MEDICAL FACTS AND MCQ'S

CTEV : congenital talipes equino varus ( club foot )

2011-09-17T11:55:00.001-07:00

INTRODUCTION :

Clubfoot can be classified as (1) postural or positional or (2) fixed or rigid. Postural or positional clubfeet are not true clubfeet. Fixed or rigid clubfeet are either flexible (ie, correctable without surgery) or resistant (ie, require surgical release, though this is not entirely true according to the Ponseti experience^1,^2,³).
The Pirani, Goldner, Di Miglio, Hospital for Joint Diseases (HJD), and Walker classifications have been published, but no classification system is universally used.^4,^{5, 6}

(See also the Medscape article Success of nonsurgical treatment of clubfoot deformity and the eMedicine Radiology article Clubfoot.)

History of the Procedure

In the past, clubfoot surgery was performed in a way that did not differentiate severity. The same procedure was performed for all patients. Bensahel has proposed a more individualized approach (ie, addressing only the structures that are required are released). The surgery is tailored to the deformity. For example, if the forefoot is well corrected and externally rotated, if there is no cavus, but if there is still significant equinus, a posterior approach alone should suffice (see Intraoperative details).⁷

Problem

See Relevant Anatomy.

Frequency

The incidence of clubfoot is approximately 1 case per 1000 live births in the

United States

. The incidence differs among ethnicities. For example, it is close to 75 cases per 1000 live births in the Polynesian islands, particularly in

Tonga

.
The male-to-female ratio is 2:1. Bilateral involvement is found in 30-50% of cases. There is a 10% chance of a subsequent child being affected if the parents already have a child with a clubfoot.

Etiology

The true etiology of congenital clubfoot is unknown. Most infants who have clubfoot have no identifiable genetic, syndromal, or extrinsic cause.
Extrinsic associations include teratogenic agents (eg, sodium aminopterin), oligohydramnios, and congenital constriction rings. Genetic associations include mendelian inheritance (eg, diastrophic dwarfism; autosomal recessive pattern of clubfoot inheritance).
Cytogenetic abnormalities (eg, congenital talipes equinovarus [CTEV]) can be seen in syndromes involving chromosomal deletion. It has been proposed that idiopathic CTEV in otherwise healthy infants is the result of a multifactorial system of inheritance.⁸ Evidence for this is as follows:

Incidence in the general population is 1 per 1000 live births.
Incidence in first-degree relations is approximately 2%.
Incidence in second-degree relations is approximately 0.6%.
If one monozygotic twin has a CTEV, the second twin has only a 32% chance of having a CTEV.

Pathophysiology

Theories of the pathogenesis of clubfeet are as follows:

Arrest of fetal development in the fibular stage
Defective cartilaginous anlage of the talus
Neurogenic factors: Histochemical abnormalities have been found in posteromedial and peroneal muscle groups of patients with clubfeet. This is postulated to be due to innervation changes in intrauterine life secondary to a neurologic event, such as a stroke leading to mild hemiparesis or paraparesis. This is further supported by a 35% incidence of varus and equinovarus deformity in spina bifida.
Retracting fibrosis (or myofibrosis) secondary to increased fibrous tissue in muscles and ligaments: In fetal and cadaveric studies, Ponseti also found the collagen in all of the ligamentous and tendinous structures (except the Achilles tendon), and it was very loosely crimped and could be stretched. The Achilles tendon, on the other hand, was made up of tightly crimped collagen and was resistant to stretching. Zimny et al found myoblasts in medial fascia on electron microscopy and postulated that they cause medial contracture.^{1, 2, 9}
Anomalous tendon insertions: Inclan proposed that anomalous tendon insertions result in clubfeet.¹⁰ However, other studies have not supported this. It is more likely that the distorted clubfoot anatomy can make it appear that tendon insertions are anomalous.
Seasonal variations: Robertson noted seasonal variations to be a factor in his epidemiologic studies in developing countries.¹¹ This coincided with a similar variation in the incidence of poliomyelitis in the children in the community. Clubfoot was therefore proposed to be a sequela of a prenatal poliolike condition. This theory is further supported by motor neuron changes in the anterior horn in the spinal cord of these babies.

Clinical

Seek a detailed family history of clubfoot or neuromuscular disorders, and perform a general examination to identify any other abnormalities. Examine the feet with the child prone, with the plantar aspect of the feet visualized, and supine to evaluate internal rotation and varus. If the child can stand, determine if the foot is plantigrade, if the heel is bearing weight, and if it is in varus, valgus, or neutral.
Similar deformities are seen with myelomeningocele and arthrogryposis. Therefore, always examine for these associated conditions. The ankle is in equinus, and the foot is supinated (varus) and adducted (a normal infant foot usually can be dorsiflexed and everted, so that the foot touches the anterior tibia). Dorsiflexion beyond 90° is not possible.
The navicular is displaced medially, as is the cuboid. Contractures of the medial plantar soft tissues are present. Not only is the calcaneus in a position of equinus but also the anterior aspect is rotated medially and the posterior aspect laterally.
The heel is small and empty. The heel feels soft to the touch (akin to the feel of the cheeks). As the treatment progresses, it fills in and develops a firmer feel (akin to the feel of the nose or of the chin).
The talar neck is easily palpable in the sinus tarsi as it is uncovered laterally. Normally, this is covered by the navicular, and the talar body is in the mortise. The medial malleolus is difficult to palpate and is often in contact with the navicular. The normal navicular-malleolar interval is diminished.
The hindfoot is supinated, but the foot is often in a position of pronation relative to the hindfoot. The first ray often drops to create a position of cavus. The Ponseti method of closed management of clubfeet through manipulations and casting describes the elevation of the first metatarsal as a first step, even if it means seemingly exacerbating the supination of the foot.
The tibia often has internal torsion. This assumes special importance in the casting management of clubfeet, where care should be taken to rotate the feet into abduction, avoiding spurious tibial rotation through the knee.
Even following correction, the foot often remains short and the calf thin.

INDICATIONS OF SURGERY :

Traditionally, surgery is indicated when a plateau has been reached in nonoperative treatment. Surgery is usually performed when the child is of sufficient size to enable anatomy to be recognized.

RELEVANT ANATOMY :

Bone

Tibia: Slight shortening is possible.
Fibula: Shortening is common.
Talus: In equinus in the ankle mortise, with the body of the talus being in external rotation, the body of the talus is extruded anterolaterally and is uncovered and can be palpated. The neck of the talus is medially deviated and plantar flexed. All relationships of the talus to the surrounding bones are abnormal.
Os calcis: Medial rotation and an equinus and adduction deformity are present.
Navicular: The navicular is medially subluxated over the talar head.
Cuboid: The cuboid is medially subluxated over the calcaneal head.
Forefoot: The forefoot is adducted and supinated; severe cases also have cavus with a dropped first metatarsal.

Muscle

Atrophy of the leg muscles, especially in the peroneal group, is seen in clubfeet.
The number of fibers in the muscles is normal, but the fibers are smaller in size.
The triceps surae, tibialis posterior, flexor digitorum longus (FDL), and flexor hallucis longus (FHL) are contracted.
The calf is of a smaller size and remains so throughout life, even following successful long-lasting correction of the feet.

Tendon sheaths: Thickening frequently is present, especially of the tibialis posterior and peroneal sheaths.
Joint capsules: Contractures of the posterior ankle capsule, subtalar capsule, and talonavicular and calcaneocuboid joint capsules commonly are seen.
Ligaments: Contractures are seen in the calcaneofibular, talofibular, (ankle) deltoid, long and short plantar, spring, and bifurcate ligaments.
Fascia: The plantar fascial contracture contributes to the cavus, as does contracture of fascial planes in the foot.

CONTRAINDICATIONS TO SURGERY :

No specific contraindications to surgery exist, although the child's size dictates that surgery is best performed at approximately age 6 months. With greater acceptance of the Ponseti conservative technique, surgery is seen to be a contentious issue. Surgery for clubfeet is no longer the only standard of care.

WORK UP :

Imaging Studies

Imaging studies generally are not required to understand the nature or the severity of the deformity. Radiographs, however, are a useful baseline prior to and following surgical correction of the feet, closed Achilles tenotomy, or a limited posterior release. Radiographs show the true gain in foot (ankle) dorsiflexion and confirm the appearance of an iatrogenic rockerbottom foot should one result. Occasionally, radiographs are necessary to diagnose clubfeet associated with tibial hemimelias.
Radiographs

Talocalcaneal parallelism is the radiographic feature of clubfeet. Simulated weight-bearing x-rays are used for infants who have not commenced walking. Positioning for foot x-rays is very important. The anteroposterior (AP) view is taken with the foot in 30° of plantar flexion and the tube at 30° from vertical. The lateral view is taken with the foot in 30° of plantar flexion.
AP and lateral views also can be taken in full dorsiflexion and plantar flexion. This is especially important when measuring the total amount of dorsiflexion achieved at the end of treatment, as well as the relative position of the talus and calcaneum.
Measure the talocalcaneal angle in the AP and lateral films. AP lines are drawn through the center of the long axis of the talus (parallel to the medial border) and through the long axis of the calcaneum (parallel to the lateral border), and they usually subtend an angle of 25-40°. Any angle less than 20° is considered abnormal.
The AP talocalcaneal lines are almost parallel in clubfeet. As the feet correct with casting or surgery, the calcaneus rotates externally, and the talus reciprocally also derotates to a lesser degree to give a convergent talocalcaneal angle.
Lateral lines are dawn through the midpoint of the head and body of the talus and along the bottom of the calcaneum, usually 35-50° Clubfoot ranges between 35° and negative 10°.
The lateral talocalcaneal lines are almost parallel in clubfeet. As the feet correct with casting or surgery, the calcaneum dorsiflexes relative to the talus to give a convergent talocalcaneal angle.
These 2 angles (AP and lateral) are added to derive the talocalcaneal index, which in a corrected foot should be more than 40°.
The AP and lateral talar lines normally pass through the center of the navicular and the first metatarsal.
In older children, a flat top talus can be demonstrated radiologically if the talus is sufficiently calcified, but care is required for positioning of the foot.
A lateral film with the foot held in maximal dorsiflexion is the most reliable method of diagnosing an uncorrected clubfoot, since the absence of calcaneal dorsiflexion is evidence that the calcaneus is still locked in varus angulation under the talus.

TREATMENT :

Medical therapy

Aims of medical therapy are to correct the deformity early and fully and to maintain the correction until growth stops.
Traditionally, 2 categories of clubfeet are identified, as follows:

Easy or correctable clubfeet correct with manipulation, casting, and splintage alone.
Resistant clubfeet that require surgery are those that respond poorly to splintage and relapse quickly following seemingly successful manipulative treatment. These clubfeet require early operative management. These feet are said to be associated with a thin calf and a small high heel.

The Pirani scoring system, devised by Shafiq Pirani, MD, of Vancouver, BC, consists of 6 categories, 3 each in the hindfoot and the midfoot. The categories are curvature of the lateral border (CLB) of the foot, medial crease (MC), uncovering of the lateral head of the talus (LHT), posterior crease (PC), emptiness of the heel (EH), and degree of dorsiflexion (DF). The first 3 constitute the midfoot score, and the last 3 constitute the hindfoot score.^{4, 12}
Each category is scored as 0, 0.5, or 1. The least (best) total score for all categories combined is 0, and the maximum (worst) score is 6. The Pirani scoring system can be used to identify the severity of the clubfoot and to monitor the correction.
It is necessary to provide counsel and advice to parents. They should be reassured that they are in no way responsible for the deformity and that it is unlikely to be reproduced in subsequent pregnancies.
Traditional nonoperative treatment
With traditional nonoperative treatment, splintage begins at 2-3 days after birth. The order of correction is as follows:

Forefoot adduction
Forefoot supination
Equinus

Attempts to correct equinus first may break the foot, producing a rockerbottom foot. Force must never be used. Merely bring the foot to the best position obtainable and maintain this position by either strapping every few days or by changing casting weekly until either full correction is obtained or until correction is halted by some irresistible force.
The corrected position is maintained for several months. Surgery should be used as soon as it is obvious that conservative treatment is failing (persisting deformity, rockerbottom deformity, or rapid relapse after correction has stopped).
By 6 weeks, it is usually apparent that the foot is easy or resistant; this is confirmed on x-ray due to the orientation of the bones. Reported success rates for these traditional casting methods are 11-58%.
Ponseti method
This method was developed by Ignacio Ponseti, MD, of the University of Iowa. The premise of the method is based on the cadaveric and clinical observations of Dr. Ponseti.^1,³ Steps are as follows:

The calcaneal internal rotation (adduction) and plantar flexion is the key deformity. The foot is adducted and planter-flexed at the subtalar joint, and the goal is to abduct the foot and dorsiflex it. In order to achieve correction of the clubfoot, the calcaneum should be allowed to rotate freely under the talus, which also is free to rotate in the ankle mortise. The correction takes place through the normal arc of the subtalar joint. This is achieved by placing the index finger of the operator on the medial malleolus to stabilize the leg and levering on the thumb placed on the lateral aspect head of the talus while abducting the forefoot in supination. Forcible attempts at correcting the heel varus by abducting the forefoot while applying counter pressure at the calcaneocuboid joint prevents the calcaneum from abducting and therefore everting.
Foot cavus increases when the forefoot is pronated. If cavus is present, the first step in the manipulation process is to supinate the forefoot by gently lifting the dropped first metatarsal to correct the cavus. Once the cavus is corrected, the forefoot can be abducted as outlined in step 1.
Pronation of the foot also causes the calcaneum to jam under the talus. The calcaneum cannot rotate and stays in varus. The cavus increases as outlined in step 2. This results in a bean-shaped foot. At the end of step 1, the foot is maximally abducted but never pronated.
The manipulation is carried out in the cast room, with the baby having been fed just prior to the treatment or even during the treatment. After the foot is manipulated, a long leg cast is applied to hold the correction. Initially, the short leg component is applied. The cast should be snug with minimal but adequate padding. The authors paint or spray the limb with tincture of benzoin to allow adherence of the padding to the limb. The authors prefer to apply additional padding strips along the medial and lateral borders to facilitate safe removal of the cast with a cast saw.

The cast must incorporate the toes right up to the tips but not squeeze the toes or obliterate the transverse arch. The cast is molded to contour around the heel while abducting the forefoot against counter pressure on the lateral aspect of the head of the talus. The knee is flexed to 90° for the long leg component of the cast. The parents can soak these casts for 30-45 minutes prior to removal with a plaster knife. The authors' preferred method is to use the oscillating plaster saw for cast removal. The cast is bivalved and removed. The cast then is reconstituted by coapting the 2 halves. This allows for monitoring of the progress of the forefoot abduction and, in the later stages, the amount of dorsiflexion or equinus correction.

Forcible correction of the equinus (and cavus) by dorsiflexion against a tight Achilles tendon results in a spurious correction through a break in the midfoot, resulting in a rockerbottom foot. The cavus should be separately treated as outlined in step 2, and the equinus should be corrected without causing a midfoot break.

It generally takes up to 4-7 casts to achieve maximum foot abduction. The casts are changed weekly. The foot abduction (correction) can be considered adequate when the thigh-foot axis is 60°

After maximal foot abduction is obtained, most cases require a percutaneous Achilles tenotomy. This is performed in the cast room under aseptic conditions. The local area is anesthetized with a combination of a topical lignocaine preparation (eg, EMLA cream) and minimal local infiltration of lidocaine. The tenotomy is performed through a stab incision with a round tip (#6400) Beaver blade. The wound is closed with a single absorbable suture or with adhesive strips.

The final cast is applied with the foot in maximum dorsiflexion, and the foot is held in the cast for 2-3 weeks.

Following the manipulation and casting phase, the feet are fitted with open-toed straight-laced shoes attached to a Dennis Brown bar. The affected foot is abducted (externally rotated) to 70° with the unaffected foot set at 45° of abduction. The shoes also have a heel counter bumper to prevent the heel from slipping out of the shoe. The shoes are worn for 23 hours a day for 3 months and are worn at night and during naps for up to 3 years.
In 10-30% of cases, a tibialis anterior tendon transfer to the lateral cuneiform is performed when the child is approximately 3 years of age. This gives lasting correction of the forefoot, preventing metatarsus adductus and foot inversion. This procedure is indicated in a child aged 2-2.5 years with dynamic supination of the foot. Prior to surgery, cast the foot in a long leg cast for a few weeks to regain the correction.

Surgical therapy

Surgery is indicated if nonoperative treatment has not been successful.

Preoperative details

The operating room is kept warm, and a general anesthetic is used. The usual position is supine with the foot resting over the contralateral leg in a figure-of-four position. Some surgeons prefer the lateral decubitus or even a prone position. A tourniquet generally is used, and the surgery is performed using optical loupe magnification.

Intraoperative details

Incision
Options for incisions include the following:

Cincinnati: This is a transverse incision that extends from the anteromedial (region of navicular-cuneiform joint) to the anterolateral (just distal and medial to the sinus tarsi) aspect of the foot and over the back of the ankle at the level of the tibiotalar joint.¹³
Turco curvilineal medial or posteromedial incision: This incision can lead to wound breakdowns, especially at the corner of the vertical and medial limbs.^{14, 13} To avoid this, some surgeons prefer the following options:

Three separate incisions - A posterior vertical, a medial, and a lateral
Two separate incisions - Curvilinear medial and posterolateral

Any approach should be able to address the release in all quadrants, which are as follows:

Plantar: Plantar fascia, abductor hallucis, flexor digitorum brevis, long and short plantar ligaments
Medial: Medial structures, tendon sheaths, talonavicular and subtalar release, tibialis posterior, FHL, and FDL lengthening
Posterior: Ankle and subtalar capsulotomy, especially releasing post talofibular and tibiofibular ligaments and the calcaneofibular ligaments
Lateral: Lateral structures, peroneal sheath, calcaneocuboid joint, and completion of talonavicular and subtalar release

Surgical clubfoot release
In the past, clubfoot surgery was performed in a way that did not differentiate severity. The same procedure was performed for all patients. Bensahel proposed a more individualized approach (ie, addressing only the structures that are required are released). The surgery is tailored to the deformity. For example, if the forefoot is well corrected and externally rotated, if there is no cavus, but if there is still significant equinus, a posterior approach alone should suffice.^{15, 16,}⁷
Any approach should afford adequate exposure. Structures to be released or lengthened are the following:

Achilles tendon (ETA)
Tendon sheaths of the muscles crossing the subtalar joint
Posterior ankle capsule and deltoid ligament
Inferior tibiofibular ligament
Fibulocalcaneal ligament
Capsules of the talonavicular and subtalar joints
Division of associated ligaments around the subtalar joint
Plantar fascia and intrinsic muscles

The longitudinal axis of the talus and calcaneum should be separated by about 20° in the lateral projection, and the calcaneal angle should be a right angle to the shaft of the tibia.
The correction is held with wires at the talocalcaneal joint, talonavicular joint, or both, possibly with a plaster cast. The wound should never be forcibly closed. It can be left open to granulate and heal by secondary intention or even grafted using split-thickness skin grafts.
Surgical treatment should take into account the age of the patient.

In children younger than 5 years, correction can be achieved with soft-tissue procedures.
Children older than 5 years require bony reshaping (eg, dorsolateral wedge excision of the calcaneocuboid joint [Dillwyn Evans procedure] or osteotomy of the calcaneum to correct varus).
Lateral wedge tarsectomy or triple fusion (arthrodesis) is required if the child is older than 10 years (salvage procedures).

Posterior release steps, in brief, are as follows:

Longitudinal incision
Z-lengthened tendocalcaneus
Identify neurovascular (NV) bundle
Tendon sheaths of FHL, FDL, and tibialis posterior released; tendons not elongated
Ankle joint capsule opened; talofibular, calcaneofibular, and seep portion of deltoid ligaments released (blunt dissection)
Release of distal tibial and fibula ligaments
Posterior release of the subtalar joint
With foot held just above neutral, tendocalcaneum is repaired and skin closed
Plaster-of-Paris cast in corrected position for 4 weeks, followed by splints until maturity

A posteromedial release is performed as follows (Turco procedure^14,¹³):

Make a medial incision 8-9 cm long from the base of the first metatarsal to the tendocalcaneum, curving it just inferior to the medial malleolus without undermining skin. Mobilize and expose the tendons of the tibialis posterior, FDL, FHL, tendo Achilles, and posterior neurovascular bundle.
Continuing the incision in the sheaths of FDL and FHL, divide the master knot of Henry beneath the navicular. Divide the spring ligament, detaching it from the sustentaculum tali and the origin of the abductor hallucis. Release the remaining contractures starting posteriorly. Lengthen the tendo Achilles (Z-plasty), detaching the medial half of the tendon insertion. Retract the neurovascular bundle and FHL anteriorly to expose the posterior aspect of the ankle and subtalar joints. Then incise the posterior capsule of the ankle joint under direct visualization and the posterior talofibular ligament if required at this time. Divide the subtalar capsule and calcaneofibular ligament.
Retract the neurovascular bundle posteriorly, and divide the tibiocalcaneal part of the deltoid ligament. Lengthen the tibialis posterior if it is contracted. Open the talonavicular joint and divide its capsule but avoid damaging the articular surface. Then release the subtalar ligaments and reduce the navicular onto the head of the talus, which should properly align the other tarsal bones. Ensure that the relationship of the talus to the calcaneus and navicular is correct, and stabilize the foot with Kirschner wires. The first wire is passed from the dorsum of the foot across the first metatarsal shaft, the medial cuneiform, the navicular, and into the talus. A second wire fixes the subtalar joint, and this should maintain the foot in the corrected position.
Apply an above-the-knee plaster-of-Paris cast, which is changed at 3 weeks and maintained to 6 weeks. The foot is initially held in slight equinus if there is tension on the skin closure, which is corrected at the time of cast change. Splintage is continued for at least 4 months after surgery, and night splints are used for several years.

The Ilizarov correction^{17, 18, 19} is as follows:

Ilizarov correction is used for recurrent clubfeet, especially in conditions such as arthrogryposis.
The calcaneum is held with 2 opposing olive-tipped wires.
The distractor force, in the form of heel-pushing distractors, must be posteriorly directed to prevent anterior subluxation of the talus in the ankle mortise.

Postoperative details

Pay meticulous attention to the wound after surgery. If the skin closure is difficult, it is better to leave the wound open and allow it to granulate for a delayed primary or secondary closure or allow it to heal by granulation tissue. Skin grafts also can be used to cover the defect. The plaster splint should be only lightly applied, and the wound should be inspected regularly.

Follow-up

The transfixion pins usually are removed in 3-6 weeks. The foot requires splintage in appropriate footwear for 6-12 months.

COMPLICATIONS :

Infection (rare)
Wound breakdown: Release skin retractors every 30 minutes, and release the tourniquet before closing the wound and applying a cast.
Stiffness and restricted range of motion: Early stiffness correlates with a poor result.
Avascular necrosis of the talus: A 40% incidence of avascular necrosis of the talus occurs with combined simultaneous medial and lateral release.
Persistent intoeing: This is quite common. Persistent intoeing is not due to tibial intorsion but rather is due to insufficient external rotation correction of the subtalar joint.

Overcorrection is associated with the following:

Release of the interosseous ligament of the subtalar joint
Excess lateral displacement of the navicular on the talus
Overlengthening of tendon units

For residual deformity following the initial surgery, rule out neurologic causes of recurrence (eg, tethered cord). Residual deformity may be one of the following:

Dynamic: With residual adductus and supination that are fully correctable passively, treat with split anterior tibial tendon transfer.
Fixed: If scarring is not excessive and the patient is younger than 5 years, repeat the release. If the patient is older than 5 years, a bony procedure to straighten the lateral border of the foot is required.

Options to correct adductus are as follows:

Metatarsal osteotomy: This operation creates a secondary deformity to compensate for a primary deformity in the tarsus. It rarely is indicated.
Cuboid decancellation
Calcaneocuboid fusion: The Dillwyn Evans procedure consists of a medial and post release together with excision and fusion of the calcaneocuboid joint.²⁰ It should be reserved for patients older than 4 years. These 2 procedures aim to shorten the lateral side of the foot.
Lengthening the medial side of the foot (eg, talar osteotomy): This is not widely performed. Avascular necrosis is a risk.

Options to correct residual hindfoot deformity are the following:

For heel varus: Open the medial wedge, or close the lateral wedge. Lateral displacement os calcis osteotomy is preferred.
For residual cavus and adductus: Perform a wedge tarsectomy.
For failed feet: Perform a triple arthrodesis.

OUTCOME AND PROGNOSIS

Approximately 50% of clubfeet in newborns can be corrected nonoperatively. Ponseti reports an 89% success rate using his technique (including an Achilles tenotomy). Others report success rates of 10-35%. Most series report 75-90% satisfactory results of operative treatment (appearance and function of the foot). The amount of motion in the joints of the foot and ankle correlates with the degree of patient satisfaction.^{21, 22, 23}
Satisfactory results were obtained in 81% of cases, and the range of ankle movement was a major factor in determining the functional result, which again was influenced by the degree of talar dome flattening (suggesting that the primary bone deformity present at birth dictates the eventual result of treatment). Forty-four percent of patients had no dorsiflexion beyond neutral, and 38% of patients required further surgery (nearly two thirds of these were bony procedures).
Recurrence rates of deformity were reported at around 25%, with a range of 10-50%. Menelaus reported a 38% recurrence rate.^{24, 25}
The best results were obtained with children older than 3-4 months with a foot large enough to perform the surgery without compromise (longer than 8 cm, as specified by Simons^{26, 27}). The age at operation is directly related to the result. Less than satisfactory results may be associated with overcorrection, which occurs in approximately 15% of cases.
Previous surgery seems to have a deleterious effect on the result.
FUTURE AND CONTROVERSIES :

As small infants with operated clubfeet have grown into heavy adults, they have been prone to painful stiff feet, despite good correction.

Deitz and Cooper published a 30-year follow-up study of patients treated with the Ponseti method.²⁸ These cases had comparatively pain-free supple feet. The Ponseti method is gaining mainstream acceptance as evidenced by the emergence of Ponseti clubfeet centers at major teaching hospitals across the United States.
Of the patients who have been monitored long term, those who are heavy and those in jobs involving long periods on their feet (especially performing manual labor) were found to be more likely to have painful feet. This correlated with the trend seen in the general population at large.

Gustilo's classification of open fractures

2011-09-17T05:43:00.001-07:00

Gustilo Classification
I	Low energy, wound less than 1 cm
II	Wound greater than 1 cm with moderate soft tissue damage
III	High energy wound greater than 1 cm with extensive soft tissue damage
	IIIA	Adequate soft tissue cover
	IIIB	Inadequate soft tissue cover
	IIIC	Associated with arterial injury

Enneking staging of bone tumors

2011-09-17T05:42:00.001-07:00

Enneking System for Staging Benign and Malignant Musculoskeletal Tumors

BENIGN

Latent

Active

Aggressive

MALIGNANT

Stage	Grade	Site	Metastases
IA	Low	Intracompartmental	None
IB	Low	Extracompartmental	None
IIA	High	Intracompartmental	None
IIB	High	Extracompartmental	None
III	Any	Any	Regional or distant metastases

Mangled Extremity Severity Score

2011-09-17T05:41:00.000-07:00

*LIMB SALVAGE Vs AMPUTATION :

*To predict which limbs will be salvageable after trauma, available scoring systems include the predictive salvage index, the limb injury score, the limb salvage index, the mangled extremity syndrome index, and the mangled extremity severity score. Of these, the Mangled extremity severity score was found to be most useful.

*This system, which is easy to apply, grades the injury on the basis of the energy that caused the injury, limb ischemia, shock, and the patient's age.

*The system was subjected to retrospective and prospective studies, with a score of 6 or less consistent with a salvageable limb. With a score of 7 or greater, amputation was the eventual result.

*Although we do not strictly follow these guidelines in all patients, we do calculate and document a mangled extremity severity score in the chart whenever we are considering primary amputation versus a complicated limb salvage.

*Mangled Extremity Severity Score

Type	Characteristics	Injuries	Points
1	Low energy	Stab wounds, simple closed fractures, small-caliber gunshot wounds	1
2	Medium energy	Open or multiple-level fractures, dislocations, moderate crush injuries	2
3	High energy	Shotgun blast (close range), high-velocity gunshot wounds	3
4	Massive crush	Logging, railroad, oil rig accidents	4
Shock Group
1	Normotensive hemodynamics	BP stable in field and in OR	0
2	Transiently hypotensive	BP unstable in field but responsive to intravenous fluids	1
3	Prolonged hypotension	Systolic BP <90 mm Hg in field and responsive to intravenous fluid only in OR	2
Ischemia Group
1	None	Pulsatile limb without signs of ischemia	0^[*]
2	Mild	Diminished pulses without signs of ischemia	1^[*]
3	Moderate	No pulse by Doppler, sluggish capillary refill, paresthesia, diminished motor activity	2^[*]
4	Advanced	Pulseless, cool, paralyzed, and numb without capillary refill	3^[*]
Age Group
1	<30 y		0
2	>30 – <50 y		1
3	>50 y		2

BP, blood pressure; OR, operating room.

^*	Points × 2 if ischemic time exceeds 6 hours

Differences between Gout and Pseudogout

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GOUT	PSEUDOGOUT
1. Smaller joints	1. Large joints
2. Intense pain	2. Moderate pain
3. Joint inflamed	3. Joint swollen
4. Hyperuricemia	4. Chondrocalcinosis
5. Uric acid crystals	5. Calcium pyrophosphate crystals
6. Rod shaped crystals	6. Rectangular shaped crystals
7. Negatively bisfringent	7. Positive bisfringence

Causes of Pseudoarthrosis

2011-09-17T05:34:00.001-07:00

*Pseudoarthrosis is a false joint, that may develop after a fracture that has not united properly.

*It may be idiopathic.

*Causes of Pseudoarthrosis (in decreasing order of frequency are) :
1. Non union of a fracture - including pathological fracture.
2. Congenital - in the middle to lower third of the tibia with or without fibula. 50% present in the first year. Later there may be cupping of the proximal bone and pointing of the distal bone end.
3. Neurofibromatosis - seen in 50% of patients with pseudoarthrosis.
4. Osteogenesis imperfecta
5. Cleidocranial dysplasia - congenitally in the femur
6. Fibrous dysplasia
7. Ankylosing spondylitis - in the fused bamboo spine .

Causes of Osteolytic and Osteoblastic metastases

2011-09-17T05:32:00.001-07:00

*Causes of Osteolytic metastasis :
- Kidney (expansile) and Thyroid : Expansile lytic osseous metastasis are characteristic of Renal cell carcinoma (kidney) and Thyroid .
- Lung
- Gastrointestinal tract (GIT)
- Breast (occasionally)
- Less commonly melanoma, carcinoma of bronchus and pheochromocytoma may also present with Expansile lytic lesions.

*Causes of Osteoblastic metastasis :
- Prostate
- Breast (may be mixed)
- Seminoma
- Carcinoid
- Neurogenic tumors
- Uterus - Ovary

Common sites for Common Bone tumors

2011-09-17T05:31:00.001-07:00

*EPIPHYSEAL TUMORS :
- Chondroblastoma
- Osteoclastoma (Giant cell)

*METAPHYSEAL TUMORS :
- Osteochondroma
- Osteoblastoma
- Simple bone cyst
- Aneurysmal bone cyst
- Osteosarcoma
- Chondromyxoid fibroma
- Fibrous cortical defect
- Non ossifying fibroma
- Enchondroma

*DIAPHYSEAL TUMORS :
- Ewing's sarcoma
- Multiple myeloma
- Eosinophilic granuloma (Langerhans cell histiocytosis)
- Osteoid osteoma
- Adamantinoma
- Enchondroma

Pulsating Tumors of the Bone

2011-09-17T05:30:00.001-07:00

*Primary tumors that may present as pulsating lesions :
- Telengiectatic Osteogenic sarcoma
- Angioendothelioma/Angiosarcoma of bone
- Aneurysmal bone cyst
- Giant cell tumor (rarely)

*Secondaries/Metastases that may present as pulsating lesions :
- Metastasis from Renal cell carcinoma
- Metastasis from Thyroid carcinoma

Markers of Bone formation and Bone resorption

2011-09-17T05:17:00.001-07:00

*MARKERS OF BONE FORMATION :
- Serum Bone specific Alkaline phosphatase
- Serum Osteocalcin
- Serum propeptide or type I procollagen

*MARKERS OF BONE RESORPTION :
- Urine and Serum N-telopeptide
- Urine and Serum C-telopeptide
- Urine total free deoxypyridinoline
- Urine Hydroxyproline
- Serum tartarate resistant Acid phosphatase
- Serum Bone Sialoprotein
- Urine hydroxylysine glycosides

Congenital Dislocation of Hip ( CDH or DDH)

2011-09-17T04:46:00.000-07:00

*Synonyms: CDH, Developmental dysplasia of the hip, DDH, dysplasia of the hip

*Developmental dysplasia of the hip (DDH) was formerly referred to as congenital dislocation of hip. DDH is now the preferred term to reflect that DDH is an ongoing developmental process, which is variable in presentation and not always detectable at birth. Developmental dysplasia of the hip refers to a range of developmental hip disorders, from a hip that is mildly dysplastic, concentrically located, and stable, to one that is severely dysplastic and dislocated.

*There is a suggestion that the national screening programme in the UK, that has operated since 1969, has not resulted in any less operations for late disease. Even the use of ultrasound has failed to improve the situation. It may be that the use of more experienced clinicians in the screening programme, rather than very junior doctors, produces better results.

*Epidemiology
-The prevalence of developmental dysplasia of the hip varies with age and method of assessment. In unscreened populations the median prevalence of persistent and clinically diagnosed hip dysplasia is estimated to be 1·3 per 1,000 births.
-The prevalence of neonatal clinical hip instability detected through the Ortolani and Barlow manoeuvres is higher, ranging from 1·6 to 28·5 per 1,000.1
-The left hip is dislocated more often than the right. This is thought to be due to the common position of the baby's left hip against the mother's sacrum, restricting movement.
-It is more common in cultures that use swaddling of babies, forcing the hips into extension and adduction.
-The pictures below show SWADDLING.

*Risk factors:
-There are racial differences. In the USA it is found that native Americans and Laplanders have a high incidence whilst Chinese and African Americans have a low incidence.
-Positive family history of the condition increases the risk in a manner suggestive of multifactorial inheritance.
-About 80 to 85% are female.
-About 60% of cases are first-born, compared with about 40% of all babies.
-Breech presentation increases the risk of DDH. The extended breech position in which knees are extended is a higher risk than with knees flexed. Elective caesarean section reduces the risk compared with vaginal breech delivery but it remains higher than after a cephalic presentation.
-Restriction of movement as with oligohydramnios increases the risk.
-It is commoner with neuromuscular disorders, such as cerebral palsy, meningomyelocele and arthrogryposis.

*Examination :
-The Ortolani and Barlow tests are the most common clinical tests for newborn babies.
-In the Ortolani test, the examiner applies forward pressure to each femoral head in turn, in an attempt to move a posteriorly dislocated femoral head forwards into the acetabulum. Palpable movement suggests that the hip is dislocated or subluxed, but reducible.
-In the Barlow test, backward pressure is applied to the head of each femur in turn, and a subluxable hip is suspected on the basis of palpable partial or complete displacement.
-A clunk feels like turning a light switch on or off. A click is much lighter and probably of no significance.
-Some units insist that the neonatal test should be performed only by an experienced person, usually an orthopaedic surgeon or a paediatrician, and not repeated by others less skilled for fear that numerous tests, perhaps with a little more force than necessary, may encourage instability of the joints.
-An early sign is limitation of hip abduction.

*Children between 1 and 2 months
-True dislocation has occurred and the Ortolani test is used.
-The thigh is flexed and abducted and the femoral head is lifted anteriorly into the acetabulum. Reduction will be felt as a clunk rather than heard as a click. This procedure is impossible after 2 months because soft tissue contractures develop.

*3 to 6 months
-The physical signs are rather different and so are the requirements of examination.
-If the hip is dislocated it is in a fixed position.
-The Galeazzi sign:
o The child is examined lying supine with the hips and knees flexed.
o A positive sign is that one leg appears shorter than the other.
o This is usually due to dislocation of the hip but any discrepancy of limb length will produce a positive sign.
-Other physical signs for late dislocation include asymmetry of the gluteal thigh or labral skin folds, decreased abduction on the affected side, standing or walking with external rotation of the affected leg, and leg length inequality.

*Bilateral dislocation of the hip:
-Can be quite difficult to diagnose, especially after the neonatal period.
-There is often a waddling gait with hyperlordosis.
-The Galeazzi sign for hip shortening is often absent as are asymmetrical thigh and skin folds, or asymmetrically decreased abduction.
-Careful examination is needed with a high level of suspicion.

*Investigations :
-Dynamic ultrasound is used to assess hip stability and acetabular development in infants.
-It is the technique of choice and has even been suggested as a universal screening procedure.
-In older children, x-rays of the pelvis are required.
-Arthrography, CT and MRI may also be needed.

*Management
-Bracing is first-line treatment in children younger than six months. Surgery is an option for children in whom nonoperative treatment has failed and in children diagnosed after six months of age.
-It is important to diagnose developmental dysplasia of the hip early to improve treatment results and to decrease the risk of complications.
-However early detection and treatment does not entirely avoid the need for subsequent surgery, and surgery is needed by up to 5% of infants treated with abduction splinting.
-The indications for the various procedures and the most effective management interventions remain controversial.

*At birth
-It is usually sufficient to maintain the unstable hip in a position of flexion and abduction for one or two months.
-This keeps the femoral head in the correct position and allows time for the ligamentous structures to tighten as well as bones to grow normally.
-The Pavlik harness, Frejka splint and other orthoses are kept in place until the hip is clinically stable with normal ultrasound or X-ray measurements. Controversially, double or triple nappies are often used in newborns for 2 or 3 weeks until splints and harnesses will fit properly.

*Between 1 and 6 months :
-A true dislocation may have occurred, so the aim of treatment is to reduce the femoral head into the acetabulum.
-The Pavlik harness is used to put the hip in the normal position by flexing it at least 90° and preferably 100 to 110°, with relatively full but gentle abduction of 50 to 70°.
-Spontaneous reduction of the femoral head usually occurs within 3 or 4 weeks and the harness is continued until X-ray measurements are normal. This is usually 3 months for dislocation and 2 months for subluxation.
-If this does not occur, closed surgical reduction is attempted with 1 to 3 weeks of preliminary skin traction to place the femoral head opposite the acetabulum, percutaneous adductor tenotomy, closed reduction and a hip spica cast.

*Between 6 months and 2 years :
-Surgical closed reduction may work but, if there is significant instability, an open reduction may be necessary.
-Open reduction is required for children above 2 years of age. A hip spica cast is worn for 6 weeks with a gradual return to full activities.
-Pelvic osteotomies and femoral shortening may be required.

*Complications :
-Surgery can result in a number of complications including re-dislocation, stiffness, blood loss and avascular necrosis of the capital femoral epiphysis (which occurs in 5 to 15% cases).
-Avascular necrosis of the capital femoral epiphysis is caused by compression of cartilage when reducing the femoral head under pressure, which may result in occlusion of intra-articular and extraosseous epiphyseal blood vessels causing infarction.
-Although it revascularises, there may be abnormal growth and development. -Extreme abduction, especially combined with extension and internal rotation, results in a higher rate of avascular necrosis.

Milestones of Prenatal development

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1^st week of gestation : fertilization and implantation , beginning of embryonic period.

2^nd week of gestation : endoderm and ectoderm appear ( bilaminar embryo ).

3^rd week of gestation : first missed menstrual period, mesoderm appears ( trilaminar embryo ), somites begin to form.

4^th week of gestation : neural folds fuse, folding of embryo into human like shape, arm and leg buds appear , crown-rump length is 4 to 5 mm .

5^th week of gestation : lens placodes,primitive mouth,digital rays on hands.

6^th week of gestation : primitive nose , philtrum ,primary palate , crown-rump length 21 – 23 mm

7^th week of gestation : eyelids begin

8^th week of gestation : ovaries and testes distinguishable

9^th week of gestation : fetal period begins, crown-rump length is 5 cms, weight is 9 grams .

10^th week of gestation : external genitalia distinguishable

20^th week of gestation : usual lower limit of viability ,weight is 460 grams , length is 19 cms

28^th week of gestation : eyes open , fetus turns head down , weight is 1000 grams

38^th week of gestation : term.

Nadas criteria

2011-09-17T04:41:00.001-07:00

The assessment of a child for the presence or absence of heart disease can be done with the help of some guidelines suggested by nadas and are called "nadas criteria" . the criteria are divided into major and minor criteria. Presence of one major and two minor criteria are essential for indicating the presence of heart disease.

The major criteria are :

1. systolic murmur grade 3 or more specially with a thrill
2. diastolic murmur
3. cyanosis
4. congestive cardiac failure

The minor criteria are :

1. systolic murmur less than grade 3 in intensity
2. abnormal second heart sound ( S2)
3. abnormal ECG
4. abnormal X-ray
5. abnormal blood pressure

Juvenile Myoclonic Epilepsy ( Janz syndrome )

2011-09-17T04:38:00.003-07:00

Here are some important points about Juvenile Myoclonic Epilepsy :

1. Juvenile Myoclonic Epilepsy is otherwise called Janz syndrome .

2. It usually begins between the ages of 12 and 16 year .

3. It accounts for approximately 5 % of all the epilepsies .

4. Patients note frequent myoclonic jerks on awakening , making hair combing and tooth-brushing difficult .

5. As the myoclonus tends to abate later in the morning, most patients do not seek medical advice at this stage and some deny the episodes .

6. A few years later, early morning generalized tonic-clonic seizures develop in association with the myoclonus.

7. The EEG shows a 4-6/sec irregular spike and wave pattern, which is enhanced by photic stimulation .

8. The neurologic examination is normal.

9. Majority of the patients respond dramatically to valproate, which is required lifelong .

10. Discontinuance of the drug causes a high rate of recurrence of seizures .

Live and Killed vaccines

2011-09-17T04:35:00.001-07:00

*All the live attenuated vaccines are contraindicated in pregnancy.

Treatment of Vesicoureteral reflux in children

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Grade I	Reflux into a non-dilated ureter
Grade II	Reflux into the upper collecting system without dilatation
Grade III	Reflux into dilater ureter and/or blunting of calyceal fornices
Grade IV	Reflux into a grossly dilated ureter
Grade V	Gross dilatation of the ureter, renal pelvis and calyces: Calyces show loss of papillary impression

*TREATMENT RECOMENDATION FOR VUR diagnosed following a UTI :

GRADE	AGE(YRS)	SCARRING	INITIAL TREATMENT	FOLLOW-UP
I-II	Any	-	Antibiotic prophylaxis	-
III-IV	0-5	-	Antibiotic prophylaxis	Surgery
III-IV	6-10	-	U/L: Antibiotic prophylaxis	Surgery
III-IV	6-10	-	B/L: Surgery
V	less than 1	-	Antibiotic prophylaxis	Surgery
V	1-5	No	U/L: Antibiotic prophylaxis	Surgery
V	1-5	No	B/L: Surgery
V	1-5	Yes	Surgery
V	6-0	-	Surgery

Differential diagnosis of Neonatal Jaundice

2011-09-17T04:31:00.001-07:00

A. Jaundice consisting of either direct or indirect bilirubin, that is present at birth or appears with in the first 24 hours of life causes :
*Erythroblastosis fetalis (high direct bilirubin - in infants who were given intrauterine transfusions)
*Concealed hemorrhage
*Sepsis
*Congenital infections like syphilis, CMV, rubella and toxoplasmosis
*Jaundice secondary to extensive eccymosis or blood extravasation may occur during the first day or later, especially in premature infants

B. Jaundice which first appears on the second or third day causes :
*Physiological jaundice
*Familial non-hemolytic icterus (Crigler-Najjar syndrome)
*Early onset breast feeding jaundice

C.Jaundice appearing after the third and within 1 week causes :
*Bacterial sepsis
*Urinary tract infection
*Infections like syphilis, toxoplasmosis, CMV or enterovirus

D. Jaundice first recongnised after the first week of life causes :
*Breast milk jaundice
*Septicemia
*Congenital atresia or paucity of the bile ducts
*Hepatitis
*Galactosemia
*Hypothyroidism
*Cystic fibrosis
*Congenital hemolytic anemia crises related to red cell morphology and enzyme deficiencies

E. Persistant jaundice during the first month of life causes :
*Hyperalimentation associated cholestasis
*Hepatitis
*Cytomegalic inclusion disease
*Syphilis
*Toxoplasmosis
*Familial non-hemolytic icterus
*Congenital atresia of the bile ducts
*Galactosemia
*Inspissated bile syndrome following hemolytic disease of newborn
*Rarely physiologic jaundice may be prolonged for several weeks, as in infants with hypothyroidism or pyloric stenosis.

P.S: Polycythemia may also lead to early jaundice.

Features of Prematurity

2011-09-17T04:23:00.000-07:00

*Premature babies are those babies who are born before 37 completed weeks.

*Physical criteria of prematurity :
- Skin texture : Shiny gelatinous, thin, plethoric skin.
- Lanugo : abundant.
- Plantar creases : Single deep crease over anterior 1/3rd of sole or no deep creases. Sole may be full of superficial creases.
- Genitals : Males : Both testes are at external inguinal ring or above. Empty scrotum with scanty rugosities.
- Genitals : Females : Labia majora are widely separated with labia minora fully exposed with hypertrophied clitoris.
- Breast nodules : Lesser than 5 mm and nipple is small or absent.
- Ear cartilage : Cartilage is deficient or absent with poor recoil.

- Hair : Brownish-black, fuzzy or woolly.

*Neurological criteria :
- Posture : Hypotonic, arms and legs extended.
- Arm recoil : No recoil or returns to incomplete flexion.
- Popliteal angle : Usually greater than 90 degrees. (120-180 degrees).
- Scarf sign : Elbow usually crosses the midline of chest. (video below)
- Square window : Degree of flexion at wrist limited.
- Head lag
- Various neonatal reflexes absent or incomplete

Vascular ring and Double aortic arch

2011-09-17T04:14:00.000-07:00

What is vascular ring ?

vascular ring is a malformation of the aortic arch that results in vascular branches or major blood vessels encircling the trachea and esophagus. In the double aortic arch (one of the most common vascular ring malformation patterns)It is most often due to persistence of the double aortic arch "right-sided aortic arch is present" after the second month of fetal life.

The two arches surround the esophagus and trachea which, if sufficiently constrictive, may cause breathing or swallowing difficulties.

In other vascular ring malformations, vascular remnants such as the ligamentum arteriosum (formerly the ductus arteriosus) may be part of the ring encircling the trachea and esophagus.

The esophagus and trachea are both compressed by the vascular ring made by malformation of the brachial arch vessels.

Systemic Sclerosis

2011-09-13T09:36:00.000-07:00

Systemic sclerosis is an autoimmune connective tissue disorder. Other similar diseases include SLE, RA, Sjogren’s syndrome and mixed connective tissue disease. There is a lot of overlap in the symptoms of these diseases. Many will require immunosuppressive therapy.

- You should always consider connective tissue diseases in ill patients with multisystem involvement, when there is no infection present.

- Also remember that, even though the common presentations are discussed below, connective tissue disorders can present with strange symptoms, that can be just about anything!

Systemic sclerosis is sometimes referred to as CREST. This is a mnemonic you can use to remember some of the main symptoms of the disorder:

- C – Calcinosis – calcium deposits, usually seen in the fingers

- R – Raynaud’s phenomenon

- E – Esophogeal Dysmotility

- S – Scleodactyly – thickening of the skin

- T – Telangiectasia – red spots on the skin

You might also here systemic sclerosis referred to ‘scleroderma’.

There are two main types of systemic sclerosis:

- Limited cutaneous scleroderma – aka scleroderma – in this variation, the signs are mostly confined to the hands, arms and face – i.e. mostly to the skin. In 80% there is also pulmonary hypertension.

o 5 year survival is >90%

o 10 year survival is >75%

o Generally only those with pulmonary involvement with have life threatening illness

o Usually skin changes on the upper limb are distal to the elbow.

- Diffuse cutaneous scleroderma – aka systemic sclerosis – tends to be more rapidly progressing and severe. Affects larger areas of the skin, and there is multi-systemic involvement. Can be life-threatening, e.g. if the heart/lungs/liver/kidneys become involved.

o 5 year survival is 70%

o 10 year survival is 55%

o Skin changes can occur anywhere, and in advanced cases, may cover the whole body!

o Patches typically appear on the trunk

Epidemiology / Aetiology

- 4x as common in women

- Prevalence is about 1 per 1000

- Peak incidence is between 30-50

- Children sometimes affected in localised patches

Pathology

- The disease is the result of vascular damage within the skin and organs.

- Organ damage is usually the result of fibrosis.

- Renal and pulmonary complications are the most life-threatening

- In normal disease progression, there can be some element of disease regression. This might include periods, perhaps a few weeks long, where the patient says their symptoms feel much less severe – although they are usually still apparent.

Clinical features

- Sclerodactyly – thickening of the skin

- Skin pigmentation changes – commonly a loss of pigment around the affected areas, but there may also be patches of hyperpigmentation.

- Calcinosis of the fingertips – calcium deposits at the fingertips. You may see these at little pits near the finger tips (digital pitting), and they are often visible as white dots around the distal phalanges on radiograph.

- Reynaud’s phenomenon – the result of vascular spasms that reduce the blood supply to the fingers, usually when the hands get cold. The phenomenon may also be triggered by emotional stress. There is a classic pattern of colour change – the fingers will go white, then blue, then as they warm up, or the episode passes, they will become red. The red part of the cycle is the result of hyperaemia that occurs after a period of reduced blood flow. The episodes are often painful.

o Sometimes also occurs in the tongue, toes, nose and ears.

o Occurs in 4-30% of all women. Rarer in men

o Can be divided into primary and secondary disease. In primary disease, it is often symmetrical, and will usually not result in any complications (e.g. ulceration, tissue necrosis, gangrene).

o Secondary disease is what we see when it is related to connective tissue disease. This is often asymmetrical, and extremely painful. There may be associated ulceration, tissue necrosis, and gangrene.

§ Smoking is a big risk factor!

o It is important to remember when asking about this in the history that everybody’s fingers will turn white/blue if they are cold for long enough! You need to find out if they think these changes are abnormal, and often there may be a clear horizontal line across the fingers where the colour changes appear.

o It commonly occurs in association with connective tissue disorders, but it can be idiopathic, and occur in isolation, or in association with other disorders, or as the result of medications (e.g. β-blockers). It also occurs in pregnancy.

o Treatment

§ Avoid sudden changes in temperature / low temperature. This might involve wearing gloves in moderate weather

§ Stop smoking

§ Try medications

· Calcium channel blockers

· Angiotensin-II agonsits

· SSRI’s may also be useful

- Oesophageal Complications

- Telangactasia

- Pulmonary complications

- Reduced movements of the fingers/wrists – due to the swelling and skin thickening involved in the disease

General features of connective tissue disease

- Mouth ulcers

- Dry eyes

- Dry mouth

- Hair loss – mostly associated with SLE – but remember there is a lot of overlap! If symptoms of more than one connective tissue disorder are present, we would probably call it mixed connective tissue disease.

- Hand, foot and leg ulcers – particularly when Reynaud’s phenomenon is present.

If you are a bit stuck when taking a history, going through a systems review is likely to yield lots of useful information in a patient with connective tissue disease

Investigations

- Anti-centromere antibodies – associated with limited cutaneous scleroderma. Foud in 70% of cases.

- Anti-Scl-antibodies – associated with Diffuse cutaneous scleroderma. Found in 40% of cases.

Treatment

Organ complications are managed individually. Treatment for the organ involvement has come a long way in the last 20 years, and has dramatically improved survival. However, scleroderma is still one of the least treatable rheumatological conditions.

Specific organ treatments, e.g.

- Renal crisis – ACE inhibitors often used

- Oesophageal involvement – PPI’s widely used

- Pulmonary Hypertension – usually treated with calcium channel blockers. However, in many patients this is not sufficient. Other drugs can be used IV, e.g. prostacyclin.

o In very severe cases, patients may be considered for lung transplant.

- Interstitial lung disease – occurs in many patients, and restrictive lung disease will occur in about 20% of patients, and many of these will die as a result. This may be treated with low dose long term corticosteroid therapy (e.g. 7mg daily of prednisolone), and can also be treated with cyclophosphamide. However, it is often resistant to treatment.

You should also monitor annually, e.g. with:

- ECG

- Spirometry

Immunosuppressive therapy

This may be used in those with more aggressive disease. This may involve therapies such as IV cyclophosphamide during flare-ups, to try and induce remission.

Mixed Connective Tissue Disease

This often presents with a combination of symptoms from:

- Systemic sclerosis

- SLE

- Polymyositis

Some clinicians still argue over whether it is actually a separate diagnosis

Fractures

2011-09-13T09:35:00.000-07:00

General Info

- fractures can involve part of, or all of the bone cortex

- Open fracture – the skin or overlying mucous membrane is breached

- Closed – there is no damage to the overlying skin or mucous membrane

Types of Fracture

Presentation

- Fractures are always Painful!

- Often involve trauma (except for pathological fractures)

- Tender

- Often swollen

- Mobile at fracture site

- Loss of limb function

Investigations

See also the article on Bone Radiology

- X-ray – the investigation of choice.

o All suspected fractures should be x-rayed from 2 planes! – AP, and lateral. This allows you to judge the level of displacement, comminution, angulation and rotation.

- Bone scans – if you can’t see a fracture on X-ray, but you still highly suspect one, then you might do a bone scan. There is increased Tc uptake at sites of fracture, so fractures will appear dark on bone scan.

o This effect is less apparent in older patients due to reduced osteoblastic responses to the scan. In such patients, you might have to wait one week between giving the radioactive isotope and performing the scan.

o Particularly useful for femoral neck and sacral injuries in the elderly, and carpal tunnel injuries in the young.

- CT – useful if you can’t see anything on x-ray. Make sure the scan is perpendicular to the fracture line!

o CT is also very good at detecting new bone formation, and so can detect subtle stress fractures.

- MRI – once again, useful if x-ray looks normal. MRI’s can be especially good, as a T1 scan can show the fracture immediately, (bone scans and CT can take a while), whilst T2 are good for showing up older fractures.

General Complications of Fracture

Early Complications

- DVT and PE – possibly the most common post fracture complication. Take preventative measures:

o Stockings

o Leg pumps

o LMW heparin (in selected patients!)

· Compartment Syndrome – full article

- Vascular injury

o May lead to avascular necrosis - this usually occurrs in the hip, but can occur at the end of any long bones, sometimes without associated fracture! Tends to occur within 48 hours of fracture. Needs to be treated with a total hip replacement (THR). X-ray will be normal in the early stages, but in the late stages, affected bone areas will be darker than normal bone, due to collapse of subchondral bone.

o Generally rare, but well recognised

o Usually associated with open, high energy fractures.

o Signs include; cold pulseless limb, ischaemia, paralysis and parasthesia of the limb

§ If there is ischaemia, you should presume there is arterial disruption until proven otherwise.

§ Angiography confirms the diagnosis

§ Treated with surgery to revascularise the limb. Also, often just closing an open fracture can help perfusion.

· You may use a temporary vascular shunt to prevent damage whilst more permanent surgery is performed

· The limb should be fixated before you operate on the vessels

· Nerve Injury – particularly if the fracture affects the knee (common peroneal nerve). Look for foot drop and loss of sensation on the dorsum of the foot. The majority of nerve injuries are traction neuropraxia – i.e. the nerve remains intact, but is temporarily unable to transmit nerve impulses. These will subsequently recover. Prognosis for nerve injury is generally good, but many patients might not make a complete recovery

§ It is important to make detailed notes and examination of nerve injury before surgery – as if the nerve injury is only noted after surgery, the surgeon may be liable.

- Vascular injury

Late Complications

· Mal-union – the separate areas of bone heal, but with incorrect alignment. Proper placement and reduction of the fracture at the time of injury can prevent it.

· Nonunion – the separated areas of bone do not fuse. More common in smokers. Often occurs when a fracture is ‘missed’ on x-ray and thus the correct treatment is not administered (e.g. immobilisation in a cast). There is usually some tissue development between the two fractured pieces. This can be scar tissue, or more rarely, a pseudo joint forms with cartilaginous articulating surfaces. If union has not occurred by 6 months, then it is unlikely to do so without intervention. Typically a diagnosis of non-union requires an x-ray at >6 months demonstrating non-union of the fractured ends of the bone.

· Delayed union – difficult to distinguish between delayed and non-union. X-ray at 6 months is definitive. In both instances the joint is likely to be painful throughout.

Other complications

- Infection

o Can be early or late

o Gas Gangrene

§ Infection, usually by chlostridium, that produces gas within tissues. Rare.

· Algodystrophy – (Sudeck’s Atrophy) – aka Reflex sympathetic dystrophy syndrome and Complex regional pain syndrome

· This typically occurs in the hand or foot after injury, sometimes after only mild injury. It occurs after about 5% of all trauma injury, and can also occur after frostbite and long periods of immobilisation.

· It results from injury to the sympathetic nervous system which inturn, affects the blood supply to the affected region.

· Typical features include:

o Burning pain in the affected area

o Skin changes – skin often becomes thinned and shiny

o Swelling

o excessive sweating – at the affected site

o Pain stiffness and muscle wasting may become worse with progression, as the patient is reluctant to use the affected body part.

· It can often be successfully treated with physiotherapy, although this can be a lengthy process

Hip Fractures - Neck of Femur (NoF)

- The most common type of fracture

- Typically occur in elderly female patients

o Also common in high impact trauma – e.g. car accident

o Female:Male ratio is 4:1

o Mean age is 75

o Dementia or cognitive impairment present in 30% of cases

- The majority will require corrective surgery

- Mortality – 20-35% in the first year

o 80% of mortality is in women

Types

- Extracapsular – fractures that occur outside the joint capsule

o Do not affect the blood supply to the femoral head

o Typically occur in well vascularised bone, and thus complications of bone union are rare

o Often described as stable, ort unstable. Unstable fractures are generally those where there is detatched fragment of lesser trochanter.

- Intracapsular – fractures that occur inside the joint capsule

o Can affect the blood supply to the femoral head, especially if the fracture is displaced.

o Complications of fracture union are common

Presentation

- History of fall / trauma

- Leg may be shortened and externally rotated in displaced fractures.

- 10% will have a fracture at another site, usually proximal humerus, or distal radius.

- Neurovascular injury is rare…

o But avascular necrosis is more common here than at other sites. Often treated with total hip replacement.

o The sciatic nerve is at risk in dislocation fractures, and dislocations (15% of patients).

Treatment

Nearly all will be given surgery, unless there are significant CI’s.

- Intracapsular fractures:

o Undisplaced – up to 15% will displace without treatment, and thus the usual treatment is internal fixation using a dynamic hip screw.

§ 5% will have avascular necrosis – these cases will need arthroplasty (hip replacement)

o Displaced – can be treated by reduction and fixation, but there is a high risk of non-union, and fixation failure. 15% will have avascular necrosis .40% of patients treated in this way will require arthroplasty at some point in the future. Thus, many surgeons opt for hemiarthroplasty as the first line. In hemiarthroplast, the femoral head but not the actebular cup is replaced. (In a total hip replacmenet both the head and the cup are replaced, but this is typically reserved for osteoarthritis).

§ Reduction and fixation more likely to be carried out in younger patients – as long as there is no underlying pathology (e.g. steroids causing osteoporosis) that caused the initial fracture.

- Extracapsular fractures

o Internal fixation is the treatment of choice for inter-trachanteric fractures. as the blood supply to the femoral head is not in danger. Unlike intracapsular fixation, a sliding screw and plate fixation (aka Dynamic Hip Screw) is used. AN intra-medually hip screw is used for sub-trochanteric fractures. Other methods may be used for fractures that extend a long way down the femoral shaft. Complications include:

§ Failure of fixation (5%)

§ Non-Union – (1-2%)

§ Infection (5%)

*Dynamic Hip Screw*	Intra-Medually Screw

Prognosis

This is generally poor, irrespective of the fracture location. This tends to be a reflection of the general health of the patient’s involved (elderly women), and not an indication of the surgery itself.

- 1 year mortality – 30%

- 1 year mortality in those with dementia – 50%

- Only 70% of patients will return home. The rest will require residential care for the rest of their lives

Upper Limb Fractures and Dislocations

Dislocated Shoulder

- Patient will support affected arm with other arm

- Dislocation usually occur anteriorly – thus traumatic injury is often from behind

- Should may appear ‘flattened’

- Palpation of the joint will reveal displaced greater tuberosity, and unusual bulge below the clavicle.

- May be anaesthesia over the attachment of deltoid (axillary nerve)

- Treatment

o Can try to relocate the joint manually

o Will often spontaneously resolve in a few weeks

Fracture of shaft of humerus

- Common traumatic injury in the elderly

- Extensive bruising of the upper arm

- Wrist drop common – due to involvement of the radial nerve which runes in the spiral groove of the humerus. In closed injuries, the nerve damage is usually reversible (traction neuropraxia)

- Treatment – as long as the injury is closed, then splitting of the wrist and a cast over the break is usually sufficient to allow for healing

Suprecondylar fracture of the humerus

- Only really seen in childhood, but it is a common fracture for children

- The humerus fractures just above the condyles, and the distal fragment can be pushed and twisted. This poses a risk to the brachial artery, which if damaged, can result in severe limb ischaemia.

o You should always check the radial pulse, which checks the integrity of the brachial artery!

Fracture of the Radial Head

- Often caused by falling onto an outstretched hand

- Can be difficult to see on x-ray, particularly if it is not displaced

- Typical signs include:

o Displacement of the fat pad

o Effusion of the elbow

o Decreased range of elbow movement – particularly extension – which can last for several months

Colle’s Fracture – distal radial fracture

- Results from falling onto dorsiflexed (outstretch) hands, with resultant posterior displacement of the wrist. The term colle's fracture however is however sometimes applied to almost any fracture of the distal radius.

- Management - if undisplaced, cast alone is usually successful. slight displacement and angulation can be treated with closed reduction (manipulation of the fracture without surgery, with plenty of pain releif!). Large displacements require surgery to correct, and often there is some form of internal fixation. rarely, external fixation is also used.

- Complications -

Colle's Fracture

Smith’s Fracture – distal radial fracture

- The reverse of Colle’s – results from falling onto a palmar flexed wrist

Chauffer’s fracture – distal radial fracture

- Fracture of the styloid of the radius, usually due to compression of this against the scaphoid

- Caused by falling onto a dorsiflexed hand

- Gets its name from the fractures seen when Chauffer’s, when cranking a car’s engine would break their wrist when the engine backfired.

Scaphoid Fracture

- The scaphoid bone articulates with the distal end of the radius.

- Nearly always the result of falling onto a dorsiflexed (outstretched) hand

- Often the diagnosis is missed as it can be very difficult to see on x-ray

o May be misdiagnosed as a ‘sprained wrist’

- Patients will have a feeling of ‘fullness’ or pain in the anatomical snuffbox – the depression between the tendons of the thumb when the thumb is fully extended and abducted

- Treatment

o Immobilisation of the joint is necessary. Without this, the scaphoid may not unite properly, and there can be necrosis of the scaphoid, resulting in prolonged pain and weakness in the wrist.

Fracture of the Elbow

- Often result from falling on an outstretched arm

- Can be very difficult to see on x-ray. Look for the posterior fat pad sign which is visible as a slight dark patch behind the elbow, on the lateral view.

o Fracture may be more visible after 10 days, so consider re-x-ray

- Pain relief is important for the first couple of weeks, but it is really important to mobilise the joint early to prevent permanent loss of extension. Patients should be encouraged to use the affected arm as much as pain allows and as soon as possible.

Osteoarthritis

2011-09-13T09:32:00.000-07:00

Osteoarthritis is a condition that results from the loss of cartilage at synovial joints, and is often accompanied by degeneration of the underlying bone.

Radiologically there are:

- osteophytes

- Joint space narrowing

Epidemiology

- most common type of arthritis

- 80% people >60 will have some radiographic features

o Only ¼ of these (20% of total population >65) is symptomatic

- rare to present below the age of 55

- OA of the hip is less common in black Africans and Chinese populations than in Caucasians

- radiologic signs are more common than symptoms

- More commonly affects women (M:F - 1:3)

- The leading cause of disability in those over 55

Aetiology

- there is a genetic component. Most commonly disease with interphalangeal involvement (nodal OA), and primary generalised OA are the inherited types

- Genes that encode for ‘collagen type II’ are thought to be involved. Several have been identified, but it is thought that many genes are involved.

- Previous trauma of a joint increases the risk of having OA in this joint.

- Obesity

- Hypermobility of joints predisposes you to OA

- Osteoporosis REDUCES the risk of OA

- Occupation – e.g. involving manual labour. Miners get OA of the hip, knee and shoulder, cotton workers – in the hand, and farmers – in the hip, sports people – often get OA in the lower limb.

Pathology

- focal destruction of articular cartilage. Inflammation is usually not involved.

- There is massive variability in the bone changes. In some individuals there may be no underlying bone change, but in others there can be massive new bone formation at the costal margins.

- Bone underneath cartilage is referred to as subcostal bone.

What is cartilage

It is 75% water, and the rest is mainly collagen (type II), mixed in with chondrocytes, fibroblasts and proteoglycans. Cartilage is smooth and can absorb impact. It is constantly damaged in normal use, but this is repaired by chondrocytes. This damage and repair is a balanced process in normal function. Chondrocytes are found throughout the cartilage itself – and along with fibroblasts, are the only cells found in cartilage.

- Cartilage itself has no blood supply, and no nervous supply.Nutrients diffuse into the cartilage from the synovial fluid, and from the nearest blood supply – in the bone marrow.

- So where does the pain come from in OA?! The reasons are pretty complex, but it is probably mainly due to irritation of the bone surface after the cartilage has worn away.

- The precursor cells of chondrocytes also differentiate into osteoblasts.

- The structure of collagen means water molecules are held in place by electrostatic forces between sidechains of the collagen. This gives cartilage its ability to absorb impact. Throughout the day, the collagen becomes dehydrated, and we lose height, due to the intervertebral discs becoming thinner. During the night when we are laid flat, the collagen rehydrates.

In OA, this balance between damage and repair is lost. There is chondrocytes proliferation, and excess cartilage is produced, however, it is oedematous. This leads to focal erosions forming. This leads to rapid destruction of the cartilage, for which, the chondrocytes cannot compensate, and later in the progression of the disease the chondrocytes die, through apoptosis.

Adjacent areas of cartilage try to take over the role of repair, but this is inadequate.

Eventually, the synthesis of new matrix stops completely. The surface becomes fistulated and fibrillated.

- Fibrillations are small ridges in the articular cartilage surface.

Now the bone has lost its protection, it is exposed to extra stressors. The two bones of a joint may rub directly against eachother. There may be microfractures, and cyst formation.

The bone attempts to re-grow itself, but this process can be too great, and it results in the formation of osteophytes.

The disease is progressive. This can be gradual, or stepwise. In some very rare cases, radiological improvement has been seen. This could be the basis for future treatments.

Clinical features

Can affect most joints, and there are lots of different patterns. Disability results when knee and hip joints are affected.

- joint pain and stiffness

o Initially, the pain is intermittent, and often described as an ache. Typically it is provoked by movement of the affected joint, and relieved by resting the joint, but in later disease, there may also be pain at rest. It may be severe enough to wake the patient at night.

o Stiffness is typically worse after long periods of inactivity – e.g. first thing in the morning. However – it typically on lasts a few minutes – as opposed to the long periods of morning joint stiffness seen in RA.

- joint gelling – stiffness and pain of the joint after a period of immobility

- Joint instability

- Loss of function as a result of the above

- Synovitis can also occur

o This is inflammation of the synovial membrane – the membrane which forms the border of the joint capsule.

o Causes joint swelling, as synovial fluid volume increases

o Also lumps (nodes) may be palpable

o Painful, especially on joint movement

o More common in RA than OA. Can also occur in other joint disorders (e.g. gout).

o Treatment depends on the cause, but when associated with OA, may just be treated with NSAID’s

Signs

- Joint line tenderness

- Limited range of movements

- Joint crepitus on movement

- Bony swelling

- Joint effusion with may or may not include inflammation

- Muscle wasting

- Limitation of range of movement

- There may also be occasional joint effusions

- Audible joint crepitus

- Nodes

o Heberden’s nodes – seen at the DIP joints

§ To help remember this you can remember ‘Outer Hebrides’ - to remember that Heberden’s Nodes are distal

o Bouchard’s Nodes – seen at the PIP joints

- Squaring of the hand – this refers to a deformity of the CMC joint of the thumb. The joint appears more prominent than in a normal hand, thus making the base of the hand appear ‘squared’.

Classifications of OA

OA can be classified by cause, or presentation.

Cause

Primary

i.e. idiopathic – there is no underlying cause. Genetic factors likely to be involved.

Secondary

Likely to be the result of another underlying disease process or event. E.g.:

- Trauma

- Rheumatoid arthritis

- Gout

- Acromegaly

Presentation

Nodal OA

- Joints of the hand affected. Usually one joint at a time, over a period of several years

- DIP’s more than PIP’s

o If PIP’s are affected, it may mimic rheumatoid arthritis. The patient may even be positive for rheumatoid factor – but this is not diagnostic, and is of no significance.

- Commonly presents around the time of the menopause in women

- Usually bilateral

- Often occurs in painful episodes – there may be joint swelling and some inflammation (although – a typical of feature of OA – despite inflammation – CRP and ESR are usually normal!)

- Functionally, patients do not usually have any problems, although if there is severe involvement of the DIP’s, there may be reduced grip strength.

- The inflammation will gradually reduce over time (months to years), and it often leaves behind stiffness deformity. Deformity is usually in the form of painless bony nodules

o Heberden’s nodes – found at DIP

o Bouchard’s nodes – found at PIP

§ These nodes are the result of osteophyte formation

- On x-ray

o Joint space narrowing

o Nodes at essentially large osteophytes

Other types of Hand OA

- Often OA of the carpometocarpal joints and metocarpophalangeal joints co-exists with nodal OA. This may be painful at first, but pain declines overtime, as the joint stiffens.

- Unlikely to serious affect function

- May cause a squared hand, due to fixed adduction of the thumb.

- Polyarticular hand OA – is associated with ↑risk of OA at other sites

- in all types of hand OA:

o symptoms are episodic, and may settle after time

o rarely disabling

o family history

Hip OA

- Affects 7-25% of Caucasians. Less common in black Africans.

- There are two main types:

o Superior pole OA – joint space narrowing, sclerosis of both head of femur and acetabulum. More common in men, often presents unilaterally, but can spread to become bilateral

§ Pain is usually felt in the groin – typically on exercise. May also radiate to the buttocks, anterior thigh, knee and ever lower leg – this is why it is important to examine the joints above in your orthopaedic examination!

§ Another typical presentation is the patient is unable to reach down to tie their shoelaces/cut their toenails/put on their socks. As the disease progresses, pain in the knee becomes more common. Pain may also disturb sleep in later disease.

o Medial cartilage loss – less common. Usually in women. Associated with hand OA. Rapidly disabling. Bilateral

o Other clinical features

§ Pain on internal rotation – early disease

§ Pain on external rotation – late disease

§ Limb shortening

§ Quads and gluteal wasting and weakness

§ Reduced range of movement in all directions. Rotation movements are usually the most painful.

§ Fixed flexion deformity

§ Tenderness is rare

Knee OA

- Present in 40% of over 75’s

- More common in women

- Strongly associated with obesity

- Usually bilateral

- Strongly associated with polyarticular OA of the hand in elderly women

- Medial compartment more often affected. Leads to varus deformity.

- Risk factors:

o Previous trauma

o Meniscal and ligament damage

- Symptoms:

o Knee pain

o Pain on walking – particularly upstairs

o Antalgic gait – a limb adopted to avoid pain. Will have short stance phase on one side usually.

o Difficulty getting out of chairs

o Joint line tenderness

o Joint line bony swelling

o Crepitus

o Quads wasting

o Varus deformity (valgus possibly but rare)

o Fixed flexion deformities

Primary generalised OA aka nodal generalised OA

- Less common than nodal OA, but often co-exists with it

- More common in women

- Strong familial association

- Onset is often sudden and severe

- Associated with immune complex deposition – may have an autoimmune cause.

- Affects:

o Knees

o Hip

o Intervertebral joints

o Hands

Erosive OA

- Rare

- Affects DIP’s and PIP’s in a similar presentation to nodal OA

- Functional outcome is poor (unlike nodal OA)

- Can develop in RA – some believe it is not truly OA

Crystal Associated OA aka crystal arthropathy, crystal arthritis.

- Usually the result of calcium pyrophosphate deposition in the cartilage. This phenomenon is known as chondrocalcinosis.

- Chondrocalcinosis is usually asymptomatic, and frequency increases with age

- Usually affects the knees and wrists

- Causes a chronic arthropathy, sometimes referred to as pseudo-OA. Usually marked cyst formation and oseteophytes of x-ray. Also, calcification of the cartilage is often seen.

- May be rapid onset and disabling. Most commonly seen in elderly women.

- Associated with pseudo-gout.

Secondary OA

- 50% of patients who have an open knee menisectomy will get OA within 21 years

- After hip dislocation fracture, average time to get OA is 7 years

Course of the disease

Joint pain and stiffness ≫ joint destruction ≫ joint replacement

The disease typically progresses slowly, although some patients can remain stable for years. Even with early intervention, up to 65% of patients will show deterioration 15 years after diagnosis.

Investigations

- X-ray – often signs may be visible on x-ray, before symptoms of pain and stiffness develop. X-rays are usually AP. Often, the correlation between radiographic features and symptoms is poor, although in larger joints, it is more reliable than smaller ones. X-rays are not very useful to distinguish types of arthritis if the history is inconclusive. To assess joint space narrowing, you need to take a weight bearing x-ray! On the left is a normal knee radiograph, on the right, a knee with OA.

- Blood tests – there aren’t really any useful tests. ESR and CRP will be negative. Rheumatoid factor and antinuclear antibodies are also usually negative, but if positive, are unhelpful. May be useful to exclude other differentials.

- MRI – can show bone and cartilage changes. Not widely used for OA at the moment, but may be able to detect early cartilage changes in the future.

- Arthroscopy – is good for looking at early changes of the cartilage (e.g. fissuring, surface erosion).

Treatment

- Treat the symptoms! – don’t treat the radiological findings.

o Depression often co-exists.this, along with quadriceps wasting, is the main predictor of pain.

- Education of the patient about the disease mechanisms and prognosis can reduce pain, and aid compliance

Physiotherapy and exercise

Initial treatment should be around ‘self-help’, such as exercise and life-style changes.

Physio and exercise are particularly important in lower limb OA, especially so for the knee. Increasing quadriceps strength helps knee stability.

- Physiotherapists should be used to teach patients how to exercise, and not relied upon for regular care.

- Thick, soft soled shoes can make a lot of difference to some patients.

Hydrotherapy may be useful in lower limb OA. Massage and heat & ice packs are often useful.

NSAID’s are debateable – many patients take them, but the proven benefit is marginal, if any. Have been associated with avascular necrosis and destructive arthropathy.

- You should add some sort of gastro protection (e.g. omeprazole), or use a selective COX2 NSAID, to reduce the risk of gastritis.

- Be wary of NSAID’s in patients with cardiovascular risk factors – which will be a lot of patients in this age range!

Weight reduction is helpful in those with BMI >28

In patella-femoral OA, performing a patellar tap can relieve symptoms!

Alternative treatments

TENS and acupuncture have been shown to be beneficial to some patients.

Pain relief

Is important to help function.

- First – try paracetomol. Up to 4g daily. If uncontrolled:

- Second – add codeine

- Third – if still not well controlled, consider NSAID’s – but only give in short doses.

Steroids

Despite the fact that OA is usually not inflammatory, local injections of steroids during severe exacerbations are often helpful. Do not do this more than twice a year. This is particularly useful when there is joint effusion.

Other treatments

- Hyaluronate – some recommend this for those unresponsive to intra-articular steroid injections. It is also administered by intra-articular injection. Other question the benefit

- Glucosamine and chondroitin – these agents appeared in early trials as though they may alter the long term outcome of the disease, but have subsequent trials have been ineffective.

Joint replacement

Used widely in severe disease. Only really used for hip and knee joints. very good outcomes in those whose disease is not well controlled with physical and pharmacological intervention. Also can greatly reduce functional deficits. The risk of complications is equal in both (about 1%). For the vast majority of patients pain is greatly reduced, and function greatly increased, after joint replacement.

- Don’t let the young age of a patient put you off

- In those unfit for general anaesthesia, they can be performed under spinal agents.

- Contraindications:

o Ongoing sepsis / leg ulcer

o Peripheral vascular disease

Hip replacement

- 90% of cases will be pain free after recovery

- Vast majority of cases have large functional improvement

- 75% of patients will not need a second hip replacement.

o The lifespan of a hip replacement is typically 10-20 years, although some patient have been known to have them for 30+. 90% of prosthesis will survive >15 years.

o The second replacement (revision surgery)carries greater procedural risk, and often the outcome is not as satisfactory as the first.

o Younger patients may have a metal on metal replacement – as these may have a long lifespan. These type of replacements tend to involve more of a resurfacing of the articular surface, than the large replacements seen with other types of surgery.

- Total hip replacement – ARTHROPLASTY – this is the most common form of joint replacement. About 50 000 are performed each year in the UK.

o Usually a metal ‘ball’ in a plastic ‘socket’, to reduce the friction co-efficient.

o Can be cemented, or uncemented. In the UK, nearly all are ‘cemented’. In the uncemented version, the prosthesis is held in place as a result of the elastic quality of bone, and a very tight fitting prosthetic joint!

- Partial hip replacement – the acetabulum may be left in place, and only the head of femur is replaced.

- Recovery

o Mobilise the patient ASAP. This prevents long-term stiffness and aids a full range of movement

o Average recovery time is 6/7 months, before full activities can be resumed

o Recovery can be painful, but usually pain is well controlled with medication. After a full recovery there should be little pain

- Risks

o Common:

§ DVT

· Risk minimised with stockings, pumps to aid bloodflow. You must inform the patient that there is a risk of death – but balance this by describing what you will do to reduce the risk, and reassure that it is rare (death from DVT is <0.1%, total risk of death is about 1%)

§ Infection (2% - 1% deep infection, 1% superficial infection)

· Bacteria are able to adhere to the prosthesis. This is dangerous, as they can then protect themselves from oral AB’s with a biofilm, making the infection impossible to treat in this way. The prosthesis has to be removed whilst the patient recovers; meaning 2 operations and no functioning hip joint, before they can start again!

· The risk is greatly reduced with prophylactic antibiotics. Also reduced with MRSA screening, laminal air-flow theatre. Don’t ever perform if there is an ongoing infection!

· Prophylactic AB used - commonly cephalosporins – e.g. cefazolin (first generation), or cefuroxime (

§ Altered leg length

· Patient will probably still have better function and less pain than without the op though!

§ Dislocation

· Risk is about 2%

Consent

- You must inform the patient that there is a risk of death – but balance this by describing what you will do to reduce the risk, and reassure that it is rare (roughly 1%)

- Remember – OA isn’t life threatening – but surgery can be! Although the risk is tiny, and OA can be very disabling.

- When disclosing risks – remember to lay out the alternatives – in this case – pretty much carry on as you are!

- Who can take consent –best practice if it is the surgeon who will carry out the procedure – but it doesn’t have to be. Has to be somebody who is properly qualified, competent, and understands and is able to explain the procedure.

Knee Surgery

- Tibial osteotomy – an alternative to knee replacement in suitable patients (young, physically active). In an osteotomy, bones are cut, shaped and re-aligned, to allow better functioning of the joint. This can prolong the need for knee replacement by up to 10 years.

o It is particularly useful when there is varus/valgus deformity.

o Recovery process is often longer and more difficult than knee replacement

- Knee replacement – full recovery often seen within about 4 months. Pain relief is great, but range of movement may not be improved.

- Hip and knee OA is the same leg – the hip is usually replaced first.

SLE - Systemic Lupus Erythematosus

2011-09-13T09:30:00.001-07:00

SLE (Systemic Lupus Erythematosus) is an autoimmune connective tissue disease, similar to systemic sclerosis, RA, and mixed connective tissue disease. Often, symptoms of these diseases overlap. In cases where you unable to distinguish exactly which condition is present,we would normally say that mixed connective tissue disease is present.

Like those other disorders, ANA’s (anti-nuclear antibodies) can be found in blood of many affected patients.

The four key features of Connective Tissue disease:

- Inflammation

- Fibrosis / scarring

- Vasospasm (including Reynaud’s Syndrome)

- Vascular Thrombosis

The main clinical features, and organ complications associated with connective tissue disease can often be put down to one of the above pathological mechanisms.

There are also specific types of antibody that are more readily associated with the different types of connective tissue disease.

Epidemiology

- Prevalence is roughly 1 in 4000

- About 10x more common in women!

- 2-3x more common in Indian Asians than Caucasians

- 5x more common in black Africans than in Caucasians

- Can present at any age

o Peak incidence is between 25-35

o Second peak of incidence is between 50-60

Aetiology

- Strong genetic disposition

o Family members 50x more likely to have the disease than members of the general population

- UV light exposure thought to increase the risk

- Drugs

o Chlorpromazine, hydralazine, isoniazid

Clinical features

- Photosensitive rash – can occur anywhere on the body, but typically seen on the face – as this area is most likely to be exposed to the sun!

- Nonspecific symptoms

o General malaise

o Fatigue

o Low-grade fever

o Weight loss

o Alopecia

o Mucosal ulceration – particularly in the mouth

- Vascular Changes

o Nail edge infarcts

o Splinter haemorrhages

- Arthritis

o Usually:

§ Symmetrical

§ Small joints

§ Polyarthritis

o The arthritis is non-erosive, and thus the joint is permanently damadged, but there may be ligament damage, which can cause deformities similar to rheumatoid arthritis. However, unlike in RA, the deformities are reducible, and they should not affect joint function – e.g. the patient should be able to make a fist.

- Systemic involvement

o Nephrosis – anti DNA antibodies are toxic to the kidney

§ You should always do a urine dipstick on any patient you suspect with SLE! If there is nephro damage it will show up as excess protein on dipstick! – there will also possibly excess blood.

o Nervous system involvement

Diagnosis

According to the American College of Rheumatology (ACR), for a patient to be diagnosed with SLE, they must fulfil 4 of the following 11 criteria:

- Malar Rash

- Oral ulceration

- Discoid Rash – a round or oval rash seen on areas of skin exposed to sunlight

- Arthritis

- Photosensitivity

- Serositis

- Neurological disorders

o Seizures

o Psychosis

- Renal disorder

o Proteinuria (>0.5g/day)

o Red cell casts in urine

- Haematological disorder

o Leucopenia

o Lymphopenia

o Haemolyitc anaemia

o Thrombocytopaenia

- Immunological disorder

o Antibodies to double stranded DNA

o Anti-Sm antibodies

- ANA positive

Investigations

- Urine dipstick – increased protein, red cell casts

- Blood – anaemia, thrombocytopenia, leukopenia, lymphpaenia, raised ESR and CRP.

- ANA testing – positive in 90% of patients. Concentration of antibodies does not reflect the severity of the disease.

- Antibodies to doublestranded DNA (dsDNA)– highly specific for SLE – but only present in 60% of cases. In some patients, the concentration of the antibodies may reflect the severity of the underlying disease

Complications

- Increased incidence of atherosclerotic disease

- Increased risk of thrombosis

- Increased risk of infection

o Usually due to immunosuppressive treatment of the disorder

Management

Mild disease

No specific therapies may be needed. The patient may be able to use topical NSAID therapy on the affected areas, and make lifestyle changes (E.g. avoiding the sun), and may otherwise need no further therapy

Mild to moderate disease

Treatment is essentially similar to RA. Patients may use disease modifying drugs (e.g. methotrexate, leflunomide, azathiaprin, hydroxychlorequine), and use steroid injections for specific flare ups.

Organ involvement

Treat the organ complications as separate entities with specific treatments

Neurological disorders

Also treated with specific therapies, e.g. anticonvulsants

Rheumatoid Arthritis

2011-09-13T09:29:00.001-07:00

RA is a type of inflammatory arthritis. Other examples include seronegative spondyarthritides, reactive arthritis, lyme arthritis, crystal arthritis and postviral arthritis.

Features of inflammatory arthritis

- Pain and stiffness worse in the morning and after rest

o Early morning pain and stiffness may last several hours (in OA, duration is much shorter)

- Inflammatory markers (ESR, CRP) usually raised

- Often accompanied by normochromic, normocytic anaemia

Rheumatoid Arthritis

This is a chronic symmetrical arthritis. When we say it is symmetrical, we don’t necessarily mean a mirror image, just that the same joints are affected on both sides of the body. it is also important to remember that RA is a systemic condition, with many extra-articular manifestations.

Typically it affects the peripheral joints, and there is inflammation of the joint (synovitis). Deformity is common and the course is extremely variable.

Epidemiology and Aetiology

- affects 0.5-3% of the population worldwide

- can present at any age (from childhood to old age), but the peak incidence is between 30-50 years

- women affected more than men (M:F – 1:2)

o before the menopause, risk is 3x higher for women

o after the menopause it is equal

§ suggests sex hormones involved in some way

§ the contraceptive pill can delay the onset, but does not reduce the risk

- Genetic factors are involved. Certain HLA variants are implicated, especially in severe forms of the disease:

o HLA-DR4 – occurs in 50-75% of patients, and is associated with a particularly poor prognosis

o HLA-DR1 is another variant associated with RA, and poor prognosis

- Environmental factors:

o Smoking

o Stress

o Infection

Clinical features

Most commonly, the condition will present as progressive over weeks to months. These patients have the worse prognosis. But in some cases it can come on in days, or even overnight. Also, it is almost always a polyarthritis, but some cases do present as monoarthritis, most commonly of the knee or shoulder, or with carpal tunnel syndrome.

- symmetrical swollen distal joints

o Often warm and tender joints

o sometimes presents as a sudden onset of widespread arthritis, but this is rare.

o Typically the joint of the hand (MCP, DIP and PIP’s)and the distal metatarsals of the foot.

§ Sometimes it affects the wrists, elbows, shoulders, knees and ankles.

§ Hips are very rarely affected

o Limitation of movement

o Muscle wasting

- Pain and stiffness – worse in the morning, may improve with activity. It is often described as an ache type pain.

- Disturbed sleep

- Nodules – in the early and mild stages of the disease, there are relatively few inflammatory cells in the joints. As the disease progresses, these increase in number and there may be nodular masses of inflammatory cells within the joint. Rheumatoid nodules occur when these inflammatory cells form similar inflammatory structures outside of the joint capsules. The nodules are usually pink/red and have a rubbery texture. They are painless. You should always check the elbows in a hand exam, looking for rheumatoid nodules!

- Osteoporosis – often occurs in the bones immediately around the affected joints, particularly in the fingers. This may be the first sign of RA.

- Secondary Osteoarthritis

- Deformity – as the joint capsule is destroyed, and the articular surface damaged, deformity occurs. Specific examples include

o Hands

§ Swan necking – the fingers become hyperextended at the PIP, and flexed at the DIP

§ Z-thumb

§ Subluxation of the MCP –not that this is not swelling!

§ Muscle wasting – “guttering” – ‘gutters’ seen between the extensor tendons on the back of the hand.

· Why do the muscles waste so quickly in joint disease? – in a normal individual, if you don’t use a muscle, it will waste at a rate of about 1% of its mass/day. However, in joint disease, the rate of wasting in much greater. This is because in joint disease, there is inhibition of nerve afferents, for nerves that innervate the muscles around a joint. This alters the muscle tone/reflex feedback loop, leading to decreased innervation of the muscle, and as a result, wasting occurs very quickly.

§ Inflamed flexor tendon sheaths – these serious impair function.

§ Carpal tunnel syndrome is common

§ Ulnar deviation – the fingers point towards the ulnar side

§ Fixed flexion deformity – aka buttonhole or boutonniere deformity

o Shoulders - Shoulders are commonly affected, and at first it may mimic rotator cuff tendonitis. Later, the joint becomes stiffened. Rotator cuff tears can occur late on.

o Elbows – less commonly affected. Flexion may be lost, which makes eating very difficult

o Knees – massive synovitis and effusion. These respond well to steroid injection and aspiration. A persistent effusion may increase the risk of cyst formation, and these can rupture. Varus or valgus deformity can occur, and there may be joint space narrowing and secondary OA.

§ Knee replacement can restore much of the function, and relieve pain.

o Cervical spine – pain in the neck is more commonly muscular, but you can get joint disease itself in the cervical spine. There can be bone destruction, which poses a risk to the spinal cord.

§ Be wary loss of sphincter control, or unexplained weakness in late RA – could be due to cord compression!

o Feet – often the first signs of the disease may only be in the feet. The patient may describe an uncomfortable sensation that feels like walking on marbles. This is due to subluxation of the heads of the metatarsals in the feet.

The symptoms of RA are often worse in the summer / hot weather – eg. If the patient goes on holiday.

Systemic Signs:

- Subcutaneous rheumatoid nodules

o Occur in about 1/3 of patients, usually in severe progressive cases. They most commonly occur on the extensor surfaces of the forearm, and sometimes on the dorsum of the foot. Sometimes also on the Achilles tendon.

o They have a central area of necrotic collagen, surrounded by marcophages and fibroblasts. This resembles the synovitis – but there is no synovium!

o Usually 2-4cm in diameter

o Firm

o Occur over pressure points

o Can be removed surgically, or injected with corticosteroids, and they will reduce over time if they are problematic, however, they will usually recur.

- Swollen bursae – particularly the olecranon.

- Anaemia – almost always occurs, and is usually normocytic, normochromic. Can sometimes be iron-deficiency related, due to NSAID’s.

- Lymphadenopathy - nd can

- Splenomegaly

- Sjogren’s syndrome

o Salivary glands and tear ducts are destroyed, resulting in dry eyes and dry mouth

- Vasculitis – a very poor prognostic sign. Usually occurs in the fingers. It is the result of immune complex deposition in arterial walls. Smoking greatly increases the risk. Signs to look out for include:

o Nail fold infarcts

o Necrosis of the skin

o Bowel infarction – due to arterial involvement in the bowels

- Septic arthritis – serious! – has a high mortality and morbidity. May present as a sudden onset effusion. Any sudden onset effusion needs to be aspirated! Staph. A. is the most common causatory organism. Treat with systemic AB’s and drainage.

- Amyloidosis

- Heart signs:

o Pericarditis – rare, but in 30% of seropositive patients, there is some pericardial involvement

o Endocarditis – even rarer

- Reynaud’s phenomenon

- Peripheral neuropathies – often in glove and stocking pattern. May also get mononeuritis multiplex (many nerves involved, but not in a distinct pattern). Usually sensory.

- Kidneys – nephrotic syndrome and renal failure

- Ruptured tendons

- Ruptured Baker’s cysts

- Depression – commonly occurs in RA patients

Eye Changes in Rheumatoid arthritis

The best way to remember them is to go from the outside, in.

- Dry eyes – can be managed with artificial tears.Also common in Sjogren’s syndrome – an autoimmune disorder associated with RA

- Episcleritis – inflammation of the covering of the sclera. Often causes mild discomfort and redness of the eye. Managed with steroid eye drops and anti-inflammatories. The blood vessels can look particularly prominent.

- Scleritis – inflammation of the sclera (White part of the eye). Causes constant severe eye pain. More serious than episcleritis, and often requires referral to ophthalmology. May be managed with steroid eye drops and anti-inflammatories.

o in scleritis and episcleritis, the sclera can become thin. It may appear blue due to thinning, and sometimes the contents of the eye ball can bulge out,

- Cataracts – can be caused by inflammatory processes within the eye, but also can be caused by the steroid eye drops used to treat this inflammation. Usually causes cloudy, blurred and/or dim vision. The only effective treatment is surgery for lens replacement.

- Uveitis – inflammation of the interior of the eye. Acute visual loss and pain. May affect the iris, where the boarder of the iris becomes jagged. Treatment is with steroid eye-drops and anti-inflammatories

- Glaucoma – in the case of RA, usually caused by inflammatory processes within the eye. Very dangerous, as it can lead to visual loss. May present with eye pain, gradual visual loss, blurred vision. Can be treated with eye drops, but may require surgery.

- Hydrochlorequine – can cause ‘floaters’ and retinopathy. This is usually reversible and will disappear when the drug is stopped

- Steroids – can cause an eye muscle myopathy – eye movements may be reduced. They can also impair the immune system, making patients more prone to conjunctivitis.

Pulmonary changes in RA:

- Diffuse pulmonary fibrosis – will shows as a restrictive pattern on spirometry. SOB in RA patients can be particularly serious. Often RA patients have very poor mobility, and so when they get SOB,you know it must be sever, because they are hardly doing anything to increase the O2 requirements!

o How to remember the causes of lung fiobrosis:

§ All connective tissue diseases cause lower lobe fibrosis – except AS, which causes upper lobe fibrosis

§ All occupational diseases cause upper lobe fibrosis, except asbestosis, which causes lower lobe fibrosis

§ Remember, the ‘A’ are the exception!

- Lung nodules – these are specific to RA, and are basically the same as the subcutaneous nodules, but they form in the lungs. They are generally asymptomatic, but they can cavitate.

- Pleural effusion – often recurrent

Presentations

- Palindromic – monoarticular attacks, last 24-48 hours. 50% of cases will progress to other types of RA

- Transient – lasts <12 months, then permanently remits. Usually seronegative. No lasting damage

- Remitting – may be active for several years at a time, before remitting. Lasting damage is minimal

- Chronic, persistent – the most common form. May be seronegative or seropositive. Follows a relapsing remitting course over many years. Seropositive patients have worse joint disease and higher risk of long-term disability.

- Rapidly progressive – rapid progression occurs over several years. Severe joint damage, disability and high rate of complications

Pathology

RA is a kind of autoimmune disease. We say that the patient is in a hyperimmune state. There is thought to be some T cell activity, but factors released by T cells (IL-2 and IL-4 amongst others) are not very abundant. Macrophages products and activity however, are very prominent. This means that rather than RA being a ‘true’ autoimmune disease, involving some dysfunction in the immune system, the dysfunction is on a cellular level.

In most cases, the reaction is mediated by rheumatoid factor. This is an antibody against IgG (which is itself and antibody!). Specifically, RF is an antibody against the Fc portion of the IgG molecule. Rheumatoid factor and IgG will join together to from immune complexes , which activates complement, and sets of the inflammatory process.

- Various rheumatoid factors are produced in normal individuals, and physyiologically, they are used to remove old IgG from the blood. But in RA, something goes wrong

- About 70% of cases of RA have RF’s in the blood

o Patients who have seronegative disease tend to have a baetter prognosis, and disease that is just limited to synovial manifestations

- RF’s are themselves generally IgM, but they can be IgG or IgA.

Cases of RA where rheumatoid factor is not present are referred to as seronegative RA.

Rheumatoid factor is also present in many individuals who do not have RA.

It is thought that like many other ‘autoimmune’ type disease, an infection earlier in life may be implicated.

In terms of joint disease, RA results in:

- Chronic inflammatory synovitis

- Progressive erosion of articular cartilage, which exposes the underlying bone.

- Pannus- this is essentially the inflamed synovium. It damages the cartilage by restricting its normal nutrient flow, and by released inflammatory factors.

Systemic pathology:

- Cytokines cause:

o fatigue

o atherosclerosis

o increased bone turnover rate

Investigations

The diagnosis can be made clinically. The American college of rheumatology (ACR) criteria are:

- Morning stiffness >1 hour

- > 3 joints involved

- Hands and wrist involvement

- Symmetrical

- Nodules

- Positive RF

- Radiological changes

- Symptoms present for > 6 weeks

These criteria are only really used for studies, and are not useful in early disease.

Normal initial investigations

- Blood count:

o Anaemia

o ESR/CRP raised due to inflammation. Monitoring levels of these can be used to assess treatment

- Serology – check for rheumatoid factor – only present in 70% of cases.

o ANA’s – anti-nuclear antibodies – these are also regularly tested for, and show up in 30% of cases

o Anti-CCP – testing for this is becoming more common.

- X-ray – useful to get a baseline reading at the start of the disease. Normally only soft tissue swellings initially. Later there may be:

o Boney erosions

o Osteopenia (lower than normal bone density)

- Joint aspiration – in the presence of effusion. Aspirate will appear cloudy due to the presence of white cells. If the joint is suddenly painful, always aspirate to check for septic arthritis, as this can rapidly destroy joints.

- MRI – very rarely used, but can show early bone erosions. Also used in widespread disease to assess the extent of the joint damage.

Differentials

There are loads of differentials, particularly if the symptoms are only transiently present for a few weeks (eg. Viral arthritis). If you get asked in the OSCE, the main differential is SLE!

Management

Increasingly, the aim of treatment is to minimize joint damage.

Symptomatic relief

NSAID’s are widely used for symptomatic relief. They do not alter the disease progression in any way. Other basic analgesics (including paracetomol and codeine) are usually ineffective at controlling pain, but may still be used. You should aim to use a COX-2 specific drug to reduce the risk of GI complications, although in relation to RA, the outcome of selective vs non-selective agents is identical.

- In all patients over 65, and those with previous history of GI problems, you should use gastric protection (e.g. omeprazole).

Steroids

These are very useful in inducing remission. Some studies have shown that low dose (7.5mg prednisolone) reduced both symptoms, and progression of the disease. However, you need to give something to prevent osteoporosis, and many doctors have concerns about long-term steroid use. However, steroids are used widely in RA.

- The exact dose and length of regimen that is most beneficial is still not widely accepted

- Remember to look for signs of long-term steroid use when examining for rehaumatological and arthritis conditions! – if there are any, then these patients will nearly always be RA.

- Steroids are also used in acute flare ups of the disease (typically an IM preparation 80-120mg).

They are also used acutely and injected into joints that are severely inflamed.

DMARD’s – Disease modifying anti-rheumatic drugs

Functional impairment and pathological joint damage can be reduced when these are used. The earlier in the disease course they are used, the more damage can be prevented.

DMARD therapy should be initiated ASAP after diagnosis.

Nearly all DMARD therapy takes between 6-12 weeks to take effect. They will reduce symptoms (according to ACR criteria) in 20-50% of patients.

All treatments (except hydrochlorequine) require regular blood test monitoring, and should all generally be avoided during pregnancy.

Most DMARD’s act by inhibiting cytokines. This reduces irreversible joint damage.

- Methotrexate –an antifolate drug, often used to treat cancer, but also effective in autoimmune disease. Probably the most widely used drug in RA. It generally targets cells with a high turnover rate (folic acid is needed to synthesize DNA)this is generally the first line treatment and is often given weekly (25mg dose) or daily (3mg).

o Side effects – can cause mouth ulcers, GI upset, (due to the high cell turnover rates in these areas) and possibly liver and haematological disturbances. GI Side effects can be reduced by giving folic acid supplement – folic acid should be taken once per week, and blood test for folate levels taken every two weeks.

§ Liver problems

§ Teratogenic

§ Myelosuppression

§ Rashes

§ Shouldn’t take with alcohol

o Less effective than anti-TNFs, but also, much cheaper, so NICE recommends try this first, before anti-TNF’s.

- Sulfasalazine – initially used in IBD, also effective in RA. Not quite as well tolerated as methotrexate.

o Mechanism - not really understood. Thought that it inhibits inflammatory mediators, but does not directly cause immunosuppression.

o Side effects –myelosuppression, nausea, rash, oral ulcers, decreased sperm count

- Leflonamide – often used as an alternative to sulfasalazine. Similar side effects. Contra-indicated in pregnancy.

- Hydrochlorequine – this is the least toxic of these agents, but probably also the least effective. Causes rashes and retinopathy – vision should be checked at least every 12 months.

- Gold – given IM. More toxic than sulfasalazine or methotrexate. Causes myelosuppression, renal toxicity, mouth ulcers, and photosensitivity

- Cyclosporin

- Azathiaprine

**DMARDS’s can be combined if they are not producing therapeutic benefit on their own**

- Myelosuppression – (bone marrow suppression) - can be caused by pretty much all of the DMARD’s. leads to a pancytopenia, and can be dangerous is it greatly increase susceptibility to infection. Can cause death if there is severe sepsis.

Anti-TNF-α - e.g. etanercept, infliximab, adalimumab

This is used in patients who fail to respond to DMARD’s. TNF-α is one of the crucial cytokines involved in the inflammatory response. It is effective in up to 70% of patients, and is growing in popularity as a treatment. It relieves symptoms, and reduces disease progression, like other DMARDS’s. it is very expensive! – hence on the NHS, other DMARD’s are tried first. NICE guidelines tate to use these only after 2 other DMARD’s have been unsuccessful.

- Side effects – can allow the reactivation of latent infections, such as TB – as TNF-α is involved in the maintenance of these latencies. However, these agents are actually usually well tolerated compared to other DMARDs.

o Long-term safety is not known. Some concerns about increased cancer risk, but unproven.

- Contra-indications – pregnancy / breast feeding, ongoing infection, heart failure

Exercise and physiotherapy – should always be encouraged.

Managing other risk factors:

- Cardiovascular disease risk is increased – due to atherosclerotic effects of RA

- Smoking –increases the risk of RA. Stopping smoking may be beneficial

As always, the best care is patient centred, and provided by a multidisciplinary team! – if you are asked about treatment in the OSCE, remember to say these two things!

General Prognosis

Modern treatments have greatly reduced the degree of deformity, and improved function for many patients. However, it many cases it is still very disabling. Many patients are able to cope remarkably well despite reduced function. Functional aids help (e.g. modified cutlery, kitchen tools, and modifications on taps (for turning) and stairways etc.).

There is also an increased mortality risk (About 1.5x that of the general population), due mainly to cardiovascular involvement of the disease. The risk of mortality is increased most in those with extra-articular manifestations, and in the most aggressive disease types.

Asthma

2011-09-13T09:15:00.000-07:00

What is asthma?

Asthma is a chronic lung condition in which there is chronic inflammation of the airways, and hypersensitivity of the airways. Symptoms include wheeze, cough, chest tightness and SOB (dyspnoea). It is often worse at night.

- The immune response is CD4 mediated, and the lungs will show an eosinophil infiltrate

It is characterised by airflow obstruction which is varied over time, and reversible. Technically, asthma exists where the obstruction is reversible by >15%, and COPD exists where it is reversible by <15%.

- Many patients will fall into a grey area near the boundary of these two diseases. These patients may typically be in their 30’s and early 40’s, and have a history of smoking, but as their airway obstruction is reversible, then they may technically be given a diagnosis of asthma. In actual fact, they are more likely to have early stage COPD. However, the diagnosis is not that significant because the treatment is very similar. Despite the fact COPD is ‘not reversible’, patient with COPD often get symptomatic relief from inhalers (although the only way to improve prognosis is to stop smoking, and give LTOT).

In the UK about 2000 patients per year die from asthma.

Three main characteristics

- Airflow limitation – this is usually reversible, either spontaneously, or with treatment

- Airway hyperresponsiveness – this occurs to a wide range of stimuli

- Inflammation of the bronchi – with infiltration by eosinophils, T cells and mast cells. there is associated plasma exudation, oedema, smooth muscle hypertrophy, mucous plugging and epithelial damage.

The disease often ‘flares up’ with viral infections – which often cause a loud wheeze.

Epidemiology

- Increasing in incidence, particularly in Western countries

- 10-20% of those in the 2^nd decade of life are affected. This is where prevalence is at its highest

- In children, boys are more likely to be affected

- After puberty, girls are more likely to be affected

- 50% of those who have childhood asthma, but then ‘grow out of it’, will relapse in adulthood

Aietiology

The disease can either be intrinsic (aka cryptogenic); where not causatory factor can be found, or extrinsice, where there is a definite external cause.

- Intrinsic – this often starts in middle age, and is sometimes called late onset asthma. You cannot find the trigger.

- Extrinsic – this usually occurs in atopic individuals who have positive skin prick test results. This type of asthma is present in 90% of childhood cases, and 50% of adults with chronic asthma. It is often accompanied by eczema.

o Non-atopic individuals can develop asthma in later life via sensitisation to e.g. occupational agents, aspirin, or as a result of taking β-blockers for hypertension or angina.

o Extrinsic asthma is basically a type I hypersensitivity reaction to something in the air

Atopy

This is a term used to describe people who often have allergies / asthma / hayfever, and where:

- The trait runs in families (i.e. genetic component)

o The ADAM33 geneis associated with airway hyperresponsiveness, and tissue remodelling

o The PHF11 geneis associated with increased IgE production

- The individuals often have skin reactions to common allergens

- The individuals have IgE antibody to many common allergens – these antibodies are present in 30-40% of the UK population, and there is a strong correlation between the levels of IgE and the prevelance of asthma and airway hyperresponsiveness

- The development of atopy – they hygiene hypothesis – this is a theory that states that growing up in a ‘clean’ environment in the early years of life can cause atopy. If you grow up in a ‘dirty’ enivronemnt, and are exposed to various bacterial, fungal and viral proteins, then this is thought to help ‘direct’ your immune system away from recognising inert particles as allergens. It is almost as if your immune system has to attack something, and in the lack of actual pathogens, it just goes for whatever it can find.

The allergens for asthma, are very similar to those that cause rhinitis. Rhinitis is inflammation of the mucosal lining of the upper respiratory tract, particularly affecting areas near the nose; thus causing a constant runny nose.

***it is thought that allergy to the faecal matter of dust mites is the largest cause of intrinsic asthma worldwide***

Airway hyperresponsiveness

You can test for this by asking the patient to inhale gradually increasing amounts of methacholine or histamine. This is known as a bronchial provocation test. This will induce transient airflow limitation in 20% of the population – and these are the patients that exhibit airway hyperresponsiveness.

Pathology

The clinical symptoms and pathology are the similar for all types of asthma, they types below are all intrinsic; the differences between types are:

- Non-atopic asthma

o Does not appear to be immunologically mediated – i.e. there is not T cell involvement

o Is associated with recurrent respiratory tract infection (viral)

o Skin tests are negative

o Bronchoconstriction due to airway hyperresponsiveness, not as much due to inflammation and leukocyte infiltration.

- Aspirin induced asthma

o Mechanism is unknown, but though to be due to increased leukotrienes and decreased prostaglandins, which leads to increased airway irritability.

o Aspirin inhibits COX enzymes. This reduces the production of prostaglandins. However, prostaglandin E2 controls leukotriene production – thus in some patients this allows overproduction of leukotrienes and allows for an abnormal inflammatory reaction.

o This can also occur with other NSAID’s such as ibuprofen. it is a relative contra-indication – as opposed to a absolute contra-indication (that would apply to everybody)

o Thus, when prescribing NSAID’s you should be careful! – you should still give them but be aware that 10% of asthmatics can have a reaction to them. So ask if they have had symptoms before, and tell them to stop immediately if they have symptoms.

- Occupational asthma

o Induced by hypersensitivity to an agent at work – probably a combination of types I and III hypersensitivity reactions

o There are several products that can cause asthma via an IgE independent route – for example paints, varnishes and sprays. These can directly bind to T cells (activating them) and other epithelial cells, without the need to IgE.

o If you can prove that the asthma is a result of allergy to a particular product at work, the in the UK, you are entitled to compensation; if you apply within 10 yeas of working there.

o Susceptible professions include; bakers (flour dust) , electrician, carpenter, painter, electrician, working with polyurethane.

o In places where there are asthma causing agents, about 10% of the population are likely to be affected.

o Typically the onset is 3-6 months after you start working at the place, although it can take years to develop.

o May be better at the weekend, although not always

- Food

o A high sodium, and low magnesium can precipitate attacks

o Magnesium sulphate is sometimes given in an acute attack – although the mechanism is uncertain.

Basic pathological principle

The pathology is not fully understood. The varying amounts of chronicity and airway hyperresponsiveness are related to an individuaos response to various antigens and to the amount of airway remodelling that occurs.

- Exposure to the antigen will make CD4 T cells differentiate into T helper cells (Th2 type, as opposed to Th1), and they will begin to secrete IL-4 and IL-5.

o IL-4 will cause B cells to become plasma cells and being secreting IgE.

o IL-5 will act on eosinophils and mast cells, making them reactive to the new antigen. Other factors are also released that are chemotaxic for eosinophils.

- This IgE will bind to mast cells in the mucosa. This initial exposure will not cause an allergic reaction. The IgE sits there on the mast cell surface, waiting to come into contact with the antigen again, perhaps for years

- Upon re-exposure to the antigen, the mast cells will be activated, and will degranulate. This will release inflammatory mediators.

- there are increased numbers of mast cells in both the airway secretion, and the epithelial lining of lung in asthmatics – thus increased response to any antigens.

o This causes the initial asthma attack. This is mainly the result of histamine and prostaglandin (as well as leukotrienes; particularly LTC₄) release by mast cells. This usually occurs within minutes of initial exposure to the antigen.

§ Thus mast cells are responsible for the early attack

§ Histamine – causes smooth muscle contraction, increased bronchial secretions, and increased vascular permeability

o The late phase reaction occurs several hours after the initial reaction. It is caused by the accumulation of eosinophils (and some neutrophils – but these are much more numerous in COPD) at the site.

§ The late phase reaction is a more sustained inflammation. The initial phase is more bronchoconstriction without as much underlying inflammation.

§ Bronchodilators (the β-adrenergics) are good at treating the initial phase reaction; the late phase reaction tends not to respond well.

§ Steroids (and other anti-inflammatories) are good for preventing the inflammation that causes the late phase reaction.

§ The late phase reaction is more likely in poorly controlled / chronic asthma, where there is already a reasonable aggregation of eosinophils in the mucosa.

§ In this phase, there may also be activation of platelets, which can lead to microthrombi in the lumen.

- Effects of the bronchoconstriction and inflammation on lung function:

o Distal airway hyperinflation and collapsed (and obviously reduced gaseous transfer to these regions)

o Mucus plugging of the bronchi – due to increased number of goblet cells – and these also secrete more than normal goblet cells

o Bronchial inflammation

o Curschmann’s spirals – these are bits of epithelium that have been shed, and can be seen on histology of the mucous plugs

o Charcot-Layden crystals – crystals that are formed as a result of eosinophil aggregation

o Thickening of the bronchial basement membrane – this is particularly important, and occurs via the process of remodelling (more on this below in the ADAM33 gene section). The submucosa becomes thickened, and this means that when the smooth muscle does contract, there is excessive narrowing of the airway in response to the contraction.

o Effects on the epithelium – the epithelium loses many of its columnar ciliated cells, and these are replaced with over-active mucous secreting cells. The mucosa also releases lots of inflammatory proteins. It is also likely to get damaged in the inflammatory processes, and this (along with the excess mucous production) increases the risk of infection.

o Effects on smooth muscle – the smooth muscle is hypertrophied, and also undergoes changes which make it more likely to contract, and more likely to stay contracted for longer.

The effect of cold air and exercise

These both dry out the mucosa of the lung, which leads makes the lining hyperosmolar. This causes mast cells to release histamine and prostaglandins, thus causing inflammation.

Typically, the asthma attack does not occur during exercise, but afterwards.

Atmospheric pollution

Large amounts of dust, cigarette smoke, car fumes, and other allergens can trigger asthma in some individuals. Large industrial incidents can trigger these on a large scale population.

Ozone has also been a trigger in the past

Diet

High intake of fruit and vegetables is protective, possibly due to the large amounts of ‘anti-oxidants’ they contain. Also, genetic variations affecting antioxidant production can affect the severity of the disease

The ADAM33 gene

It is thought that this gene is responsible for the release of factors by eosinophils. Some of these factors include MBP (major basic protein), ECP (eosinophil cationic protein) and other factors. These factors can cause remodelling of the epithelium, and stimulate growth of fibroblasts. This increases the amount of smooth muscle present, and also makes the smooth muscle more likely to contract in response to the release of inflammatory factors. This increases airway hyperresponsiveness.

The normal process of bronchoconstriction

Occurs in response to direct parasympathetic stimulation. Antagonism of this effect is produced by freely circulating epinephrine that acts upon β-receptors. Β- blockers (e.g. atenolol) have been known to induce attacks in asthmatic patients, because they prevent epinephrine from doing its job. β – agonists (e.g. salbutamol) are used to stimulate the same receptors that epinephrine uses (β₂) to cause bronchodilation.

Clinical features

- Wheezing attacks

- Periodic shortness of breath

- Symptoms often worse during the night

- Cough is frequent – and often misdiagnosed as bronchitis

- Nocturnal cough alone can be a presenting feature

- Some patients can have chronic symptoms

- Attacks precipitated by a very wide range of triggers

Investigations

There is no specific diagnostic test for asthma – although variability shown through twice daily measurements of PEF is probably the most useful test.

- Respiratory function tests –

o PEF – the peak expiratory flow. This is the most useful test in asthma. Patients should take two reading per day, to show the variability of the disease. In patients with suspected asthma, you should get them to take two weeks worth of measurements whilst at work, and 2 weeks whilst at home, to prove the cause of the disease. It can also show variation between exercise/rest, night/day, before/after bronchodilator

o Spirometry – you can show the presence of asthma by demonstrating 15% improvement in FEV1 or PEF following the inhalation of a bronchodilator

§ However – in some patients you still may not be able to show reversibility this way. For example, those in remission, or those with particularly severe chronic asthma.

§ There is a hand drawn picture in your old notes of the bronchus in both acute and chronic asthma – this is helpful for understanding the concept!

§ The picture will be that of obstructive disease

o Carbon monoxide transfer test – this is normal for asthma

o NO – nitrous oxide – for some unknown reason, levels of this are raised in the breath of those with asthma, compared to a ‘normal’ individual.

- Exercise tests – these are often used to diagnose asthma in children. The child should run on a treadmill for a max of 6 minutes – enough to increase the heart rate to at least 160bpm. Then test the peak flow before and after. Test every 15 minutes after, again looking for 15% difference. a negative test does not rule out asthma

- Histamine or metacholine bronchial provocation test – this indicates hyper-responsiveness – which is found in most asthmatics. It is useful for diagnosing patients whose main/only symptom is cough. The dose of the drug needed to produce a 20% drop in FEV1 is noted, and is called the PD₂₀ FEV1 (or PC₂₀ FEV1). Patients with airway hyper-responsiveness require only a very small dose to achieve this (<11μmol of metacholine)

o The test should not be performed on those with ‘brittle asthma’ or those who have an FEV1<1.5L

o This is really only ever done for research purposes – it is quite dangerous! And has to be done under very strict conditions.

- Trial of corticosteroids – this can be very useful in children at first presentation. You can trial them on e.g. 20mg (30mg for up to 2 weeks in adults) prednisolone just for several days. This initial dose is a one-off. Not only will this reduce the symptoms, but it will also mean they respond much better to bronchodilators. If they respond, they you know it is asthma, and you can also start them on the normal management plan (which is just a bronchodilators (e.g. salbutamol) to begin with.

o make sure you measure lung function immediately before and after the course of steroids

o >15% improvement in FEV1 demonstrates the presence of asthma

o If the steroids are given for 2 weeks or less, you can stop the drug without tailing off the dose

- Blood and sputum tests – you can test these for high number of esosinophils; and this may help form your diagnosis, but is not diagnostic on its own

- CXR – this will be normal, unless they are having a particularly bad exacerbation, in which case, overinflation may be present. The use of CXR is good at excluding the possibility of a pneumothorax.

o A pneumothorax can be a complication of asthma

- skin prick tests – you should perform these on all newly diagnosed asthmatics to help find a cause

- Allergen provocation tests – you may need to do this is cases of occupational asthma but otherwise it is not required

o Many patients are often concerned about food allergy as the cause – unless there are systemic symptoms this is highly unlikely.

Management

Controlling extrinsic factors

You may want t try and reduce the risk of a person coming into contact with a provocating factor. Dust mite faeces is a major cause, and so changing bedding regularly is a good way to manage this risk. Patients should also avoid taking β- blockers in any form. This is an absolute contra-indication.

- 50% of those with occupational asthma will have no problems if they are kept away from the cause. The other 50% may still continue to have symptoms, and will have bad exacerbations if they back into contact with the causing agent.

Using medications

Asthma is a chronic inflammatory condition – and should be recognised as such! Thus, don’t be scared to use anti-inflammatories – i.e. steroids

Long term treatment

Treatment approach

The main goal of treatment is to achieve maximum control of symptoms with the fewest medications. Optimal control should include assessment of a combination of the patient's symptoms and their PFTs. The goal for optimal control is to have the patient asymptomatic with normal PFTs if possible. Once a state of control is achieved, an attempt should be made to reduce the doses of medications while maintaining optimal control.

Stepwise management of asthma

Guidelines recommend asthma severity and control be viewed as a ladder in which medication can be stepped up or stepped down based on the severity of the disease and adequacy of the control.The stepwise approach is meant to assist, not replace, the clinical decision making required to meet individual patient needs.

Patients may start at any step of the ladder, and medications can be added (stepped up) if needed. Increasing use of short-acting beta agonist (SABA) or use >2 days a week for symptom relief (not prevention of exercise induced bronchospasm) generally indicates inadequate control and the need to step up treatment.

Regular assessment of patient's asthma control should be carried out with the aim of stepping down the ladder if disease has been well controlled for at least 3 months.

Education and environmental control

All patients at all steps of therapy should receive adequate patient education and should take environmental control measures.

Allergen vaccine immunotherapy

For well-controlled proven allergic asthma, treatment with allergen vaccine immunotherapy is an option. This treatment is only used with documented allergies either by skin testing or specific IgE serology and should only be given with adequate supervision to prevent adverse systemic allergic reactions.

	Step	PEFR	Treatment
1	Occasional symptoms – less frequent that daily	100% predicted	PRN bronchodilators – ‘2 puffs as required’ – which will deliver a dose of around 200μg
2	Symptoms more than 3x a week (used to be symptoms more than daily)	≤80% predicted	Low dose inhaled corticosteroid (start at 200-400μg up to 800μg), or sodium cromoglicate
3	Severe symptoms	50-80% predicted	Add long-acting β2 agonist, e.g. sertide and symbicort are combinations of long acting agonists and corticosteroids
4	Severe symptoms not controlled by high dose corticosteroids	50-80% predicted	Higher dose inhaled corticosteroid – up to 2000μg. Consider leukotriene receptor antagonist (e.g. monteleukast), or theophyline
5	Severe symptoms deteriorating	<50% predicted	Add prednisolone 40mg daily
6	Severe symptoms, deteriorating despite prednisolone	<30% predicted	Hospital admission

Step 1: Mild intermittent and exercise induced asthma

Defined as:

· Symptoms ≤2 times a week

· Asymptomatic and normal peak expiratory flow (PEF) between attacks

· Attacks are brief with varying intensity

· Night-time symptoms ≤2 times a month

· Forced expiratory flow at 1 second (FEV1) or PEF ≥80% of predicted

· PEF variability <20%.

For those patients with mild intermittent asthma or exercise-induced asthma, the use of a SABA as required is sufficient alone. All patients should have access to quick relief SABAs.

Increasing use of SABA or use >2 days a week for symptom relief (not prevention of exercise-induced bronchospasm) generally indicates inadequate control and the need to step up treatment.

Step 2: Mild persistent

Defined as:

· Symptoms >2 times a week but <1 time a day

· Exacerbations may affect activity

· Night-time symptoms >2 times a month

· FEV1 ≥80% of predicted

· PEF variability between 20% and 30%.

Low-dose inhaled corticosteroids (ICS) are added if control is not achieved with SABA as needed only. Second-line options that can be used instead of low-dose ICS are either sodium cromoglicate, leukotriene receptor antagonists (LRTA), nedocromil or theophylline.

Step 3: Moderate persistent

Defined as:

· Daily symptoms

· Use of short-acting beta agonists daily

· Attacks affect activity

· Exacerbations ≥2 times a week and may last for days

· Night-time symptoms >1 time a week

· FEV1 greater than 60% to less than 80% of predicted

· PEF variability >30%.

Step 3 therapy adds long-acting beta agonist bronchodilators (LABA) to step 2 therapy (low-dose ICS and SABA as needed), or increases low-dose ICS to medium-dose ICS. Second-line alternatives to either increasing ICS dose or adding a LABA are to combine SABA as needed and ICS with either LRTAs, theophylline or zileuton.

Step 4-6: Severe persistent

Defined as:

· Continual symptoms

· Limited physical activity

· Frequent exacerbations

· Frequent night-time symptoms

· FEV1 ≤60% of predicted

· PEF variability >60%.

Preferred combination treatment for step 4 is medium-dose ICS plus LABA plus SABA as needed. LABA can be substituted for either LRTA, theophylline or zileuton.

Step 5 changes medium-dose ICS to high-dose ICS. Immunomodulators (omalizumab) can be considered in stage 5 for patients with allergies.

Step 6 adds oral corticosteroids to existing treatments.

This should be re-assessed at regular intervals.

Self-management plan

Tell the patient what to do with their drugs under certain circumstances:

e.g. tell them when they could increase their dose of steroid – then this avoids them having to come and see the doctor as much!

Patients should not be given nebulizers to use at home – as this encourages them to use a nebulizer when in fact they need to seek further medical attention. This practice was carried out in the past, and is thought to have resulted in many unnecessary asthma related deaths in the 1960’s and 1980’s.

The role of Xanthines

Theophyline and aminophyline (which is metabolised to theophyline) have a role in asthma treatment. They may be added to the use of β2 agonists in an acute attack. Aminophyline can be given IV, and is metabolised to theophyline.

The role of methotrexate

Some studies have shown that giving 15mg of this per week can greatly reduce the amount of oral steroid needed to control severe chronic asthma. Methotrexate is a folic acid inhibitor (thus reduces the rate at which quickly dividing cells can divide)

The role of leaukotriene receptor antagonists

These block the crucial LTC₄ receptor, which contributes to much of the inflammatory response. These drugs should be considered in anyone who is not well controlled on a middle dose of corticosteroids. They are reasonably effective, but only in some individuals. A 4 week trial is recommended before commencing more long-term treatment.

They are always using in conjunction with β2 agonists, and are most often used in patients where a high dose of corticosteroid is proving ineffective.

Omalizumab

This is a drug that forms complexes with free IgE, and prevents it binding to inflammatory cells. Initial trials have been promising in both children and adults with chronic asthma.

It is a ‘recombinant humanized monoclonal antibody.

Antibiotics are very rarely required. Sometimes in an acute exacerbation, asthma patients may cough up yellow and green sputum. However, it is this colour because it contains eosinophils and bronchial cells, and not because there is bacterial infection. However, in such cases it is probable there is a viral infection – but still obviously, you should not give AB’s.

- Occasionally, Mycoplasma or Chlamydia can cause recurrent asthma. In such cases, you need to take a sputum sample to identify the organism responsible (if one is present)

Treating the emergency admission

Status asthmatics – this is asmtha that has failed to resolve within 12 hours of therapy use. The term is a bit old-fshioned, and we now use acute severe asthma to mean asthma that is not controlled by the patient’s current medication.

Features of acute severe asthma:

- Inability to complete a sentence in one breath

- RR>25

- Tachycardia >110 bpm

- PEF <50% predicted (or <50% patient’s own best)

Features of life-threatening attack:

- Silent chest

- Feeble respiratory effort

- Cyanosis

- Exhaustion

- Bradycardia

- Hypotenson

- Exhaustion/confusion/coma

- PEF <30% predicted normal or best – this is approximately 150L/min in adults

Pulsus paradoxis – on inspiration, the diastolic pressure decreases by 10mmHg. This is present in about 45% of cases – but you have other things to worry about.

Tension pneumothorax is a possible complication

1) Hospital management – ideally you should start treatment before doing investigations!

a. (Do an ABG – it may be life-threatening if:

i. CO2 > 5kPa (high)

ii. O2 <8kPa (low)

iii. Low pH)

b. Start on 100% O2 (non-rebreathing mask) with the patient sat upright in bed

c. Give 5mg salbutamol with 0.5mg ipratropium bromide via nebulizer on 100% O2

d. Give hydrocortisone 100mg IV or 50mg prednisolone orally. Give both if very unwell

e. Do CXR to exclude pneumothorax

2) If life-threatening signs are present:

a. Inform ITU and seniors

b. Give magnesium sulphate 1.2-2g IV over 20 minutes

c. Change the nebulized salbutamol every 15 minutes, or give 10mg continuously per hour. Only give more ipratropium every 4-6 hours

3) Repeat PEF every 15-30 minutes to assess the situation. If improving then gradually reduces O2 and nebs. If not, try and find senior, keep giving nebs, and consider aminophyline.

a. Check pulse oximetry – aiming for sats >92%

b. Check blood gasses every 2 hours, and definitely within 2 hours of admission

c. Record PEF’s and β-agonists when given / carried out

d. A maximum of 60mg/day of prednisolone can be given (orally)

e. You can give 200mg hydrocortisone every 4 hours for a max of 24 hours

f. Give oral prednisolone for 5 days after they are starting to recover

g. Ventilation is an option – you would have to call an anaesthetist

h. Patients have to have >75% predicted PEF for discharge

After recovery from a severe asthma attack, oral corticosteroids should be continued until there are no residual symptoms, especially at night, and the peak expiratory flow rate is at least 80% of the person's previous best. High doses of these drugs can be stopped abruptly if used for 3 weeks or less, or tapered off if they have been used for a longer period.

Brittle asthma – ‘catastrophic sudden severe asthma’

These patients are at risk from sudden death, even if their asthma is well controlled between attacks. An attack can come on very quickly within minutes.

Self-management plan

Tell the patient what to do with their drugs under certain circumstances:

e.g. tell them when they could increase their dose of steroid – then this avoids them having to come and see the doctor as much!

Steroids – don’t forget – these cause an increased risk of peptic ulcer!