Sunday, May 2, 2010

Singh index for femoral neck fractures

Singh Classification
This classification occasionally used in patients with intracapsular hip fractures is the Singh index.
- based on the degree of osteoporosis by fitting the pattern of proximal femoral trabecular lines into six separate categories. 
- It shows poor interobserver and intraobserver levels of agreement. 
-It does not correlate with bone mineral density as measured by DEXA scans. 
- It is of little practical value in modern orthopaedic clinical practice.
 FIGURE : Singh Index grades osteopenia from normal
(grade 6; all trabecular groups are visible) to definite (grade 3; thinned trabeculae with a break in the principal tensile group) to severe (grade 1; only the primary compressive trabeculae are visible, and they are reduced) based on the ordered reduction in trochanteric, tensile, and ultimately primary compressive trabeculae. The grade is determined from a true AP projection of an intact proximal femur. (Adapted from Singh M, Nagrath AR, Maini PS. Changes in trabecular pattern of the upper end of the femur as an index of osteoporosis. J Bone Joint Surg 1970;52A:457-467.)
ref: Rockwood & Green  Adult Fractures.

Monday, April 26, 2010

CONGENITAL PSEUDARTHROSIS OF THE FIBULA AND TIBIA

- Congenital pseudarthrosis is a specific type of nonunion that at birth is either present or incipient.
- Its cause is unknown
- Asoociated with Neurofibromatosis
- Congenital pseudarthrosis most commonly involves the distal half of the tibia and often that of the fibula in the same limb.
Fibula
- often precedes or accompanies congenital pseudoarthrosis in the ipsilateral tibia.
Grading
i. bowing of the fibula without pseudarthrosis,
ii. fibular pseudarthrosis without ankle deformity,
iii. fibular pseudarthrosis with ankle deformity, and
iv. fibular pseudarthrosis with latent pseudarthrosis of the tibia.
- Sometimes develops between the time of bone grafting of a pseudarthrosis of the tibia and skeletal maturity;
because the lateral malleolus becomes displaced proximally, a progressive valgus deformity of the ankle
develops.
Treatment
- Before skeletal maturity: the ankle can be stabilized by an ankle-foot orthosis.
- At maturity: any significant deformity can be treated by supramalleolar osteotomy made through essentially
normal bone, and union of the osteotomy can be expected.
- Synostosis (Langenskiöld)
o for children, to prevent this valgus deformity or halt its progression.
o a synostosis between the distal tibial and fibular metaphyses.
o securing union by bone grafting may be as difficult in the fibula as in the tibia, an operation that prevents the ankle deformity without grafting in fibular pseudarthrosis is useful .
Tibiofibular Synostosis Langenskiöld Technique:
• Make a longitudinal incision anteriorly over the distal fibula.
• Divide the fibula 1 to 2 cm proximal to the level of the distal tibial physis, and excise the cone-shaped part of the distal fibular shaft.
• In the lateral surface of the tibia, at the level of the cut surface of the fibula, and at the attachment of the interosseous membrane, make a hole as wide as the diameter of the fibula. Proximal to the hole, remove the periosteum and interosseous membrane from the tibia over an area of several square centimeters.
• From the ilium, obtain a bone graft the same width as that of the hole in the tibia and long enough to extend from the lateral surface of the fibula into the spongy bone of the tibial metaphysis.
• Insert the graft perpendicular to the long axis of the limb so that it rests on the cut surface of the fibula and extends into the slot in the tibial cortex.
• Pack spongy iliac bone in the angle between the proximal surface of the graft and the lateral surface of the tibia.
• Apply a cast from below the knee to the base of the toes.
Aftertreatment
 At 2 months, full weight bearing in the cast is allowed, and at 4 months the cast is discontinued.


Tibia
- Congenital pseudarthrosis of the tibia is rare,
- incidence – 1:250,000 live births.
- 50% to 90% association with neurofibromatosis, including skin and osseous lesions.

Classification
Boyd classified congenital pseudarthrosis of the tibia into the following six types:
Type I pseudarthrosis: with anterior bowing and a defect in the tibia present at birth. Other congenital deformities may be +nt, that affect its management.
Type II pseudarthrosis: with anterior bowing and an hourglass constriction of the tibia present at birth. Spontaneous #, or # after minor trauma, in <2 yrs age. This is the so-called high-risk tibia. The tibia is tapered, rounded, and sclerotic, and the medullary canal is obliterated. This type is the most common, is often @ with neurofibromatosis, and has the poorest prognosis. Recurrence of the # is common during the growth period, but decreases in frequency with age and generally ceases to occur after skeletal maturation.
Type III pseudarthrosis: develops in a congenital cyst, usually near the junction of the middle and distal thirds of the tibia. Anterior bowing may precede or follow the development of a fracture. Recurrence of the fracture after treatment is less common than in type II, and excellent results after only one operation have been reported to last well into adulthood.
Type IV pseudarthrosis: originates in a sclerotic segment of bone in the classic location without narrowing of the tibia. The medullary canal is partially or completely obliterated. An “insufficiency” or “stress” fracture develops in the cortex of the tibia and gradually extends through the sclerotic bone. With completion of the fracture, healing fails to occur, and the fracture widens and becomes a pseudarthrosis. The prognosis for this type generally is good, especially when it is treated before the insufficiency fracture becomes complete.
Type V pseudarthrosis of the tibia occurs with a dysplastic fibula. A pseudarthrosis of the fibula or tibia or both may develop. The prognosis is good if the lesion is confined to the fibula. If the lesion progresses to a tibial pseudarthrosis, the natural history usually resembles that of type II pseudarthrosis.
Type VI pseudarthrosis occurs as an intraosseous neurofibroma or schwannoma that results in a pseudarthrosis. This is extremely rare. The prognosis depends on the aggressiveness and treatment of the intraosseous lesion.
Treatment
- depends on the age of the patient and the type of pseudarthrosis.
- A true congenital pseudarthrosis of the tibia does not heal
- The decision must be made whether to attempt to secure union or if amputation is the treatment of choice. when treated by casting alone.
- Factors favoring amputation-
o anticipated shortening of  > 2 or 3 inches (5 to 7.5 cm),
o a history of multiple failed surgical procedures
o , and stiffness and decreased function of a limb that would be more useful after an amputation and fitting with a prosthesis
- For the tibia with a cyst in the medullary canal (type III): prophylactic curettage and autogenous iliac bone grafting are recommended. The limb is immobilized in plaster until the graft has united, and then a patellar tendon–bearing brace is worn until skeletal maturity is reached. .
- A tibia with anterior bowing and a narrow, sclerotic canal (type II or the high-risk tibia): often fractures during the first 2 years
- Established congenital pseudarthrosis of the tibia: was treated in the past by bone grafting or amputation. Osseous union probably is more difficult to obtain in this condition than in any other. Or, Early primary amputation with an appropriate prosthesis. of life. Initially, bracing may be beneficial for an anterolaterally bowed tibia with a narrow canal in which a fracture has not developed, stable and well-adjusted knee-ankle-foot orthosis with an anterior shield in the prepseudarthrotic stage delayed fracture and pseudarthrosis and allowed patients to reach an older age before undergoing surgical treatment. Its important because they found better healing rates in children > 3 years at the time of surgery than in younger children. When a fracture does occur, the treatment is surgery.
- Poor Prognostic factors: neurofibromatosis, dysplastic lesions, and multiple surgical procedures
- even when union is obtained, leg-length discrepancy and malalignment  may require surgical correction.
- age, the difficulty in obtaining union, and the anticipated residual shortening and other deformities of the tibia should union be obtained all must be considered.
- In an infant or young child, bone grafting is indicated as early as feasible.
- Although the likelihood of obtaining union increases with increasing age, especially after puberty, the longer grafting is delayed, the shorter and more poorly developed the leg will be.
- When union is obtained in a young child, weight bearing in a brace results in more normal development of the limb.
- The child's parents should be told that treatment often consists of several operations, and that even then amputation may be necessary later because of failure to obtain union.
- If grafting is indicated, but for some reason must be delayed, the limb should be braced to prevent increased angulation at the pseudarthrosis.
- In an older child, bone grafting is indicated unless shortness or other deformity of the limb is such that function would be better after amputation and fitting with prosthesis.
- The heavy cuff of tissue surrounding the bone at the pseudarthrosis, the presence of this tissue, whether congenital or a result of a fracture may decrease bone production and consequently healing. Any operation for congenital pseudarthrosis should include complete excision of this tissue.
- Bone grafting remain the mainstay of treatment for congenital pseudarthrosis of the tibia.
- Intramedullary rodding technique in congenital pseudarthrosis of the tibia; refractures may occur. Best results with intramedullary rod fixation, bone grafting, and the use of an osteostimulator.
- recent technique is the free vascularized bone graft with either fibular or iliac crest grafts.
- most effective methods  were the Ilizarov method and vascularized fibular grafting.

Goals of treatment of the biological problems:
(1) resection of the pseudarthrosis to provide stability, the basic requirement for bony consolidation;
(2) correction of length discrepancy and axial deformity;
(3) achievement of fusion; and
(4) correction of additional problems around the main deformity, such as alignment, leg-length discrepancy, and ankle valgus.
Plating and rodding
(1) failed to provide adequate stability for the pseudarthrosis to heal, and that
(2) when this kind of fixation was used, too little of the pseudarthrotic bone was resected.
(3) plating, rodding, and vascularized fibular transfer do not provide correction of shortening and valgus ankle deformity.
Ring fixator :
Advantages;
(1) provides excellent stability;
(2) allows complete resection of the pseudarthrotic area regardless of the size of the resected segment because the device allows lengthening or segmental bone transport;
(3) enables weight bearing during the whole time of treatment, which stimulates healing of bone and soft tissues;
(4) can be used to transport the fibula distally; and
(5) does not prohibit other treatment methods if this method fails.
Disadvantages :
(1) it is time-consuming,
(2) not easy to perform; and,
(3) varying degrees of complications, such as pin track infections, fracture, ankle valgus, and ankle stiffness.
- surgery should not be done in children <3 years old, and, if possible, surgery should be postponed until age 5 years.
- For established pseudarthroses, initial treatment should be intramedullary rodding and bone grafting. Vascularized fibular grafts may be indicated for pseudarthroses with gaps of more than 3 cm and for pseudarthroses in which multiple surgical procedures have failed.
The duration of immobilization and the type of cast are determined by the amount of healing noted on clinical and radiographic examinations. When healing is sufficient, the hip spica cast is discontinued, and an above-knee cast is applied. Removal of the cast and institution of progressive weight bearing usually are possible 3 to 9 months after surgery. A knee-ankle-foot orthosis or patellar tendon–bearing brace is worn until skeletal maturity is reached.
Complications
Stiffness of the Ankle and Hindfoot
A stiff ankle should be expected until the distal tip of the rod is proximal to the ankle joint after longitudinal growth of the distal end of the tibia. Even if stiffness persists, it rarely hampers functional results.
Refracture
Refracture is common in patients with pseudarthroses, despite apparently solid clinical and radiographic union.  Refracture can be managed with casting or removal and replacement of the intramedullary rod with additional bone grafting. Because of the likelihood of refracture, removal of the rod after union is not recommended until skeletal maturity has been reached.
Valgus Ankle Deformity
The distal tibial fragment must be fixed so that valgus deformity of the ankle is corrected at the time of placement of the intramedullary rod. Intraoperative fluoroscopy is useful for monitoring this procedure. Long-term bracing is mandatory during the growth years to minimize progressive valgus ankle deformity, or surgical treatment with the Langenskiöld procedure may be indicated.
Johnston, in  patients treated with intramedullary rods, found that valgus deformity was significantly more frequent when the fibula was left intact than when fibular osteotomy was done (with or without fibular fixation). In addition, the presence of fibular insufficiency (fracture or prepseudarthrotic lesion) was highly prognostic for subsequent valgus deformity, whether or not the fibula eventually healed.
Tibial Shortening
Tibial shortening should be anticipated in almost all these children. The maximal projected shortening was 4 cm. In selected patients, tibial shortening can be treated by a well-timed contralateral epiphysiodesis or limb lengthening of the proximal tibia.
Intramedullary nailing with bone grafting, with or without electrical stimulation, is recommended for an established pseudarthrosis. The vascularized graft or the Ilizarov technique may be useful initially in severe cases with significant shortening and a wide nonunion or in patients in whom medullary nailing and standard bone grafting procedures fail.

Ref: Canale & Beaty: Campbell's Operative Orthopaedics,11th ed.

Saturday, April 17, 2010

Orthopedic Handouts

Orthopedic Handouts
very concise, illustrative  and informative
useful for the last pre-exam review
Dr. Magdy Anwar
Orthopedic Surgeon
King Fahd Hospital - Madinah Munawrah -Saudi Arabia
http://www.madinaortho.com
 

Friday, April 16, 2010

Pronator syndrome

This is an uncommon entrapment neuropathy of the median nerve occurring in the elbow region.

Entrapment can occur typically at four sites.

1. The first occurs at the site of the ligament of Struthers. This ligament represents an anatomical variant and when present connects a small supracondyloid spur of bone to an accessory origin of pronator teres. The median nerve can be compressed as it passes under this ligament.

2. The nerve may also be trapped as it passes deep to the bicipital aponeurosis;

3. the aponeurotic edge of the deep head of pronator teres muscle;

4. or the tendinous aponeurotic arch forming the proximal free edge of the radial attachment of flexor digitorum superficialis.
The syndrome presents with pain on the volar aspect of the distal arm and proximal forearm. The symptoms may be aggravated by flexing the elbow against resistance, pronating the forearm against resistance, or flexion of superficialis to the middle finger against resistance, depending on the precise cause of the entrapment.

If the anterior interosseous nerve is also compressed there is weakness of all the muscles innervated by the median nerve, including abductor pollicis brevis and the long finger flexors, and sensory impairment on the palm of the hand.

The treatment is exploration of the nerve and surgical decompression.

Saturday, April 3, 2010

Myositis Ossificans

• Heterotopic bone formation often occurs after injury or surgery and can occur in any collagenous supportive tissue of skeletal muscles, tendons, ligaments, and fascia.

There are four clinical types; three may be seen in injured patients:

o Myositis ossificans progressiva is rare and can be genetic. It usually occurs between the ages of 5 and 10 years (younger than age 20) and proceeds relentlessly to progressive ossification of skeletal muscles. It is often present in the shoulders and neck as firm subcutaneous masses, which can be hot and tender and can undergo ossification. Often associated are microdactyly of the great toes and thumbs, ankylosis of the interphalangeal and metatarsophalangeal joints, and bilateral hallux valgus. Minor trauma often causes exacerbations. Treatment may include diphosphonate combined with surgery for severe joint malpositioning and functional impairment.

o Myositis ossificans paralytica occurs in proximal paralyzed muscles. The ossification occurs 1 to 10 months after a spinal cord injury. This process causes decreased passive range of motion. The three classic sites are in the vastus medialis, the quadratus femoris, and the hip abductors. Surgical treatment is indicated only if the position and function of the extremity are unacceptable and when the ossification has matured. After excision, the dead space created must be drained by closed suction and the wound carefully observed for a hematoma.

o Myositis ossificans circumscripta can be idiopathic but is more commonly caused by focal trauma and is common as a sports injury in the contact setting. It is more common in teenage or young adult males. It presents as an uncomfortable, indistinct mass that shows local induration and a local increase of temperature. The lesion occurs 80% of the time in the arm (biceps brachialis) but also occurs in the thigh (abductors and quadratus femoris). Roentgenograms show fluffy calcification 2 to 4 weeks after injury. In 14 weeks, the calcification has matured, and in 5 months, ossification has occurred. The differential diagnosis includes osteosarcoma and periosteal osteogenic sarcoma. Treatment is by excision, only if the lesion is unusually large or painful and after ossification is mature.

o Myositis ossificans traumatica, the most common type of hetertopic ossification presents the same way as the circumscripta type except for a clear history of trauma, with ossification of a single muscle group in the traumatized area (17). Treatment is controversial but generally is aimed at the prevention of ossification by immediate application of cold and compression to the area of muscle injury. Later, heat is applied. An operation is indicated only when the ossification causes permanent impairment and only after the process has stabilized, often as soon as 6 to 8 months after injury.

• The precise pathophysiology of myositis ossificans is not known. Preventive treatment should be designed to stop the sequence of osteogenesis

Treatment

o Rest to the part.

o Pharmacologic treatment is generally prophylactic and has historically included bisphosphonates to inhibit hydroxyapatite crystallization, mithramycin to interfere with mobilization of calcium, and cortisone to decrease bone formation at the site of injury. None of these drugs, however, has proved to be an extremely beneficial therapeutic agent. Indomethacin and Naprosyn have been shown to help minimize posttraumatic heterotopic ossification associated with acetabular fractures and arthroplasty. Similarly, low-dose irradiation with 800 to 1,000 rad has been shown to be very effective at preventing heterotopic ossification.

o When surgical treatment is indicated, traditional teaching has been to wait until the ossification is mature that is, when the bone scan is negative and the alkaline phosphatase level is decreasing. Many authors have recently advocated earlier resection before these tests have returned to normal.

Tuesday, March 30, 2010

Adult-onset Still disease (AOSD)



Essentials of Diagnosis

•    Fever that spikes in "rabbit ears" pattern with daily return to normal.

•    Salmon-colored macular rash only occurring with fever.

•    Arthritis, splenomegaly, pleuritis, pericarditis, and marked leukocytosis common.

•    Pharyngitis often the initial symptom.

General Considerations

Adult-onset Still disease (AOSD) is a multisystem inflammatory disease that typically begins with a sore throat. Nonsuppurative pharyngitis may develop days to weeks before the typical quotidian fever, evanescent rash, and joint pains begin. Other constitutional symptoms soon follow, including profound fatigue, weight loss, and anorexia. Malignancy and infectious causes of these symptoms must be excluded because AOSD is diagnosed mainly on clinical grounds.

Etiology

The cause of AOSD has yet to be identified. The presence of daily spiking fevers has focused research efforts on the possibility that the cause of AOSD is infection-related. To date, however, there has not been any infectious agent or genetic predisposition identified in patients with this disease.

Incidence

Fortunately, AOSD is rare. One series reported an incidence of 0.16 cases per 100,000 population. Women and men are equally affected. The peak onset is between ages 20 and 45, although cases have been reported in all age groups. Pediatric patients with systemic-onset juvenile idiopathic arthritis can have a recurrence of active Still disease at any age into adulthood.

Clinical Findings

There is no definitive lab test for AOSD, but a high serum ferritin and marked leukocytosis with fever, rash, and arthritis in the absence of other possible causes is highly suggestive of this diagnosis.

Symptoms and Signs

The fever of AOSD is relentless, often lasting weeks at a time before the diagnosis can be established. Temperature spikes occur daily in these patients, often in the afternoon or evening. These elevations in temperature can be associated with shaking chills and sweating. The daily return to baseline or normal temperature is a distinguishing feature that separates patients with AOSD from those with chronic infection. Typically, in patients with chronic infection the temperature remains elevated between fever spikes.

The rash of AOSD is salmon-colored, macular, and can occur anywhere on the trunk and extremities. It is evanescent, and manifests during the febrile episodes but clears completely when the temperature returns to normal. It may be mildly pruritic and extend to areas that are scratched (Koebner phenomenon). Biopsy of involved skin, even with immunofluorescence, is usually not diagnostic. The presence of this particular rash in association with a daily fever is diagnostic of AOSD, even though the rash itself is nondescript, and can easily be mistaken for a drug reaction or viral exanthem. In some patients, the rash may reappear in the identical location during subsequent flares of active disease. Usually the face, palms, and soles are spared.

Joint pain is a common feature of AOSD, but true arthritis may be slow to develop. Initially, patients often have significant joint and muscle pain without true synovitis. Marked arthralgias and myalgias may be present initially and can develop into frank arthritis over time. Arthritis develops in large joints such as the hip, knee, ankle, shoulder and wrist more often than the small joints of the hands and feet. Persistent synovitis and restricted range of motion in affected joints can occur even after the fever has resolved. Destructive arthritis occurs in 20% of patients with AOSD. Carpal and cervical ankylosis can occur as a result of arthritis in both the adult and childhood-onset forms of the disease. Avascular necrosis is a significant risk for those patients with AOSD who require glucocorticoids for control of their systemic symptoms or persistent arthritis or both. Hip involvement and persistent synovitis are poor prognostic signs and justify an aggressive treatment approach.

Pleuritis, pericarditis, lymphadenopathy, hepatomegaly, and splenomegaly are common in AOSD (Table). Biopsy specimens of lymph nodes show reactive changes due to polyclonal B-cell hyperplasia.

Table - Clinical Manifestations of Adult-Onset Still Disease
Fever
Acute pharyngitis
Arthritis/arthralgia
Severe myalgias
Lymphadenopathy
Splenomegaly
Hepatic dysfunction
Pleuritis
Pericarditis
Laboratory

There is no definitive lab test for AOSD; however, marked leukocytosis (>15,000/ L) with a predominance of neutrophils
(>80%) and a markedly elevated ESR (>90 mm/h) is seen in almost all patients with this disorder. The lab findings in AOSD suggest both acute and chronic inflammation with a low serum albumin, anemia of chronic disease, elevated C-reactive protein, and elevated complement levels. Marked elevation of the serum ferritin (above 3000 mg/mL) is seen in over 70% of AOSD patients. A high ferritin can be seen in hematologic malignancies but does not occur in other rheumatic disease syndromes. There is a low percentage (<20%) of serum ferritin that is glycosylated in patients with AOSD which may be even more specific for this disease. However, this test is not routinely clinically available. Recent studies have documented elevated levels of interleukin-18 that correlate with clinical activity of AOSD. This may help to explain the unusual pattern of inflammatory markers specific to this disease.

The antinuclear antibody test and rheumatoid factor are negative in almost all cases (Table2). Mild elevation of liver function tests are a frequent but nonspecific finding. There is no threat to renal function associated with AOSD, and the creatinine and urinalysis typically remain normal.

Table. Common Laboratory Test Abnormalities in AOSD
Elevated erythrocyte sedimentation rate
Elevated white blood cell counta
Elevated platelet count
Anemia
Elevated liver enzymes
Elevated ferritin
Negative antinuclear antibodies
Negative rheumatoid factor
TLC >15,000/ L with >80% PMN leukocytes.
 
Differential Diagnosis

Diagnostic criteria in AOSD using major and minor criteria from clinical and laboratory findings listed in Tables 1 and 2. These proposed classification systems rely on different combinations of major and minor criteria once infection, malignancy, and other rheumatic disorders have been excluded.

When patients present with sore throat, daily fever, rash, arthritis, and muscle pain, infection tops the list of possible causes. The most common causes of rash, fever, and arthritis are infectious, including viral infections (such as rubella, parvovirus, Epstein-Barr virus, cytomegalovirus, hepatitis B and C, and HIV) and bacterial infections (Borrelia burgdorferi [Lyme disease], Borrelia hermsii [relapsing fever], streptococcal-associated arthritis and rheumatic fever recurrence, and subacute bacterial endocarditis, among others). It is vitally important to exclude infection before beginning treatment for AOSD.

Malignancy can cause fever, rash, arthralgias, and also many of the nonspecific lab abnormalities seen in AOSD. Patients with hematologic malignancies often have enlarged lymph nodes, elevated ferritin levels, splenomegaly, abnormal liver function, and fever that can be difficult to distinguish from AOSD.

Other rheumatic disease syndromes can also mimic the signs and symptoms of AOSD. Sarcoidosis, polyarteritis nodosa, antineutrophilic cytoplasmic anti-body–associated vasculitis, inflammatory bowel disease, and recurrent fever syndromes such as familial Mediterranean fever, autoimmune neutropenia, hemophagocytic syndromes, and systemic lupus erythematosus must all be ruled out to establish a diagnosis of AOSD.

Treatment

Treatment of this condition can be challenging. Early in the disease course, treatment with nonsteroidal anti-inflammatory drugs can help reduce fever, joint pain, and muscle aches, but can lead to markedly elevated liver function tests. Aspirin was previously considered to be the mainstay of therapy, but frequently caused significant hepatitis. Nonsteroidal anti-inflammatory drugs are less likely to cause similar problems but the potential remains. Systemic glucocorticoids are indicated to control persistent synovitis and to treat life-threatening manifestations and constitutional symptoms that interfere with the activities of daily living. If arthritis persists, treatment with a disease-modifying agent, such as methotrexate or cyclosporine, or an anti-cytokine agent, such as anakinra (an interleukin-1 inhibitor) or etanercept (a tumor necrosis factor inhibitor), can induce remission and minimize glucocorticoid exposure. There are remarkable responses to anakinra reported in the literature. Newer biological agents that target interleukin-6 may be even more effective in treating this disease.

Therapy should be continued until laboratory parameters show no signs of inflammation and clinical examination indicates no active disease is present. Medication can then be tapered slowly with the hope of maintaining a remission on the lowest effective dose. Disease-modifying agents should be continued for a 1-year disease-free interval before being discontinued altogether.

The clinical course of AOSD is variable. One-third of patients remit after one extended symptomatic period that can last up to 1 year. One-third of patients with AOSD relapse with a polycyclic course. In these patients, remission can occur between flares. Another third of patients with AOSD will have a persistent active clinical course with chronic active arthritis the main ongoing symptom. Relapse in this disorder can occur years after the initial diagnostic episode.

Saturday, March 27, 2010

Complex Regional Pain Syndrome (CRPS)


Synonyms:
Reflex sympethatic Dystrophy Syndrome (RSDS), Sudeck's Atrophy, Causalgia, Shoulder-Hand Syndrome, Posttraumatic Dystrophy, Sympathetic maintained pain syndrome
History:

  • Recognized since the Civil War when it was called causalgia, a name chosen to describe intense, burning extremity pain after an injury.

  • Bonica coined the term reflex sympathetic dystrophy in 1953
Incidence:

1% of all conservatively treated distal radius fractures and up to 5% of operatively treated fractures.
Types:

  • CRPS type I

    • secondary to an identifiable neurologic compression or injury.

    • e.g. ulnar nerve axonotemesis or a superficial radial nerve injury due to trauma from an external fixator pin.

  • CRPS type II

    • no identifiable neurological injury

    • sympathetically mediated pain
Stages:
Acute:

  • 6-12 wks

  • persistent burning pain

  • trivial injury followed by severe & out of proportion pain

  • Localised pain, later spreads throughout extremity

  • Hypersensitivity to light touch

  • Extremity swollen & warm

  • Excessive perspiration

Dystrophic

  • Affected joint ROM restricted

  • Involved area becomes cool
Atrophic

  • Skin & muscle atrophy

  • Skin dry, shiny, glossy

  • Stiffness, intractable pain persists several weeks
Diagnosis:

  • Identify for CRPS type I or CRPS type II.

  • determine if it is a treatable source (type I).

  • Persistent burning pain after an injury is characteristic.

  • most common lesion with type I is median nerve injury, due to direct trauma or an undiagnosed compressive neuropathy.

  • causes are injury to the ulnar nerve, the superficial radial nerve, the intercarpal ligament, or the triangular fibrocartilage.

  • delayed union, incomplete union, and nonunion may also contribute to symptoms.

  • X-ray – Patchy Osteoporosis

  • Peripheral nerve conduction studies e.g. compression points around the elbow are useful, also evaluate the ulnar nerve.

  • MRI may show an incomplete union, carpal injuries, or TFCC injury.

  • Arthroscopic reveal arthrofibrosis and/or TFCC injuries.

  • Bone Scan +ve, showing regional uptake reflects ncreased blood flow
Treatment:

  • Prevention, immediate attention, control pain & swelling.

  • Restoration of motion by exercise

  • Active use of extremity despite pain

  • Edema control by limb elevation

  • Physiotherapy

  • Drugs: antidepressants, corticosteroids, calcium channel blockers
CRPS Type I:
Surgical treatment:

  • Neurolysis aimed at external compression of the median and ulnar nerve injuries.

  • Adjunctive grafting or Barrier wrapping for injury to sensory branches of the superficial radial or dorsal ulnar nerve.

  • Neuroma resection proximally and nerve stump can be buried in appropriate soft tissue.

  • External bone stimulators or revision osteosynthesis for an incomplete union.
CRPS type II

  • Multifaceted, aimed at restoring ANS control and improving physical function.

  • Early recognition and regional blockade with physical therapy is useful.
Medical management

  • guided by the appearance of the hand and wrist (Early phases marked by erythema and swelling, later phases, they may appear cool and atrophic.

  • For warm, swollen erythematous hand, treatment include gabapentin, selective serotonin reuptake inhibitors, or clonidine hydrochloride.

  • In later stages, the aim is to improve blood flow, using nifedipine or selective serotonin reuptake inhibitors.
Physical therapy

  • mobilizing the wrist and digits.

  • Focus is to improve wrist extension, causing greater mechanical advantage.

  • Adjunctive modalities such as dynamic or serial static splinting may prove effective at mobilizing the wrist and the metacarpophalangeal joints.
Prognosis

  • Recovery after CRPS treatment varies.

  • the prognosis for CRPS type I is better than that of CRPS type II.

  • When the syndrome continues for more than 1 year, it is likely that residual impairment will be present.

  • Regardless of the treatment afforded, patients experience delayed recovery.