Peripheral arthropathy in hereditary sensory and autonomic neuropathy types III and IV.

BACKGROUND: To determine the features of the underlying destructive arthropathy in the peripheral joints of children with hereditary sensory and autonomic neuropathy (HSAN) type III and to compare and contrast this to the arthropathy noted in HSAN type IV, as both groups experience decreased pain perception. METHODS: From a database of 547 patients with HSAN type III and 32 patients with HSAN type IV, we performed a retrospective chart review and radiographic analysis of all patients who presented with joint swelling and deformity. Underlying joint pathology was classified as either osteonecrosis or Charcot arthropathy. RESULTS: In the HSAN type III population, 44 (8%; 22 males and 22 females) of the 547 patients had clinical evidence of arthropathy. In 42 patients, 48 joints demonstrated radiographic evidence of osteonecrosis; 45 (94%) of the 48 joints with osteonecrosis occurred in the lower extremity. In each case of osteonecrosis of the knee (n = 19), isolated involvement of the lateral distal femoral condyle was seen consisting of varying sizes of posterolateral osteochondral fragmentation. In the 32 patients comprising the HSAN type IV population, 18 (56%) were found to have radiographic findings consistent with Charcot arthropathy in a total of 30 affected joints. One patient demonstrated Charcot arthropathy of the spine and subsequent progressive spondylolisthesis. Nine patients (12 joints) also demonstrated osteomyelitis. CONCLUSIONS: In patients with HSAN type III, osteonecrosis is the initial lesion preceding destructive arthropathy. Osteonecrosis and osteochondral fragmentation were always isolated at the lateral distal femoral condyle in the knee. This pathology may be amenable to surgical reconstruction and fixation to stabilize the knee and prevent further degeneration. Hereditary sensory and autonomic neuropathy type IV was most commonly associated with Charcot arthropathy or joint subluxation and dislocation. Late secondary changes at the articular surface may make radiographic distinction difficult. Charcot arthropathy affected both sides of the involved joint with evidence of collapse and fragmentation. With osteonecrosis, the articular process was found to be more focal.

Functional outcome in adult patients following Bernese periacetabular osteotomy.

This study evaluated functional outcome following Bernese periacetabular osteotomy. In 24 patients with mean follow-up of 3.5 years, mean dysfunction score was 15.23 on the Short Musculoskeletal Function Assessment. Eighteen patients (75%) scored = or <20, indicating a high functional level. Irrespective of preoperative Shenton line continuity, most patients demonstrated a high functional level. However, a trend toward a poorer outcome was observed in patients with preoperative noncongruent joints and Tonnis osteoarthritis grade 3. These results suggest patients with less than optimal presentation may still benefit from this surgery, delaying or eliminating the need for total hip arthroplasty.

Evaluation of an algorithmic approach to pediatric back pain.

Pediatric patients require a systematic approach to treating back pain that minimizes the number of diagnostic studies without missing specific diagnoses. This study reviews an algorithm for the evaluation of pediatric back pain and assesses critical factors in the history and physical examination that are predictive of specific diagnoses. Eighty-seven pediatric patients with thoracic and/or lumbar back pain were treated utilizing after this algorithm. If initial plain radiographs were positive, patients were considered to have a specific diagnosis. If negative, patients with constant pain, night pain, radicular pain, and/or an abnormal neurological examination obtained a follow-up magnetic resonance imaging. Patients with negative radiographs and intermittent pain were diagnosed with nonspecific back pain. Twenty-one (24%) of 87 patients had positive radiographs and were treated for their specific diagnoses. Nineteen (29%) of 66 patients with negative radiographs had constant pain, night pain, radicular pain, and/or an abnormal neurological examination. Ten of these 19 patients had a specific diagnosis determined by magnetic resonance imaging. Therefore, 31 (36%) of 87 patients had a specific diagnosis. Back pain of other 56 patients was of a nonspecific nature. No specific diagnoses were missed at latest follow-up. Specificity for determining a specific diagnosis was very high for radicular pain (100%), abnormal neurological examination (100%), and night pain (95%). Radicular pain and an abnormal neurological examination also had high positive predictive value (100%). Lumbar pain was the most sensitive (67%) and had the highest negative predictive value (75%). This algorithm seems to be an effective tool for diagnosing pediatric back pain, and this should help to reduce costs and patient/family anxiety and to avoid unnecessary radiation exposure.

Hinged distraction of the adolescent arthritic hip.

From 1996 to 2000, 11 adolescents with hip joint arthritis secondary to osteonecrosis or idiopathic chondrolysis were treated with articulated hinged distraction arthroplasty. Indications for surgery were severe pain and limited ambulation. Charts and radiographs were reviewed. Clinical status was assessed preoperatively and at latest follow-up (mean 4.8 years after surgery) using criteria of pain, range of motion, and ambulation level. Ten patients showed improved clinical status, with seven having an excellent outcome and three a good outcome. One patient failed distraction. Mean joint space was 2.6 mm before surgery and 4.8 mm at latest follow-up. Average duration of fixator use was 4.4 months. Four patients (36.4%) had complications. Articulated hip distraction was effective in eliminating pain, improving function, and preventing progressive degenerative changes in young patients' hips. It should be considered a salvage procedure for arthritic hips and an alternative to arthrodesis in this difficult-to-treat group of patients.