Bruck syndrome: neonatal presentation and natural course in three patients.

Three unrelated patients with congenital arthrogryposis and brittle bones, the main neonatal signs of Bruck syndrome, are presented. In infancy and early childhood recurrent fractures of ribs and long bones and persistent Wormian bones in the calvarium are reminiscent of osteogenesis imperfecta (OI) even with white sclerae, normal dental quality and normal hearing as important clinical negatives. The diagnosis was made before two years of age in two, and in adolescence in the third patient. The latter's radiologically documented long-term natural course reveals slow progressivity of osteopenia and growth deficiency, worsening tendon contractures and pterygia in addition to increasing spine and pelvis deformation. Mental development remains normal. Bruck syndrome is monogenic and probably due to homozygosity of an as yet unidentified gene. As no alteration in the collagens I and III is detected and molecular screening reveals no mutation in the COL1A1 and COL1A2 genes, the pathogenesis of this severe disorder of connective tissue remains largely unknown.

An acute zinc chloride poisoning in a child: was chelator therapy effective?

An acute zinc chloride poisoning due to ingestion is a rare event. Symptoms include: corrosive pharyngeal lesions, vomiting and lethargy. Laboratory findings may include hyperglycaemia, hyperamylasaemia, exocrine pancreatic insufficiency and renal insufficiency. This case report describes an accidental zinc chloride poisoning in a child, with lethargy as the most pronounced clinical sign. Clinical evaluation and chelator therapy are discussed.

Monteggia lesions in adults. A multicenter Bota study.

Sixty-seven Monteggia lesions occurring in adults were reviewed. A follow-up of 1 to 14 years revealed that 53.7% had good to excellent results and 46.3% had results which were fair or poor. All fractures were treated with open reduction and internal fixation. Complications were encountered in 29 cases or 43%. Delayed bone healing in 10 cases and the persistent dislocation of the radial head in 7 cases were noted. Excellent or good results were seen in type III and I, according to Bado's classification; fair or poor results in type IV and II. The results are less good when the olecranon process is fractured, especially in type Ia fractures. The overall result of this injury is abysmal. To improve the outcomes, the authors recommend dorsal plating of the ulnar fracture, avoidance of early resection of the radial head and immediate mobilization of the elbow.

Treatment of tibial shaft fractures by interlocking nailing.

Between 1982 and 1986, 60 tibial shaft fractures were treated by interlocking nailing. Of these, 10 were complicated by a pseudarthrosis. The results were assessed after a minimum follow-up of 2 years and were found to be good both in patients with fresh fractures and with pseudarthroses; in only 3 out of 60 cases was the outcome poor. The advantages of the techniques include: its suitability for virtually all types of shaft fractures ease of surgery early mobilization and weightbearing, with a low risk of infection rapid consolidation. Relative drawbacks are: radiation exposure longer preoperative preparation involvement of the entire shaft if infection develops irritation of the patellar tendon.

Treatment of the irreducible hip.

"Irreducible" congenital dislocation of the hip should not be regarded as a special pathological entity. Thirty-eight cases with a previous and failed treatment attempt were reviewed. Recognition of the renal cause of "irreducibility" (iatrogenic or anatomopathological) and institution of an appropriate treatment protocol result in adequate reduction in the vast majority of cases. However, osteochondritis is not an uncommon finding when the results of secondary treatment are evaluated. The percentage of more severe cases is higher after open procedures.