Musculoskeletal manifestations of Russell-Silver syndrome

The musculoskeletal manifestations of Russell-Silver syndrome were studied in 25 patients. The most common manifestations were short stature (25 patients), limb-length discrepancy (23 patients), clinodactyly (19 patients) metacarpal bone and phalangeal abnormalities (13 patients), scoliosis (9 patients), foot syndactylism (5 patients), and developmental dysplasia of the hips (3 patients). Five patients underwent lower extremity limb-lengthening procedures for discrepancies greater than 3 cm and three patients had successful pelvic and/or femoral osteotomies for hip dysplasia. All 18 patients studied had retardation of bone age, which peaked at age 7 years.     

Lower extremity compartment syndrome in an adolescent with spinal cord injury

OBJECTIVE: Describe the unusual complication of lower extremity compartment syndrome occurring in an adolescent with spinal cord injury (SCI). METHODS: Case presentation. RESULTS: A 17-year-old male with C5 ASIA A complete SCI developed a compartment syndrome of his lower leg on the ninth day postinjury. Presenting signs included an equinus deformity of the foot, blackened induration over the anterior tibia, circumferential erythematous markings over the calf, large urticarial lesions over the knee, and calf swelling. The presumed etiology of the compartment syndrome was excessive pressure from elastic wraps, which were placed over gradient elastic stockings. Pressures were 51 mmHg in the superficial posterior, 50 mmHg in the deep posterior, 33 mmHg in the anterior, and 34 mmHg in the peroneal compartments. The patient also developed rhabdomyolysis with myoglobinuria. In addition to supportive care, the patient underwent a dual incision fasciotomy for compartment release. CONCLUSIONS: The development of lower extremity compartment syndrome was probably a result of excessive pressure applied by elastic wraps. Elastic wraps should be used with caution in individuals with SCI.     

Transphyseal osteotomy of the distal tibia for correction of valgus/varus deformities of the ankle

This study is a retrospective review of the results of consecutive cases of a transphyseal osteotomy of the distal tibia. Indications for the procedure are significant valgus or varus deformities of the ankle needing acute correction because of problems with the skin and brace fit as well as progressive deformity. Twenty-one patients with a variety of underlying diagnoses, five with bilateral deformities, underwent this procedure. The technique involved making either a medially based closing or opening wedge with the distal limb of the osteotomy through the physis or the physeal scar so that it was very close to the ankle joint. A fibular osteotomy was not necessary except in three ankles. All osteotomies healed. All patients were able to ambulate and use their braces as soon as their osteotomies healed, and none had any further pressure sores or brace-related problems, although some had mild residual valgus or varus deformities. There were no significant leg-length discrepancy problems as a result of the surgery. This osteotomy is a treatment alternative for significant angular deformities of the ankle that require acute correction.     

Variability of somatosensory cortical evoked potential monitoring during spinal surgery

The intraoperative variability of somatosensory cortical evoked potentials (SCEPs) has been measured for 320 consecutive spinal surgeries and found to be a function of patient diagnosis, neuromuscular status, age, and procedural factors. In many cases, it is likely that this variability severely limits the reliability and usefulness of spinal cord monitoring in detecting early cord compromise. Patients with idiopathic scoliosis, spondylolisthesis, and pseudarthrosis have the smallest spontaneous variability and strongest amplitudes, while those with congenital, paralytic scoliosis, stenosis, or tumor have very variable, weak SCEPs. Patients with neurologic disorders, particularly cerebral palsy, myelomeningocele, Friedreich's ataxia, and peripheral deficits, also have high variability and weak amplitudes. A monitoring quality scoring system is proposed that may be useful during surgery in judging how well the SCEPs can discern surgically related changes in cord function from background variations.