Robert M. Bernstein

Arthrogryposis and amyoplasia.

&NA; Arthrogryposis (multiple congenital joint contractures) is an uncommon problem. Because there are many causes, correct diagnosis is important to predict the natural history and determine appropriate treatment. Inconsistent terminology has caused confusion about both diagnosis and treatment. Amyoplasia, the most common type of arthrogryposis, is characterized by quadrimelic involvement and replacement of skeletal muscle by dense fibrous tissue and fat. Early physical therapy and splinting may improve contractures, but surgical intervention is often necessary. Aggressive soft‐tissue releases in addition to casting may improve joint position. In more severe contractures, osseous surgery also may be needed. Deformity recurrence is common, particularly in skeletally immature patients.

Solid rod short segment anterior fusion in thoracolumbar scoliosis.

Seventeen patients with adolescent idiopathic thoracolumbar scoliosis underwent short segment anterior spinal fusion with a solid rod-screw construct. Patients were evaluated radiographically and interviewed using the Scoliosis Research Society instrument. Mean radiographic follow-up was 34 months, and mean follow-up at time of interview was 62 months. Mean curve correction was 87%, declining to 67% at 2 years. Mean correction of the instrumented curve was 114%, declining to 103% at 2 years. The thoracic curve improved 24% and remained stable throughout the follow-up period. Kyphosis over the instrumented segments increased from 4 degrees preoperatively to 10 degrees postoperatively. Although there was a trend toward increasing kyphosis over time, this was not statistically significant at final follow-up. Most patients (88%) were extremely satisfied with the surgical result. We believe that a short-segment anterior fusion with a solid rod-screw construct provides excellent curve correction with minimal kyphosis over the instrumented segment. Overcorrection of the instrumented segment must be achieved for this technique to be successful.

Neurologic risk management in scoliosis surgery.

Paraplegia resulting from the operative treatment of scoliosis is the complication most feared by surgeon and patient (5,26,31,50). Less devastating neurologic complications, including incomplete spinal cord injury and peripheral nerve injury, can also occur. Theoretically, the frequency of neurologic complications increases with more aggressive corrective techniques (50). Neurologic injury stemming from surgical treatment of scoliosis can be direct or indirect. The implications of such complications are profound, and prevention is paramount. The purpose of this paper is to review minimization of risk in scoliosis surgery. In addition, we review techniques for dealing with neurologic compromise and stress the need to maintain adequate communication with the patient and family.

Nonunion of a Hoffa fracture in a child.

The authors report a case of a Hoffa fracture of the lateral femoral condyle that subsequently went on to nonunion in an eight-year-old child. The child presented with symptoms of knee pain and snapping five years after a motor vehicle accident. The nonunion fragment involved most of the lateral femoral condylar articular surface but spared the physis. After treatment by open reduction and limited internal fixation, the nonunion has healed, and the child has virtually full range of motion of the knee and no evidence of growth disturbance.

Autogenous tibial strut grafts used in anterior spinal fusion for severe kyphosis and kyphoscoliosis

Study Design. A retrospective review was performed. Objective. To evaluate the results of autogenous tibial strut grafts for anterior fusions in children with severe kyphosis and kyphoscoliosis regarding maintenance of correction, clinical outcome, graft fractures, and donor-site morbidity. Summary and Background Data. Anterior strut grafts harvested from the iliac crest, rib, and fibula often are used to treat severe kyphosis and kyphoscoliosis. Several studies in the literature have observed graft failures, loss of correction, or donor-site morbidity when these grafts have been used. Autogenous tibial strut grafts provide some theoretical advantages with minimal donor-site morbidity. Methods. This review included 15 patients with severe kyphosis/kyphoscoliosis who underwent anterior spinal fusion with autogenous tibial strut grafts. Among these patients, 13 underwent staged or simultaneous posterior fusions, and 4 underwent cord decompression for myelopathy. An average of seven levels (range, 3–13) were fused anteriorly. Preoperative, postoperative, and latest follow-up radiographs were evaluated for graft incorporation, fracture, hardware failure, and spinal alignment. Patients were examined at the latest follow-up visit, and charts were reviewed to assess neurologic status, back pain, alignment, complications, and donor-site problems. Results. All the patients were available for clinical examination. The mean follow-up period was 3.9 years (range, 2–8 years). The mean kyphosis measured 89° before surgery, 62° after surgery, and 66° at the most recent follow-up assessment. In patients with kyphoscoliosis, the mean coronal curve measured 64° before surgery, 42° after surgery, and 46° at the latest follow-up assessment. Apparent fusion was observed in all cases with no graft fractures. One patient reported mild donor-site discomfort. Conclusions. Autogenous tibial strut grafts provide physical advantages over commonly used iliac crest, rib, and fibula grafts. The tibia provides dense cortical bone with ample length and mechanical strength, although the actual strength of each strut was not measured directly. In this study, adequate correction was maintained throughout an average follow-up period of 3.9 years, and solid fusion was obtained in all cases. The results indicate that this technique offers a reliable means of providing anterior support in the management of severe kyphosis with virtually no donor-site morbidity. Although the number of patients in this review was limited, the authors believe that anterior autogenous tibial struts are an excellent alternative for the treatment of severe kyphosis and kyphoscoliosis.

Rod diameter prediction in patients with osteogenesis imperfecta undergoing primary osteotomy.

Because the cross-sectional shape of the long bones of patients with osteogenesis imperfecta is often elliptical, the use of preoperative radiographs to determine intramedullary rod diameter in these patients undergoing osteotomy may be misleading. To investigate this, the authors correlated the narrowest inner bone diameter (NID) on preoperative radiographs to the rod diameter (RD) on postoperative radiographs. The authors evaluated 79 bones in 27 patients undergoing primary osteotomy with intramedullary fixation. Only 5% of the bones had an equal NID and RD, with 81% of bones having a smaller RD than the measured NID. Although a positive correlation was found between RD and NID (correlation coefficient 0.76), measurement of the NID on preoperative radiographs did not provide a good prediction of the actual RD used in this series of children with osteogenesis imperfecta.

The Lengthening of Short Upper Extremity Amputation Stumps

Background: Short upper extremity amputation stumps are difficult to fit with an appropriate-level prosthesis. Fitting at a more proximal level generally results in decreased function. Options in the past have included stump and prosthetic modification, both of which provide limited improvement in function. Anecdotal reports of lengthening short amputation stumps have been published. This article reviews our experience with lengthening short upper extremity amputation stumps in children. Methods: All patients who underwent an upper extremity stump lengthening at Shriners Hospital Los Angeles with at least 1-year follow-up were included. Charts and radiographs were retrospectively reviewed and prosthetic use preoperatively and postoperatively, complications, and additional procedures were documented. Stump length was measured on radiographs as the length between the proximal flexion crease and the tip of the bone. Results: Eleven patients with 14 amputation stumps underwent lengthening. Mean stump length increase was 264% (4 cm). Nine patients desired prosthetic fitting; all except 1 were able to be fit with an appropriate-level prosthesis. Two of the 9 patients reported only using the prosthesis on rare occasions. The 2 remaining patients underwent lengthening to improve function but did not desire a prosthesis preoperatively. Conclusions: Lengthening is a viable but controversial option for very short upper extremity amputation stumps and may result in better function and/or more appropriate prosthetic fitting. Complications and additional procedures are common. Soft tissue coverage seems to be the main limiting factor to lengthening. Level of Evidence: Level IV.

Mobile Pediatric Orthopaedic Education (MoPOEd): a unique program teaching sustainable pediatric orthopaedics in the developing world.

Approximately 45% of the population of the developing world is under fifteen years of age1, yet many developing countries have little or no available health care for the treatment of bone, joint, and muscle problems in children. While the World Health Organization and other organizations focus on the eradication of human immunodeficiency virus (HIV), malaria, and other diseases2 through the development of vaccines and medications, little or no effort has been directed to diseases requiring surgical treatment, which according to one estimate account for 11% of the total global disease burden and a loss of 128 million disability-adjusted life years (DALYs)3. Weiser et al. estimated that approximately 234 million major surgical procedures are performed worldwide each year, yet the poorest one-third of the worlds population undergoes only 3.5% of those procedures4. Gosselin and Heitto estimated that the cost of the surgical procedures performed at a district trauma hospital in Cambodia was

Spontaneous rupture of the tibialis anterior tendon.

77.40 per DALY averted5. They noted that this was extremely cost-effective when compared with antiretroviral therapy (

Familial aspects of Caffey's disease.

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