Observations on the development of the acetabulum following Chiari osteotomy.

A detailed analysis of acetabular development following Chiari osteotomy in ten patients with neuromuscular disease revealed an anti-Chiari effect in only one patient.

Problems with excessive residual lower leg length in pediatric amputees.

We studied six pediatric amputees with long below-knee residual limbs, in order to delineate their functional and prosthetic situations, specifically in relation to problems with fitting for dynamic-response prosthetic feet. Three patients had congenital pseudoarthrosis of the tibia secondary to neurofibromatosis, one had fibular hemimelia, one had a traumatic amputation, and one had amputation secondary to burns. Five patients had Syme's amputations, one had a Boyd amputation. Ages at amputation ranged from nine months to five years (average age 3 years 1 month). After amputation, the long residual below-knee limbs allowed fitting with only the lowest-profile prostheses, such as deflection plates. In three patients, the femoral dome to tibial plafond length was greater on the amputated side than on the normal side. To allow room for more dynamic-response (and larger) foot prostheses, two patients have undergone proximal and distal tibial-fibular epiphyseodeses (one at age 5 years 10 months, the other at 3 years 7 months) and one had a proximal tibial-fibular epiphyseodesis at age 7 years 10 months. (All three patients are still skeletally immature.) The families of two other patients are considering epiphyseodeses, and one patient is not a candidate (skeletally mature). Scanogram data indicate that at skeletal maturity the epiphyseodesed patients will have adequate length distal to their residual limbs to fit larger and more dynamic-response prosthetic feet.

Stability of myelomeningocele spines treated with the mayfield two-stage anterior and posterior fusion technique.

STUDY DESIGN: Retrospective review of the results of operative treatment for paralytic thoracolumbar lordoscoliosis in six consecutive myelomeningocele patients treated with the Mayfield two-stage combined anterior and posterior fusion and instrumentation technique. OBJECTIVES: To assess the long-term stability of myelomeningocele spines treated with the Mayfield technique. SUMMARY OF BACKGROUND DATA: An initial description of the technique by Mayfield and an early study of the evolution of the two-stage combined anterior and posterior instrumented fusion of paralytic lordoscoliosis in myelomeningocele spines are available. METHODS: Study of the six individual patients showed that the mean age at the time of surgery was 13 years and 8 months (range, 9 years 9 months to 15 years 10 months). At the time of surgery, the major lumbosacral curve averaged 81 degrees (range, 52-137 degrees ), pelvic obliquity was 28 degrees (range, 1-48 degrees ), torso decompensation was 2.8 cm (range, 0-6.0 cm), thoracic kyphosis was 60 degrees (range, 25-93 degrees ), lumbar lordosis was 106 degrees (range, 55-151 degrees ), and sacral anteflexion was 78 degrees (range, 22-117 degrees ). The mean duration of follow-up evaluation was 13 years 5 months (range, 8 years 10 months to 16 years 11 months). RESULTS: Clinical and radiographic evaluation showed that immediately after surgery, the lumbosacral curve was corrected to an average of 25 degrees and at last follow-up evaluation was 25 degrees, for a final correction of 69%. Pelvic obliquity was corrected to 7 degrees, 5 degrees at last follow-up evaluation, for a 82% correction. Torso decompensation was corrected to 1.4 cm initially and finally to 0.3 cm, for an 89% correction. Kyphosis was corrected to 52 degrees, finally to 50 degrees (17% decrease); lordosis to 73 degrees and finally to 67 degrees (37 degrees decrease); and sacral anteflexion corrected to 51 degrees and finally to 56 degrees (28% decrease). In one patient, the tip of the longest rod displaced from the uppermost hook and was replaced with maintenance of correction. Another patient had dehiscence of the pelvic portion of the posterior wound, with secondary bacterial contamination, and healed by secondary intention. CONCLUSIONS: The Mayfield technique effectively corrected and stabilized these difficult myelomeningocele spinal deformities, using distraction against square-holed hooks seated on the sacral alae, which contain the best-quality bone in these hypoplastic pelves.

Previously undescribed syndrome of spondylometaphyseal dysplasia, osteocartilaginous metaplasia of long bones, and progressive osteolysis of distal phalanges.

We present the findings and clinical course of a Caucasian woman (now age 23 1/2) who has been treated since early childhood for a previously undescribed syndrome of painful osteocartilaginous metaplasia of long bone metaphyses and painful distal phalangeal osteolysis and soft tissue swelling. Despite extensive evaluations and attempts at effective treatment, the cause and pathogenesis of her unique musculoskeletal disorder remain elusive.

Dynamics of hip joint remodeling after Chiari osteotomy. 10 patients with neuromuscular disease followed for 8 years.

We analyzed the development of 10 hips in 10 consecutive patients with neuromuscular disease (9 with spasticity, 1 with Charcot-Marie-Tooth disease) who had undergone Chiari osteotomy for painful hip subluxation or dislocation. The patients were 11 (5-19) years old at surgery and follow-up time was 8 (6-11) years. The Chiari osteotomy particularly improved and maintained femoral head coverage. These parameters did not show the postoperative deterioration noted in some other studies. The osteotomy did not improve femoral head lateral displacement. Throughout the postoperative period, the configuration of the proximal femur and the height of the joint cartilage were unchanged and undisturbed, indicating that osteotomy did not place excessive or uneven pressure on the femoral head. The ambulatory status of the patients was dependent on the severity of the underlying disease, and was not improved by osteotomy. However, pain associated with subluxation or dislocation was reduced in 9 of the patients.

Kyphoscoliosis in Williams syndrome.

A 10-year-old girl with Williams syndrome (with characteristic facies and behavior, mental retardation, and growth disturbances) was seen with scoliosis, which, despite attempted bracing, rapidly progressed to 95 degrees and required surgical stabilization. Review of the entire literature on Williams syndrome revealed hallux valgus and little-finger clinodactyly as the most commonly mentioned orthopaedic manifestations, with only brief mention of spinal deformity. As awareness of Williams syndrome increases, spine surgeons must be aware of possible rapidly progressive scoliosis and kyphosis.

Bilateral humeral head osteonecrosis following spinal cord injury: a case report illustrating the importance of adhering to the recommendations of the Second National Acute Spinal Cord Injury Study.

Five years prior to the 1990 publication of the Second National Acute Spinal Cord Injury Study (SNASCIS), a 24-year-old man sustained traumatic paraplegia, and was treated with 797 mg of dexamethasone over the ensuing 26 days. Within three years he developed symptomatic bilateral humeral head osteonecrosis. Although his total steroid dose was less than one-third of the comparable dose recommended by the SNASCIS, the duration of administration of the steroid was much longer. This case illustrates the importance of adhering to the guidelines established by the SNASCIS, especially regarding the 24-hour administration period.

Postoperative Clostridium difficile pseudomembranous colitis in idiopathic scoliosis. A brief clinical report.

The authors report two healthy young patients with progressive idiopathic scoliosis, both without allergies or histories of gastrointestinal disorders, who received perioperative preventive cephalosporin antibiotics, and developed explosive diarrhea postoperatively, confirmed as Clostridium difficile pseudomembranous colitis by stool toxin assay. Both patients had initially recovered uneventfully after posterior fusion and Cotrel-Dubousset instrumentation. Their youth, health, diagnosis, and lack of nosocomial factors made colitis unexpected. The two cases were sporadic, occurring 2 years apart over a 12-year observation period. Symptoms and signs of colitis for the two patients were markedly varied as to time of onset, order of appearance, and severity. Unexplained fever before onset of diarrhea led to renewed cephalosporin administration, potentially exacerbating the colitis. Initial symptoms and signs were nonspecific; appropriate treatment had to begin before diagnosis could be confirmed by stool toxin asay, which requires 2 days.

Intraoperative use of the mobile gamma camera in localizing and excising osteoid osteomas of the spine.

Three patients (ages 12, 13, and 15 years) had histologically verified osteoid osteomas of the spine (specifically in the facet processes of L3, L2, and C5) which were localized preoperatively with nuclear scintigraphy utilizing technetium 99m diphosphonate. Intraoperative nuclear scintigraphy utilizing the mobile gamma camera was essential in both localizing the tumor during surgical excision and ensuring adequacy of excision. The authors strongly recommend intraoperative scintigraphy, and en bloc resection rather than shelling out and curettage. All three patients required facet process excision for successful tumor removal, and therefore required local fusion with internal fixation.

The variability of response of scoliotic spines to segmental spinal instrumentation.

Ninety-two patients (71 idiopathic, 15 neurogenic, 2 Marfan's syndrome, 1 Down's syndrome, 1 osteogenesis imperfecta, 1 Prader-Willi syndrome, 1 Klippel-Trenaunay-Weber syndrome), ages 9-49 years, had posterior spine fusion and stabilization with Luque L-rods and sublaminar segmental wires (SSI) for progressive scoliosis. There were no neurologic complications. The average preoperative major curve of 52 degrees (37-113 degrees) initially corrected to 30 degrees (6-94 degrees), and at last follow-up (range, 2-7 years), was 33 degrees (8-90 degrees). There was marked variability in curvature correction and maintenance of correction, with 14 patients progressively decreasing their curvatures postoperatively. Average preoperative Pedriolle vertebral rotation angle for all 92 patients was 16 degrees (4-26 degrees), which initially corrected to 14 degrees (0-24 degrees), and at last follow-up remained 14 degrees (0-23 degrees). Although SSI had little influence on rotation, 12 patients progressively decreased their rotational deformity after operation. Aside from the positive influence of curve flexibility on the degree of postoperative curve correction, the authors could not identify factors explaining how curvature and vertebral rotation responded so variably to SSI. The authors recommend continued use of SSI to stabilize curvatures in osteopenic patients (particularly those with neuromuscular disease), but rotational deformity will probably persist.

An autosomal dominant syndrome of short stature with mesomelic shortness of limbs, abnormal carpal and tarsal bones, hypoplastic middle phalanges, and bipartite calcanei.

This paper describes seven persons in a family affected with an autosomal dominant syndrome of short stature with mesomelic shortness of upper and lower limbs, abnormal carpal and tarsal bones, hypoplastic or absent middle phalanges of hands and feet, and delayed coalescence of bipartite calcanei. All affected relatives are of normal intelligence, are free of eye problems, and have a normal skull, spine, shoulders, and hips. The digits of the hands and feet are short, broad, and angulated. The hypoplastic or absent middle phalanges effectively result in one interphalangeal joint for each digit, with decreased mobility. The bones of the carpus and tarsus coalesce with increasing age. None of the previously described syndromes or brachydactylies encompasses the findings noted in this kindred.

Surgical treatment of paralytic scoliosis associated with myelomeningocele.

The spines of forty patients with myelomeningocele and paralytic scoliosis were surgically stabilized at the Twin Cities Scoliosis Center between 1960 and 1979. Treatment with posterior spine fusion and Harrington instrumentation extending to the sacrum, combined with anterior fusion using either Dwyer or Zielke instrumentation, gave the best results, correcting scoliosis by an average of 45 degrees (comparing preoperative values with those at the last visit), lordosis by an average of 20 degrees, torso decompensation by an average of 5.7 centimeters, and pelvic obliquity by an average of 7 degrees. This combined fusion method reduced the rate of pseudarthrosis to 23 per cent (compared with 46 per cent when only posterior fusion and instrumentation were used). Prophylactic antibodies (selected on the basis of preoperative cultures of urine) reduced the infection rate to 8 per cent. Posterior fusion or anterior fusion alone was inadequate, even with instrumentation. Early mobilization wearing a bivalved polypropylene body jacket minimized osteoporosis, pressure sores, and social isolation. Unsolved technical problems remain, however, especially in relation to obtaining fusion across the lumbosacral joint.

Thoracolumbar spine fractures. Results of treatment.

Sixty-three patients with thoracolumbar spine fractures treated between 1972 and 1978 were placed into six treatment groups: fusion and instrumentation alone, laminectomy with fusion and instrumentation, laminectomy alone, laminectomy with fusion without instrumentation, fusion alone, and no operation. Patients were interviewed and examined and their histories reviewed; new radiographs were obtained, and a detailed questionnaire was answered. No treatment method improved neurologic function. Fusion with instrumentation allowed earliest ambulation with least spinal deformity. Most patients in all treatment groups adjusted effectively to their injuries and remained active in employment and hobbies. A detailed, thorough rehabilitation program gave best functional results and patient attitudes.

Congenital pseudarthrosis of long bones: a clinical, radiographic, histologic and ultrastructural study.

Sixteen patients with pseudarthrosis of the tibia and one of the radius were evaluated clinically, radiograpically, and microscopically and separated into 3 groups; 8 had neurofibromatosis clinically, 3 had fibrous dysplasia histologically, and 6 had no evidence of either neurofibromatosis or fibrous dysplasia. Prognosis and therapy were determined by correlated clinical, radiographic, and histological observations. Fracture before age 2 years carried a poor prognosis. Electron microscopy allowed neither differentiation among these fibrous lesions, nor any clue to their origin, nor did it support the concept of a neural or vascular derivation.

Aseptic necrosis of the humeral trochlea: a case report.

An 8-year-old boy was diagnosed as having unilateral aseptic necrosis of the humeral trochlea on the basis of: loss of elbow carrying angle and decreased range of motion, radiographic demonstration of trochlear collapse and sclerosis. Partial resolution of the radiographic changes occurred within 17 months. In the absence of marked functional impairement, the treatment is conservative clinical observation.

Pepsin-solubilized collagen of human nucleus pulposus and annulus fibrosus.

Human nucleus pulposus and annulus fibrosus, obtained at autopsy from patients 7-30 years of age, were extracted with 2 M guanidine-HCl (pH 5.82) to remove proteoglycans, then stirred with pepsin in 0.5 M acetic acid, followed by three 24-h extractions with 1 M NaCl (pH 7.5) and one 24-h extraction with 2 M KSCN (potassium thiocyanate) (pH 7.2). Pepsin and NaCl solubilized an average of about 30% of nucleus pulposus collagen and 18% of annulus fibrosus collagen. KSCN extracted a further 34% of nucleus pulposus collagen and only 4% of annulus fibrosus collagen. CM-cellulose chromatography of nucleus and annulus collagen purified from the pepsin, NaCl and KSCN supernatants consistently revealed only one peak, always appearing slightly ahead of the alpha1 position for rat tail tendon type I collagen. Polyacrylamide and SDS-gel electrophoresis consistently revealed only one band with the mobility of alpha1 chains. Amino acid composition of collagen from nucleus and annulus is comparable to those of mammalian and avian cartilage type II collagen, and distinctly different from those of rat tail tendonand guinea pig skin type I collagens. Periodate oxidation of nucleus and annulus collagens showed that 81% and 67%, respectively, of the hydroxylysine residues survive treatment, compared to 71% for bovine articular cartilage collagen and 17% for guinea pig skin collagen. Total hexose analysis revealed 1.8 muM and 2.0 muM hexose per muM periodate-stable hydroxylysine in nucleus and annulus collagens, respectively. Ion exchange chromatography showed the presence of glucose and galactose in a ratio of 0.92:1 in nucleas collagen and 1.07:1 in annulus collagen. Pepsin-solubilized, NaCl-extracted collagen from nucleus and annulus formed native-type fibrils in vitro. The banding patterns of ATP-induced segment-long-spacing precipitates of nucleus and annulus collagens were identical to each other and indistinguishable from those of cartilage (type II) collagen, but distinctly different from those of rat tail tendon (type I) collagen. These data suggest that the collagen which can be extracted after limited pepsin attack of human nucleus and annulus is of the form [alpha1 (II)]3.

Congenital posterolateral diaphragmatic hernia past infancy.

Twenty-seven cases of congenital posterolateral diaphragmatic hernia past infancy are reviewed in tabular form and discussed as to presenting symptoms, physical and radiographic findings, operative treatment, and final outcome. A ten year old male treated by us is presented as a detailed case report. A great contrast is noted between the acute respiratory symptoms which threaten life in the infant hernia compared with the more chronic and recurrent gastrointestinal and respiratory symptoms in pateints past infancy. Onset of symptoms did not correlate with age or sex. Chest x-ray films and gastrointestinal contrast studies were most helpful in diagnosis. Abdominal and thoracic approaches were equally effective in reducing the herniated viscera and closing the diaphragmatic defect. We believe that long-term survival of patients with congenital posterolateral diaphragmatic hernia may be due to persistence of a confining pleuroperitoneal sac. Rupture of this sac in later life may coincide with onset of the characteristic symptoms which in turn prompt diagnostic studies. Congenital diaphragmatic hernia must be considered in the differential diagnosis of patients with both recurrent gastrointestinal and respiratory complaints. Plain radiographs of the chest and contrast studies of the gastrointestinal tract are necessary to confirm diagnosis preoperatively.