Use of an intramedullary rod for the treatment of congenital pseudarthrosis of the tibia.

The use of an intramedullary rod as described by Williams, combined with implantation of an autogenous bone graft, resulted in union of an established congenital pseudarthrosis of the tibia in nine of ten patients. One patient needed additional bone-grafting before union occurred. The average age at the time of the operation was five years and three months.

Identification of a stromelysin cleavage site within the interglobular domain of human aggrecan. Evidence for proteolysis at this site in vivo in human articular cartilage.

Products generated by the digestion of human aggrecan with recombinant human stromelysin have been purified and analyzed by N-terminal sequencing and C-terminal peptide isolation. N-terminal analysis of chondroitin sulfate-bearing fragments revealed a clearly identifiable sequence initiating at residue Phe342 of human aggrecan, providing evidence for a cleavage site at the Asn341-Phe342 bond located within the interglobular domain. This cleavage site, which separates the G1 domain from the remainder of the molecule, was confirmed by isolation from the liberated G1 domain of a C-terminal tryptic peptide with the sequence YDAICYTGEDFVDIPEN (in which the C-terminal residue is Asn341).

Fractures after Wagner limb lengthening.

A retrospective review of our experience with fractures after 27 Wagner limb lengthenings (23 patients) was undertaken. Ten fractures occurred after lengthening in eight patients (six spontaneous, four traumatic). Seven patients fractured despite a "staged" removal of the fixation plate.

Use of the Green transfer in treatment of patients with spastic cerebral palsy: 17-year experience.

We retrospectively reviewed Green procedures and transfer of the flexor carpi ulnaris (FCU) in treatment of spastic forearm pronation, wrist volarflexion, and ulnar deviation deformities. Patient's ages ranged from 3 years 5 months to 16 years 5 months. Surgically, a single volar incision was made and the extensor carpi radialis brevis and/or longus (ECRB, ECRL) were used for insertion.

Post-traumatic spinal cord cysts evaluated by magnetic resonance imaging.

In order to determine a more accurate prevalence of post-traumatic spinal cord cysts (PTSCC) in spinal cord injured (SCI) patients, we retrospectively reviewed magnetic resonance scans from symptomatic imaging and asymptomatic SCI patients. We found the incidence of PTSCC to be 51% in our patient population. The only symptom that correlated to the presence of a cyst was spasticity.

Incidence of brachydactyly and hand exostosis in hereditary multiple exostosis.

Forty-two radiographs and charts of twenty-two patients with a diagnosis of hereditary multiple exostosis and hand involvement were examined to determine the incidence of hand exostoses and association with brachydactyly. An average of 11.6 exostoses were found per hand.

The Bridle procedure: a new treatment for equinus and equinovarus deformities in children.

The Bridle procedure is a tritendon anastomosis between the posterior tibialis, anterior tibialis, and peroneus longus, combined with an Achilles tendon lengthening for treating equinus and equinovarus deformities. The technique avoids problems of tendon attachment to bone and tendon placement for balance. One hundred seven procedures were performed on patients with cerebral palsy with 74% excellent and good results overall.

Immunohistologic abnormalities of the microfibrillar-fiber system in the Marfan syndrome.

Background: Indirect-immunofluorescence studies of skin and cultured dermal fibroblasts from patients with the Marfan syndrome demonstrate apparent deficiency of one element of connective tissue--the microfibrillar-fiber system--in assays using specific antibodies against fibrillin, a major microfibrillar protein. This study was designed to test whether these immunostaining abnormalities are consistent and diagnostic features of the disease.

Methods: We studied patients with either the Marfan syndrome or various other inherited connective-tissue disorders and normal subjects according to a single-blind protocol in which coded samples of skin, fibroblast cultures, or both were analyzed without knowledge of the clinical diagnosis and classified as "Marfan" or "non-Marfan" before the sample codes were broken.

In vivo and in vitro noncovalent association of excised alpha 1 (I) amino-terminal propeptides with mutant pN alpha 2(I) collagen chains in native mutant collagen in a case of Ehlers-Danlos syndrome, type VII.

The cause of the Ehlers-Danlos syndrome Type VII (EDS VII) is considered to be defective removal of the amino-terminal propeptide (N-propeptide) of Type I procollagen due to deficiency of procollagen N-proteinase, the enzyme responsible for the normal proteolytic excision of this precursor-specific domain. Molecules retaining the N-propeptide (pN-collagen molecules) are thought to cause defective fibrillogenesis and cross-linking which eventuate in dramatic joint laxity and joint dislocations, the clinical hallmark of this variety of EDS. Recent studies demonstrate that some EDS VII patients harbor small deletions of either the pro-alpha 1(I) or pro-alpha 2(I) chain of Type I procollagen.

Treatment of slipped capital femoral epiphysis. Spica-cast immobilization.

Thirty-two patients (thirty-seven hips) who had a so-called acute-on-chronic or chronic slipped capital femoral epiphysis were treated with traction for relief of symptoms and then with immobilization in a spica cast for eight to sixteen weeks. The disappearance on radiographs of a metaphyseal juxtaphyseal radiolucency, rather than closure of the physis, was used as the criterion for removing the cast. In one (3 per cent) of the thirty-seven hips, the slip progressed; possibly this could have been prevented by keeping the cast on for a longer period of time.

Cosegregation of elastin-associated microfibrillar abnormalities with the Marfan phenotype in families.

The Marfan syndrome is a serious heritable connective-tissue disorder characterized primarily by ocular, cardiovascular, and musculoskeletal abnormalities but also involving multiple other tissues and organs of the body. Inherited as an autosomal dominant disorder, the etiology and pathogenesis of the Marfan syndrome are presently unknown. We have documented consistent apparent deficient content of elastin-associated microfibrillar fibers by indirect immunofluorescent (IF) studies of Marfan skin, as well as deficient accumulation of related fibrous materials in cultures of Marfan fibroblasts as compared with normal controls and patients with other heritable disorders of connective tissue.

Intraarticular loose bodies in the adolescent hip: results of treatment of those recognized late.

Seven cases of intraarticular loose bodies, previously unrecognized, were treated with surgical removal of the fragment. The presence of an intraarticular loose body was suspected because of persistent pain, restriction of hip joint motion, and asymmetric widening of the medial clear space on an anteroposterior (AP) pelvic roentgenogram. The diagnosis was established by computed tomography (CT) scan.

Cauda equina syndrome after in situ arthrodesis for severe spondylolisthesis at the lumbosacral junction.

Relative stretching of the cauda equina over the posterosuperior border of the sacrum can be found in all patients who have Grade-III or IV spondylolisthesis at the lumbosacral junction. We identified twelve patients, all less than eighteen years old, who had cauda equina syndrome after in situ arthrodesis for Grade-III or IV lumbosacral spondylolisthesis. In all twelve patients, posterolateral arthrodesis had been done bilaterally through a midline or paraspinal muscle-splitting approach.

Results of surgical treatment of talipes equinovarus congenita.

A series of 153 feet in 103 patients were surgically treated for idiopathic clubfeet (mean follow-up period of 10.3 years). Thirty-four percent had prior surgery before referral to the authors' institution.

Transposition of the posterior tibial tendon.

The posterior tibial tendon was rerouted by the technique described by Baker and Hill in 35 feet of children with a dynamic varus deformity due to spastic cerebral palsy. The average follow-up period was 11.4 years.

Idiopathic chondrolysis of the hip.

Fourteen patients (16 hips) with idiopathic chondrolysis of the hip were retrospectively studied at the Shriners Hospital from 1973 to 1986. Follow up averaged 84 months (range 13-180 months). All 14 patients were female.

Chondro-osseous growth abnormalities after meningococcemia. A clinical and histopathological study.

The cases of nine children who survived the acute stage of meningococcal septicemia and secondary disseminated intravascular coagulation were reviewed. All of the children had major orthopaedic problems as a result of the acute disease. Detailed histological studies were performed on specimens of bone and cartilage, obtained when these patients had either acute amputation for gangrene or subsequent revision for a chondro-osseous deformity.

Immunological reactivity towards chondrocytes in rat and man: relevance to autoimmune arthritis.

Immunological investigation of articular chondrocytes obtained from rat and man have shown the presence of unique differentiation antigens on the cell surface demonstrated by poly- or monoclonal antibodies in both species, and by the analysis of T cell reactivity in the rat. Both species of chondrocytes express Class I antigens in common with all nucleated mammalian cells and, in man, individual specificities of the MHC A, B, and C locus can be identified using standard histocompatibility testing. Class II antigens are strongly expressed in the rat but are expressed poorly in the human specimens we have analyzed.

A cysteine for glycine substitution at position 1017 in an alpha 1(I) chain of type I collagen in a patient with mild dominantly inherited osteogenesis imperfecta.

Osteogenesis imperfecta (OI) is a heterogeneous group of inherited diseases of connective tissue manifested primarily by excessive fracturing of bone but associated with other abnormalities such as blue sclera, thin skin, herniae, ligamentous laxity, reduced stature and hearing loss. We report here molecular studies on a patient with the mild, dominantly inherited, variety of OI (OI type I) previously shown (Nicholls et al., 1984) to be heterozygous for an abnormal alpha 1(I) chain of type I collagen which contained cysteine near the carboxyl terminus (Steinmann et al.

Multidisciplinary team approach to the pediatric burn patient.

Burn trauma is the most devastating injury the body can sustain. Each year, 100,000 victims will sustain burn injuries serious enough to require hospitalization; 35% of these victims are children. The Shriners Hospitals for Crippled Children Burns Institute, Cincinnati Unit (SBI-Cincinnati), is a 30-bed hospital with all of the support systems of a major university medical center.

Post-traumatic fracture of polymethylmethacrylate surrounding a Harrington hook site. A radiolucent cause of instability and pain.

As polymethylmethacrylate (PMMA) is used more often in spine surgery, problems associated with its use are likely to be encountered. A case is presented of a young patient with myelomeningocele and scoliosis. The patient presented with instability and pain associated with fractured PMMA surrounding the lower Harrington hook site.

Grice subtalar arthrodesis followed to skeletal maturity.

A retrospective review of 45 patients (62 feet) who had undergone a Grice subtalar arthrodesis and who had reached skeletal maturity was undertaken. Preoperative deformities were due to flaccid and spastic paralysis, as well as congenital abnormalities. There were failures in 32% and poor results in 61%.