Long-term survival of the cemented Müller CDH stem: a minimum follow-up of 10 years.

INTRODUCTION: Total hip arthroplasty in patients with altered anatomy of the hip and femur, such as in congenital dysplasia of the hip, is challenging and often requires specially designed stems. Müller straight stems have shown excellent long-term results; however, long-term data on the analogous cemented Müller CDH stem are still missing. The aim of this study was to analyze long-term survival, identify potential risk factors for aseptic loosening, and analyze radiological outcome of the cemented Müller CDH stems. MATERIALS AND METHODS: Between 01/1985 and 06/2005, 95 Müller CDH stems (Zimmer, Winterthur, Switzerland) made up of 3 different materials were cemented using 2 different bone cements: 38 of stainless steel/high-viscosity cement, 31 of a cobalt-chrome-based alloy (CoCr)/low-viscosity cement, and 26 of a titanium-based alloy (Ti)/low-viscosity cement. All patients had a prospective clinical and radiological follow-up according to the standards of our institution. The cumulative incidence for revision of the stem was calculated using a competing risk model. To identify demographic and implant-related risk factors for aseptic loosening of the stem, a multivariate regression model for competing risks was performed. RESULTS: The cumulative risk of revision at 15 years was 12.5% (95% CI 6.6-20.5%) for aseptic loosening of the stem as endpoint, with marked differences for the various stem materials used: stainless steel 2.7% (0.2-12.3%), CoCr 12.9% (4.0-27.3%), and Ti 24.5% (9.6-43.1%). Regression modeling revealed that Ti stems in combination with low-viscosity cement (HR 10.2) and implantation with an axis deviation greater than 3° (HR 3.8) are risk factors for aseptic loosening. CONCLUSIONS: Long-term survival of the cemented Müller CDH stem is comparable to other Müller-type straight stems and uncemented implants. Similar to the original Ti Müller straight stem, the Ti Müller CDH stem also showed an increased risk for aseptic loosening and should, therefore, no longer be used.

Clinicoradiographic presentation of a girl with progressive pseudorheumatoid arthropathy.

We report the case of a girl-child who manifested the clinicoradiographic features of pseudorheumatoid arthritis. 3D-CT scan of the craniocervical junction showed distinctive features of dystopic type of os odontoideum. The report highlights the necessity to explore the craniocervical junction in patients with progressive pseudorheumatoid arthropathy.

Bruck syndrome (osteogenesis imperfecta with congenital joint contractures): review and report on the first North American case.

We describe a patient who was born with flexion contractures and pterygia at the elbows, clubfeet, torticollis, and several rib fractures. During infancy and childhood, multiple fractures of the lower limbs occurred with minimal trauma and led to disabling deformities. When evaluated at age 19 years, he was normally intelligent, but extremely short, with severe kyphoscoliosis compromising his pulmonary function. Pterygia limited elbow extension to 90 degrees, and severe lower limb deformities prevented ambulation. He did not have blue sclerae, dentinogenesis imperfecta, or hearing loss. X-ray studies showed demineralized bones, severe deformity and cystic change at old fracture sites, and vertebral wedging. Collagen studies on skin fibroblasts were normal.

Infantile systemic hyalinosis in siblings: clinical report, biochemical and ultrastructural findings, and review of the literature.

A boy presented at age 3.5 months with joint contractures, restlessness, and pain on handling. His skin was thickened and there were livid-red macular lesions over bony prominences. Infantile systemic hyalinosis (ISH) was diagnosed, a presumably autosomal recessive, progressive, and painful disorder of as yet unknown pathogenesis. Observation over three years confirmed the diagnosis as typical changes, such as nodules on both ears, pearly papules in the perinasal folds and on the neck, fleshy nodules in the perianal region, and gingival hypertrophy, developed. Skin lesions and painful joint contractures progressed in spite of intense physiotherapy, and at age 3, the child had marked motor disability. The central nervous system (CNS) appeared to be intact and the infant showed normal mental development. Radiologic findings included marked generalized osteopenia, osteolytic erosions in the metaphyses of the long bones, and cortical thinning. Electron microscopy of two skin biopsies demonstrated deposition of floccular amorphous substance that was abundant around, and appeared to originate from, small blood vessels in the dermis, partially interfering with collagen fiber formation. Lysosomal inclusions were not seen. Serum acid hyaluronidase activity was within the normal range, and the synthesis of hyaluronic acid and proteoglycans in cultured skin fibroblasts was similar to that of control cells. A younger sister presented at age two months with painful joint contractures and discrete livid-red macules over both malleoli, and showed a similar progression of the disorder over the first year of life. The diagnosis of ISH should be considered in infants and children presenting with painful joint contractures and skin lesions. The pathogenesis of this disabling and disfiguring disorder remains unclear. Our data confirm probable autosomal recessive inheritance, and do not support lysosomal storage, hyaluronidase deficiency, or a primary collagen disorder, but indicate that the amorphous material accumulating in the skin and articular soft tissues may originate from the blood circulation.

The C.C.A. syndrome (congenital contractural arachnodactyly): a new differential syndrome for Marfan's syndrome and homocystinuria.

The first case in the dental literature of congenital contractural arachnodactyly (C.C.A. syndrome) is presented. This newly delineated syndrome is an autosomal dominant heritable disorder of connective tissue. Its similarities to Marfan's syndrome and homocystinuria, as well as other syndromes, are discussed. The lack of cardiovascular disease, specific ocular anomalies, and mental retardation are presented in the differential diagnosis of the C.C.A syndrome with Marfan's syndrome and homocystinuria.

Brachygnathia superior and degenerative joint disease: a new lethal syndrome in Angus calves.

Brachygnathia superior and generalized diarthrodial degenerative joint disease were seen in 17 related, purebred Angus calves ranging in age from 2 days to 4 months. Craniometrical studies revealed decreased maxillary and palatine bone lengths and increased cranial, skull, and facial indices. Radiological evaluation of major appendicular joints demonstrated lipping of the joint margins with osteophyte formation, sclerosis of subchondral bone, and narrowing of joint spaces. Synovial fluid evaluation indicated joint degeneration but no etiologic agent. Rheumatoid factor analysis of plasma was negative. Grossly, all major appendicular joints were defective including the atlanto-occipital articulation. Lesions ranged from loss of surface luster to erosions and deep ulcers with eburnation of the subchondral bone and secondary proliferative synovitis. Histological changes were degeneration of the articular cartilage matrix, chondrocyte necrosis, flaking and fibrillation, chondrone formation, erosions and ulcers of the articular cartilage with subchondral bone sclerosis, vascular invasion with fibrosis, and chronic, nonsuppurative, proliferative synovitis. Growth plates had defective chondrocyte proliferation and hypertrophy with aberrant ossification of calcified cartilaginous matrix. Histochemical analysis of cartilage and bone failed to incriminate which component was defective, glycosaminoglycan or collagen, but indicated different distribution or absence of one or the other. Genealogic studies revealed a genetic basis for the new defect.

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.