Obesity and joint replacement.

There has been considerable discussion as to the influence of obesity on the indications for, and the outcome after, joint replacement. Attempts have been made to withhold funding for such procedures in those who are overweight. What is the justification for this? This editorial examines the current evidence concerning the influence of obesity on joint replacement and suggests that it is only in the morbidly obese, with a body mass index > 40 kg/m(2), that significant contraindications to operation are present.

Load cell analysis following silastic arthroplasty of the hallux.

The first metatarsophalangeal joint was replaced by a flexible silastic hinge prosthesis in 33 joints of 25 patients. Most patients were pleased with the operation but one third had some discomfort under the second and third metatarsal heads. We used a computer linked load cell matrix to analyse the forces under these feet during normal walking. After silastic arthroplasty there was a rise of 65% in peak load under the second and third metatarsal heads compared with that found in the opposite feet which had not undergone operation (P less than 0.001). This was associated with a fall in peak load under the hallux of 43% (P less than 0.03) compared to normal and a less significant fall averaging 23% in the peak pressure experienced under the first metatarsal head. There was a rise in peak heel loading and a reduction in the normal pronation which occurs during the stance phase of walking.

Dysplasia epiphysialis hemimelica. A clinical and genetic study.

The clinical features of nine new patients with dysplasia epiphysialis hemimelica are reported, with a long-term follow-up on a further seven patients who were described in the earliest case reports of this disease. Each of these 16 patients had only one leg involved, but 12 had multiple epiphyses affected. The distal femur, distal tibia and talus were the commonest sites and most patients presented with painless swelling or deformity. Wasting of the muscles of the affected leg was a common finding, and was occasionally disproportionate to the degree of disuse. One patient had the unique combination of involvement of the lateral and medial halves of different epiphyses in the same limb and another had unusual metaphysial changes. Diagnosis was often delayed despite typical radiographic appearances. There was no evidence for a genetic component in the aetiology nor was any common environmental factor identified. Treatment by local excision was generally effective for lesions in the vicinity of the knee, but some patients with involvement of the talus required arthrodesis around the ankle. The long-term prognosis appears to be good and so far only two of these patients have developed premature osteoarthritis.

Spondylocostal dysostosis in South African sisters.

The clinical and radiographic features of two sisters with a severe form of spondylocostal dysostosis (SCD) are described and depicted Autosomal recessive inheritance is likely.

Infantile metaphysial dysplasia or "battered babies"? A reassessment of material in the Fairbank Collection.

The Fairbank Collection in the Radiology Department of the Royal National Orthopaedic Hospital contains details of 15 patients in the section labelled "Metaphysial Dysplasia and Dysostosis". This material has been reviewed, long-term follow-up of the patients carried out and the diagnosis revised, where indicated, according to current concepts. Of the 15 patients, all children, seven had recognisable bone dysplasias. The remaining eight, all listed under the heading "metaphysial fragility", had the characteristic radiographic features of "battered" babies.

Craniometaphyseal dysplasia--variability of expression within a large family.

Fifteen individuals in five generations of a kindred with branches in South Africa and England had the autosomal dominant form of craniometaphyseal dysplasia. The majority of affected adults had mild to moderate mandibular distortion, while paranasal bossing was a transient manifestation in childhood. Facial palsy with onset in childhood was present either unilaterally or bilaterally in about 30% of the patients, while 50% had auditory dysfunction which varied from mild impairment of hearing to total deafness. Stature and intellect were normal, and bone fragility, osteomyelitis and dyshaemopoesis were not features of the condition. Five other potentially affected family members had deafness or facial palsy of uncertain aetiology in the absence of other stigmata of CMD. It is uncertain whether these complications represent minor degrees of phenotypic expression of the abnormal gene.

Osteopathia striata with cranial sclerosis. An autosomal dominant entity.

The syndromic association of striae in the long bones and pelvis, together with sclerosis of the base of the skull, has been investigated in four families. Impairment of hearing and alteration in the shape of the head are the most important clinical manifestations. Spinal abnormalities are an inconsistent feature. The distribution of affected individuals in the kindreds is compatible with autosomal dominant inheritance.

"Osteopetrosis" in the Fairbank Collection.

The "osteopetrosis" section of the Fairbank Collection in the Radiology Museum of the Royal National Orthopaedic Hospital contains radiographs and case notes of twenty-two patients. This material has been reviewed in terms of modern concepts in an attempt to obtain a long-term follow-up and a firm diagnosis in each individual. Nine patients proved to have the classical autosomal dominant form of osteopetrosis, four had the malignant autosomal recessive type, craniometaphyseal dysplasia was present in two kindreds and isolated individuals had pyknodysostosis, atypical craniodiaphyseal dysplasia and craniosclerosis with osteopathia striata. As these conditions differ greatly in their clinical and genetic prognoses, diagnostic categorisation is of practical importance.

A review of the osteopetroses.

The osteopetroses are a group of conditions which are characterized by varying combinations of bony sclerosis and modelling defects. Classical osteopetrosis may be inherited as an autosomal dominant or autosomal recessive: the former variety is benign, heterogeneous and comparatively common, while the latter is precocious, potentially lethal and rare. Many other craniotubular dysplasias and hyperostoses are loosely grouped with the osteopetroses. The commonest of these is the autosomal dominant form of craniometaphyseal dysplasia, while the others which are well known include Pyle disease, and van Buchem disease. Sclerosteosis is a progressive condition in which massive cranial thickening is associated with syndactyly and gigantism. Each of these disorders has specific clinical and radiographic features, which permit recognition. Diagnostic accuracy is crucial for treatment, prognostication and effective genetic management.