Children with a mucopolysaccharidosis or mucolipidosis suffer progressive disability of the hands, particularly in relation to dysfunction of the median nerve. This is an increasing problem because bone-marrow transplantation has dramatically improved survival without apparently changing the musculoskeletal manifestations. We have reviewed 48 children with these syndromes who required carpal tunnel decompression, recording symptoms, signs, radiological, electrophysiological and operative findings, histology and upper-limb function. In these children the carpal tunnel syndrome differs from that seen in adults. Symptoms are rare but signs such as decreased sweating, pulp atrophy, thenar wasting and manual clumsiness are much more common. At operation, the flexor retinaculum was thickened and a mass of white tenosynovium engulfed the flexor tendons. Most patients had some definite nerve constriction with a thickened epineurium. Functional improvement was seen after early decompression, with some benefit from simultaneous tendon release. Regular physiotherapy helped to maintain increased hand movement. We describe our assessment protocol, the physiotherapy and operative regime and the standard functional review which helps to maximise function in the hands and upper limbs of these children

1. Dissections have been made of the cervico-brachial region. 2. The possible causes of the scalenus anterior syndrome are discussed and an attempt is made to relate them to the anatomical findings. 3. It is suggested that a more suitable description is the "first rib syndrome," and that the most logical treatment is to remove part of the offending first rib

1 . Necropsy and dissection findings of a man of fifty-two who died from nail-patella syndrome are recorded. 2. The significance of the iliac horns are discussed and the literature concerning their development is reviewed. 3. Other anomalies are reviewed and the incidence of hereditary nephropathy is discussed. 4. The genetic origin ofthe syndrome is outlined but the involvement of such varying tissues remains unexplained

Morbid obesity and its association with obstructive sleep apnoea syndrome have been increasingly recognised in children. Orthopaedic surgeons are often the primary medical contact for older children with tibia vara, which has long been associated with obesity, but are unfamiliar with the evaluation and treatment of sleep apnoea in children. We reviewed all children with tibia vara treated surgically at one of our institutions over a period of five years. Thirty-seven patients were identified; 18 were nine years of age or older and 13 of these (72%) had morbid obesity and a history of snoring. Eleven children were diagnosed as having sleep apnoea on polysomnography. The incidence of this syndrome in the 18 children aged nine years or older with tibia vara, was 61%. All these patients required pre-operative non-invasive positive-pressure ventilation; tonsillectomy and adenoidectomy were necessary in five (45%). No peri-operative complications related to the airway occurred. There is a high incidence of sleep apnoea in morbidly obese patients with tibia vara. These patients should be screened for snoring and, if present, should be further evaluated for sleep apnoea before corrective surgery is undertaken

A review of 77 patients with traumatic central cord syndrome revealed that atypical variations are more common than the existing literature suggests and that these may be seen in a wide variety of acute injuries to the cervical spine. In general the outcome is good; a favourable prognosis on admission is suggested by good hand function, hyperpathia, Lhermitte's sign and normal perianal sensation. The study has highlighted the value of regular muscle charting and has cast doubt on previous neuroanatomical assumptions about the syndrome

Aims

The purpose of this study was to evaluate the mid-term outcomes of autologous matrix-induced chondrogenesis (AMIC) for the treatment of larger cartilage lesions and deformity correction in hips suffering from symptomatic femoroacetabular impingement (FAI).

It has been known for well over a century that the corresponding ossification centres of the hand tend to appear before those of the foot (Mall 1906), although even now the range of variability remains poorly defined. Presumably a similar asynchrony also obtains for chondrification, although precise timing is more difficult here than for ossification. Accordingly, it is tempting with respect to this syndrome to relate fusions restricted to the phalanges of the hand and to the tarsal bones to the action of a gene which during a very limited period of development exerts an effect on those small bones of the hand and foot which are in a very specific stage of development. But since there are other inherited abnormalities of the skeleton, such as brachydactyly, where homologous bones are involved, it is clear that at the descriptive level gene action can either appear to be "stage-specific" or "homologue-specific." There are also mutations affecting the axial skeleton such as the type of polydactyly of the foot described by Neel and Rusk (1963) which appear to be neither "stage" nor "homologue" specific, involving only the foot. Finally then, there are syndromes, such as Lawrence-Moon-Biedl-Bardot, in which involvement of the hands and feet (polydactyly) is associated with such other apparently completely unrelated defects as retinitis pigmentosa and hypogonadism. While it is tempting to try to construct theoretical patterns and systems of developmental processes on the basis of results such as these, it is clear that the ultimate understanding of how genes can appear to act in so many ways almost certainly depends on the identification of a genetically controlled, qualitative or quantitative biochemical lesion. With the current parallel developments in biochemical and developmental genetics, new insights into these enigmas will surely be forthcoming in the relatively near future

1. Thirty-four patients with severe lumbo-sacral subluxation have been studied. Twenty-nine of these came for advice between the ages of nine and nineteen, and of these, twenty-five developed symptoms and signs of a characteristic syndrome. 2. The details of the syndrome are described: the essential features are spinal stiffness, a lordotic gait, resistance to straight leg raising, and in some cases evidence of interference with cauda equina or nerve root. 3. The danger of attempted reduction by traction is stressed, as well as the difficulties to be encountered during posterior lumbo-sacral fusion. 4. The reasons for operating are given; the results of spinal fusion were satisfactory. 5. The traditional apprehension concerning the effect of severe subluxation on childbirth has probably been over-stressed. 6. The tendency to slip was almost completely arrested by spinal fusion

This paper describes how we came to understand the pathophysiology of Volkmann’s ischaemic contracture with references to relevant papers in this Journal, and the investigation and management of acute compartment syndrome is briefly discussed

We describe ten patients with Turner’s syndrome (karyotype 45, XO) who had leg lengthening for short stature. A high incidence of postoperative complications was encountered and many patients required intramedullary fixation as a salvage procedure. We discuss the reasons for this and highlight the differences between our findings and those of a similar series recently reported. In view of the considerable difficulties encountered, we do not recommend leg lengthening in Turner’s syndrome

1. From India a family is reported in which fifteen of fifty-seven were affected by the nail-patella syndrome. 2. Additional coincidental features not described previously were fiexion deformities of both hips and hyperextension of the interphalangeal joints of the fingers. 3. Patients affected by the condition do not always show the same blood group

From 1975 to 1988, operative treatment was performed on 50 feet in 45 patients with tarsal tunnel syndrome. The causes of this syndrome were correlated with operative findings and included ganglia in 18, and a bony prominence from talocalcaneal coalition in 15. Five feet had sustained an injury, tumours were found in three and there was no obvious cause in nine. In most cases in need of operative treatment, there was a space-occupying lesion. Classifying the results according to causes, those with coalition or a tumour fared better, and idiopathic and traumatic cases had a worse outcome. In cases with a definite lesion, an excellent result can be expected from surgical treatment carried out soon after onset of the condition

The acetabular rim syndrome is a pathological entity which we illustrate by reference to 29 cases. The syndrome is a precursor of osteoarthritis of the hip secondary to acetabular dysplasia. The symptoms are pain and impaired function. All our cases were treated by operation which consisted in most instances of re-orientation of the acetabulum by peri-acetabular osteotomy and arthrotomy of the hip. In all cases, the limbus was found to be detached from the bony rim of the acetabulum. In several instances there was a separated bone fragment, or 'os acetabuli' as well. In acetabular dysplasia, the acetabular rim is subject to abnormal stress which may cause the limbus to rupture, and a fragment of bone to separate from the adjacent bone margin. Dysplastic acetabuli may be classified into two radiological types. In type I there is an incongruent shallow acetabulum. In type II the acetabulum is congruent but the coverage of the femoral head is deficient

Degenerative changes of the knee often cause loss of extension. This may affect aspects of posture such as lumbar lordosis. A total of 366 patients underwent radiological examination of the lumbar spine in a standing position. The knee and body angles were measured by physical examination using a goniometer. Limitation of extension of the knee was significantly greater in patients whose lumbar lordosis was 30° or less. Lumbar lordosis was significantly reduced in patients whose limitation of extension of the knee was more than 5°. It decreased over the age of 70 years, and the limitation of extension of the knee increased over the age of 60 years. Our study indicates that symptoms from the lumbar spine may be caused by degenerative changes in the knee. This may be called the ‘knee-spine syndrome’

1. A case of the scapho-capitate fracture syndrome is described. 2. At operation the proximal fragment of the capitate bone was found to have rotated on a transverse and not on a vertical axis

1. Metabolic balance studies in two cases of the Fanconi syndrome are presented. 2. The actions of sodium bicarbonate and calciferol on the calcium and phosphorus balance were observed separately in the two cases. 3. The results show that sodium bicarbonate alone corrects acidosis and decreases the loss of calcium in the urine. 4. Calciferol in high dosage will increase intestinal absorption of calcium and phosphorus, but the urine calcium excretion then increases and vitamin D alone does not, therefore, give a positive balance adequate for complete healing and normal growth. 5. Alkalies and calciferol together put these cases into strongly positive calcium and phosphorus balance and promote healing of rickets, osteomalacia and pseudo-fractures. 6. Large doses of sodium bicarbonate in tablet form correct acidosis, do not adversely affect intestinal absorption of calcium, and facilitate accurate dosage and convenient administration. 7. Alkali therapy may lower serum potassium and precipitate symptoms of hypokalaemia in potassium-losing patients. This is thought to have been the cause of symptoms in several cases reported in the literature

1. A case of ischaemic necrosis of the peroneal muscles (peroneal compartment syndrome) is described. Recovery or regeneration of a seemingly necrotic peroneus brevis was noted. 2. The similarity between peroneal compartment syndrome and the anterior tibial syndrome is noted. 3. Treatment by early fasciotomy is advised

1. A case of Fanconi's syndrome, with evidence of renal tubular dysfunction, is reported. 2. Healing of the rachitic lesions followed massive doses of calciferol. There was simultaneous improvement in the biochemical state. This case is probably unique in that the amino-aciduria disappeared. 3. Two-stage osteotomies were undertaken for the correction of severe bowing of the tibiae. 4. No toxic symptoms have developed so far from the calciferol therapy