Mesomelic limb shortness: a previously unreported autosomal recessive type.

We report on sibs, the offspring of a consanguineous mating, whose mesomelic shortness and bowing of limbs with associated skin dimpling, retrognathia, mandibular hypoplasia, cleft palate, and camptodactyly represents a previously apparently unreported syndrome. The radiological findings are discussed, particularly with regard to the main known diagnostic possibilities.

A review of 240 patients undergoing distraction osteogenesis for congenital post-traumatic or postinfective lower limb length discrepancy.

BACKGROUND: We reviewed 281 lower limb lengthenings in 240 patients treated at three centers for congenital, post-traumatic, or postinfective limb length discrepancy (LLD) in the period 1984 to 1992. STUDY DESIGN: A retrospective review was done of hospital charts and radiographs of patients who had completed lengthening at least 12 months prior to the beginning of the study. The variables studied included patients' age at operation, the bone segment lengthened, whether a corticotomy or an osteotomy had been done, the amount of lengthening planned and achieved, the level(s) of corticotomy or osteotomy, the external fixator used, and the difficulties encountered. RESULTS: Limb length discrepancy was reduced within 2 cm (1.6 percent or lower) of the normal contralateral leg in 249 (89 percent) lengthenings in 208 patients. The average time spent with the fixator in situ was 186.4 days (range, 94 to 617 days), with an average healing index of 35.3 days per cm (range, 26 to 43 days per cm). Femoral osteotomies or corticotomies healed faster than tibial osteotomies or corticotomies. The healing index in post-traumatic and postinfective LLD was significantly lower than in congenital conditions. The younger patients showed a significantly decreased time to bone healing and fewer complications. CONCLUSIONS: A significantly greater number of difficulties were found in patients whose lengthening exceeded 18 percent of the original length of bone. Bifocal lengthenings healed significantly faster than single level ones. The three external fixators used were equally effective for lengthenings of less than 20 percent. The Ilizarov and the Monticelli-Spinelli circular fixators were associated with a significantly decreased number of complications when lengthenings above 20 percent were performed.

Local control of Ewing's sarcoma: an analysis of 67 patients.

Local control of Ewing's sarcoma was analysed in a series of 67 patients treated by surgery and/or radiotherapy as well as combination chemotherapy. Radiotherapy was employed with or without surgery in 60 patients and produced an overall local control rate of 55%; complete excision of the primary lesion seemed to be beneficial. There was a marked variation in control rates depending on the site of the primary lesion: limb 85%, rib 53%, pelvis 31% and other sites 33%. Primary tumours greater than 10 cm in diameter were significantly less likely to be controlled. Using daily fractions of approximately 180 cGy, total doses in excess of 6000 cGy seem more likely to produce serious late morbidity and may not increase the local control rate. No cases of second malignancy arising in irradiated tissue have been observed to date, but one patient developed acute lymphoblastic leukaemia.

Anterior and posterior displacement of the hip after innominate osteotomy.

Ten patients were treated for anterior or posterior displacement of the hip after an innominate osteotomy for congenital dislocation of the hip. All required a repeated open reduction with an additional procedure, either at the same time as reduction or as a second stage. Stable reduction was achieved in eight cases, but in two the initial attempt failed and the operation had to be repeated. The clinical features of hips with this complication are described and the technique of the salvage operation is discussed.

Lengthening of the calcaneal tendon in spastic hemiplegia by the White slide technique. A long-term review.

For equinus deformity in spastic hemiplegia, correction by the White slide technique has been studied in a group of 35 patients followed up for 14 to 20 years. It is a simple, effective method of lengthening the calcaneal tendon, and is free from significant complications with an acceptable rate of recurrent deformity. The majority of patients achieved a heel-toe gait.

Rotation osteotomy of the humerus for birth injuries of the brachial plexus.

The use of a rotation osteotomy of the humerus in 10 patients with disabling loss of lateral rotation at the shoulder due to brachial plexus birth injuries is described. The operative technique and complications are discussed. Before operation only one patient was independent, but after operation nine of them were. Restoration of lateral rotation at the shoulder can improve the function, the appearance and the independence of these handicapped patients.

Management and outcome in tibial dysplasia.

Tibial dysplasia is a rare congenital deformity which must be distinguished from the more common fibular dysplasia. We have reviewed 24 patients with 35 affected legs. The classification system of Kalamchi and Dawe (1985) was found to be preferable to that of Jones, Barnes and Lloyd-Roberts (1978) as a guide to prognosis and management. We discuss the outcome of surgical treatment, recommending selective amputation for most cases.

Fibular hypoplasia with absent lateral rays of the foot.

We describe 11 children with fibular hypoplasia and three- or four-ray feet, two bilaterally. This deformity is a less severe form of the better known congenital short tibia with absent or dysplastic fibula. If the leg-length discrepancy is minor, no treatment or a simple orthosis are used. Epiphysiodesis or leg lengthening by callotasis is appropriate for mild to moderate discrepancy. If the foot is non-functional, or the projected leg-length discrepancy is too great, early amputation and a prosthesis are advised.

The treatment of subluxation of the hip in children over the age of four years.

Subluxation of the hip presenting for the first time in a child over the age of four years is rare. We report ten cases treated over nearly 11 years by the senior author (JAF). We describe the surgical procedures and the results, at maturity, of nine of the ten patients. At a mean follow-up of nearly nine years, the clinical outcome was good in all ten children by the criteria of Ponseti. Radiological assessment showed that three hips remained subluxed, and that four had avascular necrosis of the physis. We advise a one-stage procedure, correcting both the femur and acetabulum.

Congenital convex pes valgus.

Congenital convex pes valgus (congenital vertical talus) is a rare condition. We reviewed ten feet in seven patients who had had surgical correction. All had been operated on by the senior author (JF) and the same surgical technique was used throughout, incorporating transfer of the tibialis anterior to the neck of the talus. The mean age at surgery was 31 months and the mean follow-up was nine years (6 to 14). All patients completed a questionnaire and had clinical, radiological and photographic evaluation performed by an independent examiner. None had required further surgery. All but one were satisfied with the result, and had no functional limitations. They all wore normal shoes. The mean ankle dorsiflexion was 17 degrees and plantar flexion 21 degrees. The mean arc of subtalar motion was 27 degrees. All radiological parameters measured were within the normal range, although irregularity of the talonavicular joint was common. No avascular necrosis of the body of the talus was seen. We conclude that the medium-term results of this procedure are very satisfactory.

Ultrasonographic monitoring of limb lengthening.

Limb lengthening in nine patients was monitored by radiographs and by ultrasound scans. The distraction gap appeared as a sonolucent area within which echogenic foci developed soon after distraction commenced. By seven weeks a new cortex was detected, and medullary canal began to develop between seven and eight weeks. Ultrasound scanning can be used to measure distraction, but it was not as useful as radiographs in detecting angulation. Its use in patients undergoing limb lengthening could reduce their exposure to radiation.

Horizontal pelvic osteotomies for bladder exstrophy. A preliminary report.

We have treated 11 patients aged three days to 15 years with bladder exstrophy by horizontal osteotomies of the innominate bones. The operation was originally used for older patients with severe deformity or failed previous surgery but is now applied as a primary procedure in the first week of life. The osteotomies enable the complex malformations to be corrected in a single operation without turning the patient: the pubic bones can be brought together, the abdominal wall repaired and the bladder closed with reconstruction of the urethra and external genitalia. The early results have been very satisfactory in all cases with only minor complications; we felt that a preliminary report should be made, despite a mean follow-up of only seven months.

Injuries to the physis by extravasation. A rare cause of growth plate arrest.

We report two cases of deformity of the ankle caused by physeal damage secondary to extravasation of an intravenous infusion in infancy. The possible mechanisms of injury to the physis are discussed.

Acetabuloplasty in the treatment of congenital dislocation of the hip.

The results in 102 acetabuloplasties have been assessed in eighty-six patients, with a minimum follow-up of five years. Clinical and radiological assessment was carried out according to a score system very similar to that used in previous studies, and the results were expressed as excellent, good, fair and poor according to the score obtained. Any hip requiring further stabilising procedures was automatically grouped as a failure. Subtrochanteric femoral ostestomy was used when femoral anteversion was excessive. The results revealed that 73 per cent of the hips were satifactory (excellent or good) overall, but that a steady deterioration was evident when the five- and fifteen-year groups were compared. Few of the hips were regarded as normal on radiological grounds. Homologous bone bank rib was found to be the most satisfactory graft material. Coxa magna was the most common abnormality that was consistent with a good or excellent result. Avascular necrosis of the femoral head was found in only six hips; it was more commonly present in patients subjected to previous operation. Concentric reduction of the femoral head is essential if acetabuloplasty is to maintain stability of the hip.

Talectomy for arthrogryposis multiplex congenita.

Eighteen patients (34 feet) with arthrogryposis multiplex congenita treated by talectomy for rigid equinovarus deformity were reviewed. The average follow-up was 11 years. Twenty-four feet (71%) were considered satisfactory; the remainder were improved. Seven feet required further operations to correct recurrence of the deformity, but finally all could be fitted with boots or shoes and all patients could walk. The history of talectomy is reviewed and the operative details described.

Distal fibulectomy for Ewing's sarcoma.

We treated five children with non-metastatic Ewing's sarcoma of the distal fibula by distal fibulectomy. At a mean of eight years after surgery they all had nearly normal levels of painfree function. Distal fibulectomy is a good alternative to the more radical amputations recommended in the past.

Revision of failed open reduction of congenital dislocation of the hip.

We report a review of 33 hips (32 patients) which had required repeat open reduction for congenital dislocation of the hip. They were followed up for a mean of 76 months (36 to 132). Factors predisposing to failure of the initial open reduction were simultaneous femoral or pelvic osteotomy, inadequate inferior capsular release, and inadequate capsulorrhaphy. Avascular necrosis had developed in more than half the hips, usually before the final open reduction. At review, 11 of the hips (one-third) were in Severin grade 3 or worse; five had significant symptoms and only ten were asymptomatic and radiographically normal. Once redisplacement has occurred after primary open reduction, attempts to reduce the head by closed means or by pelvic or femoral osteotomy are usually unsuccessful and a further open reduction is necessary.

The prognosis for walking in osteogenesis imperfecta.

We report a postal survey of 59 families of children with osteogenesis imperfecta. From the 51 replies we collected data on developmental milestones and walking ability and related them to the Sillence and the Shapiro classifications of osteogenesis imperfecta. Twenty-four of the patients had been treated by intramedullary rodding. Both classifications helped to predict eventual walking ability. We found that independent sitting by the age of ten months was a predictor for the use of walking as the main means of mobility with 76% attaining this. Of the patients who did not achieve sitting by ten months, walking became the main means of mobility in only 18%. The developmental pattern of mobility was similar in the rodded and non-rodded patients.

A review of the Dillwyn Evans type collateral operation in severe club feet.

A modified form of the collateral operation originally described by Dillwyn Evans has been used in severe relapsed club feet to correct the sagittally breached or bean-shaped foot. Forty-five feet in 37 patients were followed up for an average of nine years and nine months. Previous operations had been performed on 42 feet. Thirty out of 45 feet were considered satisfactory at review when evaluated by a simple, functional system of scoring. The majority of the feet were stiff but relatively free of pain and able to fit into normal shoes. The collateral operation was considered successful in 42 feet if the patient's ability to take part in any desired sporting and recreational activities was taken as the criterion for success.

The natural history and management of congenital short tibia with dysplasia or absence of the fibula.

Forty-three patients with unilateral congenital short tibia with partial or complete absence of the fibula are reviewed. The factors influencing the degree of leg shortening at maturity are considered. Serial radiographic measurements of leg length in fourteen patients covering an average observation period of 9-3 years support the hypothesis that the relative difference in growth between the two limbs remains remarkably constant. By estimating the percentage difference between the normal and abnormal leg lengths on the first measurable radiograph it is therefore possible to predict the likely shortening at maturity. This method of prediction allows the surgeon to make the decision to proceed to Syme's amputation or to the use of an extension prosthesis at about one year of age when the child starts to walk.