In order to compare the long-term results of full-thickness and split-thickness skin grafts after the correction of congenital syndactyly, 27 patients have been investigated after an average follow-up of 21 years. Post-operative functional and cosmetic results have been assessed by patient records, questionnaires and physical examination. The webs that had received split-thickness grafts showed more flexion and extension lags and the overall spreading of the operated fingers was significantly decreased compared to the control fingers. On the other hand, more re-operations because of web creep had to be performed after full-thickness grafts. Hyperpigmentation and hair growth in the grafts was found in most of the full-thickness grafts, while breakdown of the graft was found in some of the split-thickness grafts. Therefore, based on the results of this study, either full- or split-thickness skin grafts can be used when treating of congenital syndactyly.
Hepatic osteodystrophy is a complication of chronic liver disease and bone mass is known to decline further in the first year after liver transplantation. The present study focused on bone mineral density (BMD) between 1 and 15 years after liver transplantation under a prednisolone- and azathioprine-based immunosuppressive regimen. Three groups of adult patients were studied: group 1, 45 patients with a follow-up of 5-9 years after transplantation, had BMD measurements done at 1, 2 and 5 years after transplantation; group 2, 17 patients with a follow-up of 10-14 years, had BMD measurements done at 5 and 10 years; group 3, 4 patients with a follow-up of more than 15 years, had BMD measurements done at 10 and 15 years. BMD of lumbar spine (L1-L4) and proximal femur was measured using dual-energy X-ray absorptiometry, and at the same time radiographs of the spine and hips were made. Spinal BMD increased significantly, during the second post-transplant year; subsequently no significant changes were seen. Proximal femur BMD decreased slightly, but significantly during the second year, and remained stable afterwards. About one-third of patients had a BMD below the fracture threshold (= 0.798 g/cm2 for the lumbar spine and 0.675 g/cm2 for the hip) during the follow-up. In 5 of the 66 patients studied, new vertebral fractures occurred. No fractures or avascular necrosis of the hips were seen. Furthermore, after transplantation lower Z-scores of the hip were found in patients with pre-transplant cholestatic liver diseases, and lower Z-scores of the lumbar spine were found in men compared with women. Long-term follow-up of BMD up to 15 years after transplantation revealed an improvement mainly in the second postoperative year with no deterioration afterwards. Nevertheless a substantial number of patients (around one-third) kept a BMD below the fracture threshold, and new fractures may occasionally occur. The overall outcome appeared somewhat less favorable in men and patients transplanted for cholestatic liver diseases.
In 1841, Sir Alfred Poland described congenital deficiency of the pectoralis major muscle associated with brachysyndactyly. In this report a description of the variety of Poland's syndrome is presented by reviewing 23 patients treated in three different hospitals in The Netherlands. Sixteen patients were subjected to a meticulous physical examination and the medical records of all patients were reviewed. There were 15 males and 8 females in our group. Nineteen patients had right-sided Poland's syndrome, four left-sided. Two patients were related to each other (mother-daughter). The severity of the syndrome varied from mild hypoplasia of the pectoralis major muscle and mild hypoplasia of the hand to aplasia of the pectoralis major and serratus anterior muscles, rib and sternal defects, aplasia of the breast, and complete aplasia of the radius and hand. Thirteen patients required surgery for hand, chest, or breast deformities, ranging from 1 to 8 operations per patient. This study shows that Poland's syndrome is an extremely varied condition, which appears to involve an entire upper quadrant. The milder forms may easily be overlooked and do not require surgical intervention. The more severe cases may require hand, chest wall, and/or breast reconstruction. In general, expert specialist attention in a multidisciplinary setting is required for patients with this condition.
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