Chest-wall reconstruction for spondylocostal dysostosis.

A child with respiratory insufficiency due to a lethal syndrome known as spondylocostal dysostosis is described. Severe chest-wall deformities with resultant paradoxical chest-wall motion were successfully corrected by surgical reconstruction of the right thorax. We conclude that patients with the severe lethal form of the syndrome may benefit from aggressive surgical correction of the thoracic abnormality.

Calvarial deformity and remodeling following prolonged scalp expansion in a child.

A main concern in the use of scalp tissue expansion in the pediatric population has been the risk of skull deformation. Little is known about the long-term effects of tissue expansion on the skull and the ability of the skull to remodel following removal of the tissue expander. We report a recent case in which a 5-year-old boy had a fully inflated tissue expander retained under the scalp for a 15-month period. At surgery for removal of the implant the patient was noted to have a profound skull deformity characterized by severe calvarial depression and ridging. The patient underwent scalp reconstruction. Follow-up at 6 months revealed nearly complete remodeling of the skull with minimal visual deformity. This case demonstrates not only the profound bony deformity that can result from tissue expansion, but also the striking ability of the pediatric skull to remodel.

Wegener's granulomatosis involving the urogenital tract.

OBJECTIVE: To report eight cases of limited Wegener's granulomatosis (WG) affecting the urogenital tract (testis, ureter, bladder, urethra and penis) and to emphasize the importance of the anti-neutrophil cytoplasm antibody (ANCA) test in establishing the diagnosis. PATIENTS AND METHODS: Eight patients (six men and two women, aged 41-77 years) were diagnosed with WG, based on their previous medical history, the ANCA test and by biopsy. RESULTS: In each case, there were difficulties and delay in establishing the diagnosis of WG and starting appropriate treatment. The ANCA test was positive in seven cases and helped in establishing the diagnosis, in conjunction with the confirmation of vasculitis and granulomata by biopsy. CONCLUSION: We advocate ANCA testing in patients presenting with limited urogenital disease in association with a past or present relevant history of arthritis, skin vasculitis and/or biopsies showing necrosis or non-specific inflammation.

Successful repair of ectopia cordis using alloplastic materials.

Ectopia cordis is a very rare congenital anomaly associated with a high mortality rate. A successful repair of ectopia cordis with complete absence of sternum was achieved in a two-stage procedure. Initial management consisted of coverage of skin over the malpositioned heart using bilateral pectoral skin flaps. A second more definitive repair was undertaken at age 14 months. Four methyl methacrylate struts were used to reconstruct the anterior chest wall and were then covered with bilateral pectoralis major muscle flaps. At the 2.5-year follow-up there is no evidence of cardiopulmonary compromise and the development of the thorax appears normal. We advise that use of alloplastic materials is a valid option in managing this difficult congenital anomaly.

Expression of the receptor for parathyroid hormone-related protein in normal and malignant breast tissue.

Parathyroid hormone-related protein (PTHrP) is the cause of humoral hypercalcaemia of malignancy and interacts with parathyroid hormone (PTH) receptors. Breast cancer cells produce PTHrP in vitro and in vivo. The breast cancer cell line MCF-7, which products PTHrP and expresses PTHrP receptors, proliferates in response to PTHrP. The aim of these studies was to determine the tissue location of PTHrP/PTH receptors (PTHrPR) in primary breast carcinomas and to establish whether they had the potential to respond to PTHrP. The cellular location of mRNA for the PTHrP/PTH receptor was identified using in situ hybridization in primary breast carcinomas and normal breast tissue. Immunohistochemistry for PTHrP was carried out on the same specimens. Tumours were assessed and scored by two observers using the product of intensity of signal and number of positive tumour cells (possible range 0-9). Tumours were also assessed for Ki-67 expression by counting positive nuclei. Non-malignant ductular epithelium expressed mRNA for the PTHrP receptor (mean score 2.6, range 1-4). Breast carcinomas (mean score 4.4, range 0-9) showed variable expression of PTHrP receptor mRNA: eight tumours were negative, 50 had scores similar to normal breast tissue, and 49 had higher scores for the receptor. Levels of expression of the receptor within the primary breast carcinomas were unrelated to immunohistochemical detection of PTHrP or to any standard prognostic factor. There was a significant (P = 0.05) relationship between Ki-67 and PTHrPR expression in individual tumours. The presence of PTHrP and its receptor in normal breast epithelium and breast carcinomas demonstrates that most breast tumours are able to respond to PTHrP. The Ki-67 data suggest that PTHrP is a potential autocrine growth factor in primary breast carcinoma.

Expression of parathyroid hormone-related protein and its receptor in bone metastases from prostate cancer.

Studies of breast cancer suggest that parathyroid hormone-related protein (PTHrP) is important in the development of bone metastases. To determine whether PTHrP expression is important in prostate cancer metastasis, immunohistochemistry and in situ hybridization were used to assess the expression of PTHrP and its receptor in primary prostate cancer and bone metastases from both prostate and non-prostate cancers. PTHrP was expressed in more prostate primary tumours than bone metastases (p=0.003, Fisher's exact test). All bone metastases from non-prostate cancers expressed PTHrP. In contrast, PTHrP receptor was expressed in all bone metastases, but in only 19% of primary prostate tumours (p=0.001). The receptor to PTHrP was found to be highly expressed in bone metastases from prostate and other primaries, whereas PTHrP protein was found to have lower expression in the bone metastases than in the primary tumours. In conclusion, the expression of the receptor to PTHrP is increased in bone metastases from prostate cancer and may play an important role in their formation.