Periampullary tumours: advances in diagnosis and surgical treatment.

Forty-five carcinomas of the region of the ampulla of Vater were resected at the Toronto General Hospital during a 16-year period. In 26 the presenting symptom was pain and in 32 it was jaundice. The most useful means of investigation was endoscopic retrograde cholangiopancreatography. Angiography was of value in determining resectability prior to operation, and percutaneous aspiration biopsy allowed a preoperative diagnosis in the case of large pancreatic tumours. In 39 patients who had a Whipple procedure the operative mortality was 8%. Total pancreatectomy performed in two patients and local excision of ampullary carcinoma in four patients were attended by no operative deaths. Long-term survival was best in patients with ampullary carcinomas and worst in those with pancreatic cancer.     

Surgical treatment of varicocele

Varicocele as a major cause of male infertility has been well established. Increasing numbers of this operation are being performed yearly. We describe a simple technique utilizing local anesthesia which significantly reduces the risks of the operation and hospital stay. Its impact on cost effectiveness is discussed.     

Partial deletion of the long arm of chromosome 4: a clinical syndrome

Partial deletion of the long arm of chromosome 4 at q31 results in a clinical syndrome of mental retardation, characteristic ears, facial bone hypoplasia, cleft palate very prone to scarring on repair, and specific hand abnormalities. A female, aged 9 years, is described and compared with six other reported cases.     

Dysplasia of the odontoid process in Morquio's syndrome causing quadriparesis.

Eleven patients with documented Morquio's syndrome who had dysplasia of the odontoid process and resulting atlanto-axial instability were reviewed. They were found to be at risk for acute traumatic quadriparesis, chronic myelopathy of a variable and often rapid rate of progression, and sudden death by respiratory arrest. The evidence suggests that early prophylactic posterior cervical fusion is indicated for patients with this malformation and that once quadriparesis is established, recovery of function is limited.     

Porcine pancreatin as a source of salmonella infection in children with cystic fibrosis

Contamination of a powdered preparation of pancreatin with Salmonella schwarzengrund and S. eimsbuettel resulted in the infection of at least 31% of one group of paediatric patients with cystic fibrosis. The pancreatin contained very small numbers of Salmonellae, the infecting dose in at least one child being less than 44 organisms. More stringent bacteriological standards are needed for pharmaceuticals and foods used by paediatric patients.