Budd-Chiari Syndrome-A Single Center Experience From the United Kingdom.

Pediatric Budd-Chiari syndrome (BCS) is a rare cause of portal hypertension and liver disease in Europe and North America. In order to understand the long-term effect of radiological intervention on BCS we performed a single center retrospective review. Fourteen cases were identified; 6 of 14 (43%) had a congenital thrombophilia with many having multiple prothrombotic mutations. Two were managed with medical anticoagulation alone and two required super-urgent transplant for acute liver failure. The remaining 10 of 14 (71%) underwent radiological intervention: 1 of 14 thrombolysis, 5 of 14 angioplasty, and 4 of 14 transjugular intrahepatic portosystemic shunt (TIPS). Six of 14 (43%) patients required repeat radiological intervention (1 angioplasty, 5 TIPS) but none required surgical shunts or liver transplantation for chronic liver disease. The time between diagnosis and treatment did not predict the need for repeat radiological intervention. These data show that radiological intervention can be highly effective, and reduces the need for surgery, though it requires specialist multidisciplinary teams for monitoring.

Pathological fractures of the proximal humerus treated with a proximal humeral locking plate and bone cement.

Bone loss secondary to primary or metastatic lesions of the proximal humerus remains a challenging surgical problem. Options include preservation of the joint with stabilisation using internal fixation or resection of the tumour with prosthetic replacement. Resection of the proximal humerus often includes the greater tuberosity and adjacent diaphysis, which may result in poor function secondary to loss of the rotator cuff and/or deltoid function. Preservation of the joint with internal fixation may reduce the time in hospital and peri-operative morbidity compared with joint replacement, and result in a better functional outcome. We included 32 patients with pathological fractures of the proximal humerus in this study. Functional and radiological assessments were performed. At a mean follow-up of 17.6 months (8 to 61) there was no radiological evidence of failure of fixation. The mean revised musculoskeletal Tumour Society functional score was 94.6% (86% to 99%). There was recurrent tumour requiring further surgery in four patients (12.5%). Of the 22 patients who were employed prior to presentation all returned to work without restrictions. The use of a locking plate combined with augmentation with cement extends the indications for salvage of the proximal humerus with good function in patients with pathological and impending pathological fractures.