No abstract

Here we describe an 8-year old male child with homozygous sickle cell disease who presented with left parietal skull bone infarction and, during his stay in hospital, developed a right femoral deep vein thrombosis (DVT), both uncommon complications of the disease. He initially presented with severe headache and generalised tenderness of the calvarium, which did not respond to simple analgesics. Scalp swelling in and around the left frontal (including left orbit) and parietal regions developed 24 h after presentation. The differential diagnosis included incipient stroke, acute sickle bone crisis and osteomyelitis, with a possible complication of epidural haematoma, or orbital compression syndrome. An initial exchange blood transfusion did not lead to appreciable reduction in opiate requirements. Significant symptomatic relief was attained only after a second exchange transfusion. The DVT developed at the site of catheterisation (right femoral vein), and this was treated with maximal doses of enoxaparin followed by warfarin. The child is now well and off anti-coagulants. In this article we present a review of the literature and discuss possible mechanisms of these complications in our patient.

Background. Nephroblastoma is the commonest renal tumour seen in children. It has a good prognosis in developed countries with survival rates estimated to be between 80% and 90%, while in Africa it remains low. Method. Retrospective study of patients diagnosed with nephroblastoma who are seen at 4 paediatric oncology units, representing 58.5% of all South African children with nephroblastoma and treated following SIOP protocol between January 2000 and December 2010. Results. A total of 416 patients were seen at the 4 units. Over 80% of our patients were African and almost 10% of mixed ethnicity. The most common stage was stage 4. The median survival was 28 months after diagnosis with the mixed ethnicity patients recording the longest duration (39 months) and the white patients had the shortest median survival. The overall 5-year survival rate was estimated to be 66%. Stage 2 patients did significantly better (85%). Conclusions. Our patients are similar with regard to gender ratio, median age, and age distribution as described in the literature, but in South Africa the more advanced stage disease seen than in other developed countries is translated into low overall survival rate.

Background. Nephroblastoma is the most common paediatric renal malignancy, affecting 1 in 10 000 children worldwide. Between 5% and 10% present bilaterally. Objective. To review two centre's experience of bilateral Wilms' tumour in order to improve future management practices. Methods. This was a retrospective case review of nephroblastomas treated at the Chris Hani Baragwanath and Charlotte Maxeke academic hospitals from 1 January 2003 to 31 December 2013. Results. Eighteen patients (8.04%) presented with bilateral disease and were younger than those with unilateral disease. Three patients presented with metachronous disease at a median age of 23 months; initial presentation was at a median age of 2 months. The remaining 15 patients presented with synchronous disease at a median age of 27 months. Treatment followed aspects of the SIOP 9 protocol. Two patients died before surgery. Thirteen kidneys were removed. Twelve patients underwent nephron-sparing surgery, with microscopically positive resection margins seen in six kidneys post surgery. Two patients with residual microscopic disease relapsed. Three kidneys demonstrated unfavourable histology. Nephroblastomatosis was identified in one kidney. Eight patients were alive and disease free and three were alive with disease. An overall and disease-free survival rate of 66.67% and 55.56%, respectively, was found for this cohort. Neither age >2 years nor metachronous disease was associated with a poorer prognosis. Conclusion. Bilateral nephroblastoma is a complex disease. The majority of patients in this series presented with advanced local disease. Relapse was more commonly influenced by the presence of microscopically positive margins than metastatic disease at presentation.