The results of treatment of children with metastatic Wilms tumours (WT) in an African setting: do liver metastases have a negative impact on survival?

BACKGROUND: From Africa, where socio-economic circumstances differ from the developed world, there are no data regarding the influence of liver metastases on survival of children with Wilms tumour. PROCEDURE: One hundred fifty new patients with WT were seen between 2002 and 2010, 45 (30%) had metastases at diagnosis. Seven patients had bilateral disease with additional visceral metastases. Nine patients who developed liver metastases during treatment were excluded. The site of metastases and the results of pretreatment biopsies were retrieved. Neo-adjuvant chemotherapy was combined with nutritional resuscitation, and aggressive supportive care. Post-operative treatment was determined by stage and histology. RESULTS: Liver metastases were present in 19 (42%) patients but were the sole metastatic site in only 4 (9%). Overall survival at 5 years was 58.5%. Event Free Survival was 54%. Thirty-three (73%) had favourable histology, nine unfavourable and undetermined in three. No influence of histology on outcome was evident. Three patients had resection of persistent liver metastases. The pattern of metastatic disease had no influence on outcome. Despite aggressive supportive care two patients (4%) died within a week of presentation. Two patients died of chemotoxicity and two of complications following biopsy. Eight patients (17%) were lost to follow-up of whom five were on palliative treatment only. CONCLUSIONS: In Africa liver metastases do not appear to worsen the prognosis of children with Stage IV WT. Despite the poor socio-economic circumstances survival is comparable to other countries.

Conservative management of thoracobiliary fistula.

BACKGROUND: Thoracobiliary fistulas are rare manifestations of biliary disruption. Given their rarity it is not surprising that there is little consensus on the optimal management of thoracobiliary fistulas. METHODS: Patients presenting with thoracobiliary fistulas over a 5-year period (1996 to 2001) were evaluated. Initial management was conservative with tube thoracostomy or drainage of sepsis when appropriate, or both; antibiotics and somatostatin were routinely administered. Endoscopic retrograde cholangiography was performed when symptoms persisted to delineate the thoracobiliary communication and undertake sphincteroplasty. RESULTS: Eight patients with a mean age of 31.9 years (range 15 to 42) were evaluated. Biliary effusion occurred in 3 patients after hepatic injury (n = 2) and percutaneous transhepatic cholangiography (n = 1). Bilioptysis occurred in 5 patients after hepatic abscess (n = 4) and hepatic injury (n = 1) The biliary effusion (n = 3) was successfully managed by endoscopic sphincterotomy in 2 patients; the third patient underwent urgent surgical biliary drainage. Bilioptysis (n = 5) was successfully managed in 3 patients; persistence of symptoms in 2 patients prompted surgical intervention. CONCLUSIONS: Thoracobiliary fistulas may be successfully managed using a conservative approach. Surgery should be reserved for persistence of symptoms after exhaustion of this approach.