Long-term results of central venous access devices in children with haemophilia.

AIMS: Central venous access devices (CVADs) are often used to provide reliable venous access for factor VIII administration in children with haemophilia. This study investigates their long-term outcome. METHODS: A retrospective cohort study of 44 CVADs inserted into 31 children with haemophilia at a single centre between 1991 and 2006. RESULTS: Eight (18%) CVADs are still in place and working well. Fourteen (31.8%) were removed when the child was able to return to peripheral vascular access. Twenty-two (50%) were removed because of complications, most of these children needing a replacement CVAD. The median duration that the first CVAD was in place was 51 months. There were no life-threatening complications. CONCLUSIONS: CVADs function well in children with haemophilia for a long time, with a relatively low complication rate, and can tide a child over a difficult period for vascular access.

Long-term survival after spontaneous rupture of a malignant rhabdoid tumor of the liver.

A 13-month-old girl presented with a large malignant rhabdoid liver tumor that ruptured soon after admission. Six years after an emergency right hepatectomy and subsequent chemotherapy (ifosfamide, vincristine, and actinomycin D), she remains well and disease free. Previously, these rare tumors invariably have been fatal and resistant to multimodal therapy. This is the first report of long-term survival of a patient with a malignant rhabdoid liver tumor.

Hirschsprung's disease: problems with transition-zone pull-through.

BACKGROUND/PURPOSE: It is generally accepted that if surgery for Hirschsprung's disease is to be successful, ganglionic bowel must be anastomosed to the lower rectum or anal canal. Above the aganglionic distal bowel lies a transition zone (TZ) where more subtle abnormalities of innervation are apparent. The significance of this transition zone in respect to the functional outcome of surgery has received little attention. The aim of this study was to identify the incidence of transition zone pull-through (TZPT) in a cohort of children who underwent surgery for Hirschsprung's disease, to identify the reasons why TZPTs occurred, and to identify the functional consequences. The authors report the long-term outcome of these children with emphasis on bowel function and the results of subsequent surgery. METHODS: A Retrospective study was conducted of children treated at a single institution from 1979 through 1994. TZPT patients were subject to detailed review of surgical records and histopathologic material. RESULTS: Thirteen children were identified with a TZPT. In 12 cases, histopathologic errors contributed to the TZPT: in 5 cases this was caused by single point biopsies missing an asymmetrical TZ, whereas in 7 cases the histopathologic features of the TZ were not recognized. In 1 case the TZPT was caused by surgical error. As a consequence of the TZPT 7 children underwent repeat pull-through. One child is fully continent, one has daytime fecal continence, and 2 others are incontinent. Two children have permanent stomas. One child is clean with antegrade colonic washouts. Repeat pull-throughs were not attempted in 6 children. Two children have achieved full continence, 2 have permanent stomas, 1 is clean with antegrade colonic washouts, and 1 child receives regular suppositories. CONCLUSIONS: Transition zone pull-throughs occurred because of a combination of surgical and histopathologic errors. The transition zone may follow an asymmetric course around the circumference of the bowel and may be missed if single-point extramucosal biopsy specimens are taken. Recognition of the subtle histologic features of the transition zone requires an experienced pathologist. The functional consequences of a TZPT are severe, with symptoms of constipation, diarrhea, and incontinence. The results of revisional pull-through were disappointing. Serious consideration should be given to alternative procedures such as the antegrade continence enema operation.