Medium and long term central venous access in children.

From September 2000 to August 2001, 104 central venous access devices (CVAD) were inserted in 91 children, governed by a uniform protocol. Thirty catheters were inserted in neonates, 29 in infants, 37 in children and 8 in adolescents. Fifty-one were planned insertions in the operating suite and 53 were emergencies - often by the bedside. There were 12 insertion related complications-all of which were minor. Neonatal age and bedside introduction had a higher risk of insertion related problems. The incidence of non-infectious complications was 20% (rate of 13.7/1000 line days) and was influenced by the child's age and insertion site. Femoral route was the safest. Incidence of catheter associated infections (CAI) was 15.4% (rate of 11/1000 line days). Only 2 children had catheter associated bloodstream infection. Neonates were at higher risk of catheter related infections. Age, insertion site and occurence of insertion complications influenced duration of catheterization (median 7.5 days, range 2-243 days) There was no major complication, though more than 50% insertions were in neonates and infants. In our practice, use of CVAD is feasible and safe, especially in neonates and infants.

Hirschsprung's disease, associated rare congenital anomalies.

OBJECTIVE: Hirschsprung's disease may be associated with a number of congenital anomalies of which Down's syndrome and intestinal atresias are commonly encountered. The study aimed to assess the impact of rare associated anomalies on the diagnosis and management of Hirschsprung's disease. METHODS: A retrospective review of the clinical presentation, diagnosis and outcome of thirty five consecutive newly diagnosed cases of Hirschsprung's disease encountered over two years was performed. RESULTS: Besides Down's syndrome (two), intestinal atresia (one) and pigmentary ocular defects (two), three rare anomalies (Occipital meningocele, Calcific meconium cyst with anal stenosis, Malrotation) were encountered in four of thirty five cases. The clinical features, radiologic anatomy and gross morphology of the bowel were unconventional and the diagnosis was supported by intraoperative acetylcholinesterase staining of biopsies. Though the diagnosis was relatively delayed in these cases, the outcome has been comparable to the rest. The dilemma in their diagnosis and management and their possible pathoembryology is discussed. CONCLUSION: Awareness of such associations and a specific investigative protocol is imperative for timely diagnosis and minimal morbidity in complex presentations of Hirschsprung's disease.

Non specific jejunoileitis--a report of 8 cases.

Nonspecific jejuno-ileitis is a nonocclusive, necrotizing inflammation of the small intestine. We treated 8 patients of jejuno-ileitis in a short span of 8 months. Their mean age was 8.6 years. All had acute pain in abdomen and most had hematochezia. Radiology was helpful only in diagnosis of complications of the disease. Four patients responded to conservative management; the other 4 required surgery--laparotomy and lavage in 2, and multiple laparotomies with resections in 2. One patient died due to chronic malnutrition and metabolic complications. Bowel histology was suggestive of resolving vasculitis in one patient and chronic inflammation in another patient.

Management of hydrocephalus.

The present study is an analysis of 747 patients with hydrocephalus, treated and followed up in the Hydrocephalus Clinic run by the department of Paediatric Surgery at the All India Institute of Medical Sciences, New Delhi. The distribution of patients was: congenital-46%, post-meningomyelocoele excision-28%, post-meningitic-21% and others-5% (including post haemorrhagic and post encephalocoele excision hydrocephalus. The average age was 7 months in the shunted group and 10 months in the medical group with overall male to female ratio of 2.3:1. The data were analysed to study the effect of treatment on ventriculomegaly and mental development with special reference to the type of treatment (shunt versus medical) and age at starting treatment. The probability of shunt failure was also studied. A comparison of ventricular size in US/CAT scans between the time of starting treatment and last follow-up revealed improvement in ventriculomegaly in 60% of the shunted patients but only 30% of the medically treated patients. A significant difference was particularly noted in patients with severe hydrocephalus, 72% and 22%, respectively. Comparison of the mean Mental Performance Quotient (MPQ) scores in the shunted & medically treated patients also revealed significantly better MPQ scores in the shunted group (p = < 0.001). Probability of shunt survival, as depicted by the Kaplan-Meier survival curve, revealed that there is a high rate of shunt failure in the first 12 months, followed by a dramatic slowing down. Our observations support the contention that CSF shunt surgery offers better outcome than medical management even when ventriculomegaly is severe at the time of presentation.