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Twenty-one extremely-low-birth-weight (ELBW) and premature infants (<29 weeks' gestation and/or <1,000 g) underwent emergency laparotomy for acute intra-abdominal pathology (necrotising enterocolitis [NEC] 16, other bowel pathology 5) during the 4-year period from 1990 to 1993; 11 died. The neurodevelopmental outcome of the 10 survivors was assessed and compared with 20 living, otherwise normal controls matched for gestational age, birth weight, and year of birth to asses the effect of the abdominal event on quality of survival. Those who survived after laparotomy had a worse neurodevelopmental outcome than controls (P < 0.05). During this period, we also compared 24 infants in the ELBW category who developed NEC but did not require a laparotomy with the 16 ELBW infants with NEC who required a laparotomy. Those who required a laparotomy had worse disease and had significantly worse neurodevelopmental outcomes (P < 0.01). ELBW and premature infants who have acute intra-abdominal pathology requiring a laparotomy are thus at increased risk of neurodevelopmental problems and poor growth. Close long-term follow-up is important, and the families of such infants should be made aware before surgery of the increased risk the abdominal event has on their babies' developmental outcome if they survive.

Twenty-nine patients with congenital duodenal obstruction (DO) detected as a "double bubble" (DB) on antenatal ultrasound (US) or diagnosed postnatally were seen at the Women's and Children's Hospital between 1985 and 1994; 24 (83%) had antenatal scans, with 21 (87%) DBs visualised. The 3 fetuses with normal scans who developed DO postnatally had duodenal "windsocks". Five of the 24 scanned patients (21%) were found to have a DB before 20 weeks' gestation: 1 simple duodenal atresia, 2 duodenal atresias with chromosomal abnormalities (1 trisomy 21 and 1 triploidy), 1 biliary atresia with a pre-pyloric vein, and 1 malrotation. Sixteen (67%) cases were detected with scans after 20 weeks (range 28-36 weeks); 12 (75%) of these were done for polyhydramnios. In response to a questionnaire to 9 of the 16 parents whose fetuses underwent a late scan, 5 had had normal early scans elsewhere and 4 did not respond (4 of the remaining 7 had died and 3 were lost to follow-up). Eleven of the 29 patients (38%) had abnormal karyotypes, and 4 (22%) of the 18 with a normal karyotype had associated anomalies. Two sets of familial cases were noted. Early antenatal diagnosis of DO is possible in a minority of cases, and this early diagnosis may be associated with an increased risk of other pathology. Furthermore, early demonstration of a DB at routine US prior to 20 weeks allows appropriate counseling and the option of mid-trimester termination, whereas this is more difficult later in the pregnancy. In all cases detected antenatally, planning of delivery and parent counseling should be achieved.

The case is reported of a baby boy with an interstitial deletion of the long arm of chromosome 13 who, in addition to the described associations of Hirschsprung's disease and intestinal atresia, had umbilical cord ulceration resulting in massive intrapartum haemorrhage. This case provides support for the existence of a previously reported association between umbilical cord ulceration and intestinal atresia, and suggests that it is aetiologically heterogeneous.