Gastroschisis: 13 years' experience at RCH Melbourne.

OBJECTIVE: To review recent experience of gastroschisis at the Royal Children's Hospital, Melbourne. METHODOLOGY: Retrospective review of admissions over a 13 year period, June 1980-June 1993 inclusive, including an analysis of those factors influencing mortality, morbidity and complications. RESULTS: There were 49 infants, of whom two died (4%), both having an associated bowel atresia. Mode of delivery, age at operative repair, birthweight centile and silo repair had no significant effect on the duration of total parenteral nutrition (TPN) or hospital stay. The presence of short gut and/or gut resection and localized bowel narrowing or bowel atresia resulted in a significant increase in the duration of TPN and hospital stay. The presence of a central venous line was a highly significant risk factor for the development of systemic sepsis. CONCLUSION: Currently gastroschisis has a good outlook with a low mortality. Infants with short gut/resection and bowel atresia have a long duration of TPN and hospitalization, with significant morbidity and complications.

Inappropriate use of contrast studies prior to referral of neonatal gastrointestinal anomalies and bowel obstruction.

Ten cases of neonatal gastrointestinal anomalies and bowel obstruction are described, in which contrast studies were carried out prior to transfer. Nine of the 10 infants had bile-stained vomiting, indicating the need for immediate surgical referral. The contrast studies resulted in delayed referral, deferment of appropriate resuscitation, and complications associated with the study. In several cases the wrong type of study, or the wrong contrast medium was used. These 10 infants required urgent surgical referral, not a sophisticated radiological procedure in geographic and surgical isolation.

Fulminant hepatic failure with intractable ascites due to an echovirus 11 infection successfully managed with a peritoneo-venous (LeVeen) shunt.

A case is described of an infant who at 5 days of age developed an echovirus 11 infection with hepatic failure and intractable ascites, refractory to medical treatment. The ascites was successfully controlled with a peritoneo-venous (Le Veen) shunt inserted at 6 weeks of age. The shunt was subsequently removed when the infant was 4 months of age with no recurrence of ascites. At long-term follow-up he is growing and developing normally. This case shows that an adult Le Veen shunt can be successfully modified for use in the newborn with worthwhile long-term survival.

Enteric colonization in sporadic neonatal necrotizing enterocolitis.

Microflora of the gut was studied close to the onset of sporadic necrotizing enterocolitis (NEC) in neonates. Enteric flora in 25 babies with NEC differed from that in 23 matched controls. Bacteroides spp and lactobacilli were less common in babies with NEC compared with controls: 32 versus 61% (p = 0.03) and 12 versus 48% (p = 0.006), respectively. Clostridium perfringens was isolated from 40% of babies with NEC compared with 13% of controls (p = 0.03). It was present in 33% of babies with NEC aged less than 14 days but was not detected in control babies of the same age. The incidences of C. butyricum and C. difficile were similar in patients and controls. Colonization with C. perfringens in the absence of a protective barrier flora may be crucial in the pathogenesis of severe sudden onset NEC. Two potentially valuable marker factors to predict onset of this form of sporadic NEC are the use of fecal smears and estimation of fecal tryptic activity.

An unusual case of Sturge-Weber syndrome.

This report describes a case of cranio-facial angiomatosis with rapid growth of the angiomas leading indirectly to death at 3 mth of age. Extensive involvement of the cranial nerves, pituitary stalk, brain-stem meninges and liver and the presence of a Dandy-Walker malformation cyst have not been previously described in this condition.

Development of gut colonisation in pre-term neonates.

Twenty-eight pre-term babies of low birth weight were monitored for developing microflora in throat, stomach and faeces during the first 3 weeks of life. The flora at all levels of the gastrointestinal tract differed from that of healthy breast-fed and artificially fed full-term babies. Colonisation of throat and stomach was delayed beyond 4 days of life in 87% and 60% of babies respectively. Only 10% of babies had "normal" oral flora throughout the period of study. Flora of the stomach was sparse, and resembled faecal flora. Faecal flora was established more rapidly than throat or stomach flora, and 70% of babies were colonised during the first 4 days of life. Initially Bacteroides spp. were predominant (57% babies), but Escherichia coli and other aerobic gram-negative bacilli gradually increased in frequency. Colonisation by gram-positive bacteria was slow. Clostridium spp. were present in only 10% of babies during the first 4 days of life. Most strains were transient. Colonisation with C. butyricum (30%), C. perfringens (35%) and C. difficile (25%) was maximum after the first 2 weeks of life. Lactic-acid-producing bacteria usually appeared late in the third week of life. Parenteral feeding immediately after birth was associated with delayed colonisation by a restricted number of species. Parenteral antibiotics (penicillin or gentamicin or both) restricted colonisation with normal oral flora, the lactic-acid-producing bacteria and penicillin-sensitive clostridia, but had little effect on E. coli even when the colonising strain was sensitive to the aminoglycoside in the regimen. Systemic spread of bacteria via the blood stream was not detected in any babies. The pattern of colonisation of the enteric tract in pre-term infants in the special-care nursery studied, differs from that of healthy full-term babies; this merits consideration when the results of bacteriological tests of this vulnerable group of infants are being interpreted.

Infectious diseases of the newborn.

Despite the advent of antibiotics, infectious diseases remain a significant cause of mortality and morbidity in the neonatal period. Infection is the third commonest cause of perinatal mortality after hypoxia and malformations. Neonatal mortality rates from infection are of the order of 1:1000 live births. Although infections are theoretically preventable, there has been no significant change in incidence over the last 30 years.

Respiratory function after repair of congenital diaphragmatic hernia.

Respiratory function studies were carried out in 22 infants who had successful repair of diaphragmatic herniae of the Bochdalek type. Thoracic gas volume was initially reduced in only 3 of these, but subsequent studies showed that improvement occurred. There were no consistent abnormalities in either dynamic compliance or mean pulmonary conductance. This is evidence that there is rapid adaptation which compensates for any alteration in the parenchymatous tissue in the lungs or abnormalities in the bronchial tree in infants soon after the repair of congenital diaphragmetic herniae. Further studies are necessary to determine the changes in these lungs with growth.