LAUSR

Despite improved perinatal management, neurodevelopmental outcomes of infants with birth weights of ≤500 g remain unfavorable. Severe intracranial hemorrhage (IVH), cystic periventricular leukomalacia (PVL), severe necrotizing enterocolitis (NEC), surgical ligation for patent ductus arteriosus, and male sex are associated with adverse neurodevelopmental outcomes. Emergency laparotomy for NEC or intestinal perforation in extremely premature infants has a significant impact on adverse outcomes. We describe the disease course of a patient born with a birth weight of 432 g who underwent ileostomy in the neonatal period and was followed up until age 6 years. A male infant was born to a 35-year-old primipara with known diamniotic twins. Pregnancy was achieved by artificial insemination but complicated by discordancy and oligohydramnios of the smaller fetus. A cesarean delivery was performed at 28 weeks 6/7 days of gestation for non-reassuring fetal heart tracing in the smaller fetus. The child was delivered with a birth weight (SD) of 432 g ( 3.5), length of 27 cm ( 3.7), head circumstance of 21.4 cm ( 2.5), and Apgar scores of 7 and 8 at 1 and 5 min, respectively. He was intubated immediately and admitted to the neonatal intensive care unit, and was hemodynamically stable (Fig. 1). Prominent abdominal distention was observed immediately after birth. A contrast enema was performed on days 1, 2, and 6, using diatrizoic acid (Gastrografin) diluted six times, and meconium obstruction was confirmed based on the meconium plugs and small-sized colon. Laparotomy was performed on day 6 (weight, 419 g) because abdominal distension worsened despite repetitive contrast enema, which was ineffective in reaching the distended ileum even though considerable pressure was applied in each procedure. Owing to the immaturity of the premature bowels, further contrast enema seemed intolerable. Loop ileostomy and removal of the remaining meconium plugs were performed successfully. While no NEC or perforation was detected, a hard meconium plug was detected 15 cm from the oral side of the terminal ileum. Enteral feeding was started on day 18 after remission for abdominal distention. With enteral feeding with his mother’s milk and parenteral nutrition, his weight increased gradually. The ileostomy was closed on day 101 (weight, 1804 g). The child was discharged without supplemental oxygen on day 177, weighing 3245 g, with full oral feeding. No patent ductus arteriosus, IVH, PVL, or retinopathy of prematurity was observed. Growth hormone therapy was started at age 3 years, and his height and weight (SD) caught up to 105.8 cm ( 1.9) and 16.2 kg ( 1.4) at age 6 years, respectively. His developmental quotient (DQ) scores on the Kyoto Scale of Psychological Development at age 6 years were as follows: total DQ, 93; cognitive-adaptive DQ, 93; language-social DQ, 93. He entered a traditional elementary school, without a specialized educational plan. His female twin, whose birth weight was 978 g, developed normally, with a height of 111.9 cm ( 0.5 SD) and total DQ of 97 at age 6 years. Meconium obstruction in prematurity results from immature or ineffective peristalsis of the fetal intestine, which develops obstructive symptoms predisposing to intestinal perforation if not diagnosed and treated promptly. Owing to the underlying prematurity, clinical decisions on when to convert to surgical treatment in extremely premature infants with meconium obstruction remain controversial. The present case had a potential high risk of intestinal perforation without surgery because repetitive contrast enema was ineffective for improving the distention of the small intestine. The resultant early administration of enteral nutrition after remission of the abdominal distention by ileostomy, together with parenteral nutrition, was effective for the later appropriate body weight gain. Compared with his extremely low birth weight, the relative increase in maturity because of the severe fetal growth restriction might have contributed to the patient’s tolerance of the early surgery and other complications such as IVH or PVL. We described the clinical course of an infant weighing 432 g at birth who underwent ileostomy on day 6 for meconium obstruction and showed appropriate catch-up growth and Correspondence: Katsuya Hirata, MD, Department of Neonatal Medicine, Osaka Women’s and Children’s Hospital, 840 Murodocho Izumi, Osaka 594-1101, Japan. Email: [email protected] Received 16 March 2019; revised 10 June 2019; accepted 17 June 2019. doi: 10.1111/ped.14045