Esophageal pH monitoring abnormalities and gastroesophageal reflux disease in infants with intestinal malrotation.

HYPOTHESIS Infants with rotational abnormalities of the midgut mesentery are at high risk for gastroesophageal reflux disease (GERD) and for sudden infant death (SID) from GERD. DESIGN A survey of the prevalence of GERD and the risk factor for SID from GERD in a case series of infants treated for congenital anomalies that include intestinal malrotation. Eighty-one (89%) of the infants studied for GERD had a mean follow-up of 23.2 months (median, 12 months). SETTING Patients treated in 2 tertiary care centers consisting of a children's hospital and a university medical center. PATIENTS Two hundred eighty-six consecutive infants were treated for congenital anomalies from September 1, 1985, through May 31, 1998. The patients selected for study were 91 infants who had 18- to 24-hour esophageal pH monitoring performed and no prior operation on the stomach or esophagus. The studied infants had intestinal malrotation either alone (n = 55) or associated with a repaired abdominal wall defect (n = 23) or congenital diaphragmatic hernia (n = 13). Of the 91 infants, 34 were asymptomatic at the time of esophageal pH monitoring. INTERVENTIONS Eighteen- to 24-hour esophageal pH monitoring was used to determine the presence of GERD (abnormal pH score >2 hours postcibal) and the risk factor for SID from GERD (type I or III reflux pattern in combination with a prolonged mean duration of sleep reflux). MAIN OUTCOME MEASURES The prevalence of GERD and the risk factor for SID from GERD. The follow-up of GERD was reported as a combination of clinical outcome and subsequent extended esophageal pH monitoring. RESULTS Of the 91 infants studied, 80 (88%) had GERD and 26 (29%) had the risk factor for SID from GERD. Of 55 infants with intestinal malrotation alone, 52 (95%) had GERD, and 20 (36%) had the risk factor for SID from GERD. Although GERD was found in 19 (83%) of 23 patients with repaired abdominal wall defects, the prevalence of the risk factor for SID from GERD was significantly lower (13% [3 patients]; P = .03) than in patients with intestinal malrotation alone. The prevalence of GERD in infants with repaired congenital diaphragmatic hernia was significantly lower (69% [9/13]; P = .02) than in infants with intestinal malrotation alone but not for the prevalence of the risk factor for SID from GERD (23% [3/13]; P = .19). Both symptomatic and asymptomatic patients had similar prevalences of GERD (91% [52/57] vs. 82% [28/34], P = .17) and for the risk factor for SID from GERD (31% [18/57] vs. 24% [8/34]; P = .28). On follow-up, the prognosis for GERD in infants with intestinal malrotation was better in the infants who were asymptomatic than in those who were symptomatic at the initial extended esophageal pH monitoring. CONCLUSIONS The prevalence of GERD in infants with intestinal malrotation is high, and the prevalence of the risk factor for SID from GERD is a significant concern. The prevalence of GERD is lower in infants with congenital diaphragmatic hernia. Infants with repaired abdominal wall defects have a lower prevalence of the risk factor for SID from GERD. We recommend careful evaluation and follow-up of infants with intestinal malrotation for problems, such as SID, from GERD.