Hypothesis: Infants with rotational abnormalities of the midgut mesentery a
re at high risk for gastroesophageal reflux disease (GERD) and for sudden i
nfant 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 inc
lude intestinal malrotation. Eighty-one (89%) of the infants studied for GE
RD had a mean follow-up of 23.2 months (median, 12 months).
Setting: Patients treated in 2 tertiary care centers consisting of a childr
en's hospital and a university medical center.
Patients: Two hundred eighty-six consecutive infants were treated for conge
nital 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 mon
itoring performed and no prior operation on the stomach or esophagus. The s
tudied infants had intestinal malrotation either alone (n = 55) or associat
ed with a repaired abdominal wall defect (n = 23) or congenital diaphragmat
ic 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 de
termine 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 f
rom GERD. The follow-up of GERD was reported as a combination of clinical o
utcome 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 al
one, 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 sign
ificantly lower (13% [3 patients]; P = .03) than in patients with intestina
l malrotation alone. The prevalence of GERD in infants with repaired congen
ital diaphragmatic hernia was significantly lower (69% [9/13]; P = .02) tha
n in infants with intestinal malrotation alone but not for the prevalence o
f 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 followup, the prognosis for GERD in infants w
ith intestinal malrotation was better in the infants who were asymptomatic
than in those who were symptomatic at the initial extended esophageal pH mo
nitoring.
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 signi
ficant concern. The prevalence of GERD is lower in infants with congenital
diaphragmatic hernia. Infants with repaired abdominal wall defects have a l
ower prevalence of the risk factor for SID from GERD. We recommend careful
evaluation and follow-up of infants with intestinal malrotation for problem
s, such as SID, from GERD.