Journal of Perinatology (2006) 26, 375–377

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PERINATAL/NEONATAL CASE PRESENTATION

Cases
Intestinal malrotation is a relatively uncommon condition with diverse Case 1
outcomes. Familiarity with variations in the presentation of malrotation is Baby #1 is a term female (birth weight 3255 g) with a past medical
imperative as early diagnosis and prompt subsequent surgical intervention history significant for apneic episodes including a life-threatening
are essential to optimizing outcome. The most frequent clinical sign in the event on the second day of life in the well-baby nursery that
neonate is bile-stained emesis. We report three cases of unsuspected necessitated cardiopulmonary resuscitation. Extensive work-up at
malrotation that were diagnosed in neonates with a history of nonbilious the time included ruled out sepsis, as well as normal results on
emesis who were assessed for presumed gastroesophageal reflux or brain magnetic resonance imaging, electroencephalogram,
aspiration. Gastroesophageal reflux is a common condition among electrocardiogram and gastric emptying scan. A sleep study
newborns, and can be a subtle presentation of malrotation. Clinicians revealed central and obstructive apnea and, after a period of
should consider malrotation as a possible cause of reflux, particularly in observation, the infant was sent home on caffeine and a home
infants with unusually pathologic or persistent symptoms necessitating apnea monitor. She was seen in the follow-up clinic and was noted
ongoing treatment for reflux. to have recurrent episodes of nonbilious emesis with breast feeding.
Journal of Perinatology (2006) 26, 375–377. doi:10.1038/sj.jp.7211507 The patient had normal feeding, growth and bowel movement
Keywords: malrotation; gastroesophageal reflux patterns, with no report of fevers or abdominal pain. She
underwent an upper gastrointestinal (GI) tract contrast study that
revealed spontaneous GER and malrotation without any evidence
of volvulus (Figure 1). The pediatric surgery team was consulted
Introduction and the patient was admitted to the general pediatric ward. The
Intestinal malrotation is a relatively uncommon condition with following day, she underwent a Ladd’s procedure to correct the
diverse outcomes. Bilious emesis is its most common presenting malrotation, in addition to an appendectomy. Soon thereafter, her
sign.1 Gastroesophageal reflux (GER) is a relatively common, but reflux symptoms improved and her 9-month neurodevelopmental
vexing problem for neonatologists caring for infants in the testing was normal. She has had no surgical complications.
neonatal intensive care unit (NICU) and postdischarge.
Gastroesophageal reflux can present with or without emesis, but
emesis associated with GER with no obstruction, is expected to be Case 2
nonbilious. Owing to the prevalence of medical treatment for GER Baby #2 is a male born prematurely by Cesarean section at 30
in NICU infants, especially preterm infants,2 which we suspect is weeks gestation owing to maternal pre-eclampsia, (birth weight
often performed without prior radiological evaluation, we report 1330 g). During his NICU stay, respiratory distress quickly resolved
here three cases of unsuspected intestinal malrotation that were and after 48 h of antibiotics for suspected sepsis, he was discharged
diagnosed in neonates who were being assessed for presumed GER to a community hospital for an unremarkable convalescent period.
or aspiration, with a history of nonbilious emesis. The cases serve He was discharged home 3 weeks later. He was brought to the
as a reminder of the difficulties involved in diagnosing Emergency Department at the age of 2½ months status
malrotation, one very serious potential underlying cause of GER postcardiopulmonary arrest at home, was resuscitated and admitted
symptoms in infants. for further management after suffering anoxic brain injury. During
this hospitalization, he was noted to have recurrent nonbilious
Correspondence: Dr CM Cotten, Assistant Clinical Professor of Pediatrics, Clinical Research emesis without any other GI symptoms. An upper GI tract contrast
Director, Duke Neonatology, Director Special Care Nursery, Durham Regional Hospital, Box study was performed to assess the emesis and was significant for
3179 DUMC, Durham, NC 27710, USA.
E-mail: cotte010@mc.duke.edu reflux as well as malrotation (Figure 2). He subsequently
Received 29 September 2005; revised 12 March 2006; accepted 21 March 2006 underwent a Ladd’s procedure with an appendectomy, and a Nissen
 Malrotation with nonbilious emesis
K El-Chammas et al
376

Case 3
Baby #3 is a male born prematurely at 32 weeks of gestation (birth
weight 1660 g), with a history pertinent for multiple episodes of
‘feeding intolerance’ characterized solely by nonbilious emesis,
with an acute life-threatening event. During the evaluation of his
emesis at the age of 1½ months, an upper GI contrast study
revealed reflux and malrotation. Ladd’s procedure with
appendectomy and a Nissen fundoplication were performed.
Follow-up developmental testing at 18 months was significant for
growth failure, oral feeding difficulties and language delays.
Surgical complications included hospital admissions for an
incarcerated hiatal hernia, esophageal stricture and small bowel
obstruction, all necessitating further surgical intervention.

Discussion
Malrotation, the incomplete or abnormal rotation of the GI tract
about the superior mesenteric artery, is a relatively uncommon
condition associated with diverse outcomes. Familiarity with
Figure 1 Subtle case of malrotation, with duodenal–jejunal junction variations in the presentation of malrotation is imperative as early
located at the same level as the gastric antrum, but not located to the diagnosis and prompt subsequent surgical intervention are
left of the left pedicle of the spine. essential to optimizing outcome in many cases.
The actual incidence of malrotation in infants and children
beyond the neonatal period is difficult to estimate, as such
anomalies may remain asymptomatic and undetected throughout
life. Approximately 50 to 70% of cases are diagnosed during the
neonatal period; about 50% present in the first week of life, and
over 60% present before the end of the first month.1,3 A substantial
number of cases present beyond the neonatal period; most present
in infancy but up to 20% become symptomatic after the first year of
life.4,5 Male infants are more frequently affected than female
infants.6
There have been few published series that delineate the clinical
characteristics of children with malrotation. The most frequent
reported sign in neonates is bile-stained emesis, which should
always be viewed seriously and urgent investigations undertaken to
establish the cause.1 Bilious emesis is a sign of intestinal
obstruction, not exclusively malrotation. In one report of 63 infants
evaluated for bilious vomiting, 24 had a surgical diagnosis; among
these, four had malrotation.7 More chronic GI signs, such as
intermittent pain and vomiting, are more commonly manifested by
Figure 2 Upper gastrointestinal (GI) examination demonstrates older children.8 The risk of midgut volvulus is reduced in patients
failure of duodenal–jejunal junction to cross midline, consistent with over 1 month of age compared to patients in the neonatal period.4
malrotation. Abdominal radiographs may show a dilated duodenum with a
fluid level and some gas in the distal bowel; however, it may be
interpreted as normal in 20% of cases.6 The upper GI contrast study
is the preferred study in patients presenting nonacutely, despite the
fundoplication. His 18-month neurodevelopmental assessment
risk of contrast aspiration, one of the major objections to this study
showed global developmental delays with spastic quadriplegia. He
in the presence of suspected high obstructions – but in cases
has had no surgical complications of the Ladd’s procedure or
involving growing premature infants, this is not a likely concern.4
Nissen.
The appropriate position of the duodenojejunal junction is to the

Journal of Perinatology
 Malrotation with nonbilious emesis
K El-Chammas et al
377

left of the left pedicle of the spine, and at the same craniocaudal malrotation in mind as a possibility. We recommend consideration
level as the pylorus. Signs of malrotation noted with this study are of a radiographic contrast study on infants whose GER symptoms
incomplete duodenal obstruction, duodenojejunal flexure to the appear unusually severe or persistent, with or without reflux
right of the expected location, proximal jejunal loops lying medications, or will undergo an antireflux surgical procedure such
abnormally on the right side of the abdomen, and a ‘high’ cecum as fundoplication.
seen with delayed follow through films.4 Contrast enema
examination is unreliable in confirming the diagnosis, as the
position of the cecum is extremely variable.9 A contrast meal is
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Journal of Perinatology