Pediatrics International (2004) 46, 167–170

Original Article

Rare clinical presentation mode of intestinal malrotation after neonatal

period: Malabsorption-like symptoms due to chronic midgut volvulus

MUSTAFA GMAMO}LU,1 ALI ÇAY,1 HALUK SARIHAN1 AND YALAR LEN2

Departments of 1Pediatric Surgery and 2Pediatrics, Karadeniz Technical University, Faculty of Medicine,
Trabzon, Turkey

Abstract Background: Many different and non-specific clinic presentation modes of malrotation anomalies (MA) have
been reported after neonatal period. The authors describe four children with MA presented with malabsorption-
like clinical features.
Methods: Three children aged from 8 months to 7 years, with a history of long-standing diarrhea and failure
to thrive attributed to malabsorption, were referred to Department of Pediatric Surgery, for evaluation of
suspected MA. Another patient, a 10-year-old boy who was treated for malabsorption for 6 years, presented
with acute duodenal obstruction findings. The duration of symptoms averaged 35 months, ranging 8 months to
6 years. All patients had undergone extensive evaluation and empiric trials of different formulas with no
improvement in their symptoms. One underwent jejunal biopsy.
Results: Primary presentation complaints were chronic diarrhea and failure to thrive in three patients. Their
histories revealed chronic (in one) and intermittent colicky (in two) abdominal pain, and intermittent
nonbilious vomiting (in three). The remaining patient presented with acute illness, with chronic diarrhea,
failure to thrive, and intermittent abdominal pain and vomiting on his history. They were below 30th percentile
according to body weight and height. Laboratory studies revealed hypoproteinemia, hypoalbunemia, raised
liver function tests, and anemia in all patients. The patient who presented acutely had double-bubble sign on
the plain abdominal film obtained at admission. In the other three, plain films obtained during an attack of
abdominal pain and/or vomiting revealed findings of partial intestinal obstruction. The diagnosis was confirmed
by upper gastrointestinal series. At their laparotomy, a classical type of malrotation with circumstantial
evidence of chronic volvulus was noted. All patients had normal laboratory values between postoperative
3 and 5 weeks, and they were up to 30th percentile at the end of the 6 months.
Conclusion: Malrotation anomalies should be included in the differential diagnosis in a child presented with
malabsorption-like clinical features.

Key words children, failure to thrive, intestinal malrotation, malabsorption.

The age and clinical presentation of patients with malrotation
anomalies (MA) are variable.1 Bilious vomiting with other
findings of complete, or intermittent, high intestinal
obstruction is the most commonly presenting mode of MA
in the first month of life, thus the diagnosis is easily made.
However, presentation mode mainly includes a history of
vague long-standing abdominal complaints after this period.2,3
Also, the index of suspicion for MA progressively decreases
with age, so clinical diagnosis is usually not considered in the
initial evaluation. In addition, these vague symptoms are
frequently attributed to other more common conditions,
including milk intolerance, gastrointestinal allergies, mal-
absorption syndromes, or even psychogenic causes. For these
reasons, delayed diagnosis caused by misdiagnosis is a common
problem in children with late-presenting MA.1,2 We report
four cases of MA presenting with mainly malabsorption-like
symptoms after the neonatal period.

Correspondence: Mustafa Gmamo]lu, Karadeniz Technical Univer-
sity, Faculty of Medicine, Department of Pediatric Surgery, 61080
Trabzon, Turkey. Email: mimamoglu61@yahoo.com
Received 7 October 2002; revised 31 July 2003; accepted
11 August 2003.
Patients
The records of four patients were reviewed between the
years 1993–2002 (Table 1). Three patients with a history of
long-standing diarrhea and failure to thrive attributed to
168 M Gmamo]lu et al.

Table 1 Summary of the clinical findings

Case Sex/age Presentation complaints Complaints on history Duration

1 F/8 months Diarrhea, FTT
2 M/5 year Diarrhea, FTT
3 F/7 year Diarrhea, FTT
4 M/10 year Bilious vomiting, colicky
abdominal pain, distention
Nonbilious vomiting, intermittent cry attacks 8 months
Nonbilious vomiting, intermittent abdominal pain 1 year
Nonbilious vomiting, chronic abdominal pain 4 years
Diarrhea, FTT, intermittent abdominal pain 6 years

F, female; FTT, failure to thrive; M, male.

Table 2 Clinical laboratory data on admission

Normal Case 1 Case 2 Case 3 Case 4

Hb (g/dL) 10.5–14.0 7.8 9.8 9.7 9.2
TP (g/dL) 4.3–7.3 3.9 4.1 4.1 4.3
Alb (g/dL) 3.1–5.1 2.1 2.8 3.0 3.1
SGOT (U/L) 5–40 110 65 65 52
SGPT (U/L) 5–40 80 55 48 50
Weight (percentile) 5–95 5–10th 10–25th 10–25th 10–25th
Height (percentile) 5–95 5–10th 10–25th 10–25th 10–25th

Alb, albumin; Hb, hemoglobin; SGOT, serum glutamic oxaloacetic transaminase; SGPT, serum glutamic pyruvic transaminase; TP, total
protein.

malabsorption, were referred to Department of Pediatric
Surgery, Faculty of Medicine, Karadeniz Technical University,
for evaluation of suspected MA. Another patient treated for
malabsorption for 6 years was admitted with acute duodenal
obstruction findings.
All patients had undergone extensive evaluation and
empiric trials with different formulas with no complete
improvement in their symptoms. All studies for malabsorp-
tion syndromes had been reported as normal, including
allergy testing to foods, lactose breath test, sweat chloride
testing, and stool guiac. In case 1, a breath test showed
lactose intolerance but her symptoms failed to improve with
a high-fiber and lactose-free diet. Case 3 underwent jejunal
biopsy to eliminate celiac disease before referral to us, but
his biopsy showed only edematous mucosa.
Results
The duration of symptoms averaged 35 months, ranging from
8 months to 6 years. Primary presentation symptoms were
chronic diarrhea and failure to thrive in three patients. The
patient’s medical histories revealed one case of chronic and
two cases of intermittent colick abdominal pain, and three
cases of abdominal pain and intermittent non-bilious
vomiting (Table 1). Case 1 had the symptoms since birth.
The remaining case (case 4) presented with a classic picture
of acute duodenal obstruction of MA, including colicky
abdominal pain and bilious vomiting. His medical history
showed chronic diarrhea, failure to thrive, and intermittent
abdominal pain and vomiting were present. All patients were
below the 30th percentile according to body weight and
height. Laboratory studies revealed hypoproteinemia, hypo-
albunemia, raised liver function tests, and anemia in all
patients (Table 2).
A delayed (at 60 min) plain abdominal film with barium
obtained at admission demonstrated double-bubble sign of
acute complete duodenal obstruction in case 4 (Fig. 1). He
underwent urgent laparotomy, and a classical type of mal-
rotation with 270° volvulus was detected. In the other three
hospitalized children, plain films obtained during an attack
of abdominal pain and/or vomiting revealed a distended
stomach, several air-fluid levels in the upper abdomens, and
the absence of a normal colonic gas pattern. In these three
patients, the diagnosis of MA was confirmed by upper GI
series immediately after these suspect plain films. The upper
GI series revealed a distended stomach, obstruction of the
duodenum with the appearance of extrinsic compression, and
filling of the jejunal loops on the right side of the abdomen.
Delayed films (at 120 min) revealed a corkscrew appearance
in two of them (Fig. 2).
At laparotomy, a classical type of malrotation was noted in
two patients with 180° volvulus and in one patient without
volvulus. Importantly, all of our patients showed severe thick-
ened edematous intestinal wall and mesenteric venous dila-
tion, and multiple mesenteric lymph adenopathies. No patient’s
had short bowel. A standard Ladd’s procedure was performed
in all patients. Postoperative ileoileal intussusception was

Fig. 1 Delayed film (60 min) shows distended stomach and
duodenum (double-bubble sign).
developed in case 2 and he underwent second laparotomy at
the fifth postoperative day.
Without any additional medical treatment, all patients had
normal laboratory values between postoperative 3 and 5 weeks.
Their weight gains were well, and they had reached the 30th
percentile at the end of the 6 months. The follow-up period
ranged between 2 years and 8 years, and no malabsorption-
like symptoms were noted on their visits.
Discussion
After the neonatal period, patients with MA tend to be
present with chronic and non-specific symptoms, and therefore
these patients are commonly associated with delayed diagno-
sis.1,3 These rare presentation modes include only recurrent
colicky or chronic abdominal pain, constipation, hemato-
chezia and anemia, obstructive jaundice, duodenal ulcer,
chylous ascites, gastroesophageal reflux, respiratory symptoms
(asthma, aspiration), septic shock, and chronic pancreatitis.2,4
Malabsorption due to malrotation 169
Fig. 2 Delayed film (120 min) shows the position of the duo-
denojejunal junction to the right side of the spine and typical
corkscrew appearance at the jejunum, suggesting classical type
malrotation with volvulus.
Malabsorption pattern with diarrhea, protein calorie mal-
nutrition and failure to thrive is also one of the rare presenta-
tion modes of MA after the neonatal period.1,3,5,6 Among 82
patients with late presenting MA reported in the study of
Spigland et al., only three (3,6%) patients presented with this
mode.2 The authors of several studies have postulated that in
such cases chronic or recurrent volvulus could lead to
mesenteric vasculer and lymphatic obstruction, resulting in
bowel edema, loss of protein rich chyle into the bowel lumen
causing hypoprotinanemia, and impaired absorption and
nutrient transport leading eventually to protein-calorie mal-
nutrition.1,3,6 In addition, intestinal ischemia from chronic
volvulus can lead to occult gastrointestinal hemorrhage and
anemia.1 We noted evidence of chronic volvulus at laparotomy
in our cases. Similarly, our laboratory studies revealed hypo-
proteinemia, hypoalbunemia, and anemia in all patients
(Table 2).
In this type clinical presentation mode of MA, diarrea and
failure to thrive are the two primary signs of chronic midgut
volvulus.6,7 This vague mode has led many clinicians to
attribute these complaints to other more common causes; MA
170 M Gmamo]lu et al.
is easily overlooked as a potential source of malabsorption-
like symptoms, and consequently relevant investigation is
delayed.3 In patients in the study reported by Spigland et al.,
the mean delay in diagnosis was 20 months.2 In the present
cases, the mean duration of diagnosis after onset of symptoms
was <1 year in only one patient and ranged from 1 year to
6 years in the remaining three, with an average duration of
35 months. Different from the other causes of malabsorption,
patients with MA have intermittent bilious/non-bilious
vomiting attacks, and chronic, sometimes colicky, abdominal
pain due to intermittent volvulus. Therefore, in the patients
who present mainly with diarrhea and failure to thrive but
had a history of these symptoms, awareness of the possibility
of MA prevents unnecessary delays in diagnosis and
treatment, thus minimizing the morbidity.
Late presentation of MA at variable times after previously
documented normal abdominal films has been reported.2 All
four patients in the present series had films early in life that
were interpreted as normal by clinicians (air-fluid levels in
several films had been attributed to presence of diarrhea).
This also contributed to the diagnostic delay; thus, a previously
normal abdominal film does not preclude the possibility of
MA.2 Likewise, a normal abdominal film can be obtained
after an initial abnormal one because of spontaneous reduction
of volvulus. However, when an abdominal film is obtained
during a volvulus attack, a distended stomach, sometimes
with dilated proximal duedonum (double-bubble sign), and
air-fluids levels in the abdomen with the absence of a normal
colonic gas pattern could demonstrate plain radiographic
findings of malrotation.3 In one of our patients, double-
bubble sign was seen on the delayed plain film which was
thought to represent duodenal obstruction at admission
(Fig. 1). In the other three patients, abdominal films obtained
during the attack of abdominal pain and/or vomiting demon-
strated a distended stomach and air-fluids levels in the upper
abdomen with reduced colonic air pattern, and thus the
presence of MA was suspected. Upper GI contrast series are
essential for diagnosis of MA. The typical diagnostic findings
in the classical type of malrotation are obstruction of the
duodenum with the appearance of extrinsic compression,
occasionally with torsion (corkscrew appearance), and filling
of the jejunal loops on the right side of the abdomen, as
occurred in three of our patients (Fig. 2).3
Classically, the choice of operation is the Ladd procedure.
In older patients who present with acute symptoms related to
unsuspected MA, rapid diagnosis should be made and urgent
surgery is mandatory. Although there is no apparent consensus
regarding approach to the management of children with a non-
acute presentation of MA or non-specific symptoms accom-
panied by a secondary reported finding of MA on upper GI
contrast study, surgery is recommended in the patients with
chronic volvulus findings, as in three of our patients.8
In summary, our study emphasizes the diagnostic difficul-
ties in children with late-presenting MA. Therefore, a high
degree of suspicion is required in order to avoid delay and
inappropriate management of these patients. MA should be
included in the differential diagnosis of children who present
with a history of long-standing malabsorption-like symptoms
despite appropriate evaluation, even associated with normal
abdominal films.
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