INTESTINAL MALROTATION: An Atypical
and Delayed Presentation
ARSHAD S KHAN*, GIRISH D BAKHSHI**, KUNDAN K PATIL+,HEMANT G BORSE+, ANSHUL
*Associate Professor and Unit
Head; **Lecturer; +Resident, Department of Surgery, Grant Medical College
and JJ Group of Hospitals, Mumbai 400008, India.
syndromes are typically presented during the first few months of life but may
sometimes appear later in life, causing difficulties and mistakes in diagnosis.
We present a case of 15 years old female who presented with recurrent episodes
of vomiting. Diagnosis was delayed because respective symptoms were not adequately
considered in older children and adults.
Many individuals with intestinal malrotation never develop the syndrome in the
older age. Patients who were previously treated for congenital diaphragmatic
hernia or congenital abdominal wall defects have intestinal malrotation as an
associated anomaly. In most of the patients followed up
after Ladd procedure due to intestinal malrotation in infancy there was no recurrence
of the symptoms. Ladd procedure stabilizes but does not
remove the anomaly. Also, there are people who are not aware that they have
the anomaly. Minor or severe abdominal disorders may be present for a long time
or the anomaly may be completely asymptomatic.
Malrotation syndromes are clinically evident in the first month of life in 64%
of the patients with the syndrome and in 82% in the first year of life.The
onset of clinical presentation is rare later in life  and
the symptoms are obscure and usually of prolonged duration. The diagnosis is
often delayed and various others, including psychogenic disorders, are made
before a final, correct one. Bill divided intestinal malrotation
according to the time of occurrence in three embryonic stages.
Malrotation syndromes in children are most commonly presented as Ia or IIIa
type. These are also the most serious types. Clinically, they are manifested
as duodenal obstruction caused by volvulus of the midgut.
They become apparent when the gut is filled with air in the first days of life.
If a complete volvulus does not develop, the patients present with the symptoms
of subtotal obstruction of the duodenum and the ileus develops later. Other
types are very rare and are often found accidentally at operations performed
for other reasons. After the second year of life, the manifestations of malrotation
are very rare. These patients develop stable subtotal obstruction of the duodenum
and/or intermittent volvulus. The prevalence of nonrotation
without volvulus and symptoms of obstruction is not known.
A 15 year-old female presented
with recurrent vomiting episodes since 3 months. She was intensely vomiting
for three days prior to the admission and developed symptoms of dehydration.
On admission, the abdominal wall was soft with minimal distension in epigastrium.
Peristalsis was weak. Abdominal X-ray was unremarkable.Upper GI endoscopy revealed
dilated stomach and duodenum with patulous pylorus. Contrast examination revealed
giant, dilated stomach with obstruction of the third part of the duodenum (Fig.1).
Ultrasonography showed superior mesenteric vein to the left of superior mesenteric
artery, which is pathognomonic of malrotation. Contrast C.T.scan revealed (Fig.2)
a classical "whirl-pattern" seen in malrotation with midgut volvulus.
 On surgery, nonrotation of type IIIa with volvulus of the
midgut was found. There were no ischaemic changes of the gut and Ladd procedure
was per formed. Postoperatively, the clinical status improved
and normalized with weight gain of 2 kg in one month without any complications.
1 Barium study showing dilated stomach and duodenum
with obstruction of the third part of duodenum.
2 CT scan showing "Whirl sign" of midgut volvulus.
Midgut volvulus is a complication
of bowel malrotation. During embryologic development, intestinal rotation primarily
occurs in the midgut, the segment of bowel supplied by the superior mesenteric
artery. During the late stage of gut rotation, anchoring of the mesentery and
bowel to the posterior abdominal wall occurs. The small-bowel mesenteric root
forms a diagonal line from the Treitz’s ligament to the caecum. In malrotation,
the mesenteric root is shortened, which allows volvulus to occur. Malrotation
is also associated with peritoneal or Ladd’s bands, which can cause duodenal
The reason for a delayed presentation of malrotation syndromes is not known.
There is a balance between the narrowing of the duodenum and the torsion of
the midgut. The degree of the duodenal obstruction may influence clinical symptoms.
It may be obstructed enough to cause some disorders, but not enough to cause
ileus. The syndrome appears at the point of critical narrowing.
This implies that many people live with asymptomatic intestinal malrotation
or have only small disorders. Our patient remained asymptomatic till 15 years
and then started developing symptoms. Intermittent vomiting in the older child
is sometimes mis-diagnosed. Many children carry the diagnosis of psychogenic
vomiting for months or years before the correct diagnosis of malrotation is
made.  Delayed diagnosis of intestinal malrotation may
be responsible for severe dehydration. as in our case.
Modern ultrasound examination is very helpful in the diagnosis. The position
of the superior mesenteric vein left to the artery suggests malrotation in almost
all cases. The position of the vein anterior to the artery is found in 30% cases
of intestinal malrotation.  This method is applicable
as a screening test when considering a differential diagnosis. Ultrasonography
was diagnostic in our case also and CT scan was done for academic interest.
The most common type of malrotation is Ia or IIIa.  In our
case there was IIIa type of malrotation for which Ladd procedure was done. Postoperatively
patient became asymptomatic with weight gain of 2 Kg in 1 month.
In summary, malrotation leading to midgut volvulus is rare in adults, however
it should be considered as a differential possibility in the diagnosis of abdominal
disorders in older children and adults. Ultrasonography can be used for screening
in diagnostic dilemma. The CT findings in midgut volvulus can be pathognomonic
if the classic whirl pattern around a central superior mesenteric artery is
seen. CT allows the rapid diagnosis of this unusual condition in the adult patient
presenting with abdominal pain or bowel obstruction of uncertain cause. Surgery
in the form of Ladd procedure is the treatment of choice.
We would like to thank Dean and Dr. MG Rathod, Head of the Department of Surgery,
Grant Medical College and JJ Group of Hospitals for granting us permission to
publish this case report.
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