Case Challenge
Radiology
A 16-Year-Old Girl with
Chronic Intermittent Abdominal Pain
Bheesham D. Dayal, BSc; and Nida Blankas-Hernaez, MD, FAAP
A
previously healthy 16-yearold girl was admitted to the
emergency room and ultimately to the hospital floor with a
1-day history of severe abdominal
pain. Her past medical history consisted of intermittent abdominal pain for
6 years without any identifiable cause.
She had no changes in bowel habits or
history of abdominal surgery, and her
family history was negative for any
gastrointestinal diseases. Two days
before presentation, she presented to
our office with dysuria and lower abdominal pain, and sulfamethoxazoletrimethoprim was begun after urinalysis and urine culture were performed.
The urinalysis showed a trace of
Bheesham D. Dayal, BSc is a fourth-year medi-
cal student at Windsor University School of Medicine, St. Kitts and Nevis, West Indies. Nida BlankasHernaez, MD, FAAP, is an Associate Professor with
the Department of Pediatrics, Lutheran General
Hospital, Park Ridge, IL.
Address correspondence to: Nida BlankasHernaez,
MD;
fax:
847-972-1926;
email:
beeshamd@aol.com.
Mr. Dayal and Dr. Blankas-Hernaez have disclosed no relevant financial relationships.
doi: 10.3928/00904481-20111209-03
blood, but was otherwise normal. The
culture ultimately was negative.
The patient’s symptoms improved
at first, but 1 day after she started
taking the antibiotics, she developed
severe abdominal pain and vomiting.
She went to the emergency department, where intravenous fluids were
administered along with an antiemetic
(ondansetron). On physical examination, the patient’s vital signs were as
follows: temperature 97.8°F, pulse
86/min, blood pressure 115/77 mm
Hg, and respiratory rate 24 breaths/
min. She was a normally developed
girl who appeared uncomfortable but
was in no acute distress. Her abdomen
was soft without distention, and she
exhibited no peritoneal signs. Normal
bowel sounds were auscultated. There
was notable tenderness to palpation,
especially over the epigastrium, the
left periumbilical area, and her left
and right flanks. In addition, there was
also mild tenderness over McBurney’s
point, but obturator, psoas, Murphy,
and Rovsing signs were all negative.
The remainder of the examination was
unremarkable. Her rectal examination
was normal and pregnancy test was
negative. Hemoglobin, white blood
Figure 1. Upper gastrointestinal contrast study
confirms the diagnosis of malrotation as the
small bowel (SB) is found to be located entirely
in the patient’s right abdomen, while the colon
is located in the left abdomen, and the cecum (C)
is seen in the left lower quadrant. Source: Dayal
B, Blankas-Hernaez N. Reprinted with permission.
cell count, and basic chemistry panel
were all within normal values. Urinalysis showed negative nitrates and
leukocyte esterase; however, there
were a few bacteria present with one
to five squamous epithelial cells; one
to five WBC; and one to two red cells.
For her presumed urinary tract infection, the antibiotic was continued. A
computerized tomography (CT) scan
of the abdomen and pelvis with oral
and intravenous contrast and upper GI
with contrast (see Figure 1) were obtained and revealed the diagnosis.
For diagnosis, see page 10.
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Case Challenge
Diagnosis:
Intestinal Malrotation
The abdominal CT scan and upper
GI showed malpositioning of her small
bowel, which was located almost entirely on the right side of the abdomen.
In addition, the orientation of the superior mesenteric vessels was abnormal
(Figure 2), which was highly suggestive for malrotation of the bowel without evidence of volvulus. The patient
was then admitted and managed with
nasogastric tube decompression and
intravenous hydration. An upper gastrointestinal contrast study confirmed
the suspected diagnosis of intestinal
malrotation. It showed the small bowel
located entirely in the right abdomen,
the colon located in the left abdomen,
and the cecum in the left lower quadrant (Figure 1, see page 9).
DISCUSSION
Intestinal malrotation is an anomaly of fetal intestinal development believed to occur in one per 200 to one
per 500 live births, with symptomatic
malrotation occurring in one per 6,000
live births. Symptomatic malrotation
is clinically evident in the first postnatal month in 64% of patients; 82% are
diagnosed in the first postnatal year;
18% to 25% of symptomatic patients
are diagnosed at 1 year of age and older.1 Between 30% and 62% of pediatric patients with intestinal malrotation
also have an associated anomaly, such
as duodenal atresia (seen in as many
as 17%) and jejunal atresia (33%).
Disorders of intestinal rotation are
also common in infants and children
with congenital diaphragmatic hernia, gastroschisis, and omphalocele.1
However, it is difficult to determine
the true incidence of malrotation be-
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4101CaseChallenges.indd 10
Figure 2. Axial contrast-enhanced computed tomography scan obtained through mid-abdomen shows inverted relationship between the superior mesenteric artery (SMA) (A) and the superior mesenteric vein (SMV)
(B). Source: Dayal B, Blankas-Hernaez N. Reprinted with permission.
cause this condition goes undetected
during childhood in a significant number of patients.
Intestinal Malrotation
Intestinal malrotation can be defined as any deviation from the normal 270° counterclockwise rotation
of the midgut in utero. During fetal
development, the midgut supplied by
the superior mesenteric artery grows
too rapidly to be accommodated in
the peritoneal cavity. Prolapse into
the umbilical cord occurs around the
sixth week. Between the 10th and
12th weeks, the midgut returns to
the abdominal cavity, having undergone a 270° counterclockwise rotation around the superior mesenteric
artery. This rotation of intestinal development has been divided into three
stages:2 Stage I occurs between 5 and
10 weeks’ gestation; Stage II occurs
between 10 and 12 weeks’ gestation;
and Stage III lasts from 12 weeks’
gestation until term.
Symptoms of Malrotation
Many patients with malrotation are
often asymptomatic; it can be discovered incidentally during surgery for
other conditions.3 However, some may
present with chronic symptoms of intermittent bowel obstruction or vague
abdominal complaints and even fewer
may report acute episodes of agonizing abdominal pain.3 Symptoms may
arise from acute or chronic intestinal
obstruction that may be caused by the
presence of abnormal peritoneal bands
(Ladd bands) or a volvulus.4
The classic presentation of malrotation is a newborn infant with bilious
vomiting or any child with bilious
emesis and abdominal pain. Physical
examination may reveal mild distension of the abdomen, diffuse tenderness with or without signs of peritonitis, bloody stool on rectal examination, duodenal obstruction caused by
Ladd’s bands, or associated duodenal
or jejunal atresia.5 In older children,
the clinical presentation of malrota-
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Case Challenge
tion is variable and often insidious.
Approximately 30% present with intermittent vomiting (which may be
nonbilious) and 20% present with intermittent abdominal pain, as in our
case.5 Less common presentations
include failure to thrive, solid food intolerance, malabsorption, pancreatitis,
peritonitis, biliary obstruction, motility disorders, and chylous ascites.5
Volvulus in Malrotation
Volvulus is the most feared complication of intestinal malrotation and
is a clear indication for emergent surgery. It occurs when the small bowel
twists around the superior mesenteric
artery, resulting in vascular compromise to large portions of the midgut.
This leads to bowel ischemia and necrosis that becomes irreversible unless
corrected quickly.5 More than 50%
of children with malrotation present
with volvulus before 1 month of age.
Among older children with malrotation, 10% to 15% present with volvulus. The onset of symptoms is usually
acute, but some children present with
a more chronic pattern of episodic
vomiting and abdominal pain suggestive of intermittent volvulus.5
Screening and Diagnosis
Intestinal malrotation can be
screened for and diagnosed with imaging studies, including an upper gastrointestinal (GI) contrast series, plain radiographs, contrast barium enema, ultrasonography, or abdominal CT scan.
An upper GI contrast series is considered the most reliable because it allows
visualization of the duodenum and has
an accuracy of more than 80%.5 The
upper GI contrast series should be
performed under fluoroscopy and by
an experienced pediatric radiologist
whenever possible. In approximately
75% of cases, the upper GI contrast
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will see obvious signs indicating malrotation, such as a partial obstruction
of the duodenum that may have more
of a “beak” appearance if a volvulus is
present, or an abnormal positioning of
duodenum (ligament of Treitz on right
side of the abdomen) that has a “corkscrew” appearance (Figure 3).5,6
Plain radiographs are rarely diagnostic because they are neither sensitive nor specific for malrotation, so
they are usually followed by an upper
GI contrast series. However, right-sided jejunal markings (Figure 4) or the
absence of a stool-filled colon in the
right lower quadrant on a plain radiograph may suggest intestinal malrotation.7 A contrast barium enema is useful as an adjunct to the upper GI series
and can be used to diagnose volvulus
if it shows complete obstruction of the
transverse colon. Ultrasonographic
findings that suggest of malrotation
include a “whirlpool” sign, which corresponds to a clockwise wrapping of
the superior mesenteric vein (SMV)
and mesentery around the superior
mesenteric artery (SMA) and a dilated
duodenum, which indicates duodenal
obstruction by Ladd’s bands.5,8
Finally, malrotation can be diagnosed on CT by the anatomic location
of a right-sided small bowel, a leftsided colon, aplasia of the pancreatic
uncinate process, or an inverse relationship of the superior mesenteric
vessels (Figure 1, see page 9).9 The
superior mesenteric vein is normally
located to the right of the superior
mesenteric artery, but the relative positions of the vein and artery are reversed in approximately 60% of patients with malrotation.10
However, abnormalities of SMASMV orientation are not entirely diagnostic because some patients with
malrotation have a normal relationship,
whereas an inverted relationship can
Figure 3. Upper GI series clearly demonstrates
the “corkscrew” appearance of the proximal small
bowel (arrows) as it twists around the superior
mesenteric artery in malrotation with a midgut
volvulus. Source: Dayal B, Blankas-Hernaez N.
Reprinted with permission.
Figure 4. Supine frontal abdominal radiograph
shows small bowel with jejunal markings (arrowheads) on the right and colon predominately on
the left in a patient with malrotation. Source: Dayal B,
Blankas-Hernaez N. Reprinted with permission.
also be seen in patients without malrotation.9,10 Therefore, an upper GI contrast series remains the gold standard
for diagnosing intestinal malrotation.
The classic treatment for intestinal
malrotation is the Ladd procedure. It
involves counterclockwise reduction
of the volvulus if present, division
of any coloduodenal bands, widening of the mesenteric base to prevent
repeated volvulus, and prophylactic
appendectomy.11 Generally, symptomatic patients with malrotation require
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Case Challenge
surgical intervention. Management of
asymptomatic patients discovered incidentally to have malrotation is more
controversial, although some surgeons
recommend that all patients with malrotation receive laparotomy.12
CONCLUSION
On hospital day 2, the patient underwent a Ladd procedure to correct her
malrotation, along with an appendectomy. Postoperatively, the patient did
well without any complications. She
tolerated a regular diet on postoperative
day 3 and was discharged on postoperative day 5. The patient made a full
recovery; no signs of recurrence were
found after 12 months of follow-up.
Intestinal malrotation is an important
cause of abdominal pain and should be
considered in the differential diagnosis
of abdominal disorders in neonates and
older children. Many patients with malrotation are asymptomatic, while others
may present with acute or chronic symptoms, such as intermittent intestinal ob-
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4101CaseChallenges.indd 12
struction, nonbilious vomiting, or recurrent abdominal pain. Intestinal malrotation can be screened and diagnosed using various imaging studies; upper gastrointestinal contrast series is considered
the best and most accurate study. The
classic treatment for intestinal malrotation is the Ladd procedure, which entails
counterclockwise reduction of a volvulus if present; division of any coloduodenal bands; widening of the mesenteric
base to prevent repeated volvulus; and
prophylactic appendectomy.
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