Intestinal Malrotation with
Volvulus in Early Adolescence
Joshua B. Rogers, MD—Guest Author
Roslyn Stewart, MD, MS, FAAP, FACP
Lenny Feldman, MD—Column Editors
A 14-year-old boy presented to the University of
Maryland emergency department due to constant abdominal pain for 3 days and vomiting, which had been intermittent over the prior 2 weeks. He described having episodic
abdominal pain with vomiting 2 or 3 times per year for the
past 3 years. Each episode lasted approximately 2 days
before resolving spontaneously.
Physical examination revealed a 115-kg male with a
heart rate of 101 beats per minute in moderate distress.
Abdominal examination revealed an obese but soft
abdomen with normally active bowel sounds and tenderness
localized to the epigastrium. He exhibited voluntary
abdominal guarding, but no rebound tenderness. Rectal
examination was negative.
The white blood cell count was 9.0 x 109/L, bicarbonate 30 mEq/L, anion gap 17, and chloride 95 mEq/L. All
other electrolytes were within normal ranges. Urinalysis
revealed specific gravity greater than 1.030 with 3+ ketones.
Computerized tomography (CT) revealed a mid-abdominal
transition area, beyond which there was no evidence of contrast-filled small bowel. An area of concentric small bowel
was noted. There was no evidence of free fluid or free air
(Figure 1). The CT scan was suggestive of intussusception
but also as consistent with volvulus, prompting a fluoroscopic small bowel evaluation. This study revealed mild dilation of the first 2 sections of duodenum; a tapered, highly
obstructive appearance of the third portion of the duodenum; and a corkscrew configuration in the duodenal junction region—highly suggestive of volvulus (Figure 2). The
duodenal-jejunal junction was also abnormally low and
rightward in location, consistent with malrotation.
Exploratory laparotomy revealed a midgut volvulus of 540
degrees, in addition to malrotation with no evidence of
bowel ischemia or necrosis. A Ladd procedure was performed with an appendectomy, and the patient recovered
uneventfully.
Intestinal malrotation is thought to occur in 1 in 200 to
500 newborns, but is symptomatic in only 1 in 6000.1-3 Up
to 90% of malrotations with volvulus require surgical repair
during the first year of life.4 Thirty percent to 60% of
infants diagnosed with malrotation have an associated gas-
trointestinal anomaly, ranging from diaphragmatic hernia to
omphalocele.5,6 These rotational anomalies form during the
4th to 10th week of gestation. Malrotation typically results
in the cecum resting in the mid-upper abdomen, with
attachments to the posterior wall via peritoneal bands (Ladd
bands).
Figure 1.
Computed tomography scan reveals an area of concentrically small bowel, distal to which there are no contrast-filled loops.
Figure 2.
Upper gastrointestinal study demonstrating mild dilation of the first 2 portions
of the duodenum followed by a tapered and highly obstructive appearance of
the third portion of the duodenum with a “corkscrew” appearance.
Dr Rogers is with the Resident Division of Emergency Medicine, Department of Surgery, University of Maryland School of Medicine, Baltimore, Maryland.
Dr Stewart is Assistant Professor, Department of Medicine, Division of General Internal Medicine, Johns Hopkins University, Baltimore, Maryland.
Dr Feldman is Assistant Professor of Medicine, Department of Medicine, Division of General Internal Medicine–Hospitalist Service, Assistant Program Director,
Osler Medical Residency, Director, Comprehensive General Medicine Consult Service, Johns Hopkins Hospital, Johns Hopkins University, Baltimore, Maryland.
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CLINICAL IMAGES IN MEDICINE
Newborns or infants with intestinal malrotation classically and commonly, 97% of the time, present with bilious
emesis and constipation.7 Other possible findings include
hematochezia and abdominal distention, although these
symptoms may indicate intestinal ischemia.7,8 Symptoms are
more varied and nonspecific with increasing age and can
include episodic attacks of vomiting, failure to thrive, and
recurrent colicky abdominal pain.4,9-11 The intermittent
symptoms of older children often result in diagnostic delays
as the patients are diagnosed with more common ailments:
irritable bowl, peptic ulcer disease, pancreatic disease, and
psychiatric disorders.12 These delays are important because
the most serious consequence of volvulus is bowel necrosis
and resultant shock, sepsis, and death.
The treatment for volvulus is surgical. The Ladd procedure, the most common surgical technique, involves
untwisting the bowel, removing any nonviable bowel, and
placing the bowel in a position of nonrotation. A prophylactic appendectomy is usually also done.
2. Warner B. Malrotation. In: Oldham KT, Foglia RP, ed. Surgery of
Infants and Children: Scientific Principles and Practice. Philadelphia:
Lippincott Williams & Wilkins; 1997:1229.
3. Berseth C. Disorders of the intestines and pancreas. In: Taeusch WH,
ed. Avery’s Diseases of the Newborn. 7 ed. Philadelphia: WB Saunders;
1998:918.
4. Ilce Z, CelayIr S, Akova F, et al. Intestinal rotation anomalies in childhood: review of 22 years’ experience. Surg Today. 2003;33:893-895.
5. Prasil P, Flageole H, Shaw KS, et al. Should malrotation in children be
treated differently according to age? J Pediatr Surg. 2000;35:756-758.
6. Spigland N, Brandt ML, Yazbeck S. Malrotation presenting beyond
the neonatal period. J Pediatr Surg. 1990;25:1139-1142.
7. Bonadio WA, Clarkson T, Naus J. The clinical features of children with
malrotation of the intestine. Pediatr Emerg Care. 1991;7:348-349.
8. Rescorla FJ, Shedd FJ, Grosfeld JL, et al. Anomalies of intestinal rotation in childhood: analysis of 447 cases. Surgery. 1990;108:710-715;
discussion 715-716.
9. Brandt ML, Pokorny WJ, McGill CW, Harberg FJ. Late presentations
of midgut malrotation in children. Am J Surg. 1985;150:767-771.
10. Maxson RT, Franklin PA, Wagner CW. Malrotation in the older
child: surgical management, treatment, and outcome. Am Surg.
1995;61:135-138.
11. Yanez R, Spitz L. Intestinal malrotation presenting outside the neonatal
period. Arch Dis Child. 1986;61:682-685.
12. Fukuya T, Brown BP, Lu CC. Midgut volvulus as a complication of
intestinal malrotation in adults. Dig Dis Sci. 1993;38:438-444.
References
1. Dilley AV, Pereira J, Shi EC, et al. The radiologist says malrotation:
does the surgeon operate? Pediatr Surg Int. 2000;16:45-49.
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