Intestinal malrotation

Case contributed by Dr Skúli Óskar Kim

Presentation

A 47-year-old man presented to our emergency department with cramping, constant right abdominal pain. An avid cyclist, his symptoms began 12 hours before admission. A day prior, the patient started experiencing light, constant abdominal pain in the afternoon, but decided to go cycling 70 km/h. During the night, the abdominal pains grew increasingly worse in intensity and was primarily localized to the right iliac fossa with associated vomiting without relief of nausea. His last bowel movement the day before was described as normal. He had a history of surgery as a child operated on for umbilical hernia which had later recurred, bilateral open surgery due to inguinal hernia and appendectomy. The patient had also been hospitalized 2 years prior with upper abdominal pain where Mallory-Weiss lesions were discovered during gastroscopy and a year after that hospitalized with similar abdominal pain without diagnosis. He was on no current medications. On physical examination, the patient’s vital signs were: blood pressure 143/78mmHg, pulse 56/min, temperature 36,6°C and respiratory rate 20/min. He was awake, alert and oriented, a little pale, warm, dry and restless. On initial examination his abdomen was soft and with direct palpation tender mainly in the right iliac fossa with no indirect tenderness and normal bowel sounds. A bulge in the right fossa was palpated during examination. His rectal examination was normal. He was admitted and managed with intravenous fluid hydration under continuous re-evaluation. White blood cell count was minimally elevated, but hemoglobin and basic biochemistry reports were all within normal values.

Patient Data

Age: 47
Gender: Male
CT

Contrast CT scan of abdomen

Contrast CT scan of the abdomen showed malrotation of the small intestine with the jejunum and a large part of the ileum placed on the right side of the abdomen with dilated, fluid-filled ileum loops. In addition, signs of low signal in the mesentery gave suspicion of peritonitis and free intraabdominal fluid. Small air bubbles were seen in the right hypochondrium. There were visualized dilated loops of ileum up to the ileocecal junction with “fecal sign”. Based on the diagnosis of malrotation, the patient consented for exploratory laparoscopy with the possibility of conversion to laparotomy. 

Case Discussion

Intestinal malrotation is a congenital anomaly resulting in the lack of or incomplete rotation of the fetal intestine around the axis of the superior mesenteric artery. Symptoms usually present in early childhood, but are rarely seen in adults. Rotational anomalies, which may or may not be symptomatic, are estimated to occur in between 1/200 and 1/500 live births [1,2] and incidentally are discovered later in life at surgery for other conditions [3-5]. Symptomatic malrotation is estimated to occur in 1/6000 live births [1,6]. Rare adult cases have been reported [7-10]. Adult-age intestinal malrotation has a nonspecific presentation and is difficult to diagnose due to progressively decreasing suspicion for malrotation in the older population.

Intestinal malrotation is any deviation from the normal 270 degree counterclockwise rotation of the midgut around the superior mesenteric artery that begins at the 6th gestational week with a prolapse into the umbilical cord and return of the midgut into the abdominal cavity between the 10th and 12th week. This rotation has been divided into 3 stages and anomalies in rotation are associated with each stage:

Stage I anomalies are nonrotation anomalies where the midgut fails to return to the abdomen, developing an omphaloceles.

Stage II anomalies are duodenal malrotation anomalies that include nonrotation, malrotation, reversed rotation and paraduodenal hernias.

Stage III anomalies are combined duodenal and cecal malrotation anomalies that include an unattached duodenum, mobile cecum, internal hernias, midgut volvulus, incomplete fixation of hepatic flexure of the colon, intermittent duodenal obstruction by Ladd bands and an unattached small bowel mesentery [11,12]. Ladd bands are bands fixing the duodenum to the retroperitoneum and cecum that continue to form in spite of incomplete rotation of the cecum [13].

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Case information

rID: 31936
Case created: 6th Nov 2014
Last edited: 26th Sep 2015
Inclusion in quiz mode: Excluded

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