Intestinal malrotation is a congenital defect that occurs early in pregnancy when a baby's intestines fail to form into a coil in the abdomen. Malrotation means, in other words, that the bowels are abnormally located within the abdomen. This can lead to dangerous complications such as volvulus (twisting around mesentery) [1]. The definite cause of intestinal malrotation is yet to be discovered. Despite not being associated with a specific gene, there is few evidence of occurrence in familiar pattern [2]. Congenital anomalies of rotation of small intestines vary in type depending on the degree of rotation. The major presenting manifestation of these anomalies is the clinical picture of intestinal obstruction; a big interest in pediatric surgery [3].

A nine and a half years old boy, previously healthy, with a virgin abdomen, presented to our emergency department with 2 days history of diffuse intermittent abdominal pain. Pain was associated with greenish- yellowish vomiting of 3 to 4 times per day, decrease oral intake, and constipation. Patient was mildly tachycardic despite being afebrile with maintained urine output. Oxygen saturation was normal. Physical exam was significant for a soft but diffusely tender abdomen. No history of erosive agents or medications ingestion. Laboratory tests were ordered. Patient was managed symptomatically while waiting for a conclusive diagnosis. Blood count and inflammatory markers were within normal range (Table 1). Liver and pancreatic enzymes were also normal.                 

Table 1: Laboratory Tests Performed on Presentation and Day 3 after Surgery

A standing abdominal x-ray was ordered (Figure 1) and showed few distended bowel loops of the small intestine in the left upper abdominal quadrant. Image had volvulus aspect.

Figure 1: KUB done on first day of presentation (7/2/2021)

Figure 1 showing distended few bowels of the small intestine in LUQ, with tendency to volvulus aspect CT scan of the abdomen and pelvis with PO and IV contrast was performed (Figure 2), showing “intestinal malrotation, associated with volvulus and sub-occlusion at the level of the duodenum, associated with gastric and proximal duodenal distention”.

Figure 2: CT-scan done, confirming the diagnosis of volvulus, showing duodenal volvulus with intestinal malrotation

Pediatric surgery team was immediately consulted, and the patient was taken emergently to the operating room where laparotomy was performed as shown in Figure 3. De-volvulus of the duodenum followed by Ladd’s procedure (colon fixation on left, small bowels on right, and appendectomy) was performed.

Figure 3 as described by the pediatric surgeon, where de-volvulus was done followed by Ladd procedure. Patient was then admitted to regular floor. Post-op recovery was smooth and fast; he tolerated progressive PO feeding advancement. Follow-up labs were done on day 3 post-op with normal values. Patient was then discharged home on day 5 post-op and showed proper surgical recovery.

Figure 3: intestinal volvulus at the level of duodenum (boucle),

Symptomatic malrotation incidence is estimated to be 1:6000 among live births [4]. Male patients are more affected than female, with a 2:1 ratio. Patients with malrotation are at increased risk of volvulus which manifests typically with sudden onset of bilious emesis [5]. The most common age presentation is early in life. About 50% of affected patients present within the first week of life, and more than 60% before completing the first month. Only rarely do patients present later in life as in our case [6], in which the age was 9 years. Our case represents a case of congenital intestinal malrotation, complicated by midgut volvulus and sub occlusion at the level of the duodenum. The most common presentation is that of biliary emesis. All cases of intestinal malrotation that are complicated by volvulus should be treated surgically to prevent further damage to the patient, such as necrosis, secondary peritonitis, malabsorption, and severe sepsis, which can all lead to death. Early diagnosis and treatment are the cornerstones [7]. Malrotation is treated operatively ideally after appropriate resuscitation. A patient with suspected midgut volvulus should be operated emergently without any delay, as that might put the bowel at risk for irreversible ischemia [8,9,10]. During the operation, the involved bowel part is untwisted in a counter clockwise direction and observed for blood flow [10]. Next step then would depend on whether the involved segment of bowel is ischemic or viable. When bowel appears viable after detorsion, the malrotation is treated by the Ladd procedure [11,12]. In Ladd procedure, division of the adhesive peritoneal bands that are at the origin of the malrotation takes place, by that relieving obstruction and straightening duodenal course. Prophylactic appendectomy is also done because the cecum will be fixed to the left side during the procedure making presentation of appendicitis non-typical. Majority of patients undergoing Ladd procedure recover and recurrence of volvulus is possible but rare [10].

Congenital intestinal anomalies commonly present during the first year of life, and the major clinical presentation is obstruction, causing bilious vomiting, abdominal distention and obstipation. Some may manifest later, even in adulthood. The cornerstone is the early diagnosis and intervention to avoid severe and irreversible complications.

1. Millar, A.J.W. et al. “Malrotation and Volvulus in Infancy and Childhood.” Seminars in Pediatric Surgery, vol. 12, no. 4, November 2003, pp. 229–236.

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3. Filston, H.C., and D.R. Kirks. “Malrotation—The Ubiquitous Anomaly.” Journal of Pediatric Surgery, vol. 16, no. 4, 1981, pp. 614–620.

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5. Ford, E.G. et al. “Malrotation of the Intestine in Children.” Annals of Surgery, vol. 215, no. 2, 1992, p. 172.

6. Powell, D.M. et al. “Malrotation of the Intestines in Children: The Effect of Age on Presentation and Therapy.” Journal of Pediatric Surgery, vol. 24, no. 8, 1989, pp. 777–780.

7. Heidsma, C.M. et al. “[Malrotation with or without Volvulus].” Nederlands Tijdschrift voor Geneeskunde, vol. 159, 2015, p. A8859.

8. Fraser, J.D. et al. “The Role of Laparoscopy in the Management of Malrotation.” Journal of Surgical Research, vol. 156, no. 1, 2009, pp. 80–82.

9. Nehra, D., and A.M. Goldstein. “Intestinal Malrotation: Varied Clinical Presentation from Infancy through Adulthood.” Surgery, vol. 149, no. 3, 2011, pp. 386–393.

10. Tsumura, H. et al. “Successful Laparoscopic Ladd's Procedure and Appendectomy for Intestinal Malrotation with Appendicitis.” Surgical Endoscopy and Other Interventional Techniques, vol. 17, no. 4, 2003, pp. 657–658.

11. Lampl, B. et al. “Malrotation and Midgut Volvulus: A Historical Review and Current Controversies in Diagnosis and Management.” Pediatric Radiology, vol. 39, no. 4, 2009, pp. 359–366.

12. Hagendoorn, J. et al. “Laparoscopic Treatment of Intestinal Malrotation in Neonates and Infants: Retrospective Study.” Surgical Endoscopy, vol. 25, no. 1, 2011, pp. 217–220.