Malrotation with midgut volvulus associated with perforated ileal duplication.

Duplication of the alimentary tract is an important surgical condition. It may occur anywhere in the gastrointestinal tract. An important complication of this entity is perforation of the normal or abnormal gut. Malrotation with midgut volvulus can be a surgical emergency. We present a patient, who presented as malrotation with midgut volvulus associated with perforated ileal duplication. The patient was successfully managed.

INTRODUCTION

Duplication of the alimentary tract is an important surgical condition, which includes a wide variety of mass lesions.[1] It may occur anywhere in the gastrointestinal tract. An important complication of this entity is perforation of the normal or abnormal gut.

We encountered a patient who presented with perforation of the duplication, which was associated with malrotation with midgut volvulus. Being an extremely uncommon surgical emergency, it is being presented with a brief review of the relevant literature.

CASE REPORT

A 4-year-old male child was referred to us for complaint of pain in abdomen. On enquiry, there was intermittent history of bilious vomiting since infancy. There was no previous history as regard to abdominal distension. However, there was occasional history of abdominal pain, which was relieved by medication. On examination, his abdomen was distended, but it was non-tender. The weight of the patient was 9 kg. Thus he had failed to thrive. His ultrasound (USG) of abdomen suggested some fluid filled cystic spaces. A computerized tomography (CT) scan of abdomen was advised for confirmation of the diagnosis.

On next day, just after CT abdomen, the patient suddenly developed severe abdominal pain. X-ray abdomen showed pneumoperitoneum. Clinically the patient looked septic. His pulse rate was 130/min with a feeble pulse. An urgent laparotomy was planned. After initial stabilization, the patient was explored under general anesthesia. On exploration, we noticed malrotation of the gut, which was associated with two turns of midgut volvulus. The midgut did not have any evidence of ischemia or gangrene. It was complete malrotation having cecum in left hypochondrium and narrow mesentery. Ladd's bands were also present. The peritoneal cavity was grossly contaminated with fecal fluid. The perforation site was about one foot proximal to ileocecal region. The perforation was at the mesenteric side, and it was associated with a soft mesenteric mass communicating with the normal bowel. The mass was about 10 cm in length. The perforation was just at the junction of the mesenteric mass and the normal bowel [Figure 1].

Figure 1

Intraoperative photo showing perforated duplication. Mosquito forceps and one thumb forceps are in the distal duplicated lumens. The other thumb forceps is in the perforated duplication

After performing the Ladd's procedure, attention was placed towards the perforation. On attempting to resect the mesenteric mass, we noticed two lumens in the bowel distal to the perforation site and single lumen proximal to that [Figures 2 and 3]. The duplication appeared to be having all the layers of the bowel wall. When an attempt to ascertain the length of the duplication was made by inserting two Ryle's tube through the lumens, it was reaching up to ileocecal region. As the general condition of the patient was poor, complete resection of the bowel was not done, and a double barrel (triple barrel, to be more precise) was fashioned.

Figure 2

Intraoperative photo showing duplicated lumen

Figure 3

Line diagram showing duplication and normal bowel. (a) Duplication cyst, (b) junction of duplication and normal bowel, and it was the site of perforation, (c) normal ileum, (d) duplicated ileum reaching up to cecum, and (e) cecum and probable site of duplication termination

In the postoperative period, there was wound infection with superficial wound dehiscence. It was managed conservatively. The patient was discharged after 3 weeks with proper follow-up advise. During the follow-up, we had closed the ileostomy. As the duplication was reaching up to cecum, we had performed ileoascending anastomosis. The patient is fine.

DISCUSSION

Congenital anomalies of the gastrointestinal tract are a significant cause of morbidity in children. These abnormalities include developmental obstructive defects of the small intestine, anomalies of rotation and fixation, intestinal duplications, etc.[2] Duplications of the alimentary tract are rare congenital malformations and may occur anywhere in the intestinal tract. Intestinal duplication cyst has been rarely found with intestinal malrotation.[13]

In our patient, the history of bilious vomiting was suspicious of small bowel anomaly. The initial suspicion, on the basis of USG, was of some cystic mass lesion compressing the upper small bowel. The exact diagnosis, however, was confirmed only at laparotomy. The CT plates, reviewed later on, also showed twisting of mesenteric vessels suggestive of malrotation. The exact cause of perforation was not clear. Probably the perforation of the duplication was not spontaneous; it may be because of the volvulus and resultant ischemia of the wall.

As malrotation with midgut volvulus is always a surgical emergency, and perforated duplication is also a surgical emergency, a combination of the two entities is definitely a surgical emergency, which needs immediate intervention. The ideal treatment would have been a Ladd's procedure, along with resection of the complete duplication. This was, however, not possible due to the poor general condition of the patient and large length of the duplication, which may had led to extensive resection of the bowel. Hence, ileostomy was made.

The association of duplication of small bowel and malrotation, though described in the literature, is a rare one. Our English literature review revealed less than 25 patients.[2345678] None of the patients had a presentation of acute abdomen due to perforated duplication.

To conclude, this being one of a very rare combination of two pediatric surgical emergencies; needs proper surgical and postoperative management. The management needs to be tailored as per the condition of the patient. The exact preoperative diagnosis may be difficult, but a high degree of suspicion is warranted.