A 3-Day-Old Boy With Bilious Emesis

Jamie Shalkow, MD


April 14, 2016

Once the diagnosis is made, surgery must occur as soon as possible. A delay in treatment may result in bowel necrosis of a large section of small intestine (which must then be resected) or even death. A patient should be rushed into the operating room while resuscitation continues. The procedure of choice, the Ladd procedure, was first described by Dr William Ladd in 1932.[1] William Ladd is considered the "father of pediatric surgery." He was the first surgeon to devote his entire practice to the surgical care of children.

In the Ladd procedure, the abdomen is approached through a transverse supraumbilical incision. The first step is to untwist or "devolvulate" the bowel. On first inspection, it is sometimes difficult to realize in which direction the bowel has twisted. Derotation is always done in a counterclockwise fashion because the bowel always twists in a clockwise direction. The abnormal peritoneal attachments of the duodenum, right colon and cecum (Ladd bands) that create the narrow mesenteric base between the duodenum and cecum are then divided. This step widens the base of the small bowel mesentery by allowing maximal separation between the duodenum and the cecum, thereby preventing further twisting. The bowel is then returned to the abdominal cavity in a nonrotated position (leaving the small bowel in the right abdomen, and the colon on the left).

Fixing the bowel loops in this position with sutures has not proven to avoid further incidences of volvulus, and it has increased the incidence of intestinal postoperative complications, so it is not recommended.[1] An incidental appendectomy is also usually performed to avoid diagnostic dilemmas in the future. This can be done in a regular fashion. Finally, an orogastric tube is passed into the duodenum to rule out duodenal atresias or webs, which may occur concomitantly with malrotation. Postoperatively, the patient is kept NPO, with gastric decompression, administration of intravenous fluids, and antibiotic coverage. Depending on the intestinal damage encountered and the expected time to recovery, total parenteral nutrition may be required.

As much bowel should be preserved as possible during the procedure in order to avoid short bowel syndrome. No anastomoses should be performed in a bowel with questionable viability so as to avoid the risk of anastomotic leak.[5] Full intestinal resection entails several ethical considerations; these are beyond the scope of this presentation. Postoperative complications may include recurrent volvulus (2%-6%), short bowel syndrome, adhesions causing bowel obstruction, postoperative intussusceptions, and the need for total parenteral nutrition. Mortality rates have been reported to be between 2% and 24%, depending on the extent of bowel necrosis, associated anomalies, and the age of the patient.[3] Less than 10% necrosis at the time of surgery carries nearly a 100% survival rate; however, 75% necrosis at the time of surgery has only a 35% survival rate.[2]

In this case, the clinical picture and the initial plain abdominal film were considered enough to support the diagnosis of malrotation with midgut volvulus. The patient was brought to the operating room as resuscitation was continued. The abdomen was entered through a transverse supraumbilical wide incision, and the entire midgut (from the ligament of Treitz to the mid-transverse colon) was completely necrotic. The chances for survival were dismal. The case was discussed with the family. The child's surgical incision was sewn up and he was allowed to expire in a peaceful way under palliative care. This case underscores the catastrophic outcomes that these patients may have if not diagnosed and treated in a timely fashion.


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