Intestinal Malrotation Treatment & Management

Updated: Mar 02, 2017
  • Author: Denis D Bensard, MD, FACS, FAAP; Chief Editor: Carmen Cuffari, MD  more...
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Treatment

Medical Care

Medical care of intestinal malrotation is directed toward stabilizing the patient.

Where malrotation with volvulus or obstruction is suspected, seek immediate pediatric surgical consultation. Maintain patients on nothing by mouth (NPO) and adjust NG or orogastric tube to low intermittent suction. Correct fluid and electrolyte deficits. All patients require intravenous resuscitation with physiologic salt solution. Administer broad-spectrum antibiotics prior to surgery, if possible.

If a patient has signs of shock, administer appropriate fluids, blood products, and vasopressor medications to improve hypotension. Dopamine is often used as first-line therapy because of its possible effects to increase splanchnic blood flow. Dopamine can be started at an infusion rate of 3 mcg/kg/min intravenously (IV) and continued postoperatively even if the patient is not hypotensive.

Most patients require long-term intravenous access after surgery, particularly if midgut volvulus is present. Additionally, intravenous nutrition is likely to be necessary. For this reason, central venous catheters should be placed in most patients.

If the patient is unstable, do not delay surgical intervention for upper GI and laboratory studies. Quick surgical intervention, not prolonged medical management, is associated with the best results if midgut volvulus is suspected.

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Surgical Care

The Ladd procedure remains the cornerstone of surgical treatment for malrotation today. Prior to William Ladd's publication in 1936, surgical treatment for malrotation with or without volvulus had a mortality rate higher than 90%. In fact, at Ladd's own institution, the mortality rate was 100% before the development of his new technique. A classic Ladd procedure is described as reduction of volvulus (if present), division of mesenteric bands, placement of small bowel on the right and large bowel on the left of the abdomen, and appendectomy. Published reports for laparoscopic Ladd procedure are now appearing in the literature as well. [34]

Midgut volvulus

If midgut volvulus is present, the entire small intestine along with the transverse colon is delivered out of the abdominal incision, where the volvulus can be reduced. Because the volvulus usually twists in a clockwise direction, reduction is accomplished by twisting in a counterclockwise direction. Complete detorsion usually requires 2-3 twists of the bowel. After the blood supply has been restored by detorsion, the surgeon must make a decision about viability of the involved bowel. The outcome is better when no gangrenous bowel is present or when a small, localized gangrenous segment is present, which can be resected and a primary anastomosis performed.

If multiple areas of questionable viability are present, many surgeons choose to leave the areas and perform a second-look operation in 12-24 hours if the patient is not showing clinical recovery. See the image below.

This patient had malrotation with midgut volvulus. This patient had malrotation with midgut volvulus. The gut is darkened in color because of ischemia.

Grossly necrotic bowel should be resected. Primary anastomosis versus diversion of the fecal stream with a proximal ostomy should be performed at the surgeon’s discretion. Three principles should be used to guide decision making:

  • The highest priority is to preserve the maximum length of intestine required for survival.
  • Questionably viable bowel should not be anastomosed.
  • Resection of the entire small bowel require life-long parenteral nutrition or small bowel transplant. [35]

Duodenal obstruction

After the volvulus is reduced or if no volvulus was present, identify any extrinsic obstruction to the duodenum.

If peritoneal bands crossing the duodenum are found, ligate them with careful attention to protecting the superior mesenteric vessels. The bands may also obstruct the ileum or the jejunum and sometimes run to the gallbladder and liver.

Extrinsic obstruction may also be due to the cecum, colon, or superior mesenteric artery (SMA), impinging on the duodenum; relief is obtained by placing the cecum with its mesentery in the left upper quadrant and exposing the anterior duodenum through its entire length. After extrinsic obstruction has been relieved, determine that no intrinsic obstruction exists by passing an NG tube through the duodenum.

Appendectomy

Appendectomy is performed during operation for malrotation for 2 reasons [36] :

  • The normal anatomical placement of the appendix is disrupted when the cecum is placed in the left upper quadrant, making the diagnosis of future appendicitis challenging.
  • Dissection of the peritoneal bands causes damage to the appendiceal vessels.

Laparoscopy

Use of a laparoscopic approach to the correction of malrotation is feasible (level III, level IV evidence) but long-term outcome data is lacking. An open approach is thought to create adhesions, thereby reducing the risk of recurrent volvulus. Critics of the laparoscopic approach cite that laparoscopy is associated with minimal adhesion formation and inadequate widening of the mesentery and thus may not afford the patient comparable long-term benefit. [37, 38, 39]

Laparoscopy has been used to repair malrotation in clinically stable patients with or without signs of duodenal obstruction without midgut volvulus.

The Ladd procedure, including widening of the mesenteric base and dissection of peritoneal bands, has been performed successfully using a laparoscopic approach and has resulted in shorter hospital length of stay.

Laparoscopic Ladd procedure is now accepted as an initial approach to surgical correction, with recent reports showing superior short-term results including shorter term to full diet without any increase in operative duration. [40, 41, 42, 43]

Some authors have reported on the success of single-incision laparoscopic Ladd procedure for intestinal malrotation without volvulus in adults. [44]

A study reported that in children aged 6 months or older with suspicion of intestinal malrotation but not presenting with an acute abdomen or hemodynamically instability, laparoscopy should be considered as a first approach to diagnose and subsequently treat intestinal malrotation. [45]

 

Heterotaxy

Patients with heterotaxy syndromes have increased rates of abnormalities of intestinal rotation. The approach to the management of these rotational abnormalities is controversial. [46]

Some authors advocate for screening of all children with an upper GI study and elective correction of any rotational abnormality. [46] However, a retrospective review of 29 children who underwent Ladd procedure revealed that the complication rate was 57% among patients with heterotaxy who were otherwise asymptomatic, compared with only 9% in the symptomatic, nonheterotaxy group. [47] These authors recommend that asymptomatic children be managed conservatively with operative intervention reserved for those children who become symptomatic. A systematic review by Cullis et al found that out of 414 asymptomatic patients that had no screening for intestinal rotation, one patient developed malrotation symptoms which was confirmed by laparotomy. [48]

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Consultations

Consultations with the following specialists may be helpful:

  • Pediatric surgeon: The only definitive treatment for malrotation is surgical in nature
  • Dietary/intravenous nutrition team: In hospitals where available, include a nutritionist in the medical team to monitor total parenteral nutrition to ensure optimal nutrition. Children who undergo extensive small bowel resection require long-term nutritional support and benefit from early consultation with the nutrition team.
  • Pediatric gastroenterologist: Consultation with a gastroenterologist should be considered early in the postoperative course, particularly in those children who have undergone extensive small bowel resection due to the need for long-term parenteral nutrition and the resulting cholestasis in this population.
  • Physical therapist: A physical therapist can help with range of motion exercises and strength conditioning while patients are bedridden. Frequent repositioning also helps prevent decubitus pressure ulcers and head molding in infants.
  • Occupational therapist: Occupational therapists can also assist with splinting of extremities that contain central lines to prevent contractures as well as range of motion exercises.
  • Speech therapist: A speech therapist can help with oral stimulation while patients are not being fed. This stimulation can prevent feeding aversion and poor coordination when oral feeds are restarted.
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Diet

Feeding strategies are discussed under Further Inpatient Care.

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