Intestinal malrotation
- Overview
- Theory
- Diagnosis
- Management
- Follow up
- Resources
Treatment algorithm
Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer
obstruction with ischemia
emergency surgery: open laparotomy and Ladd procedure
The acutely ill patient with midgut volvulus with vascular compromise presents with bilious vomiting, acute abdominal pain, tachycardia, tachypnea, abdominal tenderness, acidosis, and potentially with signs of peritoneal catastrophe (guarding and rebound).
Emergent abdominal exploration, even without a radiographically confirmed diagnosis, is necessary. Therefore, the surgeon should be contacted early in the course of care when volvulus is clinically suspected in order to allow the surgeon opportunity to forgo further studies.
If the volvulus is associated with compromised flow of superior mesenteric artery, time is of the essence to salvage the involved intestine. Immediately on opening of the abdomen, the bowel is detorsed by turning it in a counterclockwise direction to "turn back the hands of time."[7]Shew SB. Surgical concerns in malrotation and midgut volvulus. Pediatr Radiol. 2009;39(suppl 2):S167-S171. http://www.ncbi.nlm.nih.gov/pubmed/19308380?tool=bestpractice.com Warm packs are applied to allow a period of observation to see whether the bowel will be viable. The Ladd procedure is then completed.
supportive care
Treatment recommended for ALL patients in selected patient group
Nasogastric tube, antibiotic coverage for gram-negative organisms (e.g., cefoxitin), and aggressive intravenous fluid resuscitation should be performed en route to the operating room but do not preclude an operation, as manual detorsion of the bowel is the only way to reverse an otherwise fatal course.
Postoperative care is simply supportive. The postoperative course is dictated by bowel rest until bowel activity resumes.
In the case of temporary closure or abdominal coverage with planned re-exploration, the patient should be sent to the intensive care unit, as there may be profound fluid shifts and hemodynamic perturbations that can progress rapidly. The course may mandate earlier reoperation sooner if the patient is unstable.
Primary options
cefoxitin: infants ≤3 months of age: consult specialist for guidance on dose; infants >3 months of age: 30-40 mg/kg intravenously as a single dose 30-60 minutes before surgery, then every 6 hours after surgery for no more than 24 hours
obstruction without ischemia
urgent surgery: open laparotomy and Ladd procedure
The patient with midgut volvulus without vascular compromise presents with bilious vomiting, crampy abdominal pain in waves, nontender abdomen, and no severe physiologic perturbation. Therefore, a radiographically confirmed diagnosis may be sought, but little time should be wasted obtaining an upper gastrointestinal tract contrast study, which will show the volvulus obstructing the duodenum.
Contrast may get through the torsion to give the corkscrew appearance, but this finding does not change the state of surgical urgency. Immediately on radiographic confirmation the surgeon should be called if not already involved in the care.
Immediately on opening of the abdomen, the bowel is detorsed by turning it in a counterclockwise direction. If there is any concern about bowel viability, warm packs are applied to allow a period of observation to see whether the bowel will be viable. The Ladd procedure is then completed.
supportive care
Treatment recommended for ALL patients in selected patient group
Nasogastric tube and aggressive intravenous fluid resuscitation should be performed en route to the operating room but do not preclude an operation, as manual detorsion of the bowel is the only reversal of obstruction.
Antibiotics may be used prophylactically in patients with selected risk factors for surgical-site wound infection; gram-positive coverage (e.g., cefazolin) should be used.
Postoperative care is simply supportive, and the postoperative course is dictated by bowel rest until bowel activity resumes.
Primary options
cefazolin: infants ≤1 months of age: consult specialist for guidance on dose; infants >1 months of age: 25 mg/kg intravenously as a single dose 30-60 minutes before surgery, then every 8 hours after surgery for no more than 24 hours; additional doses may be given during lengthy procedures
intermittent or partial volvulus or obstructing Ladd bands
timely surgery: Ladd procedure (open or laparoscopic)
The workup of these patients, who present with intermittent vomiting but no signs of acute illness, consists solely of an upper gastrointestinal contrast series. If there is no malrotation, intermittent volvulus or Ladd bands are ruled out but the contrast should be followed downstream with delayed images to rule out distal obstructing pathology. Malrotation diagnosed with this history should be operated on at the next feasible opportunity (e.g., next operating day), as this is not an emergency.
Laparoscopy may have a role in approaching these patients.[15]Fraser JD, Aguayo P, Sharp SW, et al. The role of laparoscopy in the management of malrotation. J Surg Res. 2009;156:80-82. http://www.ncbi.nlm.nih.gov/pubmed/19560159?tool=bestpractice.com [16]Lessin MS, Luks FI. Laparoscopic appendectomy and duodenocolonic dissociation (LADD) procedure for malrotation. Pediatr Surg Int. 1998;13:184-185. http://www.ncbi.nlm.nih.gov/pubmed/9563043?tool=bestpractice.com [17]Mazziotti MV, Strasberg SM, Langer JC. Intestinal rotation abnormalities without volvulus: the role of laparoscopy. J Am Coll Surg. 1997;185:172-176. http://www.ncbi.nlm.nih.gov/pubmed/9249085?tool=bestpractice.com [18]Draus JM Jr, Foley DS, Bond SJ. Laparoscopic Ladd procedure: a minimally invasive approach to malrotation without midgut volvulus. Am Surg. 2007;73:693-696. http://www.ncbi.nlm.nih.gov/pubmed/17674943?tool=bestpractice.com The operation remains the same with assuring no torsion around the base of the mesentery, complete duodenal mobilization, appendectomy, possible widening of the mesentery, and placement of the small intestine on the right side of the abdomen with the colon on the left.
Feeding resumes with resumption of bowel activity (which is immediate after the laparoscopic approach).
supportive care
Treatment recommended for ALL patients in selected patient group
No nasogastric tube is necessary before or after the operation if obstruction is not present.
Intravenous fluids before the operation are needed for maintenance only during the preoperative period of nothing by mouth.
Antibiotics may be used prophylactically in patients with selected risk factors for surgical-site wound infection; gram-positive coverage (e.g., cefazolin) should be used.
Primary options
cefazolin: infants ≤1 months of age: consult specialist for guidance on dose; infants >1 months of age: 25 mg/kg intravenously as a single dose 30-60 minutes before surgery, then every 8 hours after surgery for no more than 24 hours; additional doses may be given during lengthy procedures
questionable malrotation or asymptomatic finding
elective surgery: Ladd procedure (open or laparoscopic)
These patients may be asymptomatic or have intestinal malrotation discovered as an incidental finding in investigating for reflux (fussiness, arching, apneic events, reactive airways, pneumonia.).
If the contrast study confirms malrotation a Ladd procedure can be scheduled electively, either open or laparoscopic. However, the diagnosis may not be clear in this population.
The duodenum may sweep normally, not be far enough to the left side, or lie low on the contrast study. The retroperitoneal attachments may be normal (eliminating the risk of volvulus), but malrotation/nonrotation may be severe where the proximal bowel is lying to the left by circumstance.
Therefore, any radiographic findings suggesting malrotation as a possibility deserve exploration, which is preferably done laparoscopically in this scenario.[14]Ooms N, Matthyssens LE, Draaisma JM, et al. Laparoscopic Treatment of Intestinal Malrotation in Children. Eur J Pediatr Surg. 2016 Aug;26(4):376-81. https://www.doi.org/10.1055/s-0035-1554914 http://www.ncbi.nlm.nih.gov/pubmed/26086418?tool=bestpractice.com If malrotation is clearly present, the Ladd procedure can be completed laparoscopically or converted to open depending on surgeon preference.
supportive care
Treatment recommended for ALL patients in selected patient group
No nasogastric tube is necessary before or after the operation. Intravenous fluids before the operation are needed for maintenance only during the preoperative period of nothing by mouth.
Antibiotics may be used prophylactically in patients with selected risk factors for surgical-site wound infection; gram-positive coverage (e.g., cefazolin) should be used.
Primary options
cefazolin: infants ≤1 months of age: consult specialist for guidance on dose; infants >1 months of age: 25 mg/kg intravenously as a single dose 30-60 minutes before surgery, then every 8 hours after surgery for no more than 24 hours; additional doses may be given during lengthy procedures
Choose a patient group to see our recommendations
Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer
Use of this content is subject to our disclaimer