Approach
If malrotation is suspected or the diagnosis is confirmed, the timing of surgery (i.e., elective, urgent, or emergent) is predicated on the level of concern for bowel ischemia, the type of malrotation, and the underlying condition of the patient. Most patients with intestinal malrotation without volvulus require urgent surgery in the form of the Ladd procedure. Patients with midgut volvulus require an emergent Ladd procedure to potentially reverse intestinal ischemia before necrosis occurs.[6] Less frequently, the Ladd procedure may be performed electively if there are truly incidental findings without symptoms, or when the risk of surgery and anesthesia for the patient outweighs the risk of developing midgut volvulus (e.g., patients with congenital heart disease).[12]
Ladd procedure
Once bowel viability has been assessed at exploration, the Ladd procedure is completed by detorsion of volvulus when present, lysis of the Ladd bands, and separation of the duodenum and cecum by broadening the mesenteric base. Once the duodenum and cecum are separated, the small bowel is placed in the right peritoneal cavity and the colon on the left.
Controversy exists as to whether it is still beneficial to perform an incidental appendectomy for the concern of future atypical appendicitis from abnormal appendiceal location, and this decision is left to surgeon preference along with parental discussion.
The Ladd procedure can be challenging to perform for those surgeons inexperienced with the technique, particularly in the infant with volvulus. The base of the volved bowel can be disorienting, and the volvulus may inadvertently be tightened with erratic exploration.
One needs to remember that in almost all patients the twist is in a clockwise direction. Therefore, detorsion consists of "turning back the hands of time" with counterclockwise rotation 1 to 3 complete turns until the duodenum and cecum are aligned in parallel.[7] Furthermore, meticulous technique in mesenteric separation is necessary to avoid catastrophic iatrogenic injury to the vessels and full thickness opening in the mesentery that may predispose to an internal hernia.
Only tissue that is frankly necrotic or remains black after detorsion should be resected. If the viability of a long segment remains questionable it should not be resected during the initial exploration. It is more prudent to close the abdomen (or leave it open with a temporary dressing) and plan repeat exploration in 24 hours before committing to a resection that may be incompatible with life without successful enteral nutritional support.
Laparoscopic Ladd procedure
The operation can be conducted with the exact same steps as the open procedure. Laparoscopic instruments small enough for newborn operations are becoming more widely disseminated worldwide.
There have been no trials between open and laparoscopic approaches. One of the main factors thought to contribute to the effectiveness of the Ladd procedure is the development of intra-abdominal adhesions to help prevent volvulus.[6] The concern of the laparoscopic technique is that fewer adhesions may result that would potentially protect patients from future volvulus. The relative contribution in the prevention of future volvulus between newly formed abdominal adhesions from surgery versus the displacement and repositioning of the bowel by the Ladd procedure has yet to be deciphered. However, laparoscopy used in other procedures has resulted in a lowered incidence of postoperative bowel obstruction in several studies.[13]
When the diagnosis of malrotation is in question, any radiographic findings suggesting malrotation as a possibility deserve exploration that can be facilitated by the laparoscopic approach.[14] Three variables are assessed to help delineate the anatomy:
Presence/position of the ligament of Treitz
Position and attachments of the cecum and colon
Width of the base of the mesentery.
If malrotation is present, the Ladd procedure can be completed laparoscopically or converted to open depending on surgeon preference.[15]
Obstruction with ischemia (midgut volvulus with vascular compromise)
This acutely ill patient presents with bilious vomiting, severe acute abdominal pain, tachycardia, tachypnea, abdominal tenderness, acidosis, or signs of peritoneal catastrophe (guarding and rebound) and requires emergency abdominal exploration without a radiographically confirmed diagnosis.
Treatment is emergency surgery with open laparotomy and Ladd procedure. Abdominal exploration is warranted without a radiographically confirmed diagnosis.[7]
Supportive care includes nasogastric (NG) tube, broad-spectrum antibiotics, and aggressive intravenous (IV) fluid resuscitation, which should be performed en route to the operating room.
Obstruction without ischemia (midgut volvulus without vascular compromise)
The patient usually presents with bilious vomiting, crampy abdominal pain in waves, nontender abdomen, and no severe physiologic perturbation.
Treatment is urgent surgery with open laparotomy and Ladd procedure. This group of patients allows time for a radiographically confirmed diagnosis, but little time should be wasted obtaining a contrast study, which will show the volvulus causing obstruction of the duodenum.
Immediately on radiographic confirmation, the surgeon should be called if not already involved.
Supportive care includes NG tube and aggressive IV fluid resuscitation, which should be performed en route to the operating room. Antibiotics are only prophylactic and can be used as for any bowel surgery.
Intermittent or partial volvulus or obstructing Ladd bands
These patients may present with intermittent vomiting, but no signs of acute illness.
Timely surgery with open or laparoscopic Ladd procedure is required. As these patients are stable, time is available for appropriate diagnostic imaging studies.
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 treating these patients.[15][16][17][18]
Regarding supportive care, no NG tube is necessary before or after the operation if obstruction is not present. IV fluids before the operation are needed for maintenance only during the preoperative period of nothing by mouth. Antibiotics are prophylactic only with the aim toward gram-positive skin flora coverage.
Questionable malrotation or asymptomatic finding
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).
Elective surgery with open or laparoscopic Ladd procedure is performed in these patients. This group allows time for contrast studies.
No NG tube is necessary before or after the operation. IV fluids before the operation are needed for maintenance only during the preoperative period of nothing by mouth. Antibiotics are prophylactic only and gram-positive coverage is all that is required.
Patients with heterotaxy or serious comorbidities
There are rare circumstances where malrotation is diagnosed but surgery is not recommended due to the patient's underlying medical illnesses imparting a higher risk of morbidity and mortality from surgery than the risk of malrotation itself. However, one study indicates that malrotation patients with significant comorbidities of heterotaxy or congenital heart disease benefit from a Ladd procedure.[12]
How to insert a fine bore nasogastric tube for feeding.
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