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. Infants with bilious vomiting, peritonism, and a gasless abdominal x-ray require immediate treatment for hemodynamic instability in conjunction with immediate pediatric surgical referral for definitive surgery.[11] All patients with midgut volvulus require an emergent Ladd procedure to potentially reverse intestinal ischemia before necrosis occurs.[2] Most patients with intestinal malrotation without volvulus require emergent surgery in the form of the Ladd procedure. 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).[27]
Ladd procedure
Once bowel viability has been assessed at exploration, the Ladd procedure is completed by counterclockwise detorsion of volvulus when present, division 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 replaced in the right side of the abdomen 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.
It should be noted 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.[10] 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 24-48 hours before committing to a resection that may be incompatible with life without long-term parenteral 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 prospective trials comparing 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.[2] 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 determined. However, laparoscopy used in other procedures has resulted in a lower incidence of postoperative adhesive small bowel obstruction in several studies.[28] One systematic review and meta-analysis carried out in 2021 demonstrated that although laparoscopic Ladd procedure is associated with a lower rate of subsequent adhesive small bowel obstruction, the rate of postoperative volvulus is higher.[29]
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.[30] 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.[31]
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 (e.g., guarding) and requires emergency abdominal exploration without a radiographically confirmed diagnosis.[10]
Treatment is emergency surgery with open laparotomy and Ladd procedure.
Supportive care includes nasogastric (NG) tube, broad-spectrum antibiotics, and intravenous fluid resuscitation, which should be performed en route to the operating room.[11]
Ceftriaxone plus metronidazole is recommended as a suitable antibiotic regimen for low-risk adults and children.[32][33] The combination should be considered where there is increased resistance to first- and second-generation cephalosporins. Subsequent adjustments to the antibiotic regimen may be necessary depending on the patient's microbiologic profile and sensitivity tests.
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 typically have time for rapid radiologic confirmation.
Immediately on radiographic confirmation, the surgeon should be called if not already involved.
Supportive care includes NG tube and intravenous fluid resuscitation. Antibiotics are only prophylactic and can be used as for any bowel surgery.
Antibiotics (e.g., ceftriaxone plus metronidazole) are prophylactic and can be used as for any bowel surgery.[32][33]
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.[31][34][35][36]
Regarding supportive care, no NG 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 (e.g., ceftriaxone plus metronidazole) are prophylactic with the aim toward gram-positive skin flora coverage.[32][33]
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 typically performed in these patients. This group allows time for contrast studies.
Nasogastric tube is necessary after the operation. Intravenous fluids before the operation are needed for maintenance only during the preoperative period of nothing by mouth. Antibiotics (e.g., ceftriaxone plus metronidazole) are prophylactic and gram-positive/negative coverage is all that is required.[32][33]
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.[27] In this scenario, a multidisciplinary approach involving surgery, cardiology, critical care, and the patient’s caregivers can help guide a watchful waiting management plan.[26]
How to insert a fine bore nasogastric tube for feeding.
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