Gastrointestinal Duplications Treatment & Management

Before the treatment begins, gastrointestinal duplications must be evaluated based on their localization in terms of common blood supply and the search for heterotrophic gastric mucosa. In cervical esophageal duplications, the trachea must also be evaluated. Similarly, endoscopic evaluation of the genitourinary tract is recommended in selected hindgut duplications.

The preferred treatment of gastrointestinal duplications is excision;^[21] however, in colonic duplications, which feature communication proximally and distally between the duplication and the normal colon, administration of stool softeners and enemas can improve symptoms.

In general, excision is the preferred treatment of alimentary tract duplications.^[22] Because of the mesenteric location of most duplications, they share a common blood supply with the normal organ.^[23] If feasible, segmental resection may be performed. Otherwise, one may excise or "shell out" the cyst if an adequate plane is present. Mucosal stripping has also been described. If excision is not possible, for example, due to proximity to the biliary or pancreatic ducts, a drainage procedure may be performed. However, if this is planned, one must determine whether gastric mucosa is present (with technetium scan), and, if so, it must be excised to prevent future ulceration. In colon and rectal duplications below the peritoneal reflection, endoscopy of the urogenital tract should be performed preoperatively to rule out associated anomalies.

• Cervical esophageal duplications: Surgical treatment involves the excision of the cyst. Total mucosectomy is also curative.
• Thoracic and thoracoabdominal duplications: Treatment involves excision of the cyst. Combined thoracoabdominal cysts make up fewer than 2% of all duplications. If staged excision is planned, the duplication remaining after the primary excision should have a communication with the gastrointestinal tract or be drained externally to prevent compression if it expands because of hemorrhagic secretions.
• Gastric duplications: In most cases, resection can be accomplished without entering the stomach by resecting the shared wall between the stomach and the duplication. Gastric resection is generally not required.
• Duodenal duplications: Surgical resection is the treatment of choice; however, drainage of the duplication cysts into the duodenum or into a Roux limb of the jejunum is an acceptable alternative if a risk of injury to the biliary or pancreatic ductal system is observed. Preoperative endoscopic retrograde cholangiopancreatography (ERCP), percutaneous transhepatic cholangiography (PTC), or magnetic resonance cholangiography/pancreatography (MRCP) should be performed to evaluate the involvement of the biliary and pancreatic ducts. These studies may also distinguish between duodenal duplication and a choledochal cyst if the diagnosis is unclear. A cholangiogram may also be performed intraoperatively to characterize the ductal anatomy in relation to the cyst. Excision should be performed if gastric mucosa is present to avoid later ulceration. Marsupialization and external drainage have no value.
• Small-intestine duplications: Segmental resection along with the adjacent intestine is the preferred treatment for small cystic or short tubular duplications. Cystic duplications (in the absence of gastric mucosa) that cannot be resected may be drained into a Roux limb. Long tubular duplications that cannot be resected because of their length can be managed by intestinal lengthening procedures, mucosal stripping through multiple incisions (when separation is not possible), or diversion into the stomach.
• Colonic duplications

  • Cystic duplications: In cases of smaller cysts, a complete excision of the duplication and its attached normal colon is preferred. However, because long colonic duplications generally have an opening to the normal colon, enlarging these internal communications by excising a small part of the common wall to permit reentry from the duplication into the normal colon is sufficient.
  • Tubular duplications: If the duplication is fused with a normal rectum and perineal anus, the duplication can be connected transanally by excising part of the common rectal wall. Smaller duplications may also be treated by excising the fistula wall, making it part of the common rectum. Tubular duplications may also be treated by excising the lesion with the normal colon, by enlarging existing communications with the normal bowel, or by excising the common wall. Tubular duplications often extend below the peritoneal reflection, and they are associated with abnormalities of the genitourinary tract. Preoperative cystoscopy and vaginoscopy/hysteroscopy should be performed to identify duplication, fistula, or other abnormalities of the bladder, urethra, or genital tract.
• Rectal duplications: The general approach involves the transanal exposure of the cyst, incision of the posterior rectal mucosa, draining the cyst, and stripping the cyst wall mucosa. Total excision may also be performed via the transanal, posterior sagittal, or transcoccygeal (Kraske) approach.

• Surgical excision of gastrointestinal lesions is generally curative; however, follow-up is important after the treatment of certain types of duplications.
• Pancreatitis may occur after surgical management of gastric or duodenal duplications because of the proximity of the duplications to the pancreaticobiliary tree.
• Recurrent bleeding may occur after mucosal stripping of the duplication in cases in which complete excision is not possible.
• Patients undergoing medical therapy with stool softeners and who have had cystic colonic duplications require regular clinical evaluation.
• Severe duplications of external genitalia associated with tubular colonic duplications pose a cosmetic problem, and counseling is required.
• Continence studies (eg, rectal manometry) are advisable for patients who have been treated for rectal duplications.
• For patient education resources, see the Procedures Center, as well as Gastrointestinal Endoscopy.

Gastrointestinal duplications can be found along the entire length of the gastrointestinal tract; therefore, the complications that may occur after surgical excision of gastrointestinal duplications depend on the location of the lesions.

Untreated gastrointestinal duplications may lead to the following complications:

• Severe and possibly life-threatening respiratory distress may occur in patients with cervical esophageal duplications.
• Ectopic gastric mucosa in a duplication can lead to peptic ulceration, bleeding, perforation, and peritonitis.
• Recurrent pancreatitis may occur from gastric duplications communicating with the pancreatic duct.
• Neoplastic changes have been reported in patients with untreated gastrointestinal duplications.
• Hematochezia, hemorrhoids, fistulas-in-ano, or anal abscess may recur in patients with untreated rectal duplications.

The outcome of surgical (or medical) management of gastrointestinal duplications is favorable. Metaplastic changes that have been reported in untreated gastrointestinal duplications can be prevented, depending on the location of the duplication, by appropriate surgical intervention.

The severity and types of malformations associated with gastrointestinal duplications play a significant role in determining morbidity and mortality.

Gastrointestinal duplications have an extremely variable presentation, and the surgical management depends on the location, size, and shape of the duplication. Controversies and future management strategies are related to management of duplications, as well as the associated anomalies.

Alimentary tract duplications are uncommon and may present as solid or cystic tumors, intussusception, perforation, or gastrointestinal bleeding. A high index of suspicion is required in such cases. Appropriate investigations, including imaging techniques, should be directed toward adequate and planned surgery.