Approach Considerations
Surgical resection is the preferred method of treatment for most gastrointestinal (GI) duplications; however, for duodenal duplications, surgical resection is inadvisable because of the close proximity of such cysts to the biliary and pancreatic ductal system. Patients presenting with complex tubular colonic duplications may not require a surgical approach if internal communication of the duplication is adequate and the colon is normal.
Medical Therapy
Before treatment begins, GI duplications must be evaluated with respect to their localization in terms of common blood supply and the search for heterotopic 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 GI duplications is excision [32, 33] ; 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.
Surgical Therapy
Because most duplications have a mesenteric location, they share a common blood supply with the normal organ. [34] If feasible, segmental resection may be performed (see the image below). Otherwise, one may excise or "shell out" the cyst if an adequate plane is present. Mucosal stripping has also been described.
Jejunal duplication in a newborn. Intraoperative image demonstrates a cystic structure found on the antimesenterial side of the jejunum. A segmental resection was performed and the jejunum anastomosed.
If excision is not possible—for example, because of 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); if so, it must be excised to prevent future ulceration.
In colon and rectal duplications below the peritoneal reflection, endoscopy of the genitourinary tract should be performed preoperatively to rule out associated anomalies.
Laparoscopic approaches to the resection of alimentary tract duplications in children are being described with increasing frequency. [35]
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 GI 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.
Laparoscopic resection of gastric duplication cysts has been described in newborns [36] and in adults. [37]
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 cholangiopancreatography (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. Cholangiography 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.
Case reports have described successful treatment of nonmalignant duodenal duplication cysts by means of endoscopic techniques. [38, 39]
Pyloric duplications
These are extremely rare but have been detected upon endoscopic investigation. [40]
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 means of intestinal lengthening procedures, mucosal stripping through multiple incisions (when separation is not possible), or diversion into the stomach.
Colonic duplications
Cystic
In cases of smaller cysts, 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
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 to rectal duplications involves transanal exposure of the cyst, incision of the posterior rectal mucosa, drainage of the cyst, and stripping of the the cyst wall mucosa. Total excision may also be performed via the transanal, posterior sagittal, or transcoccygeal (Kraske) approach.
Complications
GI duplications can be found along the entire length of the GI tract; therefore, the complications that may occur after surgical excision of these duplications depend on the location of the lesions.
Untreated GI duplications may lead to the following complications:
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Severe and possibly life-threatening respiratory distress may occur in patients with cervical esophageal duplications
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Ectopic gastric mucosa in a duplication can lead to peptic ulceration, bleeding, perforation, and peritonitis
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Recurrent pancreatitis may occur from gastric duplications communicating with the pancreatic duct
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Neoplastic changes have been reported in patients with untreated GI duplications
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Hematochezia, hemorrhoids, fistulas-in-ano, or anal abscess may recur in patients with untreated rectal duplications
Long-Term Monitoring
Surgical excision of GI 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 where 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.
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Jejunal duplication in a newborn. Intraoperative image demonstrates a cystic structure found on the antimesenterial side of the jejunum. A segmental resection was performed and the jejunum anastomosed.
