Alimentary Tract Duplications KATIE W

39 Alimentary Tract Duplications KATIE W. RUSSELL and GEORGE W. HOLCOMB III

Alimentary tract duplications have been described for hun- distention, pain, obstruction, intussusception, bleeding, dreds of years, and multiple terms have been used in the perforation, respiratory compromise, or as a painless mass. literature. The term duplication of the alimentary tract was Generally, symptoms are related to size, location, and the coined by William Ladd in 1937.1 He described three com- presence of heterotopic mucosa. With advances in prenatal mon findings: a well-developed smooth muscle coat, an imaging, many of these masses are being diagnosed in utero epithelial lining, and attachment to the alimentary tract. (Fig. 39.1) and the majority are discovered before 2 years In 1952 Gross et al. reported the first large series describ- of age.4,5,15 A recent study from the Children’s Hospital of ing 67 patients with these findings.2 These duplications are Philadelphia (CHOP) found that 58% of duplications were relatively rare congenital anomalies found anywhere from diagnosed prenatally over a 25-year period.16 The major- the mouth to the anus with an incidence reported to be 1 in ity of duplications are cystic (Fig. 39.2), and the remaining 4500 births.3 Presentation is variable, and resection is usu- are tubular (Fig. 39.3). The ileum and jejunum are the most ally recommended in order to prevent worsening symptoms commonly affected locations, followed by the esophagus.5 or malignant transformation. The epithelial lining is usually native to the surrounding enteric tract, but heterotopic mucosa is found in approximately one-third of duplications.5 Gastric tissue is the most Embryology common type of ectopic mucosa, followed by pancreatic tissue, and there are reports of respiratory epithelium being Alimentary tract duplications take many forms, and one found as well (Fig. 39.4).17 Ectopic gastric mucosa can lead unifying embryologic theory is unlikely to encompass all to peptic ulceration with subsequent hemorrhage, anemia, variations. The associated findings of vertebral, spinal cord, or perforation (Fig. 39.5). Pressure necrosis from an adja- and genitourinary (GU) malformations, as well as malrota- cent duplication also can lead to hemorrhage or perforation and intestinal atresia, suggest a multifactorial process tion. Associated anomalies are described in approximately in their development.2,4,5 There are five prevailing theo- 20%, with the most common anomalies being malrotation, ries: partial twinning, split notochord, diverticular defects, intestinal atresia, spinal anomalies, and GU anomalies.18 canalization defects, and environmental factors. The partial twinning theory states that organs can be doubled as a result of partial twinning. This theory may be pertinent in hindgut duplications associated with GU tract duplications.6,7 The split notochord theory centers around notochord separation in the first month of gestation. This theory postulates that gaps in the notochord develop and allow gut endoderm to herniate and form diverticula. This theory could account for the association of duplications with spinal defects.8–10 A persistent embryonic diverticulum from the gastrointestinal (GI) tract was the first theory described in the literature, and a defect in lumen canalization was proposed years later. The theory of defective canalization is based on the finding that GI organs begin as solid tubes and vacuolate to form lumens. During this process, diverticula form but regress during fetal life. If they persist, duplications could form.11,12 Finally, environmental factors including hypoxia, vascular accidents, and trauma have been impli- cated in the development of these anomalies.13,14

Clinical Presentation and Fig. 39.1 This T2–weighted transverse MRI image was obtained from Diagnosis a 22-weeks gestational age fetus. The white arrow identifies a hyper- intense cystic-like structure that is a gastric duplication protruding through the native gastric wall. (From Laje P, Flake AW, Adzick NS. Pre- GI duplications are found incidentally, or they can mani- natal diagnosis and postnatal resection of intraabdominal enteric duplica- fest with a wide range of symptoms, including abdominal tions. J Pediatr Surg 2010;45:1554–1558. Reprinted with permission.) 629 630 Holcomb and Ashcraft’s Pediatric Surgery

mass, which requires further workup with either CT or MRI. Contrast studies may show a mass effect or communication with the alimentary tract and can help with the diagnosis, particularly in hindgut and foregut lesions. Technetium-99m scintigraphy may be used as adjuvant imaging but is likely unnecessary in most cases.24,25 The presence of a vertebral abnormality and a duplication is best investigated with MRI to evaluate communication with the spinal canal.26 Classification and Treatment by Location

To better understand the wide presentation and surgical treatment of duplications, they are best discussed accord- Fig. 39.2 Most alimentary tract duplications are cystic. In this laparo- ing to anatomic location. A compilation of major case series scopic photograph, note the cystic duplication being grasped by the reported in the last 75 years from 23 different institutions Maryland instrument. Its blood supply can be seen just below the tip is seen in Table 39.1.2,4,5,16,18,27–44 The goal of operative of the instrument. As opposed to a Meckel diverticulum, which is found management is to remove the duplication, but the surgi- on the antimesenteric side of the bowel, duplications are located on the mesenteric side. cal procedure should not be more radical than necessary to eliminate the patient’s complaints and prevent recurrence. Simple cyst resection without violating the native GI tract is one option, but because most share a common blood supply to the native alimentary tract, resection with primary anastomosis is often needed and curative, depending on the location. In the recent CHOP 25-year review, bowel resection was required in 34% of the cases.16 Long tubular, thoracoabdominal, and gastroduodenal duplications may present a more difficult challenge as resection can carry significant morbidity. Partial excision with mucosal stripping may be the best option in these situations. Overall prognosis is favorable, but associated malformations, location, and presenting illness can factor into the final outcome.

ESOPHAGEAL DUPLICATIONS Approximately 16% of duplications arise from the esopha- * gus (see Table 39.1). Although cervical and abdominal esophageal duplications occur, the majority are located along the thoracic esophagus and in the right chest. Most are cystic and share a muscular wall with the esophagus Fig. 39.3 This operative photograph shows a child with a tubular colonic duplication (solid arrow). The duplications are always on the but do not communicate with the lumen. Clinical presenta- mesenteric side of the native colon. In this photograph, the cecum is tion will depend on whether there is a mass effect. Esopha- marked by the asterisk and the native colon is identified with the dot- geal compression can lead to dysphagia or regurgitation ted arrow. (Photo courtesy Dr. Michael Rollins.) while respiratory symptoms such as cough or pneumonia can result from airway or lung compression. Almost 40% Although duplications are benign, the potential for malig- of esophageal duplications contain ectopic gastric mucosa nant degeneration remains a concern.19–21 (Table 39.2), so peptic ulceration leading to anemia or Multiple imaging modalities are utilized to make the hematemesis can be seen, albeit rare. Esophageal dupli- diagnosis. Prenatal ultrasound (US) can be followed with cations also can have respiratory epithelium or primitive US postnatally, which may be sufficient, especially for dis- lung tissue associated with the lesion (Fig. 39.7). Despite tal small bowel lesions. The typical sonographic appear- the wide range of potential clinical manifestations, esopha- ance of a duplication (the double wall sign) demonstrates geal duplications are most commonly asymptomatic and a cystic rim of hyperechoic serosa and an inner hyper- diagnosed incidentally either prenatally or during workup echoic rim of mucosa and submucosa with a hypoechoic for an unrelated problem such as a respiratory infection or muscular layer sandwiched between the two hyperechoic trauma. Duplications should be included in the differential layers (Fig. 39.6).22 For foregut lesions, more information diagnosis for any patient presenting with a posterior medi- in the form of computed tomography (CT) or magnetic astinal mass. resonance imaging (MRI) may prove valuable in operative Once a duplication is suspected, axial imaging with either planning.23 Plain radiographs may reveal a mediastinal CT or MRI is helpful (Fig. 39.8). When a thoracic duplication 39 • Alimentary Tract Duplications 631

A B

Fig. 39.4 These two histologic images were taken from a tubular jejunal duplication. On the left (A) the low-power image shows two lumens that are separated by a singular muscular wall. On the right (B), the medium-power image shows the gastric mucosa from the duplication (left) and the small intestinal mucosa with villi from the native jejunum (right). (Photo courtesy Dr. Walter Pipkin. From Cunningham AJ, Ham PB, King, RG, et al. Congenital jejunal tubular duplication in a patient with a congenital thoracic meningocele. Am Surg 2015;81:E332–E333.)

Fig. 39.5 This infant presented with abdominal distention and free air. Laparotomy was performed, and this perforation on the antimesenteric side of the distal small bowel was seen. The perforation occurred due to the duplication cyst located on the mesenteric border (asterisk). This patient underwent segmental small bowel resection and recovered uneventfully. Fig. 39.6 Ultrasonography is a frequent imaging modality for diagnos- ing abdominal duplications. The typical sonographic appearance of a duplication demonstrates a cystic lesion with an inner hyperechoic rim is identified, some advocate for US of the abdomen to eval- of mucosa and submucosa (dotted arrow) and an outer rim of hyper- uate for synchronous abdominal duplications as a 25% echoic serosa (solid arrow) with a hypoechoic muscular layer between incidence has been described.5 US is efficient, inexpensive, them. In this image, inspissated mucous (asterisk) is also seen. and has little risk to the patient. Esophageal duplications should be removed because of the risk of malignancy and THORACOABDOMINAL DUPLICATIONS the risk that they will become symptomatic at some point. With the increased use of thoracoscopy, many esophageal Extension of an esophageal duplication into the abdomen is duplications are being resected with a minimally invasive known as a thoracoabdominal duplication. These are quite approach rather than traditional thoracotomy.45,46 Typi- rare and account for approximately 2% of all duplications cally they can be removed by carefully creating a cleav- (see Table 39.1). Typically, they originate in the right chest age plane within the muscular wall that is shared with the and at least half communicate with the intestinal tract native esophagus. As with any chest operation, care must distally, most commonly at the level of the jejunum.4,5,47 be taken to avoid injury to adjacent structures, including Similar to esophageal duplications, presentation is variable. the phrenic and vagus nerves. If there is any concern for These duplications are tubular, and ectopic gastric mucosa esophageal violation, a postoperative esophagram should is found in a high percentage. These patients also have a be done to evaluate for leak. high incidence of vertebral anomalies (88%) (Fig. 39.9), 632 Holcomb and Ashcraft’s Pediatric Surgery

Table 39.1 Alimentary Tract Duplications by Location as Described in Literature Reports First No. D (No. Esopha- Thoracoab- Jejunum/ Author Institution Pts) gus dominal Stomach Duodenum ileum Colon Rectum Other Mejad- Children’s Hospital of 107 (104) 27 0 16 9 37 7 1 7 dam, Philadelphia 201716 Erginel, Istanbul University, 40 (40) 2 0 2 3 24 9 0 0 201744 Turkey Rattan, Pt. B.D. Sharma PGIMS 17 (17) 0 0 1 2 12 2 0 0 201743 Rohtak, India Jehangir, Christian Medical Col- 38 (35) 6 3 3 1 17 6 1 1 201542 lege, India Okur, Dicle University, Turkey 32 (32) 1 2 2 5 16 5 1 0 201441 Zouari, Habib Thameur Hospi- 12 (12) 1 0 1 4 5 1 0 0 201440 tal, Tunisia Lima, University of Bologna, 22 (22) 5 0 3 2 9 3 0 0 201239 Italy Guerin, GECI, France 114 (114) 16 0 14 7 70 6 1 0 201218 Puli- Montreal Children’s 73 (73) 0 0 6 7 51 5 4 0 gandla, 200338 Karnak, Ankara, Turkey 42 (38) 7 2 1 3 17 9 2 1 200037 Yang, NTUH, Taipei, China 20 (17) 2 0 1 0 14 3 0 0 199636 Iyer, Children’s Hospital Los 29 (27) 0 0 3 1 9 8 6 2 199535 Angeles Stringer, Hospital for Sick Chil- 77 (72) 15 6 10 3 21 10 6 6 19954 dren, London Bajpai, IIMS, New Delhi, India 15 (14) 8 1 0 1 1 3 1 0 199434 Pinter, Hungary 30 (28) 6 2 4 3 9 3 3 0 199233 Holcomb, Children’s Hospital of 101 (96) 21 3 8 2 47 15 5 0 19895 Philadelphia Bissler, Akron Children’s 11 (11) 1 0 1 2 4 2 1 0 198832 Ildstad, Cincinnati Children’s 20 (17) 6 0 1 0 5 8 0 0 198831 Hocking, RHSC, Glasgow 60 (53) 8 2 8 1 32 4 5 0 198130 Bower, Pittsburgh Children’s 78 (64) 15 1 6 6 34 12 2 2 197829 Favara, Denver Children’s 39 (37) 6 0 3 4 20 4 0 2 197114 Grosfeld, Columbus Children’s 23 (23) 4 2 1 0 9 7 0 0 197028 Basu, A. H. Children’s, Liver- 33 (28) 7 0 1 3 16 4 2 0 196027 pool Gross, Boston Children’s 68 (67) 13 3 2 4 32 10 3 1 19522 Hospital TOTALS 1098 (1041) 177 27 98 73 511 146 44 22 PERCENTAGE 16% 2% 9% 7% 47% 13% 4% 2%

Table 39.2 Ectopic Mucosa by Location First Author Esophageal Small Bowel Colorectal Mejaddam, 201716 3/27 22/46 1/8 Okur, 201441 2/3 2/13 1/6 Guerin, 201218 3/16 41/70 0/7 Puligandla, 200338 30/58 3/9 Stringer, 19954 9/21 7/24 0/16 Bajpai, 199434 9/9 2/2 1/4 Holcomb, 19895 8/24 12/49 1/20 Ildstad, 198831 2/6 5/13 0/8 Hocking, 198130 5/10 21/33 2/9 Bower, 197829 7/16 5/40 0/4 Favara, 197114 3/6 6/24 0/4 Gross, 19522 7/16 8/36 0/10 TOTALS 58/154 (38%) 161/408 (39%) 9/105 (9%) 39 • Alimentary Tract Duplications 633

Fig. 39.7 This thoracoscopic view shows an esophageal duplication (asterisk) that is adjacent to the esophagus (arrow) and attached to a piece of primitive lung. Histologic analysis confirmed the mass as a duplication with primitive lung. (Photograph courtesy Dr. Thane Blinman.)

A B

Fig. 39.8 This 16-year-old patient was found to have a posterior mediastinal mass on chest radiograph. CT scan (A) shows the duplication (arrow) to be adjacent to the trachea and the esophagus. On the right (B), the duplication is visualized at thoracoscopy and was excised without complications.

A B C

Fig. 39.9 A 3-year-old child was found to have a right paravertebral mass. (A) A large anterior defect in the vertebral bodies of the upper thoracic spine (arrow) is seen. (B) This myelogram shows the filling defect caused by a neuroenteric cyst.(C) The contrast agent from the myelogram is seen in the neuroenteric cyst (upper arrow) with extension subdiaphragmatically (black arrow) into the distal small intestine. (From Holcomb GW III, Gheissari A, O’Neill JA, et al. Surgical management of alimentary tract duplications. Ann Surg 1989;209:167–174.) 634 Holcomb and Ashcraft’s Pediatric Surgery

Spin: Tilt: *

Fig. 39.10 A neonate was found to have pyloric atresia and underwent laparoscopic correction. At laparoscopy, a large gastric duplication (asterisk) was seen emanating from the greater curvature of the stomach. The duplication was excised and the greater curvature closed. The patient recovered uneventfully and did not develop any postoperative problems. Fig. 39.11 This CT scan was taken in a 3-year-old girl with emesis. The large hypodense cystic structure in the right upper quadrant (asterisk) represents a large duodenal duplication. There is an abrupt cut-off of the stomach at its medial aspect due to the duplication. The stomach and MRI is often useful to evaluate for neuroenteric com- is markedly distended with oral contrast. (From Laje P, Flake AW, Adzick munication. The current treatment is a one-stage combined NS. Prenatal diagnosis and postnatal resection of intraabdominal enteric thoracoabdominal approach for resection. This can be done duplications. J Pediatr Surg 2010;45:1554–1558. Reprinted with permission.) open or by a minimally invasive thoracoscopic approach followed by an incision in the diaphragm to access the abdominal component. but occasionally tubular variants are seen.48,49 These frequently contain rests of gastric or pancreatic tissue. Abdom- GASTRIC DUPLICATIONS inal US is often used as an initial study, but most surgeons elect for CT or magnetic resonance cholangiopancreatogra- Gastric duplications account for 9% of alimentary tract phy (MRCP) to better delineate the anatomy prior to opera- duplications (see Table 39.1) and usually become symptom- tion (Fig. 39.11). The anatomic location and tenuous blood atic early in life, frequently presenting with pain, emesis, or supply of these duplications dictate the operative approach. melena. Most are cystic, arise from the greater curvature, Simple excision is preferred, but the intimate relationship to and do not communicate with the lumen (Fig. 39.10).5,16 the biliary and/or pancreatic ducts may warrant Roux-en- Peptic ulceration with hemorrhage or perforation can Y cystjejunostomy.50 In complicated cases, endoscopic ret- develop. Abdominal US usually can diagnose the duplica- rograde cholangiopancreatography (ERCP) may be useful tion, but pancreatic pseudocysts or choledochal cysts may in the diagnosis. Endoscopy for the treatment of duodenal have similar appearances and be difficult to differentiate. duplications also has been described.51 If there is any uncertainty, CT or MRI is recommended to help clarify the anatomy before attempting surgical resec- PANCREATIC DUPLICATIONS tion. As with most duplications, resection is recommended to prevent complications such as bleeding, perforation, and Pancreatic duplications are the rarest type of GI duplica- malignancy. Depending on the location and ease of operation. Commonly manifesting with abdominal pain that is tion, resection through the shared muscular wall or wedge often recurrent and chronic, they can easily be mistaken resection of the native stomach along with the duplication for a pancreatic pseudocyst. What differentiates them from are both acceptable. In more difficult locations, such as the other alimentary duplications is that there is communica- lesser curve, gastroesophageal junction, and pylorus, the tion with the main or accessory pancreatic duct. The cysts cyst can be partially excised in a safe manner followed by are similar to gastric duplications both grossly and micro- mucosal stripping of any remaining mucosa in order to scopically, but they may or may not have attachment to the avoid a significant gastrectomy. stomach. They can be intrapancreatic or extrapancreatic, and may be combined with duplicated pancreatic tissue DUODENAL DUPLICATIONS along an aberrant duct. The location of ductal communication can be anywhere in the pancreas. On exploration, Duodenal duplications account for 7% of all duplications there is often significant fibrosis, likely from chronic inflam- (see Table 39.1) and may be asymptomatic or can mani- mation. Intraoperative frozen section evaluation will differ- fest with bleeding, intestinal obstruction, or obstruction of entiate a duplication from a pseudocyst based on the cyst the biliopancreatic ducts causing jaundice or pancreatitis. wall cellularity. Simple cyst resection is preferred, but the Most are cystic and noncommunicating with the lumen, location may dictate a more complex resection.52–54 39 • Alimentary Tract Duplications 635

* *

A B

Fig. 39.12 This cystic small bowel duplication (asterisk) was located in the terminal ileum, and there was a second smaller duplication at the ileocecal valve (arrow) (A). This anomaly required ileocecectomy with primary anastomosis (B). (Photo courtesy Dr. Zach Kastenberg.)

A B C

Fig. 39.13 This intraoperative photo (A) shows an ileocolonic duplication. The arrow points to the bifurcation of the distal ileum. (Photo courtesy Dr. Mark Molitor.) In the first schematic (B) the terminal ileum is seen to bifurcate into native colon and a duplicate colon, which lies medial to the native colon. In this drawing, the duplicated colon ends blindly in the upper rectum. (C) In this schematic the duplicated colon communicates with the native colon and forms a common descending colon.

SMALL BOWEL DUPLICATIONS which is more common in older children.5 Abdominal US Small bowel duplications account for almost half (47%) of is usually the initial imaging study to evaluate these lesions all reported duplications and are most commonly found in and is often all that is needed for workup (see Fig. 39.6). the ileum (see Table 39.1).5,16 The vast majority are cys- Additional studies such as CT are usually less helpful than tic (Fig. 39.12), but tubular duplications are also seen (Fig. in other locations and lead to unnecessary radiation expo- 39.13).55 Tubular duplications vary in size from a few cen- sure. The presence of ectopic gastric mucosa is found in timeters to the entire length of bowel. Small bowel duplica- 80% of tubular and 20% of cystic duplications.38 Of note, tions may share a common wall or be entirely separate from these small bowel duplications can be mistaken for a Meckel the native intestine. They arise from the mesenteric side and diverticulum on technetium scanning. share a common blood supply with the native intestine (see Operative treatment of small bowel duplications will vary Fig. 39.2). Those not prenatally or incidentally diagnosed based on the type and size. Small cystic duplications can usually manifest secondary to a palpable mass, obstruction, be enucleated provided the native blood supply can be left or hemorrhage. They also may lead to segmental volvulus, intact. Small bowel resection with primary anastomosis is which is sometimes seen in neonates, or intussusception, also acceptable and arguably preferable, depending on the 636 Holcomb and Ashcraft’s Pediatric Surgery

A B

C D

Fig. 39.14 This 2-month-old infant presented with vomiting, and a distal ileal duplication was suspected on ultrasound. The infant underwent laparoscopic evaluation with a 5-mm port in the umbilicus and a 3-mm port placed through a stab incision in the left lower abdomen. Once the diagnosis was confirmed, the umbilical incision was enlarged and the distal ileum and proximal right colon were exteriorized. The duplication is marked with an asterisk (A). Segmental ileocolonic resection was performed with a primary anastomosis (B). The resected specimen is seen in (C). At the conclusion of the procedure, the umbilical incision has been closed and has a nice appearance (D). The baby recovered uneventfully. intricacy of the blood supply and length of resection. Long mucosa, GI bleeding is infrequent. Long tubular duplica- tubular duplications can pose a challenge. Resections of tions have a higher frequency of associated anomalies, large lengths of bowel increase complications and may lead including duplications of the GU system, supporting the par- to short bowel syndrome. In this situation, mucosal strip- tial twinning theory of embryogenesis.57,58 The duplicated ping through multiple enterotomies will preserve bowel colon may or may not communicate distally with the native length and decrease the risk of ulceration or hemorrhage colon and can have a duplicate anus. Similar to imperforate from the ectopic gastric mucosa.56 Laparoscopy is increas- anus, the tubular duplication may be blind ending or fistu- ingly being used for both diagnosis and treatment, thereby lize to the perineum or other parts of the GU system.59 Diag- minimizing open exploration and decreasing hospital stay nosis is typically made with CT or MRI. Contrast enema can (Fig. 39.14).3,38 help delineate the anatomy of any communication with the native GI or GU tracts (Fig. 39.16). COLONIC DUPLICATIONS The treatment of colonic duplications will vary depending on the type, size, and associated anomalies. Small cys- Colonic duplications account for approximately 13% of all tic duplications are typically treated similar to small bowel duplications (see Table 39.1). The majority are on the mes- duplications with enucleation or resection and anastomo- enteric side of the bowel, occur in the cecum, and are cystic. sis. Long tubular duplications present a challenge, and Tubular duplications are less frequent, and when present treatment needs to be tailored to each patient. Resection vary in terms of length and complexity (Fig. 39.15). Large with colectomy is thought to be overly aggressive by many bowel obstruction secondary to compression, intussuscep- surgeons. Colonic duplications rarely contain ectopic gas- tions, and volvulus are the usual presenting symptoms. tric mucosa, so mucosal stripping is not routinely needed. Because colonic duplications rarely contain ectopic gastric Long tubular duplications with distal communication 39 • Alimentary Tract Duplications 637

A B

C D

Fig. 39.15 This female infant was born with high imperforate anus and a duplicate vagina and underwent initial colostomy. At the time of the colostomy, it was noted that she had a tubular colonic duplication. (A) Note where the tubular colonic duplication starts in the transverse colon (arrow). (B) After takedown of the colostomy, the two lumens of the colonic duplication are seen (asterisk and arrow). (C) A stapler is utilized to create a common channel between the two lumens. Note the dressing on the umbilicus as laparoscopy was used to completely mobilize the colon. (D) This end-on view shows a single lumen created after using the stapler to create the common lumen. This lumen was then anastomosed to the rectum.

Mucosal septum

Rectovestibular Normal anus A B fistula

Fig. 39.16 (A) This female infant was found to have a normal anal opening and also a rectovestibular fistula. A distal and proximal cologram shows two separate lumens. The arrow points to the negative shadow of the intervening septum between the native and duplicated colons. (B) A diagram of the infant’s anatomy is depicted. The duplicated colon extended proximally to the transverse colon. The common wall between the native and the duplicated colon was divided. The rectovestibular fistula was excised using an endorectal pull-through technique, and the remaining muscular cuff was plicated and closed. (From Karkera PJ, Bendre P, D’souza F, et al. Tubular colonic duplication presenting as a rectovestibular fistula. Pediatr Gastroenterol Hepatol Nutr 2015;18:197–201. Reprinted with permission.) 638 Holcomb and Ashcraft’s Pediatric Surgery

* * *

A B

Fig. 39.17 On CT this child was seen to have a rectal duplication cyst (white asterisk) posterior to the native rectum (black asterisk) (A). At operation (B), the bulge created by the duplication can be seen through the posterior wall of the native rectum. The duplication was removed via a posterior sagittal approach. (Photos courtesy Dr. Michael Rollins.)

* *

A B

Fig. 39.18 This baby was in the neonatal intensive care unit, and an ultrasound study was performed. An incidental finding of likely two gallbladders (asterisks) was found (A). The baby needed a Nissen fundoplication, and the duplicate gallbladders were confirmed(B). A single cystic duct was seen. The gallbladders were left, and the baby continues to recover well 6 months later. (Photo courtesy Dr. Jorge Godoy.) are often treated conservatively with stool softeners. If posterior sagittal approach for more extensive duplications. there is no distal communication, one may be created to Some patients may require an initial colostomy for large or relieve obstruction (see Fig. 39.15). Fistulous tracts to the complicated duplications. perineum or other organs need to be excised and closed. Associated anomalies also need attention. Summary RECTAL DUPLICATIONS Alimentary tract duplications are rare congenital anom- Rectal duplications account for approximately 3% of dupli- alies that occur anywhere along the enteric tract, most cations (see Table 39.1) and are most often found in the commonly in the small bowel. (Gallbladder and appen- presacral space posterior to the rectum (Fig. 39.17). Chil- diceal duplications are also occasionally encountered dren typically present with constipation due to a mass [Figs. 39.18 and 39.19]). The mucosa is typically the effect. Less frequently, rectal duplications can be associated same as adjacent bowel but can contain ectopic tissue, with anorectal malformations, can fistulize to other pelvic most commonly gastric mucosa, that can cause bleed- organs, or can present in adulthood with obstruction or ing complications. They are usually cystic but also bleeding.42,60,61 Digital rectal examination may reveal a can be tubular. They are discovered prenatally or inci- mass. Multiple imaging modalities including CT, MRI, and dentally, and present with a wide variety of symptoms contrast enema can aid in making the diagnosis. Treatment depending on location. Resection is recommended in options include transanal resection, division of the sep- most instances to prevent complications and potential tum between the duplication and the native rectum, or a malignant transformation. 39 • Alimentary Tract Duplications 639

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