Formosan Journal of Surgery

ORIGINAL ARTICLE
Year
: 2021  |  Volume : 54  |  Issue : 2  |  Page : 39--44

Duplications of the alimentary tract in infants and children


Rupesh Keshri, Ramjee Prasad, Digamber Chaubey, Zaheer Hasan, Vijayendra Kumar, Vinit Kumar Thakur, Ramdhani Yadav, Rakesh Kumar, Asjad Karim Bakhtiyar, Sandip Kumar Rahul 
 Department of Paediatric Surgery, Indira Gandhi Institute of Medical Sciences, Patna, Bihar, India

Correspondence Address:
Sandip Kumar Rahul
S/O Shri Kapil kumar Jha, Qr. No. - BN-2/B, IGIMS Campus, Patna - 800 014, Bihar
India

Abstract

Background: Duplications of the alimentary tract are known for their embryonic, anatomical, clinical, and pathologic variations. Summarizing the features of these lesions would reveal these characteristics and guide appropriate management. The objectives of this study are to describe the clinical features and characteristics of all cases of duplication of alimentary tract managed at a tertiary center. Materials and Methods: A retrospective study on all cases of duplications of the alimentary tract managed at a tertiary center from July 2015 to June 2020 (5 years) was conducted after approval from the Institutional Ethics Committee. Data regarding history, demographic details, symptoms, clinical features and investigation results, and intraoperative and histopathologic findings were collected from the hospital records and analyzed. Results: Twenty-eight cases of duplication were managed during this period. They had different locations (esophageal-5 [cervical-2 and thoracic-3], gastric-1, jejunal-3, ileal – 11, cecal-3, appendicular-2, colorectal-1, rectal-1, and posterior anal canal-1) and site-specific symptoms but definite histopathology with evidence of adjacent gastrointestinal tissue on microscopic examination. Unique cases included cervical esophageal duplication, bleeding ileal duplication (ID) with heterotopic mucosa, ID with bezoar, jejunal duplication with malrotation, appendicular duplication with type 2 pouch colon and anorectal malformation, rectal duplication, and posterior anal duplication. Case-specific management ensured minimal complication without any mortality. Conclusion: Variable location and site-specific symptoms necessitate individualized case-specific management of duplication anomalies. Histopathology confirms both native and heterotopic gastrointestinal tissues and is indispensable for the diagnosis.



How to cite this article:
Keshri R, Prasad R, Chaubey D, Hasan Z, Kumar V, Thakur VK, Yadav R, Kumar R, Bakhtiyar AK, Rahul SK. Duplications of the alimentary tract in infants and children.Formos J Surg 2021;54:39-44


How to cite this URL:
Keshri R, Prasad R, Chaubey D, Hasan Z, Kumar V, Thakur VK, Yadav R, Kumar R, Bakhtiyar AK, Rahul SK. Duplications of the alimentary tract in infants and children. Formos J Surg [serial online] 2021 [cited 2021 Apr 13 ];54:39-44
Available from: https://www.e-fjs.org/text.asp?2021/54/2/39/311585


Full Text



 Introduction



Duplications of the alimentary tract (DAT) are known to involve any part of the gastrointestinal tract (GIT) and may present with a range of symptoms depending upon their size, site, local mass effect, and the presence of ectopic tissues.[1],[2],[3] They may also be seen in association with other anomalies.[4],[5],[6] Very often, they are unexpectedly encountered intra-operatively; appropriate surgical management requires that the surgeons be familiar with the anatomy and clinical characteristics of these lesions.

Through this study, we summarize the presentation, clinical features, and management of DAT at different locations in GIT at our institution in the past 5 years.

 Materials and Methods



A retrospective, descriptive, observational study was conducted on all patients of DAT managed in the department of pediatric surgery at a tertiary center after approval from the Institutional Ethics Committee with approval letter No (1694/IEC/2020/IGIMS). This study included all the patients in whom duplication of any portion of alimentary tract was either diagnosed preoperatively and confirmed at the time of surgery or found unexpectedly intraoperatively, when surgery was done for some related problems. The study period ranged from July 2015 to June 2020 (5 years). Consent of parents was obtained before the inclusion of these children in the study.

The case records and intraoperative findings of all cases of DAT were collected to get detailed history, demographic details (age, sex, and residence), presenting symptoms, examination findings, investigation results (blood, radiological, and Meckel scan), intra-operative findings, histopathological examination (HPE) results, and follow-up details. Data thus collected were analyzed.

 Results



The presenting age of the patients varied from 3 days to 8 years. 19/28 patients presented below 1 year of age with different symptoms. Males (24/28) outnumbered females (4/28). The presenting symptoms varied depending on the site and size of the lesion, its local pressure effect, and histopathology.

[Figure 1] shows the clinical and intraoperative pictures of duplication cysts at different sites at common (ileal) and uncommon locations (cervical esophageal and appendicular and posterior anal canal). [Table 1] summarizes the clinical presentation, intra-operative findings, and management of duplication cysts.{Figure 1}{Table 1}

Two cases of cervical esophageal duplications were seen; this location has been reported to be rare in the literature.[7],[8],[9] By contrast, esophageal duplication in the thoracic region is more common, and we found three such cases in our study.

Moving down in the GIT, we found a case of tubular gastric duplication adherent to the greater curvature of the stomach. Both cases of appendicular duplication were seen in neonates with anorectal malformation (ARM) and had type 2 pouch colon distally. Both these appendicular duplication were of B1 type and mimicked the appendicular anatomy seen in birds.[10]

Cecal duplication was seen in three children; two cases presented with intussusception below 6 months of age with intraluminal cecal duplication acting as a lead point to ileo-cecal intussusception. Another case of cecal duplication was antenatally detected and caused only partial luminal occlusion without causing obstruction. This child was followed up with periodic abdominal sonogram and had laparoscopy-assisted resection at 1 year of age.

Colorectal duplication was found in a child of rectovestibular fistula (RVF) with vaginal atresia. This patient was later found to have a nonfunctioning duplex ectopic right kidney and a left megaureter.

A case of rectal duplication was found while operating upon a case of RVF; the lesion could be excised totally through this approach and healthy bowel proximal to it was pulled down to perform anoplasty. There was also a rare case of the posterior anal duplication in a female child who presented with a narrow caliber luminal lesion just posterior to the normal anus.

In esophageal and gastric duplication, excision was possible without disturbing the native esophagus or stomach. However, in jejunoileal and cecal duplication, resection of involved segment and end-to-end anastomosis of healthy bowel was needed. In two cases which presented in emergency in a sick state, ileostomy was fashioned after excision of duplicated segment; stoma reversal was done after 6 months. Rectal and colorectal duplication needed excision of the bowel segment containing duplication and fashioning a neo-anus from the healthy bowel. Posterior anal duplication was excised through the posterior sagittal route and the entire tract up to the coccyx needed excision. In all cases, HPE results showed evidence of corresponding native tissue of GIT with which it was adherent; ectopic gastric and pancreatic mucosa were found in a patient of ileal duplication (ID) with blood mixed stool.

There was no mortality in the study. All these patients were symptom-free in the postoperative period. Both children (with rectal and colo-rectal duplication), in whom neo-anus was created after excision of duplicated segment and pulling down the healthy bowel did well with rectal washes and serial neo-anal dilatation. There was no incontinence in the child with posterior anal duplication as the native anus was left undisturbed within the confines of its sphincter. Child with RVF and vaginal atresia was the most complicated among these patients. She also had a nonfunctioning ectopic right duplex moiety with persistent urinary leak due to ectopic ureteric insertion; also, the left ureter was an obstructed megaureter. She needed excision of the ectopic right duplex moiety and left ureteric reimplantation and is doing well as a 3-year-old child now. Vaginal reconstruction has been planned in her at a later date at around 10 years of age or at the time of attaining puberty.

 Discussion



DAT can involve any part of GIT.[11] Their presenting features may vary depending on their site, size, associated anomalies, and HPE features.[11] Although these lesions are well-known anomalies in pediatric patients, their etiology and embryology are still not well understood with theories such as aberrant recanalization, split notochord theory, diverticular theory, or the role of environmental factors.[12],[13],[14],[15],[16],[17],[18],[19],[20],[21] It is also not known whether a single theory can explain duplication at different sites. Associated anomalies can sometimes unravel the missing links or causes behind DAT. Hence, such descriptive studies still hold relevance.

We observed that more infants presented with DAT when compared to older children and males were far more affected than females. This is in line with the observation of other investigators.[3],[11],[22],[23]

Duplication of esophagus is reported to occur more commonly in its thoracic part; cervical esophageal duplications are rare as seen in this study.[9],[24] While cervical lesions presented with an obvious swelling locally with pressure symptoms such as noisy breathing and difficulty in swallowing, thoracic duplications presented with only dysphagic symptoms. Contrast-enhanced computed tomography scan was used to delineate the anatomy before formal excision of the lesion. These lesions could be excised without opening the wall of the native esophagus. HPE showed cyst wall lined by predominantly cuboidal epithelium, focally exhibiting stratification. Subepithelium showed glands below which the double layer of smooth muscle could be appreciated. Cystic lesions must fulfil the criteria laid down by Palmar to be called duplication cysts.[5],[25] In the esophagus, this implies that the cyst should lie within the esophageal wall or be attached to its wall, it should have two muscle layers and an inner layer consisting of the epithelium of the GIT or respiratory tract.

Gastric duplications have been described to constitute around 2%–9% of DAT.[26] Most of them are seen along the greater curvature (like our case), and only a few of them along the lesser curvature. Gastric duplication in this study could be excised completely without opening the stomach wall because it was not communicating with the stomach. Most of the gastric duplication cysts reported in literature are of noncommunicating type.[26],[27] HPE features revealed the cyst wall lining, its cavity, gastric mucosal lining, and muscle layers.

Duodenal duplications are rare, comprising 2%–12% of all GIT duplication[28],[29] and have been reported with the symptoms of local mass effect leading to bilious vomiting and upper abdominal fullness. We, however, did not encounter any duodenal duplication in our series.

Duplications involving the small bowel are the most common among DAT.[11],[29],[30] In our series also, Jejuno-ID s contributed to around 50% of all DAT; also, ID were exceedingly common when compared to jejunal duplication. These cases presented differently. An 8-year-old patient presented with repeated bilious vomiting and chronic abdominal pain; he was found to have malrotation, midgut volvulus, and jejunal duplication. Such an association is rare and has been described by only a few investigators.[31],[32],[33] Ladd's bands in this patient were thick, and chronic pain could be attributed to the episodes of twisting and nontwisting of the bowel.[34],[35]

Another patient of ID presented as a 2-year-old male child with lower GIT bleeding and on doing Meckel scan (using Technetium 99-m pertechnetate scintigraphy), ectopic gastric mucosa could be found at more than one place in the small bowel. At exploratory laparotomy, there was ID and meckel diverticulum (MD) in the same patient, and it was the ID which was seen to be the site of bleeding and not the MD. This unusual case had simultaneous presence of both heterotopic gastric and pancreatic mucosa; MD had also ectopic gastric tissue, but it was not responsible for bleeding in this patient. Although ectopic gastric mucosa in duplication cysts has been previously reported and around 20%–30% of duplications have been found to have either heterotopic gastric or pancreatic mucosa,[36],[37] such simultaneous presentation with MD and both bearing ectopic mucosa but only one showing the evidence of bleeding has rarely been described. Milbrandt and Sigalet described a case of intussusception associated with both MD and ID.[38]

Another atypical presentation was in a case of ileal narrowing because of ID proximal to which massive dilatation of ileum due to a giant mixed bezoar was seen; this obstructed the small bowel. During laparotomy, ID was seen to cause luminal narrowing which led to a massive bezoar proximally. Such presentation has seldom been reported in the literature.

We also found ID in cases of high ARM, when they were operated for abdomino-perineal pull through. Because they were asymptomatic, resection was not done as these neonates were already having a major surgery and we did not want to unnecessarily prolong the surgical and anesthetic procedure. However, these children have been kept on close follow-up with periodic abdominal sonograms.

Some other modes of presentation of jejuno-IDs were by the features of intestinal obstruction, perforation, or chance finding during laparotomy for an unrelated cause. In all these cases (except perforation), resection of involved segment and anastomosis was done. Perforation patient had stoma formation and did well when stoma reversal was done after 6 months.

Appendicular duplications are rare with an incidence of around 0.004%.[39] They have been reported to be associated with other anomalies such as ARM as in our case;[40],[41] both cases showed a single cecum with two appendices, symmetrically on either side of the ileo-cecal valve (Type B1, Cave and Wallbridge classification).[10] Cecum had a type 2 pouch colon distally which ended with a fistulous communication to the urinary bladder in both neonates. Chadha and Khan described the association of Type 2 pouch colon with various appendicular anomalies such as its absence, duplication, or short stuby length.[42] After division of the fistula, we mobilized the pouch colon and took it down to fashion a neoanus without disturbing any of the two appendices. Both children are around 1 year of age now and are doing well with bowel management program consisting of serial neoanal dilatation and daily rectal washes with normal saline and glycerine.

Three patients in our series had cecal duplication. In two patients under 6 months of age, intraluminal cecal duplication was the lead point to ileo-colic intussusception and both children did well after resection and ileo-ascending anastomosis. Third child had an antenatally detected cecal duplication which was asymptomatic after birth. This patient was followed by serial ultrasound abdomen and underwent resection and anastomosis electively at 1 year of age. The rarity of cecal duplication can be judged by the findings of Radhakrishna et al. who reported that only 43 cases of cecal duplication had been reported before his publication in 2018.[43] Most of the cases reported have presentation within the 1st year of life and obstructive features of the bowel or intussusception are the common modes of presentation.[43]

Colonic and rectal duplication cysts are known to contribute to 13% and 4% of all cases of abdominal duplication cysts.[44],[45] In our series, only one case of colo-rectal duplication was found, and this was in a female child with RVF and vaginal atresia in whom laparotomy was done to do abdominoperineal pull through to create a neoanus, while doing this procedure duplication of distal sigmoid and proximal rectum was found and this anomalous segment was resected and neoanus fashioned after pulling down the healthy bowel. Later, this patient also was found to have anomalies of the urinary system. Rectal duplication was similarly found while performing posterior sagittal anorectoplasty (PSARP) for RVF. Rectal duplication has been described in the differential diagnosis of swellings in the presacral space[46] and can be associated with Currarino's triad.[47] It may present with a presacral mass causing constipation, pressure effects, tenesmus, and even urinary retention;[48] ectopic mucosa may lead to abnormal rectal bleeding; there may be pain and perineal sepsis in complicated cases.[49] We could excise the entire rectal duplication completely through PSARP and healthy bowel was then pulled down to form a neoanus in the normal position. HPE features confirmed it to be a rectal duplication.

Posterior anal duplications are very rare. In fact, they have been described as the least common digestive duplication.[50] While Choi and Park consider it to be the result of recanalization of a cloacal membrane excess in late embryonic life, Hamada et al. suggests it to be an early embryonic insult in the form of duplication of the dorsal cloaca.[15] Our case was of tubular variety, which is the more common type and it extended all the way up to the coccyx; PSARP helped us in excising the entire tract. This anomaly can occur as an isolated anomaly or as a part of the caudal duplication syndrome, so magnetic resonance imaging of the pelvis should be done to find out other possible results of twinning of hindgut derivatives.[6] In general, there is no incontinence in this isolated anomaly as the native anus with its sphincters is left undisturbed during the operative procedure. In grownup children, it may be confused with local fistulae as a result of perineal abscesses, pilonidal disease, or any local perineal pathology. Hence, awareness about this entity is necessary for appropriate management.

 Conclusion



The spectrum of DAT, its variable location, vivid presentation, and associated anomalies contribute to the complexity of its management and long-term morbidity. Proper understanding of embryology and pathology of these lesions facilitates good surgical outcome.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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