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Journal of Indian Association of Pediatric Surgeons
     Journal of Indian Association of Pediatric Surgeons
Official journal of the Indian Association of Pediatric Surgeons         
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Year : 2023  |  Volume : 28  |  Issue : 2  |  Page : 160-163

Long tubular duplication of the small intestine – Lessons learnt from a common presentation of a rare disease

1 Department of Pediatric Surgery, AMRI Hospitals, Kolkata, West Bengal, India
2 Department of Pediatric Gastroenterology, AMRI Hospitals, Kolkata, West Bengal, India
3 Department of Pediatric Surgery, Nilratan Sircar Medical College, Kolkata, West Bengal, India
4 Department of Pediatric Surgery, Institute of Child Health, Kolkata, West Bengal, India

Date of Submission17-Aug-2022
Date of Decision11-Oct-2022
Date of Acceptance26-Oct-2022
Date of Web Publication30-Nov-2022

Correspondence Address:
Poonam Guha Vaze
AMRI Hospital, Mukundapur, 230 Barakhola Lane, Behind Metro Cash n Carry, Purba Jadavpur, Mukundapur, Kolkata - 700 099, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jiaps.jiaps_114_22

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Long tubular duplication of the small intestine is one of the most difficult surgical challenges. The presence of heterotopic gastric mucosa necessitates resection of the duplicated bowel, but shared vascularity with the normal adjoining bowel makes the task daunting. We present a case of long tubular small intestinal duplication with certain special surgical and perioperative challenges which were successfully managed.

Keywords: Acute intestinal obstruction, Heterotopic gastric mucosa, Long tubular ileal duplication, Melena, Wrenn procedure

How to cite this article:
Vaze PG, Acharyya BC, Banerjee R, Biswas SK, Chattpopadhyay A, Saha S. Long tubular duplication of the small intestine – Lessons learnt from a common presentation of a rare disease. J Indian Assoc Pediatr Surg 2023;28:160-3

How to cite this URL:
Vaze PG, Acharyya BC, Banerjee R, Biswas SK, Chattpopadhyay A, Saha S. Long tubular duplication of the small intestine – Lessons learnt from a common presentation of a rare disease. J Indian Assoc Pediatr Surg [serial online] 2023 [cited 2023 Mar 29];28:160-3. Available from: https://www.jiaps.com/text.asp?2023/28/2/160/362393

   Introduction Top

Long tubular duplication of the small intestine is a life-threatening entity, which usually presents with massive gastrointestinal (GI) bleed due to the presence of heterotopic gastric mucosa (HGM). Preoperative diagnosis is a rarity and it poses a difficult surgical challenge.

Long tubular duplication of the small intestine has been managed in several ways – in those without HGM, the creation of a common lumen between the duplication and the normal bowel or drainage of the duplicated gut into the stomach has been described.[1] The presence of HGM or a history of bleed mandates the complete removal of the duplicated mucosa. Two surgical techniques described by Bianchi[2],[3] and Wrenn[4],[5] have been utilized in dealing with this anomaly.

Although there exists literature on dealing with the disease and postoperative course, we faced certain challenges during the surgery and in the postoperative period which have not been elucidated previously. Wonderful teamwork with a diligently performed surgery and good intensive care unit care led to successful recovery in our case.

   Case Report Top

A 9-month-old boy presented with a history of two episodes of self-limiting melena at 5 and 8 months of age. He underwent Meckel's scan which revealed strong uptake of tracer in the lower abdomen positive for Meckel's diverticulum.

He underwent laparoscopy which revealed long-segment tubular duplication of the small intestine, and surgery was converted to laparotomy. Intraoperative findings revealed tubular duplication of the small intestine starting from the duodenojejunal (DJ) flexure to around 30 cm from the ileocecal junction (ICJ) [Figure 1]. The duplicated bowel shared a common wall with shared vascularity for most of the length with the distal end opening into the terminal ileum. Resection of the terminal 10 cm of the duplicated bowel was done as it may contain the HGM and further stripping of the mucosa of the rest duplicated segment was done. Mucosa of around 30 cm of the duplicated bowel [Figure 2] was found to be common with that of the native bowel without any intervening muscularis/serosa. The mucosa of the normal gut was injured at two locations and there was considerable blood loss. The procedure was abandoned– a proximal anastomosis was created between the normal and duplicated bowel as a proximal limb, and the bowel from which duplicated mucosa had been stripped off as a distal limb; a distal anastomosis was performed at the site of initial resection at around 30 cm from ICJ. There was still 40–50 cm of duplicated bowel segment remaining which we planned to strip at a second sitting if required.
Figure 1: Tubular duplication of jejuno-ileum on the mesenteric side

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Figure 2: Stripped mucosa of the distal segment of the duplication during the first surgery, delivered out through the window in the mesentery and seromuscular coat of the duplicated bowel

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The child had an episode of intraperitoneal bleed on POD 0 resulting in blood in the drain, hemodynamic instability, and a drop in hemoglobin – probably due to continued ooze from mucosal stripping sites – settles on conservative management. POD 5 he was well, passing stools, nasogastric tube output had ceased and he was tolerating oral liquids. POD 6, he developed fever (which was later proven to be due to central line-induced sepsis), tachycardia, one episode of melena, features of intestinal obstruction, and a rapid fall of hemoglobin from 13 to 8 g/dl. A plain X-ray abdomen revealed grossly dilated small bowel loops. Upper GI contrast study showed a dilated duodenum and nonpassage of contrast beyond the DJ flexure by the end of 2 h [Figure 3]. After the contrast study, he passed large-volume melenic stools following which his obstruction settled.
Figure 3: Upper GI contrast study– 2 h image, showing dilated duodenum and stomach with no passage of contrast distally. GI: Gastrointestinal

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After resuscitation, the child underwent re-exploration on POD 8 with the aim of stripping out the residual duplicated mucosa. The proximal anastomosis was dismantled, and mucosal sleeve dissection was started. The common wall between the normal and duplicated bowel was very thin for a length of 20–30 cm without any intervening muscularis. The mucosal sleeve was dissected circumferentially with bipolar diathermy, not by traction, with meticulous attention to hemostasis. While proceeding proximally, after every 2–3 cm, the serosa and muscularis of the duplicated bowel were scored transversely and the mucosal tube was delivered out through the fresh incision. This was continued until we found a definite muscularis layer in between the adjoining bowels when dissection was much easier and undermining was possible for longer lengths. The whole duplicated mucosa was stripped till the DJ flexure [Figure 4]. The normal bowel was distended with saline to check for any leaks. Luminal continuity was established by end-to-end anastomosis.
Figure 4: Stripped out residual mucosa in the second surgery

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Postoperatively, the child had significant abdominal distension with delayed return of bowel motility. POD 6 when he was started on orals. The child tolerated orals and was discharged on POD 10. Histopathological examination of the stripped mucosa showed evidence of HGM throughout its length.

   Discussion Top

Long tubular duplication of the small intestine is a well-known but rare entity.

Preoperative presentation is usually GI bleed,[5],[6],[7] features of sepsis,[6] or abdominal pain.[3] The abdominal lump has been detected in cases with associated cystic duplications.[3],[5]

Preoperative radiological findings have usually identified associated cystic duplication.[3],[5] Occasionally, long tubular duplication has been picked up by preoperative sonography as a large well-defined cystic lesion with thick walls with gut signature and internal debris[6] – the patient in this report was sick with features of GI bleed and sepsis – accumulation of the contents within the duplicated bowel might have caused sepsis and enabled identification of the duplicated bowel.

Tc-99m pertechnetate scintigraphy picks up the presence of HGM, but has been traditionally “mislabeled” as “Meckel's scintigraphy.” Intestinal duplications tracer activity can be visualized in the dynamic sequence or before gastric tracer visualization in an irregular pattern – depending on the size and location of HGM.[8] Long tubular duplication with HGM shows diffuse uptake of tracer in the central abdomen corresponding to small intestinal loops and increasing in intensity with gastric uptake.[5],[6] Our case showed a focal area of increased uptake in the lower abdomen – giving an impression that HGM was concentrated near the terminal ileum.

Thus, long tubular duplication is usually not diagnosed preoperatively as in our case, and is an intraoperative surprise.

Several surgical procedures have been described for the disease depending of the length of the uninvolved small intestine and the presence or absence of HGM. Holocomb et at mentioned one child with long-segment intestinal duplication, in their series of 96 enteric duplications, who underwent staged resection of the whole involved gut.[7] In cases without evidence of HGM, procedures such as drainage in the Roux loop or division of the common wall between the native and duplicated bowel have been described.[1] Wrenn described stripping out of the mucosal tube from the entire duplicated bowel to remove all tissue-bearing HGM.[4],[5] Sham et al. reported the use of Bianchi principle to excise the duplicated bowel by dividing one leaf of the mesentery.[3] In our case, the removal of whole duplicated mucosa was possible because of the use of Wrenn principle. Dissection of the common wall would have been difficult by Bianchi technique.

Many case reports have reported staged surgery involving the management of associated cystic duplication or partial resection of duplicated segments and dealing with the rest of tubular duplication in the second sitting[5],[7] when the second surgery was necessitated by the fresh episode of bleed. In our case, dissection of the duplicated mucosa from the native mucosa was a challenge because of no intervening muscularis for a considerable length – resulting in injury to the native mucosa at two points. Furthermore, there was significant intraoperative blood loss and preoperative nuclear scan gave an impression of the HGM being localized in the terminal small bowel. Thus, we did not want to risk the native bowel further without any evidence of HGM in the remaining bowel and abandoned further attempts at stripping in the first surgery. Our patient had an episode of massive bleed very early in the postoperative period which could be diagnosed and managed effectively as he was still admitted in the hospital.

Learning points

  1. Colloquial use of the term “Meckel's scan” is misrepresentative – atypical findings in our case were not recognized preoperatively which could have led to proper preoperative preparation and counseling of the family
  2. As has been already described, the duplicated bowel is a “ticking time bomb”[5] – whole of the duplicated mucosa needs to be stripped off at the first instance – any residual mucosa can lead to torrential bleed at any point of time
  3. Blood accumulation in the duplicated bowel can lead to the development of luminal obstruction of the normal bowel and present with complete proximal small bowel obstruction
  4. The duplicated mucosa and normal mucosa can lie in very close without any intervening muscularis – the Bianchi procedure may prove dangerous and injure the mucosa of the normal gut. Wrenn procedure is safer, but utmost care is needed while dissection of common wall
  5. The serosa and muscularis of the duplicated bowel should be scored every 2–3 cm and the mucosal sleeve delivered out though the new incision till the time proper muscularis is identified between the two mucosae, when the length of undermining may be increased
  6. Thorough importance should be given to hemostasis while dissection of mucosal tube and no attempt should be made to strip by traction. Any bleeder left unaddressed may continue to ooze into the dead space and cause hemodynamic compromise
  7. Significant edema in the residual serosal tube with delayed return of motility/pseudoobstruction should be anticipated in the early postoperative period.
  8. Diligent surgery and good postoperative parenteral nutrition support can lead to a successful outcome.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal his identity, but anonymity cannot be guaranteed.


We would like to thank to Dr. Soumen Meur and Dr. Monideepa Dutta, Consultant Pediatrician and PICU Incharge, AMRI Hospitals, 230 Barakhola Lane, Purba Jadavpur, Kolkata 700099, for their excellent support in patient care which was instrumental in a successful outcome in the case.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

   References Top

Pal K. A treatise on intestinal duplications. Saudi J Med Med Sci 2015;3:8.  Back to cited text no. 1
  [Full text]  
Bianchi A. Intestinal loop lengthening – A technique for increasing small intestinal length. J Pediatr Surg 1980;15:145-51.  Back to cited text no. 2
Sham M, Phadke D, Singh D. Bowel conservation in a case of giant Jejuno-ileal duplication. J Indian Assoc Pediatr Surg 2010;15:104-5.  Back to cited text no. 3
[PUBMED]  [Full text]  
Wrenn EL Jr. Tubular duplication of the small intestine. Surgery 1962;52:494-8.  Back to cited text no. 4
Khanna V, Khanna K, Srinivas M. Total midgut duplication: A ticking time bomb. BMJ Case Rep 2018;2018:bcr2017223848.  Back to cited text no. 5
Bhattacharya A, Samujh R, Rao KL, Mittal BR. Long segment Jejuno-ileal duplication cyst with ectopic gastric mucosa detected on 99mTc-pertechnetate scintigraphy. Indian J Nucl Med 2013;28:96-8.  Back to cited text no. 6
[PUBMED]  [Full text]  
Holcomb GW 3rd, Gheissari A, O'Neill JA Jr., Shorter NA, Bishop HC. Surgical management of alimentary tract duplications. Ann Surg 1989;209:167-74.  Back to cited text no. 7
Kiratli PO, Aksoy T, Bozkurt MF, Orhan D. Detection of ectopic gastric mucosa using 99mTc pertechnetate: Review of the literature. Ann Nucl Med 2009;23:97-105.  Back to cited text no. 8


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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