CASE REPORT Bali Medical Journal (Bali MedJ) 2021, Volume 10, Number 2: 526-528 P-ISSN.2089-1180, E-ISSN: 2302-2914 Complete tubular duplication of colon presenting as rectovestibular : A case report

Published by Bali Medical Journal Muntadhar Muhammad Isa1*, Amir Thayeb2, Maria Meildi3, Muhammad Bayu Zohari Hutagalung3

ABSTRACT 1Pediatric Surgery Division, Department of Surgery, Faculty of Medicine, Background: Duplication of the is a rare congenital disorder in pediatric patients. Complete resection Universitas Syiah Kuala/Dr. Zainoel in case of a total tubular duplication that requires total or subtotal colectomy is undesirable in children. Abidin Hospital, Banda Aceh, Indonesia; Case Presentation: This case presented a simple surgical technique for treating complete colonic duplication without 2 Pediatric Surgery Division, Department colonic resection. A 2 months old female baby with abdominal distention and stool complaint came out from her vaginal of Surgery, Faculty of Medicine, vestibular and normal anus. A digital rectal exam showed a palpable mass in the posterior region. Abdominal CT Scan showed Universitas Indonesia/Dr. Cipto a rounded mass with size 2x2 cm in the left posterolateral of region. The initial diagnosis was suspected sacrococcygeal Mangunkusumo Hospital, Jakarta, Indonesia; tumor type 4 with rectovestibular fistula. Intraoperative findings showed duplication of the caecum, appendix, total colon 3Department of Surgery, Faculty of (ascending to sigmoid) and rectum (one directed to the and the other to anus). The ileostomy was done 20 cm from the Medicine, Universitas Syiah Kuala/Dr. ileocaecal junction as a temporary treatment to relieving distention with definitive repair planned in the following months. Zainoel Abidin Hospital Banda Aceh, The second stage repair was performed after 1-year-old with posterosagital anorectoplasty followed by distal separation of Indonesia. the duplicated colon using a 12 cm linear stapler. For this case, the third stage repair was done one year after the previous surgery to close the remaining ileostomy. The patient’s outcome showed good results with normal defecating function and *Corresponding author: no complication. Muntadhar Muhammad Isa; Conclusion: Complete tubular duplication is a rare case without specific clinical symptoms, but the assessment of the Pediatric Surgery Division, Department diagnosis can be assisted by radiology such as abdominal CT and barium enema. We hope this article could illustrate of Surgery, Faculty of Medicine, management for complete tubular duplication of colon presenting as rectovestibular fistula, thus reflect how important Universitas Syiah Kuala/Dr. Zainoel Abidin Hospital, Banda Aceh, Indonesia; clinical judgment and initial diagnostic in our health care system. [email protected] Keywords: Colon Duplication, Surgery, Repair. Received: 2021-04-17 Cite This Article: Isa, M.M., Thayeb, A., Meildi, M., Hutagalung, M.B.Z. 2021. Complete tubular duplication of colon presenting Accepted: 2021-06-19 as rectovestibular fistula: A case report.Bali Medical Journal 10(2): 526-528. DOI: 10.15562/bmj.v10i2.2386 Published: 2021-06-26

INTRODUCTION The treatment option for colonic presentation of this anomaly, we hope duplication is complete resection of the publishing our case will increase diagnostic Colonic duplication is a congenital duplication in the long tubular colon. precision and improve surgical treatment.9 disorder that occurs in 0.2% of the pediatric The procedure also requires total or population. Malformations are rarely subtotal colectomy because of the joint CASE REPORT found in the (only 5-6%) blood supply of the native bowel and A 2 months old female baby was admitted and can occur along the gastrointestinal duplication. The sphincter complex may into the Emergency Room (ER) with tract, with a complete tubular form need reconstruction, but this procedure 1,2 complaints of abdominal distention, and which is very rare. Clinically, colonic is not used in cases involving children. stool came out from her vaginal vestibular duplication is usually asymptomatic, Simple surgical techniques for the and normal anus. No history of same making it difficult to diagnose before treatment of complete colonic duplication condition in family members. Physical surgery. Therefore this malformation can are described.7,8 examination revealed signs of an acute only be confirmed during a laparotomy. We report a case of complete tubular abdomen with a large accumulation of fluid The clinical features of colonic duplication duplication of the colon presenting as a between the bowels. A digital rectal exam vary depending on the location, mucosal rectovestibular fistula with abdominal 3 showed a palpable mass in the posterior layer involved, and the lesion’s extent. distension. Duplication starts from the region. The abdominal x-ray did not show More than 80% of cases present before two cecum, showing two caeca and two any conclusive findings. Abdominal CT years of age as acute stomach or intestinal appendixes and two complete tubular 4,5 Scan showed a rounded mass with size obstruction, but it can occur at any age. colonic formations. There is one normal 2x2 cm in the left posterolateral of rectum However, malformations in adulthood are anus and rectovestibular fistulae. Given region. Other examination within the increasingly difficult to diagnose because the low incidence and unique clinical there is no specific clinical presentation.6 normal limit. The initial diagnosis was

526 Published by Bali Medical Journal | Bali Medical Journal 2021; 10(2):Open 526-528 access: | doi: www.balimedicaljournal.org 10.15562/bmj.v10i2.2386 CASE REPORT

et al. as recently as 2005.15 Most of the literature suggests that complete excision of duplicate channels is the preferred surgical technique.10 In this case, we performed a distal separation from the colonic duplication using 12 cm linear stapling. After dividing the septum between the normal and duplicate distal colon, the rectum acquires a single lumen, and making evacuation easier. Large communication between the Figure 1. Left: Distal separation of the duplicated colon using the stapling device; duplicated colon and the normal colon Right: Posterosagital Anorectoplasty. is required to ensure the emptying of the duplicated stool and ensure the passage of the stool, thereby relieving .7 suspected sacrococcygeal tumor type From a morphological perspective, the The study by Yucesan prefers to construct 4 with rectovestibular fistula. A precise most common type of anomaly is cystic a common channel, using mechanical preoperative diagnosis could not be duplication along the colon, followed sutures for common wall resection to established, so an urgent laparotomy was by partial tubular duplication, and very avoid accumulation feces in the duplicated performed. rare literature shows cases of complete intestine.16 Complete Tubular Colon The patient underwent exploration cecoapendicular duplications such as in Duplication has low mortality of around laparotomy. Intraoperative findingsthis patient.6,11 The most interesting aspect 4-8%. Mortality is generally associated showed duplication of the caecum, of this case was its clinical presentations: with severe malformations or malignant appendix, total colon (ascending to acute abdominal distension and transformations. Duplication of the total sigmoid) and rectum (one directed rectovestibular fistula, which are very rare tubular colon treated before complications to the vagina and the other to anus). because they are usually asymptomatic occurs generally has a good prognosis, so The ileostomy was done 20 cm from in children and found in adulthood. early diagnosis is a very important aspect.12 the ileocaecal junction as a temporary The lack of specific clinical features of treatment to relieving distention with these malformations remains a serious CONCLUSION definitive repair planned in the following diagnostic dilemma.3,12 months. Digestive tract duplication is very Complete tubular duplication is a rare One year later, the second stage difficult to diagnose at a preoperative case without specific clinical symptoms, repair was performed with posterosagital time, but ultrasound can be used to help but the assessment of the diagnosis can be anorectoplasty followed by distal make a preoperative diagnosis as well assisted by radiology such as abdominal separation of the duplicated colon using as a screening tool to treat 10-20% of CT and barium enema. This article a 12 cm linear stapler. At the next year, multiple lesions. Imaging using a chest or illustrates a two months old female baby the third stage repair was done to close abdominal CT and barium enema can also with complaints of abdominal distention, the remaining ileostomy. The patient’s help in making the diagnosis. A barium and stool came out from her vaginal outcome showed good results with normal enema examination can be used to assist vestibular and normal anus. The patient defecating function and no complication at in the diagnosis of duplication of the received 3 stage repairs. The first repair clinic visit after 1 month after last surgery. tubular colon, with opacification of two is ileostomy. The second stage repair is colons being the diagnostic sign.6,7 posterosagital anorectoplasty followed by DISCUSSION Several kinds of literature describe distal separation of the duplicated colon, various surgical techniques for the and the third stage repair to close the Colonic duplication is the most unusual management of colonic duplication. Riedel remaining ileostomy. We hope this article type of intestinal tract duplication, and operated on Grohe’s case by dividing could illustrate management for complete the complete tubular form is even less the septum between the two rectum and tubular duplication of colon presenting common. Several studies have shown the tissue between the and as rectovestibular fistula, thus reflect how a high association between colonic reconstructing the perineum.13 Another important clinical judgment and initial duplication with prostate rectal , study performed a lateral anastomosis diagnostic in our health care system. anorectal or scrotal abnormalities, between two sigmoids, then the sigmoid and myelomeningoceles present in leading to the fistula is divided under CONFLICT OF INTEREST the genitourinary or lower vertebral the anastomosis by leaving the intestinal column.9,10 The pathogenesis of these All of the authors declare that there were sac to allow the mucus to be expelled malformations is unclear but is said to no conflicts of interest in this study. through the fistula.14 A similar technique be multifactorial concerning molecular was used in the case described by Sarpel genetics.11

Published by Bali Medical Journal | Bali Medical Journal 2021; 10(2): 526-528 | doi: 10.15562/bmj.v10i2.2386 527 CASE REPORT

PATIENT CONSENT Dilemma. Eur J Pediatr Surg. 72. Available from: http://dx.doi. 2006;16(1):58–60. Available from: http:// org/10.1016/j.jpedsurg.2012.12.032 The patient’s parents had agreed and signed dx.doi.org/10.1055/s-2006-923794 10. Singh S, Ahmed I, Rawat J, Panday informed consent regarding publishing 4. Kekez T, Augustin G, Hrstic I, Smud D, A. Association of anorectal the case in an academic journal without Majerovic M, Jelincic Z, et al. Colonic malformation with duplicated colon, exposing his child’s identity. duplication in an adult who presented sacral meningomyelocele and scrotal with chronic constipation attributed to anomalies. Case Reports. 2011;2011(jul27 FUNDING hypothyroidism. World J Gastroenterol. 1):bcr1220103632–bcr1220103632. 2008;14(4):644. Available from: http:// Available from: http://dx.doi.org/10.1136/ None. dx.doi.org/10.3748/wjg.14.644 bcr.12.2010.3632 5. Merrot T, Anastasescu R, Pankevych 11. LETELIER M A, BARRÍA M C, BELTRÁN AUTHOR CONTRIBUTION T, Tercier S, Garcia S, Alessandrini P, S MA, MORENO CH C. Duplicación et al. Duodenal Duplications. Clinical intestinal: Diagnóstico y tratamiento de Substantial contributions to conception Characteristics, Embryological una condición inusual. Rev Chil cirugía. and design, or acquisition of data, or Hypotheses, Histological Findings, 2009;61(2). Available from: http://dx.doi. analysis and interpretation of data: Treatment. Eur J Pediatr Surg. org/10.4067/s0718-40262009000200011 Muntadhar M. Isa, Amir Thayeb, Maria 2006;16(1):18–23. Available from: http:// 12. Kothari PR, Shankar G, Gupta A, Meildi, Muhammad Bayu Z. Hutagalung dx.doi.org/10.1055/s-2006-923798 Rastogi A, Kulkarni B. Multiple tubular Drafting the article or revising it critically 6. Kabay S, Yucel M, Yaylak F, Hacioglu A, and cystic intestinal duplications. Algin MC, Olgun EG, et al. Combined Indian J Gastroenterol Off J Indian Soc for important intellectual content; duplication of the colon and vermiform Gastroenterol. 2004;23(6):224–5. Muntadhar M. Isa, Maria Meildi, appendix in an adult patient. World J 13. Yousefzadeh DK, Bickers GH, Jackson JHJ, Muhammad Bayu Z. Hutagalung Gastroenterol. 2008;14(4):641. Available Benton C. Tubular colonic duplication- Final approval of the version to be from: http://dx.doi.org/10.3748/ review of 1876-1981 literature. Pediatr published: Muntadhar M. Isa, Amir wjg.14.641 Radiol. 1983;13(2):65–71. Thayeb 7. Bhatnagar V, Bhadoo D, Chand K, 14. Brunschwig A, Dargeon H, Russell W. Jana M, Gupta A. Colonic duplication: Duplication of the entire colon and lower REFERENCES Treatment by limited division of common ileum, with termination of one colon into wall. J Indian Assoc Pediatr Surg. a vaginal anus. Surgery. 1948;24(6):1010. 1. Puligandla PS, Nguyen LT, St-Vil D, 2015;20(3):146. Available from: http:// 15. Sarpel U, Le MN, Morotti RA, Dolgin SE. Flageole H, Bensoussan AL, Nguyen dx.doi.org/10.4103/0971-9261.159031 Complete colorectal duplication. J Am V-H, et al. Gastrointestinal duplications. 8. Castejón-Casado J, Muñoz Miguelsanz Coll Surg. 2005;200(2):304–5. J Pediatr Surg. 2003;38(5):740–4. MA, Diaz Em, Gomez Mg, Padilla 16. Yucesan S, Zorludemir U, Ocay I. Available from: http://dx.doi.org/10.1016/ Garcia MA, Valade Rf. Acute abdomen Complete duplication of the colon. J jpsu.2003.50197 secondary to complete tubular colonic Pediatr Surg. 1986;21:962–3. 2. Jellali MA, Mekki M, Saad J, Zrig A, Elanes duplication. J Indian Assoc Pediatr Surg. I, Mnari W, et al. Perinatally discovered 2014;19(3):166. Available from: http:// complete tubular colonic duplication dx.doi.org/10.4103/0971-9261.136477 associated with anal atresia. J Pediatr Surg. 9. Sengar M, Gupta CR, Jain V, Mohta A. 2012;47(6):e19–23. Available from: http:// Colorectal duplication with prostatorectal dx.doi.org/10.1016/j.jpedsurg.2012.01.082 fistulae. J Pediatr Surg. 2013;48(4):869– 3. Al-Shanafey S, Martin HCO, Bottger S. Colonic Duplication: Diagnostic

528 Published by Bali Medical Journal | Bali Medical Journal 2021; 10(2): 526-528 | doi: 10.15562/bmj.v10i2.2386