Laparoscopic repair of long-gap rectal atresia

Laparoscopic repair of long-gap rectal atresia

Journal of Pediatric Surgery Case Reports 42 (2019) 54–57 Contents lists available at ScienceDirect Journal of Pediatric Surgery Case Reports journa...

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Journal of Pediatric Surgery Case Reports 42 (2019) 54–57

Contents lists available at ScienceDirect

Journal of Pediatric Surgery Case Reports journal homepage: www.elsevier.com/locate/epsc

Laparoscopic repair of long-gap rectal atresia a

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b

Mollie Ahn , Claire B. Cummins , Sifrance Tran , Kanika A. Bowen-Jallow a b

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School of Medicine, University of Texas Medical Branch, 301 University Boulevard, Galveston, TX, 77555-1317, USA Department of Surgery, University of Texas Medical Branch, 301 University Boulevard, Galveston, TX, 77555-0353, USA

A R T I C LE I N FO

A B S T R A C T

Keywords: Rectal atresia Laparoscopic-assisted anorectal pull-through Minimally invasive surgery Anorectal malformation

Long-gap rectal atresia, as defined by a distance greater than 3 cm, is an uncommon type of a rare anorectal malformation (ARM). . We report the successful completion of a 3-stage procedure including colostomy and mucous fistula creation, laparoscopic-assisted transanal endorectal pull-through, and colostomy take-down in a patient with long-gap rectal atresia.

1. Introduction Rectal atresia is a rare anorectal malformation (ARM) which accounts for only 1–2% of all anorectal anomalies and is characterized by normal formation of the anus without fistulous communication between the anal canal and the distal colon [1]. Long-gap rectal atresia is defined by a distance greater than 3 cm and is even more uncommon. Various surgical treatment options are available for correction of rectal atresia, but laparoscopic approaches are infrequently reported. Rectal atresia is traditional repaired by a posterior sagittal anorectoplasty (PSARP), however in patients with a long gap defect, laparoscopy may be considered. The diversity of correctional procedures reflects the challenges in treatment of this anorectal anomaly. 2. Case description A 2-day old boy born at 34 weeks gestation was referred to pediatric surgery for abdominal distention and failure to pass meconium since birth. The perineal examination revealed a normally placed anus, but on physical examination a catheter was unable to be advanced past 2 cm at the anal verge. A barium enema was subsequently performed which demonstrated a blind ending pouch and a significantly distended sigmoid colon, confirming the diagnosis of rectal atresia (Fig. 1). The patient was then taken to the operating room (OR) for a colostomy and mucous fistula creation. After operation, the patient was able to tolerate oral feeds and was discharged from the neonatal intensive care unit 5 days later with plans for a fistulogram at 3 months of age. The patient was lost to follow-up for several months due to social circumstances, but at 10 months of age continuity of care was re-established. At that time a distal mucous fistulogram was performed to



better visualize the patient's ARM (Fig. 2). A balloon catheter was inserted into the mucus fistula and contrast was used to evaluate the length of the distal colon. Additionally, the end of the anal canal was identified by inserting a radiopaque syringe marker into the anus. Imaging demonstrated a long gap rectal atresia approximately 3.5 cm above the anal junction. Three weeks later, the patient was taken back to the OR for a laparoscopic-assisted, transanal endorectal pull-through for correction of rectal atresia. The laparoscopic procedure began by using a 3 mm sealer device for mobilization of the sigmoid colon to obtain the length required for a distal anastomosis. A transition zone was noted proximal to the levator muscles with an atretic segment of bowel that was in continuity from the mucous fistula to the rectal pouch (Fig. 3). After laparoscopic mobilization of the rectum was complete, attention turned to the anus for the pull-through portion of the procedure (Figs. 4 and 5). An end-to-end anorectal anastomosis was performed using the Soave technique (Fig. 6). The atretic segment was opened on the back table and demonstrated no patency, confirming complete atresia. The patient was discharged on postoperative day 1 without complications. Eight weeks later, the patient was taken back to the OR for colostomy take down. A rectal exam 3 weeks postoperatively demonstrated no stricture and no postoperative anal dilations were performed. At his 2 month postoperative visit, the patient was eating, drinking, and passing stool with no complications. 3. Discussion ARMs are rare anomalies with a reported incidence of 2.5 in 10,000 live births [2,3]. Among such malformations, rectal atresia has an even

Corresponding author. E-mail address: [email protected] (K.A. Bowen-Jallow).

https://doi.org/10.1016/j.epsc.2018.12.018 Received 3 October 2018; Received in revised form 24 December 2018; Accepted 24 December 2018 Available online 26 December 2018 2213-5766/ © 2019 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/BY-NC-ND/4.0/).

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Abbreviations ARM PSARP

Anorectal malformation Posterior sagittal anorectoplasty

lower occurrence, making up approximately 1–2% of all ARM cases [1,3]. The severity of the malformation is determined based on the distance between the proximal rectum and the distal anorectum. Rectal atresia with a short-gap (grade 1) is most common defect. More rare is a long-gap rectal atresia, (grade 2) as discussed in our case [3]. The embryogenic etiology of rectal atresia is uncertain but is largely believed to be linked to a vascular accident during the 13th to 14th week of gestation causing an obstruction of arterial blood supply through the superior rectal branch of the inferior mesenteric artery [3,4]. The anatomical anomaly is could be considered an acquired rather than a congenital phenomenon due to the lack of associated congenital anomalies as seen with other ARMs [4]. Patients with rectal atresia generally present with a history of inability to pass meconium as a newborn despite having a normal anus on physical exam. This is followed by delayed clinical signs of abdominal distension and bilious vomiting. Rectal atresia is frequently misdiagnosed at initial assessment because of a normal appearing anal location. Proper diagnosis of this ARM most often begins with preoperative imaging displaying distance between the colon and the rectal pouch. A barium enema is subsequently performed to establish the diagnosis and assist in surgical planning [3]. Other preoperative concerns involve the detection of associated malformations, and workup can include ultrasound, rectal exam and/or abdominal magnetic resonance imaging to rule out a pre-sacral mass. In our patient, a rectal exam and retroperitoneal ultrasound demonstrated no mass. The patient described in this case presented with classic clinical signs of rectal atresia as a newborn, including abdominal distension and failure to pass meconium. Initial imaging confirmed rectal atresia with a long-gap atretic segment spanning approximately 3 cm in length. Surgical intervention included a 3-stage colostomy and mucous fistula

Fig. 2. Mucous fistulogram demonstrating 3.5 cm gap between the rectal pouch and the distal portion of the mucous fistula.

Fig. 3. Laparoscopic view of the rectosigmoid junction (solid arrow) and the proximal atretic segment (outlined arrow).

Fig. 4. Blind ending rectal pouch 2–3 cm from the anal verge.

creation, laparoscopic-assisted transanal endorectal pull-through, and colostomy take-down. Laparoscopic-assisted repair of rectal atresia is infrequently reported, and this is the first case to describe its utility in long-gap rectal atresia. The most common procedure performed for rectal atresia is a PSARP

Fig. 1. Barium enema demonstrating dilated distal colon (solid arrow) and rectal pouch (outlined arrow). 55

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with end to end anastomosis following an initial sigmoid colostomy. This technique is widely used due to its reported success in establishing fecal continence with reduced complications [5–7]. The need for minimally invasive techniques has been debated as the PSARP has proven adequate in the most common type of rectal atresia (short-gap) where colon mobilization is minimal [3,8,9]. However, PSARP involves extensive dissection to the posterior anus, rectum, and sphincter muscles. Less invasive surgical interventions under laparoscopic assistance can be beneficial in the preservation of normal anorectal structures [9,10]. Surgical correction should aim to minimize any excess dissection to preserve a normal anal canal and viable sphincter muscles [3,5]. Transanal approaches have also been reported as a viable option for surgical intervention for its reduced operating time and reduced possibility for injury to nearby anatomical structures [7]. However, in the case of long-gap rectal atresia, the plane of dissection may be difficult to identify, increasing the chances of an inadvertent injury to the urethra or vagina [5]. The use of magnets is experimental, but potentially suitable for cases with a short-gap rectal atresia with a web or membrane, but is unlikely to be successful in cases of long-gap rectal atresia [5,11]. Laparoscopic-assisted anorectal pull-through procedures were first introduced in 2000, and have been gradually gaining in popularity [12]. Laparoscopy has been frequently used in other cases of ARMs because of its less invasive nature leading to superior cosmetic results, faster return of bowel function, and decreased hospital length of stay when compared to open abdominal procedures [6,8,9]. Laparoscopic procedures are particularly helpful for cases of high ARMs, due to better visualization of the rectal fistula, gynecologic anatomy, and accurate placement of the bowel through the levator ani and sphincteric complexes [13]. The laparoscopic-assisted transanal approach has been documented in cases of short-gap rectal atresia with good outcomes [10], but not in a case of long-gap rectal atresia. As no other minimally invasive procedure has been reported as an adequate method of correction of long-gap rectal atresia, this case illustrates its utility. In this case, a laparoscopic approach was appropriate because it was not known whether a transanal approach was feasible. For our patient, continuity of the bowel was demonstrated upon operation. Had this information been available preoperatively, a transanal approach could have been utilized. In our case laparoscopic-assisted transanal approach

Fig. 6. Completed anorectal anastomosis.

for the treatment of long-gap rectal atresia was a successful alternative to PSARP and transanal procedures. Patient consent Consent to publish the case report was not obtained. This report does not contain any personal information that could lead to the identification of the patient. Funding No funding or grant support. Authorship All authors attest that they meet the current ICMJE criteria for Authorship. Conflict of interest The following authors have no financial disclosures: MA, CBC, ST, KABJ. Appendix A. Supplementary data Supplementary data to this article can be found online at https:// doi.org/10.1016/j.epsc.2018.12.018. References [1] Laamrani FZ, Dafiri R. Rectal atresia: a rare cause of failure to pass meconium. Pan Afr Med J 2014;19:198. [2] Lupo PJ, Isenburg JL, Salemi JL, et al. Population-based birth defects data in the United States, 2010-2014: a focus on gastrointestinal defects. Birth Defects Res 2017;109(18):1504–14. [3] Holschneider A. Anorectal malformations in children: embryology, diagnosis, surgical treatment, follow-up. Berlin: Springer; 2006. [4] Festen C, Severijnen RS, van der Staak FH, Rien PN. Rectal atresia: pathogenesis and operative treatment. Pediatr Surg Int 1996;11(8):559–61. [5] Lane VA, Wood RJ, Reck C, Skerritt C, Levitt MA. Rectal atresia and anal stenosis: the difference in the operative technique for these two distinct congenital anorectal malformations. Tech Coloproctol 2016;20(4):249–54. [6] Hamrick M, Eradi B, Bischoff A, Louden E, Pena A, Levitt M. Rectal atresia and stenosis: unique anorectal malformations. J Pediatr Surg 2012;47(6):1280–4. [7] Hamzaoui M, Ghribi A, Makni W, Sghairoun N, Gasmi M. Rectal and sigmoid atresia: transanal approach. J Pediatr Surg 2012;47(6):e41–4. [8] Bischoff A, Martinez-Leo B, Pena A. Laparoscopic approach in the management of anorectal malformations. Pediatr Surg Int 2015;31(5):431–7. [9] Han Y, Xia Z, Guo S, Yu X, Li Z. Laparoscopically Assisted Anorectal Pull-Through versus Posterior Sagittal Anorectoplasty for High and Intermediate Anorectal Malformations: a Systematic Review and Meta-Analysis. PLoS One 2017;12(1):e0170421. [10] Nguyen TL, Pham DH. Laparoscopic and transanal approach for rectal atresia: a novel alternative. J Pediatr Surg 2007;42(11):E25–7. [11] Russell KW, Rollins MD, Feola GP, Scaife ER. Magnamosis: a novel technique for the management of rectal atresia. BMJ Case Rep 2014;2014.

Fig. 5. Pull through demonstrating the rectal pouch (dotted arrow), atretic segment (outlined arrow), and distal mucous fistula. 56

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[12] Georgeson KE, Inge TH, Albanese CT. Laparoscopically assisted anorectal pullthrough for high imperforate anus–a new technique. J Pediatr Surg 2000;35(6):927–30. discussion 930-921.

[13] Al-Hozaim O, Al-Maary J, AlQahtani A, Zamakhshary M. Laparoscopic-assisted anorectal pull-through for anorectal malformations: a systematic review and the need for standardization of outcome reporting. J Pediatr Surg 2010;45(7):1500–4.

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