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Transanal-only Swenson-like pull-through for late diagnosed Hirschsprung disease

Transanal-only Swenson-like pull-through for late diagnosed Hirschsprung disease Hirschsprung disease (HD) is an obstructive colonic process usually diagnosed in the neonatal period. A small subset of cases are diagnosed late, present with severe constipation without enterocolitis and have low rectosigmoid disease. A transanal- only pull-through is a well-described approach but in the newborn period risks a situation whereby the transition zone is higher than the sigmoid. We present our experience with the unique patient population of older HD patients in whom the transition zone was reliably reachable via a single-stage transanal approach, performed in prone position. Patients between 2 and 6 years of age with a rectal or sigmoid transition zone and minimal proximal colonic dilation can undergo a primary transanal pull-through surgical approach. INTRODUCTION CASE REPORT Hirschsprung disease (HD) is an obstructive colonic process usu- A retrospective review was performed to identify all children ally diagnosed in the neonatal period. A small subset of cases are who underwent a transanal-only pull-through following late diagnosed late, present with a prolonged history of abdominal diagnosis of Hirschsprung disease between 2014 and 2019. A late distension and constipation prior to diagnosis and have not had diagnosis of HD was defined in any child with HD and older than episodes of enterocolitis. This clinical scenario points to low 2 years of age at the time of diagnosis. Demographics and opera- (rectosigmoid) disease and is clinically a milder form of HD. A tive details including enterocolitis episodes, fecal continence for transanal-only pull-through is a well-described approach but in those > 4 years of age were evaluated. the typical HD patient in the newborn period risks finding a tran- Of 54 primary cases of HD from our center, 19 (35%) were sition zone higher than the sigmoid, which is why a laparoscopic older than 2 years of age at diagnosis of HD, and we felt that 4 or umbilical biopsy should be the first part of the procedure. We (7%) qualified for a transanal-only primary. Diagnoses occurred present our experience with the unique patient population of at 4.4 years of age (1.6–6.1 years). The mean symptom was con- older HD in whom the transition zone was reliably reachable via stipation in all patients, and none experienced any episodes of a single-stage transanal-only approach and can be performed in enterocolitis (Table 1). All patients underwent a contrast enema prone position. and a full thickness rectal biopsy. The contrast enema in each Received: September 23, 2019. Accepted: October 19, 2019 Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author(s) 2019. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/ licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com 1 Downloaded from https://academic.oup.com/jscr/article-abstract/2019/12/rjz341/5678064 by DeepDyve user on 25 March 2020 2 R.M. Rentea et al. Table 1. Demographics Characteristic measured (n =4) Age (years, min–max) 4.4 (1.6–6.1) Gender Male = 2, female = 2 First symptom—constipation 4 (100%) Full-thickness biopsy 4 (100%) Contrast enema 4 (100%) Comorbidities 2 (50%) Operative details Age at OR (years) 4.7 (2.0–6.4) Length of case (hours) 3 (2–4) Level—rectosigmoid 4 (100%) Preoperative Length of Stay (LOS) (day) 1 Postoperative Preoperative Length of Stay (LOS) (day) 5 (4–6) Outcomes Readmissions for HAEC 0 Constipation 2 (50%) Night time fecal soiling postop 2 (50%) Laxative requirement Senna 15 mg + fiber 3 (75%) Miralax17g 1(25%) patient identified a low transition zone in the distal rectosigmoid faces include the approach to diversion in the patient population colon and no dilation of the more proximal colon (above the sig- as stomas are prone to prolapse, contain large thick bowel to moid). All patients were able to decompress their colon through decompress and mature and bleeding of the ostomy [5, 6]. increased use of laxatives prior to surgery. There is a subset of patients in whom primary transanal There were 15 patients (28% of all patients and 79% over age pull-through in those >2yearsofage at thetimeofsurgery is 2 years) who did not undergo transanal-only pull-through. These possible. While a previous case series reported that 18.8% of their patients underwent a primary pull-through using laparoscopy, late-diagnosed HD patients underwent transanal primary pull- and 11(58%) had a diverting colostomy/ileostomy in place at the through, these authors did not describe outcomes or reasons for time of pull-through. In all patients, the length of proximally choosing the operative approach [7]. In case series, the character- dilated colon was significant and involved mobilization of the istics of patients who were able to undergo primary transanal- descending colon. only pull-through include overall earlier age (2–6 years) vs. those diagnosed later. On contrast enema, they exhibited a limited degree of megacolon (Figs 1 and 2). Proximal colonic dilation was DISCUSSION absent in all four patients that we felt were good candidates for a transanal-only approach. However, proximal colonic dilation Late-diagnosed HD occurs in children and young adults and is defined variably as cases which are diagnosed more than 30 days (proximal of the sigmoid) was visualized in 15 patients, and therefore, a transanal-only approach was not offered. The ability after birth all the way to adulthood. Children who present with late diagnosed HD more often present with constipation as to identify a clear transition zone low in the rectosigmoid was also observed in each patient. Finally, the ability to empty the their primary symptom and almost never have enterocolitis [1]. colon preoperatively whether through laxatives or rectal irriga- The diagnosis is often challenging and delayed. A full-thickness tions was also present. rectal biopsy is diagnostic in 100% of cases, while an absent Prior to beginning the pull-through, the colon should be rectoanal inhibitory reflex (RAIR) was only absent on anorectal manometry (AMAN) 63.8% of the time- the rest were felt to be irrigated to make sure that any retained fecal matter has been cleared. Careful assessment of the transition zone and confirma- non-diagnostic according to the literature [2]. A meta-analysis of HD presentation after childhood demonstrated a transition zone tion of minimal proximal colonic dilatation allows for prone-only positioning and a transanal-only approach (Fig. 1). The Lone Star limited to the rectum (79.6%), rectosigmoid (12.3%), descending colon (0.8%) and total colonic (0.4%) [3]. These findings imply that retractor and full-thickness dissection begin 1.0 cm proximal to the dentate line in the Swenson plane. Biopsies should be taken children diagnosed later often have a milder form and shorter segment of disease [1] as they usually do not present with ente- in a single line so as not to lose orientation [8] and 10–15 cm past the positive biopsy for ganglion cells to assure that normal rocolitis but rather constipation [3]. The technical implications of this for the surgeon are a better prediction of the transition caliber colon is reached before performing the anastomosis. Challenges with the transanal-only approach occur when there zone and the need to manage colonic dilatation. is a large amount of proximal colonic dilation, and therefore, in Multiple operative approaches have been performed includ- this instance, we recommend a laparoscopic approach for intra- ing a Soave, Swenson, Duhamel, myectomy and low anterior operative mobilization of the descending and proximal colonic resection with myectomy or colectomy [4]. These surgeries include adjuncts of laparoscopy, laparotomy and transanal mobilization off the left retroperitoneum [9].If at the time of anastomosis of the dilated ganglionated bowel, anal caliber size variations of these approaches. Diversion with colostomy (end, loop or blowhole) as well as a ileostomy have also been described discrepancy is encountered a trick can be employed by placing four quadrant sutures and then bisecting each quadrant again as temporizing measures to help a proximally dilated colon decompress [5, 6]. Some of the greatest challenges a surgeon and again with interrupted sutures [10]. Downloaded from https://academic.oup.com/jscr/article-abstract/2019/12/rjz341/5678064 by DeepDyve user on 25 March 2020 Transanal-only Swenson-like pull-through 3 Figure 1: Representative contrast enema study. Note the lack of dilated proximal colon and defined transition zone in the low rectosigmoid colon. (Candidate for transanal only). Figure 2: Representative contrast enema study of an older child with a large amount of proximal colonic dilation of the sigmoid and descending colon, seen here across multiple views during contrast enema (Not a candidate for transanal only). Downloaded from https://academic.oup.com/jscr/article-abstract/2019/12/rjz341/5678064 by DeepDyve user on 25 March 2020 4 R.M. Rentea et al. Single-stage transanal-only pull-through performed in the 4. Wheatley MJ, Wesley JR, Coran AG, Polley TZ. Hirschsprung’s prone position is a safe and effective option in a very small group disease in adolescents and adults. Dis Colon Rectum of children diagnosed with late-diagnosed HD with transition 1990;33:622–9. zone in the rectum or mid sigmoid with minimal proximal 5. Ekenze SO, Ngaikedi C, Obasi AA. Problems and out- colonic dilation. The advantages include single stage, ability to come of Hirschsprung’s disease presenting after 1 year stay out of the abdomen and surgeon visibility. of age in a developing country. World J Surg 2011;35: 22–6. 6. Ricketts RR, Pettitt BJ. Management of Hirschsprung’s dis- CONFLICT OF INTEREST STATEMENT ease in adolescents. Am Surg 1989;55:219–25. 7. Sharma S, Gupta DK. Hirschsprung’s disease presenting All authors have no disclosures or conflicts of interest. beyond infancy: Surgical options and postoperative out- come. Pediatr Surg Int 2012;28:5–8. REFERENCES 8. Langer JC. Laparoscopic and transanal pull-through 1. Hackam DJ, Reblock KK, Redlinger RE, Barksdale EM. Diagno- for Hirschsprung disease. Semin Pediatr Surg 2012;21: sis and outcome of Hirschsprung’s disease: Does age really 283–90. matter? Pediatr Surg Int 2004;20:319–22 9. De La Torre L, Langer JC. Transanal endorectal pull-through 2. Vorobyov GI, Achkasov SI, Biryukov OM. Clinical features’ for Hirschsprung disease: Technique, controversies, pearls, diagnostics and treatment of Hirschsprung’s disease in pitfalls, and an organized approach to the management adults. Colorectal Dis 2010;12:1242–8. of postoperative obstructive symptoms. Semin Pediatr Surg 3. Doodnath R, Puri P. A systematic review and meta-analysis 2010;19:96–106. of Hirschsprung’s disease presenting after childhood. Pediatr 10. Ouladsaiad M. How to manage a late diagnosed Surg Int 2010;26:1107–10. Hirschsprung’s disease. Afr J Paediatr Surg 2016;13:82–7. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Journal of Surgical Case Reports Oxford University Press

Transanal-only Swenson-like pull-through for late diagnosed Hirschsprung disease

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Oxford University Press
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Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author(s) 2019.
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2042-8812
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Abstract

Hirschsprung disease (HD) is an obstructive colonic process usually diagnosed in the neonatal period. A small subset of cases are diagnosed late, present with severe constipation without enterocolitis and have low rectosigmoid disease. A transanal- only pull-through is a well-described approach but in the newborn period risks a situation whereby the transition zone is higher than the sigmoid. We present our experience with the unique patient population of older HD patients in whom the transition zone was reliably reachable via a single-stage transanal approach, performed in prone position. Patients between 2 and 6 years of age with a rectal or sigmoid transition zone and minimal proximal colonic dilation can undergo a primary transanal pull-through surgical approach. INTRODUCTION CASE REPORT Hirschsprung disease (HD) is an obstructive colonic process usu- A retrospective review was performed to identify all children ally diagnosed in the neonatal period. A small subset of cases are who underwent a transanal-only pull-through following late diagnosed late, present with a prolonged history of abdominal diagnosis of Hirschsprung disease between 2014 and 2019. A late distension and constipation prior to diagnosis and have not had diagnosis of HD was defined in any child with HD and older than episodes of enterocolitis. This clinical scenario points to low 2 years of age at the time of diagnosis. Demographics and opera- (rectosigmoid) disease and is clinically a milder form of HD. A tive details including enterocolitis episodes, fecal continence for transanal-only pull-through is a well-described approach but in those > 4 years of age were evaluated. the typical HD patient in the newborn period risks finding a tran- Of 54 primary cases of HD from our center, 19 (35%) were sition zone higher than the sigmoid, which is why a laparoscopic older than 2 years of age at diagnosis of HD, and we felt that 4 or umbilical biopsy should be the first part of the procedure. We (7%) qualified for a transanal-only primary. Diagnoses occurred present our experience with the unique patient population of at 4.4 years of age (1.6–6.1 years). The mean symptom was con- older HD in whom the transition zone was reliably reachable via stipation in all patients, and none experienced any episodes of a single-stage transanal-only approach and can be performed in enterocolitis (Table 1). All patients underwent a contrast enema prone position. and a full thickness rectal biopsy. The contrast enema in each Received: September 23, 2019. Accepted: October 19, 2019 Published by Oxford University Press and JSCR Publishing Ltd. All rights reserved. © The Author(s) 2019. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/ licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com 1 Downloaded from https://academic.oup.com/jscr/article-abstract/2019/12/rjz341/5678064 by DeepDyve user on 25 March 2020 2 R.M. Rentea et al. Table 1. Demographics Characteristic measured (n =4) Age (years, min–max) 4.4 (1.6–6.1) Gender Male = 2, female = 2 First symptom—constipation 4 (100%) Full-thickness biopsy 4 (100%) Contrast enema 4 (100%) Comorbidities 2 (50%) Operative details Age at OR (years) 4.7 (2.0–6.4) Length of case (hours) 3 (2–4) Level—rectosigmoid 4 (100%) Preoperative Length of Stay (LOS) (day) 1 Postoperative Preoperative Length of Stay (LOS) (day) 5 (4–6) Outcomes Readmissions for HAEC 0 Constipation 2 (50%) Night time fecal soiling postop 2 (50%) Laxative requirement Senna 15 mg + fiber 3 (75%) Miralax17g 1(25%) patient identified a low transition zone in the distal rectosigmoid faces include the approach to diversion in the patient population colon and no dilation of the more proximal colon (above the sig- as stomas are prone to prolapse, contain large thick bowel to moid). All patients were able to decompress their colon through decompress and mature and bleeding of the ostomy [5, 6]. increased use of laxatives prior to surgery. There is a subset of patients in whom primary transanal There were 15 patients (28% of all patients and 79% over age pull-through in those >2yearsofage at thetimeofsurgery is 2 years) who did not undergo transanal-only pull-through. These possible. While a previous case series reported that 18.8% of their patients underwent a primary pull-through using laparoscopy, late-diagnosed HD patients underwent transanal primary pull- and 11(58%) had a diverting colostomy/ileostomy in place at the through, these authors did not describe outcomes or reasons for time of pull-through. In all patients, the length of proximally choosing the operative approach [7]. In case series, the character- dilated colon was significant and involved mobilization of the istics of patients who were able to undergo primary transanal- descending colon. only pull-through include overall earlier age (2–6 years) vs. those diagnosed later. On contrast enema, they exhibited a limited degree of megacolon (Figs 1 and 2). Proximal colonic dilation was DISCUSSION absent in all four patients that we felt were good candidates for a transanal-only approach. However, proximal colonic dilation Late-diagnosed HD occurs in children and young adults and is defined variably as cases which are diagnosed more than 30 days (proximal of the sigmoid) was visualized in 15 patients, and therefore, a transanal-only approach was not offered. The ability after birth all the way to adulthood. Children who present with late diagnosed HD more often present with constipation as to identify a clear transition zone low in the rectosigmoid was also observed in each patient. Finally, the ability to empty the their primary symptom and almost never have enterocolitis [1]. colon preoperatively whether through laxatives or rectal irriga- The diagnosis is often challenging and delayed. A full-thickness tions was also present. rectal biopsy is diagnostic in 100% of cases, while an absent Prior to beginning the pull-through, the colon should be rectoanal inhibitory reflex (RAIR) was only absent on anorectal manometry (AMAN) 63.8% of the time- the rest were felt to be irrigated to make sure that any retained fecal matter has been cleared. Careful assessment of the transition zone and confirma- non-diagnostic according to the literature [2]. A meta-analysis of HD presentation after childhood demonstrated a transition zone tion of minimal proximal colonic dilatation allows for prone-only positioning and a transanal-only approach (Fig. 1). The Lone Star limited to the rectum (79.6%), rectosigmoid (12.3%), descending colon (0.8%) and total colonic (0.4%) [3]. These findings imply that retractor and full-thickness dissection begin 1.0 cm proximal to the dentate line in the Swenson plane. Biopsies should be taken children diagnosed later often have a milder form and shorter segment of disease [1] as they usually do not present with ente- in a single line so as not to lose orientation [8] and 10–15 cm past the positive biopsy for ganglion cells to assure that normal rocolitis but rather constipation [3]. The technical implications of this for the surgeon are a better prediction of the transition caliber colon is reached before performing the anastomosis. Challenges with the transanal-only approach occur when there zone and the need to manage colonic dilatation. is a large amount of proximal colonic dilation, and therefore, in Multiple operative approaches have been performed includ- this instance, we recommend a laparoscopic approach for intra- ing a Soave, Swenson, Duhamel, myectomy and low anterior operative mobilization of the descending and proximal colonic resection with myectomy or colectomy [4]. These surgeries include adjuncts of laparoscopy, laparotomy and transanal mobilization off the left retroperitoneum [9].If at the time of anastomosis of the dilated ganglionated bowel, anal caliber size variations of these approaches. Diversion with colostomy (end, loop or blowhole) as well as a ileostomy have also been described discrepancy is encountered a trick can be employed by placing four quadrant sutures and then bisecting each quadrant again as temporizing measures to help a proximally dilated colon decompress [5, 6]. Some of the greatest challenges a surgeon and again with interrupted sutures [10]. Downloaded from https://academic.oup.com/jscr/article-abstract/2019/12/rjz341/5678064 by DeepDyve user on 25 March 2020 Transanal-only Swenson-like pull-through 3 Figure 1: Representative contrast enema study. Note the lack of dilated proximal colon and defined transition zone in the low rectosigmoid colon. (Candidate for transanal only). Figure 2: Representative contrast enema study of an older child with a large amount of proximal colonic dilation of the sigmoid and descending colon, seen here across multiple views during contrast enema (Not a candidate for transanal only). Downloaded from https://academic.oup.com/jscr/article-abstract/2019/12/rjz341/5678064 by DeepDyve user on 25 March 2020 4 R.M. Rentea et al. Single-stage transanal-only pull-through performed in the 4. Wheatley MJ, Wesley JR, Coran AG, Polley TZ. Hirschsprung’s prone position is a safe and effective option in a very small group disease in adolescents and adults. Dis Colon Rectum of children diagnosed with late-diagnosed HD with transition 1990;33:622–9. zone in the rectum or mid sigmoid with minimal proximal 5. Ekenze SO, Ngaikedi C, Obasi AA. Problems and out- colonic dilation. The advantages include single stage, ability to come of Hirschsprung’s disease presenting after 1 year stay out of the abdomen and surgeon visibility. of age in a developing country. World J Surg 2011;35: 22–6. 6. Ricketts RR, Pettitt BJ. Management of Hirschsprung’s dis- CONFLICT OF INTEREST STATEMENT ease in adolescents. Am Surg 1989;55:219–25. 7. Sharma S, Gupta DK. Hirschsprung’s disease presenting All authors have no disclosures or conflicts of interest. beyond infancy: Surgical options and postoperative out- come. Pediatr Surg Int 2012;28:5–8. REFERENCES 8. Langer JC. Laparoscopic and transanal pull-through 1. Hackam DJ, Reblock KK, Redlinger RE, Barksdale EM. Diagno- for Hirschsprung disease. Semin Pediatr Surg 2012;21: sis and outcome of Hirschsprung’s disease: Does age really 283–90. matter? Pediatr Surg Int 2004;20:319–22 9. De La Torre L, Langer JC. Transanal endorectal pull-through 2. Vorobyov GI, Achkasov SI, Biryukov OM. Clinical features’ for Hirschsprung disease: Technique, controversies, pearls, diagnostics and treatment of Hirschsprung’s disease in pitfalls, and an organized approach to the management adults. Colorectal Dis 2010;12:1242–8. of postoperative obstructive symptoms. Semin Pediatr Surg 3. Doodnath R, Puri P. A systematic review and meta-analysis 2010;19:96–106. of Hirschsprung’s disease presenting after childhood. Pediatr 10. Ouladsaiad M. How to manage a late diagnosed Surg Int 2010;26:1107–10. Hirschsprung’s disease. Afr J Paediatr Surg 2016;13:82–7.

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Journal of Surgical Case Reports – Oxford University Press

Published: Dec 1, 2019

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