ORIGINAL RESEARCH International Journal of Surgery 9 (2011) 652e654
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Original research
Swenson’s pull-through in older children and adults: Peculiar peri-operative challenges of surgery A.O. Ademuyiwaa, *, C.O. Bodea, O.A. Lawalb, J. Seyi-Olajidea a b
Paediatric Surgery Unit, Department of Surgery, College of Medicine, University of Lagos, PMB 12003 Idi Araba, Lagos, Nigeria General Surgery Unit, Dept of Surgery, College of Medicine, University of Lagos, PMB 12003 Idi Araba, Lagos, Nigeria
a r t i c l e i n f o
a b s t r a c t
Article history: Received 18 July 2011 Received in revised form 10 August 2011 Accepted 10 August 2011 Available online 16 September 2011
Introduction: The definitive treatment of Hirschsprung’s disease is removal of the aganglionic bowel by a pull-through surgery. In most cases this surgery is performed in infancy or the neonatal period as presentation in older children and adulthood is rare. Nevertheless, pull-through in this age group may be necessary and present peculiar challenges. Materials/methods: A prospective study of patients above 5 years with Hirschsprung’s disease who presented at LUTH, Lagos between January 2007 and July 2010. Results: There were fourteen patients (10 males and 4 females). The median age was 9 years (range 5 e31 years). All the patients presented with constipation and abdominal distension. Seven patients presented with intestinal obstruction necessitating colostomy. Thirteen patients had short segment Hirschsprung’s disease limited to the rectosigmoid. The only exception had long segment disease with the transition zone located at the transverse colon. Ten patients (71.4%) had colostomy before definitive pull-through while four patients (28.6%) had primary pull-through procedure without a colostomy. The definitive pull-through procedure done in all the patients was Swenson’s abdomino-perineal pull-through and all the patients had a 2-layered colo-anal anastomoses. Due to the rigid pelvis in these patients, as well as possible scarring, simple blunt dissection was difficult in these cases. The median length of follow up was 11 months (range 6e28 months). There was one case (7.1%) of mortality. Conclusion: Older patients with Hirschsprung’s disease present with intestinal obstruction and poor nutritional status that may necessitate preoperative colostomy. Swenson’s pull-through in them poses peculiar challenges of mobilization of bowel and achieving a reliable colo-anal anastomosis, however, outcome is comparable with surgery in younger children if these challenges are overcome. Ó 2011 Surgical Associates Ltd. Published by Elsevier Ltd. All rights reserved.
Keywords: Swenson’s pull-through Adult Hirschsprung’s disease Surgical challenges
1. Introduction Hirschsprung’s disease is the congenital absence of parasympathetic ganglion cells in the submucous and myenteric plexuses of the intestine usually affecting the rectosigmoid portion of the large bowel.1,2 The definitive treatment is a pull-through procedure which can be achieved through a combined abdominal and perineal approach or a transanal approach.3e7 Three types of pull-through procedures have achieved worldwide popularity as described by Swenson, Duhamel and Soave.3e5 The outcomes from these techniques have been comparable in terms of post-operative function, complications, morbidity and mortality.8 The choice of
* Corresponding author. E-mail address:
[email protected] (A.O. Ademuyiwa).
technique is thus dependent on Surgeon’s preference and familiarity and proficiency with any of the techniques.9 At our centre, the Swenson’s procedure is performed. In developed countries, between 80 and 90% of cases of Hirschsprung’s disease are diagnosed in the neonatal period and pull-through procedure is performed during the neonatal period or infancy.10 However, in most centres in developing countries, there is often delay in diagnosis and older children and adults sometimes present with the condition.11 The Swenson’s procedure has been reported to have higher post-operative complications.12 However, Swenson13 in a 50 year review of the procedure gave excellent outcome in patients followed up for variable periods. In addition to the challenges of performing the procedure in children, the authors identified other peri-operative and intra-operative challenges in older children and adults. This communication aims to highlight some of these challenges and how to overcome them.
1743-9191/$ e see front matter Ó 2011 Surgical Associates Ltd. Published by Elsevier Ltd. All rights reserved. doi:10.1016/j.ijsu.2011.08.006
ORIGINAL RESEARCH A.O. Ademuyiwa et al. / International Journal of Surgery 9 (2011) 652e654
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4. Discussion
2. Materials and methods This is a prospective review of data collected between January 2007 and July 2010 on children above 5 years of age diagnosed with Hirschsprung’s disease at Lagos University Teaching Hospital. Demographic data including age and gender, the method of diagnosis, intra-operative localization of the transition zone, method of pull-through, type of colo-anal anastomosis and final outcome were collated. Details of the surgical procedure as documented in the operation notes with respect to technique of surgery and length of follow up were also reviewed. Children below the age of 5 years and those who required re-operation after previous pull-through procedures were excluded.
3. Results There were fourteen patients (10 males and 4 females). The median age was 9 years (range 5e31 years). All the patients had rectosigmoid disease except one who had long segment disease with the transition zone located at the junction of the proximal and middle thirds of the transverse colon. All the patients presented with constipation and abdominal distension. Seven patients presented with intestinal obstruction. They were referred from General Practitioners who had administered parenteral antibiotics and intravenous fluids. They had colostomy to relieve the obstruction in our centre. Three other patients had colostomy on account of severe colonic dilatation on barium enema and poor nutritional status (<75% of expected weight). Colostomy was performed in the latter group to allow for nutritional rehabilitation and allow faecal decompression. One (7.1%) patient each was diagnosed by either barium enema only or rectal biopsy only. All the other 13 (92.9%) patients had both investigations prior to surgery. Ten patients (71.4%) had colostomy before definitive pull-through while four patients (28.6%) had primary pull-through procedure without a colostomy. Due to the rigid pelvis in these patients as well as possible scarring, simple blunt dissection was difficult and the dissection could not get to the anal verge as in neonates and infants. The definitive pull-through procedure done in all the patients was Swenson’s abdomino-perineal pull-through and all the patients had a 2-layered colo-anal anastomoses. The median length of follow up was 11 months (range 6e28 months). All the patients had post-operative anal dilatation protocols commenced from 14th day post surgery for variable period of 3e6 months. There was no case of post-operative enterocolitis or incontinence. Three patients had constipation but this was managed conservatively with dietary modification with marked improvement. There was one case of mortality (in the patient with long segment Hirschsprung’s disease) who died of sepsis following peritonitis from posterior colonic perforation proximal to an area of anastomotic stenosis. Table 1 summarizes information about the patients.
Hirschsprung’s disease can present in the neonatal period, infancy, childhood or adulthood.14 With improved diagnostic methods, presentation in later childhood and adulthood has become less common. More than 90% of patients are diagnosed in the neonatal period in developed countries.10 The protocol for the management of this condition in neonates and infants is well established.7,10 However, surgery in older children and adults presents peculiar peri-operative challenges that must be kept in mind to ensure success. We performed Swenson’s pull-through for all our patients as this is the preference in our centre. The critical challenges in the procedure are discussed. 5. Preoperative colostomy One of the decisions that must be made before definitive pullthrough is whether or not to perform a colostomy. Due to prolonged faecal retention, there is often a markedly dilated colon proximal to the transition zone. This poses an intra-operative challenge to achieving a colo-anal anastomosis. When there is marked disproportion between the area of colonic dilatation at the proposed level for pull-through greater than three times the diameter of the anus, a disproportionate anastomosis results and in such patients a preoperative colostomy is justified to relieve the faecal retention. In our patients who required preoperative colostomy, definitive pull-through surgery was done 6 months after colostomy by which time colonic size had usually returned to normal. Ten of our patients needed colostomy for this reason; however, for the remaining four patients including a 31 year-old man, a primary pull-through was achieved without colostomy. In this series, colostomy was performed to relieve intestinal obstruction in 50% of the patients. Due to chronic constipation in these older patients, many of them have a huge load of faeces in the colon and rectum known as scybala which can become calcified over time and predispose to faecal impaction a cause of luminal intestinal obstruction. 6. Mobilization of the aganglionic segment Although mobilization of the aganglionic segment can be performed up to the anal verge in younger children and infants, this is often difficult to achieve in older children and adults due to their rigid pelvis. This is most commonly seen in males. In young infants, after creating a plane at the peritoneal reflection, blunt dissection can be performed with the fingers right on the bowel wall.
Table 1 Clinical characteristics of patients. Age
Sex
Diagnosis (type)
Contrast enema
Rectal biopsy
Preop colostomy
Rectal tubea
Outcome
31 yrs 7 yrs 9 yrs 9 yrs 5 yrs 12 yrs 11 yrs 5 yrs 6 yrs 8 yrs 5 yrs 13 yrs 16 yrs 23 yrs
M M M M F M M M M M F M F F
Rectosigmoid Rectosigmoid Rectosigmoid Rectosigmoid Transverse Rectosigmoid Rectosigmoid Rectosigmoid Rectosigmoid Rectosigmoid Rectosigmoid Rectosigmoid Rectosigmoid Rectosigmoid
Yes Yes Yes Yes Yes Yes Yes No Yes Yes Yes Yes Yes Yes
Yes Yes Yes Yes Yes Yes Yes Yes No Yes Yes Yes Yes Yes
No No Yes Yes Yes No No Yes Yes Yes Yes No Yes Yes
5cc 2cc 2cc 2cc 2cc 2cc 2cc 2cc 2cc 2cc 2cc 2cc 2cc Nil
a/w, constipation a/w a/w a/w Died, peritonitis a/w a/w; constipation a/w a/w a/w a/w a/w a/w a/w; constipation
a
Rectal tube: the patients had trans-anastomotic tubes improvised with 2cc or 5cc syringe (cut at the end where the needle is attached) without its plunger.
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However, in older children and adults, we achieve mobilization up to 2 or 3 cm from the anal verge by carefully creating and maintaining a plane just over the rectal wall at the level of the peritoneal reflection and by sharp and blunt dissection using long Mayo’s scissors and pledgets on long Kocher’s forceps. After adequate mobilization, the rectum was then everted through the anus to prepare for the anastomosis in the usual manner.3
Author contribution
7. Colo-anal anastomosis
References
Bowel anastomosis can be achieved either as a single layered or through a 2-layered procedure.15,16 While recent studies suggest that single layered method could be used to anastomose any part of the bowel, the time honoured 2-layered anastomosis was used in all our patients. This method of anastomosis achieves haemostasis faster and we have not recorded any case of anastomotic leakage with the method.
1. Belknap WM. The pathogenesis of Hirschsprung disease. Curr Opin Gastroenterol 2002 Jan;18(1):74e81. 2. Martucciello G, Pini Prato A, Puri P, Holschneider AM, Meier-Ruge W, Jasonni V, et al. Controversies concerning diagnostic guidelines for anomalies of the enteric nervous system: a report from the fourth International Symposium on Hirschsprung’s disease and related neurocristopathies. J Pediatr Surg 2005 Oct;40(10):1527e31. 3. Swenson O, Bill AH. Resection of the rectum and rectosigmoid with preservation of the sphincter for benign spastic lesions producing megacolon. Surgery 1948;24:212e20. 4. Duhamel B. A new operation for the treatment of Hirschsprung’s disease. Arch Dis Child 1960;35:38e9. 5. Soave F. A new surgical technique for the treatment of Hirschsprung’s disease. Surgery 1964;56:1007e14. 6. Weidner BC, Waldhausen JH. Swenson revisited: a one-stage, transanal pull-through procedure for Hirschsprung’s disease. J Pediatr Surg 2003;38(8): 1208e11. 7. Dasgupta R, Langer JC. Transanal pull-through for Hirschsprung disease. Semin Pediatr Surg 2005;14(1):64e71. 8. Niramis R, Watanatittan S, Anuntkosol M, Buranakijcharoen V, Rattanasuwan T, Tongsin A, et al. Quality of life of patients with Hirschsprung’s disease at 5e20 years post pull-through operations. Eur J Pediatr Surg 2008;18(1): 38e43. 9. Kleinhaus S, Boley SJ, Sheran M, Sieber WK. Hirschsprung’s disease, a survey of the members of the surgical section of the American Academy of Pediatrics. J Pediatr Surg 1979;14:588e97. 10. Cass DT. Neonatal one stage repair of Hirschsprung’s disease. Pediatr Surg Int 1990;5:341. 11. Ekenze SO, Ngaikedi C, Obasi AA. Problems and outcome of Hirschsprung’s disease presenting after 1 year of age in a developing country. World J Surg 2011;35(1):22e6. 12. Moore SW, Albertyn R, Cywes S. Clinical outcome and long-term quality of life after surgical correction of Hirschsprung’s disease. J Pediatr Surg 1996;31(11):1496e502. 13. Swenson O. Early history of the therapy of hirschsprung’s disease: facts and personal observations over 50 years. J Pediatr Surg. 1996;31(8):1003e8. 14. Miyamoto M, Egami K, Maeda S, Ohkawa K, Tanaka N, Uchida E, et al. Hirschsprung’s disease in adults: report of a case and review of the literature. J Nippon Med Sch 2005;72:113e20. 15. Puri P. Hirschsprung’s disease. In: Oldham KT, Colombani PM, Foglia RP, editors. Surgery of infants and children: scientific principles and practice. Philadelphia, New York: Lippincott-Raven; 1996. p. 1277e99. 16. Murugadas C, Raghupathy R, Kumaran V, Rajamani G, Krishnamoorthy P, Mohan NV, et al. Neonatal transanal pull through for Hirschsprung’s disease. J Indian Assoc Paediatr Surg 2001;6:69e71. 17. Bandré E, Kaboré RA, Ouedraogo I, Soré O, Tapsoba T, Bambara C, et al. Hirschsprung’s disease: management problem in a developing country. Afr J Paediatr Surg 2010;7(3):166e8. 18. Ademuyiwa AO, Sowande OA, Ijaduola TK, Adejuyigbe O. Determinants of mortality in neonatal intestinal obstruction in Ile Ife, Nigeria. Afr J Paediatr Surg 2009;6(1):11e3.
8. Outcome The mortality rate in this series was 7.1% a figure much better than quoted figures of mortality in the neonatal period within the West African sub region which is between 16% and 33%.17,18 The single patient who died was a 5 year-old girl who had peritonitis from perforation of the pulled-through segment following a staged procedure. In retrospect, it is possible she might have benefitted from a protective colostomy but this was not thought to be indicated at the time of surgery. Post mortem studies revealed that the anastomotic site was intact. There were three patients (21.4%) who presented in the post-operative period with constipation but these improved with dietary modification. None of the patients had enterocolitis or incontinence in our series. These results suggest that Swenson’s pullthrough procedure has good outcome in older children and adults. In conclusion, older patients with Hirschsprung’s disease present with intestinal obstruction and poor nutritional status that may necessitate preoperative colostomy. Swenson’s pull-through in them poses peculiar challenges of mobilization of bowel and achieving a reliable colo-anal anastomosis. Colostomy prior to pullthrough may be necessary to reduce the colo-anal disproportion. However, outcome is comparable with surgery in younger children if these challenges are overcome. Conflict of interest None declared. Funding None declared. Ethical approval Ethical approval by hospital ethics board given.
AOA: Study design, data collection, analysis and write up. COB; Study design, intellectual content and final write up. AOL: Data collection and collation and final write up. JO: Data collation, analysis and write up.