Hirschsprung’s disease and Rectal irrigation

Feasibility and efficacy of home rectal irrigation in neonates and earlyinfancy with Hirschsprung’s disease








Hirschsprung’s disease is one of the most common causes of intestinal obstruction in neonates, affecting up to approximately 1 in 5000 live births. The definitive treatment of Hirschsprung’s disease  is surgery where the aganglionic bowel segment is removed by a pull though procedure. The surgery is done in the neonatal or early infancy period either by a single stage procedure or by a multi stage procedure. Single stage pull through procedure (SSPT) is thought to be better than multi staged procedure , nevertheless SSPT in the neonatal period presents with the challenges of inaccurate diagnosis, post operative enterocolitis and delayed post operative recovery. This prospective observational study is designed to evaluate the feasibility and efficacy of home rectal irrigation in neonatal and early infancy after which a delayed and planned SSPT will be performed and outcomes will be assessed. This study will be carried out in the department of Paediatric and Neonatal surgery, Mymensingh Medical College Hospital,Mymensingh from January 2020 to April 2021 to find out about the feasibility and efficacy  of home rectal irrigation in neonates and early infancy with Hirschsprung’s disease. This study will shorten the hospital stay of the patients and will also be cost effective for the patients and will also reduce the need of a second surgery.

 










Key words: Hirschsprung’s disease, rectal irrigation, Single stage pull through procedure .


Hirschsprung’s disease (HSCR) is characterized by the absence of ganglion cells (aganglionosis) in the myenteric and submucosal plexuses of the distal gut, thus, resulting in persistent spasm in the affected bowel and functional intestinal obstruction [1]. This affects up to approximately 1 in 5000 live births globally but also a matter of concern for Bangladesh. 

 

HSCR is classified as rectosigmoid HSCR, long-segment HSCR, and total colonic HSCR based on the aganglionic segment, and definitive treatment is resecting the aganglionic portion of the colon and surgical pull-through. Over time, single-stage pull-through (SSPT) has become the most popular procedure for HSCR [2]; however, the best time to perform SSPT for a patient with HSCR has not been established. SSPT performed after the neonatal period compared to during the neonatal period for correction of HSCR has many limitations, such as lower diagnostic accuracy and higher rates of perianal excoriation, anastomotic strictures or leakage, postoperative enterocolitis, and post-operative incomplete continence [3].







 

The basic principle for the definitive surgical therapy is resection of the aganglionic bowel followed by a pull through of ganglionic bowel down to the anus[4]. In most cases, this surgery is performed in infancy or in the neonatal period as presentation in older children and adulthood is uncommon. Soave transanal one-stage endorectal pull-through is at present the most commonly used technique in neonates and infants.[5]. Rectal irrigation, a conventional and effective means for pre-operative preparation, can relieve the bowel obstruction caused by an aganglionic segment in patients with HSCR [6]. Surgical management of neonatal Hirschsprung's disease (HD) is typically performed with either a single stage pull-through (SSPT) consisting of an early primary colo-anal reconstruction in the neonatal period, or a multi-stage pull-through (MSPT) [7][8]. Neonates who are not the ideal candidates for SSPT procedure, colostomy and stage procedure is the choice of treatment[9]. But colostomy has some complications such as hemorrhage, wound infection, skin excoriation, retraction, prolapsed, para stomal hernia, stenosis etc. Home rectal irrigation may be an alternative to colostomy in the neonatal period and early infancy[10].











 

The present study was primarily designed to evaluate the feasibility and efficacy of home rectal irrigation in the neonatal period and early in infancy, followed by a delayed and planned SSPT in a prospective cohort with HSCR. [3].


Hirschsprung’s disease is the most common cause of intestinal obstruction in new borns. Colostomy followed by a trans abdominal perineal pull through procedure is the standard treatment for the disease. Using laxatives or a rectal tube and saline irrigation can successfully treat functional obstruction avoiding colostomy during neonatal and early infancy period, to overcome the complications arising in the neonates due to Hirschsprung’s disease. Thus the patient and party will be benefitted immensely due to their shorter hospital, avoid nosocomial infection and less treatment related cost.




Fig : Rectal punch biopsy forceps 



Fig : suction biopsy- a rather bed side procedure but very costly instrument wise and not available in Bangladesh.




References

1. King SK, Karpelowsky J (2017) Hirschsprung disease. ANZ JSurg 87(10):754.

           

2. Yeh YT, Tsai HL, Chen CY et al (2014) Surgical outcomes of total

           colonic aganglionosis in children: a 26-year experience in a single

           institute. J Chin Med Assoc 77(10):519–523

 

3. Lu C, Xie H, Li H, Geng H, Mo X, Tang W. Feasibility and efficacy of home rectal irrigation in neonates and early infancy with Hirschsprung disease. Springer-Verlag Gmbh Germany, September, 2019. 87(10):754

 

4. Ekenze S.O. Ngaikedi C. Obasi A. A. Problems and Outcome of Hirschsprung’s Disease Presenting 1 year of age in a Developing Country. World Journal of Surgery (2011); 77(10):519–523

 

5. Ksia A. Yengui H. Saad MA. Sahnoun L. Maazoun K. Soave transanal one-stage endorectal pull through in the treatment of Hirschsprung’s disease of the child above two-year-old: A report of 20 cases; 44(12):2352–2354

 

6. Sulkowski JP, Cooper JN, Congeni A et al (2014) Single-stage versus multi-stage pull-through for Hirschsprung’s disease: practice trends and outcomes in infants. J Pediatr Surg 49(11):1619–1625

 

7. Shah AA, Shah AV (2003) Staged laparoscopic-assisted pull-through for Hirschsprung’s disease. J Pediatr Surg 38(11):1667–1669

 

8. Vũ PA, Thien HH, Hiep PN (2010) Transanal one-stage endorectal pull-through for Hirschsprung disease: experiences with 51 newborn patients. Pediatr Surg Int 26(6):589–592

 

9. Teeraratkul S. Transanal One-Stage Endorectal Pull-Through for Hirschsprung’s Disease in Infants and Children. Journal of Pediatric Surgery, Vol 38, No 2 (February), 2003: pp 184-187

 

10. Demehri FR, Halaweish IF, Coran AG, Teitelbaum DH (2013) Hirschsprung-associated enterocolitis: pathogenesis, treat

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