Hirschsprung’s disease and home rectal irrigation

Hirschsprung’s disease and home rectal irrigation 


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. 


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]. 

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].


Fig Barium emema showing skip lesions

References:

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

            

  1. Yeh YT, Tsai HL, Chen CY et al (2014) Surgical outcomes of tota colonic aganglionosis in children: a 26-year experience in a single

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


  1. 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


  1. 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


  1. 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


  1. 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


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


  1. 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


  1. 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


  1. Demehri FR, Halaweish IF, Coran AG, Teitelbaum DH (2013) Hirschsprung-associated enterocolitis: pathogenesis, treatment and prevention. Pediatr Surg Int 29(9):873–881





Comments

Post a Comment

Popular posts from this blog

Baker’s cyst or popliteal cyst - Explained

Image
Baker’s Cyst/ Popliteal Cyst  What Is a Baker’s Cyst? A Baker’s  cyst  is a fluid-filled sac behind your  knee . It often causes a bulge and a feeling of tightness. You might hear your doctor call it a popliteal cyst. Symptoms of a Baker’s Cyst It might not cause any symptoms, but they may include: Swelling behind your  knee Swelling in your leg Knee pain Stiffness -- you won’t be able to bend your knee Causes of Baker’s Cysts Causes of a Baker's cyst may include: Swelling in the knee. This happens when the fluid that lubricates your knee joint increases. When pressure builds up, fluid squeezes into the back of the knee and creates the  cyst . Arthritis . People with all forms of arthritis often have Baker’s cysts. Injury. A sports-related injury or other blow to the knee can cause A Baker's cyst. Gout . This type of arthritis, which results from the buildup of uric acid in the  blood , can lead to a Baker’s cyst. Baker’s Cyst Diagnosis See your doctor if you have any of the s
Read more

Life in lock-down (2020 pandemic)

Image
Who would have thought what our lives would be if we were to live in cages. Being isolated from the rest of the society and almost being marooned in a deserted island gave me a lot of time to reflect on all those things which I had taken for granted. As an young adult i always enjoyed strolling around in the park, often meeting with my school friends on the weekends and grabbing a snack from the nearby french cafe while reading my favorite book laying aimlessly on that not so comfortable park bench and gentle breeze used to brush my face making a mess of my salt and pepper hair. Here in this same old-school park bench, was where I met the love of my life and in this very spot where I had asked her hand for marriage. Now in recent days, considering the horrors of world wide global pandemic COVID 19, it only seems like a dream of the past, if one is thinking of venturing into the outdoors. Different people bears different mechanisms for coping with disastrous scenario
Read more

Hirschsprung's disease - Overview

Image
Hirschsprung's disease (HD) - Overview  Hirschsprung's disease (HD) is disease of mostly unknown origin which causes problem in the large intestine. This condition revels itself from birth of the child and is related with the lack of nerve fibers in the affected portion of the bowel wall ( intestine wall). New born with HD cannot have bowel movement even beyond 24 to 48 hours after birth. Often in these times rectal wash out (rectal irrigation) with normal saline using soft rubber tube and lubricant is given with the help of doctor or nurse to evacuate the rectum. Symptoms: Considering the severity of the disease the signs and symptoms may vary which may appear  soon after birth and also in some cases later in life. The most common symptom is failure to pass stool beyond 48 hours after birth.. Other may  signs and symptoms may include: Swollen belly Vomiting, including vomiting a green or brown substance Constipation or gas, which might make a newborn fussy Diarrhea As the age
Read more