Trans-Anal Modified Endorectal Pull-Through (Soave-Boley) Procedure for Hirschsprung's Disease without Abdominal Incision in a Neonate
Figure 1:
Barium enema depicting transitional zone in recto-sigmoid colon.
Type: VSH
"Primary Trans-Anal (Incision-less) Endorectal Pull-Through for Hirschsprung's Disease in a Newborn"

3 week-old TAGA baby girl born with constipation and painless abdominal distension. 
Barium enema shows a transitional zone in recto-sigmoid colon (see figure 1 above). 
Suction rectal biopsy demonstrated no ganglion cells. Rectal irrigation's until 4 kg of weight. 
Single stage trans-anal modified endorectal pull-though (Soave-Boley) done without abdominal incision. Duration of procedure - 90 minutes. Send home one day after surgery. Doing well.

Technique in Detail
           Diagnosis of HD is made with the help of clinical manifestations, barium enemas findings and suction rectal biopsy. This technique pertains to cases with classic recto-sigmoid aganglionosis although we believe long-segments cases can also be managed similarly.
           The child receives oral electrolyte solution and bowel cleansing the day before surgery. Preoperative prophylaxis with broad-spectrum antibiotics is used. In a modified lithotomy position the abdomino-perineal area is prepared and draped. The anus is gently dilated manually. Stay sutures placed around the ano-cutaneous junction permits eversion of the entrance of the anus. Alternatively you can use a circumferential retractor system (1). Around one centimeter above the pectinate line the area is infiltrated with an epinephrine-saline 1:200,000 solution. Multiple fine silk traction sutures are placed circumferentially in the mucosa where the dissection will commence. Using sharp dissection with small curved scissors separation of the submucosa layer from the circular internal sphincteric fibers is performed bringing forth the mucosa-submucosa tube of tissue. Needle tip cauterization of small vessels may be needed. 
           Once the dissection goes smoothly the submucosal plane meets the peritoneal reflection. The transanal submucosal dissection is extended above the extra muscular plane (2). At this point the bowel wall is opened in its most anterior portion and entrance into the peritoneal cavity is obtained. The seromuscular coat is cut circumferentially and the bowel can be mobilized into the perineal area further by coagulating the vessels and ligamentous attachments near the bowel wall. Once the transitional zone has reached without tension to the anocutaneous junction a piece of bowel wall is send for frozen section confirmation of ganglion cells. The bowel is amputated and a single layer colo-anal anastomosis using polyvycryl 0000 alternated with 00000 interrupted sutures is done. 
           No postoperative bowel decompression is needed (NG). After two hours in recovery room the child is sent to the ward and po fluid liquids are given six hours after surgery. Prophylactic antibiotics are completed for no more than twenty-four after surgery. Once bowel function returns (generally twelve to eighteen hours later) the child is sent home. Two weeks after surgery a rectal exam is done. 

1- Saltzman DA, Telander MJ, Brennon WS, Telander RL: Transanal Mucosectomy: A Modification of the Soave Procedure for Hirschsprung's Disease. J Pediatr Surg 31(9): 1272-1275, 1996
2- Georgeson KE, Fuenfer MM, Hardin WD: Primary Laparoscopic Pull-Through for Hirscsprung's Disease in Infants and Children. J Pediatr Surg 30(7): 1017-1022, 1995

Iván Figueroa-Otero, MD FACS FAAP
Humberto Lugo-Vicente, MD FACS FAAP
Section of Pediatric Surgery, Department of Surgery, University of Puerto Rico School of Medicine and the University Pediatric Hospital, San Juan, Puerto Rico.

Duration of video: 18 min (Voiceless)


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 Humberto Lugo-Vicente, MD
 P.O. Box 10426
 Caparra Heights Station
 San Juan, Puerto Rico 00922  USA
Tel (787) 786-3495
Fax (787) 720-6103

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Last update: August 2020