Franco Soave, in Italy [

24 ], and Scott Boley

in the United States [ 25 ] applied the endorec- tal, submucosal dissection principle to treat Hirschsprung’s disease. Through a laparotomy, they resected the aganglionic intraperitoneal portion of the bowel as well as the most dilated part of the proximal colon. They also selected the normoganglionic piece of bowel to be pulled down (Fig. 24.19 ). An endorectal dissection of the aganglionic segment was performed trans­abdominally, from the peritoneal fl oor down to the anal canal. The dissection is carried down relatively easily and continues distally until the surgeon “feels” that he is reaching the area of the anal canal. At that point, the surgeon goes

ing than the Duhamel one. Complications with this technique have been reported and consist of leaving pieces of mucosa deep in the pelvis producing mucus and then generating abscesses and multiple fi stulas that are very diffi cult to treat [ 118 ]. We receive a number of cases oper- ated on with the Soave technique that came to us because they suffer from abscesses and fi stulas.

We had to explore those patients posterior sagit­tally or transanally and found islets of mucosa or sometimes even complete pieces of bowel left trapped between the seromuscular cuff and the bowel wall of the normoganglionic pulled­through bowel [ 118 ]. Some patients underwent an attempted repair for Hirschsprung’s and suf­fered from dehiscence and retraction. When we

Total colonic aganglionosis is a very serious form of Hirschsprung’s disease. Even when these patients are treated in a technically cor­rect way, they still suffer from very signifi cant sequelae [ 119 , 120 ]. We believe that we are not really curing this condition. As previously mentioned, this condition is defi ned as the absence of ganglion cells in the entire colon. Sometimes the aganglionic segment includes

Adult colorectal surgeons as well as pediatric surgeons, dealing with patients subjected to total colectomies for different reasons, have been trying to be very creative in dealing with the problems of diarrhea and very frequent bowel movements. Dr. Lester Martin [ 121 ] proposed an interesting idea to deal with this problem. A technique similar to the one used by Duhamel is performed, except that the aganglionic segment left includes the entire descending colon and rectosigmoid. The normoganglionic terminal ileum is pulled through, behind the rectum. A very long longitudinal anastomosis between the normoganglionic terminal ileum and the aganglionic rectosigmoid is performed (Fig. 24.21 ). The rationale of this operation was

Subsequently, Kimura et al. [ 122 ] proposed a technical modifi cation to Martin’s original idea, creating a patch using the right ascending colon, due to its great capacity to absorb water.

Kimura’s procedure was rather complex and required at least two surgical stages. Consequently, Boley modifi ed the procedure to do it in a single operation [ 123 ].

B. He or she allows the mother to pass a catheter

This means that we close these ileostomies usually in patients that are 3–5 years old [ 128 ]. This is very different to the current trend of oper­ating on patients with congenital malformations as early as possible. The reason for the manage­ment plan that we propose is that we have seen many patients with total colonic aganglionosis operated early in life. We surgeons are very proud of the fact that we can perform major operations in the newborn period. Yet, in dealing with total colonic aganglionosis, when these operations are done in very young babies, they suffer from con­stant passing of stool in the underwear and develop one of the worst diaper rashes that we have seen (Fig. 24.22 ). The surgeons usually do not become aware of this, since it is the mothers and the nurses who desperately try to control these kinds of problems. The babies, sometimes, suffer for years from what seems to be almost like a second-degree burn of the buttocks. On the other hand, the presence of an ileostomy some­times upsets the surgeons and the parents, whereas the babies are happy. The baby grows up without enterocolitis and does not care about having an ileostomy. He/she becomes aware of the ileostomy only when he/she goes to school. Before that, in general, he/she is well adapted. Care of a longstanding ileostomy requires careful observation for dehydration and monitoring of sodium losses, often necessitating oral sodium supplementation.

We like to wait until the baby is toilet trained for urine because at that time, he/she knows how to verbalize his/her desire to go to the toilet. He/ she has the capacity to hold the urine and to go to void when necessary. In addition, he/she is accus­tomed to being clean and dry in the underwear, and therefore, he/she has a greater chance to become toilet trained sooner after the ileostomy is closed. We all know that otherwise normal adult individuals, subjected to total colectomies and ileoproctoanastomosis (even when the opera­tion has been done in a technically correct man­ner), sometimes have “accidents” (passing stool accidentally in the underwear), particularly dur­ing the night. The very active, constant peristalsis (hypermotility) characteristic of the small bowel, when connected to the rectum, even if it is done in a technically correct way is not tolerated by little babies and that is why they keep passing stool constantly, producing a severe and non­manageable diaper rash. On the other hand, a 3–5-year-old child that is toilet trained for urine already knows what the toilet is all about and therefore has more possibilities to become toilet trained for stool very soon. The parents of our patients treated this way express a high degree of satisfaction. Yet, the patient still has to go through a period of many bowel movements in the under­wear. The number of bowel movements decreases with time because the patient learns to hold the stool. The terminal ileum that we anastomose to the anal canal, as time goes by, becomes dilated and populated by abnormal fl ora; therefore, they

After the ileostomy has been closed, we watch the patient very closely, as previously described, every time the patient feels sick or suffers from abdominal distention and/or vomiting, the care­giver performs a rectal irrigation that helps the child feel better and to recover the appetite. In a characteristic patient with total colonic agangli­onosis, the parents learn to hear the bowel noises from far away. In addition, we monitor radiologi­cally the degree of bowel dilatation, as well as the mucosal irregularities as signs of imminent enterocolitis and, when necessary, start irriga­tions three times daily, as well as administer oral metronidazole proactively.

due to the fact that the patient has a “cement” type of pelvis because of the severe fi brosis. It is in those cases that we use a posterior sagittal approach to obtain a direct view of the troubled area (Fig. 24.20 ). We have resected remnants of rectal mucosa or pieces of trapped bowel left in the pelvis, after attempted, failed endorectal dis­sections. With this approach, we can perform an end-to-end anastomosis under direct vision. We have been able to close acquired rectovaginal as well as rectourethral fi stulas under direct vision. Also, it is possible to mobilize normal bowel to be sure that we leave a normal bowel wall in front of the vaginal or urethral repair. We strongly recommend the use of a protective colostomy if one is obligated to use a posterior sagittal approach to repair these cases. Not open­ing a colostomy exposes these patients to suffer

The posterior sagittal approach used for dif­ferent types of problems gave us a unique oppor­tunity to be exposed directly to the posterior rectal wall and evaluate its characteristics (Fig. 24.23 ). We have not been able to identify the thickness of smooth muscle described as “internal sphincter.”

For the group of patients that came to us suffering from fecal incontinence after having an operation for Hirschsprung’s disease, we have a specifi c pro­tocol of management. Figure 24.24 shows our deci- sion-making algorithm for those patients. We perform a contrast enema and an examination under anesthesia. The purpose of the examination under anesthesia is to determine whether or not the patient has an intact anal canal and/or to determine the degree of damage that the anal canal suffered during the previous operation. One hundred and three patients come to our center suffering from fecal incontinence consecutive to an operation for Hirschsprung’s, and we found 54 cases in which the surgeons performed an anastomosis of the normo­ganglionic bowel basically to the perianal skin (Fig.

destroyed or removed during the original operation. Those patients most likely will suffer from fecal incontinence for life. Sometimes we see a very pat­ulous anus indicative of overstretching the sphincter during the pull-through (Fig. 24.26 ). When we fi nd