In this defect, which affects 10% of males, the rectum opens at the bladder neck (Fig. 20.9). The patient has a poor prognosis because the levator ani muscle, muscle complex, and external sphincter are frequently poorly developed. Consistent with the caudal regression, the sacrum and entire pelvis is often deformed and underdeveloped. The perineum is often flat, with evidence of poor muscle development.
For this repair, a total body preparation is performed; the sterile field includes the entire lower part of the patient's body. The initial incision is posterior sagittal. All of the muscle structures are divided in the midline. The channel for the rectum, which lies just under the coccyx, is created bluntly. This posterior sagittal incision can be created with the child in supine position and the legs lifted up. At this point, laparoscopy represents an excellent minimally invasive alternative to a laparotomy.
These patients have the rectum connected to the bladder neck, located approximately 2 cm below the peritoneal reflection. Interestingly, the higher the malformation, the shorter the common wall between the rectum and the urinary tract. This means that the rectum in this group of defects opens in a perpen dicular fashion into the bladder neck, which makes its dissection much easier. The laparoscopic approach provides an excellent view of the peritoneal reflection, and one can also see the ureters and the vas deferens, which tend to run in the direction of the bladder neck. During the dissection of the rectum, therefore, these structures must be kept under direct view to prevent damage to them. The serosa that covers the most distal part of the rectum should be divided, creating a plane of dissection around the rectum; it is this plane that is used to continue the dissection distally. The rectum rapidly narrows down, and this is where the fistula should be ligated.
At this point the surgeon must gain adequate length for the rectum to reach the perineum. The vessels that supply the distal rectum must be meticulously divided. Laparoscopically, or through a laparotomy, the space in the retroperitoneum for pull-through of the rectum is visualized (Fig. 20.10). An instrument or trocar from the perineum can be introduced to grab the rectum and pull it down. This allows the surgeon to see from above the tension lines that represent the vessels that it must be divided until the rectum reaches the perineum.
Tapering of the rectum, if that is required, is difficult with a laparoscopic approach, and mobilization of a very high rectum is technically challenging. In addition, passage of the trocar from perineum into the pelvis must be done carefully to avoid injury to the bladder neck and ureters.
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