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Key Points

  1. The posterior sagittal approach for anorectal malformations, first performed in 1980, led to significant implications in terms of terminology and classification, reduction of complications, and has since been expanded for many other surgical challenges in the pelvis.

  2. Correct anatomic terminology, rather than arbitrary descriptions of “low,” “intermediate,” and “high,” are useful in therapeutic and prognostic terms.

  3. The key technical points to repairing males with an ARM and a rectourethral fistula are that the lower the rectum, the longer the common wall between the anterior rectum and the posterior urethra, and that the bladderneck fistula is the only defect in which the distal rectum is at or above the peritoneal reflection. The rectum in the rectoprostatic, the rectobulbar, and the no fistula defects lie below the peritoneal reflection and are best approached posterior sagittally rather than transabdominally.

  4. An accurate preoperative distal colostogram is vital to ensure proper surgical planning, otherwise the surgeon will be looking for the distal rectum blindly, and can easily injure adjacent structures such as the vas deferens, urethra, seminal vesicles, and the bladder neck.

  5. Rectal atresia is a rare anorectal malformation with the unique feature of having a normal-appearing anal canal and dentate line, and being specifically associated with a presacral mass.

  6. Rectovestibular fistula is the most common malformation in females. The key technical concern is creating 2 walls out of 1 between the intimately attached rectum and vagina.

  7. Cloacal malformations form their own broad spectrum, with a 3-cm common channel being a dividing line between a straightforward repair able to be accomplished with a total urogenital mobilization posterior sagittally, and a complex repair requiring a laparotomy as well as special maneuvers to perform vaginoplasty, vaginal replacement, sometimes separation of the urinary tract from the gynecologic system, or preservation of the common channel as neourethra.

  8. A patient with a good prognosis defect (rectoperineal fistula, rectobulbar urethral fistula, rectovestibular fistula) with a good sacrum and spine should be expected to achieve normal bowel control.

  9. When a baby is born with an anorectal malformation, in addition to investigating for associated defects, the surgeon must decide whether to perform a colostomy or if a primary repair can be done. It takes 20 to 24 hours for enough pressure to build in the distal colon to delineate a fistula, and the decision as to what operation to pursue should wait this amount of time.

  10. The most trouble-free colostomy for anorectal malformations is a proximal sigmoid diversion, with separated stomas.

  11. For patients who cannot achieve voluntary bowel control, a bowel-management program consisting of a tailored daily enema allows the patient to remain completely clean and in normal underwear.

Historical Review

Anorectal malformations have been well known for many centuries, and described by, surgeons, sorcerers, and early medical practitioners who attempted to create an orifice in the perineum in children born with these defects. Those patients who were lucky to be born with a rectum ...

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