If a pediatric surgeon has no experience in the diagnosis and management of urologic problems, he should work together with a pediatric urologist in the initial evaluation of a patient with anorectal malformation.

All newborn babies with anorectal malformations should have a kidney ultrasound, within the first 24 h of life and before any surgical intervention. Female babies, particularly those with cloaca (single perineal orifice), must have, in addition, a pelvic ultrasound to rule out the presence of a hydrocolpos.

We believe that a male baby with a normal kidney ultrasound, that is passing urine satisfactorily, has a normal sacrum, and no evidence of tethered cord can be operated on without any further urologic tests. On the other hand, if the baby has hydronephrosis, he will require further urologic studies, including a voiding cystourethrogram. We do not believe that the voiding cystourethrogram should be done routinely, in all male babies with anorectal malformations, because in our experience, it is not a good study to diagnose the location of the recto-urinary fistula. In addition, the urethral catheterization in babies with rectourethral fistulas sometimes is difficult, because the patients frequently have kinks or narrowing of the urethra, particularly at the junction between the rectum and the urethra.

If the baby has tethered cord and/or a very abnormal sacrum (sacral ratio less than 0.4), he is at risk of suffering from deterioration of the bladder function, which eventually may affect the kidneys and therefore is considered a high-risk patient from the urologic point of view, even if the initial kidney ultrasound is normal.

The female baby with a cloaca represents a particularly difficult challenge (see Chap.​ 16). Thirty percent of patients born with a cloaca have a hydrocolpos that may produce an extrinsic compression of the ureterovesical junction, which results in megaureter and hydronephrosis. As we mentioned in the Chap.​ 16, before considering drainage of the urinary tract with a nephrostomy or ureterostomy, the surgeon must drain the hydrocolpos and reevaluate the megaureter and hydronephrosis. After the hydrocolpos has been drained, the megaureter will improve or disappear, as well as the hydronephrosis. If the hydronephrosis or the megaureter persists, the baby must be subjected to further evaluation to consider the possibility of making a ureterostomy, nephrostomy, or vesicostomy. We have seen many babies with cloacas, hydronephrosis, and megaureter, subjected to unnecessary ureterostomies, nephrostomies, and/or vesicostomies, in a futile attempt to improve the hydronephrosis, without draining the hydrocolpos (see Chap.​ 16).

There is a small group of patients with a cloaca that are born with a very narrow (almost atresia) common channel which interferes with the emptying of the bladder. After draining the hydrocolpos, an ultrasound may show that the bladder is still full and does not empty well. An attempt to catheterize the bladder may be extremely difficult; in those cases it is justified to do a cystostomy or a vesicostomy.

Another possible indication for a vesicostomy is the case of a baby with demonstrated massive vesicoureteral reflux, megaureter, hydronephrosis, and important risk factors for poor bladder function, such as poor sacrum and tethered cord.

The newborn baby should not be taken to the operating room until the surgeon has ruled out and treated important associated urologic problems. After this, the baby is taken to the operating room to perform either a colostomy or a primary repair of the anorectal malformation.

The location and type of colostomy may have important urologic implications. It is important for the neonatologist as well as the urologist to remember this (see Chap.​ 5).

The colostomy that we recommend is one created at the end of the descending colon and proximal to the sigmoid loop. The purpose of that specific location is dual, first, to avoid prolapse of the proximal stoma, since the descending colon is fixed, and, second, to leave enough distal bowel (loop of the sigmoid) to allow a successful pull-through. In addition, we specifically recommend to make the distal stoma (mucous fistula) very narrow in order to avoid prolapse, but still, we believe that is important to keep it open to be able to irrigate the distal bowel and to perform radiologic studies, most specifically, a high-pressure distal colostogram. We strongly recommend separating both stomas in order to be able to put a colostomy bag, including only the proximal stoma and not the distal one. When both stomas are too close together, the mother is forced to use a single bag to cover both stomas, which may produce fecal contamination of the distal bowel and consequently of the urinary tract. Loop colostomies, therefore, from our point of view, are formally contraindicated in anorectal malformations, because they represent a source of fecal contamination of the urinary tract.

During the creation of the colostomy, we specifically recommend the surgeons to wash the bowel distal to the mucous fistula until it is completely collapsed and cleaned of meconium. If a baby receives a colostomy and after the procedure is not doing well and suffers from frequent urinary tract infections and from episodes of acidosis, it is extremely important for the physician to look at the type of colostomy that the baby has, looking for an explanation for that behavior. A loop colostomy producing frequent urinary tract infections would require an operation to separate the stomas. If the surgeon did not wash the distal bowel, sometimes the accumulation of meconium, plus mucus passing through the fistula, produces urinary tract infections. Finally, when the colostomy was opened in the transverse colon (which we consider contraindicated), the meconium may pass into the urinary tract, but also urine passes from the urinary tract into the colon where it is absorbed, producing hyperchloremic acidosis that may interfere with the growth and development of the newborn baby. When a pediatric urologist is called to see a baby with this kind of problem, he should look at the type of colostomy that the baby has.

In our series of 909 cases of male patients with anorectal malformation, we found 102 patients suffering from a bifid scrotum (Fig. 23.8). Most of the cases of bifid scrotum occur in babies who have anorectal malformations that belong to the “bad” side of the spectrum, in other words, patients that have bladder neck or high prostatic fistulas. Therefore, a bifid scrotum should be considered as a sign of a potential complex malformation.

Through the years, we learned to repair the bifid scrotum in a rather simple way, at the same time that we repair the anorectal malformation. It is important to recognize that in cases of bifid scrotum, there is a midline portion of nonelastic skin that separates both hemiscrotums (Fig. 23.9a–e). The skin of the scrotum is recognized because it has rugae and is elastic. The skin in between the hemiscrotum lacks that kind of rugae, is rather smooth, and is nonelastic. The technique that we use consists of resecting the smooth, nonelastic portion of the skin. Figure 23.9a shows how we have marked the limits that separate the smooth, nonelastic skin from the normal rugae scrotal skin. Figure 23.9b shows the incision that is made with the needle-tip cautery. Figure 23.9c shows that the smooth skin has been resected and the beginning of a two layer suture of the skin edges (Fig. 23.9d). Both layers sutured with multiple, interrupted, 6-0 long-term absorbable sutures. Figure 23.9e shows the final aspect of the repair. Later in life, it is very difficult to know that this patient had a repair of a bifid scrotum and the parents have expressed their satisfaction.

Sixty of our male patients suffered from different types and severity of hypospadias. It may go from a subcoronal type (Fig. 23.10a) to an extreme type in which the urethral orifice is located immediately anterior to the anal opening (Fig. 23.10c). Extreme, severe types of hypospadias are frequently associated to penoscrotal inversion.

The timing for the repair of hypospadias is something rather controversial. Some pediatric urologists like to repair the hypospadias before the colostomy is closed, to avoid fecal contamination during the postoperative period. Some others prefer to wait until the patient is older to be able to deal with better tissues and have more chances of success. From the point of view of the pediatric surgeon, it is important to identify the most severe type of hypospadias (Fig. 23.10c, d) in which the urethral orifice is located immediately anterior to the anal opening. The surgeon must keep in mind that the rectum and urethra share a common wall, usually in the area of the spongiosum tissue. Those babies have a strictured anal opening located anterior to the center of the sphincter, and therefore, the operation will consist in moving the anus back and simultaneously to correct the severe chordee. In order to do that, the surgeons have to work on the common wall between the rectum and the urethra and move, if possible, the urethra a little forward. The idea is to create a reasonable space between the anal opening and the urethral opening and move the penis forward, away from the anal opening. After this repair, the urologist will take care of the problem of repairing the rest of the perineal urethra, as well as the penile urethra. Figure 23.10d shows the external aspect of a patient that underwent a repair of an anorectal malformation and separation of the rectum from the urethra in a severe perineal hypospadias.

This malformation has been reported in cases without ARM [25]. It seems to be relatively rare. We believe that it occurs more often in patients with anorectal malformations.

Twenty-one of our male patients suffer from some form of ureteral ectopia. In patients with anorectal malformations, we have seen that the ureters have a tendency to open ectopically, in the line that goes from the normal location of the ureter in the trigone toward the bladder neck. It is relatively common to see patients with a ureteral orifice located closer to the bladder neck. There are however cases in whom, definitely, we see the ureter opening in the bladder neck (Fig. 23.11a). The opening of the ureter in the bladder neck frequently is associated with some degree of ureteral obstruction and megaureter. In addition, the presence of the ureter in that abnormal location may interfere with the closure of the bladder neck, producing a certain degree of bladder neck incapacity to hold the urine. We have relocated the ureters out of the bladder neck with success, recovering urinary control in patients that were leaking urine.

The most severe type of ureteral ectopia in males occurs when the ureters are connected to the posterior urethra (Fig. 23.11b). In these cases, the patient suffers from severe megaureter, and they frequently have severe damage to the kidney that usually ends up with a nephrectomy. The surgeon should be alerted about the fact that the megaureter connected to the posterior urethra may give the false intraoperative impression of the rectum. In one specific case, the patient was operated in another city without a distal colostogram, and the surgeon entered posterior sagittally looking for the rectum, found a “tubular structure,” separated it from the posterior urethra, and pulled it down, believing it was the rectum, and it turned out to be a megaureter. The patient had actually a recto-bladder neck fistula.

As expected, the frequency of ectopic ureter is higher in patients with higher and more complex malformations. If one already made the diagnosis of an ectopic ureter located in the posterior urethra, the posterior sagittal approach represents a golden opportunity to identify the megaureter, separate it from the posterior urethra, close the posterior urethra, and push the megaureter up into the pelvis, to be recovered through a small incision in the groin, where it can be connected to the skin as a ureterostomy. It will take several months for us to determine the degree of renal damage that the kidney has and whether or not the patient will benefit from a nephrectomy or a ureteral reimplantation.

All that we said about the ectopic ureters in the trigone and bladder neck is true for female patients. The ureters also may be ectopically connected to the urethra in a female. Figure 23.12 shows a picture taken during a transpubic approach of a female patient with a complex malformation. It would be very difficult to separate the ureter with a different type of approach. We use the transpubic approach in cases that we believe would be very difficult to approach in a different way.

In patients with cloaca, it is also relatively common to find ureters connected ectopically to the vagina. Again, most of the time these ureters belong to severely damaged kidneys.

This rare anomaly has been reported in patients without anorectal malformation [26–28]. The most common clinical manifestation has been orchiepididymitis. However, we found more publications related to this malformation in cases of anorectal malformations [29–38].