NEONATAL DIAGNOSIS AND MANAGEMENT OF ANORECTAL MALFORMATIONS Female Algorithm

Simple perineal inspection provides the diagnosis in virtually 90% of ARMs in females (Fig. 89-14). For example, cutaneous and vestibular fistulas are readily identified during a perineal exam. One must immobilize the baby's legs and use good lighting. A rectovestibular fistula orifice can usually be identified just outside the hymen. A rectovaginal fistula is identified by meconium coming from inside the vagina through the hymen, but this is an exceedingly rare malformation. A perineal (cutaneous) fistula has the same prognostic and therapeutic significance as in males. A vestibular fistula tends to be competent and to remain patent with serial dilatations, which can be performed if the infant is ill. Depending on the surgeon's experience, a primary repair in the newborn period can be undertaken. The safest approach is always a colostomy with a future repair that avoids potential infection and dehiscence, which have occurred with newborn repair of rectovestibular fistulae. Passage of stool through this fistula for prolonged periods of time can induce varying degrees of megacolon and potential constipation. A single perineal orifice means the baby has a persistent cloaca. In most of these cases, there is an associated urologic emergency that requires prompt evaluation. Thus, these babies will most likely receive a colostomy, a vaginostomy or vesicostomy, or any other urinary diversion. A midline, lower abdominal mass in these newborns is pathognomonic for a hydrocolpos. It is imperative for pediatric surgeons dealing with cloacas to be aware of the fact that a very dilated vagina is a significant problem for these newborn babies. During the neonatal period, the baby should not be taken to the operating room until the urinary tract is adequately evaluated and the presence of a hydrocolpos has been ruled out. If P.1416

the baby has a hydrocolpos, it is mandatory for the surgeon not only to open a colostomy, but also to insert tubes into the dilated vagina or vaginas to decompress them and thus prevent complications, such as pyocolpos or ureteral obstruction.

If a female does not pass meconium in the first 16 to 24 hours of life, then again a cross-table lateral radiograph is indicated. In addition, an abdominal ultrasound to evaluate the kidneys and ureters is also required.

Perineal Fistula

Special consideration is given to this variant of ARM because it is frequently discovered in the pediatrician's office for the workup of constipation (131,132). Usually, the sphincteric mechanism is present and functioning. The anal orifice, however, is well forward of the muscles and a posterior shelf and pocket or cul-de-sac is palpable during rectal examination. The clinical picture is remarkably consistent. Chronic constipation persists despite all medical management using formula changes, laxatives, and stimulants. The referral to pediatric surgeons is usually to rule out Hirschsprung's disease, and in most of these patients, there is no aganglionic bowel. These patients need a thorough physical examination that includes the anus. In fact, the diagnosis can be suspected merely by inspection. The anus should normally be positioned at the midpoint of a line drawn between the base of the vagina or scrotum and the tip of the coccyx. The perineal fistula is anterior to this midpoint position and thus outside some, if not all, of the external anal sphincter. Not all these patients require repositioning operatively. Those who fail medical treatment with stool softening and have recurrent urinary tract infections should probably undergo anoplasty. Our recommendation is reconstruction using a minimal posterior sagittal approach that facilitates exposure of the external sphincter and its junction with the muscle complex. An anterior operative approach can also be performed. Restoration of the perineal body and positioning of the anus within these structures is all that is required. A colostomy is not indicated. In newborns, no bowel preparation is required. However, in older patients a very strict bowel preparation is mandatory, along with 5 to 7 days of nothing-by-mouth status that usually means peripheral alimentation.

Anoplasty

The indication for some form of anoplasty includes cases of male and female perineal fistulas (Fig. 89-15). Regardless of the type of operation performed, the goal is to restore the anus to its normal anatomic position within the external sphincter. Because postnatal bacteria flora usually takes 3 days to be present in the baby's stool, no bowel preparation is necessary if the anoplasty is performed during this time period. Prophylactic antibiotics are given, and in males a Foley catheter in the bladder is mandatory. The most frequent complication is urethral injury because, even in the low defects, the rectum and urethra are intimately attached. The operation is a minimal posterior sagittal anoplasty (Fig. 89-15). The minimal posterior sagittal anoplasty not only uses the nerve stimulator, but also takes advantage of the direct visualization of the external sphincter from anterior to posterior. Multiple 60 traction sutures are placed around the perineal orifice, and then a dissection is performed circumferentially around the rectum. The sphincters should P.1417

not be mobilized. It is the bowel that is to be dissected. Just enough bowel is mobilized to allow placement of the neoanus within the confines of the sphincter. In females, there is little to no morbidity with this procedure. Maintaining a plane immediately beyond the anus and not traveling beyond these limits facilitates protection of the bulbar urethra. Prior to the neoanoplasty, the perineal body should be restored using interrupted absorbable suture material. Two weeks after the minimal PSARP, a schedule of dilatations is begun (Table 89-5).

Colostomy

A descending colostomy is the procedure of choice for ARMs (133). In general, a completely diverting descending colostomy should be created in a newborn with a high ARM. Complete diversion is difficult, if not impossible, with a loop colostomy, and thus, the potential for continued communication between rectum and urinary tract is high. Also, a loop colostomy in contrast to a completely diverting colostomy predisposes the child to urinary tract infections. The propensity for continued overflow into the distal bowel and consequent abnormal bowel distention can compromise the definitive repair and bowel motility. Postoperative constipation can be associated with megarectum, secondary to fecal impaction, created by this overflow into the distal bowel. If a loop colostomy is created too loosely, there is the potential for prolapse of the proximal stoma. The most common error is opening the colostomy too distally, leaving one with an incomplete length of bowel for the definitive repair, and thus forcing the surgeon to gain length by performing a laparotomy. The descending colon double-barrel colostomy is our procedure of choice. Both types of stomas should be created through an oblique left lower quadrant incision using two layers of long-term absorbable sutures, one incorporating the posterior sheath and the second layer using the anterior sheath. Either stoma should be created so the distal stoma is narrow to avoid prolapse. The distal stoma (mucous fistula) should be irrigated with warm saline in the operating room so the distal bowel is cleared of meconium. A loop colostomy leads to megarectosigmoid and continued fecal contamination of the urinary tract (134).

Preoperative Colostography

A preoperative colostogram is usually performed 3 to 4 weeks following creation of the stoma. It is commonly performed on an outpatient basis because the baby has likely been discharged from the hospital. The purpose of the colostogram is to define the anatomy of the distal bowel and establish the site of fistulous connection with the urinary tract. Thus, the procedure is called a distal colostogram. In a retrospective review of boys with ARM, the risk of urologic injury was increased in those males who did not undergo a properly performed distal colostogram to define the level of the fistula (135). The single most important limitation to an adequate study is the fact that the distal rectum is surrounded by the sphincteric mechanism, which even in patients with ARMs exerts enough tone to collapse the rectum. For adequate filling of the distal bowel, enough hydrostatic pressure must be exerted to overcome the resting muscle tone and ultimately fill the distal bowel and fistula site. Therefore, we recommend injection of contrast material into the distal bowel using a Foley catheter with 2- to 3-mL inflation of the balloon. The lumen of the mucous fistula is occluded by pulling on the catheter and hand injecting a radiopaque water-soluble contrast using fluoroscopy. The anal dimple is marked with a piece of lead. In the anteroposterior position, the first piece of information to be obtained is the measurement of available length of distal bowel. Next, in the lateral projection, the fistula site is determined again by injection of contrast. The contrast material that passes into the urethra usually goes into the bladder. To complete the study, contrast should distend the bladder and the patient should be seen voiding (136). A VCUG is not necessary because the distal colostogram, if performed as outlined, provides the essential information. The bladder, urethra sacrum, and anal dimple should be visualized in all projections. Failure of a colostogram is frequently the result of lack of back pressure while injecting the contrast or muscle action around the distal rectum.
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In patients with a common cloaca, contrast should be injected through the common channel on the perineum, the distal colostomy, and the vesicostomy and vaginostomy when present (Fig. 89-16). A VCUG in children with cloacas is very difficult to obtain, except in those with a cystostomy or vesicostomy.

DEFINITIVE REPAIR

General Concepts The indications for definitive repair of an imperforate anus include all patients with nonlife-threateningassociated anomalies and nonlifethreatening chromosomal defects. Myelomeningocele, high cloaca, and absent sacrum are not contraindications to definitive repair. Absence of the colon, however, is a contraindication to PSARP. Although a variety of abdominoperineal approaches have been advocated for the repair of ARMs, the authors recommend the posterior sagittal approach for all defects. In cases of a rectobladder neck fistula, a laparotomy or laparoscopy is required to find and mobilize the distal rectum (137). In a patient with persistent cloaca with a common channel longer than 3 cm, a laparotomy may also be required.

Patient Positioning The prone position with the pelvis elevated is the optimal position for the posterior sagittal approach to ARMs. A Foley catheter is inserted into the bladder prior to positioning the patient. It is not uncommon for the catheter to follow the fistula tract into the rectum and end up in the field during the operation. It should be redirected once the rectum has been opened.

Electrical Stimulation Although the patient is paralyzed, electrical stimulation of the perineum can be conducted through a series of reflex arcs directly via muscle. Stimulation facilitates maintaining symmetry of muscle during the dissection. When stimulating on the skin, 100 to 200 mA may be required, whereas current of 20 to 40 mA is all that should be required directly on muscle.

Incision A midline incision facilitates keeping the sphincter mechanism symmetrically about the midline. Direct regional periodic electrical stimulation of the sphincteric mechanism ensures equal distribution of the voluntary striated muscle. There have been no published reports of nerves and vessels crossing the midline in this part of the body. Thus, staying in the midline raphe that seems to divide whatever spectrum of muscle is present is a prudent maneuver. Exposure using a sharp, self-retaining retractor should only be performed in a superficial plane.

Anatomy From the posterior sagittal approach, the anatomy of a normal child is different compared with the potential paucity of muscle in children with ARMs (Figs. 89-9 to 89-11). One should be able to identify an external sphincter (parasagittal fibers), vertical fibers of the striated muscle complex, and the levator. Smooth muscle, but no discrete internal sphincter, has been identified.

Posterior Sagittal Anorectoplasty

Operative Approach for Females

Rectovestibular Fistula

There is an obvious disagreement in the pediatric surgical literature concerning the appropriate management of the rectovestibular fistula (139,140,141) (Fig. 89-7). In part, this is because of a fundamental underestimation of the complexity of the defect. There is no question that this ARM can be repaired without a colostomy, but the functional prognosis should be excellent. Yet, in the absence of a protective colostomy, the repair of the vestibular fistula carries a significant morbidity characterized by wound dehiscence and infection that can jeopardize the final
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functional prognosis. Therefore, a protective colostomy is advised if the surgeon is not experienced with this meticulous dissection. A PSARP is performed at 1 month of age provided the baby is otherwise healthy. A limited posterior sagittal anoplasty incision begins anterior to the coccyx and is continued to and around the fistula in the vestibule anterior to the hymen (Fig. 89-25). Multiple 60 sutures are placed around the fistula site to exert a uniform traction, and this parachute technique is used to facilitate dissection of the rectum. The posterior wall of the rectum is identified, and the dissection proceeds laterally. Inferior hemorroidal vessels are cauterized as the dissection proceeds laterally. Separation of the rectum and vagina is facilitated by pulling on the stay sutures surrounding the fistula (Fig. 89-26). Anteriorly, there is an extensive common wall between the rectum and vagina, but two walls must be created until complete separation into a definable rectal and vaginal wall plane develops. Tension resulting from too small a dissection predisposes to dehiscence. Using electrical stimulation, the limits of the neoanus are identified and marked with sutures (see Operative Approach for Males, Rectourethral Fistula). A perineal body is restored with one or two layers of tissue separating the rectum and vagina, and including the anterior limits of the muscle complex (Fig. 89-27). The muscle complex is reconstructed by incorporating the posterior edge of bowel in the midline (Fig. 89-27). The levator muscle is usually not P.1425

exposed. An anoplasty and a skin closure are performed as previously described (see Operative Approach for Males, Rectourethral Fistula).

In cases of the rare vaginal fistula, the dissection is similar, except here the levator is divided because the rectum must be mobilized to reach the perineum.

Persistent Cloaca The repair of persistent cloaca is the most technically challenging of all types of ARMs (Fig. 89-8). The common channel represents the confluence of urinary tract, vagina, and rectum. The operative procedure begins with a long midsagittal incision extending from the middle of the sacrum through the perineum and into the common single opening. A midsagittal incision is performed, and parasagittal fibers of the external sphincter, then the muscle complex, and finally the levator muscle are divided as mentioned previously. During the initial experience with this malformation, a posterior sagittal operation, with or without a laparotomy, depending on the complexity of the defect, was used. The rectal wall is opened in the midline distally and held open with stay sutures. The rectum is separated from the vagina (Fig. 89-28), and then the vagina must be separated from the urinary tract (Fig. 89-29). Ultimately, all structures should reach the perineum. The rectum and vagina have a common wall, as described in cases of vestibular fistula. A more complex relation is the attachment of the vagina and urethra; in fact, the separation of these structures is the most difficult part of the operation. The vagina and urinary tract have a more extensive common wall such that the vagina surrounds the posterior urethra about 270 degrees, ultimately creating two cul-desacs on either side of the urethra (Fig. 89-29). The neourethra should be reconstructed using two layers of interrupted sutures (Fig. 89-30A). Then the vagina must be mobilized and placed behind the urethra so there are no facing suture lines (Fig. 89-30B). The vagina can be rotated 90 degrees to avoid this. Likewise, any damaged urethral wall should be repaired and again suture lines should not be in opposition. A perineal body should be created after the urethra and

vagina have been reconstructed, and the rectum should be situated within the limits of the sphincter and muscle complex, as previously described.

POSTOPERATIVE MANAGEMENT

Postoperative ARM children have little pain, except for those who undergo laparotomy. Patient-controlled anesthesia instead of epidural pain control is advisable. In cases of rectourethral or bladder neck fistula, the Foley catheter remains for 4 to 5 days. After a cloacal repair, the Foley catheter remains for 10 to 14 days. Suprapubic drainage may be indicated in cases of cloacas with a common channel longer than 3 cm.

Perioperative intravenous antibiotics are given for 2 to 3 days, and a topical antibiotic ointment can be used for the wound. Sitz baths are usually started by postoperative day 3 to 4.

Timing the Colostomy Closure

In general, anal dilatations are started 2 weeks after repair. The operating surgeon should perform the first one, then twice-daily dilatations should be performed by the family or health care worker. Each week the dilator can be advanced by 1 mm in caliber until the desired size is reached. Once the desired size is achieved, the stoma can be closed. Dilatations, however, must be continued twice daily until the dilator can be passed easily. Then dilatations proceed once daily for a month followed by every third day for the next month, twice a week for 1 month, once a week for 1 month, and finally once a month for 3 months. A systematic program of postoperative anal dilatation to avoid anal strictures cannot be overemphasized (Table 89-5). After closure of the colostomy, children may suffer from a diaper rash secondary to multiple daily bowel movements. A typical paste used for the diaper rash consists of vitamin A and D ointment, aloe, neomycin, Desitin, and Mylanta. Nystatin is added if the rash is the result of yeast. We encourage the use of a constipating diet to add bulk to the stool. The surgeon, however, must be cognizant of the fact that these children were born with no anus and some element of rectal atresia; thus, the major postoperative physiologic problem is a hypomotility disorder of the pulled-through bowel. In the unfortunate situation in which the rectum and colon were resected, hypermotility and intractable diarrhea may be the chief postoperative complaint.

Bowel Training Program We encourage families of children about 2 years of age with a good prognosis type of ARM to begin a bowel training program (BTP) of using the potty after each meal. If the family is unsuccessful in toilet training by the time the child is ready to attend school, then we recommend delaying the start of school and continuing with the BTP or beginning a bowel management program (BMP) for the temporary period of 1 year. We do not recommend that children with repaired ARMs be allowed to suffer in diapers at school while their peers are toilet trained.

Bowel Management Program The BMP is designed to keep artificially clean all those children who suffer temporary or permanent fecal incontinence. Therefore, the BMP is usually indicated in children with high defects and poor anatomy. Obviously, children with ARMs have a spectrum of defects, and thus, we should expect a spectrum of results regardless of how impeccable an operation. The basic principle of this program involves cleaning the colon once a day and keeping the colon quiescent (i.e., no motility or little motility) for 24 hours. This is conducted using enemas (Fleet) and colonic irrigations (via Foley catheter using Fleet enema or saline), as well as dietary manipulation and medications (i.e., Lomotil or Imodium). Once the child desires more independence, a Malone appendicostomy can be created (148,149,150,151).