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Anorectal Malformations

  • Andrea Bischoff
  • Belinda Hsi Dickie
  • Marc A. Levitt
  • Alberto PeñaEmail author
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Abstract

This is a brief presentation of the most common types of anorectal malformations. It was written for the practicing pediatrician. It meant to be a practical guide to identify and manage these pediatric problems from the newborn until adult life. It emphasizes the importance of suspecting the associated defects, as well as to try to avoid the most common errors observed in the management of these malformations. It also includes information that will allow the clinician to establish the functional prognosis for each specific types of defect.

The management of the sequelae is considered an important part of this chapter.

Keywords

Anorectal malformation Imperforate anus Cloaca 

Introduction

Anorectal malformations are serious congenital defects that present in the form of a wide spectrum, which can be treated with relatively easy surgical procedures and have good functional prognosis for bowel and urinary control. On the other extreme of the spectrum are complex anorectal and urogenital malformations that require sophisticated, technically demanding surgical procedures to be repaired and have rather poor functional prognosis for bowel, urinary, and sexual function.

It is rather regrettable that a patient born with a “good prognosis” type of defect ends up suffering from fecal and (or) urinary incontinence due to a deficient surgical care. That is the reason why in this chapter, emphasis is placed in the management of the “benign” group of anorectal malformations, which happens to include the greatest number of cases.

Incidence

Anorectal malformations occur approximately in 1 of 5000 live births (Brenner 1975). Its cause remains unknown and it seems to be multifactorial (Wang et al. 2015). Some types of anorectal malformation such as recto-perineal fistula and rectovestibular fistula occur more frequently in families (Falcone et al. 2007). In patients with Down syndrome, the most common defect found is imperforate anus without fistula, an anomaly that is uncommon in patients without Down syndrome (Torres et al. 1998).

The most common defect in females is rectovestibular fistula, whereas the most common defect in males is rectourethral fistula. Cloacal malformations are more common than formerly thought, most likely because they were previously misdiagnosed as rectovaginal fistulas (Rosen et al. 2002).

Classification

The classification system shown in Table 1 is anatomically descriptive with therapeutic and prognostic implications.
Table 1

Classification of anorectal malformations

Males

Females

Perineal fistula

Perineal fistula

Rectourethral fistula

Vestibular fistula

Bulbar

Persistent cloaca

Prostatic

≤3 cm common channel

Recto-bladder neck fistula

>3 cm common channel

Imperforate anus without fistula

Imperforate anus without fistula

Rectal atresia

Rectal atresia

Complex defects

Complex defects

Associated Malformations

Most babies (50–60%) with imperforate anus have one or more abnormalities that affect other systems (Smith and Saeki 1988; Cho et al. 2001).

Cardiovascular Anomalies

Cardiovascular anomalies are present in approximately a third of patients with imperforate anus, but only 10% require treatment in the author’s series. The most common lesions are atrial septal defect and patent ductus arteriosus, followed by ventricular septal defect and tetralogy of Fallot. The literature (Greenwood et al. 1975; Teixeira et al. 1983) mentions an incidence of 12–22%, but about one third of the cases were hemodynamically unstable.

Gastrointestinal Anomalies

Malformations of the gastrointestinal tract have been described occurring in 15–18% of cases of ARM (8–9). Esophageal atresia has been reported in 7–15% of cases of ARM (Casaccia et al. 2009; Shin et al. 2013; Hassink et al. 1996) and duodenal atresia in 3–4% (Casaccia et al. 2009; Hassink et al. 1996). Hirschsprung’s disease has been found in three patients in the author’s series of 2100 cases, and overdiagnosis of this association is probably due to the high incidence of constipation seen in patients with anorectal malformations.

Sacral Anomalies

Sacral abnormalities are very common and have significant implications. The quality of the sacrum seems to have great influence on the functional prognosis for bowel and urinary control. A sacral ratio has been created to try to quantify the sacral defect (Fig. 1) and to correlate with bowel and urinary function. A sacral ratio higher than 0.7 is considered normal. A sacral ratio lower than 0.4–0.7 has a rather mild influence on bowel control.
Fig. 1

Anterior-posterior (a) and lateral (b) sacral ratios

Spinal Cord Anomalies

Approximately 25% of patients with ARM suffer from tethered cord (Levitt et al. 1997a). The presence of the defect seems to have more influence on the urinary function and the motor activity of the lower extremities. The diagnosis of tethered cord can be done with a lumbosacral ultrasound during the first 3 months of life. After that, the diagnosis can only be made with an MRI of the spine (van den Hondel et al. 2016).

Vertebral

Hemivertebrae are commonly seen. The implication of these on bowel and urinary control has not been determined. However, they have important orthopedic implications (scoliosis).

Genitourinary Anomalies

Improved imaging has shown an increasing range of upper and lower urinary tract anomalies (Rich et al. 1988). The incidence increases with increasing complexity of the anorectal defect. Vesicoureteral reflux is the most common functional anomaly found, and renal agenesis and dysplasia is the most frequent malformation.

Gynecologic Anomalies

Gynecologic anomalies are a significant concern in patients with anorectal malformations (Levitt et al. 1998). In the newborn period, hydrocolpos can lead to urinary obstruction or can cause pyocolpos, in patients with cloacas (Bischoff et al. 2010).

Müllerian anomalies may manifest later when teenagers have obstruction of menstrual flow. Large intra-abdominal collections and peritonitis can develop, or patients may have amenorrhea because of the absence of the Müllerian structures. Obstruction to menstrual flow can occur as an atresia at any level, such as the tube, the cervix, or a hemivagina. Uterine septation abnormalities (predominantly bicornuate uterus) vaginal septum and associated vaginal atresia can also occur. During the definitive repair or at the time of colostomy closure, the intra-abdominal gynecologic structures must be inspected and its patency tested. The full obstetric impact of these anomalies is not yet known.

Anorectal Anatomy and Pathophysiology

Bowel control is the result of a complex interplay among sphincter function, anorectal sensation, and colonic motility. All these factors are affected in children with anorectal malformations.

Sphincter Mechanism

The sphincter mechanism is represented by a funnel-like structure. The exposure afforded by the posterior sagittal approach shows that the levator musculature continues down to the anal dimple with vertical striated muscle fibers called the “muscle complex.” Electrical stimulation of the upper end of the levator group pushes the rectum forward. Stimulation of the muscle complex (vertical fibers) elevates the anus, and stimulation of the parasagittal fibers closes the anus (Peña 1989). Children with anorectal malformations have varying degrees of striated sphincter muscle development from almost normal to virtually no muscle at all.

Sensation and Proprioception

Under normal circumstances, the anal canal is an exquisitely sensitive area (Duthie and Gairns 1960). It allows the individual to discriminate solids, liquids, and gas. The overwhelming majorities of children with anorectal malformations are born without an anal canal and therefore lack this kind of sensation. There is, however, proprioception, which is described as a vague feeling that is perceived when the rectum is distended, simultaneous with stretching of the voluntary muscle that surrounds the rectum (Skerritt et al. 2016). The clinical implications are that these patients lose control when they suffer an episode of diarrhea, but may have the ability to be toilet trained when they can form solid stool and can learn to perceive it.

Colonic and Rectosigmoid Motility

The intestinal contents reach the cecum in a liquid state. It then takes about 20–24 h for that liquid fecal material to reach the rectum and become formed stool as a consequence of the absorption of water that occurs in the colon. The rectosigmoid acts as a reservoir and holds the fecal material for a variable period of time usually about 24 h. The anal canal (below the pectinate line) is usually empty because of the action of the surrounding sphincteric mechanism. Peristaltic waves in the colon push the fecal material toward the anus and touch the exquisitely sensitive tissue of the anal canal, thereby providing valuable information related to the nature of the rectal contents (solid, liquid, or gas). Depending on the surrounding social circumstances, the individual may contract the sphincteric mechanism to avoid a bowel movement and then voluntarily relax at the appropriate time. Normal defecation allows massive emptying of the rectosigmoid, followed by another resting period of about 24 h, during which the rectosigmoid again acts as a reservoir.

Children with anorectal malformations suffer from rectosigmoid hypomotility of different severity. This results into constipation of different degrees, which is self-perpetuating and self-aggravating to the point that if left untreated, megasigmoid develops. In extreme cases, fecal impaction and encopresis, or overflow pseudoincontinence, may develop. It seems that constipation is worse with lower defects. Constipation should not be underestimated and must be treated aggressively.

Children with anorectal malformations, who have lost their rectosigmoid, suffer from the opposite problem (i.e., tendency to have diarrhea). These children have no reservoir capacity, are highly sensitive to certain foods, and suffer from incontinence.

Clinical Findings and Initial Management

Figures 2 and 3 show the decision-making algorithm for the initial management of male and female patients (van der Steeg et al. 2015).
Fig. 2

Algorithm for the treatment of a male newborn with an anorectal malformation

Fig. 3

Algorithm for the treatment of a female newborn with an anorectal malformation

A clinician called to see a newborn with an anorectal malformation must perform a thorough perineal inspection, which usually provides the most important clues about the type of malformation that the patient has (Figs. 4 and 5). It is important to not make a decision about a colostomy or a primary surgery before 20–24 h of age. The reason for waiting is that significant intraluminal pressure is required for the meconium to be forced through a fistula, which is the most valuable sign of the location of distal rectum in these babies. If meconium is seen on the perineum, it is evidence of a recto-perineal fistula. If there is meconium in the urine, the diagnosis of a rectourinary fistula is obvious. If a single orifice is diagnosed during the perineal inspection of a female, the diagnosis of a cloaca is made.
Fig. 4

External appearance of the perineum of male patients with anorectal malformations. (a) Perineal fistula with “bucket handle”, prone position. (b) Rectourethral bulbar fistula, prone position. (c) Flat bottom, patient in supine position

Fig. 5

External appearance of the perineum of female patients with anorectal malformations (a) Perineal fistula. (b) Vestibular fistula. (c) Cloaca

Radiologic evaluations do not show the real anatomy before 24 h because the rectum is collapsed by the muscle tone of the sphincters that surround its lower part. Therefore, radiologic evaluations done too early (before 24 h) will likely reveal a “very high rectum” and therefore yield a false diagnosis.

During the first 24 h, the newborn should receive intravenous fluids, antibiotics, and nasogastric decompression to prevent aspiration. The clinician should use these hours to evaluate for the presence of associated defects such as cardiac malformations, esophageal atresia, and urologic problems. An echocardiogram can be performed, and the baby should be checked for the presence of esophageal atresia. A plain radiograph of the lumbar spine and sacrum should be taken to evaluate for hemivertebrae and sacral anomalies. A spinal ultrasound helps screen for tethered cord. Ultrasonography of the abdomen evaluates for the presence of hydronephrosis, and pelvic ultrasonography in females with cloaca evaluates for the presence of hydrocolpos. During opening of the colostomy, it is mandatory that the hydrocolpos be drained when present. If the hydrocolpos is not large enough to reach the abdominal wall above the bladder, to be sutured to the skin, it can be drained with a pigtail tube.

If the baby has signs of a perineal or vestibular fistula, a posterior sagittal repair can be performed without a protective colostomy in the newborn period, provided the surgeon has experience with that kind of procedure. If the baby is ill from associated problems, or premature, or if the clinician chooses to wait until the baby is a little older, the fistula can be gently dilated. The repair in such cases should not be delayed more than several months.

After 24 h, if no meconium is seen on the perineum or in the urine, a cross-table lateral X-ray film with the baby in prone position should be obtained. If the gas in the rectum is located below the coccyx (Fig. 6) and the baby is in good condition with no significant associated defects, depending on the surgeon’s experience, a posterior sagittal operation without a protective colostomy can be considered. A more conservative alternative would be to perform a colostomy, with the definitive repair planned for a second stage. If rectal gas is seen above the coccyx (Fig. 7) or the patient has meconium in the urine, single perineal orifice (cloaca), significant associated defects, and/or an abnormal sacrum or a flat bottom, a colostomy is recommended with postponement of the main repair for a subsequent operation. This can be performed 2–3 months later after a distal colostogram delineating the anatomy is performed, provided that the baby is gaining weight normally.
Fig. 6

Cross-table lateral X-ray, reachable rectum

Fig. 7

Cross-table lateral X-ray, high rectum

Performing the definitive repair not later than at 2–3 months of age has important advantages for the patient, including less time with an abdominal stoma, less size discrepancy between the proximal and distal bowel at the time of colostomy closure, easier anal dilation, and no recognizable psychological sequelae from painful perineal maneuvers.

The potential advantages of an early operation must be weighed against the possible disadvantages of an inexperienced surgeon unfamiliar with the minute anatomic structures of an infant’s pelvis.

A trend to repair these defects without a protective colostomy (Albanese et al. 1999) must be balanced against the concern that such a repair without a colostomy is done without precise anatomic information about the patient’s specific type of anorectal defect. The most catastrophic complications occurred in patients operated on without a colostomy and, as a consequence, without a distal colostogram. While looking for the rectum, the surgeon might inadvertently find and injure the urethra, an ectopic ureter, the bladder neck, the vas deferens, or the seminal vesicles (Hong et al. 2002).

With emerging advancements in perinatology and prenatal ultrasound techniques, anorectal malformations are more commonly being suspected (Bianchi et al. 2013). Prenatal sonographic findings, such as a dilated rectum, hydrocolpos, or demonstration of an associated urological and spinal anomaly, can make the primatologist suspicious that the fetus may in fact have an anorectal malformation.

Limited Posterior Sagittal Anorectoplasty

When a perineal fistula is diagnosed (Fig. 5), the anal orifice is always located anterior to the sphincter mechanism. A limited posterior sagittal anoplasty can be performed in the newborn period. It is advantageous to perform this operation in the first 48 h of life because the meconium is sterile. When such an operation is delayed and performed at several months of life, a complete bowel preparation, including a postoperative period of parenteral nutrition without oral intake, is recommended to reduce the risk of perineal infection.

The baby is placed prone. In males, a urinary catheter is inserted. The distal end of the rectum is intimately attached to the posterior urethra, and urethral injury must be avoided. In females, the clinician must acknowledge an intimal contact of the rectum with the vagina, in cases of perineal fistula, and a thin common wall between rectum and vagina in cases of vestibular fistula. Multiple 6-0 silk sutures are placed at the mucocutaneous junction around the fistula orifice. The posterior sagittal incision divides the posterior sphincter in half and is continued circumferentially around the fistula. While traction is maintained on the bowel, a circumferential dissection is performed to mobilize and reposition the bowel within the limits of the sphincter. The bowel wall is sutured to the skin with fine, absorbable sutures under slight tension. The perineal body is reconstructed.

Colostomy

Except for patients with perineal fistula, a colostomy is usually performed as a first stage in the majority of cases (Fig. 8) (Peña et al. 2006).
Fig. 8

Descending colostomy

(See chapter on “Stomas of the Small and Large Bowel”.)

Management After Colostomy (High-Pressure Distal Colostogram)

Before proceeding with definitive reconstruction, the anatomy of the anorectal malformation is delineated by high-pressure distal colostography (Gross et al. 1991) (Figs. 9 and 10). This study is by far the most valuable radiologic test to determine for specific type of anomaly and best surgical approach. The high-pressure distal colostogram requires the use of a Foley catheter inserted through the mucous fistula of the colostomy. The balloon must be inflated and the radiologist must pull on the catheter to allow the balloon to act as a plug to avoid the leakage of contrast material. Water soluble contrast material must be injected with a syringe, with enough pressure to overcome the tone of the voluntary muscles that surround the lower rectum and to force the contrast through the fistula. The first films must be taken in AP position to show the length of the bowel available for the pull through and then the patient is placed in perfect lateral position with a lead marker in the anal dimple to show the fistula.
Fig. 9

Distal colostogram showing a rectourethral bulbar fistula

Fig. 10

Distal colostogram showing a recto-bladder neck fistula

Anorectal Reconstruction

Basic Principles

All defects can be repaired using a posterior sagittal approach. Approximately 10% of male patients (those with a rectum–bladder neck fistula) and about 40% of female patients with a cloaca may in addition require an abdominal approach for mobilization of a high rectum or vagina. A Foley catheter is inserted into the bladder. The catheter sometimes goes into the rectum through the fistula rather than into the bladder. To avoid this, the surgeon can direct the catheter with a lacrimal probe inserted in the tip of the Foley catheter to function as a guide. Alternatively, a “Coudee” catheter can be used. Occasionally, the catheter must be positioned intraoperatively once the fistula is exposed. The patient is placed in prone position with the pelvis elevated.

Special care must be taken to cushion all the pressure areas of the baby’s body (Fig. 11). Two small bolsters are used in front of each deltopectoral groove to avoid hyperextension of the neck and shoulders. Electrical stimulation of the perineum allows evaluation of the strength of the sphincter contraction. A midline incision is made, and the sphincteric mechanism is divided exactly in the midline, leaving equal amounts of muscle on each side. Sharp Weitlaner retractors are used to achieve good exposure.
Fig. 11

Patient in prone position with proper padding

Posterior Sagittal Anorectoplasty for Anomalies in Males

Rectourethral Fistula (Bulbar and Prostatic)

The incision usually extends from the lower portion of the sacrum through the center of the anal dimple and sometimes extended to the perineal body (Fig. 12). Below the skin and running parallel to the midline are the parasagittal muscle fibers. Medial to these muscle structures and located within the limits of the anal dimple are other muscle structures that run perpendicular to the parasagittal fibers. These structures extend from the skin to the levator muscle and are called the muscle complex. Deeper to the parasagittal fibers, the ischiorectal fat becomes evident, and below this is the levator, which is also divided in the midline. The crossing of the muscle complex fibers with the parasagittal muscle structures defines the anterior and posterior limits of the new anus. These limits can be determined most clearly with the use of an electrical stimulator.
Fig. 12

Essential steps for posterior sagittal anorectoplasty in male patients. (a) Planned posterior sagittal incision. (b) Posterior sagittal approach with the parasagittal fibers and ischiorectal fat split in the midline. (c) Posterior rectal wall exposed. (d) Posterior rectal wall opened in the midline. (e) Posterior rectal wall opened going anteriorly until the rectourethral fistula is identified. (f) Separation of the rectum from the posterior urethra with dissection above the fistula. (g) The rectum fully mobilized and in this case tapered. Sutures are placed anteriorly to close the perineal body. (h) The rectum pulled through and placed within the limits of the sphincter mechanism. (i) Closure of the levator and tacking of the posterior edge of muscle complex to the posterior rectal wall. (j) Closure of the posterior sagittal incision and completed anoplasty

In boys with a rectourethral bulbar fistula, upon opening the levator muscle, the bowel is very evident. In boys with a rectoprostatic fistula, the rectum is much smaller and located much higher in the incision just under the coccyx. The distal colostogram provides information that is valuable at this point for determining where the rectum can be found (Fig. 9). In the case of a recto-bladder neck fistula, the rectum is not visible through a posterior sagittal approach and should not be searched for (Fig. 10).

Temporary silk sutures are placed in the posterior rectal wall for traction, and the rectum is opened exactly in the midline. The incision in the rectal wall is extended distally. As the rectum is being opened, more sutures are placed to hold the edges of the rectal wall and improve the exposure of the rectal lumen. The incision must be extended distally until the fistula site is found (Fig. 13).
Fig. 13

Operative view, posterior sagittal approach. The rectourethral fistula is seen with a metallic probe in it

Because there is no distinct plane of separation between the rectum and urethra, the surgeon must be extremely careful while separating the rectum from the urinary tract. Multiple 6-0 silk sutures are placed above the fistula site in a semicircular fashion (Fig. 14). These sutures allow the surgeon to exert uniform traction on the rectum while dissecting and separating it from the urethra. Dissection of the lateral aspects of the rectum first helps to delineate the anterior plane. The dissection then proceeds in the submucosal plane, until the rectum and urethra gradually separate and become independent.
Fig. 14

Operative view. Fine silk sutures are placed through the mucosa of the cephalad hemicircumference of the fistula

Once the rectum is fully separated from the urinary tract, a circumferential perirectal dissection is performed to gain enough rectal length to reach the perineum. To achieve this, the fascia that surrounds the rectum must be divided, including blood vessels and nerves attached to the rectum. This is a key plane to identify. While the surgeon is applying traction to the rectum, the dissection continues in a circumferential manner. The vessels that hold the rectum are coagulated and divided until enough rectal length has been achieved. The rectum has an excellent intramural blood supply; even in cases of a very high prostatic fistula, sufficient length can be obtained without making the rectum ischemic. When the rectal wall is injured, however, this blood supply is damaged and ischemia may occur. Therefore, every effort must be made to perform this dissection as close as possible to the rectal wall but without damaging the bowel wall. Once enough length has been achieved to perform a tension-free bowel-to-skin anastomosis, the size of the rectum must be evaluated and compared with the available space. If necessary, the rectum can be tapered by removing part of its posterior wall and reconstructed by closing with two layers of long-term absorbable interrupted sutures. The urethral fistula is closed with interrupted absorbable sutures.

The perineal body is reconstructed by bringing together the anterior limits of the external sphincter, which was previously marked with temporary silk sutures. The levator muscle is sutured behind the rectum (Fig. 15), and the posterior edges of the muscle complex are sutured together in the midline while taking, with the same stitches, part of the posterior rectal wall to anchor the rectum. This helps to prevent prolapse (Fig. 16). The ischiorectal fossa and the subcutaneous tissue are then reapproximated, and the skin closed. The anoplasty is performed by placing the rectum within the limits of the sphincter with the use of interrupted long-term absorbable sutures (Fig. 17).
Fig. 15

Levator muscle sutured behind the rectum

Fig. 16

Sutures on the posterior edge of the muscle complex which anchor the rectum

Fig. 17

Anoplasty

Recto-bladder Neck Fistula

In patients with a recto-bladder neck fistula, an abdominal approach via either laparoscopy or laparotomy, in addition to the posterior sagittal approach, is necessary to mobilize the rectum.

Before beginning this operation, the entire body surface from chest to toes is prepared so that the surgeon can work simultaneously in the abdomen and the perineum. The abdomen is entered via either laparoscopy or laparotomy (Fig. 18a, b). The sigmoid is identified and dissected down to the bladder neck. It is very important to remember that the vas deferens and ureters run very close to the rectum; thus the dissection must be carried out as close as possible to the rectal wall. The rectum narrows very rapidly and opens into the bladder neck in a T-shaped manner. The rectum is divided at its most distal part, and the fistula is closed with absorbable sutures.
Fig. 18

Operative view of a recto-bladder neck fistula showing the rectosigmoid ending at the bladder neck. (a) Laparoscopic view. (b) View via laparotomy

The challenge, thereafter, is often the mobilization to gain adequate rectal length to reach the perineum (Bischoff et al. 2011, 2013a). The mobilization of the rectum is limited by branches of the inferior mesenteric vessels. To gain length surgeons have traditionally been advised to divide the inferior mesenteric vessels at their origin from the aorta. This maneuver is contraindicated here, since this is often the only blood supply of the rectosigmoid because the opening of the colostomy may have interfered with the continuity of the arcade that connects the middle colic vessels with the inferior mesenteric vessels. Therefore, meticulous ligation of peripheral branches of the inferior mesenteric branches must be performed being sure to leave at least two good vessels coming directly from the inferior mesenteric (Fig. 19).
Fig. 19

Ligation of the peripheral branches of the inferior mesenteric vessels with preservation of the main trunks, in order to gain length on a very high rectum

With the legs lifted up, a midsagittal incision is made and the presacral space dissected. Then the rectum is pulled through. An anoplasty should be performed as previously described. Tacking of the rectum to the muscle complex is important because these patients have poor pelvic musculature and are particularly prone to prolapse.

Imperforate Anus Without a Fistula

Anorectal malfromations (ARMs) without fistula occur in approximately 5% of all cases of ARMs. Bischoff et al. (Bischoff et al. 2014) in a series of 2186 cases of ARMs found 92 (4.2%) patients had no fistula. Thirty-seven (40%) patients had trisomy 21. In patients with an imperforate anus and no fistula, the blind end of the rectum is located at the same level as in a patient with a bulbar urethral fistula. Even when patients do not have a fistula, the rectum is still intimately attached to the posterior urethra in males or vagina in females, requiring careful dissection. The rest of the repair is performed as described for rectourethral fistulas.

Rectal Atresia and Stenosis (Hamrick et al. 2012)

Repair of rectal atresia and stenosis involves connecting the blind dilated end of the rectum proximally with the anal canal distally. Both structures are usually separated by a few millimeters of fibrous tissue. The rectum must be sufficiently mobilized to allow the performance of an end-to-end anastomosis to the anal canal. The wound is then closed by reconstructing all the muscle structures, as previously described. Since the anal canal is normal, these patients have excellent potential for bowel control. This defect in particular is frequently associated with a presacral mass, often a teratoma, which must be screened for.

Posterior Sagittal Anorectoplasty for Anomalies in Females

Vestibular Fistula

The complexity of this defect is frequently underestimated. Patients with a vestibular fistula are born with excellent potential for bowel control. Thus, every effort should be made to give these patients the best opportunity to undergo a successful reconstruction with a single operation. A protective colostomy minimizes the chances of these complications.

With the patient in prone position, a midline incision is performed. The midline incision continues around the fistula into the vestibule, and multiple 5-0 sutures are placed circumferentially at the fistula site. While traction is placed on these sutures, the rectum is dissected in a circumferential manner. The posterior rectal wall can easily be identified, and the dissection must start from the posterior aspect and be extended laterally. The last step, separation of the rectum from the vagina, is the most delicate part of the dissection. The common wall between the rectum and vagina in this kind of defect is long and extremely thin. Once fully separated, the rectum must be mobilized as previously described, to gain enough length to perform a tension-free bowel-to-skin anastomosis. The limits of the sphincteric mechanism are electrically determined and marked with temporary silk sutures. The perineal body is then reconstructed by bringing together the anterior limit of the sphincter complex. The posterior edges of the muscle complex are reapproximated taking bites of rectal wall, as described previously for males. The anoplasty and wound closure are performed as described for males. For the extremely unusual case of a rectovaginal fistula, the technique is the same except that the rectum requires more mobilization to reach the perineum.

Cloaca

Cloaca represents a spectrum of defects that goes from “benign” cloaca with a good functional prognosis that can be repaired with a relatively simple surgical technique, to very complex malformations with many anatomic variations that require different surgical maneuvers to be able to successfully reconstruct those patients (Bischoff 2015).

The group of patients born with a “benign” type of cloaca will have bowel and urinary control, will become sexually active, and may get pregnant and deliver by cesarean section. All this is possible, provided the malformation is repaired with a meticulous and delicate technique. Fortunately, this represents more than 50% of all cloacas. On the other hand, complex cloaca with a common channel longer than 3 cm should be repaired by surgeons fully dedicated to repair these malformations. The length of the common channel is better determined during cystoscopy.

Cloacas with a Common Channel Shorter than 1 cm

These patients are treated almost like those that are born with a recto-vestibular fistula (Bischoff 2016). They do not require total urogenital mobilization as the urethra is perfectly visible in its natural location, even when it is a little hypospadic. The first step consists of approaching the patient through a posterior sagittal incision, identifying the posterior wall of the rectum, clearing the posterior and lateral walls of the rectum, and then performing a very meticulous dissection to separate the anterior rectal wall from the posterior vaginal wall. Once the separation is completed and the rectum reaches the center of the sphincter mechanism without tension, the posterior and lateral walls of the vagina are mobilized in order to create an adequate introitus. The limits of the sphincter are delineated with the use of an electrostimulator and the perineal body is created. An anoplasty is performed.

Cloacas with a Common Channel Length Between 1 and 3 cm

Patients with a common channel length shorter than 3 cm can be repaired through a posterior sagittal approach. The rectum is opened in the midline and silk stiches are placed along the edges of the posterior rectal wall. The entire common channel is exposed to allow confirmation of its length under direct vision (Fig. 20). After the separation of the rectum from the vagina, the surgeon should perform a maneuver called total urogenital mobilization. To accomplish this, the posterior wall of the vagina and the entire common channel is divided to exposes the urethral and vaginal openings. Multiple 5-0 silk stitches are placed taking the edges of the vagina and common channel. Another series of stitches are placed in a horizontal manner, 5 mm proximal to the clitoris in order to provide uniform traction (Fig. 21). With the use of a needle tip cautery, the urogenital sinus is divided between the clitoris and the traction sutures. Dissection is performed between the anterior wall of the urogenital sinus and posterior aspect of the pubis. Between these two structures, there is a natural plane, mostly avascular. The dissection must be carried up until the upper edge of the pubis is identified. At this point, it is easy to identify a whitish thin membrane located between the upper edge of the pubis, the urogenital sinus, and the lateral walls of the vagina. We call this “suspensory ligaments of the urethra and vagina”. During the division of these ligaments, the surgeon should try to avoid the venous plexus located right behind the pubis. In most cases, the total urogenital mobilization is sufficient for the urethra and vagina to reach the perineum together without tension. In some cases, the posterior vaginal walls still require some extra mobilization. The urethral meatus is then sutured to the tissue behind the clitoris with multiple interrupted 6-0 vicril sutures. The lateral walls of the vagina are also sutured to the perineum and the limits of the sphincter are delineated with the use of the electrostimulator to help and determine the size of the introitus and perineal body. The anoplasty is then performed.
Fig. 20

(a) Posterior sagittal view of a typical cloaca with a common channel of 2 cm with a visible vaginal septum. (b) A cloaca with a very large vagina (hydrocolpos). The rectum opens higher in the vagina and is not seen here

Fig. 21

(a) Total urogenital mobilization (cloaca with a short common channel, <3 cm). (b) The intrinsic anatomy of the cloaca is exposed posterior sagittally. (c) The rectum is separated from the urogenital sinus. (d) Sutures are placed around the urogenital complex for uniform traction to facilitate mobilization of the urogenital sinus. (e) Urogenital sinus fully mobilized. (f) Finished repair

Cloacas with a Common Channel Longer than 3 cm

When endoscopy and a cloacagram show that the patient has a long common channel, the surgeon must be prepared to face several significant technical challenges. A more precise anatomic diagnosis can be achieved with the use of a 3-D rotational scan (Fig. 22).
Fig. 22

3D cloacagram

The repair of these complex defects usually starts with a posterior sagittal approach, separation of the rectum as previously described, and a total urogenital mobilization. Occasionally the surgeon may find that it is possible to bring the urethra and the vagina in cases with a common channel of 4 or 4.5 cm.

In the event that the total urogenital mobilization was not enough to perform an adequate urethral and vaginal repair, the next step should be a laparotomy to divide all the avascular attachments of the vagina and urethra. If that is not enough, it will be necessary to separate the bladder and urethra from the vagina(s). This is performed through the abdomen, with the bladder open, and catheters placed through the ureters. This separation represents the most technically demanding and sophisticated maneuver in the repair of cloacas; it requires experience and delicate technique.

Once the vaginas have been separated from the bladder, the surgeon must try and see if the vagina reaches the perineum. If the answer is no, then the patient will need either a vaginal replacement that can be done with rectum, colon, or small bowel, depending on the specific anatomic circumstances of the patient. The vaginal switch maneuver (Bischoff et al. 2013b) is only applicable in cases with two very large hemivaginas, with widely separated cervices, located very high in the pelvis. The surgeon may find that the distance between on the cervix and the other is longer than the vertical length of both hemivaginas. Under those circumstances one of the cervices can be resected, the vaginal septum resected, both hemivaginas are tabularized as a single vagina, and what used to be the dome of one of the hemivaginas is switched down to the perineum (Fig. 23).
Fig. 23

(a) Very large hemivaginas with a vaginal septum and long common channel. (b) Vaginal switch maneuver

Patients with a long common channel should receive a total-body preparation because it is likely that a laparotomy will be required. The presence of a very long common channel (more than 5 cm) indicates that there is no way that total urogenital mobilization will be sufficient to repair the malformation, and therefore it is advisable to leave the common channel in place, which can be used as urethra for intermittent catheterization. In this situation, the vaginas are usually connected to the bladder neck (Fig. 24), and therefore it is easier to separate them from the urinary tract through the abdomen. Once the vaginas have been separated, a partial vaginal replacement is performed. This can be done using the rectum (Fig. 25a, b), colon (Fig. 26), or small bowel (Fig. 27a–c).
Fig. 24

Extremely long common channel – hemivaginas and rectum connected to the bladder neck

Fig. 25

A and B, vaginal replacement with the rectum

Fig. 26

Vaginal replacement with the sigmoid colon

Fig. 27

Vaginal replacement with small bowel (a) and (b). Using the portion of ileum with the longest mesentery. (c) Pulling the small bowel down as a neovagina (the insert shows an anastomosis to the upper part of the vagina)

Once in the abdomen, either during the main repair or at the time of colostomy closure, the patency of the Müllerian structures is investigated by passing a No. 3 feeding tube through the fimbriae of the fallopian tubes, injecting saline, and observing the saline solution to come out through the vagina. If one of the systems is not patent, the atretic Müllerian structure should be excised, with great care to avoid damage to the blood supply of the ipsilateral ovary. When both Müllerian structures are atretic, they should be left in place. The patient must be monitored closely and further investigated with ultrasound when she reaches puberty.

After the repair of a cloaca, in cases with a common channel shorter than 3 cm, a Foley catheter is left in place for a period of 2–3 weeks. In cases of longer common channels, a suprapubic tube is left if the surgeons feel that the patient will require such diversion for less than 3 months. In complex reconstructions, a vesicostomy is recommended, particularly in cases with vesicoureteral reflux and (or) hydronephrosis. Later on, the bladder function should be evaluated to determine the possible urologic strategy to follow.

At the time of colostomy closure, endoscopy should be performed to be sure that the repair is intact, that there is no prolapse, stricture, or urethrovaginal fistula. If the cloaca repair did not require a laparotomy, the time of colostomy closure is the opportunity to investigate the patency of the Müllerian structures.

General Principles of Postoperative Care

In the absence of a laparotomy, oral feedings may begin when the child is awake. Antibiotics are given for 48 h. In males who had a rectourethral fistula, the urinary catheter should be left in place for 7 days.

An anal dilatation program is begun 2 weeks after surgery. The anus is calibrated and a dilator that fits snugly is initially used to dilate the anus twice a day. Every week, the size of the dilator is increased by one unit until the desired size is reached. The optimal size of dilator is shown in Table 2. Once the correct size is reached, the colostomy can be closed which is usually 8–12 weeks after the reconstruction.
Table 2

Anal dilatation program

Patient age

Hegar dilator

1–4 months

Size no. 12

4–8 months

Size no. 13

8–12 months

Size no. 14

1–3 years

Size no. 15

3–12 years

Size no. 16

>12 years

Size no. 17

Dilatations must continue after colostomy closure. Once the dilator can be inserted easily, the frequency of dilations is reduced to once a day for 1 month, twice a week for 1 month, once a week for 1 month, and then once a week for 3 months.

After the colostomy is closed, the patient may have multiple bowel movements and perineal excoriation may develop. A constipating diet may be helpful in the treatment of this problem. After several weeks the number of bowel movements decreases, and most patients will suffer from constipation. This constipation must be watched for and proactively treated to avoid the formation of megasigmoid and overflow pseudoincontinence (Peña and el Behery 1993). After 3–6 months, a more regular bowel movement pattern develops. A patient who has 1–3 bowel movements per day remains clean between bowel movements, shows evidence of “feeling” during the bowel movement and pushes, and generally has a good prognosis. This type of patient is trainable. A patient with multiple bowel movements or one who passes stool constantly without showing any signs of sensation or pushing typically has a poor functional prognosis for bowel control. The original type of malformation, as well as the quality of the sacrum and spine, predicts the potential for voluntary bowel movements.

Outcomes

Complications

All the usual postoperative complications may occur after these operations. In addition, some specific problems develop both early and late in the postoperative course.

Wound infection and retraction are known to occur. When the infection affects only the superficial layers of the wound, is not accompanied by dehiscence of the structures pulled through, and is promptly treated, it is likely that no functional sequelae will result. On the other hand, infection accompanied by dehiscence may reach catastrophic proportions and leave sequelae that include incontinence, strictures, acquired atresias, recurrent fistulas, and severe pelvic fibrosis.

Infections and dehiscence have occurred mainly in patients operated on primarily, without a colostomy. Therefore, although there is the benefit to operate earlier, primarily, and without a colostomy, it must be remembered that a colostomy is a very valuable adjunct in the management of these defects. Every surgeon must make a decision regarding this issue based on his personal experience.

It is very difficult to determine the precise causes of these complications; however, it seems the main contributing factors are fecal contamination, ischemia, and suture line tension, (often from the incomplete mobilization of structures).

Rectal or vaginal strictures (or both) are usually due to ischemia and (or) tension.

An anal dilatation program is recommended to avoid strictures; however, these maneuvers prevent only minor, ringlike strictures. Difficult anal dilatations usually reflect a major problem related to ischemia or tension that will result in a long narrow stricture or even acquired atresia.

Rectal mucosal prolapse may occur in less than 5% of cases; to prevent this from happening, several maneuvers are recommended, including: (A) tacking of the posterior rectal wall to the posterior edge of the muscle complex, (B) tapering a dilated rectum if necessary, and (C) performing the anoplasty under slight tension so that after the sutures of the anoplasty are cut, the rectum retracts slightly with no mucosa being visible.

Prolapse is managed by full-thickness trimming and is performed when prolapse causes excess mucus production and bleeding or interferes with the patient(s) quality of life.

A review revealed significant urologic injuries in male patients who underwent repair of anorectal malformations (Hong et al. 2002). The posterior sagittal approach, when performed without a good preoperative distal colostogram, was the most important source of these complications. Urethral, ureteral, vas deferens, and seminal vesicle injuries can occur. The laparoscopic approach when done for malformations in which the rectum ends at the bulbar urethra risks leaving behind a posterior urethral diverticulum (the original distal rectum). Postoperative neurogenic bladder in male patients who undergo a technically correct operation for the treatment of anorectal malformations must be extremely unusual.

Constipation is the most common sequel after surgical repair of anorectal malformations. The lower the malformation, the more likely the development of constipation. A vicious cycle ensues with megarectosigmoid leading to more constipation. Constipation that is not properly managed will lead to more megarectosigmoid, resulting in overflow pseudoincontinence. Patients appear to be incontinent, but if their constipation is managed appropriately, they become continent. Such patients sometimes benefit from sigmoid resection to reduce their laxative requirement (Peña and el Behery 1993).

Every effort must be made to avoid this cycle and maintain a collapsed and clean bowel from the moment the baby is born. Transverse colostomies left for a long period of time lead to severe megarectosigmoid. Loop colostomies may also contribute to distal fecal impaction, dilation, and subsequent constipation. An adequate treatment of constipation starting after colostomy closure is very important.

Continence

Bowel control is achieved in about 75% of patients.

Results according to the type of abnormality are shown in Tables 3, 4, and 5. Patients with low anomalies have done extremely well; those with high lesions and those with associated spinal or sacral problems have done less well.
Table 3

Voluntary bowel movement (VBM) and type of defect

Defect

Cases

Patients with VBMs

n

%

Atresia or stenosis

11

11

100

Perineal fistula

58

56

97

Vestibular fistula

146

131

90

Imperforate anus without fistula

40

31

78

Bulbar fistula

112

89

79

Cloaca common channel ≤3 cm

99

65

66

Prostatic fistula

109

71

65

Vaginal fistula

5

3

60

Cloaca common channel >3 cm

69

24

35

Bladder neck fistula

49

10

20

Total

698

491

70

Table 4

Soiling and type of defect

Defect

Patients with soiling

Cases

n

%

Perineal fistula

57

9

16

Vestibular fistula

135

49

36

Atresia or stenosis

10

4

40

Bulbar fistula

105

51

49

Imperforate anus without fistula

39

20

51

Cloaca common channel ≤3 cm

91

57

63

Prostatic fistula

110

86

78

Vaginal fistula

5

4

80

Cloaca common channel >3 cm

55

46

84

Bladder neck fistula

48

43

90

Total

655

369

56

Table 5

Totally continenta patients and type of defect

Defect

Cases

Totally continent

N

%

Perineal fistula

52

43

83

Atresia or stenosis

9

5

56

Vestibular fistula

135

86

64

Imperforate anus without fistula

36

18

50

Bulbar fistula

101

46

46

Cloaca common channel ≤3 cm

91

32

35

Vaginal fistula

5

1

20

Prostatic fistula

105

19

18

Cloaca common channel >3 cm

52

6

12

Bladder neck fistula

46

3

7

Total

632

259

41

aVoluntary bowel movements and no soiling

All patients with anorectal malformations, regardless of the complexity of the defect, can be kept completely dry of urine and clean of stool after the age of 3, either because they achieve bowel and urinary control or because a bowel management program is implemented to keep them artificially dry and clean, with the use of enemas. This program is used in the 25% group of patients that suffer from fecal incontinence (Bischoff and Tovilla In press; Bischoff et al. 2009; Peña 1990). With the rational administration of enemas and, sometimes, diet and drugs, most patients remain clean for 24 h after the administration of the enema. Only patients with non-manageable loose stool secondary to an absent or a short colon need a permanent colostomy.

Patients suffering from fecal incontinence are evaluated and classified into those with constipation or those with increased motility (tendency to have diarrhea). In the first group, the saline enema must be of large volume, with additives (such as glycerin, soap, and/or phosphate) to help empty the colon. This program takes advantage of the decreased bowel motility in constipated patients; they remain clean for the next 24 h. No laxatives or diets are given in these types of patients. The second group (patients who suffer from increased bowel motility because of loss of the rectosigmoid), require a constipating diet, medication to decrease bowel motility, and a smaller daily saline enema (Bischoff and Tovilla In press; Bischoff et al. 2009; Peña 1990).

The treatment is adjusted by trial and error over a period of 1 week with radiologic monitoring. Approximately 95% of patients become clean. Bowel management is started just before the patient has to go to school and join their classmates who are already wearing regular underwear. Most patients and parents are very happy with the implementation of this program.

When the patient reaches the age of 7–12 years or sometimes younger, more independence is usually desirable. At that point, creation of a continent appendicostomy (Malone procedure) is very beneficial (Levitt et al. 1997b). This operation creates a communication between the abdominal wall and the cecum through the patient’s appendix (Fig. 28). A one-way valve mechanism is created by placating the cecum around the appendix which allows catheterization of the cecum but prevents leakage of stool. Patients are able to administer their own enema while sitting on the toilet. A significant number of patients do not have an appendix. In such cases an appendix is made with a tubularized flap of the cecum and then plicate the cecum around it (Fig. 29) (Chatoorgoon et al. 2011). The stoma is also exteriorized through the umbilicus. Most patients who have undergone this operation express a great deal of satisfaction.
Fig. 28

Diagram showing appendicostomy

Fig. 29

Neoappendix made from a tabularized flap of colon

Some patients benefit from an enema program early on, but if they have potential for bowel control, when they are a little older, a laxative trial can be employed to help their colon empty, with careful radiologic monitoring. This may allow them to demonstrate the capacity for voluntary bowel movement and thus eliminate the need for enemas.

Certain patients in whom the rectum was mislocated during the original operation may be candidates for a reoperation. This is recommended only in patients who were born with a good sacrum, good sphincters, and a malformation with a good prognosis. The results of this procedure vary, with worthwhile continence achieved in more than half of patients (Peña et al. 2007).

In patients with a cloaca, those with a common channel shorter than 3 cm require intermittent catheterization one third of the time. Patients with common channels longer than 3 cm require intermittent catheterization or a continent diversion 70–80% of the time.

Conclusion and Future Directions

Recent progress in the management of anorectal malformations and their associated urogenital deformities allows us to provide an efficient and effective treatment to those patients born with defects with demonstrated good functional prognosis, allowing them to have bowel and urinary control and to have a normal social life (Nam et al. 2016).

For those patients born with complex malformations with poor functional prognosis, there is a full program for bowel and urinary rehabilitation that allows these patients to be artificially clean of stool and dry of urine, which will allow them to have an acceptable social life.

Cross-References

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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  • Andrea Bischoff
    • 1
  • Belinda Hsi Dickie
    • 2
  • Marc A. Levitt
    • 3
  • Alberto Peña
    • 4
    • 1
    Email author
  1. 1.International Center for Colorectal CareChildren’s Hospital ColoradoAuroraUSA
  2. 2.Colorectal and Complex Pelvic Malformation Center, Department of SurgeryBoston Children’s HospitalBostonUSA
  3. 3.Center for Colorectal and Pelvic ReconstructionNationwide Children’s HospitalColumbusUSA
  4. 4.Colorectal Center for Children, Division of Pediatric SurgeryCincinnati Children’s Hospital Medical CenterCincinnatiUSA

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