History case

A child was born in term after normal pregnancy and delivery with body weight at birth of 3000 gr.

The condition after birth is satisfactory. The pathologies of heart and lungs have not been determined. The abdomen is soft and slightly distended.

On the examination of perineum the absence of anal orifice was attested. On the medial line the cutaneous protuberance and ischial tuberosity are converging.

The peritoneum irritation signs are negative.

The child is 1 day old.

Blood analyzes pH 7,32; pCO2 36 mmHg; BE - -4; Hb 220 g/l; Ht 65%. What are the presumptive diagnosis, investigation plan and treatment tactics?

History case

Cross-table X-ray with Hagar dilator in anal canal

High anorectal malformation

N. Testemitanu State University of Medicine and Pharmacy Department of Pediatric Surgery, Orthopedics and Anesthesiology

Anorectal malformations

Jalba Alexandru, MD, PhD, associate professor

Chisinau 2012

Imperforate anus and cloacal malformations

Background
o Imperforate anus has been a well-known condition since antiquity. For many centuries, physicians, as well as individuals who practiced medicine, tried to help these children by creating an orifice in the perineum. Many patients survived, most likely because they suffered from a type of defect that is now recognized as low. Those with a high defect did not survive treatment.
In 1835, Amussat was the first to suture the rectal wall to the skin edges, which could be considered the first actual anoplasty. During the first 60 years of the 20th century, most surgeons performed a perineal anoplasty without a colostomy for the so-called low malformations. In 1953, Stephens proposed an initial sacral approach followed by an abdominoperineal operation, if necessary. The posterior sagittal approach for the treatment of imperforate anus was performed first in 1980, and its description was published in 1982.

o o

o o

Incidence, types of defects, and terminology

o Anorectal atresia occurs in one of every 4000 to 5000 newborns and is slightly more common in males. o The estimated risk for a couple having a second child with an anorectal malformation is approximately 1%. o The most frequent defect in male patients is anorectal atresia with a rectourethral fistula, and is anorectal atresia with a rectovestibular fistula in females. o Imperforate anus without a fistula is a rather unusual defect. It occurs in about 5% of the entire group of malformations, and is associated with Down syndrome. o Historically, persistent cloaca was considered an unusual defect, whereas a high incidence of rectovaginal fistula was reported in the literature. It is likely that most patients suffering from a persistent cloaca were erroneously thought to have a rectovaginal fistula. Many of these patients underwent surgery with repair of the rectal component but were left with a persistent urogenital sinus.

Embriology

The rectum and anus are believed to develop from the dorsal potion of the hindgut or cloacal cavity when lateral ingrowth of the mesenchyme forms the urorectal septum in the midline. This septum separates the rectum and anal canal dorsally from the bladder and urethra. Downgrowth of the urorectal septum is believed to close this duct by 7 weeks' gestation. During this time, the ventral urogenital portion acquires an external opening; the dorsal anal membrane opens later. The anus develops by a fusion of the anal tubercles and an external invagination, known as the proctodeum, which deepens toward the rectum but is separated from it by the anal membrane. This separating membrane should disintegrate at 8 weeks' gestation.

Embriology

Interference with anorectal structure development at varying stages leads to

various anomalies, ranging from anal stenosis, incomplete rupture of the anal
membrane, or anal agenesis to complete failure of the upper portion of the cloaca to descend and failure of the proctodeum to invaginate. Continued communication between the urogenital tract and rectal portions of the cloacal plate causes rectourethral fistulas or rectovestibular fistulas.

The external anal sphincter, derived from exterior mesoderm, is usually present but has varying degrees of formation, ranging from robust muscle

(perineal or vestibular fistula) to virtually no muscle (complex longcommonchannel cloaca, prostatic or bladder-neck fistula).

Classification

Anorectal malformations represent a wide spectrum of defects.

The terms low, intermediate, and high are arbitrary and not useful
in therapeutic or prognostic terms.

Within the group of anorectal malformations traditionally referred to as high, there are defects included with different therapeutic and prognostic implications. For instance, retroprostatic fistula and rectobladder neck fistula were both considered high, yet the first can be repaired with a posterior sagittal approach alone and the second requires

an additional abdominal approach. Furthermore, the prognosis for each

type is completely different. Therefore, a more therapeutic and prognostically oriented classification is depicted on the slide below.

Classification

Males Cutaneous (perineal fistula) Rectourethral fistula Bulbar Prostatic Rectobladder neck fistula Imperforate anus without fistula Rectal atresia Females Cutaneous (perineal fistula) Vestibular fistula Imperforate anus without fistula Rectal atresia Cloaca Complex malformations

MALE

ANORECTAL DEFECTS

Rectoperineal fistulas

Rectoperineal fistula is what traditionally was known as a low defect. The rectum is located within most of the sphincter mechanism. Only the lowest part of the rectum is anteriorly mislocated (Fig. 1, 2). Sometimes, the fistula does not open into the perineum but rather follows a subepithelial midline tract, opening somewhere along the midline perineal raphe, scrotum, or even at the base of the penis. This diagnosis is established by perineal inspection. No further investigations are required. Usually, the anal fistula opening is narrow (stenotic).
The terms covered anus, anal membrane, anteriorly mislocated anus, and bucket - handle malformations all refer to perineal fistulas.

Rectoperineal fistulas

Figure 1. This drawing shows the course of a perineal fistula in a male. The rectum is located within most of the muscle complex. Only the most distal aspect of the rectum is misplaced

Figure 2. Newborn with imperforate anus and a rectoperineal fistula.

Rectourethral fistulas

Imperforate anus with a rectourethral fistula is the most frequent defect in male patients. The fistula may be located at the lower (bulbar) (see figure 3A) or the higher (prostatic) part of the urethra (see figure 3B).

Figure 3. Anorectal atresia with rectourethral fistulas. A, Rectourethrobulbar fistula B, Rectourethroprostatic fistula

Rectourethral Fistulas

Immediately above the fistula, the rectum and urethra share a common wall. The lower the fistula, the longer is the common wall. This is an important anatomic fact with significant technical and surgical implications. The rectum is usually distended and surrounded laterally and posteriorly by the levator muscle. Between the rectum and the perineal skin, a portion of striated voluntary muscle called the muscle complex is present. The contraction of these muscle fibers elevates the skin of the anal dimple. At the level of the skin, a group of voluntary muscle fibers, called parasagittal fibers, are located on both sides of the midline.

Rectourethral Fistulas

Lower urethral fistulas are usually associated with good-quality muscles, a well-developed sacrum, a prominent midline groove, and a prominent anal dimple. Higher urethral fistulas are more frequently associated with poorquality muscles, an abnormally developed sacrum, a flat perineum, a poor midline groove, and a barely visible anal dimple.

Of course, exceptions to these rules exist. Occasionally, the infant passes meconium through the urethra, which is an unequivocal sign of a rectourinary fistula.

Recto-Bladder Neck Fistulas

In this defect, the rectum opens into the bladder neck (Fig. 4). The patient usually has a poor prognosis for bowel control because the

levator muscles, the striated muscle complex, and the external

sphincter frequently are poorly developed.

The sacrum is often deformed and short. In fact, the entire pelvis seems to be underdeveloped.

The perineum is often flat, which is evidence of poor muscle development.

About 10% of males with anorectal atresia fall into this category.

Recto-Bladder Neck Fistulas

Figure 4. Schematic representation of

a recto-bladder neck fistula. Note that the fistula enters into the bladder neck near the junction between the urethra

and the bladder.

Imperforate Anus without Fistula

Interestingly, most patients with imperforate anus without fistula (Fig.5) have a well-developed sacrum and good muscles, and have a good prognosis in terms of bowel function. The rectum usually terminates approximately 2 cm from the perineal skin. Although the rectum and urethra do not communicate, these two structures are separated only by a thin, common wall. About half of the patients with no fistula also have Down syndrome, and more than 90% of patients with Down syndrome and imperforate anus have this specific defect, suggesting a chromosomal link. The fact that these patients have Down syndrome does not seem to interfere with their good prognosis for bowel control.

Imperforate Anus without Fistula

Figure 5. A boy with imperforate anus

Rectal Atresia

In this extremely unusual defect in male patients (~1% of the entire group of malformations), the lumen of the rectum is totally (atresia) or partially (stenosis) interrupted. The upper pouch is represented by a dilated rectum, whereas the lower portion is represented by a small anal canal that is in the normal location and is 1 to 2 cm deep (Fig. 6).

Figure 6. This newborn was found to have rectal atresia. A, Note the normal anal position and short depth of the anal canal. B, Operative repair. A dilator has been inserted into the anus and the anastomosis (arrow) is identified just proximal to the distal anal canal.

Rectal Atresia

These two structures may be separated by a thin membrane or by dense fibrous tissue.

The repair involves a primary anastomosis between the upper pouch and anal canal (see Fig. 6B) and is ideally approached posterior sagittally.
Patients with this defect have all the necessary elements to be continent and have an excellent functional prognosis. Because they have a well-developed anal canal, they have normal sensation in the anorectum and have almost normal voluntary sphincters. Patients with rectal atresia must be screened for a presacral mass.

FEMALE

ANORECTAL DEFECTS

Rectoperineal Fistulas

From the therapeutic and prognostic viewpoint, this common defect is equivalent to the perineal fistula described in the male patient.

The rectum is well positioned within the sphincter mechanism, except for its lower portion, which is anteriorly located.

The rectum and vagina are well separated (Fig. 7). The key anatomic issues are the anal opening in relation to the sphincter mechanism, and the length of the perineal body.

Rectoperineal Fistulas

Figure 7. Schematic representation of a recto-bladder neck fistula. Note that the fistula enters into the bladder neck near the junction between the urethra and the bladder.

Rectovestibular Fistulas

Rectovestibular fistula is the most common defect in girls and has an excellent functional prognosis. The diagnosis is based on clinical examination. A meticulous inspection of the neonatal genitalia allows the clinician to observe a normal urethral meatus and a normal vagina, with a third hole in the vestibule, which is the rectovestibular fistula (Fig. 8). About 5% of these patients will have two hemivaginas with a vaginal septum.

A number of pediatric surgeons repair this defect without a protective colostomy. This is a well- recognized trend in the management of anorectal malformations. The advantage of this approach isthat it avoids the potential morbidity of a colostomy and reduces the number of operations to one from as many as three (colostomy, main repair, and colostomy closure).

Rectovestibular Fistulas

Figure 8. A, Schematic drawing of a rectovestibular fistula. B, A female neonate with a rectovestibular fistula. Note the patient is in the prone position and the rectal fistula (arrow) is located in the posterior aspect of the vestibule.

FOURCHETTE FISTULA

If perineal fistula is not identified, the labia are separated to search for a vestibular fistula. A Fourchette fistula is a type of vestibular fistula that straddles the spectrum of malformation between perineal and vestibular; it is characterized by wet mucosa of the vestibule anteriorly and a dry anoderm posteriorly at the junction of the vestibule and perineum (see the image).

Figure 9. Fourchette fistula. This malformation is somewhere halfway between perineal fistula and vestibular fistula. The fistula has a wet vestibular mucosal lining on its anterior half, but the posterior half is dry perineal skin.

Rectovestibular Fistulas

Many patients do very well with a primary neonatal operation without a protective colostomy.

However, a perineal infection followed by dehiscence of the anal

anastomosis or perineal body, or recurrence of the fistula provokes severe fibrosis that may interfere with the sphincter function.

If these complications occur, the patient may have lost the best opportunity for an optimal functional result because secondary operations do not render the same prognosis as a successful primary operation.

Thus, a protective colostomy is still the best way to avoid these

complications.

Rectovestibular Fistulas

The decision to perform a colostomy or primary repair in these cases must be made individually by the surgeon, taking into consideration his or her

experience and the clinical condition of the patient.

In our Academician Natalia Gheorghiu National Scientific and Practical Center of Pediatric Surgery, neonates born with this anomaly, without

significant associated defects, undergo operation without a colostomy.

The term vaginal fistula is frequently erroneously used in patients who actually have a vestibular fistula or a cloaca. A true vaginal fistula occurs in less than 1% of all cases and is not considered part of the proposed classification.

Imperforate Anus without Fistula

This defect in female patients (Fig. 10) carries the same therapeutic and prognostic implications as described for male patients

Figure 10. A girl with imperforate anus

Persistent Cloaca

This group of defects represents the extreme in the spectrum of complexity of female malformations. A cloaca is defined as a defect in which the rectum, vagina, and urinary tract meet and fuse, creating a single common channel. The diagnosis of persistent cloaca is a clinical one. This defect should be suspected in a female born with imperforate anus and small-looking genitalia (Fig. 11). Careful separation of the labia discloses a single perineal orifice. The length of the common channel varies from 1 to 7 cm. This distance has technical and prognostic implications. Common channels longer than 3 cm usually are associated with complex defects (Fig. 12 A).

Persistent Cloaca

If no fistula is visible and only one opening between shortened labia is observed, the child has a cloaca.

Figure 11. Cloaca. This is the classic appearance of a girl with a cloacal malformation with a single perineal orifice. The genitals appear quite short, which is a finding consistent with cloaca.

Persistent Cloaca

o Mobilization of the vagina is difficult in such cases. Therefore, in patients with a long common channel, some form of vaginal replacement is often needed during the definitive repair.

Figure 12A. Schematic diagram of a long common channel in a female with a cloacal anomaly.

Persistent Cloaca

A common channel of less than 3 cm usually means that the defect can be repaired with a posterior sagittal operation without opening the abdomen (Fig, 12B).

Figure 12B. The more commonly encountered short common channel cloaca is depicted.

Persistent Cloaca

When the rectum opens high into the dome of the vagina (Fig. 13), an abdominal approach must be utilized to mobilize the bowel.

Figure 13. A schematic diagram of the rectum inserting high into the posterior vagina with a short common urethral and vaginal channel is shown.

Persistent Cloaca

Figure 14. Schematic depiction of a cloacal anomaly with insertion of the rectum into the common channel and subsequent vaginal obstruction with hydrocolpos.

Frequently, the vagina is abnormally distended and full of secretions (hydrocolpos) (Fig. 14). The distended vagina compresses the trigone and interferes with drainage of the ureters and is frequently associated with megaureters. This condition may be diagnosed prenatally. The dilated vagina can also become infected (pyocolpos) and may lead to perforation and peritonitis. However, such a large vagina may represent a technical advantage for the repair, providing more vaginal tissue to facilitate the reconstruction.

Persistent Cloaca

Figure 15. Schematic depiction of a cloacal anomaly and uterine duplication. The rectum is shown entering between the two hemivaginas.

A frequent finding in cloacal malformations is the presence of different degrees of vaginal and uterine septation or duplication (see Fig. 15). The rectum usually enters between the two hemivaginas. Rarely, patients may have cervical atresia. During puberty, they are unable to drain menstrual blood through the vagina. These patients accumulate menstrual blood in the peritoneal cavity and often require emergency operations

Persistent Cloaca

An evaluation of a patients Mullerian anatomy, either at the time of the definitive repair or at the colostomy closure, is vital and can prevent future problems.

Low cloacal malformations (<3 cm) are usually associated with a welldeveloped sacrum, a normal-appearing perineum, and adequate muscles and nerves.

Therefore, a good functional prognosis should be expected.

COMPLEX MALFORMATIONS

Unusual and bizarre anatomic arrangements can be seen. Each case represents a unique challenge to the surgeon, with different prognoses and therapeutic implications.

No general guidelines can be drawn for the management of these patients. Each case must be individualized.

ASSOCIATED DEFECTS

SACRUM AND SPINE

Sacral deformities appear to be the most frequently associated defect. One or several sacral vertebrae may be missing (Fig. 16). A single missing vertebrae does not seem to have any important prognostic implications. However, more than two absent sacral vertebrae represent a poor prognostic sign in terms of bowel continence and, sometimes, urinary control. A hemisacrum is usually associated with a presacral mass and poor bowel control (Fig. 17). Other sacral abnormalities, such as spinal hemivertebra, have a negative implication for a patients bowel control.

SACRUM AND SPINE

Figure 16. Sacral radiography (see the picture) - Absent lumbosacral vertebrae, a severe vertebral anomaly.

Figure 17. Hemisacrum presacral mass.

with

SACRUM AND SPINE

A sacral ratio allows for a more objective evaluation of the sacrum (Fig.18). The normal sacral ratio in children is 0.77. Children with anorectal malformations suffer from different degrees of sacral hypodevelopment, and the sacral ratio can vary from 0.0 to 1.0. We have never seen a patient develop good bowel control with a sacral ratio of less than 0.3.

Figure 18. Drawings with landmarks necessary for the calculation of the sacral ratio. A, Lateral view. B, Anteroposterior view. The normal ratio is 0.77.

SACRUM AND SPINE

Emphasis has been placed on the diagnosis and treatment of a tethered cord (Fig. 19), which is a defect frequently associated with anorectal malformations. It has been assumed that the presence of a tethered cord is associated with poor functional prognosis in these children. A review of our own series showed that 25% of patients with an anorectal malformation suffer from tethered cord. Although it is true that most of these children have a poor prognosis, the presence of a tethered cord by itself coincides with a very high defect, very abnormal sacrum, or spina bifida. Therefore, it is difficult to know whether the tethered cord itself is responsible for the poor prognosis. Also, we have not found evidence that the operation to release the tethered cord changes the functional bowel prognosis of the patient.

SACRUM AND SPINE

Figure 19. MRI. Tethered spinal cord

GENITOURINARY DEFECTS

The frequency of associated genitourinary defects varies from 20% to 54%. The higher the malformation, the more frequent are the associated urologic abnormalities. Patients with persistent cloacas or recto-bladder neck fistulas have a 90% chance of having an associated genitourinary abnormality (Fig. 20, 21). Conversely, children with low defects (perineal fistulas) have less than a 10% chance of having an associated urologic defect. Hydronephrosis (Fig. 22), urosepsis, and metabolic acidosis from poor renal function represent the main sources of morbidity in infants with anorectal malformations. Thus, a thorough urologic investigation is mandatory in cases of high defects, but is not as urgent in cases of rectovestibular and rectourethral fistulas.

GENITOURINARY DEFECTS

Figure 20. Voiding cystourethrography. Vesicoureteral reflux.

Figure 21. Voiding cystourethrography. Neurogenic bladder

GENITOURINARY DEFECTS

Figure 22. Ultrasonography demonstrating hydronephrosis in a newborn with imperforate anus.

NEWBORN MANAGEMENT

NEWBORN MANAGEMENT (MALE)

A decision-making algorithm for the initial management in male patients is seen in Fig. 23.
Newborn Male Anorectal Malformation Perineal inspection

20-24 hours

Spine Kidney U/S Urinalysis R/O esophageal atresia

Sacrum Spinal U/S Cardiac echo

Re-evaluation and cross table lateral film

Perineal fistula

Rectal gas below coccyx No associated defects

Rectal gas below coccyx Associated defects Abnormal sacrum Flat bottom

Anoplasty

Consider PSARP with or without colostmy

Colostomy

Figure 23. Algorithm for the management of male newborns with anorectal malformations based on the physical examination and radiographs. PSARP, posterior sagittal anorectoplasty; U/S, ultrasonography; R/O, rule out.

NEWBORN MANAGEMENT (MALE)

During the past few years, the tendency by the pediatric surgical community has been to operate on patients with anorectal malformations primarily without a protective colostomy. However, to avoid the potential negative consequences, these operations shouldnt be performed without the necessary preoperative evaluation and experience. A thorough perineal inspection usually gives the key clues to the type of malformation. The decision about a colostomy or primary operation shouldnt be made before 24 hours of life, because of the fact that significant pressure is needed for the meconium passage through fistulous orifice, as well as radiologic findings may not show the correct anatomy, because the rectum is collapsed. During the first 24 hours, the neonate should receive intravenous fluids and antibiotics and be evaluated for associated defects that may represent a threat to life (cardiac malformations, esophageal atresia, and urinary defects).

NEWBORN MANAGEMENT (MALE)

If the neonate has signs of a perineal fistula, we recommend that an anoplasty, without a protective colostomy, be performed during the first 48 hours of life. After 24 hours, if there is no meconium on the perineum, we recommend obtaining a cross-table lateral radiograph with the patient in the prone position(Fig. 24) or invertography (Fig. 25) .

Figure 24. Technique for a cross-table lateral radiograph. A, A roll has been placed beneath the hips of the infant to elevate the buttocks and allow air to migrate superiorly to the end of the rectum. B, Actual crosstable lateral radiograph. Air is visualized distal to the coccyx (arrow).

NEWBORN MANAGEMENT (MALE)

If air in the rectum is located below the coccyx, (Fig. 24) and the patient is in good condition with no significant associated defects, one may consider performing a posterior sagittal operation without a protective colostomy. A more conservative alternative would be to perform the posterior sagittal repair and a protective colostomy at the same stage.

Figure 24. Technique for a cross-table lateral radiograph. A, A roll has been placed beneath the hips of the infant to elevate the buttocks and allow air to migrate superiorly to the end of the rectum. B, Actual crosstable lateral radiograph. Air is visualized distal to the coccyx (arrow).

Invertography
Lateral pelvic radiography at 24 hours: Children who could not be diagnosed based solely on physical examination findings, traditionally underwent invertography, which consisted of holding the baby upside down and using lateral radiography to observe the level of gas in the distal rectum (Fig. 25).

Figure 25. Invertography

NEWBORN MANAGEMENT (MALE)

Conversely, if the rectal gas does not extend beyond the coccyx, or the patient has meconium in the urine, an abnormal sacrum, or a flat bottom, we strongly recommend a colostomy. This allows for a future distal colostogram (Fig. 26), which will precisely delineate the anatomy. We would then perform a posterior sagittal anorectoplasty 1 to 2 months later, provided the neonate is gaining weight appropriately. Performing the definitive repair at age 1 to 3 months has important advantages, including less time with an abdominal stoma, less size discrepancy between the proximal and distal bowel at the time of colostomy closure, and easier anal dilation (because the infant is smaller). In addition, at least theoretically, placing the rectum in the right location early in life may represent an advantage in terms of the potential for acquired local sensation.

AUGMENTED-PRESSURE DISTAL
COLOSTOGRAPHY
o

o o

o o

This is the single most important diagnostic test used to clarify the anatomy in all children with malformations who require colostomy. It is personally performed by the colorectal surgeon in many centers. In a fluoroscopy suite, a balloon catheter is placed into the distal stoma, and the balloon is inflated. The catheter is pulled back, and water-soluble contrast is injected by hand. This pressure is required to overcome the pressure of the levator muscles and to allow the contrast to flow into the lowest part of the colon and reveal any fistula. In patients with a fistula to the urinary tract, the bladder often fills, and the study is continued to obtain as much information as is provided with voiding cystourethrography. If no fistula is present, the distal pouch has a rounded appearance, and no urinary extravasation is visible. (See the picture below Fig. 26)

Figure 26. Augmented-pressure distal colostography demonstrating rectourethral fistula only when adequate pressure is used. Note the flat rectum on the left, which represents compression of the distal rectum in the funnel-like sphincteric mechanism.

NEWBORN MANAGEMENT (MALE)

However, all of these above mentioned potential advantages of an early operation must be weighed against the possible disadvantages of an inexperienced surgeon who is not familiar with the minute anatomic structures of an infants pelvis. A temptation to repair these defects without a protective colostomy always exists. Repair without a colostomy limits the anatomic information (provided by a distal colostogram) that may be very helpful to the surgeon. The worst complications involve patients operated on without a colostomy or a properly performed distal colostogram. Proceeding with the posterior sagittal approach looking blindly for the rectum has resulted in a spectrum of serious complications, including damage to the urethra, complete division of the urethra, pull-through of the urethra, pull-through of the bladder neck, injury to the ureters, and division of the vas deferens or seminal vesicles.

NEWBORN MANAGEMENT (FEMALE)

A decision-making algorithm for the initial management of newborn females is seen in Fig. 16.
Newborn Female Anorectal Malformation Sacrum Esophagus Cardiac echo Kidney and abdominal U/S Spinal U/S (tethered cord) Lumbar spine

R/O serious potentially lethal associated defects

Perineal inspection

Single perineal orifice Cloaca

Urologic evaluation R/O hydrocolpos

Perineal fistula

Vestibular fistula

No vizible fistula (<10%)

Anoplasty or dilatations

Cross table lateral view

Colostomy Drain hydrocolpos Urinary diversion (if necessary)

Colostomy or primary repair

Rectum below coccyx

High rectum

Colostomy

Figure 27. Decision-making algorithm for female newborns with anorectal malformations. U/S, ultrasonography; R/O, rule out.

NEWBORN MANAGEMENT (FEMALE)

Again, the perineal inspection is the most important step to guide diagnosis and decision making. The first 24 hours should also be used to evaluate for serious associated defects, as previously described. The perineal inspection may disclose the presence of a single perineal orifice. This single finding establishes the diagnosis of a cloaca, which carries a high risk of an associated urologic defect. The patient needs a complete urologic evaluation, including abdominal and pelvic ultrasonography, to look for hydronephrosis and hydrocolpos.

NEWBORN MANAGEMENT (FEMALE)

Patients with a cloaca require a colostomy. It is important to perform the divided sigmoid colostomy in such a manner as to leave enough redundant, distal rectosigmoid colon to allow for the subsequent pull-through (Fig. 28)

Figure 28. An ideal colostomy for infants with high anorectal malformations is seen in the drawing (A) and infant (B). Note the colostomy (1) and mucous fistula (2) are separated and that adequate distal colonic length remains for the subsequent rectal pull-through.

NEWBORN MANAGEMENT (FEMALE)

When performing the colostomy, it is mandatory to drain the hydrocolpos when present. Because a significant number of these patients have two hemivaginas, the surgeon must be certain that both hemivaginas are drained. It is the hydrocolpos that compresses the trigone and causes bilateral hydronephrosis, and drainage of the hydrocolpos allows for decompression of the urologic system. Rarely, if the common channel is very narrow and does not allow the bladder to drain, the neonate may require a vesicostomy or suprapubic cystostomy to decompress the bladder. Endoscopic examination of the cloaca is recommended to delineate the anatomy. This is best done later (in several months) during a separate anesthetic because the neonatal perineum is swollen and endoscopy is difficult.

NEWBORN MANAGEMENT (FEMALE)

The presence of a vestibular fistula represents the most common finding in female patients (see Fig. 8). When newborns with a vestibular fistula undergo primary repair, the patient should be kept for 5 to 7 days without any oral intake, on parenteral nutrition. Conversely, when the patient undergoes a primary repair of a vestibular fistula or perineal fistula without colostomy later in life, preoperative bowel irrigation for 24 hours preoperatively will ensure that the intestine is completely clean. Then the patient is kept on parenteral nutrition 7 to 10 days with nothing by mouth, all in an attempt to avoid a perineal infection.

NEWBORN MANAGEMENT (FEMALE)

The perineal inspection may show the presence of a perineal fistula. When present, we recommend performing a primary anoplasty without a colostomy. In fewer than 5% of girls, there is no visible fistula and there is no evidence of meconium after 24 hours of observation. This small group of patients requires a cross-table lateral prone radiograph (see Fig. 24). If the radiograph shows gas in the rectum very close to the skin, it means that the patient very likely has a very narrow perineal fistula. Conversely, if the distal extent of the rectal gas is located 1 to 2 cm above the skin, the patient most likely has an imperforate anus with no fistula. If the patient is in stable condition, one can perform a primary operation without a colostomy, depending on the surgeons experience. Most of these patients with no fistula also have Down syndrome. In the event that associated conditions make the rectal repair unfeasible in the newborn period, a colostomy should be performed, with definitive repair later.

NEWBORN MANAGEMENT (FEMALE)

Occasionally, if the infant with a rectoperineal or rectovestibular fistula has severe associated defects or is ill, the surgeon may elect to dilate the fistula to facilitate emptying of the colon while these other issues are addressed. Definitive repair can be performed in a few months. A divided descending colostomy is ideal for the management of anorectal malformations (see Fig. 28). The completely diverting colostomy provides bowel decompression as well as protection for the final reconstruction of the malformation. In addition, the colostomy is used for the distal colostogram, which is the most accurate diagnostic study to determine the detailed anatomy of these defects.

NEWBORN MANAGEMENT

The descending or upper sigmoid colostomy has definitive advantages over a right or transverse colostomy. It is important to have a relatively short segment of defunctionalized distal colon, but not too short as to interfere with the subsequent pull-through (Fig. 29) .

Figure 29. A, It is important not to create the colostomy too distal because there will not be sufficient rectal length to allow for pull-through. B, This problem is seen on the lateral view of the barium enema, where there is insufficient distal rectal length for the pullthrough. This is because of an inappropriately placed colostomy and mucous fistula.

SURGICAL TREATMENT (GENERALITIES)

Historically, there have been many surgical techniques to repair anorectal malformations. These include endorectal dissection, anterior perineal approaches, and many different types of anoplasties. Most pediatric surgeons now use the posterior sagittal approach with or without laparotomy or laparoscopy to repair these malformations. The debate recently has been centered more on the possibility of performing these operations primarily without a colostomy, or for which cases a laparoscopic approach is best suited.

POSTERIOR SAGITTAL APPROACH

The patient is placed in the prone position with the pelvis elevated. An electrical stimulator is used to elicit muscle contraction during the operation. The demonstrated contraction serves as a guide to keep the incision in the midline, leaving an equal amount of muscle on both sides. The length of the incision varies with the type of defect and can be extended to achieve the necessary exposure needed to have a satisfactory repair.

POSTERIOR SAGITTAL APPROACH

Thus, a perineal fistula requires a minimal posterior sagittal incision (2 cm), whereas higher defects may require a full posterior sagittal incision that runs from the lower portion of the sacrum toward the base of the scrotum in the male or to the single perineal orifice in females with a cloaca. The incision includes the skin and subcutaneous tissue and splits the parasagittal fibers, muscle complex, and levator muscles in the midline. In simple defects (perineal and vestibular), the incision divides only the parasagittal fibers and the muscle complex in the midline.

POSTERIOR SAGITTAL APPROACH

It is not usually necessary to open the levator muscle. Once the sphincter mechanism has been divided, the next most important step of the operation is the separation of the rectum from the urogenital structures, which represents the most delicate part of the procedure. Any kind of blind maneuver at this point in the operation exposes the patient to the possibility of serious injury. About 90% of defects in boys can be repaired via a posterior sagittal approach without opening the abdomen and without laparoscopy. Each case has individual anatomic variants that mandate technical modifications. An example is the size discrepancy frequently seen between an ectatic rectum and the space available for the pull-through. If the discrepancy is significant, the surgeon must tailor the rectum to fit.

POSTERIOR SAGITTAL APPROACH

If a colostomy has been done, the posterior sagittal approach should never be attempted without a technically adequate high-pressure distal colostogram to determine the exact position of the rectum and the fistula (Fig. 30, 31).

Figure 30. Distal colostogram, posteroanterior view. The initial phase of augmented-pressure distal colostography aims to determine where the colostomy was placed in the colon and how much colon is available for pull-through, without taking down the colostomy.

Figure 31. Distal colostogram, lateral view. image shows that the rectal pouch joins the urinary tract at the level of the bulbar urethra, a relatively common malformation in boys.

MALE DEFECTS

REPAIR OF RECTOPERINEAL FISTULA

The operation in these infants is performed in the prone position with the pelvis elevated. Multiple 6-0 silk stitches are placed in the fistula orifice. An incision is created dividing the sphincter mechanism located just posterior to the fistula. The sphincter is divided, and the posterior rectal wall is identified by its characteristic whitish appearance. Dissection of the rectum continues laterally following this specific plane. The last part of the dissection consists of separating the anterior rectal wall from its intimate relation to the urethra. The patient must have a urinary catheter inserted preoperatively. The best way to avoid urethral injury is to continuously be aware of the fact that the common wall has no plane of dissection and that the surgeon must create two walls out of Fig.32. Rectoperineal fistula in a male one. newborn

REPAIR OF RECTOURETHRAL FISTULAS

Fig. 33. Rectourethral bulbar fistula

Fig. 34. Rectourethral prostatic fistula

REPAIR OF RECTOURETHRAL FISTULAS

o o o o We like to perform a cystoscopy in all patients,which helps the surgeon confirm the location of the fistula. A Foley catheter is inserted and the patient is placed in prone position with the pelvis elevated. The incision is a posterior sagittal one, in between both buttocks, running from the middle portion of the sacrum down to the anal dimple, which is electrically determined. The incision goes through skin, subcutaneous tissue, parasagittal fibres,muscle complex and levator muscle (Fig. 35 a, b, c). When the surgeon is dealing with a rectourethral bulbar fistula (Fig. 33), he expects to see a bulging rectum as soon as he opens the levator muscle. In cases of the rectoprostatic fistula, (Fig. 34) the rectum is much smaller, it may not bulge through the incision, and the surgeon expects to find the rectum immediately below the coccyx. The surgeon should not look for the rectum in the lower part of the incision in patients with rectoprosatic fistula. Looking for a rectum without evidence that the rectum is there is the main source of complications in this approach. The surgeon will find the urethra, vas deferens, prostate, and seminal vesicles and will damage the nerves important for urinary control and sexual potency.

b Fig. 35. Posterior sagittal approach.

REPAIR OF RECTOURETHRAL FISTULAS

The posterior rectal wall is easily identified by its characteristic whitish appearance (Fig. 36 a, b). The surgeon must keep in mind that there is a fascia that covers the rectum posterior and laterally that must be removed. The dissection of the rectum must be performed staying as close as possible to the rectal wall without injuring the rectal wall itself. The posterior rectal wall is opened in the midline, in between two 5/0 silk stitches. The incision is continued distally, staying in the midline while applying stitches in the edges of the rectum. The traction on these stitches will allow the surgeon to see the lumen of the rectum. When the incision continues, extending distally in the midline, it ends directly into the rectourethral fistula that is identified usually as a 12 mm orifice.

b Fig. 36. Posterior sagittal approach.

REPAIR OF RECTOURETHRAL FISTULAS

Multiple 6/0 silk sutures are placed in a semi-circumferential fashion surrounding the fistula site above and lateral to the fistula (Fig. 37). These multiple sutures serve the purpose of exerting uniform traction on the rectal mucosa to facilitate the separation of the rectum from the urinary tract.

The fascia that covers the rectum is removed, creating the lateral planes of the rectum.
The mucosa of the anterior rectal wall distal to the multiple 6/0 silk sutures is Fig. 37. Posterior sagittal approach. Applying silk sutures in a semi-circumferential fashion divided about 1 mm deep. surrounding the fistula site above and lateral
to the fistula

REPAIR OF RECTOURETHRAL FISTULAS

The dissection continues between the rectum and urinary tract in a submucosal plane for approximately 510 mm and then gradually becomes a fullthickness dissection, looking at the lateral planes until the rectum is completely separated from the urinary tract. The separation of the urethra from the rectum is the most delicate part of the operation. Most of the serious complications occur during this part of the procedure. The fistula site is closed with three or four 6/0 long-term absorbable sutures (Fig. 38 a, b).

a Fig.

38. Posterior sagittal approach. Surgical dissection between the rectum and urinary tract and closing the fistula.

REPAIR OF RECTOURETHRAL FISTULAS

The surgeon should then evaluate the size of the rectum and compare it to the available space to decide if the rectum has to be tapered to be accommodated within the limits of the sphincters (Fig. 39). In the past, we performed many of these tapering procedures and we believe that is because patients came later in life, had inadequate colostomies, and therefore suffered from severe megarectum. Currently, babies are referred to us earlier, surgeons are opening better, totally diverting colostomies, and we therefore do not see megarectums as often. Consequently, tapering is rarely necessary. When a tapering procedure is required, we recommend to remove an adequate portion of the posterior rectal wall, closing this wall into two layers of interrupted long-term absorbable sutures. Tapering on the anterior wall is absolutely contraindicated as it would leave a rectal suture line against the urethral fistula repair and a recurrent fistula may develop.

Fig. 39. Posterior sagittal approach. Tapering of the rectum.

REPAIR OF RECTOURETHRAL FISTULAS

The limits of the sphincter are then electrically determined and marked with temporary silk sutures. The perineal body is reconstructed when the incision extends anterior to the centre of the sphincter. The anterior limits of the sphincter must be reconstructed (Fig. 40).

Fig. 40. Posterior sagittal approach. Marking the sphincter and reconstruction of the perineal body and anterior limits of the sphincter.

REPAIR OF RECTOURETHRAL FISTULAS

The posterior edge of the levator muscle is electrically determined, and the rectum is placed in front of the levator. The posterior edges of the levator muscle are sutured together with interrupted 5/0 long-term absorbable sutures. The distal continuation of the levator muscle is arbitrarily called the muscle complex. The posterior edge of this muscle structure is sutured together in the midline with interrupted 5/0 long-term absorbable sutures. These sutures also take a bite of the posterior rectal wall in order to anchor the rectum in a good position to avoid retraction and/or prolapse (Fig.41).
Fig. 41. Posterior sagittal approach.

REPAIR OF RECTOURETHRAL FISTULAS

The rest of the incision is closed meticulously reapproximating all the layers of the wound. An anoplasty is performed with 16 circumferential stitches of 6/0 long-term absorbable sutures, trimming off that part of the rectum that has been damaged or does not have adequate blood supply. (Fig. 42 a, b).
a b

Fig. 42. Posterior sagittal approach. Closing the wound and anoplasty.

REPAIR OF RECTO-BLADDER NECK FISTULA

This malformation is the highest of all defects seen in male patients (Fig.43). Fortunately, only 10% of male patients suffer from this particular defect. Associated defects occur in 90% of these patients. Usually, the sacrum is hypodeveloped. This particular group of malformations is the only one that, in order to be repaired, requires not only a posterior sagittal approach, but also an abdominal one, either by laparotomy or laparoscopy.
During the laparotomy or laparoscopy, the surgeon must separate the rectum from the urinary tract. Fortunately, in these very high malformations, the common wall between the rectum and the urinary tract is very short. In other words, the rectum connects to the bladder neck in a T fashion.

Fig. 43. Recto-bladder neck fistula.

REPAIR OF RECTO-BLADDER NECK FISTULA

Total body preparation should be performed. The operation is started via a posterior sagittal approach. All the sphincter mechanisms are divided in the midline.

No attempt should be made to find the rectum through this approach.

A rubber tube is placed in the presacral space behind the urethra and located, following the tract the rectum will subsequently occupy. The perineal body, the posterior edge of the levator muscle as well as the muscle complex are reconstructed around the rubber tube that represent the rectum (Fig. 44 a, b).

a Fig. 44. Recto-bladder neck fistula repairing.

REPAIR OF RECTO-BLADDER NECK FISTULA

The patient is then turned onto a supine position and a laparotomy is performed. The bladder is pulled anteriorly and the rectosigmoid is found. In these very high defects, the rectobladderneck fistula is usually located within 1 to 2 cm below the peritoneal reflection and, therefore, the pelvic dissection necessary to mobilize it is minimal (Fig. 44 a, b).

a Fig. 44. Recto-bladder neck fistula repairing.

REPAIR OF RECTO-BLADDER NECK FISTULA

Ureters and vas deferens run very close to the rectum to approach the trigone of the bladder. This should be kept in mind to avoid any damage. The dissection of the rectosigmoid must be performed while staying in direct contact with the bowel wall itself. The rectum opens into the bladderneck in a T fashion. This means that there is no common wall above the fistula as described in lower malformations. The fistula is divided and the bladder end is sutured with interrupted long-term absorbable sutures. The rubber tube is easily found in the presacral space. The decision is made to taper the rectum when necessary and then to anchor the rectum to the rubber tube. The legs are lifted up and the rubber tube is pulled down, pulling together the rectum that will be placed in the desired location. The anoplasty is performed as previously described and the abdomen is closed (Fig. 44 a, b).

a Fig. 44. Recto-bladder neck fistula repairing.

REPAIR OF IMPERFORATE ANUS WITHOUT FISTULA

This particular malformation is unique. When we say imperforated anus without fistula, we do not have to refer to the height of the defect because in all cases the rectum is located approximately 12 cm above the perineal skin, at the level of bulbar urethra. This malformation only happens in 5% of all cases and half of these have Downs syndrome. The patients with these defects have good prognosis, good sacrum and good sphincters. Ninety percent of our patients with this defect without Downs syndrome have bowel control and 80% of our Downs syndrome patients have bowel control.

Fig.45. Imperforate anus repairing.

REPAIR OF IMPERFORATE ANUS WITHOUT FISTULA

The technique to repair this malformation is not necessarily simpler than the one for rectourethral bulbar fistula since the rectum is intimately attached to the posterior urethra. The surgeon has to open the posterior rectal wall and still has to create a plane of dissection between the anterior rectal wall and the urethra, a manoeuvre that requires a meticulous dissection (Fig.45). Fig.45. Imperforate anus repairing.

FEMALE DEFECTS

FEMALE DEFECTS

Female defects are represented by a similar spectrum. However, over the last 22 years, we have been learning a great deal about these defects,

particularly with regard to cloacas.

We have learned, for instance, that the so-called rectovaginal fistula is an almost nonexistent defect. Most of the patients that were referred to us after having an operation to repair a rectovaginal

fistula, actually never suffered from that particular defect.

Most of them suffered originally from a cloaca that was misdiagnosed; the surgeon repaired the rectal component of the defect and left the patient with a persistent urogenital sinus.

In other more unfortunate cases, the babies were born with rectovestibular fistulas, the surgeons mislabelled and misdiagnosed it as a rectovaginal fistula and performed an abdominal perineal procedure for a malformation that could otherwise have been repaired posterior sagittally with good results, leaving the patient totally incontinent for faeces.

REPAIR OF RECTOPERINEAL FISTULA

This defect is equivalent to the recto-perineal fistula in males already described. Bowel control exists in 100% of our patients and less than 10% of them have associated defects. The patients are faecally continent with and without an operation. Constipation is a constant sequela and should be treated energetically. This is true also for the male patients with perineal fistulas. We have learned that the lower the defect the more there is a chance of constipation. We have also learned that constipation is a selfperpetuating and self-aggravating condition that eventually produces severe megacolon, chronic faecal impaction and overflow pseudoincontinence, and must vigilantly be avoided (Fig.46).

Fig.46. Rectoperineal fistula in a female newborn

REPAIR OF RECTOPERINEAL FISTULA

In our opinion the better way to repair this defect is to perform the operation at birth. The main reasons are to avoid cosmetic, psychological and potential obstetric sequelae in the future. We perform this operation before the baby leaves the hospital during the newborn period. The patient is placed in prone position with the pelvis elevated. Multiple 6/0 silk stitches are placed around the fistula site. The incision is about 1.52 cm long and divides the entire sphincter mechanism in the posterior midline. We dissect the rectum as previously described in the case of perineal fistulas in male patients. The perineal body is reconstructed as shown and the rectum is anchored to the posterior edge of the muscle complex. An anoplasty is performed. These patients can eat on the same day of the surgery, and since they are only passing noncolonized meconium, we give antibiotics for 48 h.

Fig.46. Rectoperineal fistula in a female newborn

REPAIR OF RECTOVESTIBULAR FISTULA

This defect is perhaps the most important anorectal malformation in females.

This is because it is by far the most common defect seen in the females. It is also a malformation that has an excellent functional prognosis when managed correctly.
Also, paradoxically and unfortunately, girls with these defects are the ones that we have seen suffer from more complications after a failed attempt to repair. For many years, it has been very controversial whether this malformation should be treated with a previous protective colostomy or should be operated primarily at birth.

Fig.47. Rectovestibular fistula in a female newborn

REPAIR OF RECTOVESTIBULAR FISTULA

Again, we believe that this depends very much on the experience of the surgeon.

When a baby is born with this malformation at our institution and the baby is otherwise healthy, we repair this malformation during the newborn period.
If the baby is premature or has associated defects, it is always safer to open a protective colostomy. The surgeon must keep in mind that dehiscence and infection in patients with anorectal malformations not only represent a few more days in the hospital and an ugly scar but also represents the possibility of changing the prognosis for bowel control. Fig.47. Rectovestibular fistula in a female newborn

REPAIR OF RECTOVESTIBULAR FISTULA

Bowel control exists in 93% of our patients; 70% of them have constipation that is incurable but manageable and it should be treated aggressively. The most important anatomical feature that should be recognized by the surgeon is that the rectum and vagina share a long common wall that must be separated, creating a plane of dissection where it does not exist, in order to mobilize the rectum and put it in the right place. We believe that most of the complications that we have seen from treatment of this malformation originate from the lack of separation of these two structures, or the defective separation of these two structures.

Fig.47. Rectovestibular fistula in a female newborn

REPAIR OF RECTOVESTIBULAR FISTULA

These patients are also placed in prone position with the pelvis elevated. The incision runs usually from the coccyx down to the fistula site. Again, multiple 6/0 sutures are placed in a circumferential manner in the fistula opening. Traction always facilitates the dissections of these delicate structures. The entire sphincter mechanism is divided posteriorly until we identify the posterior rectal wall, and then the plane of dissection is established removing the fascia that covers the rectum. The dissection then continues laterally.

Fig.48. Rectovestibular fistula repairng in a female newborn

REPAIR OF RECTOVESTIBULAR FISTULA

The surgeon must put a special emphasis on a very meticulous separation of the rectum from the vagina. The purpose of this dissection is to make two walls out of one. He or she should try at all costs to avoid making perforations in the rectal wall or the vaginal wall. Once the rectum has been completely separated from the vagina, the surgeon determines the limits of the sphincter electrically and reconstructs the perineal body, bringing together the anterior limits of the sphincter.

Fig.48. Rectovestibular fistula repairng in a female newborn

REPAIR OF RECTOVESTIBULAR FISTULA

The rectum is then placed within the limits of the sphincter as well as the muscle complex. We can see only the lower part of the levator because the incision is rather limited in this operation. The rectum is anchored to the posterior edge of the muscle complex and the anoplasty is performed like we discussed in the previous cases. When the patient is a newborn, we keep the baby 2 or 3 days with nothing by mouth postoperatively, while receiving intravenous antibiotics.

Fig.48. Rectovestibular fistula repairng in a female newborn

REPAIR OF PERSISTENT CLOACA

Technical variant called total urogenital mobilization (Fig. 49): The rectum is separated from the vagina in the same way that they were separated in the cases of rectovestibular fistula. Once we expose the malformation, multiple 6/0 silk stitches are placed taking the edges of the common channel and the edges of the vaginal walls. We use those stitches to exert a uniform traction. Then urethra and vagina together are mobilized in what we call total urogenital mobilization. Another set of sutures is placed in a transverse fashion taking the mucosa of the common channel 5 mm proximal to the clitoris and then the entire common channel is divided, full thickness, creating a plane of dissection between the pubis and the common channel.

Fig.49. Total urogenital mobilization.

REPAIR OF PERSISTENT CLOACA

This is a natural plane of dissection and therefore it is very easy to dissect. We reach the upper part of the pubis and there we can identify avascular fibrous structures that fix the genitourinary structures to the pelvis. These are divided and that allows an immediate gain of length of the urogenital structures of approximately 24 cm,which is enough to repair the malformations with short common channels. The goal of this mobilization is to move what used to be a urethral meatus all the way down, to be placed immediately behind the clitoris to be visible and accessible for future urethral catheterizations when indicated. The vagina comes down together with the urethra and the edges of the vagina are sutured to the skin of the perineum forming the new labia.

Fig.49. Total urogenital mobilization.

REPAIR OF PERSISTENT CLOACA

What used to be the common channel is divided in the midline creating two flaps that are preserved and sutured to the new labia to enrich that tissue. All this reconstruction is performed with interrupted 6/0 long term, absorbable sutures. With these kinds of techniques, in patients with a good sacrum, 80% of them have bowel control and approximately 80% of them can empty the bladder voluntarily without intermittent catheterization, and remain dry of urine; 20% of them require intermittent catheterization to empty the bladder. Fortunately, they have a good bladder neck and they remain dry between catheterizations. When the sacrum is very abnormal, the results are not as good.

Fig.49. Total urogenital mobilization.

REPAIR OF PERSISTENT CLOACA

The repair of patients suffering from cloacas with longer common channels (>3 cm) represents a real technical challenge and requires a great deal of experience in the management of these cases. For that kind of patient, the surgeon should have experience in the management of the urinary structures including bladder reconstructions, bladderneck reconstructions, ureteral reimplantations, bladder augmentation and Mitrofanoff procedures. In cases with long common channels, if the pediatric surgeon and/or pediatric urologist both have experience with these malformations they continue with the repair, performing first what we call a total body preparation in order to have access both to the perineum and the abdomen.

Fig.49. Total urogenital mobilization.

REPAIR OF PERSISTENT CLOACA

If the common channel is longer than 5 cm, we recommend opening the abdomen directly in the midline because, in that particular case, rectum and vaginas are not accessible posterior sagittally and are more easily accessed through the abdomen. If the common channel is between 3 and 5 cm, the surgeon can open posterior sagittally, still will find the vagina or vaginas and can try to repair them by performing the total urogenital mobilization. If the total urogenital mobilization proves not be enough to repair the malformation then the operation must be completed through a laparotomy.

Fig.49. Total urogenital mobilization.

REPAIR OF PERSISTENT CLOACA

The separation of the rectum from the vagina is not difficult in very high malformations since it is similar to what we described in the separation of the rectum from the bladder neck in male patients. On the other hand, the separation of the vagina from the urinary tract in a case of a cloaca with a long common channel is a very delicate manoeuvre that requires expertise and finesse. Once the vagina has been completely separated, which is a manoeuvre that may take several hours, the surgeon then has to make important decisions considering the way to repair the vagina. The separation of the vagina from the urinary tract should be performed with the bladder open in the midline and with ureteral catheters.

Fig.49. Total urogenital mobilization.

REPAIR OF PERSISTENT CLOACA

The ureters run through the common wall that separates the vagina from the bladder and therefore, the surgeon should be ready to deal with the fact that the ureters may be right in the plane of separation. Once the vagina has been separated, the surgeon should evaluate the size of the mobilized vaginas and the length that he needs to bring the vagina down. It may be that the patient has a very large vagina that reaches the perineum.

Fig.49. Total urogenital mobilization.

CONLUSSION

Clinical results are different for each type of the malformations. A recent review of the authors series showed that 100% of patients with rectal atresia and perineal fistula had voluntary bowel movements, those with vestibular fistula 93.8%, bulbar urethral fistula 87.5%, imperforate anus without fistula 85%, cloaca 83.3%, rectourethral prostatic fistula 76.5%, and bladderneck fistula 28.6%. Soiling in patients who enjoy voluntary bowel movements usually represents a manifestation of faecal impaction and when the constipation is treated properly, the soiling usually disappears. Patients who enjoy voluntary bowel movements and never soil are considered totally continent. Constipation is a common sequela seen after the repair of an anorectal malformation. Interestingly, patients with lower defects, and therefore with better prognosis for bowel control, suffer a higher incidence of constipation and vice versa.

Constipation correlates directly with the degree of rectosigmoid dilation at the time of colostomy closure.
Therefore, every effort should be made to try to keep the rectosigmoid empty and decompressed from day 1 in these patients.

CONLUSSION

Urinary control can be expected in the overwhelming majority of male patients after repair of imperforate anus provided a good surgical technique was performed. Urinary incontinence is a concern only in male patients with absent sacrum or in some female patients with cloaca. In this last defect, when the common channel is shorter than 3 cm, approximately 20% of patients require intermittent catheterization to empty the bladder. The remaining 80% enjoy urinary control. When the common channel is longer than 3 cm it was observed that 68% of the patients require intermittent catheterization to empty the bladder. Fortunately, after the repair of a cloaca, patients have a good bladderneck; they have the capacity to empty the bladder. Thus, when the bladder becomes completely full, the patients start suffering from overflow urinary incontinence. Intermittent catheterization keeps these patients completely dry.

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