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Background
Anorectal malformations comprise a wide spectrum of diseases that affect boys and girls and can
involve malformations of the distal anus and rectum, as well as the urinary and genital tracts.
Malformations range from minor, easily treated defects that carry an excellent functional
prognosis, to complex defects that are difficult to treat, are often associated with other anomalies,
and carry a poor functional prognosis.

Throughout the centuries, doctors have seen and attempted to treat infants born with imperforate
anus. Because few patients have been described in the early literature, most are assumed to have
died without treatment. Paulus Aegineta recorded the earliest account of successful surgery for
imperforate anus. He suggested rupturing an obstructing membrane with the finger or knifepoint
and then dilating the tract until healing was complete. This approach was used for many years.

Almost 1000 years later, in 1660, Scultet used dilatation to treat an infant with anal stenosis. In
1676, Cooke used incision and dilatation and advised care of the sphincter muscles. In 1787, Bell
suggested using a midline perineal incision to find the bowel. In 1783, acting on Littre's
suggestion from 1710, Dubois performed an inguinal colostomy for imperforate anus. Other
surgeons followed suit, but almost all of the infants died; thus, colostomy remained unpopular
and a procedure only of last resort.

In 1835, Amussat described formal perineal proctoplasty (ie, mobilization of the bowel through a
perineal incision and suturing to the skin). This technique gained rapid acceptance. Strictures
were less common than observed in earlier procedures. In addition to Amussat, Dieffenbach
described anal transposition (1826); Chassaignac used a probe through a stoma to guide the
perineal dissection (1856); and Leisrink (1872), McLeod (1880), and Hadra (1884)
recommended opening the peritoneum if the bowel was not encountered from below.

In 1930, Wangensteen and Rice first advocated imaging to delineate the abnormality. Single-
stage abdominoperineal procedures became widely used after reports by Rhoads, Pipes, Randall,
Norris, Brophy, and Brayton (1948-1949). In 1953, Stephens described this procedure with
specific emphasis on preservation of the puborectalis muscle. This surgery and its modifications
were the standard approach until 1980.

In 1980, the surgical approach to repairing anorectal malformations altered dramatically with the
introduction of the posterior sagittal approach, because it allowed pediatric surgeons to clearly
view the anatomy of anorectal malformations and to repair them under direct vision, with better
visualization and understanding of the anatomic relationships than previous approaches allowed.

Pathophysiology and Etiology


Understanding the true anatomy is helpful to prevent damage to important structures during
surgical repair and to preserve the best potential for bowel control.
Anatomic visualization has allowed surgeons to eliminate many previous misconceptions. For
instance, the previous classification of these defects into high, intermediate, and low
malformations was a misleading oversimplification that did not adequately describe the spectrum
of anorectal anomalies.

Improved imaging techniques and a more thorough knowledge of the anatomy of the pelvic
structures at birth have refined diagnosis and early treatment. Analysis of large series of patients
has allowed better prediction of associated anomalies and functional prognosis.

The surgeons primary concern in correcting these anomalies is bowel control. Problems with
urinary control and sexual function must also be considered.

Early diagnosis, treatment of associated anomalies, and efficient and meticulous surgical repair
provide patients the best chance for a good functional outcome.

Some patients experience fecal and occasional urinary incontinence despite excellent anatomic
repair. Associated problems such as a poorly developed sacrum and tethered cord and/or
myelomeningocele likely contribute to an inability to achieve continence. For patients with fecal
incontinence, an effective bowel management program has been devised to improve quality of
life (see Bowel Management).

Although the etiology remains unknown, a slight genetic predisposition appears to exist.

Epidemiology
Anorectal malformations occur in approximately 1 per 5000 live births.

Prognosis
When evaluating the results of treatment of anorectal defects, patients should not be grouped into
the traditional high, intermediate, and low categories. For instance, within the traditional high
group, malformations have different treatments and carry different prognoses (eg, rectoprostatic
fistula compared with rectobladderneck fistula). Both of these would be considered high in the
traditional nomenclature; however, the malformations are so different they should not be grouped
together. An anatomic classification is of more clinical value.

The functional results of repair of anorectal anomalies have improved significantly since the
advent of the posterior sagittal approach. However, the results of this approach are difficult to
compare with those of other methods because terminology and classification are inconsistent.

Approximately 75% of all patients with anorectal malformations have voluntary bowel
movements. Approximately 50% have occasional soiling accidents. Episodes of soiling are
usually related to constipation; when constipation is treated properly, the soiling usually
disappears. Approximately 40% of all patients have voluntary bowel movements and no soiling.
About 25% of patients with anorectal malformations have fecal incontinence and must receive a
bowel management regimen with a daily enema to keep clean (see Bowel Management).

Apart from the anorectal anomaly, the status of the sacrum, spine, and muscles greatly affects a
patient's fecal continence. Even with a perfect reconstruction, a patient with a poor sacrum or
spine may not achieve bowel control.

Bowel control must be evaluated when the child is older than 3 years.

Patients with low defects (eg, rectoperineal fistula, rectal atresia) have excellent outcomes. Girls
with rectovestibular fistulas have very good outcomes, except for a tendency to develop
constipation. Approximately 60% of boys with rectourethral fistulae and normal sacra have good
outcomes. More than 80% of patients with persistent cloaca and a normal sacrum have bowel
control. Patients with very high malformations (eg, rectobladderneck fistula in boys) have poor
outcomes.

The sacrum is a good predictor of outcome. Patients with a normal sacrum are much more likely
to have fecal continence. Patients with a hypodeveloped sacrum are much more likely to be
incontinent. A sacral ratio has been developed to allow for a more objective assessment of the
sacrum (see the image below). Patients with a sacral ratio less than 0.3 only rarely achieve
continence. A hypodeveloped sacrum is also a good predictor of associated spinal problems, such
as tethered cord.

Calculation of the sacral ratio.

View Media Gallery

A child's outcome may be predicted. Parents can be realistically informed of their child's
potential for bowel control, even in the newborn period. This avoids a great deal of frustration
later in life. Establishing the functional prognosis early is vital to avoid raising false expectations
in the parents.

Once the diagnosis of the specific defect is established, the functional prognosis can be
predicted. The status of the spine, sacrum, and perineal musculature are all factors that affect the
counseling given to the parents.

If a given defect carries a good prognosis, such as rectovestibular fistula, rectoperineal fistula,
rectal atresia, rectourethral bulbar fistula, or imperforate anus without fistula, expect the child to
have voluntary bowel movements by age 3 years. Such children require supervision to avoid
fecal impaction, constipation, and soiling.

Certain defects indicate a poor prognosis, such as a high cloaca (common channel >3 cm) or a
rectobladderneck fistula. Parents should be informed that the child may require a bowel
management program to remain clean. The program should be implemented at age 3-4 years (see
Bowel Management).

Patients with rectoprostatic fistulas carry an almost equal chance of voluntary bowel movements
or incontinence. Toilet training should be attempted at age 3 years, and if unsuccessful, a bowel
management program should be initiated. Each year, during vacation, bowel control should be
attempted, and if unsuccessful, the bowel management should be restarted. As the child grows
older and more cooperative, the likelihood of achieving bowel control improves. Once it is
determined that a daily enema is needed, those can be given antegrade via a Malone
appendicostomy.

Urinary incontinence occurs in boys with anorectal malformations only when they have an
extremely defective or absent sacrum, an abnormal spine, or when the basic principles of surgical
repair are not followed and important nerves are damaged during the operation. The vast
majority of boys have urinary control. This is also true for girls, not including the group with
persistent cloaca who not infrequently need intermittent catheterization (see Cloacal
Malformations). Careful, regular observation is necessary in these patients to accurately reassess
their prognosis and to avoid problems that can dramatically affect their ultimate functional
results.

History
For neonates born with an anorectal anomaly, early treatment is crucial.

During the first 24-48 hours of life in a newborn with an anorectal malformation, the following 2
questions should be answered:

Does the newborn have any associated anomalies that need to be addressed
immediately?
Should the neonate undergo a primary procedure without a protective
colostomy, or should he or she undergo a protective colostomy with definitive
repair at a later date?

The surgeon must also determine whether female neonates have a distended vagina
(hydrocolpos), if it should be drained, and whether urinary diversion is required. These
maneuvers are intended to prevent sepsis and metabolic acidosis (see Cloacal Malformations).

Physical Examination
Colostomy versus anoplasty

The decision to perform an anoplasty during the early newborn period or to delay the repair and
perform a colostomy is based on physical examination findings in the newborn, the appearance
of the perineum, and changes that occur during the first 24-36 hours of life.

Operating earlier and in a single stage is potentially beneficial to the patient, but this should be
decided carefully, based on the specific circumstances of the newborn and the experience of the
surgeon. A more conservative approach is warranted in neonates with low birth weight and
associated cardiac or respiratory conditions.

The indications for colostomy versus anoplasty for newborn boys are as follows:

Anoplasty - Rectoperineal fistula


Colostomy - Rectobulbar urethral fistula, rectoprostatic urethral fistula,
rectobladderneck fistula, imperforate anus without fistula, rectal atresia

The indications for colostomy versus anoplasty for newborn girls are as follows:

Anoplasty - Rectoperineal fistula, rectovestibular fistula


Colostomy - Imperforate anus without fistula, persistent cloaca, rectal atresia,
rectovaginal fistula

Decision-making in newborn boys with anorectal anomalies

In 80-90% of newborn boys, clinical evaluation and urinalysis provide enough information for
the surgeon to decide whether a colostomy is required.

After birth, an intravenous line is placed for fluids and antibiotics. A nasogastric tube is inserted
to keep the stomach decompressed to avoid the risk of vomiting and aspiration.

Meconium is not usually observed at the perineum in a newborn with rectoperineal fistula until
at least 16-24 hours of life. Abdominal distension does not develop during the first few hours of
life but is required to force meconium through a rectoperineal fistula, as well as through a
urinary fistula. This is because the most distal part of the rectum in these children is surrounded
by a funnel-like voluntary muscle structure that keeps part of the rectum collapsed and empty.
The intra-abdominal pressure must be high enough to overcome the tone of the muscles that
surround the rectum to force meconium through the fistula. Therefore, the decision to perform a
colostomy or an anoplasty must be delayed for 16-24 hours while the surgeon evaluates for
clinical evidence of the newborns anorectal anomaly.

Clinical inspection of the buttocks is important. A flat bottom or flat perineum, as evidenced by
the lack of a midline gluteal fold and the absence of an anal dimple, indicates that the patient has
poor muscles in the perineum. These findings are associated with a high malformation; therefore,
a colostomy should be performed.
Perineal signs found in patients with low malformations include the presence of meconium at the
perineum, a bucket-handle malformation (ie, a prominent skin tag located at the anal dimple,
below which an instrument can be passed), and an anal membrane (through which meconium is
visible).

Newborn boys with rectoperineal fistula do not require a colostomy. They can undergo a
posterior sagittal anoplasty.

Newborn boys with evidence of a rectourinary tract communication should undergo fecal
diversion with a colostomy.

If none of the clinical signs to reveal the location of the anorectal anomaly are evident by 24
hours, obtaining radiographs can help. This situation is necessary in only approximately 10% of
patients. Obtain cross-table lateral radiographs with the newborn prone, with the pelvis elevated,
and with a radiopaque marker placed on the perineum (see the image below). Rarely,
radiography reveals the column of air in the distal rectum to be within 1 cm of the perineum; in
these instances, treatment is similar to that for rectoperineal fistula, and a newborn perineal
operation may be performed. If the air column is more than 1 cm from the perineum, a
colostomy is indicated.

Cross-table lateral radiograph of


a patient in which the air column in the distal rectum can be observed close to the
perineal skin.

View Media Gallery

A definitive repair in the newborn period avoids a colostomy; however, because the surgeon does
not know the precise anorectal defect, the urinary tract is at considerable risk. To definitively
determine the patient's anorectal defect, perform distal colostography, which requires the
presence of a colostomy. Without this information, an operation in the newborn period is,
essentially, a blind perineal exploration. The surgeon may not be able to locate the rectum and
may find and damage other unexpected structures (eg, posterior urethra, seminal vesicles, vas
deferens, ectopic ureters) during the search for the rectum. Finally, without fecal diversion, the
risk of dehiscence and infection exists. These complications may compromise the chance of
achieving bowel function.

The rare exception occurs when the cross-table lateral radiograph obtained at 16-24 hours reveals
that the rectum is located below the coccyx. In this case, the rectum may be reliably reached
from the posterior sagittal approach.

Obtain urinalysis and place gauze over the penis to reveal the presence of fecal matter in the
urine, which is considered evidence of a rectourinary fistula.

Abdominal ultrasonography must be performed to evaluate for the presence of an obstructive


uropathy. At the same time, spinal ultrasonography may be performed to evaluate for spinal
anomalies, including the presence of a tethered cord.

Methods to reveal the location of the distal rectum before 16 hours of life are flawed because of
the contracted state of the rectum within the funnel-like sphincter mechanism. Typically, the
funnel-shaped muscle structure is contracted unless overcome with a distending force. Tests such
as MRI, ultrasonography, CT scanning, or injection of contrast through the perineum locate the
distal rectum as falsely high in the pelvis.

Distal colostography (typically obtained 1 mo after colostomy opening) must have adequate
pressure to demonstrate a fistula from the rectum to the urinary tract. If pressure is inadequate,
this method also falsely locates the distal rectum as high in the pelvis.

Once the patient recovers from colostomy and demonstrates good growth and development, the
definitive operation can be planned for 4-12 weeks later.

Decision-making in newborn girls with anorectal anomalies

The decision to perform a colostomy is less complicated in newborn girls. In 90% of patients, a
meticulous perineal inspection demonstrates the anorectal defect. Waiting 16-24 hours for
enough abdominal distension to demonstrate the presence of a rectoperineal fistula or
rectovestibular fistula also applies to newborn females.

The most common anomaly in newborn girls is a rectovestibular fistula. Perineal inspection
reveals a typical urethra, typical vagina, and another orifice, which is the rectal fistula in the
vestibule.

In newborns with clinical evidence of a rectovestibular fistula, a diverting colostomy is the safest
option for surgeons without extensive experience in anorectal anomalies. Colostomy prior to the
main repair avoids the complications of infection and dehiscence. Definitive repair of this
anomaly in the newborn period should be reserved for surgeons who have significant experience
repairing these defects. Once this anomaly is repaired, patients have an excellent prognosis;
therefore, complications that may affect continence must be avoided.

Patients with rectovestibular fistulas in whom primary repair failed in the newborn period are the
most common referrals for re-operations to tertiary centers that care for anorectal anomalies.
Occasionally, the fistulas are large enough to decompress the gastrointestinal tract. They may be
dilated to facilitate fecal drainage until the baby is older and a definitive repair is performed.
Definitive repair involves a posterior sagittal approach. The most delicate part of this operation is
the separation of the rectum and vagina, which share a common wall.

As in boys, newborn girls may also have a rectoperineal fistula, which requires an anoplasty
procedure in the newborn period.

Similar to boys, the rectum location is not clinically evident in less than 5% of newborn girls
after 24 hours. They may have imperforate anus with no fistula. Cross-table lateral radiography
should be performed, which helps determine the need for a colostomy.

The presence of a single perineal orifice in a patient is clinical evidence of a persistent cloaca
(see the image below). See Cloacal Malformations for details regarding treatment of a persistent
cloaca. In patients with a persistent cloaca, abdominal examination may reveal a mass that likely
represents a distended vagina (hydrocolpos), which is present in 50% of patients with persistent
cloaca. Abdominal ultrasonography is helpful to reveal the presence of an obstructive uropathy
and hydrocolpos.
Perineum of a newborn with persistent
cloaca. Note the single perineal orifice.

View Media Gallery

During the perineal inspection, newborn girls are commonly given an incorrect diagnosis of
imperforate anus with rectovaginal fistula; however, all 3 structures (ie, urinary tract, vagina,
rectum) actually meet in a common channel, and the baby has persistent cloaca. This
misconception carries important therapeutic implications.

Making the correct determination is vital, because 90% of newborns with persistent cloaca have
an associated urologic problem and 50% have hydrocolpos. The urinary tract and distended
vagina both may require treatment in the newborn period to avoid serious complications.

Missing the diagnosis of persistent cloaca commonly means that an obstructive uropathy is
overlooked. The patient may then receive only a colostomy, resulting in subsequent sepsis,
acidosis, and, occasionally, death.

The other implication of missing the diagnosis of persistent cloaca involves repairing only the
rectal component of the anomaly, leaving the patient with a persistent urogenital sinus.

Types of urinary tract malformations are as follows:

Renal agenesis
Vesicoureteral reflux

Neurogenic bladder
Renal dysplasia

Megaureter

Hydronephrosis

Ectopic ureter

Imaging Studies in Newborn Period


The radiologic evaluation of a newborn with imperforate anus includes abdominal
ultrasonography to evaluate for urologic anomalies. In patients with persistent cloaca, a
distended vagina (hydrocolpos) may be identified.

Plain radiography of the spine may reveal spinal anomalies, such as spina bifida and spinal
hemivertebrae.

Plain radiography of the sacrum in the anterior-posterior and lateral projections may demonstrate
sacral anomalies, such as a hemisacrum and sacral hemivertebrae. In addition, the degree of
sacral hypodevelopment may be assessed, and a sacral ratio can be calculated by measuring the
distances between key bony structures (see the image below).

Calculation of the sacral ratio.

View Media Gallery

Spinal ultrasonography in the newborn period and up to age 3 months (at which point the sacrum
ossifies) may be performed to find evidence of a tethered spinal cord and other spinal anomalies.

Cross-table lateral radiography may help demonstrate the air column in the distal rectum in the
small percentage of patients in whom clinical evidence does not delineate the likely anorectal
anomaly in 16-24 hours.

Imaging Studies After Newborn Period


High-pressure distal colostography is performed on an outpatient basis, after the colostomy has
been created. Hydrosoluble contrast material is injected into the distal stoma to demonstrate the
precise location of the distal rectum and its likely urinary communication.
Hydrostatic pressure under fluoroscopic control is required. A Foley catheter is placed in the
mucous fistula, and the 3-cm3 balloon is inflated and pulled back to occlude the stoma during
contrast injection. The hydrostatic pressure must be high enough (manual syringe injection) to
overcome the muscle tone of the striated muscle mechanism that surrounds the rectum and keeps
it collapsed. This is the best way to demonstrate a rectourinary communication and determine the
rectum's true height.

The contrast material usually fills the proximal urethra and bladder through the fistula. The
injection is continued until the child voids, and pictures are taken during micturition to reveal, in
a single picture, the sacrum, rectum height, perineum, fistula location, bladder, vesicoureteral
reflux (if present), and urethra. When colostography is performed correctly, voiding cystography
and cystoscopy are not necessary.

Colostography is vital in determining the anatomy to plan definitive repair. In 10% of patients,
the fistula is at the level of the bladder neck; in these instances, during the main repair, the
surgeon knows that the rectum can be found only through the abdomen, and a combined
posterior sagittal and abdominal or laparoscopic approach is used.

The anorectal defect of imperforate anus without fistula may also be demonstrated with this
radiologic evaluation. Occurring in approximately 5% of patients, imperforate anus without
fistula has a good functional prognosis and is common in individuals with Down syndrome.

In most newborn girls with anorectal malformations (except for those with persistent cloaca),
distal colostography is not necessary because the fistula is clinically evident.

If the spine was not evaluated with ultrasonography in the newborn period, magnetic resonance
imaging is necessary after age 3 months to exclude the presence of tethered cord and other spinal
anomalies.

Approach Considerations
Indications for surgical treatment (colostomy vs anoplasty) are discussed more fully elsewhere
(see Presentation). No absolute contraindications to surgery exist. The presence of associated
malformations, such as prematurity and congenital cardiac disease, often postpones definitive
colorectal reconstruction.

Potential methods of evaluation for anorectal malformations, including prenatal diagnosis and
genetic karyotyping to reveal familial disposition, are areas of rapid advance.

Medical Therapy
Associated malformations must be addressed.
Genitourinary defects

Approximately 50% of all patients with anorectal malformations have an associated urogenital
anomaly, which commonly varies with the type of anorectal defect.

A list of anorectal defects and the percentages of patients with associated urogenital anomalies is
as follows:

Persistent cloaca - 90%


Rectobladderneck fistula - 84%

Rectoprostatic urethral fistula - 63%

Rectovestibular fistula - 47%

Rectobulbar urethral fistula - 46%

Rectoperineal fistula - 26%

Imperforate anus without fistula - 31%

All patients must be examined at birth for these defects; the most valuable screening test is
abdominal and pelvic ultrasonography. Urologic evaluation prior to colostomy provides the
surgeon the necessary information to address the urologic problem if needed.

Tethered cord

A tethered spinal cord refers to the intravertebral fixation of the phylum terminale. Tethered cord
has a known association with anorectal malformation; approximately 25% of patients with
anorectal malformation have a tethered spinal cord. The prevalence of this anomaly increases
with increasing height and complexity of the anorectal anomaly. In addition, patients with a
hypodeveloped sacrum and associated urologic problems are more likely to have tethered cord.
Motor and sensory disturbances of the lower extremities may result.

Patients with anorectal malformations and tethered cord have a poorer prognosis for bowel and
urinary function; they also have higher anorectal defects, less-developed sacra, other spinal
problems, and less-developed perineal musculature. The actual impact of tethered cord alone on
functional prognosis remains unclear.

The neurosurgical literature indicates that untethering the cord avoids motor and sensory
problems. No evidence suggests that this operation affects the functional prognosis of patients
with anorectal malformation, but may improve bladder emptying capacity.

Spinal ultrasonography in the first 3 months of life and magnetic resonance imaging
(MRI) thereafter are useful radiologic modalities to establish the diagnosis.
Sacral and spinal defects

The sacrum is the most commonly affected bony structure. Traditionally, the number of sacral
vertebral bodies was counted to evaluate the degree of sacral deficiency. A more objective
assessment of the sacrum may be obtained by calculating a sacral ratio. The sacrum is measured
and its length is compared with bony parameters of the pelvis (see the image below). The lateral
view is more accurate than the anteroposterior view because its calculation is not affected by
pelvic tilt.

Calculation of the sacral ratio.

View Media Gallery

Assessment of sacral hypodevelopment correlates with the patient's functional prognosis. Normal
sacra have a calculated sacral ratio greater than 0.7. Bowel control has rarely been observed in
patients with calculated sacral ratios of less than 0.3. Hemisacrum is almost always associated
with a presacral mass (teratomas or anterior meningoceles). Hemivertebrae may also affect the
lumbar and thoracic spine, predisposing to scoliosis.

Patients may have spinal anomalies other than tethered cord, such as syringomyelia and
myelomeningocele.

Surgical Therapy
As discussed previously, the surgeon must decide in the newborn period whether the child
requires fecal diversion with a colostomy or if a primary procedure is possible.

Colostomy

A descending colostomy with separated stomas is recommended. The advantages of this type of
colostomy include the following:

Only a small portion of distal colon is defunctionalized, but with an adequate


amount of rectosigmoid for the future pullthrough
Washing and cleaning the portion of the colon distal to the colostomy is
relatively easy

Distal colostography is easy to perform


The separated stomas prevent spillage of stool from proximal to distal bowel,
which avoids impacted distal stool and urinary tract infections

Prolapse with this technique is uncommon; proximal stoma prolapse in a


normally rotated colon should not occur with this technique, because the
colon is well fixed to the retroperitoneum just before the colostomy rises to
skin level; because the distal stoma is in a mobile portion of the colon, it may
prolapse; the distal stoma must intentionally be made small, both to avoid
prolapse and because it is used only for irrigations and radiologic studies

When performing a colostomy in the newborn, the distal bowel should be irrigated to remove all
the meconium. This prevents formation of a megasigmoid, which may lead to constipation after
the colostomy ultimately gets closed.

Colostomy errors include the following:

Too-distal sigmoidostomy - In this most common error, the colostomy is


placed too distal and interferes with the pullthrough procedure
Right upper sigmoidostomy - Instances of inadvertent sigmoid colostomy
placed in the right upper quadrant during an attempt to perform a transverse
colostomy have occurred. Inadvertent anchoring of the sigmoid in the right
upper quadrant interferes with the pullthrough procedure

Incomplete diversion of stool - A loop colostomy does not divert the stool
completely and allows for distal stool impaction and urinary tract infections

Megarectum - Transverse colostomies may produce megarectum, due to


passage and accumulation of mucus

Definitive repair

Repair of an anorectal malformation requires a meticulous, delicate technique and a surgeon with
experience in treating these defects. The posterior sagittal approach is ideal for defining and
repairing anorectal anomalies.

Anorectal abnormalities in 90% of newborn boys may be repaired solely with a posterior sagittal
approach, whereas 10% require an additional abdominal component (with laparotomy or
laparoscopy) to mobilize a very high rectum. All anorectal malformations in newborn girls may
be repaired with the posterior sagittal approach, with the exception of approximately 30% of
instances of persistent cloaca. In this 30%, the rectum or vagina is high enough to also require an
abdominal approach.

A Foley catheter is placed first; then, patients are placed in the prone position with the pelvis
elevated.

The posterior sagittal incision length varies depending on the anorectal defect. Perineal fistulas
are repaired with a minimal posterior sagittal incision that is large enough to divide the external
sphincter and to mobilize the anus back to the center of the sphincter complex. The sphincter
mechanism is always located posterior to the fistula site. This operation may be performed in the
neonatal period without a protective colostomy.

The posterior sagittal approach is based on the fact that nerves do not cross the midline.
Remaining exactly in the midline, the surgeon preserves the innervation of all the important
pelvic structures.

An electrical stimulator helps reveal the location of the sphincteric mechanism. The parasagittal
fibers, the muscle complex, and the levators are identified during the dissection. The external
sphincter is represented by muscle fibers that run parallel to the midline in a parasagittal fashion.
A muscle structure termed the levator mechanism lies medial to these fibers and represents the
lower end of the funnel-like voluntary muscle.

The levator mechanism extends in continuum down to the skin, but this was not known prior to
the use of the posterior sagittal approach. Electrically stimulated, the parasagittal fibers elicit a
contraction that results in shortening of the same fibers. The rectum pushes forward toward the
pubic bone when the upper portion of the levators are stimulated. Stimulating the lower part of
the funnel-like muscle structure elevates the anal dimple. This group of muscle fibers, termed the
muscle complex, extends from the levator mechanism down to the skin and is located
immediately medial to the parasagittal fibers.

The surgeon opens posterior sagittally. The skin and subcutaneous tissue are divided, and the
parasagittal fibers below are divided in the midline, as is the muscle complex. The levator muscle
is then opened, and the rectum is found, except in patients with a true supralevator malformation
(10% of cases), in whom the surgeon will instead find a genitourinary structure with this
approach.

When the rectum is located, its posterior wall is opened in the midline to demonstrate the
presence of a fistula. This posterior incision in the rectum is carried down to the fistula site. The
rectum and urethra share a common wall. Meticulous dissection is required to separate the distal
rectum from the urethra. A submucosal dissection must be performed in the first 5 mm above the
fistula site.

The rectum is separated from the urinary tract. This dissection is facilitated through placing
multiple 6-0 silk sutures in the rectal mucosa to exert uniform traction. The opening in the
urethra is then closed with absorbable suture.

Once the rectum is separated, it is then mobilized down to reach the perineum by
circumferentially dividing the bands and vessels that hold the rectum up in the pelvis. The
intramural blood supply of the rectum is excellent; therefore, the rectum remains viable. Because
the newborn depends on this intramural blood supply, preventing damage to the rectal wall is
vital.

Once the rectum is fully mobilized, the size of the rectum and the available space must be
assessed. The rectum occasionally requires tapering to fit the limits of the sphincteric
mechanism. The tapering should take place on the posterior wall so that the suture lines of the
tapered rectum and the closed urethral fistula do not lie next to each other.

The rectum is placed in the limits of the sphincter mechanism, which is reconstructed around it
in the midline. The rectum is sutured to the perineal skin (anoplasty).

In 10% of newborn boys with this defect, the rectum enters the urinary tract at the bladderneck
level. The repair of this malformation involves a posterior sagittal incision and an abdominal
component. The distal rectum is separated from the urinary tract, mobilized, and pulled through
to lay within the sphincteric funnel. The pathway is just under the coccyx and sacrum in the area
of the pelvic retroperitoneum.

To mobilize the rectum off of the bladderneck, an abdominal component is required via
laparoscopy or laparotomy. The fistula is ligated with great care to avoid injuring the ureters and
vas deferens: the plane of dissection must be made close to the bowel wall of the rectosigmoid,
2-3 cm above the peritoneal reflection. The rectum is then mobilized for adequate length to reach
the perineum without tension.

The fistula is divided and sutured with absorbable material. The rectum is passed through to the
posterior sagittal incision and an anoplasty is performed. This part can be done with legs lifted
up, remaining in supine position.

In patients with imperforate anus without fistula, the same meticulous dissection is required to
separate the distal rectum from the urinary tract as in patients with rectourinary fistulae, because
the rectum and urethra still share a common wall.

In patients with rectovestibular fistula, the posterior sagittal incision may be shorter than in
newborn boys with rectourethral fistulae. Often, the entire levator mechanism does not need to
be divided; only the external sphincter, muscle complex, and part of the lower portion of the
levator mechanism require division. The rectum and posterior vagina share a common wall; this
separation is the most difficult part of the operation. Once the rectum is completely mobilized,
the perineal body is constructed, and the rectum is placed within the limits of the sphincter
mechanism.

A rare malformation, rectal atresia, occurs in 1% of patients. The anal canal is normal, and,
externally, the anus appears typical. However, a blockage exists 1-2 cm from the anal skin and is
usually found when the nurse tries to pass a thermometer. These babies should undergo
colostomy at birth; definitive repair involves a posterior sagittal approach and an anastomosis
between the posterior rectum and the anal canal. This defect is particularly associated with a
presacral mass, which must be sought.

Postoperative Details
Postoperative management

The posterior sagittal incision is relatively painless.


In patients with a rectourethral fistula, the Foley catheter usually stays in place for approximately
5-7 days.

If the colostomy is untouched during the operation and laparotomy or laparoscopy was not
necessary, oral feedings may be started immediately postoperatively. If a laparotomy or
laparoscopy was necessary, the patient may require a period of fasting and nasogastric
decompression.

At 2 weeks after surgery, anal calibration is performed, followed by a program of anal


dilatations. The anus must be dilated twice daily, and the size of the dilator is increased every
week. The final size to be reached depends on the patients age. Once the desired size is reached,
the colostomy may be closed. Dilatations are continued afterward according to a prescribed
protocol. Dilatations are a vital part of postoperative treatment to avoid an anoplasty stricture.

After colostomy closure, severe diaper rash is common, because the perineal skin has never
before been exposed to stool.

Postoperative functional disorders

Constipation is the most common problem encountered after treatment for imperforate anus. It is
the most important problem to avoid after definitive repair in newborn girls with rectovestibular
or rectoperineal fistula and in newborn boys with rectobulbar urethral fistula, imperforate anus
without fistula, and rectoperineal fistula. Failure to avoid constipation may result in megarectum
and megasigmoid and can lead to fecal impaction and overflow incontinence. See Bowel
Management for further discussion.

The origin of the constipation problem is unknown. Originally, the perirectal dissection was
believed to cause a degree of denervation that resulted in constipation. However, on careful
review of the largest patient series, those with the most benign defects (ie, the least amount of
perirectal dissection) experienced the worst constipation.

The presence of a megarectum prior to the pull-through procedure correlates with postoperative
constipation. Megarectum is more common in patients who underwent a transverse or loop
colostomy during the newborn period.

Constipation appears to be a hypomotility disorder secondary to chronic bowel dilatation;


alternatively, the hypomotility may cause the dilatation. Dilatation causes constipation, creating a
vicious cycle. Patients who have undergone an older operation, an abdominoperineal operation
for imperforate anus that included rectum resection, are prone to develop diarrhea because of
lack of a rectal reservoir. Incontinence in these patients is much more difficult to treat because
stool constantly passes (see Bowel Management).

Occasionally, constipation becomes so severe that patients develop chronic fecal impaction and
constant soiling. These patients are often referred to as having fecal incontinence. However, if
the patient has a type of anorectal anomaly with a good prognosis, this incontinence is often
overflow pseudoincontinence. Once the constipation is treated, the patient regains continence.
When constipation is severe and the patient has a megasigmoid (and the patient is fecally
continent), resection of the sigmoid has been found to dramatically reduce the patient's laxative
requirements. The descending colon with normal caliber and motility is anastomosed to the
rectum at the peritoneal reflection. This procedure is useful for the select group of patients who
require enormous amounts of daily laxatives to keep their colons clean. The rectal reservoir
should be preserved to avoid the problem of diarrhea-related incontinence.

Complications
Complications of surgery include dehiscence and infection, which may be avoided with
colostomy before the main repair. These complications may compromise the chance of achieving
typical bowel function.

With inadequate preoperative anatomic information, the urinary tract is at considerable risk
because the surgeon does not know the precise anorectal defect and if the surgeon approaches a
low rectum transabdominally, they risk leaving behind the very distal rectum, which becomes a
posterior urethral diverticulum.

Long-Term Monitoring
The key in these patients is to treat constipation proactively and, if possible, avoid it after the
pull-through procedure altogether. Patients must be regularly monitored, and laxatives and
dietary manipulations are begun at the first sign of constipation.

Patients may experience soiling. This may represent fecal incontinence in patients with very high
imperforate anus or in those with poor muscles and an abnormal sacrum. These patients require a
bowel management program (see Bowel Management). However, in a patient with a good
prognosis, soiling may represent overflow incontinence, and constipation must be treated.

The childs bowel movement pattern before toilet-training may provide important information
concerning the potential for continence. For example, a 1-year-old child who has undergone a
pullthrough procedure for imperforate anus and has one to three discrete bowel movements per
day has good potential for future fecal continence. Signs of feeling are demonstrated while the
child is pushing during a bowel movement.

On the other end of the spectrum, a child who has fecal incontinence passes stool constantly
without evidence of pushing or feeling. Whereas a child with a typical bowel movement pattern
is trainable, a child with the pattern of fecal incontinence likely requires a bowel management
program with a daily enema. The child with true fecal incontinence should not be expected to
achieve voluntary bowel control.

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