CHAPTER

rectosigmoid, followed by another resting period of

about 24 hours, during which the rectosigmoid again acts
as a reservoir. The importance of rectosigmoid motility
and its relevance for bowel control have largely been
underestimated.
Children with anorectal malformations have a spectrum
of rectosigmoid motility disorders. Children subjected
to surgical techniques that preserve the rectosigmoid
suffer from constipation. Constipation, one of the most
important functional sequelae, is probably the result of
hypomotility of the rectosigmoid. The hypomotility is self
perpetuating and self-aggravating to the point that if left
untreated, megasigmoid develops. In extreme cases, fecal
impaction and encopresis or overflow pseudoinconti
nence may develop. It seems that constipation is worse
with lower defects. Knowing this and the fact that hypo
motility can begin a vicious cycle leading to megasigmoid,
it is incumbent on the pediatric surgeon to avoid
the cycle
of hypomotility,
constipation,
and
megasigmoid. Aggressive patient follow-up plus dietary,
mechanical, and pharmacologic treatment prevents this
cycle.
Children with anorectal malformations who, for what
ever reason, have lost their rectosigmoid suffer from the
exact opposite (i.e., tendency for diarrhea). These children
have no reservoir capacity, are highly sensitive to fruits and
vegetables, and worst of all, suffer from incontinence.

CLINICAL FINDINGS AND INITIAL MANAGEMENT

Figure 101-2 shows the decision-making algorithm for
the initial management of male patients.
A clinician called to see a newborn male with an anorec
tal malformation must perform a thorough perinea!
inspection, which usually provides the most important
clues about the type of malformation that the patient has
(Fig. 101-3). It is important to not make a decision about
colostomy or primary surgery before 24 hours of age.
The reason for this delay is that significant intraluminal
pressure is required for meconium to be forced through
a fistula orifice. Passing of meconium through a fistula is
the most valuable sign of the location of the fistula in

Uf.\jijJflMifJ Algorithm for the

treatment of a male
newborn with an anorectal malformation. PSARP,
posterior
sagittal anorectoplasty; R/0, rule
out.

101

Anorectal Malformations

1569

these babies. If meconium is seen on the perineum, it

is evidence of a rectoperineal fistula. If there is
meconium in
the urine,
the
diagnosis of a
rectourinary fistula is obvious (see Fig. 101-3).
Radiologic evaluations do not show the real anatomy
before 24 hours because the rectum is collapsed. It takes
a significant amount of intraluminal pressure to over
come the muscle tone of the sphincters that surround
the lower part of the rectum. Therefore, radiologic eval
uations done too early (before 24 hours) will most likely
reveal the presence of a "very high rectum" and yield a
false diagnosis.
During the first 24 hours, the baby should receive
intravenous fluids, antibiotics, and nasogastric decom
pression to prevent aspiration. The clinician should use
these hours to rule out the presence of associated defects
such as cardiac malformations, esophageal atresia, and
urologic problems. An echocardiogram can be per
formed, and the baby should be checked for the presence
of esophageal atresia. A plain radiograph of the lumbar
spine and sacrum should be taken to evaluate for herniver
tebrae and sacral anomalies. Spinal ultrasound helps
screen for tethered cord and other spinal problems.
Ultrasonography of the abdomen evaluates for the pres
ence of hydronephrosis.
If the baby has signs of a perinea! fistula, anoplasty can
be performed without a protective colostomy during the
first 48 hours of life.
After 24 hours, if no meconium is seen on the perineum
or in the urine, we recommend obtaining a cross-table
lateral x-ray film with the baby in prone position.w If the
gas in the rectum is located below the coccyx and
the baby is in good condition with no significant
associated defects, depending on the surgeon's
experience, a poste rior sagittal operation without a
protective
colostomy can be considered. A more
conservative alternative would be
to
perform
a
colostomy, with the definitive repair planned for a
second stage.
If rectal gas is seen above the coccyx and the patient
has meconium in the urine, significant associated defects,
and an abnormal sacrum or a flat bottom, a colostomy is
recommended with postponement of the main repair for

.
>

Newborn Male - Anorectal Malformation

Perial

(R/0 serious, potential y lethal associated defects)

Inspection

Perinea!
fistula

Rectal gas below coccyx

No associated defects

Rectal gas above coccyx

Associated defects
Abnormal sacrum
Flat bottom

Anoplasty

Consider PSARP
with or without
colostomy

Colostomy