Notes:

CLOACA- The cloaca is a structure in the development of the urinary and

reproductive organs.
The hind-gut is at first prolonged backward into the body-stalk as the tube of
the allantois; but, with the growth and flexure of the tail-end of the embryo,
the body-stalk, with its contained allantoic tube, is carried forward to the
ventral aspect of the body, and consequently a bend is formed at the
junction of the hind-gut and allantois.

This bend becomes dilated into a pouch, which constitutes the endodermal
cloaca; into its dorsal part the hind-gut opens, and from its ventral part the
allantois passes forward.
At a later stage the Wolffian duct and Müllerian duct open into its ventral
portion.

The cloaca is, for a time, shut off from the anterior by a membrane, the
cloacal membrane, formed by the apposition of the ectoderm and entoderm,
and reaching, at first, as far forward as the future umbilicus.

Behind the umbilicus, however, the mesoderm subsequently extends to form

the lower part of the abdominal wall and symphysis pubis.

By the growth of the surrounding tissues the cloacal membrane comes to lie
at the bottom of a depression, which is lined by ectoderm and named the
ectodermal cloaca.

ALLANTOIS- Allantois (plural allantoides or allantoises) is a part of a

developing animal conceptus (which consists of all embryonic and extra-
embryonic tissues). It helps the embryo exchange gases and handle liquid
waste.

This sac-like structure is primarily involved in nutrition and excretion, and is

webbed with blood vessels.

The function of the allantois is to collect liquid waste from the embryo, as
well as to exchange gases used by the embryo.

HINDGUT- The hindgut (or epigaster) is the posterior (caudal) part of the
alimentary canal. It includes the distal third of the transverse colon and the
splenic flexure, the descending colon, sigmoid colon and rectum. The back
part of the embryonic gut, which gives rise to part of the large intestine, the
rectum, bladder, and urinary ducts.
EMBRYOLOGY OF THE ANUS AND
RECTUM

A series of developmental steps are involved in the formation of the

normal anatomy of the lower end of the anus, rectum, and
genitourinary (GU) tract. By the fourth week of embryologic
development, the cloaca and the cloacal membrane are present
(Figs. 61-1 and 61-2A). This membrane separates the internal from
the external portions of the cloaca. At this stage, the internal portion
of the cloaca receives the allantois, the wolffian ducts, and the
portion of the hindgut that becomes the rectum. Between the fourth
and sixth weeks of development, the upper part of the cloaca begins
to be divided by the urorectal septum, growing caudally from
above. The descent of the urorectal septum is associated with
simultaneous
lateral ingrowths that result in an anterior chamber receiving the
allantois and the wolffian ducts and a posterior chamber receiving
the rectum. Failure of the cloacal membrane to develop posteriorly
results in the development of an anteriorly placed hindgut opening
(Fig. 61-2B). The extent of the failure of development of the
posterior aspect of the cloacal membrane determines the degree of
misplacement of the fistula: A mild failure results in a perineal
fistula, whereas a severe developmental failure results in a
rectourethral or even a rectovesical fistula in boys and a
rectovaginal fistula in girls. Failure of development of the anterior
and posterior cloacal membranes may result in the formation of a
cloaca with a common hindgut and urogenital opening (Fig. 61-
2C).
During the time that the previously mentioned events are
occurring, at the level of the perineum, mesodermal tissue
develops on the surface, resulting in the formation of a genital
tubercle, genital folds, and an anal tubercle in the region of the
external cloaca. By the sixth week of
development, the cloacal membrane gradually atrophies,
permitting the future GU tract and rectum to empty into the
external cloaca. Between 6 and 10 weeks' gestation, the urorectal
septum, now representing a uroanal septum, gradually grows
caudally into the external cloaca, and at the same time, there is
inward migration of the genital folds on either side, gradually
fusing to form the perineum, separating the genitourinary and anal
canals. The anal orifice develops as a separate structure by the
ringlike fusion of the right and left anal tubercles around the anal
orifice. Patency of the anal canal usually is established by 7 or 8
weeks' gestation followed by a secondary occlusion resulting from
adhesion of the anorectal walls followed later by recanalization
secondary to apoptotic cell death. Failure of recanalization of the
secondarily occluded anal canal results in a normally located
imperforate anal membrane, such as that observed with a
membranous covering, or a bucket-handle deformity. Between this
point and 10 to 12 weeks' gestation, there is continued elongation
of the urethra and anal canals, but the external genitalia are not yet
developed. By 14 to 16 weeks' gestation, male and female
differentiation becomes evident.

NORMAL ANORECTAL ANATOMY AND FUNCTION

Continence is related to normal function of the muscle sphincters

surrounding the anus and rectum and the degree to which they are present
and have appropriate nerve stimulation. Development of the sacrum occurs
at around the same time as development of the anus, rectum, and
sphincters. This is an important consideration because the nerves that are
located near the sacrum supply the muscle sphincters that control
continence. If the sacrum does not develop normally, these nerves may not
develop or function normally either. During normal development there are
sensory receptors in the lining of the anal canal that are important for
continence. These may be absent in children with imperforate anus. Humans
normally have 3 muscle groups around the anus and rectum that are
important for continence, the external sphincter, internal sphincter, and
levator complex. Children born with imperforate anus may have dysfunction
or absence of any one of these components. The internal and external
sphincters control our ability to squeeze the anus shut. The various portions
of the levator ani muscle are shaped like a cone surrounding the anus and
rectum. When these muscles squeeze they pull the rectum forward,
increasing the angle of the bowel before it enters the anal canal. A proper
rectoanal angle helps to maintain continence by preventing formed stool
from entering the anal canal. The levator muscles are also supplied by
nerves that are close to the sacrum. This is important because as a general
rule, if a part of the sacrum is missing, the nerve that was associated with it
is likely absent also.

Innervation of the Anus, Rectum, and their Sphincters

Nerve segments coming from portions of the sacrum supply the anus and
rectum, urethra, bladder, and vagina, including the various components of
the levator ani complex (the muscles of the pelvis). These nerves also serve
as the skin sensory receptors of the anus and surrounding skin areas. The
lining to the anal canal and skin around the anus are extremely sensitive to
pain, touch, cold, pressure, tension, and friction. Evidence suggests that
similar sensory receptors are located in the surrounding pelvic musculature.
These receptors are able to distinguish between rectal contents that are
solid, liquid, and gas. The anal canal and the rectum above the anal lining
are mostly insensitive to pain but are sensitive to distention. Fecal
continence is possible only when appropriate sensation from the anal lining,
the rectal wall, and the surrounding muscles are received and processed
normally within the brain and appropriate signals are then sent back out to
the various muscles that control continence. Under normal circumstances,
the anal canal is closed except when having a bowel movement. When
defecation is to occur, abdominal pressure increases and causes the pelvic
floor (levator complex) to drop down as the various muscles of continence
relax. The feces are evacuated followed by return of the levator and
continence muscles to baseline tone.

IMPERFORATE ANUS

Classification

In very simplified terms, during development the rectum descends into the
pelvis and is supposed to end at the anal opening. In both sexes, anorectal
deformities are divided into high, intermediate, and low anomalies. These
classifications depend on how far into the pelvis the rectum descended, its
relation to the surrounding muscles and whether there is a fistula (abnormal
connection) to the urinary tract in males or the vagina in females. The
incidence of anorectal malformations is approximately 1 in 5000 live births.
They occur slightly more commonly in boys, and boys are twice as likely as
girls to have high or intermediate anomalies. In boys with an intermediate or
high anomaly, 85% have a rectourinary fistula (abnormal connection
between the rectum and urethra) In terms of low-lying anomalies, 35% of
boys have a thin membrane covering the anal fistula, whereas 93% of girls
have an external fistula. Although imperforate anus may occur as an isolated
malformation, it may coexist with duodenal atresia, esophageal atresia,
vertebral and renal anomalies, Down syndrome, and congenital heart
disease in 50% to 60% of patients. The VATER or VACTERL syndrome
(vertebral, anal, cardiac, tracheoesophageal, renal, and radial limb
anomalies) occurs in approximately 15% of patients. A tethered cord and
other types of spinal cord abnormalities are observed in half of patients with
imperforate anus. Approximately 60% of patients with high or intermediate
forms of imperforate anus have some form of associated genitourinary (GU)
malformation or vesicoureteral reflux, a condition where urine goes back
upstream from the bladder to the kidneys. The incidence of GU malformation
with low anomalies is only 15% to 20%; however, associated anomalies of
the GU tract are particularly important to recognize early if problems with
the kidneys are to be avoided.
Initial Management of the Newborn with Imperforate Anus

The first decision in the assessment of the newborn with an anorectal

anomaly is whether a colostomy is required. A colostomy is when the
surgeon sutures a portion of the intestine to the abdominal wall and the child
passes stool into a plastic bag for a period of time. This is usually a
temporary colostomy. Although it is always better to err on the side of
performing a colostomy, there are many anomalies in which one is
unnecessary. Examination of the perineum (the area of the body around
where the anus is supposed to be) is important because it may provide
evidence of whether the imperforate anus is a high, intermediate or low
lesion. If possible it is best to wait 24 hours prior to any surgery to allow
progression of gas or meconium through the bowel down as far as it can
pass before finalizing the assessment.

In the literature, some controversy still exists about the normal and
abnormal development of the human anorectum. Therefore, a three-
dimensional and histological study was performed on human embryos. In
early anorectal development (< or = 49 days postfertilization), the cloaca
plays a crucial role, separated from the amniotic cavity by its cloacal
membrane. In the cloaca, the yolk sac/primitive hindgut and
allantois/primitive urogenital sinus enter. During the embryonic caudal
folding process, incorporation of these structures occurs, including their
surrounding extraembryonic mesoderm, which fuses to form the urorectal
septum. Consequently, this septum does not grow in the direction of the
cloacal membrane, and fusion of these structures is likewise never observed.
The cloaca remains as such until the cloacal membrane ruptures by
apoptotic cell death. The dorsal part of the cloaca then becomes part of the
amniotic cavity, and is by no means involved in the development of the
anorectum. The tip of the urorectal septum will become the perineal area.
Soon after rupture of the cloacal membrane, during late anorectal
development (> or = 49 days postfertilization), a secondary occlusion of the
anorectal canal occurs, first due to adhesion, followed by formation of an
epithelial "plug" at the level of the anal orifice. Recanalization, by apoptotic
cell death, of this secondary occluded anal orifice occurs later during
development. Based on these embryological observations, congenital
anorectal malformations with an abnormal communication to the exterior are
best explained as early embryonic defects. The abnormal communications,
usually called fistulae, should be regarded as ectopic anal orifices. Anorectal
malformations with the anus in normal position are best explained as late
embryonic defects.
In the past, several theories have been proposed to explain the occurrence
of anorectal malformations. Most investigators believe that these
malformations are the result of an impaired process of septation. However,
in 1986 vd Putte challenged all theories that tried to explain anorectal
malformations by a faulty fusion of lateral ridges of the cloaca. To elucidate
the principles of normal and abnormal cloacal development, the authors
studied the morphology of this region in normal embryos of rats and
abnormal embryos of SD mice, which often have abnormal cloacas. Using
scanning electron microscopy (SEM), 245 normal rat embryos and 80
abnormal SD-mice embryos were observed. The results were as follows.(1) In
normal embryos the region of the future anal opening can be identified soon
after the establishment of the cloacal membrane. This part is a fixed point in
cloacal development.(2) In abnormal embryos the cloacal membrane is too
short. The region of the future anal opening is missing.(3) In abnormal
embryos a spectrum of malformed cloacas can be observed. This is in
accordance with the spectrum of anorectal malformations clinically observed
in humans.(4) The authors' observations support recent findings that the
"fistula" in anorectal malformations resembles a normal anus at an ectopic
position.

In Emman’s analysis and understanding, IN SHORT: A series of

developmental steps are involved in the formation of the normal
anatomy of the lower end of the anus, rectum, and genitourinary (GU)
tract. By the fourth week of development, the cloaca and a structure
called the cloacal membrane are present. The cloaca is a normal
structure in birds and is present for a short period of time during the
development of the human before birth. A cloaca is a structure into
which the colon, urinary tract and genitalia all drain and exit the body
with a single opening. A human goes thorough a stage of development
where a cloaca normally exists and then goes on to develop separate
openings for the rectum, urinary tract and in girls, the vagina. This
normal development is in much part due to the development of a
structure called the cloacal membrane. If the membrane does not
develop normally the cloaca may persist at birth, imperforate anus
happens, which of which is the ½ dorsal rectal component that failed
to develop AND RECANAL ITSELF, and the ventral urogenital part
became the urinary tract and the genitals.

The hindgut comes in contact with the cloacal membrane during the
6th week of fetal development. At this time, the hindgut is divided into
a ventral urogenital and dorsal rectal component. By the 8th week, the
dorsal 1/2 perforates to the exterior. In imperforate anus, the process
is arrested during this critical period.