-The upper 2/3s of the anal canal is derived from the hindgut (endoderm), the
lower 1/3 of the anal canal is derived from the proctodeum (surface ectoderm).
-The pectinate line (structure A) marks the junction between the endoderm and
the surface ectoderm. The lower third derived from the surface ectoderm is also
known as the anal mucosa or the anoderm.
(e.g A patient with a transphincteric, high type fistula. The fistula should be
ligated rather than resecting the sphincter).
Continence Mechanisms
1- Sphincter
2- Anoderm
3- Presence of a hollow organ to act as a reservoir (which is the colon and
the rectum)
4- Peristaltic activity of the large bowel
Congenital Anomalies
-Imperforate Anus
Definition
Incidence
Boys> Girls
Presentation/Symptoms
Look at the patient’s bottom. You won’t be able to see the anal canal or the anal
verge. The diagnosis is made often shortly after birth.
Diagnose if the defect is high type or low type using an invertogram (see later)
and make sure there are no other congenital anomalies
Pathophysiology
The terminal part of the hindgut, the cloaca, is an endoderm lined chamber in
contact with the surface ectoderm at the cloacal membrane. The cloaca, is in
connection with the allantois ventrally which is a fingerlike diverticulum of the
yolk sac. –Moore’s Before We are born
The cloaca is a common chamber between the hindgut and the allantois. By the
12th week of pregnancy the bowel has herniated and forms outside the abdomen.
By the end of the first trimester the bowel returns back and the allantois remains
as the connection between the umbilicus and the cloaca.
The allantois is also connected to the future bladder through the urachus. The
urachus degenerates to form the median abdominal fold (covered with
peritoneum). A patent urachus can cause some urine to be excreted from the
umbilicus.
Future patency between the bowel lumen and the umbilicus can lead to a patent
omphalomesenteric duct which can cause succus entericus to be seen at the
umbilicus. Succus is the content of the small bowel that will be converted into
stool as it reaches the large intestine. The connection of the duct with the
intestine is 2 feet from the ileocecal valve.
The image to the left shows a child with an imperforated anus. The condensation
seen is not an opening but is rather a skin dimple developed by the aggregation of
the muscles of the external sphincter. In patients where the natal cleft (the cleft
that separates the buttocks into a right and left buttock) is absent, the muscles of
the sphincter are scattered and hence no anal skin dimple is seen. This signifies
that the rectum is higher up and the lesion is of the high type. This can mean that
the prognosis is worse regarding continence (things will become clearer after
reading the classification section).
Classification
I- Low type
II- High type
The classification depends on the distance of the hollow of the rectum from the
surface of the skin. The distance is measured using an invertogram (X-ray image
taken with the child in the prone position or hung upside down. A piece of metal
is attached to the anal dimple and an X-ray is taken. The distance between the
hollow of the rectum and the skin’s surface).
The invertogram should be taken no earlier than 24 hours after birth to make
sure that the gas has reached the rectum and hence avoid misdiagnosis
High type >2.5 cm between the rectum and the anal dimple on the invertogram
Low type <2.5 cm between the rectum and the anal dimple on the invertogram
I- Low type
This is one of the most common types of imperforated anus with anal atresia.
It is also known as anterior ectopic anus.
B- Low type imperforated anus with persistent anal membrane (possibly seen
in Down’s syndrome)
In this case there is no fistula. The anal membrane however is persistent and
seals the anal opening. Treatment is by incising the membrane and dilating the
anal canal.
The exact location of the fistula is in the center of the trigone. This means that
its surrounded laterally by the ureter openings, superiorly by the vas deferens,
and inferiorly by the prostate and the seminal vesicle. The location of the
fistula is critical as the bladder will be neurogenic and in this case often
incontinence for urine and stool is seen even after corrective surgery*
Cloacal ectopia: is a severe birth defect wherein much of the abdominal organs
(the bladder and intestines) are exposed. It often causes the splitting of
both male and female genitalia (specifically, the penis and clitoris respectively),
and the anus is occasionally sealed.
VACTERL
Vertebral anomalies (especially sacral anomalies). See the image below for an
explanation of sacral ratio index, a parameter that can be used clinically to
determine the presence/absence of sacral anomalies. Sacral anomalies can be
associated with problems in the sacral plexus causing losses of urinary and stool
continence
Anal atresia
Renal Agenesis
Limb defects
Treatment
1- Receive patient
2- Control sepsis
3- Support nutrition
4- Draw anatomy
5- Do Surgery
If the defect is of low type and the meconium is seen just behind the anal
membrane, there is no need for diversion and simple incision and anal dilation
will resolve the problem.
Surgical correction:
Stage 1: Left iliac fossa laparotomy and placing a sigmoid colostomy. A separated
(divided) colostomy rather than a loop colostomy needs to be created. This is the
initial treatment to allow the child to feed before correcting the anomaly later in
life.
Stage 2: Rectal pull through( rectum is taken down and implanted in the sphincter
mechanism).
New procedure developed by Alberto Pena where the skin is incised along with
the sphincter along the midline and other layers, then the rectum is taken and
pulled through and implanted into the sphincter again.