Professional Documents
Culture Documents
Anorectal Malformations
Anorectal Malformations
Anorectal Malformations
PRESENTED BY :
FREQUENCY:
Anorectal malformations occur in approximately 1 in 5000 live births.
EMBROYOLOGY :
The terminal part of hind gut enters into the cloaca, an endoderm- lined cavity in
direct contact with surface ectoderm. A transverse ridge, the urorectal septum arises in
the angle between allantois and hind gut. The septum grows caudally thereby
dividing the cloaca into an anterior portion, the primitive urogenital sinus and a posterior
anorectal canal. The cloacal membrane is then divided into posterior anal membrane and
anterior urogenital membrane.
In the meantime the anal membrane is surrounded by mesenchymal swelling and
in the eighth weeks it is located at the bottom of and ectodermal dipression known as anal
pit or proctodeum in the ninth weeks anal membrane ruptures and open pathway with
outside is formed. Any abnormality in the above proccess leads to the various anorectal
malformations.
CLASSIFICATION :
1. Wingspread classification :
The most common previous international classification was referred to as the
Wingspread classification of anorectal malformations, elaborated in Wingspread, Wis, in
1984. This classification distinguished between high, intermediate, and low anomalies in
the male and female, with special groups established for cloacal and rare malformations.
High-type anorectal malformations were subdivided into anorectal agenesis with and
without fistula, and rectal atresis. The intermediate malformations were classified as
rectovestibular and rectovaginal fistula in the female and rectobulbar fistula in the male
as well as anal agenesis without fistula in both sexes. The low-type malformations were
classified as anvovestibular fistula in the female and, both saxes, as anocutaneous fistula
and anal stenosis.
2. Pena’s classification :
In 1995, pena proposed a classification based on the type of the fistula present. He
distinguished between perineal, vestibular, bulbar, postatic, and bladder neck fistulas;
imperforate anus without fistula; vaginal fistulas; cloacal fistulas; and rectal atresia or
stenosis.
3. Krickenbeck classification :
A modification of the classification of Pena according to the type of the fistula
and including rare/regional variants was proposed. The major clinical groups were
classified as perineal (cutaneous) fistulas, rectourethral fistulas (prostatic and bulbar),
rectovesical fistulas, vestibular fistulas, cloacal malformations, patients with no fistula,
and anal stenosis. Rare/regional variants were subclassified as puch colon, rectal atresia/
stenosis, rectovaginal fistulas, H-type fistulas and other.
The new international classification enables the different operative procedures to
be more comparable to each other than with the Wingspread classification.
Initial assessment:
During the first 24-48 hours of life in a baby born with an anorectal malformation, answer the following 2 questions:
· Are any associated anomalies present that threaten the baby’s life and need to be addressed immediately?
Should the infant undergo a primary procedure with no protective colostomy or a protective colostomy with a definitive repair
at a later date?
For babies born with persistent cloaca, the surgeon must also determine whether a dilated vagina is present and if it should
be drained and whether urinary diversion is required. These maneuvers are intended to prevent sepsis or metabolic
acidosis.
The decision to perform an anoplasty in the newborn period or to delay the repair and perform a colostomy is based
on the infant’s physical examination findings, the appearance of the perineum, and any changes that occur over the first 24
hours of life.
Operating earlier and in a single stage is potentially beneficial to the patient, but this should be decided carefully, weighing
the specific circumstances of the baby and the experience of the surgeon. A more conservative approach is warranted in
babies with low birth weight and associated cardiac or respiratory conditions. Indications for colostomy versus anoplasty
based on sex are as follows:
· Males
· Colostomy - Recto-bulbar urethral fistula, recto-prostatic urethral fistula, rectovesical fistula, imperforate anus without
fistula, rectal atresia
· Females
· Colostomy - Rectovestibular fistula, imperforate anus without fistula, persistent cloaca, rectal atresia, rectovaginal
fistula
Imaging Studies:
In neonatal period :
Invertogram
Indication : when the perinel inspection and urin analysis are inconclusive wheather the diffect is high or low.
Procedure:it is done after18 hours of birth,so that the meconium reaches the rectum and with sufficient pressure that it can
negotiate the pressure exerted bylevator muscle complex. A radio opaque object is placed in the anal pit of the baby. Baby
is inverted for about three minutes and lateral view x-ray is taken .
Interpretation:if the lenght of the aircolumn is more than 1 cm, than a high anorectal maleformation is diagnosed. If it is less
than 1cm a low type of arm is diagnosed.
Crosstable lateral film: This procedure is same as invertogram expect the position of the baby, which is prone here. Thus
the disadvantages of invertogram because of the position is not here.
Other investigations :
Ultrasonography abdomen: This is done to rule out any associated urological anomalies.
X-ray spine : Anteroposterior and lateral films are taken to rule out any congenital spinal abnormalities like spina bifida.
Colostomy cases :
High pressure colostography : This is done to delineate the acurate anatomy of the anorectal malformation.
Associated malformations
· Genitourinary defects
· Approximately 50% of all patients with anorectal malformations have an associated urogenital anomaly, which
frequently varies with the type of anorectal defect.
· A list of anorectal defects and the percentages of patients with associated urogenital anomalies is as follows (Rich,
1988):
· Cloaca - 81%
· Recto-prostatic - 40%
· Rectovestibular - 9%
· Recto-bulbar - 4%
· Rectoperineal - 0%
· Overall - 48%
· All patients must be examined at birth to evaluate for one of these defects; the most valuable screening test is
abdominal and pelvic ultrasonography.
· Urologic evaluation before colostomy provides the surgeon the necessary information needed to address the
urologic problem at the time of the colostomy. The surgeon must be prepared to perform a urologic diversion if necessary.
· 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 have a higher likelihood of having
tethered cord.
· Untethering the cord is indicated in the neurosurgical literature to avoid motor and sensory problems. No evidence
exists to suggest that this operation has an impact on the functional prognosis for patients with anorectal malformation.
· Spinal ultrasonography in the first 3 months of life and MRI thereafter are useful radiologic modalities to establish the
diagnosis.
· The sacrum is the most frequently affected bony structure. Traditionally, to evaluate the degree of sacral deficiency,
the number of sacral vertebral bodies was counted. A more objective assessment of the sacrum can be obtained by
calculating a sacral ratio. The sacrum
is measured and its length is compared with bony parameters of the pelvis. The lateral view is more accurate than the
anteroposterior view because its calculation is not affected by the tilt of the pelvis.
· Assessment of the hypodevelopment of the sacrum correlates with the patient’s functional prognosis. Normal sacra
have a calculated sacral ratio of 0.77. Bowel control has never been observed in patients with calculated sacral ratios less
than 0.3.
Rough assessment of post operative continence can be done on the basis of presence of number of sacral vertebras like
>3 sacral vertebras-- continence expected.
expected.
· A hemisacrum is always associated with a presacral mass, which commonly includes dermoids, teratomas, or
anterior meningoceles.
· Hemivertebrae may also affect the lumbar and thoracic spine, leading to scoliosis.
· Patients may have spinal anomalies other than tethered cord, such as syringomyelia and myelomeningocele.
Management :
: As discussed previously, the surgeon must decide in the newborn period whether the child will need colostomy or primary
procedure.
DEFINITIVE REPAIR
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; however, occasionally, this period is longer.
If the colostomy is untouched during the operation and no laparotomy was necessary, oral
feedings may be started immediately postoperatively. If a laparotomy was necessary, the
patient may require a period of fasting and nasogastric decompression.
At 2 weeks’ postoperation, 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 age of the patient.
Once the desired size is reached, the colostomy can be closed.
Dilatations are continued afterward according to a prescribed protocol.
Dilatations are a vital part of the postoperative management to avoid a stricture at the
anoplasty.
After colostomy closure, severe diaper rash is common because the perineal skin has
never before been exposed to stool.
Complication :
Complications of surgery include dehiscence and infection. These complications
can be avoided by colostomy before the main repair.
A
SHORT NOTE ON
HYDATID DISEASE
CONTENTS :
- INTRODUCTION
- CUSATIVE ORGANISM
- LIFE CYCLE
- MODE OF INFECTION
- EVOLUTION OF HYDATID CYST
- CHARACTERISTICS OF HYDATID FLUID
- CLINICAL FEATURES
- INVESTIGATIONS
- DIFFERENTIL DIAGNOSIS
- TREATMENT
- PREVENTION
Echinococcosis (Hydatid Disease ) is an infection of human caused by the larval stage ofEchinococcus
granulosus , E. multilocularis or E. vogeli . The disease is world wide in distribution, prevalent in areas
where livestock ( cattle , sheep etc.) is raised in association with dogs .
CAUSATIVE ORGANISM : E. granulosus , the most common causative organism of hydatid disease ,
is a small tapeworm , measuring 3 to 6 mm in length comprises of a head ( scolex) , neck and strobila ,
consisting of three segments .
The adult worms lives in the small intestine of definitive host and discharge large no.
of eggs in their feces .
These eggs are swallowed by the intermediate hosts . In their duodenum eggs are
hatched to form embryos .
Embryos are carried to the liver to get lodge in the sinusoidal capillaries ( the liver acts
as the first filter )
Some of the embryos pass through the hepatic capillaries , enter the pulmonary
circulation to get lodge in the lungs .( lungs act as the second filter )
A few embryos even pass the pulmonary circulation to enter the systemic circulation
and lodge in various organs of the body .
The embryos that get settled in liver , lung or other organ of the body , get transformed
into larval stage forming a hollow bladder like cavity containing fluid commonly known as hadatid
cyst .
From the inner side of the cyst , brood capsules with a number of scolices are
developed .
These fertile hydatids when ingested by a dog are capable of growing into adult worms
in the intestine .
MODE OF INFECTION :
The infection is generally acquired in childhood but the disease usually manifest in adulthood only .
CLINICAL FEATURES :
The disease usually involves both sexes equally and it generally manifest at an average age of 45 years
.About three fourth of hydatid cyst are located in the right liver and are unilocular .
For many years the disease remain asymptomatic . Symptoms arises only after ,
- cyst becomes large enough to have space occupying effect in the organs
involved
pressure effect on the surrounding structures .
- the cyst gets ruptured
- the cyst gets secondarily infected
Liver hydatid cyst often present with
-abdominal pain or a palpable mass in the right upper quadrant
-compression of the billiary duct can result in jaundice
-Rupture or episodic leakage from a hydatid cyst may produce fever, pruritus
urtiaria, eosinophilia or anaphylaxis
INVESTIGATIONS:
1. Casoni test
2. Blood examination may reveal generalized eosinophilia
3. Serological test : Detection of antibody to specific ecchinococcal antigens by immunoblotting
has the highest degree of specificity
4. Exploratory cyst puncture: examination of hydatid fluid under the microscope for scolices
or
hooklets gives an accurate diagnosis but it may have serious
consequences and so not advised .
5. X ray : Plain picture abdomen may miss a hydatid cyst of liver unless the cyst wall is calcified.
Chest x ray usually picks alveolar hydatid cyst as a irregular mass of uniform density .
6. Ultrasonography : most commonly applied radiological examination . It shows
- Well circumscribed cystic area with budding cystic membrane
- may show hyperechogenic hydatid sand
- a rossete appearance is seen when daughter cyst is present
7. CT scan : shows cystic area with no enhancement in contrast study . The most pathognomonic
finding in CT scan are
- presence of membrane like structure in the dependent part of the cavity
-daughter cyst within the mother cyst , if demonstrable.
8. MRI : same finding as CT scan . No added advantage over CT
9. ERCP or PTC : may be necessary in patients with suspected billiary involvement
DIFFERENTIAL DIAGNOSIS:
TREATMENT:
a) Medical : Albendazole therapy is the mainstay of treatment in majority of the patients .Albendazole
is given at the dosage of 15mg/kg/day in two divided doses for 12 weeks to 6 months .
Response to treatment is assessed by serial imaging studies with attention to cyst size and
Consistency . It results in cure in 30% of the cases and improvement in another 50% of
the
cases .
Steps of operation :
-an incision is made through the liver parenchyma overlying the cyst until the ectocyst is
exposed
-the ectocyst is incised to expose the rubbery endocyst
-gently endocyst is removed with the parasites inside
-After the cyst has been excised , mobilized omentum is frequently packed into the liver cavity
but alternatively the area can be left open to the peritoneal cavity .
It is very important not to rupture the endocyst with subsequent spillage of viable parasites . To
reduce the consequences of such a mishap the liver is surrounded by packs which may be
soaked in scolicidal solution such as hypertonic saline .
PREVENTION
-dogs should be kept separated from livestock to break the life cycle of E. granulosus
-avoid intimate fondling of the dogs
-Praziquantal to infected dogs
-vaccination of sheep
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