Anal atresia is a congenital anomaly that is detected in the neonatal period and means that the anus is closed. It has very different types in the form of a fan, from opening only the breech to the skin, to severe urinary tract and genital anomaly. Depending on the type, treatment type(s) may vary. This typing is done according to how far the anus, which is the last part of the alimentary canal, is to where the breech should be, and where the large intestine is connected with inside. Likewise, according to this typing, patients may have other congenital anomalies. While the simple type patients return to their normal lives with an operation, a series of surgeries await the more complex patients.
In low-type disease, the breech is formed with a single operation under general anesthesia and the patient usually returns to his normal life in a very short time.
Things are more complicated in complicated disease, in almost all cases there is a connection between the intestine and the urinary tract or genitals of girls. In these patients, first of all:
Colostomy: Surgery is performed to open the intestine from the abdomen to allow the stool to be thrown out of the body. In the meantime, the connection between the mentioned intestinal-urinary tract/sexual area is partially protected from continuous fecal contamination, which increases the risk of infection. These patients pass their stools in a bag attached to their abdomen for a while.
Corrective surgery: There is no connection between “corrective surgery” and both the intestinal-urinary tract/sexual area at the time considered appropriate by the doctors according to your child’s development, additional anomalies and the type of disease (this period is usually between 2-3 months). In addition, the intestines that have remained above are pulled down and the breech is formed where it should be. The patient is taken to the breech expansion program until the next surgery.
Colostomy closure: Approximately two months after the corrective surgery, the intestinal circulation, which was opened to the abdomen with the “colostomy closure” operation, is provided again through the abdomen, the patient will now be able to pass the stool through the anus formed by the previous surgery.
The success of the outcome in these patients depends on the patient’s anatomical structure, comorbidities, if any, and family-patient compatibility (colostomy washing, compliance with the expansion program). The duration of the operation is between 2-5 hours under normal conditions, and this time may vary depending on the course of the operation and patient factors. The chance of success is approximately 30-90%, although it varies from patient to patient, the distance of the blind-ended intestine from the anus, and the presence and severity of additional anomalies seen in the patient. After the surgery, the patient is followed up in the clinic for approximately 2-7 days. During this period, the patient may not be fed orally and total parenteral nutrition (TPN) is applied.
The most common genital anomaly in men is undescended testis and hypospadias. Undescended testis surgery should be performed within the first year of life. The most common reason for surgical intervention during ARM repair is high-grade VUR, subureteric injection does not provide improvement in these patients and the most effective treatment method is ureteroneocystostomy.
The most common surgical procedure among spinal anomalies is the tethered cord (tethered cord syndrome). Especially the association of sacrum and spinal anomalies increases the possibility of urinary incontinence. The indication for surgery for the tethered cord in asymptomatic patients and the effect of tethered cord repair on fecal incontinence are controversial.
Problems that may be encountered during the recovery period and in the long term:
Colostomy tip slipping back into the abdomen.
The escaping of the lowered intestine upwards and the closure of the anus.
Stenosis, obstruction in the urinary tract.
Newly formed rectal stenosis.
Patient non-compliance in the colostomy washing and breech enlargement program
Variable rates depending on the type of anomaly
Absence of voluntary bowel movements,
More or less poop incontinence,
Urinary incontinence,
Constipation: No matter how successful the surgery is, it is a serious problem in these patients.
Expectations and Problems in the Late Period:
The most common reason for admission in patients with anorectal malformation over the age of 5 is stool and urinary incontinence. Stool habit should be established with a bowel training program between 3-5 years of age. Type of anorectal malformation, surgical repair and post-operative course, sacrum and spinal anomalies are the variables that affect stool and urinary incontinence.
ARM type should be determined preoperatively by distal colostogram (medicated film). Unsuccessful repair without colostomy, inability to fully replace the anus with surgery, development of stenosis, repair of rectourinary fistula or Currarino Triad without diagnosis, unsuccessful lowering of the bowel are the most common causes of re-ARM repair.
Hirschsprung’s disease is unlikely to coexist in cases of treatment-resistant constipation and rectal biopsy should not be a priority.
In the bowel training program, it is tried to ensure the accumulation of stool for 24 hours without incontinence and its complete emptying with voluntary bowel movement or enema. Fecal incontinence can occur at different rates. Enema content and laxative dose need to be adjusted. Some of the patients who are given enema and clean intermittent catheterization treatment for the treatment of fecal and urinary incontinence, and who are successful, stop the treatment and become incontinent. In order to increase the success of the bowel education program, patient follow-up and communication should be done more frequently.
