Question

Describe briefly : H Type anorectal malformation in male.

Hint:

Early diagnosis and surgical correction are critical for optimal outcomes in patients with H Type anorectal malformation.

Answer 1

H Type anorectal malformation is a form of congenital defect where the rectum and the anus are improperly connected, usually through a fistula, creating an abnormal passage between the two organs. This type of malformation is more commonly seen in male infants.
Step 1: Embryology and Pathogenesis:
1. Abnormal Development: During fetal development, the rectum and anus fail to form separately, and an abnormal connection (fistula) develops between the rectum and the urinary tract or skin.
2. H Type Fistula: This malformation is characterized by a fistula that connects the rectum to the perineum or the urethra in males, with no external anal opening.
Step 2: Clinical Presentation:
1. Absence of Anal Opening: The infant presents with the absence of a normal anal opening and may have a perineal fistula or a connection to the urinary tract.
2. Urinary Symptoms: Some infants may experience difficulty urinating or show signs of urinary tract infections due to the abnormal connection.
Step 3: Diagnosis:
1. Physical Examination: The absence of the anal opening is immediately apparent, and a perineal or vaginal fistula may be palpated.
2. Imaging: X-rays and MRI may be used to assess the extent of the fistula and any other associated malformations.
Step 4: Management:
1. Surgical Repair: Surgical intervention is required to repair the abnormal fistula and create a functioning anus. The surgery is typically performed in stages and may involve the use of a colostomy in the early stages.
2. Postoperative Care: After surgery, the child must be closely monitored for bowel function and any potential complications, such as infections or incontinence.