Complex Cloacal Malformation

• 2-day-old female is admitted with bilious emesis, abdominal distention and failure to pass meconium.
• Birth weight was 3.5 kg.
• On physical exam she was noted to have a single perineal orifice.
• She underwent diverting colostomy, cystostomy and hydrocolpos drainage.
• Cystostomy was maintained days for 5 d. It was removed once the infant was draining urine.
• Operative findings included hemiuteri, left ovarian macrocystic dysplasia and right ovarian agenesis.
• Baby became cyanotic postoperatively.
• Echocardiogram identified to have tetralogy of Fallot.
• Patient was referred to the pediatric surgery cardiovascular department but heart surgery was postponed until correction of anorectal malformation.
• MRI showed cloaca with short common channel and sacral agenesis.

Challenge

• What is the most effective surgical strategy for this patient?

Takeaways

• Within the first 24 h of life, babies born with anorectal malformation should have evaluation of associated anomalies. This includes echocardiogram to evaluation for congenital heart disease. Thirty percent of patients with anorectal malformation will have congenital heart disease.
• Ultrasound should be obtained. If hydrocolpos is present it should be drained.
• This patient’s imaging is concerning for tethered cord. She should be referred to neurosurgery.
• Since the patient has a short sacrum, the length of the common channel cannot be confirmed.
• To measure the length of the common channel, a distal stoma study could be performed in radiology. Contrast should be injected into the mucous fistula with the patient in lateral position. A radiopaque marker can be placed at the anal impression. Then the distance of the rectum to the marker give a good estimate of the length of the common channel.
• The most ideal method of measuring the common channel is with cystscopy.
• Vaginostomy should be placed to drain the hydrocolpos. Drain should remain in place in order to protect the kidneys.