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Virtual Pediatric Hospital: Correlapaedia - a Correlative Encyclopedia of Pediatric Imaging, Surgery, and Pathology: Case 28

Correlapaedia - a Correlative Encyclopedia of Pediatric Imaging, Surgery, and Pathology

Case 28

Michael P. D'Alessandro, M.D.,
Steven J. Fishman, M.D.,
Deborah E. Schofield, M.D.

Peer Review Status: Internally Peer Reviewed
Chief Complaint:
Eleven month old female with a single perineal opening.

Clinical History:
This patient was an 11 month old female with a very extensive cloacal malformation including absent anus and rectum, high rectal-vesical fistula, atresia of two vaginas, bilateral ectopic ureters, and right hydronephrosis. The patient's surgical history is remarkable for prior right colostomy with prolapse.

Clinical Physical Exam:
Single perineal opening.

icon gif icon gif Images 1 and 2

Clinical Labs:

Clinical Differential Diagnosis:
Cloacal malformation

Imaging Findings:
An abdominal film showed multiple vertebral anomalies in the sacrum. A cloacal study demonstrated a rectovesical fistula, a long urethra-urogenital sinus, and a possible rudimentary vagina. An MRI (not shown) showed the conus medullaris to be at L1-L2, and the sacrum to be dysplastic. A pelvic ultrasound (not shown) showed a uterus and ovaries.

icon gifImage 3

icon gif icon gif icon gif Images 4, 5. and 6

Imaging Differential Diagnosis:
Cloacal malformation

Operative Findings:
At 11 months of age the patient underwent an extensive cloacal reconstruction lasting 15 hours including reduction of the prolapsed right colon, take down of the high retrovesical fistula, pull-through of the colon to the perineum to create an anus and rectum, a cut back of the urogenital sinus, cross trigone reimplantation of both ureters, creation of a small bowel vagina from ileum, right hysterectomy, left partial hysterectomy and anastomosis of upper left uterus to small bowel vagina, revision of the right colostomy, and revision of the left colostomy.

Pathological Findings:

Final Diagnosis:
Cloacal Malformation

Follow-up and Prognosis:
The patient had an uncomplicated post-operative course.

Similar Cases:
Case 35, Case 51


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