Newborn male with features of covered cloacal exstrophy with prolapsing bladder plate at penoscrotal hypospadic opening
LB Cornwell, M Alagiri, S Marietti, G Chiang, KA Swords
Rady Children's Hospital, San Diego, CA
A full-term infant was born in July 2019 after a pregnancy notable for IUGR, oligohydramnios. Multiple prenatal ultrasounds were performed secondary to difficulty in ascertaining fetal sex with the final determination as female. At birth the infant was noted to have ambiguous genitalia with an absent anus, a bifid scrotum with bilateral palpable gonads, a small hypospadiac phallus, and a large mucosal opening at the penoscrotal junction that was draining stool and urine. Upon valsalva, the bladder prolapsed and ureteral orifices were visualized. The umbilicus was low-lying and a rectus belly diastasis was palpable beneath the skin. Renal function returned within normal limits. Pelvic ultrasound revealed no evidence of female organs and a karyotype returned 46,XY. Sacral x-ray revealed a wide pubic symphysis diastasis and sacral anomaly. After surgical consultations, the decision was made for intestinal diversion, cystoscopy, and exam under anesthesia. Due to the large size of the mucosal opening, cystoscopic exam was difficult to achieve, however, communicating surfaces of both intestinal and urothelial mucosa were visualized within the opening. The space was unable to be adequately distended to perform a fistulogram. Diagnostic laparoscopy revealed an absent colon and rectum with a short cecum entering the posterior bladder. An ileostomy and mucous fistula were created. Pelvic MRI demonstrated the bladder prolapse, mild right hydronephrosis, and lack of visualization of sacrococcygeal elements. His bladder is currently being managed with saran wrap covering, and reconstruction options remain in discussion.
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