Journal of pediatric urology · 2025
Outcomes and management of mullerian anomalies in female patients with complex congenital birth defects of cloacal exstrophy and anorectal malformation.
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Broadwell Nora Haney, Pantoja Geneva, Boyne Aidan Michael, Alexander Juliet, Paz Alisha, Nguyen Catherine, Yong Raymond, Balthazar Andrea K, Passoni Niccolo M
Аннотация
INTRODUCTION: Females born 46,XX with the severe malformations of cloacal exstrophy (CE) and cloacal anomalies (CA) have high rates of mullerian abnormalities leading to outflow tract obstruction (OTO) of menses, invasive surgeries, and/or chronic pain. This study aims to discuss long-term surgical outcomes of mullerian structures in a single major institution with a high volume of CE and CA patients.
METHODS: A prospectively maintained database was reviewed for CE and CA patients. The patient database was received for CE and CA female patients. Data on mullerian anatomy at birth, hormone suppression, and surgical procedures were evaluated.
RESULTS: 91 females (46XX, 17 CE and 74 CA) were included with median age of 14.5 [0.17, 30.5] years. Of those, 68.1 % had duplicated uteri. Vaginal anatomy was duplicated in 52.7 % with 18.7 % having complete atresia. 23.1 % of patients underwent a hysterectomy (41.2 % CE, 18.9 % CA). Hysterectomy was performed prior to menarche in 52.4 % and post menarche in 47.6 %. Hormone suppression was used in 24.2 % (22/91) of the entire cohort, with 45.2 % (10/22) of those patients proceeding to hysterectomy. In those with hormone suppression alone (54.5 %, 12/22), either vaginoplasty to relieve obstruction or hysterectomy of obstructed horn is planned for the future once the patient is ready to participate in surgical discussion. All hysterectomies were performed on patients with duplicated uteri. Reasons for pre-menarchal hysterectomy included nonfunctional or noncommunicating uteri, little chance of safe pregnancy, and/or family desire for minimal surgical intervention. Post-menarchal hysterectomy reasons included pelvic pain secondary to hematocolpos, desire to discontinue hormone suppression and/or need for vaginostomy, and/or vesicouterine fistula.
CONCLUSION: This study demonstrates high rates of surgical removal of mullerian structures in females with CE and CA. Further study would be beneficial for early identification of CE and CA patients at risk of undergoing hysterectomy while maintaining fertility potential in those with low risk of mullerian complications.
METHODS: A prospectively maintained database was reviewed for CE and CA patients. The patient database was received for CE and CA female patients. Data on mullerian anatomy at birth, hormone suppression, and surgical procedures were evaluated.
RESULTS: 91 females (46XX, 17 CE and 74 CA) were included with median age of 14.5 [0.17, 30.5] years. Of those, 68.1 % had duplicated uteri. Vaginal anatomy was duplicated in 52.7 % with 18.7 % having complete atresia. 23.1 % of patients underwent a hysterectomy (41.2 % CE, 18.9 % CA). Hysterectomy was performed prior to menarche in 52.4 % and post menarche in 47.6 %. Hormone suppression was used in 24.2 % (22/91) of the entire cohort, with 45.2 % (10/22) of those patients proceeding to hysterectomy. In those with hormone suppression alone (54.5 %, 12/22), either vaginoplasty to relieve obstruction or hysterectomy of obstructed horn is planned for the future once the patient is ready to participate in surgical discussion. All hysterectomies were performed on patients with duplicated uteri. Reasons for pre-menarchal hysterectomy included nonfunctional or noncommunicating uteri, little chance of safe pregnancy, and/or family desire for minimal surgical intervention. Post-menarchal hysterectomy reasons included pelvic pain secondary to hematocolpos, desire to discontinue hormone suppression and/or need for vaginostomy, and/or vesicouterine fistula.
CONCLUSION: This study demonstrates high rates of surgical removal of mullerian structures in females with CE and CA. Further study would be beneficial for early identification of CE and CA patients at risk of undergoing hysterectomy while maintaining fertility potential in those with low risk of mullerian complications.