Treatment guidelines for persistent cloaca, cloacal exstrophy, and Mayer–Rokitansky–Küster–Häuser syndrome for the appropriate transitional care of patients

• Published in: Surgery today (Tokyo, Japan) Japan), 2019-12, Vol.49 (12), p.985-1002
• Main Authors: Kubota, Masayuki, Osuga, Yutaka, Kato, Kiyoko, Ishikura, Kenji, Kaneko, Kazunari, Akazawa, Kohhei, Yonekura, Takeo, Tazuke, Yuko, Ieiri, Satoshi, Fujino, Akihiko, Ueno, Shigeru, Hayashi, Yutaro, Yoshino, Kaoru, Yanai, Toshihiro, Iwai, Jun, Yamaguchi, Takanori, Amae, Shintaro, Yamazaki, Yuichiro, Sugita, Yoshifumi, Kohno, Miyuki, Kanamori, Yutaka, Bitoh, Yuko, Shinkai, Masato, Ohno, Yasuharu, Kinoshita, Yoshiaki
• Format: Article
• Language: English
• Subjects: 46, XX Disorders of Sex Development - surgery; Anorectal Malformations - surgery; Anus, Imperforate - surgery; Congenital Abnormalities - surgery; Female; Hernia, Umbilical - surgery; Humans; Infant, Newborn; Male; Medicine; Medicine & Public Health; Mullerian Ducts - abnormalities; Mullerian Ducts - surgery; Practice Guidelines as Topic; Pregnancy; Quality of Life; Review Article; Scoliosis - surgery; Surgery; Surgical Oncology; Transitional Care; Urogenital Abnormalities - surgery
• ISSN: 0941-1291; 1436-2813
• DOI: 10.1007/s00595-019-01810-z

We developed treatment guidelines (TGs) for appropriate transitional care of the genitourinary system in patients with persistent cloaca (PC), cloacal exstrophy (CE), or Mayer–Rokitansky–Küster–Häuser syndrome (MRKH). These TGs are in accordance with the Medical Information Network Distribution Service (Minds), published in 2014 in Japan. Clinical questions (CQs) concerning treatment outcomes of the genitourinary system, pregnancy and delivery, and quality of life in adulthood were prepared as six themes for PC and CE and five themes for MRKH. We were able to publish statements on chronic renal dysfunction, hydrometrocolpos, and pregnancy, based on four CQs about PC, four about CE, and two about MRKH, respectively. However, due to the paucity of proper manuscripts, we were unable to make conclusions about the correct timing and method of vaginoplasty for patients with PC, CE, and MRKH or the usefulness of early bladder closure for patients with CE. These TGs may help clarify the current treatments for PC, CE, and MRKH in childhood, which have been carried out on an institutional basis. To improve clinical outcomes, systematic clinical trials revealing comprehensive clinical data of the urinary and reproductive systems, especially the length of the common channel in PC, are essential.