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

Masayuki Kubota, Yutaka Osuga, Kiyoko Kato, Kenji Ishikura, Kazunari Kaneko, Kohhei Akazawa, Takeo Yonekura, Yuko Tazuke, Satoshi Ieiri, Akihiko Fujino, Shigeru Ueno, Yutaro Hayashi, Kaoru Yoshino, Toshihiro Yanai, Jun Iwai, Takanori Yamaguchi, Shintaro Amae, Yuichiro Yamazaki, Yoshifumi Sugita, Miyuki KohnoYutaka Kanamori, Yuko Bitoh, Masato Shinkai, Yasuharu Ohno, Yoshiaki Kinoshita

Research output: Contribution to journalReview article

Abstract

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.

Original languageEnglish
Pages (from-to)985-1002
Number of pages18
JournalSurgery today
Volume49
Issue number12
DOIs
Publication statusPublished - Dec 1 2019

All Science Journal Classification (ASJC) codes

  • Surgery

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    Kubota, M., Osuga, Y., Kato, K., Ishikura, K., Kaneko, K., Akazawa, K., Yonekura, T., Tazuke, Y., Ieiri, S., Fujino, A., Ueno, S., Hayashi, Y., Yoshino, K., Yanai, T., Iwai, J., Yamaguchi, T., Amae, S., Yamazaki, Y., Sugita, Y., ... Kinoshita, Y. (2019). Treatment guidelines for persistent cloaca, cloacal exstrophy, and Mayer–Rokitansky–Küster–Häuser syndrome for the appropriate transitional care of patients. Surgery today, 49(12), 985-1002. https://doi.org/10.1007/s00595-019-01810-z