Cloacal malformations (CM), also called persistent cloaca (PC) or partial urorectal septum malformation (pURSM), are characterized by the confluence of the urethra, vagina, and rectum into one common channel (CC), which is identified on the perineum as a single orifice (Fig. 11.1). Occurring only in women, CM is the most complex type of anorectal malformations (ARM) with large anatomical variation among patients [1]. The incidence was estimated to be about 1 in 50,000 newborn girls, causing limited experience in diagnosis and treatment [2, 3]. The pathogenesis remains largely unknown, which may be related to the abnormal development of the urorectal septum during the embryonic stage. CM affects the urinary system, digestive system, and female reproductive system at the same time. The operation aims to restore the normal anatomy and function of the urethra, vagina, and rectum, which is complicated and requires long-term follow-up with multidisciplinary cooperation. This section focuses on the reproductive tract obstruction caused by CM combined with Müllerian duct system malformation.

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Cloacal Malformations

  • Jiali Duan,
  • Lan Zhu

摘要

Cloacal malformations (CM), also called persistent cloaca (PC) or partial urorectal septum malformation (pURSM), are characterized by the confluence of the urethra, vagina, and rectum into one common channel (CC), which is identified on the perineum as a single orifice (Fig. 11.1). Occurring only in women, CM is the most complex type of anorectal malformations (ARM) with large anatomical variation among patients [1]. The incidence was estimated to be about 1 in 50,000 newborn girls, causing limited experience in diagnosis and treatment [2, 3]. The pathogenesis remains largely unknown, which may be related to the abnormal development of the urorectal septum during the embryonic stage. CM affects the urinary system, digestive system, and female reproductive system at the same time. The operation aims to restore the normal anatomy and function of the urethra, vagina, and rectum, which is complicated and requires long-term follow-up with multidisciplinary cooperation. This section focuses on the reproductive tract obstruction caused by CM combined with Müllerian duct system malformation.