Congenital cloaca: Long-term follow-up results with emphasis on outcomes beyond childhood
Congenital cloaca: Long-term follow-up results with emphasis on outcomes beyond childhood
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DOI：
10.1053/j.sempedsurg.2015.11.011
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发表时间：
2016-04-01
影响因子：
1.7
通讯作者：
Rintala, Risto J.
中科院分区：
文献类型：
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作者：
Rintala, Risto J.
持续性泄殖腔仍然是儿科外科医生和泌尿科医生面临的挑战。泄殖腔畸形重建手术旨在修复肛门直肠、泌尿道和生殖器官，实现大小便节制以及具有性活动和怀孕能力的生殖道功能。不幸的是，即使在最有经验的人手中，这些目标也并不总是能够实现。肠道、泌尿和生殖功能的功能发育的终点是患者生长和性成熟的完成。在这些时间点之后不太可能出现任何显着的功能改进。大约一半的泄殖腔患者在生长期后能够实现大便和尿失禁。剩下的一半通过辅助措施保持清洁或干燥，例如通过灌肠或 ACE 通道进行肠道管理，以及节制尿流改道或间歇性导尿。与生殖器官相关的问题，如月经不调、闭经和阴道口狭窄等，很常见，通常需要二次手术。令人鼓舞的是，尽管经常进行复杂的阴道初次和二次重建，但大多数青少年和成年患者仍能够进行性生活。此外，泄殖腔畸形并不妨碍怀孕，尽管这种情况仍然相当罕见。泄殖腔妊娠患者需要特殊护理和随访，以保证顺利妊娠并保留肛门直肠和泌尿功能。建议对泄殖腔患者进行剖腹产。泄殖腔患者自我报告的生活质量似乎与患有不太复杂的肛门直肠畸形的女性患者相当。 (C) 2016 Elsevier Inc. 保留所有权利。
Persistent cloaca remains a challenge for pediatric surgeons and urologists. Reconstructive surgery of cloacal malformations aims to repair the anorectum, urinary tract, and genital organs, and achieve fecal and urinary continence as well as functional genital tract capable for sexual activity and pregnancy. Unfortunately, even in most experienced hands these goals are not always accomplished. The endpoint of the functional development of bowel, urinary, and genital functions is the completion of patient's growth and sexual maturity. It is unlikely that there will be any significant functional improvement beyond these time points. About half of the patients with cloaca attain fecal and urinary continence after their growth period. The remaining half stay clean or dry by adjunctive measures such as bowel management by enemas or ACE channel, and continent urinary diversion or intermittent catheterization. Problems related to genital organs such as obstructed menstruations, amenorrhea, and introitus stenosis are common and often require secondary surgery. Encouragingly, most adolescent and adult patients are capable of sexual life despite often complex vaginal primary and secondary reconstructions. Also, cloacal malformation does not preclude pregnancies, although they still are quite rare. Pregnant patients with cloaca require special care and follow-up to guarantee uncomplicated pregnancy and preservation of anorectal and urinary functions. Cesarean section is recommended for cloaca patients. The self-reported quality of life of cloaca patients appears to be comparable to that of female patients with less complex anorectal malformations. (C) 2016 Elsevier Inc. All rights reserved.