title: Long-term survival with cloacal dysgenesis sequence.
abstract: Cloacal dysgenesis sequence (CDS) is a rare and lethal malformation. We report such a case of long-term survival, currently to 12 years of age. In the fetal period, she received a timely placement of vesico-amniotic shunt for a megabladder due to a severe urethral obstruction. Postnatally, cystostomy and colostomy were created because of no perineal opening of urethra, vagina, and anus. Anorectoplasty, construction of efferent conduit, and colostomy closure were performed at 4 years of age. Ileovaginoplasty and ileovesicostomy which was a Mitrofanoff-type of conduit, and labioplasty were performed at the age of 11 years. To the best of our knowledge, only five survivors with CDS over 1 year of age have been reported.
authors.affiliation: Department of Pediatric Surgery, Ibaraki Children's Hospital, 3-3-1 Futabadai, Mito-city, Ibaraki, 311-4145, Japan. to.yanai@ibaraki-kodomo.com
authors.forename: Toshihiro
authors.initials: T
authors.lastname: Yanai
authors.forename: Yasuhisa
authors.initials: Y
authors.lastname: Urita
authors.forename: Teruyoshi
authors.initials: T
authors.lastname: Amagai
authors.forename: Hajime
authors.initials: H
authors.lastname: Kawakami
authors.forename: Hiroko
authors.initials: H
authors.lastname: Watayo
authors.forename: Takayuki
authors.initials: T
authors.lastname: Masuko
authors.forename: Satoshi
authors.initials: S
authors.lastname: Matsuda
authors.forename: Misako
authors.initials: M
authors.lastname: Hirai
authors.forename: Toshihiro
authors.initials: T
authors.lastname: Muraji
authors.forename: Hiromi
authors.initials: H
authors.lastname: Hamada
mesh_terms: D000015:Abnormalities, Multiple; D000328:Adult; D001003:Anal Canal / Q000002:abnormalities* / Q000601:surgery; D002988:Cloaca / Q000002:abnormalities* / Q000196:embryology / Q000601:surgery; D003125:Colostomy / Q000379:methods; D003937:Diagnosis, Differential; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007231:Infant, Newborn; D011247:Pregnancy; D011256:Pregnancy Outcome; D019651:Plastic Surgery Procedures / Q000379:methods*; D013997:Time Factors; D016216:Ultrasonography, Prenatal; D014521:Urethra / Q000002:abnormalities* / Q000601:surgery; D014621:Vagina / Q000002:abnormalities* / Q000601:surgery