Gender assignment for newborns with 46XY cloacal exstrophy: a 6-year followup survey of pediatric urologists.

J Urol. 2011 Oct;186(4 Suppl):1642-8. doi: 10.1016/j.juro.2011.03.101.

Gender assignment for newborns with 46XY cloacal exstrophy: a 6-year followup survey of pediatric urologists.

Diamond DA, Burns JP, Huang L, Rosoklija I, Retik AB.



Gender assignment for newborns with ambiguous genitalia remains a challenge. An initial survey of colleagues on this subject was performed in 2004. Our objective was to understand the basis for the attitudes and practices of pediatric urologists in regard to gender assignment for 46XY cloacal exstrophy in a 6-year followup survey.


A survey on a case of 46XY cloacal exstrophy was completed by 191 of the 263 fellows (73%) in the Urology Section, American Academy of Pediatrics. Questions referred to gender assignment, surgery timing, clinical outcomes and respondent demographics.


Of the fellows 79% favored male gender assignment. The most important factor in male assignment remained androgen brain imprinting (97%) while in female assignment it was surgical success in creating functional genitalia (96%). Respondent characteristics associated with assigning female gender were longer practice duration (greater than 15 years) (p <0.03), having trained in programs where female gender was always or usually assigned (p <0.02) and not being a fellowship program director (0 of 27 respondents, p <0.03). There was an evolution among respondents from female gender assignment earlier in the career to male assignment currently (p <0.0001).


Most pediatric urologists favor male gender assignment for 46XY cloacal exstrophy, which is a significant increase in 6 years. This change represents an evolution from female to male gender assignment and virtual unanimity among fellowship directors to gender assign male. Longer practicing clinicians perceived better outcomes for female gender assignment. If this reflects true clinical outcomes, the trend toward the eventual disappearance of female gender assignment for 46XY cloacal exstrophy is concerning.

Copyright © 2011 American Urological Association Education and Research, Inc. Published by Elsevier Inc. All rights reserved.


Long-term outcome of male genital reconstruction in childhood.

J Pediatr Urol. 2013 May 9. pii: S1477-5131(13)00092-2. doi: 10.1016/j.jpurol.2013.03.017.

Long-term outcome of male genital reconstruction in childhood.

Tourchi A, Hoebeke P.

Division of Pediatric Urology, Brady Urological Institute, The Johns Hopkins Medical Institutions, Baltimore, Maryland, USA. Electronic address: alitourchi@pedurol.com.


Hypospadias, epispadias with or without exstrophy, and disorders of sex development are among the most common anomalies of genitalia that occur during childhood. Considering the tremendous effect of genitourinary reconstruction on adult life, the evaluation of the long-term results of different techniques of genitoplasty in pediatrics is of the utmost importance. After reviewing the literature, the authors summarize the available long-term outcomes of genitoplasty in childhood, specifically focusing on the cosmetic, psychosocial, psychosexual and functional results, and emphasize that, contrary to the widely available data on early outcomes of genital reconstruction in the pediatric population, very few well described controlled studies have evaluated the long-term effect of genitoplasty in puberty and adulthood, in the sense that the surgeon should describe the peroperative findings in more detail and also be more structured in evaluating the postoperative result at follow-up visits. Finally, the authors conclude that more attention should be paid to the impact of these techniques on cosmetic aspects and psychosexual development in these patients after puberty, as they play a crucial role in their adult quality of life.


Gender identity outcome in female-raised 46,XY persons with penile agenesis, cloacal exstrophy of the bladder, or penile ablation.

Arch Sex Behav. 2005 Aug;34(4):423-38.

Gender identity outcome in female-raised 46,XY persons with penile agenesis, cloacal exstrophy of the bladder, or penile ablation.

Meyer-Bahlburg HF.
SourceNew York State Psychiatric Institute, New York, NY 10032, USA. meyerb@child.cpmc.columbia.edu


This review addresses the long-term gender outcome of gender assignment of persons with intersexuality and related conditions. The gender assignment to female of 46,XY newborns with severe genital abnormalities despite a presumably normal-male prenatal sex-hormone milieu is highly controversial because of variations in assumptions about the role of biological factors in gender identity formation. This article presents a literature review of gender outcome in three pertinent conditions (penile agenesis, cloacal exstrophy of the bladder, and penile ablation) in infancy or early childhood. The findings clearly indicate an increased risk of later patient-initiated gender re-assignment to male after female assignment in infancy or early childhood, but are nevertheless incompatible with the notion of a full determination of core gender identity by prenatal androgens.




窪田 昭男 奈良 啓悟 川原 央好 米田 光宏 田附 裕子 谷 岳人 合田 太郎 中畠 賢吾 

大阪府立母子保健総合医療センター小児外科 (2012)














高野, 泰江
信州大学医学部附属病院看護研究集録 1988: 114-123(1988)


 今回私達は,総排滑腔外反症(贋帯ヘルニア,勝耽外反,尿道上裂,回腸勝朕凄,総排他腔異常,恥骨結合解離) という極めて稀な症例に出会った。そこで, この児の成長過程での問題は何であるか予測し,看護計画を立てて援助してみた。



 そこで,最終目標を「発育に伴い児が精神的に自立できるようにする」とし,その為には母親の児の受容と精神的安定・自立が必要と考え看護してきたので, ここにその経過を発表する。


Discordant Sexual Identity in Some Genetic Males with Cloacal Exstrophy Assigned to Female Sex at Birth

W.G. Reiner and J.P. Gearhart(2004)


背 景

 総排泄腔外反症は,胚形成時に起る骨盤全体および骨盤内組織のまれな複合欠損であり,遺伝的には男性である患児において重篤な陰茎発育不全や陰茎欠損と関連する.陰茎発育不全の問題に対処するため,この 25 年間,罹患した男児に対しては新生児期に性別を女性とすることが推奨されてきたが,転帰に関するデータは依然として少ない.

方 法

 われわれの総排泄腔外反症クリニックにおいて,組み入れ時に 5〜16 歳の遺伝的には男性である患児計 16 例を評価した.14 例は,新生児期に社会的・法律的に,そして外科的処置を行って性別を女性とした;残る 2 例の両親はこれを拒否した.詳細な質問票を用いて,対象者自身による継続的な性別の申告によって定義した性的役割および性同一性の発達について,広範囲にわたって評価した.

結 果

 性別を女性とされた 14 例の対象者のうち,8 例がこの研究の過程で自分は男性であると申告し,一方,男性として育てられた 2 例は男性のままであった.対象者は,表明された性同一性に従ってグループ分けが可能であった.5 例は女性として生活していた;3 例は,うち 2 例が自分は男性であると申告していたが,性同一性が曖昧な状態で生活していた;8 例は男性として生活し,うち 6 例が性別を女性から男性へと変更していた.16 例の対象者は全員,中等度から高度の男性に典型的と考えられる態度や関心を示した.追跡期間は 34〜98 ヵ月であった.

結 論