Patil K K, Duffy P G, Woodhouse C R J, Ransley P G
Department of Paediatric Urology, Great Ormond Street Hospital for Children, London, United Kingdom.
J Urol. 2004 Apr;171(4):1666-9. doi: 10.1097/01.ju.0000118139.28229.f5.
Intra-abdominal testes in boys with prune-belly syndrome have been conventionally managed by 1 or 2-stage orchiopexy with division of the gonadal vessels. We reviewed a series of adults with prune-belly syndrome to assess the morphological and functional outcome of orchiopexy in childhood with specific reference to the spontaneous onset of puberty, hormonal profiles and sexual function.
A total of 41 boys were divided into 3 groups depending on the type of orchiopexy performed, namely group 1-20 with bilateral 1-stage orchiopexy, group 2-10 with unilateral 1-stage and contralateral 2-stage orchiopexy, and group 3-11 with bilateral 2-stage orchiopexy.
In group 1 9 of 20 patients had good scrotal testes bilaterally, 6 had a good scrotal testis on 1 side and 3 had small testes on each side. Two boys required testosterone supplementation but 18 had normal hormonal and sexual function. In group 2 6 of 10 patients had good scrotal testes bilaterally and 4 had a good scrotal testis on 1 side. All patients underwent spontaneous puberty with good sexual function. In group 3 7 of 11 boys had good scrotal testes bilaterally and 3 had 1 good testis with normal puberty and sexual function. These 10 patients underwent spontaneous puberty with good sexual function.
The majority of boys with prune-belly syndrome had a satisfactory outcome after orchiopexy with division of the gonadal vessels with testicular function sufficient to induce puberty and maintain satisfactory sexual function in adult life.
对于合并梅干腹综合征的男孩,传统上其腹腔内睾丸的处理方式是采用一期或二期睾丸固定术并切断性腺血管。我们回顾了一系列合并梅干腹综合征的成年患者,以评估儿童期睾丸固定术的形态学和功能结果,特别关注青春期的自然启动、激素水平及性功能。
根据所施行的睾丸固定术类型,将41名男孩分为3组,即第1组20例,行双侧一期睾丸固定术;第2组10例,一侧行一期睾丸固定术,另一侧行二期睾丸固定术;第3组11例,行双侧二期睾丸固定术。
第1组20例患者中,9例双侧睾丸在阴囊内位置良好,6例一侧睾丸在阴囊内位置良好,3例双侧睾丸较小。2名男孩需要补充睾酮,但18名男孩激素及性功能正常。第2组10例患者中,6例双侧睾丸在阴囊内位置良好,4例一侧睾丸在阴囊内位置良好。所有患者青春期自然启动,性功能良好。第3组11名男孩中,7例双侧睾丸在阴囊内位置良好,3例一侧睾丸位置良好,青春期及性功能正常。这10例患者青春期自然启动,性功能良好。
大多数合并梅干腹综合征的男孩在切断性腺血管的睾丸固定术后预后良好,睾丸功能足以启动青春期并在成年后维持满意的性功能。