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首页> 外文期刊>BJU international >A posterior-based omega-shaped flap vaginoplasty in girls with congenital adrenal hyperplasia caused by 21-hydroxylase deficiency.
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A posterior-based omega-shaped flap vaginoplasty in girls with congenital adrenal hyperplasia caused by 21-hydroxylase deficiency.

机译:由21-羟化酶缺乏症引起的先天性肾上腺皮质增生的女孩的后方Ω形皮瓣阴道成形术。

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摘要

OBJECTIVE: To describe a technical modification for constructing a vagina in girls with congenital adrenal hyperplasia caused by 21-hydroxylase deficiency, using a narrower skin base but rich subcutaneous tissue, aiming to obtain both longer and larger vaginal segments with better cosmesis of the external genitalia. PATIENTS AND METHODS: From August 1997 to February 2001, 28 girls (aged 5 months to 17 years) had a neovagina constructed using a posterior-based omega-shaped flap. Twenty-six patients had a low vagina entering into the urogenital sinus and two had a high vagina that entered the urogenital sinus. In those with a high vagina the flap procedure was combined with the Passerini-Glazel technique. All the patients were scheduled for vaginal "calibrations" during the first year after surgery and, according to the result, would then undergo vaginal dilatation. RESULTS: Six children were re-operated; five had plastic surgery to correct genital folds that had regained a scrotal aspect, whereas one with a high vagina developed a urethral stricture, with urinary dribbling and infection, and had the urethra reconstructed. These six children are currently well. No hormone therapy was given to one child for 1 year who is scheduled for further surgery for a re-virilized clitoris. Two patients were lost to follow-up. Up to the last visit, 19 girls had not developed a vaginal stricture and the cosmesis of their external genitalia was deemed good. CONCLUSION: The posterior-based omega-shaped flap enabled both the construction of wider vaginal segments with a low risk of developing stenosis in those with a low vagina, and increased vaginal dimension when associated with the Passerini-Glazel technique for those with a high vagina. However, despite good cosmesis of the external genitalia, the follow-up is too short to confirm whether this technique will meet all the expectations.
机译:目的:描述一种技术改造,以利用较窄的皮肤基层但丰富的皮下组织,在由21-羟化酶缺乏症引起的先天性肾上腺增生的女孩中构建阴道,以期获得更长和更大的阴道段,并具有更好的外生殖器美感。患者与方法:从1997年8月到2001年2月,有28个女孩(年龄5个月至17岁)使用后方的欧米伽状皮瓣构造了一个新阴道。 26例患者的阴道进入泌尿生殖窦的位置较低,而两个患者的阴道进入泌尿生殖窦的位置较高。在阴道高的患者中,皮瓣手术与Passerini-Glazel技术相结合。所有患者都计划在手术后的第一年进行阴道“校准”,然后根据结果进行阴道扩张。结果:6名儿童再次手术。五名接受整形手术以矫正阴囊的阴部皱褶,而阴部高的一位则出现尿道狭窄,尿尿带菌和感染,并重建了尿道。这六个孩子目前状况良好。一名儿童在一年内未进行任何激素治疗,该儿童计划接受再次手术治疗阴蒂再次阴蒂。两名患者失去随访。截至最后一次访视,有19名女孩尚未出现阴道狭窄,其外生殖器的美感被认为是良好的。结论:基于后方的欧米茄状皮瓣既可在阴道较矮的人群中构建较宽的阴道段,降低发生狭窄的风险,又可通过Passerini-Glazel技术为阴道较高的患者增加阴道尺寸。但是,尽管外部生殖器美观,但随访时间太短,无法确定该技术是否能够满足所有期望。

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