De Castro Roberto, Merlini Emilio, Rigamonti Waifro, Macedo Antonio
Department of Pediatric Surgery, Ospedale Maggiore of Bologna, Bologna, and Department of Urology, University of Padua, Italy.
J Urol. 2007 Mar;177(3):1112-6; discussion 1117. doi: 10.1016/j.juro.2006.10.095.
Female gender has been assigned to 46,XY newborns affected by aphallia, possibly resulting in subsequent gender dysphoria. Prenatal and postnatal effects of the androgens on the brain and sexual orientation cannot be modified later. Therefore, patients affected by aphallia should be raised as males. Because definitive forearm flap phalloplasty is generally not recommended before puberty, we performed a preliminary penile reconstruction during childhood in 4 patients.
Four patients with aphallia who had no sex reassignment at birth were treated at age 9, 17 and 36 months, and 12 years in a single operation. The urethral channel was dissected through an anterior-sagittal-transanorectal approach, and then phalloplasty and urethroplasty were carried out using an abdominal skin flap and a bladder/buccal mucosa free graft.
Immediate postoperative outcome was excellent in all the patients. One patient had excellent functional and cosmetic results at 5 years, while 2 had a partial dorsal urethral dehiscence resulting in an epispadiac urethra at 2 years, and 1 had necrosis of the distal urethra and was voiding through a scrotal urethrostomy at 9 months postoperatively. Phalloplasty survived and provided an adequate male appearance in all patients.
Opposite gender should not be assigned in patients affected by penile agenesis, who are better raised according to their karyotype and hormonal production. Definitive phalloplasty in adults may achieve good results. Nevertheless, this procedure is generally performed in postpubertal boys and it is not easily available everywhere. Therefore, we believe that social and psychological concerns justified this type of phalloplasty as a palliative preliminary procedure in 3 of our patients. In those countries where definitive forearm phalloplasty is not available our method may also be justified in older children (as in 1 of our patients) as an attempt at a definitive procedure.
对于患有无阴茎症的46,XY新生儿，已将其性别指定为女性，这可能会导致随后的性别焦虑。雄激素对大脑和性取向的产前和产后影响在后期无法改变。因此，患有无阴茎症的患者应作为男性抚养。由于一般不建议在青春期前进行确定性的前臂皮瓣阴茎成形术，我们对4例患者在儿童期进行了初步阴茎重建。
4例出生时未进行性别重新分配的无阴茎症患者分别在9个月、17个月、36个月和12岁时接受了单次手术治疗。通过前矢状经肛门直肠入路解剖尿道通道，然后使用腹部皮瓣和膀胱/颊黏膜游离移植物进行阴茎成形术和尿道成形术。
所有患者术后即刻效果均良好。1例患者在5岁时功能和外观效果极佳，而2例在2岁时出现部分背侧尿道裂开，导致尿道上裂，1例在术后9个月出现远端尿道坏死，通过阴囊尿道造口排尿。阴茎成形术均存活，所有患者均呈现出足够的男性外观。
对于患有阴茎发育不全的患者，不应指定其为相反性别，根据其核型和激素分泌情况抚养更为合适。成人进行确定性阴茎成形术可能会取得良好效果。然而，该手术通常在青春期后的男孩中进行，并非随处都能轻易开展。因此，我们认为，出于社会和心理方面的考虑，对于我们的3例患者而言，这种阴茎成形术作为一种姑息性初步手术是合理的。在那些无法进行确定性前臂阴茎成形术的国家，我们的方法对于年龄较大的儿童（如我们的1例患者）作为确定性手术的尝试也可能是合理的。
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