关键词: Aphallia hormonal replacement hydronephrosis penile reconstruction vaginoplasty

Mesh : Abnormalities, Multiple / diagnosis Counseling Diagnosis, Differential Humans Infant, Newborn Male Parents Penis / abnormalities

来  源:   DOI:10.1177/0049475515576675   PDF(Sci-hub)

Abstract:
Complete penile agenesis (aphallia) is a very rare congenital anomaly. Around 80 cases have been reported to date. Diagnosis of this rare anomaly is made by clinical examination, but treatment options and parental counselling for gender assignment is difficult and challenging for the treating doctor. We report a case of an infant with complete penile agenesis with recto-urethral fistula, who was referred on day 6 of life. This baby developed obstructive uropathy which required vesicostomy. Surgical management consists of gender assignment after discussing in detail the pertinent situation with the parents and accepting their final decision.
摘要:
完全阴茎发育不全(aphallia)是一种非常罕见的先天性异常。迄今为止,已报告约80例。这种罕见异常的诊断是通过临床检查得出的,但是对于治疗医生来说,治疗选择和性别分配的父母咨询是困难和具有挑战性的。我们报告了一例阴茎完全发育不全并伴有尿道瘘的婴儿,在生命的第六天被转介。这个婴儿出现了阻塞性尿路病,需要进行膀胱造口术。手术管理包括与父母详细讨论相关情况并接受他们的最终决定后的性别分配。
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