PubMed · 15407513
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J SEILER. 1949-11-15. [Intersexuality].. https://doi.org/10.1007/bf02165249
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The Leydig cell hypoplasia is a rare and well defined form of male pseudohermaphroditism with autosomal recessive inheritance pattern. An inadequate fetal testicular Leydig cell differentiation and, consequently a low androgenic production during intra uterine and post natal periods, result in absence or incomplete virilization in patients with 46,XY karyotype. These patients exhibit a wide clinical spectrum, ranging from complete female external genitalia to male external genital with micropenis, low serum testosterone levels associated with high LH levels. Inactivating mutations of the LH/hCG receptor gene have been identified in affected families in the last decade. However, the low frequency of inactivating mutations in this gene, and the lack of segregation of intragenic polymorphisms among affected members from families with typical phenotype of Leydig cell hypoplasia, suggest the genetic heterogeneity of this condition.
PURPOSE OF REVIEW: To review the relevant publications since 2004 on surgery for female pseudohermaphroditism. Current controversies exist regarding the timing and technical aspects of female genital reconstruction and indeed the wisdom of performing these procedures in childhood. RECENT FINDINGS: Nine articles, published between 2004 and the present time, were found worthy of being included in this review. The topics include surgical technique, short-term and long-term results, voiding function after feminizing genitoplasty, and the timing of surgery. SUMMARY: Although results for surgery for congenital adrenal hyperplasia have been less than satisfactory when adults who had surgery in childhood are evaluated, all present reports include patients operated on using a variety of techniques many years ago. Rather than abandoning the efforts to repair this malformation early, we favor the continued development of more refined surgical techniques that may yield better results in the future.