Department of Pediatrics, University of Rennes 1, Rennes, France
Department of Pediatrics, University of Rennes 1, Rennes, France.
Pediatrics. 2017 Oct;140(4). doi: 10.1542/peds.2016-1807.
Neonatal clitoromegaly is mainly attributed to in utero androgen exposure secondary to congenital adrenal hyperplasia. We report on 2 extremely premature girls with clitoromegaly, increased androgen levels, no salt wasting syndrome, and ovarian cyst. In case 1, the cyst liquid was aspired during ovarian hernia surgery and revealed high androgen levels. After aspiration, serum androgen levels decreased, as did clitoral size. In case 2, an ovarian cyst was seen on pelvic ultrasound. Aspiration was not indicated. The cyst regressed spontaneously on iterative pelvic ultrasounds, and her clitoromegaly decreased. Case 1 demonstrates the ovarian origin of this transient virilization. Cyst formation seems to be linked to the physiologic maturation of the hypothalamic-pituitary-ovarian axis. Thirteen cases of clitoromegaly with hyperandrogenism, without salt wasting syndrome, have been reported in extremely premature infants. In the context of clitoromegaly, we recommend ruling out in utero androgen exposure, adrenal hyperandrogenism, and disorders of sex development. We further recommend affirming hyperandrogenism by androgen assay and confirming ovarian origin with gonadotrophin assays and pelvic ultrasound. Drug therapy abstention and clinical and ultrasound monitoring are recommended because spontaneous regression of clitoral hypertrophy seems to be the most common outcome in the literature, as it was in our 2 observations.
新生儿阴蒂肥大主要归因于先天性肾上腺增生导致的子宫内雄激素暴露。我们报告了 2 例极早产儿出现阴蒂肥大、雄激素水平升高、无盐耗综合征和卵巢囊肿。在病例 1 中，在卵巢疝手术中抽吸了囊肿液体，结果显示雄激素水平升高。抽吸后，血清雄激素水平下降，阴蒂大小也随之减小。在病例 2 中，盆腔超声检查发现卵巢囊肿。未行抽吸。囊肿在反复盆腔超声检查中自发消退，阴蒂肥大也随之减轻。病例 1 表明这种短暂性男性化是卵巢来源的。囊肿形成似乎与下丘脑-垂体-卵巢轴的生理性成熟有关。在极早产儿中，已有 13 例报道出现无盐耗综合征的阴蒂肥大伴高雄激素血症。在阴蒂肥大的情况下，我们建议排除子宫内雄激素暴露、肾上腺雄激素过多症和性发育障碍。我们进一步建议通过雄激素测定来确认高雄激素血症，并通过促性腺激素测定和盆腔超声来确认卵巢来源。建议避免药物治疗，并进行临床和超声监测，因为文献中似乎最常见的结果是阴蒂肥大自发消退，就像我们的 2 例观察结果一样。
