Griebel M L, Redman J F, Kemp S F, Elders M J
Department of Pediatrics, University of Arkansas, College of Medicine, Little Rock.
Urology. 1991 Apr;37(4):337-9. doi: 10.1016/0090-4295(91)80260-e.
Although clitoral involvement with neurofibromatosis is rare, all cases previously reported have described clitoral hypertrophy due to neurofibromas of the clitoral corpora. We report on a patient who had localized enlargement of the prepuce only, with no evidence of neurofibromatous infiltration. In all cases, diagnosis of clitoromegaly requires basic chromosomal and endocrinologic evaluation. However, recognition of its association with neurofibromatosis due to either neurofibromas of the genitalia or localized genital hypertrophy may spare the patient an unnecessary or invasive evaluation. Because of an association with urinary tract neurofibromas, the patient with genital involvement should have cystoscopy. Clitoroplasty with sparing of the neurovascular bundle and glans is the preferred method of management of the enlarged clitoris.
虽然阴蒂受累于神经纤维瘤病很罕见，但之前报道的所有病例均描述为因阴蒂体神经纤维瘤导致的阴蒂肥大。我们报告了一名仅包皮局部增大的患者，没有神经纤维瘤浸润的证据。在所有病例中，阴蒂肥大的诊断需要进行基本的染色体和内分泌评估。然而，认识到其与因生殖器神经纤维瘤或局部生殖器肥大导致的神经纤维瘤病的关联，可能使患者避免不必要的或侵入性的评估。由于与泌尿道神经纤维瘤有关联，生殖器受累的患者应进行膀胱镜检查。保留神经血管束和阴蒂头的阴蒂成形术是处理增大阴蒂的首选方法。
