Abstract
No information is available about therapeutic management of young boys with persistence of Mullerian structures. We therefore report the first total surgical correction in a boy aged 3 8/12 years. Bilateral cryptorchidism was noted at birth. At age 13 mo. at left inguinal herniorrhaphy a uterus and a gonad were found in the hernial sac. Histological: Reduction of spermatogonias by 50%, Karyogram: XY. Normal basal urinary excretion of C19 and C21 steroids determined by capillary GLC; following 25 μg LH-RH i.v.: max. ΔLH:65 ng/ml; max. ΔFSH: 210 ng/ml; normal rise of plasma testosterone after infusion of HCG. At age 3 8/12 yrs. a laparatomy was performed. Uterus and Fallopian tubes were removed. The gonads were placed into the scrotum together with their adnexa and the distal end of the vas deferentia. Leydig cell function was re-examined by an i.v. HCG test: normal rise of plasma testosterone. The etiology of persistent Mullerian structures in a chromosomal male is unknown. Insufficient secretion of testosterone during early fetal life or the absence of a factor similar to that described by Josso (1) may be responsible. (1) Josso, N.: Fed. Res. & (1974), 755.
Supp. by DFG; SFB 87, Proi. C3.
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Homoki, J., Mildenberger, H., Herrmann, H. et al. EARLY SURGICAL MANAGEMENT OF A BOY WITH PERSISTENT MULLERIAN STRUCTURES. Pediatr Res 9, 670 (1975). https://doi.org/10.1203/00006450-197508000-00035
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DOI: https://doi.org/10.1203/00006450-197508000-00035