Abstract

We report the case of a 6 years old child, admitted for management of testicular ectopia, the clinical examination found an empty scrotum, a normal looking penis and a urethral meatus in place. The scrotal ultrasound showed an intra-abdominal testicle (left illiac fossa) of homogeneous echostructure on the left side, while the right testicle was not visualized. The gonadotropins LH and FSH levels were low, as well as the level of testosterone, the secretion of which is stimulated after administration of Pregnyl. Laparoscopy revealed 2 gonadal formations on the same side, 2 tubes and a median formation reminiscent of the uterus. The karyotype of the patient was male 46XY, the dosage of anti Müllerian hormone (AMH) was high, confirming an abnormality of its receptors, which explains the persistence of the Müllerian ducts in this child. The child underwent a left gonadectomy with bilateral hysterectomy and adnexectomy, associated with a lowering of the right testicle. At 9 years, pubertal development was classified as T3P4 according to Tanner's classification, scrotal ultrasound showed a free left testicular compartment with testicular calcifications classified as grade 3, and tumor marker levels were normal.

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