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Incomplete polyorchidism (also called bilobed testicle) is an extremely uncommon congenital anomaly. Only 3 cases of bilobed testicle were previously reported in the available literature. We describe a case of a 4-year-old boy who presented with a 6-month history of an asymptomatic scrotal mass located in the upper pole of the left testicle mimicking testicular tumour. After partial orchiectomy, macroscopic and pathological examination of the lesion confirmed the diagnosis of normal testicular tissue., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Müllerian inhibiting substance (MIS), a gonadal hormone important in sexual differentiation, is high (10 to 70 ng/mL) in human male serum postnatally for several years before declining during the peripubertal period, but is undetectable in female serum until the onset of puberty. The sexually dimorphic secretion of MIS suggested possibilities for its use in several clinical settings. Thirty-one patients with intersex and gonadal anomalies from 17 institutions were therefore evaluated between 1989 and 1992 with an MIS enzyme-linked immunosorbent assay (ELISA). Serum MIS levels correlated with the presence of testicular tissue in two patients with suspected anorchia, five patients with male pseudohermaphroditism, and eight other intersex patients with undescended testes, dysgenetic gonads, or ovotestes. In these latter patients, serial MIS values were also helpful to confirm complete removal of gonadal tissue postoperatively. MIS may be a more sensitive marker for the presence of testicular tissue than serum testosterone levels, both before and after the neonatal androgen surge, and, consequently, may obviate the need for human chorionic gonadotropin stimulation in the evaluation of certain intersex disorders. In values were useful in differentiating the underlying etiology of the disorder. Four patients with undetectable levels have presumptive MIS gene mutations, while 7 others with MIS values of 2 to 45 ng/mL may have bioinactive hormone of MIS receptor defects. Finally, two young girls with ovarian granulosa cell tumors had elevated MIS values that fell from 18 to 2 ng/mL and from 6.5 to 1 ng/mL during postoperative follow-up.(ABSTRACT TRUNCATED AT 250 WORDS)

We report a 5 1/2-year-old boy with preperitoneal ectopic testes. This condition has been reported only once before. Both testes were attached to the umbilicus and at operation were successfully placed in the scrotum.