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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.)

Determination of a mammal’s sex begins at conception with the establishment of genotype and continues from there as the expression of specific genes directs the bipotential gonad to develop. The gonad further directs the sexual differentiation of the individual. Deviations from either of these pathways at any stage results in disorders of sexual development. Definitive diagnosis minimally requires a karyotype, histopathologic evaluation of the gonads, and gross description of the genital anatomy, with more complete diagnostic answers achieved through other diagnostic tests. This article covers normal and abnormal development of the reproductive organs with emphasis on diagnosis and treatment.

CT and MR imaging can both contribute valuable clinical information in women with benign and malignant ovarian masses; the superior soft tissue contrast and multiplanar capabilities provided by MR imaging make it a valuable tool to evaluate the normal ovary, polycystic ovaries, endometriosis, and disorders of sexual differentiation. CT is currently the recommended modality to stage ovarian carcinoma, and peritoneal implants as small as 5 mm can be visualized. With the addition of contrast-enhanced images to conventional MR sequences, however, improved visualization of intratumoral architecture has also allowed for accurate MR characterization of benign versus malignant ovarian masses. Fat saturation techniques used with conventional MR sequences can also be used for definitive characterization of benign fat-containing teratomas and differentiate these tumors from hemorrhagic ovarian cysts or endometriomas. Finally, the detailed visualization of the normal-sized ovary on MR images provided by both the body coil and the new phased-array coil allows detection of the normal ovaries, ovarian follicles, and ovarian cysts throughout the premenopausal and postmenopausal years. Findings of polycystic ovarian syndrome, which cannot be evaluated by CT, are characteristic on MR images. MR is unparalleled in the evaluation of disorders of sexual differentiation because MR images can depict ectopic and normally positioned gonads and provide multiplanar depiction of the other pelvic organs and perineum as well. CT and MR imaging can provide valuable and specific clinical information about the ovary and benign and malignant ovarian disease.

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.