Case notes
A 13-year-old girl came to our hospital complaining of a left vulval mass. Although it had first appeared more than 1 year earlier, it had begun growing rapidly in the 6 months before her visit ( Figure 1 ). At age 5 months, the patient had undergone excision of a left vulval protruding lesion, which had been located just to the left of the present mass. At that time, pathology testing showed ovotestes and G-banding chromosomal analysis disclosed a karyotype of 46,XX. The patient received a diagnosis of ovotesticular disorder of sexual development, previously known as true hermaphroditism. After these results were obtained, a laparotomy had been performed, and no uterus, ovaries, or testes were found in her peritoneal cavity.
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When the patient presented on this occasion, a physical examination demonstrated well-developed breasts and a slightly enlarged clitoris (about 15 mm) with the urethra below it ( Figure 2 ). No vagina was found. Posterior labial fusion was observed with an anogenital ratio of 0.58; the ratio was determined by dividing the distance from the anus to the posterior fourchette, which was 5.5 cm, by the distance from the anus to the base of the clitoris, which was 9.5 cm. Palpation disclosed no abnormalities in the right vulval and inguinal region; this was corroborated by computed tomography and an ultrasound scan.
Conclusions
The mass located on the left vulva was cystic with 2 solid oval bodies within it, according to palpation and imaging results. A translucency test was positive. The patient underwent surgery with an epidural anesthetic. Opening of the cyst wall exposed a clear yellow fluid within. Further dissection led us to discover that the structures seen on imaging were a rudimentary uterus, an underdeveloped or “hypogenetic” fallopian tube, and an ovary ( Figures 3 and 4 ). Histopathology confirmed the identity of our findings. The rudimentary uterus was removed, although the ovary and the fallopian tube, preserved for conservation of hormonal supply, were moved to the peritoneal cavity via the left inguinal canal ( Figure 5 ).
