RESUMO
Herlyn-Werner-Wunderlich (HWW) syndrome is a rare and complex congenital anomaly of the genitourinary system characterized by uterus didelphys, an obstructed hemivagina, and ipsilateral renal agenesis. It is the result of the maldevelopment of both the Mullerian and Wolffian ducts. Clinical manifestations of dysmenorrhea, dysuria, and urinary retention appear after menarche due to the accumulation of menstrual blood and distention of the obstructed hemivagina. Diagnosis of this anomaly is often delayed, and the obstructive nature of the condition is missed due to cyclic menstruation from the one canalized hemivagina. This case report represents a typical form of HWW syndrome to highlight the importance of considering this rare entity in an adolescent girl presenting with signs of dysmenorrhea and urinary complaints. A 13-year-old patient was presented at the obstetrics and gynecology clinic due to low abdominal pain and urinary retention on the fifth day of menstruation. For the last 6 months, the patient suffered progressive cyclic low abdominal pain associated with menstruation. Inspection of the external genitalia was unremarkable, and the hymenal membrane had a normal oval opening. An ultrasound examination revealed the sonographic features of the didelphys uterus and was suggestive of a right obstructed hemivagina with hematocolpos. Magnetic resonance imaging and computed tomography urography showed evidence of didelphys uterus, right blind obstructed hemivagina associated with huge hematocolpos, and ipsilateral renal agenesis. The diagnosis of HWW syndrome was established. Surgical resection of the vaginal septum was undertaken without delay, and the patient was discharged the following days.
RESUMO
Although rare, sacrococcygeal teratoma is the most common congenital neoplasm, occurring in 1 in 40,000 infants. Approximately 75% of affected infants are female. The aim of the present study was to correlate ultrasonography and magnetic resonance imaging (MRI) findings in patients with fetal sacrococcygeal teratoma. Three pregnant women in 27th week of gestation underwent fetal MRI after ultrasonography examination, with findings suggestive for fetal sacrococcygeal teratoma. Tumor size, location, extent and content were evaluated both by MRI and ultrasonography. Findings regarding tumor location, size and content were similar for both methods. There was a large well-circumscribed mixed, cystic/solid oval mass, originating from right sacro-gluteal region and projecting into the amniotic cavity, 132 × 110 × 76 mm in size. The mass had a heterogeneous appearance. The T1 high signal suggested fat component of the tumor, while T1 and T2 hypointense components suggested calcified/bony components. There was also T1 hypointense component consistent with cystic and fluid component. The imaging findings were characteristic for sacrococcygeal teratoma. There was not obvious lumbar or thoracic spinal involvement. There was no gross intrapelvic or abdominal extension, and even sacrum and coccyx appeared deformed. The amount of amniotic fluid was increased. MRI was superior to ultrasonography in the evaluation of the exact tumor extent, accurately demonstrating pelvic involvement in all of the three cases. Fetal MRI has shown to be a valuable adjunct to obstetric sonography in the evaluation of fetal sacrococcygeal teratoma, because of its higher accuracy in the determination of tumors extent and content, playing a significant role in the therapeutic planning and increasing the chances of cure for these fetuses.
