PMC5867592_01
Morphological anomalies seen at antenatal ultrasound. (a) Cystic perineal mass (C), (b) equinovarus left foot, and (c) umbilical cord with a single artery.
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A perineal cyst, a left equinovarus foot, and a single umbilical artery were demonstrated during third trimester obstetric ultrasound (Figure 1).
Postnatal photographs of the newborn on day 2 of life. (a) Cystic vulvar mass with an orifice at the anterior superior part of the mass (broken arrow) emitting urine intermittently. (b) Below the cyst was another orifice (full arrow) emitting urine more regularly. (c) Equinovarus left foot. (d) Emptying of the mass after voiding.
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Physical examination of the newborn confirmed the presence of a cystic mass attached to the vulva, with an orifice in the anterosuperior part that occasionally emitted urine, with the mass emptying and gradually filling with the urine (Figure 2).
Postnatal pelvic and perineal ultrasound of the baby. (a-b) Suprapubic cross sections showing bladder (BL) and transperineal view showing cystic mass (C). (c) Uterus didelphys with the endometrial echogenic lines of the two hemiuteri (arrows).
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Examination of the urinary tract revealed normal kidneys and a normally located bladder, behind which two uterine hemimatrices suggestive of a uterus didelphys were visualized (Figure 3).
Radiographs centered on the anomalies of the bony structures (frontal view of the pelvis, lateral views of the lumbosacral spine and the left leg): VACTERL. Pubic diastasis, fusion of the last sacral vertebrae with agenesis of the coccyx, agenesis of the distal half of the tibia with fibular curvature, and an equinovarus deformation of the left foot.
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The analysis of the bony structures revealed a fusion of the last two sacral vertebrae with coccygeal agenesis and pubic diastasis and partial agenesis of the distal half of the left tibia with an equinovarus distortion of the foot (Figure 4).
Opacification with iodine-based contrast media. (a) and (b) Through the orifice below the cyst: evidence of two hemiuteri and tubes suggestive of a uterus didelphys. (c) and (d) Through the anterosuperior orifice: opacification of the vulvar cyst and of the phallic accessory urethra with the onset of bladder filling on the lateral view.
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Opacification through the orifice below the cyst initially led to the filling of two oblong cavities, each continuous by a filamentous and tortuous structure suggestive of the tubal morphology, with the left being opacified right up to the distal portions and peritoneal spillage of the contrast medium, thus confirming the uterus didelphys (Figures 5(a) and 5(b)). Pressure maneuvers revealed a short urethral pathway to the bladder that was partially opacified (Figures 5(c) and 5(d)).
(a-b) Intraoperative view: resection of the cyst and phallic accessory urethra. (c) Illustrative diagram of urethral duplicity: (1) accessory phallic urethra, (2) main urethra, (3) urogenital sinus, (4) anus, and (5) clitoral cyst.
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