Anat Cell Biol.  2022 Dec;55(4):475-482. 10.5115/acb.22.082.

Distal vaginal atresia: a report of a rare type found a late-term fetus and its histological comparison with the normal pelvis

• Kim JH
• Jin ZW
• Abe H
• Murakami G
• Rodríguez-Vázquez JF
• Hinata N

• KMID: 2537467
• DOI: http://doi.org/10.5115/acb.22.082

Abstract

Solitary distal vaginal atresia is generally caused by a transverse septum or an imperforate hymen. We found a novel type of distal vaginal atresia in a late-term fetus (gestational age approximately 28 weeks) in our histology collection. This fetus had a vaginal vestibule that was closed and covered by a thick subcutaneous tissue beneath the perineal skin in the immediately inferior or superficial side of the imperforate hymen. The uterus, uterine tube, anus, and anal canal had normal development. The urethral rhabdosphincters were well-developed and had a normal topographical relationship with the vagina, but the urethrovaginal sphincter was absent. Thus, vaginal descent seemed to occur normally and form the vestibule. However, the external orifice of the urethra consisted of a highly folded duct with hypertrophied squamous epithelium. Notably, the corpus cavernosum and crus of the clitoris had poor development and were embedded in the subcutaneous tissue, distant from the vestibule. Normally, the cloacal membrane shifts from the bottom of the urogenital sinus to the inferior aspect of the thick and elongated genital tubercle after establishment of the urorectal septum. Therefore, we speculate there was a failure in the transposition of the cloacal membrane caused by decreased elongation of the genital tubercle. The histology of this anomaly strongly suggested that the hymen does not represent a part of the cloacal membrane, but is instead a product that appears during the late recanalization of the distal vagina after vaginal descent. The transverse septum was also likely to form during this recanalization.

Keyword

Imperforate hymen; Genital tubercle; Cloacal membrane; Corpus cavernosum of the clitoris; Anomaly

Figure

• 

  Fig. 1 Topographical anatomy of vaginal atresia in a fetus with a CRL of 235 mm (sagittal sections). (A) is an almost-midsagittal section, and the five inner squares appear at higher magnification in Fig. 2A–E; (C) is the most lateral plane and the inner square appears at higher magnification in Fig. 2F. Inserts on the left of each figure show poorly developed cavernous tissues: the CC of the clitoris, which is surrounded by the developing prepuce (top); the crus of the clitoris (middle); and the vestibular bulb (bottom). Arrows in (B) and (C) indicate an end part of a lateral recess of the closed vestibule. The clitoris is embedded in a thick subcutaneous tissue that covers and closes the vestibule, and the urethra and vagina open to the vestibule. The EAS, IAS are normal. Asterisks in (A) indicate an artifact space from the histological procedure. (A–C) were at the same magnification and the inserts were at the same magnification. Scale bar: (C) 5 mm; (bottom insert) 1 mm. CRL, crown-rump length; CC, corpus cavernosum; EAS, external anal sphincter; IAS, internal anal sphincter; BSM, bulbospongiosus muscle; GMX, gluteus maximus muscle; ICM, ischiocavernosus muscle; LAM, longitudinal anal muscle.

• 

  Fig. 2 Higher magnification of major structures at and around the vaginal atresia (five squares from Fig. 1). (A) shows the urethral rhabdosphincter (external sphincter). (B) shows the external orifice of the urethra with hypertrophied squamous epithelium. (C) shows a sandwiched area of the transient zone between the columnar and squamous epithelia (arrow heads). (D) shows hypertrophied squamous epithelium covering a short longitudinal septum of the lower vagina. (E) shows a thick subcutaneous tissue closing the vestibule that contains smooth muscle-like fibers. (F) shows an area immediately lateral to the vestibular bulb. The arrow in (F) indicates the lateral end of a recess of the vestibule. (A–D) were at the same magnification, and (E and F) were at the same lower magnification. Scale bars: (A–F) 1 mm. BSM, bulbospongiosus muscle; EAS, external anal sphincter; IAS, internal anal sphincter; ICM, ischiocavernosus muscle.

• 

  Fig. 3 Topographical anatomy of the uterus, vagina, and pelvic floor muscles (contralateral side of Fig. 1). (A) is 1.6 mm lateral to (B). The levator ani muscle was cut tangentially (surrounded by the dotted line in A). The uterine cervix was normal (insert, bottom right of A). A candidate of the Bartholin gland is present in the anterolateral side of the vestibule (insert, bottom right of B). (A and B) were at the same magnification and the inserts were at the same magnification. Scale bar: (A) 5 mm; (A insert) 1 mm. EAS, external anal sphincter; IAS, internal anal sphincter; GMX, gluteus maximus muscle.

• 

  Fig. 4 Normal genital morphology at the final phase of vaginal descent in a fetus with CRL of 61 mm (sagittal sections near the midsagittal plane). (A) is the most lateral plane and (D) is the most medial plane. (E–G) are higher magnification views of the squares in (A–C), respectively. (B and C) show that the future vestibule of the vagina has a deep, gulf-like appearance. (F and G) show that the vaginal lumen is closed, and has a thick line-like appearance before recanalization. (F and G) (arrows) show the distal end of the vagina merges with the urethra near the external urethral orifice, and the vaginal descent was not yet complete. (E) shows that a longitudinal septum (star) was present in the vestibule. (E and F) show that the urethral rhabdosphincter (surrounded by the dotted line) extended inferiorly or distally along the anterior aspect of the vestibule. (C and D) show a large glans of the clitoris at the distal end of the thick CC. (A–D) were at the same magnification and (E and F) were at the same magnification. Scale bars: (A–G) 1 mm. CRL, crown-rump length; CC, corpus cavernosum.

Reference

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