Distal vaginal agenesis and differential diagnosis of other causes of hematocolpos
Authors:
P. Hobzová 1,2; Nikoleta Chubanovová 1
; Marta Nováčková 1
Authors place of work:
Gynekologicko-porodnická klinika 2. LF UK a FN Motol, Praha
1; Gynekologicko-porodnické oddělení, Nemocnice Havlíčkův Brod, p. o.
2
Published in the journal:
Ceska Gynekol 2022; 87(5): 350-355
Category:
Review Article
doi:
https://doi.org/10.48095/cccg2022350
Summary
Distal vaginal agenesis is an obstructive congenital malformation of the female reproductive tract. The distal part of the vagina is replaced by fibrous tissue and the outflow of the menstrual blood and secretion of the cervical glands is disabled. This congenital anomaly most often manifests during expected menarche by primary amenorrhea and cyclic lower abdominal pain caused by cryptomenorrhea with gradually increasing hematocolpos and hematometra. The diagnosis is based on gynecological examination and the suspected anomaly is confirmed by ultrasound examination and magnetic resonance imaging. Therapy of distal vaginal agenesis is exclusively surgical. A pull-through vaginoplasty is the method of choice for distal vaginal agenesis not exceeding 3 cm. With a greater extent of agenesis and the risk of postoperative vaginal stenosis, replacement of the missing part of the vagina with other tissues or modified balloon vaginoplasty can be used. The aim of the treatment is to enable the evacuation of the menstrual blood, ensure quality sexual intercourse and the possibility of reproduction.
Keywords:
hematocolpos – distal vaginal agenesis – distal vaginal atresia – hematometra – pull-through vaginoplasty
Zdroje
1. Tanitame K, Tanitame N, Urayama S et al. Congenital anomalies causing hemato/hydrocolpos: imaging findings, treatments, and out-comes. Jpn J Radiol 2021; 39 (8): 733–740. doi: 10.1007/s11604-021-01115-7.
2. Dietrich JE, Millar DM, Quint EH. Obstructive reproductive tract anomalies. J Pediatr Adolesc Gynecol 2014; 27 (6): 396–402. doi: 10.1016/ j.jpag.2014.09.001.
3. Gágyor D, Pilka R, Ondrová D et al. Distální vaginální ageneze. Ceska Gynekol 2019; 84 (4): 283–288.
4. Kang J, Mao M, Zhang Y et al. Congenital anal atresia with rectovestibular fistula, scoliosis, unilateral renal agenesis, and finger defect (VACTERL association) in a patient with partial bicornuate uterus and distal vaginal atresia: a case report. Medicine (Baltimore) 2018; 97 (45): e12822. doi: 10.1097/MD.0000000000012822.
5. Passos IM, Britto RL. Diagnosis and treatment of müllerian malformations. Taiwan J Obstet Gynecol 2020; 59 (2): 183–188. doi: 10.1016/ j.tjog.2020.01.003.
6. Dural O, Ugurlucan FG, Yasa C et al. A case of distal vaginal agenesis presenting with recurrent urinary tract infection and pyuria in a prepubertal girl. J Pediatr Adolesc Gynecol 2017; 30 (1): e7–e10. doi: 10.1016/j.jpag.2016.08. 007.
7. Kapczuk K, Kędzia W. Primary amenorrhea due to anatomical abnormalities of the reproductive tract: molecular insight. Int J Mol Sci 2021; 22 (21): 11495. doi: 10.3390/ijms222111495.
8. Moegni F, Meutia AP, Kouwagam AD et al. Secondary pyosalpinx after reconstructive surgery of vaginal agenesis patient with bilateral hematosalpinx: a case report. Int J Surg Case Rep 2021; 85: 106166. doi: 10.1016/j.ijscr.2021.106166.
9. Bischoff A, Alaniz VI, Trecartin A et al. Vaginal reconstruction for distal vaginal atresia without anorectal malformation: is the approach different? Pediatr Surg Int 2019; 35 (9): 963–966. doi: 10.1007/s00383-019-04512-2.
10. Scanlan KA, Pozniak MA, Fagerholm M et al. Value of transperineal sonography in the assessment of vaginal atresia. AJR Am J Roentgenol 1990; 154 (3): 545–548. doi: 10.2214/ajr.154.3.2106219.
11. Xu S, Zhang J, Wang S et al. MRI features and differential diagnoses of congenital vaginal atresia. Gynecol Endocrinol 2019; 35 (9): 777–781. doi: 10.1080/09513590.2019.1588875.
12. Hořejší J et al. Dětská gynekologie. Praha: Mladá fronta a. s. 2017.
13. Management of acute obstructive uterovaginal anomalies: ACOG committee opinion, number 779. Obstet Gynecol 2019; 133 (6): e363–e371. doi: 10.1097/AOG.0000000000003281.
14. Zhang M, Meng L, Du Y et al. Modified balloon vaginoplasty for high position vaginal atresia. Pediatr Surg Int 2022; 38 (4): 631–635. doi: 10.1007/s00383-022-05078-2.
15. Mansouri R, Dietrich JE. Postoperative course and complications after pull-through vaginoplasty for distal vaginal atresia. J Pediatr Adolesc Gynecol 2015; 28 (6): 433–436. doi: 10.1016/ j.jpag.2014.12.007.
16. Kresowik J, Ryan GL, Austin JC et al. Ultrasound-assisted repair of a unique case of distal vaginal agenesis. Fertil Steril 2007; 87 (4): 976.e9–976.e12. doi: 10.1016/j.fertnstert.2006.06.062.
17. Miller RJ, Breech LL. Surgical correction of vaginal anomalies. Clin Obstet Gynecol 2008; 51 (2): 223–236. doi: 10.1097/GRF.0b013e31816d2181.
18. Ugur MG, Balat O, Ozturk E et al. Pitfalls in diagnosis and management of distal vaginal agenesis: 10-year experience at a single centre. Eur J Obstet Gynecol Reprod Biol 2012; 163 (1): 85–90. doi: 10.1016/j.ejogrb.2012.03.024.
19. Zhang X, Tang X, Ding Y et al. Management of partial vaginal agenesis according to the Wharton-Sheares-George technique – a long- -term follow-up. J Obstet Gynaecol 2020; 40 (5): 715–717. doi: 10.1080/01443615.2019.1655721.
20. Ciftci I. Laparoscopic-assisted perineal pull- -through vaginoplasty. J Pediatr Surg 2012; 47 (4): e13–e15. doi: 10.1016/j.jpedsurg.2011.11.070.
21. Wester T, Tovar JA, Rintala RJ. Vaginal agenesis or distal vaginal atresia associated with anorectal malformations. J Pediatr Surg 2012; 47 (3): 571–576. doi: 10.1016/j.jpedsurg.2011.09.040.
22. Fei YF, Quint EH, Hryhorczuk AL et al. Distal vaginal atresia with spontaneous perforation: a case report. J Pediatr Adolesc Gynecol 2022; 35 (3): 383–386. doi: 10.1016/j.jpag.2021.10.018.
23. Chubanovová N, Chmel R Jr, Kelčík R et al. Hymenální atrezie – vzácná kongenitální anomálie s rizikem pozdního stanovení diagnózy. Ceska Gynekol 2022; 87 (2): 118–123. doi: 10.48095/cccg2022118.
24. Brander EP, Vincent S, McQuillan SK. Transverse vaginal septum resection: technique, timing, and the utility of dilation. A scoping review of the literature. J Pediatr Adolesc Gynecol 2022; 35 (1): 65–72. doi: 10.1016/j.jpag.2021.09.002.
25. Adair L 2nd, Georgiades M, Osborne R et al. Uterus didelphys with unilateral distal vaginal agenesis and ipsilateral renal agenesis: common presentation of an unusual variation. J Radiol Case Rep 2011; 5 (1): 1–8. doi: 10.3941/jrcr.v5i1.572.
26. Tzialidou-Palermo I, von Kaisenberg CS, Garcia-Rocha GJ et al. Diagnostic challenges of hemihematocolpos and dysmenorrhea in adolescents: obstructed hemivagina, didelphys or bicornuate uterus and renal aplasia is a rare female genital malformation. Arch Gynecol Obstet 2012; 286 (3): 785–791. doi: 10.1007/ s00404-012-2392-5.
27. Chmel R Jr, Pastor Z, Mužík M et al. Syndrom Mayer-Rokitansky-Küster-Hauser – ageneze dělohy a pochvy: aktuální znalosti a terapeutické možnosti. Ceska Gynekol 2019; 84 (5): 386–392.
28. Khollová S, Hrdonková E, Pomahačová R. Syndrom úplné androgenní insenzitivity – kazuistika. Ceska Gynekol 2014; 79 (1): 38–42.
Štítky
Paediatric gynaecology Gynaecology and obstetrics Reproduction medicineČlánok vyšiel v časopise
Czech Gynaecology
2022 Číslo 5
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