Congenital Obstructive Müllerian Anomaly: The Pitfalls of a Magnetic Resonance Imaging-Based Diagnosis and the Importance of Intraoperative Biopsy

Kim, Do Young; Nam, Gina; Lee, Sa Ra; Kim, Sung Hoon; Chae, Hee Dong; Kang, Byung Moon

doi:10.3390/jcm10112414

Cited by 4 publications

(7 citation statements)

References 38 publications

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“…The benefits of TMV vaginoplasty are preserved in MVLESS and streamlined. Patients who underwent the novel procedure achieved 100% anatomic success and functional efficacy, which was consistent with prior research findings ( 23 ). Patients with vaginal agenesis also had near-normal sexual function and satisfactory sexual experiences after treatment according to the postoperative FSFI evaluations.…”

Section: Discussionsupporting

confidence: 88%

Retrospective comparative cohort study of neovagina creation by modified Vecchietti-laparoendoscopic single-site surgery for Mayer-Rokitansky-Küster-Hauser syndrome

Zhang¹,

Zhang²,

Wang³

et al. 2022

Ann Transl Med

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Background: Although traditional multi-incision laparoscopic surgery is minimally invasive, it can still leave surgical scars on the lower abdomen. This study reports a modified Vecchietti-laparoendoscopic singlesite (MVLESS) procedure using self-made instruments to create a neovagina for Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS) and to determine the efficacy and safety.Methods: We conducted a retrospective comparative cohort study analysis of the clinical data from MRKHS patients treated in the Department of Difficult Gynecological Disease at Xi'an People's Hospital between January 2010 and December 2020.Women who were diagnosed with MRKHS and underwent a MVLESS procedure using self-made instruments to create a new vagina were selected as the study group (n=14). Patients who underwent a traditional multi-incision Vecchietti (TMV) procedure were selected as the control group (n=22). All participants in both groups were required to complete a Female Sexual Function Index (FSFI) and Female Genital Self-Image Scale (FGSIS) questionnaire.Results: All patients in the study group were diagnosed with type I MRKHS and 3 (21.43%) were sexually active. Twenty-one patients in the control group were diagnosed with type I (95.45%) MRKHS, 1 (4.55%) had type II MRKHS (absent left kidney), and 6 (27.27%) were sexually active. The neovaginas in both groups were wide with good elasticity, softness, smoothness, and lubrication. There were no significant differences in operative time (37±6 vs. 39±4 minutes, P=0.98), intraoperative bleeding (28.32±9.82 vs. 29.45±3.84 mL, P=0.86), postoperative anal exsufflation time (18±4 vs. 20±4 hours, P=0.82), and postoperative hospital stay (7±2 vs. 8±2 days, P=0.84) between the MVLESS and TMV groups. The FSFI and FGSIS scores in both groups showed participants had a good quality of sexual life based on the 12-month follow-up data; however,function was significantly better in the study group than in the control group [lubrication (4.72±0.86 vs. 4.64±1.01, P=0.023), visual analog scale (VAS; 4.26±0.52 vs. 4.45±0.39, P=0.041), total FSFI scores (30.21±4.32 vs. 28.42±2.21, P=0.048), and FGSIS score (23.21±1.98 vs. 22.14±2.04, P=0.012)]. Conclusions:The MVLESS procedure is a simple, safe, and minimally invasive procedure that provides an anatomical and functional neovagina for MRKHS patients. It is an alternative method for creating a neovagina to achieve satisfactory anatomic and sexual function. However, it is still a difficult problem to solve fertility problems for patients with congenital absence of vagina.

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Section: Discussionsupporting

confidence: 88%

Retrospective comparative cohort study of neovagina creation by modified Vecchietti-laparoendoscopic single-site surgery for Mayer-Rokitansky-Küster-Hauser syndrome

Zhang¹,

Zhang²,

Wang³

et al. 2022

Ann Transl Med

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“…MRI usually shows the exact obstruction sites, providing detailed information for surgical strategy planning [ 5 ]. However, considering the results of our previous study, an intraoperative biopsy at the caudal leading edge is recommended for obstructive genital anomalies, particularly for presumptive vaginal or cervical dysgenesis [ 14 ].…”

Section: Discussionmentioning

confidence: 99%

A New Case of Herlyn–Werner–Wunderlich Syndrome: Uterine Didelphys with Unilateral Cervical Dysgenesis, Vaginal Agenesis, Cervical Distal Ureteral Remnant Fistula, Ureterocele, and Renal Agenesis in a Patient with Contralateral Multicystic Dysplastic Kidney

Lee

Choi

et al. 2021

Diagnostics

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The aim of this study was to present a new case of congenital Herlyn–Werner–Wunderlich syndrome, a rare anomaly of the female reproductive tract, and review the related literature. A 12-year-old girl presented with severe dysmenorrhea since menarche and magnetic resonance imaging showing a bicornuate uterus, double cervix, right hematometra, and hematosalpinx with ipsilateral renal agenesis, accompanied by a remnant distal ureter with hydroureter. A diagnostic cystoscopy and a reduced-port robot-assisted laparoscopy with chromopertubation were performed in order to identify the anomaly. Uterine didelphys and right cervical dysgenesis with ipsilateral vaginal agenesis, cervical distal ureteral remnant fistula, ureterocele, and renal agenesis were diagnosed on the basis of histopathologic findings, and she subsequently underwent a robotic unilateral right total hysterectomy with salpingectomy. This case report reinforces the importance of the intraoperative biopsy for an accurate diagnosis, despite magnetic resonance imaging being considered the gold-standard diagnostic tool.

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“…Esto hace que sea de gran utilidad para la planificación quirúrgica de todas las anomalías uterinas, incluido el MRKH. (48,49,50)…”

Section: Exámenes Complementarios De Imágenesunclassified

Síndrome de Mayer-Rokitansky-Küster-Hauser

Pacheco Coello,

Bajaña Jiménez,

Zúñiga Fuentes

et al. 2023

Afecciones Infrecuentes en La Práctica Quirúrgica

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El síndromede Mayer-Rokitansky-Küster-Hauser (MRKH), denominado también aplasia mülleriana, es un trastorno caracterizado por agenesiadel útero y de la regiónsuperior dela vagina enpacientes femeninascon características sexuales secundarias normales y un cariotipo femenino normal (46XX). Tiene una prevalencia estimada de 1 en 5000 mujeres nacidas vivas. Suele dignosticarse durante la adolescencia, al estudiarse a las pacientesporamenorrea primaria. Se clasifica como aplasia útero-vaginalaislada tipo I o,asociado a manifestaciones extragenitales,tipo II. Las anomalías extragenitalessuelen incluir anomalías renales, esqueléticas, del oído, o malformaciones cardíacas. Aún hoy se desconoce su etiología exacta, lo cual se debe al nivel de complejidad de las vías genéticas implicadas durante el desarrollo embriogenético de los conductos müllerianos.La ecografía y la resonancia magnética constituyen los medios diagnósticos fundamentales más usados. El tratamiento para la agenesia vaginal se enfoca en procederes encaminados a la creación de una neovagina, ya sea con dilataciones vaginales no invasivas, las cuales se recomiendan como terapia de primera línea, o por cirugía.Este síndromeimplica infertilidad por factor uterino absoluto, cuyas opciones terapéuticas principales son la fertilización in vitro,utilizando ovocitos autólogos y unaportadora gestacional sustituta, así como el trasplante de útero para lograr la maternidad biológica.El impacto psicosexual que implica este síndromerequiere de asesoramiento psicológico continuo y educación terapéutica

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Congenital Obstructive Müllerian Anomaly: The Pitfalls of a Magnetic Resonance Imaging-Based Diagnosis and the Importance of Intraoperative Biopsy

Cited by 4 publications

References 38 publications

Retrospective comparative cohort study of neovagina creation by modified Vecchietti-laparoendoscopic single-site surgery for Mayer-Rokitansky-Küster-Hauser syndrome

Retrospective comparative cohort study of neovagina creation by modified Vecchietti-laparoendoscopic single-site surgery for Mayer-Rokitansky-Küster-Hauser syndrome

A New Case of Herlyn–Werner–Wunderlich Syndrome: Uterine Didelphys with Unilateral Cervical Dysgenesis, Vaginal Agenesis, Cervical Distal Ureteral Remnant Fistula, Ureterocele, and Renal Agenesis in a Patient with Contralateral Multicystic Dysplastic Kidney

Síndrome de Mayer-Rokitansky-Küster-Hauser

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