A rare form of Mayer–Rokitansky–Küster–Hauser syndrome associated with ovarian endometrioma: a case report

Naem, Antoine; Shamandi, Anwar; Al-Kurdy, Bashar

doi:10.1093/jscr/rjaa393

Cited by 4 publications

(4 citation statements)

References 10 publications

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“…Several case reports have reported management of endometriosis in patients with MRKH. All of them underwent removal of the uterine remnants, and most reported complete remission of pain after surgery and no recurrence of endometriosis [1, 11-13, 19, 21-27]. Similarly, all our patients reported uneventful recovery after the operation, and none complained of recurrent pelvic pain or recurrence of endometriosis in the follow-up.…”

Section: Discussionmentioning

confidence: 72%

Clinical Features and Management of Endometriosis among Patients with MRKH and Functional Uterine Remnants

Tian¹,

Chen²,

Liang³

et al. 2021

Gynecol Obstet Invest

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Objective: To determine the clinical features of Mayer–Rokitansky–Küster–Hauser syndrome (MRKH) patients with functional uterine remnants and endometriosis in a large cohort of Chinese patients. Design: This was a retrospective study. Participants: This study had a cohort of 511 MRKH patients. A total of 34 MRKH patients with functional remnant were finally included. Setting: Tertiary referring hospitals in China. Methods: Patients with MRKH diagnosed and treated at Peking Union Medical College Hospital from January 2009 to January 2020 were recruited. A cohort of 511 MRKH patients were retrospectively screened and a total of 34 MRKH patients with functional remnant were finally included. Relevant clinical data were reviewed retrospectively from medical charts. Results: Of 34 patients with MRKH and functional uterine remnants, 23 (68%) had endometriosis. These patients had a greater mean age at MRKH diagnosis than patients without endometriosis (15.9 ± 3.3 years vs 13.2 ± 3.5 years; P=0.03). Similarly, these patients experienced a longer time between age at onset of symptoms and age at operation than patients without endometriosis (45.5 ± 39.6 years vs 19.8 ± 13.2 years; P =0.04). In addition, the CA125 level was significantly higher in patients with endometriosis than in those without it (64.9 ± 85.9 U/ml vs 25.5 ± 19.1 U/ml; P = 0.03). Limitations: The number of patients with MRKH analyzed in this study was low as we restricted inclusion to patients with at least one functional uterine remnant or endometriosis. Conclusions: It is reasonable to monitor the uterine remnant of patients with MRKH closely, regardless of age, to achieve early intervention. The level of CA125 might be helpful to differentiate active uterine remnants with endometriosis and schedule individualized treatments.

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Section: Discussionmentioning

confidence: 72%

Clinical Features and Management of Endometriosis among Patients with MRKH and Functional Uterine Remnants

Tian¹,

Chen²,

Liang³

et al. 2021

Gynecol Obstet Invest

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“…In addition, pelvic anatomy can be altered by the presence of rudimentary horn. Indeed, 15% of cases we reviewed had associated renal system anomalies (renal agenesis or ectopic kidney), and 20.45% of cases were associated with endometriosis, which could explain the presence of adhesions as documented in some cases [3,[32][33][34][35][36].…”

Section: Discussionmentioning

confidence: 99%

“…Noncommunicating cavitated rudimentary horns are usually associated with pelvic pain (70%), hematometra (50%), endometriosis (20-40%) [1]. In patients with Mayer-Rukitansky-Kuster-Hauser Syndrome is it possible to have rudimentary uterine horns coexisting with a unicornuate uterus, as two entities completely separated from each other and from the vaginal vault [3].…”

Section: Introductionmentioning

confidence: 99%

Laparoscopic Management of Rudimentary Uterine Horns in Patients with Unicornuate Uterus: A Systematic Review

Abboud

Giannini

D’Oria

et al. 2022

Gynecol Obstet Invest

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Introduction: Unicornuate uterus is a rare Müllerian anomaly. Its potential association with a rudimentary uterine horn can cause a diagnostic delay. The most common consequences are pelvic pain, hematometra and endometriosis. Diagnosis of a unicornuate uterus is usually done by imaging combining ultrasound (US) and Magnetic Resonance Imaging (MRI). The aim of this systematic review is to assess the role of laparoscopic approach in the management of this rare condition. Methods: A comprehensive search was performed in PubMed, EMBASE, SCOPUS and Web of Science databases prior to 1 may, 2022 according to Preferred Reporting Items for Systematic Reviews and Meta- Analyses statement (PRISMA). The inclusion criteria were: cases of rudimentary horn managed through laparoscopy only; laparoscopic treatment of communicating or non-communicating uterine horn. Results: The search strategy identified 45 articles. After this first screening, 37 studies were evaluated. The full text of remaining articles was examined. 35 studies were finally included in this article. All included studies were case reports, due to the rarity of this condition. Rudimentary horns were non-communicating in all cases. Conclusion: The laparoscopic removal of a rudimentary uterine horn could be considered a feasible therapeutic option. An accurate preoperative evaluation is mandatory to assess anatomic variants and to select the optimal and tailored surgical approach, based also on the symptoms complained by the patient.

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“…In particular, rare cases of vaginal atresia may require by some gynaecologists the vaginal vault intact. In these cases, colpotomy should be avoided [ 15 ]. Finally, attention should be given to the appropriate administration of new techniques.…”

Section: Discussionmentioning

confidence: 99%

Hymen conservative techniques for vaginal surgery – a practical approach

Sleiman¹,

Ayed²,

Christoforou³

et al. 2022

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Vaginal approach remains one of the safest and easiest surgical procedures to treat many gynaecological pathologies such as cervico-vaginal fibroids or vaginal septum.These classical procedures are, however, hindered by the disruption of the integrity of the hymen, an issue that remains controversial if not unfavourable in certain conservative geographical areas such as the Middle East.Herein, we describe 3 alternative surgical laparoscopic techniques to deal with the issue of hymen conservation. In the first 2 cases we suggest a laparoscopic approach through an anterior or a posterior colpotomy to treat large cervico-vaginal fibroids. In the third case, we propose a laparoscopic technique for the resection of a vaginal septum in a young girl with obstructed hemivagina and ipsilateral renal agenesis syndrome.

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A rare form of Mayer–Rokitansky–Küster–Hauser syndrome associated with ovarian endometrioma: a case report

Cited by 4 publications

References 10 publications

Clinical Features and Management of Endometriosis among Patients with MRKH and Functional Uterine Remnants

Clinical Features and Management of Endometriosis among Patients with MRKH and Functional Uterine Remnants

Laparoscopic Management of Rudimentary Uterine Horns in Patients with Unicornuate Uterus: A Systematic Review

Hymen conservative techniques for vaginal surgery – a practical approach

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