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Background:
Systematic Review

Endometriosis Coinciding with Uterus Didelphys and Renal Agenesis: A Literature Review

by
Davut Dayan
1,*,
Florian Ebner
1,2,
Wolfgang Janni
1,
Katharina Hancke
1,
Duygu Adiyaman
1,
Beate Huener
1,
Michelle Hensel
1,
Andreas Daniel Hartkopf
1,3,
Marinus Schmid
1 and
Stefan Lukac
1
1
Klinik für Frauenheilkunde und Geburtshilfe, Universitätsklinikum Ulm, 89075 Ulm, Germany
2
Abteilung für Frauenheilkunde und Geburtshilfe, Alb-Donau Klinikum Ehingen, 89584 Ehingen (Donau), Germany
3
Department für Frauengesundheit, Universitäts-Frauenklinik Tübingen, 72076 Tübingen, Germany
*
Author to whom correspondence should be addressed.
J. Clin. Med. 2024, 13(24), 7530; https://doi.org/10.3390/jcm13247530
Submission received: 2 October 2024 / Revised: 15 November 2024 / Accepted: 7 December 2024 / Published: 11 December 2024
(This article belongs to the Section Obstetrics & Gynecology)
Browse Figure
Versions Notes

Abstract

:
Background/Objectives: Endometriosis and urogenital malformation with uterus didelphys and renal agenesis might occur concomitantly, and the question arises whether both entities are associated with each other. Methods: A literature search was conducted in PubMed and Web of Science, using the following search terms: “endometriosis and uterine malformation, endometriosis and Herlyn–Werner–Wunderlich syndrome”, “endometriosis and OHVIRA (Obstructed Hemivagina and Ipsilateral Renal Anomaly) syndrome” and “uterus didelphys, renal agenesis and endometriosis”. Results: We identified and examined 36 studies, comprising a total of 563 cases with coinciding endometriosis and OHVIRA. The most prevalent symptoms were dysmenorrhea and lower abdominal pain. Renal agenesis occurred more frequently on the right side. In the majority of cases, vaginal septum resection was performed to alleviate hematometrocolpos. Among the 97 cases necessitating abdominal exploration, endometriosis was identified in 61 patients (62.9%), although this figure is most likely an overestimation. However, a significantly heightened risk of endometriosis was evident. Conclusions: This literature review highlights the importance of considering the potential for urogenital malformation and endometriosis in cases of dysmenorrhea during adolescence. Ultrasound examination has proven to be a valuable diagnostic tool for identifying uterine abnormalities and guiding subsequent diagnostic and, if necessary, surgical interventions. Thorough assessment and appropriate management are imperative to mitigating the long-term consequences associated with deep infiltrating endometriosis.

1. Background

Endometriosis is a benign, chronic condition characterized by the development of endometrial-like lesions outside the uterine cavity. Endometriosis occurs in approximately 10% of women of reproductive age and can significantly impair quality of life and fertility [1,2,3]. Endometriosis risk factors include genetic, immunological and environmental factors [4], but the etiology has not yet been conclusively determined. A widely accepted cause is Sampson’s retrograde menstruation theory, proposed more than 100 years ago [5]. This phenomenon is more prevalent in the presence of obstructive uterine anomalies. Several studies have established a correlation between such anomalies and the development of endometriosis [6,7,8,9,10]. While retrograde menstruation could account for the pathogenesis of endometriosis, additional evidence suggests that immunological or genetic factors might also contribute [11,12]. To date, the prevalence of endometriosis in adolescents remains uncertain. In a retrospective study, conducted by Haas et al., involving 42,079 histologically confirmed endometriosis cases, 0.05% were patients aged 10 to 15 years and 1.93% patients aged 15 to 20 years. Notably, the proportion of patients increased in higher age groups, with 6.11% of cases referring to patients aged 20 to 25 years and 78.37% to patients aged 20 to 45 years [13]. In line with this study, Eisenberg et al. observed a 0.8% endometriosis rate in adolescents between 15 and 19 years in their retrospective analysis of 2 million patients [14].
A rare Müllerian duct anomaly is the congenital Obstructed Hemivagina and Ipsilateral Renal Anomaly, first described in 1922. This condition is known as Herlyn–Werner–Wunderlich syndrome (HWWS), named after the authors who reported the initial cases between 1971 and 1976, or as spacing OHVIRA syndrome—an acronym that summarizes the main features of the condition. These patients show uterus didelphys, ipsilateral vaginal obstruction and ipsilateral renal agenesis [15,16,17]. The syndrome arises from aberrant development of the paramesonephric (Müllerian) and mesonephric (Wolffian) ducts [15]. Anomalies stem from disruptions in the formation, canalization, fusion or absorption of the Müllerian ducts during the 8th to 12th week of embryonic development [18,19,20]. Although the precise prevalence remains uncertain, current estimates range from 0.1% to 3.8% [16]. The severity of these anomalies varies and depends on the extent of the development of the Müllerian system. The standardized terminology of the American Society for Reproductive Medicine (ASRM) classification is used to precisely identify each anomaly and to simplify the communication of these anomalies [21]. One of the most important symptoms is cyclical lower abdominal pain after menarche and, in the case of vaginal obstruction, pelvic distension [17,20,22,23].
In a recent literature review on HWWS, Liu et al. assessed 1673 HWWS patients presenting with symptoms and anomalies [24]. The most common symptoms were dysmenorrhea (53.8%), abnormal uterine bleeding (28.9%) and vaginal discharge (26.6%). Frequently observed anomalies included a right-obstructed hemivagina (57.3%), hematocolpos (81.7%), uterus didelphys (88.8%) and ipsilateral renal agenesis (93.1%). Vaginal septum excision was the primary treatment (91.8%), with minimally invasive surgery performed in 48.5% of cases, often following vaginal surgery (61.9%). Endometriosis was identified in 9.6% of these patients, with 52% involving ipsilateral ovarian endometriotic cysts.
Endometriosis is not universally more common in patients with Müllerian anomalies, but it is more frequently seen in those with outflow obstructions, such as hematosalpinx, hematometra or hematocolpos. An association between OHVIRA and deep infiltrating endometriosis (DIE) has been reported [20,23,25]. In a recent case report by Choridah and Pangastuti, an adolescent patient with cyclic pain and an endometriotic cyst on one side, accompanied by hemivaginal obstruction and several endometriotic nodules on the peritoneum and diaphragm, presented and didelphys was diagnosed by MRI [26]. In another recent case series, Takahashi et al. analyzed 12 adolescent patients who had primary surgery for obstructive Müllerian anomalies and 31 patients in the same age group who underwent surgery for ovarian endometrioma. Of the patients with obstructive Müllerian anomalies, including four cases of Herlyn–Werner–Wunderlich syndrome, two cases with a non-communicating functional uterine horn and two cases of cervical aplasia, 50% developed endometriosis [27].
Some studies have correlated the presence of Müllerian duct malformations with the incidence of severe endometriosis [23,28]. An increased risk of endometriosis in uterine malformation has not yet been conclusively determined; however, it is theorized that obstruction of the genital tract results in increased retrograde menstruation, thereby leading to the development of endometriosis [8]. Another possible explanation is that a family history of these two conditions predisposes individuals who suffer from one to the occurrence of the other [29]. Although the link between pelvic endometriosis and HWWS is still not well understood, Sampson’s theory of retrograde menstruation and implantation is commonly accepted but does not account for all cases of pelvic endometriosis. Detecting an association between endometriosis and OHVIRA, alongside identifying overlapping symptoms, might help with the early identification of patients at particular risk of impaired fertility and diminished quality of life and the selection of corresponding, therapeutic options.
Here we review the literature on previous reports of both entities coinciding in adolescent patients. The available data highlight the need for the timely diagnosis of uterine malformations possibly associated with endometriosis in adolescent patients with cyclical abdominal pain.

2. Methods

A systematic literature review was conducted to assess the available literature on endometriosis co-occurring with uterine malformations in general and OHVIRA in particular. The PubMed and Web of Science databases were searched for relevant publications, using the following search terms: “endometriosis AND uterine malformation”, “endometriosis AND Herlyn–Werner–Wunderlich syndrome”, “endometriosis AND OHVIRA (Obstructed Hemivagina and Ipsilateral Renal Anomaly) syndrome” and “uterus didelphys, renal agenesis AND endometriosis”. The search was conducted without limits and adhered to the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines [30]. This systematic review was registered in the Prospero database, under the registration number CRD42023462124. Two independent reviewers evaluated the suitability of papers for inclusion in the systematic review. Exclusion criteria encompassed papers not written in English, video material, conference abstracts, editorial letters, reviews and studies lacking complete or original data.
A total of 664 studies were identified using the search term “endometriosis and uterine malformation”. However, many of these studies did not pertain to OHVIRA, and so subsequent search terms were specified. The study selection process using these search terms is shown in Figure 1. When narrowing the search to “endometriosis and Herlyn–Werner–Wunderlich syndrome”, only 24 studies were retrieved. Similarly, using the terms “endometriosis and OHVIRA syndrome”, 20 studies were identified, while the search term “uterus didelphys, renal agenesis and endometriosis” yielded 30 studies. Therefore, 74 publications were identified in the initial search in both databases using the three different search term combinations. A comparison of the search results from both databases revealed 28 duplicates (n = 10 studies on OHVIRA and n = 18 studies on OHVIRA and uterus didelphys, renal agenesis and endometriosis) that were excluded, and the full texts of the remaining 46 studies were screened for eligibility. In this step, a further ten studies were removed based on the inclusion and exclusion criteria (language: n = 3, video material: n = 2, review: n = 4, letter to the editor: n = 1). Therefore, 36 studies were included in the final analysis. The included studies were assessed for the following parameters: existing cases of uterine malformations associated with renal agenesis, age distribution at menarche, diagnostic methods (visual inspection, biopsy), patient symptomatology, distribution of malformations and types of surgical treatment administered. The studies were further examined to determine whether the authors performed a diagnostic laparoscopy, cystectomy, adhesiolysis, hysterectomy, vaginoplasty or surgical examination to diagnose and/or treat endometriosis. A focused investigation was specifically conducted on cases involving renal agenesis and uterine malformation to determine whether any abdominal interventions were performed and to explore any potential associations with endometriosis.

3. Results

The initial literature search with a broader search term combination (“endometriosis and urogenital malformation”) yielded many unspecific and irrelevant results, so we decided to narrow the search to a particular urogenital anomaly in the context of endometriosis, OHVIRA. The results presented here, therefore, pertain to adolescent patients with OHVIRA or a similar anomaly and endometriosis.
The findings of the 36 finally included studies are presented in Table 1. These studies entailed a total of n = 563 cases. Of the 36 studies, 21 reported a single case [31,32,33,34,35,36,37,38,39,40,41,42,43,44,45,46,47,48,49,50,51], 1 study reported two cases [52], 1 study reported four cases [53], 1 study reported five cases [54] and 12 studies presented ten or more cases [15,23,25,55,56,57,58,59,60,61,62,63]. The majority of patients were in their adolescence. The most prevalent symptoms included dysmenorrhea, vague lower abdominal pain and foul-smelling vaginal discharge after menstrual periods.
Details about abdominal exploration via laparoscopy or laparotomy were available for 97 cases, as abdominal intervention was unnecessary in most instances. Information regarding ipsilateral renal agenesis (IRA) was available for n = 275 cases. IRA occurred on the right side in n = 168 cases (61%), on the left side in n = 107 cases (39%), and no specific side was indicated for the remaining cases. Most cases exhibited congenital malformations in the development of the Wolffian and Müllerian ducts, characteristic of the triad consisting of obstructed hemivagina, ipsilateral renal anomaly and uterus didelphys [64]. Information about endometriosis involvement was found in 20 studies [23,25,31,32,33,36,43,44,46,47,52,53,54,55,56,57,58,59,60,61].
Effective treatment involved vaginal septoplasty for the symptom management of imperforate vagina. Among these 97 cases, endometriosis was identified in 61 (62.9%), compared to a rate of approximately 10% in women of reproductive age. Studies with larger case numbers, such as Acién P. et al., demonstrated a 20% endometriosis rate in 60 cases with bicornuate/didelphys uterus [55]; Tong J. et al. reported an endometriosis rate of 17.1% (n = 12) in n = 70 cases, with abdominal exploration mentioned in only 9 cases [60]. One year later, Tong et al. observed an endometriosis rate of 19.15% (18/94) in patients with OHVIRA, where patients with complete hemivaginal obstruction exhibited a higher rate of 37.0% (10/27) compared to those with incomplete obstructions at 11.9% (8/67) [23]. Another study on this malformation was conducted by Fedele et al. In their case series from 1981 to 2011, they examined 87 female patients and described the various anatomical variants of uterus didelphys with unilateral cervico-vaginal obstruction and ipsilateral renal anomalies and highlighted the significant clinical and surgical implications of these malformations. They reported a higher incidence of unilateral obstruction on the right side: right-sided in 53 of 87 cases (60.9%) and left-sided in 34 of 87 cases (39.1%). All patients underwent diagnostic laparoscopy to assess the uterine morphology and confirm the external uterine profile. Although the main aim of their study was not to investigate the association between HWWS and endometriosis, endometriosis was diagnosed in 12 of the 87 patients (13.8%), in 10 cases on the peritoneum, in 5 cases on the ovary and in 1 case each on the fallopian tube and diaphragm [65].
The studies assessed in the present review show a frequent association between genitourinary malformation and endometriosis [6,8]. In their case series on urogenital malformations, Moon et al. report the outcomes of five patients, averaging 16.6 years of age (range 13–27). Among the subset of four patients requiring laparoscopic intervention due to these malformations, three exhibited evidence of endometriosis [54].
In their study involving 60 patients with a mean age of 26.6 years (range 11–62), Acién et al. reported an endometriosis rate of 20% [55]. In another paper, Tong et al. documented an endometriosis rate of 17.1% in patients with a mean age of 21.39 years (range 10–50), with abdominal exploration mentioned in only 12.9% of cases [60]. One year later, Tong et al. observed that the diagnosis of endometriosis occurred at a mean age of 17.8 years, while OHVIRA was diagnosed at 20.5 years. They identified an endometriosis rate of 19.15% in patients with OHVIRA, noting a higher rate of 37.0% in those with complete hemivaginal obstruction, compared to those with incomplete obstruction (11.9%) [23]. It should be noted that, in these three studies, it is not clear whether all patients underwent abdominal exploration as a means of assessing for endometriosis [23,55,60]. The majority of the 563 patients included in this review found transvaginal septum resection and vaginoplasty to be a sufficient treatment, with only 97 cases requiring laparoscopic or laparotomic intervention. Among the 97 patients requiring abdominal exploration, endometriosis was diagnosed in 61 (62.9%).

4. Discussion

OHVIRA describes the association of uterus didelphys with ipsilateral vaginal obstruction and ipsilateral renal agenesis [22]. Renal agenesis is present in 30% of cases of uterine didelphys and is the most common anomaly [20]. Renal agenesis often occurs on the right side. Several studies suggest a prevalence of right renal agenesis in 66–75% of cases [22,66,67]. In our review, we also observed a higher prevalence of renal agenesis on the right side.
The results of our literature review revealed that rare urogenital malformations are increasingly being diagnosed prenatally or in early childhood, partly owing to advancements in ultrasound imaging quality and sensitivity, enhanced expertise in examinations and the increasing use of ultrasound in both prenatal diagnostics and routine clinical assessments [63]. Affected individuals typically remain asymptomatic prior to menarche [15,68]. In their review, Lecka-Ambroziak et al. also emphasize the necessity of screening for congenital anomalies of the genital tract in patients diagnosed with renal anomalies and vice versa, to recognize the consequences of these malformations and potentially avoid emergency surgeries [17]. Symptoms, such as progressive lower abdominal pain, dysmenorrhea, hematocolpos, pelvic mass and, in some cases, abnormal vaginal discharge, vomiting, fever, acute urinary retention or infection manifest only after menarche [15,69,70]. Surgical intervention is generally recommended after menarche, aiming to treat symptoms, alleviate obstructions, prevent complications and preserve fertility. The standard surgical procedure involves a transvaginal one-stage drainage of the hematocolpos/hematometrocolpos, vaginal septum resection and vaginoplasty (a restoration of the continuity of the vaginal mucosal wall to minimize the risk of postoperative stenosis and adhesions). Abdominal exploration in OHVIRA syndrome is reserved for select cases, such as for defining the anatomy more precisely, confirming the presence of endometriosis, treating a residual ureter or Gartner’s cyst or addressing symptoms resistant to therapy and findings requiring further clarification [15].
The cause of increased endometriosis risk in cases of urogenital malformations is still unclear, and while retrograde menstruation might certainly play a role, genetic, immunological and environmental factors likely contribute. These factors might also explain the occurrence of urogenital malformations. The concomitant occurrence of both entities could be explained by the fact that the anatomical and physiological changes associated with uterine malformations cause an impairment of uterine peristalsis, resulting in the occurrence of more frequent subendometrial myometrial contraction waves than in healthy individuals [71,72]. Due to the obstruction and/or stenosis of the cervical canal, this could cause increased resistance to the outflow of blood through the cervical canal and thus to increased retrograde menstruation, which, in turn, leads to the development of endometriosis [8,73,74]. Genetic predisposition is also a risk factor for endometriosis [75,76,77]. There is a possibility that genetic factors acting together in both conditions might provide an additional plausible explanation for the association between them.
One key problem in the diagnosis of endometriosis is the time delay between the first symptoms to histopathological confirmation. Sonography is the gold standard for evaluating the pelvic organs and is an excellent tool in the diagnosis of genitourinary malformation [20]. If vaginal sonography is refused or not feasible for any reason, transrectal sonography might be a feasible alternative, with very good diagnostic value. Renal sonography is an important standard examination to monitor the kidney or, in rare cases, to evaluate renal agenesis. If uterine didelphys and renal agenesis are present, an MRI of the pelvic abdomen should be performed for radiological assessment, differential diagnosis and the exclusion of other malformations. Renal scintigraphy to exclude a functional abnormality in the existing kidney and ureter is also required [20,25]. In therapy-resistant symptomatic adolescent patients with a clinically identified genitourinary malformation, a laparoscopic examination could be performed to detect the malformation of the uterus and to exclude endometriosis and pelvic adhesions that cannot be visualized via imaging [78]. Based on the majority of studies identified in this review, reporting single cases only, a clinical recommendation on whether or not to perform laparoscopy cannot be deduced.
The early diagnosis and appropriate treatment of endometriosis can prevent further destruction of the infiltrated organs and normal anatomy. Involvement of the ureter in endometriosis is a rare but serious condition that can lead to significant health problems. When the ureter is strangulated by endometriosis, hydronephrosis and ultimately renal failure might occur [79,80,81,82]. This is especially important in renal agenesis, as patients have only one kidney, and renal loss would be life-threatening. The appropriate symptomatology through guided clinical examination enables timely diagnosis and the appropriate, correct planning of further treatment. Laparoscopic repair of symptomatic endometriosis significantly improves quality of life and enables the avoidance of endometriosis-related complications and late sequelae.

Limitations

As abdominal exploration was not required in all cases, and our analysis was limited to those cases where practitioners deemed abdominal exploration necessary and subsequently performed surgery, the reported prevalence might be overestimated. Despite this, our findings align with the existing literature, which still implies a significant association, indicating an increased risk of endometriosis in cases of urogenital malformations [6,23,25,55,56,57,60]. Another limitation of this study is the fact that our search study might have been biased for cases with concomitant endometriosis and urogenital malformations, potentially resulting in an overestimation of endometriosis prevalence in the targeted population. Lastly, the rarity of HWWS and endometriosis occurring concomitantly limits the amount of controlled studies with large patient volumes and, therefore, many findings, and the conclusions are based on case reports and case series.

5. Conclusions

Endometriosis appears to occur earlier in adolescents with concomitant HWWS, and this points to the need for a timely and specific evaluation of patients presenting with such anomalies. Adolescents with severe dysmenorrhea should be evaluated for urogenital malformation and endometriosis. Sonography provides a good diagnostic tool to plan further diagnostic and surgical treatment to avoid the late effects of DIE.

Author Contributions

Conceptualization, D.D.; methodology, D.D. and S.L.; validation, F.E., W.J., K.H. and A.D.H.; formal analysis, K.H. and M.S.; resources, D.D. and S.L.; data curation, D.D. and S.L.; writing—original draft preparation, D.D.; writing—review and editing, F.E., W.J., K.H., D.A., B.H., M.H., A.D.H., M.S. and S.L.; visualization, D.D. and S.L.; supervision, F.E., W.J., K.H., D.A., B.H., M.H., A.D.H., M.S. and S.L.; project administration, D.D. All authors have read and agreed to the published version of the manuscript.

Funding

This research received no external funding.

Institutional Review Board Statement

This systematic review was registered in the Prospero database under the registration number CRD42023462124, approval date 20 September 2023.

Informed Consent Statement

Informed consent is not applicable to this study.

Data Availability Statement

Publicly available datasets were analyzed in this study.

Acknowledgments

I would like to express my sincere thanks to Rachael Seitz, a native English speaker, for her time and effort in correcting my wording and improving the manuscript.

Conflicts of Interest

The authors declare no conflicts of interest.

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Jcm 13 07530 g001
Figure 1. Flowchart of study identification, screening, eligibility and inclusion.
Figure 1. Flowchart of study identification, screening, eligibility and inclusion.
Jcm 13 07530 g001
Table 1. Studies on adolescent patients with urogenital malformations and endometriosis.
Table 1. Studies on adolescent patients with urogenital malformations and endometriosis.
StudyCountryPatientsSymptomsAge at Diagnosis in YearsAge at Menarche in Years, Menstrual CycleRenal Agenesis Right (R)/Left (L)MalformationTreatmentAbdominal Exploration No (N), Yes (Y), Not Available (NA)Endometriosis
Studies with ≥50 patients
Kapczuk K. et al., 2023 [57]Poland5015/50 anomalies associated with cryptomenorrhea, and 35/50 were menstruating13.5 (range 11.1–18.5)2 girls detected in childhood27 (15R/12L)27/50 IRA; 22/27 UD, OHV; 1/27 UD with unilateral cervical atresia; 4/27 unicornuate uterus with RUH 17/50 only VSR (9/17 with IRA); 33/50 AE with LSC or laparotomy (18/33 with IRA)33/50 Y24/33 endometriosis (15/18 with IRA)
Acién P. et al., 2010 [55]Spain60pelvic pain/dysmenorrhea, metrorrhagia26.6 (range 11–62)NA60 (32R/28L)60 IRA; 10 (8R/2L) UD; 8 (5R/3L) bicornis–unicollis uterus; 27 (15R/12L) bicornis–bicollis uterus; 10 (2R/8L) unicornuate uterusHSG and/or LSCNABicornuate/didelphys uterus 20% Endometriosis
Tong J. et al., 2013 [60]China7045/70 (64.3%) dysmenorrhea; 28/70 (40.0%) intermittent mucopurulent discharge; 18/70 (25.7%) metromenorrhagia21.39 (range 10–50)NA70 (42R/28L)70 IRA, UD, OHV (20/70 complete obstruction with HC; 50/70 incomplete obstruction)all patients VSR; 9/70 (12.9%) underwent AE via laparotomy or laparoscopy9/70 YEndometriosis was observed in 12 (17.1%) patients who underwent AE
Tong J. et al., 2014 [23]China94NAendometriosis 17.8 (range 13–32)
HWWS 20.5 (range 13–37)		NANANANA Pelvic endometriosis in 18/94 (19.15%) of patients with HWWS (patients with complete hemivaginal obstruction 10/27 (37.0%) higher than in those with incomplete obstructions 8/67 (11.9%))
Fei F.Y., 2022 [63]USA125abdominal pain and/or dysmenorrhea14.5 ± 6.5 (r 0–32)12.3 ± 1.6 (r 9–19)NAVS 41.6% (52/125), including 39/52 with oblique septum or obstructed hemivagina–ipsilateral renal agenesis (OHVIRA)45 VSRNNA
Case series with ≤28 patients
Güdücü N. et al., 2012 [52]Turkey2Pat I: dysmenorrhea, Pat. II: foul-smelling vaginal discharge after her menstrual periods13, 21Pat. I: 12; regular Pat. II: NA2 RPat. I + II: UD, right OHV with HC, left uterine cavity, cervix and vagina normalPat. I: laparotomy, hemi-hysterectomy with salpingectomy. Par. II: VSRPat I:Y/Pat. II:NPat. I: DIE endometriosis, Pat. II: AE not performed
Kimble R. et al., 2009 [53]Austalia4dysmenorrhea, abdominal pain14 (range 12–17)11,7 (range 10–14)4 (3R/1L)4 IRA, UD, OHVLSCY2 endometriosis, 2 no endometriosis
Moon L.M. et al., 2023 [54]USA5dysmenorrhea (80%), irregular bleeding (40%), acute onset left lower quadrant pain (20%), and abdominal mass (20%)16.6 (r 13–27)12 (r 11–13)5 (1R/4L)5 IRA, UD, (3 left uterine horn distended with HM and HS, 3 left cervico-vaginal agenesis, 1 left cervical dysgenesis, 1 right cervical dysgenesis)3/5 LSC (2, robotic) uterine horn resection,
1/5 Cervico-vaginal anastomosis + LSC ovarian cystectomy
1/5 cervico-vaginal anastomosis		4Y, 1N3 endometriosis
Kudela G. et al., 2021 [58] Poland109/10 (90%) lower abdominal pain; 1/10 asymptomatic13.35 (range 11–15)mean 12.55 10 (7R/3L)10 IRA, UD, OHV1/10 LSC hemihysterectomy and vaginectomy; 1/10 LSC conversion to laparotomy hemihysterectomy, vaginectomy and salpingectomy; 8/10 only VSR2/10 Y1 endometriosis, 1 no endometriosis
Sabdia S. et al., 2014 [59]Austalia10dysmenorrhea12 (range 10–14)NA10 (7R/3L)NA8 VSR, 1 vs. excision, 2 patients with cervical aplasia geht laparoscopic hemi-hysterectomyNA6 endometriosis
Stassart J.P. et al., 1992 [25]USA15dysmenorrhea and increasing pelvic pain14.5 (range 6–26)11,7 (range 10–15), 1 premenarch15 (11R/4L)15 IRA, UD, OHV (9/15 complete obstruction with HC; 6/15 incomplete obstruction)13/15 underwent AE, 2/15 not AE 13/15 Y7/13 endometriosis
Zhang H. et al., 2017 [61]China2116/21 primary amenorrhoea with cyclic pelvic pain; 5/21 progressive dysmenorrhea14 (range 10–18)NA6 (4R/2L)6/21 IRA; 4/6 UD; 2/6 unicornuate uterus1 hymen incision, 4 vaginoplasty; 3 VSR, 11 resection of uterus, 1 resection of RUH, 1 hemihysterectomy11/21 Y6/11 endometriosis. 3/6 HWWS cases underwent AE and 2/3 were diagnosed with endometriosis
Kapczuk K. et al., 2017 [56]Poland22dysmenorrhea13.1 (range 11.4–18.2)12.2 (range 11–15.6)22 (13R/9L)22 IRA; 18/22 (81.8%) UD + OHV; 1/22 (4.5%) UD + unilateral cervical atresia; 3/22 (13.6%) unicornuate uterus + RUH with functional noncommunicating cavity2/22 laparotomy + VSR; 2/22 LSC + VSR; 3/22 laparotomy + RUH removal, 1/22 cervical fenestration + hemihysterectomy; 13/22 only VSR8/22 Y4/8 pelvic endometiosis stage 1
Zhang J. et al., 2020 [62]China2621/26 (80.8%) dysmenorrhea; 16/26 (61.5%) cystic mass; 11/26 (42.3%) irregular vaginal discharge; 6/26 (23.1%) prolonged menstrual periods15.5 (range 10–31)11.7 (range 10–15)24 NA24/26 IRA; 20/26: UD, 2/26 uterus bicornuate, 4/26 uterus septate. 25/26 OHV (17/25 complete obstruction; 8/25 incomplete obstruction)25/26 VSR, 1/26 LSC hemi-hysterektomyNANA
Zarfati A. et al., 2022 [15]Italy28abdominal pain, dysmenorrhea, vaginal discharge, irregular menstruation, infection, palpable abdominal mass, rectal tenesmus11.9 (r 0,5–15,7) (7/28 (25%) before menarche, 1/28 (3%) perinatally)NA23 (18R/5L)23 IRA, 5 multicystic-dysplastic kidney25/28 VSR, 1/28 VSR + LSC ovarian cystectomy (before menarche),
2/28 not yet operated on as not yet in menarche		1Y (before menarche), 27 NNo endometriosis
Case reports with individual cases (n = 1)
Ahmad Z. et al., 2013 [31]India1primary infertility2213; regularLIRA, UD, left OHVlaparotomy, cystectomy and adhesiolysis YEndometrioma and peritoneal endometriosis
Altchek A. et al., 2009 [32]USA1dysmenorrhea and right lower quadrant pain and rectal pressure1311; regularRIRA, UD, right HC + HMLSCYPeritoneal endometriosis
Arakaki R. et al., 2023 [33]Japan1dysmenorrhea and abnormal vaginal discharge1211; regularLIRA, UD, left OHVLSCYEndometriosis at the left fallopian tube
Basnet T. et al., 2020 [34]Nepal1mass associated with pain at vaginal introitus1714; irregularlyRIRA, UD, right HCVSRNAE not performed
Borges A.L. et al., 2023 [35]Portugal1foul discharge, dysmenorrhea1711, regularlyLIRA, UD, left OHVVSRNAE not performed
Cheung V. et al., 2008 [36]USA1dysmenorrhea, irregular menstrual cycles 17NALIRA, UD, cyst in the left ovaryLSC cystectomieYEndometioma
Cox D. et al., 2012 [37]USA1cyclic abdominal pain, enlarged abdominal mass1712; regularLIRA, UD, left OHVVSRNAE not performed
Girardi Fachin C. et al., 2019 [38]Brazil1dysmenorrhea, nausea, vomiting, fever and a palpable abdominal mass in the hypogastric region1211; irregularLIRA, uterus bicornis (left hemiuterus was distended and connected to the right one only by a myometrial strand)LSC hemi-hysterectomyYNA
Hayat A.M. et al., 2022 [39]Afghanistan1dysmenorrhea1513; regular1RIRA, UD, right HC + HM, left uterine horn and cervical canal normalVSRNNA
Khaladkar S.M. et al., 2016 [40]India1dysmenorrhea1312; regular1LIRA, UD, left OHV, right uterine cavity, cervix and vagina normallaparotomy, hemi-hysterectomyYNA
Kulkarni A. et al., 2023 [41]India1pelvic pain12premenarchal1RIRA, UD, right HC + HM; left uterine horn and cervical canal normalvaginoscopic VSRNAE not performed
Mabuchi Y. et al., 2021 [42]Japan1fever and lower abdominal pain2511; regular1RIRA, UD, right HC + HM; left uterine horn and cervical canal normalvaginoscopic VSRNAE not performed
Miyazaki Y. et al., 2020 [43]Japan1dysmenorrhea, cystic mass in the lower pelvic cavity2013; regular1LIRA, unicornuate uterus + RUH with functional noncommunicating cavityLSC left endometrioma and hematosalpinx.YEndometrioma
Nawfal K. et al., 2011 [44]USA1dysmenorrhea2012; irregular1RIRA, UD, OHV, HCLSC, supracervical hemi-hysterektomy right with in-situ pregnancyYPeritoneal endometriosis
Nishu D.S. et al., 2019 [45]Bangladesh1dysmenorrhea, right lower abdominal pain1515; regular1RIRA, UD, OHV, HCvaginoplasty VSRNAE not performed
Patterson D. et al., 2006 [46]USA1progressive cyclic rectal pain, chronic constipation10NA1RIRA, UD, OHV, HC+ HMLSC and vaginoscopy YNo endometriosis
Shah D.K. et al., 2011 [47]USA1dysmenorrhea and metrorrhagia12NA1RIRA, UD, OHV, HMVSR, 2 years later LSKYEndometriosis stage 1
Shim J. et al., 2020 [48]USA1dysmenorrhea and increasing pelvic pain15141RIRA, UD, HM, OHV with communication between the right hemivagina and the left cervixrobot-assisted hemihysterectomy and fallopian tubeYNA
Sijmons A. et al., 2023 [49]Netherlands1anuria and intralabial mass1 day 1Rmulticystic dysplastic kidney, UD, OHV and an ectopic ureteric insertion. hymen incision, nephrectomyNANA
Widyakusuma L.S. et al., 2018 [50]Indonesia1acute pelvic pain, dysmenorrea, cystic mass with a smooth surface in the right lateral region of the midline2312; regular1R1 IRA, UD, OHVVSRNNA
Yamada Y. et al., 2023 [51]Japan1dysmenorrhea and lower abdominal pain12121R1 IRA, UD, OHVvaginoscopic VSRNNA
IRA: ipsilateral renal agenesis, UD: uterus didelphys, RUH: rudimentary uterine horn, HC: hematocolpos, HM: hematometra; HS: hematosalpinx, OHV: obstructed hemivagina, VS: vaginal septum, AE: abdominal exploration, VSR: vaginal septum resection, HSG: hysterosalpingography, LSC: laparoscopy, NA: not available.
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MDPI and ACS Style

Dayan, D.; Ebner, F.; Janni, W.; Hancke, K.; Adiyaman, D.; Huener, B.; Hensel, M.; Hartkopf, A.D.; Schmid, M.; Lukac, S. Endometriosis Coinciding with Uterus Didelphys and Renal Agenesis: A Literature Review. J. Clin. Med. 2024, 13, 7530. https://doi.org/10.3390/jcm13247530

AMA Style

Dayan D, Ebner F, Janni W, Hancke K, Adiyaman D, Huener B, Hensel M, Hartkopf AD, Schmid M, Lukac S. Endometriosis Coinciding with Uterus Didelphys and Renal Agenesis: A Literature Review. Journal of Clinical Medicine. 2024; 13(24):7530. https://doi.org/10.3390/jcm13247530

Chicago/Turabian Style

Dayan, Davut, Florian Ebner, Wolfgang Janni, Katharina Hancke, Duygu Adiyaman, Beate Huener, Michelle Hensel, Andreas Daniel Hartkopf, Marinus Schmid, and Stefan Lukac. 2024. "Endometriosis Coinciding with Uterus Didelphys and Renal Agenesis: A Literature Review" Journal of Clinical Medicine 13, no. 24: 7530. https://doi.org/10.3390/jcm13247530

APA Style

Dayan, D., Ebner, F., Janni, W., Hancke, K., Adiyaman, D., Huener, B., Hensel, M., Hartkopf, A. D., Schmid, M., & Lukac, S. (2024). Endometriosis Coinciding with Uterus Didelphys and Renal Agenesis: A Literature Review. Journal of Clinical Medicine, 13(24), 7530. https://doi.org/10.3390/jcm13247530

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