{"paper_id":"4fd71257-9190-4f1c-bac6-7a527aaff0cf","body_text":"55Vol. 46, No. 2, June 2024\nCase report\nUnicornuate uterus with a functional, non-communicating\nrudimentary cavity associated with ovarian endometriosis and\nipsilateral renal agenesis\nH D Manamendrab, W Abeykoonb, R M D B Ranatungac\nCase report\nThis is an open-access article distributed under the terms of the Creative Commons Attribution 4.0 International License, which\npermits unrestricted use, distribution and reproduction in any medium provided the original author and source are credited.\nSri Lanka Journal of Obstetrics and Gynaecology   2024; 46:  55-58\nDOI:  https://doi.org/10.4038/sljog.v46i2.8151\nCorrespondence: HDM, e-mail: hesharamanamendra99@gmail.com\nhttps://orcid.org/0009-0003-9399-7486\na Undergraduate, Faculty of Medicine, University of Peradeniya, Sri Lanka\nb Consultant in Obstetrics and Gynaecology, National Hospital Kandy, Sri Lanka\nc Senior Registrar in Obstetrics and Gynaecology, National Hospital Kandy, Sri Lanka\nAbstract\nIntroduction: Müllerian anomalies are rare developmental anomalies of the female reproductive tract, that are usually\nassociated with renal abnormalities.\nCase Presentation:  A 16-year-old Sri Lankan schoolgirl, presented with progressive dysmenorrhea and inter-menstrual\nlower abdominal pain for 2 years. There was recent lower abdominal distention over 6 months. Examination revealed a non-\ntender mass in the suprapubic area.\nA subsequent CT scan, followed by an ultrasound abdomen, revealed features of a right-sided unicornuate uterus with a\nnon-communicating left-sided horn containing endometrium and a homogenous large cystic lesion between the non-\ncommunicating left horn and the uterine body, associated with ipsilateral renal agenesis.\nShe underwent a laparotomy and resection of the left-side rudimentary horn of the uterus with a left-side ovarian cystectomy,\nas intra-operative findings suggested an endometrioma.\nConclusion: Clinical presentations of Müllerian anomalies are largely non-specific. Early diagnosis and prompt treatment are\nuseful in avoiding complications that arise as a consequence of Müllerian anomalies.\nIntroduction\nMüllerian anomalies are rare developmental anomalies\nof the female reproductive tract. Uterine anomalies have\nbeen classified according to the American Society for\nReproductive Medicine, which divides uterine malfor-\nmations into nine main groups: Müllerian agenesis,\ncervical agenesis, unicornuate uterus, uterus didelphys,\nKeywords: Müllerian anomalies, ovarian endometriosis, renal agenesis, unicornuate uterus\nbicornuate uterus, septate uterus, longitudinal vaginal\nseptum, transverse vaginal septum, and complex\nanomalies. Unicornuate uterus develops as a result of\nunilateral failure of the normal Müllerian system\ndevelopment. In most instances, the abnormal\nMüllerian duct has partially developed into a rudimentary\nuterine horn1.\nReceived 3rd March 2024\nAccepted 13th June 2024\n\n56 Sri Lanka Journal of Obstetrics and Gynaecology\nCase report\nIn this article, we describe a rare case of unicornuate\nuterus with a functional non-communicating rudi-\nmentary horn, associated with ovarian endometriosis\nand ipsilateral renal agenesis to improve our unders-\ntanding of this rare Müllerian anomaly, along with the\nproper diagnosis methods and treatment options.\nCase presentation\nA 16-year-old Sri Lankan schoolgirl, presented with\nprogressive dysmenorrhea and inter-menstrual lower\nabdominal pain that developed after one year of\nmenarche, affecting her quality of life. She attained\nmenarche at the age of 13 and had regular periods.\nThere is no heavy menstrual bleeding, intermenstrual\nbleeding, vaginal discharge, or urinary symptoms.\nLower abdominal distention developed gradually over\nthe last 6 months with mild discomfort. Past medical,\nsurgical, and family histories were not significant.\nOn examination, there was a visible mass in the\nsuprapubic area. It was a rounded, firm mass of around\n10 × 9 cm with regular margins and a smooth surface,\nwhich was pelvic in origin and extended into the\nabdominal cavity.\nA transabdominal ultrasound scan suggested a right-\nside ovarian cyst measuring 13×15 cm. Subsequent\nCT scan revealed features of a right-sided unicornuate\nuterus with a non-communicating left-sided horn\ncontaining endometrium categorized in Class II\nMüllerian duct anomaly, type alb (American Fertility\nsociety classification), a distended left endometrial\ncavity with high-density fluid collection within\n(5×3.5×3.5 cm), and a homogenous large cystic lesion\nbetween the non-communicating left horn and the\nuterine body, which very much represent endometriotic\nhematometra secondary to repetitive shedding of the\nrudimentary horn in size of 9 (A)×13 (Trans) ×15 (CC)\ncm. Left-side renal agenesis was identified with a\nhypertrophied right kidney and BOSNIAK type 1 renal\ncyst in the interpolar region of the right kidney.\nShe underwent a laparotomy and resection of the left-\nside rudimentary horn of the uterus with a left-side\novarian cystectomy. Intra-operative findings were a\nlarge homogeneous cyst, which was suggestive of\nendometrioma of the left side ovary of 15×10×8 cm in\nsize, and a functional left side rudimentary horn, which\nwas 5×8×6 cm in size with hematometra. The left-\nside fallopian tube was not visible. The right-side\nfunctional uterine horn was normal in size, and the\nright-side fallopian tube and right-side ovary appeared\nto be normal as well. An excised sample of the rudi-\nmentary horn and ovary was sent for histopathological\ndiagnosis. Histology report findings were an endo-\nmetriotic cyst in the left ovary and the rudimentary\nhorn with late secretory phase endo-metrium. We plan\nto follow up on her until 10-15 years from now, to\nassess her pregnancy outcomes as well.\nFigure 1. Endometrioma of the left ovary (A) Altered blood filled ovary\n(B) After the evacuation of filled blood.\n(A)\n(A)\n(B)\n\n57Vol. 46, No. 2, June 2024\nCase report\nDiscussion\nThe overall prevalence of Müllerian anomalies was\n9.8% of the general population, with a 1.6% prevalence\nof unicornuate uteri\n2.\nMüllerian ducts (Paramesonephric ducts), whose\ndevelopment is preceded by Wolffian (mesonephric)\nducts within the paired urogenital ridges, provide critical\nparacrine growth factors for Müllerian duct growth\n3.\nTherefore, Müllerian anomalies are usually associated\nwith urinary tract anomalies. Statistically, 40.5% of\nthe patients with a unicornuate uterus presented with\nurinary tract anomalies such as renal agenesis, ectopic\nkidney, horseshoe kidney, double renal pelvis, and/ or\nunilateral medullary sponge kidney\n4. In our case, the\npatient had a unicornuate uterus with congenital renal\nagenesis on the side of the rudimentary horn, along\nwith ovarian endometriosis.\nThe incidence of a unicornuate uterus with endo-\nmetriosis on the side of the rudimentary horn is 20-\n40%\n5. The pathogenesis of endometriosis remains con-\ntroversial. According to the theory of retrograde\nmenstruation, endometrial cells and tissues derived from\nmenstruation implanted retrogradely into the abdominal\ncavity invade and induce a local inflammatory response\nthat is accompanied by angiogenesis, adhesion,\nfibrosis, scarring, and anatomical distortion\n6, which is\nFigure 2. Specimen of the rudimentary\nuterine horn.\nthe most applicable theory in our patient as she had\ncollected blood in the functional rudimentary cavity of\nthe uterus, which probably led to the development of\nendometriosis of the left ovary.\nA similar case study, which was regarding an 18-year-\nold girl who presented with progressive dysmenorrhea\nand was diagnosed to have a unicornuate uterus with\na left-sided cavitary rudimentary horn with left hemato-\nmetra and hematosalpinx and left renal agenesis,\nmentioned that she underwent a successful laparotomy\nand excision of the left-sided cornu along with left\nsalpingo-oophorectomy\n7. Another study of a 15-year-\nold girl with progressive dysmenorrhea, who was\ndiagnosed to have a left unicornuate uterus with a non-\ncommunicating right cavitary horn and hematometra,\nhad laparoscopic removal of the right fallopian tube\nand rudimentary horn\n8. In both cases, sonographic\nexamination was the first-line imaging modality used,\nfollowed by MRI in diagnosing.\nMRI has proved effective in preoperative evaluation\nof Müllerian duct anomalies\n9. Combined hysteroscopy\nand laparoscopy is considered to be the gold standard\nin diagnosis10. Our patient underwent a CT scan before\nsurgery due to the unavailability of an MRI.\nIt has been proven that a unicornuate uterus is asso-\nciated with an increased risk of adverse pregnancy\noutcomes, specifically functional rudimentary horns,\nwhich are at particularly high risk of both pelvic\nendometriosis and rudimentary-horn pregnancy\n5.\nTherefore, surgical excision of the rudimentary horn\nwas done to prevent potentially serious complications\nin pregnancy, and it is recommended to reduce the\nrisk of recurrent or de novo endometriosis\n11. Removal\nof the left ovary, which consists of endometriosis, was\nnecessary for our patient to alleviate dysmenorrhea.\nLaparoscopy proves to be an effective surgical ap-\nproach for the removal of the cavitated, non-com-\nmunicating rudimentary horn in patients with a uni-\ncornuate uterus\n12. If facilities are available, laparoscopic\nexcision should be offered, especially to young girls,\nas it is less morbid and cosmetically more acceptable.\nOur patient underwent a CT scan before surgery, which\nhas poorer soft tissue resolution than an MRI and does\nnot give adequate information about the condition to\nproceed with laparoscopic surgery. Furthermore, the\nlack of infrastructure led us to proceed with laparo-\ntomy and resection of the rudimentary horn and the\novarian cyst.\n\n58 Sri Lanka Journal of Obstetrics and Gynaecology\nCase report\nConclusion\nThe clinical presentations of rudimentary uterine horn\nwith ovarian endometriosis, including dysmenorrhea\nand non-menstrual pelvic pain, are largely non-specific.\nTherefore, when evaluating dysmenorrhea, especially\nin adolescents, it is important to consider the rare\npossibility of mullerian anomalies. Early diagnosis and\nprompt treatment are useful to avoid future gyne-\ncological and obstetrical complications. Laparoscopic\nsurgery is the preferred mode of management if the\nfacilities are available.\nConflicts of interest\nThe authors declare that they have no conflicts of\ninterest.\nReferences\n1. Pfeifer SM, Attaran M, Goldstein J, Lindheim SR,\nPetrozza JC, Rackow BW, et al. ASRM müllerian\nanomalies classification 2021. Fertility and Sterility\n[Internet]. 2021 Nov;116(5):1238-52. Available\nfrom: https://www.asrm.org/globalassets/asrm/\nasrm-content/news-and-publications/practice-\nguidelines/for-non-members/mac2021_\nmanuscript.pdf\n2. Dreisler E, Stampe Sørensen S. Müllerian duct\nanomalies diagnosed by saline contrast sono-\nhysterography: prevalence in a general population.\nFertility and Sterility. 2014; 102(2): 525-9.\n3. Zhao F, Grimm SA, Jia S, Yao HHC. Contribution\nof the Wolffian duct mesenchyme to the formation\nof the female reproductive tract. Bartolomei M,\neditor. PNAS Nexus. 2022; 1(4).\n4. Fedele L, Bianchi S, Agnoli B, Tozzi L, Vignali M.\nUrinary Tract Anomalies Associated with\nUnicornuate Uterus. Journal of Urology. 1996;\n155(3): 847-8.\n5. Tellum T, Bracco B, De Braud LV , Knez J, Ashton-\nBarnett R, Amin T, et al. Reproductive outcome in\n326 women with unicornuate uterus. Ultrasound\nin Obstetrics and Gynecology. 2023; 61(1): 99-\n108.\n6. Sourial S, Tempest N, Hapangama DK. Theories\non the Pathogenesis of Endometriosis.\nInternational Journal of Reproductive Medicine.\n2014; 2014(179515): 1-9.\n7. Choudhary R, Verma S, Meena A, Sitaram\nGothwal. A Case of Rare Mullerian Anomaly-\nFunctional Rudimentary Uterine Horn: As an\nUnusual Cause of Unilateral Dysmenorrhea in\nPostmenarchal Adolescent Girl. Journal of SAFOG.\n2012; 4(1): 56-8.\n8. Vijay NR, Santosh Mhatardev Dahifale. Uni-\ncornuate Uterus with Noncommunicating\nFunctional Rudimentary Horn as A Rare Cause of\nSecondary Dysmenorrhea: A Case Report. Journal\nof SAFOG. 2023; 15(3): 365-7.\n9. Marten K, V osshenrich R, Funke M, Obenauer S,\nBaum F, Grabbe E. MRI in the evaluation of\nmüllerian duct anomalies. Clinical Imaging. 2003;\n27(5): 346-50.\n10. Saravelos SH, Cocksedge KA, Li TC . Prevalence\nand diagnosis of congenital uterine anomalies in\nwomen with reproductive failure: a critical\nappraisal. Human Reproduction Update. 2008;\n14(5): 415-29.\n11. Fujimoto VY, Klein NA, Miller PB. Late-onset\nhematometra and hematosalpinx in a woman with\na noncommunicating uterine horn. A case report.\nThe Journal of Reproductive Medicine [Internet].\n1998 May 1 [cited 2024 Feb 7]; 43(5): 465-7.\nAvailable from: https://pubmed.ncbi.nlm.nih.gov/\n9610474/\n12. Fedele L, Bianchi S, Zanconato G, Berlanda N,\nBergamini V . Laparoscopic removal of the\ncavitated noncommunicating rudimentary uterine\nhorn: Surgical aspects in 10 cases. 2005; 83(2):\n432-6.","source_license":"CC0","license_restricted":false}