Uterine rudimentary horn and ovarian endometriosis with a special emphasis on VACTERL association: a report of two cases

Uterine rudimentary horn and ovarian endometriosis with a special emphasis on VACTERL association: a report of two cases DOI: https://doi.org/10.18203/2320-1770.ijrcog20241793Keywords: Mullerian duct anomalies, Unicornuate uterus, Rudimentary uterine horn, Endometriosis, VACTERLAbstract Mullerian duct anomalies are a wide and complex spectrum of congenital abnormalities that are often associated with renal as well as axial skeletal anomalies or can be a part of multiple malformation syndrome and may cause numerous other gynecological complications. We herein describe two young girls who presented with a common complaint of abdominal pain and had normal secondary sexual characteristics.The first case had a history of imperforate anus, and urinary incontinence due to renal anomalies including ectopic kidney, renal agenesis, duplex ureter along with sacral agenesis which were confirmed on imaging. VACTERL (V-vertebral anomalies, A-anorectal malformations, C-cardiovascular anomalies, T- tracheoesophageal fistula, E-esophageal atresia, R-renal anomalies, and L-limb defects) association was considered. To the best of our knowledge, this is the fourth such case with concomitant VACTERL association. Surgical resections were performed in both cases, after clinical-radiological correlation. Histopathological examination of specimens revealed a unicornuate uterus with a rudimentary horn and ovarian endometriosis. In conclusion, early identification and prompt intervention through a multidisciplinary approach are paramount for managing such anomalies as they can have many detrimental implications. Metrics References Sharma S. A rare incidental case of an accessory fallopian tube. Fertil Sci Res. 2020;7(1):117-20. Caserta D, Mallozzi M, Meldolesi C, Bianchi P, Moscarini M. Pregnancy in a unicornuate uterus: a case report. J Med Case Rep. 2014;8:130. Li S, Qayyum A, Coakley FV, Hricak H. Association of renal agenesis and mullerian duct anomalies. J Comput Assist Tomogr. 2000;24(6):829-34. Osman NM, Hamza AM, Elamin HAM. Congenital vaginal obstruction in a newborn. Sudan J Paediatr. 2019;19(2):145-8. Messina M, Severi FM, Bocchi C, Ferrucci E, Di Maggio G, Petraglia F. Voluminous perinatal pelvic mass: a case of congenital hydrometrocolpos. J Matern Fetal Neonatal Med. 2004;15(2):135-7. Shaw LM, Jones WA, Brereton RJ. Imperforate hymen and vaginal atresia and their associated anomalies. J R Soc Med. 1983;76(7):560-6. Heinonen PK. Unicornuate uterus and rudimentary horn. Fertil Steril. 1997;68(2):224-30. Dane C, Dane B, Erginbas M, Cetin A. Imperforate hymen-a rare cause of abdominal pain: two cases and review of the literature. J Pediatr Adolesc Gynecol. 2007;20(4):245-7. Nunes N, Karandikar S, Cooper S, Jaganathan R, Irani S. VATER/VACTERL syndrome (vertebra/anus /cardiac/trachea/esophogus/radius/renal/limb anomalies) with a noncommunicating functioning uterine horn and a unicornuate uterus: a case report. Fertil Steril. 2009;91(5):1957.e11-2. Obeidat RA, Aleshawi AJ, Tashtush NA, Alsarawi H. Unicornuate uterus with a rudimentary non-communicating cavitary horn in association with VACTERL association: case report. BMC Womens Health. 2019;19(1):71. Roman JD. Co-existence of a rudimentary non-communicating horn with a unicornuate uterus in association with 2 components of the vacterl association: a case report. Case Rep Obstet Gynecol Rep. 2021;3(2):1-4. Lupean RA, Ștefan PA, Csutak C, Lebovici A, Măluțan AM, Buiga R, et al. Differentiation of Endometriomas from Ovarian Hemorrhagic Cysts at Magnetic Resonance: The Role of Texture Analysis. Medicina (Kaunas). 2020;56(10):487. Koninckx PR, Barlow D, Kennedy S. Implantation versus infiltration: the Sampson versus the endometriotic disease theory. Gynecol Obstet Invest. 1999;47 Suppl 1:3-9; discussion 9-10. Matsuura K, Ohtake H, Katabuchi H, Okamura H. Coelomic metaplasia theory of endometriosis: evidence from in vivo studies and an in vitro experimental model. Gynecol Obstet Invest. 1999;47 Suppl 1:18-20; discussion 20-2. Konrad L, Dietze R, Kudipudi PK, Horné F, Meinhold-Heerlein I. Endometriosis in MRKH cases as a proof for the coelomic metaplasia hypothesis? Reproduction. 2019;158(2):R41-R47. Abboud K, Giannini A, D'Oria O, Ramadan A, Ayed A, Laganà AS, et al. Laparoscopic Management of Rudimentary Uterine Horns in Patients with Unicornuate Uterus: A Systematic Review. Gynecol Obstet Invest. 2023;88(1):1-10.