{"paper_id":"1a1d57cd-5759-42cc-8c7e-bd75b4351fbf","body_text":"Copyright © 2023 by Author/s and Licensed by Modestum. This is an open access article distributed under the Creative Commons Attribution License which permits \nunrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.  \n \nElectronic Journal of Medical and Dental Studies \n2023, 13(1), em0103 \ne-ISSN: 2753-2658 \nhttps://www.ejmds.com  Letter to the Editor OPEN ACCESS \n \n \nPerineal scar endometriosis \n \nCarl Abi Semaan 1 , Amarelle Chamoun 2 , Jad Ghauch 1 , Hiba Hamdar 3*  \n \n1 University Saint Esprit Kaslik, Jouniyeh, LEBANON \n2 Faculty of Medical Sciences, Lebanese University, Beirut, LEBANON \n3 Plovdiv Medical University, Plovdiv, BULGARIA \n*Corresponding Author: hiba.hamdar@live.com  \n \nCitation: Abi Semaan C, Chamoun A, Ghauch J, Hamdar H. Perineal scar endometriosis. ELECTR J MED DENT STUD . 2023;13(1):em0103. \nhttps://doi.org/10.29333/ejmds/12813 \n \nARTICLE INFO  ABSTRACT \nReceived: 15 Dec. 2022 \nAccepted: 03 Jan. 2023 \n The presence of ectopic endometrial tissue outside the uterine cavity is referred to as endometriosis. It is a \nharmless condition that is commonly seen in women of reproductive age. It can happen in both the pelvic and \nextra-pelvic areas. Endometriosis in an episiotomy scar is extremely rare, but due to local infiltration, it can cause \nsignificant morbidity in patients. The presence of the classic clinical triad of episiotomy history, tender nodule at \nthe scar site, and cyclical pain can be used to diagnose this condition. Magnetic resonance imaging is a very useful \nimaging modality for diagnosing and assessing the lesion’s deeper extent. \nKeywords: perineal scar endometriosis, aetiology, pathogenesis, lesions, diagnosis, surgery \n \nINTRODUCTION \nEndometriosis is a benign condition that typically affects \nthe pelvic area and is linked to an inflammatory process that is \noestrogen-dependent and causes the formation of \nendometrial tissues and glands outside the uterine cavity. \nEndometriosis can affect the umbilicus, abdominal wall, lungs, \nor even the vulva or perineum outside of the pelvic area [1]. Its \naetiology is unknown, but it involves complex interactions \nbetween genetic makeup, hormonal functionality, menstrual \ncycle, different levels of inflammation, and immunological \ncomponents [2]. It affects about 10% of reproductive-age \nwomen and 35-50% of women with infertility and chronic \npelvic pain. While extra-perineal endometriosis (PEM) is less \ncommon than uterine endometriosis, accounting for only 12% \nof cases, perineal scar endometriosis is even less common, \naccounting for only 0.03% to 0.15% of all cases [3, 4].  \nThe pathogenesis of endometriosis has been the subject of \nnumerous theories, some of which include retrograde \nmenstruation or the implantation of uterine endometrial cells \nvia lymphatic dissemination, direct implantation, \nhaematogenous spread, or coelomic metaplasia. The \nmechanical transplantation of endometrial cells in the months \nfollowing an open episiotomy scar induced by vaginal delivery \nis thought to be the cause of episiotomy scar endometriosis [5]. \nA history of episiotomy, tender nodules at the scar site, and \nmenstrual pain are the typical clinical triad of symptoms of \nperineal scar endometriosis. These symptoms are only \nobserved in half of the cases, resulting in frequent \nmisdiagnosis. As a result, MRI is required to make a final \ndiagnosis and characterize the severity of the condition. The \nprimary treatment for perineal scar endometriosis is surgical \nexcision with clear wide margins [3]. \nDISCUSSION  \nPEM, also known as episiotomy scar endometriosis, was \nfirst described in 1923 and is defined by the presence of \nendometrial tissue in the perineal region, in either the vulva or \nsubcutaneous tissue. Anal sphincter involvement is seen in \napproximately half of the patients, resulting in dyspareunia, \nfaecal incontinence, and surgical fistula. As a result, early \ndetection and treatment are critical [1, 6, 7]. PEM is an \nextremely rare disease that affects only 0.3% to 1% of women \n[8]. Vulvo-PEM is classified into two types: cystic and nodular \nlesions, each with its own aetiology and treatment options [4]. \nPEM is most common after a vaginal delivery. It can also occur \nfollowing Bartholin gland excision sites within the vulva [9]. \nOther factors that contribute include lymphatic dissemination, \nhaematogenous spread, familial factors, immunological \nfactors, and mullerian remnant metaplasia. As a result, the \nproclivity for PEM is polygenic and multifactorial. According to \none study, having a first-degree relative with endometriosis \nincreases a woman’s risk of developing the condition seven \ntimes [10].  \nDespite the fact that perineal trauma is the most common \ncause of vulvo-PEM, scar endometriosis can also be caused by \nother general surgical procedures such as appendectomy, \ninguinal hernial repair, laparoscopic cholecystectomy, or \nlaparoscopic gastric by-pass. However, the occurrence of PEM \nfollowing these surgeries is extremely rare [1]. Undifferentiated \nstem cells may be the underlying cause of distant \nendometriosis lesions [2]. The body mass index BMI is one of \nseveral risk factors for the development of PEM and can affect \nits incubation period.  \n\n\n2 / 4 Abi Semaan et al. / ELECTR J MED DENT STUD, 2023;13(1):em0103 \nAccording to research, the higher the BMI during delivery \nand within one month afterward, the shorter the incubation \nperiod of PEM. This is explained by the increased presence of \nfat in the perineum and lochia in patients with a high BMI, \nwhich leads to blood accumulation in the vagina and \nperineum, and thus endometrial cell implantation in the \nperineal incision. Furthermore, steroid sulfatase and 17 beta-\nhydroxysteroid dehydrogenases in ectopic endometrial cells \nactivate sulphated steroid, the inactive oestrogen secreted by \nadipocytes [2, 11]. As a result, oestrogen’s local action is \namplified, resulting in a positive feedback loop that stimulates \nthe growth of endometrial cells in the perineum. The age of \nonset, delivery age, breastfeeding period, and time of return to \nmenstruation, on the other hand, have no effect on the \nincubation period of PEM. The duration of the pain, the size and \nnumber of nodules, involvement of the perianal muscles, and \npelvic pain were all found to be unrelated to the pain in the \nlesions [2]. Other factors, such as genetic predisposition, \nimmunological and familial factors, may also contribute to \nPEM pathogenesis [2, 10]. PEM is classified as primary or \nsecondary, and its aetiology is unknown. Many theories, \nhowever, have been put forth, including direct implantation, \nwhich describes secondary PEM, retrograde menstruation, \nlymphatic dissemination, coelomic metaplasia, or \nhaematogenous spread, which are signs of primary PEM [2, 5]. \nEndometrium may implant into the perineum episiotomy scar \nduring vaginal delivery and grow into a PEM lesion, according \nto the “implanted theory” [12]. Endometrial cells may \ndisappear spontaneously during vaginal birth, but they can \nalso develop into endometriomas through cyclic recurrence, \nwhich causes clinically unpleasant symptoms [13]. It is \nimportant to note that simply transplanting these cells is \ninsufficient because, as with peritoneal endometriosis, only a \nminority of pregnant patients with episiotomy or caesarean \nsection develop endometriosis, implying that there are still \nunknown individual factors that favour cell transplant \nacceptance [1]. PEM, on the other hand, can be found on the \nopposite side of the episiotomy scar, and even nulliparous \nwomen can be diagnosed with it.  \nAnother theory is retrograde menstruation, in which \nmenstrual endometrium fragments pass through the fallopian \ntubes and implant and persist on peritoneal surfaces, which \ncould explain why the Douglas cul-de-sac, ovaries, and uterus \ncontinue to be the primary sites of endometriotic lesions. \nHowever, neither of these theories can account for the \ndisease’s various locations [6]. Furthermore, the \ntransplantation hypothesis explains how perineal trauma, \nsuch as perineal tearing or episiotomy after vaginal delivery, \ncan result in PEM [11]. However, cases of PEM without anterior \nvulvo-vaginal trauma have been reported, in which retrograde \nmenstruation metastases through the peritoneum with \nlymphovascular dissemination to the lungs, GI tract, perineum, \nand vagina, a phenomenon known as the “metastatic theory” \n[14]. This theory explains the pathogenesis of endometriosis \nlesions that develop spontaneously. This theory explains the \npathogenesis of endometriosis lesions that develop \nspontaneously. Endometriosis in the labia majora may be \ncaused by the spread of pelvic endometriosis, but it can only be \nattributed to coelomic metaplasia in Bartholin’s gland [2]. \nAnother proposed theory is the transformation of pluripotent \nperitoneal mesothelium. Furthermore, a neurologic theory \nrecently proposed that the lesions migrate from the site of the \noriginal lesions and invade the large bowel along the nerves \n[10]. \nPerineal scar endometriosis has specific diagnostic criteria \nthat, if found in a patient, guarantee a 100% accuracy in \ndiagnosing the condition. The following are the criteria: a \nhistory of vaginal delivery perineal episiotomy, a painful mass \nor nodule at the perineum, and cyclic perineal pain during \nmenstruation [15]. Early diagnosis is critical because delayed \ndiagnosis can result in malaise caused by movements of \nsurrounding structures during defecation, such as the anal \nsphincter and the rectum [3]. Patients frequently present to \ntheir healthcare providers after experiencing several months of \nperineal pain and swelling. The agonizing condition \nsignificantly disrupts the patients’ daily lives; several reported \ncontinuous discomfort throughout the days that was not \nrelieved by pain medication, as well as dyspareunia in some \ncases [16]. A large proportion of those affected have had an \nepisiotomy following a vaginal delivery in the previous months \nor even years, whereas nulliparous women suffer from perineal \nscar endometriosis at a much lower rate.  \nPerineal scar endometriosis should be considered in a \nnulliparous patient who has nodules at the perineum that swell \nand hurt during menstruation [17]. Perineal scar endometriosis \nshould be considered in a nulliparous patient who has nodules \nat the perineum that swell and hurt during menstruation. \nDespite the fact that endometriosis is an oestrogen-dependent \ninflammation that occurs before or during menstruation, \npatients do not report a change in cycle length or flow volume. \nEndometrial stroma and glandular infiltrations were found at \nthe superficial level of the muscles adjacent to the scar, \nincluding the levator ani and the external anal sphincter, on \npathologic examination of the excised nodules. Some patients \nhad slightly elevated levels of serum cancer antigen 125 \n(CA125) [2, 6]. Scar endometriosis at the perineum is frequently \nmisdiagnosed because its symptoms are frequently confused \nwith hypertrophic scar tissue, abscesses, granulomas, \nmalignancies, metastatic carcinomas, hernias, desmoids \ntumours, hematomas, neuromas, and other more common \ndisorders [18].  \nPhysical examination and laparoscopy alone cannot \ndetermine the extent of perineal scar endometriosis within \ndeep pelvic tissues. Transvaginal ultrasounds and rectal \nsonographies, for example, do not detect deep pelvic lesions \nwithin the affected structures. As a result, magnetic resonance \nimaging (MRI) is the most rigorous modality for this evaluation \n[9]. The masses appear multi-lobular on MRI, with inner \nhaemorrhaging, which is typical of vulvar endometriosis. \nInvasion of surrounding muscles of the external anal sphincter, \nas well as more distal structures damaged by the disease, can \nalso be clearly identified [8]. If not treated promptly, perineal \nscar endometriosis can spread to neighbouring structures, \nmost notably the rectum and the perineal muscles: the levator \nani and the external anal sphincter. Faecal incontinence may \nresult from deterioration of these structures [6, 8].  \nPEM is usually diagnosed based solely on a history and a \nphysical exam. The sporadic growth of the lesion and \ndiscomfort during menstruation are diagnostic indicators. \nWhen patients first present, the majority have a sensitive, \npalpable subcutaneous swelling next to or inside the surgical \nscar [11, 13]. Because the typical symptoms are only present in \n50% of patients, the presence of the traditional symptoms \nallows for the clinical diagnosis of perineal scar endometriosis \n[3, 6], and imaging is helpful in the diagnosis. In addition, \nimaging aids in determining the extent of surgical treatment in \ncases of larger lesions and in the preoperative assessment of \n\n Abi Semaan et al. / ELECTR J MED DENT STUD, 2023;13(1):em0103 3 / 4 \nsphincter involvement, as well as ruling out other possibilities \nsuch as keloid, hematoma, granuloma, abscess, cysts, and \ntumour [5, 10, 19], but also anal fistula, atheroma, and \nhidradenitis [6, 12], which are differential diagnoses for \nperineal scar [2]. Ultrasound is frequently used to diagnose \nperineal scar endometriosis as the first imaging technique [3, \n10]. A hypoechoic or anechoic lesion with fine internal echoes \nmay exist at the site of the scar [3, 8, 11]. Peripheral vascularity \nmay be visible as well.  \nTransrectal ultrasound with a high-frequency probe can \ndiagnose rectal, recto-vaginal, or recto-sigmoid endometriosis, \nbut penetration is poor [3, 9]. However, computed tomography \n[3, 20, 21] and transvaginal ultrasound are less useful in \nassessing perineal scar endometriosis. Some case studies show \nthat the high-frequency power doppler angiographic \nappearance can also be used to identify scar endometriosis. \nFurthermore, because serum CA125 levels are usually normal \nor slightly elevated, it does not appear to be a reliable predictor \nof PEM [11, 13]. MRI is a great tool for determining the extent of \na localized disease before surgery because it is a non-invasive \nimaging technique [8, 10]. It also has a high spatial resolution, \nwhich allows for good tissue characterization and multiplanar \nevaluation, and because it is highly sensitive, it can distinguish \nendometriomas from nearby tissue [22]. Nonetheless, it was \nhighlighted utility of MRI in the preoperative evaluation of this \ncondition in their report on a case of perineal scar \nendometriosis with anal sphincter involvement [3]. \nEndometriosis is also more likely to be detected when T1 and \nT2 hyperintensities are present, but saturation is absent [8]. \nDue to its excellent contrast resolution, MRI can determine size \nof the lesion and its relationship to the anal sphincter complex. \nEndometriosis in the perineal scar is best treated with \nsurgical excision with wide, clear margins. Recurrence is a \npossibility. As a result, complete removal of the lesion is \ncritical. According to [3], the only way to prevent recurrence is \nto completely remove the lesion, including healthy margins [1, \n2, 5, 9, 10, 22, 23]. Furthermore, any cystic lesions must be \ncompletely removed in order to prevent cyst fluid from \ninfecting and implanting normal tissue [2]. Delaying surgery \nmay cause the lesion to worsen and involve the anal sphincter \n[5, 24]. As a result, patients with anal sphincter involvement \nwho did not receive complete excision experienced recurrence \n[23]. Following surgery, leuprolide, a gonadotropin-releasing \nanalog, can be used to reduce the risk of recurrence [8, 24]. \nHence, PEM patients treated with GnRH-agonists had a lower \nrecurrence rate after surgery than PEM patients who did not \nreceive GnRH-agonists. However, the decision to have PEM \nsurgery should be made after considering the patient’s \nexpectations for the surgery’s outcome, age, and desire for \npregnancy. Furthermore, perineal scar endometriosis can be \navoided by ensuring that the episiotomy scar is not \ncontaminated with debris and blood. Gloves should be \nreplaced before repairing an episiotomy wound [3]. \nSphincteroplasty may be required to reduce the risk of faecal \nincontinence when the anal sphincter is affected. \nCONCLUSION \nPerineal scar endometriosis is a rare and harmless disease. \nTo identify this illness in patients who present with no typical \nclinical symptoms, a high level of suspicion is required, and the \nearlier detection and intervention, the lower the morbidity and \ncomplications. Preoperative MRI, as well as ultrasound in some \naspects of endometriosis, is extremely helpful in making the \ndiagnosis and determining its local scope. Furthermore, they \ncan rule out endometriosis in other areas of the pelvis. \nFollowing surgery, follow-up care and certain drug treatments \nare critical for preventing recurrence. \nAuthor contributions: All authors have sufficiently contributed to the \nstudy and agreed with the results and conclusions. \nFunding: No funding source is reported for this study. \nEthical statement: Authors stated that the consent was not required \nfor this letter to the editor, which aims to spread the education \nregarding the subject between medical students and medical \nprofessionals as it is considered rare among the well known diseases \nand illnesses. \nDeclaration of interest: No conflict of interest is declared by authors. \nData sharing statement: Data supporting the findings and \nconclusions are available upon request from the corresponding author. \nREFERENCES \n1. Botezatu R, Turcu-Duminica A, Ciobanu AM, Gica N, Peltecu \nG, Panaitescu AM. Episiotomy scar endometriosis. Case \npresentation. Maedica (Bucur). 2021;16(4):713-6. \nhttps://doi.org/10.26574/maedica.2021.16.4.713 \n2. Liang Y, Zhang D, Jiang L, Liu Y, Zhang J. Clinical \ncharacteristics of perineal endometriosis: A case series. \nWorld J Clin Cases. 2021;9(5):1037-47. https://doi.org/10. \n12998/wjcc.v9.i5.1037 PMid:33644167 PMCid:PMC7896645 \n3. Jayanthan SS, Shashikala G, Arathi N. Perineal scar \nendometriosis. Indian J Radiol Imaging. 2019;29(4):457-61. \nhttps://doi.org/10.4103/ijri.IJRI_366_19 PMid:31949353 \nPMCid:PMC6958888 \n4. Maillard C, Cherif Alami Z, Squifflet JL, et al. Diagnosis and \ntreatment of vulvo-perineal endometriosis: A systematic \nreview. Front Surg. 2021;8:637180. https://doi.org/10.3389/ \nfsurg.2021.637180 PMid:34046423 PMCid:PMC8148344 \n5. Dadhwal V, Sharma A, Khoiwal K, Nakra T. Episiotomy scar \nendometriosis. Med J Armed Forces India. 2018;74(3):297-\n9. https://doi.org/10.1016/j.mjafi.2017.06.004 PMid: \n30093779 PMCid:PMC6081211 \n6. Liu Y, Pi R, Luo H, Wang W, Zhao X, Qi X. Characteristics and \nlong-term outcomes of perineal endometriosis: A \nretrospective study. Medicine (Baltimore). \n2020;99(23):e20638. https://doi.org/10.1097/MD.00000000 \n00020638 PMid:32502046 PMCid:PMC7306333 \n7. Eray IC, Topal U. Perineal scar endometriosis involving the \nanal sphincter. A case report and review of the literature. \nAnn Ital Chir. 2021;10:S2239253X2103173X. \n8. Hakim H, Ben Halima S, Zouari A, et al. Perineal \nendometriosis: A rare case of a unique sizeable nodule. Pan \nAfr Med J. 2021;38:47. https://doi.org/10.11604/pamj.2021. \n38.47.27737 PMid:33854676 PMCid:PMC8017358 \n9. Matalliotakis M, Matalliotaki C, Zervou MI, Krithinakis K, \nGoulielmos GN, Kalogiannidis I. Abdominal and perineal \nscar endometriosis: Retrospective study on 40 cases. Eur J \nObstet Gynecol Reprod Biol. 2020;252:225-7. \nhttps://doi.org/10.1016/j.ejogrb.2020.06.054 PMid: \n32623253 \n10. Bindra V, Reddy N, Reddy CA, Swetha P, Alapati KV, Nori M. \nRecurrent perineal scar endometriosis: A case report. Case \nRep Womens Health. 2022;36:e00457. https://doi.org/10. \n1016/j.crwh.2022.e00457 PMid:36281243 PMCid: \nPMC9587519 \n\n4 / 4 Abi Semaan et al. / ELECTR J MED DENT STUD, 2023;13(1):em0103 \n11. Barisic GI, Krivokapic ZV, Jovanovic DR. Perineal \nendometriosis in episiotomy scar with anal sphincter \ninvolvement: Report of two cases and review of the \nliterature. Int Urogynecol J Pelvic Floor Dysfunct. \n2006;17(6):646-9. https://doi.org/10.1007/s00192-005-\n0022-5 PMid:16231117 \n12. Uzuncakmak C, Guldas A, Ozcam H, Dinc K. Scar \nendometriosis: A case report of this uncommon entity and \nreview of the literature. Case Rep Obstet Gynecol. \n2013;2013:386783. https://doi.org/10.1155/2013/386783 \nPMid:23762683 PMCid:PMC3665185 \n13. Li J, Shi Y, Zhou C, Lin J. Diagnosis and treatment of \nperineal endometriosis: Review of 17 cases. Arch Gynecol \nObstet. 2015;292(6):1295-9. https://doi.org/10.1007/ \ns00404-015-3756-4 PMid:26041323 \n14. Tangri MK, Lele P, Bal H, Tewari R, Majhi D. Scar \nendometriosis: A series of 3 cases. Med J Armed Forces \nIndia. 2016;72(Suppl 1):185-8. https://doi.org/10.1016/ \nj.mjafi.2016.07.002 PMid:28050109 PMCid:PMC5192233 \n15. Blanco RG, Parithivel VS, Shah AK, Gumbs MA, Schein M, \nGerst PH. Abdominal wall endometriomas. Am J Surg. \n2003;185(6):596-8. https://doi.org/10.1016/S0002-9610(03) \n00072-2 PMid:12781893 \n16. Kaplanoglu M, Kaplanoglu DK, Dincer Ata C, Buyukkurt S. \nObstetric scar endometriosis: Retrospective study on 19 \ncases and review of the literature. Int Sch Res Notices. \n2014;2014:417042. https://doi.org/10.1155/2014/417042 \nPMid:27379258 PMCid:PMC4897354 \n17. Kokuba EM, Sabino NM, Sato H, Aihara AY, Schor E, Ferreira \nLM. Reconstruction technique for umbilical endometriosis. \nInt J Gynaecol Obstet. 2006;94(1):37-40. https://doi.org/10. \n1016/j.ijgo.2006.04.034 PMid:16781715 \n18. Jain D. Perineal scar endometriosis: A comparison of two \ncases. BMJ Case Rep. 2013;2013:bcr2013010051. \nhttps://doi.org/10.1136/bcr-2013-010051 PMid:23897379 \nPMCid:PMC3736109 \n19. Gunes M, Kayikcioglu F, Ozturkoglu E, Haberal A. Incisional \nendometriosis after cesarean section, episiotomy and \nother gynecologic procedures. J Obstet Gynaecol Res. \n2005;31(5):471-5. https://doi.org/10.1111/j.1447-0756. \n2005.00322.x PMid:16176520 \n20. de Paula Andres M, Lopes LA, Baracat EC, Podgaec S. \nDienogest in the treatment of endometriosis: Systematic \nreview. Arch Gynecol Obstet. 2015;292(3):523-9. \nhttps://doi.org/10.1007/s00404-015-3681-6 PMid:25749349 \n21. Watanabe M, Kamiyama G, Yamazaki K, et al. Anal \nendosonography in the diagnosis and management of \nperianal endometriosis: Report of a case. Surg Today. \n2003;33(8):630-2. https://doi.org/10.1007/s00595-003-\n2545-z PMid:12884104  \n22. Odobasic A, Pasic A, Iljazovic-Latifagic E, et al. Perineal \nendometriosis: A case report and review of the literature. \nTech Coloproctol. 2010;14(Suppl 1):S25-7. https://doi.org/ \n10.1007/s10151-010-0642-8 PMid:20862505 \n23. Grimstad FW, Carey E. Periclitoral endometriosis: The \ndilemma of a chronic disease invading a rare location. J \nMinim Invasive Gynecol. 2015;22(4):684-6. https://doi.org/ \n10.1016/j.jmig.2015.02.002 PMid:25680686 \n24. Cinardi N, Franco S, Centonze D, Giannone G. Perineal scar \nendometriosis ten years after Miles’ procedure for rectal \ncancer: Case report and review of the literature. Int J Surg \nCase Rep. 2011;2(6):150-3. https://doi.org/10.1016/j.ijscr. \n2011.04.001 PMid:22096711 PMCid:PMC3199622","source_license":"CC0","license_restricted":false}