{"paper_id":"3bb73d25-5fd6-4242-b7a1-5440485270c5","body_text":"Open Access, Volume 5 \nPrimary endometriosis mimicking a strangulated umbilical \nHernia: A case report and review of literature\nCase Report\nwww.jcimcr.org\nJournal of\nClinical Images and Medical Case Reports\nReceived: Jun 20, 2024\nAccepted: Jul 26, 2024\nPublished: Aug 02, 2024  \nArchived: www.jcimcr.org\nCopyright: © Aghahowa ME (2024).\nDOI: www.doi.org/10.52768/2766-7820/3191\n*Corresponding Author: Michael E Aghahowa\nDepartment of Surgery, College of Health Sciences, \nNile University of Nigeria, Abuja, Nigeria.\nEmail: Michael.ehienagudia@nileuniversity.edu.ng \n& mikeaghahowa@yahoo.com\nISSN 2766-7820\nIntroduction\nEndometriosis is the presence of functional endometrial \nglands and stroma outside the uterine cavity. It is a common \nbenign gynaecological condition, affecting 5-10% of women of \nreproductive age and found in at least a third of women un -\ndergoing a diagnostic laparoscopy for pelvic pain or infertility \n[1]. The condition is oestrogen-dependent and tends to resolve \nafter the menopause. It is usually found within the pelvis, locat-\ned along the peritoneum lining the pelvic side walls, pouch of \nDouglas, uterosacral ligaments and urinary bladder and bowel. \nIt occurs less frequently in extra-pelvic sites including the um -\nbilicus, surgical scars (especially after caesarean sections and \nin laparoscopic port sites), and pleural cavity, and occasionally \ndevelops spontaneously [1,2]. Endometriotic tissues respond \nAbstract\nEndometriosis is a benign gynaecological condition characterised by \nthe presence of functional endometrial glands and stroma outside the \nuterine cavity. It commonly develops in the pelvis but can be found in \nother extra-pelvic sites. Umbilical endometriosis is rare and primary \n(spontaneous) umbilical endometriosis associated with umbilical her -\nnia can present a diagnostic challenge to the surgeon as the presenting \npainful swelling may mimic an obstructed hernia. We present a 34-year \nold nulliparous woman who presented with a tender umbilical nod -\nule with no previous abdominal surgical scar and no known history of \nendometriosis. A surgical excision of the nodule with umbilical herni -\norrhaphy was done and histological examination of the excised tissue \nconfirmed a primary umbilical endometriosis. Further gynaecological \nevaluation was unremarkable and the patient has remained symptom-\nfree. A high index of suspicion and a histopathological examination of \nthe excised surgical specimen are mandatory for the diagnosis of pri -\nmary umbilical endometriosis in a woman of reproductive age present-\ning with a tender umbilical nodule.\nKeywords: Primary umbilical endometriosis; Umbilical hernia; \nVillar’s nodule; Surgical excision.\nMichael E Aghahowa1,2*; Alu FE3,4; Okonkwo DI5,6; Osisanya K7,8; Duum CN9; Gali BM1,2\n1Department of Surgery, College of Health Sciences, Nile University of Nigeria, Abuja, Nigeria.\n2Department of Surgery, Asokoro District Hospital, Abuja, Nigeria.\n3Department of Obstetrics and Gynecology, College of Health Sciences, Nile University of Nigeria, Abuja, Nigeria.\n4Department of Obstetrics and Gynecology, Asokoro District Hospital, Abuja, Nigeria.\n5Department of Anatomic Pathology, College of Health Sciences, Nile University of Nigeria, Abuja, Nigeria.\n6Department of Anatomic Pathology, Maitama District Hospital, Abuja, Nigeria.\n7Department of Surgery, Baze University, Abuja, Nigeria.\n8Department of Surgery, Maitama District Hospital, Abuja, Nigeria.\n9Department of Obstetrics and Gynecology, Maitama District Hospital, Abuja, Nigeria.\n\nwww.jcimcr.org                Page 2\nCitation: Aghahowa ME, Alu FE, Okonkwo DI, Osisanya K, Duum CN, et al. Primary endometriosis mimicking a strangulated \numbilical hernia: A case report and review of literature. J Clin Images Med Case Rep. 2024; 5(8): 3191.\nFigure 1: Photograph showing supra umbilical swelling.\nto hormonal changes and undergo cyclical bleeding and local \ninflammatory reactions. The cyclical episodes of bleeding and \nhealing lead to fibrosis and adhesion formation. Affected wom-\nen traditionally present with severe cyclical non-colicky pelvic \npain restricted to around the time of menstruation which may \nstart a few days before the commencement of menstruation, \nand continue until the end of menstruation. The pain may be \nin form of dysmenorrhoea, deep dyspareunia, and dyschezia. \nEndometriosis is frequently associated with irritable bowel syn-\ndrome. Extra-pelvic endometriosis can cause local symptoms \nlike cyclical epistaxis, cyclical haemoptysis/haemopneumotho -\nrax, cyclical rectal bleeding, cyclical haematuria, and cyclical \npain, swelling and bleeding at abdominal surgical scars and the \numbilicus [1]. The aetiology of endometriosis is unknown but \nseveral theories have been postulated to explain it, including \nthe Sampson’s theory of retrograde menstruation, implantation \ntheory (surgical implantation, vascular and lymphatic spread); \ncoelomic metaplasia; and genetic and immunological factors \n[3].\nDiagnosis is mainly clinical aided by ultrasound scan and \nlaparoscopy. Laparoscopy is the traditional method of diagnosis \nwhich is based on the accurate visual identification of endome-\ntriotic lesions [4]. It has the added advantage of allowing biopsy \nof the lesions for histological confirmation of diagnosis and for \nconcurrent surgical diathermy ablation and/or excision of the \nendometriotic lesions. Magnetic Resonance Imaging (MRI) is \nthe imaging technique of choice for diagnosis as it can detect \nlesions of >5 mm in size [5]. Management of endometriosis in -\ncludes both medical and surgical treatment, with medical treat-\nment reserved only for preoperative relief of symptoms as it is \nassociated with side effects and limited to short-term use [6].\nExtra-pelvic endometriosis is rare and often presents a di -\nagnostic challenge to the surgeon when evaluating a female \nwho presents with cyclical pain, bleeding and/or swellings [3]. \nUmbilical endometriosis is rare and develops mainly in surgical \nscars. Primary Umbilical Endometriosis (PUE) without previous \npelvic surgery is however extremely rare [6-8]. We present a \n34-year old female who had an undiagnosed primary umbilical \nendometriosis but presented with features mimicking a stran -\ngulated umbilical hernia.\nCase presentation\nA 34-year old unmarried nulliparous lady who presented to \nthe surgical outpatient clinic with a history of an umbilical swell-\ning of 4 years duration associated with recurrent umbilical pain \nespecially during her menstruation and with associated dys -\nmenorrhoea and lower abdominal and pelvic pain relieved by \ningestion of NSAIDs, but with no visible bleeding from the um -\nbilicus. The swelling gradually increased in size over the last one \nmonth prior to presentation. There were no specific intestinal \nor other associated symptoms. Her past surgical and medical \nhistories were not significant. She had also not been evaluated \nby any gynaecologist.\nExamination showed a 3 cm by 4 cm nodular swelling around \nthe upper edge of the umbilicus, darkish in colour, tender, ir -\nreducible, and not pulsating. The umbilicus was inverted. Ab -\ndominal ultrasound scan confirmed a swelling abutting on the \numbilicus. The patient had excision biopsy of the nodule and \na simple umbilical defect repair. Histopathological examination \nrevealed the presence of endometrial glands and stroma pre -\ndominantly within the dermis. Some of the glands were dilated \nand filled with red blood cells. Also seen were haemosiderin-\nladen macrophages, infiltrates of lymphoplasmatic inflamma -\ntory cells and intervening dense fibrotic stroma; the overlying \nepidermis was hyperplastic and there was no evidence of malig-\nnancy; all features are in keeping with endometriosis. She was \nreferred to the gynaecologists for further evaluation following \nthe histology report and the findings were unremarkable. The \npatient has remained free of symptoms for the past 6 months. \nFigure 2: Photograph showing excision of swelling.\nFigure 3: Mid-power photomicrograph showing endometrial \nglands filled with blood clots and stroma laden with haemosiderin \nmacrophages (H&E X 10).\n\n\nwww.jcimcr.org                Page 3\nDiscussion\nUmbilical endometriosis is a rare presentation accounting \nfor 0.5-1% of all extra-pelvic endometriosis [10]. Primary Um -\nbilical Endometriosis (PUE) coexisting with an umbilical hernia, \nas seen in our patient, is also a rare entity and poses a diagnos-\ntic challenge to the Surgeon, as it may mimic an obstructed or a \nstrangulated hernia, requiring a high index of suspicion to make \na diagnosis [5,10-12]. It frequently develops in previous umbili-\ncal scars by implantation or following surgical manipulations, \nbut very rarely presents as primary (spontaneous) umbilical en-\ndometriosis as seen in the case reported. Only a limited number \nof cases of PUE have been reported in the literature [3,13-15]. \nThe umbilicus is a physiological scar with a predilection to en -\ndometriosis [10]. Haematogenous and lymphatic spread to the \numbilicus and direct migration of endometrial cells through the \nround ligament or the remnants of the obliterated umbilical \nvessels may explain the occurrence of PUE [8]. Umbilical endo -\nmetrioma (nodule) as seen in our case was first described by \nVillar in 1886, the so-called “Villar’s nodule” and is the typical \npresentation in umbilical endometriosis [7,9,12]. Most cases of \nPUE will present with cyclical symptoms related to menstrua -\ntion. The accompanying pain may however be confused with \nthe pain of a recurrent obstructed or strangulated hernia in a \npatient previously diagnosed with a hernia, especially where \nthere is no visible bleeding or discharge from the umbilicus, \nand the patient had not had any abdominal surgery or prior \ngynaecological evaluation. This was the diagnostic difficulty \nwe encountered in the case reported. Recognised differential \ndiagnoses of umbilical endometriosis include an umbilical cyst, \nabscess, melanoma, lipoma, suture granuloma or a deposit of \nsystemic malignancy [13]. Victory et al. [12] reported that 90% \nof cases of umbilical endometriosis will present with umbili -\ncal swelling, less than 50% will present with bleeding, and 80% \nwith cyclical pain resulting from tissue inflammation, distension \nand cyclical changes, while up to 25% occur with pelvic endo -\nmetriosis. There is usually discolouration of the nodule due to \nbleeding into the lesion with deposition of haemosiderin which \ncan be demonstrated on histopathological examination as seen \nin our patient and shown in Figure 1.\nIn a case review series of five African patients, Makena et al. \n[11] reported an age range of 31-47 months, while duration of \nsymptoms ranged between 3 months and 60 months. All the \npatients presented with umbilical swelling and pain with lesions \nranging from 1.6 cm to 4 cm in diameter. Their findings were \nconsistent with those of our patient who was 34 years of age \nand presented with a painful umbilical swelling of 48 months \nduration and measuring 3 cm by 4 cm. A similar review by Bin -\ndra et al. [14] and Adewole et al. [15] showed similar findings. \nAlthough laparoscopy is the traditional method of diagnosis \nbased on the accurate visual identification of endometriotic le-\nsions, this is only applicable to pelvic endometriosis. Diagnosis \nin our patient was mainly based on clinical suspicion. Ultra -\nsound imaging was useful in the preoperative evaluation of the \nnodule and assessment for involvement of surrounding tissues \nand to exclude other pelvic pathology. The diagnosis was how -\never confirmed by histopathological examination of the excised \nnodule. MRI has been recommended as the imaging technique \nof choice for diagnosis of endometriosis [5]. This imaging tech -\nnique is not usually readily available in low-resource setting like \nours and where available the cost may be a challenge. It was not \nused in our patient. Surgical excision of the umbilical lesion with \na hernia repair is considered the preferred treatment option \nand this was offered to our patient (Figure 2). All the reported \ncases in the literature had surgical management [3,11,14]. The \nsurgical option also helped in providing tissue for histological \ndiagnosis since the diagnosis was only a differential preopera -\ntively.\nThe histopathological appearance of an endometriosis sec -\ntion is usually characterized by irregular glandular lumina that \nare embedded in the stroma with an elevated vascular and cel-\nlular component similar to the stroma of the functional endo -\nmetrium [6]. The histological findings of haemosiderin-laden \nmacrophages, infiltrates of lymphoplasmatic inflammatory cells \n(Figure 3) and intervening dense fibrotic stroma with hyperplas-\ntic overlying epidermis in the case presented tend to point to \nlymphatic migration of endometrial cells as the probable aetio-\nlogical factor. This further highlights the importance of always \npreserving surgical specimens for histopathological evaluation \nto aid in diagnosis, especially where preoperative diagnosis is \na challenge.\n Other treatment options include use of hormonal therapy \nlike Gonadotropin-Releasing Hormone (GnRH) analogues or Da-\nnazol® which can be used preoperatively for relief of symptoms \nand to reduce the size of the lesion [1]. Their prolonged use is \nhowever associated with side effects. Our patient was referred \nto the Gynaecologists for further gynaecological evaluation for \nany associated pelvic endometriosis and follow-up.\nConclusion\nPUE mimicking a strangulated umbilical hernia is a rare en -\ntity presenting a preoperative diagnostic challenge. A high index \nof suspicion and adequate clinical evaluation of a woman with \na painful umbilical nodule, with specific enquiry on the cycli -\ncal nature of the pain and its association with her menstrual \ncycle, is key to the diagnosis of umbilical endometriosis in the \npresence of an umbilical hernia. Surgical excision with umbilical \nherniorrhaphy and histopathological examination of the surgi -\ncal specimen are important management strategies.\nDeclarations\nConflict of interest: The authors declare no conflict of inter-\nest.\nFinancial support and sponsorship: Nil\nConsent: The authors certify that a written consent was ob -\ntained from the patient to publish the clinical images and this \ncase report.\nAcknowledgement: We are grateful to the Departments of \nSurgery and Histopathology, Maitama District Hospital, Abuja, \nNigeria, for allowing access to the records of the patient.\nReferences\n1. Clark TJ. Benign conditions of the ovary and pelvis. 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