{"paper_id":"5f33f2b6-4efc-4c37-9b76-28aaf2961e19","body_text":"INTERNATIONAL JOURNAL OF HEALTH & MEDICAL RESEARCH \nISSN(print): 2833-213X, ISSN(online): 2833-2148 \nVolume 03 Issue 08 August 2024 \nDOI : 10.58806/ijhmr.2024.v3i08n12 \nPage No. 605-607 \nIJHMR, Volume 3 Issue 08 August 2024                  www.ijhmr.com                                                 Page 605 \nSymptomatic Primary Umbilical Endometriosis: A Case Report and A \nReview of the Literature \n \nBACHAR Amine 1, JAMALEDDINE Khalid 2, MASTAR Hajar 3, ESSAIDI Zakaria 4, ELABBASSI Taoufik 5, \nLEFRIYEKH Mohamed Rachid6 \n1,2,3,4,5,6 Department of general surgery, IBN ROCHD University hospital of Casablanca, Casablanca, Morocco  \n \nABSTRACT: Endometriosis is a condition defined as extrauterine functional endometrial tissue, typically seen on pelvic peritoneal \nsurfaces, leading to symptoms such as cyclic pain, dysmenorrhea, dyspareunia, and infertility. Extra -pelvic endometriosis is \nuncommon, especially the umbilical form. We present an interesting case of primary umbilical endometriosis (Villar’s nod ule) in a \npatient with no medical or surgical history, who presented with umbilical pain and bleeding. She was found to have a bluish umbilical \nnodule, which was surgically removed and sent to the pathology to confirm the diagnosis. The report aims to highlight the diagnosis \nand management of umbilical endometriosis and the possibility of its occurrence even in patients without prior surgical histo ry. \nKEY WORDS: Primary Umbilical endometriosis – Umbilical bleeding – Cyclic umbilical swelling – Surgical excision \n \nINTRODUCTION  \nEndometriosis (EM) is a benign gynecological disorder [1] characterized by the presence of functional endometrial tissue outside \nthe uterus. It frequently affects the pelvic peritoneum, ovaries, or rectovaginal septum. Symptoms vary based on the affected organs \nand inflammation level but typically include dysmenorrhea and dyspareunia, with about 20–25% of cases being asymptomatic. This \nchronic condition impacts 6 –15% of women of reproductive age and 6% of postmenopausal women, with infertility occurring in \n30–50% of affected individuals [2]. \nEndometriosis in extra-pelvic sites is rare, with less common locations including the diaphragm, lungs, and anterior abdominal wall \n[3]. Among patients with endometriosis, 0.5 –1% develop umbilical endometriosis, which can be primary or secondary. Secondary \numbilical endometriosis arises post-laparoscopic procedures, while primary umbilical endometriosis (PUE), first identified by Villar \nin 1886, is particularly rare [2]. \nHere, we present the case of a 36-year-old nulliparous woman with primary umbilical endometriosis. \n \nPRESENTATION OF CASE \nA 36-year-old nulliparous woman with no comorbidities or history of abdominal or pelvic surgeries presented to the emergency \nroom with dysmenorrhea and a sharp, throbbing pain originating from a swelling, bleeding, bluish nodule in the umbilicus (figure \n1). The pain began five days prior to admission, and the bleeding started on the day of admission. Her last menstrual period began \nfour days before admission, and she reported that this condition had been recurring for four years, with symptoms worsening during \nmenstruation. She did not take oral contraceptive pills and found that analgesics only provided temporary relief. The patient denied \nother symptoms such as umbilical/abdominal trauma, fever, chills, vomiting, diarrhea, nor constipation.  \nUpon admission, her vital signs were within normal limits. Clinical examination revealed multiple ill -defined bluish papules \nsurrounded by erythematous skin in  the inferior aspect of the umbilicus. A gynecological evaluation was normal. Laboratory tests \nshowed a hemoglobin level of 11.2 g/dL, a platelet count of 264,000/mm³, and a negative blood β -hCG. \n \n \n \n \n \n \n \n\nSymptomatic Primary Umbilical Endometriosis: A Case Report and A Review of the Literature \nIJHMR, Volume 3 Issue 08 August 2024                  www.ijhmr.com                                                 Page 606 \n \n \n   \n \n \n \n \n \n \n \n \nFigure 1: initial physical examination showing 1.5-cm size endometrial implant on the inferior aspect of the umbilicus. \n \nAn abdominal wall ultrasound revealed a small collection in the umbilical region with central necrosis, extending 27x10x9 mm,  \nwith a rounded edge and a fistulous path measuring 2.5 mm in diameter and 10 mm in length, limited to the parietal fatty layer. The \ndiagnosis of endometriosis was suggested based on the cyclical nature of her symptoms.  \nA pelvic MRI showed a normal -sized uterus, ovaries, and vaginal tract with no endomet rial or cervical thickening and no \nlaterouterine mass. It revealed an oblong lesion in the pouch of Douglas, measuring 7.4x6 mm, with T2 hypersignal and gadolinium \nenhancement, attaching the uterus to the rectum. Additionally, subcutaneous thickening in th e umbilical area with gadolinium \nenhancement measured 14.4x24 mm. These findings suggested endometriosis (figure 2).  \n \n \n \n \n \n \n \n \n \n  \n \n  \n \n \n \n \n \n \nFigure 2: Pelvic MRI images of the patient showing a subcutaneous hypersignal T2 (red arrow and red circle), referring to \nendometriosis in the umbilical area. \n \nThe decision was made to treat the affected tissue with radical local excision and umbilical reconstruction. The surgery, per formed \nat the end of the menstrual cycle, involved excising the nodule with a 1-cm safety margin up to the rectus fascia and performing an \numbilicoplasty to close the umbilical defect. Pathology confirmed the presence of endometrial implants within the umbilical m ass. \nAt the postoperative visit, the patient reported satisfaction with the results. She was applying a topical antibiotic and healing cream \nto the umbilicus as instructed. Clinical examination showed excellent healing of the umbilicoplasty.  \n \nDISCUSSION  \nEndometriosis is a benign gynecological disorder characterized by the presence of endometrial tissue outside the uterus, often on \npelvic organs such as the ovaries, fallopian tubes, pelvic peritoneum, uterosacral ligaments, and broad ligaments. Approximat ely \n12% of endometriosis lesions are extragenital, occurring in locations such as the brain, lungs, gastrointestinal tract, urinary system, \nand musculature. Among extragenital sites, the abdominal wall (AWE) is the most common location for endometriosis.  \nUmbilical endometriosis (UE) involves the presence of endometrial glands  and/or stroma within the umbilicus. It is a rare form of \nendometriosis, accounting for 30-40% of AWE cases, 0.4-4% of extragenital lesions, and 0.5-1% of all endometriosis cases [1], [4], \n[5]. \n\nSymptomatic Primary Umbilical Endometriosis: A Case Report and A Review of the Literature \nIJHMR, Volume 3 Issue 08 August 2024                  www.ijhmr.com                                                 Page 607 \nThere are two types of UE: \n Primary UE: First described by Villar in 1886, it is also known as Villar’s nodule [6]. Primary UE occurs without a history of \nsurgery. Its pathogenesis is unclear, but theories include coelomic metaplasia, c ongenital presence of displaced endometrial \ntissue, direct extension through the round ligament or the patent omphalo -mesenteric duct, and mechanical seeding of \nendometrial tissues via the lymphatic or venous system. \n Secondary UE: This type develops on scar tissue following abdominal procedures such as laparoscopy. \nUnderstanding the distinction between primary and secondary UE is important for comprehending the disease's pathogenic \nmechanisms. According to Hirata et al. (2020), the risk of malignant transformation of UE is about 3% [2], [5]. \nDue to its low frequency, limited data are available on the prevalence of primary and secondary UE and the associated symptom s \n[1]. Diagnosis of umbilical endometriosis is primarily clinical, with histopathology confirming the diagnosis [3]. Common clinical \nsymptoms include cyclic, menstrual-related focal abdominal pain, swelling, and bleeding [2]. UE typically presents as a red, purple, \nor black umbilical nodule, measuring between 0.5 and 3 cm in diameter [5]. \nImaging techniques can improve diagnostic accuracy: \n- Ultrasound: Although not specific for an abdominal endometrioma, it may show a mass in the abdominal wall that appears \nsolid, hypoechoic, and may contain internal vascularity and cystic areas. The differential diagnosis includes neoplasms (sarcoma \nor lymphoma), suture granuloma, ventral hernia, abscess, or hematoma. \n- CT and MRI: These modalities also show a solid mass in the abdominal wall and are useful for characterizing the extent of the \ndisease/mass preoperatively, though they cannot definitively diagnose endometriosis. \nWhile ultrasound-guided fine needle aspiration can provide a definitive diagnosis, wide surgical excision is recommended for both \ndiagnosis and treatment of an abdominal wall mass [4].  \nSurgery may involve resection of the umbilical mass with wide local excision (en -bloc omphalectomy) and, if possible, evaluation \nfor pelvic endometriosis via laparoscopy. Surgical treatment is strongly recommended despite limited evidence on long -term \nefficacy and complications. \nMedical treatment, such as combined oral contraceptives or progestins to reduce the stimulation and inflammatory effects of \nendometriotic implants, is weakly recommended due to limited supporting data and a lack of comparative studies bet ween medical \nand surgical treatments for umbilical endometriosis [1], [3]. \n \nCONCLUSION \nThe umbilical endometriosis remains a very uncommon condition. However, it should be considered in the differential diagnosis of \numbilical lesions. Our case report is an opportunity to highlight the importance of recognizing UE whenever a case of umbilical \nswelling or bleeding and cyclic pain occurs. It is not mandatory, as depicted in our patient, to have a surgical history.  \n \nREFERENCES \n1) D. Dridi et al., “Umbilical Endometriosis: A Systematic Literature Review and Pathogenic Theory Proposal,” JCM, vol. \n11, no. 4, p. 995, Feb. 2022, doi: 10.3390/jcm11040995. \n2) D. Boesgaard -Kjer, D. Boesgaard -Kjer, and J. J. Kjer, “Primary umbilical endometrio sis (PUE),” European Journal of \nObstetrics & Gynecology and Reproductive Biology, vol. 209, pp. 44 –45, Feb. 2017, doi: 10.1016/j.ejogrb.2016.05.030. \n3) I. Ogamba, S. Napolitano, L. Chuang, D. August, and K. LaVorgna, “Primary umbilical endometriosis presentin g with \numbilical bleeding: A case report,” Case Reports in Women’s Health, vol. 36, p. e00441, Oct. 2022, doi: \n10.1016/j.crwh.2022.e00441. \n4) C. Davies, A. M. Davies, L. -G. Kindblom, and S. James, “Soft Tissue Tumors with Muscle Differentiation,” Semin \nMusculoskelet Radiol, vol. 14, no. 02, pp. 245–256, Jun. 2010, doi: 10.1055/s-0030-1253165. \n5) A. Hamouie, E. Brunn, J. Orzel, S. R. Shehr, and J. K. Robinson, “Joint Treatment of De Novo Umbilical Endometriosis \nwith Plastic Surgery and Minimally Invasive Gynecologic Surgery”. \n6) D. Makena, T. Obura, S. Mutiso, and F. Oindi, “Umbilical endometriosis: a case series,” J Med Case Reports, vol. 14, no. \n1, p. 142, Dec. 2020, doi: 10.1186/s13256-020-02492-9.","source_license":"CC0","license_restricted":false}