Umbilical endometriosis represents 30–40% of abdominal wall endometriosis and around 0.5–1.0% of all cases of endometriosis. The aim of this systematic review is to revisit the epidemiology, signs, and symptoms and to formulate a pathogenic theory based on literature data. We performed a systematic literature review using the PubMed and Embase databases from 1 January 1950 to 7 February 2021, according to the PRISMA guidelines. The review was registered at PROSPERO (CRD42021239670). Studies were selected if they reported original data on umbilical endometriosis nodule defined at histopathological examination and described as the presence of endometrial glands and/or stromal cells in the connective tissue. A total of 11 studies (10 retrospective and one prospective), and 14 case series were included in the present review. Overall, 232 umbilical endometriosis cases were reported, with the number per study ranging from 1 to 96. Umbilical endometriosis was observed in 76 (20.9%; 95% CI 17.1–25.4) of the women included in studies reporting information on the total number of cases of abdominal wall endometriosis. Umbilical endometriosis was considered a primary form in 68.4% (158/231, 95% CI 62.1–74.1) of cases. A history of endometriosis and previous abdominal surgery were reported in 37.9% (25/66, 95% CI 27.2–49.9) and 31.0% (72/232, 95% CI 25.4–37.3) of cases, respectively. Pain was described in 83% of the women (137/165, 95% CI 76.6–88.0), followed by catamenial symptoms in 83.5% (142/170, 95% CI, 77.2–88.4) and bleeding in 50.9% (89/175, 95% CI 43.5–58.2). In the 148 women followed for a period ranging from three to 92.5 months, seven (4.7%, 95% CI 2.3–9.4) recurrences were observed. The results of this analysis show that umbilical endometriosis represents about 20% of all the abdominal wall endometriotic lesions and that over two thirds of cases are primary umbilical endometriosis forms. Pain and catamenial symptoms are the most common complaints that suggest the diagnosis. Primary umbilical endometriosis may originate from implantation of regurgitated endometrial cells conveyed by the clockwise peritoneal circulation up to the right hemidiaphragm and funneled toward the umbilicus by the falciform and round liver ligaments.

Although dyspareunia (pain during intercourse) is common in women with endometriosis, there is poor qualitative evidence describing women’s subjective experience of this symptom. This systematic review of qualitative research aimed to provide an in-depth exploration of women’s lived experience of dyspareunia (i.e., how they perceive and describe their pain, how they deal with it, how it affects their psychological health and intimate relationships). A total of 17 published articles were included. Our findings, derived from thematic analysis, highlighted that endometriosis-related dyspareunia manifests itself in multiple forms (deep, introital, and/or positional dyspareunia, at orgasm, during and/or after intercourse). Women use a variety of coping strategies to deal with sexual pain, such as interrupting or avoiding intercourse, enduring pain to seek pregnancy, and/or finding alternative ways to enjoy sexuality. Dyspareunia impairs women’s psychological health, especially in terms of poor self-esteem and sense of femininity and has negative consequences on intimate relationships. Unfortunately, both women and physicians are often reluctant to discuss sexual issues. Sexual health should be routinely assessed during counselling with endometriosis patients. Helping women find targeted strategies to enjoy sexuality despite endometriosis may significantly improve their psychological health and quality of life.