• Indian ocean islands 
• Pregnancy 
• episiotomy site scar endometriosis
• genital (female) 
• vaginal birth with episiotomy

• Humans
• Female
• Endometriosis/complications
• Endometriosis/diagnosis
• Adult
• Episiotomy/adverse effects
• Cicatrix
• Perineum/pathology
• Ultrasonography

• Comparison
• Cutaneous
• Endometriosis
• Primary cutaneous endometriosis
• Secondary cutaneous endometriosis

• abdominal wall endometriosis
• perineal scar endometriosis
• scar endometriosis

Objective: To describe the available knowledge on vulvo-perineal endometriosis including its diagnosis, clinical management and recurrence rate.

Methods: We followed the PRISMA guidelines for Systematic Reviews and our study was prospectively registered with PROSPERO (CRD42020202441). The terms “Endometriosis” and “Perineum” or “Vulva” were used as keywords. Cochrane Library, Medline/Pubmed, Embase and Clinicaltrials.gov were searched. Papers in English, Spanish, Portuguese, French or Italian from inception to July 30, 2020 were considered. Reference lists of included articles and other literature source such as Google Scholar were also manually scrutinized in order to identify other relevant studies. Two independent reviewers screened potentially eligible studies according to inclusion criteria.

Results: Out of 539 reports, 90 studies were eligible including a total of 283 patients. Their mean age was 32.7 ± 7.6 years. Two hundred sixty-three (95.3%) presenting with vulvo-perineal endometriosis have undergone either episiotomy, perineal trauma or vaginal injury or surgery. Only 13 patients (4.7%) developed vulvo-vaginal endometriosis spontaneously i.e., without any apparent condition favoring it. The reasons that motivated the patients to take medical advice were vulvo-perineal cyclical pain increasing during menstruations (98.2% of the patients, n = 278). Out of the 281 patients for whom a clinical examination was described, 274 patients (97.5%) showed a vulvo-perineal nodule, mass or swelling while six presented with bluish cutaneous lesions (2.1%) and 1 with bilateral polyps of the labia minora (0.4%). All but one patients underwent surgical excision of their lesions but only 88 patients (28.1%) received additional hormonal therapy. The recurrence rate was 10.2% (29 patients) considering a median follow-up period of 10 months (based on 61 studies).

Conclusion: In conclusion, vulvo-perineal endometriosis is a rare entity with approximately 300 cases reported in the literature since 1923. With the available knowledge shown in this systematic review, we encourage all practitioners to think about perineal endometriosis in case of perineal cyclical pain with or without previous perineal damage. Diagnosis should be done with clinical exam, perineal ultrasound and pelvic MRI when available. In case of anal sphincter involvement, perianal ultrasound should be performed. Surgical excision of the lesion should be realized in order to remove the lesion and to confirm the diagnosis histologically. Hormonal treatment could be proposed to attempt to decrease the size of a large lesion before surgery or to avoid recurrence of the lesion. As evidence-based approach to the diagnosis, treatment and recurrence rate of affected patients remains a challenge given its low prevalence, the variations in management found in the articles included and the limited quality of available studies, we suggest that a prospective database on vulvo-perineal endometriosis should be generated to increase knowledge but also awareness among healthcare professionals and optimize patients' care.

Systematic Review Registration:https://www.crd.york.ac.uk/prospero/, identifier: CRD42020202441.

• cyclical pain
• endometriosis
• episiotomy
• extrapelvic endometriosis
• perineal nodule
• perineal pain
• perineum
• vulva

The prevalence of perineal endometriosis (PEM) is low among women with endometriosis (EM) treated by surgery. It manifests as hard or cystic nodules with pain in the perineal wounds and surrounding areas. Implantation theory is regarded as the main pathogenesis of PEM. There are few clinical studies on the incidence and clinical characteristics of PEM. This study aims to summarize the clinical data of 14 PEM cases and analyze the factors that may be related to the incubation period and pain.

To analyze the medical history, clinical manifestations, diagnosis, treatment and treatment effect of PEM.

The present study is a case series. We collected the clinical data and follow-up data of 14 patients with PEM who visited The International Peace Maternal and Child Health Hospital Affiliated to Shanghai Jiaotong University from January 2009 to December 2019. Paired t test and Pearson correlation analysis were used for statistical analysis. P < 0.05 was considered statistically significant.

The 14 patients included had a history of vaginal delivery. All patients underwent PEM lesion resection. Three patients were treated by levator ani muscle repair at the same time and 1 patient underwent extensive PEM lesion resection and anal sphincter repair. Body mass index (BMI) at delivery and BMI within 1 mo after delivery were negatively correlated with the latent period, respectively (R² = 0.53/0.86, P < 0.05). The average visual analog scale score in lesions at the third month after surgery was 0.57 ± 1.28 for all patients, which was significantly lower than that prior to surgery (P < 0.05). One patient relapsed during the sixth month after surgery, and to date, no recurrence occurred after the second surgery.

The higher the BMI during delivery and within 1 mo after delivery, the shorter the incubation period of PEM. It is very important to evaluate the location of lesions before surgery. Surgical resection of the lesion is the best treatment for PEM and results in significant alleviation of symptoms. Therefore, following the diagnosis of PEM, immediate surgery is recommended.

• Perineal endometriosis
• Incubation period
• Pain in the perineum
• Surgery
• Body mass index
• Clinical characteristics

Endometriosis is the presence of endometrial tissue in abnormal locations outside the uterine cavity. These locations are usually the ovaries, the peritoneum, and the uterine ligaments. Less frequently, the endometrial tissue can affect the perineum especially after surgical procedures or obstetric lesions. In this case report, we describe the case of a patient suffering from pain and swelling, with a sizeable nodule in an old episiotomy scar. Clinical examination, ultrasonography (USG) and magnetic resonance imaging (MRI) helped evoke the diagnosis of perineal endometriosis, and there were no signs of other endometriosis locations. Hormonal treatment was provided at first, but there was no clinical improvement after three months, so the treatment had to be surgical. Histopathological examination of the endometriotic mass confirmed the diagnosis. There were no immediate postoperative complications, and no clinical symptoms or recurrence signs six months and one year after.

• Perineal
• case report
• endometriosis
• sizeable
• surgical

A 23-year-old patient with an asymptomatic anterior vaginal wall cyst was referred to gynecology for evaluation and treatment. Preoperative assessment with physical examination and magnetic resonance (MR) imaging of the pelvis was most consistent with Gartner’s cyst. Following resection of the cyst wall, histologic evaluation demonstrated endometrial glands, hemosiderin-laden macrophages and inflammation, consistent with vaginal wall endometrioma. This case highlights challenges in the diagnosis of endometriosis in the vagina and in other rare locations, possible mechanisms of development, and proposed treatments.

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Endometriosis implants can occur widely throughout the body, but rarely in the vagina.

• Endometrioma
• Endometriosis
• Vagina