Diagnosis and Treatment of Vulvo-Perineal Endometriosis: A Systematic Review

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.

Perineal Endometriosis: What to Do in These Cases - Analysis of 13 Patients

Objective: To analyse the clinical features of perineal endometriosis (PEM), its treatment and outcome. Methods: Prospective, single-centre study with 13 patients with PEM who were treated between 2011-2018 at Domingo Luciani Hospital and mean followed up for 58.4 months. Results: Mean age was 32,2 years. All cases had a history of vaginal delivery with an episiotomy. All complained of perineal pain related to the menstrual cycle; the perineal mass progressively increased in size and was tender during menstrual periods. Mean VAS was 7. 69,2% with rectal bleeding. The mean size of the lesion was 3.42 cm. CA125 levels were measured in all patients, 3 (23,1%) with abnormal range; all patients were subjected to transvaginal, endoanal ultrasonography (EUS) and FNAB before surgery. Anal sphincter (AS) involvement was demonstrated by EUS in 46.2% (6). Mean EUS pre-treatment volume 18.98 ml. First, these 6 patients received hormonal therapy based on GnRH and evaluated response. Mean EUS post-treatment volume 10.21 ml p < 0.05. Complete local excision was performed on all cases. Mean CCFIS preoperative was 2.46 and postoperative 3.01 p=0.01. No major complications or recurrences were noted. Conclusion: PEM presents with typical clinical features when it involves the AS, it could benefit from first a hormonal therapy before surgery. EUS is a useful preoperative tool to decide what we should do. The main idea at the time of surgery is performed a complete local excision with non-touch AS, and in cases where these aren’t possible, a sphincteroplasty is mandatory with good continence results, minor complications and no recurrences.

Ultrasonography and Atypical Sites of Endometriosis

In the present pictorial we show the ultrasonographic appearances of endometriosis in atypical sites. Scar endometriosis may present as a hypoechoic solid nodule with hyperechoic spots while umbilical endometriosis may appear as solid or partially cystic areas with ill-defined margins. In the case of endometriosis of the rectus muscle, ultrasonography usually demonstrates a heterogeneous hypoechogenic formation with indistinct edges. Inguinal endometriosis is quite variable in its ultrasonographic presentation showing a completely solid mass or a mixed solid and cystic mass. The typical ultrasonographic finding associated with perineal endometriosis is the presence of a solid lesion near to the episiotomy scar. Under ultrasonography, appendiceal endometriosis is characterized by a solid lesion in the wall of the small bowel, usually well defined. Superficial hepatic endometriosis is characterized by a small hypoechoic lesion interrupting the hepatic capsula, usually hyperechoic. Ultrasound endometriosis of the pancreas is characterized by a small hypoechoic lesion while endometriosis of the kidney is characterized by a hyperechoic small nodule. Diaphragmatic endometriosis showed typically small hypoechoic lesions. Only peripheral nerves can be investigated using ultrasound, with a typical solid appearance. In conclusion, ultrasonography seems to have a fundamental role in the majority of endometriosis cases in “atypical” sites, in all the cases where “typical” clinical findings are present.

A rare case of perineal endometriosis with anal sphincter involvement

Perineal endometriosis is a rare entity which can be explained by direct implantation of endometriotic cells over the fresh episiotomy wound and subsequent development of scar endometriosis. Perineal scar endometriosis incidence is reported to be 0.3% to 1%. 28 years old, P1L1, presented with pain and swelling near episiotomy site which is associated with menstruation. Examination during menstruation revealed swelling was tender, erythematous and slightly increased in size. Clinical diagnosis of scar endometriosis was made after clinical examination. Mass excised and sent for histopathological examination. Although diagnosis essentially remains clinical, preoperative evaluation with perineal ultrasound and MRI was performed. Wide local excision remains treatment of choice and follow up for recurrence is recommended. Histopathological examination is obligatory to exclude rare possibility of malignant changes.

Episiotomy site endometriosis: a rare case report

Endometriosis is defined as the presence of endometrial tissue apart from its usual location. Scar endometriosis is an infrequent type of extra pelvic endometriosis. Viable decidual endometrial cells are likely to be transplanted in the episiotomy wounds during normal delivery and subsequent growth may occur. We present here a case of 22 year old female with perineal endometriosis at the episiotomy site. The clinical, operative and pathological findings are reported.