Reflectance confocal microscopy features of vulvar lichen sclerosus in Chinese juvenile girls.

Vulvar lichen sclerosus (VLS) is a chronic inflammatory dermatosis of unknown pathogenesis, characterized by porcelain-white atrophic plaques around the vulvar and anal areas in girls. With this communication, we performed the study on 16 female girls with clinically and histologically confirmed VLS, described the main identifying characteristics of the lesions in reflectance confocal microscopy (RCM) and elucidated the corresponding relationship between RCM findings and histology. We recommend RCM, a noninvasive technique, as a complementary diagnostic tool for VLS.

Magnetic resonance imaging features of a solitary fibrous tumor of the vulva: a case report.

Solitary fibrous tumors (SFTs) are commonly seen in the pleura. SFT involvement of the vulva is rare, and clinical diagnosis is mainly based on histopathological and immunohistochemical analyses. We herein describe the imaging features of a 69-year-old woman with an SFT of the vulva. The SFT was hypointense on T1-weighted images, similar to muscle; however, it showed inhomogeneous hyperintensity predominantly on fat-suppressed T2-weighted images. An area of low signal intensity was evident on T2-weighted images, and the tumor showed progressive enhancement in delayed phases. The tumor also displayed heterogeneous and prolonged, persistent enhancement, and serpentine vessels were present in the peritumoral area as signal voids. Pathological examination confirmed that the lesion was an atypical SFT originating from the vulva, and it was composed of spindle cells and perivascular and stromal hyalinization. This case reveals the characteristic imaging findings of vulvar SFT and their association with the relevant pathological findings, thus contributing to the primary diagnosis and preoperative evaluation of this potentially aggressive tumor.

Portable three-dimensional imaging to monitor small volume enhancement in face, vulva, and hand: A comparative study.

Multiple handheld three-dimensional (3D) systems are available on the market, but data regarding their use in detecting small volumes are limited. The aim of this study was to compare different portable 3D technologies in detecting small volumetric enhancement on a mannequin model and a series of patients. Five portable 3D systems (Artec Eva, Crisalix, Go!Scan, LifeViz Mini, and Vectra H1) were tested in a controlled environment with standardised volumes and in a clinical setting with patients undergoing small volume fat grafting to face, vulva, and hand. Accuracy was assessed with absolute and relative technical error measurement (TEM and rTEM); precision with intra- and inter-observer reliability (rp and ICC); and usability in clinical practice with the following parameters: portability, suitability of use in operating theatre/clinic, ease of use of hardware and software, speed of capture, image quality, patient comfort, and cost. All tested devices presented overall good accuracy in detecting small volumetric changes ranging from 0.5 to 4 cc. Structured-light laser scanners (Artec Eva and Go!Scan) showed high accuracy, but their use in clinical practice was limited by longer capture time, multiple wiring, and complex software for analysis. Crisalix was considered the most user-friendly, less bothering for patients, and truly portable, but its use was limited to the face because the software does not include vulva and hand. Three-dimensional technologies exploiting the principle of passive stereophotogrammetry such as LifeViz Mini and Vectra H1 were the most versatile for assessing accurately multiple body areas, representing overall the best long-term value for money. Therefore, 3D portable technology is a non-invasive, accurate, and reproducible method to assess the volumetric outcome after facial, vulval, and hand injectables. The choice of the 3D system should be based on the clinical need and resources available.

Study on the application and imaging characteristics of optical coherence tomography in vulva lesions.

In this study, a prospective study was conducted by using optical coherence tomography (OCT) in the in vivo detection of vulvar diseases. The clinical efficacy of the OCT we investigated in the detection of vulvar diseases, and the characteristics of the OCT images were defined. Overall, this study recruited 63 patients undergoing the colposcopy for vulvar lesions in three Chinese hospitals from December 20th, 2018 and September 24th, 2019. The colposcopy and the OCT examination were performed successively, and the OCT images were compared with the relevant tissue sections to characterize different lesions. The OCT diagnoses where categorized into 7 types, including normal and inflammatory vulva, condyloma acuminata, papilloma, lichen sclerosus, atrophic sclerosing lichen, fibrous epithelial polyp as well as cysts. The structural characteristics of the vulva tissue can be clearly observed in the OCT image, which are consistent with the characteristics of the tissue section. Compared with the pathological results, the sensitivity, specificity and accuracy of the OCT examination reached 83.82% (95% confidence interval, CI 72.5%-91.3%), 57.89% (95% CI 34.0%-78.9%) and 78.16%, respectively. The OCT is found with the advantages of being noninvasive, real-time and sensitive and with high resolution. It is of high significance to screening vulva diseases, and it is expected as one of the methods to clinically diagnose vulva diseases.

Multizonal anogenital neoplasia in women: a cohort analysis.

BACKGROUND: There is currently a lack of information on full anogenital evaluation of women with a previous history of anogenital neoplasia. METHODS: Retrospective analysis of the Homerton Anogenital Neoplasia Service records from January 2012 to March 2017, to identify all new referrals of women with previous anogenital neoplasia, who had had at least one complete examination of all anogenital sites. Multizonal anogenital disease (MZD) was defined as the presence of high-grade squamous intraepithelial lesions (HSIL)/carcinoma concurrently at two or more of the following sites/zones: perianus, anal canal, vulva, vagina or cervix. RESULTS: 253 women were included, mean age was 47 (SD=15) years and median duration of follow-up was 12 (IQR=21) months. Fifty-six women (22%) were diagnosed with MZD at first assessment and/or during follow-up. Current smokers (RR=1.84, 95% CI 1.21-2.79, p=0.004) and women on immunodulators/immunosuppressive drugs (RR=2.57, 95% CI 1.72-3.86, p<0.001) had an increased risk for MZD. The risk was lower for women without a previous history of anogenital high-grade lesions/cancer compared to those with this history (RR=0.06, 95% CI 0.01-0.45, p=0.006). CONCLUSIONS: Multizonal assessment was important to diagnose occult areas of disease and should be especially considered in current smokers, pharmacologically immunocompromised and those with a previous history of anogenital HSIL/cancer.

Extraskeletal Myxoid Chondrosarcoma of the Vulva Confirmed by EWSR1 FISH: A Case Report and Review of the Literature.

Extraskeletal myxoid chondrosarcoma of the vulva is a very rare tumor, with less than 10 cases reported in the literature. We report a case of a 45-yr-old woman with extraskeletal myxoid chondrosarcoma of the vulva confirmed by EWSR1 fluorescence in situ hybridization. Given the unusual site and prominent myxoid morphology, a broad differential diagnosis and a variety of ancillary testing was required. This article aims to review extraskeletal myxoid chondrosarcoma of the vulva, the differential diagnosis of a myxoid spindle cell neoplasm of the vulva, and the diagnostic importance of immunohistochemistry and EWSR1 fluorescence in situ hybridization.

Cancer of the Vulva: A Review.

BACKGROUND: Vulvar cancers, although rare, are becoming an increasingly serious threat to women's health. Cancer of the vulva accounted for 0.3% of all new cancers in the United States in 2019, with 6,070 newly diagnosed cases. This review details the epidemiology, pathogenesis, diagnosis, staging, and treatment of vulvar malignancies. OBJECTIVE: To review cancer entities of the vulva, including vulvar intraepithelial neoplasms, squamous cell carcinoma (SCC), malignant melanoma, basal cell carcinoma, neuroendocrine tumors, and adenocarcinomas. MATERIALS AND METHODS: Literature review using PubMed search for articles related to cancer of the vulva. RESULTS: Vulvar intraepithelial neoplasms represent premalignant precursors to SCC of the vulva. There are several different histopathologic subtypes of SCC, and treatment is dependent on characteristics of primary tumor and lymph node involvement. Melanoma is the second most common cancer to affect the vulva, and staging is based on tumor, node, and metastatic spread. CONCLUSION: Vulvar malignancies are rare, and diagnosis is dependent on biopsy and pathologic evaluation. Treatment for vulvar malignancies depends on histopathologic diagnosis but ranges from wide local excision with or without lymph node biopsy or dissection to radiation therapy with chemo- or immunotherapy. Overall survival varies by diagnosis.

Is location of urethral kinking a confounder of association between urethral closure pressure and stress urinary incontinence?

OBJECTIVES: Intact urethral support and normal sphincter function are deemed important for urinary continence. We aimed to test whether the location of urethral kinking (as the probable anatomical correlate of maximal pressure transmission) is associated with stress urinary incontinence and/or urodynamic stress incontinence. METHODS: This was a retrospective study of women seen at a tertiary urogynecological center in 2017. Patients had undergone an interview, multichannel urodynamic testing and four-dimensional translabial ultrasound examination. Those with a history of anti-incontinence surgery, absence of urethral kinking on ultrasound and/or missing or inadequate ultrasound volume data were excluded. Volume data were used to assess urethral mobility using a semi-automated Excel® urethral motion profile program. Mobility vectors were calculated using the formula √((x valsalva - x rest )2 + (y valsalva - y rest )2 ), where x and y are the coordinates of six equidistant points along the length of the urethra from the bladder neck to the external urethral meatus. The location of urethral kinking was identified as a concave contour of the urethra on the vaginal side in the midsagittal plane on maximum Valsalva maneuver. The distance between the center of the kink and the bladder neck was measured and expressed as a centile in relation to the total length of the urethra, using the formula: (distance from bladder neck/total length of urethra) × 100. Univariate and multivariate analyses were performed to test the associations of stress urinary incontinence and urodynamic stress incontinence with age, maximum urethral pressure, urethral mobility vectors and location of urethral kinking. RESULTS: Of 450 women seen during the study period, 61 were excluded owing to previous incontinence surgery and 82 owing to absence of urethral kinking, inadequate volume data or missing data, leaving 307 women included, of whom 227 (74%) complained of stress urinary incontinence and 211 (69%) complained of urgency urinary incontinence. 190 (62%) of the women were diagnosed with urodynamic stress incontinence. On multivariate analysis, maximum urethral pressure (36 vs 50 cmH2 O; P < 0.001), mid-urethral mobility (2.27 vs 2.03 cm; P = 0.003) and location of urethral kinking (63.1st vs 59.7th centile; P = 0.002) were associated significantly with urodynamic stress incontinence. The location of urethral kinking was associated with stress urinary incontinence on univariate analysis (P = 0.026) but not on multivariate analysis (P = 0.21). CONCLUSIONS: The location of urethral kinking is associated with urodynamic stress incontinence. The further urethral kinking is from the mid urethra, the more likely is urodynamic stress incontinence. This provides circumstantial evidence for the pressure-transmission theory of stress urinary continence. © 2020 International Society of Ultrasound in Obstetrics and Gynecology.

[Labium majus enlargement in a 6-year old girl: benign or alarming?] / Zwelling van een labium majus bij een 6-jarig meisje.

A 6-year old girl was referred because she had a painless enlarged left labium majus. There were no further symptoms. Ultrasound and MRI revealed no hernias, malignancy or cystic abnormalities. We diagnosed CALME, Childhood Asymmetry Labium Majus Enlargement, a non-neoplastic expansion of normal vulvar tissue in response to hormonal changes. Expectant management is preferred.

Clinical and Dermoscopic Features of Vulvar Melanosis Over the Last 20 Years.

Importance: Vulvar melanosis is a common pigmentary change that accounts for most pigmented vulvar lesions. It presents as single or multiple asymptomatic macules or patches of varying size and color that may be asymmetric with poorly defined borders. The differential diagnosis of melanocytic lesions includes melanoma, which creates anxiety for patients and the physicians who diagnose the condition and treat the patients. Objective: To evaluate the clinical and dermoscopic features of vulvar melanosis and their changes over time. Design, Setting, and Participants: In this cohort study, patients with vulvar melanosis were recruited and followed up in the Department of Dermatology, University of Florence, Florence, Italy, between January 1, 1998, and June 30, 2019. Data on patient characteristics and on both the clinical and dermoscopic features of the vulvar lesions were collected. Each lesion was photographed clinically and dermoscopically at initial evaluation and at annual follow-up visits. Main Outcomes and Measures: The clinical, dermoscopic, and histopathologic features of vulvar melanosis and their changes over time. Results: This cohort study included 129 women (mean age at diagnosis, 46 years [range, 19-83 years]) with vulvar melanosis. A total of 87 patients (67%) with vulvar melanotic lesions were premenopausal, and 84 patients (65%) had received some type of hormone therapy. The most frequent location for vulvar melanosis was the labia minora (55 [43%]), followed by the labia majora (33 [26%]). In 39 of 129 cases (30%), the lesions increased in size and changed color after initial evaluation but ultimately stabilized. No malignant evolution was documented in any patient during a median follow-up of 13 years (range, 5-20 years). Conclusions and Relevance: This study suggests that vulvar melanosis was a benign entity, and changes in lesions over time did not signify malignant transformation. An association between hormonal status and vulvar melanosis may be hypothesized.

A Case of Recurrent Vulvar Carcinoma With Cardiac Metastasis: Case Report and Review of the Literature.

A 67-yr-old female patient was diagnosed with squamous cell vulvar carcinoma and treated with a radical vulvectomy and bilateral sentinel lymphadenectomy. Three months after the surgery, the patient presented with local recurrence and underwent surgical excision of the mass, followed by chemotherapy. Eight months later, the patient was admitted due to weakness and pleural effusion. The patient underwent a chest computed tomography and echocardiogram, which revealed a large mass in the right ventricle penetrating into the pericardium and an additional mass residing on the tricuspid valve. She underwent a pericardial biopsy, and the pathology revealed a moderately differentiated squamous cell carcinoma metastasis. The patient was admitted thereafter in the oncological department for additional chemotherapy treatment. Because of a rapid deterioration in the patient's condition, only palliative treatment was given, and the patient died shortly after. Secondary cardiac tumors are very rare and have not been extensively studied in oncology. Therefore, optimal management is not entirely clear. It is extremely rare for vulvar cancer to metastasize to the heart, and only a handful of cases have been reported in the literature.

Spontaneous symmetrical giant keloids at the bilateral labia majora: a case report.

Keloids are caused by an imbalance between collagen matrix decomposition and production during wound repair. Spontaneous keloids are a rare type of keloid that arise without a significant history of trauma or surgery. We herein report a case involving a 59-year-old woman with symmetric neoplasms at the bilateral labia majora that had persisted for about 3 years and a >10-year history of pruritus and pain at the bilateral labia majora with folliculitis. The bilateral labia majora gradually swelled and lost their normal physiological morphology. The patient was diagnosed with keloids. Under general anesthesia, the keloids were resected. The right incision was sutured directly and the left was closed using an inguinal flap. Adjuvant radiotherapy was administered after the surgery. No recurrence of the keloids was observed at the 1-year follow-up, and the patient was satisfied with the appearance of the vulvar region. This is the first report of spontaneous symmetrical giant keloids at the bilateral labia majora, and it emphasizes the importance of the effect of abnormal inflammation on keloid formation. Repairing genital defects using an inguinal flap has only a minor effect on the patient's postoperative movement, and an ideal vulvar appearance can be achieved.

Should Genital Hiatus/Perineal Body Be Measured at Rest or on Valsalva?

OBJECTIVE: Genital hiatus (Gh) and perineal body (Pb) are part of the Pelvic Organ Prolapse Quantification assessment system, but it is unclear whether measurements should be taken at rest or on Valsalva. This study was designed to assess the predictive value of Gh and Pb measurements obtained at rest and on Valsalva for signs and symptoms of pelvic organ prolapse (POP). METHODS: This is a retrospective study involving 416 women who presented to a tertiary urogynecology unit with symptoms of pelvic floor dysfunction. Genital hiatus and Pb were measured at rest and on maximal Valsalva. The strength of association between binary markers of POP and measurements of Gh/Pb was estimated using logistic regression analysis. Receiver operator characteristic statistics were used to compare predictive values of Gh and Pb measurements obtained at rest and on Valsalva. RESULTS: A total of 451 women were seen during the study period. Thirty-five were excluded owing to missing data, leaving 416. Fifty-four percent (n = 223) complained of POP symptoms. On examination, 80% (n = 332) had significant POP (stage 2+ in anterior or posterior compartments or stage 1+ in the central compartment). On imaging, significant POP was diagnosed in 66% (n = 275). Mean hiatal area was 22 cm (SD, 7; range, 5-49 cm) at rest and 30 cm (SD, 10; range, 11-69 cm) on Valsalva. Genital hiatus and Pb measured on Valsalva were consistently stronger predictors of prolapse symptoms and objective prolapse (by clinician examination and by ultrasound) than at Gh and Pb measured at rest. The corresponding area under the curve values were significantly larger for Gh/Pb measures on Valsalva after adjusting for multiple confounders. CONCLUSIONS: Genital hiatus/Pb measured on maximal Valsalva is a superior predictor of symptoms and signs of POP compared with Gh/Pb at rest.

Childhood Asymmetry Labium Majus Enlargement (CALME): Description of Two Cases.

Background: Childhood asymmetry labium majus enlargement (CALME) is an uncommon, benign condition that occurs in pre- and early pubertal girls and is characterized by a painless, fluctuating, non-tender labial swelling with normal overlying skin. Recognition of this benign condition is essential. Differentiation with several other diseases that mimic CALME and require different diagnostic and therapeutic approaches is mandatory. Two cases of CALME are described in this report. Differential diagnoses and therapeutic approaches are highlighted. Case presentation: The first case was an 11-year-old Caucasian girl referred to our hospital for the evaluation of right labium majus, which showed a palpable, painless, soft, non-tender, non-erythematous enlargement measuring approximately 2 cm with indistinct borders. Ultrasound showed a mass 23 × 18 × 12 mm in diameter. Surgical excision of the mass was performed and in the histopathological evaluation, the tissue specimens were composed of haphazardly arranged vascular channels, adipose tissue and nervous elements that were components of the vulvar soft tissue and were compatible with the diagnosis of CALME. Case 2 was a 6-year-old Caucasian girl who presented a post-traumatic painless mass of left labium majus swelling that progressively increased in volume. Ultrasound study evidenced an ill-defined heterogeneous echotexture mass 26 × 15 × 10 mm in diameter and magnetic resonance imaging confirmed these findings. Histopathological examination was performed after bioptic sampling evidencing normal constituents of vulvar soft tissue, including fibroblast, collagen, adipose tissue, blood vessels and nerves compatible with CALME. Conclusions: CALME is a particular clinical condition that occurs mainly in pre-pubertal girls and has a benign course but poses numerous problems in differential diagnosis that can be solved only with careful clinical observation and with a careful use of radiological imaging techniques. Our cases, in agreement with recent literature, suggest that radical excision is not recommended and that surgical biopsy should be taken into consideration only in cases of doubt.

Diagnosis and Treatment of Vulvar Lichen Sclerosus: An Update for Dermatologists.

Vulvar lichen sclerosus is an important skin disease that is common in women in their 50 s and beyond; however, it can also affect females of any age, including children. If not treated, it has the potential to cause significant and permanent scarring and deformity of the vulvar structure. In addition, if untreated, it is associated with a 2-6% lifetime risk of malignant squamous neoplasia of the vulva. Lichen sclerosus has been considered a difficult to manage condition; however, both serious complications can potentially be prevented with early intervention with topical corticosteroid, suggesting that the course of the disease can be treatment modified.

[Intrapartum Translabial Ultrasound: A Systematic Analysis of The Fetal Head Station in The First Stage of Labor]. / Ultraschall unter der Geburt: Eine systematische Analyse des Höhenstandes des kindlichen Kopfes in der Eröffnungsperiode der Geburt.

INTRODUCTION: This prospective study aimed to define the angle of progression (AOP) in relation to the height position of the fetal head during the first stage of labour. It was investigated if it is possible to predict the mode of delivery or the duration of labour by AOP. METHODS: Influencing factors on delivery were head circumference, birth-weight, administration of oxytocin, epidural anaesthesia (EA) and parity, and their impact on AOP was analysed. AOP was calculated using three different formulas. Inclusion criteria were vaginal delivery of singletons in cephalic, occipito-anterior presentation. RESULTS: 30/80 recruited women met the study criteria. 90% delivered spontaneously vaginally, 10% had instrument-assisted vaginal delivery. The average AOP in spontaneous vaginal deliveries was 100.9° at cervical dilation less than 5 cm, and 125.3° at cervical dilatation more than 5 cm. The average AOP in instrument-assisted births was 93° and 113.9° when the cervical os was less than 5 cm and more than 5 cm, respectively. Analysis identified a predictive trend towards the duration of labour only by use of the first AOP formula but not regarding the mode of delivery. CONCLUSION: Sonographically assessed AOP during first stage of labour indicates trends regarding the duration of labour.

[Cylindric Abdominoperineal Rectum Exstirpation with Partial Vulvar and Vaginal Resection as well as Perineal and Vaginal Defect Reconstruction by a Vertical Rectus Abdominis Myocutaneous (VRAM) Flap]. / Zylindrische abdominoperineale Rektumexstirpation mit partieller Vulva- und Vaginaresektion sowie perinealer und vaginaler Defektrekonstruktion durch einen vertikalen Rectus-abdominis-Muskel-Haut-Lappen (VRAM-Lappen).

Introduction Patients with low rectal cancer or anal cancer undergoing abdominoperineal excision (APE) benefit from extended surgery and the subsequent avoidance of surgical "waisting" at the level of the puborectalis muscle. The method of cylindrical APE was introduced by T. Holm and led to a reduction of intraoperative perforations and involvement of circumferential resection margins, and subsequently reduced the risk of local recurrence. The use of myocutaneous flaps reduces perineal wound complications, which occur in up to 60% of patients with primary closure of perineal defects, especially following neoadjuvant radiochemotherapy. Flaps obliterate pelvic dead space, recruit well-vascularised tissue into irradiated regions, facilitate wound closure and allow for vaginal and perineal reconstructions. This video shows the technique of extended cylindrical APE with partial vulvar and vaginal resection and subsequent reconstruction of the posterior vaginal wall and the pelvic floor defect by a vertical rectus abdominis myocutaneous (VRAM) flap. Indication Locally advanced anal cancer with infiltration and fistula to the posterior vaginal wall without metastatic spread following neoadjuvant radiochemotherapy. Procedure Extended cylindric APE with partial vulvar and vaginal resection, construction of a descending colostomy with parastomal intraperitoneal onlay mesh augmentation, pelvic reconstruction with a VRAM flap and inlay mesh augmentation of the anterior rectus sheath. Conclusion From the oncological point of view, extralevator APE is superior to standard surgery. The use of myocutaneous flaps improves postoperative wound healing and quality of life.

MR Imaging of the Female Perineum: Clitoris, Labia, and Introitus.

The female perineum has a complex anatomy and can be involved by a wide range of pathologies. In this article, we specifically focus on the clitoris, labia, and introitus. We discuss the normal anatomy of these structures, the MR imaging techniques to optimize their evaluation, and several common and uncommon entities that may affect them, including benign and malignant tumors, as well as infectious and inflammatory, vascular, iatrogenic, and developmental entities.