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Related Topics

  • Imperforate Hymen
  • Imperforate Hymen
  • Vaginal Agenesis
  • Vaginal Agenesis

Articles published on Labial fusion

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  • Research Article
  • 10.1093/humrep/deaf253
Association between in utero exposure to acetaminophen and external genital tract malformations in boys and girls: a systematic review and meta-analysis.
  • Mar 1, 2026
  • Human reproduction (Oxford, England)
  • Lina Eletri + 7 more

Is the use of acetaminophen during pregnancy associated with external genital tract malformations in boys and girls? This meta-analysis found no evidence linking in utero acetaminophen exposure to external genital malformations in boys but further research focusing on girls and considering relevant confounding factors is needed. Acetaminophen is widely used by pregnant women, but findings are conflicting regarding a possible increased risk of genital malformations in the offspring of both sexes. In this systematic review and meta-analysis, we searched MEDLINE, EMBASE, Cochrane Central Register of Controlled Trials, International Clinical Trials Registry Platform, ClinicalTrials.gov on 18 April 2024, and subsequently updated the search on 20 September 2025 for randomized controlled trials and observational studies. This meta-analysis included randomized controlled trials and observational studies focusing on the association between in utero exposure to acetaminophen, with no restriction on publication dates and languages. Two reviewers independently screened studies, extracted data, and assessed risk of bias. The two primary outcomes were a composite endpoint including cryptorchidism, hypospadias, and penile hypoplasia in boys, and a composite outcome including clitoral enlargement, labial fusion, vulvar malformations, and rectovaginal fistula in girls. Secondary outcomes included individual genital malformations and differences in anogenital distance (AGD). GRADE was used to evaluate the level of certainty. Ten observational studies were included. Regarding boys, meta-analysis of the predefined primary outcome could not be performed as no studies reported penile hypoplasia. Another composite endpoint including cryptorchidism and/or hypospadias was reconstructed and no significant association was found with acetaminophen, pooled crude odds ratio (OR) 1.27 [95% CI (0.18, 8.94), studies = 2, participants = 155362, I2=79%, phet=0.03, random-effects model (REM)]. No significant association was found for cryptorchidism or hypospadias separately based on adjusted estimates with pooled OR of 1.02 [95% CI (0.78, 1.35), studies = 3, participants = 155852, I2=32%, phet=0.23, REM] and 1.02 [95% CI (0.89, 1.17), studies = 3, participants = 159572, I2=40%, phet=0.19, REM], respectively. No significant difference was found for short AGD. No meta-analysis was possible for girls for any of the predefined outcomes due to lack of data. The level of certainty was low to very low. The predefined primary outcomes in boys and girls could not be fully evaluated. Small study effects could not be assessed as the number of included studies was limited. There was significant heterogeneity in the reporting of results and information regarding maternal characteristics was lacking. Lastly, all included studies had a serious or critical risk of bias due to the limited control of confounding factors and the level of certainty was low to very low. Acetaminophen remains an indispensable medication and abstaining from pain and fever treatment during pregnancy may have harmful effects on the developing fetus. Further research on external genital malformation risks following in utero exposure to acetaminophen should be directed toward developing validated and robust drug-exposure and outcome assessment tools, that control for confounding factors and confounding by indication. No funding was used to conduct this study. No relationship or activity could appear to have influenced the submitted work. CRD42024536483, available from: https://www.crd.york.ac.uk/prospero/display_record.php?ID=CRD42024536483.

  • Research Article
  • 10.3389/fendo.2026.1791297
Case Report: Integrating clinical presentation and genetic analysis in P450 oxidoreductase deficiency: a novel mutation and systematic review.
  • Jan 1, 2026
  • Frontiers in endocrinology
  • Chen Zhang + 6 more

Cytochrome P450 oxidoreductase deficiency (PORD) is an ultra-rare autosomal recessive disorder caused by mutations in the POR gene and characterized by highly heterogeneous skeletal, genital, and endocrine manifestations. Owing to this complexity, PORD remains frequently underrecognized in clinical practice, and integrated clinical-genetic syntheses remain limited. A retrospective analysis was conducted on the clinical data of a PORD patient treated at Shenzhen Children's Hospital. Relevant literature was retrieved from PubMed, Web of Science, and China National Knowledge Infrastructure (CNKI). Reported cases were analyzed with respect to sex, age, geographic distribution, clinical manifestations, and POR gene variants. The patient from our hospital, a 7-month-old infant, presented with characteristic features including frontal bossing, craniosynostosis, flat nasal bridge, proximal radioulnar synostosis, clitoromegaly, partial labial fusion, and steroid hormone abnormalities. Genetic testing identified compound heterozygous variants, p.G146fs*111, a novel mutation, and p.R457H. The patient underwent bilateral mandibular distraction osteogenesis and cranial reconstruction, which alleviated airway obstruction, swallowing difficulty, and craniosynostosis. A total of 50 eligible studies were identified, comprising 167 patients (male:female = 77:90). The major clinical findings were skeletal deformities in 124 cases (74.25%), gonadal deformities in 121 (72.46%), hormonal abnormalities or delayed puberty in 127 (76.05%), and adrenal insufficiency or crisis in 108 (64.67%). Additionally, ovarian cysts were observed in 36 female patients (40.00%). Among all patients, the allele frequency of the p.R457H variant was 27.25%, while that of the p.A287P variant was 14.97%. In the 22 Chinese patients, the allele frequency of the p.R457H variant reached 38.64%. We report the clinical features of a PORD patient carrying a novel POR mutation, p.G146fs*111. PORD typically presents with skeletal and genital malformations as well as adrenal insufficiency. Management requires multidisciplinary collaboration, including individualized steroid replacement, regular blood pressure monitoring, and surgical intervention when necessary. The p.R457H variant may represent a hotspot mutation in East Asian populations.

  • Research Article
  • 10.69614/ejrh.v17i2.477
A case series of labial adhesion in reproductive age women
  • Apr 30, 2025
  • Ethiopian Journal of Reproductive Health
  • Getu Dinku + 3 more

Labial adhesion also known as labial fusion , labial agglutination or synechia vulvae . it is one of rare condition in reproductive age group unlike in prepubertal and postmenopausal women . We report two cases who were apparently health women presented with labial adhesion and surgical adhesiolysis was done in our hospital .

  • Research Article
  • 10.1093/jsxmed/qdaf068.097
(109) WOMEN’S SEXUAL HEALTH: A BIOPSYCHOSOCIAL PRECLINICAL CURRICULUM
  • Apr 25, 2025
  • The Journal of Sexual Medicine
  • E Martinbianco + 2 more

Abstract Introduction In 2009, the International Consultation on Sexual Medicine advocated for the establishment of a comprehensive national standard for sexual health education in medical schools (E. Coleman et al., 2013). However, more than half of medical students in the United States and Canada report feeling inadequately prepared to address their patients’ sexual health concerns (Shindel AW et al., 2010). A 2022 study found that, aside from basic female anatomy, over half of medical schools in the Chicago area did not include female sexual functioning in their curricula (N. Codispoti et al., 2022). Additionally, many practicing physicians express similar concerns, with a lack of education and training in sexual health identified as a key barrier to providing care and screening for sexual dysfunction (Parish SJ et al., 2009). This second part of our study proposes curriculum changes aimed at addressing this gap, specifically evaluation of a second year module on women’s sexual health. Objective Assess the need for additional preclinical curriculum content by assessing students’ knowledge with women’s sexual health and the perceived importance of a female sexual health curriculum. Evaluate a second-year medical school educational module on women’s sexual health. Methods This cross-sectional, self-controlled survey study explores the initial knowledge and views of second-year medical students regarding women’s sexual function (WSF) and sexual dysfunction (WSD). The study involved 25 students, roughly one-sixth of the class. The effectiveness of the educational materials was evaluated using quiz questions based on the content. Additionally, students’ opinions on the importance of this topic in their medical education were compared before and after the educational intervention. Results Results after second-year medical students completed a module on Women’s Sexual Health and Dysfunction showed a statistically significant increase in correct answers for items 1,2, and 7 (p < 0.05). Correct responses improved for questions on the DSM-5 definition of Female Sexual Interest/Desire Disorder, Basson’s Cycle, and labial fusion. Pre-test responses (N = 28) and post-test responses (N = 25) were compared using McNemar chi-square tests. Conclusions With statistically significant improvement in knowledge of women’s sexual development and function through our proposed curriculum including integration into course material and supplemental modules, we advocate for implementing a standardized preclinical women’s sexual health curriculum. Our curriculum can improve competency in female sexual health across all medical specialities, as our cohort indicated interest in every field. Closing the gap of knowledge during medical student’s preclinical years can provide a stronger foundation and enhance their confidence in discussing and educating patients about sexual health and dysfunction. This early understanding is also crucial in preventing delayed diagnosis of conditions that can significantly impact the health and quality of life of female patients. Furthermore, our ongoing studies focus on the development of a curriculum directed at the pathology and treatments of female sexual dysfunction from a biopsychosocial perspective, resulting in a comprehensive curriculum. Disclosure No.

  • Research Article
  • 10.14235/bas.galenos.2024.25349
Labial Fusion in Childhood: Management and Treatment Strategies
  • Jan 27, 2025
  • Bezmialem Science
  • Ayşe Filiz Gökmen Karasu + 1 more

Labial fusion (LF) is frequently an asymptomatic condition and hypoestrogenism plays an important role in the pathopysiology. In the presence of a hypoestrogenic environment friction i.e. in the form of vigorous perineal cleaning leads to inflamation and desquamation of the epithelium which results in LF. Expectant management and reassurance of the family is key in asymptomatic patient. However if the condition results in complications such as urinary retention, recurrent urinary tract infection or vaginitis topical therapy with estrogen or bethametazone is the first treatment option. For patients who do not respond to a course of medical treatment or for cases in which recurrence occurs manuel or surgical seperation may be offered. Regardless of the choice of treatment the family should be counselled of the possibility of recurrence. Recurrence rates decrease with increasing age and the commence of endogenous estrogen production. Factors that contribute to recurrence are poor genital hygiene, recurrent vulvovaginitis and vulvar dermatoses.

  • Research Article
  • 10.4103/ijru.ijru_9_25
Labial fusion in an adolescent girl: A rare case requiring surgical management
  • Jan 1, 2025
  • International Journal of Reconstructive Urology
  • Kumbha Siva Naga Jyoti + 2 more

ABSTRACT Labial fusion refers to the complete or partial adhesion of the labia minora. It is also known as synechia of the vulva, labial adhesion, labial agglutination, or vulvar fusion. This condition is commonly observed in prepubescent girls due to low estrogen levels and typically resolves spontaneously with puberty. Although often asymptomatic, treatment options include manual or surgical separation of the adhesions, as well as topical application of estrogen or betamethasone cream. Here, we report a case of labial fusion in a postpubertal adolescent girl, successfully managed through surgical intervention.

  • Research Article
  • 10.1111/ped.70011
Clinical features, diagnostic approach, and outcome of imperforate hymen: A single institution case series.
  • Jan 1, 2025
  • Pediatrics international : official journal of the Japan Pediatric Society
  • Masashi Kadohisa + 4 more

Imperforate hymen is uncommon but it is one of the most common obstructive lesions of the female genital tract. Hydro/hematometrocolpos, derived from retained uterine and vaginal secretions and menstrual blood, causes various nonspecific symptoms. This study aimed to examine the clinical features, diagnostic approach, and outcomes of imperforate hymen at a single institution in Japan. We retrospectively reviewed all patients with imperforate hymen at Osaka Red Cross Hospital between January 2012 and November 2023. Six patients were identified. Five patients were diagnosed at puberty (range: 10-13 years) and one was diagnosed in the neonatal period. A neonatal case was diagnosed incidentally during the follow-up of labial fusion. In the adolescent cases, various initial symptoms (e.g., lower abdominal pain, lower back pain, urinary retention, constipation, and abdominal distension) caused the first visit to various clinical departments, such as Internal Medicine, Emergency, Pediatrics, Obstetrics and Gynecology, Orthopedics, and Surgery. In all cases, a large cystic lesion in the lower abdomen, suggestive of hematometrocolpos, was easily detected by abdominal ultrasonography, which resulted in consultation with the appropriate clinical department or performance of additional imaging tests, such as magnetic resonance imaging, in some cases. All patients were treated with a hymenectomy or hymenotomy and circumferential suture at the vaginal introitus. Postoperative stenosis or reclosure during follow-up was not observed. Physicians should consider imperforate hymen as a possible diagnosis when examining adolescent girls who have not yet had menarche and present with refractory lower abdomen-related symptoms, and abdominal ultrasonography should be performed.

  • Research Article
  • 10.14228/jpr.v1i2.53
Synechia of Major Labia and It’s Operative Technique: A Case Report
  • Dec 11, 2024
  • Jurnal Plastik Rekonstruksi
  • Muhammad Samiadji + 3 more

Background: Labial synechia (labial fusion or labial adhesion) is a clinical entity rarely seen in adults. Labial synechia are usually caused by a combination of local inflammation, chronic infection and estrogen deficiency. This condition is not life threatening, but severe cases usually result in urinary problems.Methods: A case report based on the medical and surgical records was done. We found one case of labial synechia on a 65 years old female which was referred to plastic and reconstructive surgery department from the Gynecology department.Results: The reconstructive surgery was performed with two stage. Local anaestethic adhesiolysis as the first treatment then continued with general anesthesia reconstruction using both labial advancement flap to close the mucous defect. Patient was stay in the hospital in 5 days, and the flap to reconstruct the labia was vital.Conclusion: Multifactorial causes such as chronic infection, chronic inflammation, poor hygiene and history of systemic disease can be the etiology. Surgical approach is the best choice for this case because the synechia causes urinary problems.

  • Research Article
  • 10.51271/kmj-0152
Labial fusions and age periods
  • Sep 1, 2024
  • Kastamonu Medical Journal
  • Sevgi Ulusoy Tangül + 1 more

Aims: Although labial fusion disease is usually seen in infancy, it can also be seen in other age groups. Our study aimed to evaluate whether labial fusion disease creates significant clinical problems across age periods by considering childhood periods. Methods: Following ethics committee approval, the files of patients who came to the Pediatric Surgery outpatient clinic with complaints of labial fusion between 2017 and 2023 were retrospectively examined. These patients were divided into groups related to their childhood periods and the causative cause, other accompanying problems, treatment method, number of recurrences of labial fusion, and reasons for recurrence. These patients were evaluated by grouping them as 0-2 months, 2 months-2 years, 2 years-6 years, and over six years of age. Additionally, the results obtained were evaluated in the light of the literature. Results: Of the 94 patients with labial fusion complaints, one was two months old, 51 were 2 months-2 years old, 34 were 2-6 years old, and eight were over six years old. A hygiene problem was identified in only 1 of these patients. In others, the reason for the complaint needed to be clarified. One patient had urinary complaints, and 4 had constipation. Fusion excision was performed as a treatment under outpatient clinic conditions. Recurrence occurred in 16 patients. It recurred once in 13 patients and twice in three patients. Estrogen-containing cream was used in 10 of the relapsed patients. Relapses were performed for recurrent patients under outpatient clinic conditions. Conclusion: Labial fusion is rarely seen in the neonatal period. This situation may be considered pleasing, but it may also cause people to think that perineal examinations were not performed well. We have implemented practices that can be said to be directly proportional to the literature for infants and school-age children. In patients over the age of six, no apparent cause could be identified. In treatment, only one patient required a surgical procedure, such as a straightforward fusion opening. The presence of additional complaints in some patients rose whether constipation, especially constipation, affected the formation of labial fusion, although its detection was proportionally low.

  • Research Article
  • Cite Count Icon 1
  • 10.1002/uog.29089
Abstracts of the 34th World Congress on Ultrasound in Obstetrics and Gynecology, 15-18 September 2024, Budapest, Hungary.
  • Sep 1, 2024
  • Ultrasound in obstetrics & gynecology : the official journal of the International Society of Ultrasound in Obstetrics and Gynecology
  • C Fakih + 4 more

Labial fusion typically impacts prepubertal girls and postmenopausal women, but it can rarely occur during the reproductive years without predisposing factors such as vulvar infections, dermatitis, trauma, female circumcision, or lichen sclerosis. We present the case of a 26-year-old female with Down syndrome who experienced urinary retention and abdominal pain. Obtaining a comprehensive medical history was challenging, but her parents reported regular menstrual cycles. The patient refused physical examination. A urine culture revealed Klebsiella sensitive to ciprofloxacin. Ultrasound identified a large 8cm hematocolpos. To rule out congenital malformations, an MRI was conducted, revealing a triangular-shaped fluid collection draining the urethra in the vagina and above the major and minor labia (see figures 1,2). Under general anesthesia, a physical examination unveiled complete labial fusion, which was surgically corrected through blunt dissection. Simultaneously, cystoscopy returned normal results. The patient was discharged on the same day, and her postoperative recovery was uneventful. Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.

  • Research Article
  • Cite Count Icon 3
  • 10.1111/jdv.20306
AI-powered visual diagnosis of vulvar lichen sclerosus: A pilot study.
  • Aug 28, 2024
  • Journal of the European Academy of Dermatology and Venereology : JEADV
  • Philippe Gottfrois + 8 more

Vulvar lichen sclerosus (VLS) is a chronic inflammatory skin condition associated with significant impairment of quality of life and potential risk of malignant transformation. However, diagnosis of VLS is often delayed due to its variable clinical presentation and shame-related late consultation. Machine learning (ML)-trained image recognition software could potentially facilitate early diagnosis of VLS. To develop a ML-trained image-based model for the detection of VLS. Images of both VLS and non-VLS anogenital skin were collected, anonymized, and selected. In the VLS images, 10 typical skin signs (whitening, hyperkeratosis, purpura/ecchymosis, erosion/ulcers/excoriation, erythema, labial fusion, narrowing of the introitus, labia minora resorption, posterior commissure (fourchette) band formation and atrophic shiny skin) were manually labelled. A deep convolutional neural network was built using the training set as input data and then evaluated using the test set, where the developed algorithm was run three times and the results were then averaged. A total of 684 VLS images and 403 non-VLS images (70% healthy vulva and 30% with other vulvar diseases) were included after the selection process. A deep learning algorithm was developed by training on 775 images (469 VLS and 306 non-VLS) and testing on 312 images (215 VLS and 97 non-VLS). This algorithm performed accurately in discriminating between VLS and non-VLS cases (including healthy individuals and non-VLS dermatoses), with mean values of 0.94, 0.99 and 0.95 for recall, precision and accuracy, respectively. This pilot project demonstrated that our image-based deep learning model can effectivelydiscriminate between VLS and non-VLS skin, representing a promising tool for future use by clinicians and possibly patients. However, prospective studies are needed to validate the applicability and accuracy of our model in a real-world setting.

  • Research Article
  • Cite Count Icon 1
  • 10.33425/2689-1085.1063
A Rare Presentation of Vulvar Lichen Sclerosus in a Pediatric Patient: A Case Report and Review of the Literature
  • Mar 31, 2024
  • Journal of Pediatrics & Neonatology
  • Tinatin Gagua + 3 more

Lichen Sclerosus is a benign, chronic dermatological condition characterized by marked inflammation, epithelial thinning, lymphocytic infiltration, and symptoms of vulvar pruritus, labial adhesion, and pain. There are two peaks of onset, one in low estrogen state - among prepubertal girls and the other in peri or postmenopausal women. We present a rare case of lichen scleorsus in a pediatric patient, emphasizing the importance of recognizing this condition in the pediatric population. Seven y/o female presented with a 6-month history of difficulty voiding, severe pruritus, and vulvar pain. Clinical examination showed white atrophic vulvar papules, partial labial fusion, and purpuric plaque in the anogenital region. Histological examination confirmed the diagnosis of lichen sclerosus. The patient was treated with topical corticosteroids and started on maintenance therapy to reduce recurrence and minimize the risk of vulvar carcinoma. Although rare in children, lichen sclerosus should be considered in diagnosing pediatric genital lesions. Given its high risk of recurrence and increased risk of vulvar carcinoma, long-term complications must be avoided by early detection and prompt management.

  • Research Article
  • 10.61745/jsmsau.1399333
APPROACHES TO LABIAL FUSION: 3 YEARS EXPERIENCE OF A TRAINING AND RESEARCH HOSPITAL IN THE BLACK SEA REGION
  • Dec 30, 2023
  • Atatürk Üniversitesi Tıp Fakültesi Cerrahi Tıp Bilimleri Dergisi
  • Ezgi Gün Soytürk

Objective: Labial fusion is a prevalent pediatric gynecological condition. This study aimed to conduct a retrospective analysis of patients diagnosed with labial fusion and treated in the Pediatric Surgery department of a tertiary hospital. The primary focus was on evaluating the topical and interventional modalities employed in the treatment of labial fusion and assessing the rates of recurrence. Materials and Methods: A retrospective evaluation was conducted on 520 patients treated for labial fusion at the Pediatric Surgery Clinic of Samsun Training and Research Hospital between January 1, 2020, and January 1, 2023. The investigation encompassed an analysis of patients' ages at the time of diagnosis, presenting symptoms, the application of topical, surgical, and combined therapeutic strategies, and the post-treatment recurrence rates of labial fusion. Results: Labial fusion was diagnosed in 520 patients, with ages ranging from 1 to 148 months and a mean age of 17.05±19.56 months. While a considerable proportion of patients were asymptomatic, symptomatic cases manifested urinary and vaginal symptoms. Initial treatment involved the application of topical agents to all patients. Notably, many cases referred to pediatric surgery clinics had previously undergone unsuccessful topical treatments administered by pediatricians at different medical institutions, prompting subsequent referral for manual separation. Success was achieved in 128 patients with topical treatment (98 estrogen, 30 betamethasone). Interventional procedures were performed on 392 patients, with an additional two weeks of topical treatment following the intervention for all relevant groups. Among the 95 patients undergoing manual separation, 1-5 recurrences were observed, while no recurrences were noted with repeated combined treatment. Statistically, higher labial fusion recurrence rates were identified in older age groups. Conclusion: In the management of labial fusion, we advocate for the importance of employing topical estrogen or betamethasone as non-invasive and secure treatment modalities. Considering the potential risks associated with prolonged topical therapy, manual or surgical separation is contemplated for patients exhibiting inadequate response to a two-week treatment course. Furthermore, we underscore the imperative for additional research to assess the long-term success of pre-pubertal labial adhesions and highlight the efficacy of post-surgical topical treatment as a prophylactic measure.

  • Research Article
  • 10.47407/kr2023.4.11.00301
Вульвит и инфекция мочевыводящих путей у девочек
  • Nov 28, 2023
  • Clinical review for general practice
  • Natalia A Chekeneva

Vulvitis is one of the most common disorders with which people come to visit obstetrician-gynecologist. nonspecific bacterial, atopic, infectious vulvitis and vulvitis associated with helminthiasis are most often found in girls under the age of 3 years. Hyperemic mucous membrane of the external genitalia, pain, burning, itching while urinating and at rest are specific clinical features of the disorder; abnormal genital discharge of various types can be also found. Vulvitis often comes along with perineal and perianal dermatitis. Labial fusion and urinary tract infections are common complications of nonspecific inflammation. obstetricians-gynecologists are recommended to conduct diagnosis, treatment, rehabilitation and further follow-up of such girls in cooperation with other related experts, involve pediatric urologist, gastroenterologist, pediatrician; if necessary – dermatovenerologist, enT physician, dentist, infectious disease specialist.

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  • Research Article
  • 10.24018/ejmed.2023.5.5.1917
Labial Adhesions
  • Oct 9, 2023
  • European Journal of Medical and Health Sciences
  • Shayista Shayista Nabi

Labial adhesion is characterized by partial or complete fusion of labia minora or labia majora. It is rare in menopausal women. It is usually a triad of estrogen deficiency, inflammatory skin disorders and lack of sexual activity. Patients are mostly asymptomatic until labial fusion is complete. The most common symptoms are urinary retention and urinary tract infections and incontinence. It is usually diagnosed on examination of external genitalia. The treatment is not well established, owing to low prevalence in menopausal population. The most common methods of treatment are manual separation and surgical excision. Postoperative application of local estrogen cream is vital to prevent recurrence.

  • Research Article
  • 10.1210/jendso/bvad114.238
FRI243 Hypertension And Virilization In A 4 Year Old Female
  • Oct 5, 2023
  • Journal of the Endocrine Society
  • Esraa Ismail + 2 more

Abstract Disclosure: E. Ismail: None. J.L. Miller: None. A.V. Bernier: None. Background: Congenital adrenal hyperplasia (CAH) is the most common cause of virilization of an XX female neonate. More than 95% of CAH cases are due to 21 hydroxylase deficiency which is detected in the newborn screen by detection of elevated 17 hydroxyprogesterone (17 OHP). 11 beta hydroxylase deficiency (11 BOHD) is the second most common cause of CAH, but considered extremely rare accounting for only 0.2-8% of cases. 11-BOHD is not part of the newborn screen, as it does not present with life-threatening salt wasting crisis. Hypertension (HTN) is observed in 2/3 of cases and is due to mineralocorticoid excess in the form of deoxycorticosterone (DOC). Therefore, 11-BOHD should be strongly considered in a virilized female with HTN and accelerated growth. Clinical case: 4-year-old female admitted for evaluation of severe persistent HTN. She had surgical repair of posterior labial fusion at 5 months of age. She was seen at 21 months of age for concerns of precocious puberty and virilization. Her work up included XX karyotype, 17 OHP and electrolytes within normal limits, however, she had an elevated DHEAS and testosterone level, with a bone age of 5 years at 21 months of age. Hypertension was recorded at the visit, but she was lost to follow up. At a primary care appointment her blood pressure was notably elevated, so she was sent to the ED. On admission, she was found to have both weight and height >99% for age, hyperpigmentation of gums and scars, and virilization (clitoromegaly with tanner 3 pubic hair). She had no thelarche. Initial hormonal evaluation revealed prepubertal gonadotropins, low aldosterone and low plasma renin activity. High dose ACTH stimulation test revealed elevated 11 deoxycortisol and 11 deoxycorticosterone levels. Her peak stimulated cortisol was inadequate at 8.7 mcg/dl (normal=>18 mcg/dl). Genetic testing for CAH revealed compound heterozygosity for 2 variants in the CYP11B1 gene, confirming the diagnosis of 11-BOHD. She was started on hydrocortisone replacement (12 mg/m2/day) and required spironolactone to achieve adequate blood pressure control. At the time of diagnosis, her father reported that he also had hypertension before 10 years of age, as well as early puberty, and significantly darker skin color than his family members. Conclusions: The HTN seen with 11-OHD is associated with suppressed aldosterone/renin activity and hypokalemia and is responsive to glucocorticoid therapy. However, this cause of CAH is not included in the newborn screen so can go unrecognized until features of virilization and rapid growth with bone age advancement are noted. If HTN is persistent despite glucocorticoid treatment, spironolactone is the preferred antihypertensive, as it is both a mineralocorticoid and androgen receptor antagonist. This case serves as a reminder to pay attention to BP at every clinic visit. Presentation: Friday, June 16, 2023

  • Research Article
  • 10.1093/jsxmed/qdad062.159
(172) SEXUAL FUNCTION AND QUALITY OF LIFE IN WOMEN WITH LICHEN SCLEROSUS
  • Jul 6, 2023
  • The Journal of Sexual Medicine
  • Gibrael Dr Hevy S + 2 more

Abstract Objectives Lichen sclerosus (LS) is a chronic inflammatory skin disease that most commonly affects the anogenital region in women. LS can be asymptomatic in some patients. In others, it can lead to irreversible anatomical changes in the vulvar area, such as reabsorption of the labia minora, burial of the clitoris, labial fusion and introital stenosis. Symptoms can include severe itch, paresthesia, and dyspareunia. However, there is a paucity of data concerning the effects of LS on sexual functioning. The aim of this study was to evaluate sexual function and quality of life in women with LS. Methods A literature review was performed based on extensive searches in PubMed. References were assessed for relevance by title and abstract by 2 independent reviewers, yielding 14 research papers for inclusion in the study. Results Women with LS had significantly decreased sexual activity and less satisfying sexual activity when compared with controls with no history of LS, and this included all types of sexual activity (vaginal, oral, anal and masturbation). Sexual function was significantly reduced among women with LS compared to controls, and this applied for all subdomains (desire, lubrication, arousal, orgasm, and pain). Women with LS had significant worse genital self-image, more anxiety, more depressive symptoms, and impaired health-related quality of life compared to controls. Despite adequate medical treatment, women with LS continued to have significant sexual distress. Sexual distress decreased but did not resolve. Conclusions LS can have a considerable impact on sexual function and quality of life. Thus, it is important that healthcare professionals consider LS from a bio-psycho-social perspective, where focus is not restricted to the biological aspects of the disease, but also on its psychosocial ramifications. Conflicts of Interest No interest of conflict.

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  • Research Article
  • 10.59324/ejmhr.2023.1(1).10
Chronic Bladder Retention Due to Lip Coalescence: A Case Report
  • Jul 1, 2023
  • European Journal of Medical and Health Research
  • Lounas Benghanem + 7 more

Coalescence of the labia minora is defined as partial or complete adhesion of the labia minora. Complete labial fusion is a rare condition in adults. Coalescence of the labia minora generally occurs in early childhood and in prepubertal girls. It is a rare entity in women of childbearing age, except in cases of hypo oestrogenism. In general, micturition dysfunction is a rare complication of labia minora coalescence. We report the case of an 18-year-old female with complete and severe labia minora coalescence complicated by bladder retention.

  • Research Article
  • 10.1093/jsxmed/qdad061.067
(071) Vulvo-vaginal Obliteration: Presentation of 6 Cases
  • May 24, 2023
  • The Journal of Sexual Medicine
  • A Dominguez-Bali + 5 more

Abstract Introduction Between December 2018 and July 2022, six cases of vulvovaginal fusion (obliteration) were diagnosed, evaluated, and treated at our centers. Less than 30 cases have been reported according to our reviews of the medical literature. All of the cases presented shared certain features such as more than 2 decades without sex or masturbation, no use of hormone replacement therapy, and a history of chronic urinary tract infections. Objective 1. Present information from 6 cases of a rare genitourinary disorder, its diagnosis, medical & surgical management, follow-up, and resolution. 2. Place vulvovaginal obliteration or labial fusion as one of the most extreme late consequences of untreated genitourinary syndrome of menopause. 3. Emphasize not only the importance of the use of local estrogen in all women going through menopause but also clarify and promote the importance of the maintenance of penetrative sexual activity in the lives of older women. This can be achieved either naturally or with the use of dilators and sexual toys. Methods We analyzed 6 cases of patients with vulvo-vaginal obliteration that presented in our facilities. Results Analysis of the 6 cases showed: In 3 out of the 6 cases, the patient didn’t have a partner, while in the other 3 cases, they were not sexually active with their partner in any way. In 2 of the cases, the patient’s main complaint was the inability to urinate due to obstruction of the urethral outlet by a fused labia. In both cases, urinary leakage from overflow was present in the area of the posterior fourchette, along with urocolpos, an extremely distended urinary bladder, bilateral megaureter, and hydronephrosis. The ages of these 6 women were between 72 to 94. The most severe case involved a patient with the longest time without penetration (> 30 years). Conclusions Total and partial vulvovaginal obliteration is a disease that hasn’t been commonly described. By obtaining the sexual history of each patient and the practice of geriatric gynecology, we have been able to diagnose this disorder. There is no doubt that vulvovaginal obliteration, also known as labial fusion, is one of the most extreme complications of the genitourinary syndrome of menopause but has not been medically described as part of its findings up till now. The prevention and treatment include not only the local use of estrogen but also the education of the patients on the need to maintain penetrative sexual activity in order to prevent the atrophy of the genitourinary area and maintain its functional ability. This can be done with a partner, penetrative dilators, or sexual toys. Disclosure No

  • Research Article
  • 10.1093/jsxmed/qdad060.268
(285) From the Proscription to the Prescription of Masturbation
  • May 22, 2023
  • The Journal of Sexual Medicine
  • A Dominguez-Bali + 1 more

Abstract Introduction The number of new women entering menopause each day in the US is around 6000. Unfortunately, in later life, it becomes increasingly common that a woman may arrive at this stage alone due to separation, divorce, the passing of a partner, or that the partner's decreased health and sexual function constrain the woman's sexual activity, satisfaction, and desire. The number of women with the aforementioned sexual limitations is higher than the number of women that maintain their sexual functioning. Additionally, the lack of sexual activity has been proven to accelerate the deterioration of the structures and functions of the pelvic floor and the genitourinary area. Objective All of the signs and symptoms of the Genitourinary Syndrome of Menopause (atrophy, dryness, recurrent UTI, dyspareunia, anal incontinence, pelvic organ prolapse, vaginal stenosis, labial fusion, or vulvar obliteration) can be easily found in women without sexual penetrative activity. However, this could be prevented and/or treated in great part with the maintenance of the partnered or solitary penetrative sexual activity. Methods An analysis of peer-reviewed articles on the topic of masturbation and its outcomes on women’s health, along with its data were used to assess the need for increasing the prescription of masturbation for the benefit of improved genitourinary health. Results Masturbation decreases as we age, markedly in women, studies and reports on masturbation activity show; 53% activity in Caucasian men aged 55 - 64, and 19% in women of the same age. At ages 64 – 65 there is a decrease to 33% in men and 10% in women. The result is that these women develop a stark increase in the number of genitourinary pathologies. Frequently absent in those who remain sexually active with a partner or through penetrative masturbation. Not only does the mechanical action of engaging in sexual physical therapy with a partner or with penetrative masturbation preserve the structures and functions of the genitourinary system. But in accordance with several neuroendocrinological studies, arousal and orgasms, increase the number of steroid receptors in the vital organs of the body improving their metabolism and integrity thus increasingly maintaining our health. In addition, if they don't receive hormone replacement therapy for the preservation of sexual function and prevention of genitourinary pathologies, the final results are usually catastrophic. Conclusions The number of women affected by the mentally castrating sexual education in which masturbation is not accepted is extremely high. This is despite the fact that masturbation among both younger and older women is related to a more consistent orgasm, compared with partnered sex. Historically speaking masturbation has been forbidden or taboo for centuries, mainly by religion, and has been extremely difficult to change in the mind of most adults, especially women. There is no doubt that partnered sexual activity and/or penetrative masturbation play a very important role in the health of the pelvic floor functions, just as in the general, psychological, and emotional health. Disclosure No

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