Labial Fusion Research Articles

AI-powered visual diagnosis of vulvar lichen sclerosus: A pilot study.

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.

Labial Fusion in a Postmenopausal Woman Presenting with Urinary Incontinence

ABSTRACT Genitourinary syndrome of menopause refers to the collection of signs and symptoms associated with decrease in estrogen and other sex steroids involving changes in labia majora/minora, clitoris, vestibule/introitus, vagina, urethra, and bladder. This term was coined in 2014 to reduce the stigma associated with genital symptoms after menopause. Our patient is a 65-year-old woman, menopausal for the past 15 years, who presented with dribbling of urine for 1 month. On examination, she was diagnosed with complete labial fusion. Diabetes was incidentally detected and antidiabetic treatment was started. Considering dense labial adhesion, the patient underwent surgical separation of labia under anesthesia. Postoperatively, she was put on topical estrogen therapy and was advised to maintain proper hygiene. She was followed up regularly for a month to prevent refusion.

APPROACHES TO LABIAL FUSION: 3 YEARS EXPERIENCE OF A TRAINING AND RESEARCH HOSPITAL IN THE BLACK SEA REGION

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.

Вульвит и инфекция мочевыводящих путей у девочек

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.

Labial Adhesions

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.

FRI243 Hypertension And Virilization In A 4 Year Old Female

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

Chronic Bladder Retention Due to Lip Coalescence: A Case Report

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.

(071) Vulvo-vaginal Obliteration: Presentation of 6 Cases

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

(285) From the Proscription to the Prescription of Masturbation

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

(463) The Increasing & Lethal Spectrum of the Genitourinary Syndrome of Menopause

Abstract Introduction In 2014, the Genitourinary Syndrome of Menopause replaced the previous concept of vaginal and genital atrophy. The spectrum of this syndrome includes and is not limited to atrophy, dryness, recurrent UTI, dyspareunia, anal incontinence, pelvic organ prolapse, vaginal stenosis, labial fusion, and/or vulvar obliteration. However, with time and the continuous exposure to women who have reached more advanced ages, such as centenarians, the spectrum of the GSM has been increasingly showing new entities and disorders never described before. Objective To update and expand the literature on the spectrum of GSM affecting the well-being, sexual functioning, and quality of life of postmenopausal women including the new observations seen in centenarians. Methods Multiple peer reviewed articles of both cohort and observational studies were evaluated to assess the increased spectrum of GSM symptoms in the centenarian women population. Results Nearly 60% of women in menopause experience GSM. Despite a vast majority of signs and symptoms, only 7% of providers ask women about this condition implying that GSM continues to be underdiagnosed and undertreated. In the early extreme of the spectrum of GSM, we show the new finding of the presence of false abnormal cervical dysplasia in women who have never had an abnormal PAP smear and have maintained a stable sexual relationship with their partner. This misconstrue is due to early vaginal and cervical atrophy which produces cells that, under the microscope, are very similar to dysplastic cells to the pathologist. This scenario is frequently seen in more than 80% of cases with those epidemiological characteristics, which is extremely easy to resolve with 4 months of regular local estrogen therapy. In the other extreme of the spectrum of GSM, in the elderly, the combination of lack of HRT and sex for prolonged periods of times produced the partial or total obliteration of the labia majora or minora with fusion in several women. Thus, preventing the exit of urine through the urethra with significant retention of fluid affecting the urinary bladder, ureters, and kidneys. Finally, having been exposed to the care of centenarian women in home care (women over 90 years old), we have realized that a significant number of them died with the diagnosis of pneumonia. However, when reviewing the analyzed cases retrospectively, we found that the real diagnosis was undiagnosed recurrent UTIs with poor clinical manifestations (which is common in these types of patients). The algorithm goes as follows: undiagnosed recurrent UTIs with poor clinical manifestations such as fever, leukocytosis, and poor verbal manifestations due to the age of patients, that progress to sepsis and the pneumonia that has been the part that really draws the attention of the physician. Conclusions The new findings of this review highlight the clinical implications when analyzing the two extremes of the spectrum of GSM throughout women’s lives. In all these cases, local or systemic HRT was never used. Appropriate management of GSM will enhance the quality of life and sexual function of these women. Disclosure No

(460) A Case Series on Vulvovaginal Obliteration

Abstract Introduction Between Dec. 2018 & June 2020, 5 cases of Vulvovaginal Obliteration, also known as Labial Adhesion were diagnosed, evaluated and treated. All of the presenting cases had a history which shared certain features, including more than 2 decades without sexual activity, no history of masturbation, no use of hormone replacement therapy and chronic urinary tract symptoms. The most severe case had the longest period without vaginal penetration (>30years). In 3 of the 5 cases the patients didn't have a partner, while the other 2 patients were not sexually active with their partner in any way. Objective To add new cases to the literature about this rarely described disorder. To show this condition is one of the most extreme consequences of Genitourinary Syndrome of Menopause. To demonstrate the importance of vaginal penetration and hormone replacement therapy (HRT) to prevent postmenopausal labial adhesion. Methods This is a case series reviewing the effective management of labial adhesion in post-menopausal women. Results In 2 of the cases the patients main complaint was their inability to urinate due to obstruction of the urethral outlet by the fused labia. This obstruction caused urocolpos, extremely distended urinary bladder, megaloureters, & bilateral hydronephrosis. Leakage of urine also occurred at the posterior fourchette. All of these urinary symptoms disappeared after an urgent introital incision was performed in the OR. In both cases the exclusive use of local estrogens postoperatively failed to prevent recurrent closure. Only the combined use of local estrogens and the regular use of dilators (3-4 times/day/2 weeks, then 1-2 times /day/2 weeks, then PRN) prevented recurrence. In 3 of the 5 cases, regular use of the dilators caused them to reach orgasm for the first time in their lives. The ages of these women were between 76 & 92 years old. The last 2, and more mild cases had 50% labial fusion, without urinary retention. The use of progressively increasing sizes of dilators and estrogen cream resolved the problem without surgical interventions. Conclusions Labial adhesion is one of the worst consequences of GSM, due to progression towards urinary retention. It is caused by insufficient maintenance of sexual activity after menopause, combined with lack of prevention with HRT, and is successfully managed with surgery, dilators and topical estrogens. Disclosure No

Çocukluk döneminde labial füzyona yaklaşımımız: Tek merkezde sekiz yıllık deneyim.

Aim: In this study; we aimed to determine the clinical information and treatment results of labial fusion patients who applied directly to or consulted the Pediatric Surgery Outpatient Clinic of our hospital.
 Material and Method: Female patients in the prepubertal age group with labial fusion who applied to the Pediatric Surgery Outpatient Clinic of our hospital were retrospectively reviewed in terms of age, complaints, recommended treatment, recurrence rates, time to recurrence, and complications.
 Results: This study involved 438 patients with labial fusion aged between 32 days and 10 years in our hospital. Labial fusion was detected in 359 (82%) of the patients, a nearly complete opening only in the labia minora, and additional problems related to labial fusion in 180 (41%) patients. The patients were treated with topical estrogen and creams containing the raw material of the Centella Asiatica plant and manual fusion separation. Recurrence of labial fusion was seen in 18(4%) of these patients. It was found that the patient's age at the time of detection and additional problems related to labial fusion were effective on the recurrence of the disease.
 Conclusion: Older age and additional problems may increase the recurrence rate of labial fusion. We think that we can get results faster, with less risk of recurrence, by detecting the pathology early, applying the necessary precautions, and using the creams containing the raw material of Centella Asiatica as well as the raw material of the Centella Asiatica plant in combination, if necessary.

Complete Labial Fusion in a Post Menarche Adolescent Girl

Abstract: Labial fusion is commonly seen in pre-pubertal girls but is rarely reported in adolescent females after menarche. Its main cause is low estrogen levels. A patient can be completely asymptomatic and self-limiting or present with a wide range of symptoms which can vary from urinary tract infection, urine retention, incontinence, dyspareunia, etc. We present a case of a 12-year-old female who presented in our department with a complaint of difficulty in voiding and lower urinary tract symptoms. After a detailed examination, she was diagnosed with labial fusion for which adhesiolysis was done and patient remained asymptomatic after it.

Case Series on Labial Fusion in Perimenopausal and Postmenopausal Woman

Labial fusion or agglutination is a diagnosis of exclusion we come across in perimenopausal and postmenopausal women and sometimes also in young females. Here we highlight a series of cases which had presented with urinary disturbances and turned out to be labial fusion. Labial fusion can be managed conservatively or might require surgical interference which is also quite simple. Hence vaginal examination is a must for women presenting with urinary disturbance to rule out this condition.

Retrospective evaluation of labial fusion in girls

Aim: The aim of this study was to determine the factors affecting fusion in girls treated for labial fusion retrospectively over a three-year period. 
 Material and Method: The research has a cross-sectional design. The research sample consists of girls with ICD10 code Q52.5 who were brought to the hospital with labial fusion symptoms in a three-year period (January 2018-December 2020). The data were obtained from the hospital information system. Variables that were effective in labial fusion recurrence were determined by Chi-Square analysis, and the marginal effects of effective variables on recurrence were analyzed by Poison Regression analysis. 
 Results: 52.9% of 308 cases were younger than one year old (mean age 4.36±1.10 months). Symptom presentation is 10.4% in children younger than one year of age, and 84.83% in older. The most commonly presented symptoms are pain, burning, soiling of underwear, and bad odor during urination. Labial fusion recurrence is 14.1% in children younger than one year of age, and 62.8% in older. Manual opening was applied in all cases, and weekly follow-ups were performed with topical treatment. The Poison Regression analysis revealed that a history of allergy (1.31 times; z:3.61, p:0.000), winter (0.86 times; z:3.22, p:0.001), and diaper dermatitis (1.22 times; z:5.19, p:0.000) increased the number of labial fusion recurrence. 
 Conclusion: The findings of our study are similar to the literature in terms of factors causing labial fusion and treatment type. The recurrence rate was found to be higher in our study. It should be kept in mind that labial fusion is asymptomatic, especially in girls in the first year of life. Considering the possibility of recurrence of labial fusion, mothers and physicians examining the child should be aware of this issue.

Approach to labial fusion in children: 16 years of experience

Aim: We aimed to retrospectively evaluate the patients with labial fusion who presented to or were referred to the pediatric surgery clinic in a tertiary pediatric hospital.
 Material and Method: Between 01/01/2005 and 31/12/2020, 889 patients admitted or consulted to the pediatric surgery clinic due to labial fusion, age at the time of diagnosis, complaints on admission, which clinic referred the patient to the pediatric surgery clinic, treatments, recurrence, and complications were evaluated retrospectively.
 Results: The mean age of the patients was 2.21 (0.1–11) years. Most of the patients (82.5%) were asymptomatic. Parents noticed the condition and brought their child directly to the pediatric surgery clinic in 72.3% of the cases. Otherwise, patients were consulted to the pediatric surgery clinic from pediatric clinics, the pediatric endocrinology clinic, the pediatric nephrology clinic, or the pediatric emergency department. During the initial examination, manual separation was performed in 885 patients, and surgery was required for four patients. After the procedures, hydrotherapy with warm water and topical estrogen therapy were applied to all patients for 15 days. Recurrence was detected in 80 (9.0%) patients who were treated by manual separation. Manual separation was performed again in 78 of the patients, while surgical separation was performed in two (2.5%) patients who had severe fibrotic fusions.
 Conclusion: In the treatment of labial fusion, we recommend the combination of manual separation and topical estrogen cream treatment because it can be applied safely in the clinic, and the recurrence rate is low. Surgical separation is preferred in severe, thick, and fibrotic labial fusions.

D2 MTE4 Case 11: Adult CAH, how do I optimize treatment?

28 years, female, born of non-consanguineous marriage, assigned female gender had genital ambiguity and seizures on day 10 of life. She presented to us at 9 years with genital ambiguity and hyperpigmentation. Tanner stage A3P4B2, external genitalia: phallic like structure 4.5 cm, posterior labial fusion, single opening, no palpable gonads. Unstimulated 17 OHP 192 ng/ml, testosterone and DHEAS elevated, low basal cortisol, other biochemical parameters normal, CT scan- bilateral adrenal enlargement, so a diagnosis of simple virilizing type of congenital adrenal hyperplasia was made and was started on T. Hydrocortisone and T. Fludrocortisone, after which pigmentation decreased. Gender role and identity as female. Underwent clitoral reduction and vaginal reconstruction. She had spontaneous puberty with pubarche at 7 years, thelarche at 10.5 years and menarche at 12.5 years. Her 17 OHP remained elevated and had early epiphyseal fusion leading to short stature. She had hirsutism at 16 years so she was started on T. Dexamethasone but was again changed to Hydrocortisone as she developed cushingoid features after 2 years. She is married for past 3 years and is eumenorrhic but now presents with primary infertility. Recent investigations revealed 17 OHP 50 ng/ml (Target: 4-12 ng/ml) and T 6.3 nmol/l. Currently on T. Hydrocortisone divided in 5 doses and T. Fludrocortisone 150 mcg. How to further manage fertility and long-term complications.?

Genital abnormalities and management outcomes as seen in the university of port harcourt teaching hospital, Nigeria

Background: Genital abnormalities are a source of concern and anxiety to parents and patients and in some cases, for the physicians who may have difficulty making the pathological and eventually genetic diagnosis. They range from simple small penis and labial adhesions to complex genital ambiguity and disorders of sex development (DSD). Aim: The aim is to determine the genital abnormalities presenting in the Paediatric endocrinology unit of the University of Port Harcourt Teaching Hospital. , Materials and Methods: A retrospective cohort review of children presenting to the Endocrinology unit of the Paediatrics Department with genital abnormalities was undertaken from January 1, 2013, to December 31, 2017. The evaluation of the children included detailed history, physical examination, age and sex of rearing at presentation, clinical presentation, investigations, management, and outcome of treatment. Results: There were 31 children presenting with genital abnormalities of various kinds. Sex assigned to these children was 8 females and 23 males irrespective of complete pathological diagnosis. The median age of presentation was 13 months with a range of 0.1–168 months. The most common diagnosis was micropenis (32.2%) with various forms of DSD being the second-most common (29%) and most females had labial fusion (16.1%). Females with labial fusion had complete resolution following estrogen cream application, and 4 of the 7 children with DSD died. Conclusion: Making diagnosis and managing complex genital abnormalities like DSD in UPTH remains challenging because of the lack of diagnostic equipment and drugs, but simpler conditions have better and long-term outcomes.

Labial fusion: A rare cause of obstructive lower urinary tract symptoms in old age females

Labial fusion: A rare cause of obstructive lower urinary tract symptoms in old age females

Fractional CO2 laser treatment as adjunctive therapy to topical steroids for managing vulvar lichen sclerosus

ObjectivesUncontrolled vulvar lichen sclerosus (VLS) is often associated with distressful symptoms of genital itch, irritation, and pain and can lead to a pathological process including anatomical changes, scarring, and an elevated risk of cancer in the genital area. First‐line topical corticosteroid as monotherapy is frequently not adequate to fully suppress disease activity and control symptoms. This study evaluated the efficacy of fractional CO2 laser treatments as adjunctive therapy where recalcitrant VLS had been improved, but not adequately controlled, with topical corticosteroid treatment. Outcomes were evaluated up to 12 months after a series of CO2 laser treatments delivered via a fractional handpiece.Materials and MethodsWomen with a diagnosis of VLS supported by histologic findings on biopsy and/or clinical signs on physical examination received up to five monthly laser treatments. Subjects maintained existing topical corticosteroid and any exogenous hormone treatment during the study. Investigators assessed severity (0 = not present, 1 = mild, 2 = moderate, or 3 = severe) of clinical signs and architectural changes present before adjunctive study interventions and at follow‐up visits. Subjects reported the presence of clinical symptoms and impact on quality of life on 4‐ or 5‐point Likert scales. The validated Female Sexual Function Index (FSFI) was used to assess changes in sexual function. Four subjects were biopsied before adjunctive laser treatment and at follow‐up.ResultsTwelve females, 11 postmenopausal, with a mean age of 57 ± 10 years received three to five monthly CO2 laser treatments. Significant improvement in all prominent clinical signs and architectural changes were reported at the 3‐ and 6‐month follow‐ups after the treatment series. Significant improvement was maintained at the 12‐month follow‐up, with 89% of subjects showing at least one‐point improvement in elasticity compared to baseline; 86% in lichenification; 88% in sclerosis; and 80% in whitening and parchment‐like skin. Labial fusion and the extent of disease improved in 50% of patients. Ulcerations present in three subjects at baseline resolved after treatment. Subjects reported 86% improvement in dyspareunia and 83% in skin tearing. Quality of life improved significantly after treatment (p < 0.01). The 6‐month follow‐up FSFI showed significant improvement in sexual function compared to baseline (p < 0.05), with a mean point improvement of 4.5. Histology findings after treatment showed some positive improvement, as a decrease in dermal hyalinized zone thickness. There were no treatment complications or adverse events related to the treatment.ConclusionsFractional CO2 laser treatment outcomes showed improvement in predominant clinical signs and architectural changes in VLS recalcitrant to topical corticosteroid treatment. Adjunctive laser treatment relieved symptoms and improved quality of life as well as sexual function. Fractional CO2 laser treatment may provide an advanced treatment modality for the management of recalcitrant VLS with improved patient care and sustainable outcomes. Further study in a larger population and with CO2 laser treatment to both vulvar tissue and the vaginal canal should be explored.