Labial Fusion Research Articles

Voiding dysfunction due to long-standing labial fusion in an elderly woman: a case report

Labial adhesions or fusion in the elderly are rare, and only few cases are described in the English literature. We describe a case of near-complete labial fusion in an elderly woman presenting with voiding difficulty, its management and literature review.

COMPILATION OF AN INTERDISCIPLINARY LONGITUDINAL TREATMENT HISTORY FOR COMPLEX PATIENTS WITH PSYCHOSIS

To better understand the precise role of sensory corpuscles within the female external genitalia.After IRB approval, waste tissue samples were obtained from 10 normal girls (aged 2–9 years) who underwent surgery for labial fusion. Immunocytochemistry against protein gene product 9.5 (PGP 9.5), neuron-specific enolase (NSE), vasoactive intestinal peptide (VIP), 5-hydroxytryptamine transporter (5HTT), 5-hydroxytryptamine receptor 1A (5HT1A), Neuronal Peptide Y (NPY), neuronal nitric oxide synthase (nNOS), and estrogen receptors (ER) α and β was performed.Pacinian-like corpuscles were identified in epithelium of labia minora of prepubertal girls. A central structure composed of an axon surrounded by a central core, outer core, external capsule, surrounded by encapsulated stroma, and a subsidiary innervation in the outer aspect of the corpuscle stroma stained for PGP 9.5 in the outer core and layers of the external capsule, NSE positive cells in layers of the outer core, 5HTT in stroma of the corpuscle and cells located in layers of the outer core, 5HT1A in cells of outer core, NPY in stroma of the corpuscle, and nNOS in external core and external capsule of the central structure. ERα was present in stroma, external core, and external capsule, and ERβ in stroma of the corpuscle with subsidiary innervation in the stroma positive to PGP 9.5, VIP, and NPY.PGP 9.5, NSE, ERα, nNOS, and 5HTT immunoreaction detected in the outer core and external capsule could indicate these areas may play an important role in the functional aspects of the Pacinian-like corpuscle.

Oestrogen receptors and their relation to neural receptive tissue of the labia minora

To assess the cellular distributions of oestrogen receptors alpha and beta (ER alpha and ER beta) and neuronal nitric oxide synthase (nNOS) in the labia minora, as knowledge about ER type and function may clarify the role of oestrogens in vaginal scar formation and improve outcomes in female genital surgery. Labial samples were taken from 10 girls (aged 2-9 years) who underwent surgery for labial fusion. The waste tissue strips obtained were used for immunohistochemical identification of ER alpha and ER beta, and nNOS in the labia minora. There was ER alpha nuclear staining in the stroma of the labia minora close to the clitoris, and basal and suprabasal in the epidermal cells membrane restricted to superficial sections of the labia minora. ER beta was found in the stroma of the labia minora closer to the clitoris and in superficial sections, in the basal epidermal cells membrane and apocrine glandular epithelial cells membrane. There was also ER beta cell membrane staining in the basal and suprabasal epithelial cells and fibroblasts in the lamina propria. Established ER presence allows the consideration of the introitus of the vagina as a target for oestrogen therapy in various clinical and surgical situations. Continuing elucidation of the immunohistochemistry of this external genital tissue might assist in the development of molecular tools to treat genital abnormalities. Details of this immunohistochemistry may also advance the understanding of the effects of sexual differentiation on the brain and other organ systems.

Labial fusion in the reproductive age group: a rare presentation

Labial fusion is a common condition in prepubertal girls due to hypoestrogenism or in postmenopausal women with lichen sclerosis. It can also be seen as a result of chronic inflammation, urinary tract infection, and sexual abuse or following female genital mutilation. We present a rare case of almost complete labial fusion in a woman of the reproductive age group without any identifiable cause.

La coalescence des nymphes au service de chirurgie pédiatrique du CHU de Yopougon : à propos de 108 cas

Labial fusion is a benign genital disorder in girls. It may be either congenital or acquired, sometimes due to poor hygiene. Parental panic about this "absent vagina" contrasts with its simple, rapid, radical treatment. The study reports the result of simplified treatment. This retrospective study covers cases from 1 January 1992 through 31 December 2006 and includes only the cases of young girls treated as outpatients. All patients underwent outpatient surgical treatment. They were placed in gynaecological position. After asepsis of the vulva, a curved Halsted mosquito forceps was inserted into the opening for partial adhesions or across the medial transparent membrane for complete fusion. The forceps were then opened gently until complete detachment occurred. Local anaesthesia with EMLA cream ensured the absence of pain; there was sometimes minimal bleeding. All patients had local antiseptic treatment afterwards. During this study period, 108 patients (including two sisters) with a mean age of 22 months were treated for labial fusion (101 cases of total fusion and 7 partial). Only seven were older than 5 years of age. Overall, 84 patients underwent this basic treatment of section and follow-up antisepsis; none had a recurrence. Twenty girls also received oestrogen cream, and 4 had the surgical section alone. This benign disorder is rare and generally isolated in our regions, unlike in Western countries where it is often associated with hormonal deficits. The principal approach has been either therapeutic abstention or oestrogen therapy, both currently recommended in developed countries. Oestrogen treatment is a long procedure (3 or 4 months), however, and follow-up is far from certain. In Africa, all genital disorders are considered serious. Early repair is desirable for that reason and to prevent urinary tract infections and traditional "treatment". Simple outpatient treatment by surgical section with local antiseptic treatment is effective. Vulvar hygiene is essential to prevent recurrence. This benign disorder can be treated by any physician, but the psychological impact of the site and the necessary speed suggest the choice of management in paediatric units.

Pattern of childhood gynaecological presentations in a Nigerian tertiary health facility

Gynaecological problems in children and adolescents are often both medically and psychologically unique and require a highly skilled approach differing from those utilized for an adult female population. There is paucity of data on childhood gynaecological problems in our environment. The purpose of this study was to document the prevalence and pattern of common gynaecological problems in the prepubertal child at Ahmadu Bello University Teaching Hospital Zaria, Northern Nigeria. This was a retrospective study involving case file-based data analysis over a 10-year period (1995-2004) of 62 children aged 1 month-12 years at the Gynaecology unit of Ahmadu Bello University Teaching Hospital Zaria, Northern Nigeria. Sixty-two children were seen, 17 infants, 14 under 5 years of age, and the rest (31) were aged 6-12 years. The commonest condition was labial fusion (33.9%), urethral prolapse (14.5%), and suspected sexual assault (12%). Ambiguous genitalia (9.7%), vaginitis (6.5 %), and ovarian tumour (4.8%) were also encountered. Labial fusion, urethral prolapse, and suspected sexual assault are the commonest childhood gynaecological morbidities in Zaria. Provision of gynaecological services at every level of health care system to cater for young females is advocated.

Genital lesions complicating female genital cutting in infancy: a hospital-based study in south-east Nigeria

Despite the global outcry against female genital cutting (FGC), the practice continues in many African communities. The morbidity of this practice on the girl child deserves more attention. To determine the genital lesions complicating childhood FGC and the underlying factors that sustain this practice among the Igbos in south-east Nigeria. Prospective evaluation of girls with genital complications of FGC between January 2003 and June 2005 at the Federal Medical Centre, Owerri, south-east Nigeria. The girls' mothers were interviewed at presentation and subsequent visits to determine their perception and attitudes towards FGC. The average age at presentation was 3.5 years (range 1-5) and the genital cutting procedures were performed 8-90 days after birth. The procedure was undertaken by traditional practitioners in 14 (66.7%) girls and by nurses in 7 (33.3%) girls. Twelve girls (57.1%) had type I genital cutting and nine (42.9%) type II. Inclusion clitoral dermoid cyst and labial fusion were the complications in 13 (61.9%) and eight (38.1%), respectively. Treatment involved complete excision of the cysts and operative division of the labial fusion. Post-operative complications were wound infection (5) and labial adhesion (2). With duration of admission ranging from 3 to 5 days, the average cost of managing each child was $120. Of the girls' mothers, 15 (71.4%) had been educated to secondary level and 17 (80.1%) were aware of the campaign against FGC. Regrettably, all the mothers had genital cutting themselves during childhood and 13 (61.9%) stated that they would like to perpetuate the practice for socio-cultural reasons. Despite this, they all volunteered that FGC is enforced by the child's paternal relations. Childhood FGC contributes to appreciable morbidity among girls, a large proportion of whom are not managed in a hospital setting. The cost of managing these complications can be enormous, especially in low-resource settings. Girl child education to tertiary level and the involvement of traditional rulers and local decision-makers in the campaign against FGC might help to eradicate the practice.

Labial fusion causing pseudoincontinence in an elderly woman

Adhesions of the labia are rare in the adult population with only a few cases described in the English literature. The etiology of labial adhesions probably relates to vaginal inflammation or irritation. Once the superficial epithelium of the labia is denuded subsequent healing leads to fibrous adhesions between the labia. Etiological factors for labial fusion in adult women are local inflammation senile vaginitis hypoestrogenism lack of sexual activity local trauma vaginal laceration following childbirth female genital cutting (infibulation) and recurrent urinary tract infections. There could be skin pathology in the form of genital herpes or lichen sclerosus. (excerpt)

Treatment Not Needed for Most Pediatric Labial Fusion

Treatment Not Needed for Most Pediatric Labial Fusion

P-237A Labial fusion in early infancy(Urology 2, the 44th Annual Meeting of Japanese Society of Pediatric Surgeons)

P-237A Labial fusion in early infancy(Urology 2, the 44th Annual Meeting of Japanese Society of Pediatric Surgeons)

Significance of Topical Estrogens to Labial Fusion and Vaginal Introital Integrity

Purpose One theory for labial fusion is low prepubertal estrogen levels. Topical estrogens remains the mainstay of therapy. Some patients require surgical lysis of the adhesion. Estrogen's action in regard to collagen may influence recurrent adhesions and adhesions that form after manual disruption or surgical separation. This study assesses the efficacy of topical estrogen to separate the labia, recurrence, and estrogen related side effects. Estrogens may have a role in vaginal healing in genital surgery. Materials and Methods Retrospective chart review of 109 girls from 3 months to 10 years old (mean 44 months) who had labial fusion. Data was collected on the length of topical estrogen treatment, rate of successful separation, rate of recurrence, percentage requiring surgery, and post-operative outcomes. Results Mean length of topical estrogen treatment was 3.7 months (range 0 to 36 months), with separation in 79% (85/107) of patients. Forty-one percent (44/107) had recurrence of labial fusion one to five times (range 2 to72 months). Surgery was required in 21% (22/107). Ten percent of patients (2/21) had recurrence of labial fusion post-operatively (age 6,10 years, at 1, 18 month post-operatively). Neither of these two patients was responsive to topical estrogen cream post-operatively and required a second surgical separation. Side effects of estrogen were minimal breast development in 6 children and vaginal bleeding in one child. Discontinuance resulted in resolution of side effects. Conclusion Topical estrogens were effective treatment for labial fusion. After surgical separation of adhesions there was a 10% recurrence rate. Estrogen therapy initiated after surgical failure was unsuccessful.

Mutation analysis of the FRAS1 gene demonstrates new mutations in a propositus with Fraser syndrome

Fraser syndrome (OMIM 219000) is a rare, autosomal recessive condition with classical features of cryptophthalmos, syndactyly, ambiguous genitalia, laryngeal, and genitourinary malformations, oral clefting and mental retardation. Mutations causing loss of function of the FRAS1 gene have been demonstrated in five patients with Fraser syndrome. However, no phenotype-genotype correlation was established and there was evidence for genetic heterogeneity. Fraser syndrome is rare and the FRAS1 gene has 75 exons, complicating mutation screening in affected patients. We have screened two patients who fulfilled the diagnostic criteria for Fraser syndrome and three patients with related phenotypes (two patients with Manitoba oculotrichoanal syndrome and one patient with unilateral cryptophthalmos and labial fusion) for mutations in FRAS1 to increase the molecular genetic data in patients with Fraser syndrome and related conditions. We report two new mutations in a patient with Fraser syndrome, a frameshift mutation and a deletion of two amino acids that we consider pathogenic as both alter the NG2-like domain of the protein. Although we are still unable to clarify a phenotype-genotype relationship in Fraser syndrome, our data add to the list of mutations associated with this syndrome.

Treatment of Labial Fusion with Topical Estrogen Therapy

Twenty girls with labial fusion that covered at least 50% of the vaginal opening were treated with a topical estrogen cream. The cream was precisely applied to the fused area twice a day until the adhesions were totally lysed. After separation of the adhesions, a petroleum ointment (Vaseline) was applied twice a day to the labia minora for at least 1 month. The introital area was carefully rinsed twice each day before application of either the estrogen cream or the petroleum ointment. The mean age of the studied population was 13.2 months (range, 2 to 38 months). The mean duration of estrogen treatment was 2.4 months (range, 1 to 3.5 months). All patients were successfully treated. Five patients developed vulval pigmentation, which lasted for a mean of 1.5 months. One patient developed breast enlargement, which lasted for 1 month. The mean duration of follow-up was 3.1 months, and there was no recurrence of labial fusion during the period of follow-up. We conclude that treatment of labial fusion with topical estrogen therapy is safe and effective.

Abnormal steroidogenesis in three patients with Antley–Bixler syndrome: Apparent decreased activity of 17α‐hydroxylase, 17,20‐lyase and 21‐hydroxylase

Antley-Bixler syndrome (ABS) is characterized mainly by abnormal skeletal morphogenesis such as craniosynostosis and radiohumeral synostosis, and by ambiguous genitalia in some cases. The mechanisms resulting in these deformities have not been determined. The adrenal and gonadal function of three Japanese ABS patients were evaluated. Patient 1 (17-year-old-male) had bilateral cryptoorchidism, delayed puberty and symptoms of glucocorticoid deficiency. Patient 2 (14-year-old male) and patient 3 (4-year-old female) presented with emaciation. Additionally, patient 3 had partial labial fusion and common urogenital sinus. In each patient, blood sampling for steroid analysis before and after rapid adrenocorticotropic hormone (ACTH) stimulation was carried out. Additionally, urinary steroids were quantified. Molecular analysis of CYP17 and CYP21A2 were also performed. All patients showed elevated basal 17alpha-deoxysteroid levels. Although the 17alpha-deoxysteroid levels further increased after rapid ACTH stimulation, 17alpha-hydroxysteroids including cortisol did not respond, suggesting impaired 17alpha-hydroxylation. Patient 1 and patient 2 showed low adrenal androgen blood levels both before and after rapid ACTH stimulation. Patient 3 showed lower than normal excretions of urinary androgens. Additionally, a prolonged ACTH stimulation in patient 3 failed to elicit significant increase of adrenal androgens. These findings suggested impaired 17,20-lyase activity. In contrast to attenuated 17alpha-hydroxycorticosteroids, notably cortisol, elevated 17alpha-hydroxyprogesterone (17OHP) levels were observed, not only in pubertal patients (1 and 2) but also in prepubertal patient 3, indicating impaired 21-hydroxylation. This assumption was supported by increased urinary 21-deoxycortisol metabolite excretion in patients 2 and 3. With the exception of a heterozygous mutation of CYP17 in one of the patients, other mutations of this gene or CYP21A2 were identified in any of the patients. Combined decreased 17alpha-hydroxylation, 17,20-lyase activity and 21-hydroxylation was detected in three ABS patients. Considering that the enzymes responsible are all cytochrome P450 enzymes and that another cytochrome P450 enzyme, lanosterol 14alpha-demethylase, has recently been shown to be impaired in an ABS patient, we speculate that dysfunction of a system which commonly regulates cytochrome P 450 activity may be responsible for the ABS phenotype.

Labial Adhesions in a Girl with Isolated Premature Thelarche: The Importance of Estrogenization

Background: Premature thelarche, a benign condition that affects young girls, has been associated with elevated estrogen levels. On the contrary, labial fusion, a common pediatric gynecological problem, has been associated with low estrogen status. These two conditions are not known to coexist in the same patient. Case: We report the case of a 2-yr-old girl with premature thelarche and labial fusion occurring contemporaneously. She presented with a 7-month history of perivaginal itchiness and a palpable painless breast bud. Examination revealed unilateral breast enlargement (Tanner stage 2) and partially fused labia minora. Pelvic ultrasound findings and gonadotrophin responses to intravenous LHRH were consistent with a diagnosis of isolated premature thelarche. Furthermore, elevated serum estradiol levels were found. Comment: The combination of isolated premature thelarche and labial adhesions in our patient suggests the existence of other factors as well as estrogen insufficiency in the etiology of the latter.

Luteoma Recorrente da Gravidez com Virilização Materna e Fetal

Os luteomas da gravidez são pseudotumores ovarianos diagnosticados pelo exame ultra-sonográfico ou durante realização de cesariana e laqueadura pós-parto. Determinam, na segunda metade da prenhez, sinais de virilização materna em um quarto dos casos, o mesmo ocorrendo com a metade dos fetos femininos destas gestantes virilizadas nos quais se observa hipertrofia clitoridiana ou fusão labial. As dosagens séricas maternas dos hormônios androgênicos durante a prenhez e do sangue umbilical por ocasião do parto revelam taxas significativamente aumentadas. No exame ultra-sonográfico apresentam-se como estruturas sólidas ou cístico-sólidas, que após o parto tendem a regredir com o ovário readquirindo as dimensões normais em poucas semanas. Os autores apresentam uma paciente que em duas gestações sucessivas apresentou virilização materna e fetal. Ao exame ultra-sonográfico foram evidenciadas imagem ovariana nodular e dosagens elevadas dos androgênios plasmáticos.

Female circumcision

Female circumcision, more accurately known as female genital mutilation, is still a common practice in parts of Africa. This ritual genital operation can involve partial or complete excision of the clitoris and labia minora as well as infibulation (labial fusion). The case reported here involves a 16-year-old African girl with a complication of this mutilating procedure. J Pediatr Surg 36:1068-1069. Copyright © 2001 by W.B. Saunders Company.

Non-classic 21-hydroxylase deficiency in an 18-year-old female athlete: A case report

Background: In non-classic 21-hydroxylase deficiency, age at presentation and genital findings are variable. Late diagnosis with dramatic signs of virilization precludes early treatment and thus prevention of anatomic and psychosocial consequences. The following case illustrates the complexity of late diagnosis.Case: An 18-year-old West Indian female was seen for evaluation of clitoromegaly and hirsutism discovered in the emergency department when she presented after sexual assault. She had allegedly been drugged and raped in her dorm room. She was a college student with an athletic scholarship and had a striking masculinized, broad-shouldered appearance. She denied any use of anabolic steroids or other drugs. Menarche was at age 16 with infrequent menses. She was sexually active with 4 life-time partners, all male. On physical exam, her height was 152 cm, weight 50 kg, blood pressure 110/70 mm Hg. Breasts were hypoplastic with hyperpigmented nipples. She was hirsute with a Ferriman-Gallwey score of 14. Genitalia were abnormal with clitoris measuring 5.5 × 1.5 cm and posterior labial fusion. Initial non-fasting serum 17-hydroxyprogesterone level was 2890 ng/dL, testosterone was 274 ng/dL, and cortisol was 9 μg/dL. Chromosome analysis was 46, XX and ACTH stimulation test confirmed the diagnosis of 21-hydroxylase deficiency. The patient was initially reluctant to begin glucocorticoid treatment because of concern that it would decrease her muscle mass and negatively impact on her athletic performance and scholarship support. One year after diagnosis and 10 months after beginning treatment, she elected surgical correction of her clitoromegaly because of extreme embarrassment over having erections during sex. She underwent excision of most of the corpus cavernosum with repositioning of the glans. The neurovascular elements were preserved. The patient is pleased with the cosmetic result and reports no change in achieving orgasm. She has not notices any change in muscle mass or athletic performance since beginning glucocorticoid therapy.Conclusion: This case illustrates the somatic and genital abnormalities as well as the psychosocial impact of a delayed diagnosis of 21-hydroxylase deficiency in this young female athlete.

Vulvar disease in children: a clinical audit of 130 cases.

We evaluated 130 prepubertal girls presenting with a vulvar complaint to determine the spectrum and frequency of conditions seen in this age group. Of the patients, 41 (33%) had atopic or irritant dermatitis, 23 (18%) had lichen sclerosus, 21 (17%) had psoriasis, 15 (12%) had vulvar lesions, most often hemangiomas and nevi, and 13 (10%) had streptococcal vulvovaginitis. Diagnoses less frequently seen were staphylococcal folliculitis (four patients), labial fusion (three patients), genital warts (two patients), molluscum contagiosum of the vulva only (one patient), vulvar bullous pemphigoid (two patients), scabies nodules (one patient), erythema annulare centrifugum (one patient), tinea (two patients), and vitiligo (one patient). We also encountered vulvar presentations of systemic diseases (varicella, staphylococcal scalded skin syndrome, and Henoch-Schönlein purpura, all one patient each). We did not see candidal vulvovaginitis in this age group nor did we encounter bacterial infection with pathogens other than Staphylococcus aureus and S. pyogenes.

Phenotypic diversity in siblings with partial androgen insensitivity syndrome

The androgen insensitivity syndrome is a heterogeneous disorder with a wide spectrum of phenotypic abnormalities, ranging from complete female to ambiguous forms that more closely resemble males. The primary abnormality...