Labia Majora Research Articles

Labia Majora Flap Scrotoplasty and Perineal Reconstruction in Phalloplasty Patients: Technique and Outcomes

Labia Majora Flap Scrotoplasty and Perineal Reconstruction in Phalloplasty Patients: Technique and Outcomes

Female Genital Cutting and Deinfibulation: Applying the Theory of Planned Behavior to Research and Practice

At least 200 million girls and women across the world have experienced female genital cutting (FGC). International migration has grown substantially in recent decades, leading to a need for health care providers in regions of the world that do not practice FGC to become knowledgeable and skilled in their care of women who have undergone the procedure. There are four commonly recognized types of FGC (Types I, II, III, and IV). To adhere to recommendations advanced by the World Health Organization (WHO) and numerous professional organizations, providers should discuss and offer deinfibulation to female patients who have undergone infibulation (Type III FGC), particularly before intercourse and childbirth. Infibulation involves narrowing the vaginal orifice through cutting and appositioning the labia minora and/or labia majora, and creating a covering seal over the vagina with appositioned tissue. The WHO has published a handbook for health care providers that includes guidance in counseling patients about deinfibulation and performing the procedure. Providers may benefit from additional guidance in how to discuss FGC and deinfibulation in a manner that is sensitive to each patient's culture, community, and values. Little research is available to describe decision-making about deinfibulation among women. This article introduces a theoretically informed conceptual model to guide future research and clinical conversations about FGC and deinfibulation with women who have undergone FGC, as well as their partners and families. This conceptual model, based on the Theory of Planned Behavior, may facilitate conversations that lead to shared decision-making between providers and patients.

Intralymphatic Rosai-Dorfman Disease Associated With Vulvar Lymphedema: A Case Report of an Extremely Rare Phenomenon

Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease (RDD), is a self-limited histiocytic disorder of unclear etiology which most commonly presents with cervical lymphadenopathy. Purely extranodal presentation of RDD is uncommon, and isolated intralymphatic/intravascular confinement of this entity has not previously been described. We report a 16-yr-old female who presented with vaginal swelling and mass-like enlargement of the right labia. The mass had been present for nearly a year without pain or tenderness. Clinically, the lesion was thought to be a Bartholin gland cyst. Following surgical resection, histologic examination demonstrated a hypocellular myxedematous stroma with a mixture of ectatic thin and thick-walled vessels within which there were numerous collections of histiocytes, lymphocytes, and plasma cells. The histopathologic differential diagnosis included localized vulvar lymphedema, a specialized genital tract neoplasm, and childhood asymmetric labium majus enlargement. The histiocytes showed occasional plasma cells and lymphocytes within their cytoplasm, consistent with emperipolesis. Immunohistochemical studies showed that the histiocytes expressed CD163 and S100, while ERG and D2-40 highlighted their intralymphatic confinement, ultimately leading to the diagnosis of intralymphatic RDD. Intralymphatic RDD may present as vulvar lymphedema and can potentially mimic other myxedematous neoplasms of the vulvovaginal region.

Spontaneous vulvar hematoma as a rare manifestation of congenital hypofibrinogenemia. Case report

Introduction: Congenital fibrinogen disorders are rare conditions in which there are quantitative and qualitative alterations of factor I; the vast majority of patients are asymptomatic.Case presentation: A 19-year-old female patient with a history of congenital hypofibrinogenemia presented with spontaneous vulvar hematoma along with hypotension, tachycardia, stupor and hematoma of 20cm in the right labium majus. On admission, the young woman had hemoglobin 6.6 g/dL, fibrinogen 74 mg/dL and prolonged clotting times. She received red blood cells transfusion and cryoprecipitates, followed by surgical drainage and intravenous fibrinogen replacement, adjusting the dose according to fibrinogen levels in plasma. The patient presented progressive improvement without hemorrhagic recurrence and fibrinogen levels within the target values until hospital discharge.Discussion: Afibrinogenemia and hypofibrin­ogenemia are part of the quantitative factor I disorders; in the first case, there is total absence of circulating fibrinogen, and in the second case the levels are below 150 mg/dL. Sponta­neous vulvar hematoma as a severe hemorrhagic manifestation is not frequent in symptomatic patients; its treatment is based on fibrinogen replacement in an individualized manner and surgical management when required.Conclusion: Hypofibrinogenemia is a rare dis­ease, and fibrinogen replacement is one of the mainstays of treatment.

Use of Er:YAG laser in the treatment of vulvar lichen sclerosus.

ObjectiveThe aim of this research was to evaluate the efficacy and safety of Er:YAG lasers to improve signs and symptoms of vulvar lichen sclerosus.MethodsThe study population consisted of 28 women with symptomatic vulvar lichen sclerosus. Three nonablative, thermal-only Er:YAG laser treatment sessions (7 J/cm2, 2 Hz, 7 mm spot) were performed at 4-week intervals. Each patient was asked about the presence of symptoms, such as itching, pain, and coital pain. Clinical diagnosis was established on the basis of the presence of lesions such as hypopigmentation, ecchymosis, hyperkeratosis, excoriations, or fissures. The affected areas were divided by zones (clitoris, introitus, labia minora, labia majora, perineum, labial fusion, and effacement) and were visually evaluated. Based on the presence of symptoms, lesions, and affected zones, a scale with a maximum of 14 scores was established for use before and after treatment. The impact of lichen sclerosus on patients' lives before and after treatment was evaluated with an 11-point visual analogue scale, and treatment discomfort was assessed at each session.ResultsAfter analysis of each scoring component, individual statistically significant reductions were observed in itching, pain, ecchymosis, excoriations, and hypopigmentation.ConclusionThe Er:YAG laser is a safe, well tolerated, and effective method for the adjuvant treatment of vulvar lichen sclerosus. Three sessions with monthly intervals using the indicated parameters can quantifiably reduce the impact of lichen sclerosus on patients' lives.

Tilapia fish skin as a new biologic graft for neovaginoplasty in Mayer-Rokitansky-Kuster-Hauser syndrome: a video case report

To describe a McIndoe procedure technique for surgical management of Mayer-Rokitansky-Kuster-Hauser syndrome with the use of Nile tilapia skin as a scaffold for the proliferation of new vaginal epithelium. Surgical video article. Local Institutional Review Board approval and written permission from the patient were obtained. There were no conflicts of interest. University hospital. A 17-year-old woman who presented at our gynecology department with the complaint of primary amenorrhea. At physical examination, she had a phenotypically normal vulva with no vaginal canal. Magnetic resonance imaging of the abdomen and pelvis revealed normal ovaries and absence of uterus and vaginal canal. No other congenital malformations were found. Karyotype was 46,XX. The McIndoe procedure involved only a vaginal approach. Labia minora were separated, and a transverse midline incision of 3cm was made. The vesicorectal space was progressively dissected. Blunt dissection was performed initially with digital separation of tissues. The neovagina was then inspected with the introduction of a vaginal speculum, allowing for review of hemostasis. Blunt dissection was continued with the aid of the speculum, to reach the appropriate vaginal dimensions. Subsequently, a vaginal acrylic mold covered with two pieces of processed and sterilized tilapia fish skin was inserted and accommodated into the newly created cavity. The external side of the tilapia skin, which maintained its grayish coloration after the removal of the scales, stayed in contact with the acrylic mold, while the white internal side of the tilapia skin, which was previously attached to the fish's muscle, stayed in contact with the walls of the neocavity. The mold was held in position by four multifilament polyglactin 1.0 sutures in the labia majora, thus preventing expulsion. Anatomic data, such as measurement of the final canal length, and histomorphologic analysis, 180days after surgery. The patient remained on bed rest for 9days, after which the tilapia fish skin had been partially reabsorbed. After this time, the acrylic mold was removed. A larger plastic mold was then inserted and the patient was advised to wear it day and night for the first postoperative month. The vaginal mold had to be worn each night until normal sexual intercourse was possible. The final canal length 180days after surgery was between 8 and 9cm. For the histopathologic analysis, fragments of the lateral vaginal wall were removed 180days after surgery and showed the presence of stratified squamous epithelium with five cell layers, ectasic blood vessels, and occasional desquamated epithelial cells. The procedure described offered this patient an anatomic and functional neovagina by means of a simple, safe, easy, effective, quick, and minimally invasive procedure. Limitations include the experimental nature of this study, based on a single case report with no long-term outcome results. The tilapia fish skin is a low-cost and widely available biomaterial.

Self-Resolving Vulvar Breast Tissue Arising in the Post-Partum Setting: A Case Report and Review of Literature

During the postpartum period, breast engorgement in preparation for lactation may trigger the onset of vulvar labial nodules that present with pain and discomfort. These labial nodules may originate from ectopic breast tissue and can rarely present in women during the postpartum period in the labia majora. A 37-year-old African American female, gravida 1 para 1, presented to the Loma Linda University Medical Center with complaints of new onset labial swelling worsening 6 days following her full term spontaneous vaginal delivery. Additionally, our respondent complained of difficulty breastfeeding due to intermittent lack of milk production. She reported bilateral breast engorgement with tenderness, despite frequent attempts at breastfeeding. The respondent's presentation did not meet the criteria for other common differentials due to the physical characteristics of the nodules. The location of the nodules along the milk line led physicians to believe that the respondent was presenting with engorged extra-mammary breast tissue in the labia majora. The participant was told to observe her course over the next few days as she began to have milk production and ejection. The respondent was seen in clinic for her 6-week postpartum visit, and was no longer complaining of difficulty with breastfeeding. The labial nodules had resolved spontaneously. A literature search yielded no case reports that described a case of an extra-mammary vulvar mass that self-resolved with resolution of breast engorgement. The infrequent presentation of extra-mammary vulvar tissue makes it difficult to conclude a guideline for diagnosis and management.

Carcinoma sebáceo extraocular de vulva. Reporte de caso

El carcinoma sebáceo es un tumor maligno poco frecuente de la piel que generalmente aparece en la región de cabeza y cuello. Su curso clínico es agresivo y las recidivas locales / metástasis a distancia son habituales. Se han notificado algunos casos de carcinoma sebáceo del tracto genital femenino. Aunque las glándulas sebáceas son prominentes en la vulva, los carcinomas sebáceos rara vez ocurren en esta ubicación. Poco se conoce sobre el comportamiento y pronóstico de este tipo de cáncer. Se presenta un caso de paciente de 69 años con antecedentes de un tumor de 3 × 2 centímetros por 9 meses, en el labio mayor izquierdo. No tenía antecedentes médicos o familiares de neoplasias malignas. Se observó aumento de tamaño durante este período; por lo demás, estaba asintomático. La lesión fue extirpada totalmente. El tumor estaba compuesto por lóbulos de células tumorales con diferenciación sebácea. El informe histopatológico reveló carcinoma sebáceo de vulva. Debe diferenciarse de otros carcinomas vulvares morfológicamente similares, pero biológicamente distinto.

Gebe bir kadında vulvada dev fibroepitelyal polip.

Vulvar fibroepithelial polyp in a pregnant woman is a rare clinical condition. In this study, a case of a giant fibroepithelial polyp of vulva in a pregnant woman is reported. A pregnant woman presented with a pedunculated mass over right labium majus that grew up during pregnancy period was surgically removed. S urgical excision of the tumour was performed and postoperative recovery and pregnancy follow-up were uneventful. Fibroepithelial polyps are common lesions of skin but unusual locations have been rarely reported in literature. To the best of our knowledge , the presented case is one of the largest fibroepithelial polyp of vulva in a pregnant woman.

Labiaplasty with Clitoropexy.

Supplemental Digital Content is available in the text.

Total Body Skin Examination Practices: A Survey Study Amongst Dermatologists at High-Risk Skin Cancer Clinics.

Although total body skin examination (TBSE) is the primary screening mechanism for melanoma, there is no consensus on which anatomic sites a screening TBSE should include. We sought to establish which anatomic sites are examined during routine (>90%) TBSEs of patients at high risk for skin cancer. A Google survey was emailed to 173 international dermatologist skin cancer specialists. More than 75% of participants reported routinely examining the scalp, ears, face and neck, trunk, breasts, inframammary areas, axillae, extremities, palms and soles, nails, interdigital spaces, and buttocks. The least frequently inspected anatomic sites included genitalia, with male genitalia more frequently examined than female (penis n = 39; 52%; labia majora n = 21; 28%; P = 0.003), the perianal region (n = 26; 34.7%), and the ocular conjunctiva and oral mucosa (n = 35; 46.7%). Participants cited not screening these areas because of perceived patient discomfort, low prevalence of malignancy, and the expectation that other specialists examine the area. The role of routine surveillance of neglected anatomic sites is unclear and warrants further discussion weighing potential mortality benefit against the incidence of melanoma in obscure sites, morbidity of intervention in sensitive sites, cost-effectiveness, and potential for patient discomfort.

Complete Labial Fusion in a Postmenopausal Woman Presenting with Retention of Urine

Complete labial fusion with retention of urine is a rare clinical entity. It occurs when the labia are fused in midline, forming a raphe. It usually develops in postmenopausal women with hypoestrogenism. The usual treatment is with topical estrogen and surgical separation followed by some dilatation. Here we present a case of a 58 years’ postmenopausal,unmarried, nullipara woman who came to our emergency ward with retention of urine.She had history of incomplete voiding and dribbling of urine since six months. General examination and investigations were normal. Her clitoris was normal in size, and the labia majora and anus were visualized well; however, the urethral meatus and vagina were not seen because of fused labia minora.Patient required surgical excision of fused labial folds to relieve the retention.Keywords: Labial fusion; postmenopausal; urinary retention.

97. A Rare and Extreme Case of Vulvar Swelling in the Setting of Nephrotic Syndrome in a Prepubertal Girl

Idiopathic nephrotic syndrome is a glomerular disease characterized by proteinuria which leads to hypoalbuminemia and peripheral edema. Dependent edema is a common sequela of the protein-deficient state, often starting as periorbital edema and migrating to the lower extremities and sacral region. In boys with protein-wasting disorders, this may result in scrotal edema. Less frequently described in the literature is edema in the female homologue, the labia majora. We present a case of unusually severe dependent labial edema in a prepubertal girl with idiopathic nephrotic syndrome.

Sigma-lead Male-to-Female Gender Affirmation Surgery: Blending Cosmesis with Functionality.

Current male-to-female (MtF) sex-reassignment-surgery techniques have not been fully successful to achieve the ideal objectives. The ordeal of multiple procedures, associated complications, and suboptimal results leads to high rate of dissatisfaction. We have tried to overcome functional inadequacy and address the esthetic issues for outer genitalia and vagina with our innovative "true shape sigma-lead SRS: Kaushik's technique," which has now become the technique of choice for MtF genital SRS for our patients. Between April 2007 and April 2017, authors performed 386 sigma-lead SRS in MtF transsexuals. Results were analyzed based on complications, resurgeries, and esthetic/functional outcomes. Corrective SRS using rectosigmoid constituted 145 cases and is not a part of this study. Maximum follow-up was 7 years (average 34 months). Seventy-eight (20.2%) patients had complications, majority being minor (97.4%). Forty-four (11.4%) required resurgeries, 10 (2.6%) were corrective for introital stricture and mucosal prolapse, whereas 34 (8.8%) opted for optional minor esthetic enhancement. The overall satisfaction rate for cosmetic and functional outcomes was 4.7 out of 5. In addition to review of the literature, innovations in the technique have been explained. Kaushik's sigma-lead MtF SRS technique is a step short to become the gold standard of genital SRS because it has proven to be safe and reliable. It allows faster healing, minimal dilation, and nearly natural cosmetic results in the form of clitoris/clitoral hood, labia minora, labia majora along with self-lubricating, fully deep, and sensate neovagina with orgasmic capabilities. This is perhaps the largest reported series of rectosigmoid use in transsexuals carried out for primary vaginoplasty.

33. Management of Gonadal Cysts in Patients Without Gonadectomy in Complete Androgen Insensitivity Syndrome

Complete Androgen Insensitivity Syndrome (CAIS), is an X-linked genetic disease, where female-appearing 46XY patients have testes located in the internal inguinal ring or in the labia majora. In the past, prophylactic gonadectomy has been performed, given risks of germ cell tumor development. However, gonadal surveillance algorithms have been proposed as an alternative, due to challenges with replacement therapy (HRT) and ethical concerns. In this case study, we review ultrasonographic changes in gonads of 3 patients with CAIS, and pathology results with one patient who elected for laparoscopic cystectomy as part of her surveillance.

Extensive Squamous Metaplasia in Hidradenoma Papilliferum of Vulva: Illusion of Squamous Cell Carcinoma

Hidradenoma papilliferum is a rare, slow-growing, benign, cystic, papillary tumour occurring in middle-aged females, in the anogenital region commonly. It involves the anogenital mammary-like glands and is usually asymptomatic. Simple excision is curative. We present a case of hidradenoma papilliferum of long-standing duration with extensive squamous metaplasia. A post-menopausal lady presented with an exophytic growth on the left labia majora and part of mons pubis. It was initially misdiagnosed on the basis of vulvar biopsy and managed as squamous cell carcinoma of the vulva. The final histopathology revealed an epithelial tumour involving the vulva with extensive squamous metaplasia. The tumour involved mainly the dermis and the subcutaneous tissue. This is the first case in the literature where the diagnosis of hidradenoma papilliferum was confused with squamous cell carcinoma of the vulva due to its clinical presentation and extensive squamous metaplasia. Diagnosis of hidradenoma papilliferum should be kept in mind if a woman presents with vulval papillary or nodular lesions. An expert clinical and histopathological review should be done to avoid misdiagnosis.

A 5-year follow-up of vulval swelling due to extraskeletal myxoid chondrosarcoma: A rare case report.

Vulval extraskeletal myxoid chondrosarcoma (EMC) is a rare cause of vulval swelling, reported <10 times in the literature to date. EMC in this location is frequently misdiagnosed due to its rarity, and patients may incur delays in diagnosis and treatment. We herein present the diagnosis and management of the case of vulval EMC in a 42-year-old Caucasian female patient who presented in 2011 with a swelling on the right labium majus. The tumour was initially misdiagnosed as a Bartholin's cyst and managed conservatively. The tumour was ultimately diagnosed as EMC and treated by radical surgical excision and adjuvant radiotherapy. The aim of the present study was to report the results after a long-term follow-up period and review the available relevant literature.

Superficial Genital Infantile Hemangiomas

Abstract Introduction. Superficial infantile hemangiomas (IH) are rarely localized in the genital region. Case Report. We present 8 infants with IHs in the genital region (3 boys and 5 girls). Our patients had only superficial IHs, their internal organs were not affected and there were no associated anomalies. In the boys, IHs were localized on the scrotum and the foreskin in 2 cases and 1 case, respectively. In the girls, IHs were on the labia majora and near the vaginal introitus. None of the IHs showed any IH-related complications. No therapy for IHs was administered in any of the presented infants, either because IHs were small and non-aggressive, or because their parents rejected the proposed therapy. Conclusion. Genital IHs have increased tendency for ulceration, so thorough examination and follow-up are of outmost importance. If therapy is indicated, oral propranolol is the treatment of choice for genital IHs.

Altered mRNA expression due to insertion or substitution of thymine at position +3 of two splice-donor sites in the androgen receptor gene.

We have discovered two types of 5' intronic gene mutation that impair androgen receptor (AR) mRNA expression severely, and cause complete androgen insensitivity. Labium majus skin fibroblasts (LMSF) hemizygous for each mutation had negligible specific androgen binding, and did not react to an antibody against an N-terminal peptide of the AR. Both mutations were detected by direct sequencing of exons PCR-amplified with flanking primers. One mutation is an adenine to thymine transversion at position +3 of the intron 6 splice-donor site. Using LMSF mRNA, RT-PCR of a portion of the AR androgen-binding domain yielded a small amount of a 302-bp mutant fragment instead of a 433-bp wild-type product. Sequencing established that exon 5 was followed, out of frame, by exon 7: exon 6 was skipped. The other mutation is a thymine insertion at the +3 position of the intron 1 donor-splice site. RT-PCR and sequencing revealed a small amount of normal-size mRNA with normal exon 1-exon 2 splicing. Quantitative RT-PCR on mutant LMSF showed AR mRNA levels were well below 10% of normal; hence, most of the aberrant AR mRNA resulting from each mutation is probably unstable. The misbehavior caused by these two mutations indicates that in the AR the splice-donor site +3 adenine is critical; indeed, 57% of eukaryotic introns have adenine in the +3 position, while only 2% have thymine.

Management of vulvar lymphedema of filarial origin in a pregnant woman and its outcome: A case report

Lymphedema of filarial origin affecting the vulva is extremely rare. It is a dilemma if seen in a pregnant woman as there are no guidelines regarding excision and the mode of delivery. With the World Health Organization-driven global program to eradicate filaria, it is unfortunate to see such cases. We report of a woman who had massive lymphedema of both the labia majora following filarial infection with a small secondary ulcer. She presented to our outpatient department in early pregnancy. After detailed counseling with the couple, a decision was taken for excision. The same was carried out. The wound healed well but the lymphedema recurred after 6 weeks. She was thereafter managed symptomatically. Pregnancy advanced without any complication. Her intrapartum management for a successful vaginal delivery is outlined in the report.