A long-term complication of clitoral cyst after female genital mutilation.

Female genital mutilation (FGM) was seen in 30 countries, especially in Africa and also in Asia and the Middle East. According to WHO data, Somalia is where FGM is performed most frequently. Our study aimed to evaluate the recordings of patients with FGM who were diagnosed with a traumatic clitoral cyst. We identified the clitoral cyst cases between February 2015 and August 2020. We collected clinical, surgical, sociodemographic, and histopathological details such as age, marital status, patient resume, age at which FGM was performed, complaints, size of the cyst consultation reasons, FGM procedural long-term complications, sexual function, husband polygamic relationship status, and histological findings. A total of 21 patients diagnosed with clitoral cysts were included in the study. The technique was easily applied in every patient, and the cysts were removed intact, except in 2 patients. There were no intraoperative complications; only minimal bleeding was seen. Except for one patient, all had unilocular cysts, and the final pathological examination revealed an epidermal inclusion cyst. We observed a neuroma developed due to genital trauma due to FGM in one of our patients. Female circumcision and its consequences are not familiar to many healthcare professionals in the developed world. We want to increase awareness of female circumcision and its long-term complication of clitoral cysts among healthcare professionals worldwide.

Clitoral preputial edema can be mistaken for clitoromegaly: a clinical analysis of ten cases.

Background and objectives: We herein reported ten, female neonates with transient clitoral preputial edema, which was mistaken for clitoromegaly. Although it is well known that the clitoris is prominent in premature, female neonates, there are as of yet no reports of clitoral preputial edema in full-term neonates. The present study was conducted to clarify the clinical course of clitoral preputial edema. Methods: Seventeen, Japanese patients aged < 6 months with suspected clitoromegaly were enrolled, and their clinical course was analyzed retrospectively. Clitoral preputial edema was defined by 1) a normal clitoral glans despite edema; and 2) the absence of established differences of sexual development, such as 21-hydroxylase deficiency. Results: Ten of the 17 patients with suspected clitoromegaly had clitoral preputial edema; eight of the ten patients were full-term, and the remaining two were preterm neonates. The median age at the first visit was 8 days. Edema of the labia minora and labia majora, rugosity of the labia majora, and hymenal polyps often accompanied the clitoral preputial edema. Seven patients were examined at our division during the neonatal period, and three patients were examined in the post-neonatal period. Age at reduction of the clitoral width to < 7 mm ranged from 8 to 74 days in four of the seven neonatal patients. In the three post-neonatal patients, age to reduction in the clitoral width ranged from 107 to 243 days. Conclusions: Transient clitoral preputial edema is often mistaken for clitoromegaly. The key to diagnosing clitoral preputial edema lies in its characteristic appearance and improvement course.

Fused Clitoral Hood Presenting as a Periclitoral Mass.

BACKGROUND: Periclitoral masses are seldom reported. One cause is a congenitally fused clitoral prepuce (or clitoral hood). CASE: A patient was referred to our gynecologic oncology practice with a suspicious periclitoral mass for evaluation of possible malignancy. The clitoral hood was, in fact, fused such that the glans was completely covered. Surgical excision of the distal prepuce (or clitoral hood) expelled trapped sebaceous material and revealed a normal-appearing glans clitoris. Clitoral hood reconstruction restored the patient's external genitalia to normal anatomy. CONCLUSION: Fused clitoral prepuce causing trapped sebaceous material can mimic an expanding periclitoral mass and should be considered in the differential diagnosis.

Corporoplasty: A simplified technique for clitoroplasty.

INTRODUCTION: Clitoroplasty constitutes an important step in feminizing surgery for congenital adrenal hyperplasia (CAH) (1). In this video we present a technique that aims to preserve clitoral sensitivity and engorgement while minimizing the risk of neurovascular lesion. MATERIALS AND METHODS: We present a video of a three-year-old girl with history of CAH classical form, PRADER-III, who underwent clitoroplasty. After an initial endoscopic evaluation of the urogenital sinus, the clitoris was degloved and a rectangular incision was made on the ventral corpora cavernosa 15mm above the corpora bifurcation and 0.5 mm below the coronal sulcus. The cavernous tissue was partially resected. The upper and lower borders of the rectangular gap were closed by a 5-0 PDS running suture similar to the Mikulicz technique. Next, the edge of the glans was deepithelialized to reduce its size. For improved clitoral positioning, the clitoris was sutured to the pubic fat. From that point onward the procedure followed that of a standard vaginoplasty using the en-bloc technique (2-4). Thus far we have performed this technique in 33 patients, with 31 of them being girls with CAH and 2 being women with clitoral hypertrophy. CONCLUSION: Corporoplasty is a simplified technique for clitoroplasty, with the advantage being that is faster and safer than the technique that involves the dissection of the neurovascular bundle. In addition, corporoplasty has the possible benefit of preserving the cavernosal blood flow that permits the engorgement of the clitoris during sexual arousal.

Clinical and diagnostic approach of male pseudo hermaphroditism with os-clitoris in French bulldog: A case report.

INTRODUCTION: Hermaphroditism is less frequently reported in dogs and is often associated with infertility. CASE REPORT: An 8-month-old French bulldog weighing 5 kg with an enlarged structure protruding from the vulva was clinically diagnosed with hermaphroditism. Physical, hormonal assay, computed tomography, and gross and histological studies were done in addition to successfully detailed surgical correction. On physical examination, the dog showed the presence of an os-clitoris protruded from the vulvar labia. Hormonal levels of estradiol, testosterone, and progesterone were 6.39 pg/ml, 0.4 ng/ml, and 8.67ng/ml, respectively. Surgical removal of internal gonadal tissues and os clirectomy operations were conducted after the exploratory laparotomy. The removed gonadal tissues were identical to that of a female with testicles instead of ovaries, according to a gross examination. Histological examination confirmed gonads as testis, with inactive seminiferous tubules and epididymis attached to uterine horns. CONCLUSION: The congenital anomalies in the present case were diagnosed as male pseudohermaphroditism (MPH). Surgical correction was performed, and the owner's satisfaction was achieved.

Management of Clitoral Melanoma Presenting as an Exophytic Clitoral Mass: A Case Report and Review of the Literature.

Primary mucosal melanomas of the female genital tract account for one percent or less of all cases of melanoma with even fewer originating in the clitoris. Given the rarity of diagnosis of clitoral melanoma, there is a paucity of data guiding management. There is no supporting evidence that radical vulvectomy (with or without inguinal lymphadenopathy) is associated with improved disease-free or overall survival compared to partial vulvectomy or wide local excision. Additionally, there is no data to evaluate the role of sentinel lymph node biopsy or extensive lymphadenectomy in clitoral melanoma, however previous evidence demonstrates the utility of regional lymph node sampling in predicting survival in women with female genital tract mucosal melanoma. Adjuvant therapy considerations are often extrapolated from their use in treating cutaneous melanomas, including immune checkpoint inhibitors and other immunotherapy agents. Adjuvant radiation therapy has limited utility except in cases of bulky, unresectable disease, or when inguinal lymph nodes are positive for metastasis. The 52 year-old patient presented in this review was diagnosed with locally invasive advanced stage clitoral melanoma presenting as an exophytic clitoral mass. She underwent diagnostic primary tumor resection, which demonstrated ulcerative melanoma with spindle cell features extending to a Breslow depth of at least 28 mm. She subsequently underwent secondary wide local excision with groin sentinel lymph node biopsy, and adjuvant treatment with pembrolizumab. This article also emphasizes the importance of a multidisciplinary team involving gynecologic oncology, medical oncology, radiology, and pathology for management of this rare type of primary mucosal melanoma of the female genital tract.

Three-Step Excision: An Easy Way for Composite Labia Minora and Lateral Clitoral Hood Reduction.

BACKGROUND: Hypertrophy of the labia minora with lateral clitoral hood redundancy is common, but the excess clitoral hood is often overlooked during labiaplasty, which may result in imbalanced and unsatisfactory outcomes. The purpose of this study was to present an easy method of three-step excision for composite labia minora and lateral clitoral hood reduction. METHODS: The procedure was performed in three steps: the prominent clitoral hood skin parallel to the labia majora and the clitoral hood sulcus was removed first; then, the protuberant portion of the labia minora was removed by wedge resection; and finally, a triangle at the junction between the labia minora and the clitoral hood was trimmed. Patients were required to return for examination on the first day and within 14 days after surgery. Follow-ups were arranged on the Internet or at the outpatient clinic. Satisfaction questionnaires were completed during follow-up. RESULTS: Between January of 2016 and January of 2020, 136 patients underwent composite labia minora and lateral clitoral hood reduction. Ages ranged from 9 to 55 years. Six patients experienced complications (4.4 percent) and five underwent revision surgery (3.7 percent). The overall satisfaction rate within 14 days and after 3 months following surgery was 91.0 percent and 95.5 percent, respectively. Preoperative discomfort was resolved in 43.0 percent and significantly relieved in 54.2 percent, and sex life was improved in 70.9 percent. CONCLUSIONS: Three-step excision is effective and safe for composite labia minora and lateral clitoral hood reduction with very high satisfaction. Considering the advantages of easy preoperative design and controllable tissue removal, this method could be a better alternative to the present procedures. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Co-occurrence of TCF3-PBX1 gene fusion, and chromosomal aberration in a pediatric pre-B cell acute lymphoblastic leukemia with clitoris swelling: A case report and literature review.

RATIONALE: Clitoris swelling as the initial clinical presentation of acute lymphoblastic leukemia (ALL) is extremely rare. These patients may be misdiagnosed with acute myeloid leukemia or solid tumor, and the main treatment can also be delayed. PATIENT CONCERNS: A 2.10-year-old girl was referred to the pediatric surgery clinic with a worsening onset of clitoris swellings. The patient was afebrile and well appearing. Multiple retroperitoneal mass were confirmed by computed tomography (CT) and high serum neuron-specific enolase level was high. She was scheduled for an abdominal biopsy from the retroperitoneal mass suspicious of neuroblastoma. DIAGNOSES: The child was eventually diagnosed as having precursor B cell ALL with central nervous system involved, with TCF3-PBX1 fusion gene and additional chromosomal aberrations, based on examinations of the bone marrow and brain magnetic resonance imaging. INTERVENTIONS: Before the diagnosis of leukemia, the patient was given symptomatic treatment for 1 week. She was treated with chemotherapy in accordance with the Chinese Children's Cancer Group protocol 2015 after confirmed diagnosis. OUTCOMES: After induction chemotherapy for ALL, although the girl had transiently clinical remission, the bone marrow aspirate indicated a poor outcome. Our patient discontinued treatment and discharged. From literature review, there was only 1 case of in acute myeloid leukemia with clitoris swelling as the initial symptom. LESSONS: The clinical symptoms of ALL with clitoris swelling are not typical, with a high rate of misdiagnosis. When the cause of clitoris swelling is unknown, ALL should be considered. Bone marrow aspiration must be done before doing a more invasive investigation like biopsy.

Anthropometric biomarkers for abnormal prenatal reproductive hormone exposure in women with Mayer-Rokitanksy-Küster-Hauser syndrome, polycystic ovary syndrome, and endometriosis.

OBJECTIVE: To study whether markers of prenatal exposure to reproductive hormones are related to Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, polycystic ovary syndrome (PCOS), and endometriosis. DESIGN: Case-control study. Comparison of sex hormone-related external genital and digital characteristics in cases and controls. SETTING: University hospital. PATIENT(S): We enrolled 172 women in four groups-women with MKRH, women with PCOS, women with endometriosis, and controls (43 in each group). INTERVENTION(S): Measurement of two anthropometric biomarkers: anogenital distance and digit ratio. MAIN OUTCOME MEASURE(S): Anogenital distance was measured from the anus to the anterior clitoral surface (AGDac) and from the anus to the posterior fourchette (AGDaf). For the digit ratio we used a direct, as well as a computer-assisted graphic measurement to measure the length of the second and fourth digit. RESULT(S): After adjustment for body mass index and age, AGDac was the shortest in endometriosis and the longest in PCOS groups, with a mean difference of 10 mm (95% confidence interval 3.1-16.8). AGDaf but not AGDac measures were found to be significantly larger in the MRKH group, with a mean difference compared with controls of 2.6 mm (95% confidence interval 0.1-5.2). The digit ratio was not significantly different between the groups. CONCLUSION(S): In this study we did find limited evidence for androgen exposure during the development of MRKH. This is compatible with the hypothesis that the uterovaginal agenesis may have been the result of temporary prenatal exposure to altered gonadal hormone concentrations. For endometriosis and PCOS we confirm previously observed associations for anogenital distance reflecting possible estrogen-based and androgen-based intrauterine origins, respectively. DUTCH TRIAL REGISTRATION NUMBER: NTR7492.

The Nomogram of Clitoral Length and Width in Iranian Term and Preterm Neonates.

Background and Objectives: Clitoromegaly is an important parameter in the evaluation of ambiguous genitalia in neonates, but the normative data for clitoral size in newborns have racial/ethnic differences. The present study aimed to determine clitoral length (CL) and clitoral width (CW) values and establish cutoff measurement to define clitoromegaly in both term and preterm Iranian neonates for the first time. Methods: A total number of 580 female newborn infants delivered at 28-42 weeks of gestation were enrolled in the study, and their CL and CW were measured on the first 72 h of birth. Data about birth weight (BW), body length (BL), and head circumference (HC) of newborns; mothers' age; and gestational age (GA) were recorded, too. Results were presented as mean ± standard deviation (SD) for quantitative variables and were summarized by frequency (percentage) for categorical variables. Backward stepwise regression analysis was used for prediction of CL and CW. Results: Among 580 Iranian female newborns studied, 187 were term neonates and the other 393 newborns were preterm. Mean ± SD values of CL were 6.11 ± 0.39 mm in term infants and 5.45 ± 0.64 mm in preterm infants (P < 0.001). Mean ± SD values of CW were 4.22 ± 0.43 in term infants and 3.68 ± 0.53 in preterm infants (P < 0.001). Regression analysis showed that CL was correlated with GA considered by last menstrual period, BL, BW, and HC; and CW was associated with GA, BL, and BW. Conclusion: This study suggests normative values (mean + 1, 2, and 3 SD) of CL and CW according to GA, which can be used as a reference for Middle East's newborns, especially Iranian newborn babies.

Clitoral leiomyoma in a premenopausal woman: a case report.

BACKGROUND: Extrauterine leiomyomata is an uncommon lesion that can lead to several problems of differential diagnosis, especially when localized in the external genitalia. There are few reports in the English literature and a novel association with Alport's syndrome has been investigated since the 1980s. CASE PRESENTATION: Here, we describe the case of a premenopausal woman who presented with an indolent swelling of the right interlabial fossa that resulted in a Bartholin cyst. In addition to this cyst, a benign leiomyoma of the right side of the clitoris was also found and removed. Our patient refused any further examination, although she was informed that genetic counselling could be organized to rule out an association with Alport's syndrome. CONCLUSIONS: Extrauterine leiomyomata localized in the external genitalia is an uncommon lesion arising from smooth muscle cells around vascular epithelium or erectile tissue. Since an association between extrauterine leiomyomata and Alport's syndrome has been described, genetic testing can be proposed to these patients. Upper intestinal tract symptoms such as dysphagia should prompt a gastroenterological evaluation as an association with an esophageal leiomyomatosis has been described.

Clitoral Epidermal Inclusion Cyst Leading to Anorgasmia: A Case Report and Literature Review.

BACKGROUND: Clitoral epidermal inclusions cysts are most frequently seen following trauma, especially female genital mutilation. Spontaneous clitoral epidermal inclusion cysts are rare with an unclear etiology and their impact on later sexual function has not been described. CASE: A 15-year-old spontaneously developed a clitoral mass that progressively enlarged over seven years, ultimately leading to secondary anorgasmia. Surgical removal resulted in restoration of normal anatomy and complete return of clitoral function. Final pathology revealed the mass to be an epidermal inclusion cyst. SUMMARY AND CONCLUSION: Clitoral epidermal inclusion cysts typically present in childhood or early adolescence and can lead to sexual dysfunction if left untreated. Physicians must consider the potential sequelae of these cysts when counseling and managing these patients.

Genital Neurofibromatosis Presenting as Painful Clitoromegaly.

Neurofibromatosis type 1 (NF1) is a neurocutaneous disorder caused by a mutation on chromosome 17 of the gene for neurofibromin. Patients with this condition are prone to develop benign and malignant tumors of the central and peripheral nervous systems. Genitourinary involvement in children with NF1 is uncommon and primarily affects the bladder. External genital manifestations of NF1 are rare with sporadic reports of children presenting with enlargement of their penis, clitoris, or labia due to a neurofibroma. We present a case of painful clitoromegaly due to a plexiform neurofibroma in a child.

A rare cause of clitoromegaly: Epidermoid cyst.

Clitoromegaly due to non-hormonal causes is rare. In this case, we aimed to present an epidermal cyst that caused clitoromegaly after traditional female circumcision. A 22-year-old African female was referred to our clinic with enlarged clitoris. There is a mobile, soft, nonfluctuant mass with a size of 6 cm originating from the clitoral region at physical examination. Under spinal anesthesia the clitoral mass was excised totally and labioplasty was performed. Histopathologic examination was reported as epidermal cyst. Epidermal cyst should be considered after hormonal reasons are excluded in patients with clitoromegaly who have a history of trauma.

Case report: epidermoid inclusion cyst of the clitoris as a long-term complication of female genital mutilation.

BACKGROUND: Female genital mutilation is a common procedure in sub-Saharan Africa that causes serious short- and long-term complications. Although physicians can overcome these complications sometimes, they can be very confusing to diagnose. In this report, we discuss the surgical management of a patient with an epidermal inclusion cyst of the clitoris as a long-term complication of type III female genital mutilation. CASE PRESENTATION: A healthy 43-year-old African woman who was a nonsmoker and nonalcoholic presented with a large genital mass causing difficulty in urination and sexual discomfort. The patient had three full-term spontaneous vaginal deliveries without any complications. Perineal examination revealed a 6 × 10-cm, well-circumscribed, mobile, nontender, rounded cystic swelling in the right periclitoral area that was obstructing the urinary meatus and vaginal introitus. A surgical procedure was performed for total excision of the clitoral mass. Pathological findings showed an epidermoid inclusion cyst. CONCLUSIONS: Besides increasing clinicians' awareness of female genital mutilation and its long-term complications, public education campaigns should be designed to eradicate this practice.

An Adolescent Girl with a Complaint of Clitoral Swelling.

BACKGROUND: We present the case of an adolescent girl with prominent clitoral swelling as the first symptom when she presented to the emergency department, and who was subsequently diagnosed with nephrotic syndrome. CASE: A 14-year-old adolescent girl was admitted with painless clitoral swelling. She denied recent masturbation, itching, or discharge. She was within the last few days of menstruation. Physical examination revealed clitoral edema without erythema or genital edema. Urine dipstick test and microscopic evaluation revealed protein 2+, blood 3+, abundant erythrocytes and 9-10 leukocytes. A few days later, additional clinical findings, such as pretibial and facial edema, were diagnosed as nephrotic syndrome. SUMMARY AND CONCLUSION: This case is a reminder that clitoral swelling is to be considered a sign in the diagnosis of nephrotic syndrome, even when it occurs alone.

Female Pseudo Hermaphroditism: Late Onset Congenital Adrenal Hyperplasia.

Non-classic congenital adrenal hyperplasia is a genetic condition caused by deficiency of 21- hydroxylase deficiency (NCAH). It is a milder and later onset form of a genetic condition known as congenital adrenal hyperplasia. We present four cases of non-classical congenital adrenal hyperplasia presented in gynae OPD foundation university medical college Fauji foundation hospital from Jan 2016 to March 2017. The presenting complaints were hirsuitim, menstrual problem and virilization of genitalia. Two girls were having primary amenorrhea while rests of two were having secondary amenorrhea. Two patients were already diagnosed as non-classical congenital adrenal hyperplasia due to ambiguous genitalia at the time of birth while the rest of two with marked clitoromegaly were diagnosed during workup in gynae OPD. Menarche was achieved successfully among those with primary amenorrhea after treatment. All four girls were referred to plastic surgery for clitoral reduction surgery. The post-surgery patient satisfaction level was high. Correct diagnosis of the disease can cure the patient instead of letting her live a life of being labeled with social stigmata of an intersex individual.

Primary amenorrhoea and clitoromegaly in a nulliparous woman: successful medical and surgical management.

Clitoromegaly is an important sign of virilisation and poses difficulty in sex determination, when present since birth. The diagnosis and treatment in an adult is a major challenge to the treating gynaecologist. The primary reason for its development is androgen excess due to congenital adrenal hyperplasia, polycystic ovarian syndrome, ovarian virilising tumours, neurofibromas, adrenal neoplasm and prolonged intake of anabolic steroids. A case of young nulliparous married woman who presented with primary amenorrhoea and clitoromegaly and was managed successfully has been reported.

A 15-Month-Old Girl Presenting With Clitoromegaly and a Chest Mass.

A 15-month-old girl was initially referred for endocrine evaluation for clitoromegaly and subsequently found to have an adrenal mass that tracked along the paravertebral region and was associated with increased vascular markings along the skin. Neurologic examination was normal. Magnetic resonance imaging of the chest mass demonstrated a serpiginous lesion along the intercostal margins. Initial differential diagnosis included neuroblastoma, ganglioneuroblastoma, vascular lesion, or nerve sheath tumor. Biopsy was consistent with plexiform neurofibroma. Subsequent examination revealed features consistent with neurofibromatosis type 1. A review of clinical features of NF1 is provided in the case report.