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.

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.

Canine Clitoral Carcinoma: A Clinical, Cytologic, Histopathologic, Immunohistochemical, and Ultrastructural Study.

Vaginal and vulvar tumors are uncommon in dogs. Knowledge of canine primary clitoral neoplasia is restricted to a few case reports, and only carcinomas have been reported. Cytologic and histologic features reported in the literature seem to overlap with those of canine apocrine gland anal sac adenocarcinoma (AGASA). Clinical features also recall those of canine AGASA, such as locoregional metastases and hypercalcemia of malignancy (HM). In this study, 6 cases of primary canine clitoral carcinomas (CCCs), with and without HM, were investigated by means of cytology, histopathology, electron microscopy, and immunohistochemistry for neuroendocrine markers including chromogranin A (CGA), synaptophysin (SYN), neuron-specific enolase (NSE), and S-100. In all 6 tumors, cytologic findings were consistent with malignant epithelial neoplasia of apocrine gland origin. The tumors examined were classified into 3 different histological patterns representing different degrees of differentiation: tubular, solid, and rosette type. Both CGA and SYN were mildly expressed in 2 of 6 tumors, while NSE was consistently expressed in all 6 cases. None of the tumors were S-100 positive. Transmission electron microscopy revealed electron-dense cytoplasmic granules compatible with neuroendocrine granules in all 6 cases. CCCs presented clinicopathologic features resembling AGASAs with neuroendocrine characteristics, and 2 of 6 neoplasms were considered as carcinomas with neuroendocrine differentiation and were positive for 3 neuroendocrine markers. CCCs can often present with HM, and long-term outcome is likely poor. Our study concludes that CCC seems to be a rare tumor, but it might be underestimated because of the overlapping features with AGASA. Further studies should aim to define the true incidence of this disease.

Os clitoris in dogs: 17 cases (2009-2017).

Medical records of 17 client-owned dogs diagnosed with os clitoris on physical examination or diagnostic imaging were reviewed to describe the clinical signs and surgical management of this condition. All dogs were phenotypically female. The most common presenting complaints included an enlarged clitoris (n = 10), urinary tract infection (n = 5), and licking of the vulva (n = 3). Other frequently reported clinical signs included vaginal discharge and/or lower urinary tract signs such as pollakiuria. Ten dogs were surgically managed with os clitorectomy. Concurrent related procedures included gonadohysterectomy (n = 7), and episioplasty (n = 3). Clitorectomy in the surgically managed dogs created a more normal female anatomy and resolved clinical signs associated with the exposed clitoris.

Os clitoridien chez les chiennes : 17 cas (2009­2017). Les dossiers médicaux de 17 chiennes appartenant à des clients qui avaient été diagnostiquées avec l'os clitoridien lors de l'examen physique ont été étudiés afin de décrire les signes cliniques et la gestion chirurgicale de cette affection. Tous les chiens étaient phénotypiquement femelles. Les plaintes les plus communes à la présentation incluaient un clitoris élargi (n = 10), une infection des voies urinaires (n = 5) et le léchage de la vulve (n = 3). Les autres signes cliniques fréquemment signalés incluaient l'écoulement vaginal et/ou des symptômes des voies urinaires inférieures comme la pollakiurie. Dix chiennes ont été gérées chirurgicalement par une clitoridectomie. Les interventions concomitantes incluent la gonado-hystérectomie (n = 7) et l'épisioplastie (n = 3). La clitoridectomie chez les chiennes gérées par chirurgie a créé une anatomie femelle plus normale et a donné lieu à une résolution des signes cliniques associés au clitoris exposé.(Traduit par Isabelle Vallières).

Clitoral and labial sizes in women with PCOS.

The aim of this study was to examine the external genital features in Turkish women with polycystic ovary syndrome (PCOS). Thirty-two newly diagnosed PCOS cases and 35 healthy women were included the study. All women underwent a thorough gynaecological examination. Clitoral length, and labia minora length and width were recorded. The groups were compared for features of external genital structures. Clitoral and labial lengths were significantly higher in PCOS group. There was a strong correlation between clitoral length and modified Ferriman-Gallwey score. The most effective predictor of PCOS was found to be clitoral length. Clitoral length alone predicted 99.9% of PCOS patients. There were some subclinical genital changes in women with PCOS. These changes in PCOS patients may be a sign of hyperandrogenism and might have diagnostic value in indistinct cases.

Etiology of primary ovarian insufficiency in a series young girls presenting at a pediatric endocrinology center.

UNLABELLED: The cause of the primary ovarian insufficiency (POI) remains unknown in the majority of cases. A retrospective study was carried out in 17 girls with POI and normal 46,XX karyotype evaluated before 20 years of age. The etiology of POI was determined in eight girls (group 1) and remained idiopathic in nine girls (group 2). In group 1, five patients had a medical history: cerebellar ataxia due to congenital disorder of glycosylation (CDG) 1 in three cases, mitochondrial disease in one case, and autoimmune deficiencies in one case. The diagnosis of POI was made on pubertal delay or primary amenorrhea in these five patients, whilst the others presented with clitoral hypertrophy at birth or short stature and pubertal delay in two cases with NR5A1 mutation or with short stature and learning difficulties in one case with mitochondrial disease. In group 2, associated diseases were arthrogryposis malformative, gut, and bladder malformations and kidney failure or parieto-occipital tumor. The genes tested (NR5A1, BMP15, GDF9, and NOBOX) showed no mutation. CONCLUSIONS: The frequency of defined etiologies (47%) is high. This is probably because of the recruitment of the cases at the pediatric center, where other somatic anomalies can lead to the accurate determination of the etiology.

Periclitoral endometriosis: the dilemma of a chronic disease invading a rare location.

Endometriosis affects 6% to 10% of women of reproductive age; extrapelvic endometriosis is considered a rare event with perineal endometriosis being even rarer still (only a few cases of spontaneous episodes described, the majority being from episiotomy scars). We present a unique case of periclitoral endometriosis, which to the best of our knowledge is the first in the literature. It is a 29-year-old nulligravida female with a painful fluctuant right periclitoral mass that had been growing with no response to antibiotic therapy. At the initial removal, pathology reported the lesion as endometriosis. The patient was placed on oral contraceptives, and she was noted to have monthly swelling and shrinking of the site with her menstrual cycles. When she went off hormonal contraception, she represented with the growing lesion 3.5 weeks after her last menses; she underwent re-excision. Because of the extension of the lesion medially and its adherence to the clitoral body, the decision was made to evacuate only as much of the capsule that could be safely identified to minimize the risk of damaging the clitoris. Complete excision in this case was difficult without sacrificing a portion of the clitoris and potentially resulting in decreased sexual function and persistent clitoral pain. In a patient in whom complete excision is not possible, there is potential for mass recurrence in the setting of residual tissue. Reviewing the literature suggests that there are risks with both recurrence and clitoral excision. We found that in-depth patient counseling, hormonal suppression, and close follow-up are necessary when dealing with periclitoral endometriosis postexcision.

Hair-thread tourniquet syndrome of the hypertrophic clitoris in a 6-year-old girl.

Hair-thread tourniquet syndrome is defined as the ischemic strangulation of hair resulting in edema and severe pain and may cause amputation of organs. Strangulation of the external genitalia (clitoris, labia minora) has rarely been described in girls. Here, we present a case of hypertrophic clitoris injury secondary to hair strangulation in a 6-year-old girl.

Lesions of the clitoris: a review.

The clitoris may become involved by vulvar lesions. There are also lesions arising from the clitoris. A familiarity with these lesions is necessary for the high index of suspicion needed for their diagnosis.

Partially Plexiform Neurofibroma of the Labia Minora and Clitoral Hood-A Prognostic Dilemma.

BACKGROUND: Unlike solitary neurofibromas, neurofibromatosis type 1 (NF1 or von Recklinghausen disease) has been associated with an increased risk of malignancies. Differentiation between these 2 entities may not be straightforward. CASE REPORT: We present the exceptional case of a girl with one congenital plexiform neurofibroma of the labia minora and clitoral hood in whom the diagnosis NF1 could not been made. Although this presented a prognostic dilemma, we advised against a screening program for any of the malignancies associated with NF1. Still, long-term follow-up seems indicated for recurrence of the partially plexiform neurofibroma and a possible occurrence of malignant peripheral nerve sheath tumor.

Physical examination of the female internal and external genitalia with and without pelvic organ prolapse: A review.

Pelvic organ prolapse, a herniation of pelvic organs through the vagina, is a common condition in older women. Pelvic organ prolapse distorts vaginal anatomy making pelvic examination difficult. A clinician must accurately identify anatomic landmarks both in women presenting with symptoms of prolapse and in women noted to have coincidental prolapse during routine gynecologic examination. We present a systematic approach to the female pelvic examination including anatomic landmarks of the external genitalia, vagina, and uterus in women with normal support as well as changes that occur with pelvic organ prolapse. Knowledge and awareness of normal anatomic landmarks will improve a clinician's ability to identify defects in pelvic support and allow for better diagnosis and treatment of pelvic organ prolapse.

Clitoral size and location in relation to sexual function using pelvic MRI.

INTRODUCTION: The female sexual response is dynamic; anatomic mechanisms may ease or enhance the intensity of orgasm. AIM: The aim of this study is to evaluate the clitoral size and location with regard to female sexual function. METHODS: This cross-sectional TriHealth Institutional Board Review approved study compared 10 sexually active women with anorgasmia to 20 orgasmic women matched by age and body mass index (BMI). Data included demographics, sexual history, serum hormone levels, Prolapse/Incontinence Sexual Questionnaire-12 (PISQ-12), Female Sexual Function Index (FSFI), Body Exposure during Sexual Activity Questionnaire (BESAQ), and Short Form Health Survey-12. All subjects underwent pelvic magnetic resonance imaging (MRI) without contrast; measurements of the clitoris were calculated. MAIN OUTCOME MEASURES: Our primary outcomes were clitoral size and location as measured by noncontrast MRI imaging in sagittal, coronal, and axial planes. RESULTS: Thirty premenopausal women completed the study. The mean age was 32 years (standard deviation [SD] 7), mean BMI 25 (SD 4). The majority was white (90%) and married (61%). Total PISQ-12 (P < 0.001) and total FSFI (P < 0.001) were higher for orgasmic subjects, indicating better sexual function. On MRI, the area of the clitoral glans in coronal view was significantly smaller for the anorgasmic group (P = 0.005). A larger distance from the clitoral glans (51 vs. 45 mm, P = 0.049) and body (29 vs. 21 mm, P = 0.008) to the vaginal lumen was found in the anorgasmic subjects. For the entire sample, larger distance between the clitoris and the vagina correlated with poorer scores on the PISQ-12 (r = -0.44, P = 0.02), FSFI (r = -0.43, P = 0.02), and BESAQ (r = -0.37, P = 0.04). CONCLUSION: Women with anorgasmia possessed a smaller clitoral glans and clitoral components farther from the vaginal lumen than women with normal orgasmic function.

Treatment of clitoral hypertrophy containing an os clitoris and urethra in a young greyhound dog: a detailed surgical description.

This report provides a detailed description and images of a clitorectomy with a urethral transposition. As described, the clitorectomy is a straight-forward procedure, creating more normal female-like anatomy, and it resolves the clinical signs resulting from the exposed clitoris.

Traitement d'une hypertrophie du clitoris contenant un orifice de clitoris et l'urètre chez un jeune chienne Greyhound: description chirurgicale détaillée. Ce rapport fournit une description détaillée et des images d'une clitorectomie avec une transposition urétérale. Tel que décrite, la clitorectomie est une intervention simple, qui crée une anatomie femelle plus normale et règle les signes cliniques découlant d'un clitoris exposé.(Traduit par Isabelle Vallières).

[Clitoral epidermoid cyst causing clitoromegaly. A rare case report]. / Clitoridomégalie dans le cadre un kyste épidermoïde géant: à propos d'un cas.

Clitoromegaly is uncommon. It is mostly congenital, hormonal or tumoral. Epidermoid cyst is rare. It can be the consequence of trauma, but in some situations the cyst can be non-traumatic. We report the case of a 53-year-old woman who presented a misdiagnosis of clitoromegaly due to hormonal condition. Surgical exploration has highlighted an epidermoid cyst. This observation underlines the importance to evoke a cystic origin for clitoral hypertrophy and encourages us to propose imaging (ultrasound, MRI) in case of etiological doubt. The preoperative diagnosis must be made to preserve vascularization and innervation of the clitoris.

Peri-clitoral Epidermal Inclusion Cyst as Initial Presentation of Lichen Sclerosus in a Pediatric Patient.

BACKGROUND: Lichen sclerosus (LS) is a chronic inflammatory disorder, presenting with pruritis and hypopigmentation of the vulvar and anogenital skin. LS presenting as a peri-clitoral mass has not been previously described. CASE: A 5-year-old patient with vulvar pruritis and ultrasound showing a homogenous mass was referred for suspected clitoromegaly with normal labs. Examination demonstrated a prepubertal patient with a mobile, soft, peri-clitoral mass and surrounding hypopigmentation consistent with LS. The cyst was excised surgically; pathology revealed an epidermal inclusion cyst. Postoperatively, she began using topical steroids for LS with symptom resolution. CONCLUSION: Thorough workup of clitoromegaly negative for hormonal causes requires further investigation to determine an alternative etiology of the mass. We suspect that inflammatory changes of LS and pruritus resulted in the peri-clitoral inclusion cyst.

Clitoral subdermal hoodoplasty for medical indications and aesthetic motives. A new technique.

OBJECTIVE: To present a newly developed surgical intervention (clitoral subdermal hoodoplasty) for an asymmetrical clitoral prepucial thickness hypertrophy and to evaluate its applicability and outcomes. STUDY DESIGN: The study was conducted on 3 consecutive patients with symptomatic, asymmetrical-in-thickness clitoral prepucial hypertrophy. A clitoral subdermal hoodoplasty surgical technique for asymmetrical clitoral prepucial thickness hypertrophy has never been described before. An observational prospective, multiple time series clinical study was conducted. Primary outcome measures were to determine applicability, outcomes, and potential complications of the new surgical intervention. Data collected included demographics, patient selections, intraoperative and postoperative complications, and outcomes of the newly developed surgical intervention. RESULTS: All subjects demonstrated subdermal hypertrophy of the clitoral prepuce. The newly developed procedures were successfully applied. Complications were not observed. The surgical intervention resulted in resolving medical symptoms and signs and provided pleasing aesthetic outcomes. In this study the procedure was simple to execute and well-tolerated by all subjects, without short-term and long-term complications. CONCLUSION: Clitoral subdermal hoodoplasty is a useful method in the treatment of clitoral subdermal hypertrophy.

Plexiform schwannoma of the clitoris in a young girl: a case report.

BACKGROUND: Schwannoma (neurilemoma) is a benign, slow-growing tumor of the nerve sheath. These tumors are rarely found in the female genitalia and to date only 1 case of clitoral schwannoma has been reported in a young girl. We report here the second case of schwannoma of the clitoris. CASE: A 6-year-old girl presented with an enlarging clitoris. An alteration in her clitoral appearance had first been noted at 2 years of age. However, the size had further increased in the year prior to presentation. On examination her clitoris was normal in size but beneath the clitoral hood, predominantly on the left, there was a 3 x 2 cm irregular mobile mass. Her karyotype revealed normal 46XX female genotype. Magnetic resonance imaging of the abdomen and pelvis showed an isolated finding of diffuse enlargement of the clitoris with edema of the mons pubis. Surgical excision of the paraclitoral mass was performed. Intraoperatively the clitoral tip and shaft did not appear to be involved. However, the mass was found to be more diffuse, less well-defined, and more extensive than the clinical findings had suggested. On histology long spindle cells with nuclear palisading and focal Verocay body-like structures were found. There was mild to moderate pleomorphism. No mitotic figures were identified. There was diffuse staining of interweaving bundles for S100 protein and glial fibrillary acidic protein without staining for actin, desmin, or neurofilament. These features are consistent with a plexiform schwannoma. CONCLUSION: Although benign schwannomas rarely occur in the clitoris, we suggest that it should be considered as differential diagnosis for any young girl with clitoral or paraclitoral asymmetrical irregular mass.

Giant epidermoid inclusion cyst of the clitoris mimicking clitoromegaly.

We describe a rare case of clitoromegaly due to a large clitoral cyst that occurred spontaneously without any declared previous female genital mutilation. The cyst was excised successfully with good cosmetic results.