Surgical treatment of vulvar venous malformations without preoperative sclerotherapy: Outcomes of 18 patients.

BACKGROUND: Our aim was to evaluate the benefit of surgical resection of the venous malformations (VMs) of the external female genitalia. METHODS: Over the period of 2009-2019, 18 consecutive females underwent surgical resection for vulvar VM. Evaluations included preoperative Doppler ultrasound, MRI, and pre-and postoperative photographic imaging. The main outcomes were: residual pain, cosmetic distortion, residual VM, and quality of life. RESULTS: Over a 10 year periods, 18 females, mean age 35 years (range 9-71) were included in this study. All patients were symptomatic: 16 had intermittent pain or discomfort, 1 had bleeding and 2 requested cosmetic treatment. Of these cases, there were 5 isolated vulvar VM, 12 associated VM: 3 of the clitoral hood, 3 troncular pelvic vein insufficiency and 12 of the lower limb. Eight patients had undergone previous procedures: 2 sclerotherapy treatments (1-3 sessions), 4 partial surgical resections. There were 18 single resections in the vulva (7 focal, 11 complete), 2 partial resections in clitoral hood and 2 had resection of a VM in the perirtoneovaginal canal at the same time. The mean follow-up was 42. 9 months (range 6-120). Two patients were lost to followup at 6 months. For all patients, elimination of pain and soft tissue redundancy was achieved. Two patients had persistent discomfort and 2 requested cosmetic treatment. CONCLUSION: Surgical resection of vulvar VM can be the best approach with few postoperative complications, good functional and cosmetic results. Appropriate preoperative evaluation is required to identify isolated VM or VM associated with ovarian vein or internal iliac vein insufficiency requiring to be treated before surgery.

Safety and effectiveness of percutaneous sclerotherapy for venous disorders of the labia majora in patients with vascular malformations.

OBJECTIVE: The aim of this study was to evaluate the safety and clinical outcomes of percutaneous sclerotherapy of venous disorders of the labia majora in patients with vascular malformations of the lower limbs. METHODS: Thirty percutaneous sclerotherapy treatments were performed over a 6-year period among 17 female patients with symptomatic venous malformation (VM) or secondary varicosis of the labia majora. Four patients were treated with sclerotherapy alone, 13 patients had additional procedures to control the VM before sclerotherapy. Polidocanol was used as sclerosant. Indications for sclerotherapy included pain, bleeding, thrombophlebitis, and swelling. Genitourinary symptoms were recorded. The number of treatments and procedure-related complications were registered. Complications were classified according to the Society of Interventional Radiology (SIR) classification system (grade A-E). The 3-month postintervention follow-up included magnetic resonance imaging, clinical examination, and a symptom-related questionnaire. If no reintervention was necessary, consultation was scheduled biannually. RESULTS: All patients had local swelling and pain; only a fraction of the patients had further symptoms with bleeding or thrombophlebitis (47% each). Eight patients required reintervention. No major complications were observed; minor complications such as postprocedural swelling occurred in 29% (SIR grade A), pain occurred in 17% (SIR grade B), and skin blistering developed in 5% (SIR grade B). Upon follow-up examination after a median of 40 months, 76% showed complete relief of symptoms, and 23% reported partial relief. All patients reported a substantial reduction in pain (75% >5 points in visual analogue scale) and swelling (88% complete cessation). CONCLUSIONS: Percutaneous sclerotherapy is a safe and effective treatment option of VM and secondary varicosis of the labia majora.

Reduction of Hypertrophic Labia Minora by Posterior-Lateral Wedge Resection with Preservation of the Central Blood Vessels and Nerve Bundle.

OBJECTIVE: The aim of this study was to introduce a new method of labiaplasty. Here, we describe the surgical procedure, outcomes and the advantages of this method. METHOD: The medical records of 21 patients aged between 20 and 45 years who underwent reduction of the labia minora from February 2015 to June 2017 were reviewed. The procedures performed in these studies used posterior-lateral wedge resection with preservation of the central blood vessels and nerve bundle. RESULTS: All the surgeries were performed successfully, and 21 patients experienced an uneventful postoperative period. A minor dehiscence occurred in one patient, who recovered with no requirement for additional treatment. All of the patients were satisfied with the eventual esthetic appearance. CONCLUSION: The posterior-lateral wedge resection with preservation of the central blood vessels and nerve bundle is a simple and safe method that is associated with satisfactory outcomes. LEVEL OF EVIDENCE IV: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .

Internal iliac artery balloon occlusion as a hemostatic method for spontaneous rupture of vulvar hematoma during delivery: A case report.

Spontaneous rupture of a vulvar hematoma during delivery is a relatively uncommon event and may cause excessive hemorrhage. Exact identification of anatomic structures and bleeding points is challenging. We herein present a case involving a pregnant woman at 39 weeks' gestation with a large vulvar hematoma that spontaneously ruptured during the second stage of labor, likely due to rupture of varices in the vulva or vagina. It was difficult to accurately expose and suture the deep bleeding points. The estimated blood loss volume was 1591 mL, and the hemoglobin concentration dropped from 132 g/L before delivery to 84 g/L after delivery. To prevent hemorrhagic shock, bilateral internal iliac artery balloon occlusion was performed and proved to be an effective way to achieve hemostasis. Once hemostasis was established, ligation of the bleeding sites and suturing of all dead space were rapidly completed. Bilateral internal iliac artery balloon occlusion can be used as an effective treatment for excessive vaginal bleeding. The presence of varices or hemangiomas in the vulva or vagina should be carefully checked during antenatal care.

Vulvar Varicosities: A Review.

BACKGROUND: Vulvar varicosities (VV) are dilated and tortuous veins occurring within the external female genitalia. Patients may seek treatment of these varices for both medical and cosmetic purposes. In some patients, VV may be associated with a chronic pelvic pain syndrome called pelvic congestion syndrome (PCS). OBJECTIVE: To review the English language literature on VV in both pregnant and nonpregnant women. MATERIALS AND METHODS: A literature search pertaining to vulvar varicosities and PCS was performed using PubMed and Google Scholar databases. RESULTS: There is an overall paucity of literature discussing VV, particularly in nonpregnant women without PCS. Management options for VV include compression, sclerotherapy, embolization, and surgical ligation. Treatment can be dependent on the coexistence of pelvic or leg varicosities and may require referral to a vein specialist for advanced imaging techniques and procedures. Direct sclerotherapy to VV may not provide adequate treatment if pelvic or leg varices are also present. CONCLUSION: In women with persistent VV, imaging studies should be obtained before treatment to evaluate the surrounding venous anatomy of the pelvis and leg, as the results often affect the treatment approach. Patients presenting with VV and chronic pelvic pain should be evaluated for PCS.

A Vulvovaginal Mass in a 16-Year-Old Adolescent: A Case Report.

BACKGROUND: Vulvar varicosities might be misdiagnosed as vulvar venous malformations in the prepubertal or pubertal age. If seen isolated, they can mimic other masses that are usually seen in this anatomic area like a hernia or a cyst. Depending on the associated symptoms and clinical findings, imaging modalities investigate the nature of such a mass and might provide a therapeutic alternative besides surgery. CASE: We report a case of an isolated asymptomatic vulvar varicosity in a 16-year-old adolescent. A pelvic ultrasound followed by a Doppler and a magnetic resonance imaging scan confirmed the diagnosis. We used guided direct sclerotherapy for successful treatment. SUMMARY AND CONCLUSION: Optimal management and outcome are assured using a multidisciplinary approach. Guided sclerotherapy provides a safe, effective, and minimally invasive procedure to treat vulvar varicosities.

Feasibility and Safety of Video Endoscopic Inguinal Lymphadenectomy in Vulvar Cancer: A Systematic Review.

OBJECTIVE: To systematically review previous studies and to evaluate the feasibility and safety of video endoscopic inguinal lymphadenectomy (VEIL) in vulvar cancer. METHODS: We conducted a comprehensive review of studies published through September 2014 to retrieve all relevant articles. The PubMed, EMBASE, Web of Science, Cochrane Library, Wan Fang Data and Chinese National Knowledge Infrastructure databases were systematically searched for all relevant studies published in English or Chinese through September 2014. Data were abstracted independently by two reviewers, and any differences were resolved by consensus. RESULTS: A total of 9 studies containing 249 VEIL procedures involving 138 patients were reviewed. Of the 249 VEIL procedures, only 1 (0.4%) was converted to an open procedure for suturing because of injury to the femoral vein. The range of operative time was 62 to 110 minutes, and the range of estimated blood loss was 5.5 to 22 ml. The range of the number of harvested lymph nodes was 7.3 to 16. The length of hospital stay varied from 7 to 13.6 days across reports. The incidence of lymph node metastasis was 19.7% (27/138), and the recurrence rate was 4.3% (3/70) within 3 to 41 months of follow-up. One or more short-term complications were documented in 18 of 138 (13.0%) patients. Complications after VEIL were observed in 14 (10.13%) patients and in 15 (6.0%) of the VEIL cases, including major lymphocyst formation in 9 (3.6%), lymphorrhea in 2 (0.8%), inguinal wound infection without wound breakdown in 3 (1.2%) and lymphedema in 1 (0.4%). CONCLUSIONS: VEIL appears to be a feasible procedure in the management of vulvar cancer. There may be potential benefits that result in lower morbidity compared to traditional methods, but this has yet to be objectively proven.

A Cadaveric Study of the Arterial Blood Supply of the Labia Minora.

BACKGROUND: Labiaplasty has become a rather common operation. The main complication of the different surgical techniques is the dehiscence of the suture line. The dehiscence rate varies among the different techniques, and this may imply that the vascular anatomy is not respected in some cases. The detailed arterial anatomy of labia minora is not well described, so the aim of the authors was to describe this anatomy with a cadaveric study. METHODS: Eleven fresh cadavers were dissected, and arterial study was made with injected computed tomography scans and rotational angiography. At the end, a cast of the arterial network was made and chemically exposed to verify the radiological findings. RESULTS: The findings of this study allowed identification of a dominant central artery that was named "C" artery, two posterior arteries named "P1" and "P2," and one small anterior artery, "A." Furthermore, a connection between the anterior system of the external pudendal artery and the posterior system of the internal pudendal artery was confirmed. CONCLUSIONS: The arterial network of the labia minora was identified with this study. This may help surgeons orientate the wedge excision when they perform labiaplasties. More precisely, when this wedge is placed at the most anterior part of the labia minora, the least perfused area is removed, and a posterior flap is created that will preserve a robust blood perfusion.

Pilot study of the suitability of dorsal vulval skin as a transposition flap: vascular anatomic study and clinical application.

OBJECTIVE: To identify the blood supply to the vulval fold and adjacent skin, and evaluate it as a transposition flap for closing perineal wounds in dogs. STUDY DESIGN: Prospective study. ANIMALS OR SAMPLE POPULATION: Five female canine cadavers and 2 cases referred for excision of mast cell tumors adjacent to the vulva. METHODS: Dissection was performed to identify the vascular supply to the vulval fold in two cadavers following arterial injection of red latex and methylene blue, respectively. In three cadavers, barium sulfate mixed 1:1 with water was injected into the terminal aorta. The vulval fold and surrounding perineal skin was excised and radiographed. Transposition flaps using the vulval fold and adjacent skin were used to close skin defects in two dogs presented for wide excision of mast cell tumors situated ventro-lateral and dorso-lateral to the vulva, respectively. RESULTS: The vulval fold and adjacent skin was perfused bilaterally by branches of the ventral perineal and external pudendal arteries, which entered dorsally and ventrally, respectively. As incisions used to create a transposition flaps from the skin surrounding the vulval fold transect these vessels, the flap is dependent on the sub-dermal plexus for survival. There was 100% survival of transposition flaps in the 2 clinical cases and healing proceeded uneventfully with acceptable cosmetic and functional results. CONCLUSIONS: The vulval fold and surrounding skin can be used as a subdermal plexus flap to close large perineal defects in dogs. CLINICAL RELEVANCE: Availability of a defined local skin flap will improve treatment of diseases resulting in large perineal skin defects in female dogs.

An unusual site for calciphylaxis: a case report.

BACKGROUND: Calciphylaxis is a rare condition characterized by calcification of the tunica media of small arteries with or without endovascular fibrosis, extravascular calcification and vascular thrombosis, leading to tissue ischemia and hence necrosis of tissues supplied by respective vessel. CASE REPORT: An 83-year-old lady presented with a 2-week history of rapidly progressing painful necrotic vulval lesion. This patient was being treated medically with bisphosphonates for mild hypercalcaemia secondary to a parathyroid adenoma. The diagnosis of calciphylaxis was made by biopsy of lesion, revealing extensive necrotic areas and multiple abscesses with numerous thrombosed and calcified blood vessels. CONCLUSIONS: This case shows an unusual presentation of calciphylaxis, in a patient with primary hyperparathyroidism, in the absence of end stage renal failure. The pathogenesis of the condition is still relatively unknown. Particularly of note in this case is the presentation of the lesion 9 months after the start of treatment with bisphosphonate after the relative decrease of serum parathyroid levels and serum calcium levels. This leads to the question of initiation of the pathology - did the bisphosphonate treatment have an input in initiation of the lesion? The case under review adds a new differential diagnosis to necrotic vulval lesions, other than malignancy.

Management of a Klippel-Trenaunay syndrome in pregnant women with mega-cisterna magna and splenic and vulvar varices at birth: a case report.

Klippel-Trenaunay syndrome (KTS) is a rare, sporadic and complex malformation characterized by the clinical triad of: (i) capillary malformation (port-wine stain); (ii) soft tissue and bone hypertrophy or occasionally, hypertrophy of one lower limb; and (iii) atypical lateral varicosity. The maternal and fetal risks associated with pregnancy in women with KTS are proportional to disease severity, which can be exacerbated by pregnancy. Complications include bleeding, disseminated intravascular coagulation, thromboembolic events and pain. Here, we report the case of a pregnant woman with KTS who had an uneventful pregnancy, labor and postpartum course, but had splenic and large vulvar vein varices. The obstetrical course of women with KTS varies. Management is largely conservative and multidisciplinary approaches form the mainstay for managing these patients based on their symptoms.

Untangling a web: an unusual case of labial necrosis in an adolescent female.

BACKGROUND: Hair tourniquets are commonly described in the pediatric literature. Prompt recognition of a hair tourniquet and treatment with complete removal of the hair by pediatricians, pediatric emergency room physicians, or gynecologists is essential to prevent ischemia and necrosis of affected tissue. CASE: Herein we present the case of a 12-year-old female referred to the pediatric surgery clinic for labial pain and swelling. She was found to have a hair tourniquet of the labia minora caused by pubic hair. The patient was taken to the operating room for examination under anesthesia and removal of the hair. Her post-operative course was unremarkable and she was discharged home the following day. On follow-up visit to the clinic her labial edema had completely resolved and she was pain free. SUMMARY AND CONCLUSIONS: Unlike previous case reports that describe hair tourniquets as originating from hair on the head, our patient had a hair tourniquet caused by pubic hair. In adolescents where personal hygiene of the perineum is difficult, clinicians need to be aware of the possibility of a hair tourniquet forming from pubic hair.

Cigarette smoking decreases the genital vascularization in young healthy, eumenorrheic women.

INTRODUCTION: Cigarette smoking is a major health hazard and may impair the normal sexual response. AIM: To evaluate (in the early follicular phase, and independently from sexual stimulation) in young, eumenorrheic, healthy, lean women the general and genital vascular effects of the smoking habit. METHODS: One hundred thirty-seven patients undertook, in the early follicular phase of the menstrual cycle (day 3-5): the administration of the two-factor Italian McCoy Female Sexuality Questionnaire (MFSQ); two-dimensional (2-D) color Doppler evaluation of the ophthalmic, carotid, uterine, clitoral, and labia minora arteries; three-dimensional (3-D) analysis of clitoral and labia minora vascularization; and blood pressure evaluation. Fasting blood samples were drawn to test plasma estradiol, androstenedione, and testosterone circulating levels. MAIN OUTCOME MEASURES: The two-factor Italian MFSQ; the pulsatility index (PI) of internal carotid, ophthalmic, uterine, clitoral, and labia minora arteries; the vascularization index (VI), the flow index (FI), and the vascularization flow index (VFI) of clitoral body and labia minora, blood pressure measurement, and hormonal assay. RESULTS: The subjects were divided in: nonsmokers (Group I; N=72); current (>2 years) light smokers-1 to 10 cigarettes/day-(Group II; N=35); and current heavy smokers->10 cigarettes/day-(Group III; N=30). The 2-D Doppler analysis of the ophthalmic and internal carotid arteries showed significant increased resistance in Group III compared with Group II and Group I. The genital vessels (uterine, clitoral, and labium minus arteries) showed the lowest PIs in the nonsmokers. The 3-D power Doppler histogram analysis of clitoral and labium minus blood flow showed the worst vascular indices (VI, FI, VFI) in the heavy smokers. CONCLUSIONS: Cigarette smoking may favor a reduced central and peripheral tissue perfusion with consequent increased risk of cerebrovascular and cardiovascular diseases and with genital vessels stiffness and impaired sexual performances.

Angiokeratoma of the clitoris with evident vulvar varicosity.

Angiokeratomas of the vulva are uncommon, benign vascular lesions that are generally located on the labia. However, the clitoris is an extremely rare location, with only three published cases. We report a case of clitoral angiokeratoma in a 22-year-old nulligravida with a history of surgery to remove a clitoral mass at 6 years of age. The case described herein is distinguished from the other case reports by an accompanying varicose structure involving the vulva.

The anterior Obturator Artery Perforator (aOAP) flap: surgical anatomy and application of a method for vulvar reconstruction.

OBJECTIVE: Vulvar reconstruction following oncologic resection is challenging. Some flaps used for reconstruction can show adverse characteristics such as excessive tissue bulk or increased distance to the defect. Region of the sulcus genitofemoralis is of thin and pliable tissue proximate to the vulva. Vasculature and suitability of that region used for vulvar reconstruction were focused in this work. METHODS: Vascular architecture of the region comprising the sulcus genitofemoralis was examined bilaterally on 10 female corpses (n = 20 specimens). In addition, tissue characteristics and suitability of that region to form a fasciocutaneous flap for vulvar reconstruction were anatomically examined and clinically proven. RESULTS: Vasculature of the sulcus genitofemoralis is reflected by either a musculocutaneous perforator (80%, 16/20) piercing the gracilis muscle or a septocutaneous perforator (20%, 4/20) passing the posterior border of the gracilis muscle 1.3 ± 0.3 [cm] laterally to the inferior pubic ramus. Both types of perforators derive from the anterior branch of the obturator artery and accompanying vein. This perforator, the anterior obturator artery perforator (aOAP), supplies a skin territory of about 7 × 15 [cm] centered on the sulcus genitofemoralis. The aOAP flap proved its suitability and versatility for vulvar reconstruction. CONCLUSIONS: The sulcus genitofemoralis is of a constant vascular anatomy reflected by the aOAP vessel. Especially the tunneled aOAP island flap offers outstanding characteristics beneficial for reconstruction of a more anatomically normal vulva. Scars are limited within anatomic borders of the urogenital region improving self-image. The aOAP flap clearly enlarges the surgical options available to restore the form and function of the vulva.

Resection of a vulvar arteriovenous malformation in a premenarchal patient.

BACKGROUND: Arteriovenous malformation of the vulva is described in only a few case reports in the literature. Given the complex anatomy of the vulva, arteriovenous malformations in this location present a particularly challenging treatment dilemma. CASE: An 11-year-old premenarchal girl with a large vulvar arteriovenous malformation was monitored for several years. After three episodes of bleeding, despite conservative management with embolization, she was taken urgently to the operating room for resection of the lesion. A multidisciplinary team participated in the 12-hour procedure. Several months after the initial surgery, the patient is without complaints and adjusting well. Labial reduction will be required once she is fully grown. CONCLUSION: Arteriovenous malformations of the vulva require treatment before the onset of menarche because of the potential for massive hemorrhage. If first-line conservative treatment with embolization fails, a multidisciplinary team should be assembled for surgical treatment.