Diagnostic radiology findings and spectrum of therapeutic interventions in gynaecological and urogenital vascular anomalies.

Vascular anomalies represent a rare congenital disease with manifestation at diverse anatomical sights and presenting with heterogenous symptoms. Undetected, they can progress and create acute and chronic complications with functional impairment. The manifestation in the female and male pelvis and the urogenital tract represents a multidisciplinary challenge for physicians. Especially outpatient management in gynaecology and urology is affected. Diagnostic Radiology holds an important supportive role in early diagnosis of the underlying urogenital vascular anomaly and referral to interventional radiology, either for minimal invasive treatment, or to surgery for further assessment. This pictorial review creates awareness for the spectrum of vascular anomalies of the gynaecological and urogenital tract, their characteristic imaging findings and dedicated interventional treatment options. The individual description of vascular anomalies, based on an appropriate nomenclature and classification standard, is a guide for radiologists to distinguish the underlying vascular anomaly from other vascular disorders and to accelerate diagnosis as well as therapeutic proceedings. In consequence, interdisciplinary management of patients with vascular anomalies of the female and male pelvis will benefit.

Genital Hair-Thread Tourniquet Syndrome.

BACKGROUND: Hair-thread tourniquet syndrome is a rare disorder that occurs when a hair or other fiber becomes wrapped around an appendage, resulting in swelling, pain, or even loss of the appendage. Some cases affecting the female genitals have been reported. CASE: The case of a 10-year-old girl with a 3-day history of genital pain is presented. During examination, a hair tourniquet was found at the base of a swollen and painful clitoris. The hair was removed under deep sedation, producing immediate relief. SUMMARY AND CONCLUSION: The most important concern in genital hair-thread tourniquet syndrome is a high index of suspicion and prompt resolution in order to save the affected tissue. It should be considered on the differential diagnosis for all girls with vulvar swelling and indication of pain.

Alpelisib Treatment for Genital Vascular Malformation in a Patient with Congenital Lipomatous Overgrowth, Vascular Malformations, Epidermal Nevi, and Spinal/Skeletal Anomalies and/or Scoliosis (CLOVES) Syndrome.

BACKGROUND: Most patients with phosphoinositide-3-kinase, catalytic, alpha polypeptide (PIK3CA)-related overgrowth spectrum become symptomatic early in life and need treatment before puberty. Recently, the specific inhibition of PIK3CA pathways has been proposed as a therapeutic option for these patients improving their surgical options and quality of life. Alpelisib, a specific alpha fraction inhibitor, has shown promising results. CASE: A 17-year-old girl presented with severe involvement of her external genitalia with a combined vascular malformation in the context of congenital, lipomatous, overgrowth, vascular malformations, epidermal nevi and spinal/skeletal anomalies and/or scoliosis syndrome, needing frequent blood transfusions for anemia due to vaginal bleeding and use of a crutch for walking. After failure of treatment with rapamycin, compassionate treatment with alpelisib was started with excellent response. SUMMARY AND CONCLUSION: PIK3CA inhibitors might become a new option of treatment for PIK3CA-related overgrowth spectrum patients.

Impact of Immune System Activation and Vascular Impairment on Male and Female Sexual Dysfunction.

INTRODUCTION: Male and female sexual dysfunction (SD) is considered a multifactorial condition. Numerous studies have shown the involvement of inflammatory processes in this pathological condition. Sexual intercourse requires healthy and functioning vessels to supply the pelvic region in both males and females, generating penile erection and clitoral and vaginal lubrication, respectively. Cardiovascular diseases and associated risk factors may contribute negatively to pelvic blood flow, possibly through immune system activation. AIM: The study aimed to address the correlation between vascular inflammation driven by immune system activation and SD in males and females. METHODS: A literature review was performed to identify articles addressing male and female SD and vascular inflammation. Key words included "male and female sexual dysfunction," "vascular inflammation," "iliac and pudendal arteries dysfunction," "genitourinary tract," and "blood flow." MAIN OUTCOME MEASURES: Management of systemic and local inflammation may be a useful alternative to improve SD and reduce the risk of cardiovascular diseases in the future. RESULTS: Increased levels of cytokines and chemokines have been detected in humans and animals with hypertension, obesity, and diabetic conditions. Chronic activation of the innate immune system, especially by pathogen- or damage-associated molecular patterns, and metabolic-related disorders may act as triggers further contributing to an increased inflammatory condition. Due to the reduced size of vessels, SD and retinal vascular impairments have been shown to be predictive factors for cardiovascular diseases. Therefore, considering that blood flow to the genitalia is essential for sexual function, endothelial dysfunction and vascular remodeling, secondary to chronic immune system activation, may be implicated in male and female vasculogenic SD. CONCLUSIONS: Several conditions appear to play a role in SD. In the present review, we have identified a role for the immune system in generating vascular and tissue impairments contributing to erectile dysfunction and female SD. Calmasini FB, Klee N, Webb RC, et al. Impact of Immune System Activation and Vascular Impairment on Male and Female Sexual Dysfunction. Sex Med Rev 2019;7:604-613.

Imaging Appearance and Nonsurgical Management of Pelvic Venous Congestion Syndrome.

Pelvic venous congestion syndrome (PVCS) is a challenging and complex cause of chronic pelvic pain in female patients. PVCS due to incompetent vein valves is the combination of gonadal vein reflux and pelvic venous engorgement in patients with chronic pelvic pain without other causes. However, pelvic venous engorgement and gonadal vein reflux can be seen in patients without pelvic pain, which makes obtaining a detailed history and physical examination important for workup and diagnosis. The underlying cause of PVCS may be incompetent gonadal vein valves or structural causes such as left renal vein compression with an incompetent gonadal vein valve (nutcracker syndrome) or iliac vein compression (May-Thurner configuration) with reflux into the ipsilateral internal iliac vein. Venography is considered the criterion standard for imaging diagnosis; however, more recently, US and MRI have been shown to provide adequate accuracy for diagnosis. Noninvasive imaging studies aid in the diagnosis of PVCS and also aid in pretreatment planning. When PVCS is caused by incompetent gonadal vein valves, treatment typically is performed by means of embolization via a minimally invasive catheter with excellent technical and clinical success rates. When PVCS is caused by venous obstruction, the obstruction must be treated first before gonadal vein embolization and sclerotherapy are considered. ©RSNA, 2019 Online supplemental material is available for this article.

[Possibilities of local phlebectomy in correction of pelvioperineal venous reflux]. / Vozmozhnosti lokal'noi flebéktomii v korrektsii pel'vio-perineal'nogo venoznogo refliuksa.

The work was based on the results of examination and treatment of 43 female patients presenting with varicose transformation of pelvic, perineal, and lower-limb veins. The inclusion criteria were as follows: the presence of visually determined varicose transformation of the veins of the external genital organs, perineum, posterior surface of the thighs, as well as valvular insufficiency of the mentioned veins by the findings of ultrasonographic angioscanning (USAS). The following exclusion criteria were applied: the presence of pregnancy, symptoms of pelvic venous plethora (PVP), and varicothrombophlebitis. In 33 women correction of the pelvioperineal reflux (PPR) was performed with the help of local phlebectomy on the large pudendal lips and perineum, with the maximally possible mobilization of the vessel within the limits of the operative wound. Miniphlebectomy with the use of Varady phleboextractors was performed in 10 women presenting with isolated varicose transformation of the subcutaneous veins of the posterior femoral surface. The duration of follow up of patients amounted to 3 years. The criteria for efficiency of the carried out treatment were as follows: freedom from varicose syndrome both in the perineum and on the lower extremities, as well as no PVP symptoms during the whole term of follow up. Varicose syndrome of the external genital organs, perineum and posterior surface of the femurs was successfully eliminated in 100% of patients. Meticulous mobilization and removal of the veins of the labia majora, perineum and subcutaneous femoral veins is a reliable method of removing pathological reflux of blood from the intrapelvic to superficial veins of the perineum and lower limbs. 100% of our patients were found to be free from relapses of either vulvar or perineal varicosity, with no evidence of lower limb varicose veins. Local phlebectomy is an efficient method of elimination of varicose syndrome induced by PVP in patients with dilatation of intrapelvic, vulvar and perineal veins.

Recent Update of Embolization of Postpartum Hemorrhage.

Postpartum hemorrhage (PPH) is a life-threatening condition and remains a leading cause of maternal mortality. Transcatheter arterial embolization (TAE) is an effective therapeutic strategy for PPH with the advantages of fast speed, repeatability, and the possibility of fertility preservation. We reviewed the vascular anatomy relevant to PPH, the practical details of TAE emphasizing the timing of embolization, and various clinical conditions of PPH according to a recent literature review.

Cardiometabolic Risk and Female Sexuality-Part II. Understanding (and Overcoming) Gender Differences: The Key Role of an Adequate Methodological Approach.

INTRODUCTION: Although basic science and clinical research indicate that the vascular physiopathology of male and female sexual dysfunction (FSD) is similar, to date the association between FSD and cardiovascular (CV) diseases has been only marginally explored. AIM: To discuss the potential reasons for differences in the role of CV diseases and risk factors in sexual function in women vs men in the 2nd part of a 2-part review. METHODS: A thorough literature search of peer-reviewed publications on the topic was performed using the PubMed database. MAIN OUTCOME MEASURES: We present a review of the main factors that could account for this gap: (i) actual physiologic discrepancies and (ii) factors related to the inadequacy of the methodologic approach used to investigate CV risk in patients with FSD. A summary of the available methods to assess female sexual response, focusing on genital vascularization, is reported. RESULTS: The microanatomy and biochemistry of the male and female peripheral arousal response are similar; in contrast, there are differences in the interplay between the metabolic profile and sex steroid milieu, in the relative weighting of cardiometabolic risk factors in the pathogenesis of CV disease, and their clinical presentation and management. CV diseases in women are under-recognized, leading to less aggressive treatment strategies and poorer outcomes. Moreover, evaluation of hemodynamic events that regulate the female sexual response has thus far been plagued by methodologic problems. CONCLUSION: To clarify whether sexuality can be a mirror for CV health in women, the female genital vascular district should be objectively assessed with standardized and validated methods. Studies designed to establish normative values and longitudinal intervention trials on the effect of the treatment of CV risk factors on FSD are urgently needed. Maseroli E, Scavello I, Vignozzi L. Cardiometabolic Risk and Female Sexuality-Part II. Understanding (and Overcoming) Gender Differences: The Key Role of an Adequate Methodological Approach. Sex Med Rev 2018;6:525-534.

Postpartum hemorrhage from non-uterine arteries: clinical importance of their detection and the results of selective embolization.

Background Identification of the source of postpartum hemorrhage (PPH) is important for embolization because PPH frequently originates from non-uterine arteries. Purpose To evaluate the clinical importance of identifying the non-uterine arteries causing the PPH and the results of their selective embolization. Material and Methods This retrospective study enrolled 59 patients who underwent embolization for PPH from June 2009 to July 2016. Angiographic findings and medical records were reviewed to determine whether non-uterine arteries contributed to PPH. Arteries showing extravasation or hypertrophy accompanying uterine hypervascular staining were regarded as sources of the PPH. The results of their embolization were analyzed. Results Of 59 patients, 19 (32.2%) underwent embolization of non-uterine arteries. These arteries were ovarian (n = 7), vaginal (n = 5), round ligament (n = 5), inferior epigastric (n = 3), cervical (n = 2), internal pudendal (n = 2), vesical (n = 1), and rectal (n = 1) arteries. The embolic materials used included n-butyl cyanoacrylate (n = 9), gelatin sponge particles (n = 8), gelatin sponge particles with microcoils (n = 1), and polyvinyl alcohol particles (n = 1). In 13 patients, bilateral uterine arterial embolization was performed. Re-embolization was performed in two patients with persistent bleeding. Hemostasis was achieved in 17 (89.5%) patients. Two patients underwent immediate hysterectomy due to persistent bleeding. One patient experienced a major complication due to pelvic organ ischemia. One patient underwent delayed hysterectomy for uterine infarction four months later. Conclusion Non-uterine arteries are major sources of PPH. Detection and selective embolization are important for successful hemostasis.

The baboon (Papio sp.) as a model for female reproduction studies.

BIOLOGY AND REPRODUCTION: Due to their size and anatomical similarity to humans, baboons make an excellent model for reproductive studies. Baboons have a simple short cervix, muscular uterus, ovaries just lateral to the uterus and similar vasculature to that of humans. Because of the size of the animals, instruments designed for use in women can be readily used on baboons. Noninvasive determination of phase of estrous cycle is readily made by observation of changes in perineal sexual skin turgor and color. ADVANTAGES: Some advantages of use of baboons compared to other nonhuman primates is that they are nonseasonal breeders allowing for studies to be conducted year round, have minimal infectious disease risks to humans as they do not carry Herpes B and have a social structure allowing for easy group formation. Baboons serve as good models for many conditions in humans and should be considered for studies investigating reproductive issues.

The pharmacology of the human female orgasm - its biological and physiological backgrounds.

The female orgasm has been examined over the years by numerous scientific disciplines yet it still has many secrets to be disclosed. Because its physiology, especially its neurophysiology, is sparingly understood its pharmacology is necessarily limited based mainly on the side effects of drugs. Few published studies have used a placebo group as controls. The paucity of focussed studies is well illustrated by the fact that there still is no approved medication to treat female orgasmic dysfunction. The present brief overview examines the most important aspects of its biology and especially its physiology highlighting the many questions that need answering if we are to have a comprehensive pharmacology of the female orgasm.

Evaluation of the efficacy of endovascular treatment of pelvic congestion syndrome.

AIM: To assess the efficacy of venous embolization treatment for the pelvic congestion syndrome (PCS). PATIENTS AND METHODS: Retrospective study of 33 female patients undergoing pelvic venous embolization between January 2008 and May 2012 in Bordeaux. The inclusion criteria were clinical symptoms of PCS documented by transabdominal Doppler ultrasound and/or pelvic magnetic resonance imaging. Patients with pelvic varicose veins feeding saphenous varicose veins were excluded. The efficacy of treatment was assessed on a Visual Analog Scale (VAS). RESULTS: Thirty-three patients were included and the mean follow up period was 26months (3-59months). The VAS was 7.37 (standard deviation: 0.99) before embolization and 1.36 (standard deviation: 1.73) after embolization (P<0.0001). Twenty patients reported that their symptoms had completely disappeared, 11 had partially disappeared and two had gained no improvement. A significant fall was found in the number of patients with dyspareunia (P<0.0001). A single technical embolization failure was reported. CONCLUSION: Our series demonstrates the efficacy of embolization treatment with a significant fall in the VAS in patients with PCS.

[The role of the computed tomography in the identification of the syndrome of pelvic congestion]. / El papel de la tomografía computada en la identificación del síndrome de congestión pélvica.

BACKGROUND: Pelvic congestion syndrome is a condition not yet fully understood, hence provokes controversy. It is cause of up to 40% of visits to the doctor; affecting women of reproductive age who experience non-specific symptoms such as characteristic pelvic pain with more than six months of evolution and difficult to treat dyspareunia in which even narcotics are insufficient for control. OBJECTIVE: To recognize the vascular anatomy of the pelvic cavity and identify the characteristics of pelvic congestion syndrome demonstrable by computed tomography. MATERIAL AND METHODS: A descriptive, observational, cross-sectional and retrospective study at Hospital Angeles del Pedregal, in the Department of Radiology and Imaging with patients who reported imaging studies with key findings to recognize the pelvic congestion syndrome. All women with incidental finding of abnormal dilation of the gonadal vein were included, allowing to suggest pelvic congestion syndrome as a possible diagnosis. RESULTS: There were 17 cases (0.9%) of patients with abdominopelvic pain syndrome who underwent multislice computed tomography to 3 mm, with extension from the lung bases to the pubic symphysis. Predominance of left gonadal vein is conditioned by the anatomical arrangement of the left gonadal vein. During the arterial phase opacification of the gonadal vein was identified in 11 patients (65%), a circumstance that correlates with retrograde venous flow valve incompetence. In computed tomography findings of pelvic congestion syndrome were also identified 12 patients (70%) with abdominopelvic pain syndrome. CONCLUSIONS: Pelvic congestion syndrome is a rare condition that radiologists do not consider because they don't know it and the clinical diagnoses give no clinical data to suggest this condition. But if one takes into account the literature, it refers to it as the origin of up to 40% of the visits to the gynecologist, and there may be more cases that will increase its prevalence.

Anti-VEGF- and anti-VEGF receptor-induced vascular alteration in mouse healthy tissues.

Systemic therapy with anti-VEGF drugs such as bevacizumab is widely used for treatment of human patients with various solid tumors. However, systemic impacts of such drugs in host healthy vasculatures remain poorly understood. Here, we show that, in mice, systemic delivery of an anti-VEGF or an anti-VEGF receptor (VEGFR)-2 neutralizing antibody caused global vascular regression. Among all examined tissues, vasculatures in endocrine glands, intestinal villi, and uterus are the most affected in response to VEGF or VEGFR-2 blockades. Thyroid vascular fenestrations were virtually completely blocked by VEGF blockade, leading to marked accumulation of intraendothelial caveolae vesicles. VEGF blockade markedly increased thyroid endothelial cell apoptosis, and withdrawal of anti-VEGF resulted in full recovery of vascular density and architecture after 14 d. Prolonged anti-VEGF treatment resulted in a significant decrease of the circulating level of the predominant thyroid hormone free thyroxine, but not the minimal isoform of triiodothyronine, suggesting that chronic anti-VEGF treatment impairs thyroid functions. Conversely, VEGFR-1-specific blockade produced virtually no obvious phenotypes. These findings provide structural and functional bases of anti-VEGF-specific drug-induced side effects in relation to vascular changes in healthy tissues. Understanding anti-VEGF drug-induced vascular alterations in healthy tissues is crucial to minimize and even to avoid adverse effects produced by currently used anti-VEGF-specific drugs.

[Rectal cancer and Trousseau syndrome. Case report]. / Síndrome de Trousseau y cáncer de recto. Informe de un caso.

BACKGROUND: The Trousseau syndrome, first described in 1865, is the relationship of venous thromboembolisms and cancer. We present a case with rectal cancer and Trousseau syndrome. CLINICAL CASE: Female 40 years old, went to the Coloproctology Service for painless bleeding. A computed tomography report showed a tumor of 5 by 6 cm up 5 cm from the anal margin. Ultra-low anterior resection with colonic reservoir and loop ileostomy surgery was performed. The pathology report showed a semidiferenciate adenocarcinoma of the rectum and we established the stage as T3N0M0. Within 72 hours of her operation, she experienced sudden hypotension and painful abdominal distention. A second surgery was done finding necrosis of the colon from the splenic angle until the colonic reservoir with thrombi in the left colic artery, ischemic signs of bilateral fallopian tubes, ovaries, uterus, pelvic floor and the small intestine, 40 cm before ileostomy and ileon. Left hemicolectomy and colostomy was done. She was taken to intensive care where continuous administration of heparin was given; she died within 5 days because of multiorgan failure. CONCLUSIONS: The mechanism for this syndrome was unknown but there are several hypotheses, suggesting that hematological cancer patients are at an increased risk of deep vein thrombosis. Pancreatic cancer is the most common presentation with this syndrome (in 50% of cases). We suggested continuing with the standards of prevention of thromboembolism.

antecedentes: el síndrome de Trousseau se describió por primera vez en 1865; es la relación entre tromboembolismo venoso y cáncer. Objetivo: informar el caso de una paciente con cáncer de recto y síndrome de Trousseau. Caso clínico: paciente femenina de 40 años de edad que acudió al servicio de Coloproctología por rectorragia indolora. La TAC reportó un tumor de 5 por 6cm y del margen anal a 5cm. Se efectuó resección anterior ultrabaja, con reservorio colónico e ileostomía de protección. El reporte de patología fue de: adenocarcinoma semidiferenciado del recto, con clasificación T3N0M0. A las 72 horas del postoperatorio tuvo hipotensión arterial súbita y distensión abdominal dolorosa. En la reintervención quirúrgica se encontró: necrosis del colon desde el ángulo esplénico hasta el reservorio colónico, con trombos en meso, signos de isquemia en el útero, trompa de Falopio y ovarios, piso pélvico y 40 cm de intestino delgado, antes de la ileostomía e íleon. Se realizó hemicolectomía izquierda y colostomía. Se trasladó a la unidad de terapia intensiva donde continuó con la administración de heparina; falleció a los cinco días por insuficiencia multiorgánica. Conclusiones: el mecanismo de este síndrome se desconoce pero existen varias hipótesis: se ha sugerido que los cánceres hematológicos son los que tienen mayor riesgo de trombosis venosa profunda. El cáncer de páncreas se relaciona con este síndrome en 50% de los casos. Se sugiere continuar con las normas de prevención del tromboembolismo.

Correlación de las curvas Doppler de arterias uterinas con el desarrollo puberal de los genitales internos de niñas y adolescentes: experiencia en un servicio de imagenología pediátrico / Correlation of uterine artery Doppler patterns and the development of the internal genitalia of girls and adolescents: experience in a pediatric imaging department

Ultrasound allows evaluate in girls, the internal genitals development and their follow up during puberty. Doppler of the uterine arteries (UA) has demonstrated be a complementary parameter to detect the onset of puberty. Objective: To show through our experience, the correlation between the internal genital development and the Doppler of UA morphology in girls and adolescents. We analyzed in 84 ultrasounds (US); uterine morphology and the relation body/cervix, endometrial thickness, ovarian volume and the pattern of Doppler UA. We obtained a relationship between the anatomic changes usually studied and the patterns of the Doppler UA, since childhood to puberty. Conclusion: The diastolic flow changes in the UA can be complementary for the diagnosis of the degree of puberty.

El ultrasonido permite la evaluación del desarrollo de los genitales internos en las niñas y seguimiento a través de la pubertad. La curva del Doppler de arterias uterinas (AU) ha demostrado ser un parámetro complementario en la detección del inicio de la pubertad.Objetivo: mostrar la correlación del desarrollo de los genitales internos, con la morfología de las curvas Doppler de AU en niñas y adolescentes a través de nuestra experiencia. Analizamos en 85 ultrasonidos (US); morfología uterina, relación cuerpo/cervix, grosor endometrial, volumen ovárico y morfología de la curva del Doppler de AU. Constatamos una asociaciónentre los cambios morfológico estudiados habitualmente con los distintos patrones de las curvas del Doppler desde la niñez a la pubertad. Conclusión: Los cambios en el flujodiastólico de las arterias uterinas permiten complementar el diagnóstico del grado de progreso de la pubertad.

Angiogenesis and microvasculature in the female reproductive organs: physiological and pathological implications.

The female reproductive organs such as ovary, uterus, and placenta are some of the few adult tissues that exhibit regular intervals of rapid growth, and are highly vascularized and have high rates of blood flow. Angiogenesis is a process of vascular growth that is mainly limited to the reproductive system in healthy adult animals. The development of new blood vessels in the ovary and uterus is essential to guarantee the necessary supply of nutrients and hormones. The genetic and molecular mechanisms that control the development of capillary blood vessels in the reproductive organs are beginning to be elucidated. Reproductive organs contain and produce angiogenic factors which may act alone or in concert to regulate the process of vasculature. Vascular endothelial growth factors (VEGFs) and fibroblast growth factor (FGFs) are key factors for vascular system in the reproductive organs. Recent numerous studies reported several roles of VEGFs and FGFs on ovarian and uterine functions. In this review, we focus on the involvement of VEGFs and FGFs as angiogenic factors on reproductive organs and vascular therapy for diseases of reproductive organs using anti-angiogenic agents.