Acquired vulvar lymphangioma: risk factors, disease associations, and management considerations: a systematic review.

Acquired vulvar lymphangioma (AVL) is not well-characterized. Diagnosis is delayed and the condition is often refractory to therapy. Objective: The objective of this study was to provide a systematic review of AVL including risk factors, disease associations, and management options. Methods: A primary literature search was conducted using 3 databases: PubMed, CINAHL, and OVID, from all years to 2022. Results: In total, 78 publications with 133 patients (48 ± 17 years) were included. Most studies were case reports/series. The most common disease association was prior malignancy (70 patients, 53% of cases) and inflammatory bowel disease (6 patients, 5% of cases). The most common malignancy was cervical cancer (57 patients, 43% of cases). Most patients had prior radiation or surgery, with 36% (n = 48) treated with radiation, 30% (n = 40) with lymph node dissection, and 27% (n = 36) with surgical resection. Common presenting symptoms included discharge/oozing, pain, and pruritus. Most patients underwent surgical treatment for AVL with 39% treated with excision, 12% with laser therapy (the majority used CO2), and 11% with medical therapies. Most patients had failed prior therapies and there was a diagnostic delay. Limitations: Retrospective nature. Most studies were limited to case reports and case series, with interstudy variability and result heterogeneity. Conclusion: AVL is an underrecognized entity and should be considered in patients with a history of malignancy or radiation to the urogenital area. Treatment should include multidisciplinary care and address underlying lymphatic changes, manage any existing inflammatory conditions, and utilize skin-directed therapies and barrier agents while addressing symptoms of pruritus and pain. Prospective studies are needed to further characterize AVL and develop treatment guidelines.

Vulvovaginal Complaints as the First Manifestation of Crohn's Disease in Children: Case Report.

BACKGROUND: Crohn's disease, a chronic gastrointestinal inflammatory condition, can involve gynecological structures. Rectovaginal or rectovestibular involvement may be the first sign in the pediatric population and may result in delayed diagnosis and treatment. CASE: A 9-year-old premenarchal female with chronic constipation and poor growth presented to the pediatric gynecologist for evaluation of persistent vulvovaginal discharge and vulvar irritation. Examination under anesthesia revealed a rectolabial fistula; colonoscopy was diagnostic of Crohn's disease. Immunotherapy resulted in improvement of symptoms and anatomical changes. SUMMARY AND CONCLUSION: In cases of persistent vulvar complaints in a child without clear diagnosis, a high index of suspicion for a non-gynecologic diagnosis is necessary. Collaboration between pediatric gynecologists, gastroenterologists, and surgeons can result in prompt diagnosis and treatment of genital Crohn's disease.

Massive vulvar edema during pregnancy: A case report.

INTRODUCTION: Massive vulvar edema is an unusual complication of pregnancy that may be due to underlying systemic pathology but has also been associated with preeclampsia. It is likely to interfere with vaginal delivery. It has been associated with maternal mortality in the postpartum period. OBSERVATION: A 34 years old female patient, having already delivered a child by vaginal route, without any other particular pathological history, admitted for severe pre-eclampsia on an unattended pregnancy estimated at 36 weeks of amenorrhea. The examination on admission showed a blood pressure of 170/110 mmHg, a proteinuria of three crosses on the urine dipstick. Examination of the vulva showed massive vulvar edema. The massive vulvar edema was explained by hypoprotidemia secondary to renal damage in the context of severe pre-eclampsia. The evolution was marked by the rapid normalization of the tentional figures and a spectacular regression of the vulvar edema, and its complete disappearance in fifteen days. DISCUSSION: Edema may be seen in 80 % of pregnant women, but isolated massive vulvar edema is rare in pregnancy. Massive vulvar edema has been reported in the literature after tocolysis, vulvovaginitis, Crohn's disease and pre-eclampsia. Treatment of vulvar edema is necessary because it can be alarming to the patient and may lead to occlusion of the vulvar orifices. The patient with vulvar edema deserves special attention, and identification and treatment of the associated factors are essential to its management. CONCLUSION: Massive vulvar edema is rare in pregnancy but requires special attention because it can be associated with maternal and fetal complications. Treatment is symptomatic and etiologic whenever an underlying cause is found and the evolution is often favorable with proper treatment.

Prolonged labor presenting as vulvar edema during pregnancy.

Isolated vulvar edema is a rare complaint during pregnancy with a long list of differential diagnosis. Here, we describe a case of vulvar edema due to obstructed labor presenting to a rural primary healthcare center in Nepal.

Massive vulvar edema revealing idiopathic systemic capillary leak syndrome.

Idiopathic systemic capillary leak syndrome is a rare disease characterized by recurrent episodes of hypotension, hypoalbuminemia and peripheral edema caused by capillary hyperpermeability with approximately 350 documented cases worldwide. We report herein the case of a 22-year-old primiparous patient with an unusual compartment syndrome represented with spontaneous massive vulvar edema. Treatment consisted of volume replacement and intravenous polyvalent immunoglobulins perfusions. Vulvar edema was treated surgically by incision and drainage with favorable outcome. The pregnancy follow-up did not show any fetal abnormalities. The childbirth at 35 weeks was natural and the newborn was healthy.

Preeclampsia asociada a debut de lupus eritematoso sistémico y edema vulvar masivo gestacional / Severe preeclampsia associated with the onset of systemic lupus erythematosus and massive gestational vulvar edema / Pré-eclâmpsia grave associada a lúpus eritematoso sistêmico e edema vulvar gestacional maciço

El debut del lupus eritematoso sistémico (LES) durante el embarazo, intrincado con preeclampsia grave, asociado a edema vulvar masivo gestacional, es raro y de difícil diagnóstico. Se reporta una paciente de 19 años, con 35 semanas de gestación, que debutó con LES durante el tercer trimestre del embarazo, y que consulta por manifestaciones cutáneas, dadas por eritemas en pulpejos de dedos de manos. Se constata preeclampsia severa. Se realiza cesárea de urgencia, e ingresa a la unidad de cuidados intensivos. Instala edema de vulva masivo que incapacita la deambulación.

The onset of systemic lupus erythematosus during pregnancy, complicated with severe preeclampsia, associated with massive gestational vulvar edema, is rare and difficult to diagnose. A 19-year-old patient is reported, with 35 weeks of gestation, who started with systemic lupus erythematosus during her third trimester of pregnancy, and consulted due to cutaneous manifestations caused by erythema on the pads of the fingers. Severe preeclampsia was observed. An emergency cesarean section was performed, and the patient was admitted to the Intensive Care Unit. She developed a massive vulvar edema that disabled ambulation.

O aparecimento de lúpus eritematoso sistêmico durante a gravidez, intrincado com pré-eclâmpsia grave é raro e de difícil diagnóstico. É relatada uma paciente de 19 anos, com 35 semanas de gestação, que estreou com lúpus eritematoso sistêmico no terceiro trimestre de gestação, e que se consultou por manifestações cutâneas causadas por eritema nas pontas dos dedos das mãos. Também existe uma pré-eclâmpsia grave. E realizada cesárea de emergência e ela é internada na Unidade de Terapia Intensiva. Instala um edema vulvar maciço que impede a deambulação.

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

BACKGROUND: We present a rare case of severe vulvar edema secondary to steroid-refractory nephrotic syndrome in a prepubertal girl. CASE: The patient is an 8-year-old girl who presented during nephrotic syndrome relapse. She exhibited severe mons pubis and labial edema. She was treated with local symptomatic measures such as sitz baths, barrier ointment, and labial sling, with minimal relief. Improvement ultimately occurred after bilateral nephrectomy. SUMMARY AND CONCLUSION: Vulvar edema is rare in prepubertal girls. In this case, the edema was secondary to steroid-refractory nephrotic syndrome and was not responsive to local treatment measures. There is a paucity of data on effective treatment of vulvar edema in young girls. Our goal is to raise awareness of such pathology, which might lead to development of uniform guidelines for treatment of this condition.

Tratamiento del edema vulvar con terapia de frío en el puerperio inmediato: serie de casos / Vulvar edema management with cold therapy in the immediate puerperium: A case series

Resumen OBJETIVO: Describir los desenlaces de la terapia en frío aplicada a pacientes con edema vulvar patológico en el puerperio. MATERIALES Y MÉTODOS: Estudio observacional, descriptivo y retrospectivo consistente en la revisión de los registros clínicos de pacientes con diagnóstico de edema vulvar posparto atendidas en 2018 en el Instituto Nacional de Perinatología Isidro Espinosa de los Reyes. Se analizaron las comorbilidades, los reportes del laboratorio, los tratamientos efectuados y su desenlace. RESULTADOS: Se reportaron cuatro casos de edema vulvar en el puerperio de pacientes primigestas, de edad promedio de 20 años, tres con parto instrumentado. Ninguna con feto macrosómico. Dos pacientes requirieron episiotomía medio lateral y una sufrió desgarro de tercer grado. Todas con comorbilidades asociadas. Todas se trataron con terapia de frío, antiinflamatorios, furosemida o albúmina intravenosa. A las 48 horas de haber iniciado el tratamiento las 4 pacientes reportaron mejoría, con estancia hospitalaria promedio de 3 días. CONCLUSIONES: El tratamiento con terapia de frío disminuyó el edema vulvar; es un procedimiento de fácil aplicación y sin eventos adversos.

Abstract OBJECTIVE: The aim of this study is to describe the outcomes of patients with vulvar edema, who received treatment with cold therapy during the puerperium. MATERIALS AND METHODS: Observational, descriptive and retrospective study consisting of the review of the clinical records of patients diagnosed with postpartum vulvar edema treated in 2018 at the Isidro Espinosa de los Reyes National Institute of Perinatology. Comorbidities, laboratory reports, treatments performed, and their outcome were analyzed. RESULTS: Four cases of vulvar edema were reported in the puerperium of primitive patients, with an average age of 20 years, three with instrumented delivery. None with macrosomic fetus. Two patients required middle lateral episiotomy and one suffered a third-degree tear. All with associated comorbidities. All were treated with cold therapy, anti-inflammatory, furosemide or intravenous albumin. At 48 hours after starting treatment, all patients reported improvement, with an average hospital stay of 3 days. CONCLUSIONS: Treatment with cold therapy decreased vulvar edema; it is a procedure of easy application and without adverse events.

Unilateral Labial Edema in a Female Adolescent: A Gynecologic Presentation of Rhabdomyolysis.

BACKGROUND: Rhabdomyolysis can be the result of vigorous physical activity. Typical signs and symptoms at presentation are muscle pain, weakness, or dark colored urine. There is no scientific literature associating rhabdomyolysis with gynecologic signs and symptoms. CASE: We present a case of a 16-year-old girl who presented to our pediatric emergency department with complaints of progressive left vulvar swelling. The patient underwent magnetic resonance imaging, which showed polymyositis in the rectus abdominus, external and internal obliques, and bilateral vastus lateralis muscles. Laboratory evaluation showed elevated liver transaminases, creatine kinase, and myoglobin serum levels. SUMMARY AND CONCLUSION: Exertional rhabdomyolysis is associated with muscular damage after vigorous exercise. This case is an example of a patient with rhabdomyolysis presenting with an unusual external gynecological manifestation, as unilateral labial edema.

Vulvar involvement in pediatric Crohn's disease: a systematic review.

PURPOSE: The aim of the study is to report a systematic review (from 2000 to 2017) of all pediatric cases of vulvar Crohn's disease (VCD) and to highlight the key-points for a correct diagnosis and management of this rare condition. METHODS: An electronic search using the Pubmed/Medline, Scopus, EMBASE, Cochrane database and Google Scholar database was performed according to PRISMA guidelines. RESULTS: Twenty pediatric studies and 22 cases of VCD were included for analysis. All the articles reported a single case, except two articles where two cases, respectively, are described. Clinical vulvar examination showed the following main manifestations: vulvar erythema (9/22 cases, 40.9%), vulvar swelling (8/22 cases, 36.4%), vulvar edema (8/22 cases, 36.4%), vulvar ulcers (4/22 cases, 18.2%). Perianal and/or anal involvement (fissures, vegetations, skin tags, erythema, papules, nodules) were recorded in ten cases (45.4%). Steroids per os and/or topical administration were the most prescribed treatment, achieving clinical remission in 11 cases (50%), used alone or in combination with metronidazole or 5-aminosalicylic acid, azathioprine or sulphasalazine/mesalazine. CONCLUSIONS: This review shows that pediatric VCD is an uncommon disease, difficult to be diagnosed as either symptoms or clinical lesions are not specific. A multidisciplinary approach is advised to reach a correct diagnosis and plan clinical treatment.

Manual lymphatic drainage and multilayer compression therapy for vulvar edema: a case series.

BACKGROUND AND OBJECTIVE: Vulvar edema is a condition rarely reported and without defined treatment that may result in functional limitation. The aim of the report is to describe a case series of patients with disabling vulvar edema of different etiologies that were treated with manual lymphatic drainage (MLD) and multilayer compression therapy (MCT). CASE SERIES: Four cases of vulvar edema are described: one in a woman with cervical cancer; one in a woman in the postoperative period of bilateral adrenalectomy for pheocromocytoma; and two in pregnant women with preeclampsia. All cases were treated with MLD and MCT during hospitalization. OUTCOMES: Total resolution of the edema occurred in 2 to 5 d of treatment. CONCLUSION: The present case series is the first to report the use of the MLD and MCT in the successful management of female genital edema. This report suggests that the vulvar edemas for these four patients treated with MLD and MCT seem to resolve faster than expected based on previously reported untreated edemas or edemas treated with different therapeutic approaches.