Tratamiento supermicroquirúrgico del linfocele inguinal con linfedema intercurrente: restauración de la fisiología linfática / Supermicrosurgical approach to inguinal lymphocele with lymphedema: restoring the lymphatic physiology

Resumen Introducción: El linfocele es una patología que puede ocurrir por la disrupción linfática durante una cirugía, siendo frecuente luego de una linfadenectomía inguinal. Existen diversos enfrentamientos para prevenirlo o minimizarlo, sin embargo, los resultados son inconsistentes. Caso clínico: Reportamos el caso de una mujer, con linfocele recurrente en zona inguinal derecha y linfedema distal de la extremidad secundario a una biopsia ganglionar, tratado exitosamente mediante supermicrocirugía para restaurar el drenaje linfático. La extremidad inferior tenía un exceso de volumen de 7,03%. Se realizaron estudios preoperatorios con linfografía por resonancia magnética y linfografía con verde de indocianina para identificar los vasos linfáticos y realizar anastomosis linfático-venosas (ALV). Se identificaron tres vasos linfáticos aferentes y se realizó una capsulectomía total. Se realizaron tres ALV término-terminales supermicroquirúrgicas en zona inguinal y una ALV distal en pierna. Durante seguimiento no hubo recidiva del linfocele, evidenciándose una reducción del exceso de volumen de la extremidad afectada de un 105,26%. El linfocele inguinal y linfedema pueden ser tratados exitosamente mediante supermicrocirugía, restaurando el flujo linfático de manera fisiológica, evitando la recurrencia de linfocele y mejorando los síntomas del linfedema.

Introduction: Lymphocele may occur after the disruption of lymphatic channels during a surgical procedure. After inguinal lymphadenectomy are very common, and many different approaches have been tried to prevent or minimize the formation of lymphoceles with inconsistent results. Clinical Case: We report a case of a female patient who presented with right recurrent inguinal lymphocele and lower limb lymphedema after lymph-node biopsy that was successfully treated with lymphatic supermicrosurgery restoring the lymph flow. Lower extremity had an excess volume of 7,03% compared to the healthy contralateral limb. Preoperative study with magnetic resonance lymphangiography and indocyanine green lymphography were done to identify intraoperatively lymphocele afferent and distal lymphatic vessels to perform lymphovenous anastomosis (LVA). Three different afferent lymphatics were identified and total capsulectomy was performed. Three end-to- end supermicrosurgical LVA in the groin and one distal LVA on the leg were performed. The surgery was uneventful, and there were no postoperative complications. In the follow-up, no lymphocele was noticed and lymphedema had visibly reduced with a reduction of excess volume of 105.26%. Inguinal lymphocele and lymphedema can be successfully treated with supermicrosurgery since it is a physiological approach to restore the lymphatic flow, in order to avoid lymphocele recurrence and to improve lymphedema symptoms.

Linfedema escrotal masivo: Reporte de caso y revisión de la literatura / Massive scrotal lymphedema: case report and literature review

El lindefema masivo localizado es una condición rara pero su incidencia se encuentra en aumento. El linfedema puede ser primario o secundario a infecciones, radioterapia, cirugías, cáncer, enfermedades del sistema inmune o a compresión. Algunos autores han sugerido un incremento en su incidencia debido a la epidemia de obesidad. El linfedema escrotal produce una morbilidad importante debido a mala higiene, infecciones, incontinencia urinaria, disfunción sexual, pérdida de la libido e inclusive limitación en la deambulación. Las opciones de tratamiento no quirúrgico de la linfedema incluyen el drenaje linfático manual, prendas de compresión, terapia física y ejercicio. Usualmente, el tratamiento conservador falla debido a la dificultad anatómica que presenta la región genital para la aplicación de dichas terapias. El tratamiento quirúrgico puede ser efectivo en casos graves y moderados. Existen diferentes variantes a la técnica quirúrgica, pero la resección del tejido afectado y la reconstrucción con tejido sano es primordial. El pronóstico de esta patología tiende a ser bueno; sin embargo, se reporta una alta tasa de recidiva.

Massive localized lymphedema is a rare but rising condition that could be primary in nature or secondary to infections, radiation, surgery, malignancies, autoimmune or compression. Some authors have suggested and increased incidence due to the raising epidemic of obesity. Scrotal lymphedema causes significant morbidity such as poor hygiene, infections, urinary incontinence, sexual dysfunction, loss of libido and a limited ambulation. Although non operative treatment options for lymphedema include manual lymph drainage, compression garments, exercises, sequential gradient pump. Usually, non-operative techniques fail due to the difficult in the anatomic area. Surgical treatment can be effective in moderate to severe cases of giant scrotal lymphedema. There are many variations of the surgical technique, however, resection of the affected tissue and reconstruction with healthy skin is primordial. The prognosis tends to be good, although there is reported a significant amount of relapse, mostly dependent on the weight lost and diet of the patients

Genetic analysis and clinical phenotype of a family with lymphedema-distichiasis syndrome / 浙江大学学报·医学版

OBJECTIVE@#To identify the genetic causes of a family with lymphedema-distichiasis syndrome (LDS).@*METHODS@#The whole exome sequencing was performed in a aborted fetus as the proband, and a candidate gene was identified. Peripheral blood of 8 family members were collected. Genotypic-phenotypic analysis were carried out through PCR amplification and Sanger sequencing.@*RESULTS@#The proband, and the mother, grandmother, uncle, granduncle of the proband all had distichiasis or varix of lower limb carried a @*CONCLUSIONS@#The

Morbihan disease: a therapeutic challenge

Abstract: Morbihan disease is a rare condition characterized by chronic and persistent erythematous solid edema localized on the face. It is believed to be a complication of rosacea and may occur at any stage of the disease. Features of this condition include variable therapeutic response and great refractoriness. We report a case of a 61-year-old man with rosacea history diagnosed with Morbihan disease, who showed excellent therapeutic response with the combination of deflazacort and oral isotretinoin but developed recurrence after corticosteroid discontinuation. We believe that in severe cases of lymphedema of the face this combination is effective and corticosteroid suspension should be done slowly and gradually.

Translation and adaptation to Brazilian Portuguese of the Lymphedema Rating Scale in Head and Neck Cancer / Tradução e adaptação para o português brasileiro da Lymphedema Rating Scale in Head and Neck Cancer

ABSTRACT Objective: Translate to brazilian portuguese, culturally adapt and test the rating and classification scales of cervicofacial lymphedema of the MD Anderson Cancer Center Head and Neck Lymphedema Protocol (MDACC HNL) in patients undergoing treatment for head and neck cancer. Methods: The process followed international guidelines and translation stages by two head and neck surgeons, and back translation independently by two native Americans. The test of final version was based on the evaluation of 18 patients by one speech pathologist and one physical therapist who applied the scales in Portuguese. Results: The translation of the three scales was carried out independently and the translators reached a consensus for the final version. Minor modifications were made by translating two terms into the Assessment of the Face. Versions of back-translation were similar to each other. The instrument was successfully applied to patients independently. Conclusion: The translation and cultural adaptation of the assessment and rating scale of the cervicofacial lymphedema of the MD Anderson Cancer Center Head and Neck Lymphedema Protocol to the Brazilian Portuguese were successful.

RESUMO Objetivo: Traduzir, para o português brasileiro, adaptar culturalmente e testar as escalas de avaliação e classificação do linfedema cérvico-facial do MD Anderson Cancer Center Head and Neck Lymphedema Protocol (MDACC HNL) em pacientes submetidos ao tratamento para o câncer de cabeça e pescoço. Métodos: O processo seguiu as diretrizes internacionais e as etapas de tradução por dois cirurgiões de cabeça e pescoço, além de retrotradução de forma independente por dois nativos norte-americanos. O teste da versão final foi realizado a partir da avaliação de 18 pacientes por um fonoaudiólogo e um fisioterapeuta, por meio da aplicação das escalas em português. Resultados: A tradução das três escalas foi realizada de forma independente, e os tradutores chegaram a um consenso para a versão final. Foram feitas pequenas modificações, ao serem traduzidos dois termos em Assessment of the Face . As versões da retrotradução foram semelhantes entre si. O instrumento foi aplicado com sucesso nos pacientes de forma independente. Conclusão: A tradução e a adaptação das escalas de avaliação e classificação do linfedema cérvico-facial do MD Anderson Cancer Center Head and Neck Lymphedema protocol para o português foram bem sucedidas.

Ground reaction force patterns during gait in patients with lower limb lymphedema / Forças de reação ao solo durante a marcha de pacientes com linfedema de membro inferior

Although gait problems have been reported in patients with lower limb lymphedema (LLL), the gait pattern (GP) changes have not been documented yet. However, it is possible that patients with LLL show abnormal GP that can be related to biomechanical complications related to osteoarthritis or falls affecting the quality of life. Ground reaction force analysis during gait allows objective assessment of the patients and it can be used to plan a rehabilitation approach. Objective: To analyze the GRF during gait in patients LLL. Methods: An experimental descriptive study was realized with twenty-three LLL patients, both unilateral and bilateral and classified as moderate and severe, participated in the experiments. The patients walked on a force plate while the three ground reaction force (GRF) components, vertical, mediolateral (M-L) and anteroposterior (A-P), under their feet were recorded and analyzed. Results: In the patients with unilateral lymphedema, either moderate or severe, the vertical GRF components of the affected limb were similar to the sound one and also resembling those found in healthy adults. The M-L GRF was smaller in the non-affected side. In patients with bilateral lymphedema gait speed was significantly slower. More interestingly, the vertical GRF pattern was flat, not showing the typical 2-peak shape. Finally, the large M-L forces found suggest gait stability problems. Conclusions: The patients showed abnormal GRF patterns, including compensation with the non-affected leg. The GRF variability was higher in the patients with severe unilateral lymphedema. Bilateral lymphedema results in lower A-P forces. Stance phase duration was longer in patients with bilateral and severe lymphedema

Porokeratosis simulating Bowen's disease on dermoscopy

Abstract Porokeratosis is a disorder of epidermal keratinization characterized by the presence of annular hyperkeratotic plaques. Its etiopathogenesis is not yet fully understood, but a relationship with immunosuppression has been reported. Dermoscopic examination revealed a classic yellowish-white ring-like structure that resembled “volcanic crater contour” – the so-called cornoid lamella. We describe a case of porokeratosis in a female patient with chronic lymphedema, which was similar to Bowen’s disease due to the many glomerular vessels seen on clinical examination and dermoscopy.

Stewart Treves Syndrome

Stewart-Treves Syndrome is characterized by the presence of lymphangiosarcoma on limb extremities. Rare, it occurs in 0.5% of patients who have undergone radical mastectomy with axillary node dissection. The main cause is chronic lymphedema with endothelial and lymphatic differentiation, with no direct relationship to breast cancer. Seven years after a radical right-side mastectomy with lymph node dissection and adjuvant therapy, the patient developed a lesion on her right arm. The dermatological examination revealed an erythematous nodule with bleeding surface on chronic right forearm lymphedema. After the biopsy, a lymphangiosarcoma on chronic lymphedema was diagnosed. Infrequent, this syndrome is relevant because of its associated mortality. Early diagnosis is important to improve survival and reduce complications.

Stewart-Treves Syndrome of the Lower Extremity

Stewart-Treves syndrome is a rare cutaneous angiosarcoma that develops in long-standing chronic lymphedema. Though most commonly this angiosarcoma is a result of post mastectomy lymphoedema, it also develops in Milroy disease, idiopathic, congenital, traumatic and filarial lymphoedema. Despite the rarity of this syndrome and its poor prognosis, early diagnosis associated with radical surgery can provide improved survival. We report a case of angiosarcoma in the lower limb in a patient with chronic lymphedema associated with history of repeated erysipela episodes.

Unusual manifestation of the yellow nail syndrome - Case report

The yellow nail syndrome is a rare disorder characterized by the classic triad of yellow and dystrophic nails, lymphedema and pleural effusion. We report in this paper a case of yellow nail syndrome, presenting the classic triad of the disease, associated with an unusual lymph accumulation in the abdomen region.

Síndrome de Stewart-Treves: Caso clínico / Stewart-Treves syndrome: Report of one case

Angiosarcomas are malignant tumors derived from the endothelium of blood vessel (hemangiosarcomas) or lymph vessels (lymphangiosarcomas). Lymph edema of the limbs is considered secondary when extrinsic injuries are observed and primary when these injuries are not present. Stewart-Treves syndrome or a Lymphangiosarcoma, developed over a chronic lymph edema, is a rare complication described in mastectomized patients but it can be observed in lymph edemas located elsewhere. It appears as nodular skin lesions that grow, multiply quickly and frequently metastasize. We report a 40-year-old mole with an angiosarcoma associated with primary chronic lower limb lymph edema. The patient consulted for a history of weight loss and malaise and appearance of violaceous lesions over the zone of lymph edema and inguinal lymph node involvement. A CT scan showed bilateral lung lesions and enlargement of inguinal and iliac lymph nodes. A biopsy of one of the skin lesions disclosed an angiosarcoma, diagnosis that was confirmed with immuno-histochemistry Chemotherapy was started but the patient died five months after the diagnosis.

Enfermedad de morbihan / Morbihan´s disease

La enfermedad de Morbihan (morbus Morbihan) es una entidad poco frecuente, que se caracteriza por la aparición recurrente, a nivel del tercio medio y superior del rostro, de eritema y edema, que conducen a una induración desfigurante. Actualmente se plantea que la urticaria de contacto inmunológica desempeña un rol crucial en la patogenia. Presentamos un paciente de 65 años con enfermedad de Morbihan de 2 años de evolución.

Morbihan’ s disease (morbus Morbihan) is an infrecuent entity, characterized by the appearance of relapsing erithema and edema on the center and upper aspect of the face, leading to disfi guring induration.Nowadays, immunologic contact urticaria plays a crucial role in the pa-togenesis. We present a 65 year-old patient with Morbihan’ s disease, with its onset 2 years ago.

Linfangite granulomatosa de genitália infantil: relato de caso e revisão da literatura / Granulomatous lymphangitis of children's genitalia: report of a case and review of the literature

Linfangite granulomatosa de genitália não-infecciosa é causa rara de linfedema genital autolimitado e idiopático em crianças(4, 6, 9). A maioria dos casos de linfedema com granuloma não-infeccioso de genitália em pacientes jovens ocorre em associação à doença de Crohn subseqüente ou concomitante(1, 3, 4, 9). O caso relatado é de um menino de 14 anos que apresentava história de linfedema genital principalmente em região dorsal do corpo do pênis, poupando prepúcio, parte distal do pênis e saco escrotal. Estudos laboratoriais não revelavam doenças sistêmicas. O exame anatomopatológico da peça revelou tratar-se de linfangite granulomatosa não-infecciosa.

Non-infectious granulomatous lymphangitis of genitalia is a rare cause of self-limited and idiopathic genital lymphoedema in children(4, 6, 9). Most cases of lymphoedema with non-infectious genital granulomas in young patients occur subsequent to or concomitant with Crohn's disease(1, 3, 4, 9). The case described is of a 14-year-old boy with history of genital lymphoedema mainly on the dorsal region of the body of the penis, sparing the prepuce, the distal part of the penis and the scrotum. Laboratory studies did not reveal systemic diseases. The histopathological analysis showed it was non-infectious granulomatous lymphangitis.

Linfedema post mastectomía / Postmastectomy lymphedema

El abordaje quirúrgico de la axila ha sido parte del tratamiento del cáncer de mama, desde el final del siglo pasado, después de la publicación de los trabajos de Halsted y Meyer. Por otro lado, la radioterapia en la axila, aunque no sea actualmente indicada conjuntamente con la mastectomía, aún puede ser utilizada en algunos casos específicos, como en el de pacientes con diagnóstico de neoplasia en el estadio III. Ambos procedimientos serán causantes de un bloqueo en el sistema linfático que drena el miembro superior del lado tratado. Este hecho será la causa principal del edema miembro superior. El aumento del diámetro del brazo y el edema del miembro superior. El aumento del diámetro del brazo y el edema conforman una situación que, por si sola, puede aumentar el stress de la pacientes en relación a la enfermedad, una vez que esa alteración tiende a recordarla, de una manera muy frecuente, su condición de portadora de cáncer de mama. Eso también llama la atención de las demás personas, transformando la paciente en motivo de curiosidad y dificultando su convivencia social. A su vez el médico tiende a no restar mucha atención a estas molestias de la paciente, una vez que las mismas no causan ningún daño o consecuencias más serias para la mujer y, de cierta forma, llega hasta a abandonar a la paciente con sus temores respecto a ese problema específico

Clinical manifestations of bancroftian filariasis with special reference to lymphoedema grading.

We report on some aspects of progression of chronic disease and its association with acute manifestations with special reference to grades of lymphoedema in bancroftian filariasis. These analyses were based on the clinical history and clinical findings of 1300 individuals at the time of their first visit to the filariasis clinic at a centre in south India. The mean number of adenolymphangitis (ADL) attacks in one year was 4.9 +/- 1.7, 5.5 +/- 0.9 and 10.4 +/- 3.2 in patients with grade I, grade II and grade III lymphoedema respectively. The mean duration of oedema was 47.4 +/- 5.9 days, 6.2 +/- 0.5 and 8.6 +/- 0.9 yr in patients with grade I, grade II and grade III lymphoedema respectively. These findings suggest that the progression of lymphoedema from one grade to the next in bancroftian filariasis is associated with increased frequency of ADL attacks.

Clinical manifestations in malayan filariasis infection with special reference to lymphoedema grading.

In a door-to-door survey in Shertallai area of Kerala state in southern India, 7,766 persons were examined for clinical manifestations of filariasis. The prevalence of disease was 9.85 per cent and chronic persistent oedema (grade-II) was the predominant clinical presentation in both sexes. There was no significant difference in the age prevalence of disease between the sexes. Incidence of episodic filarial fever was higher (4.73/1000) compared to other manifestations (2.49/1000) in children aged 5-15 yr and hence this may be an useful indicator of the degree of occurrence of fresh cases of clinical filariasis. Classification of patients with lymphoedema into three grades showed that 1.65 per cent of those examined had developed lymphoedema within 6 months. Persistence of oedema (grade II) for prolonged period (13.64 +/- 1.17 yr) without skin changes indicated that the development of elephantiasis is a slow process. The clinical picture of malayan filariasis is similar to that reported elsewhere in the world except for the rarity of abscess formation is inguinal area.

Hydroa vacciniforme / Hydroa vacciniforme

Se presenta el caso de un paciente portador de Hydroa Vacciniforme con una larga historia evolutiva en el que la enfermedad adopta una inusual agresividad, con importantes lesiones cicatrizales en áreas expuestas, secuelas oculares y un excepcional compromiso de áreas cubiertas con lesiones cicatrizales en zona genitoperineal. Se asocia como complicación un linfedema crónico de pene.

Subcutaneous phycomycosis mimics elephantiasis.

A case of subcutaneous phycomycosis involving the right thigh for 1 year duration which mimic elephantiasis was reported in a 21-year-old military man. An initial diagnosis was that of a subcutaneous tissue lesion attributed to parasitic manifestation but turned out to be that of subcutaneous fungal infection both clinically and histologically. The lesion was successfully treated with cotrimoxazole for a period of one month, and followed up for 4 1/2 months with complete cure.

Linfedema congenito informe de dos casos en hermanos / Lymphedema congenital inform of two cases in brothers

El linfedema congenito ocurre en la enfermedad de Milroy y tambien como parte del sindrome de disgenesia gonodal. El linfedema adquirido, pueda ocurrir en forma secundaria a prosesos inflamatorios del sistema linfatico o despues de la irradiacion y cirugia, debido a la obliteracion de los canales o ganglios linfaticos. El linfedema se caracteriza por la presencia de edema difuso, permanente y secundario a una obstruccion linfatica de cualquier area del organismo. El lindefema es mas frecuente en las extremidades inferiores. En este informe presentamos a dos hermanos (mujer y hombre) que nacieron de padres normales, sin historia familiar de la enfermedad, teniendo entre ellos una hermana completamente normal. Probablemente se trata de dos casos de lindefema congenito esporadico; el estudio de los casos no revelo ninguna otra patologia.