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3.
J Plast Reconstr Aesthet Surg ; 71(11): 1625-1630, 2018 11.
Artigo em Inglês | MEDLINE | ID: mdl-30327121

RESUMO

The purpose of this paper is to report on the relationship between lymphoscintigraphic findings and the operative results of lymphaticovenous anastomosis (LVA) as well as the resection of genital acquired lymphangiectasia (GAL). Seventeen patients with GAL who underwent lymphoscintigraphy between April 2012 and June 2016 were included in this retrospective study. LVA and GAL resections were performed for 14 patients. The GALs were resected in a spindle shape to the full thickness of the genital skin, and the wounds were closed primarily. Following the procedure, we investigated the recurrence rate and compared the results with the lymphoscintigraphic findings. The average follow-up period was 19.2 months. Six patients were without recurrence, and eight patients experienced a small recurrence of GAL. The average period from operation to recurrence was 16.8 months (range: 1-41 months). Of the six patients who had genital accumulation in lymphoscintigraphy, two patients experienced recurrence (33.3%), whereas six of the eight patients without genital accumulation experienced recurrence (75.0%). The frequency of cellulitis was observed to be reduced postoperatively in all patients, and recurrence was observed in one patient at 11 months postoperatively. In conclusion, lower limb LVA combined with genital skin tumor resection was effective for preventing the recurrence of GAL in patients who demonstrated communication between the lymphatic vessels in the lower limb and genitals. This procedure also had the benefit of reducing the frequency of cellulitis for an average of 19.2 months of follow-up.


Assuntos
Doenças dos Genitais Femininos/cirurgia , Linfangiectasia/cirurgia , Vasos Linfáticos/cirurgia , Veias/cirurgia , Adulto , Idoso , Anastomose Cirúrgica , Feminino , Seguimentos , Doenças dos Genitais Femininos/diagnóstico por imagem , Doenças dos Genitais Femininos/etiologia , Humanos , Linfangiectasia/diagnóstico por imagem , Linfangiectasia/etiologia , Linfocintigrafia , Pessoa de Meia-Idade , Recidiva , Estudos Retrospectivos , Resultado do Tratamento
4.
Br J Ophthalmol ; 102(1): 54-58, 2018 01.
Artigo em Inglês | MEDLINE | ID: mdl-28500230

RESUMO

BACKGROUND: Fabry disease (FD) is a treatable multisystem disease caused by a defect in the alpha-galactosidase gene. Ocular signs of FD, including corneal verticillata, are among the earliest diagnostic findings. Conjunctival lymphangiectasia (CL) has not previously been associated with FD. METHODS: We examined the eyes of a cohort of 13 adult patients, eight men and five women, with documented classic FD, all treated with enzyme replacement therapy (ERT) at the University of Alabama at Birmingham between February 2014 and April 2015. The average age was 48 years with a range of 35-55 years for men and 21-71 years for women. The mean duration of ERT was 8.4 years (men 8.9 years, women 7.6 years) with a range of 4-14 years. Classical Fabry mutations included Q283X, R227X, W236X and W277X. A high resolution Haag-Streit BQ-900 slit lamp with EyeCap imaging system was used to record conjunctival images. RESULTS: CL was observed in 11 of the 13 patients (85%) despite long-term ERT. Clinical presentations included single cysts, beaded dilatations and areas of conjunctival oedema. Lesions were located within 6 mm of the corneal limbus. Ten of the 13 subjects (77%) had Fabry-related cataracts and all 13 demonstrated bilateral corneal verticillata. Twelve of the 13 patients had evidence of dry eye, 9 of whom were symptomatic, and 10 had peripheral lymphoedema. CONCLUSION: CL represents a common but under-recognised ocular manifestation of FD, which persists despite ERT, and is often accompanied by peripheral lymphoedema and dry eye syndrome.


Assuntos
Túnica Conjuntiva/irrigação sanguínea , Doenças da Túnica Conjuntiva/etiologia , Doença de Fabry/complicações , Linfangiectasia/etiologia , Adulto , Idoso , Doenças da Túnica Conjuntiva/diagnóstico , Feminino , Humanos , Linfangiectasia/diagnóstico , Masculino , Pessoa de Meia-Idade , Adulto Jovem
5.
Skinmed ; 15(4): 311-313, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28859749

RESUMO

A 64-year-old white woman was originally diagnosed with histiocytic lymphoma in 1977. She had bilateral lymph node biopsies of the groin, chemotherapy, and radiation therapy after her diagnosis had been confirmed pathologically. She was treated with prednisone and vincristine.


Assuntos
Linfangiectasia/etiologia , Linfangioma/cirurgia , Neoplasias Vulvares/cirurgia , Vulvectomia/efeitos adversos , Feminino , Humanos , Pessoa de Meia-Idade , Remissão Espontânea , Vulvectomia/métodos
6.
J Perinat Med ; 45(9): 1023-1030, 2017 Dec 20.
Artigo em Inglês | MEDLINE | ID: mdl-28258976

RESUMO

Congenital pulmonary lymphangiectasia (CPL) is a rare but fatal disease, usually having an onset from the first few hours to days after birth. Inconsistent nomenclatures were used for CPL in the past decades. Patients often present with intractable respiratory failure, hydrops fetalis and even sudden death. The etiologies of CPL remain unclear. Previous hypotheses suggested that CPL might be caused by conditions preventing normal regression of the lymphatics after the 18th-20th week of gestation. Up-to-date biological studies on lymphatic development, lymphatic valve formation and occurrence of hydrops fetalis revealed possible causative relations with mutations of genes of the vascular endothelial growth factor receptor (VEGFR), RAS/MAPK, PI3K/AKT and NF-κB signaling pathways. Lung biopsy with subsequent histological and immunohistochemical studies is a gold standard of CPL diagnosis. Apart from symptomatic and supportive treatments, novel regimens including sirolimus, a mammalian target of rapamycin (mTOR) inhibitor, one of the inhibitors of the pertinent signaling pathways and ethiodized oil lymphatic embolization under ultrasound-guided intranodal lymphangiography have shown encouraging short-term therapeutic effects for lymphatic anomalies. Surgical operations (lobectomy or pneumonectomy) can be the treatment of choice for patients with CPL confined to one lobe or one lung. Patients with CPL usually have a poor prognosis and often die during the neonatal period. Their prognoses are expected to improve with the development of modern therapeutic agents.


Assuntos
Pneumopatias/congênito , Linfangiectasia/congênito , Humanos , Pulmão/patologia , Pneumopatias/diagnóstico , Pneumopatias/etiologia , Pneumopatias/patologia , Pneumopatias/terapia , Linfangiectasia/diagnóstico , Linfangiectasia/etiologia , Linfangiectasia/patologia , Linfangiectasia/terapia , Prognóstico
9.
Lymphat Res Biol ; 14(3): 172-80, 2016 09.
Artigo em Inglês | MEDLINE | ID: mdl-27599121

RESUMO

BACKGROUND: Sometimes acquired lymphangiectasia (lymphangioma circumscriptum), the pathological mechanism of which is unknown, accompanies lymphedema. The purpose of this study was to better understand the pathological changes present in acquired lymphangiectasia. METHODS AND RESULTS: We examined the pathological characteristics of acquired lymphangiectasia with lymphedema among patients treated at the University of Tokyo Hospital from March 2008 to December 2015. In total, 16 biopsies from 10 patients were investigated. The average age of the patients was 57.2 years (range 43-69), and all were female with secondary lymphedema. Surgical specimens were fixed in formalin, and tissue sections were stained with hematoxylin-eosin. Additional immunostaining (podoplanin, lymphatic vessel endothelial hyaluronan receptor [LYVE] -1, CD4, CD8, CD20, and CD31) was performed in cases 1-3 and 8-10. Dilation of lymphatic vessels in the papillary dermis was present in all 10 cases. Infiltration of inflammatory cells, most of which were lymphocytes, was also observed in the dermis and the epidermis in all cases, even though there were no clinical signs of inflammation. The infiltrating lymphocytes were mainly CD4+ T cells, and less commonly, CD8+ T cells and CD20+ B cells. The number of three types of lymphocytes was significantly larger in the superficial layer of the dermis than in the deep layer, which may indicate that they oozed out from the dilated lymphatic vessels located in the superficial dermis. CD8+ T cells infiltrated the epidermis in seven of eight specimens. In case 4, coagulated lymphatic fluid inside the lymphatic vessel was observed. Proliferation of collagenous fiber in the dermis and acanthosis were observed. CONCLUSIONS: Lymphatic dilation and proliferation of collagenous fiber in the dermis were seen in cases of acquired lymphangiectasia (lymphangioma circumscriptum). Constant infiltration of lymphocytes in the dermis and the epidermis may have a relation to frequent cellulitis, which is often seen in lymphedema patients.


Assuntos
Derme/patologia , Epiderme/patologia , Extremidade Inferior/patologia , Linfangiectasia/patologia , Linfedema/patologia , Neoplasias Ovarianas/complicações , Linfócitos T/patologia , Neoplasias do Colo do Útero/complicações , Adulto , Idoso , Células Cultivadas , Derme/imunologia , Endotélio Vascular/imunologia , Endotélio Vascular/patologia , Epiderme/imunologia , Feminino , Humanos , Inflamação/imunologia , Inflamação/patologia , Linfangiectasia/etiologia , Linfedema/etiologia , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Linfócitos T/imunologia
11.
J Med Case Rep ; 9: 34, 2015 Feb 09.
Artigo em Inglês | MEDLINE | ID: mdl-25880780

RESUMO

INTRODUCTION: Nephrotic syndrome is considered a rare cause of chylous ascites. Intestinal lymphangiectasia in a background of chylous ascites and without any lymphatic obstruction has been reported in association with yellow nail syndrome, which is a rare clinical occurrence in itself. The existence of chylous ascites, duodenal and splenic lymphangiectasia (without any lymphatic obstruction) and nephrotic syndrome in the form of focal segmental glomerulosclerosis in the same patient makes this case the first of its kind to be reported in the literature. CASE PRESENTATION: Here we report the case of a 54-year-old Asian man who presented with recurrent episodes of anasarca for approximately 25 years. He was subsequently found to have chylous ascites, lymphangiectasia and persistent proteinuria. A renal biopsy revealed focal segmental glomerulosclerosis, not otherwise specified. A lymphangiogram, which was performed with the purpose of addressing the intestinal lymphangiectasia, failed to demonstrate any abnormality of lymphatic channels. He was put on oral steroids with consequent remission of his oedema and proteinuria. CONCLUSIONS: This case highlights the fact that duodenal and splenic lymphangiectasia can exist in a scenario of chylous ascites without any obvious obstruction of lymphatic channels and in the absence of yellow nail syndrome. This case also signifies that chylous ascites may be a rare presenting feature of nephrotic syndrome and hence this aspect should be considered while in diagnostic dilemma regarding such a clinical presentation.


Assuntos
Ascite Quilosa/etiologia , Glomerulosclerose Segmentar e Focal/complicações , Linfangiectasia/etiologia , Ascite Quilosa/diagnóstico , Humanos , Masculino , Pessoa de Meia-Idade , Síndrome Nefrótica/complicações , Proteinúria/urina , Tomografia Computadorizada por Raios X
13.
World J Gastroenterol ; 20(25): 8320-4, 2014 Jul 07.
Artigo em Inglês | MEDLINE | ID: mdl-25009412

RESUMO

Lymphangioma is an uncommon benign tumor that develops in the lymphatic system. Abdominal lymphangiomatosis is extremely rare in adult patients, and the clinical symptoms of this condition are complicated and atypical. We report a case of abdominal lymphangiomatosis in a 38-year-old female who presented with intestinal bleeding and protein-losing enteropathy, as well as lesions in the lung and bones. A computed tomography scan revealed multiple small cystic lesions without enhancement. Histological examination revealed microscopic cysts were submucosal, with walls composed of thin fibrous tissue, and D2-40 stained highlight the lining of the lymphatic channels by immunohistochemical method. We make a comparison with the cases reported before, and also discuss the diagnose of diffuse pulmonary lymphangiomatosis and Gorham's disease.


Assuntos
Neoplasias Abdominais/diagnóstico , Linfangioma/diagnóstico , Neoplasias Abdominais/complicações , Neoplasias Abdominais/patologia , Adulto , Biópsia , Endoscopia Gastrointestinal , Feminino , Hemorragia Gastrointestinal/diagnóstico , Hemorragia Gastrointestinal/etiologia , Humanos , Imuno-Histoquímica , Pneumopatias/congênito , Pneumopatias/diagnóstico , Pneumopatias/etiologia , Linfangiectasia/congênito , Linfangiectasia/diagnóstico , Linfangiectasia/etiologia , Linfangioma/etiologia , Linfangioma/patologia , Osteólise Essencial/diagnóstico , Osteólise Essencial/etiologia , Enteropatias Perdedoras de Proteínas/diagnóstico , Enteropatias Perdedoras de Proteínas/etiologia , Tomografia Computadorizada por Raios X
14.
Acta Clin Belg ; 69(4): 294-5, 2014 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-24846179

RESUMO

OBJECTIVE AND IMPORTANCE: Physicians are likely to encounter patients with penis disorders and can be caught off guard by these uncommon pathologies, especially because they occur in a sensitive anatomical location. CLINICAL PRESENTATION: Here, we report the case of a patient presenting with benign transient lymphangiectasis of the penis (BTLP), including its differential diagnosis and treatment. Conclusion headings: BTLP is not an uncommon pathology and diagnosis is based only on medical history and clinical examination. The differentiation between Mondor's disease and BTLP is not necessary for treatment.


Assuntos
Linfangiectasia/diagnóstico , Doenças do Pênis/diagnóstico , Diagnóstico Diferencial , Humanos , Linfangiectasia/etiologia , Linfangiectasia/terapia , Masculino , Pessoa de Meia-Idade , Doenças do Pênis/etiologia , Doenças do Pênis/terapia
17.
Wien Med Wochenschr ; 163(7-8): 155-61, 2013 Apr.
Artigo em Alemão | MEDLINE | ID: mdl-23595137

RESUMO

Lymphedema is a chronic disease associated with a congenital or acquired disorder of the lymphatic vessels or lymph nodes. Untreated lymphedema can lead to complications and disability. Clinical Lymphology deals not only with lymphedema of the extremities but also of the head, the genitals and the internal organs (lymphostatic enteropathy, chylaskos, chylothorax, chylopericard etc). Symptoms of this disorder are often misdiagnosed or not recognized. Ignorance and trivialization of lymphedema causes insufficient treatment, which then is not carried out to the extent as it is possible today by scientific findings. Even today delayed or not optimal treatment causes a long ordeal for many patients.The fact that lymphedema for those affected is a major psychological and social burden, which is limiting the quality of life, has also often been unregarded. The knowledge of anatomy, physiology and pathophysiology as well as the knowledge of causes are necessary for diagnosis, so that early treatment can be initiated.


Assuntos
Linfedema/patologia , Linfedema/fisiopatologia , Angiodisplasia/classificação , Angiodisplasia/diagnóstico , Angiodisplasia/etiologia , Angiodisplasia/patologia , Angiodisplasia/fisiopatologia , Diagnóstico Diferencial , Humanos , Linfa/fisiologia , Linfangiectasia/classificação , Linfangiectasia/diagnóstico , Linfangiectasia/etiologia , Linfangiectasia/patologia , Linfangiectasia/fisiopatologia , Linfangioma/classificação , Linfangioma/diagnóstico , Linfangioma/etiologia , Linfangioma/patologia , Linfangioma/fisiopatologia , Sistema Linfático/anormalidades , Sistema Linfático/patologia , Sistema Linfático/fisiopatologia , Linfedema/classificação , Linfedema/diagnóstico , Linfedema/etiologia
18.
J Assoc Physicians India ; 61(7): 487-9, 2013 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-24772754

RESUMO

28 yr female presented with (grade III) chyluria, with nephrotic range proteinuria and Ig M mesangial deposition in immunofluorescence, secondary to filariasis which was confirmed by serology and microfilaria in glomerulus,and successfully treated by Renal Pelvic instillation sclerotherapy with 0.2% povidine and medical treatment (Diethylcarbamazine). She was asymptomatic with follow up period of 19 months.


Assuntos
Quilo , Filariose Linfática/complicações , Síndrome Nefrótica/etiologia , Adulto , Filariose Linfática/diagnóstico , Filariose Linfática/urina , Feminino , Humanos , Linfangiectasia/diagnóstico por imagem , Linfangiectasia/etiologia , Síndrome Nefrótica/patologia , Proteinúria/etiologia , Espaço Retroperitoneal , Tomografia Computadorizada por Raios X , Urina
19.
PLoS One ; 7(9): e45261, 2012.
Artigo em Inglês | MEDLINE | ID: mdl-23028890

RESUMO

Adrenomedullin (AM) and its receptor complexes, calcitonin receptor-like receptor (Calcrl) and receptor activity modifying protein 2/3, are highly expressed in lymphatic endothelial cells and are required for embryonic lymphatic development. To determine the role of Calcrl in adulthood, we used an inducible Cre-loxP system to temporally and ubiquitously delete Calcrl in adult mice. Following tamoxifen injection, Calcrl(fl/fl)/CAGGCre-ER™ mice rapidly developed corneal edema and inflammation that was preceded by and persistently associated with dilated corneoscleral lymphatics. Lacteals and submucosal lymphatic capillaries of the intestine were also dilated, while mesenteric collecting lymphatics failed to properly transport chyle after an acute Western Diet, culminating in chronic failure of Calcrl(fl/fl)/CAGGCre-ER™ mice to gain weight. Dermal lymphatic capillaries were also dilated and chronic edema challenge confirmed significant and prolonged dermal lymphatic insufficiency. In vivo and in vitro imaging of lymphatics with either genetic or pharmacologic inhibition of AM signaling revealed markedly disorganized lymphatic junctional proteins ZO-1 and VE-cadherin. The maintenance of AM signaling during adulthood is required for preserving normal lymphatic permeability and function. Collectively, these studies reveal a spectrum of lymphatic defects in adult Calcrl(fl/fl)/CAGGCre-ER™ mice that closely recapitulate the clinical symptoms of patients with corneal, intestinal and peripheral lymphangiectasia.


Assuntos
Proteína Semelhante a Receptor de Calcitonina/genética , Edema/genética , Intestinos/patologia , Limbo da Córnea/patologia , Linfangiectasia/genética , Vasos Linfáticos/patologia , Pele/patologia , Adrenomedulina/genética , Adrenomedulina/metabolismo , Animais , Antígenos CD/genética , Antígenos CD/metabolismo , Caderinas/genética , Caderinas/metabolismo , Proteína Semelhante a Receptor de Calcitonina/deficiência , Edema/etiologia , Edema/metabolismo , Edema/patologia , Células Endoteliais/efeitos dos fármacos , Células Endoteliais/metabolismo , Células Endoteliais/patologia , Feminino , Deleção de Genes , Expressão Gênica , Vetores Genéticos , Mucosa Intestinal/metabolismo , Intestinos/efeitos dos fármacos , Limbo da Córnea/efeitos dos fármacos , Limbo da Córnea/metabolismo , Linfangiectasia/etiologia , Linfangiectasia/metabolismo , Linfangiectasia/patologia , Vasos Linfáticos/efeitos dos fármacos , Vasos Linfáticos/metabolismo , Masculino , Camundongos , Camundongos Transgênicos , Transdução de Sinais , Pele/efeitos dos fármacos , Pele/metabolismo , Tamoxifeno/efeitos adversos , Proteína da Zônula de Oclusão-1/genética , Proteína da Zônula de Oclusão-1/metabolismo
20.
Bull Cancer ; 99(5): 599-604, 2012 May.
Artigo em Francês | MEDLINE | ID: mdl-22504738

RESUMO

Gorham's disease is a rare condition of unknown etiology, which is characterized by intra-osseous vascular abnormalities leading to osteolysis and sometimes, extension to adjacent tissues. Although there is no histological evidence of malignancy, the lesions display an aggressive potential responsible of osteolysis with pathological fractures and respiratory failure. The extension of the disease varies from one location to multiple bone lesions throughout the skeleton and the clinical presentations depend on the sites of involvement. In the ISSVA (International Society for the Study of Vascular Abnormalities) classification, its affiliation to the group of vascular tumors or vascular malformations is still debated. To date, there is no standard treatment defined for this disease. The proposed treatments are inhibitors of bone resorption (bisphosphonates) and interferon alpha therapy or anti-angiogenic molecules. Radiation therapy and surgery may also have a place in the therapeutic strategy.


Assuntos
Osteólise Essencial , Doenças Raras , Quilotórax/etiologia , Humanos , Linfangiectasia/etiologia , Osteólise Essencial/complicações , Osteólise Essencial/patologia , Osteólise Essencial/terapia , Prognóstico , Doenças Raras/complicações , Doenças Raras/patologia , Doenças Raras/terapia
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