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3.
BMJ Case Rep ; 12(4)2019 Apr 23.
Artigo em Inglês | MEDLINE | ID: mdl-31015245

RESUMO

General lymphatic anomaly (GLA) is a very rare disorder, characterised by multifocal lymphatic malformations into various tissues that is due to congenital abnormalities of lymphatic development. No treatment has ever proved its efficiency.We report a 22-year-old man with recurrent bronchial casts due to thoracic involvement of GLA. After a 6-month treatment with sildenafil (20 mg three times a day), a phosphodiesterase 5 inhibitor, chest CT scan showed a complete regression of ground-glass opacities and lung function test results improved substantially and remained stable for 1 year. The treatment was well tolerated.This observation suggests that sildenafil may be a therapeutic approach to be tested in thoracic involvement of GLA.


Assuntos
Pneumopatias/congênito , Pneumopatias/tratamento farmacológico , Linfangiectasia/congênito , Linfangioleiomiomatose/tratamento farmacológico , Citrato de Sildenafila/uso terapêutico , Humanos , Pneumopatias/diagnóstico por imagem , Pneumopatias/patologia , Linfangiectasia/diagnóstico por imagem , Linfangiectasia/tratamento farmacológico , Linfangiectasia/patologia , Linfangioleiomiomatose/congênito , Linfangioleiomiomatose/diagnóstico por imagem , Anormalidades Linfáticas/diagnóstico por imagem , Masculino , Inibidores da Fosfodiesterase 5/uso terapêutico , Doenças Raras , Testes de Função Respiratória/métodos , Citrato de Sildenafila/administração & dosagem , Tomografia Computadorizada por Raios X/métodos , Resultado do Tratamento , Adulto Jovem
4.
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
5.
Skeletal Radiol ; 47(9): 1293-1297, 2018 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-29468291

RESUMO

Lymphangiomatosis is an uncommon disease process characterized by multisystem lymphatic malformations that can involve numerous body systems, including organs, muscles, soft tissues, and bones. Involvement of the nervous system is rare and has even been previously described as a site of sparing. We present a case of a 24-year-old female with known lymphangiomatosis, presenting with acute onset of lower extremity paresthesias, weakness, and new urinary retention. MRI of the pelvis revealed lymphangiomatosis of the sacral plexus, which has not been previously reported. We will review the clinical and imaging manifestations of lymphangiomatosis and provide a differential diagnosis for masses of the lumbosacral plexus. Although lower extremity pain and weakness encountered in the emergency department or outpatient setting is most frequently caused by lumbar spine pathology, occasionally, abnormalities of the lumbosacral plexus may prove to be the cause. While peripheral nerve sheath tumors lead the differential diagnosis of tumor or tumor-like entities involving the lumbosacral plexus, lymphangiomatosis is a rare differential consideration.


Assuntos
Plexo Lombossacral/diagnóstico por imagem , Linfangiectasia/diagnóstico por imagem , Diagnóstico Diferencial , Feminino , Humanos , Perna (Membro) , Vértebras Lombares , Linfangiectasia/complicações , Imagem por Ressonância Magnética , Debilidade Muscular/etiologia , Parestesia/etiologia , Reflexo Anormal , Adulto Jovem
6.
BMC Ophthalmol ; 17(1): 194, 2017 Oct 23.
Artigo em Inglês | MEDLINE | ID: mdl-29061139

RESUMO

BACKGROUND: Conjunctival lymphangiectasia is a rare condition presumably caused by the obstruction of lymphatic channels or by an abnormal connection between conjunctival lymphatic and blood vessels. Diagnosis is based on clinical appearance and histology. We report a case of conjunctival lymphangiectasia in which anterior segment optical coherence tomography (OCT) was used to assist the diagnosis and the planning of the biopsy location. CASE PRESENTATION: A 31-year-old woman was referred with repeated episodes of conjunctival "hemorrhages" and chemosis with extended recovery periods over the last months. Other symptoms were dryness, redness, burning sensation and itching. Photo documentation, anterior segment OCT, ultrasound, computer tomography (CT) and magnetic resonance imaging (MRI) of the brain were performed. MRI revealed dilated atypical Virchow-Robin space (VRS). Conjunctival biopsy was taken and the location of the biopsy was selected based on OCT findings. Based on the clinical appearance we suspected the case to be conjunctival lymphangiectasia or lymphangioma. Histology and immunhistochemistry confirmed the diagnosis of conjunctival lymphangiectasia. CONCLUSIONS: Anterior segment OCT is a non-invasive tool, useful in the evaluation of conjunctival lesions and planning surgery.


Assuntos
Doenças da Túnica Conjuntiva/diagnóstico por imagem , Linfangiectasia/diagnóstico por imagem , Tomografia de Coerência Óptica/métodos , Adulto , Hemorragia Ocular/diagnóstico por imagem , Feminino , Humanos
7.
Ren Fail ; 39(1): 533-539, 2017 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-28639460

RESUMO

BACKGROUND: Renal lymphangiectasia is rarely reported benign renal disorder of lymphatic malformation. Though found incidentally; it presents with nonspecific symptoms and shows characteristic findings in radiological imaging studies. AIM: Here, we report eight patients with symptoms, laboratory and imaging findings compatible with renal lymphangiectasia. This report describes clinical and laboratory characteristics, treatment, Imaging findings and outcome of a series of patients with renal lymphangiectasia and reviews the literature. METHODS AND MATERIAL: Eight patients (mean age 45 years, male:female ratio 3:1) from 1st January 2011 to 30th June 2016; showing renal lymphangiectasia as incidental finding on CT IVP were included in the series. Imaging and laboratory findings were reviewed. Two out of eight patients (25%) underwent aspiration of collection and laboratory findings confirmed the diagnosis of renal lymphangiectasia. Four out of eight patients (50%) did not undergo aspiration of fluid and were offered conservative treatment. Two out of eight patients (25%) were donors for renal transplantation who were managed conservatively. RESULTS: Renal lymphangiectasia was diagnosed on CT IVP. In each case, where aspiration of collection fluid was offered, the laboratory diagnosis of renal lymphangiectasia was confirmed and patients were managed conservatively. However, large collection in one patient was relieved by percutaneous aspiration. CONCLUSIONS: Renal lymphangiectasia can be diagnosed with CT scan and confirmed by laboratory tests. As it may be confused with other cystic lesions of kidney; proper diagnosis and exclusion of other differentials can be effectively offered by CT scan IVP, which can avoid unnecessary invasive treatment options.


Assuntos
Nefropatias/diagnóstico por imagem , Linfangiectasia/diagnóstico por imagem , Adulto , Idoso , Meios de Contraste/administração & dosagem , Feminino , Humanos , Iohexol/administração & dosagem , Masculino , Pessoa de Meia-Idade , Tomografia Computadorizada por Raios X/métodos , Ultrassonografia
8.
Pediatr Radiol ; 47(11): 1441-1451, 2017 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-28631156

RESUMO

BACKGROUND: Secondary pulmonary lymphangiectasia is a complication of congenital heart disease that results from chronic pulmonary venous obstruction. OBJECTIVES: We aimed to evaluate the performance of chest ultrasound (US) in diagnosing secondary pulmonary lymphangiectasia and to review the clinical course of children with secondary pulmonary lymphangiectasia. MATERIALS AND METHODS: Chest US was performed on 26 children with hypoplastic left heart syndrome, total anomalous pulmonary venous connection or cor triatriatum in a prospective observational study. Thirteen children had pulmonary venous obstruction (62% male; median age: 17 days old, range: 1-430 days old) and 13 children did not have obstruction (62% male; median age: 72 days old, range: 4-333 days old). US features of secondary pulmonary lymphangiectasia were documented and diagnostic performance was determined. Clinical course of patients with secondary pulmonary lymphangiectasia was reviewed. RESULTS: Eleven of 13 (84.6%) patients in the obstructed group had a clinical and/or biopsy diagnosis of secondary pulmonary lymphangiectasia. Statistically significant chest US criteria for diagnosis were presence of irregular lung surface (likelihood ratio [LR] 6.8, 95% confidence interval [CI] 1.9-25.1), subpleural cystic appearing structures (LR 3.6, 95% CI 1.2-10.7), and combination of subpleural cystic appearing structures and surface irregularity together (LR 10.9, 95% CI 1.6-75.0). Seven of 11 (63.6%) patients with secondary pulmonary lymphangiectasia died during follow-up, the majority due to cardiopulmonary failure or complications. CONCLUSION: Chest US is an accurate and reproducible bedside method for diagnosing secondary pulmonary lymphangiectasia in patients with pulmonary venous obstruction. These patients may have worse prognoses.


Assuntos
Cardiopatias Congênitas/complicações , Pneumopatias/congênito , Linfangiectasia/congênito , Veias Pulmonares/anormalidades , Ultrassonografia/métodos , Feminino , Cardiopatias Congênitas/cirurgia , Humanos , Lactente , Recém-Nascido , Pneumopatias/diagnóstico por imagem , Pneumopatias/cirurgia , Linfangiectasia/diagnóstico por imagem , Linfangiectasia/cirurgia , Masculino , Prognóstico , Estudos Prospectivos , Sensibilidade e Especificidade
9.
AJR Am J Roentgenol ; 208(2): 300-305, 2017 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-27845836

RESUMO

OBJECTIVE: The aim of this study was to analyze the findings of MDCT performed after direct lymphangiography in patients with diffuse pulmonary lymphangiomatosis. MATERIALS AND METHODS: Twenty-three patients (13 male and 10 female patients) diagnosed with diffuse pulmonary lymphangiomatosis on the basis of clinical features and findings from imaging, bronchoscopy, and pathologic analysis were retrospectively evaluated. All patients underwent pulmonary MDCT after direct lymphangiography, surgical operation or open lung biopsy, and histopathologic examination. MDCT images were analyzed by two experienced radiologists independently. RESULTS: MDCT after direct lymphangiography revealed numerous intrathoracic abnormalities, including abnormal distribution of contrast medium, dilatation of lymphatic channels, mediastinal soft-tissue infiltration, and peribronchovascular thickening. Nineteen patients had interlobular septal thickening, 18 had diffuse ground-glass opacities, 22 had pleural effusion, 14 had extrapleural soft-tissue thickening, 20 had pericardial effusion and thickened pericardium, 10 had multiple lymphadenopathy in mediastinum, and five had mediastinal, pericardial, and thoracic aeroceles. CONCLUSION: MDCT performed after direct lymphangiography is well suited to clarify the characteristics of intrathoracic disorders and is an excellent tool in the diagnosis of diffuse pulmonary lymphangiomatosis.


Assuntos
Pneumopatias/congênito , Linfangiectasia/congênito , Linfografia/métodos , Tomografia Computadorizada Multidetectores/métodos , Imagem Multimodal/métodos , Intensificação de Imagem Radiográfica/métodos , Adolescente , Adulto , Criança , Pré-Escolar , Diagnóstico Diferencial , Feminino , Seguimentos , Humanos , Lactente , Pneumopatias/diagnóstico por imagem , Linfangiectasia/diagnóstico por imagem , Masculino , Reprodutibilidade dos Testes , Sensibilidade e Especificidade , Adulto Jovem
11.
Tech Vasc Interv Radiol ; 19(4): 299-304, 2016 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-27993326

RESUMO

Pulmonary lymphatic diseases have been recognized for many years and have been referred as pulmonary lymphangiectasia, pulmonary lymphangiomatosis, plastic bronchitis, and idiopathic chylothorax or chylopericardium. The lymphatic etiology of these conditions has been determined by detection of cystic lymphatic structures on biopsy or postmortem examination. Development of new imaging techniques such as dynamic contrast-enhanced magnetic resonance lymphangiography has allowed better understanding of pathophysiology of these conditions. Dynamic contrast-enhanced magnetic resonance lymphangiography demonstrated that the common denominator of these disorders is an abnormal pulmonary lymphatic flow from the thoracic duct toward pulmonary parenchyma. This abnormal lymphatic flow propagates into mediastinum, lung parenchyma, pleural surfaces, and bronchial submucosa and has been termed as pulmonary lymphatic perfusion syndrome (PLPS). Known clinical presentation of PLPS includes spontaneous chylothorax or pericardium, neonatal chylous effusions, and plastic bronchitis. PLPS has been observed in all age groups and can be considered as a congenital anatomical lymphatic variant. The onset of the clinical symptoms can be provoked by increase of the lymphatic flow owing to elevated central venous pressure that results in lymphatic distention, trauma, and severe upper respiratory infection. Reported treatment of PLPS is obliteration of these abnormal lymphatic pathways by percutaneous embolization, a technique similar to thoracic duct embolization in chylothorax.


Assuntos
Bronquite/terapia , Quilotórax/terapia , Embolização Terapêutica/métodos , Pneumopatias/congênito , Linfangiectasia/congênito , Anormalidades Linfáticas/terapia , Sistema Linfático , Derrame Pericárdico/terapia , Radiografia Intervencionista/métodos , Bronquite/diagnóstico por imagem , Bronquite/fisiopatologia , Quilotórax/diagnóstico por imagem , Quilotórax/fisiopatologia , Meios de Contraste/administração & dosagem , Embolização Terapêutica/efeitos adversos , Humanos , Recém-Nascido , Pneumopatias/diagnóstico por imagem , Pneumopatias/fisiopatologia , Pneumopatias/terapia , Linfangiectasia/diagnóstico por imagem , Linfangiectasia/fisiopatologia , Linfangiectasia/terapia , Anormalidades Linfáticas/diagnóstico por imagem , Anormalidades Linfáticas/fisiopatologia , Sistema Linfático/diagnóstico por imagem , Sistema Linfático/fisiopatologia , Linfografia/métodos , Imagem por Ressonância Magnética , Derrame Pericárdico/diagnóstico por imagem , Derrame Pericárdico/fisiopatologia , Valor Preditivo dos Testes , Radiografia Intervencionista/efeitos adversos , Resultado do Tratamento
12.
Pediatr Emerg Care ; 32(12): 888-891, 2016 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-27898632

RESUMO

Kaposiform lymphangiomatosis is a generalized lymphatic disorder complicated by consumptive coagulopathy and pericardial and pleural effusions. We present the case of a 13-year-old female adolescent given a diagnosis of a large pleural effusion by point-of-care thoracic ultrasonography, which led to further evaluation and diagnosis of this rare disorder. We review the use of point-of-care thoracic ultrasonography for the diagnosis of pleural effusion.


Assuntos
Pneumopatias/congênito , Linfangiectasia/congênito , Derrame Pleural/diagnóstico por imagem , Sistemas Automatizados de Assistência Junto ao Leito , Ultrassonografia/métodos , Adolescente , Gerenciamento Clínico , Feminino , Humanos , Pneumopatias/diagnóstico por imagem , Linfangiectasia/diagnóstico por imagem , Derrame Pleural/etiologia
13.
Zhonghua Er Ke Za Zhi ; 54(5): 360-4, 2016 May.
Artigo em Chinês | MEDLINE | ID: mdl-27143078

RESUMO

OBJECTIVE: To analyze the clinical characteristics and diagnosis of 2 cases with diffuse pulmonary lymphatic disease. METHOD: Clinical manifestations of the children were retrospectively analyzed. Two patients were both from Beijing Children Hospital in 2013 and 2014.Diffuse pulmonary lymphatic disease was diagnosed by pathology of pleura in case 1 and by lymphoscintigraphy in case 2. RESULT: The first patient was a male aged 10-year-5-month who presented with a history of pleural effusion for 2 years. Examination revealed chylothorax and pericardial effusion. After pleural decortications and ligation of thoracic duct was performed, he still had recurrent chylothorax. Pathology of pleura revealed lymphangioma. Physical examination showed diminished breath sound and heart sounds. A bronchoscope revealed too much white viscous secretions. Thorax ultrasound revealed lymphangioma in bilateral lower thoracic cavity and post inferior mediastinum. Chest computed tomography showed diffuse thickening of the interlobular septa and bronchovascular bundle in both lungs, many punctate calcification in the lower field. Abdomen ultrasound revealed a small quantity seroperitoneum. Lymphoscintigraphy revealed radioactivity enhancement in bilateral thoracic cavity. The second patient was a female aged 6 years and 9 months, who presented with a history of recurrent cough for 2 years and 9 months. Physical examination showed normal result. Pulmonary function showed mixed ventilation function disturbance. A bronchoscopy showed extensive viscous secretions. Mediastinum, heart and abdomen were normal on ultrasound. Chest computed tomography showed diffuse thickening of the interlobular septa and bronchovascular bundle in both lungs and bilateral pleural thickening. Lymphoscintigraphy revealed diffuse lymphangiectasis in both lungs. Both patients received a diagnosis of diffuse pulmonary lymphatic disease. Case 1 who had died was diagnosed with diffuse pulmonary lymphangiomatosis. Case 2 had no exacerbation after 9 months' treatment with prednisone. CONCLUSION: Diffuse pulmonary lymphatic disease might present with cough and shortness of breath and result in diffuse interstitial disease with thickening of the interlobular septa, refractory chylothorax, multiple lymphangioma in mediastinum, pericardial effusion and seroperitoneum. It could be diagnosed by pleura or lung tissue pathology and lymphoscintigraphy.


Assuntos
Pneumopatias/congênito , Linfangiectasia/congênito , Linfangioma/diagnóstico por imagem , Broncoscopia , Criança , Quilotórax/diagnóstico por imagem , Tosse , Feminino , Humanos , Pulmão , Pneumopatias/diagnóstico por imagem , Linfangiectasia/diagnóstico por imagem , Linfocintigrafia , Masculino , Mediastino/patologia , Derrame Pericárdico/diagnóstico por imagem , Derrame Pleural/diagnóstico por imagem , Estudos Retrospectivos , Tórax/patologia , Tomografia Computadorizada por Raios X
14.
Int J Dermatol ; 55(9): e482-7, 2016 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-26967121

RESUMO

BACKGROUND: Acquired lymphangiectasia of the vulva (ALV) is a rare condition thought to be secondary to pelvic lymphatic obstruction. Although benign, this entity often occurs after previous malignancy and can be confused with conditions such as genital warts. We sought to clarify the clinicopathologic features of ALV by studying affected patients from our institution and from the existing literature. METHODS: We reviewed retrospectively the clinical and histopathologic characteristics of patients with ALV from our institution and performed an extensive literature search to identify all published cases of ALV. RESULTS: Eleven patients with ALV were identified from our institution. Three patients had Crohn disease, three had malignancies involving the pelvic region (rectal adenocarcinoma, cervical carcinoma, endometrial carcinoma), and three (two with pelvic malignancy) had previous radiation therapy. One patient had Proteus syndrome. Of 67 distinct cases of ALV identified in the literature, 41 (61%) had a history of malignancy. CONCLUSIONS: In any acquired or clinically unusual lymphatic process in the skin and soft tissues, a lesion or obstructive process of the draining lymphatics should be investigated.


Assuntos
Linfangiectasia/complicações , Linfangiectasia/diagnóstico por imagem , Neoplasias/complicações , Doenças da Vulva/complicações , Doenças da Vulva/diagnóstico por imagem , Adolescente , Adulto , Idoso , Doença de Crohn/complicações , Feminino , Humanos , Linfangiectasia/patologia , Imagem por Ressonância Magnética , Microscopia , Pessoa de Meia-Idade , Neoplasias/radioterapia , Tomografia Computadorizada por Raios X , Doenças da Vulva/patologia , Adulto Jovem
15.
Chest ; 149(1): e25-8, 2016 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-26757302

RESUMO

A 42-year-old white woman presented to the pulmonary clinic for evaluation of abnormal chest imaging. Twenty years prior to presentation, she was noted to have an abnormal chest radiograph during a routine preemployment evaluation. A subsequent bronchoscopy was nondiagnostic. She was followed up with annual imaging, which demonstrated little or no progression of her disease. She remained symptom free throughout this period. A year before her visit to the pulmonary clinic, she developed abdominal discomfort and was found to have ascites. Subsequently, she underwent three paracenteses with analysis revealing chylous fluid. She was a nonsmoker without a history of exposures or travel.


Assuntos
Ascite Quilosa/diagnóstico por imagem , Pneumopatias/diagnóstico por imagem , Linfangiectasia/diagnóstico por imagem , Adulto , Ascite Quilosa/complicações , Ascite Quilosa/terapia , Feminino , Humanos , Pneumopatias/complicações , Pneumopatias/terapia , Linfangiectasia/complicações , Linfangiectasia/terapia , Tomografia Computadorizada por Raios X
17.
Pediatr Radiol ; 46(4): 483-9, 2016 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-26691156

RESUMO

BACKGROUND: Hypoplastic left heart syndrome (HLHS) is the third most common cause of critical congenital heart disease in newborns, and one of the most challenging forms to treat. Secondary pulmonary lymphangiectasia has been recognized in association with HLHS, an appearance described on fetal MRI as the "nutmeg lung." OBJECTIVE: To investigate the association of fetal nutmeg lung with HLHS survival. MATERIALS AND METHODS: A retrospective search of the fetal MRI database was performed. The nutmeg lung pattern was defined as T2 heterogeneous signal with tubular structures radiating peripherally from the hila. Postnatal echocardiograms and charts were reviewed. RESULTS: Forty-four fetal MR studies met inclusion criteria, of which 4 patients (9%) had the nutmeg lung pattern and 3 of whom also had restrictive lesions. Mortality in this nutmeg lung group was 100% by 5 months of age. Of the 40 patients without nutmeg lung, mortality/orthotopic heart transplant (OHT) was 35%. Of these 40 patients without nutmeg lung, 5 had restriction on echo, 3 of whom died/had OHT before 5 months of age (60% of patients with restriction and non-nutmeg lung). There was a significantly higher incidence of restrictive lesions (P = 0.02) and mortality/OHT (P = 0.02) in patients with nutmeg lung compared to those without. CONCLUSION: The nutmeg lung MR appearance in HLHS fetuses is associated with increased mortality/OHT (100% in the first 5 months of life compared to 35% with HLHS alone). Not all patients with restrictive lesions develop nutmeg lung, and outcome is not as poor when restriction is present in isolation. Dedicated evaluation for nutmeg lung pattern on fetal MR studies may be useful to guide prognostication and aid clinicians in counseling parents of fetuses with HLHS.


Assuntos
Síndrome do Coração Esquerdo Hipoplásico/diagnóstico por imagem , Síndrome do Coração Esquerdo Hipoplásico/mortalidade , Pneumopatias/congênito , Linfangiectasia/congênito , Diagnóstico Pré-Natal/estatística & dados numéricos , Causalidade , Pré-Escolar , Comorbidade , Feminino , Mortalidade Fetal , Humanos , Síndrome do Coração Esquerdo Hipoplásico/embriologia , Lactente , Recém-Nascido , Pulmão/diagnóstico por imagem , Pulmão/embriologia , Pneumopatias/diagnóstico por imagem , Pneumopatias/mortalidade , Linfangiectasia/diagnóstico por imagem , Linfangiectasia/mortalidade , Imagem por Ressonância Magnética/estatística & dados numéricos , Masculino , Pennsylvania/epidemiologia , Prognóstico , Fatores de Risco , Taxa de Sobrevida
18.
Ann Dermatol Venereol ; 141(11): 663-70, 2014 Nov.
Artigo em Francês | MEDLINE | ID: mdl-25442470

RESUMO

BACKGROUND: Primary lymphoedema (LE) is the consequence of lymphatic insufficiency, usually associated with hypoplasia of the lymph vessels and/or nodes. Lymphoscintigraphy allows early diagnosis. OBJECTIVES: To assess the frequency of contralateral LE by lymphoscintigraphy in children with unilateral primary LE of one limb; to assess facets of clinical bilateralization of LE. METHODS: This retrospective single-centre study included children with unilateral LE followed up at the Tours university hospital centre between 2004 and 2014. Parents were contacted by phone to obtain follow-up data. RESULTS: Of the 13 children included, 6 (46%) displayed bilateral lymphatic anomalies on lymphoscintigraphy. Within a median follow-up period of 6 years, clinical bilateralization was observed in one girl after progression of her LE for 7 years; in her case, bilateral lymphatic insufficiency was detected at the initial lymphoscintigraphy. CONCLUSION: While contralateral subclinical lymphatic insufficiency is frequent in children with unilateral primary LE of the limbs, clinical bilateralization appears only rarely.


Assuntos
Linfedema/diagnóstico por imagem , Linfocintigrafia/métodos , Adolescente , Criança , Pré-Escolar , Diagnóstico Tardio , Progressão da Doença , Feminino , Seguimentos , Humanos , Lactente , Extremidade Inferior/diagnóstico por imagem , Linfangiectasia/diagnóstico por imagem , Vasos Linfáticos/anormalidades , Vasos Linfáticos/diagnóstico por imagem , Masculino , Estudos Retrospectivos , Extremidade Superior/diagnóstico por imagem
19.
J Perinatol ; 34(9): 720-2, 2014 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-25179384

RESUMO

Historically, congenital pulmonary lymphangiectasia (CPL) has had poor treatment outcomes despite aggressive therapy. There are recent reports of ethiodized oil (Lipiodol) lymphangiography successfully treating lymphatic leakage in adults. In this report, we describe two infants with CPL complicated by chylothoraces successfully treated by instillation of ethiodized oil into the lymphatic system. Congenital atresia of the thoracic duct was demonstrated on the lymphangiogram in both patients before treatment. Both patients have shown good short-term outcomes without supplemental oxygen or fat restricted diets at 9 months of age. Ethiodized oil lymphangiography represents a new treatment modality for some patients with CPL.


Assuntos
Meios de Contraste/uso terapêutico , Óleo Etiodado/uso terapêutico , Pneumopatias/congênito , Linfangiectasia/congênito , Linfografia , Embolização Terapêutica , Feminino , Humanos , Lactente , Pneumopatias/diagnóstico por imagem , Pneumopatias/tratamento farmacológico , Linfangiectasia/diagnóstico por imagem , Linfangiectasia/tratamento farmacológico
20.
Lymphology ; 47(1): 28-39, 2014 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-25109167

RESUMO

We performed lymphoscintigraphy on 31 patients (newborns and children) affected by congenital lymphatic dysplasia according to our previously published protocol. Congenital lymphatic dysplasia may present with various degrees of clinical severity, ranging from nonimmune hydrops fetalis with visceral effusions to lymphedema alone. We recommend that lymphoscintigraphy should be strongly considered in all patients with signs of lymphatic dysplasia, including those with minimal and initial signs of lymphatic impairment, in order to obtain a very early diagnosis and to start treatment. Lymphoscintigraphy is safe and useful in the diagnosis of lymphatic dysplasia in the newborn and children. Moreover, it is well tolerated by patients and well accepted by their parents.


Assuntos
Doenças Linfáticas/diagnóstico por imagem , Sistema Linfático/anormalidades , Linfocintigrafia , Criança , Pré-Escolar , Quilotórax/congênito , Quilotórax/diagnóstico por imagem , Ascite Quilosa/diagnóstico por imagem , Humanos , Hidropisia Fetal/diagnóstico por imagem , Lactente , Recém-Nascido , Pneumopatias/congênito , Pneumopatias/diagnóstico por imagem , Linfangiectasia/congênito , Linfangiectasia/diagnóstico por imagem , Linfangiectasia Intestinal/diagnóstico por imagem , Doenças Linfáticas/congênito , Doenças Linfáticas/terapia , Linfedema/diagnóstico por imagem , Derrame Pericárdico/diagnóstico por imagem , Valor Preditivo dos Testes , Prognóstico , Índice de Gravidade de Doença
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