RESUMO
Renal lymphangiectasia (RL) is a rare condition in which lymphatic vessels are dilated giving rise to cyst formation in peripelvic, perirenal and intrarenal locations. Knowledge about RL is limited and based upon individual case reports. This can be genetic or acquired. There is no significant association with any gender or age. It can be manifested as focal or diffuse forms and can be unilateral or bilateral. Most of the cases present with abdominal or flank pain. The diagnosis is based on radiological imaging. Due to rarity of diseases, it has potential to be misdiagnosed as other cystic disease of kidneys. The treatment is mainly conservative but prolonged follow up for associated complications like hypertension and renal vein thrombosis is required. We have presented a case of bilateral renal lymphangiectasia with the review of available literature.
Assuntos
Nefropatias , Linfangiectasia , Humanos , Linfangiectasia/diagnóstico , Linfangiectasia/diagnóstico por imagem , Nefropatias/diagnóstico por imagem , Nefropatias/diagnóstico , Feminino , Masculino , AdultoRESUMO
Despite being rarely discussed, perinephric lymphatics are involved in many pathological and benign processes. The lymphatic system in the kidneys has a harmonious dynamic with ureteral and venous outflow, which can result in pathology when this dynamic is disturbed. Although limited by the small size of lymphatics, multiple established and emerging imaging techniques are available to visualize perinephric lymphatics. Manifestations of perirenal pathology may be in the form of dilation of perirenal lymphatics, as with peripelvic cysts and lymphangiectasia. Lymphatic collections may also occur, either congenital or as a sequela of renal surgery or transplantation. The perirenal lymphatics are also intimately involved in lymphoproliferative disorders, such as lymphoma as well as the malignant spread of disease. Although these pathologic entities often have overlapping imaging features, some have distinguishing characteristics that can suggest the diagnosis when paired with the clinical history.
Assuntos
Nefropatias , Linfangiectasia , Humanos , Rim/patologia , Diagnóstico por Imagem , Sistema Linfático/diagnóstico por imagem , Nefropatias/diagnóstico por imagem , Nefropatias/patologia , Linfangiectasia/diagnóstico , Linfangiectasia/patologiaRESUMO
Conjunctival lymphangiectasia is a rare pathology that represents the enlargement of the lymphatic vessels localized in the conjunctiva. Patients may be asymptomatic or experience symptoms such as foreign body sensation, congestion, irritation, dryness, and blurry vision. There are various methods of therapy for patients with severe and symptomatic conjunctival lymphangiectasia. Surgical excision has the lowest rates of recurrence. We present a case of a 24-year-old woman with conjunctival lymphangiectasia and a history of left lower limb enlargement and bilaterally enlarged submandibular and upper jugular lymph nodes without an identifiable cause, who presented to the ophthalmology clinic accusing ocular discomfort, foreign body sensation and transparent conjunctival cystic lesions in the left eye for the last five months. Abbreviations: OD = right eye, OS = left eye, OCT = optical coherence tomography, VEGF = vascular endothelial growth factor.
Assuntos
Doenças da Túnica Conjuntiva , Corpos Estranhos , Linfangiectasia , Vasos Linfáticos , Doenças Vasculares , Feminino , Humanos , Adulto Jovem , Adulto , Linfangiectasia/diagnóstico , Linfangiectasia/patologia , Linfangiectasia/cirurgia , Fator A de Crescimento do Endotélio Vascular , Doenças da Túnica Conjuntiva/diagnóstico , Doenças da Túnica Conjuntiva/cirurgia , Vasos Linfáticos/patologia , Corpos Estranhos/patologiaRESUMO
BACKGROUND Renal lymphangiectasia is a rare benign disorder with unknown pathophysiology. Renal lymphangiectasia can affect both males and females with no known age predilection. Its diagnosis can be accomplished with radiological images and confirmed by aspiration of lymphatic fluid in certain cases. However, there is no clear presentation to be relied on, apart from incidental findings or presentation of complications, such as hypertension due to mass effect on the kidney or renal failure from chronic compression and obstruction or renal vein thrombosis. Management is directed toward symptomatic relief and protection of the kidneys from failure or obstruction. The timing of possible complications and the duration of conservative therapy are undetermined. CASE REPORT Here, we present a case of a healthy 39-year-old woman with bilateral renal lymphangiectasia. It was initially discovered and confirmed to be lymphangiectasia when she was 13 years old and underwent bilateral renal aspiration. She recently presented to the Emergency Department with abdominal symptoms that were found to be caused by diverticulitis. The radiological images showed the persistence of her previous diagnosis of bilateral renal lymphangiectasia. She has had the same condition for more than 25 years, with no related complications or further intervention beyond conservative management. CONCLUSIONS Renal lymphangiectasia has a benign long-term course with insignificant and minor effects in certain patients regardless of the considerable size of cysts and bilateral involvement of the kidneys. The findings of our case could reassure patients with a diagnosis of a similar condition.
Assuntos
Nefropatias , Linfangiectasia , Adolescente , Adulto , Feminino , Humanos , Rim , Linfangiectasia/diagnóstico , Masculino , Morbidade , Tomografia Computadorizada por Raios XRESUMO
Conjunctival lymphangiectasia, a rare condition characterized by dilated lymphatic channels in the bulbar conjunctiva, generally develops as a consequence of a local lymphatic scarring or distal mechanical outflow obstruction following surgery, radiotherapy, neoplastic disease, or other disease processes. The actual cause often remains unknown. We report a case of a unilateral conjunctival lymphangiectasia in a 7-year-old girl with neurofibromatosis type 1 (NF-1) and discuss a possible association between the two entities.
Assuntos
Doenças da Túnica Conjuntiva , Linfangiectasia , Neurofibromatose 1 , Criança , Túnica Conjuntiva , Doenças da Túnica Conjuntiva/diagnóstico , Doenças da Túnica Conjuntiva/etiologia , Feminino , Humanos , Linfangiectasia/diagnóstico , Linfangiectasia/etiologia , Neurofibromatose 1/complicações , Neurofibromatose 1/diagnósticoRESUMO
BACKGROUND: Intestinal lymphangiectasia is a rare disease. Thus, prospective studies are impossible, and therapy is still controversial. Several medicines are suggested for treatment but there are no existing indications for drug choice and treatment guidelines. We aimed to introduce the action mechanism of each drug and treatment overview in a single-center experience and a review of the literature on second-line therapy for primary intestinal lymphangiectasia. METHOD: Children under 18 years old diagnosed with intestinal lymphangiectasia from June 2000 to June 2020 were included and retrospectively reviewed in the study. Capsule endoscopy, MR lymphangiography, or whole-body MRI for investigating the extent of abnormal lymphatic vessels in addition to endoscopy and biopsy were conducted. The individual treatment approaches depended upon the lymphangiectasis locations involved. RESULTS: Only one patient showed a response to dietary therapy. One patient was successfully cured after two therapeutic lymphatic embolization. Octreotide was tried for two patients who had extensive lymphangiectasis. Lymphangiectasis recurred when octreotide was used for 3 months in one patient, and there was no effect in the other patient. Sirolimus was tried for four patients. Two of them had abnormal lymphatic lesions only in the intestine, and the others had extensive lymphangiectasis. The former group showed clinical improvement after 3-4 months of sirolimus treatment, whereas the latter group showed clinical improvement only after 1 month of sirolimus treatment. CONCLUSION: Surgery or embolization is a potential therapeutic option for patients with focal abnormal lymphatic lesions. Octreotide is not an optimal choice for patients with extensive lymphangiectasis. Sirolimus is an effective and safe drug and can be the first drug of choice for patients with extensive lymphangiectasis.
Assuntos
Linfangiectasia Intestinal , Linfangiectasia , Adolescente , Criança , Humanos , Linfangiectasia/diagnóstico , Linfangiectasia/tratamento farmacológico , Linfangiectasia Intestinal/diagnóstico , Linfangiectasia Intestinal/tratamento farmacológico , Imageamento por Ressonância Magnética , Estudos Prospectivos , Estudos RetrospectivosRESUMO
CASE PRESENTATION: A 35-year-old man was admitted for recurrent episodes of pneumonia. He complained of a 2-month history of exertional dyspnea and productive cough with whitish and viscous sputum which was poorly responsive to antibiotic therapy. He also reported a weight loss of 5 kg since the first symptoms appeared. There was no dysphagia, fever, or chest pain. He currently did not use medication and did not have a relevant medical history except a current 10 pack-year smoking history. He did not report any history of trauma or respiratory exposure to fats.
Assuntos
Quilotórax , Dieta com Restrição de Gorduras/métodos , Pneumopatias/congênito , Pulmão , Linfangiectasia/congênito , Vasos Linfáticos , Pneumonia , Adulto , Biópsia/métodos , Quilotórax/diagnóstico , Quilotórax/etiologia , Diagnóstico Diferencial , Humanos , Pulmão/diagnóstico por imagem , Pulmão/patologia , Pneumopatias/diagnóstico , Pneumopatias/dietoterapia , Pneumopatias/fisiopatologia , Linfangiectasia/diagnóstico , Linfangiectasia/dietoterapia , Linfangiectasia/fisiopatologia , Vasos Linfáticos/anormalidades , Vasos Linfáticos/diagnóstico por imagem , Linfografia/métodos , Imageamento por Ressonância Magnética/métodos , Masculino , Pneumonia/diagnóstico , Pneumonia/etiologia , Recidiva , Testes de Função Respiratória/métodos , Escarro , Tomografia Computadorizada por Raios X/métodosRESUMO
Visceral vascular anomalies are common in patients with vascular malformations in other parts of the body and can include lymphatic, venous, and arteriovenous malformations. Depending on the organ or organs involved they may present differently and pose different treatment challenges. Defining the malformation and understanding its extent is paramount in devising management regimens. Medical, interventional, and surgical therapies are often required in combination to treat these complex lesions. There are new and promising advances in the development of therapeutic agents targeting the PI3K/AKT/mTOR pathway. Due to the complex nature of these lesions a coordinated, multi-disciplinary approach is necessary to manage and mitigate symptoms and complications of this diverse group of vascular malformations.
Assuntos
Malformações Arteriovenosas , Quilotórax , Neoplasias Gastrointestinais , Linfangiectasia , Anormalidades Linfáticas , Nevo Azul , Neoplasias Cutâneas , Malformações Vasculares , Vísceras/patologia , Malformações Arteriovenosas/diagnóstico , Malformações Arteriovenosas/patologia , Malformações Arteriovenosas/terapia , Criança , Quilotórax/diagnóstico , Quilotórax/patologia , Quilotórax/terapia , Neoplasias Gastrointestinais/diagnóstico , Neoplasias Gastrointestinais/patologia , Neoplasias Gastrointestinais/terapia , Humanos , Linfangiectasia/diagnóstico , Linfangiectasia/patologia , Linfangiectasia/terapia , Anormalidades Linfáticas/diagnóstico , Anormalidades Linfáticas/patologia , Anormalidades Linfáticas/terapia , Nevo Azul/diagnóstico , Nevo Azul/patologia , Nevo Azul/terapia , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/patologia , Neoplasias Cutâneas/terapia , Malformações Vasculares/diagnóstico , Malformações Vasculares/patologia , Malformações Vasculares/terapiaRESUMO
Pulmonary lymphatic disorders are characterized by the presence of the abnormal lymphatic tissues in the thoracic cavity, presenting clinically as chylothorax, chylopericardium, chyloptysis, interstitial lung disease and plastic bronchitis. These conditions include: neonatal chylothorax, cardiac and non-cardiac plastic bronchitis, non-traumatic chylothorax, post congenital cardiac surgery chylothorax and complex lymphatic malformations. Recently developed lymphatic imaging techniques, such as intranodal lymphangiography and dynamic contrast enhanced magnetic resonance lymphangiography demonstrated abnormal pulmonary lymphatic flow from thoracic duct into pulmonary parenchyma as a pathophysiological mechanism of these diseases. Novel minimally invasive lymphatic interventions, such as thoracic duct embolization, interstitial lymphatic embolization and surgical lympho-venous anastomosis, provide an effective treatment of these conditions.
Assuntos
Pneumopatias/diagnóstico , Pneumopatias/terapia , Doenças Linfáticas/diagnóstico , Doenças Linfáticas/terapia , Bronquite/diagnóstico , Bronquite/terapia , Quilotórax/diagnóstico , Quilotórax/terapia , Gerenciamento Clínico , Cardiopatias Congênitas/cirurgia , Humanos , Pneumopatias/congênito , Neoplasias Pulmonares/diagnóstico , Neoplasias Pulmonares/terapia , Linfangiectasia/congênito , Linfangiectasia/diagnóstico , Linfangiectasia/terapia , Linfangioma/diagnóstico , Linfangioma/terapia , Anormalidades Linfáticas/diagnóstico , Anormalidades Linfáticas/terapia , Linfografia , Imageamento por Ressonância Magnética , Neoplasias do Mediastino/diagnóstico , Neoplasias do Mediastino/terapia , Síndrome de Noonan/diagnóstico , Síndrome de Noonan/terapia , Osteólise Essencial/diagnóstico , Osteólise Essencial/terapia , Derrame Pericárdico/diagnóstico , Derrame Pericárdico/terapia , Complicações Pós-Operatórias/diagnóstico , Complicações Pós-Operatórias/terapiaAssuntos
Linfangiectasia/diagnóstico , Lesões por Radiação/diagnóstico , Dermatopatias Vesiculobolhosas/diagnóstico , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias da Mama/radioterapia , Neoplasias da Mama/terapia , Carcinoma Ductal de Mama/radioterapia , Carcinoma Ductal de Mama/terapia , Terapia Combinada/efeitos adversos , Diagnóstico Diferencial , Feminino , Humanos , Linfangiectasia/etiologia , Mastectomia , Radioterapia Conformacional/efeitos adversos , Dermatopatias Vesiculobolhosas/etiologia , Tamoxifeno/administração & dosagemRESUMO
CASE PRESENTATION: A 34-year-old man presented to a community hospital with sudden-onset pleuritic chest pain on a background of a 12-month indolent history of progressive exertional dyspnea. He denied cough, fevers, night sweats, or weight loss. He reported some low back pain and ache. He had a history of gastroesophageal reflux and was a current smoker with a 20-pack year history. There were no known occupational or environmental exposures and there was no family history of any lung disease.
Assuntos
Osso e Ossos , Quilotórax , Pneumopatias/congênito , Linfangiectasia/congênito , Dor Musculoesquelética , Pleura , Derrame Pleural , Sirolimo/administração & dosagem , Toracentese/métodos , Adulto , Antibióticos Antineoplásicos/administração & dosagem , Biópsia , Osso e Ossos/diagnóstico por imagem , Osso e Ossos/patologia , Dor no Peito/diagnóstico , Dor no Peito/etiologia , Quilotórax/diagnóstico , Quilotórax/etiologia , Quilotórax/fisiopatologia , Diagnóstico Diferencial , Humanos , Imuno-Histoquímica , Pneumopatias/complicações , Pneumopatias/diagnóstico , Pneumopatias/fisiopatologia , Pneumopatias/terapia , Linfangiectasia/complicações , Linfangiectasia/diagnóstico , Linfangiectasia/fisiopatologia , Linfangiectasia/terapia , Masculino , Dor Musculoesquelética/diagnóstico , Dor Musculoesquelética/etiologia , Pleura/diagnóstico por imagem , Pleura/patologia , Derrame Pleural/diagnóstico , Derrame Pleural/etiologia , Tomografia Computadorizada por Raios X/métodos , Resultado do TratamentoRESUMO
BACKGROUND: Renal lymphangiectasia is a rare and poorly understood lymphatic disease associated with lymphatic dilation and leakage. To our knowledge, no cases have been described in the context of a transplanted kidney. METHODS: We describe 2 cases of renal lymphangiectasia in transplanted kidneys, both from pediatric donors. RESULTS: The cases of allograft lymphangiectasia are characterized by severe, symptomatic ascites refractory to attempts at medical and surgical management, and ultimately requiring allograft nephrectomy. CONCLUSIONS: While lymphatic complications, particularly lymphoceles, are not uncommon in renal transplantation, lymphangiectasia is a distinct condition which should be considered in renal transplant patients with ascites, after all other sources have been ruled out.
Assuntos
Aloenxertos/patologia , Transplante de Rim/efeitos adversos , Rim/patologia , Linfangiectasia/diagnóstico , Complicações Pós-Operatórias/diagnóstico , Aloenxertos/diagnóstico por imagem , Biópsia , Criança , Feminino , Humanos , Rim/diagnóstico por imagem , Laparoscopia , Linfangiectasia/etiologia , Linfangiectasia/patologia , Linfangiectasia/cirurgia , Masculino , Pessoa de Meia-Idade , Paracentese , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/patologia , Tomografia Computadorizada por Raios X , Resultado do TratamentoRESUMO
Objective: To investigate the clinical symptoms, treatment intervention and prognosis of Kaposiform lymphangiomatosis(KLA). Methods: Medical information and clinical characteristics data of 8 KLA patients who were admitted to Department of Pediatric Surgery of West China Hospital of Sichuan University from January 2016 to February 2019 were retrospectively reviewed and analyzed. There were 5 males and 3 females with age of 5.8 years old (from 8 months to 29 years old). Results: The lesions in all patients were diffusely distributed. In all 8 patients, the lung and mediastinum were involved with different degrees. Three cases had lesions involving pelvic and abdominal organs. Three cases had lesions involving bones. One case simultaneously involved pelvic and abdominal organs, and 1 case was involved laryngeal and neck. The clinical characteristics were mainly respiratory symptoms. In the laboratory tests, 6 patients had different degrees of thrombocytopenia (minimum 3 × 10(9)/L), and 4 patients had severe fibrinogen reduction (minimum 0.42 g/L). Three patients had prolonged activated partial thromboplastin time (up to 64.2 seconds) and 3 patients had prolonged prothrombin time (up to 18.6 seconds). After surgery (including thoracotomy, chest tube, pericardiocentesis, splenectomy) and empiric medicine therapy (vincristine, sirolimus and corticosteroid), the symptoms improved in 1 case, 2 cases died of complications, 2 cases were stable and 3 cases progressed up to February 2019. Conclusions: KLA is a rare disease that should be differentiated from other types of vascular diseases. Currently, there is no consensus treatment guidelines exist. Accurate diagnosis in KLA can be a challenge. The situation in patients with KLA is prone to rapid deterioration and progress. Future research efforts should seek to develop target-specific drugs for KLA.
Assuntos
Linfangiectasia/diagnóstico , Linfangiectasia/terapia , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Linfangiectasia/complicações , Doenças Linfáticas/complicações , Doenças Linfáticas/diagnóstico , Doenças Linfáticas/terapia , Masculino , Prognóstico , Estudos Retrospectivos , Adulto JovemAssuntos
Linfangiectasia/etiologia , Protectomia/efeitos adversos , Lesões por Radiação/etiologia , Neoplasias Retais/terapia , Pele/patologia , Abdome , Biópsia , Humanos , Linfangiectasia/diagnóstico , Linfangiectasia/patologia , Masculino , Pessoa de Meia-Idade , Pênis , Lesões por Radiação/diagnóstico , Lesões por Radiação/patologia , Radioterapia Adjuvante/efeitos adversos , EscrotoRESUMO
BACKGROUND: Kaposiform lymphangiomatosis (KLA) and kaposiform hemangioendothelioma (KHE) are rare and aggressive vascular disorders. The aim of this study was to examine the clinical features and prognosis of KLA and KHE involving the thorax. METHODS: The clinical features, imaging and pathological findings, treatments and outcome were retrospectively reviewed for 6 patients with KLA and 7 patients with KHE involving the thorax. RESULTS: The mean ages at the time of the presentation of signs/symptoms were 26.7 months and 4.1 months for KLA and KHE, respectively. Respiratory symptoms, pericardial and pleural effusion, thrombocytopenia and coagulopathy were common in both KLA and KHE. Diffuse lesions involving the lung and extrathoracic sites were observed in KLA but not in KHE. Histopathologically, all lesions had spindled tumor cells, which were immunoreactive for CD31 and D2-40. In KLA, the spindle cells were distributed in sparse and poorly marginated clusters, whereas the spindle cells formed more defined and confluent vascularized nodules in KHE. Unlike the refractory behavior of KLA, the majority of patients with KHE responded to medical treatments with regression of the lesion and normalization of the hematologic parameters. CONCLUSIONS: The presenting and histological characteristics of KLA can overlap with those of KHE. The presence of diffuse vascular lesions in the mediastinum and lung with refractory thrombocytopenia and coagulopathy should suggest the diagnosis of KLA. Given the rarity and high morbidity and mortality of these disorders, the diagnostic process and therapeutic approach should include a multidisciplinary team review and consensus.
Assuntos
Hemangioendotelioma/diagnóstico por imagem , Hemangioendotelioma/diagnóstico , Síndrome de Kasabach-Merritt/diagnóstico por imagem , Síndrome de Kasabach-Merritt/diagnóstico , Linfangiectasia/diagnóstico por imagem , Linfangiectasia/diagnóstico , Sarcoma de Kaposi/diagnóstico por imagem , Sarcoma de Kaposi/diagnóstico , Diagnóstico Diferencial , Feminino , Humanos , Lactente , Recém-Nascido , Imageamento por Ressonância Magnética , Masculino , Prognóstico , Estudos RetrospectivosRESUMO
A 62-year-old man presented with a 1-month history of right eye pain unresponsive to artificial tears and topical steroids. At presentation, bullous yellowish fluid collection was noted in the nasal conjunctiva. Corneal thinning and opacity were observed at the 3 o'clock position of the cornea. High-frequency radiowave ablation and biopsy were performed at the affected area. Conjunctival lymphangiectasia was confirmed by excisional biopsy. An improvement in the degree of corneal dellen and chemosis was evident 1 week after ablation. Use of a high-frequency radiowave electrosurgical device may be a simple and effective treatment option for symptomatic conjunctival lymphangiectasia.
Assuntos
Túnica Conjuntiva/cirurgia , Doenças da Túnica Conjuntiva/cirurgia , Distrofias Hereditárias da Córnea/complicações , Eletrocirurgia/instrumentação , Linfangiectasia/cirurgia , Biópsia , Túnica Conjuntiva/irrigação sanguínea , Doenças da Túnica Conjuntiva/complicações , Doenças da Túnica Conjuntiva/diagnóstico , Distrofias Hereditárias da Córnea/diagnóstico , Distrofias Hereditárias da Córnea/cirurgia , Desenho de Equipamento , Humanos , Linfangiectasia/complicações , Linfangiectasia/diagnóstico , Vasos Linfáticos/diagnóstico por imagem , Vasos Linfáticos/cirurgia , Masculino , Pessoa de Meia-Idade , Tomografia de Coerência ÓpticaRESUMO
Unilateral congenital pulmonary lymphangiectasia (CPL) is an extremely rare disease of the pulmo nary lymphatic vessels. OBJECTIVE: to present a case of CPL in a premature newborn. CLINICAL CASE: premature male newborn with severe respiratory failure at 2 hours of extrauterine life was treated with exogenous surfactant, catecholamines and high frequency oscillatory ventilation (HFOV). Chest computed tomography (CT) scan showed bullae and air trapping of the left lung; the histopathological study showed cystic dilation of the bronchoalveolar lymphatic channels. The diagnosis of secondary unilateral CPL was made. The clinical course up to 19 months of age was normal and the chest CT scan showed few emphysematous bullae. CONCLUSIONS: CPL must be one of the differential diagnoses in neonates with unexplained respiratory distress. The prognosis will depend on the type of CPL and lung involvement.
Assuntos
Doenças do Prematuro/diagnóstico , Pneumopatias/congênito , Linfangiectasia/congênito , Humanos , Recém-Nascido , Recém-Nascido Prematuro , Pneumopatias/diagnóstico , Linfangiectasia/diagnóstico , MasculinoRESUMO
A 56-year-old man presented with multiple, skin-colored, raised eruptions of the scrotum that had been present for 2 years. Their onset had been gradual, and they had been increasing in size, resulting in cosmetic disfigurement. A year previously, he had been operated on for a bilateral vaginal hydrocele with partial excision and eversion of the sac (Jabouley method).1 There had been no extramarital or unprotected sexual contact, other hospitalizations, or major surgery, swelling of the legs, or long periods of incumbency. Cutaneous examination revealed multiple, discrete and/or coalescing verrucous papules distributed on the upper portion of the scrotum and associated with edema of the penis (Figure 1). The inguinal lymph nodes were not enlarged. Complete blood counts and ultrasonography of the abdomen were normal. Tissue sections stained with hematoxylin and eosin showed hyperkeratosis and multiple ectatic vessels, primarily confined to the papillary dermis, abutting the overlying epidermis, and demarcated by a single endothelial lining. The dilated vessels contained homogenous eosinophillic material (Figure 2).
Assuntos
Linfangiectasia/diagnóstico , Escroto , Dermatopatias/diagnóstico , Humanos , Linfangiectasia/complicações , Masculino , Pessoa de Meia-Idade , Dermatopatias/etiologiaRESUMO
Smoking-related interstitial fibrosis is a distinct form of fibrosis, found in smokers, which has striking histopathological features. We present a case of pulmonary interstitial fibrosis with cysts in a 58-year-old woman who was a significant active smoker, presenting with a 7 month history of progressive dyspnea. TAC revealed thin-walled pulmonary cysts. An open lung biopsy was performed and the histopathological study showed hyaline fibrous thickening of the alveolar septa, respiratory bronchiolitis and cysts in the thickness of the interlobar septa. Immunohistochemically, the absence of an epithelial, vascular or lymphatic endothelial lining of the cysts would suggest that the cysts had been caused by pulmonary interstitial emphysema. Immunohistochemistry is essential in the differential diagnosis that includes, in this case, true cysts, pseudocysts and pulmonary lymphangiectasia.
Assuntos
Bronquiolite/patologia , Fumar Cigarros/efeitos adversos , Cistos/patologia , Imuno-Histoquímica , Doenças Pulmonares Intersticiais/patologia , Enfisema Pulmonar/complicações , Biomarcadores/análise , Biópsia , Cistos/diagnóstico por imagem , Cistos/metabolismo , Diagnóstico Diferencial , Feminino , Humanos , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Doenças Pulmonares Intersticiais/etiologia , Doenças Pulmonares Intersticiais/metabolismo , Linfangiectasia/diagnóstico , Pessoa de Meia-Idade , Fumar , Tomografia Computadorizada por Raios XRESUMO
La linfangiectasia pulmonar congénita (LPC) unilateral es una enfermedad extremadamente rara de los vasos linfáticos pulmonares. OBJETIVO: presentar un caso de LPC en un recién nacido prematuro. CASO CLÍNICO: recién nacido masculino, prematuro, con insuficiencia respiratoria severa a las 2 horas de vida extrauterina, recibió tratamiento con surfactante exógeno, catecolaminas y ventilación de alta frecuencia oscilatoria (VAFO). La tomografía axial computarizada (TAC) de tórax reveló bulas y atrapamiento de aire de pulmón izquierdo, el estudio histopatológico describió dilatación quística de los canales linfáticos broncoalveolares. Se diagnosticó LPC unilateral secundaria. La evolución clínica hasta los 19 meses de edad fue normal y la TAC de tórax mostró escasas bulas enfisematosas. CONCLUSIONES: La LPC debe ser uno de los diagnósticos diferenciales en neonatos con dificultad respiratoria inexplicable. El pronóstico dependerá del tipo de LPC y de la afectación pulmonar.
Unilateral congenital pulmonary lymphangiectasia (CPL) is an extremely rare disease of the pulmo nary lymphatic vessels. OBJECTIVE: to present a case of CPL in a premature newborn. CLINICAL CASE: premature male newborn with severe respiratory failure at 2 hours of extrauterine life was treated with exogenous surfactant, catecholamines and high frequency oscillatory ventilation (HFOV). Chest computed tomography (CT) scan showed bullae and air trapping of the left lung; the histopathological study showed cystic dilation of the bronchoalveolar lymphatic channels. The diagnosis of secondary unilateral CPL was made. The clinical course up to 19 months of age was normal and the chest CT scan showed few emphysematous bullae. CONCLUSIONS: CPL must be one of the differential diagnoses in neonates with unexplained respiratory distress. The prognosis will depend on the type of CPL and lung involvement.