Linfangiomatosis pulmonar difusa con compromiso pleural y pericárdico. Reporte de un caso pediátrico / Diffuse pulmonary lymphangiomatosis with pleural and pericardial involvement. Pediatric case report

La linfangiomatosis pulmonar difusa es una enfermedad rara caracterizada por una marcada proliferación y dilatación de los vasos linfáticos en los pulmones, la pleura y el mediastino. Se desconoce la prevalencia, y la etiología no se comprende completamente.Una niña de 22 meses ingresó por poliserositis, con derrame pericárdico y pleural. Requirió pericardiocentesis y avenamiento pleural, y presentó drenaje de quilo (1,5-4 litros/día) sin respuesta al tratamiento médico (ayuno, nutrición parenteral y octreotide). Se realizó biopsia pulmonar. La anatomía patológica mostró hallazgos compatibles con linfangiomatosis difusa pulmonar. Comenzó tratamiento con sirolimus y propanolol, que disminuyeron las pérdidas por el drenaje pleural a la semana. Presentó buena evolución; suspendió aporte de oxígeno y se retiró el drenaje pleural. Se externó al cuarto mes de internación. El diagnóstico temprano de la linfangiomatosis pulmonar difusa es difícil de lograr, pero permite aplicar terapéuticas que evitan la progresión de enfermedad y disminuir la morbimortalida

Diffuse pulmonary lymphangiomatosis is a rare disease characterized by marked proliferation and dilation of lymphatic vessels in the lungs, pleura, and mediastinum. The prevalence is unknown and the etiology is not fully understood.A 22-month-old girl was admitted for polyserositis, with pericardial and pleural effusion. She required pericardiocentesis and pleural drainage, presenting chyle drainage (1.5-4 liters/day) without response to medical treatment (fasting, parenteral nutrition and octreotide). A lung biopsy was performed. The pathological anatomy showed findings compatible with diffuse pulmonary lymphangiomatosis. Treatment with sirolimus and propanolol began, decreasing losses due to pleural drainage one week after treatment. She progressed well, discontinued oxygen supply and pleural drainage was removed, leaving the patient after the fourth month of hospitalization.Early diagnosis of diffuse pulmonary lymphangiomatosis is difficult to achieve, but it allows the application of therapies that prevent disease progression, reducing morbidity and mortality.

A mechanism for chronic filarial hydrocele with implications for its surgical repair.

BACKGROUND: Chronic hydrocele is the most common manifestation of bancroftian filariasis, an endemic disease in 80 countries. In a prospective study, we evaluated the occurrence of intrascrotal lymphangiectasia, gross appearance/consistency of the testis, and the efficacy of complete excision of hydrocele sac in patients living in a bancroftian filariasis endemic area who underwent hydrocelectomy at the Center for Teaching, Research and Tertiary Referral for Bancroftian Filariasis (NEPAF). METHODOLOGY/PRINCIPAL FINDINGS: A total of 968 patients with uni- or bilateral filarial hydrocele (Group-1) and a Comparison Group (CG) of 218 patients from the same area who already had undergone hydrocele-sac-sparing hydrocelectomy elsewhere were enrolled at NEPAF. Twenty-eight patients from the Comparison Group with hydrocele recurrence were re-operated on at NEPAF and constitute Group-2. In Group-1 a total of 1,128 hydrocelectomies were performed (mean patient age of 30.3 yr and mean follow-up of 8.6 yr [range 5.3-12]). The hydrocele recurrence rates in Group-1 and in the Comparison Group (mean age of 31.5 yr) were 0.3%, and 19.3%, respectively (p<0,001). There was no hydrocele recurrence in Group-2 (mean patient age of 25.1 yr and mean follow-up of 6 yr [range 5-6.9]). Per surgically leaking or leak-prone dilated lymphatic vessels were seen in the inner or outer surface of the hydrocele sac wall or in surrounding tissue, particularly in the retrotesticular area, in 30.9% and in 46.3% of patients in Group-1 and Group-2, respectively (p = 0.081). The testicles were abnormal in shape, volume, and consistency in 203/1,128 (18%) and 10/28 (35.7%) of patients from Group-1 and Group-2, respectively (p = 0,025). CONCLUSIONS/SIGNIFICANCE: Lymph fluid from ruptured dilated lymphatic vessels is an important component of chronic filarial hydrocele fluid that threatens the integrity of the testis in an adult population living in bancroftian filariasis endemic areas. To avoid hydrocele recurrence the authors advise complete excision of hydrocele sac and when identified, leaking or leak-prone dilated lymphatic vessels should be sutured or excised.

Congenital pulmonary lymphangiectasia: CT and pathologic findings.

Congenital pulmonary lymphangiectasia is a rare disease characterized by dilation of lymphatic channels without lymphatic proliferation. The disease is seen almost exclusively in infancy and early childhood. The authors report 2 cases of pulmonary lymphangiectasia. The patients were a 12- and a 25-year-old male who presented with progressive dyspnea and hemoptysis. The diagnosis was confirmed by open lung biopsy. The radiographic findings consisted of bilateral reticular changes, peribronchial cuffing, and bilateral pleural effusions. High-resolution CT demonstrated extensive bilateral septal and peribronchovascular interstitial thickening, areas of ground-glass attenuation, and bilateral pleural effusions. The histologic findings consisted of ectatic and tortuous lymphatic channels in the interlobular septa, bronchovascular sheaths, and pleura.

Cutaneous vascular anomalies. Part I. Hamartomas, malformations, and dilatation of preexisting vessels

Classification of cutaneous vascular anomalies is difficult because conceptual confusion persists between vascular neoplasms and malformations. However, hemangiomas of the infancy fulfill criteria both for hyperplasia and neoplasm because they result from proliferation of endothelial cells, but often undergo complete regression. Despite these pitfalls we have classified cutaneous vascular anomalies into the following categories: hamartomas, malformations, dilatations of preexisting vessels, hyperplasias, benign neoplasms, and malignant neoplasms. In this first part of our clinicopathologic review of vascular anomalies, hamartomas, malformations, and dilatation of preexisting vessels are covered. Hamartomas include several combined vascular and melanocytic proliferations grouped as phakomatosis pigmentovascularis and the so-called eccrine angiomatous hamartoma that consists of proliferations of both eccrine glands and blood vessels. Vascular malformations result from anomalies of embryologic development, and in some of them the abnormalities of the involved vessels are more functional than anatomic, as is the case of nevus anemicus. In contrast, other cutaneous vascular malformations show striking morphologic abnormalities of the vascular structures. These anatomic vascular malformations are subdivided into the following groups: capillary, venous, arterial, lymphatic, and combined anomalies. Spider angioma, capillary aneurysm-venous lake, and telangiectases are not vascular proliferations at all, but dilations of preexisting vessels. In our opinion, most of the lesions described with the generic term of "angiokeratoma" are not authentic vascular neoplasms, but hyperkeratotic malformations of capillaries and venules or acquired telangiectases of preexisting blood vessels of the papillary dermis. Therefore the first group of these "angiokeratomas" are included in the vascular malformations section, and the second group are covered in the section of dilation of preexisting vessels. Lymphangiectases are considered the lymphatic counterpart of angiokeratomas because they result from ectasia of preexisting lymphatic vessels of the papillary dermis.

[Electron microscopy of the intestinal mucosa in a case of lymphangiectasis]. / Microscopía electrónica de la mucosa intestinal en un caso con linfangiectasia.

The morphology of the intestinal mucosa was reviewed using light microscopy and transmission electron microscopy from a two year-old male patient with protein losing primary intestinal lyphangiectasis. Diagnostic studies ruled out recognized forms of secondary intestinal lymphangiectasia (e.g cardiac, hepatic, renal diseases or lymphoreticular cancer). Furthermore, certain features distinguish this patient from the secondary type: decreased immunoglobulin and albumin levels, lymphocytopenia, chylous ascitis and delated lymphatics on small-bowel biopsy.

[Congenital pulmonary lymphangiectasis and hyaline membrane. Report of a case]. / Linfangiectasia pulmonar congénita y membrana hialina. Presentación de un caso.

The case of a newborn with pathological confirmation of congenital pulmonary lymphangiectasis and hyaline membrane is reported. The lymphagiectasis corresponded to type III, with histological characteristics that make it different from other types of lymphatic dilatation. This association is quite uncommon and should be considered in the differential diagnosis of acute respiratory failure in the newborn period.