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PubMed1999

Acquired lymphangiectasis.

Acquired lymphangiectasis is a dilatation of lymphatic vessels that can result as a complication of surgical intervention and radiation therapy for malignancy. Acquired lymphangiectasis shares clinical and histologic features with the congenital lesion, lymphangioma circumscriptum. Diagnosis and treatment of these vesiculo-bullous lesions is important because they may be associated with pain, chronic drainage, and cellulitis. We describe two patients who had these lesions after treatment for cancer and review the pertinent literature. Although a number of treatment options are available, we have found CO2 laser ablation particularly effective.

Adenocarcinoma↗
PubMed2004

Autopsy case of congenital pulmonary lymphangiectasis.

Congenital pulmonary lymphangiectasis (CPL) is a rare anomaly. We report a female infant born at 39 weeks of gestation who was found to have CPL. Cyanosis and tachypnea were noted immediately after birth, and, at room air, PaO2 was 30.7 mmHg, PaCO2 was 82.5 mmHg and pH was 7.12. The infant's symptoms did not improve even with the initiation of artificial ventilation. Chest X-ray film showed cotton-like infiltrates in both lungs and an air-leak surrounding the cardiac shadow. Echocardiography study showed no abnormality. The neonate died 3 days after birth due to hypoxemic cardiac failure. At autopsy, the pleural surface contained numerous dilated vessels that had the appearance of lymphatics. Microscopic features of the lungs were marked lymphatic dilatation of the perivascular, subpleural and interlobular areas. Lymphangiectasis was found in the liver, kidney, pancreas, thyroid and alimentary canals, such as the esophagus, stomach and rectum. Patients with lymphatic dilatations in extrapulmonary organs have mild pulmonary involvement and symptoms and a better prognosis. However, a few cases of CPL with lymphatic dilatations in extrapulmonary organs and an aggressive course, such as the present case, have been reported. The clinical behavior and prognosis of CPL depend on the extent of pulmonary involvement of the lymphatic dilatations regardless of systemic lymphatic dilations.

Fatal Outcome↗
PubMed1983

Congenital pulmonary lymphangiectasis. A case complicated by chylothorax.

A case is reported of an infant with features of Noonan Syndrome and congenital pulmonary lymphangiectasis. Soon after birth, persistent respiratory distress developed, and, at 4 months of ge, a chylothorax was noted. Medium chain triglyceride therapy by the enteral route was tried, but parenteral alimentation and chest-tube drainage were required. Diagnosis of pulmonary lymphangiectasis was confirmed by biopsy.

Chylothorax↗
PubMed2006

Endothelial, inducible and neuronal nitric oxide synthase in congenital pulmonary lymphangiectasis.

Abnormal growth and development of lymphatic pulmonary structures leads to severe hypoxia in congenital pulmonary lymphangiectasis (CPL). This case study aims to determine the cellular source and topographical distribution of the nitric oxide synthases in CPL. It studies the post mortem tissue of a term newborn with the clinical course and histological findings of CPL and three controls without pulmonary pathology. It was found that endothelial cells of pulmonary arteries and lymphatic structures stained significantly more for endothelial nitric oxide synthase protein in the CPL patient compared to the controls. The authors conclude that synthesis of endothelial nitric oxide synthase is upregulated in vascular and lymphatic endothelial cells in congenital pulmonary lymphangiectasis.

Adult↗
PubMed2004

Acquired lymphangiectasis after breast conservation treatment for breast cancer: report of a case.

Acquired lymphangiectasis is a dilatation of lymphatic vessels that can result as a complication of surgical intervention and radiation therapy for malignancy. Acquired lymphangiectasis shares clinical and histological features with the congenital lesion, lymphangioma circumscriptum. Diagnosis and treatment of these vesiculobullous lesions is important because they may be associated with pain, chronic drainage, and cellulitis. We describe patient who had these lesions after treatment for cancer. Although a number of treatment options are available, we have found cryosurgery and electrocautery to be particularly effective.

Aged↗
PubMed1988

Conjunctival hemorrhagic lymphangiectasis.

Conjunctival hemorrhagic lymphangiectasis is a benign condition resulting from an abnormal communication between conjunctival lymphatics and conjunctival blood vessels. In this paper, two cases of conjunctival hemorrhagic lymphangiectasis are presented and the possible mechanisms giving rise to this entity are discussed. To aid in clinical differential diagnosis, a short discussion of conjunctival lymphangioma is also presented.

Conjunctival Diseases↗
PubMed1985

[Non-Hodgkin lymphoma arising in a case of Waldmann's intestinal lymphangiectasis].

A case of high grade malignant lymphoma in a young woman with congenital disseminated lymphangiectasis, exudative enteropathy and immunodeficiency is reported. Lymphoid malignancies occur with a high frequency in the course of this rare disease, possibly as a consequence of immunodeficiency. In this case, a selective defect of the OKT4+ peripheral blood lymphocyte subset has been shown. The relationships between congenital lymphangiectasis, immunodeficiency and malignant lymphoma are discussed.

Adult↗
PubMed1995

Progressive lymphangiectasis and recurrent chylothorax in a dog after thoracic duct ligation.

A 2-year-old Bernese Mountain Dog was examined to determine the cause of bilateral pleural effusion. Torsion was diagnosed, and a lobectomy of a lung lobe was performed. Chylothorax developed 12 days after lung lobectomy. Mesenteric lymphangiography revealed lymphangiectasis Lymphangiography immediately after surgical thoracic duct was completely obstructed, but chylothorax persisted after thoracic duct ligation. Lymphangiography was repeated 50 days after ligation of the thoracic duct and revealed multiple patent thoracic duct branches and progressive lymphangiectasis. A second attempt to ligate the thoracic duct caused the effusion to become serosanguineous. A pleuroperitoneal shunt with a manually operated pump chamber was used to remove the pleural effusion. Chylothorax was again detected 50 weeks after placement of shunt. Mesenteric lymphangiography revealed multiple patent thoracic duct branches and a lymphatic plexus that extended across the thoracic cavity.

Animals↗
PubMed1981

Familial occurrence of congenital pulmonary lymphangiectasis. Genetic implications.

Congenital pulmonary lymphangiectasis (CPL) is a rare, generalized disease of the lung, consisting of lymphatic cysts in the subpleural and interlobular connective tissue. This disorder typically manifests a clinical picture of acute respiratory distress with cyanosis shortly after birth, with death occurring in the neonatal period. Several cases of this disorder have been described in the literature, but there has been no family with more than one affected child. We report the first instance, to our knowledge, of familial cases of CPL, which raises an important question regarding a possible genetic component in this disorder. The implications of this are discussed.

Female↗
PubMed1999

Congenital unilobar pulmonary lymphangiectasis.

A premature female infant is described in whom a cystic malformation of the left lung was observed on the second day of life. A lobectomy was carried out on day 31 of life, and histological examination revealed congenital pulmonary lymphangiectasis with isolated left upper lobe involvement.

Cystic Adenomatoid Malformation of Lung, Congenita↗
PubMed1981

Response of lymphangiectasis to radiotherapy.

A 14-year-old girl with lymphangiectasis of the skull causing rapid extensive destruction of the left orbit, zygoma, mandible, sphenoid, and occiput underwent radiotherapy with 2000 rad to the entire skull, mandible, and upper cervical vertebrae. Three years later, progression of the disease has ceased, and the involved bone is slowly remineralizing.

Adolescent↗
PubMed1993

Congenital pulmonary lymphangiectasis with chylothorax: a heterogeneous lymphatic vessel abnormality.

We report on 7 perinatal autopsy cases of primary congenital pulmonary lymphangiectasis (CPL) with bilateral chylothorax. This study demonstrates that primary CPL is often complicated by chylous pleural effusions with ensuing pulmonary hypoplasia. Conversely, CPL appears to be a constant pathological finding in spontaneous congenital chylothorax. These observations indicate a common pathogenesis for both disorders. The basic defect is not an intrinsic lung abnormality, but a developmental error of the lymphatic system resulting in a pulmonary lymphatic obstruction sequence. The cause of CPL is heterogeneous. Apparently, most cases are sporadic occurrences. We report the second instance of CPL in sibs. This indicates that some cases are genetically determined with autosomal recessive inheritance. CPL may also be part of a multiple congenital anomalies (MCA) syndrome such as Noonan, Ullrich-Turner, and Down syndrome.

Abnormalities, Multiple↗
PubMed1993

Persistence of müllerian derivatives, lymphangiectasis, hepatic failure, postaxial polydactyly, renal and craniofacial anomalies.

We describe 3 unrelated newborn males with a previously unreported constellation of congenital anomalies. All 3 died neonatally of hepatic failure. Clinically, they presented with a pattern of malformations characterized by prenatal linear growth deficiency, hypertrophied alveolar ridges, redundant nuchal skin, and postaxial polydactyly. All 3 cases had male external genitalia with cryptorchidism, and 2 of them, a small penis. Necropsies showed similar internal anomalies, consisting of müllerian duct remnants, lymphangiectasis, and renal anomalies. The karyotypes were normal (46, XY) in skin fibroblasts (Case 1) and in peripheral blood lymphocytes (Case 3). Although this pattern of congenital anomalies must be differentiated from several other lethal syndromes, to our knowledge, no similar cases have been described previously. Cause of this syndrome is unknown. Because Case 2 had a previous brother with similar anomalies, we suspect that this new entity probably is an autosomal recessive or X-linked trait.

Abnormalities, Multiple↗
PubMed2006

Congenital pulmonary lymphangiectasis sequence: a rare, heterogeneous, and lethal etiology for prenatal pleural effusion.

OBJECTIVE: Case report and literature review for congenital pulmonary lymphangiectasis (CPL) CASE REPORT: Male fetus with bilateral pleural effusion, thoracoamniotic shunt, preterm delivery, and prolonged neonatal course with neonatal death at 3 months. Autopsy-identified CPL. DISCUSSION: Review of pathology, clinical course, and genetics of CPL. CONCLUSION: This postnatal diagnosis of CPL/Hennekam syndrome must be considered with prenatal counseling regarding a fetus with bilateral pleural effusions. This pathological entity is autosomal recessive and has a significant risk of lethality.

Adult↗
PubMed2002

Thoracic lymphangiectasis presenting with chyloptysis and bronchial cast expectoration.

A 70-year-old man with recurrent undiagnosed episodes of bronchial cast expectoration and pulmonary infiltrates on chest radiography for 15 years is described. The diagnosis of chyloptysis was established by chemical analysis of the bronchial aspiration. We emphasize the radiological findings of this rare observation. The CT-associated lymphangiography showed mediastinal lymphangiectasis with retrograde opacification of mediastinal and hilar lymph nodes as well as submucosal lymphatic vessels protruding into the lumen of the tracheo-bronchial tree without evidence of thoracic duct obstruction as well as a "crazy-paving appearance." Congenital incompetence of the valves of the lymphatic vessels originating from the thoracic duct is held to be the cause. Chyloptysis and pulmonary lymphatic disorder should be sought in cases of bronchial cast expectoration.

Aged↗
PubMed1977

Acquired vulvar lymphangiectasis in a child.

A unique case of extensive acquired vulvar lymphangiectasis without lymphedema in a child is described. This lesion developed after pelvic exenteration, lymphadenectomy, and radiation therapy for rhabdomyosarcoma of the bladder. These acquired etiologic factors simulated the pathophysiology of congenital lymphangioma. She was successfully treated with surgical excision and vulvar reconstruction.

Child↗
PubMed2004

Unilobar congenital pulmonary lymphangiectasis mimicking congenital lobar emphysema: an underestimated presentation?

BACKGROUND: Congenital lobar emphysema (CLE) is characterized by unilobar alveolar distension secondary to bronchomalacia or absent cartilage. In contrast, congenital pulmonary lymphangiectasis (CPL) is defined as distended lymphatics in the bronchovascular bundle, in the interlobular septa, and in the subpleural space. Little information is available regarding the radiologic presentation of CLE as it correlates with histological diagnosis. METHODS: In a retrospective chart review from 1995 to 2002, 8 patients (5 boys and 3 girls) with clinical and radiologic diagnosis of CLE were reviewed. RESULTS: The mean age at diagnosis was 26 months (range, 11 days to 10 years). All but one had classic respiratory symptoms of CLE. Six of 7 chest computed tomography (CT), scans were suggestive of CLE. Of 8 patients, 3 were treated without pulmonary resection with resolution of symptoms. Five patients underwent lobectomies, and histology results showed CPL in 3. CT failed to identify CPL in all cases. CONCLUSIONS: Diagnosis of CLE is not as straightforward as the literature suggests. Even retrospectively, radiologic distinction between CLE and CPL could not be achieved by an experienced pediatric radiologist. CPL, thus, mimics CLE clinically and radiologically and, therefore, should be considered in the differential radiologic diagnosis of CLE.

Child↗