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Monday, July 31, 2006

Pulmonary Lymphangiectasia

Pulmonary Lymphangiectasia


A congenital disease involving the lung characterized by greatly dilated lymphatic ducts throughout the lung.



age of onset:neonatal period and after the neonatal periodrisk factors:Noonan's SyndromeTurner's Syndrome


(after Noonon (1970))*:

1. Group Idilated pulmonary lymphatics are part of a generalized form of lymphangiectasis: thoracic and extrathoracic (intestinal) lymphangiectasiaassociated with
lymphedemahemihypertrophydiffuse angiomatosis in which bone is the most common site of involvementless severe form and has a much better prognosis than the other two forms

2. Group IIdue to a cardiac lesiondilated pulmonary lymphatics are acquired in utero due to obstruction of the pulmonary venous flow secondary to a cardiac lesion which may interfere with the normal regression of the lymphatic tissue elements after the 16th week of fetal lifevery poor prognosis

3. Group IIIdue to a defect in the primary development of the lungLawrence (1955) postulated that lymphangectasis is due to a developmental error in which the normal regression of connective tissue elements in the lung fails to occur after the 16th week of life. Subsequently the lymph vessels do not regress in size and remain enlarged in relation to the parenchyma of the lung (Noonan, 1970).male predominancemay be associated with congenital malformations including cardiac lesionsclinical picture characterized by over expanded lungs with alveolar hypoventilation and periodic wheezing and a relapsing coursedilated lymphatics may result in marked restriction of respiratory movements poor prognosis

*first report of CPL by Virchow (1856) and first named by Lawarence in 1955


presents in two forms:1. Neonatal Periodpresents with respiratory distress and cyanosis at birth(usually term) with rapid demisecan also present as a stillbirth2. Post Neonatal Periodpresents with respiratory difficultiesprogressive relapsing courseassociated with chylopericardium, chylothorax, chronic cough, congestive heart failure3. Associated Abnormalitiescongenital abnormalities in 50% of cases:1. CardiacTAPVR, mitral valve atresia, hypoplastic left heart, aortic coarctation, VSD, PDA, ASD

2. Otherscystic hygromas, diffuse angiomatosis (bones), pneumothoraxINVESTIGATIONS:

1. Lung Biopsycystic dilation of the lymphatics in the subpleural and intralobular regions and perivascular spaces give the lungs a subpleural reticulated pattern of fine interconnecting white lines with air-filled cystic areas

2. Imaging Studies

Chest X-Rayhyperexpansiondiffuse reticular pattern




Pulmonary Lymphangiectasia:

Viewcases of pulmonary lymphangiectasis


Pulmonary lymphangiectasia is a rare disorder that is seen more frequently in males. It occurs secondary to a developmental defect which results in obstructive, dilated lymphatics and subsequently "wet lungs" (ie: there is arrested lymphatic development). Patients may present with severe neonatal respiratory distress, cyanosis, and death. There are questionable associations with familial cases, Noonan's syndrome, and Ichthyosis congenita [1]. There are 3 forms of the disorder:

1- Isolated Pulmonary: The developmental defect is isolated to the pulmonary lymphatics. Felt to be related to failure of regression of the pulmonary lymphatics after the 16th week of gestation. It results in severe interstitial lung disease and has a poor prognosis. Rarely children may live beyond the neonatal period.

2- Secondary to Pulmonary Venous Obstruction: This form is associated with congenital heart disease and accounts for 30% of cases. Cardiac abnormalities include: Hypoplastic left heart, total anomalous pulmonary venous return type III, and pulmonic vein atresia.

3- Generalized: (Systemic)- Characterized by lymphangiectasia throughout the body. The pulmonary manifestations are typically mild. Patients may have an associated lymphangioma.



Congenital dilatation of the pulmonary lymphatics

SN Javett, I Webster and JL Braudo904 Medical Centre Jeppe Street, Johannesburg, South Africa

The disease variously referred to as congenital dilatation of the pulmonary, lymphatics congenital pulmonary cystic lymphangiectasis, and congenital lymphangiomatosis of the lung is a rarity known almost exclusively to pediatric pathologists. Up to 1959, 21 cases had appeared in world literature, with only two reports in clinical journals. Congenital dilatation of the pulmonary lymphatics has been observed as a chance finding in fetuses dying in utero, stillborn infants, and neonates, without any mention being made as to its possible significance. The great majority of those born alive have suffered in the immediate neonatal period from an acute respiratory disease exhibiting dyspnea and cyanosis, death following within a few hours. As a consequence, the opportunity for clinical observation and investigation has been strictly limited. Confirmation has been possible only by autopsy. With virtually no clinical information to work on, the pathologists have centered interest almost entirely on the identification of lymphatics, the histopathology, and theories of causation without being in a position to offer correlation between histologic findings and symptoms. In the absence of any clinical build-up, too, it is not surprising that the disease has escaped the attention of most clinicians. We have had the opportunity of observing and investigating a patient with congenital dilatation of the pulmonary lymphatics, still alive, albeit not well, at the age of 8 months. It is the purpose of this paper to describe as a clinical entity a condition a clinical entity a condition hitherto regarded as a pathological curiosity.



Primary pulmonary lymphangiectasia in infancy and childhood

P.M. Barker1, C.R. Esther, Jr1, L.A. Fordham2, S.J. Maygarden3 and W.K. Funkhouser3
Depts of 1 Paediatrics (Division of Paediatric Pulmonology), 2 Radiology, and 3 Pathology, University of North Carolina at Chapel Hill, Chapel Hill, NC, USA Correspondence: P.M. Barker, Division of Paediatric Pulmonology, Dept of Paediatrics, 200 Mason Farm Road, CB 7220, University of North Carolina at Chapel Hill, Chapel Hill, NC, 27599, USA. Fax: 1 9199666179. E-mail:

Keywords: Growth, pathology, radiology, spirometry

Received: February 4, 2004Accepted May 20, 2004

C.R. Esther Jr is supported by a Parker B. Francis Pulmonary Fellowship.

Primary pulmonary lymphangiectasia (PPL) is a rare disorder of unknown aetiology characterised by dilatation of the pulmonary lymphatics. PPL is widely reported to have a poor prognosis in the neonatal period and little is known about the clinical features of patients who survive the newborn period.

The current authors report the outcome in nine patients diagnosed in infancy with PPL over a 15-yr period at a single university-based hospital clinic and followed for a median of 6 yrs.

Although all of the patients initially experienced respiratory distress, respiratory symptoms improved in most patients after infancy and were notably better by the age of 6 yrs. Many patients had poor weight gain in the first years of life, which eventually improved. Radiological scans showed progressive resolution of neonatal infiltrates, but were characterised by hyperinflation and increased interstitial markings in older children. Most patients had evidence of bronchitis and grew pathogenic organisms from quantitative bronchoalveolar lavage culture. Pulmonary function tests showed predominantly obstructive disease that did not deteriorate over time.

In conclusion, these results suggest that primary pulmonary lymphangiectasia does not have as dismal a prognosis as previously described and symptoms and clinical findings improve after the first year of life.



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