[MIM 265 300]

Extremely rare. Dilation of the lymphatic vessels draining interstitial and subpleural lung areas. Histology: dilation of the subpleural, interlobar, peribronchial and perivascular lymphatic vessels. Pleural effusions are chylous (ditto for pericardial and ascitic effusions if present).

Many clinical presentations are possible:

*        in utero: one of the many causes of fetal hydrops

*        neonatal form: major respiratory distress, cyanosis, tachypnea: artificial ventilation and drainage of the effusion.

*        post-neonatal form: more progressive (dyspnea, pulmonary subedema) or chylothorax or chylopericardium. The diagnosis of chylous effusion can be difficult if the child is not fed enterally .

Children who survive the neonatal period improve progressively on a respiratory point of view but present with both obstructive and restrictive chronic lung disease.

Two subgroups:

• primary pulmonary lymphangiectasies: it is either a congenital abnormality of lung development or the local expression of a systemic anomaly of the lymphatic vessels

-        primary pulmonary development anomaly

-        anomaly of the regression of the lymphatics on the 20th week of gestation

-        dilatation of the lung lymphatics

-        body hemihypertrophy and lymphedema

-        syndromic (see below)

• secondary pulmonary lymphangiectasies: following an anomaly of the heart or the large lymphatic vessels

-        hypoplasia of the left ventricle, pulmonary vein atresia, congenital mitral stenosis, cor triatum

-        agenesis of the thoracic duct

-        post-infectious obstruction of the lymphatics

Some syndromes may be associated with a primary pulmonary lymphangiectasia, they are:

• yellow nail syndrome [MIM 153 300]
• Noonan [MIM 163950]
• PEHO [MIM 260 656]
• German [MIM 231 080]
• Knobloch [MIM 267 750]
• Urioste [MIM 235 255]
• intestinal lymphangiectasies  [MIM 152 800]
• Hennekam lymphangiectasies  [MIM 235 510]
• lymphedema-hypoparathyroidy [MIM 247 410]
• lymphedema-  cerebral arterioveinous malformation[MIM 152 900]
• hypotrichosis - lymphedeme - telangiectasies [MIM 607 823]
• mandibulofacial dysostosis
• campomelia, Cumming type [MIM 211 890]

Anesthetic implications:

difficult ventilation, hypoproteinemia, failure to thrive (loss of proteins, immunoglobulins, and lymphocytes in pleural effusions)

References : 

-         Kamada N, Mitani Y, Kihira K, Iwao A et al. 
Fatal pulmonary lymphangiectasia manifesting after repeated surgery for intractable chylopericardium and chylothorax in a 20-month-old girl. 
Circulation 2008; 117: 1894-6.

-         Barker PM, Esther CR, Fordham LA, Maygarden SJ, Funkhouser WK. 
Primary pulmonary lymphangiectasia in infancy and childhood. 
Eur Resp J 2004; 24: 413-19.

-        Bellini C, Boccardo F, Campisi C, Bonioli E. 
Congenital pulmonary lymphangiectasia. 
Orphanet J Rare Diseases 2006 : 1 :43

       

Updated: October 2018