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congenital pulmonary lymphangiectasis

Types

- CPL, Noonan Group 1
- CPL, Noonan Group 2
- CPL, Noonan Group 3
- unilobar congenital pulmonary lymphangiectasis (UCPL) (#15136998#)

Associations

- Ivemark syndrome with asplenia

  • total anomalous pulmonary venous connection (TAPVC)
  • atrioventricular communis
  • septal defects

- cardiovascular malformations

  • total anomalous pulmonary venous connection (TAPVC)
  • aortic arch malformation
  • hypoplastic left heart (left heart hypoplasia)
  • atrioventricular communis
  • ventricular septal defect (VSD)

- congenital lobar emphysema (#4715376#, #5936105#)
- renal malformations
- ichtyosis congenita
- cystic fibrosis
- generalized lymphangioma
- omphalocele (exomphalos)

See also

- pulmonary lymphangiectases

References

- Congenital pulmonary lymphangiectasis: report of an autopsy case masquerading as pulmonary interstitial emphysema. Yamada S, Hisaoka M, Hamada T, Araki S, Shiraishi M. Pathol Res Pract. 2010 Jul 15;206(7):522-6. PMID: #20399027#

- Chapdelaine J, Beaunoyer M, St-Vil D, Oligny LL, Garel L, Bütter A, Di Lorenzo M. Unilobar congenital pulmonary lymphangiectasis mimicking congenital lobar emphysema: an underestimated presentation? J Pediatr Surg. 2004 May;39(5):677-80. PMID: #15136998#