Alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV)—awareness prevents extended or futile ECMO use
- 93 Downloads
Alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV) is a rare, fatal, congenital lung disorder involving abnormal development of the capillary vascular system around the alveoli of the lungs, which clinically presents as persistent pulmonary hypertension of the newborn (PPHN) refractory to treatment. It has been linked to the gene FOXF1 on chromosome 16q24.1–q24.2. Histopathological examination by lung biopsy is the gold standard for diagnosis.
Materials and methods
We present four cases of ACD/MPV who were referred for ECMO support with a diagnosis of PPHN with no apparent congenital anomalies.
All the newborns had an overwhelming course, with PPHN and hypoxemia refractory to treatment. The diagnosis of ACD/MPV was established by ante-mortem lung biopsy in all cases. Intensive care treatment was withdrawn post diagnosis, with none of the four surviving.
Early lung biopsy for a histological diagnosis allows expensive and ineffective treatment to be avoided. Lung biopsy can be performed with low risk and high-diagnostic yield for alveolar capillary dysplasia.
KeywordsPulmonary veins Lung biopsy Alveolar capillary dysplasia
The authors would like to thank Dr. O’neill (histopathologist).
Conflicts of interest
- 1.Abu-Shaweesh JM. Respiratory disorders in preterm and term infants. In: Martin RJ, Fanaroff AA, Walsh MC, editors. Fannaroff and Martin’s neonatal-perinatal medicine, diseases of the fetus and infant. 9th ed. St Louis: Elsevier Mosby; 2011. p. 1141–70.Google Scholar
- 9.Extracorporeal Life Support Organization. ECLS Registry Report, International Summary. Ann Arbor, MI: Extracorporeal Life Support Organization; 2013. pp. 1–26.Google Scholar
- 14.Hagood JS. Children’s Interstitial Lung Disease (ChILD): eMedicine Pediatrics: General Medicine. 2009. Available from: http://emedicine.medscape.com/article/1003631-overview. Accessed 15 Aug 2012.
- 16.Children’s hospital, St. Luis: new medical approach helps baby with rare disease [internet] 2012 [updated July 18, 2012 cited Feb 3, 2014] Available from: http://www.stlouischildrens.org/articles/features/2012/new-medical-approach-helps-baby-with-rare-disease