Alveolar capillary dysplasia: An emerging syndrome

Abstract

Alveolar capillary dysplasia is rare, and each new report adds important new information to the incomplete knowledge of this syndrome. Is this a new unreported case?1Montgomery V Buchino JJ Clinical pathologic conference: a newborn infant with pulmonary hypertension.J Pediatr. 1998; 133: 157-161Abstract Full Text Full Text PDF PubMed Scopus (10) Google Scholar The authors state that primary pulmonary hypertension (PH) is said to be rapidly fatal. 1Montgomery V Buchino JJ Clinical pathologic conference: a newborn infant with pulmonary hypertension.J Pediatr. 1998; 133: 157-161Abstract Full Text Full Text PDF PubMed Scopus (10) Google Scholar, 2Murphy JD Rabinovitch MR Goldstein JD Reid LM The structural basis of persistent pulmonary hypertension of the newborn infant.J Pediatr. 1981; 98: 962-967Abstract Full Text PDF PubMed Scopus (243) Google Scholar This was true in 1981, but survival and cure of all types of PH treated with extracorporeal membrane oxygenation is presently greater than 80%.3Zwischenberger JB Bartlett RH ECMO Extracorporeal Cardiopulmonary Support in Critical Care. Extracorporeal Life Support Organization.Ann Arbor (MI). 1995; : 255Google Scholar It should also be emphasized that greater than 75% of patients will have associated anomalies.4Garola RE Thibeault DW. Alveolar capillary dysplasia, with and without misalignment of pulmonary veins: an association of congenital anomalies.Am J Perinata1. 1998; 15: 103-107Crossref PubMed Scopus (45) Google Scholar Some anomalies such as Hirschsprung’s disease may be overlooked even with an autopsy. Misalignment of the veins, which is easily identified by light microscopy, is not a constant association with ACD,4Garola RE Thibeault DW. Alveolar capillary dysplasia, with and without misalignment of pulmonary veins: an association of congenital anomalies.Am J Perinata1. 1998; 15: 103-107Crossref PubMed Scopus (45) Google Scholar suggesting that ACD may be underdiagnosed. The light microscopic diagnosis of ACD can be enhanced by the study of lungs of infants dying of PH with the use of immunohistochemistry with collagen IV and CD34 antibodies and with thin sections.4Garola RE Thibeault DW. Alveolar capillary dysplasia, with and without misalignment of pulmonary veins: an association of congenital anomalies.Am J Perinata1. 1998; 15: 103-107Crossref PubMed Scopus (45) Google Scholar, 5Haraida S Lochbuhler H Heger A Nerlich A Diebold J Wiest I et al.Congenital alveolar capillary dysplasia: rare cause of persistent pulmonary hypertension.Pediatr Pathol Lab Med. 1997; 17: 959-975Crossref PubMed Scopus (22) Google Scholar Finally, a statement attributed to Cater et al indicated that PH with ACD was the result of chronic hypoxia.6Cater G Thibeault DW Beatty EC Kilbride HW Huntrakoon M Misalignment of lung vessels and alveolar capillary dysplasia: a cause of persistent pulmonary hypertension.J Pediatr. 1989; 114: 293-300Abstract Full Text PDF PubMed Scopus (75) Google Scholar However, a reading of this article will show no reference to hypoxia. Their conclusion was that chronic in utero vascular structural changes with superimposed vasoconstriction after birth led to the hypoxemia seen on clinical evaluation.