A neonate with coexisting congenital cystic adenomatoid malformation of the lung and alveolar capillary dysplasia: a case report with review of literature.

Abstract

OBJECTIVE:

First report of a term neonate with coexistent congenital cystic adenomatoid malformation (CCAM) of the lung and alveolar capillary dysplasia (ACD).

METHODS AND DESIGN:

Case report and literature review. Our institutional review board waived the need for consent.

SETTING:

We describe a term neonate with antenatally diagnosed CCAM and persistent pulmonary hypertension of the newborn (PPHN) who underwent right upper lobe resection on day 9 of life. Histology confirmed CCAM but closer examination also showed ACD. Postoperatively pulmonary hypertension persisted despite high-frequency oscillation and inhaled nitric oxide, and she was placed on extracorporeal membrane oxygenation. Due to the lack of any improvement, intensive care treatment was withdrawn 4 days later.

CONCLUSIONS:

This is the first description of an association between these two rare malformations. Although a causative link between CCAM and ACD is possible, it is unlikely. ACD should always be considered as a cause of severe PPHN when persistent beyond 10 days, even if another etiology of PPHN is present.

• Authors: Peter Paul Roeleveld, Jeff Martin, C W Chow, Trevor Duke
• Journal: Pediatric critical care medicine : a journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies (Pediatr Crit Care Med) Vol. 9 Issue 2 Pg. e10-3 (Mar 2008) ISSN: 1529-7535 [Print] United States
• PMID: 18477924 (Publication Type: Case Reports, Journal Article, Review)

Topics

• Abnormalities, Multiple
• Capillaries (pathology)
• Cystic Adenomatoid Malformation of Lung, Congenital (diagnostic imaging, physiopathology, surgery)
• Dilatation, Pathologic (congenital, diagnosis)
• Female
• Humans
• Infant, Newborn
• Persistent Fetal Circulation Syndrome (physiopathology, therapy)
• Pulmonary Alveoli (pathology)
• Radiography
• Treatment Outcome