Presumptive intrahepatic congenital cystic adenomatoid malformation

B. Keegan Markhardt, Diana L Farmer, Roy A. Filly

Research output: Contribution to journalArticle

1 Scopus citations

Abstract

Congenital cystic adenomatoid malformation (CCAM) and extralobar pulmonary sequestration (EPS) are 2 rare types of bronchopulmonary malformations that typically appear in infancy and childhood. A CCAM is a hamartomatous lesion of the lung, thought to be the result of the cessation of bronchiolar maturation with overgrowth of mesenchymal elements. An EPS is a discrete mass of lung tissues separate from the tracheobronchial tree and is supplied by a systemic vascular source (usually aortic). As well, it forms outside the normal pleural investment. Conran and Stocker1 analyzed 50 cases of EPS and found that 50% (23 of 46) were associated with a coexistent CCAM. Rare cases of intra-abdominal EPS (IEPS) have been reported in the literature, and in these cases, CCAMs commonly occur within the pulmonary sequesterant.2-11 We present an interesting case of CCAM manifesting within the liver of a patient treated prenatally for a large pulmonary type II CCAM and review the reported cases of IEPS with CCAM.

Original languageEnglish (US)
Pages (from-to)531-536
Number of pages6
JournalJournal of Ultrasound in Medicine
Volume22
Issue number5
StatePublished - May 1 2003

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging
  • Radiological and Ultrasound Technology
  • Acoustics and Ultrasonics

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