Cantrell syndrome with complex cardiac malformations: a case report

Abstract Cantrell syndrome is a rare condition of congenital defects of 5 developmentally associated structures: the abdominal wall, sternum, diaphragm, pericardium, and heart. Few patients survive, and even fewer survive with good outcomes of quality of life. We present a case with the pentalogy an...

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Bibliographic Details
Published in:Journal of pediatric surgery 2011-07, Vol.46 (7), p.1455-1458
Main Authors: Wen, Li, Liao, Jun-lin, Hao, Jia, Zhang, Dong, Zou, Li-guang, Dai, Shu-hua, Liu, Wei-jin, Gao, Yun-hua
Format: Article
Language:English
Subjects:
Abdominal Wall - surgery
Angiography
Cantrell syndrome
Cardiac Surgical Procedures
Echocardiography
Heart Defects, Congenital - diagnostic imaging
Heart Defects, Congenital - pathology
Heart Defects, Congenital - surgery
Hernia, Umbilical - surgery
Humans
Infant, Newborn
Infant, Small for Gestational Age
Male
Pediatrics
Pentalogy of Cantrell - pathology
Pentalogy of Cantrell - surgery
Quality of Life
Radiography
Reoperation
Surgery
Ultrasonography
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Summary:Abstract Cantrell syndrome is a rare condition of congenital defects of 5 developmentally associated structures: the abdominal wall, sternum, diaphragm, pericardium, and heart. Few patients survive, and even fewer survive with good outcomes of quality of life. We present a case with the pentalogy and profound cardiac malformations. Despite repeated life-threatening conditions, the boy survived to have his first operation at 5 months old. The operation reduced the heart within the thoracoabdominal area, repaired the abdominal wall defect, and released multiple stenoses of the heart and great vessels. The patient recovered uneventfully. At 4.5 years old, he is healthy and developed normally.
ISSN:0022-3468
1531-5037
DOI:10.1016/j.jpedsurg.2011.03.071