Congenital Diaphragmatic Hernia with Liver Herniation into the Pericardial Sac in a 30-Week Gestation Infant.
Pediatr Dev Pathol
; 20(5): 421-425, 2017.
Article
de En
| MEDLINE
| ID: mdl-28812464
ABSTRACT
Anterior diaphragmatic defects with pericardial involvement are extremely rare and diagnostically challenging entities encountered perinatally. While a majority of diaphragmatic defects occur in isolation, others are associated with multiple defects forming a complex of syndromes such as Pentalogy of Cantrell. Liver herniation into the pericardial sac poses a particular challenge and can mimic a pericardial tumor on prenatal ultrasound, yielding a different management course. The following case is an unusual presentation of a 30-week gestation female with an anterior midline diaphragmatic defect with liver herniation mimicking as a pericardial tumor, diagnosed at time of autopsy. Postmortem studies also found multiple congenital anomalies including an atrioventricular septal defect and midline gumline defect suggesting at least a partial Pentalogy of Cantrell or variant. Early recognition and screening for associated anomalies are essential for management in this subset of patients.
Mots clés
Texte intégral:
1
Collection:
01-internacional
Base de données:
MEDLINE
Sujet principal:
Péricarde
/
Malformations multiples
/
Hernies diaphragmatiques congénitales
/
Cardiopathies
/
Maladies du prématuré
/
Maladies du foie
Type d'étude:
Diagnostic_studies
Limites:
Female
/
Humans
/
Newborn
Langue:
En
Journal:
Pediatr Dev Pathol
Sujet du journal:
PATOLOGIA
/
PEDIATRIA
Année:
2017
Type de document:
Article
Pays d'affiliation:
États-Unis d'Amérique