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Joseph Junewick, MD FACR
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Congenital Diaphragmatic Hernia

Case Detail

Anatomy: Chest
Junewick
Joseph Junewick, MD FACR
Diagnostic Category: Developmental or Congenital
Created: over 5 years ago
Updated: over 5 years ago
Tags: PEDS
Modality/Study Types: CR FL
Activities:
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History

Newborn with respiratory distress


Case Images


Diagnosis

Congenital Diaphragmatic Hernia

Findings

CR – Air-filled bowel loops in the left hemithorax with contralateral mediastinal shift.

Discussion

Most congenital diaphragmatic hernias occur through the posterolateral diaphragm (Bochdalek). Bochdalek hernias are thought to arise from malformation of the pleuroperitoneal fold or failure or delay of the pleuroperitoneal fold and septum transversum to fuse properly with intercostal muscles. Most congenital diaphragmatic hernias are diagnosed at antenatally by US but some are not diagnosed until birth or later with respiratory distress. Most Bochdalek hernias occur on the left side.

CDH is associated with respiratory distress. Although most believe that herniation of abdominal viscera causes pulmonary hypoplasia due to compression during a critical period of lung development, abnormal pulmonary vasculature with a hypermuscular vessel wall and increased vasoreactivity resulting in pulmonary hypertension also contributes.

Associated anomalies are seen in up to half of patients with CDH. These include cardiac defects (VSD, ASD, and Tetrolgy of Fallot), limb anomalies, genetic defects (Trisomy 18 and 21, Tetrasomy 12p), neural tube defects, hydrocephalus, cryptorchidism, cleft palate, and esophageal atresia. The differential diagnosis is for CDH includes bronchopulmonary foregut malformation, sequestration, congenital cystic adenomatoid malformation, and pulmonary agenesis or hypoplasia.

Reference

Chavhan GB, Babyn PS, Cohen RA et al. Multimodality imaging of the pediatric diaphragm: Anatomy and pathologic conditions. Radiographics (2010); 30: 1797-1817.



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