Pulmonary hypoplasia with esophageal bronchus

Case contributed by Dr Hidayatullah Hamidi

Presentation

Recurrent chest infection, respiratory distress.

Patient Data

Age: 1 year
Gender: Female

Hypoplastic right lung with mediastinal shift towards right side.
Non-visualization of right main bronchus.
Esophageal bronchus is seen to hypoplastic/collapsed right lung lower lobe.
Hypoplastic right pulmonary artery (3 mm in diameter) and hypoplastic right pulmonary vein.

Left lung is hyper inflated and herniated to right side. No consolidation or nodule in left lung.
Sub-segmental collapse in the left lung upper lobe.
Trachea, left main bronchus and left lung bronchial tree are normal.
Cardiac chambers are normal. The pulmonary trunk measures 13 mm in diameter. The left pulmonary artery measures 10 mm. 
Normal left pulmonary veins are seen.
NG tube in situ.

Absent right main bronchus and right esophageal bronchus are depicted.

* NG tube.

The patient underwent resection surgery of the hypoplastic right lung

X-ray taken 10 months after surgery shows:

  • complete mediastinal shift to right hemithorax
  • clear left lung

Case Discussion

A rare combination of two rare anomalies:

  • hypoplasia of right lung with absent right main bronchus
  • esophageal bronchus of hypoplastic right lung lower lobe (esophageal lung)

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