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Medical Cases

March 2016

Star Imaging and Research Centre Medical case list

HISTORY

Case Courtesy:

Dr. Sachin Shah

2.5 year old child presented with cough and difficulty in breathing

Chest X-ray suggestive of right upper lobe collapse with hyperinflation of left lung and ipsilateral mediastinal shift

Bronchoscopy was done in view of suspicion of foreign body. No foreign body was found. Mucus plug noted in right upper lobe bronchus. Child was discharged

Child presented with similar complaints after 1 month

CT was suggested in view of abnormal persistent findings on X-ray

FINDING & CONCLUSION

CT findings

Right upper lobe is hypoplastic
Rudimentary bronchus for right upper lobe
Right main pulmonary artery is reduced left side. No upper lobe branch of the right pulmonary artery is seen representing aplasia
There is anomalous drainage of the right inferior pulmonary vein into the infradiaphragmatic IVC
Anomolous arterial supply to the right lower lobe from the hypertrophied right phrenic artery

DIAGNOSIS

Pulmonary venolobar syndrome also known as Scimitar Syndrome or Hypogenetic Lung Syndrome

Key points

It is differentiated from sequestration because tracheobronchial tree is still connected
2D Echo should be done to rule out associated congenital heart disease like ASD, VSD, PDA, TOF. Often patient present with cardiac symptoms
The presence of a left-to-right shunt may lead to development of pulmonary hypertension and Eisenmenger physiology
Plain film may not always show Turkish sword (scimitar) along right cardiac margin

Management

Medical management in respiratory infections
Surgical correction should be considered if significant left to right shunt and development of pulmonary hypertension
Intermittent follow-up to assess right heart size and pressures and cardiac function and rhythm is necessary in patients who did not undergo surgical treatment