Thursday, September 27, 2007

Pierre Robin Syndrome with Cleft Palate, Aspiration Pneumonia and Partial Intestinal Obstruction

In infant of 4 months presented with cough, respiratoy distress, abdominal distension and constipation.

He was deliverd normally. Then in the first week he developed Pneumonia for which he was admitted in the hospital. He had occassional asphyxia and advised to be kept in prone position.

On examination, he was ill-looking and dysponeac and found to have micrognathia, high arched palate and posterior cleft palate. His growth was compromised. Abdomen was distended and hypertympanic. He had no neurological sign.

P/R examination was normal.

For the abdominal distenstion and constipation, initially Hirschsprug was suspected. But as the suction biopsy was found normal and abdominal distension was increasing our surgical collegue did the laparotomy and found a fibrous band extended from the meckl’e diverticulum to the mesentry.

The band was released and the distension gradually decreased.

The patient was finally lebelled as Pierre Robin Syndrome with Cleft Palate, Aspiration Pnemonia and Partial Intestinal Obstruction due to fibrous band.

The patient was recovered with antibiotic, surgery and other supportive measures and discharged.

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