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Page 6 / Case 11.02

On day 11 of life he was taken to operating room for diaphragmatic plication. On day 15 of life was extubated for the third time. Again he had respiratory distress and stridor. CPAP and heliox were tried for 1 day with no improvement. On day 16 his PCO2 was 63 mmHg, so he was reintubated for fourth time.

Clinical Progress
On day 26 a second bronchoscopy was performed which showed normal airways. After extubation the vocal cords had normal movements, with mild laryngomalacia. The baby was put on CPAP again for stridor and high PCO2. Day 28 he had deterioration with PCO2 of 81. The chest radiograph showed a complete "white-out" of the right lung. Bi-PAP noninvasive ventilation was initiated. He remained on ventilatory support (BIPAP), not tolerating CPAP.

Further Investigations
Neurology assessment. o MRI of the brain and spine was normal. o Peripheral nerve conduction, EMG and CK were normal. o Right phrenic nerve conduction was inconclusive Genetics: no specific diagnosis

Fluoroscopy of the diaphragm after plication showed: poor movement on the left and no movement of the right while on and off CPAP. Ultrasound confirmd this.

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