Severe Restrictive Lung Disease with Osteogenesis Imperfecta

A 23 yrs old male presented to the emergency department of Tirumala Medicover hospitals in Vizianagaram on 5th Nov 22 with severe difficulty in breathing, cough, cold and high-grade fever for four days. He was already suffering from osteogenesis imperfecta with kyphoscoliotic deformity. He maintained an O2 saturation of 64% with room air, blood pressure of 90/60 mm hg, temperature of 101 ° F, pulse rate of 146/min regular, and respiratory rate of 34/min. The patient was observed to be using the accessory musculature for his breathing.

The patient was put on 14L of oxygen with a face mask but was not responding properly as the tachypnoea has not come down. Then NIV was tried for which also he was not tolerating and consequent ABG revealed respiratory acidosis with pco2 levels of 88 mm hg and po2 levels of 40 mm hg.

On the 2nd day of admission, the patient was ventilated on acv pc mode. HRCT chest revealed bilateral diffuse ground glass opacities with consolidation. Aggressive medical management was continued. The patient was extubated successfully on the 4th day and had satisfactory parameters PR 100/min, BP 110/70 mm, SPO2 97%, and 40% PO2. He was discharged on 14th Nov 22 with the satisfactory general condition. The relation between the osteogenesis imperfecta and severe restrictive lung disease is that it often becomes very difficult for these types of patients to recover due to the physical deformity of the chest which worsens the respiratory efforts of the patient. The patient recovered successfully with the combined efforts of pulmonologist, ortho surgeon, and critical care physician.