Dr. Mehta’s Hospitals Successfully Manages Rare and Complex Case of Brittle Bone Disease with Airway, Lung, Spine & Hearing Challenges

2025-08-04 13:32:40
Dr. Mehta’s Hospitals Successfully Manages Rare and Complex Case of Brittle Bone Disease with Airway, Lung, Spine & Hearing Challenges
Dr. Mehta’s Hospitals Successfully Manages Rare and Complex Case of Brittle Bone Disease with Airway, Lung, Spine & Hearing Challenges

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Chennai, July 2025

Osteogenesis imperfecta (OI) is an inherited disease of connective tissue that affects bone, sclera, and the inner ear, caused by the mutations of the collagen type I – COLIA1 and COLIA2 genes. Anaesthesiologists are challenged by difficult airways, short neck, risk of odontoaxial dislocation, cervical vertebrae, mandible, and teeth fractures during laryngoscopy and intubation. These patients may also have cardiac valvular lesions, cor pulmonale, and a restrictive pattern of lung disease.

 

A 61-year-old female known case of Osteogenesis imperfecta (OI) Type 1 presented with a fracture neck of the left femur, and she was posted for left femur screw fixation. On general examination, she was very short-statured (90 cm), weighing 25 kg, with severe kyphoscoliosis, progressive hearing loss. She had blue sclera. Respiratory system (RS) examination revealed barrel shaped chest with bilateral equal. X–ray lumbar spine revealed kyphoscoliosis. Airway examination revealed an anticipated difficult airway. Preoperative pulmonary function test revealed a severe restrictive pattern of disease.

 

Given the risks and difficulties posed by general anaesthesia, the choice of anaesthesia was decided to be real-time ultrasound-guided spinal anaesthesia. Plan B was to give general anaesthesia using awake fibreoptic bronchoscopy.  USG of spine done, and L2-L3 space was found to have the largest interlaminar space, midline identified, skin to intrathecal distance was measured to be 4cm with transverse view. After infiltrating 2% lignocaine, real-time ultrasound-guided oblique in-plane placement of the needle, done with a 25-g Quincke needle, was done to reach the intrathecal space in paragattital view. The perioperative period was uneventful. Patient discharged on the 5th day with no complications.

 

Patients's after opertaion       X-ray scan        Doctor doing opeartion