Anesthesia Management in Rare Case: Osteogenesis Imperfecta

Osteogenesis imperfecta is a rare genetic disorder and a collagen tissue disease for which preoperative preparation and intra-operative anesthesia management must be performed with great care on patients.

An operation was planned for a 5-year old female patient with Type I osteogenesis imperfecta due to a right femoral fracture. Her medical history showed that she had been operated due to a left femoral fracture 5 months ago under sevoflurane inhalation anesthesia without any complications. On her physical examination, she was observed to be a short child with growth deficiency, kyphoscoliosis, and bone and shape deformities on her extremities. Her modified Mallampati score was III and neck extension was limited. Preoperative echocardiography, complete blood cell count, coagulation profile, and biochemical values were found in normal limits. She was taken into the operation theatre and monitorized. Pressure points were supported by silicon peds. Vascular access could not be established at first because of her agitation, and then it was achieved after the patient’s anesthesia induction was performed with sevoflurane. Her neck was kept stable and laryngeal mask was placed in the mouth in the first intervention. Anesthesia was provided through 50% O2 + 50% air and 2% sevoflurane. At the end of the operation that took 90 min, the patient was extubated unevenBtfully, and taken to the recovery room.

Main anesthetic problems in patients with osteogenesis imperfecta are the difficulties in maintaining the airway management and malign hyperthermia. We used sevoflurane both at the induction and at the maintenance of anaesthesia due to the difficult vascular access of the patient, and we did not encounter any problems.

Inhalation anesthesia such as sevoflurane as well as TIVA could be used for the anesthesia for the patients with osteogenesis imperfecta. Great care must be given because of difficult airway in such patients, and necessary precautions must be taken. Laryngeal mask airway could be preferred in order to secure the airway and avoid traumatic complications.