LAUSR

Abstract Introduction: Osteogenesis imperfecta (OI), a rare congenital disorder, has a risk of bone fracture and progressive bone deformity. OI type II is the most serious subtype, and very few reports on its anesthetic management exist. Patients face several anesthetic difficulties, of which easy fracturing of OI-affected bones is critical. Herein, we report our experience with the anesthetic management of a patient with OI type II. Patient concerns and diagnoses: Through genetic testing, a 14-month-old girl (height: 45 cm, weight: 3.9 kg) was diagnosed with OI type IIB due to COL1A gene abnormality. The clinical manifestations included hydrocephalus, blue sclera, dental dysplasia, short stature, limb deformity and shortening, thoracic hypoplasia, rabbit eye, left inguinal hernia, and tricuspid valve regurgitation. Physical examination revealed an enlarged head due to skull dysplasia and hydrocephalus. The pediatrician confirmed that mask ventilation was possible even under spontaneous breathing, and that there was no history of bone fracture during mask holding. Interventions and outcomes: The patient was scheduled for gastrostomy and ventriculo-peritoneal shunt implantation. An arterial pressure line was inserted at the neonatal intensive care unit. Propofol and remifentanil were selected for general anesthesia. Confirming that mask-assisted ventilation was possible under sleep, rocuronium was administered. Attentive mask ventilation was performed via the 2-person method to avoid fractures. We were able to intubate successfully using a Macintosh laryngoscope. A microcuff endotracheal tube was used. For ventilation, pressure control ventilation was selected and sedative dosage was adjusted using the patient state index as an indicator. The patient was sedated, intubated, and returned to the neonatal intensive care unit. She was extubated on the sixth postoperative day. No bone fractures were noted. Conclusion: OI type II is the most severe subtype with high mortality, and there are few reports on its anesthetic management. Easy fractures can be a problem in airway maintenance, blood pressure measurement, and repositioning. We performed the procedure attentively, avoiding jaw and cervical fractures during mask ventilation and endotracheal intubation. For respiratory management, we chose pressure control ventilation using a cuffed tracheal tube and circulatory control was attained via an arterial line inserted preoperatively. No complications occurred.