2023 FSA Podium and Poster Abstracts
P004: SPINAL ANESTHESIA FOR A 3-MONTH-OLD WITH OSTEOGENESIS IMPERFECTA AND CERVICAL INSTABILITY
Taimoor A Khan1; Jacquelin Peck2; 1Memorial Healthcare System; 2Joe DiMaggio Children's Hospital
Background: Osteogenesis imperfecta (OI) is a rare genetically heterogeneous skeletal dysplasia disorder. Patients with OI are at increased risk for surgical and anesthetic complications. Challenges include securing a airway with potential cervical instability; the possibility of limb fractures during IV tourniquet placement, NIBP readings, or patient positioning; and possible bleeding diathesis (1). Neuraxial analgesia is an appealing alternative but has historically been relatively contraindicated in patients with OI because of concerns for bleeding and damage to the vertebral body (2,3). However, recent literature has demonstrated that regional anesthesia techniques can be safely performed in this population (4). This case highlights the use of neuraxial anesthesia in a 3-month-old male with autosomal dominant, type 3 OI.
Case: A 3-month-old 4 kg male with OI type 3 presented for surgical repair of bilateral bowel-containing inguinal hernias and a circumcision prior to hospital discharge. The patient was born via an uncomplicated c-section at 38 weeks and was admitted to the NICU for close observation given his diagnosis of OI type 3. A skeletal survey noted long bone dysplasia, subacute fractures of the ulnas and right femur, and prominence of the anterior atlantoaxial interval. Platelet count and function were within normal limits and the patient had no clinical signs of bleeding diathesis such as easy bruising or oral bleeding.
After careful positioning, spinal anesthesia with 1mg/kg 0.5% bupivacaine with an epinephrine wash was performed in the sitting position. An IV was placed in the lower extremity and a caudal block with 4.5cc 0.125% bupivacaine was performed in the right lateral decubitus position. Blood pressure readings were deferred due to risk of fracture, monitoring was completed with pulse oximetry, EKG, and mental status. After 60 mins, the patient began to demonstrate signs of mild discomfort and was soothed with cartoon entertainment via iPhone. Approximately 90 minutes after regional anesthesia, the patient occasionally seemed uncomfortable and was given mask inhalation N2O. The surgeon performed a penile block to finish the repair.
Conclusion: Caring for patients with Osteogenesis Imperfecta is complex. A 2017 best practices recommendation for patients with skeletal dysplasia recommended that ‘epidural anesthesia should not be used in patients with skeletal dysplasia.’ However, this multidisciplinary panel recommendation was based on two reports of postoperative paralysis in patients with mucopolysaccharidosis. The incidence and risk of spinal stenosis vary greatly between different forms of skeletal dysplastic diseases and a narrative review did not find any reports of spinal stenosis or paralysis in patients with OI who received neuraxial anesthesia(4). This case highlights the safe use of neuraxial anesthesia in a patient with OI that was high risk for general anesthesia.
References:
1. Rothschild L. Pediatr Anesth. 2018;28(11):1050-1058.
2. White KK. Am J Med Genet A. 2017;173(10):2584-2595.
3. Persiani P. J Pediatr Orthop B. 2018;27(4):338-343.
4. Beethe AR. Reg Anesth Pain Med. 2020;45(12):993-999.