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Management of Spinal Muscular Atrophy Type 2 on continuous BiPAP during Pregnancy
Abstract Number: S4C-6
Abstract Type: Case Report/Case Series
Spinal muscular atrophy (SMA) is an early onset, AR, neurodegenerative disease with an incidence of 1 in 6,000 - 10,000. It affects the anterior horn of the spinal cord and motor nuclei of the lower brainstem resulting in progressive, proximal muscle weakness and atrophy. Five subtypes are described with the highest mortality seen in Types 0 and 1 (Diagram).
SMA Type 2 is the intermediate form with onset between 6-18 mo with muscle weakness presenting early in childhood. Individuals are often wheelchair dependent and have severe restrictive lung disease (RLD). In this case report, we describe the challenges in managing a periparturient patient with SMA Type 2 starting at 23w6d GA until the time of delivery.
A 27 YO G2P0101 with SMA type 2 at 23w6d GA presented from an OSH with acute respiratory insufficiency due to community acquired pneumonia. Her respiratory support escalated requiring continuous BiPAP from her baseline of O2 3L NC. PMH includes severe RLD (FEV1 15%, FVC 16%) with significant contractures and kyphoscoliosis requiring Harrington rods (T2-L5) from age 11, asthma, malnutrition (BMI 17), chronic anemia (HBG 8.2), and wheelchair dependence. Her prior pregnancy was straightforward with adequate planning with MFM. She received a preinduction a-line, GETA with G1v with CMAC3, and TAPs blocks. She was extubated to BiPAP on POD #0 and transitioned off BiPAP to NC on POD #3.
For this pregnancy, she was transferred to our institution for antepartum management while on BiPAP. Her care required a multidisciplinary team approach. Recommendations included, but not limited to, continuing TPN nutrition while g-tube had stage 3 pressure ulcer, weaning BiPAP to NC, and maintaining Hgb levels above 8-9. Her airway examination was notable for a Mallampati III view.
3 months passed and the pt had her scheduled C/S. However, the airway exam changed starkly, notable for a mallampati IV view while on continuous BiPAP, limited neck flexion and extension, and continued orthopnea. Additionally, she was a poor neuraxial candidate due to her kyphoscoliosis with unpredictable spinal spread in the setting of respiratory insufficiency. She was a difficult airway requiring 4 attempts, exchange of provider hands, and use of 3 modalities for ETT positioning. The final intubation required a Glidescope, Eschman, and fiberoptic scope for ETT confirmation. To expedite extubation and minimize PONV, the pt had a TIVA anesthetic, and bilateral quadratus lumborum blocks were placed by ultrasound guidance. The patient extubated several hours after transfer to ICU, resumed BiPAP use, and discharge home with BiPAP.
Pregnancy in patients with SMA have been described many times. Various anesthetic plans have been implemented including use of spinal, general, and regional anesthesia,. Here we describe the management of a SMA Type II under GETA with a known difficult airway and poor candidate for neuraxial blockade.