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Peri-operative management of children with spinal muscular atrophy
Matthew A Halanski1, Andrew Steinfeldt2, Rewais Hanna3
1Children's Hospital of Omaha, Omaha, NE, USA.
Insights
Children with spinal muscular atrophy (SMA) types I and II undergoing spinal surgery require careful perioperative management. Anticipate challenges like difficult intubations and extended anesthesia times for optimal outcomes.
Area of Science:
- Pediatric Orthopedics
- Pediatric Anesthesiology
- Neuromuscular Disorders
Background:
- Spinal deformities are common in children with spinal muscular atrophy (SMA).
- Surgical correction is often necessary as part of multi-disciplinary care.
- Perioperative management protocols are crucial for this patient population.
Purpose of the Study:
- To evaluate the perioperative outcomes of children with SMA undergoing spinal surgery.
- To share a neuromuscular perioperative protocol for SMA patients.
Main Methods:
- Retrospective chart review of children with SMA types I and II undergoing spinal deformity correction (1990-2015).
- Assessment of pre-operative, intra-operative, and post-operative variables.
- Statistical analysis using T-tests, Wilcoxon Rank Sum, and Fisher's Exact tests.
Main Results:
- 12 SMA type I and 22 SMA type II patients were analyzed.
- SMA type I patients had higher ASA class 4 (36.4% vs 4.5%) and utilized preoperative total parenteral nutrition (TPN) more frequently (75% vs 18.2%).
- Difficult intubations occurred in ~25% of patients; anesthetic time was ~2 hours longer than surgical time for both types. ICU stay averaged 6 days for type I and 3 days for type II.
Conclusions:
- Children with SMA types I and II experience similar hospital courses.
- Perioperative considerations include TPN, non-invasive positive-pressure ventilation (NIPPV), anticipating difficult intubations, and extended anesthetic/ICU stays.
- A tailored neuromuscular perioperative protocol is recommended for SMA patients undergoing spinal surgery.
Background And Aims:
Current multi-disciplinary management of children with spinal muscular atrophy (SMA) often requires the surgical management of spinal deformities. We present the outcomes of our peri-operative experience around the time of their spinal surgery and share our neuromuscular perioperative protocol.
Methods:
A single-centre retrospective chart review was performed to evaluate all children with SMA types I and II that underwent thoracolumbar spinal deformity correction (posterior spinal fusion or growing rod insertion) from 1990 to 2015. Electronic medical records were reviewed to assess pre-operative, intraoperative, and postoperative variables. T-tests, Wilcoxon Rank Sum, Fisher's Exact tests were performed as appropriate.
Results:
Twelve SMA I and twenty-two SMA II patients were included. Type I patients tended to be smaller and had a higher percentage (36.4% vs 4.5%) of American Society of Anesthesiologists (ASA) class 4 patients. Preoperative total parenteral nutrition (TPN) was utilised in 75.0% of type I and 18.2% type II patients. A difficult intubation was experienced in around 25% of the patients (20.0% SMA I, 27.3% SMA II). Approximately two hours of anaesthetic time was required in addition to the actual surgical time in both types. The intensive care unit (ICU) length of stay averaged 6 (4.0-7.5) days for type I and 3 (3-5) days for type II (p = 0.144). Average post-operative length of stay was (8 (7-9) vs. 7 (6-8)) P = 1.0.
Conclusion:
Children with type I and II SMA have similar hospital courses. The surgical and anaesthesia team should consider perioperative TPN and NIPPV (non-invasive positive-pressure ventilation), anticipate difficult intubations, longer than usual anaesthetic times, and potentially longer ICU stays in both SMA type I and II.
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