Anesthesia and perioperative medical management of children with spinal muscular atrophy

Graham, Robert J.; Athiraman, Umeshkumar; Laubach, Anjolie E.; Sethna, Navil F.

doi:10.1111/j.1460-9592.2009.03055.x

Cited by 125 publications

(68 citation statements)

References 40 publications

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“…SMA patients have an increased risk for postanesthesia complications, which may lead to prolonged intubation, nosocomial infections, tracheotomy, and even death 63. Upper-airway obstruction during endotracheal intubation, hypoventilation and atelectasis from impaired cough, and mucociliary clearance due to anesthetic agents are possible perioperative complications that raise the need for extended preoperative evaluation of respiratory status and pulmonary situation 63.…”

Section: Perioperative Managementmentioning

confidence: 99%

“…Upper-airway obstruction during endotracheal intubation, hypoventilation and atelectasis from impaired cough, and mucociliary clearance due to anesthetic agents are possible perioperative complications that raise the need for extended preoperative evaluation of respiratory status and pulmonary situation 63. Preoperative care should include nutritional assessment as well as investigation of gastroesophageal function 64.…”

Section: Perioperative Managementmentioning

confidence: 99%

“…The consensus statement52 recommends the measurement of respiratory function and cough effectiveness in addition to physical examination and chest X-ray. Nocturnal respiration and sleep disturbances are additional risk factors; noninvasive ventilation and cough assistance can be indicated prior to surgery, and anesthesia should be performed by an experienced pediatric anesthesiologist because of frequent difficult intubation in almost two thirds of these patients 63. However, intraoperative anesthesia complications remain very low, and postoperative complications are mostly related to the individual’s respiratory status 63.…”

Section: Perioperative Managementmentioning

confidence: 99%

“…Nocturnal respiration and sleep disturbances are additional risk factors; noninvasive ventilation and cough assistance can be indicated prior to surgery, and anesthesia should be performed by an experienced pediatric anesthesiologist because of frequent difficult intubation in almost two thirds of these patients 63. However, intraoperative anesthesia complications remain very low, and postoperative complications are mostly related to the individual’s respiratory status 63. Perioperative care should be provided at a multidisciplinary pediatric center with dedicated pediatric anesthesia and postoperative intensive care capacity 52,63…”

Section: Perioperative Managementmentioning

confidence: 99%

“…However, intraoperative anesthesia complications remain very low, and postoperative complications are mostly related to the individual’s respiratory status 63. Perioperative care should be provided at a multidisciplinary pediatric center with dedicated pediatric anesthesia and postoperative intensive care capacity 52,63…”

Section: Perioperative Managementmentioning

confidence: 99%

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Proximal spinal muscular atrophy: current orthopedic perspective

Haaker¹,

Fujak²

2013

TACG

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Spinal muscular atrophy (SMA) is a hereditary neuromuscular disease of lower motor neurons that is caused by a defective “survival motor neuron” (SMN) protein that is mainly associated with proximal progressive muscle weakness and atrophy. Although SMA involves a wide range of disease severity and a high mortality and morbidity rate, recent advances in multidisciplinary supportive care have enhanced quality of life and life expectancy. Active research for possible treatment options has become possible since the disease-causing gene defect was identified in 1995. Nevertheless, a causal therapy is not available at present, and therapeutic management of SMA remains challenging; the prolonged survival is increasing, especially orthopedic, respiratory and nutritive problems. This review focuses on orthopedic management of the disease, with discussion of key aspects that include scoliosis, muscular contractures, hip joint disorders, fractures, technical devices, and a comparative approach of conservative and surgical treatment. Also emphasized are associated complications including respiratory involvement, perioperative care and anesthesia, nutrition problems, and rehabilitation. The SMA disease course can be greatly improved with adequate therapy with established orthopedic procedures in a multidisciplinary therapeutic approach.

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Section: Perioperative Managementmentioning

confidence: 99%

Section: Perioperative Managementmentioning

confidence: 99%

Section: Perioperative Managementmentioning

confidence: 99%

Section: Perioperative Managementmentioning

confidence: 99%

Section: Perioperative Managementmentioning

confidence: 99%

See 3 more Smart Citations

Proximal spinal muscular atrophy: current orthopedic perspective

Haaker¹,

Fujak²

2013

TACG

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A case series of paediatric patients with spinal muscular atrophy type I undergoing scoliosis correction surgery

Holmes

O’Brien

2021

Anaesthesia Reports

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Spinal muscular atrophy is a neuromuscular disorder with degeneration of spinal motor neurons. Type I is a severe variant that was recently shown to be amenable to treatment with the antisense oligonucleotide nusinersen. As a result of increased life expectancy with this treatment, more children with spinal muscular atrophy type I are presenting for spinal correction surgery. In this case series, we present four such patients who underwent spinal surgery at our institution over the course of one year. Pre-operative assessment showed evidence of reduced respiratory function requiring nocturnal non-invasive ventilation in all four patients. A difficult airway was encountered in two of the four patients. Postoperative complications were ubiquitous and included CSF leak, poor wound healing, metal frame exposure, frame instability and wound infection. There were no postoperative respiratory complications and all four children returned to their respiratory baseline postoperatively. All patients underwent successful lumbar puncture and intrathecal nusinersen injection following their spinal surgeries. Given the risk of complications and prolonged recovery following spinal surgery, a detailed family discussion is advisable.

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