Case Report: Respiratory management of a successful pregnancy in type II spinal muscular atrophy

Introduction. Anesthetic management of a patient with spinal muscular atrophy type II, who underwent elective cesarean section with neuraxial anesthesia is presented in this case report. Case report. A 33-year old woman with first pregnancy and no previous birth, at 39 weeks gestational age was scheduled for a cesarean section due to placenta previa. She had a history of spinal muscular atrophy type II, that confined her to a wheelchair, and a surgical history that included corrective surgery for kyphoscoliosis. The patient had predictors for a difficult intubation (limited mouth opening and reduced neck extension) so the decision was made to attempt the needle-through-needle combined spinal-epidural technique for surgical anesthesia. Harrington rods and scar tissue complicated placement of the combined spinal-epidural anesthesia, however successful placement was achieved. Conclusion. Spinal muscular atrophy in pregnancy is rare and represents big challenge for an anesthesiologist due to respiratory dysfunction, anticipated difficult intubation, severe kyphoscoliosis and limitations of the use neuromuscular blocking agents. The potential risks need to be considered when administering anesthesia in patients with spinal muscular atrophy undergoing a cesarean section.

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Enhancing Function, Fun and Participation with Assistive Devices, Adaptive Positioning, and Augmented Mobility for Young Children with Infantile-Onset Spinal Muscular Atrophy: A Scoping Review and Illustrative Case Report

Disabilities ◽

10.3390/disabilities1010001 ◽

2021 ◽

Vol 1 (1) ◽

pp. 1-22

Author(s):

Roslyn Livingstone ◽

Ginny Paleg

Keyword(s):

Case Report ◽

Spinal Muscular Atrophy ◽

Scoping Review ◽

Muscular Atrophy ◽

Assistive Devices ◽

Illustrative Case ◽

Medical Interventions ◽

Mobility Function ◽

Dynamic Mobility ◽

Mobility Device

Recent advances in medical interventions have changed the prognosis for children with infantile-onset spinal muscular atrophy (SMA-1); however, little has been published regarding rehabilitation management. A rapid scoping review was conducted in November 2020 using Medline and CINAHL databases. Evidence supporting use of assistive devices and equipment to enhance participation, mobility, function, and posture in lying, sitting, and standing positions was sought. From 239 articles, only five studies (describing use of augmentative communication, manual and power mobility, supported standing and orthotic devices) met inclusion criteria. Results are presented alongside a case report of a 5-year-old boy (treated with Nusinersen since 7 months-of-age) who uses a variety of devices to enhance his activity and participation in family life. While reclined and tilted sitting positions as well as power mobility were previously considered for children with SMA-1, this child has progressed to supported upright standing, self-propelling a lightweight manual wheelchair indoors, communicating using multiple methods and taking steps in a dynamic mobility device. Power mobility was introduced in a switch-adapted cart at 11 months and he was independently exploring indoors and outside in his power wheelchair before 20 months. Research evidence is limited, but alongside the case report highlights the importance of a comprehensive and proactive approach to enhancing function, fun and participation with family and friends through adaptive equipment for children with significant and life-limiting disabilities.

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