Pediatric anesthesia & critical care

Perioperative management and outcomes for posterior spinal fusion in patients with Friedreich ataxia: A single‐center, retrospective study

Paediatr AnaesthPublished Retrospective case series

Authors' conclusion

Patients with Friedreich ataxia are at high risk for perioperative complications when undergoing posterior spinal fusion and coordinated multidisciplinary care is required at each stage.

Study context

Population
Adolescents with Friedreich ataxia undergoing posterior spinal fusion, Children's Hospital of Philadelphia, 2007–2023included in final analysis
Participants
17 adolescents
Outcome
Perioperative management and outcomes
Methodological detailssingle-centre
  • Single-centre

Main finding

As reported in the abstract

Intraoperative TEE monitoring
53%
Changes in volume status on echo, no changes in function
12%
Wake-up tests (poor baseline neuromonitoring signals in 4, signal loss in 1)
4 (24%)
Extubated in the operating room
75%
Postoperative complications
88%

Observational data: associations, not effects.

Further findings

Preoperative characteristics

Age, mean ± SD (years)
15 ± 2
Female
47%
Wheelchair dependent
35%
Mild-to-moderate hypertrophic cardiomyopathy, preserved systolic function, no LV outflow tract obstruction
100%
On cardiac medications
29%
On pain medications
29%

Anesthetic agents

Numerous TIVA combinations; most commonly propofol, remifentanil and ketamine

Safety

Any postoperative complication
88%
Nausea/vomiting
18%
Hypotension/tachycardia
29%
ECMO support
6%
  • ECMO in one patient

Original publication

Perioperative management and outcomes for posterior spinal fusion in patients with Friedreich ataxia: A single‐center, retrospective study. Paediatr Anaesth. Published 24 April 2024. doi:10.1111/pan.14896

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