Goldenhar syndrome associated with increased risk of respiratory failure and reoperations following spinal deformity surgery.

Abstract

Background: Goldenhar syndrome is a rare congenital disease that presents with a spectrum of clinical sequelae related to the vertebrae and other organs. The spinal manifestations of the syndrome are associated with scoliosis for which fusion may be considered. The current study aimed to evaluate the risks of adverse events and reoperations following posterior spinal fusion for those with Goldenhar syndrome relative to those with adolescent idiopathic scoliosis (AIS).

Methods: Patients with Goldenhar syndrome and AIS between the ages of 10 and 17 who underwent posterior spinal fusion were abstracted from the 2010 to 2022 PearlDiver Database. The Goldenhar syndrome patients were matched 1:4 to patients with AIS based on age, sex, and Elixhauser Comorbidity Index. All 90 day postoperative adverse events, readmissions, and 5 year reoperations were identified using administrative coding. Incidence of adverse events between the cohorts were compared using multivariate logistic regression.

Results: A total of 11,742 patients with AIS and 72 (0.61%) Goldenhar syndrome undergoing deformity surgery were identified. On matched comparison, patients with Goldenhar syndromes had higher odds ratio (OR) of respiratory failure (OR: 2.99, p = 0.009), severe adverse events (p = 2.29, p = 0.01), and readmissions (p = 2.26, p = 0.02). Over 5 years, they had a significantly higher incidence of reoperation compared to those with AIS (18.1% versus 5.5%, p = 0.005).

Conclusions: In this national sample of patients with Goldenhar syndrome undergoing posterior spinal fusion, patients with Goldenhar had increased odds of respiratory failure, readmissions, and reoperations. Targeted risk mitigation strategies may be appropriately considered for those with Goldenhar syndrome undergoing such surgeries.

Level of evidence: Level III; Case-control study or retrospective cohort study.