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. 2025 Jan;13(1):205-210.
doi: 10.1007/s43390-024-00963-3. Epub 2024 Sep 9.

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

Michael J Gouzoulis  1 , Sahir S Jabbouri  1 , Anthony E Seddio  1 , Jonathan N Grauer  1 , Dominick A Tuason  2
Affiliations

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

Michael J Gouzoulis et al. Spine Deform. 2025 Jan.

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.

Keywords: Adolescent idiopathic scoliosis; Goldenhar syndrome; Reoperation; Respiratory; Revision; Scoliosis.

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Conflict of interest statement

Declarations. Conflict of interest: MJG: James G. Hirsch Medical Student Fellowship at Yale University School of Medicine, SSJ: no disclosures; AES: no disclosures; JNG: Editor in Chief of North American Spine Society Journal, Board Member of North American Spine Society; DAT: Depuy, A Johnson & Johnson Company: Paid Consultant, OrthoPediatrics: Paid Consultant, Paid presenter or speaker. Pediatric Orthopaedic Society of North America: Board or Committee member. Scoliosis Research Society: Board committee member. Ethical approval: (include appropriate approvals or waivers): Our Institutional Review Board has determined this study to be exempt from requiring review. Consent to participate: Not applicable. Consent for publication: Not applicable.

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