Peripapillary Hyperreflective Ovoid Mass-like Structures before and after Intracranial Decompression: A Longitudinal Cohort Study.

Purpose: To appraise the association of peripapillary hyperreflective ovoid mass-like structures (PHOMS) with the need for intracranial pressure (ICP) decompression surgery and to study the impact of decompression surgery on the persistence of PHOMS.

Design: Retrospective, longitudinal cohort study.

Participants: Patients with syndromic craniosynostosis evaluated at Boston Children's Hospital (2010-2023) who had a minimum of 2 consecutive optic nerve volume scans.

Methods: Consecutive OCT volume scans were reviewed by 2 neuro-ophthalmologists to determine the presence of PHOMS. Demographic and ophthalmic features and history of elevated ICP, ICP decompression procedures, presence of Chiari malformation, and obstructive sleep apnea were noted for each patient.

Main outcome measures: Primary outcomes were sensitivity, specificity, positive and negative predictive values, and odds of needing a subsequent ICP decompression procedure given the presence of PHOMS. Secondary outcome was the impact of ICP decompression procedures on persistence of PHOMS.

Results: Median age of 34 patients at first scan was 7.7 years (interquartile range [IQR], 5.1-11.2), and 59% were female. Diagnoses were Apert (n = 7); Crouzon (n = 10); Muenke (n = 3); Pfeiffer (n = 3); and Saethre-Chotzen (n = 11). Median duration of follow-up was 4.2 years (IQR, 1.4-7.7). Sensitivity and specificity of PHOMS as a predictor of having ICP decompression procedure were 85.7% and 70.4%, respectively. The positive predictive value was 42.9%, and negative predictive value was 95.0%. By using logistic regression, the odds ratio of having subsequent ICP decompression surgery was 14.3:1 (95% confidence interval, 1.47-138.3; P = 0.022) in the presence versus the absence of PHOMS. Resolution of PHOMS during the study period was more common in patients who had ICP decompression than in patients who did not (83.3% vs. 0%, P = 0.003). Kaplan-Meier curves comparing those treated and not treated with ICP decompression surgery demonstrated a faster rate of PHOMS resolution with ICP decompression procedure (log-rank test = 7.87, P = 0.005).

Conclusions: Presence of PHOMS was significantly associated with the need for a subsequent ICP decompression procedure. If PHOMS were absent, it was highly unlikely that ICP decompression was indicated. Resolution of PHOMS was highly associated with ICP decompression surgery and might prove to be a convenient noninvasive measure of success.

Financial disclosure(s): The author(s) have no proprietary or commercial interest in any materials discussed in this article.