ICP risk rare in metopic synostosis regardless of treatment

Increased intracranial pressure (ICP) is rare in patients with metopic synostosis, with no significant differences between surgical and conservative groups, according to a recent study published in JAMA Network Open.¹

Approximately 1 in 4500 live births are impacted by metopic synostosis, making it the second most common form of craniosynostosis.² Surgical intervention is recommended to correct deformities and prevent further complications, but there is no clear data about the risk of increased ICP in this condition, with a range from 0% to 33% reported.¹

“Avoiding surgery reduces perioperative risk and family burden, but evidence on safety remains limited,” wrote investigators.

Comparison conservative vs surgical management

The prospective cohort study was conducted to compare head growth and signs of increased ICP in pediatric patients undergoing surgical vs conservative management of metopic synostosis. Participants included patients presenting at the study clinic with metopic synostosis from January 1, 2017, to December 31, 2024.

Patients with prior craniofacial surgery were excluded from the analysis, and having multiple head circumference measurements across 2 years of follow-up was necessary for inclusion. Parents were offered to choose between surgical or conservative management for their children’s metopic synostosis.

In the conservative treatment group, patients received a nonsurgical approach with routine follow-up appointment. Those in the surgical group underwent a chosen surgical method based on parental preferences and age at presentation. The 2 option savailable included fronto-orbital advancement and endoscopic-strip craniectomy with helmet therapy.

The former of these options was usually performed in children aged 9 to 12 months, while the latter was usually performed in those aged 3 to 6 months. Children in the conservative group only received surgery if ICP occurred. Follow-up care through the age of 8 years was identical between groups.

Measurement and evaluation criteria

Head growth was reported as the primary outcome based on yearly longitudinal head circumference measurements performed with a measuring tape. Elevated ICP was indicated by deflected or stagnated head growth.

Proxies for ICP were reported as secondary outcomes. These included the presence or absence of both head growth deflection and papilledema. Investigators considered head growth greater than 0.5 standard deviation (SD) across 2 years to be clinically relevant.

The suture-specific metopic photo score was used to grade the initial severity of metopic synostosis. Points on this scale included normal, mild, moderate, and severe.

Patient demographics and head growth trajectories

There were 209 patients aged a median 4 months at presentation included in the analysis, 74% of whom were boys and 26% were girls. Surgical treatment was provided to 69% and 31% of these patients, respectively, with a median age of 9 months reported at surgery.

A median age of 40 months was reported at last follow-up. During the follow-up period, no patients in the conservative group required surgery, and none of their parents asked for surgery for aesthetic reasons.

Investigators observed a nonlinear trajectory with age for head circumference SD. The trajectory significantly differed between groups, at β = 0.37. Greater initial SD values were reported in the surgical group, plateauing at approximately 20 months of age at β = –1.02. In comparison, the conservative group reported a steady increase over time.

Additional growth values and ICP rates

SD values were significantly increased among female vs male patients regardless of treatment method, at β = 0.31. Severe deformities were linked to significantly reduced SD values vs mild deformities, at β = –0.39. However, the difference between moderate and mild deformities was not statistically significant.

Head growth deflection was reported in 3.1% of the surgical group and 1.1% of the conservative group after at least 2 years of follow-up. Deflection rates did not significantly differ between groups, nor did papilledema with rates of 1.4% and 2.8%, respectively. Overall, signs of ICP were rare, with no significant differences between groups.

“These findings support close clinical monitoring as a safe alternative to routine surgery for selected patients and underscore the value of individualized treatment decisions,” wrote investigators.

References

1. Tio PAE, Koehoorn EN, Clement LIF, et al. Head growth and fundoscopy as proxies for intracranial pressure in metopic synostosis treated surgically vs conservatively. JAMA Netw Open. 2026;9(2):e2559871. doi:10.1001/jamanetworkopen.2025.59871
2. Cornelissen M, Ottelander Bd, Rizopoulos D, et al. Increase of prevalence of craniosynostosis. J Craniomaxillofac Surg. 2016;44(9):1273-1279. doi:10.1016/j.jcms.2016.07.007