Delayed diagnosis of scaphocephaly after 1 year of age is associated with elevated intracranial pressure

Purpose Delayed presentation of scaphocephaly remains clinically challenging, particularly regarding the risk of elevated intracranial pressure (ICP) and the management of late surgical intervention. This study evaluated the prevalence of elevated ICP in children with delayed diagnosis and the effect of decompressive cranial vault surgery. Methods A retrospective single-center analysis included five patients with delayed diagnosis (May 2022–January 2024) and three infants treated early as a reference group. Clinical characteristics, cranial index (CI), ophthalmologic findings, and intraoperative ICP were analyzed. All delayed cases underwent modified Pi-plasty with systematic ICP monitoring. Results The delayed group had a mean CI of 0.64, compared with 0.68 in the early group. Papillary edema was present in two delayed cases and absent in controls. Mean opening ICP in delayed patients was 24 mmHg (range 16–36), significantly higher than 5 mmHg in early-treated patients ( p < 0.01). Following surgery, ICP decreased to a mean of 7.5 mmHg. Conclusion All patients with delayed diagnosis and treatment of scaphocephaly showed elevated ICP in this study. Decompressive cranial vault surgery effectively normalized ICP, highlighting the importance of early diagnosis and timely intervention to reduce the risk of intracranial hypertension.