Background Secondary surgery for raised intracranial pressure (ICP) is a recognised late complication of craniosynostosis correction. Existing literature combines syndromic, multi-suture and mixed indications, obscuring risk estimates for isolated, single-suture, non-syndromic disease, and no consensus definition of clinically significant raised ICP exists. Methods PubMed was systematically searched to identify studies reporting the incidence, diagnosis, timing, management or outcomes of secondary surgery for raised ICP in isolated single-suture non-syndromic craniosynostosis after primary correction. Given substantial heterogeneity across studies, findings were synthesised descriptively rather than by formal meta-analysis. Results Twenty-seven studies met the eligibility criteria (1998–2026), predominantly retrospective single-centre cohorts. Among cohorts with a reliable denominator, the proportion undergoing secondary surgery ranged from 0.5% to 8.5%. Routine postoperative surveillance was reported in 17 of 27 studies (63%); the remainder relied on symptom-triggered assessment. Secondary surgery typically occurred years after primary correction, with reported medians of 17 months to 6 years and individual patients presenting up to 14 years later. Papilloedema was the most consistently reported objective sign but was absent in a substantial proportion of confirmed cases, and no diagnostic threshold for raised ICP was applied consistently. Secondary surgery most often comprised further cranial vault expansion or remodelling, generally with symptomatic improvement, although long-term outcome data were limited. Conclusions Secondary surgery for raised ICP after correction of isolated single-suture non-syndromic craniosynostosis is uncommon but represents a genuine long-term risk. Surveillance and diagnostic practice remain highly heterogeneous. Standardised diagnostic criteria and prospective multicentre studies with extended follow-up are needed to enable risk-stratified surveillance.
Craniosynostosis is the premature fusion of one or more cranial sutures and affects approximately 1 in 2,000-2,500 live births. Although traditionally considered a craniofacial difference, pediatric neurologists are frequently among the first subspecialists to evaluate infants with abnormal head shape, developmental concerns, or symptoms of elevated intracranial pressure. Early recognition is critical, as delayed diagnosis may preclude minimally invasive surgical options, delay neurodevelopmental surveillance, and contribute to health care disparities. This narrative review provides a practical, diagnosis-focused overview of craniosynostosis for pediatric neurologists. This work briefly summarizes the pathophysiologic basis of craniosynostosis, characteristic cranial phenotypes associated with individual sutures, and distinguishing features of common syndromes to aid in diagnosis. The authors emphasize the importance of systematic history taking and physical examination, discuss the strengths and limitations of contemporary imaging modalities, and review common diagnostic mimics, including positional plagiocephaly. In the closing sections, the neurological complications of craniosynostosis, including intracranial hypertension, visual morbidity, and neurodevelopmental impairment are discussed, which underscore the need for prompt recognition and multidisciplinary evaluation. By emphasizing a structured diagnostic approach and appropriate referral pathways, this review aims to equip pediatric neurologists with the knowledge necessary to facilitate early diagnosis, minimize unnecessary imaging, and optimize outcomes for children with craniosynostosis.
Craniosynostosis, the premature fusion of one or more cranial sutures, affects approximately 1 in 2,000 to 2,500 live births and may impact brain development, resulting in cognitive, behavioral, and psychosocial sequelae. This narrative review synthesizes the current literature on neuropsychological outcomes in craniosynostosis and addresses outcomes by suture type, syndromic status, psychiatric and behavioral comorbidities, quality of life, risk and protective factors, and approaches to neuropsychological screening. Outcomes among youth with isolated, single-suture craniosynostosis tend to be favorable relative to youth with multisuture and/or syndromic craniosynostosis. In single-suture craniosynostosis, overall intellectual functioning is generally within the average range and strongest in sagittal suture, followed by metopic and unicoronal suture, and least well developed in lambdoid based on available data. Among syndromic forms of craniosynostosis, Apert syndrome generally confers the greatest risk for difficulties with overall intellectual functioning, and highly variable outcomes are seen across syndromes, though studies are often limited by small samples. Frequently impacted domains of functioning include attention, executive functions, processing speed, language, and academic learning. The most consistent protective factors for cognitive outcomes are sociodemographic factors, including parental education, socioeconomic status, and access to private insurance. There is variable support for biological and treatment-related risk factors, including elevated intracranial pressure and timing of surgical interventions. Given variability in outcomes, neuropsychological screening is recommended to provide individualized care. Future research including larger and more clinically homogenous samples is needed to better understand risk and protective factors and biological mechanisms underlying these variable neuropsychological outcomes.
