There are multiple options available for the surgical management of non-syndromic scaphocephaly. The goals of surgery are to correct the cranial deformity in order to optimize overall cosmetic appearance and to prevent raised intracranial pressure (ICP). At Alder Hey Children's Hospital NHS Trust, we offer a range of surgical procedures for single-suture scaphocephaly (SSS) from endoscopic strip craniectomy (ESC) with helmet therapy, sagittal suturectomy, and barrel staving - known as passive vault remodeling (PassiveVR), to an active subtotal (SVR) or total vault remodeling (TVR). We conducted a retrospective analysis of all operated scaphocephaly patients at Alder Hey. All consecutive patients from 2012 to 2023 were included. Syndromic or multisuture synostosis cases were excluded. Age, type of surgery, issues of ICP at diagnosis, cephalic index (CI) pre- and postoperative, type and indication for reoperation, and length of follow-up were gathered in the database. Four hundred fifteen patients were included; 200 underwent PassiveVR, 206 underwent SVR/TVR, 8 underwent ESC + helmet, and one was treated with springs. The median follow-up period was 46 months. Eighteen patients underwent treatment due to high ICP related to synostosis, with the median age at surgery being 54 months in this group. Reoperations for intracranial hypertension or persistent abnormal shape were done for 17 patients treated initially with PassiveVR (8.5%), for 4 patients initially treated with SVR/TVR (1.9%), and for 1 patient initially treated with ESC + helmet (12.5%). Postoperative CI was significantly higher than preoperative CI in all operated patients (paired t-testing, p < 0.01). PassiveVR and SVR/TVR are the most common procedures in our centre to treat scaphocephaly, with PassiveVR being performed earlier in life. In less than 10% of PassiveVR cases, reoperation due to increased ICP or abnormal head shape is required, with the benefit of PassiveVR being less invasive than SVR/TVR. Our experience with ESC is limited at present, but preliminary findings from this study suggest the need for long-term follow-up in order to identify early evidence of raised ICP or persistent deformity in this patient subgroup. Craniosynostosis occurs due to the premature in-utero fusion of one or more cranial sutures. These sutures allow for passage through the birth canal and, later in development, allow the expansion and growth of the underlying brain. When these sutures close prematurely, the head shape becomes altered depending on the sutures involved. Most cases involve a single suture and are classified as non-syndromic. However, Crouzon, Pfeiffer, and Apert syndromes have been associated with multi-sutural craniosynostosis. The treatment of craniosynostosis involves surgical intervention to unlock the fused sutures, ensuring unrestricted brain development and correcting cosmetic deformities.

Papilledema is swelling of the optic disk due to fluid accumulation around the optic nerve and is commonly used to detect increased intracranial (ICP) in craniosynostosis. This study aimed to assess the prevalence and utility of papilledema detection in patients with single-suture, multi-suture, and syndromic craniosynostosis and the differences before and after surgical intervention. A retrospective review of ophthalmology examinations of patients diagnosed with craniosynostosis treated between 2008 and 2023 was performed. Demographics, diagnosis, and operative data were collected. A total of 1509 ophthalmology exams of 519 patients were reviewed. The overall rate of papilledema in patients with craniosynostosis was 2.12%. The single-suture and multi-suture involvement rates were 1.15% and 5.81%, respectively. Non-syndromic patients with preoperative and postoperative examinations had an overall rate of 3.13%. Most had single suture involvement, and the average age of papilledema examination was 72 months. Syndromic patients with preoperative and postoperative examinations had an overall rate of 17.65%. The majority had multiple suture involvement, and the average age of papilledema examination was 26 months. The overall papilledema detection rate was low in patients with craniosynostosis. Multi-suture involvement and syndromic diagnosis had increased rates of papilledema. The average age of diagnosis was in older children. Syndromic patients had papilledema examination at a younger age. The authors' findings support the concept that fundoscopic ophthalmologic examination will benefit patients with multi-suture involvement, an associated syndrome, and older age.

ObjectiveWe assessed the prevalence of guardian-reported reading difficulties in children treated for non-syndromic craniosynostosis (NSC) and in relation to the location of the closed suture. Additionally, we determined correlations with guardian-reported neurodevelopmental disorders (NDDs) and associations between reported reading ability, sex, timing of surgical intervention, parental education, and heredity of reading difficulties in guardians.DesignCross-sectional, prospective, population-based study.SettingTertiary hospital.ParticipantsThe cohort included 127 participants (aged 9-10 years) treated for NSC (76 sagittal, 38 metopic, 9 unicoronal, and 1 each of bicoronal, lambdoid, frontosphenoidal, and multisuture NSC).Main outcome measuresThe prevalence of guardian-reported reading difficulties was defined as a score ≥8 out of 14 points on the Short Dyslexia Scale. We determined associations between reported reading ability and reported NDDs through binomial regression analysis.ResultsThe cohort demonstrated a 14.2% risk of of guardian-reported reading difficulties, with sagittal, metopic, and unicoronal NSCs exhibiting respective prevalences of 10.5%, 18.4%, and 23.1%. NDDs were reported in 22% of participants and significantly associated with reading difficulties.ConclusionsThe results indicated that participants with sagittal NSC showed the lowest risk of reading difficulties. Additionally, we found that NDDs represent significant risk factors for guardian-reported reading difficulties across the patient cohort. These findings underscore the need for targeted screening and support, especially for patients with metopic and unicoronal NSC, to guide clinical and educational decisions.

Craniosynostosis is a prevalent craniofacial malformation in Finland; however, comprehensive population-based epidemiological data are limited. This study aimed to estimate the total and birth prevalence of craniosynostosis in Finland from 1987 to 2010 and examine temporal trends. We collected the data from nationwide registers maintained by the Finnish Institute for Health and Welfare and Statistics Finland, as well as treating hospitals, encompassing live births, stillbirths, terminations for fetal anomalies, and infant deaths with suspected or diagnosed craniosynostosis or skull deformation. A craniofacial surgeon and a clinical geneticist reviewed 1878 medical records for diagnostic confirmation. Out of 877 craniosynostosis cases, 83% were single-suture synostoses (all live births), 10% craniosynostosis syndromes, and 7% multisutural non-syndromic synostoses. Live birth prevalence from 1987 to 2010 was 6.0/10,000 live births, ranging from 5.0/10,000 in 1987 to 7.5/10,000 in 2010. Total prevalence, including live births, stillbirths, and terminations, varied from 5.0/10,000 in 1987 to 8.0/10,000 in 2010. Sagittal synostosis was the most common synostosis, with a prevalence of 3.9/10,000 live births, followed by metopic (0.6/10,000), unicoronal (0.4/10,000), and unilambdoid (0.1/10,000) synostoses. The total combined prevalence of all craniosynostosis types significantly increased driven by a nonsignificant rise across all subgroups and a significant increase in the syndrome group. In live births increase was significant only within the syndrome subgroup, primarily due to an increase in Muenke syndrome patients. The rising prevalence of syndromes necessitates further investigation. Contrasting with trends in Europe, Australia, and the USA, Finland showed no significant increase in metopic craniosynostosis.

This study aimed to determine the cognitive profile of preschool children undergoing surgery to correct non-syndromic craniosynostosis, compare them with typically developing children, and analyze possible cognitive deficits in the most prevalent subtypes: sagittal and unicoronal. Thirty-one children aged 3 years to 5 years and 11 months with non-syndromic craniosynostosis (11 sagittal, 9 unicoronal, 4 metopic, 3 lambdoid, 4 multisutural) who underwent surgery were compared with thirty-one typically developing children. The Wechsler Preschool and Primary Scale of Intelligence-Third Edition (WPPSI-III) was used to assess cognitive function. Children with non-syndromic craniosynostosis scored below the typically developing children in the Verbal Intelligence Quotient (VIQ) and Full-Scale Intelligence Quotient (FISQ). When specific subtypes were compared, children with sagittal synostosis scored similarly to the typically developing children; in contrast, children with unicoronal synostosis had lower performance in the Processing Speed Quotient and FISQ. The proportion of participants scoring below one standard deviation on the VIQ, General Language Composite, and FISQ was greater in the non-syndromic craniosynostosis group. This study supports the finding that children with non-syndromic craniosynostosis, particularly those with unicoronal synostosis, have more cognitive difficulties than those with normal development. Assessing cognition at preschool age in children with non-syndromic craniosynostosis is important in order to detect difficulties before they become more apparent at school age.