Scaphocephaly is the most common form of isolated single-suture craniosynostosis, a condition in which the fibrous joints between the plates of a baby’s skull fuse prematurely. When the sagittal suture, which runs from front to back along the top of the head, closes too early, normal skull growth is restricted perpendicular to that suture. According to Virchow’s law, the skull compensates by expanding along the remaining open sutures, producing the characteristic elongated head shape with bossing of the forehead and back of the skull. Craniosynostosis affects roughly one in 2,000 to 2,500 live births and can occur as part of a genetic syndrome or as an isolated anomaly. Its severity is clinically gauged with the cranial index, or CI, a ratio of skull width to length; healthy children under three years average around 0.81, and none of the late-diagnosed children in this study came close, with a group mean of 0.64.

The danger of a misshapen skull, however, extends well beyond appearance. Premature suture fusion can restrict the space available for the growing brain, and elevated intracranial pressure is a recognized complication, estimated to affect between 7 and 44 percent of children with scaphocephaly. Chronic pressure on the optic nerves can cause papillary edema, the swelling of the nerve head visible on fundoscopy, and in the worst cases progress to atrophy and blindness. Ophthalmologic problems including strabismus, amblyopia, and refractive errors are also more frequent in craniosynostosis, though they occur more often in syndromic forms such as Crouzon and Apert syndromes. Because the condition is rare and presentation varies widely, pinning down the true prevalence of intracranial hypertension has proven difficult, with epidemiological studies reporting widely divergent figures depending on measurement methods, patient age, and the normative thresholds used.

The new study, conducted at the Children’s Hospital of Central Switzerland in Lucerne, focused on a specific and clinically awkward population: children with isolated scaphocephaly who were diagnosed only after their first year of life. Between May 2022 and January 2024, five such patients presented to the institution, three boys and two girls, ranging in age at surgery from 19 to 62 months. The authors compared them with a reference group of three infants, operated on at 5, 6, and 8 months, who underwent standard osteoclastic craniectomy during the same period. Children with syndromic scaphocephaly and those diagnosed and treated in infancy were excluded from the delayed cohort. All five late-diagnosed patients underwent a modified Pi-plasty, a technique originally described by Jane and colleagues, in which the skull is surgically remodeled to relieve the constriction.

The technical centerpiece of the study was systematic intraoperative intracranial pressure monitoring. In every delayed case, after the first burr hole was placed in the right frontal region just in front of the coronal suture, the dura was incised and a Raumedic Neurovent-PX probe was inserted two centimeters into the brain parenchyma and connected to the anesthesia monitoring system. Opening pressures were recorded just before the craniectomy began and closing pressures just before the reconstruction was completed, all under general anesthesia with complete neuromuscular blockade administered by the same anesthesiology team. The researchers defined an intracranial pressure of 15 mmHg or higher under general anesthesia as pathologically elevated, a threshold drawn from prior pediatric craniosynostosis literature rather than any universally accepted pediatric cutoff, which the authors acknowledge does not exist.

The results were striking in their consistency. Every child in the delayed group exceeded the threshold, with opening pressures of 16, 18, 25, 25, and 36 mmHg, a mean of 24 mmHg with a standard deviation of 7.8. In the control group, mean opening pressure was 5 mmHg with a standard deviation of 1, and the difference between groups was statistically significant at p less than 0.01. After cranial vault expansion, closing pressures in the delayed group dropped to a mean of 7.5 mmHg. Yet the clinical signs of hypertension were far subtler than the measurements: only two of the five late-diagnosed children had papillary edema on fundoscopy, and none of the infants did. All five delayed cases, however, showed a copper-beaten skull appearance on x-ray, an indirect radiological sign of chronic intracranial hypertension.

The individual case histories illustrate how easily the diagnosis can slip through the net. One five-year-old boy had persistent papillary edema of unknown origin for more than two years, and a lumbar puncture by pediatric neurologists had failed to detect raised pressure before the scaphocephalic head shape was finally recognized. An eighteen-month-old girl arrived after repeated parental concerns about her head shape, and a three-year-old girl was brought in because her brother had just been diagnosed, her workup additionally revealing a Chiari type 1 malformation. Perhaps most telling was a boy whose scaphocephaly had been suspected prenatally and evaluated in infancy, but whose parents initially refused surgery; by 27 months, with a cranial index of 0.62 and a copper-beaten skull on x-ray, the family consented, and his opening pressure proved to be 25 mmHg.

Follow-up, extending through 2026, brought encouraging news. Papillary edema resolved completely in both affected children and did not recur over two and three years of follow-up respectively. One child showed a notable leap in overall and language development within three months of surgery, though the authors are careful to note this as an observation rather than proof of causal neurocognitive benefit. Complications were limited to a minor wound dehiscence in one child that healed without revision surgery. The Chiari malformation in the fourth case remained unchanged, with no hydrocephalus at six and twelve months. No patient required reoperation, and all remain under active follow-up.

The findings sit within a broader and still contested literature on timing. Previous studies suggest the prevalence of intracranial hypertension rises with age in unoperated single-suture craniosynostosis: Arnaud and colleagues reported 26 percent in children over one year versus 7 percent in younger infants, and Tuite’s group found that children with papillary edema and raised pressure were older on average than those without. On neurocognitive outcomes the evidence is genuinely mixed. Some studies, including work by Patel and by Bellew, found higher full-scale and verbal IQs in children operated on before six or seven months, but Starr and colleagues found no strong association between age at repair and development at three years, and a systematic review split evenly, with five of ten studies supporting a benefit of earlier surgery. Meanwhile, later surgery carries its own advantages, including more stable bone reconstruction and a lower risk of reoperation, though postoperative pressure re-elevation, reported in 6.9 to 14.6 percent of one large non-syndromic series, means close follow-up remains essential.

The study also exposes the weaknesses of current surveillance tools. Fundoscopy is a useful screen but cannot rule out hypertension, since its negative predictive value is low, a point painfully illustrated by the three pressure-loaded children in this cohort who had normal optic discs. Optical coherence tomography can detect papillary edema earlier and more objectively, with one prospective study reporting 89 percent sensitivity and 62 percent specificity against intracranial measurements, but it is only feasible in children over three. Optic nerve sheath diameter measurement on ultrasound, CT, or MRI works even in infants and performs well in adults, though pediatric data are less reliable. And there is no consensus on normal intracranial pressure ranges in anesthetized children, with deep anesthesia itself lowering measured values. The authors are candid about their limitations: the retrospective design, the tiny control group of three, which makes comparisons exploratory, possible selection bias, and pressures that can fluctuate with anesthesia, ventilation, head position, and probe displacement, which in fact dislodged in two patients. They present the work as a pilot study intended to inform larger prospective trials, but its central message is hard to dismiss: in every child diagnosed late, the pressure was already up, and surgery brought it down.

Subject of Research: Intracranial pressure in children with delayed diagnosis of sagittal craniosynostosis (scaphocephaly)

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

Article References: Luz, H., Bertrand, A., Luxenhofer, M., Esslinger, P., Lehner, M., & Wendling-Keim, D. S. (2026). Delayed diagnosis of scaphocephaly after 1 year of age is associated with elevated intracranial pressure. Frontiers in Pediatrics, 14, 1928890. https://doi.org/10.3389/fped.2026.1928890

Image Credits: AI Generated

DOI: 10.3389/fped.2026.1928890

Keywords: scaphocephaly, craniosynostosis, intracranial pressure, sagittal suture, Pi-plasty, papillary edema, pediatric surgery, cranial index, fundoscopy, optical coherence tomography, Chiari malformation, early diagnosis