Lack of association of cranial lacunae with intracranial hypertension in children with Crouzon syndrome and Apert syndrome: a 3D morphometric quantitative analysis

Abstract

Purpose Cranial lacunae (foci of attenuated calvarial bone) are CT equivalents of Bcopper beating^seen on plain skull radiographs in children with craniosynostosis. The qualitative presence of copper beating has not been found to be useful for the diagnosis of intracranial hypertension (IH) in these patients. 3D morphometric analysis (3DMA) allows a more systematic and quantitative assessment of calvarial attenuation. We used 3DMA to examine the relationship between cranial lacunae and IH in children with Crouzon and Apert syndromic craniosynostosis. Methods Patients were divided into IH and non-IH groups defined on an intention-to-treat basis. Pre-operative CT scans were converted into 3D skull models and processed to quantify lacunae as a percentage of calvarium surface area (LCP). This was done on individual bone and whole skull basis. Results Eighteen consecutive children with Crouzon's syndrome and 17 with Apert syndrome were identified. Median age at CT scan was 135 days (range 6-1778). Of the 35 children, 21 required surgery for IH at median age of 364 days (range 38-1710). Of these 21 children, 14 had lacunae with mean LCP of 3% (0-28%). Of the 14 non-IH children, 8 had lacunae with mean LCP of 2% (0-8%). LCP was not significantly different between IH and non-IH groups. Parietal bones were most likely to show lacunae (IH 14/21, non-IH 9/14), followed by occipital (IH 8/21, non-IH 3/14), and frontal (IH 6/21, non-IH 2/14). Conclusion Results suggest that cranial lacunae, measured using quantitative 3DMA, do not correlate with IH, in agreement with evidence from qualitative plain skull radiograph studies.

Characteristics of the Sphenoid Bone in Crouzon Syndrome

“…To a certain extent, these complications have been solved by many studies to some degree. [16][17][18][19][20] Our study results support the claim that the base of the sphenoid bone is squeezed and cranial cavity is enlarged in patients with Crouzon syndrome and that the development of the cranial base and related facial features are significantly influenced by the major sutures. 13 These cranial deformities are likely due to the premature fusion of the cranial sutures, which limits the growth and developmental space for the brain tissue, leading to compression of the surrounding cranial base bones and the sphenoid part of the skull base, tending to be squeezed with papilledema.…”

“…21 Meanwhile, some studies found that the ocular complications of Crouzon syndrome and the attenuation of cranial lacunae may not be related to ICP. 19,20 We consider that this is because the details of the ICP severity and how much space they obtained from the surgery varied much from patient to patient. We consider decompression to be necessary in most of the Crouzon syndrome patient.…”

The use of OCT to detect signs of intracranial hypertension in patients with sagittal suture synostosis: Reference values and correlations

“…There is also a debate regarding fingerprinting as a clinical sign of ICH. Several studies report a low sensitiviy to detect ICH [38][39][40][41][42]. However, a recent study has shown that fingerprinting resolves after surgical correction for ICH.…”

Perceptive Anamnesis of Syndromic Craniosynostosis with Case Series of Crouzon Syndrome

“…6,14 It is essential to be well informed of the late-onset pansynostosis of CS, which occurs occasionally; because the slight distortion of the skull shape may disguise the presence of raised ICP. 11,13 Matter of Contention: Copper Beaten Skull: Copper beaten skull, also known as beaten silver skull or beaten brass skull, refers to the prominence of convolutional markings (gyral impressions on the inner table of the skull) seen throughout the skull vault. The primary cause may be raised intracranial pressure.…”

“…DISCUSSION CASE REPORT Mishra S et al: Perceptive Anamnesis of Syndromic Craniosynostosis with Case Series of Crouzon Syndrome https://doi.org/10.38110/ijohmr.2020.v07i01.003atleast 20% of all cases [9][10][11][12][13][14][15][16]. In 1993, Jabs et al were the first to chronicle the genetic origin of syndromic craniosynostosis.…”