Scaphocephaly and increased intra-cranial pressure in non-operated adults: A controlled anthropological study on 21 skulls - 11/02/22

Doi : 10.1016/j.jormas.2022.01.012

Maddy-Hélène Delattre ^a, Quentin Hennocq ^a, Sarah Stricker ^b, Giovanna Paternoster ^c,^d, Roman Hossein Khonsari ^a,^d,^⁎
^a Service de Chirurgie Maxillo-Faciale et Chirurgie Plastique, Hôpital Necker – Enfants Malades, Assistance Publique – Hôpitaux de Paris, Faculté de Médecine, Université de Paris, 149 rue de Sèvres, Paris 75015, France
^b Klinik für Neurochirurgie, Universitätsspial Basel, Basel, Switzerland
^c Service de Neurochirurgie, Hôpital Necker – Enfants Malades, Assistance Publique – Hôpitaux de Paris, Faculté de Médecine, Université de Paris, Paris, France
^d Centre de Référence Maladies Rares Craniosténoses et Malformations Craniofaciales CRANIOST, Filière Maladies Rares TeteCou, France

^⁎Corresponding author at: Service de Chirurgie Maxillo-Faciale et Chirurgie Plastique, Hôpital Necker – Enfants Malades, 149 rue de Sèvres, 75015 Paris, France.Service de Chirurgie Maxillo-Faciale et Chirurgie Plastique, Hôpital Necker – Enfants Malades149 rue de SèvresParis75015France

En prensa. Pruebas corregidas por el autor. Disponible en línea desde el Friday 11 February 2022
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Abstract

Aim and scope

The prevalence of increased intra-cranial pressure (ICP) in patients with scaphocephaly is controversial. Here, based on anthropological material, we aimed to determine whether adults with non-operated sagittal synostosis show indirect signs of increased ICP.

Materials and methods

Thirty-eight dry skulls (21 skulls with sagittal craniosynostosis and 17 controls) were selected from the collections of the National Museum of Natural History (Paris, France). All skulls registered as ‘fused sagittal suture’ or ‘scaphocephaly’ in the registry of the Museum were included. All had total fusion of the sagittal suture. Controls were selected within skulls of similar origin (France), without visible craniofacial anomalies. The 38 skulls were CT-scanned using a standard medical CT-scan with a protocol dedicated to dry bone imaging. Eight radiological signs associated with raised ICP were assessed: (1) calvaria and (2) skull base thinning, (3) dorsum sellae erosion, (4) sella turcica lengthening, (5) copper beaten skull, (6) suture diastasis, (7) persistent metopic suture, and (8) small frontal sinus. Scaphocephaly was assessed based on head circumference, cranial index, intra-cranial volume, fronto-nasal angle, and inter-zygomatic distance. Linear and non-linear logistic models were used to compare groups.

Results

19/21 skulls with sagittal synostosis were significantly scaphocephalic. None of the criteria for ICP were significantly different in skulls with scaphocephaly relative to controls. Nevertheless, 5 individual skulls with scaphocephaly had ≥ 3 signs in favor of a history of raised ICP.

We do not report the significant prevalence of indirect signs of raised ICP in adults with scaphocephaly. These results do not allow ruling out a history of early raised ICP or of minor prolonged raised ICP. Even though our findings support the fact that scaphocephaly is not significantly associated with prolonged raised ICP, individual cases (5/21) with clear signs in favor of a history of brain compression indicate that scaphocephaly correction should be considered as a functional procedure until the production of clear evidence. Cognitive assessments of non-operated adult patients with scaphocephaly could contribute to tackle this recurring question in craniofacial surgery.

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Keywords : Scaphocephaly, Intra-cranial pressure, Intra-cranial volume, Craniosynostosis, Suture

Abbreviations : SC, CT, MRI, ICP, MSP, IQ, CSF