Isolated Sagittal Craniosynostosis: A Comprehensive Review

Abstract

Sagittal craniosynostosis, a rare but fascinating craniofacial anomaly, presents a unique challenge for both diagnosis and treatment. This condition involves premature fusion of the sagittal suture, which alters the normal growth pattern of the skull and can affect neurological development. Sagittal craniosynostosis is characterised by a pronounced head shape, often referred to as scaphocephaly. Asymmetry of the face and head, protrusion of the fontanel, and increased intracranial pressure are common clinical manifestations. Early recognition of these features is crucial for early intervention, and understanding the aetiology is, therefore, essential. Although the exact cause remains unclear, genetic factors are thought to play an important role. Mutations in genes such as FGFR2 and FGFR3, which disrupt the normal development of the skull, are suspected. Environmental factors and various insults during pregnancy can also contribute to the occurrence of the disease. An accurate diagnosis is crucial for treatment. Imaging studies such as ultrasound, computed tomography, magnetic resonance imaging, and three-dimensional reconstructions play a crucial role in visualising the prematurely fused sagittal suture. Clinicians also rely on a physical examination and medical history to confirm the diagnosis. Early detection allows for quick intervention and better treatment outcomes. The treatment of sagittal craniosynostosis requires a multidisciplinary approach that includes neurosurgery, craniofacial surgery, and paediatric care. Traditional treatment consists of an open reconstruction of the cranial vault, where the fused suture is surgically released to allow normal growth of the skull. However, advances in minimally invasive techniques, such as endoscopic strip craniectomy, are becoming increasingly popular due to their lower morbidity and shorter recovery times. This review aims to provide a comprehensive overview of sagittal craniosynostosis, highlighting the aetiology, clinical presentation, diagnostic methods, and current treatment options.

Keywords: cranial deformation; cranial vault remodelling; craniosynostosis; scaphocephaly; surgery.

Figure 1

Computed tomography (CT) with 3D reconstructions showing sagittal craniosynostosis (A) and subsequent scaphocephalic skull deformity (B).

Figure 2

Cranial bone ultrasound confirms the synostosis of the cranial suture, as the hyperechoic bone signal continues uninterrupted, whereas a hypoechoic cranial suture should be identifiable.

Figure 3

(A,B) show the scaphocephalic head shape from a bird’s eye view, characterised by biparietal constriction and increased anteroposterior circumference. (C,D) show the lateral view, with prominent frontal convexity and flattening of the vertex.

Figure 4

A CT scan of scaphocephaly showed enlarged frontal pericerebral subarachnoid spaces (white arrow) and slightly wider frontal horns of the lateral ventricles (empty arrow).

Figure 5

The Omega sign is recognisable on CT scans. It is a bony groove in the area of the synostotic sagittal suture, which hugs the superior sagittal sinus (refer to Ref. [14].)

Figure 6

Four cases of children (A–D) with marked and persistent frontal bulging at early follow-up after RHT surgery.

Figure 7

The reshaping of the entire vault involves not only biparietal expansion but also the reshaping of the frontal bone.

Figure 8

The aim of an endoscopic procedure is to minimise the invasiveness of the surgery itself. The endoscopic approach is performed through two small incisions in the skin (arrows). The synostotic sagittal suture is then removed with the assistance of an endoscope.

Figure 9

One of the modern surgical methods of ISS treatment involves the insertion of springs into the bone defect created after the suturectomy (refer to Ref. [62]).