Scaphocephaly is characterized by an abnormally long and narrow skull, sometimes described as boat-shaped. This condition arises because the skull grows more from front to back than from side to side. Understanding the specific reason behind the head shape is necessary for determining the appropriate course of action.
What Causes Scaphocephaly?
Scaphocephaly stems from an imbalance in how the skull bones expand to accommodate the growing brain. This shape results from two underlying causes: Positional (deformational) scaphocephaly, caused by external forces molding the soft skull, and Craniosynostosis, a medical condition where a skull joint fuses prematurely.
The sagittal suture, which runs along the top of the head from front to back, is the joint involved in craniosynostosis-related scaphocephaly. When this seam closes too early, it prevents the skull from growing wider, forcing the head to grow longer. Management depends entirely on correctly identifying which of these two mechanisms is at play.
The Critical Difference: Positional Versus Craniosynostosis
Whether scaphocephaly corrects itself depends entirely on its underlying cause. Positional scaphocephaly, caused by consistent external pressure, often from the baby’s sleeping position, frequently self-corrects. Since all skull sutures remain open, the skull can reshape itself as the child spends more time off their back and develops neck control. Simple repositioning techniques, such as increasing “Tummy Time,” are typically effective in guiding the head toward a more typical shape.
Scaphocephaly caused by sagittal craniosynostosis will not correct itself and requires medical intervention. This condition involves the premature fusion of the sagittal suture, which creates a rigid barrier to sideways skull expansion. If this fused suture is not released, restricted growth can prevent the brain from expanding normally and may lead to increased intracranial pressure. The abnormal elongation will persist as the brain continues to grow.
Diagnostic Confirmation and Timing
A definitive diagnosis is necessary to distinguish between positional and craniosynostosis types. The diagnostic process begins with a physical examination where the physician assesses the head shape and measures dimensions. The doctor will also palpate the sagittal suture, looking for a bony ridge or the absence of the expected soft spot, which indicates premature fusion.
Imaging, such as X-rays or a Computed Tomography (CT) scan, confirms the physical findings. The CT scan, often with 3D reconstruction, provides a detailed view of the skull plates, allowing specialists to visualize the sutures clearly. Early diagnosis is important for treatment outcomes, especially for craniosynostosis, with intervention before six months of age generally leading to better results.
Intervention Strategies
Intervention strategies are tailored to the confirmed cause of the scaphocephaly. For positional cases that do not fully resolve with repositioning, a non-surgical approach using a cranial orthosis (helmet) is employed. This custom-fitted helmet gently redirects skull growth, applying light pressure to protruding areas while allowing space for the flattened sides to expand. Helmet therapy is most effective when started between four and eighteen months of age while the skull bones remain malleable.
For scaphocephaly caused by craniosynostosis, surgical intervention is required to correct the bony fusion. The goal of surgery is to release the fused sagittal suture, allowing the skull to reshape and providing the brain with adequate room to grow. Options include minimally invasive endoscopic surgery (often followed by helmet therapy) or a more extensive open cranial vault remodeling procedure. These procedures are generally performed between three and six months of age to achieve optimal cosmetic and functional results.