Scaphocephaly (Sagittal Craniosynostosis): Symptoms, Causes, Treatment

What are the symptoms of scaphocephaly?

Scaphocephaly is a condition characterized by an abnormal shape of the head due to premature fusion of the sagittal suture, which runs from the front to the back of the skull. The symptoms and signs of scaphocephaly typically include:

• Elongated Head Shape: The most prominent feature is an elongated and narrow head. The head may appear more oval or boat-shaped compared to a normal round head.
• Prominent Forehead: The forehead may appear more prominent or protruding.
• Flattened Back of the Head: The back of the head may be flattened or less rounded compared to the sides.
• Increased Head Size: In some cases, there may be an increase in the overall size of the head due to compensatory growth patterns.
• Abnormal Head Growth: Head growth may be uneven or abnormal compared to typical growth patterns.
• Possible Developmental Delays: In severe cases, there could be associated developmental delays or neurological issues, although this is less common.
• Occasional Physical Discomfort: Some infants might experience discomfort or pressure due to the abnormal head shape.

Scaphocephaly is often diagnosed through physical examination and imaging studies such as X-rays or CT scans. If you suspect your child has scaphocephaly, consulting a healthcare provider or specialist, such as a pediatric neurosurgeon, is important for an accurate diagnosis and appropriate management.

What are the causes of scaphocephaly?

Scaphocephaly is caused by the premature fusion of the sagittal suture, which runs along the top of the skull from front to back. This condition is a type of craniosynostosis. The primary causes and contributing factors include:

• Genetic Factors: Scaphocephaly can be associated with genetic mutations or inherited conditions that affect skull development. Some cases may occur sporadically, but genetic predisposition can play a role.
• Environmental Factors: Certain environmental factors during pregnancy, such as maternal smoking, advanced paternal age, or exposure to certain medications or substances, may contribute to the development of craniosynostosis.
• Familial Syndromes: Scaphocephaly may occur as part of a genetic syndrome that affects multiple body systems, such as Apert syndrome or Crouzon syndrome.
• Abnormal Skull Growth: The premature closure of the sagittal suture restricts normal skull growth and leads to the characteristic elongated head shape.
• Multiple Births: In some cases, multiple births (e.g., twins or triplets) can be associated with craniosynostosis, although this is less common.
• Maternal Conditions: Certain maternal health conditions or complications during pregnancy may be linked to craniosynostosis, although these associations are less well-defined.

In many cases, the exact cause of scaphocephaly is not known. Early diagnosis and management by a healthcare provider or specialist, such as a pediatric neurosurgeon or craniofacial surgeon, are important for appropriate treatment and intervention.

What is the treatment for scaphocephaly?

The treatment for scaphocephaly primarily involves surgical intervention to correct the abnormal head shape and allow for normal brain growth. The specific approach depends on the age of the patient and the severity of the condition. Here’s an overview of the treatment options:

• Surgical Correction: The primary treatment for scaphocephaly is surgery to correct the skull shape. This is typically done through a procedure called cranial vault remodeling. The surgery involves:
• Opening the Skull: The surgeon makes incisions and removes sections of the skull to reshape it.
• Reshaping the Skull: The removed sections are reshaped and repositioned to correct the elongated head shape.
• Stabilizing the Skull: The skull is then stabilized with plates, screws, or other materials to ensure proper healing and to maintain the new shape.
• Timing of Surgery: Surgery is usually performed early in life, often between 6 and 12 months of age, to take advantage of the skull’s malleability and to minimize the impact on brain development. In some cases, surgery might be done later, depending on individual circumstances.
• Postoperative Care: After surgery, the patient may need to wear a helmet or other devices to support the head and aid in proper healing. Follow-up appointments are essential to monitor recovery and ensure that the head shape is improving as expected.
• Non-Surgical Management: In some mild cases or when surgery is not an immediate option, regular monitoring by a healthcare provider is essential. Non-surgical approaches may include physical therapy or head positioning strategies, although these are not typically sufficient to correct scaphocephaly.
• Long-Term Follow-Up: Ongoing evaluation may be necessary to monitor the child’s growth and development, ensuring that there are no residual issues or complications.

Early diagnosis and treatment are crucial for the best outcomes, so consulting with a specialist in pediatric neurosurgery or craniofacial surgery is recommended for tailored management and care.