Managing Atypical Sagittal Craniosynostosis: When Surgery is Needed

Understanding Sagittal Craniosynostosis

Sagittal craniosynostosis involves the premature fusion of the sagittal suture in the skull. This condition often leads to a boat-shaped head, but atypical cases present differently. The focus here is on whether surgery is necessary for atypical forms discovered after the age of one, where the head shape does not resemble a typical boat head.

Expert Recommendations on Surgery

Globally, experts agree that surgery is recommended for sagittal craniosynostosis detected before age one. However, for cases identified later, especially atypical ones, opinions vary among medical centers and surgeons. Some specialists advocate surgery up to age ten or beyond if it improves appearance or addresses underlying issues. Surgery is generally considered when the condition affects appearance significantly, as non-surgical options cannot correct it.

Treatment Guidelines for Atypical Cases

For atypical sagittal craniosynostosis discovered after one year, clear treatment protocols are limited. Research in this area is sparse, with only a few studies published in recent years.

Key Study from a European Center

A 2010 study in the Journal of Plastic Surgery examined 193 patients with sagittal craniosynostosis, focusing on 8 with atypical head shapes. Follow-up ranged from 12 months to 9 years. Six showed signs of potential increased intracranial pressure or developmental delays via imaging. The study recommends monitoring intracranial pressure with a detector. Among these, four had elevated pressure, defined as over 15 mmHg—a critical threshold, as normal pediatric intracranial pressure is not well-established.

The findings highlight that atypical cases have a higher risk of elevated intracranial pressure compared to typical boat-shaped head cases.

Recommended Procedures from the Study

• If the head resembles a boat shape, surgery is advised regardless of age.
• For atypical shapes, check for signs of increased intracranial pressure through clinical symptoms or imaging, such as developmental delays or CT scans.
• If no signs, continue monitoring with biennial CT scans and genetic consultation.
• If signs are present, install an intracranial pressure detector.
• If pressure is elevated, recommend surgery; if not, maintain monitoring and refer to genetics.

Another Study from a U.S. Medical Center

A 2017 study analyzed 52 patients with sagittal craniosynostosis diagnosed after age one, averaging 6 years old (range 1.3 to 17.2 years). Follow-up was short, averaging 14.4 months (0 to 37 months), with 36 atypical cases.

Among them, 9 underwent surgery: 2 with elevated intracranial pressure (over 20 mmHg), 5 with abnormal eye exams (optic disc edema or visual evoked potentials), and 2 with boat-shaped heads.

Procedures from This Study

• If the head is boat-shaped, recommend surgery.
• For atypical shapes, perform an ophthalmic exam.
• If abnormal, recommend surgery; if uncertain, measure intracranial pressure.
• If pressure is elevated, advise surgery.
• If exams are normal, continue monitoring with eye checks every six months until at least age ten.

Current Approaches to Intracranial Pressure Monitoring

Monitoring intracranial pressure typically requires surgical placement of a detector under anesthesia, followed by ICU care. For atypical cases discovered after one year, consult ophthalmology for exams, review clinical development and imaging, and involve neurosurgeons. If surgery is recommended and agreed upon, proceed accordingly.

Characteristics of Atypical Sagittal Craniosynostosis

Atypical cases are often diagnosed later due to subtle signs, typically after one year. Features include depressions behind the anterior fontanelle and coronal suture, with a narrow mid-skull. Initial skull X-rays can help, followed by CT scans if needed.