The Management of Trigonoscaphocephaly as a Result of Combined Metopic and Sagittal Synostosis.

BACKGROUND

The combination of sagittal and metopic synostosis is rare, resulting in a scaphocephalic shape, but with an absence of frontal bossing and therefore varying degrees of trigonocephaly and occipital prominence. Treatment is primarily surgical, with a combination of procedures to address both the scaphocephaly and trigonocephaly required involving multiple operations. The authors discuss their experience of treating combined trigonoscaphocephaly in a single-stage procedure and propose a management strategy based on the severity of the presenting deformity.

METHODS

The Oxford Craniofacial Unit database was searched from inception in October of 2004 to August of 2013 to identify all patients with combined sagittal and metopic synostosis. Case notes were then manually searched to identify those patients who had true trigonoscaphocephaly.

RESULTS

Of 2856 patients in the authors' database, a total of nine were identified as having had true trigonoscaphocephaly. Seven of these patients underwent a combined single-stage procedure with an average cephalic index of 68.7 percent preoperatively and 80.3 percent postoperatively.

CONCLUSIONS

Management of trigonoscaphocephaly has been traditionally performed by multiple, staged surgical procedures. The authors propose that it can instead be managed in a single surgical procedure, with the choice of procedure determined by the severity of the deformity. If the deformity is mild to moderate with no occipital bullet, a combined fronto-orbital advancement remodeling and subtotal calvarial remodeling can be performed; however, if there is an occipital bullet, the authors propose the combination of fronto-orbital advancement remodeling and total calvarial remodeling performed in one operation with the patient turned from prone to supine intraoperatively.

CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.