Plagiocephaly due to Frontosphenoidal Suture Synostosis: Report of 2 Cases and Literature Review

Abstract

Background/Aims: The most common cause of anterior plagiocephaly is the fusion of the unilateral coronal suture. In some rare cases, however, the fusion of the frontosphenoidal suture may lead to a resembling pattern with specific clinical and radiological features. The aim of the present study is to further enlighten this entity as it is misdiagnosed most of the time. Methods: A report of 2 cases of plagiocephaly due to frontosphenoidal synostosis with a review of the literature. Results: One female and one male baby were identified. The mean age at presentation was 11.5 months (range: 1-22). Head circumference was normal in each patient. The two fusions were on the left side. Unilateral frontal flattening and recession of the supraorbital rim were the most important physical findings. Computed tomography confirmed the patency of the frontoparietal suture and the closure of the frontosphenoidal suture. Fronto-orbital advancement was performed in both cases with good cosmetic outcome. Conclusion: Frontosphenoidal synostosis should be suspected and carefully searched when dealing with plagiocephaly with patent coronal suture. Good outcome requires a prompt diagnosis and early correction.

Isolated Frontosphenoidal Synostosis: Early Clinical Management of a Rare Condition With Distraction Osteogenesis

“…The suggested timeline to undergo FSS surgery varies among the literature with reports ranging from 6 to 18 months. 17,18 Ruiz et al affirms that among synostotic patients, elevated intracranial pressure does not typically manifest during the first year of life, thus reducing the urgency of surgical intervention within this time frame. 5 Hence, surgical timing is determined based on the goal of improving esthetic outcome at a time that assures patient health and stability.…”

The 27 Facial Sutures: Timing and Clinical Consequences of Closure

“…45 Since Francel et al first documented frontal plagiocephaly caused by an isolated frontosphenoidal suture synostosis, there have been over 20 published cases. [94][95][96][97][98][99][100][101][102][103][104] Puente-Espel et al characterized the phenotype of isolated frontosphenoidal suture synostosis as ipsilateral frontal flattening and retrusion, and inferolateral positioning of the ipsilateral orbit with compensatory medial elongation. 96 Variation in orbital deformity is the key differentiator between isolated coronal and frontosphenoidal suture synostoses.…”