Current Trends in Management of Nonsyndromic Unilateral Coronal Craniosynostosis: A Cross-sectional Survey

Abstract

Background: Although the natural history of nonsyndromic unilateral coronal craniosynostosis has been extensively described, optimal management remains controversial due to lack of Level 1 evidence. This study aims to assess the current state of practice among craniofacial surgeons. Methods: Ninety-four craniofacial surgeons were approached to complete a survey consisting of 15 questions. Data were collected assessing surgeons’ primary surgical indication, timing of intervention, preoperative imaging, and choice of technique for patients presenting with nonsyndromic unilateral coronal craniosynostosis. Choice of technique and timing of intervention in case of recurrence were also investigated. Results: After 5 mailings, the response rate was 61%. The combination of both appearance and raised intracranial pressure was the primary indication for treatment for 73.2% of surgeons. Preoperative CT scan of the skull was “always” performed by 70.1% of respondents. Open surgical management was most commonly performed at 8–10 months of age (38.6%). Bilateral frontal craniectomy with remodeling of the supraorbital bandeau and frontal bone was the most common choice of procedure (84.2%). In case of mild to moderate and moderate to severe recurrences at 1 year of age, 89.5% and 47.4% of surgeons opted for conservative management, respectively. Optimal timing for repeat cranioplasty was after 4 years of age (65.5%). Overall, 43.4% quoted lack of evidence as the greatest obstacle to clinical decision-making when dealing with unilateral synostosis. Conclusion: This survey exposes the lack of consensus and the disparity of opinion among craniofacial surgeons regarding the management of nonsyndromic coronal synostosis, particularly in the setting of recurrence.

Postoperative helmet therapy following fronto‐orbital advancement and cranial vault remodeling in patients with unilateral coronal synostosis

“…Fronto-orbital advancement (FOA) and cranial vault remodeling (CVR) are the standard corrective procedures for UCS (Mesa, Fang, Muraszko, & Buchman, 2011;Moderie et al, 2019;Soleman, Thieringer, Beinemann, Kunz, & Guzman, 2015). Although the optimal timing is not agreed upon, the surgery is typically performed at 8-10 months of age (Moderie et al, 2019).…”

A novel surgical rotation overlapping craniotomy technique for the management of non-syndromic anterior plagiocephaly

“…Over many years, different techniques have been developed to treat AP due to non-syndromic UCS, but the lack of quality level evidence supporting a technique's superiority over another has resulted in practical discrepancies. The lack of both evidence-based medicine and agreement between surgeons are regarded as the major obstacles in decision making in UCS management [5].…”

“…Premature coronal suture fusion combined with the rapidly expanding infant brain results in the typical AP morphology [5]. UCS produces restriction of regional growth and compensatory expansion of adjacent regions with an evident fronto-orbital dysmorphology [2].…”

“…Most literature available on the surgical management of this pathology includes retrospective studies and case series. Consequently, there is a wide discrepancy in the opinions for the best treatment of non-syndromic AP and dissatisfaction with the existing surgical procedures among many surgeons [5].…”

Facial Asymmetry in Nonsyndromic and Muenke Syndrome–Associated Unicoronal Synostosis: A 3-Dimensional Study Based on Facial Surfaces Extracted From CT Scans

“…According to the golden standard guidelines, children with UCS should be treated surgically, preferably during the first year of life. The main goals of the surgical procedure are to prevent elevated intracranial pressure by expanding the intracranial volume and to reshape the cranial vault to improve the aesthetic appearance (Derderian and Seaward, 2012; Warren et al, 2012; Moderie et al, 2019). Regarding the surgical outcome, in terms of restoring a symmetrical facial appearance, several previous studies have found a poorer outcome and a higher reoperation rate in children with MS-associated UCS (MS-UCS) compared to nonsyndromic UCS (NS-UCS; Cassileth et al, 2001; Thomas et al, 2005; Honnebier et al, 2008; Öwall et al, 2016; Öwall et al, 2019).…”