Surgical management of Mercedes-Benz pattern synostosis with early endoscopic strip craniectomy

The incidence of nonsyndromic multisuture craniosynostosis is rare, accounting for < 5% of cases. Concurrent isolated bilateral lambdoid and sagittal synostosis (BLSS), colloquially known as Mercedes-Benz pattern synostosis, constitutes < 1% of all craniosynostosis cases. Patients with BLSS benefit from surgical correction in infancy to correct the characteristic frontal bossing, a high forehead with significant downward sloping toward the occiput, a prominent occiput with a notable point, and occipital concavity that starts immediately below that region. However, due to its rarity, there is a paucity of literature describing the surgical management of BLSS. In this study, the authors evaluated a single institution's clinical experience treating this condition with early endoscopic strip craniectomy (ESC).

Medical records for patients with a diagnosis of concurrent sagittal and bilateral lambdoid synostosis who underwent ESC between 2006 and 2023 at a single institution were reviewed for demographic, operative, and follow-up data. Patients with additional fused sutures or other syndromic diagnoses were excluded as those cases constitute a distinct pathology from pure Mercedes-Benz synostosis. All patients with less than 1 year of follow-up were also excluded.

Seven patients underwent early ESC as the primary treatment for Mercedes-Benz pattern synostosis. The mean age at surgery was 3.0 months (range 1.60-5.97 months). The mean operative time was 67 minutes, with a time under anesthesia of 155 minutes. There were no complications intra- or postoperatively. No patients required readmission within 30 days or a subsequent surgical procedure. The mean length of follow-up was 3.5 years.

Early endoscopic surgery is a safe and effective treatment for infants with Mercedes-Benz synostosis. Based on clinical criteria and limited imaging in some cases, the children have done extremely well with no need for additional surgery, but additional long-term follow-up is in process.