Non-syndromic bilambdoid and sagittal craniosynostosis

Non-syndromic bilambdoid and sagittal craniosynostosis (also known as Mercedes Benz pattern craniosynostosis) is a rare congenital craniofacial condition characterized by the premature fusion of both lambdoid sutures and the sagittal suture of the skull. Unlike syndromic forms of craniosynostosis, this condition occurs in isolation without other systemic anomalies or known genetic syndrome associations. The premature closure of these three sutures restricts normal skull growth, leading to an abnormal head shape that typically presents with a shortened skull from front to back (brachycephaly), a turricephalic (tower-shaped) head, and a characteristic flattening of the back of the skull. Increased intracranial pressure may develop if the condition is not treated, potentially affecting brain development. The condition is apparent at birth or in early infancy, as the abnormal skull shape becomes evident during the period of rapid brain growth. Key clinical features include an abnormal head shape with prominent frontal bossing, occipital flattening, and a palpable bony ridge along the fused sutures. Affected infants may also exhibit signs of raised intracranial pressure such as irritability, vomiting, or developmental concerns if left untreated. Diagnosis is typically confirmed through clinical examination and imaging studies including CT scanning with three-dimensional reconstruction, which clearly demonstrates the pattern of sutural fusion. Treatment is primarily surgical and aims to release the fused sutures and reshape the skull to allow normal brain growth and to correct the cosmetic deformity. Surgical intervention, often performed in the first year of life, may involve open cranial vault remodeling or, in select cases, endoscopic strip craniectomy followed by helmet therapy. Long-term follow-up is important to monitor for potential re-fusion of sutures and to assess neurodevelopmental outcomes. With appropriate surgical management, the prognosis for most affected children is generally favorable.

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