A Unique Case of Pure Bilateral Lambdoid Craniosynostosis and Progression to Mercedes-Benz Syndrome Following Spring Cranioplasty.
Sebastian Kuriakose, Yasmine Soubra, Sarah Anne Frommer et al.
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Case in brief
A 3-month-old girl with isolated bilateral lambdoid craniosynostosis and mendosal fusion had a distinctive posterior vault deformity. She underwent suturectomy with cranial spring placement; the springs were removed five months later. Segmented CT showed intracranial volume increasing from 727.4 cm³ before surgery to 1371.9 cm³ after removal. Recovery was uncomplicated, with minimal blood loss. Imaging after spring removal demonstrated complete sagittal synostosis, raising the possibility of delayed Mercedes-Benz pattern synostosis or secondary fusion related to the springs.
What made this case unusual
Unexpected complete sagittal fusion followed treatment of apparently isolated bilateral lambdoid synostosis, creating a possible Mercedes-Benz pattern; whether this reflected delayed disease progression or a mechanical effect of the springs was uncertain.
Diagnostic / clinical pearl
After spring-assisted treatment of lambdoid craniosynostosis, follow-up imaging may reveal sagittal fusion; this case could not distinguish delayed pattern progression from mechanically induced secondary synostosis.
Why it matters
The authors identify the possible relationship between cranial springs and secondary synostosis as a question warranting further investigation.