Posterior Cranial Decompression in ERF-Mutated Multisuture Craniosynostosis

Multisutural craniosynostosis, involving premature fusion of cranial sutures, leads to abnormal skull shape and elevated intracranial pressure (ICP), threatening neurodevelopment. This case report describes a 6-day-old infant with multisutural craniosynostosis associated with an ERF gene mutation. Imaging confirmed elevated ICP. At 2 months, posterior cranial and foramen magnum decompression effectively reduced ICP, deferring fronto-orbital advancement. By age 2, the child had achieved normal developmental milestones with stable cranial morphology and no Chiari I malformation. Early posterior decompression is a viable first-line intervention for syndromic craniosynostosis, promoting natural bone reformation and reducing secondary surgeries. Multidisciplinary management is essential.

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Publication Details

Journal
Journal of Craniofacial Surgery
Published
2026-01-21
DOI
https://doi.org/10.1097/scs.0000000000012457
Primary Topic
Craniofacial Disorders and Treatments
Type
article
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article

Posterior Cranial Decompression in ERF-Mutated Multisuture Craniosynostosis

Achara Sathienkijkanchai, Sarut Chaisrisawadisuk
Journal of Craniofacial Surgery
Craniofacial Disorders and Treatments
article

Posterior Cranial Decompression in ERF-Mutated Multisuture Craniosynostosis

Achara Sathienkijkanchai, Sarut Chaisrisawadisuk
article en

Abstract

Multisutural craniosynostosis, involving premature fusion of cranial sutures, leads to abnormal skull shape and elevated intracranial pressure (ICP), threatening neurodevelopment. This case report describes a 6-day-old infant with multisutural craniosynostosis associated with an ERF gene mutation. Imaging confirmed elevated ICP. At 2 months, posterior cranial and foramen magnum decompression effectively reduced ICP, deferring fronto-orbital advancement. By age 2, the child had achieved normal developmental milestones with stable cranial morphology and no Chiari I malformation. Early posterior decompression is a viable first-line intervention for syndromic craniosynostosis, promoting natural bone reformation and reducing secondary surgeries. Multidisciplinary management is essential.

Journal of Craniofacial Surgery
Siriraj Hospital (TH), Mahidol University (TH), Women's and Children's Hospital (AU)
Good health and well-being
Openalex Percentile: Top 82%
Craniofacial Disorders and Treatments
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