Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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November 19, 2015
Nonsyndromal Multisuture Synostosis
- Background:
- True lambdoid synostosis is an extremely rare condition, with an incidence of 3%, and is even rarer when combined with other sutural synostoses. Multisuture synostosis is often associated with syndromes such as Apert syndrome, Crouzon syndrome, and Pfeiffer syndrome. In this case, the patient did not have an associated syndrome. Premature closure of the intra-occipital synchondroses was also not present in this patient.
- Relevant Clinical Information:
- Often presents within the first year of life with atypical craniofacial morphology
- Early fusion of the intra-occipital synchondroses can result in a decrease in the size of the foramen magnum and jugular foramina, leading to collateral venous drainage pathways, increased intracranial pressure, and cerebellar tonsil herniation.
- Cross-sectional imaging is needed to evaluate for hydrocephalus, venous collaterals from jugular foraminal stenosis, and abnormalities of the intracranial structures prior to surgical intervention.
- Key Diagnostic Features:
- Plain film: Lack of a lucent suture line, sutural sclerosis, abnormal head shape or size, copper beaten skull
- CT: Parenchymal structural and venous drainage anomalies, foraminal stenosis at the skull base
- MR: Used to assess associated congenital anomalies
- Differential Diagnosis:
- Synostotic plagiocephaly
- Nonsynostotic plagiocephaly
- Treatment:
- Emergent intervention for protection of airways and eyes, maintenance of feeding, and relief of elevated intracranial pressure
- Elective surgical management for cosmetic correction of craniofacial deformity and prevention of future increased intracranial pressure
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