Reduced Intercarotid Artery Distance in Syndromic and Isolated Brachycephaly

Publication date: Available online 9 October 2017
Source:Pediatric Neurology
Author(s): Reza Assadsangabi, Mehrdad Hajmomenian, Seyed Ali Nabavizadeh, James Eric Schmitt, Arastoo Vossough
IntroductionThe morphology of the skull base can be altered in craniosynostoses. The objective of this study is to evaluate the reduced intercarotid artery distance in the lacerum segment in patients with syndromic and isolated brachycephaly.Patients and MethodsThe distances between the inner walls of the carotid canals at the lacerum segment were measured on high-resolution CT scans in children with Crouzon (25), Pfeiffer (21), Apert (26), Saethre–Chotzen (7) syndromes, isolated bicoronal synostosis (9), and compared to an age matched control group (30).ResultsA significantly smaller mean distance between carotid canal walls was observed in Crouzon (11.1±4.9mm), Pfeiffer (9.6±5.1mm), Apert (12.3±4.3mm), Saethre–Chotzen (14.8±3.0mm) syndromes and isolated bicoronal synostosis (14.9±3.7mm) as compared to the control group (19.7± 2.4mm, p = <0.001, <0.001, <0.001, 0.005 and 0.002, respectively). In comparison to Saethre–Chotzen and isolated bicoronal synostosis, there was statistically significant reduction of the mean intercarotid canal distance in patients with Crouzon and Pfeiffer.There is no statistically significant difference in intercarotid canal distance among the Apert, Saethre–Chotzen and isolated bicoronal synostosis groups. Overall, the brachycephalic group showed reduced intercarotid canal distance comparing to controls (p≤ 0.001).Discussion and ConclusionsThere is significant reduction of the distance between carotid canals in brachycephalic patients. This distance is more significantly altered in FGFR-related brachycephaly syndromes (esp. Crouzon and Pfeiffer), than Saethre–Chotzen syndrome (TWIST-1 mutation) and isolated non-syndromic bicoronal synostosis. This study highlights the importance of FGFRs in shaping the skull base. Altered vascular course of the internal carotid arteries can have important implications in planning skull base surgery in brachycephalic patients.



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