Abstract | BACKGROUND:
Craniofrontonasal dysplasia (CFND) is a rare congenital craniofacial syndrome characterized by single suture synostosis, hypertelorism, other clinical facial features, and abnormalities in the upper extremities. There are only a few studies in the applicable literature that address hypertelorism management for CFND patients and outcomes and complication rates. METHODS: A retrospective study was performed on consecutive late presenting CFND patients referred to our hospital with substantially completed craniofacial skeleton growth, who underwent hypertelorism correction between 2007 and 2019 following intracranial pressure screening, and who received at least 1 year of follow-up care. None of the patients in this study underwent prior craniofacial surgery. Only those patients with a confirmed mutation of the EFNB1 gene were included in this study. All patients in this study underwent hypertelorism correction by facial bipartition or box osteotomy. RESULTS: A total of ten late presenting CFND patients (all female) were treated at our hospital during the study period. None of the patients presented signs of elevated intracranial pressure. The average patient age at hypertelorism correction was 13.4 ± 7.68 years of age. Major complications, defined as complications requiring a return to the operating room, were limited to infection of the frontal bone, which required partial bone removal, and cerebrospinal fluid (CSF) leak, which was completely resolved by insertion of a lumbar shunt for a 7-day period. CONCLUSION:
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Authors | Cassio Eduardo Raposo-Amaral, Gabriel Resende, Rafael Denadai, Enrico Ghizoni, Cesar Augusto Raposo-Amaral |
Journal | Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
(Childs Nerv Syst)
Vol. 37
Issue 9
Pg. 2873-2878
(09 2021)
ISSN: 1433-0350 [Electronic] Germany |
PMID | 33864106
(Publication Type: Journal Article)
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Copyright | © 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature. |
Topics |
- Craniofacial Abnormalities
(complications, surgery)
- Female
- Frontal Bone
- Humans
- Hypertelorism
(surgery)
- Retrospective Studies
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