Hyaline Fibromatosis Syndrome: Early Outcomes Following Major Craniofacial Mass Excision
Issued Date
2024-07-01
Resource Type
ISSN
10492275
eISSN
15363732
Scopus ID
2-s2.0-85197586182
Journal Title
Journal of Craniofacial Surgery
Volume
35
Issue
5
Start Page
e492
End Page
e495
Rights Holder(s)
SCOPUS
Bibliographic Citation
Journal of Craniofacial Surgery Vol.35 No.5 (2024) , e492-e495
Suggested Citation
Chaisrisawadisuk S., Rattana-Arpa S., Vathanophas V., Sathienkijkanchai A. Hyaline Fibromatosis Syndrome: Early Outcomes Following Major Craniofacial Mass Excision. Journal of Craniofacial Surgery Vol.35 No.5 (2024) , e492-e495. e495. doi:10.1097/SCS.0000000000010401 Retrieved from: https://repository.li.mahidol.ac.th/handle/123456789/99657
Title
Hyaline Fibromatosis Syndrome: Early Outcomes Following Major Craniofacial Mass Excision
Author's Affiliation
Corresponding Author(s)
Other Contributor(s)
Abstract
Hyaline fibromatosis syndrome (HFS) is a rare congenital disorder characterized by abnormal hyaline deposition within soft tissues. Hyaline fibromatosis syndrome manifests in 2 distinct forms: (1) infantile systemic hyalinosis and (2) juvenile hyaline fibromatosis. Infantile systemic hyalinosis, the more severe form, typically emerges in early childhood with extensive systemic involvement. In contrast, juvenile HFS is less severe, allowing patients to survive into adulthood. Common clinical manifestations include thickened skin, hyperpigmented patches, gingival hypertrophy, skin nodules, and progressive severe joint contractures, leading to significant morbidity and potential mortality. This case report describes a 7-year-old child who was diagnosed with HFS and presented with a very large, ulcerated, rapidly expanding craniofacial mass. The patient underwent successful treatment involving a multidisciplinary medical team and strategic surgical intervention, achieving favorable postoperative outcomes.