TY - JOUR
T1 - Pitfall of Monobloc Advancement in Beare-Stevenson Syndrome
AU - Sakamoto, Yoshiaki
AU - Miwa, Tomoru
N1 - Publisher Copyright:
© 2024 Lippincott Williams and Wilkins. All rights reserved.
PY - 2024/5/1
Y1 - 2024/5/1
N2 - Beare-Stevenson syndrome (BSS) is an extremely rare form of craniosynostosis. Half of patients with BSS died within the first year of life due to cardiorespiratory arrest or unexpected sudden death. In addition, there have only been fewer than 30 cases, which limits prognostication for clinicians. We currently have an 8-year-old patient who is being followed up. She underwent tracheostomy, cranioplasty, and shunting. After waiting for growth, for improvement of ocular herniation, monobloc advancement was performed at the age of 5 years. The temporal fossa was quite narrow due to severe midface hypoplasia. In addition, even though midface advancement, it was difficult to remove tracheostomy. This was because midfacial hypoplasia was so severe that it was hard to notice, but there was also micrognathia. We report our experience with this midface treatment.
AB - Beare-Stevenson syndrome (BSS) is an extremely rare form of craniosynostosis. Half of patients with BSS died within the first year of life due to cardiorespiratory arrest or unexpected sudden death. In addition, there have only been fewer than 30 cases, which limits prognostication for clinicians. We currently have an 8-year-old patient who is being followed up. She underwent tracheostomy, cranioplasty, and shunting. After waiting for growth, for improvement of ocular herniation, monobloc advancement was performed at the age of 5 years. The temporal fossa was quite narrow due to severe midface hypoplasia. In addition, even though midface advancement, it was difficult to remove tracheostomy. This was because midfacial hypoplasia was so severe that it was hard to notice, but there was also micrognathia. We report our experience with this midface treatment.
KW - Beare-Stevenson syndrome
KW - Le Fort
KW - midface
KW - monobloc
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U2 - 10.1097/SCS.0000000000010074
DO - 10.1097/SCS.0000000000010074
M3 - Article
AN - SCOPUS:85192236201
SN - 1049-2275
VL - 35
SP - E294-E295
JO - Journal of Craniofacial Surgery
JF - Journal of Craniofacial Surgery
IS - 3
ER -