An Unoperated Crouzon Family Treated with Monobloc Distraction: Challenges and Lessons

Background:. Crouzon syndrome (CS) is a rare form of craniosynostosis characterized by bicoronal craniosynostosis and facial features including severe midface hypoplasia, exophthalmos, and hypertelorism. Most patients are diagnosed and treated in early childhood; however, there are a few reports of...

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Autores principales:	Justin Hart, DO, Stephen Lu, MD, Konstantinos Gasteratos, MD, Kongkrit Chaiyasate, MD, FACS
Formato:	article
Lenguaje:	EN
Publicado:	Wolters Kluwer 2021
Materias:	Surgery RD1-811
Acceso en línea:	https://doaj.org/article/3128947614f745f198036a18834923ad
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spelling	oai:doaj.org-article:3128947614f745f198036a18834923ad2021-11-25T07:58:04ZAn Unoperated Crouzon Family Treated with Monobloc Distraction: Challenges and Lessons2169-757410.1097/GOX.0000000000003869https://doaj.org/article/3128947614f745f198036a18834923ad2021-11-01T00:00:00Zhttp://journals.lww.com/prsgo/fulltext/10.1097/GOX.0000000000003869https://doaj.org/toc/2169-7574Background:. Crouzon syndrome (CS) is a rare form of craniosynostosis characterized by bicoronal craniosynostosis and facial features including severe midface hypoplasia, exophthalmos, and hypertelorism. Most patients are diagnosed and treated in early childhood; however, there are a few reports of Crouzon patients treated as adults with monobloc facial advancement. To our knowledge, this is the first report of a family affected by CS treated sequentially with monobloc facial advancement using combined internal and external distraction osteogenesis (rigid external distraction). Methods:. We present a family from Jamaica (mother 47 years old, older daughter 17, and younger daughter 9) who were brought to our craniofacial clinic with stigmata of CS and no previous surgical intervention. Patients had bicoronal craniosynostosis and exorbitism, with varying severity, sequelae, and comorbidities. Here, we delineate our technique of monobloc distraction osteogenesis with advancement osteotomies using dual “push–pull” method, elevation of a split anteriorly based tunneled pericranial flap to seal off nasal cavity, and internal and external distraction. Results:. Our patients had favorable outcomes after reconstruction to reduce ocular symptoms and improve midface hypoplasia and aesthetic appearance. No intracranial injury, hardware/soft-tissue infection, hardware failure, or (new) loss of vision were encountered in 10 months follow-up. Conclusions:. Dual “push–pull” monobloc distraction is safe and effective for a range of ages in CS; it allows good vector control, accommodates patient compliance, and allows early rigid external distraction device removal with sufficient time for consolidation. This surgery can be performed with highly satisfactory results.Justin Hart, DOStephen Lu, MDKonstantinos Gasteratos, MDKongkrit Chaiyasate, MD, FACSWolters KluwerarticleSurgeryRD1-811ENPlastic and Reconstructive Surgery, Global Open, Vol 9, Iss 11, p e3869 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Surgery RD1-811
spellingShingle	Surgery RD1-811 Justin Hart, DO Stephen Lu, MD Konstantinos Gasteratos, MD Kongkrit Chaiyasate, MD, FACS An Unoperated Crouzon Family Treated with Monobloc Distraction: Challenges and Lessons
description	Background:. Crouzon syndrome (CS) is a rare form of craniosynostosis characterized by bicoronal craniosynostosis and facial features including severe midface hypoplasia, exophthalmos, and hypertelorism. Most patients are diagnosed and treated in early childhood; however, there are a few reports of Crouzon patients treated as adults with monobloc facial advancement. To our knowledge, this is the first report of a family affected by CS treated sequentially with monobloc facial advancement using combined internal and external distraction osteogenesis (rigid external distraction). Methods:. We present a family from Jamaica (mother 47 years old, older daughter 17, and younger daughter 9) who were brought to our craniofacial clinic with stigmata of CS and no previous surgical intervention. Patients had bicoronal craniosynostosis and exorbitism, with varying severity, sequelae, and comorbidities. Here, we delineate our technique of monobloc distraction osteogenesis with advancement osteotomies using dual “push–pull” method, elevation of a split anteriorly based tunneled pericranial flap to seal off nasal cavity, and internal and external distraction. Results:. Our patients had favorable outcomes after reconstruction to reduce ocular symptoms and improve midface hypoplasia and aesthetic appearance. No intracranial injury, hardware/soft-tissue infection, hardware failure, or (new) loss of vision were encountered in 10 months follow-up. Conclusions:. Dual “push–pull” monobloc distraction is safe and effective for a range of ages in CS; it allows good vector control, accommodates patient compliance, and allows early rigid external distraction device removal with sufficient time for consolidation. This surgery can be performed with highly satisfactory results.
format	article
author	Justin Hart, DO Stephen Lu, MD Konstantinos Gasteratos, MD Kongkrit Chaiyasate, MD, FACS
author_facet	Justin Hart, DO Stephen Lu, MD Konstantinos Gasteratos, MD Kongkrit Chaiyasate, MD, FACS
author_sort	Justin Hart, DO
title	An Unoperated Crouzon Family Treated with Monobloc Distraction: Challenges and Lessons
title_short	An Unoperated Crouzon Family Treated with Monobloc Distraction: Challenges and Lessons
title_full	An Unoperated Crouzon Family Treated with Monobloc Distraction: Challenges and Lessons
title_fullStr	An Unoperated Crouzon Family Treated with Monobloc Distraction: Challenges and Lessons
title_full_unstemmed	An Unoperated Crouzon Family Treated with Monobloc Distraction: Challenges and Lessons
title_sort	unoperated crouzon family treated with monobloc distraction: challenges and lessons
publisher	Wolters Kluwer
publishDate	2021
url	https://doaj.org/article/3128947614f745f198036a18834923ad
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An Unoperated Crouzon Family Treated with Monobloc Distraction: Challenges and Lessons

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