Orbital and Eyelid Characteristics, Strabismus, and Intracranial Pressure Control in Apert Children Treated by Endoscopic Strip Craniectomy versus Fronto-Orbital Advancement

Background:. Apert syndrome is characterized by eyelid dysmorphology, V-pattern strabismus, extraocular muscle excyclorotation, and elevated intracranial pressure (ICP). We compare eyelid characteristics, severity of V-pattern strabismus, rectus muscle excyclorotation, and ICP control in Apert syndr...

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Main Authors: Jenny C. Dohlman, MD, Sanjay P. Prabhu, MD, Steven J. Staffa, MS, Melissa D. Kanack, MD, Sarah Mackinnon, BSc, OC(C), COMT, Vivekanand U. Warkad, MD, John G. Meara, MD, DMD, MBA, Mark R. Proctor, MD, Linda R. Dagi, MD
Format: Article
Language:English
Published: Wolters Kluwer 2023-05-01
Series:Plastic and Reconstructive Surgery, Global Open
Online Access:http://journals.lww.com/prsgo/fulltext/10.1097/GOX.0000000000004937
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author Jenny C. Dohlman, MD
Sanjay P. Prabhu, MD
Steven J. Staffa, MS
Melissa D. Kanack, MD
Sarah Mackinnon, BSc, OC(C), COMT
Vivekanand U. Warkad, MD
John G. Meara, MD, DMD, MBA
Mark R. Proctor, MD
Linda R. Dagi, MD
author_facet Jenny C. Dohlman, MD
Sanjay P. Prabhu, MD
Steven J. Staffa, MS
Melissa D. Kanack, MD
Sarah Mackinnon, BSc, OC(C), COMT
Vivekanand U. Warkad, MD
John G. Meara, MD, DMD, MBA
Mark R. Proctor, MD
Linda R. Dagi, MD
author_sort Jenny C. Dohlman, MD
collection DOAJ
description Background:. Apert syndrome is characterized by eyelid dysmorphology, V-pattern strabismus, extraocular muscle excyclorotation, and elevated intracranial pressure (ICP). We compare eyelid characteristics, severity of V-pattern strabismus, rectus muscle excyclorotation, and ICP control in Apert syndrome patients initially treated by endoscopic strip craniectomy (ESC) at about 4 months of age versus fronto-orbital advancement (FOA) performed about 1 year of age. Methods:. Twenty-five patients treated at Boston Children’s Hospital met inclusion criteria for this retrospective cohort study. Primary outcomes were magnitude of palpebral fissure downslanting at 1, 3, and 5 years of age, severity of V-pattern strabismus, rectus muscle excyclorotation, and interventions to control ICP. Results:. Before craniofacial repair and through 1 year of age, none of the studied parameters differed for FOA versus ESC treated patients. Palpebral fissure downslanting became statistically greater for those treated by FOA by 3 (P < 0.001) and 5 years of age (P = 0.001). Likewise, severity of palpebral fissure downslanting correlated with severity of V-pattern strabismus at 3 (P = 0.004) and 5 (P = 0.002) years of age. Palpebral fissure downslanting and rectus muscle excyclorotation were typically coexistent (P = 0.053). Secondary interventions to control ICP were required in four of 14 patients treated by ESC (primarily FOA) and in two of 11 patients initially treated by FOA (primarily third ventriculostomy) (P = 0.661). Conclusions:. Apert patients initially treated by ESC had less severe palpebral fissure downslanting and V-pattern strabismus, normalizing their appearance. Thirty percent initially treated by ESC required secondary FOA to control ICP.
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spelling doaj.art-19743f95f18947179636b6420e479c142023-05-29T03:33:14ZengWolters KluwerPlastic and Reconstructive Surgery, Global Open2169-75742023-05-01115e493710.1097/GOX.0000000000004937202305000-00008Orbital and Eyelid Characteristics, Strabismus, and Intracranial Pressure Control in Apert Children Treated by Endoscopic Strip Craniectomy versus Fronto-Orbital AdvancementJenny C. Dohlman, MD0Sanjay P. Prabhu, MD1Steven J. Staffa, MS2Melissa D. Kanack, MD3Sarah Mackinnon, BSc, OC(C), COMT4Vivekanand U. Warkad, MD5John G. Meara, MD, DMD, MBA6Mark R. Proctor, MD7Linda R. Dagi, MD8From the * Department of Ophthalmology, Boston Children’s Hospital, Boston, Mass.† Department of Radiology, Boston Children’s Hospital, Boston, Mass.‡ Department of Anesthesiology, Critical Care and Pain Medicine, Boston Children’s Hospital, Boston, Mass.§ Department of Plastic and Oral Surgery, Boston Children’s Hospital, Boston, Mass.From the * Department of Ophthalmology, Boston Children’s Hospital, Boston, Mass.From the * Department of Ophthalmology, Boston Children’s Hospital, Boston, Mass.§ Department of Plastic and Oral Surgery, Boston Children’s Hospital, Boston, Mass.¶ Department of Neurosurgery, Boston Children’s Hospital, Boston, Mass.From the * Department of Ophthalmology, Boston Children’s Hospital, Boston, Mass.Background:. Apert syndrome is characterized by eyelid dysmorphology, V-pattern strabismus, extraocular muscle excyclorotation, and elevated intracranial pressure (ICP). We compare eyelid characteristics, severity of V-pattern strabismus, rectus muscle excyclorotation, and ICP control in Apert syndrome patients initially treated by endoscopic strip craniectomy (ESC) at about 4 months of age versus fronto-orbital advancement (FOA) performed about 1 year of age. Methods:. Twenty-five patients treated at Boston Children’s Hospital met inclusion criteria for this retrospective cohort study. Primary outcomes were magnitude of palpebral fissure downslanting at 1, 3, and 5 years of age, severity of V-pattern strabismus, rectus muscle excyclorotation, and interventions to control ICP. Results:. Before craniofacial repair and through 1 year of age, none of the studied parameters differed for FOA versus ESC treated patients. Palpebral fissure downslanting became statistically greater for those treated by FOA by 3 (P < 0.001) and 5 years of age (P = 0.001). Likewise, severity of palpebral fissure downslanting correlated with severity of V-pattern strabismus at 3 (P = 0.004) and 5 (P = 0.002) years of age. Palpebral fissure downslanting and rectus muscle excyclorotation were typically coexistent (P = 0.053). Secondary interventions to control ICP were required in four of 14 patients treated by ESC (primarily FOA) and in two of 11 patients initially treated by FOA (primarily third ventriculostomy) (P = 0.661). Conclusions:. Apert patients initially treated by ESC had less severe palpebral fissure downslanting and V-pattern strabismus, normalizing their appearance. Thirty percent initially treated by ESC required secondary FOA to control ICP.http://journals.lww.com/prsgo/fulltext/10.1097/GOX.0000000000004937
spellingShingle Jenny C. Dohlman, MD
Sanjay P. Prabhu, MD
Steven J. Staffa, MS
Melissa D. Kanack, MD
Sarah Mackinnon, BSc, OC(C), COMT
Vivekanand U. Warkad, MD
John G. Meara, MD, DMD, MBA
Mark R. Proctor, MD
Linda R. Dagi, MD
Orbital and Eyelid Characteristics, Strabismus, and Intracranial Pressure Control in Apert Children Treated by Endoscopic Strip Craniectomy versus Fronto-Orbital Advancement
Plastic and Reconstructive Surgery, Global Open
title Orbital and Eyelid Characteristics, Strabismus, and Intracranial Pressure Control in Apert Children Treated by Endoscopic Strip Craniectomy versus Fronto-Orbital Advancement
title_full Orbital and Eyelid Characteristics, Strabismus, and Intracranial Pressure Control in Apert Children Treated by Endoscopic Strip Craniectomy versus Fronto-Orbital Advancement
title_fullStr Orbital and Eyelid Characteristics, Strabismus, and Intracranial Pressure Control in Apert Children Treated by Endoscopic Strip Craniectomy versus Fronto-Orbital Advancement
title_full_unstemmed Orbital and Eyelid Characteristics, Strabismus, and Intracranial Pressure Control in Apert Children Treated by Endoscopic Strip Craniectomy versus Fronto-Orbital Advancement
title_short Orbital and Eyelid Characteristics, Strabismus, and Intracranial Pressure Control in Apert Children Treated by Endoscopic Strip Craniectomy versus Fronto-Orbital Advancement
title_sort orbital and eyelid characteristics strabismus and intracranial pressure control in apert children treated by endoscopic strip craniectomy versus fronto orbital advancement
url http://journals.lww.com/prsgo/fulltext/10.1097/GOX.0000000000004937
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