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Published on: August 11, 2015
Orbital and Eyelid Characteristics, Strabismus, and Intracranial Pressure Control in Apert Children Treated by
Jenny C Dohlman1, Sanjay P Prabhu2, Steven J Staffa3
1From the Department of Ophthalmology, Boston Children's Hospital, Boston, Mass.
Insights
Early endoscopic strip craniectomy (ESC) in Apert syndrome patients resulted in less severe eyelid and strabismus issues compared to later fronto-orbital advancement (FOA). However, some patients treated with ESC required subsequent FOA for intracranial pressure management.
Area of Science:
- Craniofacial Surgery
- Pediatric Ophthalmology
- Neurosurgery
Background:
- Apert syndrome presents with characteristic eyelid dysmorphology, V-pattern strabismus, extraocular muscle excyclorotation, and elevated intracranial pressure (ICP).
- Treatment timing for craniofacial abnormalities in Apert syndrome is a critical factor influencing outcomes.
Purpose of the Study:
- To compare eyelid characteristics, V-pattern strabismus severity, rectus muscle excyclorotation, and ICP control in Apert syndrome patients treated with early endoscopic strip craniectomy (ESC) versus later fronto-orbital advancement (FOA).
Main Methods:
- A retrospective cohort study of 25 Apert syndrome patients treated at Boston Children's Hospital.
- Primary outcomes included palpebral fissure downslanting, V-pattern strabismus severity, rectus muscle excyclorotation, and ICP interventions at 1, 3, and 5 years of age.
Main Results:
- Early ESC treatment showed statistically significant less palpebral fissure downslanting and V-pattern strabismus severity at 3 and 5 years compared to later FOA.
- Palpebral fissure downslanting correlated with V-pattern strabismus severity and often coexisted with rectus muscle excyclorotation.
- ICP control required secondary interventions in 30% of ESC patients (often followed by FOA) and 18% of FOA patients.
Conclusions:
- Initial endoscopic strip craniectomy (ESC) in Apert syndrome patients leads to improved eyelid appearance and reduced strabismus severity.
- While early ESC offers cosmetic and functional benefits, some patients may still require later fronto-orbital advancement (FOA) for effective intracranial pressure management.
Abstract:
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.
