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Updated: May 31, 2026
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An In Vitro Model for the Study of Cellular Pathophysiology in Globoid Cell Leukodystrophy
Published on: October 21, 2014
The globe and orbit in Laron syndrome.
L Kornreich1, O Konen, P Lilos
1Imaging Department, Schneider Children's Medical Center of Israel, Petah Tiqwa, Israel. korn@netvision.net.il
AJNR. American Journal of Neuroradiology
|July 16, 2011
Summary
Patients with Laron syndrome (LS) have smaller eyes and shallower, wider orbits compared to controls, potentially due to insulin-like growth factor 1 (IGF-1) deficiency.
Area of Science:
- Ophthalmology
- Endocrinology
- Radiology
Background:
- Laron syndrome (LS) is characterized by growth hormone resistance.
- This resistance leads to a failure in generating insulin-like growth factor 1 (IGF-1).
Purpose of the Study:
- To evaluate and compare the size of the eye and orbit in patients with LS.
- To investigate potential differences in ocular and orbital dimensions in LS.
Main Methods:
- Retrospective review of brain MRI scans from 9 patients with LS.
- Measurement of various ocular and orbital parameters, including globe diameter, orbital dimensions, and distances.
- Comparison with 20 control subjects.
Main Results:
- Patients with LS exhibited significantly smaller maximal globe diameters.
- Orbits in LS patients were shallower and wider, with shorter lateral walls and a larger orbital angle.
- The ratio of anterior orbital diameter to globe size was greater in LS patients, with more anteriorly positioned globes.
Conclusions:
- Laron syndrome is associated with shallow, wide orbits and relatively small globes.
- These ocular and orbital findings may be a consequence of IGF-1 deficiency in LS.
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