Collagen fibril abnormalities in the extraocular muscles in Ehlers-Danlos syndrome.
The dermal collagen fibrils and the fibrils of the extraocular muscles and the conjunctivas of both eyes of a child afflicted with Ehlers-Danlos syndrome (EDS) were studied ultrastructurally. The results were evaluated statistically. Biopsy material was used in all instances. The collagen fibrils from all involved tissues showed a high percentage of size variation. Abnormalities of shape were noted. In the reticular dermis, 48% of fibrils were of normal diameter, 23% were enlarged, and 29% were thinner than normal. In the extraocular muscles, 77% were of normal size, 14.5% were larger, and 8.5% were smaller. In the conjunctivas, 73% of fibrils were normal-sized, 22% were enlarged, and 5% were of small diameter. No small-sized fibrils were encountered in control extraocular muscles.
Meyer
E
E
Department of Ophthalmology, Faculty of Medicine, Technion-Israel, Rambam Medical Center, Haifa.
Ludatscher
R M
RM
Zonis
S
S
D002675:Child, Preschool; D003094:Collagen / Q000502:physiology*; D003228:Conjunctiva / Q000648:ultrastructure; D004535:Ehlers-Danlos Syndrome / Q000473:pathology*; D006801:Humans; D008297:Male; D008854:Microscopy, Electron; D009801:Oculomotor Muscles / Q000648:ultrastructure*; D012016:Reference Values; D012867:Skin / Q000648:ultrastructure
D003094:Collagen