Purpose: To propose a classification of retinal astrocytic hamartoma based on spectral domain optical coherence tomography and correlate each class with systemic manifestations of tuberous sclerosis complex. Methods: Retrospective chart review conducted at four international referral medical retina centers. There were 43 consecutive patients with an established diagnosis of tuberous sclerosis complex based on presence of at least 2 major or 1 major and 2 minor features of the diagnostic criteria. Clinical and spectral domain optical coherence tomography features regarding retinal astrocytic hamartoma were documented. Results: The mean patient age at presentation was 16.2 years. The retinal astrocytic hamartoma was classified as Type I (n 41), Type II (n 25), Type III (n 20), or Type IV (n 12). Patients with Type II showed greater number of cutaneous fibrous plaques (odds ratio 64.8; 92% confidence interval: 64.2-65; P < 0.001); those with Type III displayed higher incidence of subependymal giant-cell astrocytomas (odds ratio 43.2; 95% confidence interval: 43.0-43.3; P < 0.001); and those with Type IV showed higher incidence of pulmonary lymphangiomyomatosis (odds ratio 126; 95% confidence interval: 122-128; P < 0.001). Conclusion: Retinal astrocytic hamartoma can be classified into four morphologic groups, based on spectral domain optical coherence tomography. There are important systemic tuberous sclerosis complex correlations with each class.

Retinal astrocytic hamartoma / F. Pichi, D. Massaro, M. Serafino, P. Carrai, G.P. Giuliari, C.L. Shields, C. Veronese, A.P. Ciardella, P. Nucci. - In: RETINA. - ISSN 0275-004X. - 36:6(2016), pp. 1199-1208. [10.1097/IAE.0000000000000829]

Retinal astrocytic hamartoma

F. Pichi
;
P. Nucci
Ultimo
2016

Abstract

Purpose: To propose a classification of retinal astrocytic hamartoma based on spectral domain optical coherence tomography and correlate each class with systemic manifestations of tuberous sclerosis complex. Methods: Retrospective chart review conducted at four international referral medical retina centers. There were 43 consecutive patients with an established diagnosis of tuberous sclerosis complex based on presence of at least 2 major or 1 major and 2 minor features of the diagnostic criteria. Clinical and spectral domain optical coherence tomography features regarding retinal astrocytic hamartoma were documented. Results: The mean patient age at presentation was 16.2 years. The retinal astrocytic hamartoma was classified as Type I (n 41), Type II (n 25), Type III (n 20), or Type IV (n 12). Patients with Type II showed greater number of cutaneous fibrous plaques (odds ratio 64.8; 92% confidence interval: 64.2-65; P < 0.001); those with Type III displayed higher incidence of subependymal giant-cell astrocytomas (odds ratio 43.2; 95% confidence interval: 43.0-43.3; P < 0.001); and those with Type IV showed higher incidence of pulmonary lymphangiomyomatosis (odds ratio 126; 95% confidence interval: 122-128; P < 0.001). Conclusion: Retinal astrocytic hamartoma can be classified into four morphologic groups, based on spectral domain optical coherence tomography. There are important systemic tuberous sclerosis complex correlations with each class.
retinal astrocytic hamartoma; spectral domain optical coherence tomography; tuberous sclerosis complex; ophthalmology
Settore MED/30 - Malattie Apparato Visivo
2016
Article (author)
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2434/444441
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