Long-term follow-up of patients with familial subepithelial amyloidosis of the cornea

J. Shimazaki, T. Hida, M. Inoue, H. Saito, Kazuo Tsubota

Research output: Contribution to journalArticle

22 Citations (Scopus)

Abstract

Purpose: To investigate the clinical course of patients with familial subepithelial amyloidosis of the cornea (FSA). Methods: The authors retrospectively investigated the clinical course of seven Japanese patients with FSA. Corneal specimens obtained at the time of keratoplasty were examined histopathologically. Results: The mean follow-up period was 20.6 years, including four patients followed for more than 25 years. In all patients, the initial symptoms of photophobia and epiphora started in the first decade of life. All except one patient had their first keratoplasty before 30 years of age. The seven Japanese patients with FSA had a total of 35 keratoplasties, each of which was followed by a severe recurrence of disease. In each patient, sub-epithelial haziness developed in the graft within 1 year of keratoplasty (mean, 7.9 months). Amyloid deposition typically recurred within a few years (mean, 26.6 months) followed by a deterioration of vision. There was a high incidence of postkeratoplasty complications such as wound dehiscence, glaucoma, and cataract. Histopathologic findings demonstrated that, in the early phase of recurrence, amyloid was deposited between the basal cell of the epithelium and Bowman's layer. Conclusion: Patients with FSA have ocular symptoms with a deterioration in vision from an early decade of life. Conventional surgical approaches were complicated by subepithelial haziness in the first postoperative year, which was followed by a severe recurrence in each patient. New Surgical approaches may be indicated in FSA.

Original languageEnglish
Pages (from-to)139-144
Number of pages6
JournalOphthalmology
Volume102
Issue number1
Publication statusPublished - 1995

Fingerprint

Familial Amyloidosis
Cornea
Corneal Transplantation
Amyloid
Recurrence
Lacrimal Apparatus Diseases
Photophobia
Glaucoma
Cataract
Epithelium

ASJC Scopus subject areas

  • Ophthalmology

Cite this

Long-term follow-up of patients with familial subepithelial amyloidosis of the cornea. / Shimazaki, J.; Hida, T.; Inoue, M.; Saito, H.; Tsubota, Kazuo.

In: Ophthalmology, Vol. 102, No. 1, 1995, p. 139-144.

Research output: Contribution to journalArticle

Shimazaki, J, Hida, T, Inoue, M, Saito, H & Tsubota, K 1995, 'Long-term follow-up of patients with familial subepithelial amyloidosis of the cornea', Ophthalmology, vol. 102, no. 1, pp. 139-144.
Shimazaki, J. ; Hida, T. ; Inoue, M. ; Saito, H. ; Tsubota, Kazuo. / Long-term follow-up of patients with familial subepithelial amyloidosis of the cornea. In: Ophthalmology. 1995 ; Vol. 102, No. 1. pp. 139-144.
@article{3115e8d25763417fbce8c168a250a21e,
title = "Long-term follow-up of patients with familial subepithelial amyloidosis of the cornea",
abstract = "Purpose: To investigate the clinical course of patients with familial subepithelial amyloidosis of the cornea (FSA). Methods: The authors retrospectively investigated the clinical course of seven Japanese patients with FSA. Corneal specimens obtained at the time of keratoplasty were examined histopathologically. Results: The mean follow-up period was 20.6 years, including four patients followed for more than 25 years. In all patients, the initial symptoms of photophobia and epiphora started in the first decade of life. All except one patient had their first keratoplasty before 30 years of age. The seven Japanese patients with FSA had a total of 35 keratoplasties, each of which was followed by a severe recurrence of disease. In each patient, sub-epithelial haziness developed in the graft within 1 year of keratoplasty (mean, 7.9 months). Amyloid deposition typically recurred within a few years (mean, 26.6 months) followed by a deterioration of vision. There was a high incidence of postkeratoplasty complications such as wound dehiscence, glaucoma, and cataract. Histopathologic findings demonstrated that, in the early phase of recurrence, amyloid was deposited between the basal cell of the epithelium and Bowman's layer. Conclusion: Patients with FSA have ocular symptoms with a deterioration in vision from an early decade of life. Conventional surgical approaches were complicated by subepithelial haziness in the first postoperative year, which was followed by a severe recurrence in each patient. New Surgical approaches may be indicated in FSA.",
author = "J. Shimazaki and T. Hida and M. Inoue and H. Saito and Kazuo Tsubota",
year = "1995",
language = "English",
volume = "102",
pages = "139--144",
journal = "Ophthalmology",
issn = "0161-6420",
publisher = "Elsevier Inc.",
number = "1",

}

TY - JOUR

T1 - Long-term follow-up of patients with familial subepithelial amyloidosis of the cornea

AU - Shimazaki, J.

AU - Hida, T.

AU - Inoue, M.

AU - Saito, H.

AU - Tsubota, Kazuo

PY - 1995

Y1 - 1995

N2 - Purpose: To investigate the clinical course of patients with familial subepithelial amyloidosis of the cornea (FSA). Methods: The authors retrospectively investigated the clinical course of seven Japanese patients with FSA. Corneal specimens obtained at the time of keratoplasty were examined histopathologically. Results: The mean follow-up period was 20.6 years, including four patients followed for more than 25 years. In all patients, the initial symptoms of photophobia and epiphora started in the first decade of life. All except one patient had their first keratoplasty before 30 years of age. The seven Japanese patients with FSA had a total of 35 keratoplasties, each of which was followed by a severe recurrence of disease. In each patient, sub-epithelial haziness developed in the graft within 1 year of keratoplasty (mean, 7.9 months). Amyloid deposition typically recurred within a few years (mean, 26.6 months) followed by a deterioration of vision. There was a high incidence of postkeratoplasty complications such as wound dehiscence, glaucoma, and cataract. Histopathologic findings demonstrated that, in the early phase of recurrence, amyloid was deposited between the basal cell of the epithelium and Bowman's layer. Conclusion: Patients with FSA have ocular symptoms with a deterioration in vision from an early decade of life. Conventional surgical approaches were complicated by subepithelial haziness in the first postoperative year, which was followed by a severe recurrence in each patient. New Surgical approaches may be indicated in FSA.

AB - Purpose: To investigate the clinical course of patients with familial subepithelial amyloidosis of the cornea (FSA). Methods: The authors retrospectively investigated the clinical course of seven Japanese patients with FSA. Corneal specimens obtained at the time of keratoplasty were examined histopathologically. Results: The mean follow-up period was 20.6 years, including four patients followed for more than 25 years. In all patients, the initial symptoms of photophobia and epiphora started in the first decade of life. All except one patient had their first keratoplasty before 30 years of age. The seven Japanese patients with FSA had a total of 35 keratoplasties, each of which was followed by a severe recurrence of disease. In each patient, sub-epithelial haziness developed in the graft within 1 year of keratoplasty (mean, 7.9 months). Amyloid deposition typically recurred within a few years (mean, 26.6 months) followed by a deterioration of vision. There was a high incidence of postkeratoplasty complications such as wound dehiscence, glaucoma, and cataract. Histopathologic findings demonstrated that, in the early phase of recurrence, amyloid was deposited between the basal cell of the epithelium and Bowman's layer. Conclusion: Patients with FSA have ocular symptoms with a deterioration in vision from an early decade of life. Conventional surgical approaches were complicated by subepithelial haziness in the first postoperative year, which was followed by a severe recurrence in each patient. New Surgical approaches may be indicated in FSA.

UR - http://www.scopus.com/inward/record.url?scp=0028795757&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0028795757&partnerID=8YFLogxK

M3 - Article

C2 - 7831029

AN - SCOPUS:0028795757

VL - 102

SP - 139

EP - 144

JO - Ophthalmology

JF - Ophthalmology

SN - 0161-6420

IS - 1

ER -