Caractéristiques cliniques, radiologiques, anatomopathologiques, thérapeutiques et évolutives de l’amylose périoculaire et/ou intraorbitaire : à propos de 6 cas et revue de la littérature [Clinical, radiological, pathological features, treatment and follow-up of periocular and/or orbital amyloidosis: Report of 6 cases and literature review]

Details

Serval ID
serval:BIB_CF8210173760
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Caractéristiques cliniques, radiologiques, anatomopathologiques, thérapeutiques et évolutives de l’amylose périoculaire et/ou intraorbitaire : à propos de 6 cas et revue de la littérature [Clinical, radiological, pathological features, treatment and follow-up of periocular and/or orbital amyloidosis: Report of 6 cases and literature review]
Journal
Journal francais d'ophtalmologie
Author(s)
Martel A., Oberic A., Moulin A., Tieulie N., Hamedani M.
ISSN
1773-0597 (Electronic)
ISSN-L
0181-5512
Publication state
Published
Issued date
06/2018
Peer-reviewed
Oui
Volume
41
Number
6
Pages
492-506
Language
french
Notes
Publication types: Case Reports ; Journal Article ; Observational Study
Publication Status: ppublish
Abstract
To assess demographic, clinical, radiological, pathological features, treatment and follow-up of periocular or/and orbital amyloidosis.
We conducted an observational retrospective monocentric study from January 2004 to April 2017 in patients diagnosed with histologically proven periocular or/and orbital amyloidosis.
Six patients were included (2 females, 4 males). Mean age was 76.8 years (range 66-88 years). Mean time between first ophthalmological symptoms and diagnosis was 27 months (range 11-36 months). The main symptoms were subconjunctival infiltration (6 patients; 100%), periocular pain or discomfort (4 patients; 66.6%) and subconjunctival hemorrhage (1 patient; 16.6%). Clinical findings included ptosis (4 patients; 66.6%), keratitis (3 patients; 50%) leading to corneal perforation in one patient, and proptosis (3 patients; 50%). One-half of the patients showed bilateral involvement. AL amyloidosis was identified on immunohistochemistry in 5 patients (83.3%). One case of B cell marginal zone orbital lymphoma was diagnosed. Systemic work-up was negative for all patients. Treatment consisted of simple monitoring (1 patient; 16.6%), surgical debulking (3 patients; 50%), ptosis surgery (1 patient; 16.6%), eyelid or eyelash malposition surgery (2 patients; 33.3%) and orbital radiation beam therapy (2 patients; 33.3%). Mean follow-up was 14.6 months (range 6-36 months), and no progression nor recurrence were noted.
Periocular or/and orbital amyloidosis is rarely encountered. Diagnosis is based on pathological examination, and immunohistochemistry analysis should always be performed to guide systemic work-up. Orbital lymphoma and multiple myeloma should be ruled out if AL amyloidosis is diagnosed. Progression is slow, and surgery is the mainstay of treatment in symptomatic patients. Long-term multidisciplinary follow-up is advocated.
Keywords
Aged, Aged, 80 and over, Amyloidosis/diagnosis, Amyloidosis/pathology, Amyloidosis/therapy, Conjunctival Diseases/diagnosis, Conjunctival Diseases/pathology, Conjunctival Diseases/therapy, Female, Follow-Up Studies, Humans, Male, Orbital Diseases/diagnosis, Orbital Diseases/pathology, Orbital Diseases/therapy, Retrospective Studies, AL, Amylose AL, Amylose orbitaire, Amylose périoculaire, Immunohistochemistry, Immunohistochimie, Lymphoma, Lymphome, Multiple myeloma, Myélome, Orbital amyloidosis, Periocular amyloidosis
Pubmed
Web of science
Create date
11/09/2018 11:38
Last modification date
20/08/2019 16:49
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