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Klin Monbl Augenheilkd 2017; 234(04): 584-587
DOI: 10.1055/s-0042-119564
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Georg Thieme Verlag KG Stuttgart · New York

Leberʼs Hereditary Optic Neuropathy, Normal Tension Glaucoma, and Flammer Syndrome: Long Term Follow-up of a Patient

Lebersche hereditäre Optikusneuropathie, Normaldruckglaukom und Flammer-Syndrom – eine langzeitige Beobachtung eines Patienten
K. Konieczka
1   Department of Ophthalmology, University of Basel, Basel, Switzerland (Chairman: Prof. Dr. Hendrik Scholl)
,
J. Flammer
1   Department of Ophthalmology, University of Basel, Basel, Switzerland (Chairman: Prof. Dr. Hendrik Scholl)
,
J. Sternbuch
2   Private Practice, Zurich, Switzerland
,
T. Binggeli
1   Department of Ophthalmology, University of Basel, Basel, Switzerland (Chairman: Prof. Dr. Hendrik Scholl)
,
S. Fraenkl
3   Department of Ophthalmology, University of Bern, Bern, Switzerland (Chairman: Prof. Dr. Dr. Sebastian Wolf)
› Author Affiliations
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Publication History

Publication Date:
31 January 2017 (online)

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Background

In our clinic, we observed a patient with the risk constellation of a mitochondrial DNA mutation (G7444A-mutation) combined with Flammer syndrome (FS) [1], [2], [3], who developed a classical phenotype of a Leberʼs hereditary optic neuropathy (LHON) in one eye, subsequently chronic progressive normal tension glaucoma (NTG) in the other eye, and finally, optic nerve compartment syndrome (ONCS) [4] and a marked increase in the retinal venous pressure (RVP) [5] on both sides. This stimulated the discussion of whether these individual clinical and laboratory findings were an accidental concurrence, or whether they were dependent on each other.

Abbreviations

FS: Flammer syndrome
LHON: Leberʼs hereditary optic neuropathy
NTG: normal tension glaucoma
ONCS: optic nerve compartment syndrome
ONH: optic nerve head
PVD: primary vascular dysregulation
RVP: retinal venous pressure

 

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