Macular Holes Secondary to Macular Telangiectasia Type 2: Pathogenesis, Clinical Features, Imaging and Surgical Management
- 1Asociados de Macula, Vitreo y Retina de Costa Rica, Primer Piso Torre Mercedes Paseo Colón, San José 60612, Costa Rica
Abstract
Full-thickness macular holes (FTMHs) are a rare but recognized complication of macular telangiectasia type 2 (MacTel2). Unlike idiopathic FTMHs, which are primarily driven by vitreofoveal traction, MacTel2-associated holes appear to arise predominantly from progressive neurodegenerative changes. Müller cell dysfunction, intraretinal cavitation, and outer retinal atrophy progressively compromise foveal architecture, ultimately predisposing to tissue dehiscence and full-thickness defects. Although vitreofoveal traction may contribute in individual cases, it is unlikely to be the principal mechanism in most affected eyes. Surgical treatment remains challenging, as current evidence is limited to case reports and small case series. Internal limiting membrane (ILM) flap techniques and, in selected cases, retinal graft or amniotic membrane transplant procedures have yielded encouraging anatomical closure rates, whereas conventional ILM peeling alone appears less predictable. Importantly, restoration of foveal continuity does not necessarily result in meaningful visual improvement, particularly in eyes with established photoreceptor and outer retinal damage. The durability of anatomical repair may be further compromised by continued disease progression. Persistent Müller cell dysfunction, intraretinal cavitation, and progressive outer retinal atrophy can undermine foveal support and lead to recurrent architectural disruption, including reopening of a previously closed hole. Although late recurrence has been documented, its incidence and determinants remain poorly defined because of the limited size and heterogeneity of the available literature. Visual prognosis appears to be determined largely by the amount of structurally and functionally viable retinal tissue remaining at the time of intervention. Smaller defects, less extensive cavitation, and preservation of the external limiting membrane and ellipsoid zone are associated with greater potential for visual recovery. In contrast, extensive cavitation, advanced outer retinal atrophy, and substantial Müller cell or photoreceptor loss are associated with limited functional improvement and may compromise long-term anatomical stability. Overall, a FTMH secondary to MacTel2 should be regarded as a distinct form of macular hole in which surgical repair occurs against a background of ongoing macular neurodegeneration. Preoperative evaluation of hole dimensions, foveal cavitation, and outer retinal integrity may assist in prognostication and in identifying eyes with the greatest likelihood of achieving durable anatomical closure and meaningful visual recovery.
Related
- Gastric Cancer: Epidemiology, Risk Factors, Pathogenesis, Clinical Features, Diagnosis, and Management
- Imaging and Clinical Features of Secondary Spinal Cord Lymphoma: A 2-Center Case Series
- Narrative Review: Epidemiology, Pathogenesis, Diagnosis and Clinical Management of Chronic Bronchitis.
- Unmasking Primary Candidemia: Clinical Features and Implications for Management
- Status Epilepticus in Children: Clinical Features, Management, and Outcomes in a Single-Center Experience
BibTeX
@article{wu2026macular,
title = {{Macular Holes Secondary to Macular Telangiectasia Type 2: Pathogenesis, Clinical Features, Imaging and Surgical Management}},
author = {Lihteh Wu and Genesis Chen-Li},
journal = {Journal of Clinical Medicine},
year = {2026},
month = oct,
doi = {10.3390/jcm15197673},
url = {https://www.mdpi.com/2077-0383/15/19/7673},
note = {CC BY 4.0},
}
RIS
TY - JOUR
TI - Macular Holes Secondary to Macular Telangiectasia Type 2: Pathogenesis, Clinical Features, Imaging and Surgical Management
AU - Lihteh Wu
AU - Genesis Chen-Li
PY - 2026
DA - 2026/10/03
JO - Journal of Clinical Medicine
DO - 10.3390/jcm15197673
UR - https://www.mdpi.com/2077-0383/15/19/7673
AB - Full-thickness macular holes (FTMHs) are a rare but recognized complication of macular telangiectasia type 2 (MacTel2). Unlike idiopathic FTMHs, which are primarily driven by vitreofoveal traction, MacTel2-associated holes appear to arise predominantly from progressive neurodegenerative changes. Müller cell dysfunction, intraretinal cavitation, and outer retinal atrophy progressively compromise foveal architecture, ultimately predisposing to tissue dehiscence and full-thickness defects. Although vitreofoveal traction may contribute in individual cases, it is unlikely to be the principal mechanism in most affected eyes. Surgical treatment remains challenging, as current evidence is limited to case reports and small case series. Internal limiting membrane (ILM) flap techniques and, in selected cases, retinal graft or amniotic membrane transplant procedures have yielded encouraging anatomical closure rates, whereas conventional ILM peeling alone appears less predictable. Importantly, restoration of foveal continuity does not necessarily result in meaningful visual improvement, particularly in eyes with established photoreceptor and outer retinal damage. The durability of anatomical repair may be further compromised by continued disease progression. Persistent Müller cell dysfunction, intraretinal cavitation, and progressive outer retinal atrophy can undermine foveal support and lead to recurrent architectural disruption, including reopening of a previously closed hole. Although late recurrence has been documented, its incidence and determinants remain poorly defined because of the limited size and heterogeneity of the available literature. Visual prognosis appears to be determined largely by the amount of structurally and functionally viable retinal tissue remaining at the time of intervention. Smaller defects, less extensive cavitation, and preservation of the external limiting membrane and ellipsoid zone are associated with greater potential for visual recovery. In contrast, extensive cavitation, advanced outer retinal atrophy, and substantial Müller cell or photoreceptor loss are associated with limited functional improvement and may compromise long-term anatomical stability. Overall, a FTMH secondary to MacTel2 should be regarded as a distinct form of macular hole in which surgical repair occurs against a background of ongoing macular neurodegeneration. Preoperative evaluation of hole dimensions, foveal cavitation, and outer retinal integrity may assist in prognostication and in identifying eyes with the greatest likelihood of achieving durable anatomical closure and meaningful visual recovery.
SN - 2077-0383
ER -