Source Article:American Journal of Ophthalmology Case Reports 42 (2026) 102537
Authors: Sebastiano Del Fabbro, Alessandro Arrigo, Maria Vittoria Cicinelli, Francesco Bandello, and Maurizio Battaglia Parodi
Introduction & Clinical Context
Dome-Shaped Macula (DSM) is a distinct structural feature observed in highly myopic eyes, characterized on Optical Coherence Tomography (OCT) by a convex inward scleral and chorioretinal bulge at the macula. First described by Gaucher et al. in 2008, DSM has traditionally been regarded as a static or very slowly progressive structural adaptation resulting from asymmetric axial elongation. While common complications of DSM include subretinal fluid, choroidal neovascularization, and myopic traction maculopathy, its association with inflammatory conditions like Punctate Inner Choroidopathy (PIC) has not been documented previously. This 7-year longitudinal case study provides rare documentation of the de novo development of DSM within a 2-year window, followed shortly by the onset of active and inactive PIC lesions along newly formed lacquer cracks.
Patient Case Summary
- Demographics: A 46-year-old man followed longitudinally over a 7-year period.
- Ocular Baseline Status:
- High myopia with axial lengths of 28.53 mm in the right eye and 28.14 mm in the left eye.
- History of amblyopia, penetrating keratoplasties for Acanthamoeba keratitis, and pseudophakia in the right eye (BCVA: 20/50).
- Unremarkable anterior segment and BCVA of 20/20 in the left eye.
- Fundus copy showed tessellated fundi and peripapillary atrophy without lacquer cracks at baseline.
- Systemic History: Psoriatic arthritis managed with an interleukin-23 inhibitor.
Longitudinal Timeline & Clinical Progression
| Follow-Up Interval | Structural Changes (OCT & Multimodal Imaging) | Key Clinical Insights |
|---|---|---|
| Baseline to Year 5 | Linear sclerochoroidal margin under the fovea; preserved choroidal thickness; no DSM or lacquer cracks. | Stable highly myopic fundus appearance over a 5-year observation window. |
| Year 7 | De novo central subfoveal scleral bulge meeting horizontal DSM criteria; inferior macula displacement; newly appeared linear lacquer cracks in the inferior posterior pole. | Rapid formation of DSM within a 2-year timeframe; progressive reduction in subfoveal and peri-dome choroidal thickness. |
| Year 7 + 3 Months | Emergence of yellow-gray punctate chorioretinal lesions arrayed linearly along the newly formed lacquer cracks. | Onset of active and inactive PIC foci. |
Multimodal Imaging Characteristics of Associated PIC Foci
At the 7-year + 3-month mark, OCT scans perpendicular to the inferior lacquer cracks demonstrated distinct imaging hallmarks corresponding to active and inactive PIC lesions:
- Active PIC Foci:
- Focal hyperreflective disruption of the RPE-Bruch’s membrane complex.
- Subretinal hyperreflective material accumulation.
- Localized choroidal thickening beneath the active focus.
- Ellipsoid zone disruption and posterior choroidal hypertransmission.
- Inactive PIC Foci:
- Localized choroidal thinning, RPE patchy atrophy, and initial collapse of the outer retinal layers.
Diagnostic & Pathophysiological Takeaways for Practice
1. Mechanism of DSM Formation: Peri-Dome Differential Elongation
Rather than being driven solely by localized subfoveal thickening, the longitudinal vertical OCT scans demonstrated that the dome configuration emerged due to progressive inferior displacement of the macula and asymmetrical scleral elongation. The sclerochoroidal margin shifted from linear to curved with inferior concavity, supporting Dormegny’s hypothesis of differential peri-dome expansion.
Dome-Shaped Macula (DSM) — Dormegny’s Hypothesis of Differential Expansion
Differential Peri-Dome Expansion
DSM formation is driven by unequal posterior eye wall elongation rather than isolated subfoveal scleral thickening:
Inward Scleral Inward Deformation
Asymmetrical mechanical strain leads to central convex protrusion:
- Lateral Weakening: Perifoveal scleral stretch creates structural compliance disparity.
- Relative Indentation: The central submacular sclera undergoes relative inward bucking/deformation.
- OCT Biomarker: Generates the characteristic inward convex bulge typical of classic DSM.
Longitudinal Imaging Verification
Serial vertical B-scans provide direct in vivo evidence of evolving biomechanics:
Clinical Pearl: Myopic Staphyloma Dynamics
DSM should be conceptualized as an active biomechanical response to asymmetrical posterior staphyloma expansion, emphasizing the necessity of serial vertical and horizontal raster OCT cuts to monitor progression and secondary subretinal fluid.
2. Rapid Pace of Development
Prior literature noted an average DSM height increase of ~30 µm over a 10-year period in existing cases. This report proves that a full-fledged DSM can emerge from a completely normal macular profile within a 2-year window in highly myopic eyes.
3. Coexistence of DSM and PIC
Axial elongation, accompanied by Bruch’s membrane (BM) breaks (lacquer cracks) and localized sclerochoroidal remodeling, alters the microenvironment. These structural defects can enhance local immune trafficking, predisposing the tissue to secondary inflammatory focus formation such as PIC along the line of BM disruptions.
Practical Recommendations for General Ophthalmologists
- Serial OCT Monitoring in High Myopia: High myopic patients require regular structural imaging, as biomechanical shifts, lacquer cracks, and scleral contour changes can evolve rapidly over a 1–2 year interval.
- Evaluate Linear Lacquer Cracks Carefully: When new linear lacquer cracks or BM defects are identified in myopic eyes, inspect the adjacent retinal layers closely for subretinal hyperreflective material or subtle inflammatory punctate lesions.
- Consider Systemic Factors: Autoimmune conditions or systemic immunomodulatory therapies (such as IL-23 inhibitors) may influence scleral remodeling or immune responses within the eye, altering the clinical presentation of myopic maculopathy.
Caillaux’s Classification of Dome-Shaped Macula (DSM)
1. Horizontal Dome (Most Common)
Prominent convex bulge visible on vertical OCT B-scans, forming an east-west oriented horizontal ridge across the macula.
2. Vertical Dome
Prominent convex bulge visible on horizontal OCT B-scans (flat on vertical cuts), forming a north-south oriented vertical ridge across the posterior pole.
3. Round / Circular Dome
Symmetrical, equally prominent convex elevation visible on both horizontal and vertical OCT B-scans, forming a localized, round, dome-like central elevation.
Clinical Relevance & Imaging Pearl
Complications: Horizontal and round configurations carry higher risks for chronic subretinal fluid (SRF), serous macular detachment, and localized choroidal thinning.
Scanning Mandate: Always acquire both horizontal and vertical radial scans; single-orientation cuts risk missing orthogonal ridges entirely.
