Table of Contents
Torpedo Maculopathy
Characteristics
- Single oval shaped lesion of the RPE
- Located close to the fovea
- usually temporal and unilateral
- leading edge points towards the fovea
- Causes a scotoma
- Not associated with other vision loss
- OCT
- cleft of missing RPE or thin abnormal RPE
- degeneration of photoreceptors and loss of outer segments
- Fundus autofluorescence
- dark in the area of the lesion
- no leakage
- Fluorescein angiogram
- hyper-fluorescence
- In isolation not associated with other disease
- Can occur with other pigmented lesions typically seen in Familial Adenomatous Polyposis
Figure 3 from reference [2]
Variations in lesion pigmentation in torpedo maculopathy.
(A) Lesion in patient 6, entirely hypopigmented. This allows visualisation of the deeper choroidal vessels and the sclera. (B) In patient 7, there is variable degree of hypopigmentation and a temporal region of hyperpigmentation within the lesion. (C) In patient 9, the lesion consists of only mild hypopigmentation with a hyperpigmented area within it. (D) The hypopigmented lesion in patient 4 consists of a hyperpigmented temporal crescent.
Figure 1 from reference [2]
Torpedo maculopathy in patient 1. (A) Hypopigmented lesion along the horizontal raphe with a tip towards the centre of the macula and a slightly pigmented tail. (B) Lesion hyperfluorescence on fluorescein angiography, secondary to the window defect. (C) Horizontal optical coherence tomography (OCT) (Stratus OCT; Carl Zeiss Meditec, Dublin, California) image taken of the superior aspect of the lesion revealing a large cleft and a seemingly absent retinal pigment epithelium signal. (D) Humphrey visual field (10-2) testing showing a corresponding scotoma. (E) Fundus autofluorescence shows hypoautofluorescence of the lesion. (F) Same OCT (Stratus OCT; Carl Zeiss Meditec, Dublin, California) cut of the lesion 2 years later showing no change in the cleft. There is increased irregularity in the photoreceptor layer of the retina. (G) Spectralis HRA+OCT (Heidelberg Engineering, Heidelberg, Germany) taken 4 years later showing the large cleft, the abnormal outer retinal layer, and the increased signal transmission in the choroid more clearly. (H) Three-dimensional view of the lesion using Spectralis OCT.