Focal choroidal excavation and pitchfork sign in choroidal neovascularisation associated with choroidal osteoma

Abstract

Purpose: To describe a complex case of choroidal osteoma complicated by choroidal neovascularisation and focal choroidal excavation and presence of pitchfork sign. Methods: A young female patient with confirmed diagnosis of choroidal osteoma was followed up for 5 years. Multimodal imaging including optical coherence tomography and fluorescein angiography (Spectralis HRA + OCT; Heidelberg Engineering, Heidelberg, Germany) was performed. Results: Optical coherence tomography showed the presence of a unilateral choroidal osteoma in the first visit which was later followed by the appearance of focal choroidal excavation. After 3 years, choroidal neovascularisation developed in choroidal osteoma area, with subretinal fluid, subretinal hyper-reflective material, hyper-reflective foci and pitchfork sign. Decalcification of the tumour was not noted in this patient. Treatment with one intravitreal injection of Aflibercept resulted in complete disappearance of subretinal fluid, subretinal hyper-reflective material, pitchfork sign and decreased hyper-reflective foci. Conclusion: Inflammatory response can be a propulsive element in the development of focal choroidal excavation and choroidal neovascularisation in choroidal osteoma patients.

Complications, treatments, and visual prognosis of choroidal osteomas

“…A possible hormonal influence has been suggested owing to its high prevalence in women, but no endocrinological abnormalities have yet been associated with choroidal osteomas, except for a case report that showed multiple choroidal osteomas in a patient with transient secondary hypoparathyroidism [8]. In addition to the 12.2% of our patients with concurrent thyroid diseases, we found five other patients with underlying thyroid diseases in the literature [9][10][11][12]. The possibility of association between thyroid disease and choroidal osteomas has not been proposed previously.…”

“…In addition, we found that FCE frequently accompanied CNV: among the 21 eyes with CNV, FCE was present in 14 (66.7%) eyes. FCE has been shown to occur within the area of CNV in eyes with choroidal osteomas and various macular diseases, including typical agerelated macular disease, polypoidal choroidal vasculopathy, central serous chorioretinopathy (CSCR), macular dystrophy, inflammatory diseases, and idiopathic conditions [11,[21][22][23][24][25][26][27][28]. Tumor decalcification leads to outer retinal layer thinning and PR atrophy [29] and may contribute to the development of FCE and CNV in choroidal osteoma cases [28].…”

Optical Coherence Tomography Features of Choroidal Neovascularization and Their Correlation With Age, Gender, and Underlying Disease

“…11 However, the PFS has also been described in CNV related to noninflammatory conditions, making the specificity of this sign a topic of debate. 5,18 There is a need to identify features on OCT that can help in differentiating CNV related to conditions such as AMD, uveitis, and myopia, the three most common causes. In this study, we have analyzed the OCT features from a large cohort of patients with iCNV, myopic CNV, and AMD-related CNV to characterize the specific OCT features that can help in identifying the underlying etiology.…”

“…Choroidal neovascularization (CNV) is a common sight-threatening complication of several chorioretinal diseases, such as age-related macular degeneration (AMD), 1 uveitis, 2 myopia, 3 choroidal tumors, 4,5 and hereditary degenerative retinopathies. 6,7 The presence of CNV is usually accompanied by a decrease in visual acuity and requires specific therapy apart from the treatment of the underlying condition.…”

“…11 However, the PFS has also been described in CNV related to noninflammatory conditions, making the specificity of this sign a topic of debate. 5,18…”