Sclerochoroidal Calcification Research Articles

Sclerochoroidal calcification: idiopathic or associated with systemic disease?

Idiopathic sclerochoroidal calcification is a rare disorder that may be confused with a malignant tumor. In most patients, its etiology is unknown. Some patients have associated systemic disorders or syndromes. We report the case history of a patient with hypercalcemia and extensive sclerochoroidal calcifications; furthermore, we evaluate a series of seven patients with sclerochoroidal calcification, investigating calcium-phosphorus metabolism and screening for primary renal tubular hypokalemic metabolic alkalosis syndromes. One patient was diagnosed with bilateral sclerochoroidal calcification; she had primary hyperparathyroidism due to a parathyroid adenoma. None of the other seven patients met the criteria of primary renal tubular hypokalemic metabolic alkalosis syndromes or had significant problems of calcium-phosphorus metabolism. Nevertheless, two of them had a history of kidney stones, whereas two others had a history of arthritis. For all patients with sclerochoroidal calcification, it is important to exclude primary renal tubular hypokalemic metabolic alkalosis syndromes such as Bartter and Gitelman syndromes, because these patients have a higher risk of cardiovascular morbidity especially during anesthesia.

Idiopathic sclerochoroidal calcification in a 79‐year‐old woman

Sclerochoroidal calcification is an uncommon ocular condition usually seen in elderly patients. Lesions are typically bilateral and observed in the mid-peripheral fundus, frequently near the superotemporal arcade. It is often an incidental finding, observed as multiple ill-defined, elevated yellow plaque-like lesions, in otherwise asymptomatic patients.

Sclerochoroidal calcification in a patient with classic Bartter’s syndrome

To report sclerochoroidal calcification in a patient with classic Bartter's syndrome. Observational case report. A 42-year-old woman with a 26-year history of classic Bartter's syndrome was found to have bilateral fundus tumors. The patient presented initially with quivering lips and hand stiffness at age 6 years but was not diagnosed until age 16 years. Treatment included magnesium and potassium supplementation and Amiloride therapy. On ocular examination, there were multifocal, yellow-white, geographic, solid choroidal lesions along the superior and inferior retinal vascular arcades in both eyes. Ultrasonography showed echogenic, placoid calcified lesions at the level of the sclera and choroid, consistent with bilateral sclerochoroidal calcification. Sclerochoroidal calcification can be associated with classic Bartter's syndrome.

Idiopathic sclerochoroidal calcification

Background Idiopathic sclerochoroidal calcification (ISC) is an uncommon condition of calcium deposition at the level of the sclera and choroid of the posterior pole. With normal acuity and visual field, it typically manifests in asymptomatic older males. It can be diagnosed on clinical grounds, appearing as multiple geographic, yellowish, placoid-like lesions in the sclera and choroid, commonly found in the super-otemporal arcade of the midperipheral fundus in both eyes. Ocular ultrasound and fluorescein angiography have classic results. Case Report An 85-year-old white man came to us for routine examination. Best-corrected visual acuity was 20/40 O.D. and O.S. Dilated fundus examination revealed several midperipheral yellowish-white deposits with retinal pigment epithelium hyperpigmentation temporally in each eye. Ocular ultrasound revealed high reflectivity consistent with calcium. The lesions demonstrated mild hyperfluorescence in the late phase of fluorescein angiography. Laboratory testing was void of abnormal calcium or phosphorous metabolism, and a diagnosis of ISC was made. Conclusion Idiopathic sclerochoroidal calcification is a benign lesion of calcium deposition in the posterior pole, often discovered on routine examination. Although it is idiopathic, systemic evaluation is warranted to rule out dystrophic or metastatic calcification by various pathological conditions associated with abnormal calcium-phosphorus metabolism— most notably, hyperparathyroidism. Differential diagnoses include choroidal osteoma, melanoma, and metastatic carcinoma.

CME review: sclerochoroidal calcification: the 2001 Harold Gifford Lecture.

Sclerochoroidal calcification is a recently recognized ocular condition that is characterized by typical geographic yellow-white fundus lesions that usually occur bilaterally in adults. It can clinically simulate a number of intraocular tumors like choroidal metastasis, choroidal melanoma, and choroidal osteoma. Clinical evaluation with ultrasonography, computed tomography, and some histopathologic studies have supported the fact the condition represents calcium deposition in the sclera and choroid. Early reports suggested that sclerochoroidal calcification was usually associated with hypercalcemic conditions such as hyperparathyroidism and pseudohypoparathyroidism. Subsequently, many patients with sclerochoroidal calcification have had no apparent systemic associations and have been classified as idiopathic. However, very recent studies have suggested that this ocular condition can also be associated with Gitelman syndrome or Bartter syndrome. These are autosomal recessive conditions of hypokalemic alkalosis associated with hypomagnesemia and deposition of calcium salts in various tissues. Ophthalmologists should be familiar with fundus features and systemic associations of sclerochoroidal calcification.

Sclerochoroidal calcification: clinical manifestations and systemic associations.

Sclerochoroidal calcification is an unusual ocular condition that is believed to be idiopathic in most cases. To describe the clinical manifestations of sclerochoroidal calcification and to investigate its possible systemic associations. This noncomparative consecutive case series included patients diagnosed as having sclerochoroidal calcification based on clinical characteristics and diagnostic test findings. We analyzed the demographic, systemic, and ocular features of 27 such patients. Systemic evaluation included tests for calcium-phosphorus metabolism in 19 patients and renal tubular hypokalemic metabolic alkalosis syndromes (Bartter or Gitelman syndrome) in 13. All the patients were asymptomatic older (mean age, 70 years) white individuals, incidentally noted as having a choroidal lesion on routine examination. Among 38 eyes, the main referral diagnoses were choroidal metastasis in 10 eyes (26%), choroidal melanoma in 8 (21%), and choroidal nevus in 4 (11%). Sixteen patients (59%) had unilateral clinical findings, while 11 (41%) had bilateral. The Snellen visual acuity was 20/50 or better in 37 eyes (97%). Cogan scleral plaque was visible anterior to the insertion of horizontal rectus muscles in 10 eyes (26%). Among 77 foci, there were a mean of 2 foci of sclerochoroidal calcification in each eye, 41 yellow (53%), 32 yellow-white (42%), 2 white (3%), and 2 orange (3%), measuring a mean 2.6 mm in diameter and 1.1 mm in thickness. The most common locations were postequatorial in 45 (58%), along the temporal vascular arcades in 30 (39%), and in the superotemporal quadrant in 43 (56%). A-scan and B-scan ultrasonography revealed dense echoes compatible with calcium, with orbital shadowing. All the lesions remained stable in size and configuration during a mean follow-up of 38 months. One patient developed a choroidal neovascular membrane over the area of sclerochoroidal calcification. Investigations for abnormal calcium-phosphorus metabolism in 19 patients revealed primary hyperparathyroidism in 1 patient (5%). Clinical and biochemical evaluation of 13 patients demonstrated hypomagnesemia in 6 (46%). Four patients (31%) met the criteria for the diagnosis of Gitelman syndrome. Sclerochoroidal calcification usually manifests as multiple discrete yellow placoid lesions in the midperipheral fundus of asymptomatic older white individuals. Although most cases may be idiopathic in nature, some patients may have underlying systemic disorders involving abnormal calcium-phosphorus metabolism or renal tubular hypokalemic metabolic alkalosis syndromes. All patients with sclerochoroidal calcification should be tested for these treatable systemic associations.

Choroidal calcifications in patients with Gitelman’s syndrome

Gitelman’s syndrome is a renal tubular disorder characterized by a sodium and chloride reabsorption defect in distal tubular cells that determines hypokalemia, metabolic alkalosis, hypomagnesemia, and low calcium excretion. The presence of choroidal calcifications was sought in five patients with Gitelman’s syndrome by ophthalmic examination, fluorescein angiography, indocyanine green angiography, and ocular ultrasonography. Calcifications observed in the choroid of two patients were shown by ultrasonography in both patients. Ophthalmic and fluorangiographic examinations detected this alteration in one of the two subjects. Chondrocalcinosis was found in one patient with choroidal calcifications. These findings suggest that precipitation of calcium salts can occur in the choroidal tissue of patients with Gitelman’s syndrome. Deposits appeared to be well seen by ultrasonography because of their depth in ocular tissues. Sclerochoroidal calcifications may be favored by the low calcium excretion, which is associated with normal intestinal calcium absorption in patients with Gitelman’s syndrome.

Sclerochoroidal calcification associated with Gitelman syndrome

PURPOSE: To investigate sclerochoroidal calcification in a patient with Gitelman syndrome. METHOD: Case report. Bilateral fundus abnormalities observed in a 58-year-old woman were documented with fluorescein angiography and tomodensitometry. RESULTS: Symmetric yellow-white subretinal lesions were observed in the superotemporal midperiphery of the fundus of each eye. Tomodensitometry examination was consistent with calcium deposition. The medical history included Gitelman syndrome. Sclerochoroidal calcification probably resulted from the severe hypomagnesemia. CONCLUSION: Gitelman syndrome may be a cause of sclerochoroidal calcification.

Idiopathische sklerochoroidale Kalzifikation

Background Idiopathic sclerochoroidal calcification (ISC) is an unusual, benign disorder occuring at the level of the posterior choroidea and sclera. Clinical differential diagnosis includes choroidal osteoma, choroidal metastasis, choroidal melanoma, choroidal amelanotic naevus and choroiditis. Patient A 61-year old man was referred with the diagnosis of multifocal choroidal tumor in both eyes. Fundoscopy showed bilateral yellow lesions located near the superotemporal arcade. Results Fluorescein angiography shows the late staining of the choroidal lesions. Ultrasonographically highly reflective lesions (sclerochoroidal placoid lesions at the sclero-choroidal interface in the CT) confirms the clinical diagnosis. The serum calcium and phosphate metabolism is normal. Conclusion The Idiopathic Sclerochoroidal Calcification (ISC) is a rare echographical differential diagnosis to the choroidal osteoma. ISC is a heterotopic calcification of the posterior pole in both eyes, usually located near the supero-temporal arcade.

Choroidal neovascularization as a late complication of hyperparathyroidism

PURPOSE: To report choroidal neovascularization in a patient with sclerochoroidal calcification secondary to hyperparathyroidism. METHOD: Case report. Bilateral ocular fundus abnormalities observed in a 74-year-old woman were documented with fluorescein angiography and ultrasonography. RESULTS: Two yellow, irregular, oval placoid lesions were observed in the fundus of each eye. Ultrasound examination showed that these areas were extremely sonoreflective and exhibited orbital shadowing. In the left eye, one lesion was accompanied by lipid exudates, and subsequent fluorescein angiography disclosed choroidal neovascularization. Medical history included hyperparathyroidism treated with surgical excision of a parathyroid adenoma 27 years earlier. CONCLUSION: Choroidal neovascularization may be a late complication of hyperparathyroidism.

Sclerochoroidal Calcification

We studied 19 patients with sclerochoroidal calcification. The findings were bilateral in 16 patients and unilateral in the remaining three patients. The lesions, which were usually multifocal, had two characteristic appearances, plaque-like and tumorlike. Eleven patients had relatively flat, irregularly shaped, plaque-like, yellow-white lesions located between the arcades and the equator. Eight patients had more elevated tumorlike lesions, ranging up to 6 mm in height. All showed patterns on echography consistent with calcification. The calcification was often documented in both the choroid and sclera; sometimes it appeared only in the choroid, but never only in the sclera. Calcium metabolism appeared to be normal in all but two of the nine patients in whom it was investigated. Idiopathic sclerochoroidal calcification has a characteristic echographic and ophthalmoscopic appearance and may be more common than has been realized.

Idiopathic Sclerochoroidal Calcification: New Observations

Two cases of idiopathic sclerochoroidal calcification are reported with follow-up of two and ten years. In addition we have reviewed 102 cases of choroidal osteoma, including six misleading case reports which actually described idiopathic sclerochoroidal calcification and not choroidal osteoma. Clinical manifestation and the angiographic features of idiopathic sclerochoroidal calcification are outlined. The differential diagnosis of intraocular deposition of calcium salts is discussed in detail.

Idiopathic Sclerochoroidal Calcification

AbstractIdiopathic sclerochoroidal calcification is a disorder that is often overlooked, occurring at the level of the posterior sclera and choroid in older patients. Clinically, it may be confused with choroidal osteoma, choroidal metastasis, amelanotic choroidal nevus, or choroiditis. The authors present seven cases of this unusual entity. The patients ranged in age from 58 to 80 years. In all cases, the yellow lesions were clinically estimated to be at the level of the choroid and sclera, and they were usually located superior to the superotemporal retinal vascular arcade. Systemic investigation of calcium and phosphate metabolism was normal in all patients. The classic clinical manifestations and differentiation of this unusual entity from other similar fundus lesions is discussed.