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Karim M Talaat1
*, Khalid H Allam1
, Lujain T Idrees1
and Zahid Altaf2
1
Department of Ophthalmology, National Guard Hospital, Saudi Arabia
2
Department of Radiology, National Guard Hospital, Saudi Arabia
*Corresponding author: Karim Mohamed Talaat, Senior Consultant, Departments of Ophthalmology, Section Head, National Guard Hospital, PO Box
9515, Postal Code 21423, Jeddah, Saudi Arabia
Submission: January 13, 2018; Published: February 20, 2018
Choroidal Neovascular Membrane Association
with Tumoral Calcinosis, a Case Report
Case Report
A 40-year-old male with a history of Tumoral calcinosis
presented in our clinic on November 2011 complaining of
decreased visual acuity in his right eye for the past 2 weeks. His past
Medical history included arthritis in his large joints primarily his
knees, iron deficiency anemia and hyperphosphatemia. He was on
multiple medications including: Cholecalciferol, Ferrous Sulphate,
Folic Acid, Omeprazole and Multivitamins. His past Surgical history
was unremarkable.
On presentation to our ophthalmic service his visual acuity was
6/21 OD and 6/6 OS. Intraocular pressures were 13 mmhg OD and
12 mmhg OS by Tono-pen measurements. Slit Lamp examination
revealed perilimbal Calcification OU nasally otherwise a normal
anterior segment examination bilaterally. Fundus examination
showed myelinated nerve fibers, punched out chorioretinal lesions
in the periphery, angioid streaks and subretinal haemorrhage at
the macula with a central retinal thickness of 329 microns with
subretinal fluids. Flourescein angiogram confirmed presence of
subfoveal choroidal neovascular membrane OD.
Patient received Ranibizumab ( Lucentis, Novartis Pharm. Lim,
Horsham, West Sussex, UK) intravitreal injection 6th
of December
2011 , 18 days post intravitreal injection, BCVA right eye 6/24 and
OCT shows less subretinal fluid compared to first visit , in December
26 the thickness was 290 microns. Patient had his second lucentis
injection on 3rd
January 2012. On 24th
January 2012 (3 weeks
post injection) BCVA right eye 6/21, and OCT showed even less
subretinal fluid than what was previously noted.
Third Lucentis Injection was given on 31st
of January 2012, 3
weeks later the patient was followed up with a BCVA right eye 6/24
with almost no sub retinal fluid. On 20th
of March 2012 BCVA right
eye 6/60 with no sub retinal fluid. On 17th
of April 2012 BCVA of the
right eye 6/36 with a central retinal thickness of 276 microns with
no sub retinal fluid.
On 16th of May 2012 BCVA right eye is 6/36 OCT shows a small
pocket of sub retinal fluid in which we decide to inject Lucentis 4th
time which was performed on 29th
of May 2012 to regain some of
the lost visual acuity. Patient was then seen on 11th of June and the
BCVA 6/36 and the sub retinal fluid resolved. On 25th
of July OCT
showed Mild SRF under the fovea. Patient disappeared and showed
up 23rd
October 2012, with BCVA right eye 6/18-2, and OCT right
eye showing a dry macula. On 28 January 2013 BCVA 6/18 OCT Dry
Macula. On 13th
of March 2013, BCVA 6/60, OCT Macula dry, we
thought decrease visual acuity could be due to RPE atrophy, trial of
Lucentis advised, which was performed 19th
of March 2013.
One-month post injection VA didn’t improve and OCT still dry.
3 months later on 24th
of July 2013, BCVA 6/30 and left eye 6/6
patient was complaining of Visual Field disturbance in his left
eye Oct left eye shows no sub retinal fluid dry macula which was
confirmed with FFA showing NO CNV in left eye. On his last visit on
11/8/2015 his vision was CF right eye with no sub retinal fluid in
OCT and 6/6 left eye with no changes in OCT.
Discussion
Tumoralcalcinosis,isasubtypeofagroupofdisordersclassified
under calcinosis cutis. Calcinosis cutis is classified into four types
Case Report
1/2Copyright © All rights are reserved by Karim Mohamed Talaat.
Volume 1 - Issue - 3
Abstract
To report a case of Choroidal neovascular membrane in a tumoral calcinosis patient, 2 eyes from 1 patient with choroidal neovascular membrane
in one eye, a 40-year-old male with a history of Tumoral calcinosis presented with a choroidal neovascular membrane in his right eye, with his medical
work up consisting of elevated levels of phosphorus and normal levels of calcium, with radiological imaging consistent with the diagnosis of tumoral
calcinosis. We describe a case of choroidal neovascular membrane in tumoral calcinosis, and its response to Intravitreal anti-VEGF injections.
Keywords: Choroidal Neovascular Membrane; Intravitreal Injections; Tumoral Calcinosis
Medical & Surgical
Ophthalmology ResearchC CRIMSON PUBLISHERS
Wings to the Research
ISSN 2578-0360
Med Surg Ophthal Res Copyright © Karim Mohamed Talaat
2/2How to cite this article: Karim M T, Khalid H A, Lujain T I, Zahid A. Choroidal Neovascular Membrane Association with Tumoral Calcinosis, a Case Report.
Med Surg Ophthal Res. 1(3). MSOR.000514. 2018. DOI: 10.31031/MSOR.2018.01.000514
Volume 1 - Issue - 3
being metastatic, Dystrophic, Intraepidermal calcified nodules
and Idiopathic of which Tumoral calcinosis is a special form of the
Idiopathic type [1], it affects a certain age group of adolescents
to young adults [2] with special association and expressibility
in afro-Caribbean descent in particular [3]. The Term Tumoral
calcinosis was first described in literature in 1943 [4] with certain
characteristics as such of elevation of serum phosphate levels,
elevated renal tubular phosphate reabsorption and / or normal
or elevated serum 1,25-dihydroxyvitamin D [5]. It is characterized
with considerable subcutaneous deposits of calcium phosphate
near joints of upper and lower limbs such as elbows, hands
wrists, hips, knees and feet with these deposits consisting mainly
of pleomorphic calcium phosphate crystals. Keeping in mind that
calcium and parathyroid hormone levels being on a normal level
basis.
Ithasbeenlinkedwithcertainophthalmicpresentationsthatare
associated with calcification such as calcific lid lesions of the eyelid,
Band keratopathy, Conjunctival calcific lesions, angioid streaks and
choroidal neovascular membranes [6-14]. To our knowledge this
is the Third published case in the English literature that describes
the involvement of Tumoral Calcinosis with choriodal neovascular
membrane in the retina.
Perimbal calcification was present but no eyelid calcifications
which have been mentioned in previous studies [12,13], Radiologic
imagining revealed multi lobulated amorphous, cloud-like
calcification located in a periarticular distribution adjacent to the
right knee, inferior to the right shoulder and around the left hip
joint. No significant secondary bone changes.
Recent report in treatment of CNV secondary to angioid
streaks in cases of tumoral calcinosis shows great improvement
post anti-VEGF injection to affected eyes with CNV after a trial of
3 Lucentis injections and follow up over 6 months [12], however
in our particular patient the visual acuity fluctuated in response
to Anti-VEGF injections. Follow up period in total was 46 months,
which was longer than the previous 2 studies that mentioned the
association of CNVM in tumoral calcinosis patients.
With the small number of reports found in relation of tumoral
calcinosis associated with CNV it is difficult to establish a clear
treatment algorithm, further studies and investigations are needed
and patients with tumoral calcinosis are advised to visit the
ophthalmic clinic if they complain of changes in vision in order to
gain proper management.
Acknowledgments
We would like to thank Richard Florentino for his aid.
References
1.	 Maize J, Metcalf (1997) metabolic diseases of the skin. In: L Elder D
(Ed.), Lever’s Histopathology of the skin. (8th
edn), Lippincott- Raven,
Philadelphia, USA, pp. 379-382.
2.	 Datta c, Badnyopadhyay D, Bhattacharyya S, Ghosh S (2005) Tumoral
calcinosis. Indian J dermatol Venereol Leprol 71(4): 293-294.
3.	 Kluger G, Kochs A, Holthausen H (2000) Heterotopic ossification in
childhood and adolescence. J Child Neurol 15(6): 406-413.
4.	 Inclan A, Leon P, Carmejo MG (1943) Tumoral calcinosis. J Am Med Assoc
121(7): 490-495.
5.	 Mitnick PD, Goldfarb S, Slatopolsky E, Lemann J, Gray RW, et al. (1980)
Calcium and phosphate metabolism in tumoral calcinosis. Ann Intern
Med 92(4): 482-487.
6.	 Martinez S, Vogler JB, Harrelson JM (1990) Imaging of tumoral calcinosis:
new observations. Radiology 174(1): 215-222.
7.	 McKeePH,LiombaNG,HuttMS(1982)Tumoralcalcinosis:apathological
study of fifty-six cases. Br J Dermatol 107(6): 669-674.
8.	 McPhaulJJ,EngelFL(1961)Heterotopiccalcification,hyperphosphatemia
and angioid streaks of the retina. Am J Med 31: 488-492.
9.	 Bruns DE, Lieb W, Conway BP (1988) Band keratopathy and calcific lid
lesions in tumoral calcinosis. Case reports. Arch Ophthalmol 106(6):
725-726.
10.	Barton DL, Reeves RJ (1961) Tumoral calcinosis. Report of three cases
and review of the literature. Am J Roentgenol Radium Ther Nucl Med
86: 351-358.
11.	Lafferty FW, Reynolds ES, Pearson OH (1965) Tumoral calcinosis: a
metabolic disease of obscure etiology. Am J Med 38: 105-118.
12.	ClarkeE,SwischukLE,HaydenCK(1984)Tumoralcalcinosis,diaphysitis,
and hyperphosphatemia. Radiology 151(3): 643-646.
13.	McGrath E, Harney F, Kinsella F (2010) An ocular presentation of familial
tumoral calcinosis. Case Rep, BMJ, UK.
14.	Bhattacharjee H, Bhattacharjee K, Yambem D (2014) Ocular involvement
in tumoral calcinosis. Indian J Ophthalmol 62(8): 884-887.
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Choroidal Neovascular Membrane Association with Tumoral Calcinosis, a Case Report | Crimson Publishers

  • 1. Karim M Talaat1 *, Khalid H Allam1 , Lujain T Idrees1 and Zahid Altaf2 1 Department of Ophthalmology, National Guard Hospital, Saudi Arabia 2 Department of Radiology, National Guard Hospital, Saudi Arabia *Corresponding author: Karim Mohamed Talaat, Senior Consultant, Departments of Ophthalmology, Section Head, National Guard Hospital, PO Box 9515, Postal Code 21423, Jeddah, Saudi Arabia Submission: January 13, 2018; Published: February 20, 2018 Choroidal Neovascular Membrane Association with Tumoral Calcinosis, a Case Report Case Report A 40-year-old male with a history of Tumoral calcinosis presented in our clinic on November 2011 complaining of decreased visual acuity in his right eye for the past 2 weeks. His past Medical history included arthritis in his large joints primarily his knees, iron deficiency anemia and hyperphosphatemia. He was on multiple medications including: Cholecalciferol, Ferrous Sulphate, Folic Acid, Omeprazole and Multivitamins. His past Surgical history was unremarkable. On presentation to our ophthalmic service his visual acuity was 6/21 OD and 6/6 OS. Intraocular pressures were 13 mmhg OD and 12 mmhg OS by Tono-pen measurements. Slit Lamp examination revealed perilimbal Calcification OU nasally otherwise a normal anterior segment examination bilaterally. Fundus examination showed myelinated nerve fibers, punched out chorioretinal lesions in the periphery, angioid streaks and subretinal haemorrhage at the macula with a central retinal thickness of 329 microns with subretinal fluids. Flourescein angiogram confirmed presence of subfoveal choroidal neovascular membrane OD. Patient received Ranibizumab ( Lucentis, Novartis Pharm. Lim, Horsham, West Sussex, UK) intravitreal injection 6th of December 2011 , 18 days post intravitreal injection, BCVA right eye 6/24 and OCT shows less subretinal fluid compared to first visit , in December 26 the thickness was 290 microns. Patient had his second lucentis injection on 3rd January 2012. On 24th January 2012 (3 weeks post injection) BCVA right eye 6/21, and OCT showed even less subretinal fluid than what was previously noted. Third Lucentis Injection was given on 31st of January 2012, 3 weeks later the patient was followed up with a BCVA right eye 6/24 with almost no sub retinal fluid. On 20th of March 2012 BCVA right eye 6/60 with no sub retinal fluid. On 17th of April 2012 BCVA of the right eye 6/36 with a central retinal thickness of 276 microns with no sub retinal fluid. On 16th of May 2012 BCVA right eye is 6/36 OCT shows a small pocket of sub retinal fluid in which we decide to inject Lucentis 4th time which was performed on 29th of May 2012 to regain some of the lost visual acuity. Patient was then seen on 11th of June and the BCVA 6/36 and the sub retinal fluid resolved. On 25th of July OCT showed Mild SRF under the fovea. Patient disappeared and showed up 23rd October 2012, with BCVA right eye 6/18-2, and OCT right eye showing a dry macula. On 28 January 2013 BCVA 6/18 OCT Dry Macula. On 13th of March 2013, BCVA 6/60, OCT Macula dry, we thought decrease visual acuity could be due to RPE atrophy, trial of Lucentis advised, which was performed 19th of March 2013. One-month post injection VA didn’t improve and OCT still dry. 3 months later on 24th of July 2013, BCVA 6/30 and left eye 6/6 patient was complaining of Visual Field disturbance in his left eye Oct left eye shows no sub retinal fluid dry macula which was confirmed with FFA showing NO CNV in left eye. On his last visit on 11/8/2015 his vision was CF right eye with no sub retinal fluid in OCT and 6/6 left eye with no changes in OCT. Discussion Tumoralcalcinosis,isasubtypeofagroupofdisordersclassified under calcinosis cutis. Calcinosis cutis is classified into four types Case Report 1/2Copyright © All rights are reserved by Karim Mohamed Talaat. Volume 1 - Issue - 3 Abstract To report a case of Choroidal neovascular membrane in a tumoral calcinosis patient, 2 eyes from 1 patient with choroidal neovascular membrane in one eye, a 40-year-old male with a history of Tumoral calcinosis presented with a choroidal neovascular membrane in his right eye, with his medical work up consisting of elevated levels of phosphorus and normal levels of calcium, with radiological imaging consistent with the diagnosis of tumoral calcinosis. We describe a case of choroidal neovascular membrane in tumoral calcinosis, and its response to Intravitreal anti-VEGF injections. Keywords: Choroidal Neovascular Membrane; Intravitreal Injections; Tumoral Calcinosis Medical & Surgical Ophthalmology ResearchC CRIMSON PUBLISHERS Wings to the Research ISSN 2578-0360
  • 2. Med Surg Ophthal Res Copyright © Karim Mohamed Talaat 2/2How to cite this article: Karim M T, Khalid H A, Lujain T I, Zahid A. Choroidal Neovascular Membrane Association with Tumoral Calcinosis, a Case Report. Med Surg Ophthal Res. 1(3). MSOR.000514. 2018. DOI: 10.31031/MSOR.2018.01.000514 Volume 1 - Issue - 3 being metastatic, Dystrophic, Intraepidermal calcified nodules and Idiopathic of which Tumoral calcinosis is a special form of the Idiopathic type [1], it affects a certain age group of adolescents to young adults [2] with special association and expressibility in afro-Caribbean descent in particular [3]. The Term Tumoral calcinosis was first described in literature in 1943 [4] with certain characteristics as such of elevation of serum phosphate levels, elevated renal tubular phosphate reabsorption and / or normal or elevated serum 1,25-dihydroxyvitamin D [5]. It is characterized with considerable subcutaneous deposits of calcium phosphate near joints of upper and lower limbs such as elbows, hands wrists, hips, knees and feet with these deposits consisting mainly of pleomorphic calcium phosphate crystals. Keeping in mind that calcium and parathyroid hormone levels being on a normal level basis. Ithasbeenlinkedwithcertainophthalmicpresentationsthatare associated with calcification such as calcific lid lesions of the eyelid, Band keratopathy, Conjunctival calcific lesions, angioid streaks and choroidal neovascular membranes [6-14]. To our knowledge this is the Third published case in the English literature that describes the involvement of Tumoral Calcinosis with choriodal neovascular membrane in the retina. Perimbal calcification was present but no eyelid calcifications which have been mentioned in previous studies [12,13], Radiologic imagining revealed multi lobulated amorphous, cloud-like calcification located in a periarticular distribution adjacent to the right knee, inferior to the right shoulder and around the left hip joint. No significant secondary bone changes. Recent report in treatment of CNV secondary to angioid streaks in cases of tumoral calcinosis shows great improvement post anti-VEGF injection to affected eyes with CNV after a trial of 3 Lucentis injections and follow up over 6 months [12], however in our particular patient the visual acuity fluctuated in response to Anti-VEGF injections. Follow up period in total was 46 months, which was longer than the previous 2 studies that mentioned the association of CNVM in tumoral calcinosis patients. With the small number of reports found in relation of tumoral calcinosis associated with CNV it is difficult to establish a clear treatment algorithm, further studies and investigations are needed and patients with tumoral calcinosis are advised to visit the ophthalmic clinic if they complain of changes in vision in order to gain proper management. Acknowledgments We would like to thank Richard Florentino for his aid. References 1. Maize J, Metcalf (1997) metabolic diseases of the skin. In: L Elder D (Ed.), Lever’s Histopathology of the skin. (8th edn), Lippincott- Raven, Philadelphia, USA, pp. 379-382. 2. Datta c, Badnyopadhyay D, Bhattacharyya S, Ghosh S (2005) Tumoral calcinosis. Indian J dermatol Venereol Leprol 71(4): 293-294. 3. Kluger G, Kochs A, Holthausen H (2000) Heterotopic ossification in childhood and adolescence. J Child Neurol 15(6): 406-413. 4. Inclan A, Leon P, Carmejo MG (1943) Tumoral calcinosis. J Am Med Assoc 121(7): 490-495. 5. Mitnick PD, Goldfarb S, Slatopolsky E, Lemann J, Gray RW, et al. (1980) Calcium and phosphate metabolism in tumoral calcinosis. Ann Intern Med 92(4): 482-487. 6. Martinez S, Vogler JB, Harrelson JM (1990) Imaging of tumoral calcinosis: new observations. Radiology 174(1): 215-222. 7. McKeePH,LiombaNG,HuttMS(1982)Tumoralcalcinosis:apathological study of fifty-six cases. Br J Dermatol 107(6): 669-674. 8. McPhaulJJ,EngelFL(1961)Heterotopiccalcification,hyperphosphatemia and angioid streaks of the retina. Am J Med 31: 488-492. 9. Bruns DE, Lieb W, Conway BP (1988) Band keratopathy and calcific lid lesions in tumoral calcinosis. Case reports. Arch Ophthalmol 106(6): 725-726. 10. Barton DL, Reeves RJ (1961) Tumoral calcinosis. Report of three cases and review of the literature. Am J Roentgenol Radium Ther Nucl Med 86: 351-358. 11. Lafferty FW, Reynolds ES, Pearson OH (1965) Tumoral calcinosis: a metabolic disease of obscure etiology. Am J Med 38: 105-118. 12. ClarkeE,SwischukLE,HaydenCK(1984)Tumoralcalcinosis,diaphysitis, and hyperphosphatemia. Radiology 151(3): 643-646. 13. McGrath E, Harney F, Kinsella F (2010) An ocular presentation of familial tumoral calcinosis. Case Rep, BMJ, UK. 14. Bhattacharjee H, Bhattacharjee K, Yambem D (2014) Ocular involvement in tumoral calcinosis. Indian J Ophthalmol 62(8): 884-887. 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