Acta Ophthalmologica 2015
Editor, ipodystrophy is a group of heterogeneous diseases that is characterized by a depletion of subcutaneous adipose tissue. Acquired partial lipodystrophy (PLD) is a rare condition that involves a loss of fat only from the upper half of the body with a relative or absolute abundance over the lower half. Normally, adiposity of the lower limbs develops later than the atrophy of the upper parts. Laignel-Lavastine and Viard variety of partial lipodystrophy is characterized by adiposity of the lower limbs developing at the same time as the atrophy of the upper parts (Watson & Ritchie 1925). Systemic involvement is common and includes mesangiocapillary glomerulonephritis, insulin resistance and dermatomyositis. Currently, very little is known about the ocular manifestations of partial lipodystrophy. Here, we present a case of partial lipodystrophy of the LaignelLavastine and Viard type with retinal changes. A 27-year-old Caucasian woman was diagnosed with Laignel-Lavastine and Viard syndrome. On examination, she had symmetrical loss of subcutaneous fat in the upper half of the body with hypertrophy in the lower half. Her systemic examination was normal. Three years after diagnosis, she was referred for an ophthalmic examination by her optician. Visual acuity was 6/5 in both the eyes, the anterior chamber was deep and quiet, and the intraocular pressure was within normal range. Dilated fundus examination revealed round, well-defined, faintly yellow deposits resembling drusen of variable sizes in both maculae. These lesions were more in number and larger in size in the central macula (Fig. 1). The rest of the fundus examination was normal. Amsler grid testing and colour vision by Ishihara pseudoisochromatic plates were normal. Her full blood count, urea and electrolyte levels, liver function tests and renal function were normal. The antinuclear and extractable nuclear antibody (ENA) screen was negative. Complement screen showed low C3 at 0.5 g/l (0.75–1.6 g/l) and normal C4 complement (0.28 g/l). C3 factor was negative.
L
(A)
(B)
(C) Fig. 1. Fundoscopy of the left eye. There are hazy white peripapillary zones in the retina (A). Fluorescein angiography of the left eye. There is a juxtapapillary choroidal neovascularization membrane (B). Spectral domain optical coherence tomography of the left eye. There is a retinal oedema and serous neurosensory detachment involving the fovea (C).
7 days after intravitreal injection during the 4th week of pregnancy, and the second case happened 10 days after intravitreal injection during the 5th week of pregnancy. Thus, it is recommended that anti-VEGF should be given to a pregnant woman only if clearly needed, and especially not during the first trimester. Finally, it is not known whether anti-VEGF agents are secreted in human milk (Errera et al. 2013). Consequently, it is recommended to stop the lactation if injections are needed. Intravitreal ranibizumab during pregnancy was effective and safe to treat ICNV in this case report.
References Errera MH, Kohly RP, Kohly P & da Cruz L (2013): Pregnancy-associated retinal diseases and their management. Surv Ophthalmol 58: 127–142. Ho AC, Yannuzzi LA, Pisicano K & DeRosa J (1995): The natural history of idiopathic subfoveal choroidal neovascularization. Ophthalmology 102: 782–789. Ouhadj O, Degheb N, Idir S et al. (2012): Intravitreal ranibizumab injection for the treatment of idiopathic choroidal neovascularisation in young patients. J Fr Ophthalmol 35: 514–522. Petrou P, Georgalas I, Giavaras G et al. (2010): Early loss of pregnancy after intravitreal bevacizumab injection. Acta Ophthalmol 88: e136.
e598
Zhang H, Liu ZL, Sun P & Gu F (2012): Intravitreal bevacizumab for treatment of subfoveal idiopathic choroidal neovascularization: results of a 1-year prospective trial. Am J Ophthalmol 153: 300–306.
Correspondence: Le´a Jouve 28 rue de Charenton 75012 Paris France Tel: +33 140 021 507 Fax: +33 142 630 885 Email: [email protected]
Retinal changes in a patient with acquired partial lipodystrophy (Laignel-Lavastine and Viard Syndrome) Ganne Pratyusha1 and Marcela Votruba1,2 1
School of Optometry & Vision Sciences, Cardiff University, Cardiff, UK, 2Cardiff Eye Unit, University Hospital of Wales, Cardiff, UK doi: 10.1111/aos.12690
Acta Ophthalmologica 2015
Editor, revalence of myelinated retinal nerve fibres (MRNFs) in the general population have only been investigated in a few study so far. An incidence of myelinated nerve fibres of 0.3–0.6% has been reported by Duke et al. (1963). There are also not much data regarding its clinical features in large populations. Aiming to investigate the prevalence of MRNFs in the Turkish population and to determine those patients’ clinical characteristics, we performed a prospective population-based cross-sectional study including 6250 otherwise healthy individuals (aged between 8 and 75 years). All subjects were examined in Izmir University Faculty of Medicine, Department of Ophthalmology between June 2013 and December 2014. Each subject underwent full ophthalmic examination including BCVA, tonometry, fundus photography and OCT. Moreover, categorization of the MRNF lesions was made using the classification system by (Ellis et al. (1987), which was defined as follows: Type I (one temporal arcade), Type II (both temporal arcades) and Type III (not contiguous with optic disc). Readable fundus photographs were available for 5580 eyes (89.2%) with a mean age of 45.3 15.6 years. Twenty-three eyes of 19 subjects (10 men and nine women) had MRNF lesion. We found MRNFs’ incidence as 0.34% in patients and 0.21% in eyes examined. 21% of the cases were bilateral. In a series of 3,968 consecutive autopsy cases, Straatsma et al. (1981) reported that MRNFs were present in 0.98% of patients and in 0.54% of eyes examined, with bilateral involvement in 7.7% of patients. Also, similar to that study, males and females were equally affected in our series. When the eyes with MRNFs were analysed according to the lesion location, 14 eyes were classified as Type I, seven eyes as Type II and two eyes as Type III (Fig. 1). Amongst the patients with MRNFs, we observed myopia in 10 (52%), hyperopia in four (21%) and emetropia in five patients (26%). Also, 4 of 19 patients (21%) had amblyopia and myopia, but no manifest strabismus. Similar to the results of the Beijing eye study, we found no association between MRNF presence and refractive error, age and gender (You et al. 2007).
P
Fig. 1. Fundus photographs showing drusenoid changes at the macula.
Subsequent follow-up after 1 year revealed that she was still asymptomatic and her visual acuity was still 6/5 in both the eyes. There was no distortion on Amsler grid or any evidence of colour vision anomaly. The aetiology of partial lipodystrophy is uncertain. However, it is postulated that there could be an immunological destruction of adipocytes due to associated presence of C3 nephritic factor, antiphospholipid, antinuclear and anti-double-stranded DNA antibodies (Caballero et al. 2003). For a long time, ocular changes in partial lipodystrophy (retinal pigment epithelial changes and drusen) were thought to be associated with mesangiocapillary glomerulonephritis. The anatomy of the glomerulus and the complex of choriocapillaris, Bruch’s membrane and retinal pigment epithelium are similar. The drusenoid deposits in the retina are histologically similar to the dense deposits in the glomerular basement membrane (Colville et al. 2003). However, there are a few case reports suggesting ocular involvement in the absence of renal involvement. Changes noted include drusen at macula and choroidal neovascular membranes (Jansen et al. 2013; Broadhead & Chang 2013). However, to our knowledge, there are no reports of a Laignel-Lavastine and Viard type of PLD with macular changes. It has to be noted that this patient also had no obvious renal dysfunction. It may be that this patient will develop renal dysfunction in future and if so, ocular screening may help us to recognize predisposition to renal involvement in these patients. This case emphasizes the need to screen patients with partial lipodystrophy with or without renal disease for retinal changes as they can be potentially vision threatening.
References Broadhead GK & Chang A (2013): Choroidal neovascularization in acquired partial lipodystrophy. Eur J Ophthalmol 23: 439–444. Caballero CR, Mira MA, Bosch IF, Bauxauli JM & Aliaga BA (2003): Barraquer-Simons lipodystrophy associated with antiphospholipid syndrome. J Am Acad Dermatol 49: 768–769. Colville D, Guymer R, Sinclair RA & Savige J (2003): Visual impairment caused by retinal abnormalities in mesangiocapillary (membranoproliferative) glomerulonephritis type II (“dense depositdisease”).AmJKidneyDis42:E3.1–E3.4. Jansen J, Delaere L, Spielberg L & Leys A (2013): Long-term fundus changes in acquired partial lipodystrophy. BMJ Case Rep. pii: bcr2013201218. [Epub ahead of print]. Watson WN & Ritchie WT (1925): Progressive lipodystrophy. QJM 18: 224–239. Correspondence: Prof. Marcela Votruba School of Optometry & Vision Sciences Cardiff University Maindy Road Cardiff, CF24 4LU UK Tel: 00 44 29 2087 0134 Fax: 00 44 29 20874859 Email: votrubam@cardiff.ac.uk
Prevalence and clinical characteristics of myelinated retinal nerve fibres: a cross-sectional study of Turkish individuals between 8 and 75 years Esat Cinar, Mehmet Ozgur Zengin and Cem Kucukerdonmez Department of Ophthalmology, Izmir University Faculty of Medicine, Izmir, Turkey doi: 10.1111/aos.12706
e599
Editor, ipodystrophy is a group of heterogeneous diseases that is characterized by a depletion of subcutaneous adipose tissue. Acquired partial lipodystrophy (PLD) is a rare condition that involves a loss of fat only from the upper half of the body with a relative or absolute abundance over the lower half. Normally, adiposity of the lower limbs develops later than the atrophy of the upper parts. Laignel-Lavastine and Viard variety of partial lipodystrophy is characterized by adiposity of the lower limbs developing at the same time as the atrophy of the upper parts (Watson & Ritchie 1925). Systemic involvement is common and includes mesangiocapillary glomerulonephritis, insulin resistance and dermatomyositis. Currently, very little is known about the ocular manifestations of partial lipodystrophy. Here, we present a case of partial lipodystrophy of the LaignelLavastine and Viard type with retinal changes. A 27-year-old Caucasian woman was diagnosed with Laignel-Lavastine and Viard syndrome. On examination, she had symmetrical loss of subcutaneous fat in the upper half of the body with hypertrophy in the lower half. Her systemic examination was normal. Three years after diagnosis, she was referred for an ophthalmic examination by her optician. Visual acuity was 6/5 in both the eyes, the anterior chamber was deep and quiet, and the intraocular pressure was within normal range. Dilated fundus examination revealed round, well-defined, faintly yellow deposits resembling drusen of variable sizes in both maculae. These lesions were more in number and larger in size in the central macula (Fig. 1). The rest of the fundus examination was normal. Amsler grid testing and colour vision by Ishihara pseudoisochromatic plates were normal. Her full blood count, urea and electrolyte levels, liver function tests and renal function were normal. The antinuclear and extractable nuclear antibody (ENA) screen was negative. Complement screen showed low C3 at 0.5 g/l (0.75–1.6 g/l) and normal C4 complement (0.28 g/l). C3 factor was negative.
L
(A)
(B)
(C) Fig. 1. Fundoscopy of the left eye. There are hazy white peripapillary zones in the retina (A). Fluorescein angiography of the left eye. There is a juxtapapillary choroidal neovascularization membrane (B). Spectral domain optical coherence tomography of the left eye. There is a retinal oedema and serous neurosensory detachment involving the fovea (C).
7 days after intravitreal injection during the 4th week of pregnancy, and the second case happened 10 days after intravitreal injection during the 5th week of pregnancy. Thus, it is recommended that anti-VEGF should be given to a pregnant woman only if clearly needed, and especially not during the first trimester. Finally, it is not known whether anti-VEGF agents are secreted in human milk (Errera et al. 2013). Consequently, it is recommended to stop the lactation if injections are needed. Intravitreal ranibizumab during pregnancy was effective and safe to treat ICNV in this case report.
References Errera MH, Kohly RP, Kohly P & da Cruz L (2013): Pregnancy-associated retinal diseases and their management. Surv Ophthalmol 58: 127–142. Ho AC, Yannuzzi LA, Pisicano K & DeRosa J (1995): The natural history of idiopathic subfoveal choroidal neovascularization. Ophthalmology 102: 782–789. Ouhadj O, Degheb N, Idir S et al. (2012): Intravitreal ranibizumab injection for the treatment of idiopathic choroidal neovascularisation in young patients. J Fr Ophthalmol 35: 514–522. Petrou P, Georgalas I, Giavaras G et al. (2010): Early loss of pregnancy after intravitreal bevacizumab injection. Acta Ophthalmol 88: e136.
e598
Zhang H, Liu ZL, Sun P & Gu F (2012): Intravitreal bevacizumab for treatment of subfoveal idiopathic choroidal neovascularization: results of a 1-year prospective trial. Am J Ophthalmol 153: 300–306.
Correspondence: Le´a Jouve 28 rue de Charenton 75012 Paris France Tel: +33 140 021 507 Fax: +33 142 630 885 Email: [email protected]
Retinal changes in a patient with acquired partial lipodystrophy (Laignel-Lavastine and Viard Syndrome) Ganne Pratyusha1 and Marcela Votruba1,2 1
School of Optometry & Vision Sciences, Cardiff University, Cardiff, UK, 2Cardiff Eye Unit, University Hospital of Wales, Cardiff, UK doi: 10.1111/aos.12690
Acta Ophthalmologica 2015
Editor, revalence of myelinated retinal nerve fibres (MRNFs) in the general population have only been investigated in a few study so far. An incidence of myelinated nerve fibres of 0.3–0.6% has been reported by Duke et al. (1963). There are also not much data regarding its clinical features in large populations. Aiming to investigate the prevalence of MRNFs in the Turkish population and to determine those patients’ clinical characteristics, we performed a prospective population-based cross-sectional study including 6250 otherwise healthy individuals (aged between 8 and 75 years). All subjects were examined in Izmir University Faculty of Medicine, Department of Ophthalmology between June 2013 and December 2014. Each subject underwent full ophthalmic examination including BCVA, tonometry, fundus photography and OCT. Moreover, categorization of the MRNF lesions was made using the classification system by (Ellis et al. (1987), which was defined as follows: Type I (one temporal arcade), Type II (both temporal arcades) and Type III (not contiguous with optic disc). Readable fundus photographs were available for 5580 eyes (89.2%) with a mean age of 45.3 15.6 years. Twenty-three eyes of 19 subjects (10 men and nine women) had MRNF lesion. We found MRNFs’ incidence as 0.34% in patients and 0.21% in eyes examined. 21% of the cases were bilateral. In a series of 3,968 consecutive autopsy cases, Straatsma et al. (1981) reported that MRNFs were present in 0.98% of patients and in 0.54% of eyes examined, with bilateral involvement in 7.7% of patients. Also, similar to that study, males and females were equally affected in our series. When the eyes with MRNFs were analysed according to the lesion location, 14 eyes were classified as Type I, seven eyes as Type II and two eyes as Type III (Fig. 1). Amongst the patients with MRNFs, we observed myopia in 10 (52%), hyperopia in four (21%) and emetropia in five patients (26%). Also, 4 of 19 patients (21%) had amblyopia and myopia, but no manifest strabismus. Similar to the results of the Beijing eye study, we found no association between MRNF presence and refractive error, age and gender (You et al. 2007).
P
Fig. 1. Fundus photographs showing drusenoid changes at the macula.
Subsequent follow-up after 1 year revealed that she was still asymptomatic and her visual acuity was still 6/5 in both the eyes. There was no distortion on Amsler grid or any evidence of colour vision anomaly. The aetiology of partial lipodystrophy is uncertain. However, it is postulated that there could be an immunological destruction of adipocytes due to associated presence of C3 nephritic factor, antiphospholipid, antinuclear and anti-double-stranded DNA antibodies (Caballero et al. 2003). For a long time, ocular changes in partial lipodystrophy (retinal pigment epithelial changes and drusen) were thought to be associated with mesangiocapillary glomerulonephritis. The anatomy of the glomerulus and the complex of choriocapillaris, Bruch’s membrane and retinal pigment epithelium are similar. The drusenoid deposits in the retina are histologically similar to the dense deposits in the glomerular basement membrane (Colville et al. 2003). However, there are a few case reports suggesting ocular involvement in the absence of renal involvement. Changes noted include drusen at macula and choroidal neovascular membranes (Jansen et al. 2013; Broadhead & Chang 2013). However, to our knowledge, there are no reports of a Laignel-Lavastine and Viard type of PLD with macular changes. It has to be noted that this patient also had no obvious renal dysfunction. It may be that this patient will develop renal dysfunction in future and if so, ocular screening may help us to recognize predisposition to renal involvement in these patients. This case emphasizes the need to screen patients with partial lipodystrophy with or without renal disease for retinal changes as they can be potentially vision threatening.
References Broadhead GK & Chang A (2013): Choroidal neovascularization in acquired partial lipodystrophy. Eur J Ophthalmol 23: 439–444. Caballero CR, Mira MA, Bosch IF, Bauxauli JM & Aliaga BA (2003): Barraquer-Simons lipodystrophy associated with antiphospholipid syndrome. J Am Acad Dermatol 49: 768–769. Colville D, Guymer R, Sinclair RA & Savige J (2003): Visual impairment caused by retinal abnormalities in mesangiocapillary (membranoproliferative) glomerulonephritis type II (“dense depositdisease”).AmJKidneyDis42:E3.1–E3.4. Jansen J, Delaere L, Spielberg L & Leys A (2013): Long-term fundus changes in acquired partial lipodystrophy. BMJ Case Rep. pii: bcr2013201218. [Epub ahead of print]. Watson WN & Ritchie WT (1925): Progressive lipodystrophy. QJM 18: 224–239. Correspondence: Prof. Marcela Votruba School of Optometry & Vision Sciences Cardiff University Maindy Road Cardiff, CF24 4LU UK Tel: 00 44 29 2087 0134 Fax: 00 44 29 20874859 Email: votrubam@cardiff.ac.uk
Prevalence and clinical characteristics of myelinated retinal nerve fibres: a cross-sectional study of Turkish individuals between 8 and 75 years Esat Cinar, Mehmet Ozgur Zengin and Cem Kucukerdonmez Department of Ophthalmology, Izmir University Faculty of Medicine, Izmir, Turkey doi: 10.1111/aos.12706
e599
