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Severe and acute loss of visual field in a young patient with optic disk drusen
a r c h s o c e s p o f t a l m o l . 2 0 1 4;8 9(8):324–328
ARCHIVOS DE LA SOCIEDAD ESPAÑOLA DE OFTALMOLOGÍA www.elsevier.es/oftalmologia
Short communication
Severe and acute loss of visual field in a young patient with optic disk drusen夽 M. Moreno ∗ , A.M. Vazquez, R. Dominguez, M. Rosas Servicio de Oftalmología, Hospital Virgen de la Merced de Osuna, Sevilla, Spain
a r t i c l e
i n f o
a b s t r a c t
Article history:
Case report: We describe a case of a young patient with bilateral optic disk drusen, who in
Received 26 June 2011
the course of 10 months, had a sudden visual field constriction in the right eye accompanied
Accepted 11 June 2013
by moderate loss of vision, corresponding to the ophthalmological examination of changing
Available online 26 September 2014
from hidden to visible drusen.
Keywords:
a benign disorder due to the low frequency of complications that appear during their course.
Optic disk drusen
This case shows how they can become a real problem in a short period of time.
Discussion: The optic disk drusen are a fairly common disorder that is mainly considered as
Visual field constriction
˜ © 2011 Sociedad Espanola de Oftalmología. Published by Elsevier España, S.L.U. All rights reserved.
Anterior ischemic optic neuropathy
Pérdida severa y aguda de campo visual en paciente joven con drusas de papila r e s u m e n Palabras clave:
Caso clínico: Presentamos el caso de un paciente joven con drusas papilares bilaterales que
Drusas de papila
en el curso de 10 meses ha presentado una importante reducción de campo visual en ojo
Alteración del campo visual
derecho con pérdida moderada de visión, correspondiéndose la exploración oftalmológica
Neuropatía óptica isquémica
con el paso de drusas ocultas a visibles.
anterior
Discusión: Las drusas de papila son un hallazgo relativamente frecuente y considerado como una enfermedad benigna en la mayoría de las ocasiones por la baja frecuencia de complicaciones que aparecen en su evolución; este caso muestra cómo pueden convertirse en un verdadero problema en un corto periodo de tiempo. ˜ © 2011 Sociedad Espanola de Oftalmología. Publicado por Elsevier España, S.L.U. Todos los derechos reservados.
夽
Please cite this article as: Moreno M, Vazquez AM, Dominguez R, Rosas M. Pérdida severa y aguda de campo visual en paciente joven con drusas de papila. Arch Soc Esp Oftalmol. 2014;89:324–328. ∗ Corresponding author. E-mail address: [email protected] (M. Moreno). ˜ 2173-5794/$ – see front matter © 2011 Sociedad Espanola de Oftalmología. Published by Elsevier España, S.L.U. All rights reserved.
a r c h s o c e s p o f t a l m o l . 2 0 1 4;8 9(8):324–328
325
Case report A 13-year-old male who in February 2010 is seen for blurred vision in his left eye (OS) and headaches; vision improves as headache strength reduces. Personal history shows that in 2009 the patient had sweating and nausea episodes without loss of consciousness, accompanied by unilateral headache, blurred vision and drowsiness after testing negative in analytical, magnetic resonance imaging [MRI], and electroencephalogram tests.
Examination Visual acuity is 1 in both eyes, without refractive error, tropias or phorias, no pupillary defect, normal anterior segment and intraocular pressure (IOP). Fundus examination shows the following: – Right eye (OD): papillary elevation without signs of vascular stasis, negative spontaneous venous pulse. – Left eye (OS): visible optic disk drusen (Fig. 1). Ultrasound shows typical signs of optic disk drusen in both eyes (Fig. 2a). Central visual field 24-2, threshold test with Humphrey Field Analyzer (HFA II 745) shows a significant reduction in OS, not involving the central 10◦ . OD field shows nasal defect (Fig. 5a). Computed tomography (CT) shows disk calcification in both optic nerves (Fig. 2b). After diagnosing optic disk drusen, an appointment is set up to follow-up on visual fields. On August 2010, patient is seen for severe loss of vision in OD, 24 h from onset, including more frequent headaches and fatigue. Visual acuity in OD is 0.4, with severe visual field loss affecting attachment area (Fig. 5b), elevated and swollen optic disk, and no other accompanying signs (Fig. 3). OS scan is identical to the first one. Given the diagnostic uncertainty (and family pressure), patient is admitted and intravenous bolus corticoid injection is administered for 3 days (1 mg/kg weight/day). Laboratory tests were normal: mild elevation of erythrocyte sedimentation rate with standard C-reactive protein. MRI is normal. Neurological examination and photopic/scotopic electroretinogram are normal in both eyes, while visual evoked potentials of both eyes show delayed P100 wave latency. In the following weeks, both visual acuity and visual field remain unchanged. On October 2010, visible and abundant drusen appear in the papilla of OD with vascularization normalization (Fig. 4); the field is clearly worse than the previous (Fig. 5c), and visual acuity increased to 0.7. OS situation remains unchanged. Patient remains stable during follow-up.
Fig. 1 – Baseline symptoms: papilla pseudoedema in the right eye. Abundant and visible drusen in the left eye.
Discussion Optic nerve drusen have a prevalence of about 1%, and 75–85% are bilateral. It is believed they are transmitted with incomplete penetrance autosomal dominant inheritance; the main risk factor for its development is hereditary dysplasia of the optic nerve head. This dysplasia would be the cause of vascular involvement1 and alteration of axoplasmic transport, which would cause accumulation of mucopolysaccharides and proteinaceous material inside the optic nerve in front of the lamina cribrosa2 (hidden drusen), which with time and continuous deposited calcium increase in size and hardness becoming visible under ophthalmoscopic inspection (visible drusen) and resulting in visual field defects in a considerable number of patients. Visual field defects are usually slowly progressive, although sudden and severe field loss have been reported due to vascular complications such as anterior ischemic neuropathy3
326
a r c h s o c e s p o f t a l m o l . 2 0 1 4;8 9(8):324–328
Fig. 2 – (a) The low gain ultrasound B clearly shows drusen in both eyes. (b) CT reveals clearly calcification at the papilla in both eyes.
and may also be related to the mere presence of drusen4 or glaucoma. An Auw-Haedrich study in 2002 suggests that optic drusen renders the nerve more susceptible to glaucomatous damage at lower pressures than in the general population; therefore, antiglaucoma therapy must be performed in cases
Fig. 3 – Right ocular fundus appearance in August 2010, which corresponds to a decrease in visual acuity and visual field reduction.
where there is visual field deterioration and IOP in normal limits.1 Patients with drusen are usually asymptomatic, although sometimes complaints of transient obscurations of vision, with good central visual acuity. Although visual examination alone is often sufficient to confirm its presence, for hidden drusen (differential diagnosis with true papilledema is recommended), ultrasound B-mode with low gain is the most cost-effective way to diagnose. Other additional examinations are neither necessary nor cost effective to diagnose it, although sometimes they need to be performed (non-visible drusen, decreased vision, related neurological symptoms, etc. . .). This case seems very interesting in regard to the fact that it clearly shows the clinicopathological correlation. The change from hidden to visible drusen is related to decreased acuity and significant contraction of the visual field, which leads us to believe that the conflict of the space produced induces anterior compressive neuropathy (ischemic), responsible for the clinical condition. Optic disk drusen diagnosis, specifically pseudopapilledema from drusen, must be a forewarning to conduct tests, not only on the ocular fundus but also serialized vision field assessment, allowing us to detect changes and start antiglaucoma treatment although there is no clear evidence of ocular hypertension.
a r c h s o c e s p o f t a l m o l . 2 0 1 4;8 9(8):324–328
327
Fig. 4 – Visible drusen in both eyes in examination dated October 2010, which correlates with significant right eye visual field contraction.
328
a r c h s o c e s p o f t a l m o l . 2 0 1 4;8 9(8):324–328
30
30
January 2010
30
30
August 2010
30
30
October 2010 Fig. 5 – Visual field progression.
Conflicts of interest The authors declare that they have no conflicts of interest.
references
1. Auw-Haedrich C, Staubach F, Witschel H. Optic disk drusen. Surv Ophthalmol. 2002;47:515–32.
2. XXXIV Edward Jackson Memorial Lecture: drusen of the optic disc and aberrant axoplasmic transport. Ophthalmology. 1978;85:21–38. 3. Sarkies NJ, Sanders MD. Optic disc drusen and episodic visual loss. Br J Ophthalmol. 1987;71:537–9. 4. Moody TA, Irvine AR, Cahn PH, Susac JO, Horton JC. Sudden visual field constriction associated with optic disc drusen. J Clin Neuroophthalmol. 1993;13:8–13, discussion 14.
ARCHIVOS DE LA SOCIEDAD ESPAÑOLA DE OFTALMOLOGÍA www.elsevier.es/oftalmologia
Short communication
Severe and acute loss of visual field in a young patient with optic disk drusen夽 M. Moreno ∗ , A.M. Vazquez, R. Dominguez, M. Rosas Servicio de Oftalmología, Hospital Virgen de la Merced de Osuna, Sevilla, Spain
a r t i c l e
i n f o
a b s t r a c t
Article history:
Case report: We describe a case of a young patient with bilateral optic disk drusen, who in
Received 26 June 2011
the course of 10 months, had a sudden visual field constriction in the right eye accompanied
Accepted 11 June 2013
by moderate loss of vision, corresponding to the ophthalmological examination of changing
Available online 26 September 2014
from hidden to visible drusen.
Keywords:
a benign disorder due to the low frequency of complications that appear during their course.
Optic disk drusen
This case shows how they can become a real problem in a short period of time.
Discussion: The optic disk drusen are a fairly common disorder that is mainly considered as
Visual field constriction
˜ © 2011 Sociedad Espanola de Oftalmología. Published by Elsevier España, S.L.U. All rights reserved.
Anterior ischemic optic neuropathy
Pérdida severa y aguda de campo visual en paciente joven con drusas de papila r e s u m e n Palabras clave:
Caso clínico: Presentamos el caso de un paciente joven con drusas papilares bilaterales que
Drusas de papila
en el curso de 10 meses ha presentado una importante reducción de campo visual en ojo
Alteración del campo visual
derecho con pérdida moderada de visión, correspondiéndose la exploración oftalmológica
Neuropatía óptica isquémica
con el paso de drusas ocultas a visibles.
anterior
Discusión: Las drusas de papila son un hallazgo relativamente frecuente y considerado como una enfermedad benigna en la mayoría de las ocasiones por la baja frecuencia de complicaciones que aparecen en su evolución; este caso muestra cómo pueden convertirse en un verdadero problema en un corto periodo de tiempo. ˜ © 2011 Sociedad Espanola de Oftalmología. Publicado por Elsevier España, S.L.U. Todos los derechos reservados.
夽
Please cite this article as: Moreno M, Vazquez AM, Dominguez R, Rosas M. Pérdida severa y aguda de campo visual en paciente joven con drusas de papila. Arch Soc Esp Oftalmol. 2014;89:324–328. ∗ Corresponding author. E-mail address: [email protected] (M. Moreno). ˜ 2173-5794/$ – see front matter © 2011 Sociedad Espanola de Oftalmología. Published by Elsevier España, S.L.U. All rights reserved.
a r c h s o c e s p o f t a l m o l . 2 0 1 4;8 9(8):324–328
325
Case report A 13-year-old male who in February 2010 is seen for blurred vision in his left eye (OS) and headaches; vision improves as headache strength reduces. Personal history shows that in 2009 the patient had sweating and nausea episodes without loss of consciousness, accompanied by unilateral headache, blurred vision and drowsiness after testing negative in analytical, magnetic resonance imaging [MRI], and electroencephalogram tests.
Examination Visual acuity is 1 in both eyes, without refractive error, tropias or phorias, no pupillary defect, normal anterior segment and intraocular pressure (IOP). Fundus examination shows the following: – Right eye (OD): papillary elevation without signs of vascular stasis, negative spontaneous venous pulse. – Left eye (OS): visible optic disk drusen (Fig. 1). Ultrasound shows typical signs of optic disk drusen in both eyes (Fig. 2a). Central visual field 24-2, threshold test with Humphrey Field Analyzer (HFA II 745) shows a significant reduction in OS, not involving the central 10◦ . OD field shows nasal defect (Fig. 5a). Computed tomography (CT) shows disk calcification in both optic nerves (Fig. 2b). After diagnosing optic disk drusen, an appointment is set up to follow-up on visual fields. On August 2010, patient is seen for severe loss of vision in OD, 24 h from onset, including more frequent headaches and fatigue. Visual acuity in OD is 0.4, with severe visual field loss affecting attachment area (Fig. 5b), elevated and swollen optic disk, and no other accompanying signs (Fig. 3). OS scan is identical to the first one. Given the diagnostic uncertainty (and family pressure), patient is admitted and intravenous bolus corticoid injection is administered for 3 days (1 mg/kg weight/day). Laboratory tests were normal: mild elevation of erythrocyte sedimentation rate with standard C-reactive protein. MRI is normal. Neurological examination and photopic/scotopic electroretinogram are normal in both eyes, while visual evoked potentials of both eyes show delayed P100 wave latency. In the following weeks, both visual acuity and visual field remain unchanged. On October 2010, visible and abundant drusen appear in the papilla of OD with vascularization normalization (Fig. 4); the field is clearly worse than the previous (Fig. 5c), and visual acuity increased to 0.7. OS situation remains unchanged. Patient remains stable during follow-up.
Fig. 1 – Baseline symptoms: papilla pseudoedema in the right eye. Abundant and visible drusen in the left eye.
Discussion Optic nerve drusen have a prevalence of about 1%, and 75–85% are bilateral. It is believed they are transmitted with incomplete penetrance autosomal dominant inheritance; the main risk factor for its development is hereditary dysplasia of the optic nerve head. This dysplasia would be the cause of vascular involvement1 and alteration of axoplasmic transport, which would cause accumulation of mucopolysaccharides and proteinaceous material inside the optic nerve in front of the lamina cribrosa2 (hidden drusen), which with time and continuous deposited calcium increase in size and hardness becoming visible under ophthalmoscopic inspection (visible drusen) and resulting in visual field defects in a considerable number of patients. Visual field defects are usually slowly progressive, although sudden and severe field loss have been reported due to vascular complications such as anterior ischemic neuropathy3
326
a r c h s o c e s p o f t a l m o l . 2 0 1 4;8 9(8):324–328
Fig. 2 – (a) The low gain ultrasound B clearly shows drusen in both eyes. (b) CT reveals clearly calcification at the papilla in both eyes.
and may also be related to the mere presence of drusen4 or glaucoma. An Auw-Haedrich study in 2002 suggests that optic drusen renders the nerve more susceptible to glaucomatous damage at lower pressures than in the general population; therefore, antiglaucoma therapy must be performed in cases
Fig. 3 – Right ocular fundus appearance in August 2010, which corresponds to a decrease in visual acuity and visual field reduction.
where there is visual field deterioration and IOP in normal limits.1 Patients with drusen are usually asymptomatic, although sometimes complaints of transient obscurations of vision, with good central visual acuity. Although visual examination alone is often sufficient to confirm its presence, for hidden drusen (differential diagnosis with true papilledema is recommended), ultrasound B-mode with low gain is the most cost-effective way to diagnose. Other additional examinations are neither necessary nor cost effective to diagnose it, although sometimes they need to be performed (non-visible drusen, decreased vision, related neurological symptoms, etc. . .). This case seems very interesting in regard to the fact that it clearly shows the clinicopathological correlation. The change from hidden to visible drusen is related to decreased acuity and significant contraction of the visual field, which leads us to believe that the conflict of the space produced induces anterior compressive neuropathy (ischemic), responsible for the clinical condition. Optic disk drusen diagnosis, specifically pseudopapilledema from drusen, must be a forewarning to conduct tests, not only on the ocular fundus but also serialized vision field assessment, allowing us to detect changes and start antiglaucoma treatment although there is no clear evidence of ocular hypertension.
a r c h s o c e s p o f t a l m o l . 2 0 1 4;8 9(8):324–328
327
Fig. 4 – Visible drusen in both eyes in examination dated October 2010, which correlates with significant right eye visual field contraction.
328
a r c h s o c e s p o f t a l m o l . 2 0 1 4;8 9(8):324–328
30
30
January 2010
30
30
August 2010
30
30
October 2010 Fig. 5 – Visual field progression.
Conflicts of interest The authors declare that they have no conflicts of interest.
references
1. Auw-Haedrich C, Staubach F, Witschel H. Optic disk drusen. Surv Ophthalmol. 2002;47:515–32.
2. XXXIV Edward Jackson Memorial Lecture: drusen of the optic disc and aberrant axoplasmic transport. Ophthalmology. 1978;85:21–38. 3. Sarkies NJ, Sanders MD. Optic disc drusen and episodic visual loss. Br J Ophthalmol. 1987;71:537–9. 4. Moody TA, Irvine AR, Cahn PH, Susac JO, Horton JC. Sudden visual field constriction associated with optic disc drusen. J Clin Neuroophthalmol. 1993;13:8–13, discussion 14.