DIVING-RELATED VISUAL LOSS IN THE SETTING OF ANGIOID STREAKS: REPORT OF TWO CASES Maria I. Angulo Bocco, MD,* Leigh Spielberg, MD,* Greet Coppens, MD,* Janet Catherine, MD,† Claire Verougstraete, MD,† Anita M. Leys, MD, PhD*
Purpose: The purpose of this study was to report diving-related visual loss in the setting of angioid streaks. Methods: Observational case reports of two patients with angioid streaks suffering sudden visual loss immediately after diving. Results: Two young adult male patients presented with visual loss after diving headfirst. Funduscopy revealed angioid streaks, peau d’orange, subretinal hemorrhages, and ruptures of Bruch membrane. Choroidal neovascularization developed during follow-up. Both patients had an otherwise uneventful personal and familial medical history. Conclusion: In patients with angioid streaks, diving headfirst can lead to subretinal hemorrhages and traumatic ruptures in Bruch membrane and increase the risk of maculopathy. Ophthalmologists should caution patients with angioid streaks against diving headfirst. RETINAL CASES & BRIEF REPORTS 6:223–227, 2012
skin lesions can be observed in adulthood and are rarely observed in childhood. They are most prominent in neck and on the large flexor surfaces, and biopsy of these lesions usually leads to the diagnosis of PXE. Gastrointestinal bleedings and peripheral and coronary arterial occlusive disease are well-known systemic complications. The underlying defect is a mutation in the ABCC6 gene on chromosome 16p13.1, which results in abnormal transmembranous transport, accumulation of extracellular material, and calcification of elastic fibers. Because of the fragility of Bruch membrane in angioid streaks, ruptures of Bruch membrane, subretinal haemorrhages, and choroidal neovascularization (CNV) can occur, often with irreversible loss of visual acuity (VA). Although these complications can occur spontaneously, ocular and periocular trauma increases the risk. We describe two young patients who were diagnosed with angioid streaks and subretinal hemorrhages after diving headfirst.
From the *Department of Ophthalmology, University Hospitals, Leuven, Belgium; and †Department of Ophthalmology, University Hospitals Brugmann, Saint-Pierre, Brussels, Belgium.
A
ngioid streaks are subretinal lesions looking like blood vessels that extend from the peripapillary area into the peripheral fundus.1 Near the optic disk, they are often interconnected by circumferential breaks in Bruch membrane. They are caused by cracklike dehiscences in the collagenous and elastic portion of Bruch membrane. When first observed in the elderly, they are considered to be idiopathic or age related although Paget disease has to be ruled out. When angioid streaks are observed in a young adult, the suspected diagnosis is pseudoxanthoma elasticum (PXE) and the presence of peau d’orange in the ocular fundus and skin lesions are additional clinical arguments for PXE. Confirmation of PXE requires biopsy-proven skin changes or genetic testing with detection of the PXE mutation. Pseudoxanthoma elasticum–associated
Case Reports Case 1
The authors have no proprietary or commercial interest in any materials discussed in this article. Reprint requests: Anita M. Leys, MD, PhD, Department of Ophthalmology, University Hospitals, Capucijnenvoer 33, Leuven 3000, Belgium; e-mail: [email protected]
In May 1998, an otherwise healthy 19-year-old man experienced bilateral vision loss after diving headfirst into a swimming pool. He underwent ophthalmologic examination very shortly thereafter. VA was 20/20 in the right eye and 20/32 in
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the left eye, despite both central and peripheral scotomas. The anterior segments were normal bilaterally. In both eyes, funduscopy and fluorescein angiography revealed multiple large subretinal hemorrhages (and ruptures of Bruch membrane) with the macula of the left eye displaying more extensive lesions than that of the right eye (Figures 1 and 2). The examination also revealed bilateral angioid streaks and peau d’orange as well as apparently de novo choroidal rupture sites. The hemorrhages occurred both along the angioid streaks as along the fresh ruptures of the Bruch membrane. Based on the presence of angioid streaks and peau d’orange, PXE was suspected. The diagnosis of PXE was confirmed with cutaneous biopsies from the neck and axilla. The cardiovascular examination also revealed PXE-associated findings, including a sinus arrhythmia and moderate lower limb arteritis. Two months later, VA had improved bilaterally and most of the retinal hemorrhages had resolved but a macular lesion with signs of CNV had developed in the left eye (Figure 3). Treatment of the CNV was not performed because of the lack of anti–vascular endothelial growth factor agents at that time. Four months after initial presentation, VA in the left eye had again deteriorated to 20/40. Fundoscopy revealed a full-blown subfoveal CNV and recent retinal hemorrhage. The CNV remained untreated and evolved to a scar with VA 20/40 at last examination in 2005, 7 years after initial presentation (Figure 4).
Fig. 1. Patient 1 examined in May 1998 after the event with visual loss. Note subretinal hemorrhages in RE (A) and left eye (B), angioid streaks and peau d’orange. RE, right eye.
Fig. 2. Left eye. Fluorescein angiography performed on the same day reveals vertical papillomacular ruptures of Bruch membrane. In areas with dense hemorrhages, there is a blocked deep fluorescence.
Case 2 An otherwise healthy, 21-year-old, male soldier experienced visual loss after diving from a diving board in July 1989. He consulted an ophthalmologist the same day. Ocular examination showed numerous subretinal hemorrhages in the left eye and several in the right eye as well as bilateral angioid streaks. Many of the hemorrhages appeared to occur along the areas of angioid streaks. The clinical presentation suggested the diagnosis of diving headfirst-induced subretinal hemorrhages in a patient with angioid streaks (Figure 5A). Fluorescein angiography at that stage did not reveal fresh choroidal rupture sites. However, in June1991, 2 years after initial presentation, the right eye suffered a blunt trauma. Ocular examination in September 1991 revealed bilateral angioid streaks and choroidal ruptures. The locations of the choroidal ruptures corresponded with the locations of the dense hemorrhages noted in 1989 (Figure 5B) and were in the right eye probably also because of the blunt trauma in June 1991. Eighteen years later, in April 2009, the patient presented with blurred vision and a scotoma in the left eye. Visual acuity was 20/25 in the right eye and 20/40 in the left eye. Biomicroscopic examination, fluorescein angiography, and optical coherence tomography revealed active CNV nasal to the fovea with vascular leakage and edema. An intravitreal injection of bevacizumab was administered in the left eye,
Fig. 3. Two months later, most of the blood is resolved and ruptures of Bruch membrane and a left side maculopathy with CNV are clearly demonstrated on FA. FA, fluorescein angiography.
ANGIOID STREAKS AND DIVING
225
Fig. 4. Seven years after the event, the ruptures in Bruch membrane have caused in the left eye prominent long and large vertical window defects with staining on FA, whereas the angioid streaks are causing smaller window defects with a spiderweb configuration around the optic disk. Vision is severely reduced because of macular scar formation. FA, fluorescein angiography.
and in May 2009, the edema had resorbed and vision improved. The patient was referred to our medical retina department for a second opinion and discussion of therapeutic options. Biomicroscopic examination, optical coherence tomography, and fluorescein angiography showed bilateral angioid streaks and peau d’orange, and bilateral choroidal ruptures, and in the left eye, the CNV was not active but required close follow-up (Figure 6). Subsequently, activity recurred and a total of eight injections of bevacizumab was administered. In January 2010, the patient consulted with loss of vision in the right eye and clinical examination, fluorescein angiography, and optical coherence tomography showed an active occult subfoveal CNV in the right eye and an inactive CNV in the left eye. Bevacizumab treatment was started on the right side. Detection at age 21 of angioid streaks in a healthy man makes the diagnosis of PXE probable, but no skin lesions were observed, and 3 cutaneous biopsies from neck and axilla did not reveal changes because of PXE. Genetic studies are ongoing, and no results are available as yet.
Discussion We report for the first time diving-related visual loss in the setting of angioid streaks. In both patients, extensive bilateral subretinal hemorrhages were observed as well as angioid streaks and traumatic choroidal ruptures. One of our patients had a skin biopsy that confirmed the diagnosis of PXE. In the second patient, PXE could not be confirmed with biopsy and genetic testing of PXE is ongoing. The ocular phenotype of PXE is variable, and the findings also depend on the age of the patient and the presence of ocular trauma. Retinal pigment epithelial irregularities called peau d’ orange typically precede angioid streaks. Angioid streaks occur in at least 85% of patients and are usually first identified between the age of 15 and 25. Over time, other features may become prominent including reticular pigmentary dystrophy of the macula, atrophic lesions, crystalline bodies with
Fig. 5. A, Patient 2 examined in July 1989 shortly after the divingassociated event. Angioid streaks are observed in a spiderweb configuration centered on the optic nerve, and dense hemorrhages are present in several sectors masking the deep fluorescence. Two years later, the angioid streaks remain nearly unchanged, but prominent vertical trauma-induced ruptures of Bruch membrane are observed. The locations of these ruptures of Bruch membrane corresponded with locations of dense hemorrhages noted in 1989 (B).
comets, and optic nerve head drusen. Pseudoxanthoma elasticum eyes are at high risk for visual impairment, which is most often because of ruptures of Bruch membrane and CNV and occasionally because of
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Fig. 6. Eighteen years later, in April 2009, the patient presented with blurred vision and a scotoma in the LE because of CNV. Fluorescein angiography showed in the right (A) and LE (B) prominent long vertical trauma-induced ruptures of Bruch membrane and, in the LE, a CNV nasal to the fovea. The angioid streaks are symmetric in RE (C) and LE (D) and have a spiderweb configuration that is most prominent around the optic disk on the red-free photographs. LE, left eye; RE, right eye.
macular atrophy or complications of optic nerve head drusen. Reports point to the poor visual prognosis of PXE: By the age of 50, the majority of patients with PXE has bilateral poor vision, often 20/200 or worse. This is most often because of CNV. The risk of CNV increases with age; most patients are in the fifth or sixth decade of life when they present with loss of vision because of CNV. Choroidal neovascularization occurs in 72% to 86% of patients and becomes bilateral in approximately half of patients within 18 months of detection. Untreated the prognosis of PXE-associated CNV is poor, but intravitreal anti–vascular endothelial growth factor has improved the prognosis. In 1889, Doyne2 published the first case report and colored drawing of angioid streaks and retinal hemorrhages. Because the patient presented for examination after blunt ocular trauma, it was believed that these lesions were of purely traumatic origin. Later reports described angioid streaks as a characteristic finding of PXE and proposed that these eyes are fragile and predisposed for macular complications in adulthood. Vision is usually well preserved in children and young adults with PXE, unless a blunt trauma induces
complications.1,3–10 In several cases, the underlying trauma was a minor blow to the eye7,10 or a punch about the eyes or to the head without direct ocular trauma.5.8,10 After ocular trauma, macular hemorrhages, breaks of Bruch membrane, CNV, and scars can be observed at an earlier age. Two reported patients were 16 years old,5,8 and the oldest of a series of 12 reported cases3,5–7,9,10 was 29.9 The two patients discussed in this report experienced diving headfirst-related visual loss at age 19 and 21, respectively, and fundus examination revealed bilateral angioid streaks and extensive subretinal hemorrhages, some overlying the angioid streaks and some independent of the streaks. Choroidal ruptures, apparently of traumatic origin, were observed as well. Possible mechanisms for the subretinal hemorrhages are the direct blunt injury to the globe from the diving headfirst and the sudden change in pressure and barotrauma. Visual loss because of maculopathy was the first sign of angioid streaks and PXE in our patients and also in 6 of 12 previously reported cases.2,4–6,9,10 Traumatic ruptures in Bruch membrane can be overlooked in a fundus with PXE-associated angioid streaks. However, the pattern of lesions is different.
ANGIOID STREAKS AND DIVING
Angioid streaks typically occur in a spiderweb configuration centered around the optic nerve. The traumatic break formation is curved or linear, frequently vertical, and located perineurally or temporally.1,11 The present case reports illustrate that in patients with angioid streaks diving headfirst can cause subretinal hemorrhages and ruptures of Bruch membrane and increase the risk of maculopathy. Patients with angioid streaks should be cautioned not to dive headfirst. Key words: diving headfirst, angioid streaks, pseudoxanthoma elasticum, subretinal hemorrhages, traumatic rupture Bruch membrane, choroidal neovascularization. References 1. Gass JDM. Stereoscopic Atlas of Macular Diseases. 4th ed. St.Louis, Missouri: Mosby; 1997:118–121. 2. Doyne RW. Choroidal and retinal changes. The results of blows on the eyes. Trans Ophthalmol Soc UK 1889;9:128.
227 3. Streiff FH, Portmann UP. Une fratrie de stries angioides de la re´tine. Conside´rations sur l’he´re´dite´ et l’e´volution. Ophthalmologica 1951;121:87–89. 4. Levasseur JC. Angioid streaks and trauma. [Stries angioı¨des et traumatisme]. Bull Soc Fr Ophtalmol 1962;75:530–543. 5. Britten MJA. Unusual traumatic retinal haemorrhages associated with angioid streaks. Br J Ophthalmol 1966;50:540–542. 6. Bouchat J, Pouliquen Y, Joseph C, Aubry JP. Angioid streaks and ocular contusion (fluoro-retinographic aspects). [Stries angioı¨des et contusion oculaire (Aspect fluoro-retinographique)]. Bull Soc Ophtalmol Fr 1968;68:450–455; discussion Woillez 455. 7. Hagedoorn A. Angioid streaks and traumatic ruptures of Bruch’s membrane. Br J Ophthalmol 1975;59:267. 8. Levin DB, Bell DK. Traumatic retinal hemorrhages with angioid streaks. Arch Ophthalmol 1977;95:1072–1073. 9. Turut P, Malthieu D, Courtin J. Membrane neovasculaire choroidienne et rupture choroidienne traumatique sur stries angioides. Bull Soc Ophtalmol Fr 1982;4:591–594. 10. Pandolfo A, Verrastro G, Piccolino FC. Retinal hemorrhages following indirect ocular trauma in a patient with angioid streaks. Retina 2002;22:830–831. 11. Pruett RC, Weiter JJ, Goldstein RB. Myopic cracks, angioid streaks, and traumatic tears in Bruch’s membrane. Am J Ophthalmol 1987;103:537–543.
Purpose: The purpose of this study was to report diving-related visual loss in the setting of angioid streaks. Methods: Observational case reports of two patients with angioid streaks suffering sudden visual loss immediately after diving. Results: Two young adult male patients presented with visual loss after diving headfirst. Funduscopy revealed angioid streaks, peau d’orange, subretinal hemorrhages, and ruptures of Bruch membrane. Choroidal neovascularization developed during follow-up. Both patients had an otherwise uneventful personal and familial medical history. Conclusion: In patients with angioid streaks, diving headfirst can lead to subretinal hemorrhages and traumatic ruptures in Bruch membrane and increase the risk of maculopathy. Ophthalmologists should caution patients with angioid streaks against diving headfirst. RETINAL CASES & BRIEF REPORTS 6:223–227, 2012
skin lesions can be observed in adulthood and are rarely observed in childhood. They are most prominent in neck and on the large flexor surfaces, and biopsy of these lesions usually leads to the diagnosis of PXE. Gastrointestinal bleedings and peripheral and coronary arterial occlusive disease are well-known systemic complications. The underlying defect is a mutation in the ABCC6 gene on chromosome 16p13.1, which results in abnormal transmembranous transport, accumulation of extracellular material, and calcification of elastic fibers. Because of the fragility of Bruch membrane in angioid streaks, ruptures of Bruch membrane, subretinal haemorrhages, and choroidal neovascularization (CNV) can occur, often with irreversible loss of visual acuity (VA). Although these complications can occur spontaneously, ocular and periocular trauma increases the risk. We describe two young patients who were diagnosed with angioid streaks and subretinal hemorrhages after diving headfirst.
From the *Department of Ophthalmology, University Hospitals, Leuven, Belgium; and †Department of Ophthalmology, University Hospitals Brugmann, Saint-Pierre, Brussels, Belgium.
A
ngioid streaks are subretinal lesions looking like blood vessels that extend from the peripapillary area into the peripheral fundus.1 Near the optic disk, they are often interconnected by circumferential breaks in Bruch membrane. They are caused by cracklike dehiscences in the collagenous and elastic portion of Bruch membrane. When first observed in the elderly, they are considered to be idiopathic or age related although Paget disease has to be ruled out. When angioid streaks are observed in a young adult, the suspected diagnosis is pseudoxanthoma elasticum (PXE) and the presence of peau d’orange in the ocular fundus and skin lesions are additional clinical arguments for PXE. Confirmation of PXE requires biopsy-proven skin changes or genetic testing with detection of the PXE mutation. Pseudoxanthoma elasticum–associated
Case Reports Case 1
The authors have no proprietary or commercial interest in any materials discussed in this article. Reprint requests: Anita M. Leys, MD, PhD, Department of Ophthalmology, University Hospitals, Capucijnenvoer 33, Leuven 3000, Belgium; e-mail: [email protected]
In May 1998, an otherwise healthy 19-year-old man experienced bilateral vision loss after diving headfirst into a swimming pool. He underwent ophthalmologic examination very shortly thereafter. VA was 20/20 in the right eye and 20/32 in
223
224
RETINAL CASES & BRIEF REPORTS´ 2012 VOLUME 6 NUMBER 3
the left eye, despite both central and peripheral scotomas. The anterior segments were normal bilaterally. In both eyes, funduscopy and fluorescein angiography revealed multiple large subretinal hemorrhages (and ruptures of Bruch membrane) with the macula of the left eye displaying more extensive lesions than that of the right eye (Figures 1 and 2). The examination also revealed bilateral angioid streaks and peau d’orange as well as apparently de novo choroidal rupture sites. The hemorrhages occurred both along the angioid streaks as along the fresh ruptures of the Bruch membrane. Based on the presence of angioid streaks and peau d’orange, PXE was suspected. The diagnosis of PXE was confirmed with cutaneous biopsies from the neck and axilla. The cardiovascular examination also revealed PXE-associated findings, including a sinus arrhythmia and moderate lower limb arteritis. Two months later, VA had improved bilaterally and most of the retinal hemorrhages had resolved but a macular lesion with signs of CNV had developed in the left eye (Figure 3). Treatment of the CNV was not performed because of the lack of anti–vascular endothelial growth factor agents at that time. Four months after initial presentation, VA in the left eye had again deteriorated to 20/40. Fundoscopy revealed a full-blown subfoveal CNV and recent retinal hemorrhage. The CNV remained untreated and evolved to a scar with VA 20/40 at last examination in 2005, 7 years after initial presentation (Figure 4).
Fig. 1. Patient 1 examined in May 1998 after the event with visual loss. Note subretinal hemorrhages in RE (A) and left eye (B), angioid streaks and peau d’orange. RE, right eye.
Fig. 2. Left eye. Fluorescein angiography performed on the same day reveals vertical papillomacular ruptures of Bruch membrane. In areas with dense hemorrhages, there is a blocked deep fluorescence.
Case 2 An otherwise healthy, 21-year-old, male soldier experienced visual loss after diving from a diving board in July 1989. He consulted an ophthalmologist the same day. Ocular examination showed numerous subretinal hemorrhages in the left eye and several in the right eye as well as bilateral angioid streaks. Many of the hemorrhages appeared to occur along the areas of angioid streaks. The clinical presentation suggested the diagnosis of diving headfirst-induced subretinal hemorrhages in a patient with angioid streaks (Figure 5A). Fluorescein angiography at that stage did not reveal fresh choroidal rupture sites. However, in June1991, 2 years after initial presentation, the right eye suffered a blunt trauma. Ocular examination in September 1991 revealed bilateral angioid streaks and choroidal ruptures. The locations of the choroidal ruptures corresponded with the locations of the dense hemorrhages noted in 1989 (Figure 5B) and were in the right eye probably also because of the blunt trauma in June 1991. Eighteen years later, in April 2009, the patient presented with blurred vision and a scotoma in the left eye. Visual acuity was 20/25 in the right eye and 20/40 in the left eye. Biomicroscopic examination, fluorescein angiography, and optical coherence tomography revealed active CNV nasal to the fovea with vascular leakage and edema. An intravitreal injection of bevacizumab was administered in the left eye,
Fig. 3. Two months later, most of the blood is resolved and ruptures of Bruch membrane and a left side maculopathy with CNV are clearly demonstrated on FA. FA, fluorescein angiography.
ANGIOID STREAKS AND DIVING
225
Fig. 4. Seven years after the event, the ruptures in Bruch membrane have caused in the left eye prominent long and large vertical window defects with staining on FA, whereas the angioid streaks are causing smaller window defects with a spiderweb configuration around the optic disk. Vision is severely reduced because of macular scar formation. FA, fluorescein angiography.
and in May 2009, the edema had resorbed and vision improved. The patient was referred to our medical retina department for a second opinion and discussion of therapeutic options. Biomicroscopic examination, optical coherence tomography, and fluorescein angiography showed bilateral angioid streaks and peau d’orange, and bilateral choroidal ruptures, and in the left eye, the CNV was not active but required close follow-up (Figure 6). Subsequently, activity recurred and a total of eight injections of bevacizumab was administered. In January 2010, the patient consulted with loss of vision in the right eye and clinical examination, fluorescein angiography, and optical coherence tomography showed an active occult subfoveal CNV in the right eye and an inactive CNV in the left eye. Bevacizumab treatment was started on the right side. Detection at age 21 of angioid streaks in a healthy man makes the diagnosis of PXE probable, but no skin lesions were observed, and 3 cutaneous biopsies from neck and axilla did not reveal changes because of PXE. Genetic studies are ongoing, and no results are available as yet.
Discussion We report for the first time diving-related visual loss in the setting of angioid streaks. In both patients, extensive bilateral subretinal hemorrhages were observed as well as angioid streaks and traumatic choroidal ruptures. One of our patients had a skin biopsy that confirmed the diagnosis of PXE. In the second patient, PXE could not be confirmed with biopsy and genetic testing of PXE is ongoing. The ocular phenotype of PXE is variable, and the findings also depend on the age of the patient and the presence of ocular trauma. Retinal pigment epithelial irregularities called peau d’ orange typically precede angioid streaks. Angioid streaks occur in at least 85% of patients and are usually first identified between the age of 15 and 25. Over time, other features may become prominent including reticular pigmentary dystrophy of the macula, atrophic lesions, crystalline bodies with
Fig. 5. A, Patient 2 examined in July 1989 shortly after the divingassociated event. Angioid streaks are observed in a spiderweb configuration centered on the optic nerve, and dense hemorrhages are present in several sectors masking the deep fluorescence. Two years later, the angioid streaks remain nearly unchanged, but prominent vertical trauma-induced ruptures of Bruch membrane are observed. The locations of these ruptures of Bruch membrane corresponded with locations of dense hemorrhages noted in 1989 (B).
comets, and optic nerve head drusen. Pseudoxanthoma elasticum eyes are at high risk for visual impairment, which is most often because of ruptures of Bruch membrane and CNV and occasionally because of
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Fig. 6. Eighteen years later, in April 2009, the patient presented with blurred vision and a scotoma in the LE because of CNV. Fluorescein angiography showed in the right (A) and LE (B) prominent long vertical trauma-induced ruptures of Bruch membrane and, in the LE, a CNV nasal to the fovea. The angioid streaks are symmetric in RE (C) and LE (D) and have a spiderweb configuration that is most prominent around the optic disk on the red-free photographs. LE, left eye; RE, right eye.
macular atrophy or complications of optic nerve head drusen. Reports point to the poor visual prognosis of PXE: By the age of 50, the majority of patients with PXE has bilateral poor vision, often 20/200 or worse. This is most often because of CNV. The risk of CNV increases with age; most patients are in the fifth or sixth decade of life when they present with loss of vision because of CNV. Choroidal neovascularization occurs in 72% to 86% of patients and becomes bilateral in approximately half of patients within 18 months of detection. Untreated the prognosis of PXE-associated CNV is poor, but intravitreal anti–vascular endothelial growth factor has improved the prognosis. In 1889, Doyne2 published the first case report and colored drawing of angioid streaks and retinal hemorrhages. Because the patient presented for examination after blunt ocular trauma, it was believed that these lesions were of purely traumatic origin. Later reports described angioid streaks as a characteristic finding of PXE and proposed that these eyes are fragile and predisposed for macular complications in adulthood. Vision is usually well preserved in children and young adults with PXE, unless a blunt trauma induces
complications.1,3–10 In several cases, the underlying trauma was a minor blow to the eye7,10 or a punch about the eyes or to the head without direct ocular trauma.5.8,10 After ocular trauma, macular hemorrhages, breaks of Bruch membrane, CNV, and scars can be observed at an earlier age. Two reported patients were 16 years old,5,8 and the oldest of a series of 12 reported cases3,5–7,9,10 was 29.9 The two patients discussed in this report experienced diving headfirst-related visual loss at age 19 and 21, respectively, and fundus examination revealed bilateral angioid streaks and extensive subretinal hemorrhages, some overlying the angioid streaks and some independent of the streaks. Choroidal ruptures, apparently of traumatic origin, were observed as well. Possible mechanisms for the subretinal hemorrhages are the direct blunt injury to the globe from the diving headfirst and the sudden change in pressure and barotrauma. Visual loss because of maculopathy was the first sign of angioid streaks and PXE in our patients and also in 6 of 12 previously reported cases.2,4–6,9,10 Traumatic ruptures in Bruch membrane can be overlooked in a fundus with PXE-associated angioid streaks. However, the pattern of lesions is different.
ANGIOID STREAKS AND DIVING
Angioid streaks typically occur in a spiderweb configuration centered around the optic nerve. The traumatic break formation is curved or linear, frequently vertical, and located perineurally or temporally.1,11 The present case reports illustrate that in patients with angioid streaks diving headfirst can cause subretinal hemorrhages and ruptures of Bruch membrane and increase the risk of maculopathy. Patients with angioid streaks should be cautioned not to dive headfirst. Key words: diving headfirst, angioid streaks, pseudoxanthoma elasticum, subretinal hemorrhages, traumatic rupture Bruch membrane, choroidal neovascularization. References 1. Gass JDM. Stereoscopic Atlas of Macular Diseases. 4th ed. St.Louis, Missouri: Mosby; 1997:118–121. 2. Doyne RW. Choroidal and retinal changes. The results of blows on the eyes. Trans Ophthalmol Soc UK 1889;9:128.
227 3. Streiff FH, Portmann UP. Une fratrie de stries angioides de la re´tine. Conside´rations sur l’he´re´dite´ et l’e´volution. Ophthalmologica 1951;121:87–89. 4. Levasseur JC. Angioid streaks and trauma. [Stries angioı¨des et traumatisme]. Bull Soc Fr Ophtalmol 1962;75:530–543. 5. Britten MJA. Unusual traumatic retinal haemorrhages associated with angioid streaks. Br J Ophthalmol 1966;50:540–542. 6. Bouchat J, Pouliquen Y, Joseph C, Aubry JP. Angioid streaks and ocular contusion (fluoro-retinographic aspects). [Stries angioı¨des et contusion oculaire (Aspect fluoro-retinographique)]. Bull Soc Ophtalmol Fr 1968;68:450–455; discussion Woillez 455. 7. Hagedoorn A. Angioid streaks and traumatic ruptures of Bruch’s membrane. Br J Ophthalmol 1975;59:267. 8. Levin DB, Bell DK. Traumatic retinal hemorrhages with angioid streaks. Arch Ophthalmol 1977;95:1072–1073. 9. Turut P, Malthieu D, Courtin J. Membrane neovasculaire choroidienne et rupture choroidienne traumatique sur stries angioides. Bull Soc Ophtalmol Fr 1982;4:591–594. 10. Pandolfo A, Verrastro G, Piccolino FC. Retinal hemorrhages following indirect ocular trauma in a patient with angioid streaks. Retina 2002;22:830–831. 11. Pruett RC, Weiter JJ, Goldstein RB. Myopic cracks, angioid streaks, and traumatic tears in Bruch’s membrane. Am J Ophthalmol 1987;103:537–543.
![[Angioid streaks].](/assets/img/hold.png)
