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Post-traumatic bilateral blindness in a toddler
Journal of Pediatric Surgery Case Reports 51 (2019) 101292
Contents lists available at ScienceDirect
Journal of Pediatric Surgery Case Reports journal homepage: http://www.elsevier.com/locate/epsc
Post-traumatic bilateral blindness in a toddler E.W.M. Engelmann, MD a, *, C. Tinley, MD b, A.B. van As, Prof MD a a b
Trauma Unit1, Division of Paediatric Surgery, Red Cross War Memorial Children’s Hospital, Klipfontein Rd, Rondeboschm, Cape Town, South Africa Department of Ophthalmology, Division of Paediatric Surgery, Red Cross War Memorial Children’s Hospital, Klipfontein Rd, Rondebosch, Cape Town, South Africa
A R T I C L E I N F O
A B S T R A C T
Keywords: paediatric trauma Eye trauma Blindness Retinopathy
A unique case of vision loss in a two-year-old boy after sustaining a head injury as a pedestrian versus car was seen at our paediatric trauma unit. A CT brain was negative for fracture, intra-cranial haemorrhage and contusion. There were no neurological symptoms and no signs of abuse. Ophthalmological examination demonstrated retinal haemorrhages, cotton wool spots and Purtscher flecken that are pathognomonic for the diagnosis of Purtscher’s retinopathy. This occlusive microvasculopathy, associated with cranial and chest trauma, may cause severe blindness. In literature, bilaterality was described in almost two thirds of cases. Regardless of management with or without medication, visual function improved in the majority of cases. In our case, despite treatment with high-dose intravenous methylprednisolone, the child’s vision remained poor with bilateral optic atrophy and retinal pigmentary changes. Children with Purtscher’s retinopathy should be examined for signs of non-accidental injury.
1. Introduction An unusual case of vision loss in a two-year-old boy sustaining a head injury was seen at our paediatric trauma unit. More than a hundred years ago, Austrian ophthalmologist Othmar Purtscher reported a similar case of blindness in a middle-aged man with cranial trauma after falling off a tree [1]. Since then, Purtscher’s retinopathy or angiopathia retinae traumatica is described as an extremely rare post-traumatic condition in adults [2,3]. It is an occlusive microvasculopathy associ ated with cranial and chest trauma that cause blindness. Bilaterality is described in almost two thirds of cases [2]. Similar retinopathy with a non-traumatic etiology, such as severe pancreatitis and thrombotic thrombocytopenic purpura, is referred to as Purtscher-like retinopathy [2,3]. The estimated incidence of both combined is less than 0.24 per sons per million per year [3,4]. 2. Case presentation A twenty-eight-month-old boy was involved in a pedestrian versus vehicle accident (PVA). Details were unclear but the car may have driven over the head of the child. The accident happened in an informal settlement of the Cape Flats and the boy was admitted to the trauma unit of the local hospital with minor abrasions of the right cheek and right
elbow. Full-body radiography (LODOX-Statscan) and a CT brain did not detect any trauma-related pathology and the child was discharged home. Three days later, the child was taken to our paediatric hospital by his mother because of abnormal visual behaviour over the previous two days. The boy had started walking into things, was grabbing air when trying to find his bottle and his mother believed he could not see. However, there were no signs of vision loss directly after the PVA. There was no history of seizures or vomiting and no past ocular disease or other health issues. On arrival the patient was stable. The airway was patent and selfmaintained, there was no C-spine tenderness, no problems breathing, the chest was clear and circulatory status was normal. His Children’s Coma Score was 11/11. The child was alert yet irritable, kept his eyes closed and was uncooperative. There were no signs of external eye trauma, no periorbital or eyelid swelling and no conjunctival findings. The pupils were both dilated with minimal response to light. Eye movements were normal. When placed on the ground without support, the boy waddled around reaching for his mother. He only redirected in the right direction after she called him. Movement of the extremities was normal and the child talked to his mother in the usual way. Repeat CT imaging of the brain was negative for fracture, intra-cranial haemor rhage and contusion. There were no neurological symptoms and no signs of abuse. Further examination consisted of a third CT scan, which
* Corresponding author. Kennedylaan 128-2, 1079, NJ, Amsterdam, the Netherlands. E-mail addresses: [email protected] (E.W.M. Engelmann), [email protected] (C. Tinley), [email protected] (A.B. van As). 1 The study was conducted here. https://doi.org/10.1016/j.epsc.2019.101292 Received 6 August 2019; Received in revised form 21 August 2019; Accepted 27 August 2019 Available online 19 September 2019 2213-5766/© 2019 Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
E.W.M. Engelmann et al.
Journal of Pediatric Surgery Case Reports 51 (2019) 101292
showed symmetrical optic nerves, no atrophy, normal lobes, lenses, no midline shift or hydrocephalus and no other features explaining the clinical status. Ophthalmological consultation revealed decreased visual acuity. He could not fix or follow with either eye. In both eyes, diffuse retinal haemorrhages, Purtscher flecken, with a pale retina and cottonwool spots were seen. The patient was admitted to the trauma ward with the diagnosis of Purtscher’s retinopathy secondary to severe head injury. On the second day of admission, there was an impression that the child started to regain vision, yet visual acuity remained difficult to assess. Treatment with megadose (15 mg/kg in three gifts a day) of intravenous methylprednisolone was started. The boy showed improvement in behaviour and was discharged after four days. Unfor tunately, vision at three months post-PVA was very poor in both eyes. He could fix a target with the right eye only, however this not steady or maintained. and had bilateral optic atrophy. Follow-up 18 months after trauma depicted persistent optical atrophy and retinal pigmentary changes. The best vision with both eyes open and optimal spectacle correction was 1.0 LogMAR.
the visual outcome remained poor, in contrast to the findings of Tomasi [6]. 4. Conclusion Purtscher’s retinopathy is a rare condition, with sudden onset vision loss hours to days after a traumatic event distant from the eyes. Children should be examined for signs of non-accidental injury. Although spon taneous recovery may occur, it can be vision-threatening and urgent ophthalmologic examination is recommended. Treatment with intrave nous high-dose methylprednisolone may be considered, however, it is not yet clear whether the natural course of the condition can be altered. Patient consent Consent to publish the case report was not obtained. This report does not contain any personal information that could lead to the identifica tion of the patient. Funding
3. Discussion
No funding or grant support.
This case is the first report of Purtscher’s retinopathy after PVA in a child. We observed a very poor visual outcome, even after high-dose intravenous methylprednisolone. The available evidence consists of case reports and a handful of small case series of which the majority concentrated on non-traumatic, Purtscher-like retinopathies [2,5]. In infants, Purtscher’s retinopathy has only been described in two cases of battered child syndrome, following chest injury and associated with seizures and intracranial haemorrhage [6]. The exact pathophysiology remains unclear, but it is hypothesized that the microangiopathy is due to precapillary retinal arteriole occlusion caused by fat emboli or leuko-aggregation, resulting in microvascular infarction of the retinal nerve fibre layer, which in turns causes cotton wool spots [4]. The latter is described as white ischemic lesions in the nerve fibre layer [7]. Although Purtscher’s reti nopathy is a clinical diagnosis, it may be supported by fundoscopic signs such as cotton-wool spots and intraretinal haemorrhage (83–92%) and pathognomonic Purtscher flecken (polygon-shaped white lesions in the inner nuclear layer, 50%) [2,3,7]. These typical findings were present in our patient. Additional imaging by optical coherence tomography angiography and fluorescein angiography was suggested by Xiao et al., however, these techniques were not available in our clinic [8]. Two management strategies were identified in literature: observa tion without medication and treatment with intravenous corticosteroids [2,5]. Visual recovery after the administration of corticosteroids is suggested to be a result of stabilisation of damaged neuronal membranes and neurovascular channels as well as inhibition of granulocyte aggre gation related to the complement activation [9,10]. No statistically significant difference in visual acuity between patients with and without glucocorticoid therapy was found in the literature [2,5]. Regardless of the management, visual function improved in the majority of cases [2]. Given the child’s age, the low quality of available evidence and the risk of guarded visual prognosis, treatment with megadoses (15 mg/kg) of intravenous methylprednisolone was initiated in our case. Despite this,
Authorship All authors attest that they meet the current ICMJE criteria for Authorship. Conflict of interest The authors have no financial disclosures. Appendix A. Supplementary data Supplementary data to this article can be found online at https://doi. org/10.1016/j.epsc.2019.101292. References [1] Purtscher O. Noch unbekannte funde nach schadeltrauma. Ber Dtsch Ophthalmol Ges 1910;36:294–301. [2] Miguel AIM, Henriques F, Azevedo LFR, et al. Systematic review of Purtscher’s and Purtscher-like retinopathies. Eye (Lond) 2013;27(1):1–13. [3] Agrawal A, McKibbin M. Purtscher’s retinopathy: epidemiology, clinical features and outcome. Br J Ophthalmol 2007;91(11):1456–9. [4] Hollo G. Frequency of Purtscher’s retinopathy. Br J Ophthalmol 2008;92(8):1159. [5] Xia D, Chen X, Zhou Q, et al. Efficacy of Purtscher’s retinopathy treatments: a systematic review. Curr Eye Res 2017;42(6):908–17. [6] Tomasi LG, Rosman NP. Purtscher retinopathy in the battered child syndrome. Am J Dis Child 1975;129(11):1335–7. [7] Caplen SM, Madreperla SA. Purtscher’s retinopathy: a case report and review. Am J Emerg Med 2008;26(7):836. [8] Xiao W, He L, Mao Y, et al. Multimodal imaging in purtscher retinopathy. Retina 2018;38(7):59–60. [9] Agrawal A, McKibbin M. Purtscher’s retinopathies: a review. Surv Ophthalmol 2006;51:129–36. [10] Hammerschmidt DE, White JG, Craddock PR, et al. Corticosteroids inhibit complement-induced granulocyte aggregation. A possible mechanism for their efficacy in shock states. J Clin Investig 1979;63(4):798–803.
2
Contents lists available at ScienceDirect
Journal of Pediatric Surgery Case Reports journal homepage: http://www.elsevier.com/locate/epsc
Post-traumatic bilateral blindness in a toddler E.W.M. Engelmann, MD a, *, C. Tinley, MD b, A.B. van As, Prof MD a a b
Trauma Unit1, Division of Paediatric Surgery, Red Cross War Memorial Children’s Hospital, Klipfontein Rd, Rondeboschm, Cape Town, South Africa Department of Ophthalmology, Division of Paediatric Surgery, Red Cross War Memorial Children’s Hospital, Klipfontein Rd, Rondebosch, Cape Town, South Africa
A R T I C L E I N F O
A B S T R A C T
Keywords: paediatric trauma Eye trauma Blindness Retinopathy
A unique case of vision loss in a two-year-old boy after sustaining a head injury as a pedestrian versus car was seen at our paediatric trauma unit. A CT brain was negative for fracture, intra-cranial haemorrhage and contusion. There were no neurological symptoms and no signs of abuse. Ophthalmological examination demonstrated retinal haemorrhages, cotton wool spots and Purtscher flecken that are pathognomonic for the diagnosis of Purtscher’s retinopathy. This occlusive microvasculopathy, associated with cranial and chest trauma, may cause severe blindness. In literature, bilaterality was described in almost two thirds of cases. Regardless of management with or without medication, visual function improved in the majority of cases. In our case, despite treatment with high-dose intravenous methylprednisolone, the child’s vision remained poor with bilateral optic atrophy and retinal pigmentary changes. Children with Purtscher’s retinopathy should be examined for signs of non-accidental injury.
1. Introduction An unusual case of vision loss in a two-year-old boy sustaining a head injury was seen at our paediatric trauma unit. More than a hundred years ago, Austrian ophthalmologist Othmar Purtscher reported a similar case of blindness in a middle-aged man with cranial trauma after falling off a tree [1]. Since then, Purtscher’s retinopathy or angiopathia retinae traumatica is described as an extremely rare post-traumatic condition in adults [2,3]. It is an occlusive microvasculopathy associ ated with cranial and chest trauma that cause blindness. Bilaterality is described in almost two thirds of cases [2]. Similar retinopathy with a non-traumatic etiology, such as severe pancreatitis and thrombotic thrombocytopenic purpura, is referred to as Purtscher-like retinopathy [2,3]. The estimated incidence of both combined is less than 0.24 per sons per million per year [3,4]. 2. Case presentation A twenty-eight-month-old boy was involved in a pedestrian versus vehicle accident (PVA). Details were unclear but the car may have driven over the head of the child. The accident happened in an informal settlement of the Cape Flats and the boy was admitted to the trauma unit of the local hospital with minor abrasions of the right cheek and right
elbow. Full-body radiography (LODOX-Statscan) and a CT brain did not detect any trauma-related pathology and the child was discharged home. Three days later, the child was taken to our paediatric hospital by his mother because of abnormal visual behaviour over the previous two days. The boy had started walking into things, was grabbing air when trying to find his bottle and his mother believed he could not see. However, there were no signs of vision loss directly after the PVA. There was no history of seizures or vomiting and no past ocular disease or other health issues. On arrival the patient was stable. The airway was patent and selfmaintained, there was no C-spine tenderness, no problems breathing, the chest was clear and circulatory status was normal. His Children’s Coma Score was 11/11. The child was alert yet irritable, kept his eyes closed and was uncooperative. There were no signs of external eye trauma, no periorbital or eyelid swelling and no conjunctival findings. The pupils were both dilated with minimal response to light. Eye movements were normal. When placed on the ground without support, the boy waddled around reaching for his mother. He only redirected in the right direction after she called him. Movement of the extremities was normal and the child talked to his mother in the usual way. Repeat CT imaging of the brain was negative for fracture, intra-cranial haemor rhage and contusion. There were no neurological symptoms and no signs of abuse. Further examination consisted of a third CT scan, which
* Corresponding author. Kennedylaan 128-2, 1079, NJ, Amsterdam, the Netherlands. E-mail addresses: [email protected] (E.W.M. Engelmann), [email protected] (C. Tinley), [email protected] (A.B. van As). 1 The study was conducted here. https://doi.org/10.1016/j.epsc.2019.101292 Received 6 August 2019; Received in revised form 21 August 2019; Accepted 27 August 2019 Available online 19 September 2019 2213-5766/© 2019 Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
E.W.M. Engelmann et al.
Journal of Pediatric Surgery Case Reports 51 (2019) 101292
showed symmetrical optic nerves, no atrophy, normal lobes, lenses, no midline shift or hydrocephalus and no other features explaining the clinical status. Ophthalmological consultation revealed decreased visual acuity. He could not fix or follow with either eye. In both eyes, diffuse retinal haemorrhages, Purtscher flecken, with a pale retina and cottonwool spots were seen. The patient was admitted to the trauma ward with the diagnosis of Purtscher’s retinopathy secondary to severe head injury. On the second day of admission, there was an impression that the child started to regain vision, yet visual acuity remained difficult to assess. Treatment with megadose (15 mg/kg in three gifts a day) of intravenous methylprednisolone was started. The boy showed improvement in behaviour and was discharged after four days. Unfor tunately, vision at three months post-PVA was very poor in both eyes. He could fix a target with the right eye only, however this not steady or maintained. and had bilateral optic atrophy. Follow-up 18 months after trauma depicted persistent optical atrophy and retinal pigmentary changes. The best vision with both eyes open and optimal spectacle correction was 1.0 LogMAR.
the visual outcome remained poor, in contrast to the findings of Tomasi [6]. 4. Conclusion Purtscher’s retinopathy is a rare condition, with sudden onset vision loss hours to days after a traumatic event distant from the eyes. Children should be examined for signs of non-accidental injury. Although spon taneous recovery may occur, it can be vision-threatening and urgent ophthalmologic examination is recommended. Treatment with intrave nous high-dose methylprednisolone may be considered, however, it is not yet clear whether the natural course of the condition can be altered. Patient consent Consent to publish the case report was not obtained. This report does not contain any personal information that could lead to the identifica tion of the patient. Funding
3. Discussion
No funding or grant support.
This case is the first report of Purtscher’s retinopathy after PVA in a child. We observed a very poor visual outcome, even after high-dose intravenous methylprednisolone. The available evidence consists of case reports and a handful of small case series of which the majority concentrated on non-traumatic, Purtscher-like retinopathies [2,5]. In infants, Purtscher’s retinopathy has only been described in two cases of battered child syndrome, following chest injury and associated with seizures and intracranial haemorrhage [6]. The exact pathophysiology remains unclear, but it is hypothesized that the microangiopathy is due to precapillary retinal arteriole occlusion caused by fat emboli or leuko-aggregation, resulting in microvascular infarction of the retinal nerve fibre layer, which in turns causes cotton wool spots [4]. The latter is described as white ischemic lesions in the nerve fibre layer [7]. Although Purtscher’s reti nopathy is a clinical diagnosis, it may be supported by fundoscopic signs such as cotton-wool spots and intraretinal haemorrhage (83–92%) and pathognomonic Purtscher flecken (polygon-shaped white lesions in the inner nuclear layer, 50%) [2,3,7]. These typical findings were present in our patient. Additional imaging by optical coherence tomography angiography and fluorescein angiography was suggested by Xiao et al., however, these techniques were not available in our clinic [8]. Two management strategies were identified in literature: observa tion without medication and treatment with intravenous corticosteroids [2,5]. Visual recovery after the administration of corticosteroids is suggested to be a result of stabilisation of damaged neuronal membranes and neurovascular channels as well as inhibition of granulocyte aggre gation related to the complement activation [9,10]. No statistically significant difference in visual acuity between patients with and without glucocorticoid therapy was found in the literature [2,5]. Regardless of the management, visual function improved in the majority of cases [2]. Given the child’s age, the low quality of available evidence and the risk of guarded visual prognosis, treatment with megadoses (15 mg/kg) of intravenous methylprednisolone was initiated in our case. Despite this,
Authorship All authors attest that they meet the current ICMJE criteria for Authorship. Conflict of interest The authors have no financial disclosures. Appendix A. Supplementary data Supplementary data to this article can be found online at https://doi. org/10.1016/j.epsc.2019.101292. References [1] Purtscher O. Noch unbekannte funde nach schadeltrauma. Ber Dtsch Ophthalmol Ges 1910;36:294–301. [2] Miguel AIM, Henriques F, Azevedo LFR, et al. Systematic review of Purtscher’s and Purtscher-like retinopathies. Eye (Lond) 2013;27(1):1–13. [3] Agrawal A, McKibbin M. Purtscher’s retinopathy: epidemiology, clinical features and outcome. Br J Ophthalmol 2007;91(11):1456–9. [4] Hollo G. Frequency of Purtscher’s retinopathy. Br J Ophthalmol 2008;92(8):1159. [5] Xia D, Chen X, Zhou Q, et al. Efficacy of Purtscher’s retinopathy treatments: a systematic review. Curr Eye Res 2017;42(6):908–17. [6] Tomasi LG, Rosman NP. Purtscher retinopathy in the battered child syndrome. Am J Dis Child 1975;129(11):1335–7. [7] Caplen SM, Madreperla SA. Purtscher’s retinopathy: a case report and review. Am J Emerg Med 2008;26(7):836. [8] Xiao W, He L, Mao Y, et al. Multimodal imaging in purtscher retinopathy. Retina 2018;38(7):59–60. [9] Agrawal A, McKibbin M. Purtscher’s retinopathies: a review. Surv Ophthalmol 2006;51:129–36. [10] Hammerschmidt DE, White JG, Craddock PR, et al. Corticosteroids inhibit complement-induced granulocyte aggregation. A possible mechanism for their efficacy in shock states. J Clin Investig 1979;63(4):798–803.
2