CASE REPORT
Sudden Death From Ruptured Choroid Plexus Arteriovenous Malformation Andreea Cioca, MD,*Þ Dan Gheban, MD, PhD,*þ Dan Perju-Dumbrava, MD, PhD,*§ Ovidiu Chiroban, MD,*§ and Mihaela Mera, MD, PhD*Þ
Abstract: Brain vascular malformations are recognized as having potential to produce hemorrhage, but leading to sudden death in children is uncommon. Arteriovenous malformations may be situated in any region of the brain, but very rarely, they can be restricted to the choroid plexus. We report here a rare case of sudden death in a child, caused by a ruptured vascular malformation with an unusual location, which was not identified grossly but only on histological examination. The size and the location of the lesion, as well as the age of our patient, were contributing factors of the massive bleeding. Autopsy remains an important tool because it provides valuable information about the etiology of such bleedings, improves knowledge about these lesions, and enhances epidemiologic data. Key Words: arteriovenous malformation, intracerebral hemorrhage, choroid plexus, children, sudden death (Am J Forensic Med Pathol 2014;35: 100Y102)
A
rteriovenous malformations (AVMs) are abnormal collections of feeding arteries and draining veins, without an intervening capillary bed, that lead to lesions with shunting in a central nidus. Arteriovenous malformations may be situated in any region of the brain, and exceedingly rare, they can be restricted to the choroid plexus.1 The clinical presentation of AVMs and their prognosis in children seem to differ from those in adults.2 Although intracerebral hemorrhage is the most frequent clinical presentation of an AVM, sudden and unexpected death due a ruptured malformation is an unusual presentation in children.3 We report here a rare case of sudden death in a child, due to a ruptured AVM of the choroid plexus, which was not demonstrated by computed tomographic (CT) scan. To our knowledge, there are only 5 such cases reported in the literature.
CASE REPORT A 9-year-old boy, previously healthy, was admitted to the emergency department with no history of headache, migraine, or epilepsy. At admission, the patient was comatose, with Manuscript received November 22, 2013; accepted January 9, 2014. From the *‘‘Iuliu Hatieganu’’ University of Medicine and Pharmacy, Cluj-Napoca, Romania; †Department of Pathology, County Emergency Hospital, Cluj-Napoca, Romania; ‡Department of Pathology, Children’s Emergency Hospital, Cluj-Napoca, Romania; and §Department of Legal Medicine, Institute of Legal Medicine, Cluj-Napoca, Romania. The authors report no conflicts of interest. Reprints: Andreea Cioca, MD, ‘‘Iuliu Hatieganu’’ University of Medicine and Pharmacy, Street Victor Babes, no. 8, 400012, Cluj-Napoca, Romania. E-mail: [email protected]. Supplemental digital contents are available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal’s Web site (www.amjforensicmedicine.com). Copyright * 2014 by Lippincott Williams & Wilkins ISSN: 0195-7910/14/3502Y0100 DOI: 10.1097/PAF.0000000000000091
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Glasgow Coma Scale of 3 points. The pupils were dilated, without reaction to light, and the corneal and muscle reflexes were negative. Because of the respiratory insufficiency, the patient was intubated and respiratory assisted. Computed tomographic scan of the brain revealed diffuse subarachnoid hemorrhage and extensive intraventricular hemorrhage. No evidence of vascular malformation was shown. Afterward, the spontaneous respiration ceased and the apnea test result was positive, which supported the diagnosis of brain death. He died in 22 hours after admission to the hospital, and an autopsy was performed the next day. At the autopsy, there was no evidence of an intracerebral trauma. Gross examination of the brain showed cerebral edema with scattered subarachnoid hemorrhage, marked in the basilar region (Fig. 1A) and with abundant blood filling the entire ventricular system (Fig. 1B). On cut section, ischemic areas were found in the cerebellar parenchyma. There was no hemorrhage in the brain tissue. Multiple histology sections were sampled from the brain parenchyma bleeding areas and from the choroid plexus of the lateral ventricles, and then the brain was fixed in buffered formalin. Microscopic examination of the sections taken revealed only cerebral edema and hemorrhage without evidence of the etiology. Additional sections were taken from the brain previously fixed in buffered formalin and from the choroid plexus of the third and fourth ventricles. Microscopic examination revealed a vascular malformation that involved the choroid plexus of the fourth ventricle. Morphologically, there was a conglomeration of blood-filled arteries and arterialized veins with varying sizes (Fig. 2, A and B). Orcein stain was performed to demonstrate the presence of the arteries (Fig. 2, C and D). Iron stain did not reveal hemosiderin, which excludes former bleedings. The cause of death was massive intracerebral hemorrhage due to a ruptured AVM located in the choroid plexus of the fourth ventricle.
DISCUSSION Sudden nontraumatic death in apparently healthy children accounts for 10% of all deaths in children, cardiovascular pathology being the most common finding at necropsies.4 The incidence of sudden death in children varies from 0.8 to 6.2 cases per 100.000 per year, which is much lower compared with adults.5 Intracerebral hemorrhage as a cause of sudden death in pediatric population is usually the consequence of a trauma. After a traumatic etiology is excluded, other conditions associated with central nervous system causes of sudden death that should be considered are aneurysms, AVM, brain tumors, vasculitis, cerebral infections, or hematological conditions.3 Arteriovenous malformations may be situated in any region of the brain, but very rarely, they can be restricted to the choroid plexus.1 Reviewing the literature, we found that sudden death due to AVMs of the choroid plexus is exceedingly rare in Am J Forensic Med Pathol
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Volume 35, Number 2, June 2014
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Am J Forensic Med Pathol
&
Volume 35, Number 2, June 2014
Sudden Death From Ruptured Choroid Plexus AVM
FIGURE 1. Gross examination of the brain showed cerebral edema with scattered subarachnoid hemorrhage, marked in the basilar region (A), and with abundant blood filling the entire ventricular system (B). Figure 1 can be viewed online in color at www.amjforensicmedicine.com.
pediatric population because only 5 cases have been reported in children and adolescents younger than 15 years, in the period of 1990 to 2011.6Y8 Autopsy studies showed that only 12% of AVMs become symptomatic during life, whereas the rest remains clinically silent.2 Intracerebral hemorrhage is the most frequent clinical presentation of an AVM, but the tendency of bleeding is much higher in children (80%) than in adults (50%Y65%), with a worse prognosis compared with adults.2 There are several factors that are associated with a high risk for bleeding: small
AVMs, a single draining vein, high feeding arterial pressure, venous stenosis, and AVMs associated with an aneurysm. Some studies showed that AVMs in deep locations such as the basal ganglia and the periventricular or the intraventricular space increase the risk for bleeding.2 Histologically, AVMs are composed of tortuous dilated arteries and veins with varying size and wall thickness. When there is a significant difference of blood flow in an AVM compared with surrounding brain tissue, the arterial steal phenomenon may appear. In these conditions, the blood from the
FIGURE 2. Hematoxylin-eosin stain showing the choroid plexus with conglomeration of small vessels (A) and large vessels (B). Orcein stain demonstrating arterial components with internal elastic lamina at low power (C) and at high power (D). Figure 2 can be viewed online in color at www.amjforensicmedicine.com. * 2014 Lippincott Williams & Wilkins
www.amjforensicmedicine.com
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
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adjacent brain is diverted into the draining veins of AVM and leads to ischemic lesions,9 as in our case. The size of AVMs varies from giant forms to very small malformations that may be undetected by imagistic investigations (CT scan, magnetic resonance imaging, or angiography), namely, cryptic. In our case, the CT scan did not demonstrate the malformation, and because of the rapid evolution and the poor prognosis, a contrast CT was not performed. Moreover, an AVM accompanied by massive hemorrhage is not always grossly visible on autopsy examination. The pathologist should take numerous sections from the most hemorrhagic areas with adjacent brain parenchyma and from the choroid plexus. Even so, further samples may be necessary until the malformation is demonstrated.
CONCLUSIONS In this article, we reported a rare case of sudden death in a child, caused by a ruptured vascular malformation with an unusual location, which was not identified grossly but only on histological examination. The size and the location of the lesion, as well as the age of our patient, were contributing factors of the massive bleeding. Autopsy remains an important tool because it provides valuable information about the etiology of such bleedings, improves the knowledge about these lesions, and enhances the importance of the epidemiologic data.
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REFERENCES 1. Gu¨rcan F, Aribal ME, Baltacio?lu F, et al. Arteriovenous malformation of the choroid plexus. Australas Radiol. 1998;42:69Y71. 2. Forsting M, Wanke I. Intracranial vascular malformations and aneurysms. In: Christophe C, Laurent S, Laurent P, eds. Pial Arteriovenous Malformations. 2nd ed. Germany: Springer; 2006:51Y74. 3. Roger WB. Sudden Death in Infancy, Childhood, and Adolescence. Cambridge, UK: University Press; 2004:350Y358. 4. Christopher W. Sudden death in children and adolescents. Heart. 2002;88(4):426Y431. 5. Stuart B, John DK, John AT, et al. Sudden cardiac death in children and adolescents: introduction and overview. Pediatr Clin North Am. 2004;5:1201Y1209. 6. Jakob M, Ute L, Friedrich S. Intracranial arteriovenous malformations presenting as sudden unexpected deathVa report of 3 cases and review of the literature. Am J Forensic Med Pathol. 2007;28:173Y176. 7. Erich R, Christian G, Katharina C, et al. Sudden death from primary intraventricular hemorrhage in childhood. Eur J Paediatr Neurol. 1999;3:223Y226. 8. Tomcik MA, Gerig NR, Prahlow JA. Sudden death from ruptured intracranial vascular malformation. Forensic Sci Med Pathol. 2011;7:185Y191. 9. Roach ES, Lo WD, Heyer GL. Pediatric Stroke and Cerebrovascular Disorders. New York, NY: Demos Medical Publishing; 2011.
* 2014 Lippincott Williams & Wilkins
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Sudden Death From Ruptured Choroid Plexus Arteriovenous Malformation Andreea Cioca, MD,*Þ Dan Gheban, MD, PhD,*þ Dan Perju-Dumbrava, MD, PhD,*§ Ovidiu Chiroban, MD,*§ and Mihaela Mera, MD, PhD*Þ
Abstract: Brain vascular malformations are recognized as having potential to produce hemorrhage, but leading to sudden death in children is uncommon. Arteriovenous malformations may be situated in any region of the brain, but very rarely, they can be restricted to the choroid plexus. We report here a rare case of sudden death in a child, caused by a ruptured vascular malformation with an unusual location, which was not identified grossly but only on histological examination. The size and the location of the lesion, as well as the age of our patient, were contributing factors of the massive bleeding. Autopsy remains an important tool because it provides valuable information about the etiology of such bleedings, improves knowledge about these lesions, and enhances epidemiologic data. Key Words: arteriovenous malformation, intracerebral hemorrhage, choroid plexus, children, sudden death (Am J Forensic Med Pathol 2014;35: 100Y102)
A
rteriovenous malformations (AVMs) are abnormal collections of feeding arteries and draining veins, without an intervening capillary bed, that lead to lesions with shunting in a central nidus. Arteriovenous malformations may be situated in any region of the brain, and exceedingly rare, they can be restricted to the choroid plexus.1 The clinical presentation of AVMs and their prognosis in children seem to differ from those in adults.2 Although intracerebral hemorrhage is the most frequent clinical presentation of an AVM, sudden and unexpected death due a ruptured malformation is an unusual presentation in children.3 We report here a rare case of sudden death in a child, due to a ruptured AVM of the choroid plexus, which was not demonstrated by computed tomographic (CT) scan. To our knowledge, there are only 5 such cases reported in the literature.
CASE REPORT A 9-year-old boy, previously healthy, was admitted to the emergency department with no history of headache, migraine, or epilepsy. At admission, the patient was comatose, with Manuscript received November 22, 2013; accepted January 9, 2014. From the *‘‘Iuliu Hatieganu’’ University of Medicine and Pharmacy, Cluj-Napoca, Romania; †Department of Pathology, County Emergency Hospital, Cluj-Napoca, Romania; ‡Department of Pathology, Children’s Emergency Hospital, Cluj-Napoca, Romania; and §Department of Legal Medicine, Institute of Legal Medicine, Cluj-Napoca, Romania. The authors report no conflicts of interest. Reprints: Andreea Cioca, MD, ‘‘Iuliu Hatieganu’’ University of Medicine and Pharmacy, Street Victor Babes, no. 8, 400012, Cluj-Napoca, Romania. E-mail: [email protected]. Supplemental digital contents are available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal’s Web site (www.amjforensicmedicine.com). Copyright * 2014 by Lippincott Williams & Wilkins ISSN: 0195-7910/14/3502Y0100 DOI: 10.1097/PAF.0000000000000091
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Glasgow Coma Scale of 3 points. The pupils were dilated, without reaction to light, and the corneal and muscle reflexes were negative. Because of the respiratory insufficiency, the patient was intubated and respiratory assisted. Computed tomographic scan of the brain revealed diffuse subarachnoid hemorrhage and extensive intraventricular hemorrhage. No evidence of vascular malformation was shown. Afterward, the spontaneous respiration ceased and the apnea test result was positive, which supported the diagnosis of brain death. He died in 22 hours after admission to the hospital, and an autopsy was performed the next day. At the autopsy, there was no evidence of an intracerebral trauma. Gross examination of the brain showed cerebral edema with scattered subarachnoid hemorrhage, marked in the basilar region (Fig. 1A) and with abundant blood filling the entire ventricular system (Fig. 1B). On cut section, ischemic areas were found in the cerebellar parenchyma. There was no hemorrhage in the brain tissue. Multiple histology sections were sampled from the brain parenchyma bleeding areas and from the choroid plexus of the lateral ventricles, and then the brain was fixed in buffered formalin. Microscopic examination of the sections taken revealed only cerebral edema and hemorrhage without evidence of the etiology. Additional sections were taken from the brain previously fixed in buffered formalin and from the choroid plexus of the third and fourth ventricles. Microscopic examination revealed a vascular malformation that involved the choroid plexus of the fourth ventricle. Morphologically, there was a conglomeration of blood-filled arteries and arterialized veins with varying sizes (Fig. 2, A and B). Orcein stain was performed to demonstrate the presence of the arteries (Fig. 2, C and D). Iron stain did not reveal hemosiderin, which excludes former bleedings. The cause of death was massive intracerebral hemorrhage due to a ruptured AVM located in the choroid plexus of the fourth ventricle.
DISCUSSION Sudden nontraumatic death in apparently healthy children accounts for 10% of all deaths in children, cardiovascular pathology being the most common finding at necropsies.4 The incidence of sudden death in children varies from 0.8 to 6.2 cases per 100.000 per year, which is much lower compared with adults.5 Intracerebral hemorrhage as a cause of sudden death in pediatric population is usually the consequence of a trauma. After a traumatic etiology is excluded, other conditions associated with central nervous system causes of sudden death that should be considered are aneurysms, AVM, brain tumors, vasculitis, cerebral infections, or hematological conditions.3 Arteriovenous malformations may be situated in any region of the brain, but very rarely, they can be restricted to the choroid plexus.1 Reviewing the literature, we found that sudden death due to AVMs of the choroid plexus is exceedingly rare in Am J Forensic Med Pathol
&
Volume 35, Number 2, June 2014
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Am J Forensic Med Pathol
&
Volume 35, Number 2, June 2014
Sudden Death From Ruptured Choroid Plexus AVM
FIGURE 1. Gross examination of the brain showed cerebral edema with scattered subarachnoid hemorrhage, marked in the basilar region (A), and with abundant blood filling the entire ventricular system (B). Figure 1 can be viewed online in color at www.amjforensicmedicine.com.
pediatric population because only 5 cases have been reported in children and adolescents younger than 15 years, in the period of 1990 to 2011.6Y8 Autopsy studies showed that only 12% of AVMs become symptomatic during life, whereas the rest remains clinically silent.2 Intracerebral hemorrhage is the most frequent clinical presentation of an AVM, but the tendency of bleeding is much higher in children (80%) than in adults (50%Y65%), with a worse prognosis compared with adults.2 There are several factors that are associated with a high risk for bleeding: small
AVMs, a single draining vein, high feeding arterial pressure, venous stenosis, and AVMs associated with an aneurysm. Some studies showed that AVMs in deep locations such as the basal ganglia and the periventricular or the intraventricular space increase the risk for bleeding.2 Histologically, AVMs are composed of tortuous dilated arteries and veins with varying size and wall thickness. When there is a significant difference of blood flow in an AVM compared with surrounding brain tissue, the arterial steal phenomenon may appear. In these conditions, the blood from the
FIGURE 2. Hematoxylin-eosin stain showing the choroid plexus with conglomeration of small vessels (A) and large vessels (B). Orcein stain demonstrating arterial components with internal elastic lamina at low power (C) and at high power (D). Figure 2 can be viewed online in color at www.amjforensicmedicine.com. * 2014 Lippincott Williams & Wilkins
www.amjforensicmedicine.com
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
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adjacent brain is diverted into the draining veins of AVM and leads to ischemic lesions,9 as in our case. The size of AVMs varies from giant forms to very small malformations that may be undetected by imagistic investigations (CT scan, magnetic resonance imaging, or angiography), namely, cryptic. In our case, the CT scan did not demonstrate the malformation, and because of the rapid evolution and the poor prognosis, a contrast CT was not performed. Moreover, an AVM accompanied by massive hemorrhage is not always grossly visible on autopsy examination. The pathologist should take numerous sections from the most hemorrhagic areas with adjacent brain parenchyma and from the choroid plexus. Even so, further samples may be necessary until the malformation is demonstrated.
CONCLUSIONS In this article, we reported a rare case of sudden death in a child, caused by a ruptured vascular malformation with an unusual location, which was not identified grossly but only on histological examination. The size and the location of the lesion, as well as the age of our patient, were contributing factors of the massive bleeding. Autopsy remains an important tool because it provides valuable information about the etiology of such bleedings, improves the knowledge about these lesions, and enhances the importance of the epidemiologic data.
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REFERENCES 1. Gu¨rcan F, Aribal ME, Baltacio?lu F, et al. Arteriovenous malformation of the choroid plexus. Australas Radiol. 1998;42:69Y71. 2. Forsting M, Wanke I. Intracranial vascular malformations and aneurysms. In: Christophe C, Laurent S, Laurent P, eds. Pial Arteriovenous Malformations. 2nd ed. Germany: Springer; 2006:51Y74. 3. Roger WB. Sudden Death in Infancy, Childhood, and Adolescence. Cambridge, UK: University Press; 2004:350Y358. 4. Christopher W. Sudden death in children and adolescents. Heart. 2002;88(4):426Y431. 5. Stuart B, John DK, John AT, et al. Sudden cardiac death in children and adolescents: introduction and overview. Pediatr Clin North Am. 2004;5:1201Y1209. 6. Jakob M, Ute L, Friedrich S. Intracranial arteriovenous malformations presenting as sudden unexpected deathVa report of 3 cases and review of the literature. Am J Forensic Med Pathol. 2007;28:173Y176. 7. Erich R, Christian G, Katharina C, et al. Sudden death from primary intraventricular hemorrhage in childhood. Eur J Paediatr Neurol. 1999;3:223Y226. 8. Tomcik MA, Gerig NR, Prahlow JA. Sudden death from ruptured intracranial vascular malformation. Forensic Sci Med Pathol. 2011;7:185Y191. 9. Roach ES, Lo WD, Heyer GL. Pediatric Stroke and Cerebrovascular Disorders. New York, NY: Demos Medical Publishing; 2011.
* 2014 Lippincott Williams & Wilkins
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
