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A Hidden Cause of Hemorrhage: Cavernous Venous Malformation
Pediatric Neurology xxx (2016) 1e2
Contents lists available at ScienceDirect
Pediatric Neurology journal homepage: www.elsevier.com/locate/pnu
Visual Diagnosis
A Hidden Cause of Hemorrhage: Cavernous Venous Malformation Arpita Lakhotia MBBS *, Hala ElMoctar MD, Jessica Goldstein MD Pediatric Neurology, UH Rainbow Babies and Children’s Hospital, Cleveland, Ohio
Patient Description
Discussion
This 14-year-old girl presented with a focal seizure (right gaze version and tonic flexion of arms) after spending the day at an amusement park, riding on roller coasters. Computed tomography scan of head showed left parietal intraparenchymal hemorrhage (Fig 1A). Magnetic resonance imaging and magnetic resonance angiography suggested a left temporoparietal cavernous malformation (Fig 1B) with an adjacent left developmental venous anomaly (Fig 2).
Cavernous venous malformations are collections of dilated blood vessels (“caverns”) through which blood flows slowly. About 0.5% to 1% of the population has a cavernous malformation, although up to 40% never experience symptoms. Cavernous malformations can either be sporadic and isolated or familial with autosomal dominant forms
FIGURE 1. (A) Noncontrast axial computed tomography shows a left parietal parenchymal hemorrhage. (B) T2-weighted magnetic resonance imaging depicts a left temporoparietal cavernous venous malformation. The characteristic “popcorn” appearance with mixed signal intensities in the center results from blood in various stages of evolution surrounded by a hypointense hemosiderin rim.
* Communications should be addressed to: Dr. Lakhotia; Pediatric Neurology; UH Rainbow Babies and Children’s Hospital; 11100 Euclid Avenue; Cleveland, OH 44106. E-mail address: [email protected] 0887-8994/$ e see front matter Ó 2016 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.pediatrneurol.2016.07.008
where individuals tend to have multiple venous malformations throughout the body. As in this child, cerebral cavernous malformations are often found adjacent to a developmental venous anomaly, which are usually asymptomatic anomalies of draining veins. Developmental venous
2
A. Lakhotia et al. / Pediatric Neurology xxx (2016) 1e2
tomatic lesions may require surgical intervention.1 Developmental venous anomalies, on the other hand, typically are asymptomatic and incidentally found. These may increase the risk of bleeding during surgery. Hemorrhagic stroke has been documented after roller coaster rides in a child with an arterial dissection.2 Another teenager developed a subdural hemorrhage after riding a roller coaster.3 Bleeding in these patients has been attributed to high gravitational forces (G forces) on roller coaster rides, although a causal relationship has not been fully established.4 Our patient had spent the day riding roller coasters and then presented with an intracranial hemorrhage from a previously undiagnosed cavernous venous malformation. The temporal relationship of events as well as the risk of intracranial hemorrhage with cavernous venous malformations led us to attribute her hemorrhage to the G-forces exerted by the roller coaster ride. These forces may have “tipped it over the edge” from being an asymptomatic lesion to a symptomatic one.
FIGURE 2. Magnetic resonance imaging with contrast demonstrated a developmental venous anomaly with smaller veins draining into a single larger draining vein (arrow). These anomalies are usually incidental and often occur in association with a cavernous venous malformation.
anomalies consist of a collection of veins (“caput medusae”) draining into a single larger vein on imaging (Fig 2). Cavernous malformations can present with a wide variety of symptoms including headaches, focal neurological deficit, intracranial hemorrhage, or seizures, and symp-
References 1. Horne M, Flemming K, Su I, et al. Clinical course of untreated cerebral cavernous malformations: a meta-analysis of individual patient data. Lancet Neurol. 2016;15:166-173. 2. Nouh A, Vela-Duarte D, Grobelny T, Hoganson G, Pasquale D, Biller J. Internal carotid artery dissection after a roller coaster ride in a 4year-old: case report and review of the literature. Pediatr Neurol. 2015;52:349-351. 3. Roldan-Valadez E, Facha M, Martinez-Lopez M, Herrera-Mora P. Subdural hematoma in a teenager related to roller-coaster ride. Eur J Paediatric Neurol. 2006;10:194-196. 4. Pfister B, Chickola L, Smith D. Head motions while riding roller coasters. Am J Forensic Med Pathol. 2009;30:339-345.
Contents lists available at ScienceDirect
Pediatric Neurology journal homepage: www.elsevier.com/locate/pnu
Visual Diagnosis
A Hidden Cause of Hemorrhage: Cavernous Venous Malformation Arpita Lakhotia MBBS *, Hala ElMoctar MD, Jessica Goldstein MD Pediatric Neurology, UH Rainbow Babies and Children’s Hospital, Cleveland, Ohio
Patient Description
Discussion
This 14-year-old girl presented with a focal seizure (right gaze version and tonic flexion of arms) after spending the day at an amusement park, riding on roller coasters. Computed tomography scan of head showed left parietal intraparenchymal hemorrhage (Fig 1A). Magnetic resonance imaging and magnetic resonance angiography suggested a left temporoparietal cavernous malformation (Fig 1B) with an adjacent left developmental venous anomaly (Fig 2).
Cavernous venous malformations are collections of dilated blood vessels (“caverns”) through which blood flows slowly. About 0.5% to 1% of the population has a cavernous malformation, although up to 40% never experience symptoms. Cavernous malformations can either be sporadic and isolated or familial with autosomal dominant forms
FIGURE 1. (A) Noncontrast axial computed tomography shows a left parietal parenchymal hemorrhage. (B) T2-weighted magnetic resonance imaging depicts a left temporoparietal cavernous venous malformation. The characteristic “popcorn” appearance with mixed signal intensities in the center results from blood in various stages of evolution surrounded by a hypointense hemosiderin rim.
* Communications should be addressed to: Dr. Lakhotia; Pediatric Neurology; UH Rainbow Babies and Children’s Hospital; 11100 Euclid Avenue; Cleveland, OH 44106. E-mail address: [email protected] 0887-8994/$ e see front matter Ó 2016 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.pediatrneurol.2016.07.008
where individuals tend to have multiple venous malformations throughout the body. As in this child, cerebral cavernous malformations are often found adjacent to a developmental venous anomaly, which are usually asymptomatic anomalies of draining veins. Developmental venous
2
A. Lakhotia et al. / Pediatric Neurology xxx (2016) 1e2
tomatic lesions may require surgical intervention.1 Developmental venous anomalies, on the other hand, typically are asymptomatic and incidentally found. These may increase the risk of bleeding during surgery. Hemorrhagic stroke has been documented after roller coaster rides in a child with an arterial dissection.2 Another teenager developed a subdural hemorrhage after riding a roller coaster.3 Bleeding in these patients has been attributed to high gravitational forces (G forces) on roller coaster rides, although a causal relationship has not been fully established.4 Our patient had spent the day riding roller coasters and then presented with an intracranial hemorrhage from a previously undiagnosed cavernous venous malformation. The temporal relationship of events as well as the risk of intracranial hemorrhage with cavernous venous malformations led us to attribute her hemorrhage to the G-forces exerted by the roller coaster ride. These forces may have “tipped it over the edge” from being an asymptomatic lesion to a symptomatic one.
FIGURE 2. Magnetic resonance imaging with contrast demonstrated a developmental venous anomaly with smaller veins draining into a single larger draining vein (arrow). These anomalies are usually incidental and often occur in association with a cavernous venous malformation.
anomalies consist of a collection of veins (“caput medusae”) draining into a single larger vein on imaging (Fig 2). Cavernous malformations can present with a wide variety of symptoms including headaches, focal neurological deficit, intracranial hemorrhage, or seizures, and symp-
References 1. Horne M, Flemming K, Su I, et al. Clinical course of untreated cerebral cavernous malformations: a meta-analysis of individual patient data. Lancet Neurol. 2016;15:166-173. 2. Nouh A, Vela-Duarte D, Grobelny T, Hoganson G, Pasquale D, Biller J. Internal carotid artery dissection after a roller coaster ride in a 4year-old: case report and review of the literature. Pediatr Neurol. 2015;52:349-351. 3. Roldan-Valadez E, Facha M, Martinez-Lopez M, Herrera-Mora P. Subdural hematoma in a teenager related to roller-coaster ride. Eur J Paediatric Neurol. 2006;10:194-196. 4. Pfister B, Chickola L, Smith D. Head motions while riding roller coasters. Am J Forensic Med Pathol. 2009;30:339-345.