Pediatr Radiol (1992) 22:138-139
Pediatric Radiology 9 Springer-Verlag 1992
Choroid plexus angioma: a rare cause of cerebral hemorrhage in childhood L. J. Abernethy 1, E. Phelan 1, and J. V. Rosenfeld 2 1Department of Radiology and 2Department of Neurosurgery, Royal Children's Hospital, Melbourne, Australia Received: 16 January 1992/Accepted: 20 January 1992
Choroid plexus angioma is a rare cause of cerebral h a e m o r r h a g e [1]. The radiological signs of this lesion are characteristically subtle. D e l a y e d diagnosis may result in sudden death from catastrophic cerebral h a e m o r r h a g e [2]. We describe the diagnosis of a choroid plexus angioma in an 8-year-old boy, who was subsequently t r e a t e d successfully by CT guided stereotactic surgery.
Casereport An eight year old boy, previously in good health, developed sudden generalised headache with nausea and vomiting. On examination, he was mildly drowsy with moderate meningism. His pulse rate was 92/minute, blood pressure 115/50 mmHg, and he was afebrile. There were no focal neurological signs. A lumbar puncture revealed evenly bloodstained cerebrospinal fluid. Computerised
tomography (CT) revealed an intracerebral haematoma in the right parietal lobe adjacent to the lateral ventricle, with blood within the ventricles and basal cisterns (Fig. 1). Initial management was conservative, Cerebral angiography was performed seven days after presentation. Right and left carotid angiograms showed subtle asymmetry between the choroidal vasculature, with an increase in the number of small arteries, which were abnormally tortuous, and a more prominent choroidal blush in the choroid plexus of the right lateral ventricle (Figs. 2, 3). There was no evidence of early filling or enlargement of drainingveins. A left vertebral angiogram showed no abnormality. An angioma of the right choroid plexus was diagnosed on the basis of these findings. Eight days after the initial haemorrhage, a CT-guided stereotactic right parieto-occipital craniotomy was performed. The intracerebral haematoma, which extended into the right lateral ventricle, was evacuated via a posterolateral transcortical approach. Abnormal, tortuous vessels were identified in
Fig. 1. CT brain scan. Intracerebral haematoma in fight parietal lobe and choroid plexus with intraventricular haemorrhage Fig. 2. Right carotid angiogram, showing abnormal vessels in fight choroid plexus (arrow) Fig. 3. Left carotid angiogram. Normal left choroid plexus (arrow)
the trigonal and posterior portions of the choroid plexus. The posterior choroidal feeding vessels were divided and the lesion was excised. Following the operation, the child recovered uneventfully, without neurological deficit. Histopathology of the lesion showed numerous well formed small arteries mixed with many dilated, thin walled vessels, consistent with an angioma.
Discussion A n g i o m a s of the choroid plexus are rare. In a recent review by van R y b r o e k and M o o r e [2], forty r e p o r t e d cases of vascular malformations (both artefiovenous malformations and angiomas) of the choroid plexus were found in the world literature. Twenty of the r e p o r t e d cases occurred in patients less than twenty years of age. 13 of these cases were diagnosed at post mortem. Vascular malformations in the choroid plexus m a y b e angiographically occult, because they are frequently found in the glomus, which is the most vascular part of the choroid plexus. H o d g e and King [3] reviewed 18 cases, of which only 7 were d e m o n s t r a t e d on cerebral angiography. In the 40 cases found in the world
139
literature by van Rybroek and Moore, 14cases were diagnosed by angiography. Coin et al. [4] described an extensive cerebral arteriovenous malformation in which choroidal involvement
or intraventricular haemorrhage in childhood, a careful search for abnorm a l i t i e s o f c h o r o i d a l vessels is necessary.
was n o t a p p a r e n t o n c e r e b r a l a n g i o graphy, but was demonstrated by arterial contrast injection during a CT examination. Our case illustrates the subtle angiog r a p h i c a b n o r m a l i t i e s w h i c h m a y indicate a vascular malformation of the chor o i d plexus. I n t h e i n v e s t i g a t i o n o f cases of a p p a r e n t l y s p o n t a n e o u s i n t r a c e r e b r a l
References
3. Hodge C J, King RB (1957) Arteriovenous malformation of choroid plexus. A case report. J Neurosurg 42:457-461 4. Coin CG, Coin JW, Glover M G (1976) Contrast enhancement by arterial perfusion during computerised tomography. Ne uroradiolo gy 11:119-121
1. Wilson CB, Stein BM (1984) Intracranial arteriovenous malformations. Williams and Wilkins, Baltimore London, p 115 2. Van Rybroek J J, Moore S A (1990) Sudden death from choroid plexus vascular malformation haemorrhage: case report and review of the literature. Clin Neuropathol 1: 39-45
Dr. E. Phelan D e p a r t m e n t of Radiology Royal Childrens Hospital Flemington Road Parkville, Victoria 3051 Australia
Literature in pediatric radiology Cardiac involvement in mucopolysaccharidosis: echocardiographic data in eight cases. [In french]. Ata, J. et al. (Serv. de Cardiol., CHU de Sousse, Tunisie) 38,614 (1991) Aunales de Radiologie (Paris)
Duplex Doppler ultrasonography of renal parenchyma arteries: normal and pathological aspects. [In french], Sauvain, J. L. et al. (Serv. d'Imagerie mrd, H6pital Paul Morel, 46, av Aristide Briand, F-70014 Vesoul, France) 34, 237 (1991) Neonatal aortic thrombosis. [In french], Teissier, J. M. et al. (Serv. de Rad. B, CHRU, H6pital Nord, place Victor Pauchet, F-80054 Amiens Cedex, France) 34, 256 (1991) Archives Franoises de Prdiatrie (Paris)
The Currarino triad: an important recognizable complex. [In french] de Lagansie, P. et al. (Serv. de Chirurg. Pdd., H6pital Robert-Debr6, 8, bouL. Seruier, F-75019 Paris, France) 48, 631 (1991) Un cas de thrombose veineuse crrrbrale et rrnale apr~s un cathdtdrisme cardiaque. Cas radiologique du mois. Achour, D.B. et al. (24, rue Tahar-ElHaddad, Montfleury, Tunis, Tunisie) 48,641 (1991) Un cas de neurofibrome en sabher chez un enfant de 13 ans. Cas radiologique du mois. Lucidarme, O. et al. (Montagne, J.-P., Serv. de Rad. Prd., H6pital Armand-Trousseau, 26, av. du Dr A.-Netter, F-75012 Paris, France) 48,643 (1991) Archives of Orthopaedic and Trauma Surgery (Berlin)
Trauma involving the proximal tibial epiphysis. Wozasek, G. E. et aL (Second Dept. of Tranma Surgery, Univ. Med. School, Spitalgasse 23, A-1090 Vienna, Austria) 110, 301 (1991) European Journal of Nuclear Medicine (Berlin) Postitive gallium-67 citrate scintigraphy in Wiskott-Aldrich syndrome with malignant lymphoma. Sandrock, D. et al. (Dept. of Nucl. Med., Georg August Univ., Robert-Koch-Str. 40, W-3400 Grttingen, FRG) 18, 928 (1991) Radionuclide blood pool scintigraphy in a child with intestinal arteriovenous malformation (juvenile angiodysplasia). Garty, I. et al. (Nucl. Med. Dept., Central Emek Hosp., Afula 18101, Israel) 18,992 (1991) European Journal of Pedialrics (Berhn) Left ventricular thrombus in a 2-year-old boy with cardiomyopathy: lysis with recombinant tissue-type plasminogen activator. Krogmann, O.N. et al. (Dept. of Paed., Heinrich Heine Univ., Moorenstr. 5, W-4000 Dt~sseldorf, FRG) 150,829 (1991) Cartilage hair hypoplasia in infancy: a misleading chondrodysplasia. Le Merrer, M., Maroteaux, R (URA584 CNRS, H6pital des Enfants-Malades, 149, rue de SSvres, F-Paris Cedex 15, France) 150, 847 (1991)
Continued from p. 130 Chronic pancreatitis in a child with glycogen storage disease type 1. Kikuchi, M. et al. (Dept. of Paed., Tohoku Univ. School of Med., 1-1 Seiryo-machi, Aoba-ku, Sendal, 980, Japan) 150, 852 (1991) Mitral valve and tricuspidal valve prolapse in Melnick-Needles syndrome. Kriiger, G. et al. (Div. of Neonatol. and Clinical Genetics, Children's Hosp., Rembrandtstr. 16/17, 0-2500 Rostock, FRG) 150, 858 (1991) Cystic mediastinal lesions in children: evaluation by magnetic resonance and conventional imaging. Kornreich, L. et al. (Dept. of Paed. Rad., Beilinson Med. Centre, Petah Tiqva 49100, Israel) 151, 38 (1992) Connatal rickets following repeated administration of phosphate enemas in pregnancy: a case report. Rimensberger, R et al. (ICU, Univ. Children's Hosp., CH-3010 Berne, Switzerland) 151, 54 (1992) Periventricular haemorrhagic infarction associated with subependymal germinal matrix haemorrhage in the premature newborn. Report of two cases. Haddad, J. et al. (Serv. de Ndonatol., Univ. Hosp., H6pital de Hautepierre, Av. Moli~re, F-67098 Strasbourg Cedex, France) 151, 63 (1992) Fortschritte auf dem Gebiete der Riintgenstrahlen und der neuen bildgebenden Verfahren (Stuttgart)
MRI of aneurysmal bone cyst. [In germ.] Sigmund, G. et al. (Abt. Rrntgendiagn., Rad. Univ.-Klinik, Hugstetter Str. 55, W-7800 Freiburg i. Br., FRG) 155, 289 (1991) Studies on diaphragm mobility in an infant with ultrafast serial snapshot Flash-MRT. [In germ.] Urhahn, R. et al. (Klinik ffir Rad. Diagn. der Med. Fakult~t der RWTH, Pauwelsstr. 30, W-5100 Aachen, FRG) 155, 478 (1991) Klinische Piidiatrie (Stuttgart) Gelastic epilepsy and precorious puberty due to hypothatamic hamartoma. [In germ.] Dammann, O. et al. (Univ.-Kinderldinik, Martinistr.52, W-2000 Hamburg 20, FRG) 203,439 (1991) Diagnosis of coxitis fugax, the commonest disease of the hip joint in childhood. - Evaluation of 131 own cases. [In germ.] Graf, J. et al. (Sektion Experimentelle Orthopfid., Orthop. Univ.-Klinik, Schlierbacher Landstr. 200, W-6900 Heidelberg, FRG) 203, 448 (1991) Monatsschrifl Kindecheilkunde (Berlin)
Macrocephaly as an initial symptom of glutaryl-CoA-dehydrogenase deficiency (ghitaric aciduria type I). [In germ.] Trefz, E K. et al. (Univ.-Kinderklinik, Im Neuenheimer Feld150, W-6900Heidelberg, FRG) 139, 754 (1991) Nasal encephalocele as a cause of recurrent bacterial meningitis. [In germ.] K6hler, T. et al. (Anna-Hosp., Evangelisches Kinderkrankenhans, Platz der Jugend 25, 0-2755 Schwerin, FRG) 139, 783 (1991) Neuroradioiogy (Berlin)
MR imaging of the brainstem: normal postnatal development. Martin, E. et al. (Univ. Children's Hosp., Steinwiesstr. 75, CH-8032 Ztirich, Switzerland) 33, 391 (1991) continued on p. 141
Pediatric Radiology 9 Springer-Verlag 1992
Choroid plexus angioma: a rare cause of cerebral hemorrhage in childhood L. J. Abernethy 1, E. Phelan 1, and J. V. Rosenfeld 2 1Department of Radiology and 2Department of Neurosurgery, Royal Children's Hospital, Melbourne, Australia Received: 16 January 1992/Accepted: 20 January 1992
Choroid plexus angioma is a rare cause of cerebral h a e m o r r h a g e [1]. The radiological signs of this lesion are characteristically subtle. D e l a y e d diagnosis may result in sudden death from catastrophic cerebral h a e m o r r h a g e [2]. We describe the diagnosis of a choroid plexus angioma in an 8-year-old boy, who was subsequently t r e a t e d successfully by CT guided stereotactic surgery.
Casereport An eight year old boy, previously in good health, developed sudden generalised headache with nausea and vomiting. On examination, he was mildly drowsy with moderate meningism. His pulse rate was 92/minute, blood pressure 115/50 mmHg, and he was afebrile. There were no focal neurological signs. A lumbar puncture revealed evenly bloodstained cerebrospinal fluid. Computerised
tomography (CT) revealed an intracerebral haematoma in the right parietal lobe adjacent to the lateral ventricle, with blood within the ventricles and basal cisterns (Fig. 1). Initial management was conservative, Cerebral angiography was performed seven days after presentation. Right and left carotid angiograms showed subtle asymmetry between the choroidal vasculature, with an increase in the number of small arteries, which were abnormally tortuous, and a more prominent choroidal blush in the choroid plexus of the right lateral ventricle (Figs. 2, 3). There was no evidence of early filling or enlargement of drainingveins. A left vertebral angiogram showed no abnormality. An angioma of the right choroid plexus was diagnosed on the basis of these findings. Eight days after the initial haemorrhage, a CT-guided stereotactic right parieto-occipital craniotomy was performed. The intracerebral haematoma, which extended into the right lateral ventricle, was evacuated via a posterolateral transcortical approach. Abnormal, tortuous vessels were identified in
Fig. 1. CT brain scan. Intracerebral haematoma in fight parietal lobe and choroid plexus with intraventricular haemorrhage Fig. 2. Right carotid angiogram, showing abnormal vessels in fight choroid plexus (arrow) Fig. 3. Left carotid angiogram. Normal left choroid plexus (arrow)
the trigonal and posterior portions of the choroid plexus. The posterior choroidal feeding vessels were divided and the lesion was excised. Following the operation, the child recovered uneventfully, without neurological deficit. Histopathology of the lesion showed numerous well formed small arteries mixed with many dilated, thin walled vessels, consistent with an angioma.
Discussion A n g i o m a s of the choroid plexus are rare. In a recent review by van R y b r o e k and M o o r e [2], forty r e p o r t e d cases of vascular malformations (both artefiovenous malformations and angiomas) of the choroid plexus were found in the world literature. Twenty of the r e p o r t e d cases occurred in patients less than twenty years of age. 13 of these cases were diagnosed at post mortem. Vascular malformations in the choroid plexus m a y b e angiographically occult, because they are frequently found in the glomus, which is the most vascular part of the choroid plexus. H o d g e and King [3] reviewed 18 cases, of which only 7 were d e m o n s t r a t e d on cerebral angiography. In the 40 cases found in the world
139
literature by van Rybroek and Moore, 14cases were diagnosed by angiography. Coin et al. [4] described an extensive cerebral arteriovenous malformation in which choroidal involvement
or intraventricular haemorrhage in childhood, a careful search for abnorm a l i t i e s o f c h o r o i d a l vessels is necessary.
was n o t a p p a r e n t o n c e r e b r a l a n g i o graphy, but was demonstrated by arterial contrast injection during a CT examination. Our case illustrates the subtle angiog r a p h i c a b n o r m a l i t i e s w h i c h m a y indicate a vascular malformation of the chor o i d plexus. I n t h e i n v e s t i g a t i o n o f cases of a p p a r e n t l y s p o n t a n e o u s i n t r a c e r e b r a l
References
3. Hodge C J, King RB (1957) Arteriovenous malformation of choroid plexus. A case report. J Neurosurg 42:457-461 4. Coin CG, Coin JW, Glover M G (1976) Contrast enhancement by arterial perfusion during computerised tomography. Ne uroradiolo gy 11:119-121
1. Wilson CB, Stein BM (1984) Intracranial arteriovenous malformations. Williams and Wilkins, Baltimore London, p 115 2. Van Rybroek J J, Moore S A (1990) Sudden death from choroid plexus vascular malformation haemorrhage: case report and review of the literature. Clin Neuropathol 1: 39-45
Dr. E. Phelan D e p a r t m e n t of Radiology Royal Childrens Hospital Flemington Road Parkville, Victoria 3051 Australia
Literature in pediatric radiology Cardiac involvement in mucopolysaccharidosis: echocardiographic data in eight cases. [In french]. Ata, J. et al. (Serv. de Cardiol., CHU de Sousse, Tunisie) 38,614 (1991) Aunales de Radiologie (Paris)
Duplex Doppler ultrasonography of renal parenchyma arteries: normal and pathological aspects. [In french], Sauvain, J. L. et al. (Serv. d'Imagerie mrd, H6pital Paul Morel, 46, av Aristide Briand, F-70014 Vesoul, France) 34, 237 (1991) Neonatal aortic thrombosis. [In french], Teissier, J. M. et al. (Serv. de Rad. B, CHRU, H6pital Nord, place Victor Pauchet, F-80054 Amiens Cedex, France) 34, 256 (1991) Archives Franoises de Prdiatrie (Paris)
The Currarino triad: an important recognizable complex. [In french] de Lagansie, P. et al. (Serv. de Chirurg. Pdd., H6pital Robert-Debr6, 8, bouL. Seruier, F-75019 Paris, France) 48, 631 (1991) Un cas de thrombose veineuse crrrbrale et rrnale apr~s un cathdtdrisme cardiaque. Cas radiologique du mois. Achour, D.B. et al. (24, rue Tahar-ElHaddad, Montfleury, Tunis, Tunisie) 48,641 (1991) Un cas de neurofibrome en sabher chez un enfant de 13 ans. Cas radiologique du mois. Lucidarme, O. et al. (Montagne, J.-P., Serv. de Rad. Prd., H6pital Armand-Trousseau, 26, av. du Dr A.-Netter, F-75012 Paris, France) 48,643 (1991) Archives of Orthopaedic and Trauma Surgery (Berlin)
Trauma involving the proximal tibial epiphysis. Wozasek, G. E. et aL (Second Dept. of Tranma Surgery, Univ. Med. School, Spitalgasse 23, A-1090 Vienna, Austria) 110, 301 (1991) European Journal of Nuclear Medicine (Berlin) Postitive gallium-67 citrate scintigraphy in Wiskott-Aldrich syndrome with malignant lymphoma. Sandrock, D. et al. (Dept. of Nucl. Med., Georg August Univ., Robert-Koch-Str. 40, W-3400 Grttingen, FRG) 18, 928 (1991) Radionuclide blood pool scintigraphy in a child with intestinal arteriovenous malformation (juvenile angiodysplasia). Garty, I. et al. (Nucl. Med. Dept., Central Emek Hosp., Afula 18101, Israel) 18,992 (1991) European Journal of Pedialrics (Berhn) Left ventricular thrombus in a 2-year-old boy with cardiomyopathy: lysis with recombinant tissue-type plasminogen activator. Krogmann, O.N. et al. (Dept. of Paed., Heinrich Heine Univ., Moorenstr. 5, W-4000 Dt~sseldorf, FRG) 150,829 (1991) Cartilage hair hypoplasia in infancy: a misleading chondrodysplasia. Le Merrer, M., Maroteaux, R (URA584 CNRS, H6pital des Enfants-Malades, 149, rue de SSvres, F-Paris Cedex 15, France) 150, 847 (1991)
Continued from p. 130 Chronic pancreatitis in a child with glycogen storage disease type 1. Kikuchi, M. et al. (Dept. of Paed., Tohoku Univ. School of Med., 1-1 Seiryo-machi, Aoba-ku, Sendal, 980, Japan) 150, 852 (1991) Mitral valve and tricuspidal valve prolapse in Melnick-Needles syndrome. Kriiger, G. et al. (Div. of Neonatol. and Clinical Genetics, Children's Hosp., Rembrandtstr. 16/17, 0-2500 Rostock, FRG) 150, 858 (1991) Cystic mediastinal lesions in children: evaluation by magnetic resonance and conventional imaging. Kornreich, L. et al. (Dept. of Paed. Rad., Beilinson Med. Centre, Petah Tiqva 49100, Israel) 151, 38 (1992) Connatal rickets following repeated administration of phosphate enemas in pregnancy: a case report. Rimensberger, R et al. (ICU, Univ. Children's Hosp., CH-3010 Berne, Switzerland) 151, 54 (1992) Periventricular haemorrhagic infarction associated with subependymal germinal matrix haemorrhage in the premature newborn. Report of two cases. Haddad, J. et al. (Serv. de Ndonatol., Univ. Hosp., H6pital de Hautepierre, Av. Moli~re, F-67098 Strasbourg Cedex, France) 151, 63 (1992) Fortschritte auf dem Gebiete der Riintgenstrahlen und der neuen bildgebenden Verfahren (Stuttgart)
MRI of aneurysmal bone cyst. [In germ.] Sigmund, G. et al. (Abt. Rrntgendiagn., Rad. Univ.-Klinik, Hugstetter Str. 55, W-7800 Freiburg i. Br., FRG) 155, 289 (1991) Studies on diaphragm mobility in an infant with ultrafast serial snapshot Flash-MRT. [In germ.] Urhahn, R. et al. (Klinik ffir Rad. Diagn. der Med. Fakult~t der RWTH, Pauwelsstr. 30, W-5100 Aachen, FRG) 155, 478 (1991) Klinische Piidiatrie (Stuttgart) Gelastic epilepsy and precorious puberty due to hypothatamic hamartoma. [In germ.] Dammann, O. et al. (Univ.-Kinderldinik, Martinistr.52, W-2000 Hamburg 20, FRG) 203,439 (1991) Diagnosis of coxitis fugax, the commonest disease of the hip joint in childhood. - Evaluation of 131 own cases. [In germ.] Graf, J. et al. (Sektion Experimentelle Orthopfid., Orthop. Univ.-Klinik, Schlierbacher Landstr. 200, W-6900 Heidelberg, FRG) 203, 448 (1991) Monatsschrifl Kindecheilkunde (Berlin)
Macrocephaly as an initial symptom of glutaryl-CoA-dehydrogenase deficiency (ghitaric aciduria type I). [In germ.] Trefz, E K. et al. (Univ.-Kinderklinik, Im Neuenheimer Feld150, W-6900Heidelberg, FRG) 139, 754 (1991) Nasal encephalocele as a cause of recurrent bacterial meningitis. [In germ.] K6hler, T. et al. (Anna-Hosp., Evangelisches Kinderkrankenhans, Platz der Jugend 25, 0-2755 Schwerin, FRG) 139, 783 (1991) Neuroradioiogy (Berlin)
MR imaging of the brainstem: normal postnatal development. Martin, E. et al. (Univ. Children's Hosp., Steinwiesstr. 75, CH-8032 Ztirich, Switzerland) 33, 391 (1991) continued on p. 141
