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Dehydroepiandrosterone 7α-hydroxylation in human tissues: Possible interference with type 1 11β-hydroxysteroid dehydrogenase-mediated processes
Case Report Pediatr Neurosurg 2003;39:108–111 DOI: 10.1159/000071323
Received: December 5, 2002 Accepted: March 20, 2003
Ruptured Middle Cerebral Artery Aneurysm with Intramural Myxoid Degeneration in a Child Keishi Fujita a Kiyoyuki Yanaka b Takao Kamezaki a Masayuki Noguchi c Tadao Nose b a Department
and
of Neurosurgery, Ibaraki Seinan Medical Center Hospital, Sashima, Departments of b Neurosurgery, Institute of Clinical Medicine, University of Tsukuba, Tsukuba, Ibaraki, Japan
c Pathology,
Key Words Aneurysm W Dissection W Myxoid degeneration
Abstract A 9-year-old girl presented with a subarachnoid hemorrhage. Cerebral angiography showed a saccular aneurysm arising from the horizontal portion of the right middle cerebral artery. The aneurysm was successfully clipped, and the dome of the aneurysm was excised for pathological study. Histological examination of the aneurysm wall showed that the entire thickness of the wall showed an increased myxoid degeneration. No dissection was present. Most intracranial aneurysms in childhood are believed to be of the saccular type similar to that in adults, but the pathogenesis of the aneurysm formation remains controversial. Myxoid degeneration may cause intracranial saccular aneurysm with eventual rupture, even in the absence of dissection. This is the first case reported of a ruptured saccular aneurysm caused by myxoid degeneration in a child. The possible pathophysiology is discussed. Copyright © 2003 S. Karger AG, Basel
ABC
© 2003 S. Karger AG, Basel 1016–2291/03/0392–0108$19.50/0
Fax + 41 61 306 12 34 E-Mail [email protected] www.karger.com
Accessible online at: www.karger.com/pne
Introduction
Cerebral saccular aneurysms are rare in childhood, and patients under the age of 18 account for only 1–2% of the cases of aneurysmal subarachnoid hemorrhages [1]. Saccular aneurysms of cerebral arteries have as their common background a weakening of the tensile strength of a given segment of the artery. The reasons include congenital developmental defects and acquired injuries to the vessel walls in the form of atherosclerosis and various types of vasculitides. In childhood, a congenital pathogenesis appears more convincing because of the tendency to occur at peripheral sites, the high frequency of large or giant aneurysms and the association with other cerebral and vascular congenital abnormalities [2]. Most intracranial aneurysms in childhood are believed to be of the saccular type, similar to that in adults, but the pathogenesis of the aneurysm formation remains controversial. As seen in Ehlers-Danlos syndrome, a constitutional structural weakness of collagen and elastic fibers may affect the arterial walls [3]. Myxoid degeneration of the arterial wall may also contribute to the weakening of arterial walls, and can also result in dissection or dissecting aneurysms [4]. We describe herein a 9-year-old girl who presented with a subarachnoid hemorrhage from a
Kiyoyuki Yanaka, MD Department of Neurosurgery, Institute of Clinical Medicine University of Tsukuba Tsukuba, Ibaraki 305-8575 (Japan) Tel. +81 298 53 7593, Fax +81 298 53 3214, E-Mail [email protected]
1
2
Fig. 1. Computed tomography showing diffuse subarachnoid hemorrhage with hematoma in the right Sylvian fissure. Fig. 2. Cerebral angiography showing a saccular aneurysm arising from the horizontal portion of the right middle cerebral artery.
ruptured middle cerebral artery aneurysm. Myxoid degeneration was observed in the entire thickness of the wall of the aneurysm without dissection. This is the first report of a ruptured cerebral aneurysm with myxoid degeneration in a child.
Case Report A 9-year-old girl of normal mental and physical development with an uneventful history suddenly lost consciousness and showed left-sided hemiparesis. Upon admission to our hospital, she was semicomatose and had left-sided hemiparesis. Physical examination showed a pulse rate of 82/min and blood pressure of 122/76 mm Hg. She did not have any of the stigmas of Marfan’s syndrome, such as arachnodactyly, dislocation of the lens and high arched palate, or of Ehlers-Danlos syndrome, such as hyperextendability of the joints. She had no significant family history or past history. Routine laboratory tests, electrocardiogram and chest X-ray were normal. Computed tomography revealed diffuse subarachnoid hemorrhage with a large hematoma in the right Sylvian fissure (fig. 1). Cerebral angiography demonstrated a saccular aneurysm arising from the horizontal portion of the right middle cerebral artery. It was away from the M1 and M2 junction (fig. 2). No other vascular involvement was seen during four-vessel cerebral angiography. The patient was taken directly to the operating room and surgery was performed. At surgery, the hematoma in the Sylvian fissure was evacuated and successful neck clipping was performed through a right pterional approach. The neck of the aneurysm was broad-based, had a thick wall and arose directly from the horizontal portion of the middle cerebral artery. There was no arterial branch around the neck. No atherosclerotic change was seen on the surface of the intracranial vessels. The dome of the aneurysm was excised for histological examination.
Intracranial Myxoid Aneurysm
The postoperative course was uneventful, and her clouding of consciousness and left-sided hemiparesis improved gradually. About 3 weeks after surgery, the patient was discharged from our hospital without any neurological deficits. Histological examination of the aneurysmal wall demonstrated that both the intima and the media had been replaced by myxoid tissue with scattered collagen fibers. The entire thickness of the wall was composed of myxoid tissue with spidery multipolar cells that had replaced the normal fibroblasts and smooth muscle cells (fig. 3). Elastica van Gieson staining demonstrated that there was no internal elastic membrane. Immunohistological study showed positive for vimentin stain and negative for alpha-smooth muscle antigen, desmin, and epithelial membrane antigen. Colloidal iron strain for acid mucopolysaccharides showed a positive reaction in the wall matrix (fig. 4). No dissection of the wall was present. There were no inflammatory changes or atherosclerotic changes.
Discussion
Injury to the internal elastic lamina by hemodynamic stresses and absence of media resulting in aneurysm formation are generally accepted mechanisms for the pathogenesis of aneurysm formation [1]. However, such intrinsic hemodynamic factors almost certainly play less of a role in children than in adults, and mural or systemic factors are considered to be more important [5–7]. The aneurysm of the present case is distinct from those found in the adult population, both in terms of the location and the histological findings; the aneurysm arose directly from the arterial trunk without branching and showed a marked
Pediatr Neurosurg 2003;39:108–111
109
Fig. 3. Photomicrograph of the aneurysmal
wall. The entire thickness of the aneurysmal wall shows marked myxoid degeneration. No dissection is seen. HE. Original magnification !64.
Fig. 4. Photomicrograph of the aneurysmal
wall. Staining of the wall matrix suggests a myxoid change. Colloidal iron stain. Original magnification !64.
myxoid degeneration in the entire thickness of the aneurysm wall. No dissection was seen in this case. While dissection is the most common complication of myxoid degeneration, only one case of intracranial nondissecting aneurysm has been reported in the distal middle cerebral artery in an adults [4]. That case presented with aneurysmal subarachnoid hemorrhage, and underwent microsurgical clipping of the aneurysm. With regard
110
Pediatr Neurosurg 2003;39:108–111
to extracranial arteries, there have been two reports of nondissecting aneurysms caused by myxoid degeneration in the cervical portion of the carotid artery [8, 9]. Four cases of intracranial dissecting aneurysms in adults have also been reported [10–13]. Therefore, intracranial aneurysms with myxoid degeneration are extremely rare. Although such pathological changes may be underreported because aneurysmal walls or domes are not subjected to
Fujita/Yanaka/Kamezaki/Noguchi/Nose
pathological analysis in most instances, to our knowledge, there have been no reports of intracranial myxoid degeneration of arterial walls with nondissecting aneurysm formation in a child, and this case is the first reported instance. The structural weakness of the arterial wall due to myxoid degeneration may have resulted in the formation of the nondissecting aneurysm. The etiology of myxoid degeneration is unknown, but is thought to be associated with the physiological process of aging, myxedema heart disease, idiopathic familial cardiomyopathy, Marfan’s syndrome [14], Ehlers-Danlos syndrome [15] and mediolytic arteriopathy [16]. In these diseases, myxoid degeneration of the wall may worsen the arterial damage, most commonly in the form of dissec-
tions. Some authors have found the presence of myxoid degeneration in apparently healthy arteries [10–12]. Our patient had no signs or history of the above-mentioned clinical entities. The cause and mechanism of the myxoid degeneration remains to be determined, and long-term follow-up seems essential in such cases. Myxoid generation has been thought to be associated with dissecting aneurysm formation. However, this case confirms that myxoid degeneration of the arterial walls may cause saccular-shaped aneurysms with eventual rupture, even in the absence of dissection. This case also provides new insight into the pathogenesis of aneurysmal formation.
References 1 Weir B, Macdonald RL: Intracranial aneurysms and subarachnoid hemorrhage: An overview; in Wilkins RH, Rengachary SS (eds): Neurosurgey, ed 2. New York, McGraw-Hill, 1996, pp 2191–2214. 2 Ferrante L, Fortuna A, Celli P, Santoro A, Fraioli B: Intracranial arterial aneurysms in early childhood. Surg Neurol 1988;29:39–56. 3 Kato T, Hattori H, Yorifuji T, Tashiro Y, Nakahata T: Intracranial aneurysms in EhlersDanlos syndrome type IV in early childhood. Pediatr Neurol 2001;25:336–339. 4 O’Boynick P, Green KD, Batnitzky S, Kepes JJ, Pietak R: Aneurysm of the left middle cerebral artery caused by myxoid degeneration of the vessel wall. Stroke 1994;25:2283–2286. 5 Lasjaunias P: Vascular Diseases in Neonates, Infants and Children. Berlin, Springer, 1997, pp 373–392. 6 Yanaka K, Meguro K, Narushima K, Takano S, Doi M, Nose T: Basal perforating artery aneurysm within the cavum septi pellucidi. Case report. J Neurosurg 1998;88:601–604.
Intracranial Myxoid Aneurysm
7 Yanaka K, Tsuboi K, Fujita K, Aoki K, Takeuchi S, Anno I, Nose T: Distal anterior choroidal artery aneurysm associated with an arteriovenous malformation. Intraoperative localization and treatment. Surg Neurol 2000;53:546–551. 8 Barnes WT, Jacoby JE: Aneurysm of the common carotid artery due to cystic medical necrosis treated by excision and graft. Ann Surg 1962;155:82–85. 9 Martinez Perez M, Pintos Diaz G, Varela Duran J: Aneurysm of the extracranial internal carotid caused by myxoid degeneration of the middle layer (in Spanish). Rev Quir Esp 1988; 15:112–114. 10 Hamada Y, Mannoji H, Kaneko Y: A ruptured dissecting aneurysm of the vertebral artery. Comparison of angiographic and histological findings. Neuroradiology 2001;43:375–378. 11 Ide Y, Fukushima T, Yamamoto M, Tomonaga M: Vertebral dissecting aneurysm associated with medial mucoid degeneration. Case report and review of literature (in Japanese). Neurol Med Chir (Tokyo) 1986;26:888–894.
12 Manz HJ, Luessenhop AJ: Dissecting aneurysm of intracranial vertebral artery. Case report and review of literature. J Neurol 1983; 230:25–35. 13 Thal DR, Schober R, Schlote W: Carotid artery dissection in a young adult. Cystic medial necrosis associated with an increased elastase content. Clin Neuropathol 1997;16:180–184. 14 Schievink WI, Parisi JE, Piepgras DG, Michels VV: Intracranial aneurysms in Marfan’s syndrome: An autopsy study. Neurosurgery 1997; 41:866–871. 15 Rubinstein MK, Cohen NH: Ehlers-Danlos syndrome associated with multiple intracranial aneurysms. Neurology 1964;14:125–132. 16 Leu HJ: Cerebrovascular accidents resulting from segmental mediolytic arteriopathy of the cerebral arteries in young adults. Cardiovasc Surg 1994;2:350–353.
Pediatr Neurosurg 2003;39:108–111
111
Copyright: S. Karger AG, Basel 2003. Reproduced with the permission of S. Karger AG, Basel. Further reproduction or distribution (electronic or otherwise) is prohibited without permission from the copyright holder.
Received: December 5, 2002 Accepted: March 20, 2003
Ruptured Middle Cerebral Artery Aneurysm with Intramural Myxoid Degeneration in a Child Keishi Fujita a Kiyoyuki Yanaka b Takao Kamezaki a Masayuki Noguchi c Tadao Nose b a Department
and
of Neurosurgery, Ibaraki Seinan Medical Center Hospital, Sashima, Departments of b Neurosurgery, Institute of Clinical Medicine, University of Tsukuba, Tsukuba, Ibaraki, Japan
c Pathology,
Key Words Aneurysm W Dissection W Myxoid degeneration
Abstract A 9-year-old girl presented with a subarachnoid hemorrhage. Cerebral angiography showed a saccular aneurysm arising from the horizontal portion of the right middle cerebral artery. The aneurysm was successfully clipped, and the dome of the aneurysm was excised for pathological study. Histological examination of the aneurysm wall showed that the entire thickness of the wall showed an increased myxoid degeneration. No dissection was present. Most intracranial aneurysms in childhood are believed to be of the saccular type similar to that in adults, but the pathogenesis of the aneurysm formation remains controversial. Myxoid degeneration may cause intracranial saccular aneurysm with eventual rupture, even in the absence of dissection. This is the first case reported of a ruptured saccular aneurysm caused by myxoid degeneration in a child. The possible pathophysiology is discussed. Copyright © 2003 S. Karger AG, Basel
ABC
© 2003 S. Karger AG, Basel 1016–2291/03/0392–0108$19.50/0
Fax + 41 61 306 12 34 E-Mail [email protected] www.karger.com
Accessible online at: www.karger.com/pne
Introduction
Cerebral saccular aneurysms are rare in childhood, and patients under the age of 18 account for only 1–2% of the cases of aneurysmal subarachnoid hemorrhages [1]. Saccular aneurysms of cerebral arteries have as their common background a weakening of the tensile strength of a given segment of the artery. The reasons include congenital developmental defects and acquired injuries to the vessel walls in the form of atherosclerosis and various types of vasculitides. In childhood, a congenital pathogenesis appears more convincing because of the tendency to occur at peripheral sites, the high frequency of large or giant aneurysms and the association with other cerebral and vascular congenital abnormalities [2]. Most intracranial aneurysms in childhood are believed to be of the saccular type, similar to that in adults, but the pathogenesis of the aneurysm formation remains controversial. As seen in Ehlers-Danlos syndrome, a constitutional structural weakness of collagen and elastic fibers may affect the arterial walls [3]. Myxoid degeneration of the arterial wall may also contribute to the weakening of arterial walls, and can also result in dissection or dissecting aneurysms [4]. We describe herein a 9-year-old girl who presented with a subarachnoid hemorrhage from a
Kiyoyuki Yanaka, MD Department of Neurosurgery, Institute of Clinical Medicine University of Tsukuba Tsukuba, Ibaraki 305-8575 (Japan) Tel. +81 298 53 7593, Fax +81 298 53 3214, E-Mail [email protected]
1
2
Fig. 1. Computed tomography showing diffuse subarachnoid hemorrhage with hematoma in the right Sylvian fissure. Fig. 2. Cerebral angiography showing a saccular aneurysm arising from the horizontal portion of the right middle cerebral artery.
ruptured middle cerebral artery aneurysm. Myxoid degeneration was observed in the entire thickness of the wall of the aneurysm without dissection. This is the first report of a ruptured cerebral aneurysm with myxoid degeneration in a child.
Case Report A 9-year-old girl of normal mental and physical development with an uneventful history suddenly lost consciousness and showed left-sided hemiparesis. Upon admission to our hospital, she was semicomatose and had left-sided hemiparesis. Physical examination showed a pulse rate of 82/min and blood pressure of 122/76 mm Hg. She did not have any of the stigmas of Marfan’s syndrome, such as arachnodactyly, dislocation of the lens and high arched palate, or of Ehlers-Danlos syndrome, such as hyperextendability of the joints. She had no significant family history or past history. Routine laboratory tests, electrocardiogram and chest X-ray were normal. Computed tomography revealed diffuse subarachnoid hemorrhage with a large hematoma in the right Sylvian fissure (fig. 1). Cerebral angiography demonstrated a saccular aneurysm arising from the horizontal portion of the right middle cerebral artery. It was away from the M1 and M2 junction (fig. 2). No other vascular involvement was seen during four-vessel cerebral angiography. The patient was taken directly to the operating room and surgery was performed. At surgery, the hematoma in the Sylvian fissure was evacuated and successful neck clipping was performed through a right pterional approach. The neck of the aneurysm was broad-based, had a thick wall and arose directly from the horizontal portion of the middle cerebral artery. There was no arterial branch around the neck. No atherosclerotic change was seen on the surface of the intracranial vessels. The dome of the aneurysm was excised for histological examination.
Intracranial Myxoid Aneurysm
The postoperative course was uneventful, and her clouding of consciousness and left-sided hemiparesis improved gradually. About 3 weeks after surgery, the patient was discharged from our hospital without any neurological deficits. Histological examination of the aneurysmal wall demonstrated that both the intima and the media had been replaced by myxoid tissue with scattered collagen fibers. The entire thickness of the wall was composed of myxoid tissue with spidery multipolar cells that had replaced the normal fibroblasts and smooth muscle cells (fig. 3). Elastica van Gieson staining demonstrated that there was no internal elastic membrane. Immunohistological study showed positive for vimentin stain and negative for alpha-smooth muscle antigen, desmin, and epithelial membrane antigen. Colloidal iron strain for acid mucopolysaccharides showed a positive reaction in the wall matrix (fig. 4). No dissection of the wall was present. There were no inflammatory changes or atherosclerotic changes.
Discussion
Injury to the internal elastic lamina by hemodynamic stresses and absence of media resulting in aneurysm formation are generally accepted mechanisms for the pathogenesis of aneurysm formation [1]. However, such intrinsic hemodynamic factors almost certainly play less of a role in children than in adults, and mural or systemic factors are considered to be more important [5–7]. The aneurysm of the present case is distinct from those found in the adult population, both in terms of the location and the histological findings; the aneurysm arose directly from the arterial trunk without branching and showed a marked
Pediatr Neurosurg 2003;39:108–111
109
Fig. 3. Photomicrograph of the aneurysmal
wall. The entire thickness of the aneurysmal wall shows marked myxoid degeneration. No dissection is seen. HE. Original magnification !64.
Fig. 4. Photomicrograph of the aneurysmal
wall. Staining of the wall matrix suggests a myxoid change. Colloidal iron stain. Original magnification !64.
myxoid degeneration in the entire thickness of the aneurysm wall. No dissection was seen in this case. While dissection is the most common complication of myxoid degeneration, only one case of intracranial nondissecting aneurysm has been reported in the distal middle cerebral artery in an adults [4]. That case presented with aneurysmal subarachnoid hemorrhage, and underwent microsurgical clipping of the aneurysm. With regard
110
Pediatr Neurosurg 2003;39:108–111
to extracranial arteries, there have been two reports of nondissecting aneurysms caused by myxoid degeneration in the cervical portion of the carotid artery [8, 9]. Four cases of intracranial dissecting aneurysms in adults have also been reported [10–13]. Therefore, intracranial aneurysms with myxoid degeneration are extremely rare. Although such pathological changes may be underreported because aneurysmal walls or domes are not subjected to
Fujita/Yanaka/Kamezaki/Noguchi/Nose
pathological analysis in most instances, to our knowledge, there have been no reports of intracranial myxoid degeneration of arterial walls with nondissecting aneurysm formation in a child, and this case is the first reported instance. The structural weakness of the arterial wall due to myxoid degeneration may have resulted in the formation of the nondissecting aneurysm. The etiology of myxoid degeneration is unknown, but is thought to be associated with the physiological process of aging, myxedema heart disease, idiopathic familial cardiomyopathy, Marfan’s syndrome [14], Ehlers-Danlos syndrome [15] and mediolytic arteriopathy [16]. In these diseases, myxoid degeneration of the wall may worsen the arterial damage, most commonly in the form of dissec-
tions. Some authors have found the presence of myxoid degeneration in apparently healthy arteries [10–12]. Our patient had no signs or history of the above-mentioned clinical entities. The cause and mechanism of the myxoid degeneration remains to be determined, and long-term follow-up seems essential in such cases. Myxoid generation has been thought to be associated with dissecting aneurysm formation. However, this case confirms that myxoid degeneration of the arterial walls may cause saccular-shaped aneurysms with eventual rupture, even in the absence of dissection. This case also provides new insight into the pathogenesis of aneurysmal formation.
References 1 Weir B, Macdonald RL: Intracranial aneurysms and subarachnoid hemorrhage: An overview; in Wilkins RH, Rengachary SS (eds): Neurosurgey, ed 2. New York, McGraw-Hill, 1996, pp 2191–2214. 2 Ferrante L, Fortuna A, Celli P, Santoro A, Fraioli B: Intracranial arterial aneurysms in early childhood. Surg Neurol 1988;29:39–56. 3 Kato T, Hattori H, Yorifuji T, Tashiro Y, Nakahata T: Intracranial aneurysms in EhlersDanlos syndrome type IV in early childhood. Pediatr Neurol 2001;25:336–339. 4 O’Boynick P, Green KD, Batnitzky S, Kepes JJ, Pietak R: Aneurysm of the left middle cerebral artery caused by myxoid degeneration of the vessel wall. Stroke 1994;25:2283–2286. 5 Lasjaunias P: Vascular Diseases in Neonates, Infants and Children. Berlin, Springer, 1997, pp 373–392. 6 Yanaka K, Meguro K, Narushima K, Takano S, Doi M, Nose T: Basal perforating artery aneurysm within the cavum septi pellucidi. Case report. J Neurosurg 1998;88:601–604.
Intracranial Myxoid Aneurysm
7 Yanaka K, Tsuboi K, Fujita K, Aoki K, Takeuchi S, Anno I, Nose T: Distal anterior choroidal artery aneurysm associated with an arteriovenous malformation. Intraoperative localization and treatment. Surg Neurol 2000;53:546–551. 8 Barnes WT, Jacoby JE: Aneurysm of the common carotid artery due to cystic medical necrosis treated by excision and graft. Ann Surg 1962;155:82–85. 9 Martinez Perez M, Pintos Diaz G, Varela Duran J: Aneurysm of the extracranial internal carotid caused by myxoid degeneration of the middle layer (in Spanish). Rev Quir Esp 1988; 15:112–114. 10 Hamada Y, Mannoji H, Kaneko Y: A ruptured dissecting aneurysm of the vertebral artery. Comparison of angiographic and histological findings. Neuroradiology 2001;43:375–378. 11 Ide Y, Fukushima T, Yamamoto M, Tomonaga M: Vertebral dissecting aneurysm associated with medial mucoid degeneration. Case report and review of literature (in Japanese). Neurol Med Chir (Tokyo) 1986;26:888–894.
12 Manz HJ, Luessenhop AJ: Dissecting aneurysm of intracranial vertebral artery. Case report and review of literature. J Neurol 1983; 230:25–35. 13 Thal DR, Schober R, Schlote W: Carotid artery dissection in a young adult. Cystic medial necrosis associated with an increased elastase content. Clin Neuropathol 1997;16:180–184. 14 Schievink WI, Parisi JE, Piepgras DG, Michels VV: Intracranial aneurysms in Marfan’s syndrome: An autopsy study. Neurosurgery 1997; 41:866–871. 15 Rubinstein MK, Cohen NH: Ehlers-Danlos syndrome associated with multiple intracranial aneurysms. Neurology 1964;14:125–132. 16 Leu HJ: Cerebrovascular accidents resulting from segmental mediolytic arteriopathy of the cerebral arteries in young adults. Cardiovasc Surg 1994;2:350–353.
Pediatr Neurosurg 2003;39:108–111
111
Copyright: S. Karger AG, Basel 2003. Reproduced with the permission of S. Karger AG, Basel. Further reproduction or distribution (electronic or otherwise) is prohibited without permission from the copyright holder.