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Cobbs syndrome: A case of spinal arteriovenous malformation treated with endovascular embolization
European Journal of Radiology Extra 79 (2011) e15–e17
Contents lists available at ScienceDirect
European Journal of Radiology Extra journal homepage: intl.elsevierhealth.com/journals/ejrex
Cobbs syndrome: A case of spinal arteriovenous malformation treated with endovascular embolization Elvira Jiménez Gómez ∗ , Rafael Oteros Fernández, Fernando Delgado Acosta Endovascular Interventional Neuroradiology, Department of Radiology, University, Hospital Reina Sofía, Av/Menendez Pidal s/n, ZC 14004, Cordoba, Spain
a r t i c l e
i n f o
Article history: Received 14 January 2011 Accepted 5 April 2011
Keywords: Cobb syndrome Cutaneomeningospinal angiomatosis Spinal arteriovenous malformation Spinal angioma
a b s t r a c t Cobb syndrome is a rare illness that combines vascular skin nevus and spinal vascular malformation at the same metamere. We report the case of a 34-year old man that presented a light low back pain, a slowly progressive paraparesis and abnormal urinary sphincter functioning. MR imaging of the thoracic spine depicted an intramedular arteriovenous malformation. Spinal angiography confirmed these findings. The arteriovenous malformation also affected the chestwall bones and muscles with the same metameric distribution as the intramedular lesion. Due to these findings Cobb syndrome was suspected and in a second physical examination a skin angioma was noted on the patient’s back. He was treated with corticosteroids and endovascular coiling and his symptoms rapidly improved. Although pathophysiology of this syndrome is poorly understood and the correct management remains unknown, endovascular treatment has become the first choice for spinal arteriovenous malformations. We believe that combined treatment with corticosteroids and endovascular embolization is an effective procedure to alleviate symptoms and to minimize morbidity. © 2011 Elsevier Ireland Ltd. All rights reserved.
Cobb syndrome is a rare illness that combines vascular skin nevus and spinal vascular malformation in the same metamere. Also known as cutaneomeningospinal angiomatosis [1] this syndrome did not receive recognition until 1915 when Stanley Cobbs presented the case of an 8-year-old boy who developed flaccid paralysis [2]. Mean age at diagnosis is 36.4 years, with a peak between the third and fifth decades. Lesions are most frequently localized at the cervical and thoracic spinal cord [3]. We report the case of a 34-year old man with Cobb syndrome that presented a spinal arteriovenous malformation that was successfully treated by endovascular embolization.
1. Case report A 34-year old man presented a light low back pain, a slowly progressive paraparesis and abnormal urinary sphincter functioning. There was no significant past or family history. Initially, a lumbar disc herniation was suspected and the patient began an empiric treatment with no improvement.
∗ Corresponding author. Tel.: +34 957 010 292; fax: +34 957 204 763. E-mail addresses: [email protected], [email protected] (E.J. Gómez), [email protected] (R.O. Fernández), [email protected] (F.D. Acosta). 1571-4675/$ – see front matter © 2011 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ejrex.2011.04.003
On physical examination the patient demonstrated grade 2/5 motor strength in his lower extremities bilaterally with left predominance. His gait was unsteady. Plantar reflexes were extensor. He underwent MR imaging of the thoracic spine which demonstrated an intramedular mass from C6 to T5 with multiple worm-shaped lesions corresponding to an intramedular arteriovenous malformation (Fig. 1). There was also a 6 mm nodule lesion between T1 and T2 corresponding to an aneurismatic venous dilation in the malformation. Both were enhanced after contrast administration. Cord edema and signal changes were also seen at this level. Spinal angiography confirmed these findings and showed a radicular artery aneurysm that had not been seen in MR (Figs. 2A and 3). The arteriovenous malformation affected the chestwall bones and muscles with the same metameric distribution as the intramedular lesion. Due to these findings Cobb syndrome was suspected. In a second physical examination the patient was noted to have a port wine stain over his dorsal back at the same metameric location as the intramedular, muscular and bone vascular malformation (Fig. 4). A multidisciplinary approach to the patient’s care was undertaken with the involvement of neuroradiologists, interventional neuroradiologists and neurosurgeons. He was first treated with corticosteroids and endovascular coiling in the intramedular vascular malformation with epidural drainage. After this treatment his symptoms improved. He reported no pain and could walk normally, however his urinary sphincter abnormalities continued. Twenty-two weeks later, he presented
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E.J. Gómez et al. / European Journal of Radiology Extra 79 (2011) e15–e17
Fig. 1. Sagital T2 weighted MR image revealing an intramedular aretriovenous malformation. There are also vertebral angiomas in the same metamere as the medular arteriovenous malformation.
recurrent symptoms so he underwent spinal angiography and subsequent partial embolization of the vascular angioma. The largest fistulous component of the vascular malformation was closed (Fig. 2B). These procedures were performed while the patient was under general anesthesia, with sistemic heparinization and via transfemoral route. He rapidly noted symptoms improvement. After treatment the patient’s symptoms improved, he had no back pain and he could walk normally although urinary sphincter problems continued. Endovascular treatment of the angioma will be treated as symptoms progress. Radicular artery aneurism management remains unsolved.
Fig. 3. Selective spinal angiogram. The catheter is located into a radicular branch where there’s an arterial aneurysm (arrow).
2. Discussion Cobb syndrome is a rare nonfamilial condition coupling skin nevus and spinal arteriovenous malformations in the same metamere [4]. The embryologic origin of the blood supply to the vertebral and spinal cord derives from the segmental dorsal arteries. This finding
Fig. 2. (A) Selective spinal angiogram denoting a portion of the arteriovenous malformation. There is an aneurismatic venous dilatation (arrow). Note the anterior spinal artery (arrowhead). (B) Arteriovenous malformation after endovascular treatment. Embolic material can be seen (arrow).
E.J. Gómez et al. / European Journal of Radiology Extra 79 (2011) e15–e17
e17
The reduction of venous hypertension and the decreased of steal phenomenon resulting from embolization might have contributed to the clinical improvement in this patient. In all cases clinical and imaging follow up is essential to analyze the efficacy of this treatment as there is the possibility of development or growth of new or preexisting lesions [6,11]. If symptoms recur during the follow up, new embolizations can be performed. Corticosteroids therapy before intramedular embolization may reduce extension and number of spinal arteriovenous embolization procedures [12,13]. We present a case of adult Cobb syndrome successfully treated with corticosteroids and endovascular embolization of the intramedular arteriovenous malformation. However, long term follow up and outcome remains unknown, we believe that combined treatment with corticosteroids and endovascular embolization of spinal arteriovenous malformations in Cobbs syndrome is an effective procedure to alleviate symptoms and to minimize morbidity. This treatment also permits reembolization each time symptoms recur. Fig. 4. Photograph of the patient’s cutaneous angioma located on the back in the same metamere as the intramedular, bone, and muscular arteriovenous malformation.
corresponds to the metameric origin of the arteriovenous malformation vessels that create the Cobb syndrome angiomas [5]. Many theories exist regarding the etiology of the neurologic manifestations of Cobb syndrome, including blood steal syndrome (that produces cord ischemia), cord compression and venous hypertension [6,7]. There is a slight male predominance associated with this syndrome [1]. The most frequent angioma location is thoracic spine. Most symptoms in adults appear as a gradual progressive neurological deficit, as in our case. Pathophysiology of this syndrome is poorly understood so the correct management remains unknown. Angiography augments understanding of the angioarchitecture because spinal angiomas in Cobb’s syndrome have a distinct blood supply from the normal spinal cord [6,8]. The range of presentation in symptomatic Cobb syndrome is diverse and the course is unpredictable. Nowadays with the development of endovascular techniques, endovascular therapy has become the first choice for spinal arteriovenous malformations treatment [6,7,9,10]. As previously mentioned, many theories exist about the etiology of the neurologic manifestations of this syndrome. In the present case a preprocedural MR revealed an important spinal cord edema too large to be explained only by cord compression by the angioma. The spinal cord edema was probably secondary to venous estasis which results in an anterior spinal artery pressure increase and an abnormal tissue drainage.
References [1] Johnson WD, Petrie MMJ. Variety of spinal vascular pathology seen in adult Cobb syndrome. Neurosurg Spine 2009;10(May (5)):430–5. [2] Cobb S. Haemangioma of the spinal cord: associated with skin naevi of the same metamere. Ann Surg 1915;62(December (6)):641–9. [3] Zevgaridis D, Medele RJ, Hamburger C, et al. Cavernous haemangioma of the spinal cord. A review of 117 cases. Acta Neurochir (Wien) 1999;141(237):e45. [4] Shim JH, Lee DW, Cho BK. A case of Cobb syndrome associated with lymphangioma circumscriptum. Dermatology 1996;193(1):45–7. [5] Rodesch G, Hurth M, Alvarez H, et al. Angio-architecture of spinal cord arteriovenous shunts at presentation. Clinical correlations in adults and children. The Bicêtre experience on 155 consecutive patients seen between 1981–1999. Acta Neurochir (Wien) 2004;146(March (3)):217–26 [discussion 226–7. Epub 2004 Feb 23]. [6] Bao YH, Ling F. Classification and therapeutic modalities of spinal vascular malformations in 80 patients. Neurosurgery 1997;40(January (1)):75–81. [7] Halbach VV, Higashida RT, Dowd CF, et al. Treatment of giant intradural (perimedullary) arteriovenous fistulas. Neurosurgery 1993;33(December (6)):972–9 [discussion 979–80]. [8] Doppman JL, Wirth Jr FP, Di Chiro G, et al. Value of cutaneous angiomas in the arteriographic localization of spinal-cord arteriovenous malformations. N Engl J Med 1969;281(December (26)):1440–4. [9] Soeda A, Sakai N, Iihara K, et al. Cobb syndrome in an infant: treatment with endovascular embolization and corticosteroid therapy: case report. Neurosurgery 2003;52(March (3)):711–5. [10] Clark MT, Brooks EL, Chong W, et al. Cobb syndrome: a case report and systematic review of the literature. Pediatr Neurol 2008;39(December (6)):423–5. [11] Miyatake S, Kikuchi H, Koide T, et al. Cobb’s syndrome and its treatment with embolization. J Neurosurg 1990;72(March (3)):497–9. [12] Akyüz C, Yaris¸ N, Kutluk MT, et al. Management of cutaneous hemangiomas: a retrospective analysis of 1109 cases and comparison of conventional dose prednisolone with high-dose methylprednisolone therapy. Pediatr Hematol Oncol 2001;18(January–February (1)):47–55. [13] Weber TR, Connors RH, Tracy Jr TF, et al. Complex hemangiomas of infants and children. Individualized management in 22 cases. Arch Surg 1990;125(August (8)):1017–20 [discussion 1020].
Contents lists available at ScienceDirect
European Journal of Radiology Extra journal homepage: intl.elsevierhealth.com/journals/ejrex
Cobbs syndrome: A case of spinal arteriovenous malformation treated with endovascular embolization Elvira Jiménez Gómez ∗ , Rafael Oteros Fernández, Fernando Delgado Acosta Endovascular Interventional Neuroradiology, Department of Radiology, University, Hospital Reina Sofía, Av/Menendez Pidal s/n, ZC 14004, Cordoba, Spain
a r t i c l e
i n f o
Article history: Received 14 January 2011 Accepted 5 April 2011
Keywords: Cobb syndrome Cutaneomeningospinal angiomatosis Spinal arteriovenous malformation Spinal angioma
a b s t r a c t Cobb syndrome is a rare illness that combines vascular skin nevus and spinal vascular malformation at the same metamere. We report the case of a 34-year old man that presented a light low back pain, a slowly progressive paraparesis and abnormal urinary sphincter functioning. MR imaging of the thoracic spine depicted an intramedular arteriovenous malformation. Spinal angiography confirmed these findings. The arteriovenous malformation also affected the chestwall bones and muscles with the same metameric distribution as the intramedular lesion. Due to these findings Cobb syndrome was suspected and in a second physical examination a skin angioma was noted on the patient’s back. He was treated with corticosteroids and endovascular coiling and his symptoms rapidly improved. Although pathophysiology of this syndrome is poorly understood and the correct management remains unknown, endovascular treatment has become the first choice for spinal arteriovenous malformations. We believe that combined treatment with corticosteroids and endovascular embolization is an effective procedure to alleviate symptoms and to minimize morbidity. © 2011 Elsevier Ireland Ltd. All rights reserved.
Cobb syndrome is a rare illness that combines vascular skin nevus and spinal vascular malformation in the same metamere. Also known as cutaneomeningospinal angiomatosis [1] this syndrome did not receive recognition until 1915 when Stanley Cobbs presented the case of an 8-year-old boy who developed flaccid paralysis [2]. Mean age at diagnosis is 36.4 years, with a peak between the third and fifth decades. Lesions are most frequently localized at the cervical and thoracic spinal cord [3]. We report the case of a 34-year old man with Cobb syndrome that presented a spinal arteriovenous malformation that was successfully treated by endovascular embolization.
1. Case report A 34-year old man presented a light low back pain, a slowly progressive paraparesis and abnormal urinary sphincter functioning. There was no significant past or family history. Initially, a lumbar disc herniation was suspected and the patient began an empiric treatment with no improvement.
∗ Corresponding author. Tel.: +34 957 010 292; fax: +34 957 204 763. E-mail addresses: [email protected], [email protected] (E.J. Gómez), [email protected] (R.O. Fernández), [email protected] (F.D. Acosta). 1571-4675/$ – see front matter © 2011 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.ejrex.2011.04.003
On physical examination the patient demonstrated grade 2/5 motor strength in his lower extremities bilaterally with left predominance. His gait was unsteady. Plantar reflexes were extensor. He underwent MR imaging of the thoracic spine which demonstrated an intramedular mass from C6 to T5 with multiple worm-shaped lesions corresponding to an intramedular arteriovenous malformation (Fig. 1). There was also a 6 mm nodule lesion between T1 and T2 corresponding to an aneurismatic venous dilation in the malformation. Both were enhanced after contrast administration. Cord edema and signal changes were also seen at this level. Spinal angiography confirmed these findings and showed a radicular artery aneurysm that had not been seen in MR (Figs. 2A and 3). The arteriovenous malformation affected the chestwall bones and muscles with the same metameric distribution as the intramedular lesion. Due to these findings Cobb syndrome was suspected. In a second physical examination the patient was noted to have a port wine stain over his dorsal back at the same metameric location as the intramedular, muscular and bone vascular malformation (Fig. 4). A multidisciplinary approach to the patient’s care was undertaken with the involvement of neuroradiologists, interventional neuroradiologists and neurosurgeons. He was first treated with corticosteroids and endovascular coiling in the intramedular vascular malformation with epidural drainage. After this treatment his symptoms improved. He reported no pain and could walk normally, however his urinary sphincter abnormalities continued. Twenty-two weeks later, he presented
e16
E.J. Gómez et al. / European Journal of Radiology Extra 79 (2011) e15–e17
Fig. 1. Sagital T2 weighted MR image revealing an intramedular aretriovenous malformation. There are also vertebral angiomas in the same metamere as the medular arteriovenous malformation.
recurrent symptoms so he underwent spinal angiography and subsequent partial embolization of the vascular angioma. The largest fistulous component of the vascular malformation was closed (Fig. 2B). These procedures were performed while the patient was under general anesthesia, with sistemic heparinization and via transfemoral route. He rapidly noted symptoms improvement. After treatment the patient’s symptoms improved, he had no back pain and he could walk normally although urinary sphincter problems continued. Endovascular treatment of the angioma will be treated as symptoms progress. Radicular artery aneurism management remains unsolved.
Fig. 3. Selective spinal angiogram. The catheter is located into a radicular branch where there’s an arterial aneurysm (arrow).
2. Discussion Cobb syndrome is a rare nonfamilial condition coupling skin nevus and spinal arteriovenous malformations in the same metamere [4]. The embryologic origin of the blood supply to the vertebral and spinal cord derives from the segmental dorsal arteries. This finding
Fig. 2. (A) Selective spinal angiogram denoting a portion of the arteriovenous malformation. There is an aneurismatic venous dilatation (arrow). Note the anterior spinal artery (arrowhead). (B) Arteriovenous malformation after endovascular treatment. Embolic material can be seen (arrow).
E.J. Gómez et al. / European Journal of Radiology Extra 79 (2011) e15–e17
e17
The reduction of venous hypertension and the decreased of steal phenomenon resulting from embolization might have contributed to the clinical improvement in this patient. In all cases clinical and imaging follow up is essential to analyze the efficacy of this treatment as there is the possibility of development or growth of new or preexisting lesions [6,11]. If symptoms recur during the follow up, new embolizations can be performed. Corticosteroids therapy before intramedular embolization may reduce extension and number of spinal arteriovenous embolization procedures [12,13]. We present a case of adult Cobb syndrome successfully treated with corticosteroids and endovascular embolization of the intramedular arteriovenous malformation. However, long term follow up and outcome remains unknown, we believe that combined treatment with corticosteroids and endovascular embolization of spinal arteriovenous malformations in Cobbs syndrome is an effective procedure to alleviate symptoms and to minimize morbidity. This treatment also permits reembolization each time symptoms recur. Fig. 4. Photograph of the patient’s cutaneous angioma located on the back in the same metamere as the intramedular, bone, and muscular arteriovenous malformation.
corresponds to the metameric origin of the arteriovenous malformation vessels that create the Cobb syndrome angiomas [5]. Many theories exist regarding the etiology of the neurologic manifestations of Cobb syndrome, including blood steal syndrome (that produces cord ischemia), cord compression and venous hypertension [6,7]. There is a slight male predominance associated with this syndrome [1]. The most frequent angioma location is thoracic spine. Most symptoms in adults appear as a gradual progressive neurological deficit, as in our case. Pathophysiology of this syndrome is poorly understood so the correct management remains unknown. Angiography augments understanding of the angioarchitecture because spinal angiomas in Cobb’s syndrome have a distinct blood supply from the normal spinal cord [6,8]. The range of presentation in symptomatic Cobb syndrome is diverse and the course is unpredictable. Nowadays with the development of endovascular techniques, endovascular therapy has become the first choice for spinal arteriovenous malformations treatment [6,7,9,10]. As previously mentioned, many theories exist about the etiology of the neurologic manifestations of this syndrome. In the present case a preprocedural MR revealed an important spinal cord edema too large to be explained only by cord compression by the angioma. The spinal cord edema was probably secondary to venous estasis which results in an anterior spinal artery pressure increase and an abnormal tissue drainage.
References [1] Johnson WD, Petrie MMJ. Variety of spinal vascular pathology seen in adult Cobb syndrome. Neurosurg Spine 2009;10(May (5)):430–5. [2] Cobb S. Haemangioma of the spinal cord: associated with skin naevi of the same metamere. Ann Surg 1915;62(December (6)):641–9. [3] Zevgaridis D, Medele RJ, Hamburger C, et al. Cavernous haemangioma of the spinal cord. A review of 117 cases. Acta Neurochir (Wien) 1999;141(237):e45. [4] Shim JH, Lee DW, Cho BK. A case of Cobb syndrome associated with lymphangioma circumscriptum. Dermatology 1996;193(1):45–7. [5] Rodesch G, Hurth M, Alvarez H, et al. Angio-architecture of spinal cord arteriovenous shunts at presentation. Clinical correlations in adults and children. The Bicêtre experience on 155 consecutive patients seen between 1981–1999. Acta Neurochir (Wien) 2004;146(March (3)):217–26 [discussion 226–7. Epub 2004 Feb 23]. [6] Bao YH, Ling F. Classification and therapeutic modalities of spinal vascular malformations in 80 patients. Neurosurgery 1997;40(January (1)):75–81. [7] Halbach VV, Higashida RT, Dowd CF, et al. Treatment of giant intradural (perimedullary) arteriovenous fistulas. Neurosurgery 1993;33(December (6)):972–9 [discussion 979–80]. [8] Doppman JL, Wirth Jr FP, Di Chiro G, et al. Value of cutaneous angiomas in the arteriographic localization of spinal-cord arteriovenous malformations. N Engl J Med 1969;281(December (26)):1440–4. [9] Soeda A, Sakai N, Iihara K, et al. Cobb syndrome in an infant: treatment with endovascular embolization and corticosteroid therapy: case report. Neurosurgery 2003;52(March (3)):711–5. [10] Clark MT, Brooks EL, Chong W, et al. Cobb syndrome: a case report and systematic review of the literature. Pediatr Neurol 2008;39(December (6)):423–5. [11] Miyatake S, Kikuchi H, Koide T, et al. Cobb’s syndrome and its treatment with embolization. J Neurosurg 1990;72(March (3)):497–9. [12] Akyüz C, Yaris¸ N, Kutluk MT, et al. Management of cutaneous hemangiomas: a retrospective analysis of 1109 cases and comparison of conventional dose prednisolone with high-dose methylprednisolone therapy. Pediatr Hematol Oncol 2001;18(January–February (1)):47–55. [13] Weber TR, Connors RH, Tracy Jr TF, et al. Complex hemangiomas of infants and children. Individualized management in 22 cases. Arch Surg 1990;125(August (8)):1017–20 [discussion 1020].