British Journal of Neurosurgery ISSN: 0268-8697 (Print) 1360-046X (Online) Journal homepage: http://www.tandfonline.com/loi/ibjn20 Spontaneous Angiographic Disappearance of an Intracranial Arteriovenous Malformation Paul A. Gibb, Richard W. Gullan & Nindi Sandhu To cite this article: Paul A. Gibb, Richard W. Gullan & Nindi Sandhu (1988) Spontaneous Angiographic Disappearance of an Intracranial Arteriovenous Malformation, British Journal of Neurosurgery, 2:4, 529-533, DOI: 10.3109/02688698809029610 To link to this article: http://dx.doi.org/10.3109/02688698809029610 Published online: 06 Jul 2009. Submit your article to this journal Article views: 2 View related articles Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=ibjn20 Download by: [University of Saskatchewan Library] Date: 01 April 2016, At: 04:33 British Journal of Neurosurgery (1988) 2,529-533 SHORT REPORT Spontaneous Angiographic Disappearance of an Intracranial Arteriovenous Malformation Downloaded by [University of Saskatchewan Library] at 04:33 01 April 2016 PAUL A. GIBB, RICHARD W. GULLAN & NINDI SANDHU South East Thames Regional Neurosurgical Unit, Brook General Hospital, Shooters Hill Road, Woolwich, SE18 4L W, United Kingdom Abstract A deep right hemisphere AVM was demonstrated by angiography in an 8-year-old girl soon after her presentation with a subarachnoid haemorrhage. Eight years later the AVM was no longer visible on angiography. During the interval she had been completely asymptomatic and had received no treatment. There was no residual neurological deficit or epilepsy. A similar case has previously been described and is discussed briefly along with other cases of complete AVM regression. Key words: Arteriovenous malformation. Case Report In 1979 a previously healthy 8-year-old girl was referred to this unit after waking from sleep screaming and complaining of severe headache, nausea and vomiting. On examination she was irritable and restless but would obey simple commands. She had marked meningism and bilateral extensor plantar responses. There were no other neurological signs. A lumbar puncture performed at the referring hospital revealed uniformly bloodstained CSF, and a C T scan in our unit showed extensive subarachnoid blood with no evidence of an intracerebral haematoma. Bilateral carotid angiography 18 h after the haemorrhage demonstrated a deep right hemisphere ganglionic AVM, supplied mainly by enlarged lenticulostriate arteries, with a small component from the distal anterior cerebral arteries. The malformation drained into the right basal vein, the superior saggital sinus and the vein of Galen. (Fig. la,b,c). She made a slow recovery complicated by transient mild hydrocephalus and was discharged 1 month after admission with no residual neurological deficit. In 1987, with the advent of greater availability of stereotactic radiosurgery in the United Kingdom, she was readmitted for repeat angiography to reassess the malformation. She was now 15 years old and had been completely well since her previous admission with no headaches,convulsions or neurological deficit. She smoked 10 cigarettes a day and was not taking the oral contraceptive pill. Routine haematological investigations were normal. T o our suprise repeat angiography failed to demonstrate the AVM. (Fig. 2a,b,c). Discussion Regression of arteriovenous malformations is an extremely rare phenomenon. We have identified 27 cases of AVM regression which 529 Downloaded by [University of Saskatchewan Library] at 04:33 01 April 2016 530 Paul A. Gibb, Richard W.Gullan & Nindi Sandhu FIG. l.a,b,c. AP, lateral and oblique projections of right internal carotid angiogram (1979), demonstrating a deep ganglionic AVM, supplied mainly by enlarged lenticulostriate arteries. There is a small component from the distal anterior cerebral arteries. The malformation shunts early through hypertrophied and tortous vessels into the large right basal vein, superior saggital sinus and vein of Galen. are reviewed in this article. In five cases incomplete resolution o c c ~ r r e d ~and - ~ , in four other cases there was incomplete angiographic documentation (i.e. thrombosed AVM discovered at o ~ e r a t i o n ) ~ Table - ~ . I summarises the remaining 18 cases in which complete regression of an AVM has been demonstrated angiographically. The patients were grouped according to events occurring between the positive and negative angiograms that could possibly be related to disappearance of the AVM. Six patients had undergone surgery at presentation including partial excision of the AVM'OJ', clot aspiration12, ~ h u n t i n g ' ~ and J ~ , aspiration of an associated arachnoid cystI5. In two patients there was clinical and radiographic evidence of rebleeding prior to re-angiography16. Five patients developed new or increasing neurological deficit or epilepsy prompting i n v e s t i g a t i ~ n ~ . ~ Two J ~ J ~ .patients underwent re-angiography after episodes of severe headache (associated with unconsciousness in one) without clinical or radiographic evidence of rebleedinglEJ9.In the remaining three cases reangiography was not precipitated by any acute event, and there had been no deterioration in the clinical condition of the patients between Presentation Sex GroupY Deficit F 2/12 L Parietal R Parietal R Parietal Haemorrhage F 54 F Haemorrhage L Parietal + * “Symptom complex group at presentation/representation after Crawford*’: 1 Haemorrhage*epilepsy 2 neurological deficit 2 Epilepsy neurological deficit bAngiographic appearance. 16 3 Neurological deficit 4 Other - 22 - Deep Superficial 48 - Superficial Aneurysm + R MCA Superficial Aneurysm 59 - R Parietal 37 - Superficial Group 6: No change-Asymptomatic Patient 15 M Haemorrhage R Parietal 11 62 - Superficial 40 - Deep L Parietal L Parietal (previous partial excision) R Parietal Group 5: No change-Persistent Neurological Deficit or Epilepsy 41 M Epilepsy L Parietal L MCA Group 4: Severe Headache 34 F Haemorrhage (recurreutj 46 M Other Haemorrhage F 35 38 59 18 - 65 55 Asymptomatic Deficit Epilepsy Other Other Epilepsy Epilepsy Deficit Deficit Deficit Haemorrhage Haemorrhage Epilepsy Complete C T normal Complete CT enhancing lesion Complete C T low density Complete C T Aneurysm Complete Complete Complete Complete C T Astrocytoma Complete C T low density Complete Complete Complete C T low density Complete C T subdural 16 20 16 19 18 17 2 8 10 2 16 16 15 13 14 Deficit Epilepsy 54 1 Other 12 Complete Complete Complete Deficit 47 3 11 Other 42 10 Reference Complete Discovered thrombosed Complete Resolutionb Deficit 47 Representation Age Group - - Partial excision Aspiration of clot Shunt VP Shunt+ Evacuation of Subdural Evacuation of cyst Partial excision Treatment - - Deep Deep Deep Superficial ? Deep Deep Deep + Arachnoid cyst Deep L Frontal L P Fossa Deep Superficial Depthb L Parietal L Frontal R Parietal AVM Site Group 3: New or increasing Neurologrcal Deficit or Epilepsy R Parietal 34 F Deficit L Temporal 54 F Deficit Deficit L Temporal F 15 Group 2: Second Haemonhage 56 M Deficit Epilepsy F 40 Other Other 33 4/12 Deficit M M F 40 Haemorrhage F 33 Group 1: Surgical Intervention 42 M Haemorrhage Age TABLE I. Case reports of complete AVM disappearance, grouped as described in the text according to events occurring between presentation and reangiography Downloaded by [University of Saskatchewan Library] at 04:33 01 April 2016 Downloaded by [University of Saskatchewan Library] at 04:33 01 April 2016 532 Paul A. Gibb, Richard W. Gullan & Nindi Sandhu FIG. 2.a,b,c. AP, lateral and oblique projections of the right internal carotid angiogram (1987), demonstrating normal intracranial circulation. The previously demonstrated AVM was not opacified on the early or late phases of the study. angiograms. Two of these patients had suffered from persistent epilepsy and hemiparesis following presentation and angiography was performed prior to attempting surgical treatment16,20.The remaining patient16, like our own, was unique in making a complete recovery from spontaneous haemorrhage, suffering neither neurological deficit nor epilepsy. In both cases re-angiography was performed electively prior to treatment in an apparently normal patient. Pasqualin16 described the case of a 15-yearold boy presenting with sudden unconsciousness and convulsions in whom a CT scan revealed a small right parietal haematoma, and angiography demonstrated a small parietal AVM fed by the calloso-marginal artery. One year later a preoperative angiogram was normal, as was constrast enhanced CT. We did not feel that repeat scanning was indicated in our patient particularly in view of her age and parental anxiety over radiation exposure. However it is of interest that of eight patients in whom CT scans have been performed following spontaneous angiographic disappearance of their AVM, only one was normal, and this was in an asymptomatic patient without epilepsy or deficitI6. A variety Downloaded by [University of Saskatchewan Library] at 04:33 01 April 2016 Disappearance of an Arteriovenous Malformation 533 Spontaneous regression of intracranial arteriovenous of abnormalities were found, the most common malformations. J Neurosurg 1982; 57: 818-22. being an area of low attenuation related to the 3 Sasaki U, Itagaki T, Shimizu H, Takahashi M. Almost site of the AVM. In one case an Astrocytoma complete regression of cerebral arteriovenous malformation. Neurol Surg (Tokyo) 1976; 4:175-81. was discovered*. 4 Sukoff M, Barth B, Moran T. Spontaneous occlusion The case presented suggests that AVM's are of a massive arteriovenous malformation. Neurotruly capable of spontaneous resolution, and radiology 1972; 4121-3. 5 Svien HJ, Perserico L. Regression in size of arteriovarious mechanisms explaining the phenomevenous anomaly. J Neurosurg 1960; 17:493-6. non of AVM regression have been proposed. In 6 Castaigne P, Buge A, Pertuiset B, Laplane D, Corbin J. those in whom a surgical procedure has been Angiome thrombose, radiologiquement exclu. Bull Mem SOCMed Hop Paris 1961; 77:923-30. undertaken it is possible that alterations in 7 Dyck P. Spontaneous thrombosis of an arteriovenous local haemodynamics contributed to the AVM malformation. Neurosurgery 1977; 1,3:287-90. thrombosis and subsequent regre~sion'~. Com8 Eisenman JI, Alekoumbides A, Pribram H. Spontaneous thrombosis of vascular malformations of the brain. pression by intracerebral clot and associated Acta Radio1 1972; 13:77-85. oedema*, distortion after recurrent episodes of 9 Norlen G. Arteriorvenous aneurysms of the brain: bleeding", acute4 and gradual t h r o m b o s i ~ ~ ~ ~Report of ten cases of total removal of the lesion. J Neurosurg 1949; 6:475-94. and embolisation' have also been proposed as mechanisms for spontaneous regression. In 10 Fischer G, Brunon J, Thierry A, Aimard G, Rochet M, Mansuy L. Thrombose au cours de l'evolution des Pasqualin's case16 and our own thrombosis aneurysms arterio-veineux cerebraux. J Med Lyon 1969; 50~613-25. probably occurred gradually, precipitated initially by local mass effect and spasm and 11 Lakke JPWF. Regression of an arteriovenous malformation of the brain. J Neurol Sci 1970; 11:489-96. latterly by haemodynamic disturbances asso- 12 Levine J, Misko JC, Seres JL, Glenn Snodgrass R. Spontaneous angiographic disappearance of a cerebral ciated with gliosis. It is possible that the arteriovenous malformation. Arch Neurol 1973; transient hydrocephalus occuring in our 28:195-6. patient was a contributory factor. In both cases 13 Hook 0, Johanson C. Intracranial arteriovenous aneurysms. Arch Neurol Psychiat 1958; 80:39-54. spontaneous regression occurred in a healthy 14 Mabe H, Furuse M. Spontaneous disappearance of a patient with no deleterious effects. Acknowledgements We would like to thank Mr J. R. Bartlett for permission to report this case, and Mr N. Norman for his assistance with the literature search. Address for correspondence: Mr R. W. Gullan, South East Thames Regional Neurosurgical Unit, Brook General Hospital, Shooters Hill Road, Woolwich, SE18 4LW, U.K. References 1 Kushner J, Alexander E. Partial spontaneous regressive arteriovenous malformation. J. Neurosurg 1970; 32~360-6. 2 Omojola MF, Fox AJ, Vinuela FV, Drake CG. cerebral arteriovenous malformation in infancy. J Neurosurg 1977; 46:811-5. 15 Pascual-Castroviejo I, Pascual-Pascual J, Blazquez MG, Lopez-Martin V. Spontaneous occlusion of an intracranial arteriovenous malformation. Child's Brain 1977; 3:169-79. 16 Pasqualin A, Vivenza C, Rosta R, Scienza R, Da Pian R, Colangeli M. Spontaneous disappearance of intracranial arteriovenous malformations. Acta Neurochir 1985; 76~50-7. 17 Pecker J, Sabouraud 0, Javalet A. Les thromboses spontanees des aneurysmes arterio-veineux cerebraux. Neurochirurgie 1961; 7:155-7. 18 Conforti P. Spotaneous disappearance of cerebral arteriovenous angioma. J Neurosurg 1971; 34:432-4. 19 Nehls DG, Pittman HW. Spontaneous regression of arteriovenous malformations. Neurosurgery 1982; 11,6:776-80. 20 Sartor K. Spontaneous closure of cerebral arteriovenous malformation demonstrated by angiography and computed tomography. Neuroradiology 1978; 15:95-8. 21 Crawford PM, West CR, Chadwick DW, Shaw MDM. Arteriovenous malformations of the brain: natural history in unoperated patients. J Neurol Neurosurg Psych 1986; 49~1-10.