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|LETTERS TO EDITOR
|Year : 2016 | Volume
| Issue : 4 | Page : 815-817
Spontaneous non-thrombotic regression of vein of Galen malformation in a child following shunt surgery
Pankaj Gupta, Manish Agrawal, Virendra Deo Sinha
Department of Neurosurgery, S.M.S. Medical College, Jaipur, Rajasthan, India
|Date of Web Publication||5-Jul-2016|
Department of Neurosurgery, S.M.S. Medical College, Jaipur, Rajasthan
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Gupta P, Agrawal M, Sinha VD. Spontaneous non-thrombotic regression of vein of Galen malformation in a child following shunt surgery. Neurol India 2016;64:815-7
A 3-month old male boy presented in the emergency department with macrocephaly, feeble cry, refusal to feed and recurrent vomiting. The child was born as full-term normal delivery. There was no history of unconsciousness, failure to thrive, seizures, breathlessness or recurrent chest infection. On examination, Glasgow coma score was 10 (E3V2M5), head circumference was 52.5 cm, anterior fontanel was tense and bulging, and sunset sign was present. Magnetic resonance imaging (MRI) of the brain [Figure 1] showed a large vein of Galen malformation [VOGM] (size = 32 mm × 24 mm) communicating with the straight sinus, without evidence of either stagnation of contrast material in the venous sac, or outflow restriction. Cerebral computed tomography (CT) angiography [Figure 1] showed the dilated vein of Galen communicating with the prominent straight sinus. Two-dimensional echocardiography was done immediately to rule out congestive cardiac failure. Right-sided ventriculo-peritoneal shunt was done after ensuring the low flow variety of VOGM and the child improved symptomatically. Since the child was perfectly alright after surgery, no active intervention was done for the VOGM and the child was kept under regular follow-up. At the age of 3.5 years, MRI and CT angiography [Figure 2] showed complete resolution of the VOGM without evidence of thrombosis. Adjoining venous sinuses looked normal. At the age of 4 years, the 4-vessel digital subtraction angiography [Figure 3] confirmed the complete resolution of VOGM. The child is healthy, growing normally and doing well at the age of 4 years.
|Figure 1: Magnetic resonance imaging of the brain (c) and cerebral computed tomography with angiography (a, b and d) showing a large vein of Galen malformation communicating with the straight sinus, without evidence of either stagnation of contrast material in the venous sacor outflow restriction|
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|Figure 2: Post-operative computed tomography (a), magnetic resonance imaging (b), magnetic resonance venography (c) and computed tomography angiography (d) at the age of 3.5 years showing the ventriculoperitoneal shunt in situ, with no evidence of venous dilatation. A small bunch of abnormal vessels isseen in the region of vein of Galen|
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|Figure 3: The four-vessel angiography done at the age of 4 years showing the complete regression of vein of Galen malformation; no abnormal bunch of vessels was present in the vein of Galenregion; and,all other sinuses are normal except for a slightly prominent occipital sinus|
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Based on the Google and Pubmed database search, a total of 22 cases of spontaneous thrombosis of VOGM have been reported, whereas only two cases of spontaneous disappearance of VOGM without evidence of thrombosis have been reported in literature till date. Kuroki et al., had reported the first case of spontaneous regression of VOGM in a child without evidence of spontaneous thrombosis. Tawk et al., reported the second case of spontaneous regression of VOGM associated with a posterior fossa cyst. In both the cases, no surgical intervention had been performed. Kuzeyli et al., had reported the spontaneous thrombosis of VOGM after shunt surgery. Therefore, this is the first case report of spontaneous non-thrombotic regression of VOGM following shunt surgery. The mechanism responsible for regression of VOGM in our case could have been restriction of the feeding artery rather than the outflow obstruction. This could have caused a diminution of pressure in the sac because restriction of venous outflow usually results in spontaneous thrombosis of VOGM, which was not present in our case.
Therefore, it can be concluded that in a patient of low flow-mural type VOGM, presenting with minimal symptoms (without evidence of cardiac failure), we can plan treatment taking into consideration the possibility of spontaneous resolution of VOGM with or without thrombosis. Ventriculo-peritoneal shunt is contraindicated in patients with a high flow choroidal type of VOGM. In patients with medically intractable congestive cardiac failure with VOGM, emergency endovascular embolization should be performed.
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Conflicts of interest
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| » References|| |
Kuroki K, Uozumi T, Arita K, Takechi A, Matsuura R, Fujidaka M. Spontaneous disappearance of an aneurysmal malformation of the vein of Galen. Neuroradiology 1995;37:244-6.
Tawk RG, Mohasseb G, Charbel FT, Debrun GM. Spontaneous disappearance of vein of Galen malformation and posterior fossa venous pouch. Acta Neurochir (Wien) 2002;144:1225-31.
Kuzeyli K, Cakir E, Karaarslan G, Ahmetoglu A, Peksoylu B, Yazar U, et al.
Spontaneous thrombosis of vein of Galen aneurysmal malformation after ventriculoperitoneal shunting. J Clin Neurosci 2004;11:439-42.
[Figure 1], [Figure 2], [Figure 3]