The Open Neuroimaging Journal




ISSN: 1874-4400 ― Volume 13, 2019
CASE REPORT

Case Report of an Obstructive Hydrocephalus Caused by an Unruptured Mesencephalic Arteriovenous Malformation in a Boy and a Review of Literature



Furkan Diren1, Serra Sencer2, Tayfun Hakan1, 3, *
1 International Kolon Hospital, Neurosurgery Clinic, İstanbul, Turkey
2 İstanbul University, İstanbul Medical School, Neuroradiology Department, İstanbul, Turkey
3 Okan University, Vocational School of Health Services, İstanbul, Turkey

Abstract

Objective:

Arteriovenous malformation (AVM) is the most common form of intracranial vascular malformations in adults. Intracranial pediatric AVMs are rare. AVM located in the vicinity of the brain stem in children are even more rare.

Case report:

This study reports a rare case of acute obstructive hydrocephalus following aqueductal stenosis caused by an unruptured grade IV perimesencephalic arteriovenous malformation. An 11-year-old boy admitted to the hospital with progressive headache, nausea and vomiting throughout a month. A Computerized Tomography (CT) showed an obstructive hydrocephaly. A Magnetic Resonance (MR) imaging revealed a mesencephalic AVM compressing the aqueduct. The patient deteriorated in hours and an emergency ventriculoperitoneal shunting was performed. He did well in the early postoperative period. AVM examined with Digital Subtraction Angiography (DSA) in detail for maintaining the definitive treatment by means of endovascular embolization, microsurgery and stereotactic radiosurgery; but the patient was lost to follow up.

Conclusion:

A Pubmed search revealed 34 cases of hydrocephalus caused by an unruptured AVM in the literature, and only four cases were less than 18 years old with unruptured AVM locating in brain stem or posterior fossa. Although focal neurologic deficit, seizure and headache are the most common symptoms, acute neurologic deterioration due to hydrocephalus may be the presenting symptom in these cases. The decrease in intracranial pressure by changing the flow of cerebrospinal fluid (CSF) via an emergency ventriculoperitoneal (VP) shunting or Endoscopic Third Ventriculostomy (ETV) can be a lifesaving procedure that gives a chance for further treatment modalities.

Keywords: Arteriovenous malformation, Aqueduct, Digital subtraction angiography, Hydrocephalus, Mesencephalon, Ventriculoperitoneal shunt.


Article Information


Identifiers and Pagination:

Year: 2018
Volume: 12
First Page: 10
Last Page: 15
Publisher Id: TONIJ-12-10
DOI: 10.2174/1874440001812010010

Article History:

Received Date: 24/10/2017
Revision Received Date: 30/01/2018
Acceptance Date: 01/02/2018
Electronic publication date: 21/02/2018
Collection year: 2018

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© 2018 Diren et al.

open-access license: This is an open access article distributed under the terms of the Creative Commons Attribution 4.0 International Public License (CC-BY 4.0), a copy of which is available at: https://creativecommons.org/licenses/by/4.0/legalcode. This license permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.


This case was partly presented as poster in 30th Scientific Congress of the Turkish Neurosurgical Society
* Address correspondence to this author at the Okan University, Vocational School of Health Services, Caddebostan Mahallesi Ada Sokak No:2/3, 34728 Kadikoy/Istanbul, Turkey; GSM: +90 (532) 3243284; E-mail: tayfunhakan@yahoo.com





1. INTRODUCTION

AVM is the most common form of intracranial vascular malformations in adults. Intracranial pediatric AVMs are rare [1Darsaut TE, Guzman R, Marcellus ML, et al. Management of pediatric intracranial arteriovenous malformations: Experience with multimodality therapy. Neurosurgery 2011; 69(3): 540-56.[http://dx.doi.org/10.1227/NEU.0b013e3182181c00] , 2Kiriş T, Sencer A, Sahinbaş M, et al. Surgical results in pediatric Spetzler-Martin grades I-III intracranial arteriovenous malformations. Childs Nerv Syst 2005; 21(1): 69-76.[http://dx.doi.org/10.1007/s00381-004-1025-0] ]. AVM located in the vicinity of the brain stem in children are even more rare [1Darsaut TE, Guzman R, Marcellus ML, et al. Management of pediatric intracranial arteriovenous malformations: Experience with multimodality therapy. Neurosurgery 2011; 69(3): 540-56.[http://dx.doi.org/10.1227/NEU.0b013e3182181c00] , 3Di Rocco C, Tamburrini G, Rollo M. Cerebral arteriovenous malformations in children. Acta Neurochir (Wien) 2000; 142(2): 145-58.[http://dx.doi.org/10.1007/s007010050017] ]. Hemorrhage is the most common presentation of intracranial AVMs in children [3Di Rocco C, Tamburrini G, Rollo M. Cerebral arteriovenous malformations in children. Acta Neurochir (Wien) 2000; 142(2): 145-58.[http://dx.doi.org/10.1007/s007010050017] ]. Focal neurological deficit, seizure and headache are the most common symptoms for unruptured AVMs in children [4Ding D, Starke RM, Kano H, et al. International multicenter cohort study of pediatric brain arteriovenous malformations. Part 1: Predictors of hemorrhagic presentation. J Neurosurg Pediatr 2017; 19(2): 127-35.[http://dx.doi.org/10.3171/2016.9.PEDS16283] ]. An unruptured AVM presenting with an acutely worsening hydrocephalus is a rare event. We found only 34 cases of hydrocephalus caused by an unruptured AVM in the literature, and only eight of them were less than 18 years old and only 12 cases were locating in midbrain or posterior fossa (Table 1).

Table 1
Literature Review of Hydrocephalus due to Unruptured AVM.


This study illustrates a case of an unruptured pediatric perimesencephalic AVM that presented with acute neurological deterioration due to acute obstructive hydrocephalus in a child.

2. CASE REPORT

An 11-year-old male from abroad admitted to outpatient clinic with progressive headache, nausea and vomiting which had been presented for a month. Neurologic examinations showed bilateral papillary edema and right-sided central facial nerve palsy. He had been investigated for right sided facial palsy at home and was diagnosed with an AVM located in the perimesencephalic region two years ago. Head CT showed enlargement of lateral and third ventricles with transependymal cerebrospinal fluid oozing (Fig. 1B). A prompt cranial MR revealed serpiginous signal void structures around the midbrain compatible with an AVM and enlargement of the vein of Galen and internal cerebral veins causing mechanical compression of the aqueduct causing an acute obstructive hydrocephalus caused (Fig. 1A). Cerebral DSA was planned for diagnosis and treatment planning, however; due to rapid deterioration in patient’s consciousness DSA was postponed and emergency right VP shunting was done in the same day. His conciseness improved, headache and vomiting disappeared immediately in the early postoperative period. Control CT scan showed good ventricular decompression (Fig. 1B). DSA was performed via right femoral access two weeks after the operation. Bilateral injection of internal carotid arteries showed enlarged posterior cerebral arteries bilaterally, supplying a diffuse AVM by the perforating arteries of the posterior lateral, medial choroidal and posterior communicating arteries. Venous drainage was into enlarged internal cerebral veins and vein of Galen. There was also some reflux to the deep tentorial and adjacent cortical veins due to high flow. The late capillary and venous phase was prolonged because of venous hypertension (Fig. 1C). The AVM was assessed as grade IV according to the Spetzler-Martin AVM grading system. A multimodal treatment protocol including endovascular embolization and stereotactic radiosurgery to portions of residual AVM was suggested. Patient was discharged on his family’s request as neurologically intact except for a mild right-sided facial palsy and was lost to follow-up.

Fig. (1)
A) T2 weighted MR scans showing a mesencephalic AVM that caused hydrocephalus with obstruction of aqueduct in sagittal, axial and coronary planes. B) A control CT showing the enlarged lateral ventricle with transependymal edema (left), and the collapsed ventricles following VP shunt procedure, with tip of the ventricular catheter (right). C) A DSA view perimesencephalic AVM in lateral plane, with intraventricular catheter.


3. DISCUSSION

3.1. Demographic Features

Hydrocephalus caused by an unruptured AVM may be seen in both gender and in every age but rare less than 18 years [5Geibprasert S, Pereira V, Krings T, et al. Hydrocephalus in unruptured brain arteriovenous malformations: Pathomechanical considerations, therapeutic implications, and clinical course. J Neurosurg 2009; 10(3): 500-7.[http://dx.doi.org/10.3171/2008.7.JNS0815] -8Montoya G, Dohn DF, Mercer RD. Arteriovenous malformation of the vein of Galen as a cause of heart failure and hydrocephalus in infants. Neurology 1971; 21(10): 1054-8.[http://dx.doi.org/10.1212/WNL.21.10.1054] ]. Only 4 cases of pediatric patients -two female & two male- with hydrocephalus and unruptured AVM locating in midbrain or posterior fossa were reported in the literature [5Geibprasert S, Pereira V, Krings T, et al. Hydrocephalus in unruptured brain arteriovenous malformations: Pathomechanical considerations, therapeutic implications, and clinical course. J Neurosurg 2009; 10(3): 500-7.[http://dx.doi.org/10.3171/2008.7.JNS0815] , 8Montoya G, Dohn DF, Mercer RD. Arteriovenous malformation of the vein of Galen as a cause of heart failure and hydrocephalus in infants. Neurology 1971; 21(10): 1054-8.[http://dx.doi.org/10.1212/WNL.21.10.1054] , 9Nozaki K, Hashimoto N, Kikuta K, et al. Surgical applications to arteriovenous malformations involving the brainstem. Neurosurgery 2006; 58(4)(Suppl. 2): ONS-270-9.]. We added the fifth pediatric patient with this study.

3.2. Clinical Signs and Symptoms

Hydrocephalus is mostly expected to be happening after the hemorrhage of an AVM into the ventricular system or subarachnoid space [10Mindea SA, Yang BP, Batjer H. Unruptured arteriovenous malformation in a patient presenting with obstructive hydrocephalus. Case report and review of the literature. Neurosurg Focus 2007; 22: E11.[http://dx.doi.org/10.3171/foc.2007.22.4.13] ]. Hydrocephalus is an uncommon neurologic problem in patients with unruptured AVMs [5Geibprasert S, Pereira V, Krings T, et al. Hydrocephalus in unruptured brain arteriovenous malformations: Pathomechanical considerations, therapeutic implications, and clinical course. J Neurosurg 2009; 10(3): 500-7.[http://dx.doi.org/10.3171/2008.7.JNS0815] ]. Presentation of a deeply located intracranial unruptured AVM with acute obstructive hydrocephalus is a rare entity in children. Hemorrhage is usually seen with deep seated AVMs due to deep venous drainage and in unruptured AVMs mostly located in cerebral hemispheres in children [4Ding D, Starke RM, Kano H, et al. International multicenter cohort study of pediatric brain arteriovenous malformations. Part 1: Predictors of hemorrhagic presentation. J Neurosurg Pediatr 2017; 19(2): 127-35.[http://dx.doi.org/10.3171/2016.9.PEDS16283] ]. In this case however, the AVM was located around the midbrain and not caused hemorrhage. Hemorrhage, seizure, headache and focal neurologic deficits are common neurologic problems at presentation in children with intracranial AVMs [11Bristol RE, Albuquerque FC, Spetzler RF, et al. Surgical management of arteriovenous malformations in children. J Neurosurg 2006; 105(2)(Suppl.): 88-9., 12Zheng T, Wang QJ, Liu YQ, et al. Clinical features and endovascular treatment of intracranial arteriovenous malformations in pediatric patients. Childs Nerv Syst 2014; 30(4): 647-53.[http://dx.doi.org/10.1007/s00381-013-2277-3] ]. As hemorrhage is the most common symptom in ruptured AVMs, focal neurologic deficit, seizure and headache commonly seen in unruptured AVMs [4Ding D, Starke RM, Kano H, et al. International multicenter cohort study of pediatric brain arteriovenous malformations. Part 1: Predictors of hemorrhagic presentation. J Neurosurg Pediatr 2017; 19(2): 127-35.[http://dx.doi.org/10.3171/2016.9.PEDS16283] , 13Millar C, Bissonnette B, Humphreys RP. Cerebral arteriovenous malformations in children. Can J Anaesth 1994; 41(4): 321-31.[http://dx.doi.org/10.1007/BF03009913] ]. Incidental AVMs may also be encountered during childhood deterioration [11Bristol RE, Albuquerque FC, Spetzler RF, et al. Surgical management of arteriovenous malformations in children. J Neurosurg 2006; 105(2)(Suppl.): 88-9.]. Focal neurologic deficit can be seen in patients with ruptured or unruptured AVMs, but acute neurologic deterioration due to hydrocephalus with unruptured AVM is a rare entity [7Rodríguez R, Molet Teixido J. Letter to the Editor of Acta Neurochirurgica: Obstructive hydrocephalus caused by unruptured arteriovenous malformation treated with endoscopic third ventriculostomy. Acta Neurochir (Wien) 2013; 55(5): 901-2.[http://dx.doi.org/10.1007/s00701-013-1673-5] , 10Mindea SA, Yang BP, Batjer H. Unruptured arteriovenous malformation in a patient presenting with obstructive hydrocephalus. Case report and review of the literature. Neurosurg Focus 2007; 22: E11.[http://dx.doi.org/10.3171/foc.2007.22.4.13] ]. The presented case had a cranial nerve deficit for nearly two years, but acute neurological deterioration due to hydrocephalus ensued over a short period of hours. According to the patient’s history of the presented case, an intracranial AVM was detected following facial palsy two years ago, and no new sign or symptom added until one month ago. Although the mechanism -like enlargement as a hemodynamic consequence of increased flow, or stimulated proliferation because of shunt- has been a matter of controversy, the increase in size of AVM has been documented [14Kader A, Goodrich JT, Sonstein WJ, et al. Recurrent cerebral arteriovenous malformations after negative postoperative angiograms. J Neurosurg 1996; 85(1): 14-8.[http://dx.doi.org/10.3171/jns.1996.85.1.0014] ]. We do not have any knowledge about the size of previously found AVM, but we can suggest that the occurrence of obstructive hydrocephalus and deterioration of the patient is most probably due to enlargement of the size of mesencephalic AVM.

3.3. Cause of Hydrocephalus

Hydrocephalus can occur as a result of the venous outflow and hemodynamic unbalance [5Geibprasert S, Pereira V, Krings T, et al. Hydrocephalus in unruptured brain arteriovenous malformations: Pathomechanical considerations, therapeutic implications, and clinical course. J Neurosurg 2009; 10(3): 500-7.[http://dx.doi.org/10.3171/2008.7.JNS0815] , 8Montoya G, Dohn DF, Mercer RD. Arteriovenous malformation of the vein of Galen as a cause of heart failure and hydrocephalus in infants. Neurology 1971; 21(10): 1054-8.[http://dx.doi.org/10.1212/WNL.21.10.1054] , 10Mindea SA, Yang BP, Batjer H. Unruptured arteriovenous malformation in a patient presenting with obstructive hydrocephalus. Case report and review of the literature. Neurosurg Focus 2007; 22: E11.[http://dx.doi.org/10.3171/foc.2007.22.4.13] ]; or mechanical obstruction of the ventricles by drainage vein or AVM [5Geibprasert S, Pereira V, Krings T, et al. Hydrocephalus in unruptured brain arteriovenous malformations: Pathomechanical considerations, therapeutic implications, and clinical course. J Neurosurg 2009; 10(3): 500-7.[http://dx.doi.org/10.3171/2008.7.JNS0815] , 7Rodríguez R, Molet Teixido J. Letter to the Editor of Acta Neurochirurgica: Obstructive hydrocephalus caused by unruptured arteriovenous malformation treated with endoscopic third ventriculostomy. Acta Neurochir (Wien) 2013; 55(5): 901-2.[http://dx.doi.org/10.1007/s00701-013-1673-5] ], or by compression of the aqueduct [5Geibprasert S, Pereira V, Krings T, et al. Hydrocephalus in unruptured brain arteriovenous malformations: Pathomechanical considerations, therapeutic implications, and clinical course. J Neurosurg 2009; 10(3): 500-7.[http://dx.doi.org/10.3171/2008.7.JNS0815] , 15Champeaux C, Botella C, Lefevre E, et al. Obstructive hydrocephalus caused by an unruptured arteriovenous malformation successfully treated by endoscopic third ventriculostomy after shunt dysfunction. Turk Neurosurg 2016.[http://dx.doi.org/10.5137/1019-5149.JTN.19435-16.2] -17Tucker A, Tamura Y, Hanabusa K, et al. Endoscopic third ventriculostomy for hydrocephalus due to unruptured pineal AVM: Case report and review of the literature. J Neurol Surg A Cent Eur Neurosurg 2013; 74(Suppl. 1): e45-9.[http://dx.doi.org/10.1055/s-0032-1330120] ] in unruptured AVM cases. Ebuni et al. [18Ebinu JO, Matouk CC, Wallace MC, et al. Hydrocephalus secondary to hydrodynamic disequilibrium in an adult patient with a choroidal-type arteriovenous malformation. Interv Neuroradiol 2011; 17(2): 212-6.[http://dx.doi.org/10.1177/159101991101700212] ] suggested that hydrocephalus was result of reflux into periventricular and transmedullary veins instead of mechanical obstruction in their study. Overproduction of cerebrospinal fluid may also be a cause of hydrocephalus in an unruptured choroidal AVM case [19Carleton CC, Cauthen JC. Vascular (“arteriovenous”) malformations of the choroid plexus. Arch Pathol 1975; 99: 286-8.]. In the presented case, the hydrocephalus was thought to be due to mechanical obstruction of the aqueduct by AVM.

3.4. Management of Obstructive Hydrocephalus Due to an AVM

Microsurgical resection [11Bristol RE, Albuquerque FC, Spetzler RF, et al. Surgical management of arteriovenous malformations in children. J Neurosurg 2006; 105(2)(Suppl.): 88-9.], endovascular embolization [5Geibprasert S, Pereira V, Krings T, et al. Hydrocephalus in unruptured brain arteriovenous malformations: Pathomechanical considerations, therapeutic implications, and clinical course. J Neurosurg 2009; 10(3): 500-7.[http://dx.doi.org/10.3171/2008.7.JNS0815] , 12Zheng T, Wang QJ, Liu YQ, et al. Clinical features and endovascular treatment of intracranial arteriovenous malformations in pediatric patients. Childs Nerv Syst 2014; 30(4): 647-53.[http://dx.doi.org/10.1007/s00381-013-2277-3] ], stereotactic radiosurgery [4Ding D, Starke RM, Kano H, et al. International multicenter cohort study of pediatric brain arteriovenous malformations. Part 1: Predictors of hemorrhagic presentation. J Neurosurg Pediatr 2017; 19(2): 127-35.[http://dx.doi.org/10.3171/2016.9.PEDS16283] , 5Geibprasert S, Pereira V, Krings T, et al. Hydrocephalus in unruptured brain arteriovenous malformations: Pathomechanical considerations, therapeutic implications, and clinical course. J Neurosurg 2009; 10(3): 500-7.[http://dx.doi.org/10.3171/2008.7.JNS0815] ], and/or multimodal therapy [1Darsaut TE, Guzman R, Marcellus ML, et al. Management of pediatric intracranial arteriovenous malformations: Experience with multimodality therapy. Neurosurgery 2011; 69(3): 540-56.[http://dx.doi.org/10.1227/NEU.0b013e3182181c00] ] consist of current intracranial AVM treatment. Treatment of brainstem AVMs -especially with high (Grade IV and V) Spetzler-Martin grade, is a challenge [9Nozaki K, Hashimoto N, Kikuta K, et al. Surgical applications to arteriovenous malformations involving the brainstem. Neurosurgery 2006; 58(4)(Suppl. 2): ONS-270-9., 20Tong X, Wu J, Cao Y, et al. Microsurgical outcome of unruptured brain arteriovenous malformations: A single-center experience. World Neurosurg 2017; 99: 644-5.[http://dx.doi.org/10.1016/j.wneu.2016.12.088] ]. Removal of brain stem AVMs with microsurgical resection is difficult and entangled with a high surgical risk. Since they are located deeply and close to vital structures, radiosurgery also has high risk of adverse effect and hemorrhage during latency periods, and rate of obliteration is relatively low. Preoperative embolization is advised before microsurgical excision for grad IV-V unruptured AVMs locating in brainstem [20Tong X, Wu J, Cao Y, et al. Microsurgical outcome of unruptured brain arteriovenous malformations: A single-center experience. World Neurosurg 2017; 99: 644-5.[http://dx.doi.org/10.1016/j.wneu.2016.12.088] ]. In case of AVM with large size or locating in eloquent area, conservative treatment may be adopted [5Geibprasert S, Pereira V, Krings T, et al. Hydrocephalus in unruptured brain arteriovenous malformations: Pathomechanical considerations, therapeutic implications, and clinical course. J Neurosurg 2009; 10(3): 500-7.[http://dx.doi.org/10.3171/2008.7.JNS0815] ].

Obstructive hydrocephalus due to an AVM can be treated by a VP shunting [5Geibprasert S, Pereira V, Krings T, et al. Hydrocephalus in unruptured brain arteriovenous malformations: Pathomechanical considerations, therapeutic implications, and clinical course. J Neurosurg 2009; 10(3): 500-7.[http://dx.doi.org/10.3171/2008.7.JNS0815] , 18Ebinu JO, Matouk CC, Wallace MC, et al. Hydrocephalus secondary to hydrodynamic disequilibrium in an adult patient with a choroidal-type arteriovenous malformation. Interv Neuroradiol 2011; 17(2): 212-6.[http://dx.doi.org/10.1177/159101991101700212] , 21Bayri Y, Sakar M, Ozen A, et al. Drainage vein induced hydrocephalus caused by an unruptured arteriovenous malformation in an adult presenting with visual loss. Turk Neurosurg 2017; 27(1): 151-4.] or ETV [6Park SH, Chi JG, Cho BK. Congenital arteriovenous malformation associated with progressive hydrocephalus in a newborn. J Korean Med Sci 1991; 6(4): 362-6.[http://dx.doi.org/10.3346/jkms.1991.6.4.362] , 15Champeaux C, Botella C, Lefevre E, et al. Obstructive hydrocephalus caused by an unruptured arteriovenous malformation successfully treated by endoscopic third ventriculostomy after shunt dysfunction. Turk Neurosurg 2016.[http://dx.doi.org/10.5137/1019-5149.JTN.19435-16.2] , 22Rezaee O, Sharifi G, Samadian M, et al. Endoscopic third ventriculostomy for treatment of obstructive hydrocephalus. Arch Iran Med 2007; 10: 498-503.]. ETV is a technique which would not involve a change between supratentorial and infratentorial pressure relationships [7Rodríguez R, Molet Teixido J. Letter to the Editor of Acta Neurochirurgica: Obstructive hydrocephalus caused by unruptured arteriovenous malformation treated with endoscopic third ventriculostomy. Acta Neurochir (Wien) 2013; 55(5): 901-2.[http://dx.doi.org/10.1007/s00701-013-1673-5] ] and it considered as an advantage for being “shunt free” [15Champeaux C, Botella C, Lefevre E, et al. Obstructive hydrocephalus caused by an unruptured arteriovenous malformation successfully treated by endoscopic third ventriculostomy after shunt dysfunction. Turk Neurosurg 2016.[http://dx.doi.org/10.5137/1019-5149.JTN.19435-16.2] ]. Malfunctioning or over drainage may be seen following VP shunting [5Geibprasert S, Pereira V, Krings T, et al. Hydrocephalus in unruptured brain arteriovenous malformations: Pathomechanical considerations, therapeutic implications, and clinical course. J Neurosurg 2009; 10(3): 500-7.[http://dx.doi.org/10.3171/2008.7.JNS0815] ]. We chose VP shunting in this case because that procedure was the fastest treatment option in the emergency setting in our conditions [5Geibprasert S, Pereira V, Krings T, et al. Hydrocephalus in unruptured brain arteriovenous malformations: Pathomechanical considerations, therapeutic implications, and clinical course. J Neurosurg 2009; 10(3): 500-7.[http://dx.doi.org/10.3171/2008.7.JNS0815] ] to treat obstructive hydrocephalus.

Some authors primarily chose to treat AVM in cases with mild symptoms of obstruction that not required emergency [18Ebinu JO, Matouk CC, Wallace MC, et al. Hydrocephalus secondary to hydrodynamic disequilibrium in an adult patient with a choroidal-type arteriovenous malformation. Interv Neuroradiol 2011; 17(2): 212-6.[http://dx.doi.org/10.1177/159101991101700212] , 23Pribil S, Boone SC, Waley R. Obstructive hydrocephalus at the anterior third ventricle caused by dilated veins from an arteriovenous malformation. Surg Neurol 1983; 20(6): 487-92.[http://dx.doi.org/10.1016/0090-3019(83)90032-0] ].

Patient’s family requested discharge although they were fully informed about the disease and treatment options due to financial concerns and patient was lost to follow up.

CONCLUSION

Presentation of a deeply located intracranial unruptured AVM with acute obstructive hydrocephalus is a rare entity in children. Although focal neurologic deficit, seizure and headache are the most common symptoms, acute neurologic deterioration due to hydrocephalus may be the presenting symptom in these cases. The decrease in intracranial pressure by changing the flow of CSF via an emergency VP shunting or ETV can be a lifesaving procedure that gives a chance for further treatment modalities.

ETHICS APPROVAL AND CONSENT TO PARTICIPATE

The study was approved by an internal ethics committee at the specialized arthritis centre and was conducted in accordance with the recommendations of the Declaration of Helsinki.

HUMAN AND ANIMAL RIGHTS

No Animals were used in this research. All human research procedures followed were in accordance with the ethical standards of the committee responsible for human experimentation (institutional and national), and with the Helsinki Declaration of 1975, as revised in 2008.

CONSENT FOR PUBLICATION

A written informed consent was obtained from the patient when he was enrolled.

CONFLICT OF INTEREST

The authors declare no conflict of interest, financial or otherwise.

ACKNOWLEDGEMENTS

Declared none.

REFERENCES

[1] Darsaut TE, Guzman R, Marcellus ML, et al. Management of pediatric intracranial arteriovenous malformations: Experience with multimodality therapy. Neurosurgery 2011; 69(3): 540-56.[http://dx.doi.org/10.1227/NEU.0b013e3182181c00]
[2] Kiriş T, Sencer A, Sahinbaş M, et al. Surgical results in pediatric Spetzler-Martin grades I-III intracranial arteriovenous malformations. Childs Nerv Syst 2005; 21(1): 69-76.[http://dx.doi.org/10.1007/s00381-004-1025-0]
[3] Di Rocco C, Tamburrini G, Rollo M. Cerebral arteriovenous malformations in children. Acta Neurochir (Wien) 2000; 142(2): 145-58.[http://dx.doi.org/10.1007/s007010050017]
[4] Ding D, Starke RM, Kano H, et al. International multicenter cohort study of pediatric brain arteriovenous malformations. Part 1: Predictors of hemorrhagic presentation. J Neurosurg Pediatr 2017; 19(2): 127-35.[http://dx.doi.org/10.3171/2016.9.PEDS16283]
[5] Geibprasert S, Pereira V, Krings T, et al. Hydrocephalus in unruptured brain arteriovenous malformations: Pathomechanical considerations, therapeutic implications, and clinical course. J Neurosurg 2009; 10(3): 500-7.[http://dx.doi.org/10.3171/2008.7.JNS0815]
[6] Park SH, Chi JG, Cho BK. Congenital arteriovenous malformation associated with progressive hydrocephalus in a newborn. J Korean Med Sci 1991; 6(4): 362-6.[http://dx.doi.org/10.3346/jkms.1991.6.4.362]
[7] Rodríguez R, Molet Teixido J. Letter to the Editor of Acta Neurochirurgica: Obstructive hydrocephalus caused by unruptured arteriovenous malformation treated with endoscopic third ventriculostomy. Acta Neurochir (Wien) 2013; 55(5): 901-2.[http://dx.doi.org/10.1007/s00701-013-1673-5]
[8] Montoya G, Dohn DF, Mercer RD. Arteriovenous malformation of the vein of Galen as a cause of heart failure and hydrocephalus in infants. Neurology 1971; 21(10): 1054-8.[http://dx.doi.org/10.1212/WNL.21.10.1054]
[9] Nozaki K, Hashimoto N, Kikuta K, et al. Surgical applications to arteriovenous malformations involving the brainstem. Neurosurgery 2006; 58(4)(Suppl. 2): ONS-270-9.
[10] Mindea SA, Yang BP, Batjer H. Unruptured arteriovenous malformation in a patient presenting with obstructive hydrocephalus. Case report and review of the literature. Neurosurg Focus 2007; 22: E11.[http://dx.doi.org/10.3171/foc.2007.22.4.13]
[11] Bristol RE, Albuquerque FC, Spetzler RF, et al. Surgical management of arteriovenous malformations in children. J Neurosurg 2006; 105(2)(Suppl.): 88-9.
[12] Zheng T, Wang QJ, Liu YQ, et al. Clinical features and endovascular treatment of intracranial arteriovenous malformations in pediatric patients. Childs Nerv Syst 2014; 30(4): 647-53.[http://dx.doi.org/10.1007/s00381-013-2277-3]
[13] Millar C, Bissonnette B, Humphreys RP. Cerebral arteriovenous malformations in children. Can J Anaesth 1994; 41(4): 321-31.[http://dx.doi.org/10.1007/BF03009913]
[14] Kader A, Goodrich JT, Sonstein WJ, et al. Recurrent cerebral arteriovenous malformations after negative postoperative angiograms. J Neurosurg 1996; 85(1): 14-8.[http://dx.doi.org/10.3171/jns.1996.85.1.0014]
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