{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,29]],"date-time":"2026-05-29T12:27:23Z","timestamp":1780057643413,"version":"3.54.0"},"reference-count":56,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"4","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["PED"],"published-print":{"date-parts":[[2013,10]]},"abstract":"<jats:sec>\n<jats:title>Object<\/jats:title>\n<jats:p>Pial arteriovenous fistulas (PAVFs) are a rare form of cerebrovascular disease that tend to be overrepresented in the pediatric population. There have been limited studies of the clinical features and outcomes in this group of patients. Here, the authors attempt to better delineate this clinical entity with institutional cases and a review of the literature.<\/jats:p><\/jats:sec>\n<jats:sec>\n<jats:title>Methods<\/jats:title>\n<jats:p>A retrospective review of cases at our institution was performed to identify all pediatric patients treated for a PAVF between 2000 and 2012.<\/jats:p><\/jats:sec>\n<jats:sec>\n<jats:title>Results<\/jats:title>\n<jats:p>Five patients treated for a PAVF were identified. Patients had a mean age of 1.9 years at diagnosis, and the most common presenting symptoms were seizure and macrocephaly. Patients were treated primarily with embolization, and 3 patients required both <jats:italic>N<\/jats:italic>-butyl cyanoacrylate (NBCA) glue and coiling. Four of the patients had complete obliteration of the PAVF and had a pediatric overall performance category score of either 1 (n = 3) or 2 (n = 1) at follow-up. There was 1 death due to heart failure. Analysis of the literature review suggested that a younger age or presence of intracerebral hemorrhage (ICH) or congestive heart failure (CHF) at presentation likely predicts a worse prognosis. Older patients presented more often with ICH, whereas younger patients presented significantly more often in CHF. The majority of pediatric patients reported on in the literature were treated with endovascular embolization, most commonly with NBCA glue alone. Most patients (65.4%) in the literature had an excellent outcome without neurological deficit.<\/jats:p><\/jats:sec>\n<jats:sec>\n<jats:title>Conclusions<\/jats:title>\n<jats:p>Pial AVFs represent a serious yet rare form of cerebrovascular disease. Pediatric patients with ICH or CHF at presentation or those who are very young are likely to have a worse prognosis. Endovascular management of these patients has greatly changed the natural history of this disease, but the complication and mortality rates suggest the need for continued insights and advances in treatment.<\/jats:p><\/jats:sec>","DOI":"10.3171\/2013.6.peds13110","type":"journal-article","created":{"date-parts":[[2013,8,2]],"date-time":"2013-08-02T14:01:40Z","timestamp":1375452100000},"page":"344-350","source":"Crossref","is-referenced-by-count":47,"title":["An institutional series and literature review of pial arteriovenous fistulas in the pediatric population"],"prefix":"10.3171","volume":"12","author":[{"given":"Peter J.","family":"Madsen","sequence":"first","affiliation":[{"name":"Departments of Neurosurgery and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shih-Shan","family":"Lang","sequence":"additional","affiliation":[{"name":"Departments of Neurosurgery and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jared M.","family":"Pisapia","sequence":"additional","affiliation":[{"name":"Departments of Neurosurgery and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Phillip B.","family":"Storm","sequence":"additional","affiliation":[{"name":"Departments of Neurosurgery and"},{"name":"Division of Neurosurgery, Children's Hospital of Philadelphia, Pennsylvania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert W.","family":"Hurst","sequence":"additional","affiliation":[{"name":"Radiology, Hospital of the University of Pennsylvania; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gregory G.","family":"Heuer","sequence":"additional","affiliation":[{"name":"Departments of Neurosurgery and"},{"name":"Division of Neurosurgery, Children's Hospital of Philadelphia, Pennsylvania"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1754","reference":[{"key":"b1-peds13110","series-title":"J Child Neurol","doi-asserted-by":"publisher","first-page":"1015","DOI":"10.1177\/0883073810397363","article-title":"Endovascular treatment of a pial arteriovenous fistula with occipital remodeling secondary to giant torcular dilation","volume":"26","author":"Aguilar","year":"2011"},{"key":"b2-peds13110","series-title":"Childs Nerv Syst","doi-asserted-by":"publisher","first-page":"216","DOI":"10.1007\/BF01850976","article-title":"Hemispheric arteriovenous fistulae with giant venous dilation","volume":"6","author":"Almeida","year":"1990"},{"key":"b3-peds13110","series-title":"Surg Neurol","first-page":"45","article-title":"Giant hemispheric arteriovenous fistula in an infant","volume":"7","author":"Antunes","year":"1977"},{"key":"b4-peds13110","series-title":"Neurosurgery","doi-asserted-by":"publisher","first-page":"619","DOI":"10.1227\/00006123-199104000-00027","article-title":"Intracranial arteriovenous fistula manifesting as progressive neurological deterioration in an infant: case report","volume":"28","author":"Aoki","year":"1991"},{"key":"b5-peds13110","series-title":"Neurosurgery","doi-asserted-by":"publisher","first-page":"122","DOI":"10.1227\/00006123-199001000-00017","article-title":"Intracerebral arteriovenous fistulas associated with intraparenchymal varix in childhood: case reports","volume":"26","author":"Barnwell","year":"1990"},{"key":"b6-peds13110","series-title":"Interv Neuroradiol","doi-asserted-by":"publisher","first-page":"273","DOI":"10.1177\/159101990200800307","article-title":"Encephalocraniocutaneous lipomatosis syndrome in a child: association with multiple high flow cerebral arteriovenous fistulae. Case report and review","volume":"8","author":"Batista","year":"2002"},{"key":"b7-peds13110","series-title":"Cerebrovascular Diseases in Children","first-page":"132","article-title":"Endovascular treatment of cerebrovascular pathology","author":"Beltramello","year":"1991"},{"key":"b8-peds13110","series-title":"J Neurosurg","doi-asserted-by":"publisher","first-page":"1085","DOI":"10.3171\/jns.1984.60.5.1085","article-title":"Giant aneurysm arising from a single arteriovenous fistula in a child. Case report","volume":"60","author":"Carrillo","year":"1984"},{"key":"b9-peds13110","series-title":"Childs Nerv Syst","first-page":"785","article-title":"Total endovascular occlusion of a giant direct arteriovenous fistula in the posterior fossa in a case of Rendu-Osler-Weber disease","volume":"12","author":"Coubes","year":"1996"},{"key":"b10-peds13110","series-title":"Am J Hum Genet","doi-asserted-by":"publisher","first-page":"1240","DOI":"10.1086\/379793","article-title":"Capillary malformation-arteriovenous malformation, a new clinical and genetic disorder caused by RASA1 mutations","volume":"73","author":"Eerola","year":"2003"},{"key":"b11-peds13110","series-title":"J Pediatr","doi-asserted-by":"publisher","first-page":"68","DOI":"10.1016\/S0022-3476(05)82544-2","article-title":"Assessing the outcome of pediatric intensive care","volume":"121","author":"Fiser","year":"1992"},{"key":"b12-peds13110","series-title":"Crit Care Med","doi-asserted-by":"publisher","first-page":"2616","DOI":"10.1097\/00003246-200007000-00072","article-title":"Relationship of pediatric overall performance category and pediatric cerebral performance category scores at pediatric intensive care unit discharge with outcome measures collected at hospital discharge and 1- and 6-month follow-up assessments","volume":"28","author":"Fiser","year":"2000"},{"key":"b13-peds13110","series-title":"Childs Nerv Syst","doi-asserted-by":"publisher","first-page":"48","DOI":"10.1007\/BF00263834","article-title":"Congestive cardiac manifestations from cerebrocranial arteriovenous shunts. Endovascular management in 30 children","volume":"7","author":"Garc\u00eda-Monaco","year":"1991"},{"key":"b14-peds13110","series-title":"Neuroradiology","doi-asserted-by":"publisher","first-page":"60","DOI":"10.1007\/BF00588522","article-title":"Pial arteriovenous fistula in children as presenting manifestation of Rendu-Osler-Weber disease","volume":"37","author":"Garc\u00eda-M\u00f3naco","year":"1995"},{"key":"b15-peds13110","series-title":"Arq Neuropsiquiatr","doi-asserted-by":"publisher","first-page":"718","DOI":"10.1590\/S0004-282X2011000500028","article-title":"Pial arteriovenous fistula in the posterior fossa","volume":"69","author":"Guerra","year":"2011"},{"key":"b16-peds13110","series-title":"AJNR Am J Neuroradiol","first-page":"747","article-title":"Transarterial occlusion of solitary intracerebral arteriovenous fistulas","volume":"10","author":"Halbach","year":"1989"},{"key":"b17-peds13110","series-title":"Childs Nerv Syst","doi-asserted-by":"publisher","first-page":"494","DOI":"10.1007\/BF00334975","article-title":"Endovascular treatment of an infantile nongalenic cerebral arteriovenous fistula with cyanoacrylate","volume":"11","author":"Hermier","year":"1995"},{"key":"b18-peds13110","series-title":"AJNR Am J Neuroradiol","doi-asserted-by":"publisher","first-page":"1710","DOI":"10.3174\/ajnr.A3194","article-title":"Pediatric intracranial nongalenic pial arteriovenous fistulas: clinical features, angioarchitecture, and outcomes","volume":"33","author":"Hetts","year":"2012"},{"key":"b19-peds13110","series-title":"Childs Nerv Syst","doi-asserted-by":"publisher","first-page":"879","DOI":"10.1007\/s00381-009-1063-8","article-title":"Diagnosis and treatment of vein of Galen aneurysmal malformations","volume":"26","author":"Heuer","year":"2010"},{"key":"b20-peds13110","series-title":"Neurosurgery","doi-asserted-by":"publisher","first-page":"1351","DOI":"10.1097\/00006123-200112000-00011","article-title":"Surgical and endovascular flow disconnection of intracranial pial single-channel arteriovenous fistulae","volume":"49","author":"Hoh","year":"2001"},{"key":"b21-peds13110","series-title":"Pediatr Neurol","doi-asserted-by":"publisher","first-page":"300","DOI":"10.1016\/S0887-8994(02)00424-1","article-title":"Successful endovascular treatment of cerebral arteriovenous fistula","volume":"27","author":"Hung","year":"2002"},{"key":"b22-peds13110","series-title":"Neuroradiolog J","doi-asserted-by":"publisher","first-page":"783","DOI":"10.1177\/197140090601900615","article-title":"Posterior fossa arteriovenous pial fistula: diagnostic and endovascular therapeutic features","volume":"19","author":"Izzo","year":"2006"},{"key":"b23-peds13110","series-title":"Surg Neurol","doi-asserted-by":"publisher","first-page":"374","DOI":"10.1016\/0090-3019(94)90030-2","article-title":"Vascular malformations of the brain in hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber disease)","volume":"41","author":"Kikuchi","year":"1994"},{"key":"b24-peds13110","series-title":"Neurosurg Rev","doi-asserted-by":"publisher","first-page":"253","DOI":"10.1007\/BF01743632","article-title":"Angiographic architecture of intracranial vascular malformations and fistulas\u2014pretherapeutic aspects","volume":"9","author":"Lasjaunias","year":"1986"},{"issue":"3 Suppl 3","key":"b25-peds13110","series-title":"Neurosurgery","doi-asserted-by":"publisher","first-page":"S3184","DOI":"10.1227\/01.NEU.0000237445.39514.16","article-title":"The management of vein of Galen aneurysmal malformations","volume":"59","author":"Lasjaunias","year":"2006"},{"key":"b26-peds13110","series-title":"J Cerebrovasc Endovasc Neurosurg","doi-asserted-by":"publisher","first-page":"305","DOI":"10.7461\/jcen.2012.14.4.305","article-title":"Intracranial pial arteriovenous fistula presenting with hemorrhage: a case report","volume":"14","author":"Lee","year":"2012"},{"key":"b27-peds13110","series-title":"J Korean Neurosurg Soc","doi-asserted-by":"publisher","first-page":"101","DOI":"10.3340\/jkns.2008.44.2.101","article-title":"Intracranial pial arteriovenous fistulas","volume":"44","author":"Lee","year":"2008"},{"key":"b28-peds13110","series-title":"Neurol India","first-page":"87","article-title":"Endovascular management of intracranial pial arterio-venous fistulas","volume":"52","author":"Limaye","year":"2004"},{"key":"b29-peds13110","series-title":"J Neurosurg","doi-asserted-by":"publisher","first-page":"733","DOI":"10.3171\/2010.3.JNS09370","article-title":"A prospective, multicenter, randomized trial of the Onyx liquid embolic system and N-butyl cyanoacrylate embolization of cerebral arteriovenous malformations. Clinical article","volume":"113","author":"Loh","year":"2010"},{"key":"b30-peds13110","series-title":"Interventional Neuroradiology: Endovascular Therapy of the Central Nervous System","first-page":"87","article-title":"Endovascular therapy of nongalenic cerebral arteriovenous fistulas","author":"Lownie","year":"1992"},{"key":"b31-peds13110","series-title":"World Neurosurg","doi-asserted-by":"publisher","first-page":"385","DOI":"10.1016\/j.wneu.2010.01.023","article-title":"Clinical outcomes of endovascular treatment for intracranial pial arteriovenous fistulas","volume":"73","author":"Lv","year":"2010"},{"key":"b32-peds13110","series-title":"AJNR Am J Neuroradiol","doi-asserted-by":"publisher","first-page":"851","DOI":"10.3174\/ajnr.A1436","article-title":"Endovascular treatment of brain arteriovenous fistulas","volume":"30","author":"Lv","year":"2009"},{"key":"b33-peds13110","series-title":"Neurosurgery","doi-asserted-by":"publisher","first-page":"295","DOI":"10.1227\/00006123-199008000-00021","article-title":"Isolated growth hormone deficiency associated with a giant arteriovenous varix","volume":"27","author":"Martin","year":"1990"},{"key":"b34-peds13110","series-title":"Arch Dis Child","doi-asserted-by":"publisher","first-page":"903","DOI":"10.1136\/adc.2009.177584","article-title":"Management and outcome of vein of Galen malformation","volume":"95","author":"McSweeney","year":"2010"},{"key":"b35-peds13110","series-title":"Arq Neuropsiquiatr","doi-asserted-by":"publisher","first-page":"463","DOI":"10.1590\/S0004-282X2010000300026","article-title":"Endovascular treatment of intracranial pial arteriovenous fistula","volume":"68","author":"Nakiri","year":"2010"},{"key":"b36-peds13110","series-title":"Neuroimaging Clin N Am","first-page":"309","article-title":"Endovascular embolization of congenital intracranial pial arteriovenous fistulas","volume":"2","author":"Nelson","year":"1992"},{"key":"b37-peds13110","series-title":"J Neurosurg Pediatr","doi-asserted-by":"publisher","first-page":"637","DOI":"10.3171\/2011.4.PEDS10577","article-title":"Balloon-assisted Onyx embolization of cerebral single-channel pial arteriovenous fistulas. Technical note","volume":"7","author":"Newman","year":"2011"},{"key":"b38-peds13110","series-title":"Dev Med Child Neurol","first-page":"245","article-title":"Cerebral arteriovenous fistula in the Klippel-Trenaunay-Weber syndrome","volume":"30","author":"Oyesiku","year":"1988"},{"key":"b39-peds13110","series-title":"Neuroradiology","doi-asserted-by":"publisher","first-page":"380","DOI":"10.1007\/s00234-004-1323-y","article-title":"A contribution to the controversy over dimethyl sulfoxide toxicity: anesthesia monitoring results in patients treated with Onyx embolization for intracranial aneurysms","volume":"47","author":"Pamuk","year":"2005"},{"key":"b40-peds13110","series-title":"J Neurointerv Surg","doi-asserted-by":"publisher","first-page":"184","DOI":"10.1136\/neurintsurg-2011-010241","article-title":"Development, clinical presentation and endovascular management of congenital intracranial pial arteriovenous fistulas","volume":"5","author":"Paramasivam","year":"2013"},{"key":"b41-peds13110","series-title":"Neurol India","doi-asserted-by":"publisher","first-page":"434","DOI":"10.4103\/0028-3886.28124","article-title":"Surgical management of a pial arteriovenous fistula with giant varix in an infant","volume":"54","author":"Pillai","year":"2006"},{"key":"b42-peds13110","series-title":"Childs Nerv Syst","doi-asserted-by":"publisher","first-page":"1521","DOI":"10.1007\/s00381-009-0921-8","article-title":"Single hole cerebral arteriovenous fistula between the anterior choroidal artery and the basal vein of Rosenthal in a child","volume":"25","author":"Rivera","year":"2009"},{"key":"b43-peds13110","series-title":"Cases J","doi-asserted-by":"publisher","first-page":"335","DOI":"10.1186\/1757-1626-1-335","article-title":"Intracerebral atypical calcification in nongalenic pial arteriovenous fistula: a case report","volume":"1","author":"Tabatabai","year":"2008"},{"key":"b44-peds13110","series-title":"J Neurosurg Sci","first-page":"337","article-title":"Complex arteriovenous fistula of the brain in an infant. Case report","volume":"41","author":"Talamonti","year":"1997"},{"key":"b45-peds13110","series-title":"Am J Ophthalmol","doi-asserted-by":"publisher","first-page":"484","DOI":"10.1016\/j.ajo.2004.03.023","article-title":"Acquired, isolated third nerve palsies in infants with cerebrovascular malformations","volume":"138","author":"Tamhankar","year":"2004"},{"key":"b46-peds13110","series-title":"Crit Care Res Pract","first-page":"918185","article-title":"Acute respiratory distress syndrome after onyx embolization of arteriovenous malformation","volume":"2011","author":"Tawil","year":"2011"},{"key":"b47-peds13110","series-title":"J Neurosurg","doi-asserted-by":"publisher","first-page":"16","DOI":"10.3171\/jns.1993.79.1.0016","article-title":"Arteriovenous fistulas of the brain and the spinal cord","volume":"79","author":"Tomlinson","year":"1993"},{"issue":"6","key":"b48-peds13110","series-title":"Neurosurg Focus","first-page":"E8","article-title":"Nongalenic arteriovenous fistulas: history of treatment and technology","volume":"20","author":"Upchurch","year":"2006"},{"issue":"1 Suppl 1","key":"b49-peds13110","series-title":"Neurosurgery","first-page":"ONS73","article-title":"Comparison of N-butyl cyanoacrylate and onyx for the embolization of intracranial arteriovenous malformations: analysis of fluoroscopy and procedure times","volume":"63","author":"Velat","year":"2008"},{"key":"b50-peds13110","series-title":"J Neurosurg","doi-asserted-by":"publisher","first-page":"198","DOI":"10.3171\/jns.1987.66.2.0198","article-title":"Giant intracranial varices secondary to high-flow arteriovenous fistulae","volume":"66","author":"Vi\u00f1uela","year":"1987"},{"issue":"2 Suppl Pediatrics","key":"b51-peds13110","series-title":"J Neurosurg","first-page":"201","article-title":"Intracranial pial arteriovenous fistulas with single-vein drainage. Report of three cases and review of the literature","volume":"100","author":"Wang","year":"2004"},{"key":"b52-peds13110","series-title":"Acta Neurochir (Wien)","doi-asserted-by":"publisher","first-page":"17","DOI":"10.1007\/s00701-004-0341-1","article-title":"Supratentorial cerebral arteriovenous fistulas (AVFs) in children: review of 41 cases with 63 non choroidal single-hole AVFs","volume":"147","author":"Weon","year":"2005"},{"key":"b53-peds13110","series-title":"Br J Neurosurg","doi-asserted-by":"publisher","first-page":"580","DOI":"10.3109\/02688697.2011.566382","article-title":"Pial arteriovenous fistula: a review of literature","volume":"25","author":"Yang","year":"2011"},{"key":"b54-peds13110","series-title":"Neuroradiology","first-page":"474","article-title":"Posterior cranial fossa single-hole arteriovenous fistulae in children: 14 consecutive cases","volume":"46","author":"Yoshida","year":"2004"},{"key":"b55-peds13110","series-title":"World Neurosurg","doi-asserted-by":"publisher","first-page":"2","DOI":"10.1016\/j.surneu.2009.06.001","article-title":"Coil-based endovascular treatment of single-hole cerebral arteriovenous fistulae: experiences in 11 patients","volume":"73","author":"Youn","year":"2010"},{"key":"b56-peds13110","series-title":"Childs Nerv Syst","doi-asserted-by":"publisher","first-page":"1381","DOI":"10.1007\/s00381-010-1223-x","article-title":"Neurosurgical vascular malformations in children under 1 year of age","volume":"26","author":"Zuccaro","year":"2010"}],"container-title":["Journal of Neurosurgery: Pediatrics"],"original-title":[],"link":[{"URL":"https:\/\/thejns.org\/view\/journals\/j-neurosurg-pediatr\/12\/4\/article-p344.xml","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/thejns.org\/downloadpdf\/journals\/j-neurosurg-pediatr\/12\/4\/article-p344.xml","content-type":"text\/html","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/thejns.org\/downloadpdf\/journals\/j-neurosurg-pediatr\/12\/4\/article-p344.xml","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,4,15]],"date-time":"2023-04-15T12:46:34Z","timestamp":1681562794000},"score":1,"resource":{"primary":{"URL":"https:\/\/thejns.org\/view\/journals\/j-neurosurg-pediatr\/12\/4\/article-p344.xml"}},"subtitle":["Clinical article"],"short-title":[],"issued":{"date-parts":[[2013,10]]},"references-count":56,"journal-issue":{"issue":"4"},"URL":"https:\/\/doi.org\/10.3171\/2013.6.peds13110","relation":{},"ISSN":["1933-0707","1933-0715"],"issn-type":[{"value":"1933-0707","type":"print"},{"value":"1933-0715","type":"electronic"}],"subject":[],"published":{"date-parts":[[2013,10]]}}}