{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,9]],"date-time":"2026-03-09T06:11:20Z","timestamp":1773036680510,"version":"3.50.1"},"reference-count":38,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"3","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["PED"],"published-print":{"date-parts":[[2008,9]]},"abstract":"<jats:sec>\n<jats:title>Object<\/jats:title>\n<jats:p>The Dandy\u2013Walker complex is a continuum of aberrant development of the posterior fossa that has been associated with multiple congenital anomalies, radiographic abnormalities, and developmental delay. The Dandy\u2013Walker variant (DWV) is a unique entity believed to represent a milder form of the complex, and is characterized by a specific constellation of radiographic findings. In this retrospective case series, the authors report the association of the DWV with other congenital anomalies, the associated radiographic findings linked with DWV, and the developmental outcome in this population.<\/jats:p><\/jats:sec>\n<jats:sec>\n<jats:title>Methods<\/jats:title>\n<jats:p>The charts and radiographs of 10 male and 14 female patients treated between 2000 and 2006 were examined. The patients' mean gestational age was 35.6 weeks (range 23\u201341 weeks), and the mean follow-up period was 5.1 years (range 1 month\u201315 years).<\/jats:p><\/jats:sec>\n<jats:sec>\n<jats:title>Results<\/jats:title>\n<jats:p>Three patients died. Associated anomalies included cardiac (41.7%), neurological (33.3%), gastrointestinal (20.8%), orthopedic (12.5%), and genitourinary (12.5%) abnormalities. Less common were pulmonary and psychiatric findings. Developmental delay was identified in 11 of the 21 patients for whom follow-up was available. Five of 6 patients with isolated DWV had a normal developmental course. Radiographic findings associated with DWV included corpus callosum dysgenesis in 20.8%, ventricular enlargement in 29%, and vermian rotation in 8.3%. Shunts were placed in 4 of 7 patients with ventriculomegaly. Using the two-tailed Pearson correlation, the authors determined that developmental outcome was solely affected by neurological deficits and that ventricular enlargement predicted the need for shunt placement.<\/jats:p><\/jats:sec>\n<jats:sec>\n<jats:title>Conclusions<\/jats:title>\n<jats:p>The DWV was associated with both extra- and intracranial anomalies. Associated radiographic abnormalities including ventriculomegaly were observed. Hydrocephalus requiring cerebrospinal fluid diversion may be indicated. Isolated DWV was associated with a good developmental outcome.<\/jats:p><\/jats:sec>","DOI":"10.3171\/ped\/2008\/2\/9\/194","type":"journal-article","created":{"date-parts":[[2008,8,29]],"date-time":"2008-08-29T18:15:04Z","timestamp":1220033704000},"page":"194-199","source":"Crossref","is-referenced-by-count":63,"title":["The Dandy\u2013Walker variant: a case series of 24 pediatric patients and evaluation of associated anomalies, incidence of hydrocephalus, and developmental outcomes"],"prefix":"10.3171","volume":"2","author":[{"given":"Deanna","family":"Sasaki-Adams","sequence":"first","affiliation":[{"name":"Division of Neurosurgery, Department of General Surgery;"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Samer K.","family":"Elbabaa","sequence":"additional","affiliation":[{"name":"Division of Neurosurgery, Department of General Surgery;"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Valerie","family":"Jewells","sequence":"additional","affiliation":[{"name":"Division of Neuroradiology, Department of Radiology; and"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lori","family":"Carter","sequence":"additional","affiliation":[{"name":"Division of Maternal and Fetal Medicine, Department of Neonatology, University of North Carolina School of Medicine, Chapel Hill, North Carolina; and"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jeffrey W.","family":"Campbell","sequence":"additional","affiliation":[{"name":"Division of Neurosurgery, Department of General Surgery, Nemours Alfred I. duPont Hospital for Children, Wilmington, Delaware"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ann M.","family":"Ritter","sequence":"additional","affiliation":[{"name":"Division of Neurosurgery, Department of General Surgery;"},{"name":"Division of Neurosurgery, Department of General Surgery, Nemours Alfred I. duPont Hospital for Children, Wilmington, Delaware"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1754","reference":[{"key":"b1-0020194","series-title":"Pediatr Radiol","doi-asserted-by":"publisher","first-page":"124","DOI":"10.1007\/s00247-004-1316-3","article-title":"MRI of the fetal posterior fossa","volume":"35","author":"Adamsbaum","year":"2005"},{"key":"b2-0020194","series-title":"Int J Cardiol","doi-asserted-by":"publisher","first-page":"258","DOI":"10.1016\/j.ijcard.2005.09.013","article-title":"An unusual combination: aortic arch coarctation associated with Dandy-Walker variant","volume":"113","author":"Akgul","year":"2006"},{"key":"b3-0020194","series-title":"Pediatr Neurosci","doi-asserted-by":"publisher","first-page":"66","DOI":"10.1159\/000120445","article-title":"Dandy-Walker syndrome: experience at the Hospital for Sick Children, Toronto","volume":"15","author":"Asai","year":"1989"},{"key":"b4-0020194","series-title":"Neuroradiology","doi-asserted-by":"publisher","first-page":"948","DOI":"10.1007\/s002340100576","article-title":"Menkes' disease with a Dandy-Walker variant: case report","volume":"43","author":"Bekiesinska-Figatowska","year":"2001"},{"key":"b5-0020194","series-title":"J Neuropathol Exp Neurol","doi-asserted-by":"publisher","first-page":"14","DOI":"10.1093\/jnen\/13.1.14","article-title":"The Dandy-Walker syndrome or the so-called atresia of the foramen Magendie","volume":"13","author":"Benda","year":"1954"},{"key":"b6-0020194","series-title":"Pediatr Neurosurg","doi-asserted-by":"publisher","first-page":"163","DOI":"10.1159\/000120518","article-title":"Management of the Dandy-Walker syndrome","volume":"16","author":"Bindal","year":"1990"},{"key":"b7-0020194","series-title":"Dev Med Child Neurol","first-page":"285","article-title":"Dandy-Walker syndrome and agenesis of the cerebellar vermis: diagnostic problems and genetic counselling","volume":"32","author":"Bordarier","year":"1990"},{"key":"b8-0020194","series-title":"Radiology","doi-asserted-by":"publisher","first-page":"765","DOI":"10.1148\/radiology.193.3.7972821","article-title":"Sonographic detection of inferior vermian agenesis in Dandy-Walker malformations: prognostic implications","volume":"193","author":"Chang","year":"1994"},{"key":"b9-0020194","series-title":"Prenat Diagn","doi-asserted-by":"publisher","first-page":"1112","DOI":"10.1002\/pd.471","article-title":"Pre-natal diagnosis of partial trisomy 3p and partial monosomy 11q in a fetus with a Dandy-Walker variant and trigonocephaly","volume":"22","author":"Chen","year":"2002"},{"key":"b10-0020194","series-title":"J Neuropathol Exp Neurol","doi-asserted-by":"publisher","first-page":"450","DOI":"10.1097\/00005072-196307000-00007","article-title":"The Dandy Walker Syndrome","volume":"22","author":"D'Agostino","year":"1963"},{"key":"b11-0020194","series-title":"Surg Gynecol Obstet","first-page":"112","article-title":"The diagnosis and treatment of hydrocephalus due to occlusions of the foramina of Magendie and Luschka","volume":"32","author":"Dandy","year":"1921"},{"key":"b12-0020194","series-title":"Am J Dis Child","first-page":"406","article-title":"Internal hydrocephalus. An experimental, clinical, and pathological study","volume":"8","author":"Dandy","year":"1914"},{"key":"b13-0020194","series-title":"Neuroradiology","doi-asserted-by":"publisher","first-page":"639","DOI":"10.1007\/s00234-002-0783-1","article-title":"Abnormalities of cerebellar foliation and fissuration: classification, neurogenetics and clinicoradiological correlations","volume":"44","author":"Demaerel","year":"2002"},{"key":"b14-0020194","series-title":"Am J Med Genet","doi-asserted-by":"publisher","first-page":"97","DOI":"10.1002\/ajmg.1320560122","article-title":"Interstitial deletion of 8q21\u2013->22 associated with minor anomalies, congenital heart defect, and Dandy-Walker variant","volume":"56","author":"Donahue","year":"1995"},{"key":"b15-0020194","series-title":"Prenat Diagn","doi-asserted-by":"publisher","first-page":"328","DOI":"10.1002\/(SICI)1097-0223(200004)20:4<328::AID-PD806>3.0.CO;2-O","article-title":"The sonographic diagnosis of Dandy-Walker and Dandy-Walker variant: associated findings and outcomes","volume":"20","author":"Ecker","year":"2000"},{"key":"b16-0020194","series-title":"Radiology","doi-asserted-by":"publisher","first-page":"755","DOI":"10.1148\/radiology.185.3.1438757","article-title":"Dandy-Walker variant: prenatal sonographic features and clinical outcome","volume":"185","author":"Estroff","year":"1992"},{"key":"b17-0020194","series-title":"Pediatr Neurosurg","doi-asserted-by":"publisher","first-page":"86","DOI":"10.1159\/000120942","article-title":"Relationship between cerebellar appearance and function in children with Dandy-Walker syndrome","volume":"23","author":"Gerszten","year":"1995"},{"key":"b18-0020194","series-title":"Pediatr Neurosci","doi-asserted-by":"publisher","first-page":"38","DOI":"10.1159\/000120299","article-title":"Dandy-Walker syndrome and associated anomalies","volume":"13","author":"Golden","year":"1987"},{"key":"b19-0020194","series-title":"Neurology","doi-asserted-by":"publisher","first-page":"771","DOI":"10.1212\/WNL.22.8.771","article-title":"The Dandy-Walker syndrome. A clinicopathological study based on 28 cases","volume":"22","author":"Hart","year":"1972"},{"key":"b20-0020194","series-title":"Neuroradiology in Infants and Children","first-page":"1014","author":"Harwood-Nash","year":"1976"},{"key":"b21-0020194","series-title":"J Neurosurg","doi-asserted-by":"publisher","first-page":"515","DOI":"10.3171\/jns.1984.61.3.0515","article-title":"The Dandy-Walker malformation. A review of 40 cases","volume":"61","author":"Hirsch","year":"1984"},{"key":"b22-0020194","series-title":"Am J Med Genet","doi-asserted-by":"publisher","first-page":"152","DOI":"10.1002\/ajmg.1231","article-title":"Dandy-Walker variant in Coffin-Siris syndrome","volume":"100","author":"Imai","year":"2001"},{"key":"b23-0020194","series-title":"Korean J Radiol","doi-asserted-by":"publisher","first-page":"145","DOI":"10.3348\/kjr.2006.7.2.145","article-title":"Parenchymal neurocutaneous melanosis in association with intraventricular dermoid and Dandy-Walker variant: a case report","volume":"7","author":"Kim","year":"2006"},{"key":"b24-0020194","series-title":"Am J Obstet Gynecol","doi-asserted-by":"publisher","first-page":"1070","DOI":"10.1016\/j.ajog.2005.10.191","article-title":"Diagnosis of inferior vermian hypoplasia by fetal magnetic resonance imaging: potential pitfalls and neurodevelopmental outcome","volume":"194","author":"Limperopoulos","year":"2006"},{"key":"b25-0020194","series-title":"Prenat Diagn","doi-asserted-by":"publisher","first-page":"707","DOI":"10.1002\/pd.1485","article-title":"Outcome of fetal cerebral posterior fossa anomalies","volume":"26","author":"Long","year":"2006"},{"key":"b26-0020194","series-title":"Neuropathol Appl Neurobiol","doi-asserted-by":"publisher","first-page":"344","DOI":"10.1111\/j.1365-2990.2006.00719.x","article-title":"Neuropathological analysis of an asymptomatic adult case with Dandy-Walker variant","volume":"32","author":"Notaridis","year":"2006"},{"key":"b27-0020194","series-title":"Pediatr Neurosurg","doi-asserted-by":"publisher","first-page":"179","DOI":"10.1159\/000120660","article-title":"Diagnosis and management of the Dandy-Walker malformation: 30 years of experience","volume":"18","author":"Osenbach","year":"1992"},{"key":"b28-0020194","series-title":"Ultrasound Obstet Gynecol","doi-asserted-by":"publisher","first-page":"482","DOI":"10.1002\/uog.2748","article-title":"Posterior fossa and vermian morphometry in the characterization of fetal cerebellar abnormalities: a prospective three-dimensional ultrasound study","volume":"27","author":"Paladini","year":"2006"},{"issue":"Suppl 347","key":"b29-0020194","series-title":"Acta Radiol","first-page":"553","article-title":"Developmental defects of the cerebellum. A radiologic and anatomic investigation","author":"Pascual-Castroviejo","year":"1976"},{"key":"b30-0020194","series-title":"Childs Nerv Syst","first-page":"88","article-title":"Dandy-Walker malformation: analysis of 38 cases","volume":"7","author":"Pascual-Castroviejo","year":"1991"},{"key":"b31-0020194","series-title":"Ultrasound Q","doi-asserted-by":"publisher","first-page":"211","DOI":"10.1097\/RUQ.0b013e31814b162c","article-title":"The fetal cerebellar vermis: assessment for abnormal development by ultrasonography and magnetic resonance imaging","volume":"23","author":"Robinson","year":"2007"},{"key":"b32-0020194","series-title":"Am J Med Genet","doi-asserted-by":"publisher","first-page":"124","DOI":"10.1002\/ajmg.1320370129","article-title":"Dandy-Walker variant malformation, spastic paraplegia, and mental retardation in two sibs","volume":"37","author":"Stoll","year":"1990"},{"key":"b33-0020194","series-title":"Clin Dysmorphol","doi-asserted-by":"publisher","first-page":"193","DOI":"10.1097\/00019605-200107000-00008","article-title":"De novo interstitial long arm deletion of chromosome 3 with facial dysmorphism, Dandy-Walker variant malformation and hydrocephalus","volume":"10","author":"Sudha","year":"2001"},{"key":"b34-0020194","series-title":"Brain","doi-asserted-by":"publisher","first-page":"352","DOI":"10.1093\/brain\/9.3.352","article-title":"The lateral recesses of the fourth ventricle: their relation to certain cysts and tumors of the cerebellum and to occipital meningocele","volume":"9","author":"Sutton","year":"1886"},{"key":"b35-0020194","series-title":"Arch Neurol Psychiatry","doi-asserted-by":"publisher","first-page":"583","DOI":"10.1001\/archneurpsyc.1942.02290100083008","article-title":"Congenital atresia of the foramens of Luschka and Magendie","volume":"48","author":"Taggart","year":"1942"},{"key":"b36-0020194","series-title":"Clin Dysmorphol","doi-asserted-by":"publisher","first-page":"15","DOI":"10.1097\/00019605-200201000-00003","article-title":"X-linked inheritance of Dandy-Walker variant","volume":"11","author":"Wakeling","year":"2002"},{"key":"b37-0020194","series-title":"Pediatr Neurol","doi-asserted-by":"publisher","first-page":"347","DOI":"10.1016\/S0887-8994(03)00016-X","article-title":"Clinical spectrum associated with cerebellar hypoplasia","volume":"28","author":"Wassmer","year":"2003"},{"key":"b38-0020194","series-title":"Cytogenet Genome Res","doi-asserted-by":"publisher","first-page":"235","DOI":"10.1159\/000094206","article-title":"Proof of partial imbalances 6q and 11q due to maternal complex balanced translocation analyzed by microdissection of multicolor labeled chromosomes (FISH-MD) in a patient with Dandy-Walker variant","volume":"114","author":"Weimer","year":"2006"}],"container-title":["Journal of Neurosurgery: Pediatrics"],"original-title":[],"link":[{"URL":"https:\/\/thejns.org\/view\/journals\/j-neurosurg-pediatr\/2\/3\/article-p194.xml","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/thejns.org\/downloadpdf\/journals\/j-neurosurg-pediatr\/2\/3\/article-p194.xml","content-type":"text\/html","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/thejns.org\/downloadpdf\/journals\/j-neurosurg-pediatr\/2\/3\/article-p194.xml","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,4,15]],"date-time":"2023-04-15T14:02:36Z","timestamp":1681567356000},"score":1,"resource":{"primary":{"URL":"https:\/\/thejns.org\/view\/journals\/j-neurosurg-pediatr\/2\/3\/article-p194.xml"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2008,9]]},"references-count":38,"journal-issue":{"issue":"3"},"URL":"https:\/\/doi.org\/10.3171\/ped\/2008\/2\/9\/194","relation":{},"ISSN":["1933-0707","1933-0715"],"issn-type":[{"value":"1933-0707","type":"print"},{"value":"1933-0715","type":"electronic"}],"subject":[],"published":{"date-parts":[[2008,9]]}}}