{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,14]],"date-time":"2026-10-14T04:17:44Z","timestamp":1791951464842,"version":"4.3.4"},"reference-count":21,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2024,10,1]]},"abstract":"<jats:sec>\n<jats:title>OBJECTIVE<\/jats:title>\n<jats:p>Hindbrain herniation (HH) is a clinical prerequisite for prenatal repair of myelomeningocele\/myeloschisis; however, a subset of patients lack HH on initial fetal imaging and may ultimately progress to exhibit herniation on subsequent prenatal or postnatal imaging. The authors sought to explore the cohort of patients without HH at the time of initial fetal consultation for myelomeningocele\/myeloschisis repair to define their clinical characteristics and outcome.<\/jats:p>\n<\/jats:sec>\n<jats:sec>\n<jats:title>METHODS<\/jats:title>\n<jats:p>From July 2016 to July 2022, patients evaluated at the Children\u2019s Hospital of Philadelphia Center for Fetal Diagnosis and Treatment for myelomeningocele\/myeloschisis were classified into two cohorts: those with and those without HH. The diagnosis of HH was obtained from prenatal and postnatal MRI. The osseous lesion level, prenatal sac volume, and prenatal ventricular size was obtained from fetal ultrasound. The fronto-occipital horn ratio was measured on the first postnatal ultrasound. Ambulation status was obtained from postnatal evaluation in the spina bifida clinic.<\/jats:p>\n<\/jats:sec>\n<jats:sec>\n<jats:title>RESULTS<\/jats:title>\n<jats:p>A total of 176 patients with prenatal HH had postnatal follow-up, of whom 95 (54%) had HH resolution and 81 (46%) had herniation persistence. Of 73 patients without prenatal HH, 9 (12%) had herniation on subsequent prenatal imaging while 64 (88%) had no herniation on prenatal imaging. Of these 64 patients, 11 (17%) had postnatal HH, 32 (50%) had no postnatal herniation, and 21 (33%) were lost to follow-up or the pregnancy was terminated. For patients without HH throughout, the sac volume was larger (9 cm<jats:sup>3<\/jats:sup>) than those who had herniation progression or initial herniation; however, the rate of talipes was not significantly different among the groups. The majority of patients were also ambulators (with assistive devices or independent), and the atrial diameter was also &lt; 10 mm for most patients. Overall, 53% of those with initial HH compared with 35% with progression of herniation required CSF diversion, while only 25% of those without herniation required diversion.<\/jats:p>\n<\/jats:sec>\n<jats:sec>\n<jats:title>CONCLUSIONS<\/jats:title>\n<jats:p>This study demonstrates the natural history of HH in patients with a prenatal diagnosis of myelomeningocele\/myeloschisis. The majority of patients without any herniation had larger sac sizes but not higher rates of talipes and smaller ventricles and were ambulatory. These findings improve the ability to guide families during prenatal consultation.<\/jats:p>\n<\/jats:sec>","DOI":"10.3171\/2024.6.peds24170","type":"journal-article","created":{"date-parts":[[2024,10,19]],"date-time":"2024-10-19T02:28:13Z","timestamp":1729304893000},"page":"1-6","source":"Crossref","is-referenced-by-count":3,"title":["The clinical significance of lack of hindbrain herniation in fetal myelomeningocele\/myeloschisis patients"],"prefix":"10.3171","author":[{"given":"Tracy M.","family":"Flanders","sequence":"first","affiliation":[{"name":"Division of Neurosurgery, Department of Surgery,"},{"name":"Richard D. Wood Jr. Center for Fetal Diagnosis and Treatment, and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria A.","family":"Punchak","sequence":"additional","affiliation":[{"name":"Division of Neurosurgery, Department of Surgery,"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Edward R.","family":"Oliver","sequence":"additional","affiliation":[{"name":"Richard D. Wood Jr. Center for Fetal Diagnosis and Treatment, and"},{"name":"Department of Radiology, Children\u2019s Hospital of Philadelphia, Pennsylvania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sierra D.","family":"Land","sequence":"additional","affiliation":[{"name":"Richard D. Wood Jr. Center for Fetal Diagnosis and Treatment, and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sabrina J.","family":"Flohr","sequence":"additional","affiliation":[{"name":"Richard D. Wood Jr. Center for Fetal Diagnosis and Treatment, and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tom A.","family":"Reynolds","sequence":"additional","affiliation":[{"name":"Richard D. Wood Jr. Center for Fetal Diagnosis and Treatment, and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katie M.","family":"Schmidt","sequence":"additional","affiliation":[{"name":"Richard D. Wood Jr. Center for Fetal Diagnosis and Treatment, and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Danielle D.","family":"Ertz","sequence":"additional","affiliation":[{"name":"Richard D. Wood Jr. Center for Fetal Diagnosis and Treatment, and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julie S.","family":"Moldenhauer","sequence":"additional","affiliation":[{"name":"Richard D. Wood Jr. Center for Fetal Diagnosis and Treatment, and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"N. Scott","family":"Adzick","sequence":"additional","affiliation":[{"name":"Richard D. Wood Jr. Center for Fetal Diagnosis and Treatment, and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gregory G.","family":"Heuer","sequence":"additional","affiliation":[{"name":"Division of Neurosurgery, Department of Surgery,"},{"name":"Richard D. Wood Jr. Center for Fetal Diagnosis and Treatment, and"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1754","reference":[{"key":"b1","series-title":"Nat Rev Dis Primers","first-page":"15007","article-title":"Spina bifida","volume":"1","author":"Copp AJ","year":"2015","unstructured":"Copp AJ, Adzick NS, Chitty LS, Fletcher JM, Holmbeck GN, Shaw GM. Spina bifida. Nat Rev Dis Primers. 2015;1:15007."},{"issue":"1","key":"b2","series-title":"Pediatr Neurosci","first-page":"1","article-title":"The cause of Chiari II malformation: a unified theory","volume":"15","author":"McLone DG","year":"1989","unstructured":"McLone DG, Knepper PA. The cause of Chiari II malformation: a unified theory. Pediatr Neurosci. 1989;15(1):1\u201312."},{"issue":"7-8","key":"b3","series-title":"Childs Nerv Syst","first-page":"540","article-title":"The Chiari II malformation: cause and impact","volume":"19","author":"McLone DG","year":"2003","unstructured":"McLone DG, Dias MS. The Chiari II malformation: cause and impact. Childs Nerv Syst. 2003;19(7-8):540\u2013550."},{"issue":"1","key":"b4","series-title":"Neuropathol Appl Neurobiol","first-page":"29","article-title":"The association of hydrocephalus and Arnold-Chiari malformation with spina bifida in the fetus","volume":"6","author":"Bell JE","year":"1980","unstructured":"Bell JE, Gordon A, Maloney AF. The association of hydrocephalus and Arnold-Chiari malformation with spina bifida in the fetus. Neuropathol Appl Neurobiol. 1980;6(1):29\u201339."},{"issue":"11","key":"b5","series-title":"N Engl J Med","first-page":"993","article-title":"A randomized trial of prenatal versus postnatal repair of myelomeningocele","volume":"364","author":"Adzick NS","year":"2011","unstructured":"Adzick NS, Thom EA, Spong CY,  A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med. 2011;364(11):993\u20131004."},{"issue":"5","key":"b6","series-title":"Neurosurgery","first-page":"637","article-title":"Detailed analysis of hydrocephalus and hindbrain herniation after prenatal and postnatal myelomeningocele closure: report from a single institution","volume":"86","author":"Flanders TM","year":"2020","unstructured":"Flanders TM, Heuer GG, Madsen PJ,  Detailed analysis of hydrocephalus and hindbrain herniation after prenatal and postnatal myelomeningocele closure: report from a single institution. Neurosurgery. 2020;86(5):637\u2013645."},{"issue":"5","key":"b7","series-title":"Ultraschall Med","first-page":"514","article-title":"Hindbrain herniation and banana and lemon sign after open fetal myelomeningocele repair: when do these signs disappear and is shunting predictable?","volume":"42","author":"Vonzun L","year":"2021","unstructured":"Vonzun L, Winder FM, Meuli M,  Hindbrain herniation and banana and lemon sign after open fetal myelomeningocele repair: when do these signs disappear and is shunting predictable? Ultraschall Med. 2021;42(5):514\u2013519."},{"issue":"5","key":"b8","series-title":"AJNR Am J Neuroradiol","first-page":"1031","article-title":"Hindbrain herniation in Chiari II malformation on fetal and postnatal MRI","volume":"38","author":"Nagaraj UD","year":"2017","unstructured":"Nagaraj UD, Bierbrauer KS, Zhang B, Peiro JL, Kline-Fath BM. Hindbrain herniation in Chiari II malformation on fetal and postnatal MRI. AJNR Am J Neuroradiol. 2017;38(5):1031\u20131036."},{"issue":"6","key":"b9","series-title":"J Neurosurg Pediatr","first-page":"613","article-title":"Prenatal surgery for myelomeningocele and the need for cerebrospinal fluid shunt placement","volume":"16","author":"Tulipan N","year":"2015","unstructured":"Tulipan N, Wellons JC III, Thom EA,  Prenatal surgery for myelomeningocele and the need for cerebrospinal fluid shunt placement. J Neurosurg Pediatr. 2015;16(6):613\u2013620."},{"issue":"6","key":"b10","series-title":"Neuropediatrics","first-page":"359","article-title":"Reversal of hindbrain herniation after maternal-fetal surgery for myelomeningocele subsequently impacts on brain stem function","volume":"39","author":"Danzer E","year":"2008","unstructured":"Danzer E, Finkel RS, Rintoul NE,  Reversal of hindbrain herniation after maternal-fetal surgery for myelomeningocele subsequently impacts on brain stem function. Neuropediatrics. 2008;39(6):359\u2013362."},{"issue":"5","key":"b11","series-title":"Am J Obstet Gynecol","first-page":"450.e1","article-title":"Preschool neurodevelopmental outcome of children following fetal myelomeningocele closure","volume":"202","author":"Danzer E","year":"2010","unstructured":"Danzer E, Gerdes M, Bebbington MW, Zarnow DM, Adzick NS, Johnson MP. Preschool neurodevelopmental outcome of children following fetal myelomeningocele closure. Am J Obstet Gynecol. 2010;202(5):450.e1\u2013450.e9."},{"issue":"3","key":"b12","series-title":"J Pediatr Surg","first-page":"451","article-title":"Correction of hindbrain herniation and anatomy of the vermis after in utero repair of myelomeningocele in sheep","volume":"38","author":"Bouchard S","year":"2003","unstructured":"Bouchard S, Davey MG, Rintoul NE, Walsh DS, Rorke LB, Adzick NS. Correction of hindbrain herniation and anatomy of the vermis after in utero repair of myelomeningocele in sheep. J Pediatr Surg. 2003;38(3):451\u2013458."},{"issue":"19","key":"b13","series-title":"JAMA","first-page":"1826","article-title":"Improvement in hindbrain herniation demonstrated by serial fetal magnetic resonance imaging following fetal surgery for myelomeningocele","volume":"282","author":"Sutton LN","year":"1999","unstructured":"Sutton LN, Adzick NS, Bilaniuk LT, Johnson MP, Crombleholme TM, Flake AW. Improvement in hindbrain herniation demonstrated by serial fetal magnetic resonance imaging following fetal surgery for myelomeningocele. JAMA. 1999;282(19):1826\u20131831."},{"issue":"2","key":"b14","series-title":"Am J Obstet Gynecol","first-page":"256.e1","article-title":"The Management of Myelomeningocele Study: full cohort 30-month pediatric outcomes","volume":"218","author":"Farmer DL","year":"2018","unstructured":"Farmer DL, Thom EA, Brock JW III,  The Management of Myelomeningocele Study: full cohort 30-month pediatric outcomes. Am J Obstet Gynecol. 2018;218(2):256.e1\u2013256.e13."},{"issue":"2","key":"b15","series-title":"Pediatrics","first-page":"e20191544","article-title":"Prenatal repair of myelomeningocele and school-age functional outcomes","volume":"145","author":"Houtrow AJ","year":"2020","unstructured":"Houtrow AJ, Thom EA, Fletcher JM,  Prenatal repair of myelomeningocele and school-age functional outcomes. Pediatrics. 2020;145(2):e20191544."},{"issue":"2","key":"b16","series-title":"Oper Neurosurg (Hagerstown)","first-page":"158","article-title":"Improved postoperative metrics with modified myofascial closure in fetal myelomeningocele repair","volume":"18","author":"Flanders TM","year":"2020","unstructured":"Flanders TM, Madsen PJ, Pisapia JM,  Improved postoperative metrics with modified myofascial closure in fetal myelomeningocele repair. 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AJR Am J Roentgenol. 2018;211(6):1376\u20131380."}],"container-title":["Journal of Neurosurgery: Pediatrics"],"original-title":[],"link":[{"URL":"https:\/\/thejns.org\/view\/journals\/j-neurosurg-pediatr\/aop\/article-10.3171-2024.6.PEDS24170\/article-10.3171-2024.6.PEDS24170.xml","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/thejns.org\/downloadpdf\/journals\/j-neurosurg-pediatr\/aop\/article-10.3171-2024.6.PEDS24170\/article-10.3171-2024.6.PEDS24170.xml","content-type":"text\/html","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/thejns.org\/downloadpdf\/journals\/j-neurosurg-pediatr\/aop\/article-10.3171-2024.6.PEDS24170\/article-10.3171-2024.6.PEDS24170.xml","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,12,28]],"date-time":"2024-12-28T02:29:20Z","timestamp":1735352960000},"score":1,"resource":{"primary":{"URL":"https:\/\/thejns.org\/view\/journals\/j-neurosurg-pediatr\/aop\/article-10.3171-2024.6.PEDS24170\/article-10.3171-2024.6.PEDS24170.xml"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,10,1]]},"references-count":21,"URL":"https:\/\/doi.org\/10.3171\/2024.6.peds24170","relation":{},"ISSN":["1933-0707","1933-0715"],"issn-type":[{"value":"1933-0707","type":"print"},{"value":"1933-0715","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,10,1]]}}}