{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,5,13]],"date-time":"2025-05-13T14:33:16Z","timestamp":1747146796426,"version":"3.40.5"},"reference-count":20,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2023,5,25]],"date-time":"2023-05-25T00:00:00Z","timestamp":1684972800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Birth Defects Research"],"published-print":{"date-parts":[[2023,7]]},"abstract":"Abstract<\/jats:title>Background<\/jats:title>Symmetric craniorachipagus is an exceedingly rare type of bi\u2010umbilical conjoined twinning, known from only three scantily described cases.<\/jats:p><\/jats:sec>Case<\/jats:title>We identified a fourth, previously described case that was misdiagnosed as janiceps and as pygopagus. It concerned dorsally conjoined twins that were part of a triplet pregnancy, spontaneously born at 22\u2009weeks of gestation. Radiography confirmed union at the occipital craniums and the thoracolumbar vertebral columns. Both twins had their own separate umbilical cords. To delineate the phenotype of craniorachipagus and to differentiate it from rachipagus without cranial involvement, we compared the present case with the previous three reported cases and with the historical literature concerning comparable conditions. Furthermore, we discuss why exceedingly rare conditions such as these are presently underreported in the literature.<\/jats:p><\/jats:sec>Conclusion<\/jats:title>Symmetric craniorachipagus is a type of bi\u2010umbilical conjoined twinning, to date represented by four confirmed cases that share a similar phenotype. This includes dorsal conjunction at the sides of the occipital craniums and the vertebral columns, in the absence of any visceral connections. Details on its etiopathogenesis and apparent lethality await additional case investigations. No unequivocally confirmed cases of symmetric rachipagus without cranial involvement have been reported and its existence in humans has yet to be proven.<\/jats:p><\/jats:sec>","DOI":"10.1002\/bdr2.2204","type":"journal-article","created":{"date-parts":[[2023,5,25]],"date-time":"2023-05-25T08:59:20Z","timestamp":1685005160000},"page":"1174-1180","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Craniorachipagus symmetric conjoined twinning: Identification of a fourth case and delineation of the phenotype"],"prefix":"10.1002","volume":"115","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2452-8307","authenticated-orcid":false,"given":"Roelof\u2010Jan","family":"Oostra","sequence":"first","affiliation":[{"name":"Department of Medical Biology, Section Clinical Anatomy & Embryology, Amsterdam University Medical Centers, location Academic Medical Center University of Amsterdam Amsterdam The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ido","family":"Solt","sequence":"additional","affiliation":[{"name":"Department of Obstetrics and Gynecology, Division of Maternal\u2010Fetal Medicine, Rambam Health Care Campus The Rappaport Faculty of Medicine, Technion Haifa Israel"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1564-0847","authenticated-orcid":false,"given":"Lucas L.","family":"Boer","sequence":"additional","affiliation":[{"name":"Department of Imaging, Section Anatomy and Museum for Anatomy and Pathology Radboud University Medical Center Nijmegen The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2023,5,25]]},"reference":[{"doi-asserted-by":"publisher","key":"e_1_2_7_2_1","DOI":"10.1002\/ajmg.a.61847"},{"key":"e_1_2_7_3_1","first-page":"30","article-title":"\u00c9tude radiologique du squelette d'un monstre double janic\u00e9phale\u2010rachipage","volume":"58","author":"B\u00e9touli\u00e8res P.","year":"1960","journal-title":"Montpellier Medical Journal"},{"doi-asserted-by":"publisher","key":"e_1_2_7_4_1","DOI":"10.1002\/ca.23387"},{"doi-asserted-by":"publisher","key":"e_1_2_7_5_1","DOI":"10.1002\/ca.23725"},{"key":"e_1_2_7_6_1","first-page":"1878","article-title":"Een zeer merkwaardig menschelijk dubbelmonstrum: Cranioamniopaag","volume":"70","author":"Bolk L.","year":"1926","journal-title":"Nederlands Tijdschrift voor Geneeskunde"},{"doi-asserted-by":"publisher","key":"e_1_2_7_7_1","DOI":"10.1159\/000082442"},{"doi-asserted-by":"publisher","key":"e_1_2_7_8_1","DOI":"10.1002\/tera.1420250306"},{"key":"e_1_2_7_9_1","first-page":"221","article-title":"M\u00e9moire sur un monstre double monomphalien de provenance humaine, constituant un genre nouveau d\u00e9sign\u00e9 sous le nom de rachipage","volume":"3","author":"Eudes\u2010Deslongchamps J. A.","year":"1852","journal-title":"Comptes Rendus Des s\u00e9ances de la Soci\u00e9t\u00e9 de Biologie et de Ses Filiales"},{"volume-title":"Histoire g\u00e9n\u00e9rale et particuli\u00e8re des anomalies de l'organisation chez l'homme et les animaux, 2 vols","year":"1836","author":"Geoffroy Saint\u2010Hilaire I.","key":"e_1_2_7_10_1"},{"doi-asserted-by":"publisher","key":"e_1_2_7_11_1","DOI":"10.1159\/000091364"},{"volume-title":"Prodigiorum ac Ostentorum Chronicon. Quae praeter naturae ordinem, motum et operationem, et in superior. et his inferior mundi regionibus ab exordio mundi usque ad haec nostra tempora acciderunt","year":"1557","author":"Lycosthenes C.","key":"e_1_2_7_12_1"},{"volume-title":"Multiple pregnancy. Epidemiology, gestation & perinatal outcome","year":"2005","author":"Oleszczuk J. 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L.","year":"1971","journal-title":"American Journal of Diseases of Children"},{"key":"e_1_2_7_20_1","first-page":"1","volume-title":"Nieuwe Verhandelingen der Eerste Klasse van het Koninklijk Nederlandsch Instituut","author":"Vrolik W.","year":"1840"},{"doi-asserted-by":"publisher","key":"e_1_2_7_21_1","DOI":"10.1002\/uog.26016"}],"container-title":["Birth Defects Research"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/bdr2.2204","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,16]],"date-time":"2023-08-16T22:35:07Z","timestamp":1692225307000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/bdr2.2204"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,5,25]]},"references-count":20,"journal-issue":{"issue":"12","published-print":{"date-parts":[[2023,7]]}},"alternative-id":["10.1002\/bdr2.2204"],"URL":"https:\/\/doi.org\/10.1002\/bdr2.2204","archive":["Portico"],"relation":{},"ISSN":["2472-1727","2472-1727"],"issn-type":[{"type":"print","value":"2472-1727"},{"type":"electronic","value":"2472-1727"}],"subject":[],"published":{"date-parts":[[2023,5,25]]},"assertion":[{"value":"2023-01-02","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-05-15","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2023-05-25","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}