{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,30]],"date-time":"2025-11-30T22:25:23Z","timestamp":1764541523349},"reference-count":19,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2005,9,28]],"date-time":"2005-09-28T00:00:00Z","timestamp":1127865600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Prenatal Diagnosis"],"published-print":{"date-parts":[[2005,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Infantile cortical hyperostosis (ICH) is benign and self\u2010limiting when it presents near or after birth but is usually lethal when it presents earlier.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We present the clinical, ultrasonic, radiographic and pathologic findings in an instructive case of early onset prenatal ICH.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>A 40\u2010year\u2010old G2P1 woman delivered spontaneously at 22 weeks' gestation. Prenatal ultrasounds showed rib and mandibular abnormalities as well as short humeri. Post\u2010mortem radiographs showed asymmetric hyperostosis in long bones, mandible, scapulae and pelvis with sparing of spine, hands, feet and skull. The affected skeleton showed marked bony sclerosis and ballooning of the diaphyses of the long bones with periosteal sclerosis. A complete autopsy showed characteristic histologic findings in affected bones. Previous reports at 20+ weeks have described anasarca, fetal hydrops, hepatomegaly, and pulmonary hypoplasia. In our case, there was no hydrops\/anasarca; hepatomegaly, due to massive extramedullary hematopoiesis with marked myeloid hyperplasia, combined with ribcage abnormalities, caused mild pulmonary hypoplasia.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>We hypothesize that early onset fetal ICH is usually lethal because massive hepatic myeloid hyperplasia sequentially causes: (1) hepatomegaly and, in conjunction with rib abnormalities, mild pulmonary hypoplasia, (2) sinusoidal and pre\u2010sinusoidal portal hypertension followed by ascites\/hydrops, and (3) ascites\/hydrops\u2010induced severe pulmonary hypoplasia. Copyright \u00a9 2005 John Wiley &amp; Sons, Ltd.<\/jats:p><\/jats:sec>","DOI":"10.1002\/pd.1235","type":"journal-article","created":{"date-parts":[[2005,9,28]],"date-time":"2005-09-28T14:49:08Z","timestamp":1127918948000},"page":"939-944","source":"Crossref","is-referenced-by-count":11,"title":["Prenatal infantile cortical hyperostosis (Caffey's disease): a \u2018hepatic myeloid hyperplasia\u2010pulmonary hypoplasia sequence\u2019 can explain the lethality of early onset cases"],"prefix":"10.1002","volume":"25","author":[{"suffix":"Jr","given":"James R.","family":"Wright","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michiel C.","family":"Van den Hof","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marian B.","family":"Macken","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2005,9,28]]},"reference":[{"key":"e_1_2_1_2_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2002-01-0282"},{"key":"e_1_2_1_3_1","first-page":"1","article-title":"Infantile cortical hyperostosis preliminary report on a new syndrome","volume":"54","author":"Caffey J","year":"1945","journal-title":"Am J Roentgenol"},{"key":"e_1_2_1_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/0378-3782(96)01765-3"},{"key":"e_1_2_1_5_1","doi-asserted-by":"publisher","DOI":"10.1080\/00313020120083304"},{"key":"e_1_2_1_6_1","first-page":"865","volume-title":"Fetal and Perinatal Pathology","author":"Dimmick JE","year":"1998"},{"key":"e_1_2_1_7_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01959789"},{"key":"e_1_2_1_8_1","first-page":"137","article-title":"Antenatal\u2010onset cortical hyperostosis and nonimmune hydrops","volume":"16","author":"Herman TE","year":"1996","journal-title":"J Perinatol"},{"key":"e_1_2_1_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/pd.1970120803"},{"key":"e_1_2_1_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0248-8663(86)80006-6"},{"key":"e_1_2_1_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9343(91)90513-W"},{"key":"e_1_2_1_12_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2002-06-1699"},{"key":"e_1_2_1_13_1","doi-asserted-by":"publisher","DOI":"10.1136\/pgmj.65.764.394"},{"key":"e_1_2_1_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/0378-3782(92)90138-7"},{"key":"e_1_2_1_15_1","doi-asserted-by":"publisher","DOI":"10.1242\/dev.129.17.4147"},{"key":"e_1_2_1_16_1","first-page":"221","article-title":"Portal hypertension caused by intra\u2010hepatic block during chronic lymphoid leukemia","volume":"24","author":"Pauwels M","year":"2000","journal-title":"Gastroenterol Clin Biol"},{"key":"e_1_2_1_17_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.20062"},{"key":"e_1_2_1_18_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF02389527"},{"key":"e_1_2_1_19_1","first-page":"61","volume-title":"Radiology of Syndromes, Metabolic disorders, and Skeletal Dysplasias","author":"Taybi H","year":"1996"},{"key":"e_1_2_1_20_1","doi-asserted-by":"publisher","DOI":"10.1097\/00019605-199301000-00012"}],"container-title":["Prenatal Diagnosis"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fpd.1235","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/obgyn.onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/pd.1235","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,11,17]],"date-time":"2023-11-17T02:54:04Z","timestamp":1700189644000},"score":1,"resource":{"primary":{"URL":"https:\/\/obgyn.onlinelibrary.wiley.com\/doi\/10.1002\/pd.1235"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2005,9,28]]},"references-count":19,"journal-issue":{"issue":"10","published-print":{"date-parts":[[2005,10]]}},"alternative-id":["10.1002\/pd.1235"],"URL":"https:\/\/doi.org\/10.1002\/pd.1235","archive":["Portico"],"relation":{},"ISSN":["0197-3851","1097-0223"],"issn-type":[{"value":"0197-3851","type":"print"},{"value":"1097-0223","type":"electronic"}],"subject":[],"published":{"date-parts":[[2005,9,28]]}}}