{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,3]],"date-time":"2026-05-03T03:31:46Z","timestamp":1777779106113,"version":"3.51.4"},"reference-count":16,"publisher":"BMJ","issue":"4","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Med Genet"],"published-print":{"date-parts":[[1973,12]]},"DOI":"10.1136\/jmg.10.4.398","type":"journal-article","created":{"date-parts":[[2008,11,10]],"date-time":"2008-11-10T14:50:51Z","timestamp":1226328651000},"page":"398-399","source":"Crossref","is-referenced-by-count":5,"title":["Letter: The Crouzon syndrome."],"prefix":"10.1136","volume":"10","author":[{"given":"K L","family":"Jones","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"suffix":"Jr","given":"M M","family":"Cohen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[1973,12,1]]},"reference":[{"key":"ref_1","article-title":"An syndromes","unstructured":"An syndromes."},{"key":"ref_2","author":"Series","unstructured":"Series. (In press.)"},{"key":"ref_3","author":"Juberg, R.C.; Chambers, S.R.","year":"1973","unstructured":"Juberg, R. C. and Chambers, S. R. (1973)."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"107","DOI":"10.1542\/peds.23.1.107","article-title":"pedigree Craniofacial dysostosis of Crouzon: a case with emphasis on heredity","volume":"23","unstructured":"pedigree Craniofacial dysostosis of Crouzon: a case with emphasis on heredity. Pediatrics, 23, 107-112.","journal-title":"Pediatrics"},{"key":"ref_5","article-title":"Two terms, proptosis and exophthalmos, have often been used interchangeably to indicate abnormal protrusion of the eyeball. At least one authority","author":"(Hilson,","year":"1962","unstructured":"Two terms, proptosis and exophthalmos, have often been used interchangeably to indicate abnormal protrusion of the eyeball. At least one authority (TrevorRoper, 1962) has suggested that exophthalmos should refer to forward protrusion of the eyeball alone; proptosis should indicate displacement of the lids and orbital contents as well as the eyeball. Most authors have used exophthalmos in their description of patients with craniofacial dysostosis (Hilson, 1947; Flippen, 1950; Pinkerton and Pinkerton, 1952; Dodge et al, 1959; Shiller, 1959; Vulliamy and Normandale, 1966). However, proptosis would seem to be the more accurate term because the whole content of the orbit would be expected to be abnormally projected as a result of orbital shallowness.","journal-title":"et al; has suggested that exophthalmos should refer to forward protrusion of the eyeball alone; proptosis should indicate displacement of the lids and orbital contents as well as the eyeball. Most authors have used exophthalmos in their description of patients with craniofacial dysostosis"},{"key":"ref_6","article-title":"Although quantitative determination of the degree of protrusion has been possible by various designs of exophthalmometer for quite some time (the Hertel instrument dates from 1905), clinical judgement by standing behind the patient and looking over the forehead or by viewing from the side, has usually sufficed. Thus, most reports do not include quantitative assessment of the ocular abnormality but rather state only the result of clinical opinion. Consequently, some patients who by appearance have abnormal protrusion might by measurement and comparison be clearly beyond normal limits, but others might not. Normal values for 10 to 14 year olds have only recently been published","year":"1972","unstructured":"Although quantitative determination of the degree of protrusion has been possible by various designs of exophthalmometer for quite some time (the Hertel instrument dates from 1905), clinical judgement by standing behind the patient and looking over the forehead or by viewing from the side, has usually sufficed. Thus, most reports do not include quantitative assessment of the ocular abnormality but rather state only the result of clinical opinion. Consequently, some patients who by appearance have abnormal protrusion might by measurement and comparison be clearly beyond normal limits, but others might not. Normal values for 10 to 14 year olds have only recently been published (Gerber et al, 1972)."},{"key":"ref_7","article-title":"The exophthalmometric measurement of the female sib we reported (Juberg and Chambers, 1973) falls within the normal range for her age according to published data (Gerber et al, 1972), but we cannot classify her male sib because of the lack of comparative data. In our report we indicated the lack of abnormality of the position of the eyes of the two sibs judged by adult standards. However, the standards published are for Caucasians, and our patients are Negroes","volume-title":"preparation) have recently found by examination of 1219 Negroes, aged 5 to 11 years, that the means for both the internal intercanthal and the external intercanthal distances are greater than the normal values reported for Caucasians. If the width of the orbit as reflected by canthal measurements differs in racial groups, then it seems reasonable that the depth may also differ. Normal values, when they become available, will permit comparison of measurements in patients such as we reported","author":"R. C. Juberg, F. G. Sholte, Jr.","unstructured":"The exophthalmometric measurement of the female sib we reported (Juberg and Chambers, 1973) falls within the normal range for her age according to published data (Gerber et al, 1972), but we cannot classify her male sib because of the lack of comparative data. In our report we indicated the lack of abnormality of the position of the eyes of the two sibs judged by adult standards. However, the standards published are for Caucasians, and our patients are Negroes. R. C. Juberg, F. G. Sholte, Jr., and W. J. Touchstone (in preparation) have recently found by examination of 1219 Negroes, aged 5 to 11 years, that the means for both the internal intercanthal and the external intercanthal distances are greater than the normal values reported for Caucasians. If the width of the orbit as reflected by canthal measurements differs in racial groups, then it seems reasonable that the depth may also differ. Normal values, when they become available, will permit comparison of measurements in patients such as we reported."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"98","DOI":"10.1542\/peds.23.1.98","article-title":"Craniofacial dysostosis: Crouzon's disease","volume":"23","author":"Dodge, Jr., H.W.; Wood, M.W.; Kennedy, R.L.J.","year":"1959","unstructured":"Dodge, H. W., Jr., Wood, M. W., and Kennedy, R. L. J. (1959). Craniofacial dysostosis: Crouzon's disease. Pediatrics, 23, 98-106.","journal-title":"Pediatrics"},{"key":"ref_9","first-page":"90","article-title":"Cranio-facial dysostosis of Crouzon. Report of a case in which the malformation occurred in four generations","volume":"5","author":"Flippen, Jr, J.H.","year":"1950","unstructured":"Flippen, J. H., Jr. (1950). Cranio-facial dysostosis of Crouzon. Report of a case in which the malformation occurred in four generations. Pediatrics, 5, 90-96.","journal-title":"Pediatrics"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1016\/S0022-3476(72)80303-2","article-title":"Normal standards for exophthalmomentry in children 10 to 14 years of age: Relation to age, height, weight, and sexual maturation","volume":"81","author":"Gerber, F.R.; Taylor, F.H.; deLevie, M.; Drash, A.L.; Kenny, F.M.","year":"1972","unstructured":"Gerber, F. R., Taylor, F. H., deLevie, M., Drash, A. L., and Kenny, F. M. (1972). Normal standards for exophthalmomentry in children 10 to 14 years of age: Relation to age, height, weight, and sexual maturation. Journal of Pediatrics, 81, 327-329.","journal-title":"Journal of Pediatrics"},{"key":"ref_11","first-page":"64","article-title":"Scaphocephaly in a brother and sister. An unusual incidence of this form of premature synostosis of the skull bones","volume":"6","author":"Hilson, D.","year":"1947","unstructured":"Hilson, D. (1947). Scaphocephaly in a brother and sister. An unusual incidence of this form of premature synostosis of the skull bones. Clinical Proceedings, 6, 64-70.","journal-title":"Clinical Proceedings"},{"key":"ref_12","first-page":"89","article-title":"An autosomal recessive form of craniofacial dysostosis (the Crouzon Syndrome)","volume-title":"J7ournal of Medical Genetics","volume":"10","author":"Juberg, R.C.; Chambers, S.R.","year":"1973","unstructured":"Juberg, R. C. and Chambers, S. R. (1973). An autosomal recessive form of craniofacial dysostosis (the Crouzon Syndrome). J7ournal of Medical Genetics, 10, 89-94."},{"key":"ref_13","first-page":"500","article-title":"Hereditary craniofacial dysplasia","volume":"35","author":"Pinkerton, 0. D.; Pinkerton, F. J","year":"1952","unstructured":"Pinkerton, 0. D. and Pinkerton, F. J. (1952). Hereditary craniofacial dysplasia. American_Journal of Ophthalmology, 35, 500-506.","journal-title":"American_Journal of Ophthalmology"},{"key":"ref_14","first-page":"107112","article-title":"Craniofacial dysostosis of Crouzon. A case report and pedigree with emphasis on heredity","volume":"23","author":"Shiller, J.G.","year":"1959","unstructured":"Shiller, J. G. (1959). Craniofacial dysostosis of Crouzon. A case report and pedigree with emphasis on heredity. Pediatrics, 23, 107112.","journal-title":"Pediatrics"},{"key":"ref_15","article-title":"Ophthalmology: A Textbookfor Diploma Students","author":"Trevor-Roper, P.D.","year":"1962","unstructured":"Trevor-Roper, P. D. (1962). Ophthalmology: A Textbookfor Diploma Students. Year Book, Chicago."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"375","DOI":"10.1136\/adc.41.218.375","article-title":"Cranio-facial dysostosis in a Dorset family","volume":"41","author":"Vulliamy, D.G.; Normandale, P.A.","year":"1966","unstructured":"Vulliamy, D. G. and Normandale, P. A. (1966). Cranio-facial dysostosis in a Dorset family. Archives of Disease in Childhood, 41, 375-382.","journal-title":"Archives of Disease in Childhood"}],"container-title":["Journal of Medical Genetics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/jmg.10.4.398","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,18]],"date-time":"2025-10-18T05:37:28Z","timestamp":1760765848000},"score":1,"resource":{"primary":{"URL":"https:\/\/jmg.bmj.com\/lookup\/doi\/10.1136\/jmg.10.4.398"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1973,12]]},"references-count":16,"journal-issue":{"issue":"4","published-online":{"date-parts":[[1973,12,1]]},"published-print":{"date-parts":[[1973,12]]}},"alternative-id":["10.1136\/jmg.10.4.398"],"URL":"https:\/\/doi.org\/10.1136\/jmg.10.4.398","relation":{},"ISSN":["0022-2593","1468-6244"],"issn-type":[{"value":"0022-2593","type":"print"},{"value":"1468-6244","type":"electronic"}],"subject":[],"published":{"date-parts":[[1973,12]]}}}