Abstract
A nine day old boy who had the Pierre Robin syndrome also had an unusual associated hand malformation consisting of bilateral clinodactyly of the index finger. A supernumerary phalanx was inserted between the second metacarpal and the proximal phalanx of both index fingers with a radial deviation of the same phalanx. The same hand malformation has been previously described in three patients who had either the Pierre Robin syndrome or isolated cleft palate. On the basis of ours and the three previous cases, the existence of a new palato-digital syndrome is suggested.
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Bergsma: Birth Defects Atlas and Compendium. White Plains, Georgia: The National Foundation March of Dimes 1973
Catel, W.: Differentialdiagnose von Krankheitssymptomen bei Kindern und Jugendlichen, 3rd ed., vol. I, 218. Stuttgart: Thieme 1961
Centa, A., Rovei, S.: Dismelia grave non talidomidica associata a sindrome di Pierre Robin. Pathologica57, 245 (1965)
Cinque, N. A., Ena, J.: Un raro caso di Pierre Robin con artogripposi congenita e calcificazioni intraepatiche. Minerva Pediatr.17, 1809 (1965)
Holthusen, W.: The Pierre Robin syndrome. Unusual associated developmental defects. Ann. Radiol. (Paris)15, 253 (1972)
La Grutta, A., Fonti, F. E.: Sindrome di Pierre Robin e malformazioni associate. Minerva Pediatr.17, 1910 (1965)
Manzke, H.: Symmetrische Hyperphalangie des zweiten Fingers durch ein akzessorisches Metacarpale. Fortschr. Geb. Roentgenstr. Nuklearmed.105, 425 (1966)
Morger, R.: Zum Pierre Robin Syndrom. Z. Kinderchir.3, 317 (1966)
Passarge, E.: Congenital malformations and maternal diabetes. Lancet1966 I, 324
Randall, P., Krogmann, W. M., Jahins, S.: Pierre Robin and the syndrome that bears his name. Cleft Palate J.2, 237 (1965)
Smith, J., Stove, F. R.: The Pierre Robin syndrome (glossoptosis, micrognathia, cleft palate). A review of 39 cases with emphasis on associated ocular lesions. Pediatrics27, 128 (1961)
Sacrez, R., Francfort, J. J., Gigonnet, J. M., Beauvais, P., Boll, G.: A propos de la debilitè intellectuel et d'anomalies associees à la triade symtomatique du syndrome de Pierre Robin. Ann. Pediatr.14, 28 (1961)
Walter, H.: Der diastrophische Zwergwuchs. Fortschr. Allg. Klin. Humangenetik2, 31 (1970)
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Silengo, M.C., Franceschini, P., Cerutti, A. et al. Pierre Robin syndrome with hyperphalangism-clinodactylysm of the index finger: A possible new palato-digital syndrome. Pediatr Radiol 6, 178–180 (1977). https://doi.org/10.1007/BF00972113
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DOI: https://doi.org/10.1007/BF00972113