Kosztolányi G, Weisenbach J, Méhes K
Department of Pediatrics, University Medical School, Pécs, Hungary.
Am J Med Genet. 1995 Sep 11;58(3):213-6. doi: 10.1002/ajmg.1320580303.
We have evaluated an infant with a striking combination of craniofacial anomalies, arachnodactyly, and severe developmental failure. She died at the age of 5 months during a recurrent apneic episode. She also had protruding eyes, downward slant of palpebral fissures, short upturned nose, midface hypoplasia, micrognathia, extreme under-development of the epiglottis, and severe feeding difficulties. The patient closely resembled four other previously reported patients. It is suggested that these five patients represent the same malformation syndrome, a well-recognizable separate entity. Our patient also had a pericentric inversion of chromosome 10; a possible association of this with the phenotype cannot be excluded.