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Two siblings with midline field defects and Hirschsprung disease: Variable expression of Toriello-Carey or new syndrome?

✍ Scribed by Jespers, A. ;Buntinx, I. ;Melis, K. ;Vaerenberg, M. ;Janssens, G.


Publisher
John Wiley and Sons
Year
1993
Tongue
English
Weight
382 KB
Volume
47
Category
Article
ISSN
0148-7299

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✦ Synopsis


We describe 2 sibs with multiple congenital anomalies. The main manifestations include hypoplasia of the corpus callosum and/or cerebellar hypoplasia, Robin sequence, pharyngeal and laryngeal hypoplasia, abnormal ears, excessive neck skin, cardiac defect, and Hirschsprung disease. The presence in 2 sibs born to healthy, consanguineous parents suggests autosomal recessive inheritance. These anomalies must have arisen during blastogenesis; the syndrome resembles most the condition described in 1988