phenylephrine-hydrochloride and De-Lange-Syndrome

Cornelia de Lange syndrome is a collection of congenital anomalies. Its victims present considerable burdens in home care, not the least of which is feeding. The use of the modified Orticochea pharyngoplasty for correction of nasal food emission affords the opportunity for application of plastic surgical principles to seemingly unrelated clinical events. The salient features of the syndrome are presented, and the literature is reviewed.

Topics: Child, Preschool; De Lange Syndrome; Eating; Female; Humans; Nose; Pharynx; Surgical Flaps; Velopharyngeal Insufficiency

Three cases of de Lange's syndrome are described. This condition is characterized by generally severe mental retardation, reduced stature, mild microcephaly, hypertrichosis, various anomalies of hands and feet, and a peculiar facies. The most outstanding features of the latter are the low forehead, profuse, generally confluent eyebrows, abundant long eyelashes, eyes that frequently slant downwards and outwards in antimongoloid fashion, pug nose with prominent anteverted nostrils, increased distance between nose and vermilion border of upper lip, slight reduction in size of chin, and often abnormally low-placed ears. The etiology of de Lange's syndrome is at present unknown.

Topics: Adolescent; Body Height; Child; Congenital Abnormalities; De Lange Syndrome; Ear Deformities, Acquired; Ear, External; Eye; Eyebrows; Facial Expression; Hand Deformities; Hirsutism; Humans; Hypertrichosis; Intellectual Disability; Lip; Mandible; Microcephaly; Nose; Nose Deformities, Acquired