title: Congenital nasal pyriform aperture stenosis with semilobar holoprosencephaly.
abstract: We describe a child who has congenital nasal pyriform aperture stenosis with single maxillary central incisor, holoprosencephaly and central diabetes insipidus without any apparent anterior pituitary dysfunction. Conservative management of the congenital nasal pyriform aperture stenosis is adopted and management of diabetes insipidus is described. A literature review is undertaken.
authors.affiliation: Department of Paediatrics, Kwong Wah Hospital, Waterloo Road, Hong Kong, Kowloon, Hong Kong SAR, PR China.
authors.forename: E Y T
authors.initials: EY
authors.lastname: Chan
authors.forename: D K K
authors.initials: DK
authors.lastname: Ng
authors.forename: A S F
authors.initials: AS
authors.lastname: Chong
authors.forename: Y
authors.initials: Y
authors.lastname: Hui
authors.forename: Y M
authors.initials: YM
authors.lastname: Fu
mesh_terms: D000015:Abnormalities, Multiple / Q000175:diagnosis*; D001999:Bronchoscopy; D003894:Deamino Arginine Vasopressin / Q000627:therapeutic use; D020790:Diabetes Insipidus, Neurogenic / Q000175:diagnosis* / Q000628:therapy; D004750:Enteral Nutrition; D005260:Female; D016142:Holoprosencephaly / Q000175:diagnosis*; D006801:Humans; D007231:Infant, Newborn; D007442:Intubation, Intratracheal; D008279:Magnetic Resonance Imaging; D009296:Nasal Cavity / Q000002:abnormalities*; D015508:Nasal Obstruction / Q000151:congenital* / Q000175:diagnosis* / Q000628:therapy; D012076:Renal Agents / Q000627:therapeutic use; D014057:Tomography, X-Ray Computed; D014667:Vasopressins / Q000627:therapeutic use
chemical_list: D012076:Renal Agents; D014667:Vasopressins; D003894:Deamino Arginine Vasopressin