Congenital nasal pyriform aperture stenosis associated with central diabetes insipidus.

We describe a child who has central diabetes insipidus associated with congenital nasal pyriform aperture stenosis without any apparent anterior pituitary dysfunction. This association further strengthens the concept that congenital nasal pyriform aperture stenosis may be a microform of holoprosencephaly.

[1]  K. Lee,et al.  Solitary maxillary central incisor and congenital nasal pyriform aperture stenosis , 1998, European Journal of Pediatrics.

[2]  A. Drake,et al.  Congenital Nasal Pyriform Aperture Stenosis in the Monozygotic Twin of a Child with Holoprosencephaly , 1998, Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery.

[3]  S. Scherer,et al.  Mutations in the human Sonic Hedgehog gene cause holoprosencephaly , 1996, Nature Genetics.

[4]  C. Garel,et al.  Nasal pyriform aperture stenosis and absence of the anterior pituitary gland: report of two cases. , 1996, The Journal of pediatrics.

[5]  Kristina J. Liu,et al.  Report of two cases , 1995 .

[6]  J. Friedberg,et al.  Congenital nasal pyriform aperture stenosis as a presenting feature of holoprosencephaly. , 1995, International journal of pediatric otorhinolaryngology.

[7]  R. Ward,et al.  Congenital nasal pyriform aperture stenosis. Isolated abnormality vs developmental field defect. , 1992, Archives of otolaryngology--head & neck surgery.

[8]  V. P. Johnson.  Holoprosencephaly: a developmental field defect. , 1989, American journal of medical genetics.

[9]  M. Münke,et al.  Clinical, cytogenetic, and molecular approaches to the genetic heterogeneity of holoprosencephaly. , 1989, American journal of medical genetics.

[10]  C. Myer,et al.  Congenital nasal pyriform aperture stenosis , 1989, The Laryngoscope.

[11]  J. Fryns,et al.  Single central maxillary incisor and holoprosencephaly. , 1987, American journal of medical genetics.

[12]  W. Demyer,et al.  THE FACE PREDICTS THE BRAIN: DIAGNOSTIC SIGNIFICANCE OF MEDIAN FACIAL ANOMALIES FOR HOLOPROSENCEPHALY (ARHINENCEPHALY). , 1964, Pediatrics.

[13]  J. Gilbert,et al.  Growth of the nose and the septorhinoplastic problem in youth. , 1958, A.M.A. archives of otolaryngology.

[14]  R. Marion,et al.  Nasal pyriform aperture stenosis and the holoprosencephaly spectrum. , 1994, International journal of pediatric otorhinolaryngology.

[15]  M. Kappy,et al.  Sublingual administration of desmopressin. Effectiveness in an infant with holoprosencephaly and central diabetes insipidus. , 1987, American journal of diseases of children.