Early Identification of @entity4611 in Congenital Nasal Pyriform Aperture XXXX : Recommendations Based on Experience in a Single Centre.

@entity1 @entity851 @entity4611 @entity2124 @entity4614 @entity601 @entity850 @entity1224
BACKGROUND: Congenital nasal pyriform aperture @entity850 (CNPAS) is an increasingly recognised cause of @entity1224 associated with @entity601 . Studies have described @entity4611 in 40% of @entity1 . We aimed to develop guidelines for: (a) the early identification of @entity4614 to minimise surgical risk and (b) to stratify @entity1 for follow-up. METHODS: Retrospective case note review of @entity1 with CNPAS between 2000 and 2014 in a tertiary paediatric unit. RESULTS: 20 @entity1 (12 female:8 male) were analysed; 16 were diagnosed during the neonatal period while 4 were diagnosed later. There was no consistent approach in the evaluation of the @entity4611 at diagnosis. @entity4611 was identified in 3 (15%) @entity1 , 2 of whom were found during evaluation of @entity851 in mid-late childhood. Hypoglycaemia and conjugated hyperbilirubinaemia, but not the degree of @entity850 , were highly predictive of @entity4611 (p < 0.05). Available height standard deviation score (SDS) data at 1 year of 70% of our @entity1 identified both of the late-diagnosed @entity2124 -deficient @entity1 , with SDS of -2.6 and -3.6, respectively. CONCLUSION: All CNPAS @entity1 should have MRI of the brain and baseline endocrine investigations at diagnosis. Growth monitoring for at least 1 year is recommended as low, or falling, height SDS at 1 year is a good predictor of @entity4611 .    2015 S. Karger AG, Basel.
