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Early identification of pituitary dysfunction in congenital nasal pyriform aperture stenosis: recommendations based
Suet Ching Chen1, Helen McDevitt, W Andrew Clement
1Department of Paediatric Endocrinology, Royal Hospital for Sick Children, Glasgow, UK.
Insights
Congenital nasal pyriform aperture stenosis (CNPAS) patients need early pituitary evaluation. Monitoring growth for one year can predict pituitary dysfunction, minimizing surgical risks.
Area of Science:
- Pediatric Endocrinology
- Otolaryngology
- Medical Genetics
Background:
- Congenital nasal pyriform aperture stenosis (CNPAS) is linked to midline abnormalities and upper airway obstruction.
- Previous studies indicate a high incidence of pituitary dysfunction in CNPAS patients (40%).
- Current diagnostic and follow-up protocols for pituitary insufficiency in CNPAS are inconsistent.
Purpose of the Study:
- To establish guidelines for early identification of pituitary insufficiency in CNPAS patients.
- To minimize surgical risks associated with undiagnosed pituitary dysfunction.
- To develop a patient stratification strategy for appropriate follow-up.
Main Methods:
- Retrospective review of 20 CNPAS patients treated between 2000 and 2014.
- Analysis of diagnostic approaches to pituitary axis evaluation.
- Correlation of clinical findings with pituitary dysfunction incidence.
Main Results:
- Pituitary dysfunction was identified in 15% of patients, often diagnosed later in childhood.
- Hypoglycemia and conjugated hyperbilirubinemia were significant predictors of pituitary dysfunction.
- Height SDS at 1 year effectively identified patients with growth hormone deficiency.
Conclusions:
- Recommend MRI and baseline endocrine tests for all CNPAS patients at diagnosis.
- Suggest growth monitoring for at least one year post-diagnosis.
- Low or falling height SDS at one year is a reliable indicator of potential pituitary dysfunction.
Background:
Congenital nasal pyriform aperture stenosis (CNPAS) is an increasingly recognised cause of upper airway obstruction associated with midline abnormalities. Studies have described pituitary dysfunction in 40% of patients. We aimed to develop guidelines for: (a) the early identification of pituitary insufficiency to minimise surgical risk and (b) to stratify patients for follow-up.
Methods:
Retrospective case note review of patients with CNPAS between 2000 and 2014 in a tertiary paediatric unit.
Results:
20 patients (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 pituitary axis at diagnosis. Pituitary dysfunction was identified in 3 (15%) patients, 2 of whom were found during evaluation of short stature in mid-late childhood. Hypoglycaemia and conjugated hyperbilirubinaemia, but not the degree of stenosis, were highly predictive of pituitary dysfunction (p < 0.05). Available height standard deviation score (SDS) data at 1 year of 70% of our patients identified both of the late-diagnosed growth hormone-deficient patients, with SDS of -2.6 and -3.6, respectively.
Conclusion:
All CNPAS patients 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 pituitary dysfunction.
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