Three female patients were initially given conservative ODDI. Two other patients underwent surgery as initial CNPAS treatment but were placed on ODDI afterward due to ongoing symptoms from nasal obstruction. See Table 1.
Patient 1 presented after birth with cyanosis during feeding. CT showed 4.7mm pyriform aperture diameter and the interuncinate distance was 11mm. ODDI was begun after diagnosis and was tapered from two drops to one and from twice daily to once daily before she was removed. Improvement continued and surgery was unnecessary. At 1year, the PA diameter and interuncinate distance had increased to 8.8 and 15.5 mm.
Patient 2 presented with nasal obstruction at birth with CNPAS. 5 days of Afrin raised the oxygen saturation percentage from the 70's to the high 90's, but obstruction and dysphagia persisted intermittently. The patient started ODDI and after 9 days was tapered to one drop per nostril per day. Nasal obstruction and feeding improved. She was eventually weaned off ODDI.
Patient 3 presented at birth with sleep apnea, desaturations in the 80's, and nasal obstruction ODDI was commenced after diagnosis. At 2 weeks she was breathing freely through her nose, retractions subsided and dysphagia improved moderately. By two months, however, she was not receiving ODDI consistently due to caregiver noncompliance and her symptoms returned. After reinstating ODDI at one drop per nostril per day, symptoms again improved and stabilized. She was weaned completely off ODDI after 6 months.
Patient 4 was intubated when 3 days old due to severe respiratory failure from CNPAS. Surgical correction was performed at six weeks. Nasal obstruction persisted post-surgically, however, and ODDI was instituted. After little improvement, his dose was increased to four times daily. The increase improved the obstruction and allowed adequate nasal breathing. Drops were tapered until 1.5 years old. After weaning ODDI, he was sleeping restlessly. Although he had no significant sleep apnea, sleep disordered breathing persisted, and at three years old this was treated with Flonase prn, which helped alleviate his symptoms.
Patient 5 is a 4year old female who presented with obstructive sleep apnea, nasal obstruction, and a history of CNPAS. Although his outside CT was not available for review, he had undergone a sublabial repair at an outside institution, which initially improved his symptoms , but nasal obstruction and sleep disordered breathing eventually developed. He was treated with prn afrin postoperatively for several months, which was not successful at treating his symptoms. The child was a candidate for either surgical PA repair or ODDI with adenotonsillectomy. Her parents elected for the latter. Nasal obstruction continued, however, and she required valvular stenosis repair. Breathing had improved after this surgery and she required no further treatment for nasal obstruction. The patient was subsequently lost to follow up.
Results
References
Five children evaluated for CNPAS at our tertiary referral center from 2006-2009 were reviewed retrospectively. CT measurements of the pyriform aperture diameter and the midnasal diameter (interuncinate distance) were documented. All patients, initially received two drops in each nostril twice daily. Dosage was modified and tapered based on symptoms. Pediatric endocrinology was consulted for each patient to help diagnose and manage side effects. Outcome measures included the cessation of airway obstruction, ability to taper steroid drops, necessitation of surgical intervention, and evidence of adrenal suppression.
Methods and Materials
For most neonatal patients with CNPAS, surgical is generally not recommended until they reach 10 pounds, 10 inches, or 10 grams of hemoglobin. 9 Conservative therapies or ventilation support help manage symptoms until surgery can be performed in most cases. ODDI offers an effective alternative to surgery. This case series is the first to show ODDI to be an effective option of therapy for some patients with CNPAS. ODDI was effective in patients 1, 2, and 3 from the onset and averted surgery entirely rather than deferring it. In patients 2 and 3, ODDI provided rapid breathing improvements and was weaned within 2 weeks.
Patients 4 and 5 had severe cases of CNPAS that required surgery initially. ODDI was effective in alleviating post-surgical nasal obstruction in these patients. While ODDI helped Patient 4 avoid further surgery, patient 5 only had mild improvements from ODDI and required vestibular stenosis repair as well as an adenotonsillectomy.
Ingestion or absorption of dexamethasone can cause adrenal insufficiency, immunosuppression, or metabolic side effects, and patients should be closely monitored by a pediatric endocrinologist until further data on safety and side effects are available. 7 While no side effects were observed in patients 1, 2, 3, or 5, patient 4 required a longer course of ODDI, which resulted in iatrogenic Cushing symptoms and adrenal suppression. These effects are likely minimized by close monitoring by endocrinology, low dosing, and careful, slow tapering. Further studies on the safety and side effects are required.