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Congenital Nasal Pyriform Aperture Stenosis (CNPAS): Sublabial Approach to Surgical Correction

Congenital nasal pyriform aperture stenosis (CNPAS) is defined as inadequate formation of the pyriform apertures forming the bony nasal openings resulting in respiratory distress and cyanosis soon after birth. Some clues such as worsening distress during feeding and improvement during crying may indicate a nasal cause of respiratory distress rather than distal airway etiology. Inability or difficulty passing a small tube through the nasal cavities may suggest CNPAS. The presenting clinical features of CNPAS can be  similar to other obstructive nasal airway anomalies such as choanal atresia. Diagnosis is confirmed via CT scan with a total nasal aperture less than 11mm.

CNPAS may occur in isolation or it may be a sign of other developmental abnormalities such as holoprosencephaly, anterior pituitary abnormalities, or encephalocele. Some physical features of holoprosencephaly include closely spaced eyes, facial clefts, a single maxillary mega incisor, microcephaly, nasal malformations, and brain abnormalities (i.e. incomplete separation of the cerebral hemispheres, absent corpus callosum, and pituitary hormone deficiencies). It is important to rule out other associated abnormalities to ensure optimal treatment and intervention.

Conservative treatment of CNPAS includes humidification, nasal steroids, nasal decongestants and reflux control. Failure of conservative treatment defined by respiratory or feeding difficulty necessitates more aggressive intervention. The most definitive treatment for CNPAS is surgical intervention to enlarge the pyriform apertures.

Contributors:

Adam Johnson MD, PhD
Abby Nolder MD

1. After informed consent was obtained, the patient was brought to the operating room and placed supine on the operating table. Correct patient and procedure were identified and general anesthesia was induced. Once spontaneous ventilation was achieved, the patient was turned 90 degrees to the operating surgeon. 2. Demonstrated inability to pass a 2.5 mm telescope through the right and left nostril 3. A sublabial incision was outlined and injected with 1% lidocaine with 1:100,000 epinephrine. The mucosa was incised through to the periosteum of the maxilla. 4. Blunt and sharp dissection were performed using a Freer and Cottle elevator to raise the periosteum up to the pyriform aperture. The floor of the nose was gently elevated in the same periosteal plane. A round knife was then used to complete this dissection. 5. Once the pyriform aperture was completely exposed, a 2 mm diamond bur was used to enlarge the pyriform aperture bilaterally. Continuous irrigation was used and care was taken not to injure the nasal lacrimal duct. 6. Once this was complete, the soft tissue replaced and nasal endoscopy was performed with a 2.5 mm Hopkins operating telescope. We were then able to freely pass the scope through the choanae. 7. We then used the urethral sounds to serially dilate the nasal cavity on the bilaterally from 8 to 20 french. 8. A nostril retainer, size 2, was then placed in the nose and secured to the septum using one 4-0 Ethilon suture. Sublabial incision was closed in layers with 4-0 chromic gut. 9. The nostril retainer will remained in place for 1 week. After removal a trial of extubation occurred. Recommend perioperative steroids prior to extubation attempts.
Clinical presentation of respiratory distress, cyanosis, and feeding difficulty attributable to the nasal obstruction along with the diagnosis of pyriform aperture stenosis as evidenced by CT and/or MRI imaging modalities.
Absence of respiratory distress or cyanosis and absence of pyriform aperture stenosis are contraindications to this procedure. Presence of other compromising airway anomalies
Endoscopic capabilities Afrin soaked gauze Nasal stents Urethral sounds 2mm coarse diamond burr and hand drill
Thorough physical exam Ultrasound of brain Echocardiogram Chest/abdominal X-ray MRI brain w/o contrast Maxillofacial CT w/o contrast Newborn screen
Sublabial sulcus Nasal lacrimal duct Nasal septum
N/A
Bleeding, inflammation leading to compromised nasal airway, infection, and re-stenosis
none
ENT Department of Arkansas Children's Hospital
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3056623/ http://onlinelibrary.wiley.com/doi/10.1097/00005537-200205000-00025/abstract;jsessionid=B8E45F78E7CF337155FC771FE4742147.f03t04 https://www.uptodate.com/contents/congenital-anomalies-of-the-nose?source=search_result&search=piriform%20aperture%20stenosis&selectedTitle=1~150#H8 https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3574011/

Review Congenital Nasal Pyriform Aperture Stenosis (CNPAS): Sublabial Approach to Surgical Correction.

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