Etiology of Pediatric Nasal Obstruction
The major causes of nasal obstruction in children are listed in ▶ Table 16.1. Some are dealt with in more detail in Chapter 17 but are included here for the sake of completeness.
Congenital anomalies | Acquired disorders |
Skeletal:
Nasal masses:
| Inflammatory/infective:
Traumatic:
Neoplastic:
Vascular Metabolic Autoimmune Idiopathic |
16.3 Congenital Anomalies
16.3.1 Skeletal
Arhinia
Arhinia or complete nasal agenesis is extremely rare, with only 43 cases reported in the literature. It can be associated with other craniofacial anomalies due to a common embryological origin affecting the development of the nose and other structures. Partial arhinia is more common and is seen most often in facial clefting disorders (see ▶ 27).
The development of the nose occurs between the third and eighth week in utero and involves the superior frontal process and bilateral maxillary processes in the formation of the midface. The nasal placodes themselves develop in the fifth week and consist of medial and lateral nasal swellings with nasal pits between. The medial swellings fuse to form the septum, and cells within the pits migrate backward to form the nasal cavities. It is not entirely clear how arhinia arises. There may be a failure of development of the medial nasal swelling or even an overgrowth and premature fusion of this same structure resulting in an atretic plate. Most cases have normal chromosomal analysis, but there is a single case report of familial arhinia and three individuals around the world have shown some abnormal karyotyping.
Complete arhinia presents as an airway emergency at birth, which can be alleviated with a Guedel oral airway or by oral endotracheal intubation if required. Partial arhinia can also cause severe airway obstruction and feeding difficulties that may require intervention in the first few weeks or months of life. A tracheostomy may need to be considered in either situation.
Reconstruction of the nose is remarkably difficult; multiple challenges include securing a stable skeletal structure with a functional mucosa and the appropriate skin cover. A bone-anchored prosthesis can be considered and gives an excellent cosmetic result.
Choanal Atresia
Blockage of the posterior choanae can be unilateral or bilateral and is generally of a mixed bony/membranous type. It is rare, with an incidence of approximately 1 in 7,000 live births. The condition can be an isolated lesion or occur in association with other congenital anomalies. One or more features of the CHARGE syndrome (coloboma, heart defects, atresia choanae, retardation of growth, genital anomalies, and ear abnormalities) may be present. Some children have the full CHARGE syndrome due to mutations in the CHD7 gene on chromosome 8. A cardiac echo, renal ultrasound, audiology, and ophthalmology review are recommended.
Bilateral obstruction presents as an acute airway emergency at birth with cyclical cyanosis (blue spells relieved by crying).
If the diagnosis is suspected, the midwife or the attending pediatrician will find that a nasal catheter will not pass into the nasopharynx as it is held up by the atretic plate. A useful confirmatory test is to place a cold steel spatula under the baby’s nose and check for misting. If no air is exhaled through the nose, there is no misting; conversely, if there is misting, the nasal airway is patent, although it can still be partly obstructed. Flexible endoscopy can demonstrate the atretic plate. The correct placement of an oral airway can alleviate symptoms, although endotracheal intubation may be required.
If there is a strong suspicion of choanal atresia, the baby should be transferred to an appropriate center. Computed tomography (CT) scanning with 1-mm cuts (following nasal suction and the application of topical decongestant drops) aids surgical planning ( ▶ Fig. 16.1. The diagnosis is confirmed by rigid endoscopy. A 120-degree Hopkins rod provides an excellent view and facilitates surgical correction ( ▶ Fig. 16.2). Early surgery is important, and although the baby’s airway can be safely managed with a Guedel oral airway and with careful observation in a neonatal unit, it proves impossible to establish oral feeding until the atresia is corrected.
Unilateral pathology does not usually cause issues until the child is older. There is persistent mucopurulent discharge in the absence of a foreign body. Occasionally, there can be airway or feeding difficulties in an affected baby, and earlier correction is indicated.
Fig. 16.1 Computed tomography scan (axial view) showing bilateral choanal atresia (mixed bony/membranous).
Fig. 16.2 Bilateral choanal atresia as viewed from postnasal space with a 120-degree endoscope.
Management of Choanal Atresia
Different operative approaches for repair are described as follows 1, 2:
Transpalatal surgery is less common now, although it may be useful in those with significant craniofacial anomalies such as Treacher Collins’ syndrome where the dimensions of the nostrils and postnasal space provide extremely limited access.
Transnasal repairs can either involve the use of a 120-degree endoscope in the oropharynx to look back at the postnasal space with the instruments and drill being introduced through the nostrils, or the repair can be carried out with the endoscope and instruments in the nasal cavities directly.
The factors affecting successful repair have been debated in the literature. Nasal stenting post bilateral repair in the neonate is reported as standard in a series of patients, 3 whereas Teissier et al 4 who reviewed 80 cases over a 9-year period feel it is only required for 2 days in this group and not at all in unilateral cases. Ibrahim et al 5 reported on 21 cases, an equal division of unilateral and bilateral, without stents and found similar success rates to the series quoted previously. If stents are used, soft tubes (Ivory Portex, Smiths Medical) are recommended. Treatment of associated gastroesophageal reflux disease and daily washing with sodium chloride solution were shown to positively affect outcome. 4 Regular suction to clear secretions is important. Wide surgical excision with resection of the posterior aspect of the vomer and early (1 week postrepair) removal of crusting/granulation tissue under general anesthesia (GA), if required, are reported as beneficial. 4
Mitomycin C has been proposed as useful to reduce granulation tissue formation and hence fibrosis. Kubba et al 3 in a retrospective study found no difference in the outcome when 22 patients treated with mitomycin C were compared with 24 control patients. They suggested that the use of mitomycin C might just be a marker of refractory disease since it seems to be used in cases of children with poorer overall outcome.
A specific review of refractory cases 6 found an incidence of almost 10% of cases requiring repeated procedures. Risk factors for restenosis were found to be male gender, bilateral disease, associated congenital anomalies, low birth weight, and small stent size. There was no obvious relationship between the duration of stent placement and restenosis. Restenosis tended to occur early on, and so generally if the choanae were patent after the initial treatment pathway was completed, then routine outpatient follow-up was not required and the child can be reviewed as symptoms dictate.
The complications reported tend to relate to issues related to the stents, if used. Local irritation and infection are common while there are reports of injury to the nasal alar margins resulting in cosmetic deformity and even stenosis of the anterior nares.
Pyriform Aperture Stenosis
This is a very rare cause of nasal obstruction seen in the newborn and is related to bony overgrowth of the nasal process of the maxilla. Diagnosis is suggested clinically and by the inability to pass a narrow gauge nasogastric tube or 2.2-mm endoscope through the anterior part of the nose. Confirmation is through a CT scan with an aperture width of <11mm (measured on an axial CT at the level of the inferior meatus) in a term neonate ( ▶ Fig. 16.3).
There is a link between this condition and holoprosencephaly (a defect in development of brain and midline structures) and so affected individuals should have a formal review for other midline anomalies, including an assessment of function of the hypothalamic–pituitary axis and consideration of a brain magnetic resonance imaging (MRI). The solitary median maxillary central incisor syndrome is the least severe form of holoprosencephaly and three series have reported incidence rates of this condition with pyriform aperture stenosis (PAS) of 28, 50, and 60%, respectively. 7, 8, 9
This anomaly will not be seen at birth but is evident on CT scan and is suggested on examination by a single central maxillary alveolus, absent upper labial frenulum, and arch-shaped lower lip. Associated urogenital and cardiac anomalies have been described.
Fig. 16.3 Computed tomography scan (axial view) showing pyriform aperture stenosis.
Management of Pyriform Aperture Stenosis
Initial treatment for PAS involves medical therapy in the form of saline irrigation and the short-term use of decongestants or nasal steroid drops. The maximum duration for such treatment is suggested to be 2 weeks. 9 A nasopharyngeal airway can also be considered, although dilation under GA may be required to allow satisfactory placement, and softer tubes can easily be kinked by the bony deformity.
If there is significant respiratory distress or failure to thrive, then surgical repair may be necessary. A recent review has found that those with an aperture of less than 5 mm required surgical intervention. 8
A sublabial approach is recommended with a gingivobuccal sulcus incision and elevation of the soft tissue and periosteum to expose the pyriform aperture.
The bony narrowing is drilled away (diamond bur), with care being taken posterolaterally to avoid the nasolacrimal ducts and inferiorly to avoid the tooth buds. The mucoperiosteal flap is then replaced.
As with choanal atresia, the use of nasal stenting postoperatively can be considered, with a period of 7 days to 4 weeks being reported. 7, 8
Satisfactory outcomes are reported in the three largest series reported, although small numbers are involved due to the rarity of the condition.
Complications including adhesions, septal ulceration, and septal perforation are described. Careful postoperative care with nasal irrigation, avoidance of aggressive suction, and management of gastroesophageal reflux are recommended to minimize such issues. 7
16.3.2 Nasal Masses
Cysts
The most common form of nasal cyst is a dermoid. This is the most common midline nasal mass.
There are other rarer types of cyst are as follows:
Nasolacrimal duct cysts can cause nasal obstruction or related eye symptomatology, and if so, endoscopic removal is recommended.
Nasolaveolar cysts are developmental nonodontogenic maxillary cysts that usually present with due to aesthetic concerns but can result in obstruction. Excision through a sublabial approach or transnasal endoscopic marsupialization has been described.
Dentigenerous cysts can present in the nose if they arise from the crown of an unerupted tooth in the upper jaw. Treatment involves liason with the dental team.
Tornwaldt’s cyst arises in the pharyngeal recess in the midline of the posterior wall of the nasopharynx. This recess ends adjacent to the adenoids and is usually lined with normal pharyngeal mucosa. Cystic transformation of this forms the lesions that bear the name of the individual who initially described it.
The hairy polyp sometimes seen in neonates and originally thought to be a cyst has been shown to contain mature ectodermal and mesodermal tissue and is therefore more correctly described as a bigerminal choristoma.
Dermoid Cysts
These originate from ectoderm and mesoderm and so can contain all the structures of normal skin. There is debate as to how they develop. In the embryo, there are two areas that are potential candidates: the fonticulus frontalis space, which is between the developing frontal and nasal bones, and the prenasal space, which is between the nasal bones and the developing septum ( ▶ Fig. 16.4). It is unclear as to whether dermoids occur due to inclusion of dermal tissue at the fonticulus frontalis or from persistent dura in the prenasal space, which then makes contact with the skin and forms a cyst.
Clinical presentation is either as a mass in the midline, which gradually enlarges, or as a small pit on the skin surface, which can be anywhere from the glabella to the philtrum. The pit represents a sinus tract and so can intermittently discharge. Hair can also be present in the pit ( ▶ Fig. 16.5).
The investigation of choice is an MRI scan to assess for single or multiple cysts and also to delineate any intracranial component ( ▶ Fig. 16.6). A CT scan can be useful to demonstrate the bony anatomy.
The cyst can become infected and early surgical excision is recommended:
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