Pediatric Rhinology: Developmental Aspects and Surgery
Summary
Surgery of the nasal supporting skeleton generally aims to improve the function and form of the nose. In children, a third ambition is relevant: surgery should restore normal growth of the nose. This ambition requires knowledge of the postnatal development and age-specific anatomy of the nasal skeleton, the morphogenetic processes involved in nasal development, and the way these may be affected by trauma or restored by surgery. This chapter discusses the clinical aspects—diagnostics, indications, and surgery.
Introduction
The three sections in this chapter deal, respectively, with surgical aspects of the growing nasal skeleton, congenital anomalies, and acquired conditions.
Surgery of the nasal skeleton generally aims to improve the form and function of the nose; in children, a third ambition is most relevant: restoring normal growth and avoiding additional negative effects on later development of the nasal skeleton.
This ambition should be based on knowledge of the evolution of dimensional growth in boys and girls, the age-specific anatomy, the morphogenetic processes involved in the growing nasal skeleton, and the way these may be affected by fractures and cartilage necrosis and how they may be restored by surgery. The most common congenital anomalies of the nose are described in this chapter, along with an approach to the assessment and management of nasal anomalies in the neonatal period, including choanal atresia, craniofacial anomalies, and meningoencephaloceles. Many of the acquired conditions affecting the nose in childhood are similar to those seen in adults, but their assessment and management differ substantially. Allergic rhinitis, epistaxis, and tumors all occur in children, but the differences with their adult equivalents are important. We will outline these, together with the acquired conditions specific to childhood, such as adenoid hypertrophy. Finally, it is worth noting that many of the surgical techniques used in children will be very familiar to the rhinologist, although the indications for using them in children may be very different. The external rhinoplasty approach, for example, is ideal for the excision of congenital anomalies of the external nose, whereas functional endoscopic sinus surgery (FESS) is mostly confined to the management of chronic sinus disease in association with cystic fibrosis (CF).
Postnatal Growth of the Nose
Dimensional Aspects
The face of a newborn is marked by the small dimensions of the nose, maxilla, and mandible compared with the relatively large size of the brain skull at that age. In children and teenagers, the higher growth rate of the skeleton of the mid- and lower face will result in the adult profile ( Fig. 33.1 ).
Longitudinal studies demonstrated an adolescent growth spurt of the external nose in boys around the age of 13 years. In girls, the maximum growth velocity of the nose seemed to vary from 8 to 12 years of age.1
Other studies demonstrated height, depth, and inclination to be essentially completed in girls by the age of 16 years, while continuing to increase up to and even beyond 18 years of age in boys.2 Different conclusions were drawn from a study in the Aegean region where nasal dimensions reached maturity in boys at the age of 15 and in girls at 12 years.3
Anatomy of the Newborn
In the newborn, the septum cartilage reaches from the columella to the sphenoid. The upper lateral cartilages extend in the cranial direction under the nasal bones to merge with the cartilaginous anlage of the anterior cranial base ( Fig. 33.2 ). The cartilaginous septum and upper lateral cartilages are parts of an unpaired T-shaped structure, the septodorsal cartilage.4 The nasal bones originate from mesenchymal cells and are formed by desmal ossification.
The first signs of ossification of the septum cartilage preceding the formation of the perpendicular plate are found near the anterior cranial base. The anlage of the vomeral bone is represented by small areas of desmal ossification between the inferior margin of the septum and the palatal bone, with extensions (vomeral alae) on both sides of the cartilaginous septum.
The septum cartilage shows a specific pattern of thicker and thinner areas.5 The transverse diameter appears to vary from 0.4 to 3.5 mm. The thickest cartilage is found most posteriorly, where the septum is based on the sphenoid; from here, two zones of thick cartilage extend in an anterior direction, reaching to the anterior nasal spine and extending under the nasal dorsum. A slightly thickened columellar rim will contribute to the support of the nasal tip ( Fig. 33.2b,c ).
In a neonate, the anatomy of the nasal skeleton is quite specific but will gradually acquire more adult features during further growth.
Anatomical Development in Children: Clinically Relevant Aspects
In the first years of life, the intracranial parts of the dorsolateral cartilages show gradual regression; simultaneously, the bony cribriform plate is formed by desmal ossification of mesenchymal cells. Consequently, the anterior base of the skull at this age is the most vulnerable part of the superior wall of the nasal cavity. In 4-year-old children, the dorsolateral cartilages may still be found running as far as the nasofrontal suture, while in (late) teenagers and adults, the overlap by nasal bones is usually reduced to 5–10 mm.4
Ossification of the cartilaginous septum will gradually progress from near the anterior cranial base in a ventrocaudal direction. In young children, the anterior rim of the perpendicular plate is still located intracranially ( Fig. 33.3 ). This should be respected when surgery of the nasal septum is indicated in a child.
Whereas ossification of the septum cartilage contributes to dimensional growth of the bony perpendicular plate, extracartilaginous ossification results in expansion of the vomerine alae at either side of the cartilaginous septum. Both processes will result in an overlap of the expanding vomerine alae and the ossifying front of the perpendicular plate ( Fig. 33.4 ). The age at which this junction has been realized is thought to vary between 10 and 14 years. The junction of the cartilaginous septum, the perpendicular plate, and the vomer show considerable interindividual variation ( Fig. 33.5 ). The vomer includes, next to the vomeral wings, a basal part extending between the inferior edge of the cartilaginous septum, palate, and choanal rim of the nasal septum.
The sagittal dimensions of the cartilaginous septum initially demonstrate a rapid increase and reach their adult size during the first 2 to 3 years of age.6 From then on, proliferation of cells and expansion of the intercellular matrix in the septal cartilage appear to be balanced by progressive ossification of the septum cartilage, resulting in increasing dimensions of the perpendicular plate ( Fig. 33.4c ). At the same time, remodeling of the septum cartilage leads to changes in form related to a relatively more anterior position of the cartilaginous part. The adult septum does not demonstrate any signs of ongoing ossification or formation of new cartilage ( Fig. 33.4d ).
Radiograph at the age of 10 years. The anterior border of the perpendicular plate (D) demonstrates an intracranial position; the vomer wings (F) along both sides of the septal cartilage (A) do not reach the perpendicular plate (C). Anterior cranial base (B). Radiograph at 30 years. The anterior rim of the perpendicular plate has moved forward under the nasal dorsum and shows a thick caudal rim of bone, which is firmly connected to the septal cartilage to contribute to the support of the cartilaginous nasal dorsum. Histologic section through the septoethmoidal junction at the age of 10 years. Note the active ossification of the cartilaginous septum. At 30 years, there are no signs of ongoing ossification. Anatomical and histologic specimens showing postnatal ossification of the nasal septum.
The zone of thick septal cartilage supporting the nasal dorsum is initially based on the sphenoid and will shift with increasing age to the thickened anterior rim of the perpendicular plate, dorsal to the variable septovomeral junction ( Fig. 33.4 ).
The characteristic structure of the nasal septum is reflected in fracture-prone zones. Fracture lines often tend to show a specific C configuration ( Fig. 33.3 ). They run just superior to the thickened basal rim of the cartilaginous septum, continuing in the perpendicular plate to turn in an anterior direction under the nasal dorsum.7
It has been advocated that surgery of the nasal skeleton should be postponed until the growth rate of the nose has declined to a minimum to avoid negative effects on growth processes. The growth curve of the nose in boys and girls, however, demonstrate obvious gender-related differences. It seems important to recognize that in young children, the anterior rim of the perpendicular plate may still be intracranially positioned, whereas the not yet completely ossified anterior cranial base is most vulnerable.
The surgeon should be aware of the gender-specific growth curve and the specific anatomy of the nasal skeleton in children at different ages.
Morphogenetic Processes and Nasal Growth: Surgical Aspects
Rhinosurgery in children is special. Apart from the agespecific anatomy and dimensions of the nasal skeleton, morphogenetic processes involved in nasal growth are equally important. How these are affected by injury or may be restored by surgical intervention has been studied in animal models, especially in growing rabbits.8–17
Animal Studies
In growing rabbits, as well as in children, the nose and upper jaw grow faster and over a longer period than the brain skull, the cartilaginous septum and the upper lateral cartilages form a T-bar-like structure, and the septum cartilage demonstrates a similar organization with thinner and thicker parts. Moreover, in the nasal septum of both species, a zone of thicker cartilage is found between the sphenoid and nasal dorsum (sphenodorsal zone), and between the sphenoid and the anterior nasal spine (sphenospinal zone).12
Septodorsal Cartilage and Sutural Growth in Rabbits
The role of the septodorsal cartilage in relation to the development of the nose and upper jaw in growing rabbits has been studied in more detail. Some interesting conclusions may be made, as follows:
In growing rabbits, it was demonstrated that the extra growth of the nose and upper jaw primarily depends on the growth of the septodorsal cartilage.
The sphenodorsal zone of thick cartilage is essential for lengthening of the connected dorsal cartilages and indirectly of the overlying nasal bones.
The zone of thicker cartilage between the sphenoid and anterior nasal spine is responsible for lengthening and gradual forward shifting of the upper jaw.
The bilateral dorsal cartilages were found to stabilize the growing nasal septum in a median position. Resection of the anterior part of both lateral cartilages leads to instability of the growing septum with multiple deviations to either side. Shortening of the septum and nasal bones is the final result. After resection of the anterior part of the upper lateral on one side, a deviation of the anterior part of the nasal skeleton (septum and nasal bones) becomes evident during further growth.
The septodorsal cartilage may be required for normal growth of the nasal bones and upper jaw; the bone-forming potential of the sutures in the facial skeleton, however, is equally important. Corresponding sutures on both sides contribute to the symmetrical growth of the midfacial skeleton. When one or more sutures are missing, as in unilateral facial clefts, the growing septum and connected upper jaw will deviate to the noncleft side. Moreover, the maxilla on the cleft side, which is missing the connection with the growing cartilaginous septum, will not be pulled forward by the growing septum and shows in adult animals a retro-position compared with the noncleft side.18 A similar cleft syndrome has been reported in human skulls.19
Surgical Interventions: Wound Healing and Development of the Nasal Skeleton
Mucosal Elevation
Elevation of the septal mucosa seems not to interfere with further growth of the septum. It should, however, be an intraperichondrial procedure, leaving the inner layer of perichondrium firmly connected to the cartilage and elevating the outer layer of perichondrium and mucosa. Both perichondrial layers will reconnect within a few days after mucosal elevation.11
Transection of Septum Cartilage, Release of Interlocked Stresses
After submucosal transection of septum cartilage, the separated parts will demonstrate an immediate overlap. This phenomenon has been ascribed to the release of interlocked stresses within the cartilage.20,21 The overlapping parts may remain firmly reconnected side to side by their perichondrium or later demonstrate increasing deviations and angulations during further growth.22,23
Wound Healing of Cartilage and Perichondrium
Incision or transection of the septum initiates a wound reaction of the cartilage and inner perichondrium ( Fig. 33.6a ). Next to cell death, new cartilage is formed through processes of resorption, proliferation, and redifferentiation. Simultaneously, cells (fibroblasts) from the outer perichondrium migrate into the free space and cover the wound surface. As soon as the regenerating cartilage is overgrown by a new fibrous layer (perichondrium), the process of cartilage formation seems to stop ( Fig. 33.6b ). Elevated perichondrial layers adjacent to larger defects in the septum cartilage will finally be separated by a thin but firm layer of sparsely vascularized connective tissue interspersed with islands of cartilage.
Integration of reimplanted septum cartilage to fill a defect was equally inhibited by overgrowing perichondrium and followed by dislocation of the implant. This ultimately resulted in underdevelopment of the nose.
Sinonasal Wall
Partial resection of the lateral nasal wall enlarging the sinonasal ostium was shown not to interfere with midfacial growth.24
Conclusions from Animal Studies: Relevant for Children?
Despite obvious differences between various species of mammals, the anatomy of the skull—and the facial skeleton in particular—demonstrate similar components. In children and in growing rabbits, the cartilaginous septum and the upper lateral cartilages form a T-bar-like structure. In both species, the septum cartilage demonstrates a specific organization with two important zones of obviously thicker cartilage.
In the rabbit-model, a specific role of various parts of the septum and upper lateral cartilage has been demonstrated in the development of the nose and upper jaw. In the following section, observations on individual patients are presented that may confirm a similar role for the septodorsal cartilage in the postnatal development of the human midface.
Nasal Growth in Children after Injury or Surgery
Observations on individual patients also point to a definite role of the septodorsal cartilage in relation to the development of the nose and upper jaw. Poor wound healing of cartilage is believed to intervene with the repair of fractures and defects.25
Loss of the total cartilaginous septum, following a septal abscess at a young age, interrupts the (extra) growth of the nose and upper jaw, resulting in progressive underdevelopment of the midface ( Fig. 33.7 ).
The younger the child at the time septum cartilage has been destructed, the greater are the effects on the development of the nose and maxilla.26
Partial loss of the cartilaginous septum may not appear to interfere with normal nasal development when the (sphenospinal, sphenodorsal, columellar) zones of thicker cartilage remain intact.
Incomplete healing of fractures of the cartilaginous nasal skeleton in children may result in progressive deviations of the septum and nasal dorsum during further growth ( Figs. 33.8, 33.9, 33.10, and 33.11 ).
These and similar clinical observations confirm previous conclusions from animal research. The total septodorsal cartilage should be regarded as a “growth center” in the developing midface. Local injuries of the septum or dorsal cartilages will disturb development of the total dorsolateral cartilage and indirectly of the bony nasal skeleton.
Clinical Evidence: Nasal Growth after Rhinosurgery
Rhinosurgery in children is special, as it aims to restore— and certainly not disturb—nasal (midfacial) growth. Considering that the nose of children shows increasing external dimensions as well as growth and maturation of the nasal skeleton, clinical documentation should include for each patient
Age at the time of surgery
Description (including photographic documentation) of the external form of the nose and the anatomy of the nasal skeleton prior to surgical intervention (including observed fracture lines, deviations, etc.)
Facial profile at the age of 6.5 years shows a slight under-development of the bony nasal pyramid and saddling of the cartilaginous nasal dorsum, retracted columella, and retroposition of the anterior nasal spine. At the age of 17 years, these anomalies have become even more evident. Nasal tip is supported only by the alar cartilages. Six months after surgery, elevation of the nasal dorsum with an dorsum implant. Nasal development after a septal abscess at the age of 4.5 years. Evolution of a deviation of the cartilaginous nasal pyramid after previous trauma. Frontal photographs of a boy at the age of 11 (a), 15 (b), and 18 (c) years show a gradual increase of the deviation of the cartilaginous nasal dorsum (prior to surgery). (Courtesy of Dr. R. M. L. Poublon.) The same patient in the lateral view demonstrates a lowering of the cartilaginous nasal dorsum, which is only observed at the age of 18 years. (Courtesy of Dr. R.M.L. Poublon.)
Precise report of the surgical reconstruction of the nose-supporting framework
Follow-up of the development of the nose and midface, based on periodic photographic documentation and description of the nasal skeleton and patency of the nose
Follow-up to be continued until after the adolescent growth spurt has slowed down Clinical studies usually refer to small numbers of patients. The age at the time of surgery seems to vary from 1 to 16 years. The follow-up period is often too short and the available documentation incomplete. This may explain contradictory opinions regarding the outcome of surgical treatment, varying from normal growth after surgery to recurrent malformation.27–29 A lack of structured follow-up and documentation still precludes evidence-based conclusions.
The younger the child is at the time of injury to the nasal skeleton, the greater are the effects on further growth of the nose and possibly the maxilla.
Evaluation of nasal growth after treatment of nasal fractures or septal abscesses should differentiate for the age of the child at the time of injury and treatment; follow-up should be continued until after the adolescent growth spurt.
Until now, clinical evidence has not been convincing as far as restoring nasal growth by surgery is concerned.
Indications, Diagnosis, and Treatment
Indications
Rhinosurgery in children may be indicated in acute situations after recent trauma or performed as an elective procedure.
Fractures and/or dislocations of the nasal skeleton, as well as hematoma or abscess of the nasal septum or nasal dorsum, are indications for evaluation and treatment at short notice.30,31
Indications for an elective procedure are severe breathing problems due to pathology of the nasal skeleton and severe or progressing distortion of the nose.27 For children with less evident pathology, postponing surgery until after the adolescent growth spurt is advised to minimize the risk of negative effects from surgery on developmental processes.
Recent Nasal Trauma
A correct diagnosis of fractures and dislocations of the bony and cartilaginous nasal pyramid in young children is often hampered by the small dimensions of the nose and abundant swelling of the soft tissues during the first days after trauma. When in doubt, the maxilla, orbital rim, and palate should be examined (radiographically) to exclude additional fractures. As in adult patients, the diagnosis and treatment of a hematoma or abscess of the nasal septum are urgent to prevent necrosis of the septum cartilage, which could lead to sagging of the nasal dorsum or underdevelopment of the nose. Extensive soft tissue swelling may be a reason to repeat the examination after a few days. Reexamination under general anesthesia may be combined with surgical treatment. Vertical or horizontal fractures of the septum are often found. Fractures of the bony nasal pyramid do not occur as frequently in children as in adults. Occasionally, a dehiscence of the sutures bordering the nasal bones may be found.
In general, similar techniques are used for the treatment of recent traumatic deformation of the nasal skeleton as for elective surgery.
Closed Reduction in Young Children
The prevailing method of managing acute nasal fractures in young children is closed reduction under general anesthesia. Intranasal elevation of the nasal dorsum, thereby straightening the nasal septum, and digital compression of the nasal bones aim to restore the form of the nasal pyramid. In exceptional cases, a 2-mm osteotome may be used to mobilize dislocated parts of the nasal bones.
Nasal packing is tolerated only by older children and contraindicated in younger children, as they are “ programmed” for nasal breathing.
Hematoma of the Nasal Dorsum
The external branch of the anterior ethmoidal artery reaches the subcutis of the nasal dorsum via a foramen in the upper lateral cartilage near the caudal rim of the nasal bone. External trauma of the nose may cause a rupturing of an upper lateral cartilage, damaging the anterior ethmoidal artery. The resulting hematoma is diagnosed at anterior rhinoscopy as a bulging of the lateral nasal wall between the caudal edge of the upper lateral cartilage and the cephalic rim of the alar cartilage. Externally, the hematoma is often concealed by facial edema. It is recommended that the hematoma be evacuated via an intercartilaginous incision or puncture before approximating the dislocated upper lateral cartilage to the nasal bone by nasal packing to prevent infection, which can eventually result in cartilage necrosis and scar formation that may cause progressive asymmetry of the cartilaginous nasal pyramid.
Rhinosurgery in Children as an Elective Procedure
It is perhaps a sign of the times we live in that otolaryngologists are increasingly being asked to consider cosmetic rhinoplasty in children. The occasional child with a severely disfigured nose may be suffering from bullying at school, poor self-esteem, and impaired socialization severe enough to make surgery at a young age worthwhile, but even in such a case, it is preferable to leave the surgery for as long as possible. Surgery will lead to better results if delayed until after the pubertal growth spurt. Early surgery carries a risk of disrupting midfacial growth, especially if the septum is operated upon.
The following recommendations may be presented in relation to nasal surgery in children, considering the anatomical development, the interactions between the growing cartilaginous and bony nasal skeleton, and the poor wound healing of nasal cartilage.
A preliminary condition is to visualize the dimensions of the nasal cavity, the position of the anterior cranial base and choanae, and the anatomy of the nasal septum and other parts of the nasal skeleton specific to the age of the patient.
The mucoperichondrium may be elevated carefully on one or both sides of the septum. The mucosa of the nasal floor should not be elevated to prevent damage to the incisive nerves.
Identify defects or fractures of the septum; mobilize deviated or overlapping cartilaginous fragments by separating adherent fibrous tissues; adapt the form and size of the fragments to realign them in the midline.
Visualize the growth and support zones in the cartilaginous septum in relation to the progressing ossification of the septum, columellar rim, and nasal tip.
Do not separate the septum cartilage from the perpendicular plate: the septoethmoidal junction is essential for support of the cartilaginous nasal dorsum and growth of the septum.
Resection of a crista septi, spina vomeri, or deviating basal rim of the cartilaginous septum should not harm nasal development.
Do not transect the septospinal ligament: it anchors the septum in the midline and contributes to forward growth of the maxilla.
Reconstruct defects in the cartilaginous nasal septum with autologous septum cartilage to minimize the risk of later septal perforations.
Reposition a deviated caudal rim of the cartilaginous septum into a columella pocket and fixate it between the medial crura of the alar cartilages with sutures.
Alloplastic or biomaterials are not capable of growth; when implanted in the nasal septum of a child, they may support the nasal dorsum but do not restore nasal growth.
Do not separate the septum from the upper lateral cartilage on one or both sides; this bears the risk of later irregularities or deviations of the nasal dorsum.
Mobilization of nasal bones by osteotomies may not disturb further nasal growth, but the relationships between growing cartilaginous and bony nasal skeleton are complex. The development of the bony nasal skeleton is largely dependent on the growth of the septodorsal cartilage; at the same time, the cartilaginous nasal pyramid is supported by the bony structures. The hazards for further development of this mutual dependence should be considered when planning a combined correction of the bony and cartilaginous pyramid.
Nasal surgery in children is unique within otorhinolaryngology, as it concerns a complex of growing cartilaginous and bony structures whose interaction is essential for normal development of the nose and other parts of the midface. The poor wound healing and inadequate regenerative capacity of the cartilaginous nasal skeleton are still major factors in the inefficacy of nasal surgery to restore normal growth.
Current research is focused on ways to stimulate in situ wound healing of fractures or defects of the nasal cartilage.32 The next step will be to investigate how “ engineered” cartilage implanted in the nasal septum may contribute to morphogenetic processes of the growing nasal skeleton.6,18,33
As stated previously, clinical evidence concerning nasal growth after rhinosurgery in children still shows serious gaps. The patient′s parents should be informed of the restrictions of current surgery as to normalization of further nasal growth and of the long follow-up to evaluate the results of surgery. Instruction of family doctors might result in more children being evaluated by otorhinolaryngology specialists. Tertiary care centers might offer surgical expertise, multidisciplinary competence, and facilities for an adequate follow-up of the development of the nose and maxilla, which should result in evidence-based conclusions and surgical methods.
Congenital Anomalies
Physiology and Initial Assessment of Nasal Obstruction in Neonates
At birth, the larynx is positioned high up behind the tongue base so that the epiglottis and uvula interdigitate, separating the midline nasal-laryngeal airstream from the lateral food channels; food is thus directed on either side of the larynx to the piriform fossae of the hypopharynx.34 This arrangement is typical for all mammals and serves to reduce the risk of aspiration. Adult humans are unusual in having a descended larynx. Until the process of laryngeal descent begins, newborns are obligate nasal breathers, meaning that they are unable to voluntarily mouth breathe. If a newborn has an obstructed nose for any reason, he or she will struggle to breathe. In mild cases, the baby may seem to be very “snuffly” but may feed and grow without it being a major problem. The most severely affected newborns, however, will hypoventilate when their mouths are closed and become hypoxic. This leads to the baby becoming distressed, but as soon as he or she starts to cry with an open mouth, the respiratory distress resolves. The baby then settles, and a repeating cycle of cyclical hypoxia results.
It is important to assess the nasal patency of any infant with breathing difficulties. This can be accomplished simply by looking for misting on a cold metal spatula held under the nares. Misting excludes choanal atresia on that side. Failure to pass a nasogastric tube suggests a bony atresia or stenosis: if the tube cannot enter a slit-like nostril, then piriform aperture stenosis should be considered. If the nares look normal, but the tube will pass only 2 to 3 cm into the nose, choanal atresia is likely. This can be confirmed by nasal endoscopy and computed tomography (CT) scan. In most cases of nasal congestion with some reduced nasal airflow on each side but no bony narrowing, the diagnosis is neonatal rhinitis ( Fig. 33.12 ). These conditions and others occurring in newborns are described below.
Regardless of the cause, the immediate respiratory distress can easily be relieved with a Guedel oral airway, although ulceration of the soft palate will occur if this is left in place for more than 1 or 2 days. A McGovern nipple is softer and less traumatic. In many cases, an orogastric feeding tube will hold the mouth open enough to relieve the respiratory distress. Once the child is stabilized, the underlying cause can be investigated.
It is important to note that children with craniofacial anomalies often present with multiple, complex problems affecting a variety of organ systems, and many will require lifelong medical input. The role of the otolaryngologist is as part of a wider multidisciplinary team of surgeons, physicians, and therapists.
Arrhinia
Complete absence of the nose is very rare, with fewer than 40 cases reported worldwide. It has been postulated that the condition arises when the medial and lateral nasal processes fail to develop. No consistent genetic basis has been found. Arrhinia is often associated with learning difficulties, and tracheostomy may be the most appropriate treatment.35 Implant-retained prostheses can be used for cosmesis. Staged surgery to reconstruct a skin-lined bony nasal cavity and an external nose has been attempted in some centers, but the results are variable. Such extensive surgery may be appropriate for children with normal psychomotor development.
Midline Facial Clefting
A variety of names have been used to describe these rare anomalies in which children have hypertelorism, varying degrees of midline splitting of the nose, underdevelopment of the premaxilla, midline grooving of the lip, and midline clefting of the palate ( Figs. 33.13 and 33.14 ). According to the classification system used and the exact anomalies present, these may be termed midline facial clefts, frontonasal dysplasia, or internasal dysplasia. They may be associated with meningoencephaloceles and pituitary anomalies. Nasal anomalies include broadening of the nasal bridge, a bifid nasal tip, and duplication of the septum, producing stenosis of the nasal cavity. Dilation and temporary stenting of the nose (see discussion on choanal atresia below for details of nasal stents) for the first 2 or 3 months of life can produce an improvement in the nasal airway sufficient to allow the child to grow. CT and magnetic resonance imaging (MRI) scans are required for accurate assessment of the anomalies. In later childhood, extensive craniofacial corrective surgery may be undertaken.
Congenital Nasopharyngeal Stenosis
Children with syndromic craniosynostosis (Crouzon, Apert, Pfeiffer, and Saethre-Chotzen syndromes) have an underdeveloped maxilla. The nose is patent, but the maxilla is small and sits in a very posterior position, effectively producing a stenotic nasopharynx. This can produce significant upper airway obstruction, especially at night. This can be managed with dilation and bilateral nasal stents in the newborn (see discussion below for details of nasal stents) or with a unilateral nasopharyngeal airway used at night in the older child with obstructive sleep apnea.36 Severe cases will require tracheostomy. Midface advancement surgery (Le Fort III osteotomies or a frontomaxillary “monobloc” advancement with distraction) can produce major improvements in the airway,37 but the timing of such major surgery for any individual child is controversial.
Choanal Atresia
Choanal atresia is by far the most common of the craniofacial anomalies described in this chapter, occurring in ~1 in 7000 births. It arises due to failure of involution of the nasobuccal membrane of Hochstetter. It is characterized by a failure of the posterior nasal cavity to communicate with the nasopharynx. The posterior choanae are blocked by an atretic plate consisting mostly of bone (but with a central membranous component in 70% of cases) ( Fig. 33.15 ). Other bony abnormalities contributing to the obstruction are thickening of the vomer and medialization of the pterygoid plates. Slightly more than half of the cases are unilateral, the remainder bilateral.
In ~75% of cases, the atresia is associated with other congenital anomalies, and in 50% with a named syndrome.38 The most common association is with CHARGE syndrome (coloboma, heart defects, choanal atresia, retarded growth and development, genital hypoplasia, ear anomalies, and deafness). This syndrome is caused by mutations in the chromodomain helicase DNA-binding protein 7 (CHD7) gene and is present in about one-quarter of children with choanal atresia. CHARGE is more common in those with bilateral atresia, but it occurs often enough in unilateral cases to make a CHARGE screen mandatory for any child diagnosed with choanal atresia. A CHARGE screen consists of an echocardiogram, hearing testing, eye examination, and renal tract ultrasound. Of these, the echocardiogram is the one that is most essential to obtain before any surgery is contemplated because of the risk of anesthesia in an infant with an undiagnosed heart problem.
Presentation is usually with respiratory distress at birth for most bilateral cases and some unilateral ones. Most unilateral cases present in the early-preschool years with unilateral nasal discharge, or later in childhood with a complaint of nasal obstruction.
When a child is suspected of having choanal atresia, fiberoptic endoscopy of the nose can be very useful in the diagnosis. If the endoscope can be passed to the pharynx, atresia can be excluded on that side. Accumulated secretions within the nose can make visualization of the atretic plate difficult. The diagnosis is best confirmed by CT scan. The best images are obtained if the nasal cavity is suctioned of secretions and the mucosa decongested with sympathomimetic drops ( Fig. 33.16 ). This allows clear visualization of the atretic plate and enables the surgeon to estimate the extent of bony removal that will be needed at surgery.
Surgery consists of drilling to remove the atretic plate and reduce projection of the vomer and pterygoid plates. Various approaches have been used over the years. The transpalatal approach gives good access but is rarely used now because the results of transnasal surgery are just as good. Transnasal surgery involves the instruments and the drill being passed in through the nose, but this can be controlled endoscopically in different ways: either using a 0-degree endoscope alongside the instruments within the nose (“FESS-style” or anterior approach) or using a 120-degree endoscope introduced through the mouth and looking up behind the palate into the nasopharynx (posterior approach).39 The anterior approach allows for formation of mucosal flaps from the mucosa on the atretic plate, which can be used to provide cover for the bare bone of the choana, but this is technically very difficult except in the older child. In infants, visualization is difficult with the anterior approach, and injury to the soft palate and base of the skull is more likely. The posterior approach is technically much easier and allows for wide illumination of the operative site and more room for instruments with less contamination of the endoscope by blood. It is accomplished with the child in the tonsillectomy position and the operator at the head of the patient. A Boyle-Davis tonsillectomy gag is used (or a smaller cleft palate gag for infants, such as a Sommerlad gag). The palate is retracted using a suture in the base of the uvula. An assistant inserts the 120-degree endoscope into the oral cavity until it is looking up behind the soft palate at the nasopharynx. The operator introduces instruments into the nose, and they are seen on the screen emerging from the choana into the nasopharynx toward the camera. The posterior approach gives a wide view of the entire choanae, and the limits of surgery are much easier to define than with the anterior approach.
The first step in surgery is to perforate the atretic plate near its center, where it is thin and usually membranous. A 2- to 3-mm bur is then used to remove bone, taking care not to injure the ala with the shaft of the bur. Bone removal should be generous laterally due to the projection of the pterygoid plates into the choanae. The vomer can be removed with backbiting forceps to considerably enlarge the space created. It is possible to use a potassium-titanyl-phosphate (KTP) laser rather than a drill, but it has no clear advantages.40
It has been customary to insert stents after surgery. Proprietary nasal stents are available in various sizes, or they can be fashioned from cut endotracheal tubes held with a polypropylene suture through the tubes encircling the septum. Whether stents have any effect in reducing restenosis is controversial.40 They are very useful in providing a guaranteed nasal airway for infants during the obligate nasal breathing phase, so it is reasonable to use them routinely in bilateral atresia surgery in infants for 6 to 8 weeks. Their use in older children with unilateral atresia is discretionary and may be even counterproductive.41,42
Restenosis is universal to some degree, but in some children the choanae close rapidly and completely with fibrous tissue. Parents should be warned from the outset that several procedures may be required before a stable result is achieved. Repeated dilation in the early weeks after surgery may help to achieve a good result. It is possible that use of a balloon for dilation will give better results without the shearing effect of the urethral dilators that have been traditionally used in many centers. The choanae will probably never be of normal size, but as long as they are patent enough for breathing and drainage of mucus, then parents and the child will be satisfied. In addition to prolonged stenting, various measures have been tried to reduce restenosis. Corticosteroid nose drops have not been shown to have any effect.40 Mitomycin C inhibits fibroblasts and may have a role in reducing fibrous tissue formation. It can be applied topically at the time of stent removal, a few weeks after surgery. The evidence for its efficacy is limited.40,43 Uncontrolled gastronasopharyngeal reflux has been shown to be a predictive factor for restenosis, and a trial of antireflux medication may be justifiable if restenosis is problematic.42
Tips and Tricks
Top tips for success in choanal atresia surgery
Preoperative planning:
CT scan
CHARGE screen
Surgical technique:
Good endoscopic visualization
Adequate lateral bone removal with the drill
Remove the vomer with backbiting forceps
Aftercare:
Stents for 6 to 8 weeks in bilateral cases
Careful sizing and fixation of stents to prevent alar stenosis
Early, regular, repeated balloon dilation until choanae are stable and patent
Consider use of mitomycin C
Consider antireflux therapy