First the problem(s) needs a diagnosis. Prenatal diagnosis is now possible for CL but is less reliable for CP or CF deformities. At birth most clefting or CF disorders are apparent, with diagnosis based on the typical features seen. Syndromic CP or CF anomalies may also have renal, cardiac, extremity, or metabolic disorders. A genetics or dysmorphology consult is obtained especially if other anomalies are found. Family counseling can be given regarding the likelihood of future children affected, genetic testing can be initiated, and the prognosis for the affected child can be discussed by the geneticist. Also performing a social and psychological assessment provides support and resources to the parents and may prevent future problems.

Neonates
Feeding and breathing problems can develop during the neonatal period. Babies with clefts may have an ineffective suck that results in significant air swallowing, nasal regurgitation, and poor swallowing potentially leading to inadequate nutrition. A feeding nurse specialist offers expertise and strategies to overcome these feeding problems. Changing the position during feeding or using a special cleft palate nipple are two techniques used. Intimately related to feeding problems are breathing problems. Children with CF anomalies frequently have a relatively constricted pharynx due to a narrowed skull base or a small mandible. Careful attention during feeding may reveal increased work of breathing and indicate the need for an airway evaluation. Also some CF syndromes (e.g. Apert syndrome) may have deformities of the airway such as choanal atresia or tracheal stenosis. A recent review of over 100 children with CF anomalies demonstrated that 60% of these children required some form of airway intervention. Moreover, 80% of those children who required airway intervention received airway support starting in the first month of life.

Airway evaluation in an infant begins with assessing breathing patterns, airway noise, and retractions. A 5 French catheter should easily pass through a neonate's nose and exclude choanal atresia. Flexible laryngoscopy is performed at bedside and visualizes the airway from the nasal vestibule to the level of the vocal cords. If the site of airway stenosis is below the vocal cords, then bronchoscopy is done to evaluate the subglottic larynx and tracheobronchial tree. Treatment of airway obstruction is based on the site of lesion, but the approach is step-wise. Perhaps positioning or nasal oxygen is all that is necessary. If the obstruction is at the nose or tongue, then often a nasopharyngeal airway can support the airway until the child "grows out" of the obstruction. If the obstruction is severe intubation is required. However these severely affected children may ultimately need a tracheotomy for long-term airway management.

Finally, any child born with a facial deformity should have a screening audiogram. Babies with CP or CF are at risk for developing hearing loss (see below), and a baseline hearing function can be quite useful later. There are various techniques available and should be coordinated through a pediatric audiologist skilled in these techniques.

Infants
During the first year the care providers continue to monitor progress in feeding and growth. The child with a CL can then undergo surgery to reconstruct the lip. Unless there is some contraindication, most surgeons like to perform CL repair when the child is about 10 pounds and has a hemoglobin of 10 g/dl. Several techniques are available with the ultimate goal of facial symmetry. Often correction of the associated cleft nasal deformity accompanies the cleft lip repair. Children with associated CP frequently develop chronic otitis media (see below), thus some surgeons will place tympanostomy tubes simultaneous with lip repair to optimize hearing.

Hearing should also be monitored in all children with facial clefting or CF anomalies. If the child fails the screening audiogram, an auditory brainstem response (ABR) is performed. The "gold standard" remains the behavioral audiogram, but children must be at least 6 months developmental age before they can be reliably tested. Any hearing loss requires a complete otologic evaluation and hearing aids if necessary.

Infants with some CF syndromes (e.g. Crouzon, Apert) may need cranial suture release if brain growth is compromised. Also airway compromise may occur as these infants grow but the airway remains disproportionately small. If the work of breathing consumes too much energy the child could fail to thrive. Indications for airway evaluation include failure to thrive, stridor, retractions, cyanosis, or apnea.

Toddler
Many of the management principles through the first year of life continue. Now the toddler must accurately develop the important skills of speech and language in order to begin social integration. Children with CP can develop speech delay related both to the functional defect of the cleft palate and the hearing loss due to the inevitable chronic middle ear effusion. Attempts to speak by children with CP often results in compensatory misarticulation errors that must be corrected by speech therapy. To prevent speech delay and compensatory misarticulation the CP is typically repaired between 9 to 15 months of age. The CP deformity also affects the eustachian tube function, so nearly all children with CP will develop persistent middle ear effusion or chronic otitis media. Thus tympanostomy tubes are needed to clear the middle ear and optimize hearing, and if not done earlier can be done during palatoplasty. Even after CP repair it takes at least 7 years for eustachian tube function to equal age-matched normals. Newer techniques available to some CP patients repair the palatal muscle sling important to eustachian tube and palatal function. Preliminary data from a multi-center study suggests the double-opposing Z (Furlow) palatoplasty may improve speech outcomes.

Speech development needs careful monitoring by a speech pathologist. Problems with phoneme production such as compensatory misarticulations must be diagnosed early for effective habilitation, and speech delay responds best to early intervention. Also an infant with tracheotomy is at high risk for speech delay. After CP repair about 20--30% of children will develop velopharyngeal insufficiency (VPI). VPI is heard as hypernasality during vowel production and nasal air emission with consonants. These problems with speech intelligibility or speech delay require evaluation by a speech pathologist skilled in the diagnosis and treatment of VPI and CP speech.

The audiologist performs periodic hearing tests especially if there is active middle ear disease. Eustachian tube function is monitored, ossicular abnormalities seen in CF anomalies may be diagnosed, aided hearing can be assessed, and progressive sensorineural hearing loss may be discovered by the audiometric testing available at this age.

In a child with a small pharynx and skull base (i.e. CF syndrome) growth of the tonsils and adenoids may compromise the airway. Difficulty breathing at night frequently is the first symptom of this problem. A sleep study should be performed in any CF child with symptoms of obstructive sleep apnea. A history of a compromised airway indicates a high risk patient for any surgery on that airway. Only after careful analysis should surgery be considered to improve the airway. A new method to improve the airway in selected toddlers with mandibular hypoplasia is distraction osteogenesis. This technique stimulates new bone growth by "stretching the callus" after a surgical mandibular osteotomy. Not only will the mandible expand but the surrounding soft tissues of the face and neck also grow proportionately. Distraction osteogenesis produces benefits for the airway, facial symmetry, and appearance.

School Age
Prior to entering school any improvements in the CL repair or surgical correction of midface hypoplasia are performed. Also if VPI remains a problem in spite of speech therapy further intervention should be implemented. The child with VPI is evaluated using either videofluoroscopy or nasendoscopy. These procedures visualize the velum during speech. The velum consists of the combined efforts of the soft palate, posterior pharyngeal wall, and lateral pharyngeal walls to close during speech or swallowing. The velum closes during all English consonant phonemes except \m\, \n\, and \ng\. During normal speech, if the nasal cavity is not aerodynamically separated from the oral cavity--the function of the velum--hypernasal speech results. Direct observation of the velum during speech evaluation can reveal where the deficiency is. Based on this analysis of speech and velar function treatment is devised. A temporary speech appliance, or palatal obturator, is designed by a pedodontist to "plug the gap" in the velum. In some children a palatal obturator is enough to strengthen the velar musculature, and these select children eventually wean from the obturator. Other children with VPI may not tolerate the obturator (a dental appliance fit like an orthodontic retainer) or VPI may persist after obturator therapy. In these cases one of three surgical procedures are used to correct VPI. The pattern of velar closure seen at nasendoscopy or videofluoroscopy determines which procedure to use. A Furlow palatoplasty lengthens the palate in addition to reconstructing the muscles of the palate. A pharyngoplasty improves lateral pharyngeal wall motion by adding functioning muscle to the lateral velum. A pharyngeal flap uses tissue from the posterior pharyngeal wall attached to the palate to obturate or fill the velar gap. Any of these procedures alter the nasal and pharyngeal airway, but the pharyngeal flap has the highest potential for airway compromise. Thus any child with a history of airway problems in whom any of these three procedures are considered should have an airway evaluation. Nasendoscopy offers that opportunity for examination of the pharynx and larynx.

Another important issue to address during school age is dental health. Good dental hygiene taught early shows its benefits now. The eruption of permanent teeth allow the orthodontist to correct the malocclusion that is frequently seen in CP or CF children. If the dental arch (alveolus) was a part of the original cleft, then orthodontics will expand and align the maxillary dentition prior to bone grafting. Once the teeth are aligned alveolar bone grafting stabilizes the maxillary dental arch and supports the nasal base.

Some CF children (e.g. hemifacial microsomia) will have external and/or middle ear deformities. Microtia reconstruction typically is performed about age 6--7 when the normal external ear is about 90% of adult size. Correction of microtia requires up to 4 surgeries performed in a staged manner. Middle ear reconstruction follows microtia repair, primarily in bilateral cases, and requires sufficient middle ear development to be technically feasible.

It is very important to assess school performance and psychosocial adjustment in these children. For any child school years can be difficult, but for children with obvious facial deformities this time can be quite a challenge. Careful monitoring by parents, teachers, and CF team members with early and active intervention has demonstrated improved social integration for CL, CP and CF children.

Adolescence
With the completion of natural facial growth correction and refinement of the original repairs are performed. A septorhinoplasty for CL patients improves the nasal airway and achieves nasal symmetry. At this age septal cartilage can be removed safely without disturbing midface growth. Septal cartilage grafts enhance nasal appearance. For severe malocclusion not corrected by orthodontics, orthognathic surgery is considered. Either maxillary advancement or mandible repositioning, or both, should improve occlusion and appearance. Similarly CF anomalies may require further surgery to reposition the facial skeleton. As before, the school performance and psychosocial abilities of these children require monitoring and intervention as needed.

In summary, there are myriad problems that confront the child with CL, CP, or CF anomalies. A team approach relying upon the expertise of several pediatric specialists committed to the monitoring and care of these children provides a coordinated series of interventions throughout the child's life. The team's goals are functional and cosmetic, with the final result a well-adapted young adult.

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