This article reviews the most current practice guidelines in the diagnosis and management of patients born with cleft lip and/or palate. Such patients frequently have multiple medical and social issues that benefit greatly from a team approach. Common challenges include feeding difficulty, nutritional deficiency, speech disorders, hearing problems, ear disease, dental anomalies, and both social and developmental delays, among others. Interdisciplinary evaluation and collaboration throughout a patient’s development are essential.
Key points
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Proper care of a patient born with a cleft lip and/or cleft palate (CL ± P) requires a team-based approach.
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Close monitoring by members of an accredited cleft team is essential throughout the patient’s development.
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Speech and ear problems are common issues that require early evaluation and treatment.
Introduction
CL ± P is one of the most common birth defects. Patients with CL ± P are frequently encountered in general pediatric clinics but even more commonly in an otolaryngology practice. Problems with ear disease, hearing, infections, breathing, sleep disturbance, and feeding and speech disorders are often encountered with clefts. These patients often require multiple surgeries and many need therapy during their development.
Early and accurate diagnosis of a patient’s cleft is critical for proper management. The primary care provider (PCP) and cleft surgeon must be aware of the potential issues that may arise with CL ± P and be familiar with the process for appropriate management, referral, and follow-up. Referral to a cleft team accredited by the American Cleft Palate-Craniofacial Association should be made either prenatally or soon after a child’s birth.
Once referred to a cleft team’s care, a coordinated evaluation must be completed to properly identify the need for surgical and medical management as well as possible therapy. This requires a fully functioning interdisciplinary cleft team that can provide comprehensive and cohesive care.
The object of this article is to provide a general understanding of clefts and describe an overview of the specific care each patient with CL ± P may receive. The embryology, epidemiology, classification, descriptive evaluations, surgical and medical interventions, and follow-up care are described.
Introduction
CL ± P is one of the most common birth defects. Patients with CL ± P are frequently encountered in general pediatric clinics but even more commonly in an otolaryngology practice. Problems with ear disease, hearing, infections, breathing, sleep disturbance, and feeding and speech disorders are often encountered with clefts. These patients often require multiple surgeries and many need therapy during their development.
Early and accurate diagnosis of a patient’s cleft is critical for proper management. The primary care provider (PCP) and cleft surgeon must be aware of the potential issues that may arise with CL ± P and be familiar with the process for appropriate management, referral, and follow-up. Referral to a cleft team accredited by the American Cleft Palate-Craniofacial Association should be made either prenatally or soon after a child’s birth.
Once referred to a cleft team’s care, a coordinated evaluation must be completed to properly identify the need for surgical and medical management as well as possible therapy. This requires a fully functioning interdisciplinary cleft team that can provide comprehensive and cohesive care.
The object of this article is to provide a general understanding of clefts and describe an overview of the specific care each patient with CL ± P may receive. The embryology, epidemiology, classification, descriptive evaluations, surgical and medical interventions, and follow-up care are described.
Embryology
In the fourth week of embryogenesis, the developing median frontonasal prominence and paired maxillary prominences and mandibular prominences emerge around the primitive oral cavity. At the end of the fourth week of embryonic development, the inferior frontonasal prominence divides into medial nasal prominences and lateral nasal prominences. The nasal alae are formed by elevation of the lateral nasal prominences. The upper lip and primary palate complete formation at the end of the sixth week of embryogenesis by fusion of the bilateral maxillary prominences with the 2 medial nasal prominences.
The secondary palate has a distinct embryologic origin from the lip and primary palate. Bilateral palatal shelves of the maxillary processes begin a vertical growth phase in the sixth week of embryonic development and transition to horizontal growth in the seventh week. The palatal shelves then fuse in the midline and fuse to the primary palate anteriorly as well as the nasal septum. Ossification of the anterior aspect of the secondary palate occurs in the eighth week, differentiating the hard palate from the more posterior soft palate. Palatal development is complete by the 10th week of embryogenesis.
Failure of fusion during embryonic development of the face results in orofacial clefting. Clefts can be divided into CL ± P and isolated CP due to their distinct developmental patterns. Teratogen exposure and timing of insult are critical factors in labiopalatal clefting.
Epidemiology
The overall incidence of orofacial clefts is estimated at 1 in 700 live births. Prevalence varies by race and ethnicity, with high rates of oral clefts in Asian populations (0.79 to 3.74 per 1000 individuals), intermediate rates in white populations (0.91 to 2.69 per 1000 individuals), and low rates in African populations (0.18 to 1.67 per 1000 individuals). CL ± P more often affects male infants, whereas CP occurs more frequently in female infants.
Syndromic cleft malformations represent a minority of cases, accounting for 10% to 37% of patients with CL ± P and 40% to 50% of patients with CP. Gorlin and colleagues indicate more than 400 syndromes that may be implicated in orofacial clefting. Van der Woude syndrome is the most common syndrome associated with CL ± P, accounting for approximately 2% of all cases ( Fig. 1 ). It is heralded by lower lip pits and is inherited in an autosomal dominant pattern with incomplete penetrance. CHARGE syndrome and Down syndrome represent additional recognizable malformations in the cleft lip population.
Stickler syndrome is the most commonly associated syndrome in patients with isolated CP, accounting for 5% of patients in some studies. This syndrome demonstrates an autosomal dominant pattern of inheritance and is due to collagen defects. Affected individuals are at risk for SNHL and ophthalmologic involvement, including myopia, retinal detachment, cataracts, and glaucoma ( Fig. 2 ). 22q11.2 Deletion syndrome, historically referred to as velocardiofacial syndrome or DiGeorge syndrome, is the second most common syndrome observed in patients with CP; phenotypic expression may include conotruncal cardiac defects, velopharyngeal insufficiency, and characteristic facies (flattened malar eminences and square nasal root, among other features [ Fig. 3 ]). Pierre Robin sequence (PRS) is not a syndromic entity but occurs frequently in patients with CP and is commonly associated with multiple orofacial clefting syndromes. A classic triad of micrognathia, glossoptosis, and U-shaped CP occurs sequentially, resulting in postnatal airway obstruction and/or feeding difficulty. Isolated, or nonsyndromic, PRS is estimated to account for 17% to 63% of cases. A referral to a geneticist or additional subspecialists (ie, ophthalmology) is warranted for many patients with clefts.
Cases of nonsyndromic CL ± P or CP are largely due to multiple factors, including complex genetic interactions and exogenous factors. The risk of both CL ± P or CP in first-degree relatives (ie, parent, offspring, or sibling) is approximately 3% to 4% and thus higher than the general population. Maternal use of retinoids and anticonvulsants, specifically phenytoin, greatly increases the risk of having an infant with an orofacial cleft. Pregestational diabetes in mothers also increases the risk of orofacial clefts, among other congenital malformations, and seems to correlate with glycemic control. Folic acid deficiency has been implicated in the development of orofacial clefts with several case-control studies reporting a positive effect of folic acid supplementation on decreasing risk of orofacial clefting. A multicenter randomized clinical trial investigating folic acid supplementation and oral cleft prevention is currently ongoing. An additional exogenous risk factor for development of orofacial clefts is maternal smoking, which increases risk by 1.3 or 1.2 times for CL ± P or isolated CP, respectively.
Classification
Numerous classification schemes exist to describe orofacial clefting. Rare facial clefts are typically classified using the Tessier system, with clefts located along axes identified by numbers, which are assigned relative to the midline. Early classifications for oral clefts were based on observed morphology. Of these, the Veau classification divided patients into 4 subgroups :
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Clefts of the soft palate
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Clefts of the soft and hard palate, posterior to the incisive foramen
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Complete unilateral cleft lip and CP
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Complete bilateral cleft lip and CP
Cefts may more simply be described in terms of anatomy and embryologic origin. Clefts of the lip or palate can be similarly subcategorized into ( Figs. 4–11 )
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Unilateral versus bilateral
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Complete versus incomplete
A complete cleft lip describes a cleft of the lip and alveolus extending into the floor of the nose. A Simonart band can be seen in complete cleft lips and is a soft tissue band connecting the disrupted lip segments. An incomplete cleft lip has an intact nasal sill and may or may not involve a cleft alveolus. Incomplete cleft lips can be further divided as mini-microform, microform, or minor-form, depending on the height of the defect from the normal Cupid bow peak.
A complete CP involves both the primary (anterior to the incisive foramen) and secondary (posterior to the incisive foramen) palate. An incomplete CP involves the secondary palate only but may be of the hard and soft palate, soft palate only, or a submucous CP. A submucous CP describes a dehiscence of the central palatal musculature with intact palatal mucosa; patients may exhibit a bifid uvula, zona pellucida (bluish discoloration due to absent muscle centrally), and a hard palate notch due to absence of the posterior nasal spine.
Initial evaluation
Patients born with a CL ± P may present at different ages, ranging from intrauterine to adulthood. The needs at these different stages can be different, requiring a comprehensive evaluation tailored to the individual. It is important that a fully functioning and accredited cleft team becomes involved from the beginning. The composition of each team may be different but the aim is the same: to provide complete interdisciplinary care. Each team has a coordinator whose major role is to determine the most appropriate timing and place for patient evaluation and to arrange for the appropriate subspecialty assessments ( Fig. 12 ).
Prenatal Evaluation
Although a significant number of infants are born with CL ± P to parents who were unaware of the child’s condition, a growing number of women (10% of cases ) discover during routine fetal ultrasound that their child has a cleft. A vast majority of infants who are diagnosed with a cleft prenatally have a cleft lip because it is much more difficult to diagnose a CP accurately with ultrasound. Other anomalies may be identified by ultrasound at this time because there is an elevated incidence of additional congenital anomalies and genetic abnormalities (10%–25% of cases depending on the type and degree of cleft).
Receiving the news of a fetal birth defect in a developing child may be a shock to the pregnant mother and family and may result in a multitude of emotions and additional questions. Soon after diagnosis, a referral to the cleft team for a prenatal visit can prove beneficial for the mother as well as her family and friends. The majority of this visit includes counseling and education. The goal should be to provide reassurance to the family of the type and quality of care the child receives and what they can expect from an evaluation and treatment plan once their infant is born.
Nutritionist
It is helpful to include a nutritionist familiar with CL ± P (ideally the nutritionist associated with the cleft team) as part of this prenatal visit. A discussion of the feeding challenges the infant may face tempered by an encouraging description of the different feeding options is helpful.
The topic of breastfeeding may be discussed if that is something the mother is interested in pursuing. Most infants with CP struggle with breastfeeding (as well as standard bottle feeding) due to an inability to achieve an appropriate sucking mechanism. The mother may attempt breastfeeding at first, if she desires, but feeding and weight must be monitored closely to assure adequacy of nutrition and avoid negative health consequences. The use of a feeding plate is controversial and not widely used.
Most infants with CP cannot successfully breastfeed or even use standard bottles. In this setting, the mother and support team are encouraged to explore other feeding options. It is helpful to discuss and demonstrate the different specialized nipples and bottles, which the child may use during this prenatal visit (ie, Pigeon Cleft Palate Nipple and Cleft Palate Bottle, Haberman Special Needs Feeder, and/or Mead Johnson Cleft Lip/Palate Nurser). Providing them with some of these items can also prove beneficial especially if a child is delivered in an area with limited resources and little experience with CL ± P. Hubbard and colleagues found hospital admission rates for feeding issues in patients with clefts are reduced by approximately 50% when prenatal counseling is given.
As an indicator of proper nutrition, a weight gain of at least 15 to 30 g per day should be expected. To monitor this closely, weekly weight checks should be performed in the early neonatal period and should be communicated directly to the cleft team nutritionist. Rarely, alternative feeding options may be required, such as extended nasogastric tube feeding (NGT) or gastrostomy tube (GT) placement.
Nursing
In addition to visiting with a nutritionist and surgeon, a mother’s visit with a nurse who is a member of the cleft team is helpful while still pregnant. Providing reassurance to the family about the quality and type of care the child will receive is paramount. A discussion in conjunction with the surgeon regarding the timeline of evaluations and surgeries may take place at this time to help prepare the family for what may lie ahead. When possible, pictures of children before and after surgical management can be shown. Photographs may provide an excellent form of comfort because they see what their child may look like immediately at birth and also how he or she will transform through the various indicated procedures.
The overall goal of the cleft team should be discussed during this prenatal visit: to provide individualized coordinated care to allow the child to successfully engage in society and achieve healthy and appropriate development. The nurse may consider describing the other various specialists the child may encounter during clinical follow-ups to show the family the comprehensive care their child will receive.
Surgeon
A prenatal visit with the surgeon can also prove exceedingly valuable. This visit helps plant the initial seeds of trust and confidence that will prove essential especially early in the child’s health management. During this visit, the physician may ask about maternal health status, including prenatal care, teratogen exposure, and any family history of clefting, congenital anomalies, or syndromes, which may help further direct the neonatal care. A discussion about cleft etiology can take place at this time.
Many expectant mothers feel guilt as they wonder if there was something they may or may not have done to contribute to this birth defect. If done successfully, this visit can help the mother and family remove some of these negative feelings and instead develop the positive sentiments that typically come with the arrival of a new child. The opportunity to ask questions should be offered multiple times throughout the visit and, when possible, information about resources available to the family and contact information for the team should be provided.
Overall, the goal of the prenatal visit should be to answer questions and provide information and materials that allow the mother and her close associates to prepare physically and emotionally for an infant’s arrival. Women are encouraged to deliver in whatever setting their obstetrician feels is most appropriate and to make contact with the cleft team soon after the child is born.
Infant Evaluation
For any infant born with a cleft, a thorough examination is essential. As discussed previously, associated anomalies are common in patients born with clefts. A thorough head and neck examination (including airway) as well as heart and extremity evaluations can discover most of the common related findings in CL ± P. Depending on the type and degree of cleft, other specialists (such as audiology, speech, nutrition, genetics, and surgery) should be consulted to provide further evaluation and care for such infants (see Fig. 12 ).
Primary care
It is critical that each patient born with CL ± P establish a close relationship with the managing PCP. Regular follow-ups, especially early in a child’s development, are beneficial to the emotional and physical needs of patients and their families. Accurate communication between the PCP and the cleft team members is essential.
Cleft team evaluation
Once a child is stabilized, a consultation to the cleft team, including the infant’s managing cleft surgeon and nurse, should be initiated. This referral provides the infant with prompt access to the appropriate members of the cleft team whose services the child may require and establishes a mechanism for close follow-up (see Fig. 12 ).
If a prenatal consultation was not performed, the information described previously should be shared at this time. This discussion includes information about clefts; descriptions of surgical interventions, including a timeline of procedures and evaluations; specific issues the patient may encounter (eg, feeding, hearing, speech, and dental), and information about the cleft team composition and timing of evaluations. Whether the initial visit takes place while still in the hospital or in an outpatient setting, this visit is the first opportunity for the nurse and/or managing surgeon to evaluate a patient and determine the degree and type of clefting. A thorough examination and history take place at this time to evaluate the cleft and identify any associated anomalies.
Specific attention should be directed at this time toward the common problems encountered in CL ± P, including feeding, hearing, and breathing. Depending on findings, appropriate referrals should be generated and evaluations performed. In many cases, multispecialty evaluations can be accomplished during the same visit (inpatient or outpatient), which expedites patient care and provides convenience to the patient and family.
Breathing is one of the first things evaluated when an infant is born, and neonates with CL ± P are at higher risk for airway compromise. This is particularly true in the context of associated anomalies and if syndromic. PRS is the main source of upper airway obstruction in CL ± P although other causes need to be ruled out ( Fig. 13 ). If a patient is in respiratory distress, indicated interventions may be pursued (discussed later).
Speech pathology
An evaluation by a speech and language pathologist (SLP) familiar with clefts is often beneficial in neonates with CL ± P. Early assessment and proper intervention allow for determination of oral and pharyngeal function. Most infants with CP, as well as some with cleft lip alone, have difficulty with standard feeding approaches and SLP intervention can help them successfully feed and grow.
The degree of clefting as well as the presence of an associated syndrome has been shown closely associated with the severity of feeding problems. Feedings often take longer in CL ± P and frequent burping and breaks are typically required because infants often swallow large amounts of air.
The SLP can greatly support patients with learning appropriate techniques for feeding as well as determining the most effective feeding method (breastfeeding, standard bottle feeding vs specialized nipples/bottles, and so forth). The use of a palatal obturator or feeding plate is controversial although widely used in some institutions and regions of the world. This option is rarely considered, however, at the authors’ institution due to limited proved benefit in this setting combined with increased time and monetary cost.
Initially, a neonate with CL ± P may have difficulty despite using specialized nipples/bottles and having close SLP assistance. In these cases, NGT may be required. Rates of NGT placement have been found close to 30% in neonates with CP. Oral feeding efforts should continue while NGT is in place and successful oral feeding is frequently accomplished. Some infants may eventually require GT placement. Even if GT surgery is performed, regular speech therapy and close follow-up examinations by the cleft team are important to avoid or at least minimize oral aversion and eventually achieve successful oral feeding.
Nutrition
As discussed previously, feeding and nutrition difficulties are frequently present in CLP. Close weight monitoring (initially weekly) is important in neonates. Increasing formula calories or adding calories to expressed breast milk, changing formula type, and so forth may all be required. Occasionally, an infant may present with failure to thrive and require admission to the hospital. A team nutritionist can serve a pivotal role in making these decisions. A coordinated effort between SLP, nutritionist, nurse, and managing physician nearly always achieves successful feeding, nutrition and growth.
Audiology
Universal newborn hearing screening (NBHS) is mandated in a majority of states in the United States, such that more than 95% of newborn infants are screened for hearing loss. Infants born with a cleft (in particular, CP) are at a higher risk of failing their NBHS; pass rates for these patients are only 72% in some studies compared with greater than 97% in patients without clefts. In many cases, the cause of the failed hearing screen is middle ear effusion, which is present almost universally in patients with CPs. Rates of congenital sensorineural hearing loss (SNHL) have been found as high as 5% in cleft populations. Rates of conductive hearing loss (CHL) and mixed hearing loss are also much higher in CLP.
The NBHS includes otoacoustic and/or automated auditory brainstem response testing, preferably before an infant is discharged home from the hospital. If a child refers (does not pass) the screening test, an appropriate and timely referral should be made to obtain repeat hearing screening and possibly further evaluation, testing, and possible intervention as indicated. A prompt evaluation becomes especially important in CL ± P. Identification of hearing loss should take place by 3 months of age and intervention should transpire by 6 months of age for confirmed hearing loss (see Fig. 12 ).
Genetics
Many institutions consult genetics once a cleft is diagnosed. This consultation can prove helpful in identifying associated anomalies and may allow for classification into a specific syndrome. More than 400 syndromes have been found associated with CL ± P although most cleft cases are multifactorial. A thorough history and examination with investigation into teratogen exposure and maternal factors (such as smoking, diabetes, prenatal vitamins, and so forth) may elucidate the cleft etiology and allow for meaningful genetic counseling; genetic testing is also an increasingly used tool. This evaluation can serve a critical role in helping the family adjust to the news that their child was born with a cleft. A discussion about recurrence risks and prevention can also be enlightening to the family and assist with future pregnancy planning. The authors make efforts to have all cleft patients evaluated by genetics within the first 6 months of life (see Fig. 12 ).
Social work
Patients born with CL ± P may have multiple medical issues, as described previously, which require extensive work-ups, treatments, procedures, and frequent hospital visits. The financial, intellectual, and emotional issues can be challenging for many families. It is important to recruit the assistance of a social worker when indicated to provide the family with information and resources.
Childhood/adolescent new patient evaluation
Because the modern day population is dynamic and mobile, patients frequently present as a new patients to cleft teams after already having received treatments. The cleft team coordinator and director must possess a thorough understanding of the major medical and psychosocial issues that patients with CL ± P may encounter. This knowledge allows for a timely and effective approach to the evaluation of such patients (see Fig. 12 ). Newly established PCPs should also be aware of the various issues their patients may encounter to ensure quality and individualized care for their steward.
Airway and Breathing
The airway of any patient with CL ± P needs to be carefully considered and monitored. A compromised airway can result in poor weight gain, low school performance, dysfunctional behaviors, and overall health detriment. Patients with CP are in an elevated risk category for airway anomalies, especially if there is an associated syndrome. Although maxillary and mandibular hypoplasias are frequently the source of obstruction, other anomalies can be present and require a systematic and comprehensive evaluation.
The incidence of obstructive sleep apnea (OSA) or sleep disturbed breathing is also elevated in CL ± P compared with the general population and should be screened for during any evaluation, especially in patients with an identified syndrome. In many cases, sleep-related problems can be corrected but require an appropriate work-up, including a referral to an otolaryngologist and cleft surgeon. A formal sleep study is often recommended. If adenotonsillectomy is indicated to provide relief for OSA or for other reasons, such as chronic tonsillitis, a partial (ie, superior) adenoidectomy should be performed to lessen the risk of postoperative hypernasality in this at-risk group.
Otology and Audiology
As discussed previously, hearing loss of various types is common in patients with clefts. Eustachian tube dysfunction may persist longer in CL ± P and has been found to often persist into adolescence and adulthood. As such, frequent audiometric and otologic evaluations, especially during the early developmental years, are important (see Fig. 12 ). Any new patient to a cleft team should receive an audiogram and otologic examination followed by indicated intervention.
In patients with tympanostomy tubes, evaluations should take place at least yearly. Tube otorrhea is common and routine treatments should be pursued. The development of atelectasis of the tympanic membrane, tympanic membrane perforations, cholesteatoma, and other otologic diseases have been found more common in CL ± P and need to be closely monitored throughout a child’s growth. Even in adolescence and adulthood, the incidence of otologic disease is high. Regular otologic and audiometric follow-up throughout development by an otolaryngologist and audiologist is important in many patients with CL ± P.
Speech
Speech and language are critical aspects for successful communication in society. Children born with CL ± P have an elevated risk of speech delay or disorder. It is important to evaluate speech for each patient with CL ± P in a timely fashion. A majority of speech disorders in CL ± P are related to either articulation disorder or velopharyngeal dysfunction (VPD), although other disorders are also commonly identified. Providing early intervention and therapy can prove beneficial in this population and should be pursued. Speech assessment starting at or before 18 months of age is recommended with reassessment every 6 to 12 months as indicated (see Fig. 12 ).
Dental/Orthodontics
Close attention needs to be paid to dental hygiene and care in patients with CL ± P. The ability of the patient to access appropriate dental care may, however, prove challenging. The complexity of care required may also be difficult for a general dentist to manage. A vast majority of patients with CL ± P, however, are able to receive routine dental care. The type of assessments and treatments vary depending on a patient’s age. Orthodontic care is required in most patients with CL ± P. The involvement of a craniofacial-trained orthodontist as a key member of the cleft team is essential. Most patients should begin routine dental care at approximately 2 years old and receive an evaluation by an orthodontist by 6 or 7 years of age (see Fig. 12 ).
Cleft Surgery
A surgeon’s involvement with a new patient who has already been managed by a different cleft team depends on the specific diagnosis and treatments already received. The outcomes of previous interventions as well as the age and maturity of the patient are heavily considered. The surgical procedures and techniques offered and performed during this period are described in detail later. Surgeries, such as lip revision, palatal fistula repair, speech surgery, and alveolar bone grafting, among others, may be considered at this time.
Neuropsychology
Less than average IQ scores have been identified in several children with CL ± P. As such, many struggle with schoolwork and are frequently found to have learning disabilities. It is important to assess each patient for evidence of cognitive and/or behavioral deficits that might benefit from further evaluation and treatment. This is typically evaluated once formal schooling has begun (see Fig. 12 ).
Social Work
Social adjustment has been found significantly affected in many children with clefts. There are also many challenges a patient’s family may be facing and access to social work should always be considered and offered. Children with CL ± P who are of school age are at particular risk of low self-esteem, negative peer relationships, and social isolation.
Nutrition
As described previously, the nutritional status of patients with CL ± P needs to be followed closely. Dietary habits should be inquired about as well as nutritional supplementation in these growing individuals. A nutritionist can also serve a critical role in preparation for procedures, such as palatoplasty and alveolar bone grafting, where dietary restrictions are frequently used (see Fig. 12 ).
Early interventions
Airway and Breathing
If a patient with CL ± P has associated PRS, difficulty with breathing and/or feeding may be present, and conservative measures should be attempted first. These measures are successful in managing approximately 70% of cases. Prone or side positioning is successful in alleviating the obstruction in at least half of all patients with PRS. If a patient continues to struggle, a nasopharyngeal airway can be placed to bypass the obstruction and allow for appropriate ventilation.
With continued respiratory difficulty and/or dysphagia, flexible laryngoscopy should be performed to confirm the suspicion of glossoptosis and rule out associated airway anomalies, which are present in greater than 20% of cases. Depending on the results of the endoscopic evaluation, surgery may be indicated.
Multiple surgical techniques are available to address the issues related to micrognathia in PRS. Surgery is a topic of much controversy and debate, however. Tongue-lip adhesion, mandibular distraction osteogenesis, and tracheotomy each have specific indications, benefits, and complications. The decision-making and surgical techniques for management of airway compromise in PRS are, however, beyond the scope of this article.
If a patient with PRS is having significant respiratory distress, intubation may be required. This intervention can be challenging and experienced personnel should be involved. Fortunately, with proper training and instrumentation, intubation can be safely performed in nearly all cases without resulting in emergent tracheotomy or death. Additional reasons, other than PRS, can exist for respiratory distress in patients with CL ± P and should be thoroughly explored using standard protocols.
Feeding
Early intervention for dysphagia or feeding disorders was described previously. A team approach to this problem is most effective, which may include a PCP or neonatologist, SLP, nutritionist, and cleft surgeon. The cause of dysphagia should be thoroughly explored and different options for management considered. Some patients may require prolonged nasogastric feeding whereas others may require GT placement (discussed previously).
Ears
As discussed previously, the incidence of failed hearing screens is high in the cleft population. A study by Chen and colleagues discovered that 28% of infants with CP failed their hearing screen. The failed hearing screen is frequently attributable to the presence of fluid, which is almost universally found in patients with CP. Chen also found, however, that 43% of infants with failed hearing screens who go on to have pressure equalization tube (PET) placement had persistent hearing loss in follow-up evaluations. The main associated findings that predicted hearing loss after tubes in this study were isolated CP, female gender, and a syndromic diagnosis.
If hearing loss is discovered, a thorough examination is required to evaluate a patient’s anatomy and determine pathology. A sedated audiometric brainstem response (ABR) test may be indicated at this point to determine the degree and type of hearing loss. In the presence of fluid, however, the ABR frequently shows a more severe hearing loss. In this setting, most otolaryngologists recommend PET placement, which can be accomplished during the same anesthetic as the sedated ABR. This approach is supported in a recent consensus statement by the American Academy of Otolaryngology Head and Neck Surgery.
Historically, early and frequent PET placement was recommended due to research in the 1960s by Paradise showing the universality of middle ear effusions in CP. Rates of chronic otitis media with effusion in greater than 90% of ears are consistently reported. The reasoning for treating middle ear effusions is to assist with language, communication, and psychosocial development as well as prevention of hearing loss and ear disease. Paradise showed improved hearing, higher IQ, and better language skills in children who underwent PET placement and had close follow-up. Tympanostomy tube placement was found to improve hearing results in CL ± P and is recommended. The authors follow this philosophy at their institution with a majority of CL ± P patients receiving PET either early at 3 months due to hearing loss in the presence of effusion or at the time of palatoplasty if effusion has been present for greater than 3 months (usually at 9 to 12 months of age).
Some recent literature has looked, however, at the possible consequences of PET placement and questioned the developmental, otologic, and audiologic benefits of treating middle ear effusions in CL ± P. A study by Phua and colleagues retrospectively compared 2 groups of patients with CP who underwent routine PET placement compared with a selective group. They found no difference in persistent CHL between the 2 groups; however, there was an increased incidence of otologic complications in the routine tympanostomy group and they recommend a more selective approach to PET placement. The speech and language benefits of PET placement for persistent effusion have also been questioned when looking at long-term outcomes; however, these findings were in noncleft populations.
Presurgical Orthopedics
Depending on the type and degree of cleft lip, presurgical orthopedics may be considered and provide benefit. Different options, such as lip taping, nasoalveolar molding (NAM), and lip adhesions, can assist with narrowing the cleft width and improving the affiliated soft tissue, aligning the alveolar margins and improving nostril formation and position ( Fig. 14 ). The decision to used any one of these techniques is frequently based on subjective evaluations, surgeon preference and training, family compliance, and resource availability. The use of these different methods to improve surgical preparation and postoperative outcomes is controversial with variable supportive data and is discussed further later.
