Cleft Palate: A Clinical Review Mahdi A. Shkoukani*1,2,3, Lauren A. Lawrence1, Daniel J. Liebertz1, and Peter F. Svider1

Orofacial clefts, including cleft palates (CP), are one of the most common birth defects. CP have a multiplicity of effects on the individual and society in terms of economic costs, loss of productivity, psychosocial effects, and increased morbidity and mortality at all stages of life. Embryological development of the palate is well delineated, with developments in the last decade regarding the biomolecular processes involved. Etiology is complex, involving a number of genetic and environmental factors. Various techniques can be employed for the repair of CP, depending on whether the cleft is of the primary or secondary palate, the width of the cleft, whether lengthening of the palate is necessary, and with regard to concerns of disruption of midfacial growth. All surgical techniques have the goals of restoring functional speech,

Introduction Second only to trisomy 21, orofacial clefts are one of the most common congenital anomalies, occurring in approximately 17 per 10,000 live births in the United States (Parker et al., 2010). The impacts of orofacial clefts on the individual and society are multifaceted, with lifetime costs approximated at $200,000, loss of productivity, increased utilization of mental health services, and increased morbidity and mortality at all stages of life (Wehby and Cassell, 2010; Leslie and Marazita, 2013). Additionally, patients with cleft palates are often afflicted with an associated syndrome and concurrent anomalies (Marazita and Mooney, 2004; Eppley et al., 2005; Merritt, 2005; Leslie and Marazita, 2013). Orofacial clefts are typically classified as cleft lip with or without cleft palate (CL/P) or isolated cleft palate (CP), as they differ epidemiologically and etiologically (Carinci et al., 2003; Merritt, 2005; Mossey et al., 2009). The embryology of the palate has long been delineated, but the past decade has brought noteworthy advances in the understanding of the biochemical mechanisms that underlie palatal development (Gritli-Linde, 2007; Meng et al., 2009; Bush and Jiang, 2012). 1 Department of Otolaryngology—Head and Neck Surgery, Wayne State University School of Medicine, Detroit, Michigan 2 Department of Otolaryngology—Head and Neck Surgery, Division of Craniofacial Surgery, Wayne State University School of Medicine, Detroit, Michigan 3 Division of Facial Plastic and Reconstructive Surgery, Wayne State University School of Medicine, Detroit, Michigan

Received: 17 September 2014 Accepted: 27 October 2014 *Correspondence to: Mahdi A. Shkoukani, Department of Otolaryngology— Head and Neck Surgery, Wayne State University School of Medicine, 4201 St. Antoine, 5E-UHC, Detroit, MI 48201. E-mail: [email protected] Published online 10 December 2014 in Wiley Online Library (wileyonlinelibrary. com). Doi: 10.1002/bdrc.21083

C 2014 Wiley Periodicals, Inc. V

swallowing, and aesthetics. A multidisciplinary team is necessary for the longterm pre- and postoperative care of CP patients to handle complications, associated anomalies, and to optimize function and quality of life. Birth Defects Research (Part C) 102:333–342, 2014. C 2014 Wiley Periodicals, Inc. V

Key words: orofacial cleft; cleft palate; congenital anomalies; craniofacial anomalies

The repair of cleft palates ideally involves an interdisciplinary team encompassing a variety of specialties and fields, including otolaryngologists, oral maxillofacial surgeons, plastic surgeons, pediatricians, speech pathologists, audiologists, social workers, geneticists, and psychologists (Marazita and Mooney, 2004). Goals of repair include restoring functional speech and swallowing, improving aesthetics and facial symmetry, and restoring competence of the velopharyngeal apparatus. The objective of this review is to discuss these common birth defects as they relate to clinical medicine.

Embryology Normal craniofacial and palatal development is a complex, but well-described process, with recent advances regarding knowledge of the cellular and molecular processes involved with palatogenesis (Gritli-Linde, 2007; Meng et al., 2009; Bush and Jiang, 2012). Palatal development takes place between the fifth and twelfth embryonic weeks, with the most critical period occurring during the sixth to ninth weeks (Merritt, 2005). The process of palatogenesis involves correct temporal and spatial distribution of a number of growth factors, signaling factors, and other biomolecular products, as well as appropriately timed cell growth, differentiation, migration, transformation, and apoptosis (Jugessur et al., 2009; Mossey et al., 2009; Tang et al., 2013; ten Dam et al., 2013). In the fourth week of embryonic growth, development of five facial prominences begins when neural crest cells migrate, under the regulation of a number of homeobox genes (Marazita and Mooney, 2004), from the neural tube to the craniofacial region. The five facial prominences are the frontonasal, the paired maxillary, and the paired mandibular (Dudas et al., 2007; Mossey et al., 2009). Molecular studies in mice and chicks have identified a role for fibroblast growth factors (FGFs), sonic hedgehog (SHH), bone

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TABLE 1. Classification Systems used to Describe Clefts of the Palate Veau

Class I: incomplete, involving only the soft palate Class II: involves only secondary palate, both hard and soft palate Class III: complete unilateral cleft of primary and secondary palate Class IV: complete bilateral cleft of primary and secondary palates

LAHSHAL

Designated as Lip, Alveolus, Hard Palate, Soft Palate, Hard Palate, Alveolus, Lip, assigned from right to left with upper case letters to indicate a complete cleft, lower case letters to designate an incomplete cleft, an asterisk to signify an incomplete cleft, and an “X” to designate a normal structure

Striped Y

Diagram in Y shape to represent a complete bilateral cleft of the primary and secondary palate, with subdivisions numbered 1 to 9, which can be stippled to designate degree of the cleft

Van der Meulen

Divides clefts based on stage of embyrologic disruption:

Tessier

Soft tissue and bony clefts each designated by num-

zita and Mooney, 2004). During the seventh week the jaw elongates, the tongue descends, and glycosaminoglycans are hydrated, causing the palatal shelves to rise to a horizontal position. They remain adhesion incompetent as they rise into the correct position, mediated by interferon regulatory factor 6 (IRF6) and protein jagged 2 (JAG2), preventing improper fusion (Mossey et al., 2009). In mice, IRF6 is expressed highly in the medial edge of the epithelium immediately before and after fusion (Jugessur and Murray, 2005). Once properly aligned and in contact, the palatal shelves fuse rapidly, mediated by transforming growth factor (TGF)-a, epidermal growth factor receptor (EGFR), TGF-b3, Wnt, E-cadherin, and N-cadherin (Carinci et al., 2003; Gritli-Linde, 2007; Meng et al., 2009; Mossey et al., 2009; Iwata et al., 2011; Tang et al., 2013). Midline cells then undergo apoptosis (Dudas et al., 2007). The palatal mesenchyme is then replaced by intramembranous bone formation, corresponding to the area of the hard palate, while the posterior section does not ossify and becomes the soft palate (Merritt, 2005; Mossey et al., 2009; Iwata et al., 2011). In normal development, the secondary palate has completed fusion with the lip and the nasal septum by the tenth week of embryologic development (Mossey et al., 2009) and is fully formed by the twelfth week (van Aalst et al., 2008).

internasal, nasal, nasomaxillary, or maxillary

bers 0 to 14

morphogenetic proteins (BMP), and retinoic acid (RA), among others, in the initiation and growth of the facial processes (Mossey et al., 2009). During the end of the fourth week the nasal placodes develop. The nasal placodes are ectodermal thickenings on the lower half of the frontonasal prominence. Nasal pits develop bilaterally within the placodes, with paired medial and lateral nasal processes developing from the frontonasal process on either side of the nasal placodes. At the end of the sixth week, the medial nasal processes fuse with each other, forming the philtrum, and at the end of the eighth week they fuse with the adjacent maxillary processes, forming the upper lip and primary palate. Just before fusion, the nasal processes have a peak in cell division, leading to an increased susceptibility to teratogenic insults with increased potential for failures of fusion. Failures of fusion at this stage will cause CL/P (Marazita and Mooney, 2004; Merritt, 2005; Dudas et al., 2007; Mossey et al., 2009; Shkoukani et al., 2013). The secondary palate begins to develop during the sixth week. Outgrowths of the maxillary prominences develop into the palatal shelves, which orient vertically alongside the tongue. Clefts of the secondary palate are due to either failure of elevation, failure of contact and adhesion, or failure of fusion of the palatal shelves (Mara-

Classification There are a number of classification systems used to describe clefts of the palate (Table 1). Because the lip and primary palate have embryologic development distinct from the secondary palate, cleft palate is often distinguished as cleft palate alone (CP), or in conjunction with cleft lip in the category of cleft lip with or without cleft palate (CL/P) (Mossey et al., 2009). CP and CL/P also have epidemiologic and genetic differences, though they are sometimes observed in the same family (Leslie and Marazita, 2013). Both CP and CL/P can be described as unilateral or bilateral and complete or incomplete. Clefts of the primary palate are those that occur anterior to the incisive foramen, and clefts of the secondary palate occur posterior to the incisive foramen (Friedman et al., 2010). Clefts of the palate are associated with variable levels of deformity and dysfunction. Clefts of the hard palate involve bony deficiency, and clefts of the soft palate involve the mucosa and muscles. The soft palate consists of five paired sets of muscles: levator veli palatini, tensor veli palatini, palatopharyngeus, palatoglossus, and muscularis uvulae (Huang et al., 1998; van Aalst et al., 2008). These muscles form a sling and are involved in the closure of the nasopharynx during speech and swallowing, and contribute to proper functioning of the eustachian tube. With CP, these muscles are absent, hypoplastic, or inserted abnormally, leading to lack of regular palatal function and subsequent velopharyngeal insufficiency (VPI) (Friedman

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FIGURE 1. Veau classification. (A) Class I—incomplete cleft, involving only the soft palate. (B) Class II—involving only the secondary palate, both hard and soft palate. (C) Class III—complete unilateral cleft of primary and secondary palate. (D) Class IV—complete bilateral cleft of primary and secondary palates.

et al., 2010). The least severe form of cleft palate is submucous cleft palate (SMCP), in which the palatal musculature underlying the mucosa is deficient and improperly attached. Associated features that may aid in diagnosis of SMCP include a bifid uvula, a zona pellucida (bluish midline region), and a notch in the posterior hard palate. While less severe, SMCP also has functional consequences and is also associated with VPI (Gosain et al., 1996; Ha et al., 2013). Various classification systems may be used to describe clefts, with optimal utility dependent on the pathology being described. One commonly used classification system for CP is the Veau classification (van Aalst et al., 2008; Davit et al., 2014). The Veau classification system categorizes CP into four types (Fig. 1). Veau class I is an incomplete cleft involving only the soft palate; a Veau class II cleft involves both the hard and soft palate and is limited to the secondary palate. Veau class III is a complete unilateral cleft of the primary and secondary palate, while Veau class IV is a complete bilateral cleft (van Aalst et al., 2008). Other classification systems involve number and lettering systems, and may be more useful for clefts that involve the lip and alveolus (Davit et al., 2014). A lettering system developed by Krein, LAHSHAL, stands for Lip, Alveolus, Hard Palate, Soft Palate, Hard Palate, Alveolus, Lip and is assigned from right to left with upper case letters to indicate a complete cleft, lower case letters to designate an incomplete cleft, an asterisk to signify an incomplete cleft, and an “X” to designate a normal structure (Davit et al., 2014). There are also numbering systems, such as the “striped Y” and associated modifications (Koch et al., 1995; Khan et al., 2013; Davit et al., 2014). Additional classification systems exist for orofacial clefts, including Van der Meulen’s classification and Tessier’s classifications (Eppley et al., 2005). Van der Meulen’s classification has an embryologic basis and delineates orofacial clefts based on the embryological stage of disruption, while Tessier’s classification is strictly descriptive and based on observation of anatomical disruption. Classification based on anatomy is often more useful for surgeons, while a focus on embryology may be of more interest to a

geneticist (Eppley et al., 2005). Clearly, a variety of classification systems are available, with the most relevant system likely dependent on the pathology present and the role of the medical professional.

Epidemiology Orofacial clefts, specifically CP and CL/P, are one of the most common birth defects, and when considered together, is the most common birth defect. The epidemiology of CP and CL/P are distinct, with an estimated prevalence of 6.35 CP per 10,000 live births and an estimated prevalence of 10.63 CL/P per 10,000 live births (Parker et al., 2010). Prevalence of CP is consistent among races, while CL/P varies, with the highest prevalence in Native Americans (3.6 in 1000 live births) and Asian Americans (2.1 in 1000 live births) and the lowest in African Americans (0.41 in 1000 live births) (van Aalst et al., 2008; Friedman et al., 2010). Prevalence also varies with geographic location, maternal age, teratogen exposure, and socioeconomic status (Wehby and Cassell, 2010). Additionally, there are gender differences in the incidence of CP and CL/P. Clefts are more common overall in males, as are CL/P, which occur at a 2:1 ratio. CP is more common in females; in female fetuses the palatal shelves take a week longer to fuse than in male fetuses, leaving more time for exposure to teratogens that can cause failure of fusion (Marazita and Mooney, 2004; Merritt, 2005; Gundlach and Maus, 2006; van Aalst et al., 2008). In both genders, clefts of the lip and primary palate are more common on the left (Gundlach and Maus, 2006; Lithovius et al., 2014). Epidemiologic data is often difficult to compile within the U.S. and internationally, as there is not a uniform structure for collecting and reporting data. In the U.S., the National Birth Defects Prevention Network pools population based data from over 30 different surveillance programs with different collection processes, separated into active and passive depending on the method used for including and confirming cases (Parker et al., 2010). One attempt at

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TABLE 2. Syndromes Associated with Clefts of the Palate

Syndrome Ankyloblepharon-ectodermal

References Cabiling et al. (2007)

dysplasia-clefting Apert

(van Aalst et al. (2008)

Bamforth-Lazarus

Venza et al. (2011)

Beckwith Wiedermann

Laroche et al. (2005)

Branchio-oculo-facial

Milunsky et al. (2008)

Cleft lip palate ectodermal dysplasia

Marwaha and Nanda (2012)

Conotruncal anomaly face

Marazita and Mooney (2004)

Cornelia de Lange

Yamamoto et al. (1987)

Crouzon

van Aalst et al. (2008)

Ectrodactyly ectodermal

Marwaha and Nanda (2012)

dysplasia-clefting Gorlin-Goetz

Lambrecht and Kreusch (1997)

Kabuki

Iida et al. (2006)

Miller-Dieker

Carter et al. (2000)

Pfeiffer

Stoler et al. (2009)

Popliteal pterygium

Leslie and Marazita (2013)

Rapp-Hodgkin

Clements et al. (2010)

Saethre–Chotzen

Gallagher et al. (1993)

Stickler

Basta et al. (2014)

Treacher Collin’s

van Aalst et al. (2008)

Van der Woude’s

Jugessur and Murray (2005)

Velocardiofacial

Marazita and Mooney (2004)

Wolf-Hirschhorn

Roberts et al. (2009)

delineating cleft incidence by race in Europe and worldwide noted that it was impossible to fully compare data (Gundlach and Maus, 2006). Smaller countries with centralized systems of collecting patient information have more consistent results, though these are specific only for a single population and epidemiology has been noted to be quite variable among different countries and populations (Gundlach and Maus, 2006; Lithovius et al., 2014; Lowry and Sibbald, 2014). For example, epidemiology of CL/P and CP is distinct in Finland, as compared with the rest of Scandinavia (Lithovius et al., 2014). Updated epidemiology on isolated cleft palate has noted a potential increase in incidence in the U.S., which has not been seen worldwide, though comments on the article note inconsistencies in data systems may skew extrapolation of results (Tanaka et al., 2013; Lowry and Sibbald, 2014; Mahabir et al., 2014).

Etiology A number of causative factors are related to the etiology of CL/P and CP, including genetics, teratogen exposure,

environmental factors, and maternal and paternal age. Despite the number of identifiable contributing factors, etiology of most clefts is multifactorial, complex, and remains to be completely delineated (Marazita and Mooney, 2004; Merritt, 2005). Most research has focused on the contribution of genetics to clefting, as there is increased risk of familial recurrence, with one affected parent having a 3 to 5% risk of having an affected child, and a 15% risk of having a second affected child if there is one affected sibling (Friedman et al., 2010). Genetic factors can be separated into syndromic and nonsyndromic. There are over 300 syndromes associated with CL/P and CP, with approximately half of those due to a single allele and inherited in a Mendelian fashion. Of these, 50% are autosomal recessive, 40% autosomal dominant, and 10% X-linked, and all are also complicated by other aspects of Mendelian inheritance, including reduced penetrance, variable expressivity, imprinting, allelic heterogeneity, and locus heterogeneity (Marazita and Mooney, 2004). The most common syndrome with CL/P or CP as an associated feature is Van der Woude’s syndrome (Friedman et al., 2010). Van der Woude’s syndrome is caused by a mutation in the interferon regulatory factor 6 gene, which has a role in fusion of the palatal shelves (Jugessur and Murray, 2005). It has also been speculated to repress TGF-b, which has an extensively studied role in palatal shelf fusion (Jugessur and Murray, 2005; Meng et al., 2009; Iwata et al., 2011; Tang et al., 2013). Mechanical disruptions contribute to the development of CP in other syndromes, such as in Pierre Robin sequence, which is characterized by the triad of micrognathia, CP, and glossoptosis. The micrognathia causes CP, as the growth of the jaw is important in the elevation of the palatal shelves (Merritt, 2005; Friedman et al., 2010). Other syndromes with associated CL/P or CP include Treacher Collin’s, Apert, and Crouzon syndromes, though there are also many others (Table 2) (van Aalst et al., 2008; Leslie and Marazita, 2013). Chromosomal rearrangements are also the cause of some of these syndromes, including velocardiofacial syndrome and contruncal anomaly face syndrome. Velocardiofacial syndrome and conotruncal anomaly face syndrome are phenotypically similar syndromes, both caused by a deletion at 22q11.2 (Marazita and Mooney, 2004). Many other chromosome deletions, duplications, or trisomies are also associated with clefts (Marazita and Mooney, 2004). Teratogen exposure and environmental factors also play a role during the first trimester when embryologic development of the face is occurring. Exposure to cortisol increases the risk of clefting 3.4-fold. In murine studies, cortisone has been shown to have an effect on the extracellular matrix and disrupt palatal shelf elevation. Anticonvulsant exposure also greatly increases the risk for CL/P and CP, with phenytoin specifically causing a 10-fold

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TABLE 3. Surgical Techniques for Cleft Palate Repair von Lagenback

Used for incomplete clefts of the secondary palate. Bipedicled mucoperiosteal flaps are elevated.

Intravelar veloplasty

Abnormally inserted velar muscles are returned to their proper anatomical position to improve velar and pharyngeal function.

VY pushback

Oral mucoperiosteal palatal flaps are elevated and

palatoplasty

moved medially and posteriorly in a V to Y fashion to increase length of the palate.

Two-flap palatoplasty Used for unilateral or bilateral for complete cleft palate. Oral mucoperiosteal flaps and nasal mucosal

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are somewhat at odds, and have led many surgeons to perform palatoplasty between 12 and 24 months (Leow and Lo, 2008). The first author prefers to repair the cleft palate around 9 months of age. Hodges suggested early combined repair in patients with cleft lip and palate to prevent patient loss to follow up after cleft lip repair (Hodges, 2010). In this study, 10 patients underwent only cleft lip repair, with only 1 of 10 returning for CP repair. The author suggested that parents recognized the importance of cleft lip repair for cosmetic purposes, but failed to understand the importance of CP repair, and that combined early repair would prevent the potential for loss of patients to follow up. Combined repair before 10 months in this study including 106 patients had no mortality or significant complications (Hodges, 2010).

flaps are elevated. Furlow double

Used for repair of the soft palate and for lengthen-

opposing

ing of the palate. Four triangular flaps are used,

Z–palatoplasty

two from each side of the palate, with one mucosal and one combined muscle and mucosal flap on each side.

increased risk (Eppley et al., 2005). Smoke and alcohol exposure are additional risks. Maternal smoking causes an at least twofold increase in the risk of CL/P and CP, with increased smoking causing increased risk. There is also interplay between teratogen exposure and genetics, as shown by a number of studies delineating combined effects of cigarette exposure with specific alleles for genes for TGF-a and MSX1 (Eppley et al., 2005; Wu et al., 2014). Additional contributing factors include maternal and paternal age. Maternal age less than 20 or greater than 39 years has been associated with increased risk. Increase in paternal age has also been noted to have a small but significant effect on the incidence of clefting (Merritt, 2005).

Timing of Surgery There is controversy related to the optimal timing of cleft palate repair, with varying recommendations, depending on which outcome is being considered (Trier and Dreyer, 1984; Strong and Buckmiller, 2001; Leow and Lo, 2008; Hodges, 2010). Goals of palatoplasty include the separation of the nasal cavity from the oral cavity, creation of a competent velopharyngeal valve for both speech and swallowing, and preservation of midface growth (Strong and Buckmiller, 2001; Campbell et al., 2010; Friedman et al., 2010). For optimal speech development, recommendations for palatoplasty are as early as 3 to 6 months but at least before 12 months, when language acquisition begins (Leow and Lo, 2008). In contrast, with regard to optimal midface growth, recommendations vary as late as 2 to 15 years, as completion of transverse midface growth is thought not to occur until 5 years of age. These two goals

Surgical Techniques Various techniques can be employed for the repair of CP, depending on whether the cleft is of the primary or secondary palate, the width of the cleft, whether lengthening of the palate is necessary, and with regard to concerns of disruption of midfacial growth (Table 3). Repeat procedures may be necessary to fully achieve functional speech, swallowing, and dental occlusion. Before definitive surgical repair, presurgical orthopedics may be beneficial. In unilateral clefts, the focus is on decreasing the cleft distance to ease primary repair. In bilateral clefts, presurgical orthopedics may be especially important to keep the premaxilla in place and to prevent it from migrating forward (Strong and Buckmiller, 2001). The von Lagenback technique (Fig. 2) has been implemented with various modifications, and is useful for repair of incomplete clefts of the secondary palate without cleft lip or alveolar involvement. The technique involves mobilizing bipedicled mucoperiosteal flaps medially for closure of the cleft (Trier and Dreyer, 1984; Strong and Buckmiller, 2001; Leow and Lo, 2008). One modification of the von Lagenback technique is the intravelar veloplasty (Strong and Buckmiller, 2001). In intravelar veloplasty, the velar muscles, which are abnormally inserted at the cleft margins and improperly oriented, are returned to their normal anatomical position. Restoring normal anatomy has the potential to improve both velar and pharyngeal function as the proper position of the levator and palatopharyngeus muscles optimizes the ability of the palate to elevate against the pharynx, and the reconstructed palatopharyngeus can correctly work with the superior constrictor for sphincter function of the pharynx (Huang et al., 1998). The Veau-Wardill-Kilner or VY pushback palatoplasty (Fig. 3) is another modification of the von Lagenback technique and can be used for increasing palatal length and soft palate mobility to improve velopharyngeal competence (Strong and Buckmiller, 2001). Oral mucoperiosteal palatal flaps are elevated and moved medially and

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FIGURE 2. The von Lagenback palatoplasty technique. (A) Markings for incisions. (B) Bipedicled mucoperiosteal flaps elevated bilaterally and moved medially with the aid of lateral relaxing incisions. (C) Closure of the nasal mucoperiosteal layer. (D) Closure of the oral mucoperiosteal layer.

FIGURE 3. V-Y pushback palatoplasty. (A) Markings for incisions. (B) Elevation of oral mucoperiosteal flaps with preservation of the greater palatine vessels. (C) Closure of nasal mucoperiosteal layer and repair of the levator veli palatini muscle (intravelar veloplasty). (D) Completed closure of the oral mucoperiosteal layer, with areas of denuded palatal bone visible.

posteriorly. Moving the flaps posteriorly allows for increased length of the palate, but exposure of the palate has effects on midfacial growth, which is one of the disadvantages of this technique. There are also higher rates of oronasal fistula than in other techniques, because of the single layer of mucosa anteriorly (Strong and Buckmiller, 2001; Leow and Lo, 2008). Two-flap palatoplasty (Fig. 4) is commonly used for unilateral or bilateral complete clefts (Leow and Lo, 2008). Oral mucoperiosteal flaps are elevated with preservation of the greater palatine vessels on both sides, and bilateral nasal mucosal flaps are elevated from the nasal surface of the hard palate. The nasal flaps are first reapproximated to cover the cleft, followed by the oral mucosal flaps. This technique is limited by the inability to add length to the palate, but can be used in combination with the intravelar veloplasty of the Furlow double opposing Z-palatoplasty, if additional palatal length is necessary to prevent VPI (Strong and Buckmiller, 2001; Leow and Lo, 2008). The Furlow double opposing Z–palatoplasty (Fig. 5) is used for repair of the soft palate (Strong and Buckmiller, 2001). It lengthens the soft palate and reconstructs the muscular sling. Four triangular flaps are used, two from each side of the palate, with one mucosal and one com-

bined muscle and mucosal flap on each side. The two flaps containing muscle are rotated posteriorly and the two mucosa-only flaps are transposed anteriorly. Closure is not anatomic, though speech outcomes are similar to other techniques. The Furlow double opposing Z-palatoplasty can also be used to correct velopharyngeal insufficiency in patients with SMCP (Gosain et al., 1996; Strong and Buckmiller, 2001; Leow and Lo, 2008; Friedman et al., 2010).

Complications Following surgery, patients are monitored for hemostasis and respiratory distress (Assael, 1995). Postoperative bleeding is a rare complication and blood loss during surgery is generally minimal at 50 to 60 mL (Assael, 1995; Friedman et al., 2010). There is potential for airway obstruction secondary to edema from prolonged tongue ischemia. This is especially of concern in some syndromic patients, including those with Pierre Robin sequence, Treacher Collin’s, and Crouzon’s syndrome, because they are already at risk for airway obstruction because of retrodisplacement of the tongue, micrognathia, or other craniofacial anomalies (van Aalst et al., 2008; Friedman et al., 2010). Following surgery, recovery can be complicated by development of oronasal fistula. The frequency of oronasal

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FIGURE 4. Two flap palatoplasty. (A) Markings for incisions. (B) Elevation of oral mucoperiosteal flaps with preservation of the greater palatine vessels. (C) Closure of nasal mucoperiosteal layer and repair of the levator veli palatini muscle (intravelar veloplasty). (D) Completed closure of the oral mucoperiosteal layer

FIGURE 5. Furlow double opposing Z-palatoplasty. (A) Markings for incisions. (B) Four triangular flaps are used, two from each side of the palate, with one mucosal and one combined muscle and mucosal flap on each side. (C) The two flaps containing muscle are rotated posteriorly and the two mucosa-only flaps are transposed anteriorly. (D) Final appearance after complete closure.

fistula is high, with reports ranging from 8.7% to 23%, though this varies with severity of clefting, type of cleft, and technique used. Patients with clefts of the hard and soft palate, and those with SMCP, are more likely to develop fistulas than those with clefts of the soft palate alone (Andersson et al., 2008). Fistulas are also more common as age of the patient undergoing surgery increases (Andersson et al., 2008). Common sites of fistulas are the anterior hard palate and the junction of the hard and soft palate (Friedman et al., 2010). The most common complication after CP repair is VPI, the rate of which also varies with the surgical technique employed and may reach as high as 25 to 30% (Phua and de Chalain, 2008). VPI has an effect on both speech and swallowing, causing hypernasal speech, and nasal regurgitation with swallowing. VPI can also reduce the intelligibility of speech, as the degree of opening and closure of the velopharyngeal sphincter is an important component of producing specific consonant sounds (Hopper et al., 2014).

Follow-Up Various aspects of CP care are important before and after surgery, and may need continual evaluation throughout

life. VPI is a common problem following cleft repair, and patients may require multiple forms of speech evaluation and speech therapy to optimize function. Evaluation techniques for assessment of velopharyngeal function include perceptual assessment by a trained speech pathologist, oral examination, nasoendoscopy, fluoroscopy, and nasometry. Each of these techniques provides different information on the structure, movement, and closure of the velopharyngeal apparatus and allow for judgments to be made regarding the utility of additional surgery or speech therapy (Hopper et al., 2014). Other associated problems that require follow-up include otolgic disease, dental deformities, facial growth deficiencies, and psychosocial issues (Kasten et al., 2008; Friedman et al., 2010). Otologic disease is common because of impaired function of the eustachian tube. Rates of otitis media are high, with one study reporting that 96% of children with cleft palates required tympanostomy tube placement (Friedman et al., 2010). Eustachian tube function takes, on average, 6 years to recover following palatoplasty, and other otologic complications, including tympanic membrane perforation, chronic suppurative otitis media, and cholesteatoma are more common in this population in the post-operative period (Friedman et al., 2010).

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Outcomes and Quality of Life Outcomes are most typically measured through physiciancentered means that focus on function, aesthetics, and rate of complications, but patient quality-of-life (QOL) outcome measures offer a different method of determining successful treatment. Patient QOL outcome measures include selfimage, integration in society, functional status, and speech and aesthetic perceptions (Munz et al., 2011; Shaye, 2014). Additionally, studies using a sample population and health utility assessment scores have equated the perceived level of disability associated with being born with CL/P or CP as similar to being born with monocular blindness (Sinno et al., 2012). Other studies have reported that children with CL/P or CP rate their own QOL as higher than control children do (Kramer et al., 2009). This is in contrast to reports that children with CP are at increased risk for developing psychopathology, including social anxiety disorder and major depressive disorder (Demir et al., 2011). Assessment of patient QOL outcomes will benefit from continued research. Barriers to care for CL/P or CP patients are also important aspects contributing to patient QOL. In one survey of caretakers of children with orofacial clefts in North Carolina, 48% of respondents reported traveling greater than 1 hour to receive cleft care (Cassell et al., 2013). An additional study reported that there is a small but significant delay in age at repair for patients who are publicly insured or of nonwhite race/ethnicity. Geographic and access disparities could adversely affect clinical outcomes and patient QOL, and would also benefit from further investigation (Abbott et al., 2011).

Conclusion Orofacial clefts are one of the most common congenital anomalies, occurring through a combination of genetic and environmental factors with variable phenotypic presentations and levels of disability and dysfunction. A number of surgical techniques may be employed with variable benefits and drawbacks, but all with the same goals of restoring functional speech, swallowing, and aesthetics. Patients typically require extensive pre- and postoperative care from a multidisciplinary team to optimize outcomes and QOL.

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Cleft palate: a clinical review.

Orofacial clefts, including cleft palates (CP), are one of the most common birth defects. CP have a multiplicity of effects on the individual and soci...
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