MOC-CME Evidence-Based Medicine: Cleft Palate Kyle J. Chepla, M.D. Arun K. Gosain, M.D. Cleveland, Ohio
Learning Objectives: After reading this article, the participant should be able to: 1. Describe recent changes in treatment of cleft palate. 2. Compare the efficacy of different surgical treatments. 3. Assess their own knowledge of cleft palate repair. 4. Determine where further individual in-depth study and development are warranted. Summary: The Maintenance of Certification in Plastic Surgery series is designed to ensure professional development and measure continued competency within a specialty or subspecialty. The present article provides an evaluation of the interval studies regarding the management of cleft palate with a specific focus on craniofacial growth, speech outcomes, and obstructive sleep apnea since the last Maintenance of Certification in Plastic Surgery article on the subject published in 2010. This purpose of this article is to update plastic and craniomaxillofacial surgeons on recent changes in treatment of cleft palate, provide a means for accurate self-assessment, and guide further individual indepth study and development. (Plast. Reconstr. Surg. 132: 1644, 2013.)
T
he incidence of isolated cleft palate is approximately 0.1 to 1.1 per 1000 births and varies by race, without a gender difference. Multiple risk factors have been associated with cleft palate and include genetic factors, teratogens (e.g., maternal alcohol intake, maternal tobacco use, certain antiepileptic medications), and several syndromes (i.e., Pierre Robin, velocardiofacial, Van der Woude, Apert, and Crouzon). Cleft palate can involve only the soft palate to varying degrees (incomplete cleft) or both the soft and hard palate to the level of the incisive foramen (complete cleft). Selection of the proper corrective surgical technique is patient dependent and should be based on the degree of palatal involvement and the distance between the palatal shelves. Repair of the hard palate typically uses various axial pattern flaps based on the greater palatine arteries, and commonly used techniques for repair of the hard palate include von Langenbeck palatoplasty,1 Veau-Wardill-Kilner pushback,2–5 Bardach two-flap palatoplasty,6 and others.7 Repair of the soft palate requires dissection of the anomalous palatal musculature to reposition the levator palatini with variable manipulation of the palatoglossus, palatopharyngeus, and tensor veli palatini to restore the levator sling. Restoration of From the Department of Plastic Surgery, University Hospitals Case Medical Center. Received for publication June 29, 2012; accepted July 13, 2012. Copyright © 2013 by the American Society of Plastic Surgeons DOI: 10.1097/PRS.0b013e3182a80952
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the normal muscular anatomy was first described by Braithwaite8 in 1964 and subsequently radically modified in 1970 by Kriens,9 who coined the term “intravelar veloplasty.” Again, there are many commonly used techniques for recreating the levator sling, including the double-opposing Z-plasty10,11 and those described by both Cutting et al.12 and Sommerlad et al.13,14 Regardless of the technique used, the goals of cleft palate repair remain as follows: (1) lengthen the palate to minimize the incidence of postoperative velopharyngeal insufficiency and promote proper speech development, (2) minimize maxillary and alveolar growth disturbances, and (3) prevent fistula formation. The previous cleft palate Maintenance of Certification in Plastic Surgery article published in 201015 reviewed evidence-based literature with a specific focus on preoperative assessment, anesthesia/analgesia, surgical technique, and patient outcomes. In this article, we evaluate the interval Disclosure: The authors have no financial interest to declare in relation to the content of this article.
Supplemental digital content is available for this article. Direct URL citations appear in the text; simply type the URL address into any Web browser to access this content. Clickable links to the material are provided in the HTML text of this article on the Journal’s Web site (www. PRSJournal.com).
www.PRSJournal.com
Volume 132, Number 6 • Cleft Palate literature and review articles with a high level of evidence on cleft surgery with a specific focus on maxillary growth, postoperative speech changes, and obstructive sleep apnea. We also provide a synopsis of our approach and treatment of a patient with cleft palate, and videos demonstrating the Furlow double-opposing Z-plasty in a patient with submucous cleft palate and a two-flap palatoplasty in a patient with complete bilateral cleft palate. (See Video, Supplemental Digital Content 1, which demonstrates Furlow palatoplasty, available in the “Related Videos” section of the full-text article on PRSJournal.com or, for Ovid users, at http://links.lww.com/PRS/A916; and Video, Supplemental Digital Content 2, which demonstrates two-stage palatoplasty, available in the “Related Videos” section of the full-text article on PRSJournal.com or, for Ovid users, at http://links.lww.com/PRS/A917.)
Video 1. Supplemental Digital Content 1, which demonstrates Furlow palatoplasty, is available in the “Related Videos” section of the full-text article on PRSJournal.com or, for Ovid users, at http://links.lww.com/PRS/A916.
ARTICLE SEARCH AND EVALUATION A search of the PubMed database was performed using the following terms: “cleft palate,” “obstructive sleep apnea,” “maxillary growth,” “maxillary development,” “craniofacial growth,” “veloplasty,” “velopharyngeal insufficiency,” and “speech.” The search was limited to studies published since the last Maintenance of Certification in Plastic Surgery article in 2009. All abstracts were reviewed to determine level of evidence as defined by the American Society of Plastic Surgeons Evidence Rating Scale for Therapy (Table 1) and were grouped by area of focus (maxillary growth, speech, obstructive sleep apnea). Studies of patients with bilateral cleft lip and palate were excluded from review; however, studies evaluating outcomes for patients with either isolated cleft palate or unilateral cleft lip and palate were included.
MAXILLARY AND FACIAL GROWTH Cleft palate is known to alter normal facial skeletal development. Two recent studies compared facial growth in patients after surgery,
Video 2. Supplemental Digital Content 2, which demonstrates two-stage palatoplasty, is available in the “Related Videos” section of the full-text article on PRSJournal.com or, for Ovid users, at http://links.lww.com/PRS/A917.
patients who are yet to undergo surgical repair, and nonclefted controls, to better elucidate skeletal changes. Chen et al.16 compared patients with uncorrected cleft palate to noncleft controls and demonstrated a significant reduction in maxillary sagittal length (anterior nasal spine to posterior nasal spine) and maxillary retrusion (decreased sella, nasion, A point angle) in the uncorrected
Table 1. American Society of Plastic Surgeons Evidence Rating Scale for Therapeutic Studies Level of Evidence I II III IV V
Qualifying Studies High-quality, multicenter or single-center, randomized controlled trial with adequate power; or systematic review of these studies Lesser-quality, randomized controlled trial; prospective cohort study; or systematic review of these studies Retrospective comparative study, case-control study, or systematic review of these studies Case series Expert opinion; case report or clinical example; or evidence based on physiology, bench research, or “first principles”
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Plastic and Reconstructive Surgery • December 2013 cleft group. Another study of dental-arch morphology in patients with surgically corrected cleft palate demonstrated a significantly decreased maxillary arch width and anterior arch length compared with nonclefted control patients and patients with uncorrected isolated cleft palate.17 More controversial is how surgical repair, particularly the timing, number of surgical stages, and technique used, impacts maxillary growth. Several authors argue that flap elevation damages the periosteum and that subsequent postsurgical scarring negatively impacts maxillary growth, which could be minimized using a two-stage approach with delayed repair of the hard palate.18–20 However, proponents of early, single-stage repair maintain that early closure improves speech outcomes through promotion of proper phonologic development and decreases articulation disorders associated with velopharyngeal insufficiency, and that the benefits of improved speech outcome outweigh potential maxillary growth restriction that may ensue.21 Several recent studies comparing one-stage versus two-stage cleft palate repair report conflicting results with respect to subsequent maxillary growth restriction. Yamanishi et al.19,22 compared growth outcomes following a two-stage repair consisting of Furlow palatoplasty at 12 months followed by hard palate closure at 18 months, to a single-stage Wardill-Kilner pushback at 12 months of age in 30 patients (Reference 19, Level of Evidence: Therapeutic, III). Cephalometric analysis at 4 years of age demonstrated a statistically significant increase in maxillary length and anterior facial height in patients who underwent a two-stage repair. In contrast, other studies have failed to demonstrate significant maxillary growth disturbance following single-stage elevation of palatal mucoperiosteal flaps. Pradel et al.21 found an increase in anterior and posterior transverse dental arch width at 6 years of age when singlestage intravelar veloplasty of the soft palate and bipedicled mucosal flap closure of the hard palate was performed at 9 to 12 months compared with patients undergoing two-stage repair. Liao et al.18 randomized 72 patients to either one-stage or two-stage cleft palate repair with delayed closure of the hard palate. In this study, serial cephalometric analysis until 20 years of age demonstrated no difference in growth rate between the groups but a decrease in maxillary length and protrusion in patients who underwent single-stage repair. De Mey et al.23 randomized 72 patients to single-stage closure at 3 months of age or two-stage repair with soft palate closure at 3 months and hard palate
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repair at 6 months of age (Level of Evidence: Therapeutic, II). Cephalometric data at age 15 years was used to compare the patients to nonclefted controls. At 10-year follow-up, the twostage repair demonstrated significantly reduced anteroposterior maxillary growth compared with controls; however, no significant difference was seen between the two surgical groups. Finally, no difference in sagittal growth on lateral cephalograms was seen in a study evaluating single-stage cleft palate repair versus two-stage repair at 12 months (soft palate) and 30 months (hard palate) in a study by Zemann et al. (Level of Evidence: Therapeutic, III).24 Another study examined the extent of mucoperiosteal flap elevation, which is believed to cause maxillary growth disturbances, in patients undergoing single-stage repair. Kulewicz and Dudkiewicz25 evaluated long-term morphologic outcomes using lateral cephalometric analysis after singlestage surgical correction at age 7 months in 66 patients with unilateral cleft lip and palate (Level of Evidence: Therapeutic, III). The patients were divided into three groups based on the technique of hard palate repair: group I, mucoperiosteal flap elevated on both sides of the cleft; group II, minimal (2 to 3 mm) mucoperiosteal dissection on the cleft side and normal dissection on the noncleft side; and group III, single-layer closure using vomer mucoperiosteal flaps and minimal palatal mucoperiosteal elevation bilaterally. Cephalometric analysis demonstrated a significant reduction in anteroposterior maxillary prominence (group I versus groups II and III) and anterior vertical maxillary height (group I versus group III). Group III had the lowest number of significant growth disturbances on cephalometric measurements compared with noncleft age-matched controls; however, there was no statistically significant difference between the three groups. Finally, evaluation of the impact of cleft size, as a percentage of total palatal surface area in 39 infants, using lateral and posteroanterior cephalometric radiographs, demonstrated a significant correlation between increased cleft size and decreased maxillary length and protrusion.26
SPEECH OUTCOMES A properly functioning velopharyngeal mechanism is critical to proper speech development and remains one of the primary goals of cleft palate repair. Repositioning of the levator sling and recreation of the normal anatomy results in velopharyngeal competence for most patients
Volume 132, Number 6 • Cleft Palate and reduces the need for secondary pharyngeal flaps.27 In a study by Sullivan et al.,28 persistent postoperative velopharyngeal insufficiency was shown to be associated with patient age at palatoplasty (timing), and degree of palatal involvement (Veau score) (Level of Evidence: Therapeutic/ Prognostic, IV). They conclude that early, singlestage repair reduces the development of learned, compensatory misarticulations that can persist despite surgical correction of the palatal anatomy, but must be weighted against altered facial growth secondary to early disruption of the maxillary periosteum and soft-tissue envelope. Willadsen29 recently reviewed the impact of the timing of hard palate repair on speech outcomes. Thirty-four children underwent two-stage cleft surgery with velar repair at 4 months of age and repair of the hard palate at either 12 months (early) or 36 months (late) (Level of Evidence: Therapeutic, II). Evaluation 18 months postoperatively demonstrated that the group that underwent late repair of the hard palate produced fewer labial stops and more velar stops, fewer vocalizations, and consonants permissible in word-initial position. Thirty-six months postoperatively, this group demonstrated a severely restricted phonologic system with more cleft speech characteristics. Pradel et al.21 evaluated speech outcomes after either a single- or two-stage cleft palate repair (Level of Evidence: Therapeutic, III). Twelve children underwent a two-stage repair with intravelar veloplasty at age 9 to 12 months and repair of the hard palate using bipedicled flaps at age 24 to 36 months, and 12 children underwent a single-stage intravelar veloplasty and bipedicled flap repair at age 9 to 12 months. At 4 years of age, patients who underwent single-stage repair demonstrated less altered resonance and less nasal emission. When reevaluated at age 6 years, the children who underwent two-stage repair had improved their speech skills but still had not attained a level equal to patients who had undergone single-stage palate repair. This is in contrast to a study by Yaminishi et al.19 that compared 30 patients who underwent early two-stage double-opposing Z-plasty at 12 months and hard palate repair at 18 months, to 42 patients who underwent single-stage WardillKilner push-back palatoplasty at 12 months of age. In this study, no statistically significant difference was seen in the incidence of velopharyngeal insufficiency or articulation errors at 4 years of age. Two techniques of palatal repair were compared by Williams et al.,30 who randomized patients to either a Furlow double-opposing Z-plasty or a von Langenbeck palatoplasty with intravelar
veloplasty (Level of Evidence: Therapeutic, II). Postoperative velopharyngeal function, measured using a cul-de-sac test of hypernasality and mirror test of nasal air emission, was significantly improved in the group that had undergone Furlow double-opposing Z-palatoplasty.
OBSTRUCTIVE SLEEP APNEA A recent retrospective study examined the incidence of sleep-disordered breathing or obstructive sleep apnea in 459 cleft palate patients and demonstrated a significantly increased incidence of sleep-disordered breathing (37.5 percent) and obstructive sleep apnea (8.5 percent) compared with age-matched controls (Level of Evidence: Prevalence, IV).31 No recent studies have evaluated how surgical repair of the cleft palate may help or worsen this condition.
CONCLUSIONS The present literature review demonstrates that many of the important issues surrounding repair of the cleft palate remain controversial. Since the last Maintenance of Certification in Plastic Surgery article on cleft palate repair by Liau et al.,15 there is still no consensus in the interval literature regarding the proper timing of repair, number of stages, or surgical technique(s) to maximize speech outcomes and minimize the impact on long-term skeletal growth and facial development. Although cleft severity will always vary between patients, we believe that there should not be an unstandardized approach to surgical repair. We echo the assessment by Yang and Liao32 who, following a literature review of facial growth after one- or two-stage palate repair, concluded that there is still a need for well-designed, randomized, prospective, long-term studies (Level of Evidence: Therapeutic, III). Studies with a high level of evidence will possibly one day guide selection of surgical technique and the timing of repair to optimize surgical and patient outcomes. In the absence of such a consensus, we advocate a single-stage repair of the hard and soft palate at 9 to 12 months of age as demonstrated in the accompanying videos. (See Video, Supplemental Digital Content 1, http://links.lww.com/PRS/ A916; and Video, Supplemental Digital Content 2, http://links.lww.com/PRS/A917.) We believe that early surgical repair significantly improves speech outcomes and that there is currently insufficient evidence demonstrating a clinically relevant maxillary growth disturbance relative to two-stage
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Plastic and Reconstructive Surgery • December 2013 or late repairs. We institute regular orthodontic follow-up through a multidisciplinary team beginning at age 3 years, allowing us to monitor facial and dental development carefully and initiate palatal expansion with the onset of mixed dentition when necessary. Regular visits through the multidisciplinary team can identify and prepare patients who may require subsequent orthognathic surgery. We believe that late correction of skeletal deficiency can yield a normal facial profile, whereas late correction of refractory errors secondary to prolonged velopharyngeal insufficiency is a much more difficult prospect, with far less satisfying results. Arun K. Gosain, M.D. 225 E. Chicago Avenue, Box 93 Chicago, Ill. 60611 [email protected]
ACKNOWLEDGMENT
The authors offer special thanks to Matt Sugerik for help in recording and editing the surgical videos. REFERENCES 1. Von Langenbeck B. Die uranoplastik mittles ablosung des microsperiostalen gaumenuberzuges. Arch Klin Chir. 1862;2:205–287. 2. Veau V, Ruppie C. Anatomie chirurgicale de la division palatine: Considerations operatoires. Rev Chir. 1922;20:1–30. 3. Veau V. Division Palatine. Paris: Masson; 1931. 4. Kilner TP. Cleft lip and palate repair technique. In: Maingot R, ed. Postgraduate Surgery. Vol. 3. London: Medical Publishers; 1937:3800–3827. 5. Wardill WEM. Technique of operation for cleft lip and palate. Br J Surg. 1937;25:117–130. 6. Bardach J, Salyer KE. Cleft palate repair. In: Bardach J, Salyer KE, eds. Surgical Techniques in Cleft Lip and Palate. St. Louis: Mosby; 1991:224–273. 7. Sommerlad BC. A technique for cleft palate repair. Plast Reconstr Surg. 2003;112:1542–1548. 8. Braithwaite F. Cleft palate repair. In: Gibson T, ed. Modern Trends in Plastic Surgery. London: Butterworths; 1964:30–49. 9. Kriens OB. Fundamental anatomic findings for an intravelar veloplasty. Cleft Palate J. 1970;7:27–36. 10. Furlow LT Jr. Cleft palate repair by double opposing Z-plasty. Plast Reconstr Surg. 1986;78:724–738. 11. Furlow LT. Cleft palate repair by double opposing Z-plasty. Oper Tech Plast Reconstr Surg. 1995;2:223–230. 12. Cutting CB, Rosenbaum J, Rovati L. The technique of muscle repair in the cleft soft palate. Operative Tech Plast Reconstr Surg. 1995;2:215–222. 13. Sommerlad BC, Mehendale FV, Birch MJ, Sell D, Hattee C, Harland K. Palate re-repair revisited. Cleft Palate Craniofac J. 2002;39:295–307. 14. Fisher DM, Sommerlad BC. Cleft lip, cleft palate, and velopharyngeal insufficiency. Plast Reconstr Surg. 2011;128:342e–360e. 15. Liau JY, Sadove AM, van Aalst JA. An evidence-based approach to cleft palate repair. Plast Reconstr Surg. 2010;126:2216–2221.
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16. Chen ZQ, Qian YF, Wang GM, Shen G. Sagittal maxillary growth in patients with unoperated isolated cleft palate. Cleft Palate Craniofac J. 2009;46:664–667. 17. Ye B, Ruan C, Hu J, et al. A comparative study on dental-arch morphology in adult unoperated and operated cleft palate patients. J Craniofac Surg. 2010;21:811–815. 18. Liao YF, Yang IY, Wang R, Yun C, Huang CS. Two-stage palate repair with delayed hard palate closure is related to favorable maxillary growth in unilateral cleft lip and palate. Plast Reconstr Surg. 2010;125:1503–1510. 19. Yamanishi T, Nishio J, Sako M, et al. Early two-stage double opposing Z-plasty or one-stage push-back palatoplasty? Comparisons in maxillary development and speech outcome at 4 years of age. Ann Plast Surg. 2011;66:148–153. 20. Friede H, Lilja J, Lohmander A. Long-term, longitudinal follow-up of individuals with UCLP after the Gotenburg Primary Early Velopalsty and Delayed Hard Palate Closure Protocol: Maxillofacial growth outcome. Cleft Palate Craniofac J. 2012;49:649–656. 21. Pradel W, Senf D, Mai R, Ludicke G, Eckelt U, Lauer G. Onestage palate repair improves speech outcome and early maxillary growth in patients with cleft lip and palate. J Physiol Pharmacol. 2009;60(Suppl 8):37–41. 22. Yamanishi T, Nishio J, Kohara H, et al. Effect on maxillary arch development of early 2-stage palatoplasty by modified furlow technique and conventional 1-stage palatoplasty in children with complete unilateral cleft lip and palate. J Oral Maxillofac Surg. 2009;67:2210–2216. 23. De Mey A, Franck D, Cuylits N, Swennen G, Malevez C, Lejour M. Early one-stage repair of complete unilateral cleft lip and palate. J Craniofac Surg. 2009;20(Suppl 2):1723–1728. 24. Zemann W, Kärcher H, Drevenšek M, Koželj V. Sagittal maxillary growth in children with unilateral cleft of the lip, alveolus and palate at the age of 10 years: An intercentre comparison. J Craniomaxillofac Surg. 2011;39:469–474. 25. Kulewicz M, Dudkiewicz Z. Craniofacial morphological outcome following treatment with three different surgical protocols for complete unilateral cleft lip and palate: A preliminary study. Int J Oral Maxillofac Surg. 2010;39:122–128. 26. Liao YF, Prasad NK, Chiu YT, Yun C, Chen PK. Cleft size at the time of palate repair in complete unilateral cleft lip and palate as an indicator of maxillary growth. Int J Oral Maxillofac Surg. 2010;39:956–961. 27. Nyberg J, Westberg LR, Neovius E, Larson O, Henningsson G. Speech results after one-stage palatoplasty with or without muscle reconstruction for isolated cleft palate. Cleft Palate Craniofac J. 2010;47:92–103. 28. Sullivan SR, Marrinan EM, LaBrie RA, Rogers GF, Mulliken JB. Palatoplasty outcomes in nonsyndromic patients with cleft palate: A 29-year assessment of one surgeon’s experience. J Craniofac Surg. 2009;20(Suppl 1):612–616. 29. Willadsen E. Influence of timing of hard palate repair in a two-stage procedure on early language development in Danish children with cleft palate. Cleft Palate Craniofac J. 2012;49:574–595. 30. Williams WN, Seagle MB, Pegoraro-Krook MI, et al. Prospective clinical trial comparing outcome measures between Furlow and von Langenbeck palatoplasties for UCLP. Ann Plast Surg. 2011;66:154–163. 31. Robison JG, Otteson TD. Increased prevalence of obstructive sleep apnea in patients with cleft palate. Arch Otolaryngol Head Neck Surg. 2011;137:269–274. 32. Yang IY, Liao YF. The effect of 1-stage versus 2-stage palate repair on facial growth in patients with cleft lip and palate: A review. Int J Oral Maxillofac Surg. 2010;39:945–950.
Learning Objectives: After reading this article, the participant should be able to: 1. Describe recent changes in treatment of cleft palate. 2. Compare the efficacy of different surgical treatments. 3. Assess their own knowledge of cleft palate repair. 4. Determine where further individual in-depth study and development are warranted. Summary: The Maintenance of Certification in Plastic Surgery series is designed to ensure professional development and measure continued competency within a specialty or subspecialty. The present article provides an evaluation of the interval studies regarding the management of cleft palate with a specific focus on craniofacial growth, speech outcomes, and obstructive sleep apnea since the last Maintenance of Certification in Plastic Surgery article on the subject published in 2010. This purpose of this article is to update plastic and craniomaxillofacial surgeons on recent changes in treatment of cleft palate, provide a means for accurate self-assessment, and guide further individual indepth study and development. (Plast. Reconstr. Surg. 132: 1644, 2013.)
T
he incidence of isolated cleft palate is approximately 0.1 to 1.1 per 1000 births and varies by race, without a gender difference. Multiple risk factors have been associated with cleft palate and include genetic factors, teratogens (e.g., maternal alcohol intake, maternal tobacco use, certain antiepileptic medications), and several syndromes (i.e., Pierre Robin, velocardiofacial, Van der Woude, Apert, and Crouzon). Cleft palate can involve only the soft palate to varying degrees (incomplete cleft) or both the soft and hard palate to the level of the incisive foramen (complete cleft). Selection of the proper corrective surgical technique is patient dependent and should be based on the degree of palatal involvement and the distance between the palatal shelves. Repair of the hard palate typically uses various axial pattern flaps based on the greater palatine arteries, and commonly used techniques for repair of the hard palate include von Langenbeck palatoplasty,1 Veau-Wardill-Kilner pushback,2–5 Bardach two-flap palatoplasty,6 and others.7 Repair of the soft palate requires dissection of the anomalous palatal musculature to reposition the levator palatini with variable manipulation of the palatoglossus, palatopharyngeus, and tensor veli palatini to restore the levator sling. Restoration of From the Department of Plastic Surgery, University Hospitals Case Medical Center. Received for publication June 29, 2012; accepted July 13, 2012. Copyright © 2013 by the American Society of Plastic Surgeons DOI: 10.1097/PRS.0b013e3182a80952
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the normal muscular anatomy was first described by Braithwaite8 in 1964 and subsequently radically modified in 1970 by Kriens,9 who coined the term “intravelar veloplasty.” Again, there are many commonly used techniques for recreating the levator sling, including the double-opposing Z-plasty10,11 and those described by both Cutting et al.12 and Sommerlad et al.13,14 Regardless of the technique used, the goals of cleft palate repair remain as follows: (1) lengthen the palate to minimize the incidence of postoperative velopharyngeal insufficiency and promote proper speech development, (2) minimize maxillary and alveolar growth disturbances, and (3) prevent fistula formation. The previous cleft palate Maintenance of Certification in Plastic Surgery article published in 201015 reviewed evidence-based literature with a specific focus on preoperative assessment, anesthesia/analgesia, surgical technique, and patient outcomes. In this article, we evaluate the interval Disclosure: The authors have no financial interest to declare in relation to the content of this article.
Supplemental digital content is available for this article. Direct URL citations appear in the text; simply type the URL address into any Web browser to access this content. Clickable links to the material are provided in the HTML text of this article on the Journal’s Web site (www. PRSJournal.com).
www.PRSJournal.com
Volume 132, Number 6 • Cleft Palate literature and review articles with a high level of evidence on cleft surgery with a specific focus on maxillary growth, postoperative speech changes, and obstructive sleep apnea. We also provide a synopsis of our approach and treatment of a patient with cleft palate, and videos demonstrating the Furlow double-opposing Z-plasty in a patient with submucous cleft palate and a two-flap palatoplasty in a patient with complete bilateral cleft palate. (See Video, Supplemental Digital Content 1, which demonstrates Furlow palatoplasty, available in the “Related Videos” section of the full-text article on PRSJournal.com or, for Ovid users, at http://links.lww.com/PRS/A916; and Video, Supplemental Digital Content 2, which demonstrates two-stage palatoplasty, available in the “Related Videos” section of the full-text article on PRSJournal.com or, for Ovid users, at http://links.lww.com/PRS/A917.)
Video 1. Supplemental Digital Content 1, which demonstrates Furlow palatoplasty, is available in the “Related Videos” section of the full-text article on PRSJournal.com or, for Ovid users, at http://links.lww.com/PRS/A916.
ARTICLE SEARCH AND EVALUATION A search of the PubMed database was performed using the following terms: “cleft palate,” “obstructive sleep apnea,” “maxillary growth,” “maxillary development,” “craniofacial growth,” “veloplasty,” “velopharyngeal insufficiency,” and “speech.” The search was limited to studies published since the last Maintenance of Certification in Plastic Surgery article in 2009. All abstracts were reviewed to determine level of evidence as defined by the American Society of Plastic Surgeons Evidence Rating Scale for Therapy (Table 1) and were grouped by area of focus (maxillary growth, speech, obstructive sleep apnea). Studies of patients with bilateral cleft lip and palate were excluded from review; however, studies evaluating outcomes for patients with either isolated cleft palate or unilateral cleft lip and palate were included.
MAXILLARY AND FACIAL GROWTH Cleft palate is known to alter normal facial skeletal development. Two recent studies compared facial growth in patients after surgery,
Video 2. Supplemental Digital Content 2, which demonstrates two-stage palatoplasty, is available in the “Related Videos” section of the full-text article on PRSJournal.com or, for Ovid users, at http://links.lww.com/PRS/A917.
patients who are yet to undergo surgical repair, and nonclefted controls, to better elucidate skeletal changes. Chen et al.16 compared patients with uncorrected cleft palate to noncleft controls and demonstrated a significant reduction in maxillary sagittal length (anterior nasal spine to posterior nasal spine) and maxillary retrusion (decreased sella, nasion, A point angle) in the uncorrected
Table 1. American Society of Plastic Surgeons Evidence Rating Scale for Therapeutic Studies Level of Evidence I II III IV V
Qualifying Studies High-quality, multicenter or single-center, randomized controlled trial with adequate power; or systematic review of these studies Lesser-quality, randomized controlled trial; prospective cohort study; or systematic review of these studies Retrospective comparative study, case-control study, or systematic review of these studies Case series Expert opinion; case report or clinical example; or evidence based on physiology, bench research, or “first principles”
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Plastic and Reconstructive Surgery • December 2013 cleft group. Another study of dental-arch morphology in patients with surgically corrected cleft palate demonstrated a significantly decreased maxillary arch width and anterior arch length compared with nonclefted control patients and patients with uncorrected isolated cleft palate.17 More controversial is how surgical repair, particularly the timing, number of surgical stages, and technique used, impacts maxillary growth. Several authors argue that flap elevation damages the periosteum and that subsequent postsurgical scarring negatively impacts maxillary growth, which could be minimized using a two-stage approach with delayed repair of the hard palate.18–20 However, proponents of early, single-stage repair maintain that early closure improves speech outcomes through promotion of proper phonologic development and decreases articulation disorders associated with velopharyngeal insufficiency, and that the benefits of improved speech outcome outweigh potential maxillary growth restriction that may ensue.21 Several recent studies comparing one-stage versus two-stage cleft palate repair report conflicting results with respect to subsequent maxillary growth restriction. Yamanishi et al.19,22 compared growth outcomes following a two-stage repair consisting of Furlow palatoplasty at 12 months followed by hard palate closure at 18 months, to a single-stage Wardill-Kilner pushback at 12 months of age in 30 patients (Reference 19, Level of Evidence: Therapeutic, III). Cephalometric analysis at 4 years of age demonstrated a statistically significant increase in maxillary length and anterior facial height in patients who underwent a two-stage repair. In contrast, other studies have failed to demonstrate significant maxillary growth disturbance following single-stage elevation of palatal mucoperiosteal flaps. Pradel et al.21 found an increase in anterior and posterior transverse dental arch width at 6 years of age when singlestage intravelar veloplasty of the soft palate and bipedicled mucosal flap closure of the hard palate was performed at 9 to 12 months compared with patients undergoing two-stage repair. Liao et al.18 randomized 72 patients to either one-stage or two-stage cleft palate repair with delayed closure of the hard palate. In this study, serial cephalometric analysis until 20 years of age demonstrated no difference in growth rate between the groups but a decrease in maxillary length and protrusion in patients who underwent single-stage repair. De Mey et al.23 randomized 72 patients to single-stage closure at 3 months of age or two-stage repair with soft palate closure at 3 months and hard palate
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repair at 6 months of age (Level of Evidence: Therapeutic, II). Cephalometric data at age 15 years was used to compare the patients to nonclefted controls. At 10-year follow-up, the twostage repair demonstrated significantly reduced anteroposterior maxillary growth compared with controls; however, no significant difference was seen between the two surgical groups. Finally, no difference in sagittal growth on lateral cephalograms was seen in a study evaluating single-stage cleft palate repair versus two-stage repair at 12 months (soft palate) and 30 months (hard palate) in a study by Zemann et al. (Level of Evidence: Therapeutic, III).24 Another study examined the extent of mucoperiosteal flap elevation, which is believed to cause maxillary growth disturbances, in patients undergoing single-stage repair. Kulewicz and Dudkiewicz25 evaluated long-term morphologic outcomes using lateral cephalometric analysis after singlestage surgical correction at age 7 months in 66 patients with unilateral cleft lip and palate (Level of Evidence: Therapeutic, III). The patients were divided into three groups based on the technique of hard palate repair: group I, mucoperiosteal flap elevated on both sides of the cleft; group II, minimal (2 to 3 mm) mucoperiosteal dissection on the cleft side and normal dissection on the noncleft side; and group III, single-layer closure using vomer mucoperiosteal flaps and minimal palatal mucoperiosteal elevation bilaterally. Cephalometric analysis demonstrated a significant reduction in anteroposterior maxillary prominence (group I versus groups II and III) and anterior vertical maxillary height (group I versus group III). Group III had the lowest number of significant growth disturbances on cephalometric measurements compared with noncleft age-matched controls; however, there was no statistically significant difference between the three groups. Finally, evaluation of the impact of cleft size, as a percentage of total palatal surface area in 39 infants, using lateral and posteroanterior cephalometric radiographs, demonstrated a significant correlation between increased cleft size and decreased maxillary length and protrusion.26
SPEECH OUTCOMES A properly functioning velopharyngeal mechanism is critical to proper speech development and remains one of the primary goals of cleft palate repair. Repositioning of the levator sling and recreation of the normal anatomy results in velopharyngeal competence for most patients
Volume 132, Number 6 • Cleft Palate and reduces the need for secondary pharyngeal flaps.27 In a study by Sullivan et al.,28 persistent postoperative velopharyngeal insufficiency was shown to be associated with patient age at palatoplasty (timing), and degree of palatal involvement (Veau score) (Level of Evidence: Therapeutic/ Prognostic, IV). They conclude that early, singlestage repair reduces the development of learned, compensatory misarticulations that can persist despite surgical correction of the palatal anatomy, but must be weighted against altered facial growth secondary to early disruption of the maxillary periosteum and soft-tissue envelope. Willadsen29 recently reviewed the impact of the timing of hard palate repair on speech outcomes. Thirty-four children underwent two-stage cleft surgery with velar repair at 4 months of age and repair of the hard palate at either 12 months (early) or 36 months (late) (Level of Evidence: Therapeutic, II). Evaluation 18 months postoperatively demonstrated that the group that underwent late repair of the hard palate produced fewer labial stops and more velar stops, fewer vocalizations, and consonants permissible in word-initial position. Thirty-six months postoperatively, this group demonstrated a severely restricted phonologic system with more cleft speech characteristics. Pradel et al.21 evaluated speech outcomes after either a single- or two-stage cleft palate repair (Level of Evidence: Therapeutic, III). Twelve children underwent a two-stage repair with intravelar veloplasty at age 9 to 12 months and repair of the hard palate using bipedicled flaps at age 24 to 36 months, and 12 children underwent a single-stage intravelar veloplasty and bipedicled flap repair at age 9 to 12 months. At 4 years of age, patients who underwent single-stage repair demonstrated less altered resonance and less nasal emission. When reevaluated at age 6 years, the children who underwent two-stage repair had improved their speech skills but still had not attained a level equal to patients who had undergone single-stage palate repair. This is in contrast to a study by Yaminishi et al.19 that compared 30 patients who underwent early two-stage double-opposing Z-plasty at 12 months and hard palate repair at 18 months, to 42 patients who underwent single-stage WardillKilner push-back palatoplasty at 12 months of age. In this study, no statistically significant difference was seen in the incidence of velopharyngeal insufficiency or articulation errors at 4 years of age. Two techniques of palatal repair were compared by Williams et al.,30 who randomized patients to either a Furlow double-opposing Z-plasty or a von Langenbeck palatoplasty with intravelar
veloplasty (Level of Evidence: Therapeutic, II). Postoperative velopharyngeal function, measured using a cul-de-sac test of hypernasality and mirror test of nasal air emission, was significantly improved in the group that had undergone Furlow double-opposing Z-palatoplasty.
OBSTRUCTIVE SLEEP APNEA A recent retrospective study examined the incidence of sleep-disordered breathing or obstructive sleep apnea in 459 cleft palate patients and demonstrated a significantly increased incidence of sleep-disordered breathing (37.5 percent) and obstructive sleep apnea (8.5 percent) compared with age-matched controls (Level of Evidence: Prevalence, IV).31 No recent studies have evaluated how surgical repair of the cleft palate may help or worsen this condition.
CONCLUSIONS The present literature review demonstrates that many of the important issues surrounding repair of the cleft palate remain controversial. Since the last Maintenance of Certification in Plastic Surgery article on cleft palate repair by Liau et al.,15 there is still no consensus in the interval literature regarding the proper timing of repair, number of stages, or surgical technique(s) to maximize speech outcomes and minimize the impact on long-term skeletal growth and facial development. Although cleft severity will always vary between patients, we believe that there should not be an unstandardized approach to surgical repair. We echo the assessment by Yang and Liao32 who, following a literature review of facial growth after one- or two-stage palate repair, concluded that there is still a need for well-designed, randomized, prospective, long-term studies (Level of Evidence: Therapeutic, III). Studies with a high level of evidence will possibly one day guide selection of surgical technique and the timing of repair to optimize surgical and patient outcomes. In the absence of such a consensus, we advocate a single-stage repair of the hard and soft palate at 9 to 12 months of age as demonstrated in the accompanying videos. (See Video, Supplemental Digital Content 1, http://links.lww.com/PRS/ A916; and Video, Supplemental Digital Content 2, http://links.lww.com/PRS/A917.) We believe that early surgical repair significantly improves speech outcomes and that there is currently insufficient evidence demonstrating a clinically relevant maxillary growth disturbance relative to two-stage
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Plastic and Reconstructive Surgery • December 2013 or late repairs. We institute regular orthodontic follow-up through a multidisciplinary team beginning at age 3 years, allowing us to monitor facial and dental development carefully and initiate palatal expansion with the onset of mixed dentition when necessary. Regular visits through the multidisciplinary team can identify and prepare patients who may require subsequent orthognathic surgery. We believe that late correction of skeletal deficiency can yield a normal facial profile, whereas late correction of refractory errors secondary to prolonged velopharyngeal insufficiency is a much more difficult prospect, with far less satisfying results. Arun K. Gosain, M.D. 225 E. Chicago Avenue, Box 93 Chicago, Ill. 60611 [email protected]
ACKNOWLEDGMENT
The authors offer special thanks to Matt Sugerik for help in recording and editing the surgical videos. REFERENCES 1. Von Langenbeck B. Die uranoplastik mittles ablosung des microsperiostalen gaumenuberzuges. Arch Klin Chir. 1862;2:205–287. 2. Veau V, Ruppie C. Anatomie chirurgicale de la division palatine: Considerations operatoires. Rev Chir. 1922;20:1–30. 3. Veau V. Division Palatine. Paris: Masson; 1931. 4. Kilner TP. Cleft lip and palate repair technique. In: Maingot R, ed. Postgraduate Surgery. Vol. 3. London: Medical Publishers; 1937:3800–3827. 5. Wardill WEM. Technique of operation for cleft lip and palate. Br J Surg. 1937;25:117–130. 6. Bardach J, Salyer KE. Cleft palate repair. In: Bardach J, Salyer KE, eds. Surgical Techniques in Cleft Lip and Palate. St. Louis: Mosby; 1991:224–273. 7. Sommerlad BC. A technique for cleft palate repair. Plast Reconstr Surg. 2003;112:1542–1548. 8. Braithwaite F. Cleft palate repair. In: Gibson T, ed. Modern Trends in Plastic Surgery. London: Butterworths; 1964:30–49. 9. Kriens OB. Fundamental anatomic findings for an intravelar veloplasty. Cleft Palate J. 1970;7:27–36. 10. Furlow LT Jr. Cleft palate repair by double opposing Z-plasty. Plast Reconstr Surg. 1986;78:724–738. 11. Furlow LT. Cleft palate repair by double opposing Z-plasty. Oper Tech Plast Reconstr Surg. 1995;2:223–230. 12. Cutting CB, Rosenbaum J, Rovati L. The technique of muscle repair in the cleft soft palate. Operative Tech Plast Reconstr Surg. 1995;2:215–222. 13. Sommerlad BC, Mehendale FV, Birch MJ, Sell D, Hattee C, Harland K. Palate re-repair revisited. Cleft Palate Craniofac J. 2002;39:295–307. 14. Fisher DM, Sommerlad BC. Cleft lip, cleft palate, and velopharyngeal insufficiency. Plast Reconstr Surg. 2011;128:342e–360e. 15. Liau JY, Sadove AM, van Aalst JA. An evidence-based approach to cleft palate repair. Plast Reconstr Surg. 2010;126:2216–2221.
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16. Chen ZQ, Qian YF, Wang GM, Shen G. Sagittal maxillary growth in patients with unoperated isolated cleft palate. Cleft Palate Craniofac J. 2009;46:664–667. 17. Ye B, Ruan C, Hu J, et al. A comparative study on dental-arch morphology in adult unoperated and operated cleft palate patients. J Craniofac Surg. 2010;21:811–815. 18. Liao YF, Yang IY, Wang R, Yun C, Huang CS. Two-stage palate repair with delayed hard palate closure is related to favorable maxillary growth in unilateral cleft lip and palate. Plast Reconstr Surg. 2010;125:1503–1510. 19. Yamanishi T, Nishio J, Sako M, et al. Early two-stage double opposing Z-plasty or one-stage push-back palatoplasty? Comparisons in maxillary development and speech outcome at 4 years of age. Ann Plast Surg. 2011;66:148–153. 20. Friede H, Lilja J, Lohmander A. Long-term, longitudinal follow-up of individuals with UCLP after the Gotenburg Primary Early Velopalsty and Delayed Hard Palate Closure Protocol: Maxillofacial growth outcome. Cleft Palate Craniofac J. 2012;49:649–656. 21. Pradel W, Senf D, Mai R, Ludicke G, Eckelt U, Lauer G. Onestage palate repair improves speech outcome and early maxillary growth in patients with cleft lip and palate. J Physiol Pharmacol. 2009;60(Suppl 8):37–41. 22. Yamanishi T, Nishio J, Kohara H, et al. Effect on maxillary arch development of early 2-stage palatoplasty by modified furlow technique and conventional 1-stage palatoplasty in children with complete unilateral cleft lip and palate. J Oral Maxillofac Surg. 2009;67:2210–2216. 23. De Mey A, Franck D, Cuylits N, Swennen G, Malevez C, Lejour M. Early one-stage repair of complete unilateral cleft lip and palate. J Craniofac Surg. 2009;20(Suppl 2):1723–1728. 24. Zemann W, Kärcher H, Drevenšek M, Koželj V. Sagittal maxillary growth in children with unilateral cleft of the lip, alveolus and palate at the age of 10 years: An intercentre comparison. J Craniomaxillofac Surg. 2011;39:469–474. 25. Kulewicz M, Dudkiewicz Z. Craniofacial morphological outcome following treatment with three different surgical protocols for complete unilateral cleft lip and palate: A preliminary study. Int J Oral Maxillofac Surg. 2010;39:122–128. 26. Liao YF, Prasad NK, Chiu YT, Yun C, Chen PK. Cleft size at the time of palate repair in complete unilateral cleft lip and palate as an indicator of maxillary growth. Int J Oral Maxillofac Surg. 2010;39:956–961. 27. Nyberg J, Westberg LR, Neovius E, Larson O, Henningsson G. Speech results after one-stage palatoplasty with or without muscle reconstruction for isolated cleft palate. Cleft Palate Craniofac J. 2010;47:92–103. 28. Sullivan SR, Marrinan EM, LaBrie RA, Rogers GF, Mulliken JB. Palatoplasty outcomes in nonsyndromic patients with cleft palate: A 29-year assessment of one surgeon’s experience. J Craniofac Surg. 2009;20(Suppl 1):612–616. 29. Willadsen E. Influence of timing of hard palate repair in a two-stage procedure on early language development in Danish children with cleft palate. Cleft Palate Craniofac J. 2012;49:574–595. 30. Williams WN, Seagle MB, Pegoraro-Krook MI, et al. Prospective clinical trial comparing outcome measures between Furlow and von Langenbeck palatoplasties for UCLP. Ann Plast Surg. 2011;66:154–163. 31. Robison JG, Otteson TD. Increased prevalence of obstructive sleep apnea in patients with cleft palate. Arch Otolaryngol Head Neck Surg. 2011;137:269–274. 32. Yang IY, Liao YF. The effect of 1-stage versus 2-stage palate repair on facial growth in patients with cleft lip and palate: A review. Int J Oral Maxillofac Surg. 2010;39:945–950.
