CLEFT LIP and PALATE REPAIR

Transcription

1 CLEFT LIP and PALATE REPAIR Policy Number: 2016M0040A Effective Date: February 1, 2016 Table of Contents: Page: Cross Reference Policy: POLICY DESCRIPTION 2 Genetic Counseling, 2015M0039A COVERAGE RATIONALE/CLINICAL CONSIDERATIONS 2 BACKGROUND 3 REGULATORY STATUS 5 CLINICAL EVIDENCE 5 APPLICABLE CODES 10 REFERENCES 11 POLICY HISTORY/REVISION INFORMATION 13 Pediatric Dental Anesthesia, 2015M0090A Reconstructive-Cosmetic Health Services, 2015M0084A INSTRUCTIONS: Medical Policy assists in administering UCare benefits when making coverage determinations for members under our health benefit plans. When deciding coverage, all reviewers must first identify enrollee eligibility, federal and state legislation or regulatory guidance regarding benefit mandates, and the member specific Evidence of Coverage (EOC) document must be referenced prior to using the medical policies. In the event of a conflict, the enrollee's specific benefit document and federal and state legislation and regulatory guidance supersede this Medical Policy. In the absence of benefit mandates or regulatory guidance that govern the service, procedure or treatment, or when the member s EOC document is silent or not specific, medical policies help to clarify which healthcare services may or may not be covered. This Medical Policy is provided for informational purposes and does not constitute medical advice. In addition to medical policies, UCare also uses tools developed by third parties, such as the InterQual Guidelines, to assist us in administering health benefits. The InterQual Guidelines are intended to be used in connection with the independent professional medical judgment of a qualified health care provider and do not constitute the practice of medicine or medical advice. Other Policies and Coverage Determination Guidelines may also apply. UCare reserves the right, in its sole discretion, to modify its Policies and Guidelines as necessary and to provide benefits otherwise excluded by medical policies when necessitated by operational considerations. Page. 1 of 14

2 POLICY DESCRIPTION: This policy provides information on cleft lip and palate, birth defects that affect the upper lip and the roof of the mouth. A cleft lip may be just a small notch in the lip. It may also be a complete split in the lip that goes all the way to the base of the nose. A cleft palate can be on one or both sides of the roof of the mouth. It may go the full length of the palate. Other defects may include changes in nose shape and/or poorly aligned teeth. Problems that may be present because of a cleft lip or palate include: Failure to gain weight Feeding problems Flow of milk through nasal passages during feeding Poor growth Repeated ear infections Speech difficulties COVERAGE RATIONALE / CLINICAL CONSIDERATIONS: Treatment of Cleft Lip and Cleft Palate is considered MEDICALLY NECESSARY for dependent children, to the limiting age in the definition of Dependent Children. A. The surgical repair of a cleft lip and/or cleft palate is considered MEDICALLY NECESSARY and, therefore, covered to correct a congenital defect. The surgical repair plan may consist of serial surgeries as part of a reconstructive plan to achieve optimal functional outcomes; these surgeries may be considered MEDICALLY NECESSARY and, therefore, covered. Rhinoplasty may be considered a MEDICALLY NECESSARY component of the reconstructive plan when an individual has a significant impairment of nasal function. The surgical repair of any portion of the face performed solely to change appearance, in the absence of a reconstructive plan for a cleft lip and/or cleft palate, without the expectation of improving physiologic functioning may be considered COSMETIC and, therefore, is a benefit contract exclusion. Photographs demonstrating the deformity may be required to determine coverage. B. Pre-surgical oral appliances (Durable Medical Equipment) for maxillary repair to facilitate palatal expansion in preparation for bone graft surgery of the alveolar cleft, and post-surgical appliances to support bone grafting are considered MEDICALLY NECESSARY. Appliances provided pre- or post-repair of the cleft lip and/or cleft palate typically consist of two phases: Pre-surgical orthodontics: aligns the maxillary alveolar segments prior to bone graft surgery. Post-surgical orthodontics: supports the bone graft and keeps the maxillary alveolar segments in the desired position until the grafts have stabilized. Replacement of appliances required for cleft lip and/or cleft palate repair is considered MEDICALLY NECESSARY when the replacement is due to the growth of the individual. Page. 2 of 14

3 C. Dental and Orthodontic services directly related to the cleft are considered MEDICALLY NECESSARY when the treatment is directly related to the cleft. D. Dental and Orthodontic services which are not required for the treatment of the cleft lip or cleft palate are considered NOT MEDICALLY NECESSARY for this treatment. Note that other coverage provisions may apply. Note: See section Regulatory Status/Mandated Benefits Under Minnesota Law/Chapter 62A. Accident and Health Insurance. 62A.042 Family Coverage for coverage provisions related to the cleft lip and cleft palate (Page 6). Clinical Considerations: The individual's medical record must reflect the medical necessity for the care provided. These medical records may include, but are not limited to: records from the health care professional's office, hospital, nursing home, home health agencies, therapies, and test reports. Photographs demonstrating the deformity may be required to determine coverage. Relative contraindications: 1. Current illness or other medical condition that may interfere with general anesthesia 2. Possible compromise of the airway in a child with a preexisting airway problem (such as severe micrognathia) 3. Severe developmental delay 4. A short life expectancy because of other severe illnesses Laboratory Studies: Routine laboratory studies are noncontributory in otherwise healthy infants with cleft palate. Some centers obtain a blood count as a routine study before performing surgery on a child with cleft palate. The authors do not find this necessary unless some other associated medical conditions coexist. Imaging Studies: Routine imaging studies are noncontributory in otherwise healthy infants who undergo primary cleft palate repair. Diagnostic Procedures: Early collaboration with an audiologist and an otolaryngologist, including examination and early audiologic assessment, prevents long-term hearing deficits. BACKGROUND: Cleft lip and/or cleft palate occurs in 1 of every 600 babies born annually, making it the most common birth defect in the United States. Cleft lip and cleft palate are conditions affecting the upper lip and the roof of the mouth. A cleft or gap forms when the developing facial structures do not join (fuse) fully during gestation. Cleft lip affects the top lip and may present as a small gap (partial or incomplete cleft lip) or larger gap that extends into the nose (complete cleft lip). Cleft lip can occur as 1 (unilateral) or 2 (bilateral) clefts. Cleft lip occurs during the formation of the primary palate when the maxillary and medial nasal processes fail to fuse completely (Pham and Senders, 2006; Cleft Palate Foundation, 2012). Cleft palate affects the roof of the mouth. The soft palate is usually affected. In most cases of cleft palate, cleft lip is also present (Pham and Senders, 2006). Cleft palate may involve both the soft and hard palates and may also include a gap in the jaw. The hole in the roof of the mouth caused by a cleft palate connects the mouth Page. 3 of 14

4 directly to the nasal cavity. The shape of the nose may be affected, as well as the alignment of the teeth. Cleft palate occurs during the formation of the secondary palate when the lateral palatine processes, the nasal septum, and/or the median palatine processes fail to fuse completely (Pham and Senders, 2006). Prenatal ultrasound often detects cases of cleft lip and/or cleft palate in utero; however, some cases may not be detected until the newborn examination (Aminpour and Tollefson, 2008; Kasten et al., 2008). Cleft lip and cleft palate commonly occur as isolated conditions, but may occur in association with genetic disorders. Complications arising from cleft lip or palate include feeding difficulty, failure to gain weight, poor growth, conductive hearing deficits, and speech difficulties (Kasten et al., 2008). There are many causes of cleft lip and palate. Problems with genes passed down from one or both parents, drugs, viruses, or other toxins can all cause these birth defects. Cleft lip and palate may occur along with other syndromes or birth defects. Traditionally, multiple surgeries are performed to provide a functional and an aesthetically acceptable correction of cleft lip and/or cleft palate. Repair of the defect is critical to meet the individual's nutritional needs and facilitate emerging expressive language skills. There is agreement in the medical literature that a long delay in repair is not prudent because postponement may produce less-than-optimal functional and aesthetic outcomes. Delayed repair can also result in facial asymmetry due to retarded growth and/or disturbed mid-facial development. Moreover, postponement in correction may contribute to other problems, such as reduced senses of taste and smell, and hearing loss due to recurrent auditory canal infections, which are a direct result of palatal clefts. Additionally, it has been observed that significant numbers of infants with cleft lip and/or cleft palate develop maxillary hypoplasia, which may require orthognathic surgical correction; therefore, surgical treatment of the defect should occur when the facial bones are still growing. Treatment is usually done before a child is 12 months old. Because cleft lip and/or cleft palate defects can occur singly or in combination with varying degrees of complexity, the surgical repair plan is individualized and may consist of a single surgery or a series of surgeries. Repair Of Cleft Lip (Cheiloplasty): This surgical repair is typically performed immediately after birth or in early infancy (6-12 weeks of age). Nostril deformities, which are often present with cleft lips, can be improved at the time of the cheiloplasty or at a later surgery. Repair Of Cleft Palate (Palatoplasty): This surgical repair is typically performed between nine months and two years of age, and may require several stages, including: Pre-surgical maxillary repair to facilitate palatal expansion in preparation for bone graft surgery of the alveolar cleft Maxillary alveolar bone grafting The use of post-surgical appliances to support bone grafting Rhinoplasty: A surgical procedure to reshape the nose, rhinoplasty is sometimes necessary as part of the corrective repair of cleft lip and/or cleft palate. Rhinoplasty may be provided at the time of the original surgery or at a later revisional surgery. Revisional Surgery: Typically, as part of a reconstructive plan, the defects of cleft lip and/or cleft palate are repaired by the time the child has reached adulthood, but there may be circumstances where revisional surgeries may be required after the individual has reached adulthood. Revisional surgeries consist of serial Page. 4 of 14

5 surgeries as part of the reconstructive plan, and are generally performed to improve function. REGULATORY STATUS: 1. U.S. FOOD AND DRUG ADMINISTRATION (FDA): Surgical procedures for cleft lip and palate repair are not subject to FDA regulation. However, any medical devices, drugs, or tests used as part of this procedure may be subject to FDA regulation. 2. CENTERS FOR MEDICARE AND MEDICAID SERVICES (CMS): There is no Medicare Coverage Determination addressing cleft lip or cleft palate repair. 3. MINNESOTA DEPARTMENT OF HUMAN SERVICES (DHS): Comprehensive orthodontic treatment (through age 20), is considered medically necessary when adequate corrective treatment is not achievable with less extensive means, and dentition is affected by significant cleft palate, craniofacial or other congenital or developmental disorder. Authorization and documentation (including x-rays) is always required. 4. MANDATED BENEFITS UNDER MINNESOTA LAW: Chapter 62A. Accident and Health Insurance. 62A.042 Family Coverage. Available at Accessed December 22, Current health insurance benefit mandates in Minnesota law, which apply to private, fully-insured group and nongroup policies, require benefits for inpatient or outpatient expenses, arising from hospital, medical and dental services for cleft lip or cleft palate to be covered by health plans. In the case of deformities known as cleft lip or cleft palate, benefits will be available for dependent children for inpatient medical and dental treatment, including orthodontic and oral surgery treatment, for services that were scheduled or initiated prior to the dependent reaching age 19. Benefits for individuals age 19, up to the limiting age for coverage of the dependent, are limited to inpatient or outpatient expenses arising from medical and dental treatment that was scheduled or initiated prior to the dependent turning age 19. If orthodontic services are eligible for coverage under a dental insurance plan and another policy or contract, the dental plan shall be primary and the other policy or contract shall be secondary in regard to the coverage required. Payment for dental or orthodontic treatment not related to the management of the congenital condition of cleft lip and cleft palate shall not be covered under this provision. CLINICAL EVIDENCE: The presence of cleft palate has both aesthetic and functional implications for patients in their social interactions, particularly on their ability to communicate effectively and on their facial appearance with or without involvement of the lip. Midfacial skeletal growth may be affected by the surgical repair of the palate. The treatment plan focuses on two areas: speech development and facial growth. Speech development is paramount in the appropriate management of cleft palate. Many surgical techniques and Page. 5 of 14

6 modifications have been advocated to improve functional outcome and aesthetic results. The most controversial issues in the management of cleft palate are the timing of surgical intervention, speech development after various surgical procedures, and the effects of surgery on facial growth. The major goals of surgical intervention are normal speech, minimizing growth disturbances, and establishing a competent velopharyngeal sphincter. The surgeon must always keep in mind that in as many as 29% of patients, the child with cleft palate may have other anomalies. These may be more commonly associated with isolated cleft palate than with cleft lip/palate. High among the associated anomalies are those affecting the circulatory and skeletal systems. The Procedure A topic for debate has been over the treatment of the alveolar cleft that accompanies the cleft palate. The rationale for its closure includes stabilizing the maxillary arch, providing support for tooth eruption and postsurgical orthodontics, closing oronasal fistulae, and improving the aesthetics of the mid face and nose. The current trend is toward secondary bone grafting at the time of mixed dentitia, with early (primary) grafting potentially proving detrimental to midfacial growth. Much discussion has centered over the role and timing of presurgical appliances. Both the hard palate and the alveolus can be molded with passive molds and active devices, with the shared ultimate goals of facilitating surgical repair and providing an improved long-term outcome in both facial form and palatal function. Epidemiology The incidence of cleft lip/palate by race is 2.1/1000 in Asians, 1/1000 in whites, and 0.41/1000 in blacks. Isolated cleft palate shows a relatively constant ratio of /1000 births. The foremost type of clefting is a bifid uvula, occurring in 2% of the population. The second most frequent type is a left unilateral complete cleft of the palate and prepalatal structures. Midline clefts of the soft palate and parts of the hard palate are also common. Complete clefts of the secondary palate are twice as common in females as in males while the reverse is true of velar clefts. About 7-13% of patients with isolated cleft lip and 11-14% of patients with cleft lip/palate have other anomalies at birth. Recurrence risks for clefting deformities do not correspond to any Mendelian pattern of inheritance and it would appear that clefting is inherited heterogeneously. This observation is supported by evidence from studies of twins that indicate the relative roles of genetic and nongenetic influences of cleft development. For isolated cleft palate and combined cleft lip and palate, if the proband has no other affected first- or second-degree relatives, the empiric risk of a sibling being born with a similar malformation is 3-5%. However, if a proband with a combined cleft lip and palate has other affected first-degree relatives, the risk for siblings or subsequent offspring is 10-20%. Etiology In 25% of patients, there is a family history of facial clefting, which does not follow either a normal recessive or dominant pattern. The condition appears to be multifactorial. Some instances of clefting may be because of an overall reduction in the volume of the facial mesenchyme, which leads to clefting by virtue of failure of mesodermal penetration. In some patients, clefting appears to be associated with increased facial width, either alone or in association with encephalocele, idiopathic hypertelorism, or the presence of a teratoma. The characteristic U-shaped cleft of the Pierre Robin anomaly is thought to be dependent upon a persistent high position of the tongue, perhaps associated with a failure or delay of neck extension. This prevents descent of the tongue, which in turn prevents elevation and a medial growth of the palatal shelves. Page. 6 of 14

7 The production of clefts of the secondary palate in experimental animals has frequently been accomplished by drug administration. Agents commonly used are steroids, anticonvulsants, diazepam, and aminopterin. Phenytoin and diazepam may also be causative factors in clefting in humans. Infections during the first trimester of pregnancy, such as rubella or toxoplasmosis, have been associated with clefting. Pathophysiology The pathologic sequelae of cleft palate include feeding and nutritional difficulties, recurrent ear infections, hearing loss, abnormal speech development, and facial growth distortion. The communication between the oral and nasal chamber impairs the normal sucking and swallowing mechanism of the cleft infants. Food particles reflux into the nasal chamber. The abnormal insertion of the tensor veli palati prevents satisfactory emptying of the middle ear. Recurrent ear infections have been implicated in the hearing loss of patients with cleft palate. The hearing loss may worsen the speech pathology in these patients. Evidence that repair of the cleft palate decreases the incidence of middle ear effusions is inconsistent. However, these problems are overshadowed by the magnitude of the speech and facial growth problems. Speech abnormalities are intrinsic to the anatomic derangement of cleft palate. The facial growth distortion appears to be, to a great extent, secondary to surgical interventions. Along with an intact hard palate, an intact velopharyngeal mechanism is essential in production of high pressure consonants and the oral resonance of vowels. The velopharyngeal mechanism must also remain open to some degree to accomplish nasal resonance of m, n, and ng. With connected spontaneous speech, oral and nasal speech contexts are rapidly coarticulated, resulting in the complex need for millisecond timing of velopharyngeal closure and opening, depending on the speech targets. Experimentation with this velopharyngeal activity begins with babbling and continues through the early language learning years. Without sufficient velopharyngeal function during this early learning period, compensatory strategies may be adopted, with consonants produced in the pharynx to create pressure buildup. These compensatory strategies are difficult to change, and always require speech therapy intervention, often with the need for additional surgical reconstruction. Multiple studies have demonstrated that the cleft palate maxilla has some intrinsic deficiency of growth potential. This intrinsic growth potential varies from isolated cleft of the palate to complete cleft lip and palate. This growth potential is further impaired by surgical repair. Any surgical intervention performed prior to completion of full facial growth can have significant deleterious effects on maxillary growth. Disagreement exists as to the appropriate timing of surgery to minimize the harmful effects on facial growth and on what type of surgical intervention is most responsible for growth impairment. The formation of scar and scar contracture in the areas of denuded palatal bones are most frequently blamed for restriction of maxillary expansion. The growth disturbance is exhibited most prominently in the prognathic appearance during the second decade of life despite the normal appearance in early childhood. The discrepant occlusion relationship between the maxilla and the mandible is usually not amenable to nonsurgical correction. Indications Children born with a cleft palate should undergo surgical repair unless otherwise contraindicated. The main goal is to perform a functional repair of the soft palate musculature through the repositioning of the abnormally-oriented and attached muscles. This anatomic repair attempts to facilitate the development of normal speech. While separation of the oral and nasal cavities is advantageous to normalize feeding and decrease regurgitation and nasal irritation, it is not absolutely necessary for feeding. Page. 7 of 14

8 Palate repair with repositioning of the palatal musculature may be advantageous to eustachian tube function and ultimately to hearing. Because the levator and the tensor veli palatini have their origins along the eustachian tube, repositioning improves function of these muscles, improves ventilation of the middle ear, and decreases serous otitis, which further decreases the incidence of hearing abnormality. Palate repair alone does not usually completely correct this dysfunction and additional therapy frequently includes placement of ear tubes as necessary. Medical Therapy The Pierre Robin sequence is classically associated with retrognathia, glossoptosis, respiratory distress, and a cleft palate. If untreated, death may result from obstruction by the tongue, which has fallen back in the airway. The most appropriate first step in management is to place the infant in the prone position to allow the tongue to fall forward and clear the trachea. Orthodontic interventions The available data suggest that to optimize speech development, some degree of facial growth distortion may need to be accepted. One role of orthodontic intervention is to minimize the severity of the growth disturbance. Interventions vary according to the type of cleft. Many types of orthodontic appliances have been used in the treatment of patients with cleft palate. In cleft lip/palate, orthodontic appliances can be used to realign the premaxilla into a normal position prior to lip closure. Orthodontic interventions in patients with cleft palate are frequently aimed at maxillary arch expansion, correction of malocclusion, and correction of an often developing class III skeletal growth pattern. The maxillary dental arch contracture may become significant, requiring the surgical repair of the hard palate. Orthodontic interventions may be started early or delayed for several years. When orthodontic manipulation is initiated early, difficulties may occur. Maintaining orthodontic appliances in the infant population may present a challenge unless these appliances are fixed in position. The beneficial influence of these orthopedic interventions has also been questioned, especially in isolated patients with cleft palate. The most beneficial period for orthodontic interventions in isolated cleft palate may be during the mixed dentition period. At approximately age 6-8 years, the permanent incisors are erupting. During this period, children are beginning to have social interactions with their peers. The presence of grossly malaligned teeth and severe malocclusion can lead to social isolation. The incisor relation can be corrected and maintained with relatively simple interventions. Patients who undergo palatal arch expansion therapy during this period can benefit from the rapid growth phase. The orthodontic intervention can also proceed with more cooperation from the patient in this age group. Orthodontic management of arch deformities after the permanent dentition has erupted is more limited. The established malocclusion and asymmetry between the maxillary arch and mandibular arch usually require orthognathic surgery. Surgical Therapy Timing of palatal closure The timing of surgical repair of cleft palate remains controversial. The goals of palatal repair include normal speech, normal palatal and facial growth, and normal dental occlusion. Physicians believe that early palate repair is associated with better speech results but early repair also tends to produce severe dentofacial deformities. Randall and McComb as well as Lehman and colleagues consistently reported that children whose palates were repaired at an earlier age appeared to have better speech and needed fewer secondary pharyngoplasties then those whose surgeries had been delayed beyond the first 12 months. Page. 8 of 14

9 Noordhoff and associates found that children undergoing delayed palatoplasty for cleft palate had significantly poorer articulation skills before the hard palate closure than children of the same age who did not have clefts. These benefits of early cleft palate repair from the standpoint of speech and hearing must be weighed against the increased technical difficulty of the procedure at a younger age and possible adverse effects on maxillary growth. Numerous studies failed to demonstrate an observable difference in underdevelopment of the palatal arch among children undergoing operations at various ages. The surgical intervention appears to interfere with midfacial growth without regard to the age of the patient at the time surgery is performed. Sequence of operations Multiple protocols for the management of CL/P have been suggested over the years by various authors. Today, the mainstream of cleft repair calls for closure of the lip at an early age (from 6 wk to 6 mo) followed by closure of the palate secondarily approximately 6 months later. This protocol has little impact on facial development. When managing a residual alveolar defect and an associated oronasal fistula, the primary goal of surgery is to allow subsequent development of a normal alveolus. Optimal eruption of teeth at the cleft site and development of normal periodontal structures of the teeth adjacent to the cleft occur when bone grafting and final fistula closure are performed prior to eruption of the permanent canine at the cleft site. Choice of operation The list of surgical techniques used in palatal cleft closure is extensive. The repairs differ depending upon whether the cleft is an isolated cleft palate or part of a unilateral or bilateral cleft lip and palate. The 3 main categories include (1) simple palatal closure, (2) palatal closure with palatal lengthening, and (3) either of the first two techniques with direct palatal muscle reapproximation. EXTERNAL SOURCES/ GROUPS POLICY: American Cleft Palate Craniofacial Association: In its guidelines on Parameters for Evaluation and Treatment of Patients with Cleft Lip/Palate or Other Craniofacial Anomalies, the American Cleft Palate Craniofacial Association (ACPA) states that presurgical maxillary orthopedics (including NAM) to improve the position of the maxillary alveolar segments prior to surgical closure of the lip may be indicated for some infants (ACPA, 2009). SUMMARY The management of a patient with cleft palate is complex. No current universal agreement exists on the appropriate treatment strategy. Several main points should be emphasized. Normal speech should be the most important consideration in the therapeutic plan. Growth disturbance should be minimized but not at the expense of speech impairment because facial distortion can be satisfactorily managed with future surgery, whereas speech impairment can often be irreversible. The authors believe, as do many others, that repair of cleft palate to establish a competent velopharyngeal sphincter should be completed at age 6-12 months. Surgical interventions should be designed to cause minimal disruption of the palate to decrease the severity of subsequent growth problems. Cleft patients should be managed in a center with a multidisciplinary team. Cleft palate remains a significant challenge for current and future plastic surgeons. Page. 9 of 14

10 APPLICABLE CODES: The Current Procedural Terminology (CPT ) codes and HCPCS codes listed in this policy are for reference purposes only. Listing of a service or device code in this policy does not imply that the service described by this code is a covered or non-covered health service. The inclusion of a code does not imply any right to reimbursement or guarantee claims payment. Other medical policies and coverage determination guidelines may apply. HCPCS Code Level II D5931 D5932 D5933 D5936 D5954 D5955 D5958 D5959 CPT Codes Obturator prosthesis, surgical Obturator prosthesis, definitive Obturator prosthesis, modification Obturator prosthesis, interim Palatal augmentation prosthesis Palatal lift prosthesis, definitive Palatal lift prosthesis, interim Palatal lift prosthesis, modification Description Description Impression and custom preparation; surgical obturator prosthesis Impression and custom preparation; interim obturator prosthesis Impression and custom preparation; definitive obturator prosthesis Impression and custom preparation; palatal augmentation prosthesis Impression and custom preparation; palatal lift prosthesis Impression and custom preparation; speech aid prosthesis Impression and custom preparation; oral surgical splint Rhinoplasty for nasal deformity secondary to congenital cleft lip and/or palate, including columellar lengthening; tip only Rhinoplasty for nasal deformity secondary to congenital cleft lip and/or palate, including columellar lengthening; tip, septum, osteotomies Plastic repair of cleft lip/nasal deformity; primary, partial or complete, unilateral Plastic repair of cleft lip/nasal deformity; primary bilateral, 1-stage procedure Plastic repair of cleft lip/nasal deformity; primary bilateral, 1 of 2 stages Plastic repair of cleft lip/nasal deformity; secondary, by recreation of defect and re-closure Plastic repair of cleft lip/nasal deformity with cross lip pedicle flap (Abbe- Estlander type), including sectioning and inserting of pedicle Palatoplasty for cleft palate, soft and/or hard palate only Palatoplasty for cleft palate, with closure of alveolar ridge; soft tissue only Palatoplasty for cleft palate, with bone graft to alveolar ridge (includes obtaining graft) Palatoplasty for cleft palate; major revision Palatoplasty for cleft palate; secondary lengthening procedure Palatoplasty for cleft palate; attachment pharyngeal flap Lengthening of palate, and pharyngeal flap Lengthening of palate, with island flap Repair of anterior palate, including vomer flap Page. 10 of 14

11 42260 Repair of nasolabial fistula Maxillary impression for palatal prosthesis Insertion of pin-retained palatal prosthesis ICD-9 Codes Description Cleft palate, unspecified Cleft palate: Unilateral, complete Cleft palate: Unilateral, incomplete Cleft palate: Bilateral, complete Cleft palate: Bilateral, incomplete Cleft lip, unspecified Cleft lip: Unilateral, complete Cleft lip: Unilateral, incomplete Cleft lip: Bilateral, complete Cleft lip: Bilateral, incomplete Cleft palate with cleft lip, unspecified Cleft palate with cleft lip: Unilateral, complete Cleft palate with cleft lip: Unilateral, incomplete Cleft palate with cleft lip: Bilateral, complete Cleft palate with cleft lip: Bilateral, incomplete Cleft palate with cleft lip: Other combinations ICD-10 Codes Description Q35.1 Cleft hard palate Q35.3 Cleft soft palate Q35.5 Cleft hard palate with cleft soft palate Q35.7 Cleft uvula Q35.9 Cleft palate, unspecified Q36.0 Cleft lip, bilateral Q36.1 Cleft lip, median Q36.9 Cleft lip, unilateral Q37.0 Cleft hard palate with bilateral cleft lip Q37.1 Cleft hard palate with unilateral cleft lip Q37.2 Cleft soft palate with bilateral cleft lip Q37.3 Cleft soft palate with unilateral cleft lip Q37.4 Cleft hard and soft palate with bilateral cleft lip Q37.5 Cleft hard and soft palate with unilateral cleft lip Q37.8 Unspecified cleft palate with bilateral cleft lip Q37.9 Unspecified cleft palate with unilateral cleft lip CPT is a registered trademark of the American Medical Association. REFERENCES: 1. American Cleft Palate-Craniofacial Association (ACPA). Parameters for Evaluation and Treatment of Patients with Cleft Lip/Palate or Other Craniofacial Anomalies. Revised November Available at: Accessed December 22, Aminpour S, Tollefson TT. Recent advances in presurgical molding in cleft lip and palate. Curr Opin Otolaryngol Head Neck Surg. 2008;16(4): Bardach J, Mooney MP. The relationship between lip pressure following lip repair and craniofacial growth: an Page. 11 of 14

12 experimental study in beagles. Plast Reconstr Surg. Apr 1984;73(4): Bardach J, Morris HL, Olin WH. Late results of primary veloplasty: the Marburg Project. Plast Reconstr Surg. Feb 1984;73(2): Center for Children with Special Needs. Cleft Lip and Palate: Critical Elements of Care. 5th ed Seattle, WA: Seattle Children s Hospital; 2010:42. Available at: Accessed December 22, Chang CS, Por YC, Liou EJ, Chang CJ, Chen PK, Noordhoff MS. Long-term comparison of four techniques for obtaining nasal symmetry in unilateral complete cleft lip patients: a single surgeon's experience. Plast Reconstr Surg. 2010;126(4): Cleft Palate Foundation. Parents & Individuals Available at: Accessed December 22, Cohen M. Residual deformities after repair of clefts of the lip and palate. Clinics in Plastic Surgery. 2004;21(2): da Silva Filho OG, Teles SG, Ozawa TO. Secondary bone graft and eruption of the permanent canine in patients with alveolar clefts: literature review and case report. Angle Orthod. Apr 2000;70(2): Dufresne CR. Oronasal and nasolabial fistulas. In: Bardach J, Morris HL, eds. Multidisciplinary Management of Cleft Lip and Palate. 1st ed. WB Saunders Co;1991: Freni SC, Zapisek WF. Biologic basis for a risk assessment model for cleft palate. Cleft Palate Craniofac J. Oct 1991;28(4): Garfinkle JS, King TW, Grayson BH, Brecht LE, Cutting CB. A 12-year anthropometric evaluation of the nose in bilateral cleft lip-cleft palate patients following nasoalveolar molding and cutting bilateral cleft lip and nose reconstruction. Plast Reconstr Surg. 2011;127(4): Hanson JW, Murray JC. Genetic aspect of cleft lip and palate. In: Bardach J, Morris HL, eds; Multidisciplinary Management of Cleft Lip and Palate. 1st ed. WB Saunders Co;1991: Hayes, Inc. Medical Technology Directory. Presurgical Nasoalveolar Molding for Cleft Lip and Palate Repair in Children. May 08, Reviewed April 15, Hodges PL, Pownell PH. Cleft palate surgery and velopharyngeal function. Plast Surg. 1994;7(23): Johnston MC, Bronsky PT, Millicovsky G. Embryogenesis of cleft lip and palate. In: McCarthy JG, ed. Plastic Surgery. Vol 4. Philadelphia:. WB Saunders Co;1990: Kasten EF, Schmidt SP, Zickler CF, et al. Team care of the patient with cleft lip and palate. Curr Probl Pediatr Adolesc Health Care. 2008;38(5): Kaufman FL. Managing the cleft lip and palate patient. Pediatr Clin North Am. Oct 1991;38(5): Kriens O. Anatomy of the velopharyngeal area in cleft palate. Clin Plast Surg. Apr 1975;2(2): Laub DR Jr, Peterson BG. Presurgical Orthopedic Therapy for Cleft Lip and Palate. Updated April Medscape from WebMD. Available at: Accessed December 22, Lee CT, Garfinkle JS, Warren SM, Brecht LE, Cutting CB, Grayson BH. Nasoalveolar molding improves appearance of children with bilateral cleft lip-cleft palate. Plast Reconstr Surg. 2008;122(4): Lewis MB. Timing and technique of cleft palate repair. In: Marsh JL, ed. Current Therapy in Plastic and Reconstructive Surgery. Vol 1. Mosby-Year Book; Lindsay WK. Surgical repair of cleft palate. Clin Plast Surg. Apr 1975;2(2): Lubker JF. Normal velopharyngeal function in speech. Clin Plast Surg. Apr 1975;2(2): Marsh JL, Grames LM, Holtman B. Intravelar veloplasty: a prospective study. Cleft Palate J. Jan 1989;26(1): Maue-Dickson W, Dickson DR. Anatomy and physiology related to cleft palate: current research and clinical implications. Plast Reconstr Surg. Jan 1980;65(1): Nguyen PN, Sullivan PK. Issues and controversies in the management of cleft palate. Clin Plast Surg. Oct Page. 12 of 14

13 1993;20(4): O'gara MM, Logemann JA. Early speech development in cleft palate babies. In: Bardach J, Morris HL, eds. Multidisciplinary Management of Cleft Lip and Palate. 1st ed. WB Saunders Co;1991: Peet E. The Oxford technique of cleft palate repair. Plast Reconstr Surg. 1961;28: Perko M. Two-stage palatoplasty. In: Bardach J, Morris HL, eds. Multidisciplinary Management of Cleft Lip and Palate. 1st ed. WB Saunders Co;1991: Peterson-Falzone SJ. A cross-sectional analysis of speech results following palatal closure. In: Bardach J, Morris HL, eds. Multidisciplinary Management of Cleft Lip and Palate. 1st ed. WB Saunders Co;1991: Pham AM, Senders CW. Management of bilateral cleft lip and nasal deformity. Curr Opin Otolaryngol Head Neck Surg. 2006;14(4): Rohrich RJ, Byrd HS. Optimal timing of cleft palate closure. Speech, facial growth, and hearing considerations. Clin Plast Surg. Jan 1990;17(1): Ross RB. Facial growth in cleft lip and palate. In: McCarthy JG, ed. Plastic Surgery. Vol 4. Philadelphia:. WB Saunders Co;1990: Sayetta RB, Weinrich MC, Coston GN. Incidence and prevalence of cleft lip and palate: what we think we know. Cleft Palate J. Jul 1989;26(3):242-7; discussion Schultz RC. Management and timing of cleft palate fistula repair. Plast Reconstr Surg. Dec 1986;78(6): Schweckendiek W, Doz P. Primary veloplasty: long-term results without maxillary deformity. a twenty-five year report. Cleft Palate J. Jul 1978;15(3): Shetty V, Vyas HJ, Sharma SM, Sailer HF. A comparison of results using nasoalveolar moulding in cleft infants treated within 1 month of life versus those treated after this period: development of a new protocol. Int J Oral Maxillofac Surg. 2012;41(1): Silva Filho OG, Calvano F, Assuncao AG. Craniofacial morphology in children with complete unilateral cleft lip and palate: a comparison of two surgical protocols. Angle Orthod. Aug 2001;71(4): Smahel Z, Mullerova Z, Nejedly A. Effect of primary repositioning of the nasal septum on facial growth in unilateral cleft lip and palate. Cleft Palate Craniofac J. 1999;36(4): Steinberg B, Padwa BL, Boyne P, Kaban L. State of the art in oral and maxillofacial surgery: Treatment of maxillary hypoplasia and anterior palatal and alveolar clefts. Cleft Palate Craniofac J. 1999;36(4): Still MJ, Georgiade NG. Historical review of management of cleft lip and palate. In: Georgiade NG, ed. Symposium on Management of Cleft Lip and Palate. 1974: Teng E, Steinbacher DM. Repair of the Cocaine-induced Cleft Palate Using the Modified Double-opposing Z-plasty. Cleft Palate Craniofac J. Jan von Langenbeck B. Operation on congenital total cleft of the hard palate by a new method. In: McDowell F, ed. The Source Book of Plastic Surgery. Baltimore:. Lippincott Williams & Wilkins;1977: Waite PD, Waite DE. Bone grafting for the alveolar cleft defect. Semin Orthod 1996 Sep;2(3): Witzel MA, Salyer KE, Ross RB. Delayed hard palate closure: the philosophy revisited. Cleft Palate J. Oct 1984;21(4): POLICY HISTORY: DATE ACTION/DESCRIPTION 10/10/2013 New Policy. Reviewed and approved by the Interim Medical Policy Committee. 10/24/2013 Reviewed and approved by the Quality Improvement Advisory and Credentialing Council (QIACC). Page. 13 of 14

14 11/15/2013 Published to ucare.org 1/09/2016 Policy updated and approved by the Interim Medical Policy Committee. 1/28/2016 Updated and approved by the Quality Improvement Advisory and Credentialing Committee (QIACC). 2/01/2016 Published to ucare.org Page. 14 of 14