Frequency of Le Fort I Osteotomy After Repaired Cleft Lip and Palate or Cleft Palate

Phoebe M. Good, D.M.D., John B. Mulliken, M.D., Bonnie L. Padwa, D.M.D., M.D.

Objective: Diminished maxillary growth is a consequence of labiopalatal repair, and many patients with cleft lip and palate require Le Fort I advancement. The goal of this study was to determine the frequency of maxillary hypoplasia as measured by need for Le Fort I. Subjects: Retrospective cohort study of males born before 1987 and females before 1989. Records of 173 patients with cleft lip and palate and 34 with cleft palate were reviewed. Methods: Documented age, gender, cleft type, and need for Le Fort I. Pearson chi-square and Fischers exact analyses were performed to evaluate the frequency of Le Fort I. Results: Of 217 patients with cleft lip and palate or cleft palate, 40 were syndromic; of the remaining 177 patients, 69 had cleft lip, 78 had cleft lip and palate, and 30 had cleft palate. Thirty-seven of 177 patients (20.9%) required Le Fort I, subcategorized by cleft type: 0/69 for cleft lip, 37/78 for cleft lip and palate, and 0/35 for cleft palate (p .0001). Of the 37/78 (47.4%) cleft lip and palate patients, the frequency of Le Fort I correlated with severity: 5/22 unilateral incomplete cleft lip and palate; 16/33 unilateral complete cleft lip and palate; 1/2 bilateral incomplete cleft lip and palate; 2/4 bilateral asymmetric complete/incomplete cleft lip and palate; 13/17 bilateral complete cleft lip and palate (p .05). Conclusion: Overall frequency of Le Fort I was 20.9% in patients with cleft lip and palate and cleft palate. Of those with cleft lip and palate, 47.7% required maxillary advancement, but none with isolated cleft lip or cleft palate required correction. Frequency of Le Fort I osteotomy correlated with the spectrum of severity of labiopalatal clefting. KEY WORDS: cleft lip/palate, Le Fort I osteotomy, maxillary hypoplasia

Specialists treating children with cleft lip/palate have emphasized the effect of labiopalatal repair on facial growth. There are several studies showing that patients with unrepaired cleft lip/palate have normal maxillary growth (Ortiz-Monasterio et al., 1966; Mars and Houston, 1990). There are also a few studies of patients with unoperated cleft lip/palate showing

Dr. Good is a dental student at Harvard School of Dental Medicine, Boston, Massachusetts. Dr. Mulliken is Professor of Surgery, Harvard Medical School, Department of Plastic and Oral Surgery, Childrens Hospital, Boston, Massachusetts. Dr. Padwa is Associate Professor of Oral and Maxillofacial Surgery, Harvard School of Dental Medicine, Department of Plastic and Oral Surgery, Childrens Hospital, Boston, Massachusetts. Manuscript was presented orally at the annual meeting of the American Association of Oral and Maxillofacial Surgeons, San Francisco, California, October 1, 2004. Funding was provided by the Research Training Grant NIH/NIDCR DE07268 and Ofce of Enrichment Programs, Harvard Medical School. Submitted May 2006; Accepted September 2006. Address correspondence to: Dr. Bonnie L. Padwa, Department of Plastic and Oral Surgery, Childrens Hospital, 300 Longwood Avenue, Boston, MA 02115. E-mail bonnie.padwa@childrens.harvard.edu. DOI: 10.1597/06-075.1 396

decreased maxillary growth (Innis, 1962; Atherton, 1967; Isiekwe and Sowemimo, 1984; Bishara et al., 1986). The validity of these studies has been questioned, because the sample sizes were too small for statistical signicance (Will, 2000). Diminished maxillary growth is commonly observed following cleft lip/palate closure, and most investigators believe that this is secondary to the labial or palatal repair, or both (Ross, 1987a; Mars and Houston, 1990; Han et al., 1995; Capelozza Filho et al., 1996; Liao et al., 2002). Ross maintained that there is an intrinsic deciency in midfacial skeletal growth in unilateral complete cleft lip and palate (UCLP) that is accentuated by labiopalatal repair (Ross, 1987a). Several investigators have tried to determine whether it is labial or palatal repair that causes decient maxillary growth. Capelozza Filho et al. (1996) studied 93 patients with repaired UCLP and found maxillary retrusion in those who had labial repair only. Those patients who had both labial and palatal repair did not differ signicantly from the labial repaironly group. They concluded that decient maxillary growth is a consequence of labial, rather than palatal, closure. In contrast, other studies have shown that labial repair does not affect anteroposterior max-

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illary growth (Liao et al., 2005), suggesting instead that palatal repair is responsible (Ross, 1987a; Mars and Houston, 1990; Han et al., 1995; Liao et al., 2002). Mars and Houston (1990) documented that patients with UCLP had normal maxillary growth following labial repair in early infancy, whereas maxillary hypoplasia was common following palatal closure before 2 years. Han et al. (1995) compared longitudinal craniofacial growth among patients with unilateral complete cleft lip (CL), UCLP, and isolated cleft palate (CP), and found that there was less forward development of the posterior limit of the maxilla (perpendicular intersect of the palatal plane with a line from the inferior point of the pterygomaxillary ssure) in UCLP and CP patients as compared with CL patients. Liao et al. (2002) analyzed craniofacial morphology in patients with bilateral complete cleft lip and bilateral complete cleft lip and palate (BCLP), and concluded that palatal repair had a more adverse effect on maxillary length than labial repair. Whatever causes maxillary hypoplasia, it is accepted that a subgroup of patients with repaired labial and/or palatal cleft will need maxillary advancement. The frequency of Le Fort I osteotomy in UCLP and BCLP patients is reported to be 22% to 27% (Ross, 1987b; Rosenstein et al., 1991; Cohen et al., 1995; DeLuke et al., 1997). However, many of these studies have limitations, such as small sample size, large age range, different clinical management protocols, failure to separate cleft types, combining of sexes, inadequate controls, and insufcient postoperative interval. Moreover, subjective criteria often have been used to determine the need for orthognathic correction (Rosenstein et al., 1991). Clearly, further study in a dened population of patients is needed to better delineate the relationship between maxillary hypoplasia and closure of cleft lip and palate. Furthermore, it is important to examine if the cleft severity and/or the number of operative procedures correlate with abnormal maxillary growth. The goals of this study were: (1) to determine the frequency of maxillary hypoplasia, as documented by the frequency of Le Fort I correction in cleft patients treated by one surgeon using the same protocol; (2) to ascertain the frequency of maxillary advancement for various categories of clefting (CL, CLP, and CP); and (3) to establish whether cleft severity correlates with frequency of Le Fort I osteotomy. MATERIALS
AND

TABLE 1 Cleft Types in Study Groups

Cleft Type No. of Patients

Cleft lip unilateral bilateral Cleft lip and palate unilateral bilateral Cleft palate soft hard and soft

69 (39.0%) 60 (51 incomplete, 9 complete) 9 (4 incomplete, 1 asymmetric, 4 complete) 78 (44.1%) 55 (22 incomplete, 33 complete) 23 (2 incomplete, 4 asymmetric, 17 complete) 30 (16.9%) 17 13

syndromic CLP or CP (including Robin sequence) were excluded. We documented age, gender, ethnicity, cleft type, need for Le Fort I correction, and number of total operative procedures (labial, palatal, oronasal stula closure, nasolabial revision, and pharyngeal ap). Data were recorded from the patients hospital medical records, specically the Cleft Program notes and operative reports. The patients were managed according to the protocol followed in the Cleft Program at Childrens Hospital Boston from 1976 to 1989: 1. Single-stage labial repair for unilateral (complete or incomplete) or bilateral incomplete CL (3 to 5 months); 2. A dentofacial orthopedic appliance for BCCLP to retract the premaxilla, to align the dentoalveolar segments, and to bring the labial elements closer together prior to synchronous nasolabial repair (Mulliken, 1985). Some patients had a palatal device plus elastic traction to position the premaxilla. (The protocol changed in 1991 to use of the Latham pin-retained appliance for UCCLP and BCCLP, but none of these patients were included in this study.); 3. Labial adhesion for UCCLP and complete side in BACLP (1 month). Denitive nasolabial repair (3 to 6 months); 4. Soft and hard palatal repair (8 to 10 months); 5. Pharyngeal ap for velopharyngeal incompetence (5 to 6 years); 6. Labionasal revision (if needed), either at 3 to 5 years or at time of alveolar bone graft; 7. Alveolar bone graft and closure of oronasal stula (during mixed dentition); and

METHODS

This retrospective cohort study only included patients who had completed facial growth: males born before 1987 (18 years old) and females born before 1989 (16 years old). We reviewed records of 217 patients with various types of CLP (n 170) and CP (n 47) registered in the Cleft Program at Childrens Hospital Boston. All patients had their primary repair(s) by one surgeon (J.B.M.). Patients with CLP were subcategorized as: unilateral incomplete cleft lip and palate (UICLP), unilateral complete cleft lip and palate (UCCLP), bilateral incomplete cleft lip and palate (BICLP), bilateral asymmetric complete/incomplete cleft lip and palate (BACLP), and bilateral complete cleft lip and palate (BCCLP). Patients with either

FIGURE 1 Frequency of Le Fort I among all cleft types.

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FIGURE 2 Frequency of Le Fort I among CLP patients.

8. Le Fort I osteotomy and maxillary repositioning (after completion of skeletal growth). Descriptive statistics were calculated on all study variables after converting the qualitative data into quantitative values using Microsoft Excel features. Pearson chi-square and Fischers exact analyses were performed to evaluate the frequency of maxillary deciency requiring Le Fort I correction for each cleft type. These same analyses also were performed to evaluate the association between all variables (sex, ethnicity, and total number of procedures) and Le Fort I osteotomy. RESULTS Of 217 patients, 40 were either syndromic CLP or CP or had Robin sequence, leaving 177 patients for analysis (111 males, average age 21.5 years; 66 females, average age 20.8 years). This distribution of cleft types is shown in Table 1. Of the 177 patients, 37 (20.9%) required a Le Fort I procedure, subcategorized by cleft type: none for CL; 37/78 (47.4%) for CLP, and none for CP (Fig. 1). These differences

were statistically signicant (p .0001). The frequency of Le Fort I in the various subcategories of the 37/78 CLP is shown in Figure 2. Note the increased need for Le Fort I correction with the increasing extent of the cleft. The differences in frequency of Le Fort I osteotomy among unilateral and bilateral and incomplete and complete CLP were statistically signicant (p .05). Due to the small numbers, however, there were no signicant differences when comparing bilateral incomplete, bilateral asymmetric, and bilateral complete CLP. We also examined other variables and their possible association with Le Fort I correction. Patients who had Le Fort I osteotomy had signicantly (p .0001) more operative procedures (mean, 6.4; range, 4 to 9) than those who did not (mean, 2.6; range, 1 to 8). There was no correlation between sex and need for a Le Fort I procedure; 20.7% of males and 21.2% of females had this procedure. Also, ethnicity was not a predictor of need for Le Fort I; between the two most common ethnicities, 21.8% (34/156) of Caucasians and 25.0% (3/12) of Hispanics had maxillary correction. DISCUSSION The requirement for Le Fort I osteotomy for all types of clefts treated by one surgeon using one protocol was 20.9%. The need for maxillary correction correlated with the type of cleft. None of the patients with a cleft of the primary palate (CL) or isolated cleft of the secondary palate (CP) required Le Fort I advancement (i.e., did not have maxillary hypoplasia), whereas 37/78 (47.4%) of patients with CLP required osteotomy. Furthermore, the frequency of Le Fort I advancement correlated with anatomic severity, from unilateral to bilateral and from incomplete to complete. Le Fort I osteotomy among patients with UCCLP (48.5%) was similar to that of BACLP

FIGURE 3 A: Frontal and B: lateral photographs of 18-year-old man with repaired left UCCLP demonstrating minor attened midface. C: Lateral cephalogram shows edge-to-edge anterior occlusion that could have been corrected orthodontically.

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FIGURE 4 Patient in Figure 3 two years after orthodontic decompensation followed by LeFort I osteotomy and maxillary advancement, dermal graft to philtral ridge, and nasal septal resection. Note improved nasolabial and midfacial prole (A: frontal view, B: lateral view, C: cephalogram).

(50%), suggesting that the repaired complete side of the cleft is responsible for decient maxillary growth. The higher rate of Le Fort I osteotomy in our CLP patients (47.4%), compared with previous reports, requires explanation. The decision for maxillary advancement is based on subjective assessment, usually without standardized criteria, and therefore, the decision for orthognathic correction is likely to vary among centers (Rosenstein et al., 1991). Although other studies focused on patients with horizontal and vertical maxillary hypoplasia, these studies may not have included adolescents with repaired oral clefts who have a normal dental relationship, yet might benet aesthetically from orthognathic correction.

The higher frequency of Le Fort I osteotomy in our unit may reect our preference for operative correction for all patients who have poor midfacial aesthetics despite their occlusal relationship (Figs. 3 through 6); approximately 10% of this patient sample had maxillary advancement for this reason. Furthermore, other reported rates for Le Fort I osteotomy are difcult to interpret and compare, due to small sample size, failure to fully separate cleft types, and combining of patients treated by different surgeons and protocols (Ross, 1987b; Rosenstein et al., 1991; Cohen et al., 1995; DeLuke et al., 1997; Schnitt et al., 2004). For example, Ross (1987b) analyzed a large sample (n 100) of male patients with repaired UCCLP

FIGURE 5 A: Frontal and B: lateral photographs of 17-year-old girl with repaired BACLP (left complete/right incomplete). C: Clinical photographs and lateral cephalogram shows prominent naso-frontal projection, bimaxillary retrognathia, and Class I occlusion.

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FIGURE 6 Improved appearance 8 months after 8-mm bimaxillary advancement and malar implants. (A: frontal view, B: lateral view, C: cephalogram).

treated at 15 centers by various protocols and calculated a Le Fort I rate of 27%. Rosenstein et al. (1991) evaluated 36 patients treated with primary bone grafting (25 with UCCLP and 11 with BCCLP) and documented Le Fort I frequency of 6/25 (24.0%) and 2/11 (18.2%), respectively. Cohen et al. (1995) included patients with UICLP, UCCLP, BICLP, BCCLP, but did not separate cleft types based on incomplete versus complete forms. They documented Le Fort I correction in 10/ 38 (26.3%) patients with UCLP and 7/29 (24.9%) patients with BCLP. DeLuke et al. (1997) subcategorized patients and reported the frequency of Le Fort I as 2/5 (40.0%) in isolated CP, 5/15 (33.3%) in UCCLP, and 2/8 (25.0%) in BCCLP. Schnitt et al. (2004) reported that 7/22 (32%) of all UCLP patients needed Le Fort I osteotomy. Unlike many previous reports, our study included a large cohort of patients and controlled for several important variables (i.e., surgeon, operative technique, and treatment protocol) that have been shown to inuence outcomes (Ross, 1987a). Ross (1987a) suggested that patients born with UCCLP have an intrinsic deciency in the midfacial skeleton that is made worse by operations. Our data supports this concept; maxillary hypoplasia occurred in patients with CLP, but not in those with isolated CL or CP. Patients who required Le Fort I osteotomy had signicantly more operations than did those who had adequate midfacial position. Several investigators have tried to indict labial or palatal repair as being responsible for maxillary growth aberrations (Ross, 1987a; Mars and Houston, 1990; Han et al., 1995; Capelozza Filho et al., 1996; Liao et al., 2002). In our study, however, patients who had decient maxillary growth had had both labial and palatal repair; this precluded blaming either repair as the cause of maxillary hypoplasia. We found that the severity of cleft type, as well as number and extent of operative procedures, predisposes patients to maxillary hypoplasia.

The ndings of this study aid in management and counseling of cleft patients. Members of the cleft lip/palate team can more accurately inform patients and their parents as to the likely need for Le Fort I osteotomy based on cleft type. Our protocol changed in 1991 to the use of preoperative orthopedic manipulation for all infants with complete CLP. This separate cohort of patients will need a similar analysis of maxillary growth once they have reached skeletal maturity. This would be an extension of the study from our unit showing that active infant orthopedics does not affect the dental arch of preadolescent children with repaired UCCLP (Chan et al., 2003). CONCLUSION Once skeletal growth nears completion, patients with repaired CLP often exhibit a characteristic concave facial prole, which requires correction by Le Fort I osteotomy and maxillary advancement. The prevalence of maxillary hypoplasia, determined by frequency of Le Fort I osteotomy, was 20.9% for all types of patients with clefts (CL, CLP, and CP), treated by one surgeon, following one protocol. For all types of CLP, the frequency of Le Fort I osteotomy was 47.4%. The severity of the cleft type correlated with decient maxillary growth, as reected by an increased frequency of Le Fort I osteotomy.
Acknowledgments. We thank Dr. Catherine Hayes for assistance in statistical analyses.

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