The Cleft Palate–Craniofacial Journal 00(00) pp. 000–000 Month 2014 Ó Copyright 2014 American Cleft Palate–Craniofacial Association
CASE REPORT 45 Years of Simultaneous Le Fort III and Le Fort I Osteotomies: A Systematic Literature Review Matthew S. Brown, M.D., Haruko Okada, M.D., Manish Valiathan, D.D.S., M.S.D., Gregory E. Lakin, M.D Objective: To review and collectively summarize our knowledge of simultaneous Le Fort III and Le Fort I osteotomies. Design: A PubMed search using ‘‘Le Fort III,’’ ‘‘simultaneous Le Fort III and Le Fort I,’’ ‘‘combined Le Fort III and Le Fort I,’’ ‘‘dual midface,’’ and ‘‘segmental midface’’ was performed. Articles with relevant abstracts were obtained for formal review. A new case of simultaneous Le Fort III and Le Fort I is presented to describe and discuss specific operative indications and surgical decisions. Results: There were 14 articles that met inclusion criteria with reports of simultaneous Le Fort III and Le Fort I osteotomies. A total of 20 cases were present in the literature. No major complications were reported. We performed combined Le Fort III with Le Fort I osteotomies in a 25-year-old patient with Crouzon syndrome who had undergone a previous Le Fort III at the age of 4 years. The patient tolerated the procedure well, and postoperatively, her exophthalmos and class III malocclusion were corrected. Conclusions: Simultaneous Le Fort III and Le Fort I can correct differential upper and lower midface hypoplasia and is a well-tolerated procedure in the mature facial skeleton. This systematic review improves our understanding of the surgical technique and indications for a procedure that can correct complex midfacial deformities. KEY WORDS:
Le Fort I, Le Fort III, simultaneous
The Le Fort III osteotomy has been a vital surgical approach to correct midface hypoplasia since it was first described by Gillies in 1950 and first successfully performed by Tessier (Gillies and Harrison, 1950; Tessier, 1967). In his seminal work on craniofacial dysostosis, Tessier remarked on the necessity for double osteotomies in correction of differential midface and occlusal abnormalities (Tessier, 1971). Surgeons have continued to expand on the work of these pioneers, modifying the operation with additional osteotomies. For patients with complex craniofacial deformities, simultaneous or combined osteotomies have provided excellent functional and aesthetic outcomes with a single surgical procedure. Distraction osteogenesis of the Le Fort III segment is now advocated for patients in need of large advancements or children with syndromic craniofacial dysmorphias where overcorrection is necessary (Fearon, 2001; Iannetti et al., 2006). Moderate advancements without distraction on patients with mature skeletal
development can still provide an optimal outcome with a single surgery. The reports of simultaneous Le Fort III and Le Fort I osteotomies (LF III/I) are rare, and to our knowledge no large cohort has been reported. A systematic literature review was performed to review all reported data of these cases. We also present a case of a 25-year-old woman with Crouzon syndrome previously treated with a Le Fort III advancement at age 4. METHODS A systematic literature review using PubMed was conducted by two independent reviewers. Articles were searched with the following terms: ‘‘simultaneous Le Fort III and Le Fort I,’’ ‘‘combined Le Fort III and Le Fort I,’’ ‘‘dual midface,’’ ‘‘segmental midface,’’ and ‘‘Le Fort III.’’ Searches were conducted using both ‘‘Le Fort’’ and ‘‘LeFort.’’ Abstracts and their original articles, English and non-English, were selected for review from the available titles. Disagreements were resolved by discussion and third-party review. The initial PubMed search was performed on August 30, 2013. All articles with reports of simultaneously performed LF III/I osteotomies were reviewed for number of cases, diagnosis, fixation, and outcomes. Bibliographies of included articles were subsequently reviewed for additional publications.
Dr. Brown and Dr. Okada are Physician Residents; Dr. Valiathan is Associate Professor, Department of Orthodontics; and Dr. Lakin is Assistant Professor, Department of Plastic Surgery, Case Western Reserve University, Cleveland, Ohio. Submitted January 2014; Revised May 2014; Accepted May 2014. Address correspondence to: Dr. Gregory E. Lakin, Case Western Reserve University, 11000 Euclid Avenue, 5th Floor Lakeside Building, Department of Plastic Surgery, Cleveland, OH 44106. Email [email protected]. DOI: 10.1597/14-005R 0
Brown et al., SIMULTANEOUS LE FORT III AND LE FORT I
FIGURE 1
0
Preoperative and 12-month postoperative photographs.
For the presented case, the patient’s clinic notes, photographs, operative records, and hospital chart were reviewed. CASE REPORT A 25-year-old woman with Crouzon syndrome presented for evaluation of a malocclusion, headaches, proptosis, and sleep apnea (Fig. 1). The patient had previously undergone a Le Fort III advancement at age 4 with rigid fixation. Her chronic headaches were located behind the eyes and were partially alleviated by medical therapy. Lumbar puncture revealed a normal opening pressure, indicating no increased intracranial pressure. She reported constant eye irritation and dry eyes. The patient had a sleep study that showed evidence of obstructive sleep apnea (OSA), with an apneahypopnea index of 14.9 (normal , 5), that was treated with nightly continuous positive airway pressure (CPAP). A physical exam revealed she had severe midface hypoplasia, proptosis, and evidence of exposure keratitis, with the left side being more affected than the right. Globe distance as measured by an exophthalmometer showed distances of 22.5 mm on the right and 23.5 mm on the left (normal ¼ 15 to 17 mm). She had angle class III malocclusion (Fig. 2). Computed tomography and three-
dimensional (3D) rendering (Fig. 3) confirmed the physical exam findings. Midfacial advancement was needed to correct her exorbitism, malocclusion, and OSA and to improve her cosmesis. She was orthodontically prepared and it was determined that she needed an 8-mm advancement at the Le Fort I level to obtain an angle class I dental relationship. Initial surgical planning included a 6-mm advancement of her upper midface at the Le Fort III level with additional 2mm advancement of her lower midface at the Le Fort I level. Intermediate and final acrylic occlusal splints were fabricated. The surgery began with converting the oral-tracheal intubation to a submental-tracheal intubation. A 2-cm transverse submental incision was made and dissection was carried out along the inner border of the mandible, entering the oral cavity to the right of the lingual frenulum. The oral tracheal tube was passed through the opening and secured at the skin with a suture. The old scar from the prior coronal flap was excised, and a subperiosteal plane dissection was performed. The supraorbital neurovascular bundles were identified and the foramina were operated upon with an osteotome. Dissection was carried out laterally along the deep temporal fascia. Exposure continued along the lateral orbital rims
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FIGURE 2 Preoperative and 12-month postoperative occlusal views.
toward the zygomatic arches and centrally to the nasofrontal junction. Old plates from the previous Le Fort III advancement were identified and removed. Osteotomies began along the lateral orbital wall with additional cuts through the zygomatic frontal process and anterior portion of the zygomatic arch. The medial orbital wall cut was made inferior to the anterior ethmoidal artery, superior to the medial canthal tendon, and posterior to the posterior lacrimal crest and was connected to an osteotomy of the nasofrontal junction. A 3-mm osteotome was used along the orbital floor. A Tessier-Kawamoto ridged osteotome was used through lateral intraoral incisions to perform the pterygomaxillary disjunction. A protective palatal acrylic splint was then placed in the palate, and Rowe disimpaction forceps were inserted. The Le Fort III
FIGURE 3 Preoperative and postoperative 3D computed tomography.
segment was then partially down-fractured, allowing for insertion of a double-guarded, curved osteotome in the nasofrontal separation to complete the osteotomy of the perpendicular plate. The Le Fort III segment was completely mobilized and maximally advanced. The final splint was used to advance the midface 8 mm, and the patient was placed in maxillomandibular fixation (MMF). Split-thickness parietal calvarial bone grafts were harvested and placed along with rigid fixation at the zygomatic arches. The nasofrontal junction and zygomatic frontal sutures were also fixated. The patient was released from MMF and the mandible was noted to slide forward, indicating that the patient had not been in mandibularcentric relation. The intraoral incision was fully opened, and the planned Le Fort I osteotomy was performed with a
Brown et al., SIMULTANEOUS LE FORT III AND LE FORT I
FIGURE 4
Preoperative and 6-month postoperative cephalogram.
reciprocating saw across the nasomaxillary buttresses, zygomaticomaxillary buttresses, and caudal septum, avoiding the canine roots. The Le Fort I segment was advanced an additional 2 mm into the final splint. The patient was placed again in MMF and rigidly fixated with L-plates across the nasomaxillary and zygomaticomaxillary buttresses. The MMF was released and class I occlusion was noted. The submental tube was translocated back to an intraoral position and incisions were closed. The patient spent 1 night in the intensive care unit, was extubated, and received a transfusion of a unit of packed red blood cells on postoperative day 1. She was discharged TABLE 1
Obwegeser Tessier Freihofer Kahnberg et al. Polley & Figueroa Matsumoto et al. Satoh et al. Takashima et al. Vachiramon et al. Bradley et al. Cheung et al. Hariri et al.
Ungari et al.
to home on postoperative day 4. She was seen weekly for 6 weeks in the plastic surgery and orthodontic clinics to maintain her class I occlusion with dental elastic adjustments as needed. During postoperative recovery she had a small superficial infection of her coronal incision that was treated with local wound care and antibiotics. She had postoperative velopharyngeal incompetence and difficulty with mastication, which required speech therapy and resolved within 3 months. At the 12-month follow-up, she maintained excellent occlusion and improvement of her midface projection (Figs. 1 through 4; Table 3). Globe distance as measured by
Summary of Articles Included in Systematic Review
Author
Dai et al.
0
Title
Year of Publication
Cases (N)
Surgical Correction of Small or Retrodisplaced Maxillae: The ‘‘Dish-Face’’ Deformity The Definitive Plastic Surgical Treatment of the Severe Facial Deformities of Craniofacial Dysostosis. Crouzon’s and Apert’s Diseases Results After Midface Osteotomies A Method for Fixation of the Maxillo-Facial Bones in Combined Surgical LeFort I and III Osteotomies ‘‘Piggyback’’ Osteotomies in Craniomaxillofacial Surgery Segmental Distraction of the Midface in a Patient With Crouzon Syndrome Dual Midfacial Distraction Osteogenesis: Le Fort III Minus I and Le Fort I for Syndromic Craniosynostosis Dual Segmental Distraction Osteogenesis of the Midface in a Patient With Apert Syndrome A Novel Model Surgery Technique for LeFort III Advancement Roman Arch, Keystone Fixation for Facial Bipartition With Monobloc Distraction Simultaneous Modified Oblique LeFort III and Segmentalized LeFort I Osteotomies Simultaneous LeFort III and LeFort I Osteotomies for Correction of Midface Hypoplasia in Crouzon Syndrome Simultaneous LeFort I, II, and III Osteotomies for Correction of Midface Deficiency in Apert Disease Le Fort III Osteotomic Variants
1969 1971
1
1973 1982
2 1
1995 2002 2003
1 1 4
2006
1
2007 2008 2010 2011
1 2 3 1
2012
1
2012
1
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TABLE 2
Summary of Individual Cases Present in the Literature
Case
Age
Diagnosis
Osteotomy
1 2 3 4 5 6
? 21 22 35 18 20
Unspecified facial dysostosis Posttraumatic injury Apert Crouzon Crouzon Crouzon
Le Le Le Le Le Le
7
19
Crouzon
Le Fort III/I
8
26
Crouzon
Le Fort III/I
9
13
Apert
Le Fort III/I
10
17
Apert
Le Fort III/I
11 12
19 12
Le Fort III/I Le Fort III/I
13
25
Apert Acrodysostosis, bimaxillary protrusion, Frontal bossing Nonsyndromic
14
19
Nonsyndromic
15
25
Nonsyndromic
16
16
Crouzon
17 18 19 20
24 25 ? ?
Apert Crouzon Syndromic Syndromic
Fort Fort Fort Fort Fort Fort
III/I III/I III/I III/I III-/I III/I
Le Fort III, Segmented Le Fort I (4-part) Le Fort III segmented Le Fort I (2part) Le Fort III, segmented Le Fort I (4part) Le Fort III segmented Le Fort I (2part) Le Fort III, II, I Le Fort III/I Le Fort III/I Le Fort III/I
an exophthalmometer showed distances of 18 mm on the right and 17 mm on the left. The patient stopped using her CPAP machine 3 weeks after the operation on her own accord due to improved breathing. No formal sleep study was performed postoperatively. Her psychosocial wellbeing has improved and she appears to have more selfconfidence. RESULTS The broad search criteria yielded 715 individual article titles. Only 14 articles were included after a thorough review of titles and abstracts. One additional article by Polley et al. was included after review of the bibliographies from the included papers (Table 1). Another article, by Schmitz et al. (1995), was subsequently excluded from the final analysis due to large modifications to the Le Fort III osteotomy that did not include the nasal and medial canthal subunits. TABLE 3
Fixation
Advancement or Distraction
Wire osteosynthesis Wires þ MMF 4 wk Wires þ MMF 6 wk Wire over skull vertex Rigid fixation Rigid external distraction (RED) device External and internal Distraction device External and internal Distraction device External and internal Distraction device External and internal Distraction device RED II device Rigid fixation plate
Advancement Advancement Advancement Advancement Advancement Distraction
Rigid fixation miniplate Rigid fixation miniplate Rigid fixation miniplate Rigid fixation
lag screw and
Advancement
lag screw and
Advancement
lag screw and
Advancement
miniplate
Advancement
fixation miniplate fixation fixation fixation
Advancement Advancement Advancement Advancement
Rigid Rigid Rigid Rigid
Distraction Distraction Distraction Distraction Distraction Advancement
The remaining 14 articles represented 20 reported cases of combined Le Fort III/I osteotomies spanning a 45-year period. The mean patient age was 21 years, and diagnoses included Crouzon syndrome (n ¼ 7), Apert syndrome (n ¼ 5), nonsyndromic craniosynostosis (n ¼ 3), posttraumatic injury (n ¼ 1), acrodysostosis (n ¼ 1), and an unspecified facial dysostosis (n¼ 3). There were 12 patients treated with direct advancement, and eight patients were treated with distraction. A variety of fixation techniques were reported. Older reports presented wire fixation; whereas, newer descriptions included miniplates. The patients treated with distraction used both halo-type devices and internal distractors. The mean advancement of the Le Fort III segment for direct advancement was 7 mm, and mean advancement for distraction was 16 mm. Only two minor complications were reported. One patient had a scalp incision dehiscence at the coronal incision, and one patient, a temporary temporal branch of facial nerve neuropraxia
Preoperative and 6-Month Postoperative Cephalometric Measurements
Measurement
Normative
Preoperataive
Postoperative
SNA SNB ANB SNPG Facial convexity Facial depth LFA:UFA ratio
82 6 2 80 6 3 2 80 6 3 068 88 6 3 0.95 to 1.0
80 86 6 86 11 102 0.69
88 83 5 82 10 101 0.78
Brown et al., SIMULTANEOUS LE FORT III AND LE FORT I
TABLE 2
0
Summary of Individual Cases Present in the Literature
Complications
Le Fort III Movement (mm)
Le Fort I Movement (mm)
Follow-Up (mo)
Outcome
Not specified 24
Stable Stable
8 15
10 7
12 12
Stable Stable
14
14
38
Stable
18
13
16
Stable
20
14
15
Stable
21
18
10
Stable
9
Left 11.5 Right 6.5
12
Stable
Step off at nasal bone
5 to 6
12
Scalp dehiscence
5 to 6
6
Temporal branch neuropraxia
5 to 6
2.5
10
9
12
Stable
Combined 13
Combined 13
24 Not specified
Stable
that resolved in follow-up. The mean reported follow-up was 15 months, and 11 patients had documentation of stability of occlusion or facial aesthetics. One patient had a reported revision for a contour deformity at the nasofrontal junction (Table 2). DISCUSSION The combined LF III/I osteotomy was first described by Obwegeser in 1969. This operation was again advocated for select syndromic patients by Tessier in 1971, although he did not provide any operative description. Since that time, multiple authors have reported their experiences combining the LF III/I osteotomies. In the process of our review we decided to report our experience because, to our knowledge, this is the first case report of a secondary Le Fort III osteotomy with simultaneous Le Fort I osteotomy for a patient with Crouzon syndrome. Additionally, we found that the indications and specifics of the operation have not been well described. The LF III/I should be performed in patients who are skeletally mature. A Le Fort I osteotomy is of concern in a child due to the developing tooth buds. The youngest patient reported in the literature was a 12-year-old; although, the mean age for all the simultaneous procedures was 21 years. Combined osteotomies are useful when a differential advancement of the upper and lower midface is needed, or when advancement of the upper and lower
midface cannot be achieved with a Le Fort III alone due to the soft tissue restrictions. The literature reveals that a majority of cases have been reported in patients with Crouzon or Apert syndromes. We find it interesting that our patient had a diagnosis of Crouzon syndrome confirmed by genetic testing but did not have cranial vault remodeling as a child. She likely had isolated cranial base suture synchondrosis resulting in midface hypoplasia, as described in other syndromic craniosynostoses (McGrath et al., 2012). One theoretical fear of simultaneous osteotomies is devascularization of the osteotomized bony segments. The blood supply to the Le Fort I segment was initially felt to be due to the descending palatine artery. Siebert et al. (1997) proved with latex injection studies that the ascending palatine branch of the facial artery and anterior branch of the ascending pharyngeal artery are responsible for vascular integrity after osteotomy. Avascular necrosis of the maxilla after Le Fort I alone is a rare complication, with few case reports (Lanigan et al., 1990; Pereira et al., 2010) and a reported rate of less than 1% (Kramer et al., 2004). Risk factors are transversal segmentation of the maxilla, particularly with transverse expansion of the segments (Lanigan, 1997; Kramer et al., 2004). In contrast, few reports investigate the blood supply to the Le Fort III segment. Wittenberg and Meyer (1983) studied blood flow to the Le Fort III segment in macaques after multiple incisions (bilateral brow, zygomatic, and
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intraoral) versus a single coronal incision. It is interesting that the blood flow to the Le Fort III osseous segment decreased by 85% when a bicoronal incision was made; whereas, the multiple incision group decreased only by 26%, showing vascularization of the midfacial bone from facial soft tissues. Hence, Wittenberg recommended a multiple-incision approach over a coronal incision for combined Le Fort III/I cases. No major complications were noted in any of the reported cases. In our case, the Le Fort III/I was accomplished via a coronal incision without vascular compromise, but we recommend proceeding with this case with minimal periosteal stripping. Relapse or growth retardation of the upper maxilla is common in patients with Crouzon or Apert syndrome who had standard advancement procedures as children. They often present as adults with recurrent pathology that necessitates orthodontic management or secondary orthognathic surgery (Shetye et al., 2010; Nout et al., 2011; Warren et al., 2012). Therefore, the current standard of care is to perform the Le Fort III with distraction osteogenesis during childhood to overcorrect the midface, anticipating mandibular growth. Although it remains to be determined, distraction osteogenesis may obviate the need for secondary advancement procedures in these patients. Fearon reported up to a 5-year follow-up on Le Fort III distraction cases done at a mean age of 8 years. In his series no one had a secondary procedure during the follow-up period. He attributes factors determining the need of secondary surgery to include the age patients are first operated upon and overcorrection achieved (Fearon, 2005). Fearon reported an average distraction of 19-mm advancement, compared with a 6-mm advancement with a standard Le Fort III advancement (Fearon, 2001). Large, single advancements with a Le Fort III osteotomy to overcorrect the midface may be limited by the stretch of the soft tissues, especially in those with scarred tissue planes. Freihofer (1984) wrote that advancement at any level is limited to 20 mm but remarked that a maxilla that has been previously operated on is more limited. Obwegeser (1969), in his original description of the combined osteotomy, advised advancing the upper maxillary segment ‘‘as far forward as is necessary for esthetic reasons’’ (p. X). In our review of the literature we found that the mean direct advancement of the Le Fort III segment to be 7 mm, with a range of 5 to 10 mm. In the case of our patient we attempted to achieve 8 mm of advancement at the Le Fort III level. After removing the patient from MMF the mandible moved anteriorly, returning the patient to a class III malocclusion, indicating that the mandible was not in centric relation. We thought that she may have additional skin laxity due to a 100-pound weight loss that would allow us to achieve an 8-mm advancement. Ultimately, we were only able to achieve 6 mm at the Le Fort III level. The originally planned Le Fort I osteotomy was needed to advance her lower maxilla an additional 2 mm. The 6-mm advancement is consistent with the numbers reported by
others after Le Fort III osteotomy (Fearon, 2001; Iannetti et al., 2006; Cheung et al., 2010). Larger single advancements are reported in the literature but have also been noted to have complications and relapse (Freihofer, 1973). To help prevent relapse, the zygomatic arches were stabilized with split thickness calvarial bone grafts as initially described by Tessier and advocated by others (Tessier, 1967; Obwegeser, 1969; Kahnberg et al., 1982). Various authors have briefly described their general operative approach, but a step-by-step description of the operation was not found (Obwegeser, 1969; Tessier, 1971; Cheung et al., 2010). We included a detailed description of the operation we performed because most of the descriptions to date have been brief. Though not commonly advocated for use in craniofacial advancement surgeries, a submental intubation was used in our patients. Most of the operative descriptions for Le Fort III and other facial advancements describe the use of a nasotracheal intubation. A recent review of the literature on submental intubation indicated this is a safe procedure with an average operative time of 10 minutes and minimal complications (Jundt et al., 2012). Submental intubation provides several intraoperative conveniences (Lypka and Urata, 2012). It allows for easy access to all facial structures while providing the ability to take the patient in and out of MMF. It also reduces the risk of damaging the endotracheal tube during osteotomies or dislodging the tube from the trachea when moving the nasomaxillary segment forward. Additionally, the surgeon can insert the Rowe disimpaction forceps during down-fracture without a nasotracheal tube obstructing or being damaged. The only other way to achieve this type of access is with a tracheostomy, which comes with a more significant risk of long-term sequelae of the airway. A reinforced tube should be used to prevent obstructive kinking that can otherwise result from a nonreinforced tube curving at an acute angle from the submental intubation. Freihofer (1973) was the first to report complications and long-term follow-up on two patients with simultaneous osteotomies. These patients both had excellent occlusal results in follow-up, and one was followed up to 2 years. It is interesting to note that this was before the time of rigid plate fixation. Schmitz et al. (1995) provided the largest case series in the literature of combined LF III/I osteotomies with 11 patients. However, the Le Fort III procedure in this series was not standard because they did not perform a coronal incision and carry the medial canthus with the Le Fort III segment (hence, this paper was excluded from analysis). The remainder of the literature on combined osteotomies includes case reports and small case series. Ungari et al. (2012), Hariri et al. (2011), and Cheung et al. (2010) have published the use of LF III/I in both nonsyndromic and syndromic cases. Hariri et al. (2011) commented that this strategy can address the deformity of the upper and lower midface differentially in cases where the upper midface is hypoplastic and the lower maxillary
Brown et al., SIMULTANEOUS LE FORT III AND LE FORT I
dentoalveolus is hyperplastic. Bradley et al. (2008) reported two patients with simultaneous LF III/I after previous monobloc distraction with combined facial bipartition. Dai et al. (2012) published a simultaneous Le Fort I, II, and III in a patient with Apert syndrome. Among all these cases only a few minor postoperative complications have been reported. This information is relatively unreliable because it is inevitable that poor results or complications are much less likely to be reported, unless they are within a large series or cohort. However, consistent with the literature, our patient did well postoperatively. She had a 4-day hospitalization, mostly secondary to facial swelling and difficulty with secretions. In the literature, length of stay ranged from 1 to 2 weeks (Cheung et al., 2010; Hariri et al., 2011; Dai et al., 2012). During her recovery the patient had both speech and masticatory difficulties. The surgery altered her nasopharynx, causing some velopharyngeal incompetence. These issues resolved with speech therapy and are not uncommon with these procedures (Fearon, 2005). In some cases preoperative nasal endoscopy and speech evaluation may help in both counseling and preparing patients for these postoperative changes. Our patient has had no further complications, with stable occlusion at 1 year. The psychosocial benefits of this procedure cannot be underestimated. The patient had been extremely selfconscious prior to the operation. Postoperatively, she began to wear makeup around her eyes for the first time, had an improved self-image, and even discussed starting a family. Although this procedure has the potential to correct complex deformities of the midface, relatively few simultaneous LF III/I osteotomies have been reported in the literature. Underreporting may be one potential explanation for the relatively low frequency of this procedure. The more widespread use of distraction devices for Le Fort III advancements in childhood has likely reduced the frequency of these combined LF III/I surgeries. Another potential explanation to the low frequency of this procedure is the need for a surgeon to have in-depth knowledge to perform a combination of both craniofacial and orthognathic surgical techniques. Other authors have reported on the use of combinations of osteotomies to differentially distract the midface to achieve correction of a variety of complex deformities. Matsumoto et al. (2002), Takashima et al. (2006), and Satoh et al. (2003) have reported their experience with LF III/I and facial distraction, allowing larger advancements. Hopper et al. (XXXX) recently published a series that included three Apert syndrome patients who had combined Le Fort III advancement with Le Fort II distraction. One of these patients had a previously failed Le Fort III distraction. He demonstrated successful outcomes in all of his patients. Furthermore, Lakin and Kawamoto (2012)
0
performed a combined Le Fort II/Le Fort I osteotomy to differentially advance the nasomaxillary segment. In summary, we found evidence of only 20 separate cases of combined LF III/I since 1969. It is interesting to note that, to our knowledge, at least 24 facial transplants have been performed and reported thus far since the original transplant in 2005 (Dorafshar et al., 2013). In 1995 Polley commented in his article that combining facial osteotomies ‘‘has perhaps its greatest potential in reconstruction of the midface; unfortunately, the application to this area has received the least amount of attention’’ (Polley and Figueroa, 1995, p. X). Acknowledgment. We thank Dr. Mark Urata for his mentorship and surgical guidance on the case presented in this article.
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Lanigan DT. Ligation of the descending palatine artery: pro and con. J Oral Maxilliofac Surg. 1997;55:1502–1504. Lanigan DT, Hey JH, West RA. Aseptic necrosis following maxillary osteotomies: report of 36 cases. J Oral Maxilliofac Surg. 1990;48:142–156. Lypka M, Urata M. Submental intubation: a useful surgical airway for patients undergoing maxillofacial osteotomies and other adjunctive procedures Presented at the American Cleft Palate–Craniofacial Association Annual Meeting; April 2012; San Jose, California. Matsumoto K, Nakanishi H, Koizumi Y, Seike T, Okazaki M, Yokozeki M, Moriyama K. Segmental distraction of the midface in a patient with Crouzon syndrome. J Craniofac Surg. 2002;13:273– 278. McGrath J, Gerety PA, Derderian CA, Steinbacher DM, Vossough A, Bartlett SP, Nah HD, Taylor JA. Differential closure of the sphenooccipital synchondrosis in syndromic craniosynostosis. Plast Reconstr Surg. 2012;130:e681–e689. Nout E, Koudstaal MJ, Wolvius EB, Van der Wal KG. Additional orthognathic surgery following Le Fort III and monobloc advancement. Int J Oral Maxilliofac Surg. 2011;40:679–684. Obwegeser HL. Surgical correction of small or retrodisplaced maxillae. The ‘‘dish-face’’ deformity. Plast Reconstr Surg. 1969;43:351–365. Pereira FL, Yaedu RY, Sant’Ana AP, Sant’Ana E. Maxillary aseptic necrosis after Le Fort I osteotomy: a case report and literature review. J Oral Maxilliofac Surg. 2010;68:1402–1407. Polley JW, Figueroa AA. ‘‘Piggyback’’ osteotomies in craniomaxillofacial surgery. J Craniofac Surg. 1995;6:199–210.
Satoh K, Mitsukawa N, Hosaka Y. Dual midfacial distraction osteogenesis: Le Fort III minus I and Le Fort I for syndromic craniosynostosis. Plast Reconstr Surg. 2003;111:1019–1028. Schmitz JP, Tiner BD, Van Sickels JE. Stability of simultaneous modified LeFort III/LeFort I osteotomies. J Craniomaxillofac Surg. 1995;23:287–295. Shetye PR, Kapadia H, Grayson BH, McCarthy JG. A 10-year study of skeletal stability and growth of the midface following Le Fort III advancement in syndromic craniosynostosis. Plast Reconstr Surg. 2010;126:973–981. Siebert JW, Angrigiani C, McCarthy JG, Longaker MT. Blood supply of the Le Fort I maxillary segment: an anatomic study. Plast Reconstr Surg. 1997;100:843–851. Takashima M, Kitai N, Murakami S, Takagi S, Hosokawa K, Kreiborg S, Takada K. Dual segmental distraction osteogenesis of the midface in a patient with Apert syndrome. Cleft Palate Craniofac J. 2006;43:499–506. Tessier P. The definitive plastic surgical treatment of the severe facial deformities of craniofacial dysostosis. Crouzon’s and Apert’s diseases. Plast Reconstr Surg. 1971;48:419–442. Tessier P. Total facial osteotomy. Crouzon’s syndrome, Apert’s syndrome: oxycephaly, scaphocephaly, turricephaly [in French]. Ann Chir Plast. 1967;12:273–286. Warren SM, Shetye PR, Obaid SI, Grayson BH, McCarthy JG. Longterm evaluation of midface position after Le Fort III advancement: a 20-plus-year follow-up. Plast Reconstr Surg. 2012;129:234–242. Wittenberg GJ, Meyer MW. Flap design and the LeFort III osteotomy: blood flow investigation. J Oral Maxilliofac Surg. 1983;41:314–321.
CASE REPORT 45 Years of Simultaneous Le Fort III and Le Fort I Osteotomies: A Systematic Literature Review Matthew S. Brown, M.D., Haruko Okada, M.D., Manish Valiathan, D.D.S., M.S.D., Gregory E. Lakin, M.D Objective: To review and collectively summarize our knowledge of simultaneous Le Fort III and Le Fort I osteotomies. Design: A PubMed search using ‘‘Le Fort III,’’ ‘‘simultaneous Le Fort III and Le Fort I,’’ ‘‘combined Le Fort III and Le Fort I,’’ ‘‘dual midface,’’ and ‘‘segmental midface’’ was performed. Articles with relevant abstracts were obtained for formal review. A new case of simultaneous Le Fort III and Le Fort I is presented to describe and discuss specific operative indications and surgical decisions. Results: There were 14 articles that met inclusion criteria with reports of simultaneous Le Fort III and Le Fort I osteotomies. A total of 20 cases were present in the literature. No major complications were reported. We performed combined Le Fort III with Le Fort I osteotomies in a 25-year-old patient with Crouzon syndrome who had undergone a previous Le Fort III at the age of 4 years. The patient tolerated the procedure well, and postoperatively, her exophthalmos and class III malocclusion were corrected. Conclusions: Simultaneous Le Fort III and Le Fort I can correct differential upper and lower midface hypoplasia and is a well-tolerated procedure in the mature facial skeleton. This systematic review improves our understanding of the surgical technique and indications for a procedure that can correct complex midfacial deformities. KEY WORDS:
Le Fort I, Le Fort III, simultaneous
The Le Fort III osteotomy has been a vital surgical approach to correct midface hypoplasia since it was first described by Gillies in 1950 and first successfully performed by Tessier (Gillies and Harrison, 1950; Tessier, 1967). In his seminal work on craniofacial dysostosis, Tessier remarked on the necessity for double osteotomies in correction of differential midface and occlusal abnormalities (Tessier, 1971). Surgeons have continued to expand on the work of these pioneers, modifying the operation with additional osteotomies. For patients with complex craniofacial deformities, simultaneous or combined osteotomies have provided excellent functional and aesthetic outcomes with a single surgical procedure. Distraction osteogenesis of the Le Fort III segment is now advocated for patients in need of large advancements or children with syndromic craniofacial dysmorphias where overcorrection is necessary (Fearon, 2001; Iannetti et al., 2006). Moderate advancements without distraction on patients with mature skeletal
development can still provide an optimal outcome with a single surgery. The reports of simultaneous Le Fort III and Le Fort I osteotomies (LF III/I) are rare, and to our knowledge no large cohort has been reported. A systematic literature review was performed to review all reported data of these cases. We also present a case of a 25-year-old woman with Crouzon syndrome previously treated with a Le Fort III advancement at age 4. METHODS A systematic literature review using PubMed was conducted by two independent reviewers. Articles were searched with the following terms: ‘‘simultaneous Le Fort III and Le Fort I,’’ ‘‘combined Le Fort III and Le Fort I,’’ ‘‘dual midface,’’ ‘‘segmental midface,’’ and ‘‘Le Fort III.’’ Searches were conducted using both ‘‘Le Fort’’ and ‘‘LeFort.’’ Abstracts and their original articles, English and non-English, were selected for review from the available titles. Disagreements were resolved by discussion and third-party review. The initial PubMed search was performed on August 30, 2013. All articles with reports of simultaneously performed LF III/I osteotomies were reviewed for number of cases, diagnosis, fixation, and outcomes. Bibliographies of included articles were subsequently reviewed for additional publications.
Dr. Brown and Dr. Okada are Physician Residents; Dr. Valiathan is Associate Professor, Department of Orthodontics; and Dr. Lakin is Assistant Professor, Department of Plastic Surgery, Case Western Reserve University, Cleveland, Ohio. Submitted January 2014; Revised May 2014; Accepted May 2014. Address correspondence to: Dr. Gregory E. Lakin, Case Western Reserve University, 11000 Euclid Avenue, 5th Floor Lakeside Building, Department of Plastic Surgery, Cleveland, OH 44106. Email [email protected]. DOI: 10.1597/14-005R 0
Brown et al., SIMULTANEOUS LE FORT III AND LE FORT I
FIGURE 1
0
Preoperative and 12-month postoperative photographs.
For the presented case, the patient’s clinic notes, photographs, operative records, and hospital chart were reviewed. CASE REPORT A 25-year-old woman with Crouzon syndrome presented for evaluation of a malocclusion, headaches, proptosis, and sleep apnea (Fig. 1). The patient had previously undergone a Le Fort III advancement at age 4 with rigid fixation. Her chronic headaches were located behind the eyes and were partially alleviated by medical therapy. Lumbar puncture revealed a normal opening pressure, indicating no increased intracranial pressure. She reported constant eye irritation and dry eyes. The patient had a sleep study that showed evidence of obstructive sleep apnea (OSA), with an apneahypopnea index of 14.9 (normal , 5), that was treated with nightly continuous positive airway pressure (CPAP). A physical exam revealed she had severe midface hypoplasia, proptosis, and evidence of exposure keratitis, with the left side being more affected than the right. Globe distance as measured by an exophthalmometer showed distances of 22.5 mm on the right and 23.5 mm on the left (normal ¼ 15 to 17 mm). She had angle class III malocclusion (Fig. 2). Computed tomography and three-
dimensional (3D) rendering (Fig. 3) confirmed the physical exam findings. Midfacial advancement was needed to correct her exorbitism, malocclusion, and OSA and to improve her cosmesis. She was orthodontically prepared and it was determined that she needed an 8-mm advancement at the Le Fort I level to obtain an angle class I dental relationship. Initial surgical planning included a 6-mm advancement of her upper midface at the Le Fort III level with additional 2mm advancement of her lower midface at the Le Fort I level. Intermediate and final acrylic occlusal splints were fabricated. The surgery began with converting the oral-tracheal intubation to a submental-tracheal intubation. A 2-cm transverse submental incision was made and dissection was carried out along the inner border of the mandible, entering the oral cavity to the right of the lingual frenulum. The oral tracheal tube was passed through the opening and secured at the skin with a suture. The old scar from the prior coronal flap was excised, and a subperiosteal plane dissection was performed. The supraorbital neurovascular bundles were identified and the foramina were operated upon with an osteotome. Dissection was carried out laterally along the deep temporal fascia. Exposure continued along the lateral orbital rims
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FIGURE 2 Preoperative and 12-month postoperative occlusal views.
toward the zygomatic arches and centrally to the nasofrontal junction. Old plates from the previous Le Fort III advancement were identified and removed. Osteotomies began along the lateral orbital wall with additional cuts through the zygomatic frontal process and anterior portion of the zygomatic arch. The medial orbital wall cut was made inferior to the anterior ethmoidal artery, superior to the medial canthal tendon, and posterior to the posterior lacrimal crest and was connected to an osteotomy of the nasofrontal junction. A 3-mm osteotome was used along the orbital floor. A Tessier-Kawamoto ridged osteotome was used through lateral intraoral incisions to perform the pterygomaxillary disjunction. A protective palatal acrylic splint was then placed in the palate, and Rowe disimpaction forceps were inserted. The Le Fort III
FIGURE 3 Preoperative and postoperative 3D computed tomography.
segment was then partially down-fractured, allowing for insertion of a double-guarded, curved osteotome in the nasofrontal separation to complete the osteotomy of the perpendicular plate. The Le Fort III segment was completely mobilized and maximally advanced. The final splint was used to advance the midface 8 mm, and the patient was placed in maxillomandibular fixation (MMF). Split-thickness parietal calvarial bone grafts were harvested and placed along with rigid fixation at the zygomatic arches. The nasofrontal junction and zygomatic frontal sutures were also fixated. The patient was released from MMF and the mandible was noted to slide forward, indicating that the patient had not been in mandibularcentric relation. The intraoral incision was fully opened, and the planned Le Fort I osteotomy was performed with a
Brown et al., SIMULTANEOUS LE FORT III AND LE FORT I
FIGURE 4
Preoperative and 6-month postoperative cephalogram.
reciprocating saw across the nasomaxillary buttresses, zygomaticomaxillary buttresses, and caudal septum, avoiding the canine roots. The Le Fort I segment was advanced an additional 2 mm into the final splint. The patient was placed again in MMF and rigidly fixated with L-plates across the nasomaxillary and zygomaticomaxillary buttresses. The MMF was released and class I occlusion was noted. The submental tube was translocated back to an intraoral position and incisions were closed. The patient spent 1 night in the intensive care unit, was extubated, and received a transfusion of a unit of packed red blood cells on postoperative day 1. She was discharged TABLE 1
Obwegeser Tessier Freihofer Kahnberg et al. Polley & Figueroa Matsumoto et al. Satoh et al. Takashima et al. Vachiramon et al. Bradley et al. Cheung et al. Hariri et al.
Ungari et al.
to home on postoperative day 4. She was seen weekly for 6 weeks in the plastic surgery and orthodontic clinics to maintain her class I occlusion with dental elastic adjustments as needed. During postoperative recovery she had a small superficial infection of her coronal incision that was treated with local wound care and antibiotics. She had postoperative velopharyngeal incompetence and difficulty with mastication, which required speech therapy and resolved within 3 months. At the 12-month follow-up, she maintained excellent occlusion and improvement of her midface projection (Figs. 1 through 4; Table 3). Globe distance as measured by
Summary of Articles Included in Systematic Review
Author
Dai et al.
0
Title
Year of Publication
Cases (N)
Surgical Correction of Small or Retrodisplaced Maxillae: The ‘‘Dish-Face’’ Deformity The Definitive Plastic Surgical Treatment of the Severe Facial Deformities of Craniofacial Dysostosis. Crouzon’s and Apert’s Diseases Results After Midface Osteotomies A Method for Fixation of the Maxillo-Facial Bones in Combined Surgical LeFort I and III Osteotomies ‘‘Piggyback’’ Osteotomies in Craniomaxillofacial Surgery Segmental Distraction of the Midface in a Patient With Crouzon Syndrome Dual Midfacial Distraction Osteogenesis: Le Fort III Minus I and Le Fort I for Syndromic Craniosynostosis Dual Segmental Distraction Osteogenesis of the Midface in a Patient With Apert Syndrome A Novel Model Surgery Technique for LeFort III Advancement Roman Arch, Keystone Fixation for Facial Bipartition With Monobloc Distraction Simultaneous Modified Oblique LeFort III and Segmentalized LeFort I Osteotomies Simultaneous LeFort III and LeFort I Osteotomies for Correction of Midface Hypoplasia in Crouzon Syndrome Simultaneous LeFort I, II, and III Osteotomies for Correction of Midface Deficiency in Apert Disease Le Fort III Osteotomic Variants
1969 1971
1
1973 1982
2 1
1995 2002 2003
1 1 4
2006
1
2007 2008 2010 2011
1 2 3 1
2012
1
2012
1
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TABLE 2
Summary of Individual Cases Present in the Literature
Case
Age
Diagnosis
Osteotomy
1 2 3 4 5 6
? 21 22 35 18 20
Unspecified facial dysostosis Posttraumatic injury Apert Crouzon Crouzon Crouzon
Le Le Le Le Le Le
7
19
Crouzon
Le Fort III/I
8
26
Crouzon
Le Fort III/I
9
13
Apert
Le Fort III/I
10
17
Apert
Le Fort III/I
11 12
19 12
Le Fort III/I Le Fort III/I
13
25
Apert Acrodysostosis, bimaxillary protrusion, Frontal bossing Nonsyndromic
14
19
Nonsyndromic
15
25
Nonsyndromic
16
16
Crouzon
17 18 19 20
24 25 ? ?
Apert Crouzon Syndromic Syndromic
Fort Fort Fort Fort Fort Fort
III/I III/I III/I III/I III-/I III/I
Le Fort III, Segmented Le Fort I (4-part) Le Fort III segmented Le Fort I (2part) Le Fort III, segmented Le Fort I (4part) Le Fort III segmented Le Fort I (2part) Le Fort III, II, I Le Fort III/I Le Fort III/I Le Fort III/I
an exophthalmometer showed distances of 18 mm on the right and 17 mm on the left. The patient stopped using her CPAP machine 3 weeks after the operation on her own accord due to improved breathing. No formal sleep study was performed postoperatively. Her psychosocial wellbeing has improved and she appears to have more selfconfidence. RESULTS The broad search criteria yielded 715 individual article titles. Only 14 articles were included after a thorough review of titles and abstracts. One additional article by Polley et al. was included after review of the bibliographies from the included papers (Table 1). Another article, by Schmitz et al. (1995), was subsequently excluded from the final analysis due to large modifications to the Le Fort III osteotomy that did not include the nasal and medial canthal subunits. TABLE 3
Fixation
Advancement or Distraction
Wire osteosynthesis Wires þ MMF 4 wk Wires þ MMF 6 wk Wire over skull vertex Rigid fixation Rigid external distraction (RED) device External and internal Distraction device External and internal Distraction device External and internal Distraction device External and internal Distraction device RED II device Rigid fixation plate
Advancement Advancement Advancement Advancement Advancement Distraction
Rigid fixation miniplate Rigid fixation miniplate Rigid fixation miniplate Rigid fixation
lag screw and
Advancement
lag screw and
Advancement
lag screw and
Advancement
miniplate
Advancement
fixation miniplate fixation fixation fixation
Advancement Advancement Advancement Advancement
Rigid Rigid Rigid Rigid
Distraction Distraction Distraction Distraction Distraction Advancement
The remaining 14 articles represented 20 reported cases of combined Le Fort III/I osteotomies spanning a 45-year period. The mean patient age was 21 years, and diagnoses included Crouzon syndrome (n ¼ 7), Apert syndrome (n ¼ 5), nonsyndromic craniosynostosis (n ¼ 3), posttraumatic injury (n ¼ 1), acrodysostosis (n ¼ 1), and an unspecified facial dysostosis (n¼ 3). There were 12 patients treated with direct advancement, and eight patients were treated with distraction. A variety of fixation techniques were reported. Older reports presented wire fixation; whereas, newer descriptions included miniplates. The patients treated with distraction used both halo-type devices and internal distractors. The mean advancement of the Le Fort III segment for direct advancement was 7 mm, and mean advancement for distraction was 16 mm. Only two minor complications were reported. One patient had a scalp incision dehiscence at the coronal incision, and one patient, a temporary temporal branch of facial nerve neuropraxia
Preoperative and 6-Month Postoperative Cephalometric Measurements
Measurement
Normative
Preoperataive
Postoperative
SNA SNB ANB SNPG Facial convexity Facial depth LFA:UFA ratio
82 6 2 80 6 3 2 80 6 3 068 88 6 3 0.95 to 1.0
80 86 6 86 11 102 0.69
88 83 5 82 10 101 0.78
Brown et al., SIMULTANEOUS LE FORT III AND LE FORT I
TABLE 2
0
Summary of Individual Cases Present in the Literature
Complications
Le Fort III Movement (mm)
Le Fort I Movement (mm)
Follow-Up (mo)
Outcome
Not specified 24
Stable Stable
8 15
10 7
12 12
Stable Stable
14
14
38
Stable
18
13
16
Stable
20
14
15
Stable
21
18
10
Stable
9
Left 11.5 Right 6.5
12
Stable
Step off at nasal bone
5 to 6
12
Scalp dehiscence
5 to 6
6
Temporal branch neuropraxia
5 to 6
2.5
10
9
12
Stable
Combined 13
Combined 13
24 Not specified
Stable
that resolved in follow-up. The mean reported follow-up was 15 months, and 11 patients had documentation of stability of occlusion or facial aesthetics. One patient had a reported revision for a contour deformity at the nasofrontal junction (Table 2). DISCUSSION The combined LF III/I osteotomy was first described by Obwegeser in 1969. This operation was again advocated for select syndromic patients by Tessier in 1971, although he did not provide any operative description. Since that time, multiple authors have reported their experiences combining the LF III/I osteotomies. In the process of our review we decided to report our experience because, to our knowledge, this is the first case report of a secondary Le Fort III osteotomy with simultaneous Le Fort I osteotomy for a patient with Crouzon syndrome. Additionally, we found that the indications and specifics of the operation have not been well described. The LF III/I should be performed in patients who are skeletally mature. A Le Fort I osteotomy is of concern in a child due to the developing tooth buds. The youngest patient reported in the literature was a 12-year-old; although, the mean age for all the simultaneous procedures was 21 years. Combined osteotomies are useful when a differential advancement of the upper and lower midface is needed, or when advancement of the upper and lower
midface cannot be achieved with a Le Fort III alone due to the soft tissue restrictions. The literature reveals that a majority of cases have been reported in patients with Crouzon or Apert syndromes. We find it interesting that our patient had a diagnosis of Crouzon syndrome confirmed by genetic testing but did not have cranial vault remodeling as a child. She likely had isolated cranial base suture synchondrosis resulting in midface hypoplasia, as described in other syndromic craniosynostoses (McGrath et al., 2012). One theoretical fear of simultaneous osteotomies is devascularization of the osteotomized bony segments. The blood supply to the Le Fort I segment was initially felt to be due to the descending palatine artery. Siebert et al. (1997) proved with latex injection studies that the ascending palatine branch of the facial artery and anterior branch of the ascending pharyngeal artery are responsible for vascular integrity after osteotomy. Avascular necrosis of the maxilla after Le Fort I alone is a rare complication, with few case reports (Lanigan et al., 1990; Pereira et al., 2010) and a reported rate of less than 1% (Kramer et al., 2004). Risk factors are transversal segmentation of the maxilla, particularly with transverse expansion of the segments (Lanigan, 1997; Kramer et al., 2004). In contrast, few reports investigate the blood supply to the Le Fort III segment. Wittenberg and Meyer (1983) studied blood flow to the Le Fort III segment in macaques after multiple incisions (bilateral brow, zygomatic, and
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intraoral) versus a single coronal incision. It is interesting that the blood flow to the Le Fort III osseous segment decreased by 85% when a bicoronal incision was made; whereas, the multiple incision group decreased only by 26%, showing vascularization of the midfacial bone from facial soft tissues. Hence, Wittenberg recommended a multiple-incision approach over a coronal incision for combined Le Fort III/I cases. No major complications were noted in any of the reported cases. In our case, the Le Fort III/I was accomplished via a coronal incision without vascular compromise, but we recommend proceeding with this case with minimal periosteal stripping. Relapse or growth retardation of the upper maxilla is common in patients with Crouzon or Apert syndrome who had standard advancement procedures as children. They often present as adults with recurrent pathology that necessitates orthodontic management or secondary orthognathic surgery (Shetye et al., 2010; Nout et al., 2011; Warren et al., 2012). Therefore, the current standard of care is to perform the Le Fort III with distraction osteogenesis during childhood to overcorrect the midface, anticipating mandibular growth. Although it remains to be determined, distraction osteogenesis may obviate the need for secondary advancement procedures in these patients. Fearon reported up to a 5-year follow-up on Le Fort III distraction cases done at a mean age of 8 years. In his series no one had a secondary procedure during the follow-up period. He attributes factors determining the need of secondary surgery to include the age patients are first operated upon and overcorrection achieved (Fearon, 2005). Fearon reported an average distraction of 19-mm advancement, compared with a 6-mm advancement with a standard Le Fort III advancement (Fearon, 2001). Large, single advancements with a Le Fort III osteotomy to overcorrect the midface may be limited by the stretch of the soft tissues, especially in those with scarred tissue planes. Freihofer (1984) wrote that advancement at any level is limited to 20 mm but remarked that a maxilla that has been previously operated on is more limited. Obwegeser (1969), in his original description of the combined osteotomy, advised advancing the upper maxillary segment ‘‘as far forward as is necessary for esthetic reasons’’ (p. X). In our review of the literature we found that the mean direct advancement of the Le Fort III segment to be 7 mm, with a range of 5 to 10 mm. In the case of our patient we attempted to achieve 8 mm of advancement at the Le Fort III level. After removing the patient from MMF the mandible moved anteriorly, returning the patient to a class III malocclusion, indicating that the mandible was not in centric relation. We thought that she may have additional skin laxity due to a 100-pound weight loss that would allow us to achieve an 8-mm advancement. Ultimately, we were only able to achieve 6 mm at the Le Fort III level. The originally planned Le Fort I osteotomy was needed to advance her lower maxilla an additional 2 mm. The 6-mm advancement is consistent with the numbers reported by
others after Le Fort III osteotomy (Fearon, 2001; Iannetti et al., 2006; Cheung et al., 2010). Larger single advancements are reported in the literature but have also been noted to have complications and relapse (Freihofer, 1973). To help prevent relapse, the zygomatic arches were stabilized with split thickness calvarial bone grafts as initially described by Tessier and advocated by others (Tessier, 1967; Obwegeser, 1969; Kahnberg et al., 1982). Various authors have briefly described their general operative approach, but a step-by-step description of the operation was not found (Obwegeser, 1969; Tessier, 1971; Cheung et al., 2010). We included a detailed description of the operation we performed because most of the descriptions to date have been brief. Though not commonly advocated for use in craniofacial advancement surgeries, a submental intubation was used in our patients. Most of the operative descriptions for Le Fort III and other facial advancements describe the use of a nasotracheal intubation. A recent review of the literature on submental intubation indicated this is a safe procedure with an average operative time of 10 minutes and minimal complications (Jundt et al., 2012). Submental intubation provides several intraoperative conveniences (Lypka and Urata, 2012). It allows for easy access to all facial structures while providing the ability to take the patient in and out of MMF. It also reduces the risk of damaging the endotracheal tube during osteotomies or dislodging the tube from the trachea when moving the nasomaxillary segment forward. Additionally, the surgeon can insert the Rowe disimpaction forceps during down-fracture without a nasotracheal tube obstructing or being damaged. The only other way to achieve this type of access is with a tracheostomy, which comes with a more significant risk of long-term sequelae of the airway. A reinforced tube should be used to prevent obstructive kinking that can otherwise result from a nonreinforced tube curving at an acute angle from the submental intubation. Freihofer (1973) was the first to report complications and long-term follow-up on two patients with simultaneous osteotomies. These patients both had excellent occlusal results in follow-up, and one was followed up to 2 years. It is interesting to note that this was before the time of rigid plate fixation. Schmitz et al. (1995) provided the largest case series in the literature of combined LF III/I osteotomies with 11 patients. However, the Le Fort III procedure in this series was not standard because they did not perform a coronal incision and carry the medial canthus with the Le Fort III segment (hence, this paper was excluded from analysis). The remainder of the literature on combined osteotomies includes case reports and small case series. Ungari et al. (2012), Hariri et al. (2011), and Cheung et al. (2010) have published the use of LF III/I in both nonsyndromic and syndromic cases. Hariri et al. (2011) commented that this strategy can address the deformity of the upper and lower midface differentially in cases where the upper midface is hypoplastic and the lower maxillary
Brown et al., SIMULTANEOUS LE FORT III AND LE FORT I
dentoalveolus is hyperplastic. Bradley et al. (2008) reported two patients with simultaneous LF III/I after previous monobloc distraction with combined facial bipartition. Dai et al. (2012) published a simultaneous Le Fort I, II, and III in a patient with Apert syndrome. Among all these cases only a few minor postoperative complications have been reported. This information is relatively unreliable because it is inevitable that poor results or complications are much less likely to be reported, unless they are within a large series or cohort. However, consistent with the literature, our patient did well postoperatively. She had a 4-day hospitalization, mostly secondary to facial swelling and difficulty with secretions. In the literature, length of stay ranged from 1 to 2 weeks (Cheung et al., 2010; Hariri et al., 2011; Dai et al., 2012). During her recovery the patient had both speech and masticatory difficulties. The surgery altered her nasopharynx, causing some velopharyngeal incompetence. These issues resolved with speech therapy and are not uncommon with these procedures (Fearon, 2005). In some cases preoperative nasal endoscopy and speech evaluation may help in both counseling and preparing patients for these postoperative changes. Our patient has had no further complications, with stable occlusion at 1 year. The psychosocial benefits of this procedure cannot be underestimated. The patient had been extremely selfconscious prior to the operation. Postoperatively, she began to wear makeup around her eyes for the first time, had an improved self-image, and even discussed starting a family. Although this procedure has the potential to correct complex deformities of the midface, relatively few simultaneous LF III/I osteotomies have been reported in the literature. Underreporting may be one potential explanation for the relatively low frequency of this procedure. The more widespread use of distraction devices for Le Fort III advancements in childhood has likely reduced the frequency of these combined LF III/I surgeries. Another potential explanation to the low frequency of this procedure is the need for a surgeon to have in-depth knowledge to perform a combination of both craniofacial and orthognathic surgical techniques. Other authors have reported on the use of combinations of osteotomies to differentially distract the midface to achieve correction of a variety of complex deformities. Matsumoto et al. (2002), Takashima et al. (2006), and Satoh et al. (2003) have reported their experience with LF III/I and facial distraction, allowing larger advancements. Hopper et al. (XXXX) recently published a series that included three Apert syndrome patients who had combined Le Fort III advancement with Le Fort II distraction. One of these patients had a previously failed Le Fort III distraction. He demonstrated successful outcomes in all of his patients. Furthermore, Lakin and Kawamoto (2012)
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performed a combined Le Fort II/Le Fort I osteotomy to differentially advance the nasomaxillary segment. In summary, we found evidence of only 20 separate cases of combined LF III/I since 1969. It is interesting to note that, to our knowledge, at least 24 facial transplants have been performed and reported thus far since the original transplant in 2005 (Dorafshar et al., 2013). In 1995 Polley commented in his article that combining facial osteotomies ‘‘has perhaps its greatest potential in reconstruction of the midface; unfortunately, the application to this area has received the least amount of attention’’ (Polley and Figueroa, 1995, p. X). Acknowledgment. We thank Dr. Mark Urata for his mentorship and surgical guidance on the case presented in this article.
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