Orthognathic surgery with iliac bone grafting for an interpositional gap in a patient with type III hemifacial microsomia: A case report.

Hemifacial microsomia (HFM) is characterized by uni- or bilateral microtia and hypoplasia of the mandible, orbits, facial nerve, and adjacent soft tissues. Patients with Pruzansky-Kaban type III HFM show the most severe facial deformities and often encounter difficulty obtaining treatment. In recent years, orthognathic surgery for HFM-related deformities has often been performed after the patient has stopped growing. However, few detailed reports have described the difficulties of orthognathic surgery for patients with type III HFM. This report describes the case of a patient with type III HFM who underwent three unilateral mandibular reconstructions while still growing, including autogenous reconstructions and secondary distraction osteogenesis, followed by orthognathic surgery with iliac bone grafting for an interpositional gap between the proximal and distal segments after she had stopped growing to improve facial asymmetry and malocclusion.

Posterior pharyngeal wall augmentation using autologous tiered costal cartilage for velopharyngeal insufficiency.

The aim of this study was to retrospectively estimate intervention outcomes and to establish suitable indications for autogenous tiered cartilage augmentation (ATCA) of the posterior pharyngeal wall for velopharyngeal insufficiency (VPI). The sample cohort comprised 17 consecutive patients with VPI (10 boys, 7 girls), excluding syndromic cases. Pre- and postoperative findings were comprehensively evaluated using a combination of speech assessment and lateral pharyngography (LPG). Hypernasality and consonant distortion were scored (normal: 0; mild: 1; moderate: 2; severe: 3). Velopharyngeal gap size at rest (AC) and at maximum closure (BC) were measured on LPG. All patients displayed improvements in velopharyngeal function and speech score, and no patients required re-operation. Pre- and postoperative hypernasality scores were 1.9 ± 0.5 and 0.6 ± 0.5, respectively (P < .001). Pre- and postoperative consonant distortion scores were 1.9 ± 0.6 and 0.6 ± 0.5, respectively (P < .001). Pre- and postoperative AC distances were 8.2 ± 3.4 mm and 5.9 ± 2.6 mm, respectively (P < .001). Pre- and postoperative BC distances were 3.9 ± 2.4 mm and 0.3 ± 0.8 mm, respectively (P < .001). Conclusions: ATCA appears effective for surgical treatment of VPI patients with a gap less than 10 mm.

A predictor of a postoperative fistula after double-opposing Z-plasty in bilateral cleft lip and palate patients.

Furlow double-opposing Z-plasty (FDOZ) as primary palatoplasty for the bilateral cleft lip and palate (BCLP) closure often leads to a palatal fistula formation in cases with wide clefts. We examined the utility of the calculated maximum closable cleft width to determine the feasibility of a direct palatal closure without lateral incisions. In this retrospective study, we analyzed consecutive patients with a BCLP who were treated for 5 years from 2009. In sixty-three BCLP patients, the following dimensions were measured preoperatively: an actual distance between maxillary tuberosities (Actual X); actual cleft height, calculated from the line joining the maxillary tuberosities to the cleft edge (Actual Y); and actual cleft width at the hard-soft palate junction (Actual Z) and calculated maximum cleft width requiring a direct closure (CMZ). Six months postoperatively, the relationships between the fistula occurrence and actual Z/CMZ were examined. Median values (interquartile range) of Actual X, Y, and Z and CMZ were 35.0 (33.0-39.0) mm, 7.0 (6.0-8.0) mm, 10.0 (8.0-11.0) mm, and 4.6 (2.7-5.7) mm, respectively. The median age at operation was 8.0 (7.0-17.0) months. Fistulae at the hard-soft palate junction developed postoperatively in three cases with more than a 10-mm cleft width (4.76%). A receiver operating characteristic (ROC) curve analysis indicated that actual Z was a better predictor of a postoperative fistula formation than CMZ. An actual cleft width was a better predictor of the fistula occurrence than a calculated maximum cleft width after FDOZ for a BCLP repair. The low fistula rate of FDOZ suggested that FDOZ could be used to close the palate with less than a 10-mm cleft width.

Le fort II distraction osteogenesis with a hybrid system for an Apert syndrome patient: A case report.

BACKGROUND: Le Fort II advancement is considered for normalizing the facial appearance in Apert syndrome. When these procedures are performed during growth, overcorrection of midface advancement is required. We developed a system that can control the distance and vector of movement for the central midface to create more normal facial proportions. This case report shows Le Fort II distraction osteogenesis with this hybrid system for an Apert syndrome patient. CASE: The patient was a girl with Apert syndrome with midfacial-nose hypoplasia and skeletal class III malocclusion. She was healthy without respiratory problems and had no learning disabilities. She underwent our Le Fort II distraction osteogenesis with the hybrid system at 10 years and 6 months of age. Her midface was elongated 22 mm at point Or forward and moved 5° downward to the Frankfort horizontal plane compared to the standard position of average Japanese adult women on the cephalogram. Examining the facial image, the midfacial depression was improved 4 years after the operation. DISCUSSION: Overcorrection of midface advancement is required for patients to reduce the number of procedures during growth. The system that we developed could control the distance and vector of movement steadily when the central midface was overcorrected to try to create normal adult facial proportions.

Treatment of congenital short palate using bilateral buccal musculomucosal flaps.

A case of congenital short palate was treated by bilateral buccal musculomucosal flaps. The levator veli palatini muscle formed a continuous sling, but the anterior portion was attached to the posterior border of the hard palate. The speech outcome improved from severe to normal.

Evaluation of Congenital Maxillary Growth Using Computed Tomography in Patients With Bilateral Cleft Lip and Palate.

OBJECTIVE: To analyze congenital lateral maxillary growth for patients with bilateral cleft lip and palate (BCLP). DESIGN: A retrospective study. SETTING: Kanagawa Children's Medical Center. MATERIALS: Images from computed tomography (CT) of patients with BCLP and control patients that were previously used for treatment. MAIN OUTCOME MEASURES: The following landmarks were used: A, the posterior most point of the piriform aperture; B, the superior most point of the acoustic meatus; C, the point at which line A-B intersects the line drawn perpendicular from line A-B to the maxillary tuberosity; and D, the apical most point of the nasal bone. The following distances were then measured using these landmarks: (1) A-B distance; (2) A-C distance; (3) A-C/A-B; (4) the angle between lines A-B and A-D (∠BAD); and (5) B-D distance. RESULT: Mean A-B and A-C distances and A-C/A-B were significantly smaller in the BCLP group than in the control group (P < .01 each). Mean ∠BAD was significantly larger in the BCLP group than in the control group (P < .01). Mean B-D distance did not differ significantly between groups. CONCLUSIONS: Our results indicated that the lateral maxillary segments of patients with BCLP were more posterior than those of the control group, and segment length was shorter compared to the control group on 3D-CT analysis. The lateral maxillary segments of patients with BCLP were basically suggested to originally be underdeveloped.

Orthodontic premaxillary setback versus premaxillary osteotomy with gingivoperiosteoplasty for Bilateral cleft lip and palate patients: 4-year observation outcomes.

Patients with bilateral cleft lip and palate (BCLP) generally require several stages of treatment, and adequate maxillary development without orthognathic surgery is not easy to achieve. The purpose of this study was to determine short-term outcomes of orthodontic premaxillary setback (OPS) and premaxillary osteotomy (PO) for complete BCLP treatment. PATIENTS AND METHODS: Twenty-six patients with BCLP were consecutively treated for 4 years by a single surgeon. All patients were categorized by three indications for the first operation: OPS1, cheiloplasty + gingivoperiosteoplasty (GPP) + palatoplasty; OPS2, cheiloplasty + GPP; and PO, cheiloplasty + GPP + PO. Cephalograms for maxillary growth and velopharyngeal function (VPF) were judged at 4 years old, before orthodontic treatment. RESULTS: OPS1 was performed in 14 cases, OPS2 in 8 cases, and PO in 4 cases. As for cephalometric analysis at 4 years old, no significant differences between groups were seen in any cephalometric measurements. As for speech outcomes, assessment of VPF at 80.8±14.8 months was good in 17 cases, slightly impaired in 8 cases, and marginally impaired in 1 case, with no severely impaired cases. DISCUSSION: No significant differences in maxillary growth or speech outcomes were seen between OPS1, OPS2, and PO groups at 4 years old, possibly because all groups showed the same position of the premaxilla after the first operation. CONCLUSIONS: No significant differences in maxillary growth or speech outcomes were seen for 26 patients with BCLP between OPS1, OPS2, and PO groups at 4 years old. However, the long-term growth characteristics remain unclear.

Perinasal Osteotomy With Distraction Osteogenesis for a Mild Syndromic Craniosynostosis.

Le Fort II and III procedures have generally been performed for syndromic craniosynostosis with midfacial hypoplasia and skeletal class III malocclusion. However, some patients have midfacial hypoplasia without malocclusion. Perinasal osteotomy was performed with distraction osteogenesis to move the midface forward in 2 patients (a 17-year old female patient with Crouzon-like disease and a 15-year-old female patient with Antely-Bixler syndrome) with mild midface hypoplasia without malocclusion. The success of the procedure was assured by 3 features: the intermaxillary sutures were fixed by a mini metal plate to prevent separation during distraction; the distraction wires were fixed through the bone of the piriform aperture with the mini metal plates to prevent the wires from coming off; and the osteotomy line was designed in front of the palatomaxillary suture to avoid suture damage. These were expected to secure the procedure. Perinasal osteotomy with distraction osteogenesis is considered one of the recommended procedures for mild midfacial hypoplasia as seen in mild syndromic craniosynostosis without malocclusion.

Overcorrected Midface Advancement to Improve Airway Problems in Severe Pfeiffer Syndrome Types II and III.

INTRODUCTION: Treatment of patients with severe Pfeiffer syndrome types II and III is difficult. The purpose of this article is to present our method of overcorrecting midface advancement to improve airway problems in such patients. MATERIALS AND METHODS: One boy and two girls with types II and III Pfeiffer syndrome and who underwent Le Fort III midface advancement using our previously described corrected cephalometric analysis and distraction system were included in the study. RESULTS: The authors overcorrected by advancing the midface to make it look as similar as possible to an adult face. While the overcorrected midface advancement widened the upper airway spaces in the 3 patients, the tracheostomy that had already been placed during infancy could not be closed, probably because of an underlying tracheal abnormality or tracheomalacia. DISCUSSION: Overcorrected midface advancement cannot enable tracheostomy closure, probably because of severe tracheal anomalies, such as tracheomalacia, below the tracheostomy. However, with the possibility of gradual improvement of the tracheomalacia with age, closure of the tracheostomy can eventually be expected. Therefore, efforts to close a tracheostomy should be pursued even if the probability of its removal is low. CONCLUSION: Overcorrected midface advancement did not enable tracheostomy closure, probably because of severe tracheal anomalies such as tracheomalacia. However, the severe exophthalmos and angle III malocclusion were improved, and with the possibility of gradual improvement of the tracheomalacia with age, closure of the tracheostomy can eventually be expected. Therefore, efforts to close a tracheostomy should be pursued even if the probability of its removal is low.

A Survey of Orthodontic Treatment in Team Care for Patients With Syndromic Craniosynostosis in Japan.

OBJECTIVE: To understand the actual condition of orthodontic treatment in team care for patients with syndromic craniosynostosis (SCS) in Japan. DESIGN: A nationwide collaborative survey. SETTING: Twenty-four orthodontic clinics in Japan. PATIENTS: A total of 246 patients with SCS. MAIN OUTCOME MEASURE: Treatment history was examined based on orthodontic records using common survey sheets. RESULTS: Most patients first visited the orthodontic clinic in the deciduous or mixed dentition phase. Midface advancement was performed without visiting the orthodontic clinic in about a quarter of the patients, and more than a half of the patients underwent "surgery-first" midface advancement. First-phase orthodontic treatment was carried out in about a half of the patients, and maxillary expansion and protraction were performed. Tooth extraction was required in about two-thirds of patients, and the extraction of maxillary teeth was required in most patients. Tooth abnormalities were found in 37.8% of patients, and abnormalities of maxillary molars were frequently (58.3%) found in patients who had undergone midface surgery below the age of 6 years. CONCLUSIONS: Many patients underwent "surgery-first" midface advancement, and visiting the orthodontic clinic at least before advancement was considered desirable. First-phase orthodontic treatment should be performed considering the burden of care. Midface advancement below the age of 6 years had a high risk of injury to the maxillary molars. This survey is considered useful for improving orthodontic treatment in team care of patients with SCS.

Synchronous Premaxillary Osteotomy with Primary Cheiloplasty for BCLP Patients with Protrusion of the Premaxillae.

BACKGROUND: In bilateral cleft lip and palate (BCLP) patients with protrusion and/or torsion of the premaxillae, it is difficult to achieve a good outcome. We have developed a series of procedures of premaxillary osteotomy with primary cheiloplasty for BCLP patients who did not respond well to presurgical orthodontics (PSO). METHODS: A total of 27 BCLP patients with protrusion and/or torsion of the premaxillae underwent PSO. For 3 BCLP patients in whom the protruded premaxillae could not be returned to a good position, a primary premaxillary osteotomy and gingivoperiosteoplasty (GPP) with cheiloplasty were performed simultaneously. Subsequently, Furlow palatoplasty was performed by one and a half years of age. Maxillary growth was evaluated by dental occlusion at 4 years of age. RESULTS: A premaxillary osteotomy and GPP with cheiloplasty were performed at 6 months. The patients' facial structures improved, their premaxillae were positioned more superiorly, and normal inclination of the incisors was achieved. They had edge-to-edge occlusions or cross bites at 4 years of age. CONCLUSIONS: As advantages, the patients' facial structures improved, and the alveolar bones were formed by GPP. As a disadvantage, premaxillary necrosis might occur because of poor blood circulation. It is important to secure the following 2 blood supplies: from the periosteum and soft-tissue of the anterior premaxillae and from the periosteum and mucosa of the nasal septum. Synchronous premaxillary osteotomy and GPP with primary cheiloplasty are appropriate when the premaxillae cannot be properly repositioned by PSO or PSO cannot be done.

Corrected Cephalometric Analysis to Determine the Distance and Vector of Distraction Osteogenesis for Syndromic Craniosynostosis.

BACKGROUND: The purpose of this study was to confirm the utility of a corrected cephalometric analysis to facilitate the planning of distraction osteogenesis with Le Fort III osteotomy for syndromic craniosynostosis. METHODS: This prospective study involved 4 male and 2 female patients (mean patient age, 8 years 9 months; age range, 4 years 6 months to 13 years 2 months) with Crouzon syndrome who were treated with Le Fort III maxillary distraction using our previously described system of analysis of a corrected cephalogram and who underwent clinical follow-up. Lateral cephalograms were obtained immediately after device removal. RESULTS: Distraction of orbitale moved the vector downward to the adult profile, but there was slightly less elongation than the adult profile for the distraction distance. The desired and real mean angles after distraction of point A were 29.2 ± 7.9° and 6.1 ± 8.5°, respectively, and the desired and the real mean distances after distraction of point A were 30.6 ± 12.7 mm and 29.4 ± 4.1 mm, respectively. CONCLUSIONS: Using the corrected cephalometric analysis, the distance and vector of distraction osteogenesis with Le Fort III osteotomy could be determined in patients with syndromic craniosynostosis. The distraction system brought the patients' facial bones to the planned position using controlling devices.

Transmaxillary Sinus Approach for Le Fort II Osteotomy.

The Le Fort II osteotomy is a relatively rare operation. The posterior wall osteotomy of the maxillary sinus (MS) is often difficult for Le Fort III. We developed the transmaxillary sinus approach (TSA) for the Le Fort II osteotomy that cuts the posterior wall of the MS directly. This report illustrates this easy-to-use procedure for the Le Fort II osteotomy in syndromic craniosynostosis. This procedure was performed in an 18-year-old patient with Apert syndrome and a 15-year-old patient with Pfeiffer syndrome. The thin anterior walls of the MS were removed through an intraoral approach to look inside the MS. Then, the posterior walls were cut by chisel under direct vision using light. The other osteotomy was performed as usual. Distraction osteogenesis with internal and external devices was used in combination. The advantages of TSA are the direct posterior wall osteotomy of the MS with no down fracture and minimal invasiveness to the mucosa of the MS under direct vision. However, the disadvantage is that TSA becomes a blind procedure in a case with no MS or hypoplasia. We developed the TSA for the Le Fort II osteotomy, which could provide direct observation and perform the posterior wall osteotomy of the MS without down fracture. We believe that TSA is an effective surgical procedure for the Le Fort II osteotomy.

Primary Palatoplasty for Unilateral Cleft Lip and Palate Using Mucosal Grafts and Flaps.

The mucosal graft and flaps method (MG method) is a palatoplasty technique that was developed for the purpose of improving maxillary growth in patients with cleft palate. In the MG method, full-thickness buccal mucosa is grafted onto the raw surface created by pushback palatoplasty. The method is unlikely to result in severe scarring and has a favorable effect on maxillary growth. In addition, it is unlikely to result in oronasal fistula and provides good speech results. Overall, postive long-term treatment results have been obtained. Although the MG method is technically difficult and requires a lengthy surgery, the technique is considered to be effective for palate closure in terms of speech and maxillary growth.

Maxillary growth after maxillary protraction: Appliance in conjunction with presurgical orthopedics, gingivoperiosteoplasty, and Furlow palatoplasty for complete bilateral cleft lip and palate patients with protruded premaxilla.

BACKGROUND AND AIMS: In bilateral cleft lip and palate (BCLP) with premaxillary protrusion, a good outcome with adequate maxillary development is difficult to achieve. The purpose of this article is to evaluate the maxillary growth after using presurgical orthopedics (PSO), gingivoperiosteoplasty (GPP), Furlow palatoplasty, and maxillary protraction appliance (MPA) for BCLP with premaxillary protrusion. PATIENTS AND METHODS: Seven patients with complete BCLP with premaxillary protrusion were treated by PSO, cheiloplasty, GPP, and Furlow palatoplasty. MPA was used as part of the protocol for 6 months to 1 year for postoperative retardation of maxillary growth cases. Maxillary growth was evaluated by cephalometric analysis at 4 and 10 years of age, and bone formation at the alveolar cleft was evaluated by computed tomography (CT) imaging at 5 years of age. RESULTS: At 4 years of age, three of seven patients had apparent retardation of maxillary growth. The maxillary growth at 10 years of age was equivalent to the average value of normal Japanese after using MPA in three cases. At 5 years of age, only two of seven patients showed sufficient bone formation at the alveolar cleft to avoid alveolar bone grafting (ABG). Subsequently, ABG was performed in five patients. DISCUSSION: Although three of seven patients had apparent crossbite at 4 years of age, the maxillary growth of all patients at 10 years of age was approximately equivalent to the average value of normal Japanese after using MPA. A treatment protocol based on PSO, GPP, Furlow palatoplasty, and MPA may be an option, but long-term growth is unknown.

The folded buccal musculomucosal flap for large palatal fistulae in cleft palate.

BACKGROUND: A palatal fistula is a common complication of cleft palate repair. Although a buccal musculomucosal flap (BMMF) is effective for fistula repair, it does have the following problems: a second operation may be required to release the pedicle on the oral side and unilateral BMMF cannot close mucosal defects of both the nasal and oral sides. A novel fistula closure method using the folded BMMF (f-BMMF) invented by the authors is presented. CASE: A 8-year-old-boy with bilateral cleft lip and palate with anencephaly. A fistula in the hard palate occurred after palatoplasty by the Furlow method, and an f-BMMF was planned. The mucosal defects of the nasal and oral sides were covered by 2 separate islands of mucosal epithelium. Finally, no reoperation was needed to remove the pedicle of the f-BMMF. CONCLUSION: The f-BMMF is able to cover both sides without a raw surface and a mucosal graft even in cases of large fistula closure, although BMMF cannot usually cover both oral and nasal sides of a fistula. The advantages of this procedure are that it does not require second surgery to release the pedicle and that its distal island mucosa can be used to monitor engraftment. This proposed method seems to be an appealing alternative.

A osteogenesis distraction device enabling control of vertical direction for syndromic craniosynostosis.

BACKGROUND: We have developed a hybrid facial osteogenesis distraction system that combines the advantages of external and internal distraction devices to enable control of both the distraction distance and vector. However, when the advanced maxilla has excessive clockwise rotation and shifts more downward vertically than planned, it might be impossible to pull it up to correct it. We invented devices attached to external distraction systems that can control the vertical vector of distraction to resolve this problem. The purpose of this article is to describe the result of utilizing the distraction system for syndromic craniosynostosis. METHODS: In addition to a previously reported hybrid facial distraction system, the devices for controlling the vertical direction of the advanced maxilla were attached to the external distraction device. The vertical direction of the advanced maxilla can be controlled by adjustment of the spindle units. This system was used for 2 patients with Crouzon and Apert syndrome. RESULTS: The system enabled control of the vertical distance, with no complications during the procedures. As a result, the maxilla could be advanced into the planned position including overcorrection without excessive clockwise rotation of distraction. CONCLUSION: Our system can alter the cases and bring them into the planned position, by controlling the vertical vector of distraction. We believe that this system might be effective in infants with syndromic craniosynostosis as it involves 2 osteotomies and horizontal and vertical direction of elongation can be controlled.

Maxillary growth after the use of protraction head gear in conjunction with presurgical orthopedics and gingivoperiosteoplasty for complete bilateral cleft lip and alveolus patients.

INTRODUCTION: Maxillary development is often inadequate in bilateral cleft patients. The use of presurgical orthopedics (PSO) and gingivoperiosteoplasty (GPP) may promote bone formation at the alveolar cleft, but can also have detrimental effects on maxillary development. Our objective was to investigate the effect of PSO and GPP on maxillary development in bilateral cleft lip and alveolus (BCLA) patients. SUBJECTS AND METHODS: We had 3 complete BCLA patients who had received PSO. All patients underwent cheiloplasty and GPP simultaneously. At 4 years, maxillary protraction head gear was used as part of the protocol. They were evaluated by cephalometric analysis at 4 and 8 years of age, and by CT imaging at 5 years of age. RESULTS: At 4 years of age, patients with all BCLA had anterior crossbite of deciduous central incisors. As a result of maxillary protraction, jaw development at 8 years was good. Among all patients, only one showed bone formation at the alveolar cleft sufficient to avoid alveolar bone grafting (ABG). DISCUSSION: All patients presented anterior crossbite in the premaxillary region, but had good maxillary growth at 8 years old as a result of maxillary protraction. The combination of PSO and GPP can potentially eliminate the need for ABG and does not significantly retard maxillary development. PSO with GPP and protraction head gear may be an option, but long-term growth is not known.

A novel craniofacial osteogenesis distraction system enabling control of distraction distance and vector for the treatment of syndromic craniosynostosis.

BACKGROUND: Distraction osteogenesis is now an important clinical tool in craniofacial surgery. However, controlling the distance and vector of distraction in infants with syndromic craniosynostosis with good repeatability is a task that still proves difficult today. We have developed a new facial osteogenesis distraction system that combines the advantages of external and internal distraction devices to enable control of both the distraction distance and vector. This article describes the method and short-term results of this system. METHODS: Our distraction system uses both a conventional external distraction device and a newly developed internal distraction device. Postoperative control of the distraction vector is performed using the external device, whereas control of distraction distance is done with the adjustable-angle internal device. This system was used for 2 patients with Crouzon syndrome. RESULTS: The system enabled control of lengthening distance and vector, and no complications occurred during the procedures. CONCLUSIONS: We developed a facial distraction system leveraging the advantages of external and internal distraction devices, which we then used to successfully control both lengthening distance and vector. The system would be particularly indicated in patients with severe scarring due to multiple follow-up surgeries and in patients requiring distraction of 20 mm or more. Aligning the periorbital profile at 5 to 6 years old caused the maxilla to rotate counterclockwise, and we consider that a procedure combining Le Fort III osteotomy with Le Fort I and II osteotomies is required to prevent these rotations.