In support of using computer-aided design and modeling for periorbital osteotomies.

Three-dimensional virtual surgical planning using computer-aided design and modeling (CAD/CAM) has gained popularity in planning complex orthognathic and osteocutaneous free flap reconstructions of the head and neck because of its ability to guide complex geometric planning in three-dimensional space and save time in the operating room. The purpose of this study was to review our experience using CAD/CAM concepts in periorbital osteotomies. Three complex periorbital osteotomies were planned and performed: 1 case of bilateral vertical and horizontal orbital dystopia, 1 case of a Monobloc-Le Fort II in an Apert patient, and 1 case of recurrent hypertelorism in a patient with craniofrontal nasal dysplasia. The patients' charts were reviewed, including photographs, medical records, and CAD/CAM plans. The CAD/CAM planning sessions were held 3 to 6 weeks preoperatively and lasted approximately 1 hour. Both cutting guides and positioning guides were used, translating to significant precision of both the osteotomy as well as the final position of the orbits. Qualitatively, the cutting and positioning guides were easy to use and improved operating room efficiency. To conclude, in our hands, CAD/CAM virtual surgical planning is safe and effective in the performance of complex periorbital osteotomies. More work is needed to more clearly define surgical indications for this costly, new technology.

Premature closure of the spheno-occipital synchondrosis in Pfeiffer syndrome: a link to midface hypoplasia.

The spheno-occipital synchondrosis (SOS) is a critical component of midfacial and cranial base growth. Premature closure has been associated with midface hypoplasia in animal models and syndromic craniosynostosis subpopulations with Apert and Muenke syndromes. To link premature SOS closure and midface hypoplasia in patients with Pfeiffer syndrome, a retrospective case-control study was performed in patients treated at a large craniofacial center between 1982 and 2012 diagnosed with Pfeiffer syndrome. At least 1 computed tomography (CT) scan was required to assess SOS patency. Age-/sex-matched control CT scans were also assessed for SOS patency. Three independent reviewers with high interrater reliability (κ = 0.88) graded SOS patency as open, partially closed, or completely closed. Wilcoxon rank sum test compared the Pfeiffer patients with control subjects. A total of 63 CT scans in 16 patients with Pfeiffer syndrome, all with midface hypoplasia, and 63 age-/sex-matched control scans, none of whom had midface hypoplasia, met inclusion criteria. Earliest partial SOS closure in patients with Pfeiffer syndrome was seen at 5 days compared with control subjects at 7.07 years. Earliest age at complete fusion was 2.76 years in the Pfeiffer cohort and 12.74 years in control subjects. Average age at partial closure was significantly younger (4.99 ± 3.33 years; n = 31 scans) in patients with Pfeiffer syndrome compared with control subjects (10.92 ± 3.53 years) (P = 0.0005), whereas average age at complete closure (11.90 ± 7.04 years) was not significantly different than that in control subjects (16.07 ± 3.39 years). Although definitive causality cannot be concluded, a strong correlation exists between midface hypoplasia and premature SOS closure in Pfeiffer syndrome.

The impact of a parent liaison at a major craniofacial center.

The psychosocial impact of craniofacial disfigurement affects both the developing child and his/her family. The Facial Reconstruction Center at the Children's Hospital of Philadelphia has employed a Parent Liaison (PL) to provide psychosocial support to families and has been an invaluable resource in this regard. We hypothesize that a PL impacts the overall outcome of the surgery by building trust between the parents and medical institution, and increasing satisfaction. An anonymous satisfaction survey was sent to families of craniofacial patients treated between January 1976 and June 2012. All patients who had undergone at least 1 craniofacial procedure had addresses on file and were included in this study. Statistical analyses were performed using the Mann-Whitney U test.During the study, 441 surveys were mailed to families meeting the inclusion criteria. A total of 151 families returned completed surveys (34.2%), and 121 surveys were included for analysis (27.4%). In rating overall satisfaction, families who met with the PL had statistically higher scores than those who had not (P = 0.0011). Parents who met with the PL preoperatively reported greater satisfaction in time spent answering questions (P = 0.0029) and the perception that questions were adequately answered (P = 0.0039). No statistical difference was observed in postoperative preparedness between families that did and did not meet the PL. The results demonstrate that the PL is beneficial in the education, experience, and satisfaction of families treated at a large Craniofacial Center. The PL complements the surgeon's treatment of the physical by adding psychosocial support.

Monobloc minus Le Fort II for single-stage treatment of the Apert phenotype.

Treatment of the Apert syndrome phenotype aims to correct airway obstruction, exorbitism, elevated intracranial pressure, midface hypoplasia, and malocclusion. Cranial vault expansion prevents elevated intracranial pressure, normalizes head shape, and protects the globes, but variation exists in surgical timing and osteotomy to treat the midface. We present the case of an 11-year-old female patient with Apert syndrome and no prior surgical interventions who presented with severe turribrachycephaly, exorbitism, severe midface retrusion, and apertognathia. A monobloc distraction with simultaneous Le Fort II distraction was planned using computer-aided design and modeling (CAD/CAM) techniques to provide for concurrent distraction of the segments in independent vectors without bony interferences. Monobloc minus Le Fort II distraction was performed without intraoperative complications. Surgical time was 340 minutes with an estimated blood loss of 1100 mL. Distraction began on postoperative day 5 at a rate of 1.5 mm/day for the Le Fort II via an external Halo distractor and 1 mm/day for the monobloc segment via internal distractors anchored bitemporally. The monobloc was distracted a total of 17 mm in a horizontal vector, while the Le Fort II segment was distracted 18 mm horizontally and 5 mm inferiorly. The Halo distractor was removed 3 months following the procedure and the internal distractors 1 month later. Monobloc minus Le Fort II distraction enables correction of the Apert phenotype with a single-stage approach, potentially decreasing the burden of care with improved results. Utilization of CAD/CAM modeling allows for accurate planning of multisegment distraction in independent vectors without concerns for bony interferences.

Monobloc minus Le Fort II for single-stage treatment of the Apert phenotype.

Treatment of the Apert syndrome phenotype aims to correct airway obstruction, exorbitism, elevated intracranial pressure, midface hypoplasia, and malocclusion. Cranial vault expansion prevents elevated intracranial pressure, normalizes head shape, and protects the globes, but variation exists in surgical timing and osteotomy to treat the midface. We present the case of an 11-year-old female patient with Apert syndrome and no prior surgical interventions who presented with severe turribrachycephaly, exorbitism, severe midface retrusion, and apertognathia. A monobloc distraction with simultaneous Le Fort II distraction was planned using computer-aided design and modeling (CAD/CAM) techniques to provide for concurrent distraction of the segments in independent vectors without bony interferences.Monobloc minus Le Fort II distraction was performed without intraoperative complications. Surgical time was 340 minutes with an estimated blood loss of 1100 mL. Distraction began on postoperative day 5 at a rate of 1.5 mm/day for the Le Fort II via an external Halo distractor and 1 mm/day for the monobloc segment via internal distractors anchored bitemporally. The monobloc was distracted a total of 17 mm in a horizontal vector, while the Le Fort II segment was distracted 18 mm horizontally and 5 mm inferiorly. The Halo distractor was removed 3 months following the procedure and the internal distractors 1 month later. Monobloc minus Le Fort II distraction enables correction of the Apert phenotype with a single-stage approach, potentially decreasing the burden of care with improved results. Utilization of CAD/CAM modeling allows for accurate planning of multisegment distraction in independent vectors without concerns for bony interferences.

Utility of the ultrasonic scalpel in mandibular distraction osteogenesis.

The purpose of this study was to describe our technique of bilateral mandibular distraction for micrognathia and to highlight the ultrasonic scalpel as an alternative to conventional saws in performing osteotomies for mandibular distraction osteogenesis. To do so, we retrospectively reviewed all patients who underwent mandibular distraction with an ultrasonic scalpel for tongue-based upper airway obstruction due to micrognathia between 2010 and 2011. Study outcome measures include operative blood loss, length of surgery, postoperative complications, and avoidance of a tracheostomy. Excel (Microsoft) was used to calculate averages, P values (2-tailed Student t test), and SDs for operative data, sleep studies, and cephalometric analysis. Nine patients--7 females and 2 males--were distracted for a mean distance of 17 ± 6 mm. Mean blood loss was 15 ± 7 mL, and the average length of surgery was 111 ± 27 minutes. One patient returned to the operating room for debridement/washout of a wound infection, but distraction was continued without sequelae. There were no other postoperative complications. Resolution of airway obstruction was evidenced by clinical examination and avoidance of a tracheostomy in all cases. Based on these data, we feel that mandibular distraction with univector, internal distractors, and ultrasonic osteotomies at the mandibular angle is safe and efficacious at relieving tongue-based upper airway obstruction and avoiding a tracheostomy.

Comparison of spring-mediated cranioplasty to minimally invasive strip craniectomy and barrel staving for early treatment of sagittal craniosynostosis.

The treatment of sagittal craniosynostosis has evolved from early strip craniectomy to total cranial vault remodeling and now back to attempts at minimally invasive correction. To optimize outcomes while minimizing morbidity, we currently use 2 methods of reconstruction in patients younger than 9 months: spring-mediated cranioplasty (SMC) and minimally invasive strip craniectomy with parietal barrel staving (SCPB). The purpose of this study was to compare the safety and efficacy of the 2 methods. Hospital records of our first 7 SMCs and our last 7 SCPBs were analyzed for demographics, the type of operation performed, estimated blood loss, transfusion requirements, operative time, length of stay in the intensive care unit, length of hospital stay, preoperative cephalic index, postoperative cephalic index, and complications. The techniques were then compared using analysis of variance.All 14 patients successfully underwent cranial vault remodeling with significant improvement in cephalic index. Demographics, length of stay in the intensive care unit (P = 0.15), preoperative cephalic index (P = 0.86), and postoperative cephalic index (P = 0.64) were similar between SMC and SCPB. Spring-mediated cranioplasty had statistically significantly shorter operative time (P = 0.002), less estimated blood loss (P < 0.001), and shorter length of hospital stay (P = 0.009) as compared with SCPB. Complications included 1 spring dislodgment in an SMC that did not require additional management and 1 undercorrection in the SCPB group. Both SMC and SCPB are safe, effective means of treating sagittal craniosynostosis. Spring-mediated cranioplasty has become our predominant means of treatment of scaphocephaly in patients younger than 9 months because of its improved morbidity profile.

Mandibular microsurgical reconstruction in patients with hemifacial microsomia.

BACKGROUND: Although hemifacial microsomia is a relatively common craniofacial malformation, there is some debate regarding the ideal treatment of severe mandibular hypoplasia. Traditionally, patients with severe mandibular deficits have been treated with iliac or costochondral bone grafts followed by distraction osteogenesis, with mixed results. The authors present their experience with the use of the fibula osteocutaneous free flap for mandibular reconstruction in severe hemifacial microsomia patients. METHODS: From 1999 to 2006, 10 patients aged 3 to 10 years (mean, 7.2 years) underwent 10 free flap reconstructions. Of the 10 patients, six were girls and four were boys. Data were collected retrospectively from the patients' records, photographs, and radiographs. The authors report the surgical technique used, complications, and long-term outcome. RESULTS: Nine of 10 flaps were successful, for a flap survival rate of 90 percent. Donor bone length was 5 to 10 cm, with a mean of 6.3 cm. Skin paddles ranged from 8 to 36 cm, with a mean size of 18.7 cm. Mean operation time was 412 minutes and mean follow-up was 45.4 months (range, 12 to 94 months). Two patients underwent successful distraction osteogenesis subsequent to their free flap mandible reconstruction. All patients demonstrated stable bony union of the free flap by physical and radiographic examination. One major complication (a failed free flap) and two minor complications were observed. CONCLUSION: The free flap is safe and effective, and should be considered as a first choice in mandibular reconstruction in severe cases of hemifacial microsomia where distraction osteogenesis is not possible.

Videofluoroscopic analysis of temporomandibular joint ankylosis.

The causes of temporomandibular joint (TMJ) ankylosis, or hypomobility, are many, and it is important to understand the underlying etiology before treatment. Classically, TMJ ankylosis has been diagnosed by clinical evaluation and static imaging techniques such as plain radiographs, computed tomography, and magnetic resonance imaging. Static imaging demonstrates the size and location of the bones and soft tissues of the TMJ at a given moment; however, it fails to show the dynamic relationship of structures as the condylar head goes through its range of motion. The purpose of this study is to evaluate the use of videofluoroscopy as a dynamic means of assessing TMJ ankylosis. To do so, videofluoroscopy must be able to distinguish between bony fusion, fibrosis of the surrounding soft tissues, degeneration of the joint space, and mechanical causes of joint limitation. Six patients--2 healthy controls and 4 patients with known TMJ ankylosis--were submitted to standardized videofluoroscopic evaluation and thorough physical examination that included measurement of mandibular excursion. Videofluoroscopic data were compared with physical data. Condylar displacement was recorded in all patients, and values ranged from 0% to 100%. Videofluoroscopy allowed for the measurement of the TMJ joint space and for a detailed observation of bony and soft tissue components as they ranged in motion. In all cases, the exact cause limiting mandibular excursion was noted. Videofluoroscopy has become our preferred method of imaging the TMJ because it provides a detailed and dynamic evaluation at a reasonable cost.