Jaw Thrust: A Simple Predictor of Success in Mandibular Distraction Osteogenesis.

BACKGROUND: The hypoplastic mandible in the congenital condition Pierre Robin sequence (PRS) displaces the base of the tongue posteriorly, which results in upper airway obstruction (UAO) that can potentially be corrected with mandibular distraction osteogenesis (MDO). Jaw thrust (JT) is routinely performed during evaluation of the airway; similar to MDO, it projects the mandible and tongue anteriorly to open the airway. The authors demonstrate that JT can be used as a criterion to predict successful MDO outcomes in infants with PRS. METHODS: The study was a single-center, retrospective chart review of infants diagnosed with PRS between 2016 and 2023. Data regarding their demographics, comorbid diagnoses, JT success, airway anomalies, laryngeal grade of view, apnea-hypopnea index, and perioperative course were statistically analyzed. RESULTS: Of the 16 patients included in the study, 11 had successful relief of their airway obstruction with JT and proceeded with MDO. The unsuccessful JT group had significantly greater proportions of females, birth prematurity, gastrostomies, tracheostomies, and longer hospital stays. In the successful JT group, both the mean laryngeal grade of view (P=0.029) and mean apnea-hypopnea index (P=0.025) improved significantly post-MDO. Post-MDO tracheostomy was also avoided in all but 1 patient who was not previously tracheostomized. CONCLUSIONS: There is no widely accepted algorithm to guide craniofacial surgeons on the optimal intervention for relieving UAO in infants with PRS. In our institutional experience, patients whose preoperative JT relieved UAO also successfully relieved UAO with MDO. In patients with PRS, JT may be a useful criterion for selecting appropriate candidates for MDO.

The Role of Digital Surgical Planning and Surgical Guides in the Treatment of Unilateral Condylar Hyperplasia.

Unilateral condylar hyperplasia (UCH) results in facial asymmetry, malocclusion, and temporomandibular joint dysfunction. Treatment consists of both surgical and orthodontic intervention. A review was performed for 4 patients with UCH who underwent digital surgical planning (DSP)-assisted condylectomy. All patients were female, aged 14 to 35 years at the time of operation with facial asymmetry and class III malocclusion. None of the patients had prior treatment and all had perioperative orthodontic appliances to provide fixation and postoperative elastic therapy. All patients underwent DSP-guided condylectomy, and intraoperative surgical cutting guides were used for 3 of the patients. All had significant improvement in facial symmetry and occlusion. None had recurrence, and additional intervention has not been required. If UCH is recognized before marked secondary changes in the maxilla, mandible, and occlusion, future orthognathic surgery may be potentially obviated. Craniomaxillofacial surgeons should consider using DSP and surgical guides in the treatment of UCH.

Crouzon Syndrome Spanning Three Generations: Advances in the Treatment of Syndromic Midface Deficiency.

Background: Crouzon syndrome is an autosomal dominant genetic disorder characterized by craniosynostosis, midface retrusion, and exophthalmos. Over the past century, the treatment of craniofacial disorders like Crouzon syndrome has evolved significantly. Methods: An institutional review board-approved retrospective study was conducted to ascertain the treatment of three individuals with Crouzon syndrome from one family, complemented with a series of literature searches to examine the evolution of craniofacial surgical history. Results: Dr. David Williams Cheever developed the Le Fort I level to correct malocclusion, maxillomandibular malformations, and midface hypoplasia. Later, Dr. Paul Tessier introduced the Le Fort II and III osteotomies to treat syndromic midface hypoplasia. In 1978, Dr. Fernando Ortiz-Monasterio and Dr. Antonio Fuente del Campo published the first series of monobloc osteotomies, allowing for simultaneous correction of supraorbital and midface malformations, although complicated by blood loss and high infection rates. In 1992, McCarthy et al introduced the concept of gradual distraction to the craniofacial skeleton. In 1995, Polley et al performed the first monobloc advancement using external distraction. Subsequently, in 1997, Polley and Figueroa introduced a rigid external distraction device with multiple vector control to manage severe cleft maxillary hypoplasia. The technique was further refined and applied to treat syndromic midface hypoplasia, reducing complication rates. Currently, either external or internal distraction approaches are used to safely treat this challenging group of patients. Conclusion: The treatment of syndromic midface deficiency has significantly evolved over the past 50 years, as evidenced by this report of three generations of Crouzon syndrome.

Tracheostomy, the Not So Definitive Airway?: Tracheostomy Morbidity in Pediatric Craniofacial Patients.

BACKGROUND: Tracheostomy is the definitive treatment for airway management in severe cases of craniofacial-associated upper airway obstruction, like the Pierre-Robin sequence, but is associated with significant morbidity. The purpose of this study was to examine tracheostomy-associated morbidities and mortalities in craniofacial patients to identify opportunities to improve clinical care and patient prognosis. METHODS: The study was a retrospective review of pediatric craniofacial patients who were tracheostomized between 2016 and 2022. Data regarding their demographics, craniofacial diagnoses, endoscopic airway anomalies, intubation grade of view classification, tracheostomy-related complications, and causes of mortality were analyzed. RESULTS: Sixteen of the 17 tracheostomized pediatric patients had the Pierre-Robin sequence, with 5 of those patients having an additional syndromic craniofacial diagnosis. Additional airway anomalies were found in 82.4% of the patients. The mean length of hospital stay after tracheostomy was 4.08 months. Infection was the most common complication, observed in 94.1% of patients, followed by stomal granulation in 76.5% of patients. Two mortalities were observed: one following the compassionate removal of ventilator support and the other following the accidental dislodgment of the tracheostomy tube. CONCLUSIONS: Tracheostomy-related complications were observed in all craniofacial patients in this group. Compared with the general pediatric population, tracheostomized craniofacial patients may endure longer hospital stays and greater stomal granulation rates. Mandibular distraction osteogenesis may allow for tracheostomy avoidance in these patients, and future research should focus on comparing the long-term complication rates and outcomes between tracheostomy mandibular distraction osteogenesis in this challenging patient population.

TMJ Ankylosis Following Mandibular Distraction Osteogenesis: Management With Simultaneous Midface External Distraction and Bilateral Temporomandibular Joint Replacement.

Early mandibular distraction osteogenesis (MDO) can decrease upper airway and feeding complications in pediatric patients with micrognathia; however, temporomandibular joint (TMJ) complications like TMJ ankylosis (TMJA) may occur. TMJA can disturb pediatric patients' function and craniofacial growth, resulting in significant physical and psychosocial consequences. Additional surgical procedures may also be required, increasing the burden of care on patients and their families. CMF surgeons must discuss the potential complications of early MDO surgery with families as well as potential solutions should these problems occur. This report presents the case of a 17-year-old male with a severe craniofacial anomaly with features of Treacher-Collins syndrome (TCS) and a surgical history of tracheostomy, cleft palate repair, mandibular reconstruction with harvested costochondral grafts, and MDO with resultant bilateral TMJA and limited mouth opening. The patient Was treated with bilateral custom alloplastic TMJ replacements and simultaneous maxillary DO using a Rigid External Distraction (RED) device.

Uvular Dermoid Cyst: More Than Just a Lump in the Throat-Management of Resultant Velopharyngeal Insufficiency.

To seal the passage between the nasal and oral cavities during speech and swallowing, velopharyngeal closure is required. However, in velopharyngeal dysfunction, uncoupling of the nasal and oral cavities can be impaired, resulting in hypernasality, nasal air emission, and decreased vocal intensity. Velopharyngeal dysfunction can develop following velopharyngeal mislearning, oral surgery, or a congenital palatal malformation. Rare dermoid cysts of the palate may interrupt normal palatal development, resulting in velopharyngeal insufficiency (VPI). While speech therapy is the standard treatment, some cases may necessitate surgical correction of structural insufficiencies. In this report, we present the case of a 7-year-old female with a past surgical history of a uvular dermoid cyst removal at 14 months of age with VPI that was treated with Furlow Z-palatoplasty. To the author's knowledge, this is one of but a few cases of a uvular dermoid cyst with VPI.

Long-Term Outcomes After External Maxillary Distraction Surgery in Patients With Down Syndrome.

BACKGROUND: Patients with Down syndrome have severe facial deformities that can precipitate functional consequences and social stigmatization. Craniofacial surgical intervention can play a role in improving these symptoms and patient quality of life. The objective of this study was to investigate the long-term outcomes of distraction osteogenesis and orthognathic surgical intervention in patients with Down syndrome. MATERIALS AND METHODS: Charts of 3 patients with Down syndrome who were treated with external maxillary distraction osteogenesis were retrospectively reviewed. The patients' caregivers were prospectively interviewed between 10 and 15 years after surgery to determine surgical stability, long-term function, and quality of life status. RESULTS: All patients and their caregivers reported excellent results with improvements in function and quality of life. Facial skeletal changes have been stable over time. The cephalometric analysis demonstrated significant maxillary advancement in all 3 patients and mandibular changes to correct mandibular prognathism and asymmetry in the patient who underwent finishing orthognathic surgery. CONCLUSIONS: External maxillary distraction osteogenesis and orthognathic surgery may be considered in select patients with Down syndrome as part of their multidisciplinary health care. These interventions can result in long-term improvements in patient function and quality of life.

Spontaneous Closure of Congenital Cranial Defect: Is Early Surgical Intervention Warranted?

Infantile cranial development typically occurs in a predictable sequence of events; however, less is known about how the development occurs in isolated, nonsyndromic congenital craniofacial anomalies. Furthermore, the timing of pediatric cranioplasty has been extrapolated from adult studies. Thus, the management of nonsyndromic congenital craniofacial anomalies presents with unique challenges to the craniofacial surgeon. The authors describe the case of a baby girl who was born with right Tessier 3 cleft, cleft palate, anophthalmos, and severe left craniofacial microsomia with Pruzansky grade III left mandibular anomaly. By analyzing 3-dimensional chronological models of the patient, the authors found that her abnormal fontanelle initially increased in size until 22 weeks of age, with subsequent spontaneous closure at a rate of 60.53 mm2/y. Although similar cranial anomalies are typically surgically corrected early in life, delaying treatment until after 2 years of age may be appropriate in some patients, obviating surgical morbidity in the newborn period.

Is There a Role for Comfort Care in Neonates With Severe Craniofacial Anomalies? Case Report and Review of Quality-of-Life Literature.

This article aims to determine how quality of life (QoL) is defined and assessed in cases of severe craniofacial anomalies, as well as the impact such considerations may have on the treatment of a neonate with these conditions with respect to palliative neonatal care. Our literature review found insufficient evidence to suggest that craniofacial anomalies result in consistently poor QoL. Based on these findings and in line with the current acceptable standards for the ethical care of neonates, with the exception of rare cases, resuscitative efforts should always be performed on patients with isolated craniofacial anomalies, as demonstrated in the management of this reported patient.

Using Active-Phonation Cone-Beam Computed Tomography to Evaluate Pediatric Patients With Suspected Velopharyngeal Dysfunction, a Pilot Study.

ABSTRACT: Velopharyngeal dysfunction (VPD) is described as the incomplete closure of the velopharyngeal port during a speech production. Nasopharyngoscopy and/or multiplanar videofluoroscopy have been utilized for decades to assess the degree and nature of the dysfunction. Cone-beam computed tomography (CBCT) is presented as an additional diagnostic tool, allowing for clear visualization of the affected structures and the ability to obtain accurate measurements (within 100 microns) of the involved anatomy and defect. This prospective pilot study aims to test the feasibility of using "active-phonation" CBCT to assess suspected VPD in the pediatric and young adult populations and compare the results to nasopharyngoscopy; the current standards of care.Six patients, ages 6 to 26 years, with suspected VPD, defined as the inability to completely close off the nasal airway during an oral speech, seen at an urban medical outpatient craniofacial care center, served as subjects for this pilot study. Each patient received a comprehensive speech evaluation and participated in both active-phonation CBCT and nasopharyngoscopy.Both active-phonation CBCT and nasopharyngoscopy revealed incomplete closure of the velopharyngeal port during a speech in all 6 patients (100%). Two patients (33%) were unable to tolerate a complete nasendoscopic examination. There was no difference between CBCT or nasopharyngoscopy in determining the presence of VPD and noting the severity on a 3-point scale, (P = 0.61) as judged by 4 experienced clinicians.As a functional imaging modality, active-phonation CBCT is a useful adjunct tool for accurate diagnosis of VPD and may be more easily tolerated during a thorough VPD assessment than nasopharyngoscopy. It also provides quantitative data that is useful to augment treatment optimization and surgical planning in this population. Further studies are needed to validate these results.

Alloplastic Temporomandibular Joint Reconstruction Following Recurrent Ameloblastoma Resection.

ABSTRACT: Ameloblastomas are benign tumors that most commonly affecting the mandible. The current standard of treatment for ameloblastomas is resection followed by reconstruction that has historically been accomplished through the use of a microsurgical vascularized flaps taken from the iliac crest or fibula. Alloplastic reconstruction methods have gained popularity over recent years with success reported in the reconstruction of many pathologies, including ankylosis, condylar fracture, neoplasia involving extensive resection, severe inflammatory/degenerative temporomandibular joint (TMJ) disease, and congenital TMJ abnormalities. The authors present a patient who successfully underwent ameloblastoma resection and TMJ reconstruction with a custom TMJ Concepts alloplastic implant. The authors also present a review of the literature on alloplastic TMJ reconstruction following ameloblastoma resection. To our knowledge, this is the second report in the literature on the use of a TMJ Concepts implant after ameloblastoma resection.

Intra-Lesional Injection of Triamcinolone to Palatoplasty Scar to Aid Reversal of Transverse Maxillary Relapse after Orthognathic Surgery.

ABSTRACT: A 32-year-old female with a repaired right unilateral cleft lip and palate underwent several surgical and orthodontic procedures during the rehabilitation process of her condition. Nine years after this extensive treatment she underwent transverse relapse of her maxilla and requested a consultation for its correction as she felt her speech and chewing were negatively affected. She presented with a transverse maxillary arch collapse on the cleft side with significant palatal scarring secondary to multiple palate procedures. A course of maxillary expansion and dental alignment with fixed orthodontic appliances was carried out. in addition, she had 2 triamcinolone injections 7 months apart while undergoing orthodontic treatment and one 10 months after completion to soften the scarred palatal tissues. The maxillary arch was successfully expanded and aligned. She was retained with a removable chrome cobalt palatal frame to be used full-time and assure stability of the correction. She has been followed for 4 years with no clinical evidence of relapse. Triamcinolone injection into significant palatal scarring in cleft palate patients with a decreased transverse maxillary dimension can be considered an adjunct procedure in conjunction with orthodontic treatment.

Cleft Lip and Palate: LeFort I Distraction with Halo and Hybrid Internal Maxillary Distractors.

Distraction osteogenesis is a viable treatment option for patients with a cleft associated with severe maxillary retrusion. A rigid external distraction device and a hybrid internal maxillary distractor have been used to advance the maxilla allowing for predictable and stable results. These techniques can be applied by itself or as an adjunct to traditional orthognathic procedures. The technical aspects are presented. These procedures tend to be simpler and demonstrate great stability compared to traditional surgical methods. The reasons for stability are discussed.

Custom Alloplastic Temporomandibular Joint Reconstruction: Expanding Reconstructive Horizons.

Custom alloplastic temporomandibular joint (TMJ) reconstruction has been well established for the management of end-stage TMJ disease. However, its use in congenital TMJ deformities is limited. Here, the authors present initial outcomes of skeletally mature patients who underwent custom alloplastic TMJ reconstruction and simultaneous orthognathic surgery.A retrospective case series of patients who underwent custom alloplastic TMJ reconstruction concurrent with orthognathic surgery between 2014 and 2019 was completed. Functional, aesthetic and orthodontic outcomes as well as complications were recorded.Seven TMJs in 5 skeletally mature patients (4 female, 1 male, ages 16-30) (2 bilateral, 3 unilateral) were replaced. All but 1 patient had previous attempts at reconstructive surgery with poor results. All cases were prepared using virtual surgical planning and underwent concomitant maxillomandibular orthognathic surgery. All patients demonstrated improved post-operative occlusions. Four of 5 patients achieved >30 millimeters of post-operative MIO. Complications included ear canal perforation and facial nerve dysfunction.There were no infections or other implant-related complications. Mean follow up was 2 years and 15 days. Alloplastic TMJ reconstruction at the time of skeletal maturity for patients with congenital mandibular TMJ defects is an alternative to existing management options. Further long-term prospective outcomes studies are ongoing.

Optimizing the management of cutaneous melanoma in the elderly.

BACKGROUND: The incidence of melanoma in patients aged ≥ 65 years is increasing. Melanoma characteristics appear to be different in the elderly, and outcomes worse. We undertook this study to characterize our experience with melanoma in the elderly and identify factors associated with outcome. METHODS: We studied 244 elderly consecutive melanoma patients with clinically localized disease. Mean follow-up was 73 ± 3.7 months. RESULTS: One-hundred thirty-two patients (54%) were male. The most common site was extremity (44%), histology superficial spreading (51%), mean thickness 1.91 mm, 16% ulcerated. T stage included 36% T1, 14% T2, 14% T3, 9% T4 tumors. Undertreatment of the primary tumor occurred in 22%, overtreatment in 6%, and inadequate lymph node staging/treatment in 22%. 23% of patients recurred, 21% died of unrelated causes, and 12% died of melanoma. Sex, tumor thickness, mitotic index, ulceration, and lymph node status were significant factors affecting disease-free survival, while tumor thickness, mitotic index, and lymph node status were significant predictors of overall survival. CONCLUSION: The features of melanoma in elderly patients were different from younger patients, but prognostic factors were similar. Most patients received appropriate treatment and survived >5 years. Strategies to improve early detection to facilitate optimal treatment of melanoma in the elderly are warranted.

Primary prevention of overweight and obesity: an analysis of national survey data.

BACKGROUND: Obesity is rapidly approaching tobacco as the leading cause of preventable morbidity and mortality. Health care providers have the opportunity to address this through primary prevention strategies. OBJECTIVE: To assess whether health care professionals provide primary prevention for overweight and obesity by examining the percentage of healthy-weight (body mass index [BMI] = 18.5-24.9 kg/m(2)) individuals who report being advised to maintain a healthy weight. DESIGN: Cross-sectional analysis of the 2003 Behavioral Risk Factor Surveillance Survey data. PARTICIPANTS: Noninstitutionalized U.S. adults >18 years of age. RESULTS: Among healthy BMI respondents, only 2.6% reported receiving primary prevention. Logistic regression analyses yielded that healthy-weight adults receiving primary prevention were more likely to report: being 18-49 years of age, annual household incomes <$35,000, having at least 1 comorbidity, having a health care provider, changed eating habits to include less fat or fewer calories, and using physical activity to maintain or lose weight. Men were also more likely to receive primary prevention. CONCLUSIONS: Only a very small proportion of healthy-weight adults received primary prevention, which suggests that physicians are missing opportunities to help address the epidemic of adult obesity in the US.