Simultaneous standard total joint prosthesis reconstruction with SSRO and Le Fort I osteotomy in the treatment of unilateral temporomandibular joint ankylosis with jaw deformity: a case cohort study.

OBJECTIVE: Unilateral temporomandibular joint ankylosis with jaw deformity (UTMJAJD) may require simultaneous total joint prosthesis (TJP) reconstruction, sagittal split ramus (SSRO), and Le Fort I osteotomies. The purpose of this study was to evaluate outcomes in patients treated with these procedures. METHODS: Patients diagnosed UTMJAJD between 2016 and 2018 were selected for the study. Mandible-first procedure was performed after ankylosis release with TJP on the ankylosed side and SSRO on the contralateral side. Le Fort I osteotomy with and without genioplasty was lastly performed. Maximal incisor opening (MIO), facial symmetry, and jaw and condyle stability were compared before, after operation, and during follow-ups. RESULTS: Seven patients were included in the study. Their average chin deviation was 9.5 ± 4.2 mm, and maxillary cant was 5.1 ± 3.0°. After operation, jaw deformity significantly improved, with chin deviation corrected 7.6 ± 4.1 mm (p = 0.015) and advanced 5.9 ± 2.5 mm (p = 0.006). After an average follow-up of 26.6 ± 17.1 months, MIO significantly increased from 11.4 ± 9.3 to 35.7 ± 2.6 mm (p = 0.000). The occlusion was stable with no significant positional or rotational changes of the jaw (p > 0.05). There was no obvious condylar resorption during follow-ups. CONCLUSION: Simultaneous TJP reconstruction, SSRO, and Le Fort I osteotomy are reliable and effective methods for the treatment of UTMJAJD.

Feasibility of simultaneous TMJ arthroscopy in ADDwoR patients undergoing orthognathic surgery for jaw deformity.

This study evaluated the feasibility of simultaneous temporomandibular joint (TMJ) arthroscopy and orthognathic surgery as a new treatment strategy for anterior disc displacement without reduction (ADDwoR) patients with severe jaw deformities. Twelve ADDwoR patients with facial deformities who underwent arthroscopy and orthognathic surgery between September 2015 and December 2019 were retrospectively evaluated. Pre- and postoperative maximum incisal opening (MIO) and joint pain were recorded. Computed tomography (CT) and three-dimensional cephalometric analysis were performed at 3 (T1) and ≥6 (T2) months postoperatively. Magnetic resonance imaging (MRI) of the TMJ was performed before, ≤7 days after and ≥6 months after surgery. The lateral profile radiological findings, the symmetry of the maxilla and mandible, and the MRI measurements were compared. Anterior disc displacement did not recur, and the maximum incisal opening (MIO) increased from 27.4 mm to 32.7 mm after surgery (p < 0.05). No significant differences were found in the lateral profile, symmetry indices or condylar height via MRI between T1 and T2. Joint morphology and the position of both the maxilla and mandible remained stable during postoperative follow-up, while joint symptoms were markedly relieved and facial appearance was noticeably improved. Combined arthroscopy and orthognathic surgery is effective and recommended for ADDwoR patients with jaw deformities.

Marcus-Gunn jaw winking syndrome: case series study and management algorithm.

PURPOSE: To identify a rationale for treatment of patients with Marcus Gunn jaw winking syndrome (MGJWS). METHODS: Retrospective review of 38 consecutive patients with MGJWS referred to a single tertiary institution. Clinical data included visual acuity, ocular motility, side of jaw-wink, presence or absence of ptosis, levator function, clinical photographs, and management undertaken. Thirty-two patients were operated on with customized surgery by a senior surgeon (FQL). RESULTS: Cases with no ptosis or mild ptosis were managed conservatively. Levator advancement (LA) was successful in case of moderate ptosis and negligible synkynesis but resulted in a more evident synkinesis. Levator resection (LR) in patients with severe ptosis was associated with high rate of ptosis recurrence. Ptosis was adequately corrected in all patients submitted to uni- or bilateral levator excision (LE) and bilateral frontalis suspension (FS) or unilateral frontalis flap (FF). Jaw winking resolved in all patients submitted to LE but recurred in three cases at a later stage. Strabismus surgery was performed simultaneously in case of associated esotropia or hypotropia. CONCLUSIONS: Moderate ptosis can be corrected with LA, but success is not related to levator function and synkinesis becomes more evident postoperatively. In severe ptosis, LR showed unpredictable results. In case of severe ptosis and severe synkinesis, uni- or bilateral LE and bilateral FS are recommended; unilateral FF is an alternative in patients who refuse bilateral treatment, as the cosmetic outcome is usually better than after unilateral FS.

Machine Learning Effectively Diagnoses Mandibular Deformity Using Three-Dimensional Landmarks.

BACKGROUND: Jaw deformity diagnosis requires objective tests. Current methods, like cephalometry, have limitations. However, recent studies have shown that machine learning can diagnose jaw deformities in two dimensions. Therefore, we hypothesized that a multilayer perceptron (MLP) could accurately diagnose jaw deformities in three dimensions (3D). PURPOSE: Examine the hypothesis by focusing on anomalous mandibular position. We aimed to: (1) create a machine learning model to diagnose mandibular retrognathism and prognathism; and (2) compare its performance with traditional cephalometric methods. STUDY DESIGN, SETTING, SAMPLE: An in-silico experiment on deidentified retrospective data. The study was conducted at the Houston Methodist Research Institute and Rensselaer Polytechnic Institute. Included were patient records with jaw deformities and preoperative 3D facial models. Patients with significant jaw asymmetry were excluded. PREDICTOR VARIABLES: The tests used to diagnose mandibular anteroposterior position are: (1) SNB angle; (2) facial angle; (3) mandibular unit length (MdUL); and (4) MLP model. MAIN OUTCOME VARIABLE: The resultant diagnoses: normal, prognathic, or retrognathic. COVARIATES: None. ANALYSES: A senior surgeon labeled the patients' mandibles as prognathic, normal, or retrognathic, creating a gold standard. Scientists at Rensselaer Polytechnic Institute developed an MLP model to diagnose mandibular prognathism and retrognathism using the 3D coordinates of 50 landmarks. The performance of the MLP model was compared with three traditional cephalometric measurements: (1) SNB, (2) facial angle, and (3) MdUL. The primary metric used to assess the performance was diagnostic accuracy. McNemar's exact test tested the difference between traditional cephalometric measurement and MLP. Cohen's Kappa measured inter-rater agreement between each method and the gold standard. RESULTS: The sample included 101 patients. The diagnostic accuracy of SNB, facial angle, MdUL, and MLP were 74.3, 74.3, 75.3, and 85.2%, respectively. McNemar's test shows that our MLP performs significantly better than the SNB (P = .027), facial angle (P = .019), and MdUL (P = .031). The agreement between the traditional cephalometric measurements and the surgeon's diagnosis was fair. In contrast, the agreement between the MLP and the surgeon was moderate. CONCLUSION AND RELEVANCE: The performance of the MLP is significantly better than that of the traditional cephalometric measurements.

A Recurrent Nonsense Mutation in NECTIN4 Underlying Ectodermal Dysplasia-Syndactyly Syndrome with a Novel Phenotype in a Consanguineous Kashmiri Family.

EDSS1, a syndrome characterized by ectodermal dysplasia-syndactyly, is inherited in an autosomal recessive manner due to mutations in the NECTIN4/PVRL4 gene. Clinical manifestations of the syndrome include defective nail plate, sparse to absent scalp and body hair, spaced teeth with enamel hypoplasia, and bilateral cutaneous syndactyly in the fingers and toes. Here, we report a consanguineous family of Kashmiri origin presenting features of EDSS1. Using whole exome sequencing, we found a recurrent nonsense mutation (NM_030916: c.181C > T, p.(Gln61 ∗)) in the NECTIN4 gene. The variant segregated perfectly with the disorder within the family. The candidate variant was absent in 50 in-house exomes pertaining to other disorders from the same population. In addition to the previously reported clinical phenotype, an upper lip cleft was found in one of the affected members as a novel phenotype that is not reported by previous studies in EDSS1 patients. Therefore, the study presented here, which was conducted on the Kashmiri population, is the first to document a NECTIN4 mutation associated with the upper lip cleft as a novel phenotype. This finding broadens the molecular and phenotypic spectrum of EDSS1.

Metopic and Sagittal Craniosynostosis in Williams Syndrome.

Craniosynostosis has been previously reported in patients with Williams syndrome. Due to the associated significant cardiovascular anomalies, with an attendant increased risk of death under anaesthesia, most patients have been managed conservatively. Here we report the multidisciplinary approach in a 12-month-old female infant with Williams syndrome who has metopic and sagittal craniosynostosis. The child successfully underwent calvarial remodelling procedures, with the clinical outcome demonstrating dramatically improved global development after surgery.

Digital template-guided genioplasty for patients with jaw deformity resulting from temporomandibular joint ankylosis: A comparison between single- and double-layer genioplasty.

The aim of this study was to compare single- and double-layer digital template-assisted genioplasty for the correction of jaw deformity resulting from temporomandibular joint ankylosis (TMJA). Thirteen patients with jaw deformity resulting from TMJA who underwent lateral arthroplasty, costochondral graft, or total joint replacement combined with single- or double-layer digital template-assisted genioplasty were included. Computed tomography data were obtained for the preoperative design. Digital templates were designed and manufactured using three-dimensional printing to assist with the chin osteotomy and repositioning in single- or double-layer genioplasty. Of the 13 patients included, seven underwent single-layer genioplasty and six underwent double-layer genioplasty. The digital templates precisely reflected the osteotomy planes and repositioning of the chin segments intraoperatively. The radiographic evaluation showed that the patients who underwent double-layer genioplasty exhibited more chin advancement (11.95 ± 0.92 mm vs 7.50 ± 0.89 mm; P < 0.001) with a slightly larger mean surface error (1.19 ± 0.14 mm vs 0.75 ± 0.15 mm; P < 0.001) than those who underwent single-layer genioplasty. This indicates that double-layer genioplasty better promoted chin advancement and improved the facial shape, but was accompanied by more surgical error compared with the preoperative design. Furthermore, hardly any nerve damage was observed. Digital templates are useful for assisting in surgical procedures.

Application of Computer-Assisted Preoperative Planning in Treating Congenital Bilateral Zygomatico-Maxillo-Mandibular Fusion: A Case Report and Literature Review.

Congenital fusion of the jaws (syngnathia) is a rare facial malformation with an unknown etiology. This disease may vary in severity with adhesion of soft tissue and bony fusion. It can be anterior fusion, unilateral or bilateral fusion, and complete fusion. The main problem of these patients is the difficulty of airway maintenance and feeding, and the most common postoperative complication is the relapse of bony fusion. Here, we report a young male patient with bony syngnathia, involving bilateral fusion of the ascending ramus and body of the mandible with the maxillary complex. We performed bone isolation by computer-assisted preoperative planning and used an insertional temporalis flap to fix the wound surface to prevent refusion of bone.

The Esthetic Perception of Morphological Severity in Scaphocephalic Patients is Correlated With Specific Head Geometrical Features.

OBJECTIVE: To investigate the relationship between perception of craniofacial deformity, geometric head features, and 3D head shape analyzed by statistical shape modeling (SSM). PATIENTS: A total of 18 unoperated patients with scaphocephaly (age = 5.2 ± 1.1m)-6 were followed-up after spring-assisted cranioplasty (SAC) (age = 9.6 ± 1.5m)-and 6 controls (age = 6.7 ± 2.5m). MAIN OUTCOME MEASURES: 3D head shapes were retrieved from 3D scans or computed tomography (CTs). Various geometrical features were measured: anterior and posterior prominence, take-off angle, average anterior and posterior lateral and horizontal curvatures, cranial index (CI) (cranial width over length), and turricephaly index (TI) (cranial height over length). SSM and principal component analysis (PCA) described shape variability. All models were 3D printed; the perception of deformity was blindly scored by 9 surgeons and 1 radiologist in terms of frontal bossing (FB), occipital bulleting (OB), biparietal narrowing (BN), low posterior vertex (LPV), and overall head shape (OHS). RESULTS: A moderate correlation was found between FB and anterior prominence (r = 0.56, P < .01) and take-off angle (r = - 0.57, P < .01). OB correlated with average posterior lateral curvature (r = 0.43, P < 0.01) similarly to BPN (r = 0.55, P < .01) and LPV (r = 0.43, P < .01). OHS showed strong correlation with CI (r = - 0.68, P < .01) and TI (r = 0.63, P< .01). SSM Mode 1 correlated with OHS (r = 0.66, p < .01) while Mode 3 correlated with FB (r = - 0.58, P < .01). CONCLUSIONS: Esthetic cranial appearance in craniofacial patients is correlated to specific geometric parameters and could be estimated using automated methods such as SSM.

Delayed Presentation of Unicoronal Craniosynostosis-Masked by Ipsilateral Posterior Deformational Plagiocephaly.

Despite a greater awareness of the differential diagnosis of head shape abnormalities among pediatricians, the effect of deformational forces on calvarial morphology can complicate the diagnosis of craniosynostosis. In this report, we describe 2 patients diagnosed with unicoronal craniosynostosis (UCS) in a delayed fashion due to the presence of concomitant posterior deformational plagiocephaly (PDP). In both cases, the severity of each patients' PDP obscured changes typically associated with UCS. This unique presentation underscores the importance of having a high index of suspicion for possible premature suture fusion despite the presence of concomitant PDP.

Rare variant of dens invaginatus with accessory root and labial talon cusp in maxillary lateral incisor-Case report.

Dens invaginatus (DI) often causes dysmorphic changes in both the crown and root. This case report presents a complicated type 3a DI in a maxillary lateral incisor with labial talon cusp and root bifurcation. Although lingual talon cusp is common in maxillary lateral incisor, labial talon cusp is rare. An auxiliary palatal root in maxillary lateral incisor is also unusual. No such case involving all three variations has been described in the literature. The DI was classified as type B4 according to Schulze and Brand, as it involved division of pulp and root. DI was managed by orthograde MTA, radisectomy and periodontal regeneration was done for the palatal root. Labial talon associated with DI and can lead to early periodontal/pulpal involvement. Type 3 DI can affect the root with marked dilatation and division. Additional palatal root should be carefully detected in type 3a DI and managed with the aid of CBCT.

Early Surgical Intervention of Congenital Syngnathia.

Congenital syngnathia is a rarely reported malformation when there is a fusion between the maxilla and the mandible. It is necessary to modify it in childhood because congenital syngnathia causes incongruity in pronunciation, diet, and esthetics during the growth process. In this case report, 1 case of syngnathia, a rare craniofacial anomaly, is presented with a review of reports. Prompt diagnosis and surgery were performed right after birth for the present case. A partial limitation point was resolved for further growth. Herein, the authors present the case of a female infant (7 d after birth) diagnosed with congenital syngnathia and treated by early surgical intervention.

The role of anterior segmental osteotomies in orthognathic surgery for protrusive faces in a Southeast Asian population: 10-year retrospective data of 51 patients treated in a single centre.

Bimaxillary protrusion is a dentofacial deformity common in the East Asian population. It is often difficult to decide between orthodontic and surgical retraction to treat patients with bimaxillary protrusion, especially if surgery to correct skeletal base discrepancies is also required. The objective of this study was to investigate the treatment outcomes of surgical versus orthodontic retraction in patients with bimaxillary protrusion undergoing orthognathic surgery. A retrospective cohort study was conducted, where the medical records and radiographs of 51 patients with bimaxillary protrusion who had an anterior segmental osteotomy concomitant with orthognathic surgery were examined. Patients who had bimaxillary anterior segmental osteotomies were more likely to achieve an inter-incisal angle of 135 ± 5° at the end of treatment, when compared to patients who had orthodontic retraction (P < 0.01). The total treatment duration of patients who had bimaxillary anterior segmental osteotomies was significantly shorter as compared to that of the patients who only had these osteotomies in the maxilla (P < 0.001) or mandible (P = 0.012). Complications and permanent long-term sequelae following anterior segmental osteotomies were uncommon. It is concluded that bimaxillary anterior segmental osteotomies are a viable alternative for patients with concomitant bimaxillary protrusion and skeletal base discrepancies. This allows for a significantly shorter treatment time and greater correction of inter-incisal angles, without a significant increase in complications.

Congenital mandibular coronoid process hyperplasia and associated diseases.

Coronoid process hyperplasia (CPH) is an oral and maxillofacial surgical disease that can result in restricted jaw movement due to an enlarged and elongated mandibular coronoid process. It is characterized by the painless progressive restriction of unilaterally or bilaterally mouth opening. Clinically, unexplained bilateral CPH is less common and therefore often overlooked or misdiagnosed, and coronoidectomy can be very effective on improving mouth opening. Currently, the exact etiology and mechanism of congenital CPH have not yet been fully understood, but it is generally believed to be genetically related. In this paper, the relationship of the congenital mandibular CPH with the related diseases was examined based on cases collected in our clinic and literature review for the clinical diagnosis and treatment of patients with restricted mouth opening associated with CPH.

Free Fibula Flap for the Treatment of Agnathia in a 10-Year-Old With Severe Agnathia-Otocephaly Complex.

Agnathia-otocephaly complex (AOC), a first branchial arch defect, is characterized by mandibular hypoplasia or aplasia, ear abnormalities, microstomia, and macroglossia and is a rare and often fatal diagnosis. Herein, the technical considerations and details of mandibular reconstruction using virtual surgical planning (VSP) and a vascularized free fibula flap for further mandibular reconstruction in a 10-year-old boy are presented. The patient's preoperative examination was consistent with agnathia (absence of mandibular symphysis, bilateral mandibular bodies, condyles, coronoids, rami, and temporomandibular joint), severe microstomia, and a Tessier # 30 cleft (maintained to allow oral access until later in treatment). Virtual surgical planning was utilized to plan a 3-segment fibula for the reconstruction of the mandibular symphysis and bilateral body segments, and bilateral costochondral grafts were planned for the rami. To the authors' knowledge, this represents the first application of virtual surgical planning for mandibular reconstruction with a vascularized free fibula flap in a pediatric patient with severe agnathia-otocephaly complex.

Crocodile tear syndrome treated with lacrimal gland incobotulinum toxin A injection: a report of two cases.

Crocodile tear syndrome (CTS) is a late complication of facial nerve palsy characterized by unilateral lacrimation in response to gustatory stimulation. We present 2 cases of patients diagnosed with CTS after recovering from unilateral idiopathic facial nerve palsy. Both patients underwent transconjunctival lacrimal gland incobotulinumtoxinA injection, with doses of 5-16 units. The patients were seen in clinic for post-treatment follow-up at 2 weeks, 3 months, and 6 months. Outcomes were measured by treatment efficacy and adverse drug effects. Following treatment, both patients reported resolution of gustatory lacrimation. The patient treated with 16 U experienced transient ptosis and diplopia following injection, whereas the patient treated with 5-7.5 U experienced no adverse effects.

Condiciones de aplicación del injerto ilíaco óseo en la sustitución de defectos mandibulares: un estudio retrospectivo de 11 años de experiencia / Conditions of iliac bone grafts application in mandibular defects replacement: a retrospective study of 11-years’ experience

Antecedentes: Este estudio tenía como objetivo aclarar la aparición y las causas de complicaciones postoperatorias en pacientes con defectos mandibulares continuos, reconstruidos con injertos óseos ilíacos libres. Pacientes y métodos: Los pacientes con defecto de continuidad mandibular con pérdida de tejido óseo, que dio lugar a una brecha de 2 cm o más, se sometieron a reconstrucción con injerto óseo de cresta ilíaca no vascularizado. La variable de resultado fue el fracaso del injerto, que se definió como su pérdida por infección postoperatoria, exposición del injerto, reabsorción completa o no unión del injerto. Los factores predictivos fueron el momento de la reconstrucción, la longitud del defecto óseo, la presencia preoperatoria de defecto mucoso y/o dehiscencia y el método de fijación. Todas las variables explicativas y predictoras se analizaron mediante regresión logística binomial univariante.Resultados: El estudio incluyó los resultados de 50 reconstrucciones mandibulares con injerto óseo de cresta ilíaca no vascularizado que se observaron retrospectivamente. El seguimiento de los pacientes incluidos osciló entre 6 meses y 11 años. 34 pacientes (68 %) tuvieron resultados quirúrgicos satisfactorios. En 16 pacientes (32 %) se perdieron los injertos óseos. Todos ellos debido al desarrollo de infección, supuración y/o exposición del injerto durante los primeros 6 meses del periodo de observación. En el análisis univariante, las principales variables predictoras de fracaso fueron el tabaquismo (OR 5,8; IC: 1,48-22,7; p = 0,002), el momento de la reconstrucción (OR 7,94; IC: 1,88-33,5; p = 0,004) y el defecto o dehiscencia de la mucosa (OR 8,49; IC: 2,21-32,6; p = 0,002). El análisis multivariante también reveló la afectación sinfisaria del defecto (OR 5,63; IC: 1,14-27,8; p = 0,034) como factor predictivo significativo de fracaso en un caso de reconstrucción inmediata. ... (AU)

Background: This study aimed to clarify the occurrence and causes of postoperative complications in patients with continuous mandibular defects, reconstructed with free iliac bone grafts.Patients and methods: Patients with mandibular continuity defect with bone tissue loss, resulting in a 2 cm gap or more underwent reconstruction with non-vascularized iliac crest bone graft. The outcome variable was graft failure which was defined as its loss due to the postoperative infection, graft exposure, full resorption or non-unition of the graft. The predictors were timing of reconstruction, length of the bone defect, preoperative presence of mucosa defect and/or dehiscence and fixation method. All explanatory and predictor variables were analyzed with univariate binomial logistic regression.Results: The study included the results of 50 mandibular reconstructions with non-vascularized iliac crest bone graft which were observed retrospectively. The follow-up of included patients ranged from 6 months to 11 years. 34 patients (68 %) had successful surgery results. In 16 patients (32 %) the bone grafts were lost. All of them due to the infection development, suppuration and/or graft exposure during the first 6-months of the observation period. In univariate analysis, the main failure predictor variables were smoking (OR 5.8, CI 1.48-22.7, p = 0.002), timing of reconstruction (OR 7.94, CI 1.88-33.5, p = 0.004) and mucosa defect or dehiscence (OR 8.49, CI 2.21-32.6, p = 0.002). The multivariate analysis also revealed symphyseal involvement of defect (OR 5.63, CI 1.14-27.8, p = 0.034) as the significant failure predictor in a case of immediate reconstruction. The length of defect and fixators type remained statistically non-significant for mandibular reconstruction with NVICG (p < 0.05).Conclusion: ... (AU)

Estimation of the predictive effect of body mass index-percentile on skeletal maturation in a Nigerian population

To determine the predicting the effect of BMI-index percentile on the skeletal maturation of Nigerian children. Design: A prospective cross-sectional study. Setting: This study was conducted in a tertiary health care facility in North-Central Nigeria. Participants: Children between the ages of 5-17 years consisting of 44 males and 30 females that presented in the Child Dental clinic over a period of eight months were recruited for the study. Main outcome measured: Skeletal maturation was assessed using the middle phalanx of the third finger (MP3) while the standard WHO growth chart specific for age (2-20 years) and gender was used for grading BMI-percentile. The unpaired t-test was used to compare mean chronological age of the stages of MP3 according to gender. Multinomial logistic regression used to determine the predictive effect of age, gender and BMI percentile on pubertal growth spurt. Results: The BMI-percentile had weak correlation with the pubertal growth spurt (r=0.089, p=0.448). Gender (p=0.004) and chronological age had significant (p<0.001) predictive effect on the skeletal maturation. A one-percentile increase in the BMI-percentile decreases the likelihood of healthy children to be in the peak-pubertal by 1.504 when compared to obese children (p=0.305). Conclusions: This study showed that BMI-percentile is a weak predictor of skeletal maturation. However, obese children had a tendency towards advanced skeletal maturation than healthy participants. It is therefore suggested that orthodontists should consider early implementation of jaw modification treatments among obese children.

Cleft palate-lateral synechiae syndrome: a case report.

Cleft palate-lateral synechiae syndrome (CPLSS) is an extremely rare congenital malformation syndrome with undetermined etiology, characterized by a cleft palate and lateral intraoral synechiae linking the free borders of the palate to the mouth floor. We report a case of a female neonate, admitted for suckling difficulties with a cleft lip and palate associated to multiple lateral intraoral synechiae. Resection of the synechiae allowed oral feeding. Cleft palate-lateral synechiae syndrome is an exceptional syndrome as only seventeen cases have been reported in the literature. Synechiae can be isolated or more frequently in association with other congenital anomalies such as cleft lip and/or palate. These synechiae can cause functional deficits, especially in the respiratory and feeding tracts, language disorders or recurrent otitis. Although it is exceptional, this malformative entity must be known by medical practitioners in order to set up a well-adapted therapeutic protocol.