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1.
Clin Oral Investig ; 28(9): 502, 2024 Aug 28.
Article in English | MEDLINE | ID: mdl-39196436

ABSTRACT

OBJECTIVES: To compare the facial asymmetry after bimaxillary surgery between mild craniofacial microsomia (CFM) and non-syndromic class II asymmetry. MATERIALS AND METHODS: Cone-beam computed tomography scans of adults with Pruzansky-Kaban types I and IIA CFM (CFM groups, n = 20), non-syndromic skeletal class II asymmetry (Class II group, n = 20), and normal controls (control group, n = 20) were compared. The area asymmetry of lower face and jaw bones was quantified. Landmark-based method was used to evaluate the lower facial asymmetry regarding midline, cants, and contour. RESULTS: There were no significant postoperative differences in the hemi-facial and hemi-jaw area asymmetry between CFM and Class II groups, both of which were significantly larger than the control group. No significant difference was found in the midline deviation and lip and occlusal cants between CFM and Class II groups. The vertical contour asymmetry in CFM group became significantly larger than Class II group. Compared to the control group, the deviation of pronasale, subnasale, and soft-tissue menton, lip and occlusal cants, and sagittal and vertical contour asymmetry in CFM group were significantly larger, as were the deviation of subnasale and soft-tissue menton and vertical contour asymmetry in Class II group. CONCLUSIONS: The vertical contour asymmetry of mild CFM was significantly larger than non-CFM class II after surgery, while the area asymmetry, midline deviation, cants, and sagittal contour asymmetry of lower face showed no significant difference. CLINICAL RELEVANCE: Be aware that correcting vertical asymmetry of contour, lip, and dentition in CFM is still challenging.


Subject(s)
Cone-Beam Computed Tomography , Facial Asymmetry , Goldenhar Syndrome , Malocclusion, Angle Class II , Orthognathic Surgical Procedures , Humans , Facial Asymmetry/surgery , Facial Asymmetry/diagnostic imaging , Male , Orthognathic Surgical Procedures/methods , Female , Malocclusion, Angle Class II/surgery , Malocclusion, Angle Class II/diagnostic imaging , Adult , Goldenhar Syndrome/diagnostic imaging , Goldenhar Syndrome/surgery , Treatment Outcome , Anatomic Landmarks , Adolescent , Case-Control Studies
2.
Cleft Palate Craniofac J ; 61(4): 584-591, 2024 Apr.
Article in English | MEDLINE | ID: mdl-36628544

ABSTRACT

OBJECTIVE: To characterise the morphology of temporal bone in patients with craniofacial microsomia (CFM). DESIGN: A retrospective study. SETTING: A craniofacial centre. PATIENTS: Ninety-four patients with unilateral craniofacial microsomia. INTERVENTIONS: Mimics 21.0 (Materialise Inc., Belgium) was used to locate temporal bone landmarks on preoperative computed tomography data. The spatial Cartesian coordinate system was established in 3-matic 13.0 (Materialise Inc., Belgium). The coordinates of each landmark and the distances and angles between the landmarks were calculated. A classification system was used to quantify the severity of the zygomatic arch deformity. MAIN OUTCOME MEASURE(S): The bilateral differences in coordinates, linear and angular measurements, and the severity of temporal bone deformity (TTL δ, Psag δ, Paxiδ, and Tsag δ) among the groups were compared using the paired t-test and Kruskal-Wallis test, respectively. RESULTS: Compared to those of the unaffected side, the landmarks of the inner ear and petrous part on the affected side showed a decrease in the Z-coordinate or an increase in the X-coordinate. A superolateral rotation tendency of the temporal bone on the affected side was found. There were no significant differences in the linear and angular measurements between the groups. The degree of zygomatic arch deformation was lower in the mild group; however, no significant difference was found between the moderate and severe groups. CONCLUSIONS: In patients with CFM, asymmetry of the temporal bone and its inner organs (vestibulocochlear organ, facial nerve, and vessels) exists in multiple dimensions; its severity is not completely consistent with the degree of mandibular involvement.


Subject(s)
Goldenhar Syndrome , Humans , Goldenhar Syndrome/diagnostic imaging , Retrospective Studies , Mandible , Temporal Bone/diagnostic imaging , Tomography, X-Ray Computed , Facial Asymmetry
3.
J Craniofac Surg ; 34(4): 1283-1286, 2023 Jun 01.
Article in English | MEDLINE | ID: mdl-36658685

ABSTRACT

INTRODUCTION: The oculoauriculovertebral spectrum (OAVS) are anomalies of the first and second pharyngeal arches, causing craniofacial changes, principally facial asymmetry, as well as anomalies in the spine, that can be a cause of instability of the craniocervical junction - manipulation of an unstable spine can result in morbimortality. However, few studies have related OAVS to craniocervical instability. OBJECTIVES: Correlate patients with OAVS through radiography with craniocervical instability and prediction of its occurrence. MATERIAL AND METHODS: Through a radiographic study of the cervical spine, the assessment of vertebral malformations and the presence of craniocervical instability was performed and clinical assessment using the OMENS score as a phenotypic criteria for patients with OAVS, by a specialized multidisciplinary team. Student's t test, Kolmogorov-Smirnova, and χ 2 were performed. RESULTS: Twenty-six patients with OAVS were evaluated, 7 (26.9%) had craniocervical instability, the OMENS score was 3 times higher, but without statistical significance. All patients with instability also had spinal malformations, and of those without craniocervical instability (19 patients), 57.8% had spinal malformations. Vertebral malformations had a similar incidence in those with and without instability, the most common being scoliosis. There was no statistical significance in the presence or absence of spinal malformations with the presence or absence of craniocervical instability. Extracraniofacial findings were found in all patients with instability; 71.4% of them were radial. CONCLUSIONS: Patients with higher OMENS scores had more craniocervical instability but without statistical significance. It also did not show statistical relevance between the presence of malformations and craniocervical instability.


Subject(s)
Goldenhar Syndrome , Spinal Diseases , Humans , Goldenhar Syndrome/diagnostic imaging , Facial Asymmetry , Cervical Vertebrae/diagnostic imaging , Radiography
4.
J Craniofac Surg ; 34(1): 295-301, 2023.
Article in English | MEDLINE | ID: mdl-35949011

ABSTRACT

Although there are multiple variations of accessory foramina described in the literature, to our knowledge there is only 1 report of an isolated mandibular foramen and inferior alveolar neurovascular bundle that courses through the lateral ramus, particularly in those with branchial arch syndromes. Goldenhar syndrome, of the oculo-auriculo-vertebral spectrum, is a rare congenital condition, which most characteristically presents with hemifacial microsomia. Depending on the severity of hemifacial microsomia, there are predictable treatment modalities to correct the consequent facial asymmetry. We report on a patient with Goldenhar syndrome who was found to have a unique course of the inferior alveolar nerve during orthognathic surgery work-up and treatment.


Subject(s)
Goldenhar Syndrome , Orthognathic Surgery , Humans , Goldenhar Syndrome/diagnostic imaging , Goldenhar Syndrome/surgery , Mandible/diagnostic imaging , Mandible/surgery , Facial Asymmetry , Branchial Region
5.
Cleft Palate Craniofac J ; 60(11): 1513-1516, 2023 11.
Article in English | MEDLINE | ID: mdl-37448161

ABSTRACT

Goldenhar syndrome (GS) is a rare congenital disorder characterized by multiple facial anomalies. This case report describes a GS presenting with bilateral cleft lip and palate and bilateral transverse facial cleft. We performed a single-stage surgery to repair the bilateral cleft lip and bilateral transverse facial cleft when the patient was 4-months-old. Bilateral cleft lip repair using the Mulliken method was performed first, and then the bilateral transverse facial cleft was corrected. Orbicularis oris muscle repair was done at each clefts. Anatomical approximation technique was used and the final oral commissure was determined considering symmetry. Satisfactory outcomes were achieved without complications.


Subject(s)
Abnormalities, Multiple , Cleft Lip , Cleft Palate , Goldenhar Syndrome , Humans , Infant , Cleft Lip/diagnostic imaging , Cleft Lip/surgery , Goldenhar Syndrome/diagnostic imaging , Goldenhar Syndrome/surgery , Cleft Palate/diagnostic imaging , Cleft Palate/surgery , Lip/surgery , Facial Muscles/surgery , Abnormalities, Multiple/surgery
6.
Am J Med Genet A ; 188(5): 1515-1525, 2022 05.
Article in English | MEDLINE | ID: mdl-35119197

ABSTRACT

A diagnosis of oculo-auriculo-vertebral spectrum (OAVS) is established when microtia is present in association with hemifacial hypoplasia (HH) and/or ocular, vertebral, and/or renal malformations. There is no consensus on which imaging studies should be used to rule out variable expressivity and distinguish "sporadic" from "familial" patients. This observational and descriptive study was performed in a Mexican population of 51 patients (32 males, 19 females, 0-18 years old) with microtia/OAVS, and their available parents. A clinical history, genealogy, and physical examination were obtained from all included patients, as were a computed tomography (CT) scan of the ear, audiological evaluation, orthopantomography, complete spine radiography, and renal ultrasound. The same approach was completed in their available parents (51 mothers and 40 fathers), excluding the CT scan and audiological evaluation. By genealogy, 53% of patients were classified as "sporadic"; of the "familial" patients, at least 79.1% had suggestion of a multifactorial inheritance. In the available parents, orthopantomography, complete spine X-ray, and renal ultrasound identified the following OAVS-related manifestations: HH (16.2%, n = 14/86), vertebral alterations (10.9%, n = 10/91), and renal anomalies (2.2%, n = 2/90). Our evaluation of the parents allowed three patients to be reclassified from "sporadic" to "familial" (5.8%, n = 3/51). Our proposed clinical and imaging approach allowed the identification of variable expressivity that more clearly distinguished between "sporadic" and "familial" OAVS patients, which is of utmost importance in providing proper genetic counseling to these families.


Subject(s)
Congenital Microtia , Goldenhar Syndrome , Adolescent , Child , Child, Preschool , Female , Goldenhar Syndrome/diagnostic imaging , Goldenhar Syndrome/genetics , Humans , Infant , Infant, Newborn , Male , Mexico , Spine/diagnostic imaging , Tomography, X-Ray Computed
7.
Acta Anaesthesiol Scand ; 66(7): 823-832, 2022 08.
Article in English | MEDLINE | ID: mdl-35416276

ABSTRACT

BACKGROUND: Hemifacial microsomia (HFM) is a congenital craniofacial malformation, and its features include hypoplasia and asymmetry in skeletal tissue and soft tissue. These features are usually associated with a difficult airway. Mandibular distraction osteogenesis can achieve mandibular advancement, but its effect on the subsequent laryngoscopic views has not been adequately documented. AIM: The aim of this study was to determine the change in laryngoscopic views after mandibular distraction osteogenesis (MDO) in children with HFM and to examine the radiological changes after MDO by using computed tomography (CT)-based three-dimensional (3-D) airway reconstruction. METHODS: An observational prospective study was carried out in children with HFM undergoing MDO. All children underwent 3-D airway reconstruction preoperatively before the placement and removal of the distractor. The temporomandibular joint lesions in children were classified by the Pruzansky-Kaban classification. The Cormack-Lehane grade was assessed after induction under direct laryngoscopy. The relationship between difficult laryngoscopy and the Pruzansky-Kaban classification was assessed. Changes in the upper pharyngeal airway parameters and laryngoscopic views were analyzed. RESULTS: Forty children (ages ranging from 5 to 16 years, males = 26, females = 14) with HFM underwent 80 anesthesia for MDO. The incidence of difficult laryngoscopy before mandibular distraction was 5/12 (41.7%) in type IIa, 19/24 (79.2%) in type IIb and 1/4 (25%) in type III HFM. The mean difference in the incidence of difficult laryngoscopy before and after MDO was 33.3% (95% CI: -6.7% to 73.3%), 50.0% (95% CI: 8.4% to 91.6%), and 0% for type IIa, type IIb, and type III, respectively. In type IIb, 12/19 cases of difficult laryngoscopy became easy after mandibular distraction: this was associated with an increase in the palatopharyngeal and glossopharyngeal CT parameters (p < .05). In type IIa, 5/5 cases of difficult laryngoscopy became easy after mandibular distraction, and 1/7 cases of easy laryngoscopy became difficult laryngoscopy. However, there were no significant changes in the palatopharyngeal, glossopharyngeal, and hypopharyngeal CT parameters in type IIa and type III HFM (p > .05). CONCLUSIONS: MDO can improve the laryngoscopic views in children with HFM. According to the Pruzansky-Kaban classification, children with type IIb showed the highest incidence of difficult laryngoscopy before MDO. After MDO, the improvement in laryngoscopic views under direct laryngoscopy in type IIb was related to the increase in the palatopharyngeal and glossopharyngeal volume.


Subject(s)
Anesthetics , Goldenhar Syndrome , Osteogenesis, Distraction , Adolescent , Child , Child, Preschool , Female , Goldenhar Syndrome/diagnostic imaging , Goldenhar Syndrome/surgery , Humans , Male , Mandible/diagnostic imaging , Mandible/surgery , Osteogenesis, Distraction/methods , Prospective Studies , Retrospective Studies
8.
BMC Pediatr ; 22(1): 102, 2022 02 21.
Article in English | MEDLINE | ID: mdl-35189859

ABSTRACT

BACKGROUND: Oculo-auriculo-vertebral spectrum is a heterogeneous group of genetic disorder, also known as Goldenhar Syndrome, which has several phenotypic features including craniofacial anomalies, cardiac, vertebral and central nervous system defects. Cardiovascular anomalies include ventricular septal defects, atrial septal defects, patent ductus arteriosus, Tetralogy of Fallot, double outlet right ventricle, aberrant right subclavian artery, coarctation of aorta, transposition of the great arteries, double inlet left ventricle, cor triatriatum, pulmonary artery stenosis, aortic stenosis, persistent left superior vena cava, partially or totally abnormal pulmonary venous return and bicuspid aortic valve. Persistent fifth aortic arch, also named as double lumen aortic arch, is a very rare cardivascular anomaly and usually associate other cardiac defects. CASE PRESENTATION: We present a 7 month old patient with oculo-auriculo-vertebral spectrum signs as facial asymmetry, short neck, choanal atresia, cleft palate, bilateral preauricular skin tags, bilateral hypoplastic ear lobes, epibulbar dermoid cyst, rib, vertebrae and cardiovascular anomalies. Cardiovascular anomalies detected with echocardiography and computed tomography were malalignment ventricular septal defect and double lumen aorta, known as persistent fifth aortic arch. CONCLUSION: Various cardiovascular anomalies may accompany Goldenhar Syndrome. We present a case with persistent fifth aortic arch and Oculo-auriculo-vertebral spectrum and this is a new association that was not reported before in the literature.


Subject(s)
Cardiovascular Abnormalities , Goldenhar Syndrome , Heart Defects, Congenital , Heart Septal Defects, Ventricular , Transposition of Great Vessels , Aorta, Thoracic/abnormalities , Aorta, Thoracic/diagnostic imaging , Cardiovascular Abnormalities/complications , Cardiovascular Abnormalities/diagnostic imaging , Goldenhar Syndrome/diagnosis , Goldenhar Syndrome/diagnostic imaging , Heart Defects, Congenital/genetics , Humans , Infant , Transposition of Great Vessels/surgery , Vena Cava, Superior
9.
Clin Oral Investig ; 26(6): 4603-4613, 2022 Jun.
Article in English | MEDLINE | ID: mdl-35218426

ABSTRACT

OBJECTIVES: To compare the characteristics of mandibular asymmetry in patients with unilateral craniofacial microsomia (CFM) and class II asymmetry. MATERIALS AND METHODS: Pretreatment cone-beam computed tomography of consecutive adults with Pruzansky-Kaban type I and IIA CFM (CFM group) was analyzed by 3D cephalometry. Fourteen mandibular landmarks and two dental landmarks were identified. The mandibular size and positional asymmetry were calculated by using landmark-based linear and volumetric measurements, in terms of asymmetry ratios (affected/non-affected side) and absolute differences (affected - non-affected side). Results were compared with non-syndromic class II with matched severity of chin deviation (Class II group). Statistical analyses included independent t test, paired t test, chi-square test, and ANOVA. RESULTS: CFM group (n, 21; mean age, 20.4 ± 2.5 years) showed significantly larger size asymmetry in regions of mandibular body, ramus, and condyle compared to Class II group (n, 21; mean age, 27.8 ± 5.9 years) (p < 0.05). The curvature of mandibular body was asymmetric in CFM. Regarding the positional asymmetry of mandibular body, while a comparable transverse shift and a negligible yaw rotation were found among the two groups, the roll rotation in CFM was significantly greater as well as the occlusal (6.06° vs. 4.17°) and mandibular (7.84° vs. 2.80°) plane cants (p < 0.05). CONCLUSIONS: Mild CFM showed significantly more severe size asymmetry and roll rotation in mandible than non-CFM class II asymmetry. CLINICAL RELEVANCE: To improve the mandibular size and positional asymmetry in CFM, adjunct hard tissue augmentation or reduction in addition to OGS orthodontics with a meticulous roll and yaw planning is compulsory, which is expected to be distinct from treating non-CFM class II asymmetry.


Subject(s)
Goldenhar Syndrome , Adolescent , Adult , Cephalometry/methods , Cone-Beam Computed Tomography/methods , Facial Asymmetry/diagnostic imaging , Goldenhar Syndrome/diagnostic imaging , Humans , Imaging, Three-Dimensional/methods , Mandible/diagnostic imaging , Young Adult
10.
J Craniofac Surg ; 33(4): 1154-1158, 2022 Jun 01.
Article in English | MEDLINE | ID: mdl-34743153

ABSTRACT

ABSTRACT: This study aimed to explore correlations between mandible and ear deformities and quantitative volumetric relations between condylar structures and external ear in hemifacial microsomia. The authors reconstructed three-dimensional craniofacial models from 212 patients with unilateral hemifacial microsomia (the unaffected side as the controls). Patients were evaluated by Pruzansky-Kaban and Marx classification, and divided into 3 age groups (0-6, 7-12, and >12 years of age). The mandible condylar structures, including condyle and the condylar skeletal unit, were selected (except the classification of the mandibular or ear deformities (M3)). Along with the external ear (except the classification of the mandibular or ear deformities (E4)), their volumes were measured and analyzed. Spearman correlation coefficient analysis was applied. There was a positive correlation between the mandible and ear deformities (r = 0.301, P   <  0.001). Either between the condyle and external ear ( P  = 0.071-0.493) or between the condylar unit and external ear ( P  = 0.080 - 0.488), there were no volumetric relations on the affected side, whereas on the unaffected side were (r = 0.492-0.929 for condyle, r = 0.443-0.929 for the condylar unit, P  < 0.05). In most cases, the condylar structures of the classification of the mandibular or ear deformities (M2b) were significantly smaller than the classification of the mandibular or ear deformities (M2a). Results suggested deformities of mandibular condylar structures and ear did not correlate, although deformities of mandible and ear did. The condylar deformity might develop independently from microtia and be more severe within relatively more abnormal temporomandibular joints.


Subject(s)
Goldenhar Syndrome , Child , Ear, External/diagnostic imaging , Facial Asymmetry/diagnostic imaging , Goldenhar Syndrome/diagnostic imaging , Humans , Mandible/abnormalities , Mandible/diagnostic imaging , Mandibular Condyle/abnormalities , Mandibular Condyle/diagnostic imaging , Temporomandibular Joint
11.
J Craniofac Surg ; 33(2): 534-538, 2022.
Article in English | MEDLINE | ID: mdl-34723918

ABSTRACT

BACKGROUND: Patients with hemifacial microsomia (HFM) may undergo unilateral mandibular distraction osteogenesis (MDO) before skeletal maturity in an effort to improve facial symmetry. Mandibular distraction osteogenesis's effect on airway volumes have been studied in the past, though to our knowledge, none have accounted for the effect of head and neck posture on airway morphology. This study aimed to tackle this shortcoming, using imaging to analyze the upper airway of patients with HFM before and after surgical intervention with MDO. METHODS/DESCRIPTION: The authors retrospectively reviewed patients with a diagnosis of unilateral HFM whom underwent unilateral MDO with an oblique vector at age 4 to 14 years at a single institution from 2004 to 2019. Patients with pre- and post-MDO three-dimensional computed tomography scans of the upper airway within 12 months of distractor placement and removal, respectively, were included. Head and neck postures were determined by craniocervical, pitch, roll, and yaw angles. Pre- and post-operative pharyngeal airway volumes, pharyngeal surface area, minimum retropalatal cross-sectional areas (RP CSA) and retroglossal (RG) CSA and associated anteroposterior distances were measured using Mimics 22.0 (Materialise; Leuven, Belgium). Comparison was done using Kruskal-Wallis tests and linear mixed-effects models controlling for head and neck postures. RESULTS: Ten patients met inclusion criteria. Mean age at pre-distractor placement computed tomography scan was 99 ±â€Š35 months, and mean duration between pre- and post-surgery scans was 220 ±â€Š90 days. Head and neck posture were found to be significant predictors of all airway dimensions. After controlling for significant factors with fixed effects linear modeling, surface area was found to be significantly smaller in patients after MDO by 189.48 mm2 (F[10.8] = -3.47, P = 0.0053), compared to their preoperative measurements. Surgery was not a significant predictor of changes in airway volume (F[11.6] = 0.52, P = 0.61), minimum RP CSA (F[12.2] =  -0.64, P = 0.53), minimum RG CSA (F[12.6] =  -1.64, P = 0.13), RP anteroposterior distance (F[14.0] = 0.30, P = 0.77), or RG anteroposterior distance (F[20.0] =  -0.04, P = 0.97). CONCLUSIONS: Oblique vector MDO in patients with HFM is associated only with statistically significant changes in the surface area of the upper airway, and is not associated with statistically significant changes in dimensions like volume, CSA, or anteroposterior dimension. This is an important finding, as it may guide discussions surrounding risk/benefit ratio for MDO in childhood.


Subject(s)
Airway Obstruction , Goldenhar Syndrome , Osteogenesis, Distraction , Pierre Robin Syndrome , Adolescent , Airway Obstruction/surgery , Child , Child, Preschool , Goldenhar Syndrome/complications , Goldenhar Syndrome/diagnostic imaging , Goldenhar Syndrome/surgery , Humans , Mandible/diagnostic imaging , Mandible/surgery , Osteogenesis, Distraction/methods , Pierre Robin Syndrome/surgery , Retrospective Studies , Treatment Outcome
12.
Am J Orthod Dentofacial Orthop ; 161(5): 708-726, 2022 May.
Article in English | MEDLINE | ID: mdl-35031193

ABSTRACT

Treatment of hemifacial microsomia is challenging and often requires multiple interventions to restore function and facial esthetics. In this article, the combined orthodontic-surgical treatment of a young patient exhibiting Pruzansky I hemifacial microsomia is reported. The patient was aged 15 years, but his bone age was determined to be 18 years. His facial asymmetry was severe, with the nose and a retrusive chin deviated to the left side and a canted smile. The presurgical phase was aimed at centering the mandibular midline to the center of the chin through the distal movement of the mandibular left buccal dentition. The surgery was planned with 3-dimensional computer-aided surgical simulation and included a LeFort I and unilateral sagittal split osteotomies combined with a genioplasty. This report illustrates the therapeutic stages and a 4-year follow-up of a unique and complex orthognathic surgical approach, chosen among other alternatives and leading to improved function and appearance and stable results.


Subject(s)
Goldenhar Syndrome , Orthognathic Surgical Procedures , Facial Asymmetry/diagnostic imaging , Facial Asymmetry/surgery , Follow-Up Studies , Goldenhar Syndrome/complications , Goldenhar Syndrome/diagnostic imaging , Goldenhar Syndrome/surgery , Humans , Mandible/surgery , Orthognathic Surgical Procedures/methods
13.
Orthod Craniofac Res ; 24(4): 575-584, 2021 Nov.
Article in English | MEDLINE | ID: mdl-33713375

ABSTRACT

OBJECTIVES: The objectives of this study were to characterize the craniofacial and airway morphology of oculo-auriculo-vertebral spectrum (OAVS) individuals using computed tomography (CT) examination. SETTING AND SAMPLE POPULATION: This sample included individuals in the age range from 5 to 14 years, consisted of a group of 18 OAVS individuals (12 females and 6 males), Pruzansky-Kaban1 IIB and III and by a paired control group matched by age and sex for comparison of morphometric and airway variables. MATERIALS AND METHODS: Through the CT examination, airway analysis was performed using Dolphin Imaging® Software, and seven morphometric measurements were performed to evaluate craniofacial morphology by Materialize Mimics® Software. To compare airway and morphometric variables, the control group was used. Student's t test and Mann-Whitney U test were performed to compare differences between the groups. RESULTS: Statistically significant differences were showed between the control and OAVS groups for the variables: total airway (TA) area, volume and MAA, RP area, RP volume, RP MAA, RG volume, RG MAA, total posterior height diff, Md incl and y-axis asymmetry. Pearson and Spearman's correlation showed mostly moderate correlations between Mand Occlusal canting AS with TA area and RP volume, Ax-Gn with TA area and Hy-C3 with TA volume. CONCLUSIONS: The OAVS's airway was altered and worse than the control group. Our results suggest that the contralateral side of OAVS individuals is unaffected; however, longitudinal assessments are needed to confirm it. Hyoid bone and postural measures play an important role in interpreting airway features of individuals with and without OAVS.


Subject(s)
Goldenhar Syndrome , Female , Goldenhar Syndrome/diagnostic imaging , Humans , Hyoid Bone/diagnostic imaging , Male , Tomography, X-Ray Computed
14.
J Oral Maxillofac Surg ; 79(8): 1750.e1-1750.e10, 2021 08.
Article in English | MEDLINE | ID: mdl-33939960

ABSTRACT

PURPOSE: To assess the condylar hypoplasia and its correlation with craniofacial deformities in adults with unilateral craniofacial microsomia (CFM). METHODS: Pretreatment cone-beam computed tomography scans of consecutive adults (mean age: 20.4 ± 3.0 years; range: 17.3 to 31.4 years) with Pruzansky-Kaban type I and IIA CFM were reconstructed in 3D. Both condyles were segmented. Asymmetry ratios (affected side/contralateral side) of condylar volume were calculated to indicate the extent of condylar hypoplasia. 3D cephalometry was performed to quantify the maxillomandibular morphology and facial asymmetry. The correlations in between were assessed by using Pearson's or Spearman's correlation coefficients. RESULTS: Thirty-six subjects were enrolled, consisting of 22 subjects with Pruzansky-Kaban type I and 14 subjects with type IIA. The condyles in type IIA group were significantly more hypoplastic in height (asymmetry ratio: 40.69 vs 59.95%, P = .006) and volume (18.16 vs 47.84%, P < .001) compared to type I group. Type IIA group had a significantly smaller SNB value than type I group (72.94° vs 77.41°, P = .012), and a significantly greater facial asymmetry (P < .05). The hypoplastic extent of condylar volume and Pruzansky-Kaban types were significantly correlated with SNB (r = 0.457 and ρ = -0.411, respectively), upper incisor deviation (r = -0.446 and ρ = 0.362), chin deviation (r = -0.477 and ρ = 0.527), upper occlusal plane cant (r = -0.672 and ρ = 0.631), and mandibular plane cant (r = -0.557 and ρ = 0.357, P < .05). CONCLUSION: For unilateral CFM adults, greater condylar hypoplasia in volume along with more severe mandibular retrusion and facial asymmetry objectively indicated a higher scale of Pruzansky-Kaban classification (type IIA). These quantitative distinctions are expected to enhance the diagnostic reliability of CFM.


Subject(s)
Goldenhar Syndrome , Adolescent , Adult , Cone-Beam Computed Tomography , Facial Asymmetry/diagnostic imaging , Goldenhar Syndrome/diagnostic imaging , Humans , Imaging, Three-Dimensional , Mandible , Mandibular Condyle/diagnostic imaging , Reproducibility of Results , Young Adult
15.
J Craniofac Surg ; 32(3): 896-901, 2021 May 01.
Article in English | MEDLINE | ID: mdl-33405441

ABSTRACT

PURPOSE: Pruzansky-Kaban III hemifacial microsomia (HFM) is a rare congenital facial deformity, and it is challenging to reconstruct the facial appearance. The aim of the present study was to describe a technique of application of virtual planning for three-dimensional (3D) guided maxillofacial reconstruction of Pruzansky-Kaban III HFM using custom made fixation plate. METHODS: With the help of 3D models, a preoperative virtual planning and surgical simulation were performed. Computer-aided design/computer-aided manufacture (CAD/CAM) patient customized guides and custom fixation plates were designed to reconstruct the maxillofacial skull intraoperatively. Assessment was achieved through evaluation of the postoperative effects, such as imaging, facial appearance recovery and operation time. RESULTS: Five patients with Pruzansky-Kaban III HFM were enrolled into this study. The results showed an exceptional accuracy between the preoperative virtual planning and the outcomes actually achieved postoperatively. Intraoperative measurements were no longer needed and the different surgical steps became more simple and easier. The total time was distributed as: 160 minutes for the surgical time, 40 minutes for preoperative virtual plan, and 80 minutes for designing the patient specific cutting guides and custom fixation plates. The operating time and tissue damage were reduced. All cases underwent uneventful healing without any complications. CONCLUSION: The technique of patient specific guides and custom fixation plates is a reliable method of conveying the virtual plan to the operative field with higher efficiency in patients with Pruzansky-Kaban III HFM.


Subject(s)
Goldenhar Syndrome , Orthognathic Surgical Procedures , Surgery, Computer-Assisted , Bone Plates , Computer-Aided Design , Goldenhar Syndrome/diagnostic imaging , Goldenhar Syndrome/surgery , Humans , Imaging, Three-Dimensional
16.
J Craniofac Surg ; 32(3): 1083-1086, 2021 May 01.
Article in English | MEDLINE | ID: mdl-33405458

ABSTRACT

ABSTRACT: Hemifacial microsomia is the second most frequent pathology in craniofacial malformations. Clinical findings are broad, mainly affecting the mandible. Several classifications of mandibular compromise exist that guide the best treatment option in each patient. The authors present a case of an unusual complication following fibular free flap mandibular reconstruction in a patient with hemifacial microsomia prada type IV, who presented with ankylosis at the skull base and simultaneous fibula pseudoarthrosis at the union with the residual mandible. These dual findings allowed the patient to have a functional mouth aperture, which give us time to let him grow and wait for final management. Treatment options and follow up are discussed, knowing that there is no literature to support any protocol with this patient, so we present his evolution.


Subject(s)
Free Tissue Flaps , Goldenhar Syndrome , Mandibular Reconstruction , Plastic Surgery Procedures , Tooth Ankylosis , Fibula , Goldenhar Syndrome/diagnostic imaging , Goldenhar Syndrome/surgery , Humans , Male , Mandible/diagnostic imaging , Mandible/surgery
17.
J Craniofac Surg ; 32(7): 2416-2420, 2021 Oct 01.
Article in English | MEDLINE | ID: mdl-34260455

ABSTRACT

ABSTRACT: This is a retrospective study to evaluate the postsurgical position of the maxilla and mandible in 5 matured craniofacial patients with unilateral craniofacial microsomia who underwent 2 jaw surgical procedures using computerized surgical planning. The craniofacial surgeon and orthodontist completed the virtual surgical treatment plan with a biomedical engineer's assistance via a web meeting. The treatment plan of each patient included 2 jaw surgery with genioplasty. At the maxillary dental midline, the planned mean advancement was 4 mm; yaw, a rotational correction towards the unaffected side was 4.96 mm; and impaction was 2.74 mm. The mean advancement measured at point B was 10.5 mm, and the rotational correction towards the unaffected side was 6.58 mm. The mean advancement following genioplasty was 8.43 mm, and the mean transverse correction was 6.33 mm towards the midsagittal plane. The intermediate surgical splint, final surgical splint, bone graft templates, and cutting guides were constructed utilizing computer-aided design/computer-aided manufacturing technology. The surgeon executed the treatment plan in the operating room using appropriate computer-generated guides and splints. A postsurgical cone-beam computed tomography scan was obtained and superimposed on the surgical treatment plan using Simplant OMS 10.1 software. The cranial base was used as a reference for superimposition. Three-dimensional color-coded displacement maps were generated to visually and quantitatively assess the surgical outcome. There was a mean error of 0.88 mm (+0.30) for the position of the maxillary anatomical structures from the planned position, and the anterior mandibular anatomical structures were on average 0.96 mm (+0.26) from the planned position.


Subject(s)
Goldenhar Syndrome , Orthognathic Surgical Procedures , Surgery, Computer-Assisted , Computer-Aided Design , Facial Asymmetry/diagnostic imaging , Facial Asymmetry/surgery , Goldenhar Syndrome/diagnostic imaging , Goldenhar Syndrome/surgery , Humans , Imaging, Three-Dimensional , Retrospective Studies
18.
J Craniofac Surg ; 32(4): 1331-1333, 2021 Jun 01.
Article in English | MEDLINE | ID: mdl-33710054

ABSTRACT

OBJECTIVE: This study focused on evaluating different parts of the airway in patients with hemifacial microsomia classified by the Pruzansky-Kaban classification system. METHODS: Volumetric and morphologic airway parameters of 66 children with hemifacial microsomia were measured on 3D models. Using the Pruzansky-Kaban system, the patient cohort was composed of seven I-type, 14 IIa type, 27 IIb type, and 18 III type patients. RESULTS: The total airway volume was not statistically different among groups. In the 3D oropharynx models, volume and surface area of the oropharynx decreased from the type I group to the type IIb group. However, no statistically significant difference was found for length (P = 0.965) or minimum cross-sectional area (mini-CSA, P = 0.550) of the oropharynx in the type III group compared to the other groups. In the 3D laryngopharynx models, the mean-CSA (P = 0.413) and mini-CSA (P = 0.378) were not statistically different among groups. In contrast, volume (P = 0.014), length (P = 0.005), and surface area (P = 0.032) of the laryngopharynx were reduced from type I to type III. Kruskal-Wallis analysis indicated statistically significant differences of volume (P = 0.004), length (P = 0.017), and surface area (P = 0.010) of the laryngopharynx among groups I, IIa, and IIb. The mean-CSA (P = 0.247) and mini-CSA (P = 0.206) of the laryngopharynx were not correlated with the different clinical types. CONCLUSION: The mean-CSA varied significantly from type I to IIb at the level of oropharynx. In addition, the volume of the laryngopharynx decreased from type I to type III. However, type III was unique in this study with nonseverely airway measurement results.


Subject(s)
Goldenhar Syndrome , Child , Goldenhar Syndrome/diagnostic imaging , Humans , Hypopharynx/diagnostic imaging , Imaging, Three-Dimensional , Mandible , Oropharynx/diagnostic imaging
19.
Cleft Palate Craniofac J ; 58(9): 1086-1093, 2021 09.
Article in English | MEDLINE | ID: mdl-33251829

ABSTRACT

OBJECTIVE: The purpose of this study is to utilize cone-beam computed tomography (CBCT) to compare the bone mineral density (BMD) on the affected versus nonaffected side, among individuals with hemifacial microsomia (HFM). METHODS: This retrospective study included 9 patients with HFM. Pretreatment CBCT volumes were imported into Invivo5 software, which was used to measure BMD through Hounsfield units (HU) in 3 regions of the mandible; inferior to the lower lateral incisors, inferior to the first molar and at the ramus inferior to the sigmoid notch. Each region was measured at the buccal cortical bone, lingual cortical bone, and cancellous bone. The densities on the right and left sides were compared by Wilcoxon signed-rank test. RESULTS: Overall, the BMD on the affected side tended to show slightly lower values when compared with the nonaffected side. Differences ranged from 14 HU at the lingual cortical plate of the first molar region to 234 HU at the buccal cortical plate of the ramus region. Differences were only statistically significant at the ramus region for the buccal (P = .002) and lingual (P < .001) cortical plates and at the lower incisor region at the buccal cortical plate (P = .016) and cancellous bone (P = .044). The differences, however, did not seem to be clinically significant. CONCLUSIONS: The current study shows that the quality of bone on the affected side may be slightly reduced but did not seem to be clinically significant. This should be accounted for during surgical and orthodontic planning for patients with HFM.


Subject(s)
Goldenhar Syndrome , Bone Density , Cone-Beam Computed Tomography , Goldenhar Syndrome/diagnostic imaging , Humans , Mandible/diagnostic imaging , Retrospective Studies
20.
Orthod Craniofac Res ; 23(4): 479-485, 2020 Nov.
Article in English | MEDLINE | ID: mdl-32515152

ABSTRACT

OBJECTIVE(S): The aim of this study was to evaluate the long-term effectiveness of costochondral graft in hemifacial microsomia (HFM) type III patients. SETTINGS AND SAMPLE POPULATION: A sample of 10 patients affected by HFM type III treated during growth in the same Centre with costochondral graft (CCG patients group) is compared with a control group (CG) sample of 10 non-treated patients affected by the same malformation in order to understand whether surgery during growth provides advantages in terms of bony and facial symmetry after an 8-year follow-up. MATERIALS AND METHODS: The growth of the CCG was assessed on panoramic X-rays. To assess facial symmetry, a photometric evaluation on the frontal view was carried out. RESULTS: In CCG patients group the graft grown in mean less than the healthy ramus, a good facial symmetry was achieved after surgery, but was lost in the majority of the cases at the most recent control. In CG, occlusal canting slightly increased and facial asymmetry was relatively stable during the years. CONCLUSION: In patients with a congenital deformity, restoring the height of the ramus leads to an immediate restitution of facial symmetry, but in the long term, there is a return to the asymmetrical pattern. In CG, the asymmetry is stable during years with no increase of the facial deformity.


Subject(s)
Goldenhar Syndrome , Facial Asymmetry/diagnostic imaging , Facial Asymmetry/surgery , Follow-Up Studies , Goldenhar Syndrome/diagnostic imaging , Goldenhar Syndrome/surgery , Humans , Mandible , Radiography, Panoramic , Treatment Outcome
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