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1.
Front Pediatr ; 12: 1453270, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-39252751

RESUMEN

Introduction: Mandible distraction osteogenesis (MDO) is widely used to reconstruct the mandible in patients with mild Hemifacial microsomia (HFM). However, the masseter's response to mandible distraction remains unclear. Methods: In this study, we analyze the effect of the surgical intervention on masseter muscle by a retrospective analysis. The procedure consisted of a five-day latent period, a three-week distraction period, and a six-month consolidation period. CT data were manually segmented and measured with Mimics software before surgery, within 3 months, and more than 1 year postoperatively. Masseter volume, masseter length, masseter width, and mandible ramus height were measured and analyzed using paired t-test, Pearson, and Spearman correlation analysis. Results: We included 21 patients with HFM who underwent mandible distraction osteogenesis from 2015 to 2020. The masseter volume on the affected side increased immediately after surgery from (6,505.33 ± 3,671.95) mm3 to (10,194.60 ± 5638.79) mm3, but decreased to (8,148.38 ± 3,472.57) mm3 at the second follow-up correlated to mandible ramus height (r = 0.395, P = 0.038). A similar trend was observed in changes in masseter length. Symmetry and width of masseter muscle had no longitudinal statistical significance. Discussion: Masseter muscle involvement benefits from MDO in the short term. To achieve long-term efficacy, more attention should be paid to muscle reconstruction.

2.
J Plast Reconstr Aesthet Surg ; 98: 100-102, 2024 Sep 02.
Artículo en Inglés | MEDLINE | ID: mdl-39243711

RESUMEN

This paper describes our experience with mixed reality (MR) intra-operative guides in patients with congenital craniofacial malformations. The first case was a patient with bilateral hemifacial microsomia. He underwent bilateral mandibular distraction osteogenesis. Pre-operative virtual planning determined the sites of osteotomy. Standard Tessellation Language (STL) files of mandibular 3D models with osteotomy sites were uploaded onto the HoloLens 2® MR glasses (Microsoft®, Washington, USA). The superimposed hologram denoted the osteotomy line. This was validated with a physical cutting guide. The second case was a patient with Crouzon's syndrome. A modified Lefort 2 advancement was performed to correct his midfacial deficiency. Pre-operative virtual planning was performed to determine the sites of osteotomies. Superimposed hologram using the Hololens 2® denoted the osteotomy sites. These were validated with a conventional intra-operative navigation system. The advantages of using MR include its immediate availability for use; saving time and costs. MR allows surgeons to maintain continuous line-of-sight within the operative field. A robust registration system is required to anchor the hologram onto the patient's skull without variations in hologram position from different angles of gaze. MR has the potential to function as an adjunct and possible replacement for conventional cutting guides and intra-operative navigation. LAY SUMMARY: We describe the use of mixed reality intra-operative guides in patients with congenital craniofacial malformations. Our experience shows the potential MR has as an adjunct and possible replacement for conventional cutting guides and intra-operative navigation.

3.
Stomatologiia (Mosk) ; 103(4): 28-32, 2024.
Artículo en Ruso | MEDLINE | ID: mdl-39171340

RESUMEN

THE STUDY OBJECTIVE: Was to assess the qualitative and quantitative characteristics of the bone regenerate in the distant period following compression-distraction osteosynthesis. MATERIAL AND METHODS: Results from the treatment of 30 patients with mandibular micrognathia, were analyzed. Compression-distraction osteosynthesis (CDO) was performed in the area of the mandibular angle. The patients were divided into two groups: group 1 consisted of 16 patients with congenital micrognathia, and group 2 comprised 14 patients with acquired (post-traumatic) micrognathia. For the assessment of qualitative and quantitative characteristics of the bone regenerate, three-dimensional modeling, morphometric measurements of the regenerate, and evaluation of bone density using the Hounsfield scale through multislice computed tomography were used. RESULTS AND DISCUSSION: The study of the bone regenerate parameters in group 1 yielded the following results: the length of the bone regenerate decreased by 2.14%; the thickness of the bone regenerate increased by 8.75%; the height of the bone regenerate increased by 1.75%. In group 2, the values were 3.14; 3 and 3.76%, correspondingly. In patients of group 1, the length of the mandibular branch increased by 4%; the length of the mandibular body increased by 5.35%. In group 2, the length of the mandibular branch increased by 1.94%; the length of the mandibular body increased by 2.2%. The density of the cortical bone of the regenerate corresponds to the density of the intact bone, while the density of the cancellous bone is slightly reduced. CONCLUSION: Qualitative and quantitative characteristics of bone regenerate in the long-term period after CDO correspond to the parameters of intact bone. CDO can be considered not only as a preparatory stage for reconstructive operations after the growth of the skull bones has completed, but also as an independent stage of treatment.


Asunto(s)
Regeneración Ósea , Mandíbula , Humanos , Mandíbula/cirugía , Mandíbula/diagnóstico por imagen , Masculino , Femenino , Adolescente , Osteogénesis por Distracción/métodos , Adulto , Niño , Densidad Ósea , Fijación Interna de Fracturas/métodos , Adulto Joven
4.
Cureus ; 16(7): e64499, 2024 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-39139347

RESUMEN

Hemifacial microsomia (HFM) presents a complex congenital anomaly characterized by the asymmetric underdevelopment of facial structures, predominantly affecting the ear, mouth, and mandible on one side of the face. This case study examines the intricacies of HFM through the presentation of a 23-year-old female with congenital deformities of the left ear and mandibular hypoplasia. Clinical and radiological evaluations revealed significant facial malformations, including mandibular hypoplasia, left temporomandibular joint fusion, and maxillary abnormalities, confirming the diagnosis of HFM. Management of HFM necessitates a multidisciplinary approach involving otolaryngologists, maxillofacial surgeons, orthodontists, and audiologists to comprehensively address functional and aesthetic concerns. Early diagnosis and intervention, along with psychosocial support, are essential for optimizing outcomes and improving the quality of life for individuals with HFM.

5.
J Plast Reconstr Aesthet Surg ; 97: 212-220, 2024 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-39173574

RESUMEN

BACKGROUND: This study aimed to prospectively investigate the reference values for masseter muscle thickness and hardness using ultrasonography and shear wave elastography, respectively, in patients with hemifacial microsomia (HFM). METHODS: We enrolled 51 patients, aged 5-20 years, with HFM including 31 males and 20 females. The upper-lower, left-right, and anterior-posterior diameters of 102 masseter muscles and stiffness of 98 masseter muscles were determined by examining the unaffected and affected sides of each participant's face. RESULTS: The upper-lower, left-right, and anterior-posterior diameters of the masseter muscle were significantly smaller at rest (4.26 ± 0.83, 2.94 ± 0.75, and- 0.80 ± 0.25 cm, respectively) and during contraction (3.95 ± 0.78, 2.71 ± 0.78, and 0.87 ± 0.29 cm, respectively) in the affected side than those in the healthy side (5.45 ± 0.66, 3.87 ± 0.49, and 0.97 ± 0.20 cm, respectively, at rest and 4.99 ± 0.45, 3.49 ± 0.53, and 1.07 ± 0.23 cm, respectively, during contraction, p < 0.05). In the resting state, the hardness of the masseter muscle on the affected side (0.77 ± 0.66 m/s) was significantly greater than that on the healthy side (0.42 ± 0.41 m/s; p < 0.05). The magnitude of changes in the upper-lower, left-right, and anterior-posterior diameters of the biting muscle in the occlusal state were significantly smaller on the affected side (-0.30 ± 0.27, -0.23 ± 0.17, and 0.08 ± 0.08 cm, respectively) than those in the healthy side (-0.47 ± 0.38, -0.37 ± 0.25, and 0.10 ± 0.12 cm, respectively, p < 0.05). CONCLUSIONS: The knowledge of these values allows for better understanding of the disease characteristics of HFM, which may be used for its diagnosis, treatment, and prognosis. Patients experiencing different severity levels exhibited significant differences in the morphology and function of the masseter muscle on the affected-side (p < 0.05). EVIDENCE LEVEL: Level III.


Asunto(s)
Diagnóstico por Imagen de Elasticidad , Músculo Masetero , Humanos , Músculo Masetero/diagnóstico por imagen , Masculino , Femenino , Adolescente , Niño , Diagnóstico por Imagen de Elasticidad/métodos , Adulto Joven , Estudios Prospectivos , Preescolar , Ultrasonografía/métodos , Síndrome de Goldenhar/diagnóstico por imagen , Contracción Muscular/fisiología , Valores de Referencia
6.
In Vivo ; 38(5): 2550-2556, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-39187366

RESUMEN

BACKGROUND/AIM: Hemifacial microsomia (HFM) is the second most common congenital anomaly of the craniomaxillofacial region after the cleft lip and palate. This malformation is characterized by unilateral mandible and ear hypoplasia. Treatment varies and depends on different phenotypes. Severe deficiencies require multiple reconstructive surgeries to address facial asymmetries. This study aimed to review the surgical approach and evaluate the postoperative results of a case with right hemifacial microsomia and anotia. CASE REPORT: This is the case of a 35-year-old female patient who, after multiple graft operations in the right mandible due to hemifacial microsomia, was operated for auricle reconstruction. Initially, a three-dimensional custom made Medpor (porex) was used, covered by the superficial temporal fascia. Subsequently, due to inflammation and partial exposure of this porous polyethylene implant (PPI), a temporalis muscular flap along with the deep temporal fascia were used as a salvage operation. Ten months later, the patient underwent deep plane face lift combined with open rhinoplasty. Lefort I osteotomies and transoral lip lengthening through a transection of the levator nasi septi muscle were also performed. Ear helix reconstruction was completed with a rotation scalp flap after tissue expansion. The patient had an uncomplicated postoperative course with an aesthetically acceptable result. CONCLUSION: As a congenital disorder, hemifacial microsomia is present at birth and successful reconstruction is of fundamental importance for the smooth integration of these individuals into society. The multiple asymmetries, the affected topographic area of the face, as well as the onset in neonatal age constitute a challenge for reconstructive surgery.


Asunto(s)
Síndrome de Goldenhar , Procedimientos de Cirugía Plástica , Humanos , Femenino , Adulto , Procedimientos de Cirugía Plástica/métodos , Síndrome de Goldenhar/cirugía , Resultado del Tratamiento , Asimetría Facial/cirugía , Colgajos Quirúrgicos
7.
Oral Maxillofac Surg ; 2024 Jul 02.
Artículo en Inglés | MEDLINE | ID: mdl-38954312

RESUMEN

PURPOSE: This scoping review explores various parameters of the mandible in progressive facial asymmetry (FA) in hemifacial microsomia (HFM) patients, highlighting its relationship with sex, population, and age group. METHODS: The review was based on a comprehensive search of PubMed, EBSCOhost, and Web of Science. Eligible studies that met the inclusion criteria form part of the selection study. The included studies were appraised using screening and quantitative criteria of mixed-method appraisal tools. The authors utilised a pre-set data extraction form to obtain information from the included studies. RESULTS: Eleven studies met the inclusion criteria. The mandible parameters used were angular measurements, chin point, ramal height, body length, and total length. There was no relationship between FA and sex in HFM patients in the included studies. Most of the studies were comprised of European participants (55%), followed by Americans (36%) and Chinese (9%). The age groups included in the selected studies were categorised as dentition age (18%), early-to-middle childhood (18%), and varied ages (64%). The data presented in this review only pertains to the anomalous characteristics recorded on the affected side in HFM patients. No concomitant control data was recorded in this review. CONCLUSION: An assessment of the included studies revealed that FA does not increase with age in HFM. Hence, FA is non-progressive in HFM patients. This information is relevant to diagnosing and managing HFM patients. More reports are needed on the progression of FA in HFM patients.

8.
J Clin Med ; 13(12)2024 Jun 12.
Artículo en Inglés | MEDLINE | ID: mdl-38929967

RESUMEN

Background: The current high standards in orthognathic surgery demand surgical solutions that are both ⁠ functionally ⁠ effective and aesthetically pleasing. Our approach offers one for enhanced stability, attractiveness, and nerve protection ⁠ with improved accessibility ⁠ in the majority of orthognathic scenarios ⁠ compared to an inverted L osteotomy. Methods: A case series is presented to illustrate the application and outcomes of HSSO, an optimised approach that combines the advantages of a transoral inverted L osteotomy with specific enhancements and increased versatility, ⁠ with accessibility and exposure similar to a BSSO. Results: HSSO as a completely transoral technique, demonstrate the ability to perform significant counterclockwise rotations of the mandible, eliminating the need for trocars or skin incisions. We experinced high postoperative stability when HSSO was performed in conjunction with a three-piece LeFort 1 osteotomy on a dynamic opposing arch. In comparison to an inverted L approach, we postulated that HSSO offers advantages in stability, due to the increased segmental overlap of the proximal and distal segments of the mandible. This approach is designed to enhance the safety of the inferior alveolar nerve compared to traditional sagittal split methods. Furthermore, HSSO represents an alternative to total joint replacement in select cases of idiopathic condylar resorption and is effective for correcting mandibular asymmetries while maintaining jawline aesthetics. This is achieved through the manipulation of the mandibular angle, ramus height, and inferior border without creating a step deformity in the soft tissue. Conclusions: The outcomes of HSSO highlight its capacity to deliver predictable, functional, and aesthetically pleasing results, offering a viable alternative to more traditional orthognathic techniques.

9.
Sleep Breath ; 2024 Jun 06.
Artículo en Inglés | MEDLINE | ID: mdl-38842644

RESUMEN

PURPOSE: Patients with syndromic hemifacial microsomia (SHFM) are at risk of obstructive sleep apnea (OSA). The aim of the study was to describe the prevalence of OSA and its management, especially in patients with Goldenhar syndrome (GS). METHODS: The respiratory polygraphies and clinical management of 15 patients, aged 2 to 23 years, evaluated at a national reference center, were analyzed. RESULTS: Four (27%) patients had no OSA, 4 (27%) had mild OSA, and 7 (46%), of whom 5 were ≤ 2 years old, had severe OSA. None of the patients had central apneas. Only one patient had alveolar hypoventilation, and another one had nocturnal hypoxemia. Two patients had severe OSA despite prior adenoidectomy or mandibular distraction osteogenesis. Median duration of follow-up was 3.5 years (range 0.5-9 years). None of the patients without OSA or with mild OSA at baseline respiratory polygraphy developed OSA during the follow up. Among the 7 patients with severe OSA, 3 required continuous positive airway pressure or noninvasive ventilation, and one patient required a tracheostomy. CONCLUSION: In conclusion, patients with SHFM are at high risk of severe OSA at any age, underlining the importance of systematic sleep studies to diagnose and evaluate the severity of OSA. Individualized treatment should be privileged, based on a careful examination of the entire upper airway, taking in account potential associated risk factors. All patients with SHFM should be managed by a pediatric expert multidisciplinary medical/surgical team until the end of post pubertal growth.

10.
Int J Mol Sci ; 25(9)2024 Apr 26.
Artículo en Inglés | MEDLINE | ID: mdl-38731925

RESUMEN

Hemifacial microsomia (HFM) is a rare congenital genetic syndrome primarily affecting the first and second pharyngeal arches, leading to defects in the mandible, external ear, and middle ear. The pathogenic genes remain largely unidentified. Whole-exome sequencing (WES) was conducted on 12 HFM probands and their unaffected biological parents. Predictive structural analysis of the target gene was conducted using PSIPRED (v3.3) and SWISS-MODEL, while STRING facilitated protein-to-protein interaction predictions. CRISPR/Cas9 was applied for gene knockout in zebrafish. In situ hybridization (ISH) was employed to examine the spatiotemporal expression of the target gene and neural crest cell (NCC) markers. Immunofluorescence with PH3 and TUNEL assays were used to assess cell proliferation and apoptosis. RNA sequencing was performed on mutant and control embryos, with rescue experiments involving target mRNA injections and specific gene knockouts. CDC27 was identified as a novel candidate gene for HFM, with four nonsynonymous de novo variants detected in three unrelated probands. Structural predictions indicated significant alterations in the secondary and tertiary structures of CDC27. cdc27 knockout in zebrafish resulted in craniofacial malformation, spine deformity, and cardiac edema, mirroring typical HFM phenotypes. Abnormalities in somatic cell apoptosis, reduced NCC proliferation in pharyngeal arches, and chondrocyte differentiation issues were observed in cdc27-/- mutants. cdc27 mRNA injections and cdkn1a or tp53 knockout significantly rescued pharyngeal arch cartilage dysplasia, while sox9a mRNA administration partially restored the defective phenotypes. Our findings suggest a functional link between CDC27 and HFM, primarily through the inhibition of CNCC proliferation and disruption of pharyngeal chondrocyte differentiation.


Asunto(s)
Síndrome de Goldenhar , Pez Cebra , Animales , Pez Cebra/genética , Humanos , Masculino , Femenino , Síndrome de Goldenhar/genética , Síndrome de Goldenhar/patología , Apoptosis/genética , Cresta Neural/metabolismo , Secuenciación del Exoma , Proliferación Celular/genética , Fenotipo , Mutación , Técnicas de Inactivación de Genes
11.
Maxillofac Plast Reconstr Surg ; 46(1): 18, 2024 May 11.
Artículo en Inglés | MEDLINE | ID: mdl-38733452

RESUMEN

BACKGROUND: Hemifacial microsomia is characterized by the hypoplasia of the mandible and temporomandibular joint, involving a variety of abnormalities of the craniofacial area. Since it gradually worsens as patients grow, it is necessary to understand the characteristics of facial bone growth and facial deformity in hemifacial microsomia patients in order to determine appropriate treatment timing and treatment methods. MAIN BODY: Appropriate classification of hemifacial microsomia would facilitate accurate diagnosis, selection of treatment methods, and prognosis prediction. Therefore, in this article, we review previously published hemifacial microsomia classification and provide an overview of the growth of the facial skeleton and the characteristics of hemifacial microsomia-related facial deformities. The OMENS system is the most comprehensive classification method based on the characteristics of hemifacial microsomia deformity, but it needs to be improved to include malar/midface abnormalities and nerve involvement. In hemifacial microsomia, growth is progressing on the affected side, but to a lesser degree than the unaffected side. Therefore, surgical intervention in growing patients should be performed selectively according to the severity of deformity. CONCLUSION: Understanding growth patterns is important to develop appropriate treatment protocols for correcting asymmetry in adult patients and to minimize secondary anomalies in growing patients.

12.
Int J Oral Maxillofac Surg ; 53(9): 746-751, 2024 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-38631989

RESUMEN

This study aimed to document the prevalence, severity, and risk factors of velopharyngeal dysfunction (VPD) in craniofacial microsomia (CFM) and to analyse differences in VPD-related speech characteristics between CFM patients without cleft lip and/or palate (CL/P), CFM patients with CL/P, and CL/P patients without CFM (control). A total of 223 patients with CFM were included, of whom 59 had a CL/P. Thirty-four CFM patients had VPD, including 20 with a CL/P. VPD was significantly more prevalent in CFM with CL/P than in CFM without CL/P (odds ratio (OR) 4.1, 95% confidence interval (CI) 1.9-8.7; P < 0.001). Multivariate logistic regression showed a significant association between CL/P and VPD in CFM patients (OR 7.4, 95% CI 2.1-26.3; P = 0.002). The presence of VPD was not associated with sex, the laterality or severity of CFM. Speech problems related to VPD appeared to be similar among the different groups (CFM without CL/P, CFM with CL/P, CL/P without CFM). As 15.2% of all CFM patients and 8.5% of CFM patients without CL/P had VPD, it is proposed that all patients with CFM, with or without CL/P, should be assessed by a speech and language therapist for the potential risk of VPD.


Asunto(s)
Síndrome de Goldenhar , Insuficiencia Velofaríngea , Humanos , Masculino , Femenino , Estudios Retrospectivos , Insuficiencia Velofaríngea/fisiopatología , Niño , Síndrome de Goldenhar/complicaciones , Factores de Riesgo , Adolescente , Prevalencia , Preescolar , Trastornos del Habla/etiología , Trastornos del Habla/fisiopatología , Adulto , Fisura del Paladar/complicaciones
13.
Am J Med Genet A ; 194(8): e63594, 2024 08.
Artículo en Inglés | MEDLINE | ID: mdl-38553895

RESUMEN

Craniofacial microsomia (CFM) primarily includes specific head and neck anomalies that co-occur more frequently than expected. The anomalies are usually asymmetric and affect craniofacial features; however, there are frequently additional anomalies of variable severity. Published prenatal findings for CFM are limited. This study contributes 11 cases with CFM and their anomalies identified prenatally. Cases born between January 1, 1997 and December 31, 2019 with CFM were abstracted from the Alberta Congenital Anomalies Surveillance System, which is a population-based program ascertaining congenital anomalies for livebirths, stillbirths, and termination of pregnancies for fetal anomalies. There were 11 cases ascertained with prenatal findings including facial anomalies: one each with left cleft lip, right microtia, and bilateral microphthalmia. Two cases had vertebral anomalies. In addition, anomalies of the kidneys, brain, heart, and radial ray were identified. Six (55%) had a single umbilical artery, five (45%) were small for gestational age, and three (27%) were from a twin pregnancy that were discordant for anomalies. Four (36%) overlapped another proposed recurrent constellations of embryonic malformation condition. This study describes prenatal findings for 11 cases with CFM. Comparable to prior published cases, there were recurring anomalies on prenatal imaging, including anomalies of the brain, eye, heart, kidneys, and radial ray, which may aid in the prenatal diagnosis of CFM.


Asunto(s)
Síndrome de Goldenhar , Humanos , Femenino , Embarazo , Masculino , Síndrome de Goldenhar/genética , Síndrome de Goldenhar/epidemiología , Síndrome de Goldenhar/diagnóstico , Síndrome de Goldenhar/patología , Alberta/epidemiología , Diagnóstico Prenatal , Adulto , Recién Nacido , Labio Leporino/epidemiología , Labio Leporino/patología , Labio Leporino/genética , Labio Leporino/diagnóstico , Labio Leporino/diagnóstico por imagen , Ultrasonografía Prenatal , Anomalías Múltiples/epidemiología , Anomalías Múltiples/genética , Anomalías Múltiples/patología , Anomalías Múltiples/diagnóstico
14.
Oral Maxillofac Surg Clin North Am ; 36(3): 303-315, 2024 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-38462395

RESUMEN

Pediatric temporomandibular joint (TMJ) disorders represent a broad range of congenital and acquired diagnoses. Dentofacial deformities, including facial asymmetry, retrognathism, and malocclusion, commonly develop. Compared with adult TMJ conditions, pain and articular disc pathology are less common. Accurate diagnosis is paramount in planning and prognostication. Several specific considerations apply in preparation for skeletal correction, including timing in relation to disease progression and growth trajectory, expectation for postcorrection stability, reconstructive technique as it applies to expected durability and need for future revision, management of occlusion, and need for ancillary procedures to optimize correction. This article reviews common conditions and treatment considerations.


Asunto(s)
Trastornos de la Articulación Temporomandibular , Humanos , Trastornos de la Articulación Temporomandibular/cirugía , Trastornos de la Articulación Temporomandibular/diagnóstico , Trastornos de la Articulación Temporomandibular/terapia , Niño
15.
BMC Ophthalmol ; 24(1): 81, 2024 Feb 22.
Artículo en Inglés | MEDLINE | ID: mdl-38388885

RESUMEN

BACKGROUND: To report a case of a 4-year-old patient with Goldenhar syndrome. CASE PRESENTATION: The author presents a rare case report involving a 4-year-old boy with multiple malformations. A comprehensive examination showed that the patient primarily had a limbal dermoid. He also has bilateral microtia and ear canal deformities. The skull CT scan and spine X-ray showed Maxillofacial Abnormalities and scoliosis. Whole Exome Sequencing revealed potential gene variations related to microtia. Although certain circumstances prevented us from initiating follow-up treatment for the patient, we have provided a detailed account of the diagnostic methodologies used for this condition. CONCLUSIONS: Goldenhar syndrome is a congenital condition, predominantly presenting as sporadic cases. Its diagnosis and management typically necessitate the involvement of multiple disciplines, including otolaryngology and craniofacial surgery. The syndrome encompasses a variety of craniofacial features, which can facilitate early diagnosis and guide subsequent therapeutic interventions.


Asunto(s)
Anomalías Múltiples , Microtia Congénita , Neoplasias del Ojo , Síndrome de Goldenhar , Masculino , Humanos , Preescolar , Síndrome de Goldenhar/diagnóstico , Síndrome de Goldenhar/genética , Microtia Congénita/diagnóstico , Anomalías Múltiples/diagnóstico , Tomografía Computarizada por Rayos X
16.
J Craniomaxillofac Surg ; 52(4): 469-471, 2024 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-38369394

RESUMEN

The aim of this study is to present a sequential strategy of soft-tissue, non-osteogenic distraction with a novel device, followed by microvascular bony reconstruction for severe cases of mandibular hypoplasia. The case of a 21-year-old woman with Goldenhar syndrome is presented, whose mandible remained severely hypoplastic despite previous attempts at distraction and was not suitable for further osteogenic distraction. Soft tissue deficiency and pin track scarring prevented free fibular transfers. A personalized distractor, anchored to the cranium and the mandibular symphysis, was designed to expand the soft tissues while allowing for physiological temporomandibular joint (TMJ) movement without compression forces. Internal distractors were placed along the osteotomies to prevent condylar luxation. After completion of the soft tissue distraction, the native mandible was resected except for the condyles and reconstructed with two free fibula flaps. This report represents the proof of concept of a sequential approach to severe lower face soft-tissue and bone deficiency, which preserves TMJ function and avoids the transfer of poorly matched skin to the face.


Asunto(s)
Síndrome de Goldenhar , Micrognatismo , Osteogénesis por Distracción , Procedimientos de Cirugía Plástica , Femenino , Humanos , Adulto Joven , Adulto , Síndrome de Goldenhar/diagnóstico por imagen , Síndrome de Goldenhar/cirugía , Mandíbula/diagnóstico por imagen , Mandíbula/cirugía , Mandíbula/anomalías , Micrognatismo/cirugía , Cráneo/cirugía
17.
Cleft Palate Craniofac J ; 61(4): 584-591, 2024 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-36628544

RESUMEN

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.


Asunto(s)
Síndrome de Goldenhar , Humanos , Síndrome de Goldenhar/diagnóstico por imagen , Estudios Retrospectivos , Mandíbula , Hueso Temporal/diagnóstico por imagen , Tomografía Computarizada por Rayos X , Asimetría Facial
18.
Orthod Craniofac Res ; 27(1): 15-26, 2024 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-37533308

RESUMEN

Hypoplastic asymmetry due to hemifacial microsomia (HFM) often represents the most difficult reconstruction in the craniomaxillofacial clinic. Although autogenous grafts are generally used for temporomandibular joint reconstruction (TMJR), the use of TMJR prostheses is not well established. The aim of this review was to identify, collect and analyse the use of extended TMJR (eTMJR) prostheses in patients with HFM, describing clinical features, surgical procedures and postoperative complications. Online searches of all major databases were performed according to PRISMA guidelines. All studies with HFM patients treated with the eTMJR prostheses were included. Descriptive statistics were used for data analysis. A total of 19 studies, including 08 case studies, 06 case series and 05 retrospective cohort studies, met the inclusion criteria, where a total of 42 HFM patients were reported from 18 countries, mostly from the United States (05; 26%). Fifteen of the 42 cases (~36%) were male. The mean ± SD (range) age of patients in all studies was 19.79 ± 5.81 (9-36) years. The mean ± SD (range) of patient follow-up was 41.30 ± 35.50 (6-136) months. A total of 5 (10.6%) patients were implanted with bilateral eTMJR prostheses. The Pruzansky classification was used in 18 (~89.5%) studies, OMENS classification in 01 (~5%) study, whereas no classification was reported in one study. Only 01 (7.1%) study had documented the eTMJR classification for the prosthesis used. In growing patients with or without a history of failed autogenous tissues, TMJR prostheses may provide a viable alternative. Randomized studies with large cohorts are warranted to validate these preliminary results.


Asunto(s)
Síndrome de Goldenhar , Prótesis Articulares , Trastornos de la Articulación Temporomandibular , Adolescente , Adulto , Femenino , Humanos , Masculino , Adulto Joven , Asimetría Facial , Síndrome de Goldenhar/cirugía , Síndrome de Goldenhar/complicaciones , Prótesis Articulares/estadística & datos numéricos , Estudios Retrospectivos , Articulación Temporomandibular/cirugía , Trastornos de la Articulación Temporomandibular/cirugía , Niño
19.
J Craniomaxillofac Surg ; 52(1): 40-47, 2024 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-38129190

RESUMEN

The study analyzed vascular variations in microtia associated with hemifacial microsomia (HFM). A retrospective analysis was conducted on 47 patients with microtia and HFM, who underwent computed tomography angiography between November 2011 and May 2022. The vascular course and branching supplying the TPF were analyzed. Craniometric measurements were conducted to determine the horizontal distance from the porion and fronto-zygomatic suture (F-Z suture) to the vessels. On the affected side, the TPF was primarily supplied by either the superficial temporal artery (STA) or the postauricular artery-originated STA (Po-STA). The Po-STA (n = 29) was more prevalent than the STA (n = 18), and mostly exhibited a single frontal branch (n = 20). Craniometric analysis revealed that the Po-STA was closer to the porion, ear vestige, and F-Z suture than the STA on the non-affected side. Furthermore, a significant correlation was observed between the severity of mandibular hypoplasia and presence of Po-STA variation (Cramer's V = 0.498, p = 0.005). Microtia associated with HFM exhibits vascular variations in the TPF - in particular, a unique Po-STA variation. The Po-STA is prone to injury during ear reconstruction because of its proximity to the external auditory canal and ear vestige. Surgeons should be cautious of these anatomical variations for safer ear reconstruction procedures, and utilize preoperative imaging for meticulous planning.


Asunto(s)
Microtia Congénita , Síndrome de Goldenhar , Humanos , Síndrome de Goldenhar/diagnóstico por imagen , Síndrome de Goldenhar/cirugía , Síndrome de Goldenhar/complicaciones , Estudios Retrospectivos , Asimetría Facial/complicaciones , Microtia Congénita/cirugía , Fascia/trasplante
20.
Innov Surg Sci ; 8(3): 159-183, 2023 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-38077487

RESUMEN

Objectives: This article describes the many anomalies within and outside the head and neck of hemifacial microsomia (HFM). Methods: The OMENS+ classification system is described with particular reference to the mandibular features classified by Pruzansky and modified by Kaban. The application of virtual surgical planning (VSP) to HFM, largely in children, is described and taken through to aspects indicated in maturity. Results: VSP is demonstrated with clinical cases examples in HFM patients for (1) grafts and flaps replacing missing parts of the zygomatic bone, temporo-mandibular joint and mandible, (2) distraction osteogenesis for lengthening of the mandibular ramus, advancement of the mandibular body, widening of the face and simultaneous mid-face and mandibular rotation, (3) implants and correction of microtia for bone anchored ear prostheses, (4) correction of microtia by autogenous ear construction, and (5) end stage rotational bimaxillary osteotomies. Conclusions: 3D virtual and physical planning is a valuable adjunct to the treatment of this complex condition.

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