A Filipino child with schinzel-giedion syndrome

@#Schinzel-Giedion syndrome is a rare condition characterized by dysmorphic features, neurologic features, urogenital abnormalities, and radiographic changes. The etiology has been traced to mutations in the SETBP1 gene. We report a Filipino patient with features suggestive of Schinzel-Giedion Syndrome and the first to be confirmed through molecular testing.

Paranasal augmentation using autologous costal cartilage as an adjunct to rhinoplasty / 中华医学美学美容杂志

Objective:To investigate the postoperative effect of paranasal concavity augment by block united with diced autologous costal cartilage.Methods:The data of 76 patients who were underwent costal cartilage rhinoplasty together with paranasal augmentation from January 2016 to December 2019 were analyzed retrospectively. The surgical technique was described in detail. Medical charts and operative records were reviewed to summary the complications. Patients′subjective satisfaction of the postoperative nasal appearance was self-evaluated with grading (1 worse, 2 no change, 3 improved, and 4 much improved). Cosmetic effects were evaluated by the measurements of the nasolabial angle and the distance between ACJ and intertragic notch preoperatively and postoperatively.Results:The postoperative follow-up duration was 6 to 34 months. Overall, functional and aesthetic outcome was satisfactory in most patients, and the mean score by the patients′self-evaluation was 3.4±0.5. Graft exposure, mobility, or significant resorption, pneumothorax or significant donor-site pain were not observed. The average nasolabial angle was changed from (78.2±13.2)° preoperatively to (89.8±10.2)° 6 months postoperative at the last follow-up ( t=152.00, P<0.01). And the distance from the alar-cheek junction to the left tragus was increased by 3.8±2.1 (2.2-6.1) mm. Conclusions:Costal cartilage rhinoplasty combined with augmentation of parasal area can improve parasal depression, with stable postoperative effect and fewer postoperative complications, and has a good effect for patients with normal occlusal relationship or mild abnormality.

Clinical effect of mid-face lifting combined with autologous compound fat transplantation in facial rejuvenation / 中华医学美学美容杂志

Objective:To investigate the clinical effect and safety of mid-face lifting combined with compound fat transplantation in facial rejuvenation.Methods:From October 2016 to May 2020, 26 patients with mid-facial aging were enrolled, including 10 males and 16 females, with an average age of (47±6) years (38-65 years). The facial superficial musculoaponeurotic system was stripped, folded and suspended through the temporal hairline incision to lift the middle of the face. Liposuction was performed and compound fat was prepared, in which structural fat was injected into the area marked with loss of facial volume before operation, and extravascular matrix component gel was injected into the static wrinkle dermis. Six and 12 months after operation, the global aesthetic improvement scale of the observer and the global aesthetic improvement scale of the patient were scored, and the postoperative complications and patients＇ satisfaction were counted.Results:At the end of 6 months, improvements in mid face were reported in all patients by blinded reviewers. 12 months after operation, 53.8% (14 cases) of patients had significant improvement in facial aging, 30.8% (8 cases) had moderate improvement, and 15.4% (4 cases) had slight improvement; 25 patients (96%) were satisfied with the postoperative results. Postoperative hemorrhage occurred in one patient, scar hyperplasia occurred in one patient, and no obvious complications occurred in the other patients.Conclusions:The method of midface lifting combined with compound fat transplantation can improve the facial soft tissue and supplement the facial missing capacity, improve the static wrinkles of the face, and comprehensively solve the aging problem of midface. It has a definite clinical effect, fewer complications, and is safe and reliable. It has definite clinical effect with less complications.

Características del Trauma Maxilofacial de Tercio Medio Atendido en Unidad de Emergencia del Complejo Asistencial Barros Luco

RESUMEN: El Trauma Maxilofacial (TMF) es una patología que presenta alta frecuencia en la actualidad, constituyendo un problema global de salud para los centros de alta complejidad y los equipos multidisciplinarios involucrados en su manejo y resolución. (neurología, maxilofacial, otorrinolaringología, oftalmología). Su etiología y epidemiología varía significativamente en la literatura. El objetivo de este estudio es caracterizar a los pacientes atendidos en un hospital del s ector sur de Santiago, Chile, identificando sus factores sociales y clínicos, clasificando los principales tipos de fractura a nivel de tercio medio facial. Se realizó un estudio observacional retrospectivo con los Datos de Atención de Urgencia (DAU) de los pacientes atendidos en el Servicio Dental de la Unidad de Emergencia del Complejo Asistencial Barros Luco (CABL), analizando el sexo, edad, comuna de procedencia, etiología del trauma, categorización según el "Emergency Severity Index", horario de ingreso y evaluación, estructuras óseas comprometidas de los pacientes con diagnóstico final de Fractura en Tercio Medio Facial. Se utilizaron estimadores puntuales de porcentaje, media y desviación estándar para representar las variables. Se calculó la significancia de relación de variables independientes, con valor de p < 0,05 estadísticamente significativo por el tamaño muestral. Población principalmente afectada corresponden a hombres versus mujeres (2.4:1), y entre segunda y tercera década de vida. Principal etiología identificada fue accidentes en el hogar (n = 73), seguidos de agresiones (n = 55). La mayoría de los pacientes ingresan con un riesgo C4 (n = 107) y C3 (n = 64). Fractura más frecuentemente es la nasal (46 %), seguidas de complejo orbitario (11.3 %) y arco cigomático (7.9 %). Es necesario mantener actualizada la epidemiología del TMF considerando la complejidad de su manejo temprano, morbilidades al mediano y largo plazo, con el objetivo de tener un rol activo en su prevención y tratamiento.

ABSTRACT: Maxillofacial Trauma (TMF) is a frequent pathology nowadays and presents a global health problem for specialized trauma centers and the multidisciplinary teams involved in its management and treatment (neurology, maxillofacial, otolaryngology, ophthalmology). The objective of this study is to characterize the patients seen in a hospital in the southern sector of Santiago, Chile, identifying their social and clinical factors, classifying the main types of fracture at the level of the facial third middle. A retrospective observational study was conducted with the Emergency Care Data (DAU) of the patients attended in the Dental Service of the Emergency Unit of the Barros Luco Assistance Complex (CABL), extracting the sex, age, commune of Origin, etiology of the trauma, categorization according to the "Emergency Severity Index" evaluation time and compromised bone structures of patients with diagnosis of Fracture in the Third Facial Mid-Section. Point estimators of percentage, mean and standard deviation were used to represent the variables. The significance of the relationship of independent variables was calculated, with a value of p <0.05 statistically significant for the sample size. Mainly affected population corresponded to men versus women (2.4: 1), and between the second and third decade of life. Main etiology identified were accidents at home (n = 33), followed by assaults (n = 55). Most of the patients entered with a risk of C4 (n = 107) and C3 (n = 64). The most frequent fracture was nasal (46%), followed by an orbital complex (11.3 %) and a zygomatic arch (7.9 %). It is necessary to keep the epidemiology of TMF up to date considering the complexity of its early management and morbidities in the medium and long term.

Application of multi-point and multi-level suspension and fixation through palpebral margin incision in midface lift / 中华整形外科杂志

Objective@#To explore the clinical effect of the multi-point, multi-level suspension as well as fixation using midfacial soft-tissue spaces for midface lifting.@*Methods@#A total of 65 patients with aging midface were admitted at the First Affiliated Hospital of Fujian Medical University from October 2017 to February 2019. Among them, 47 patients underwent primary blepharoplasty and midface lifting. Eighteen patients, including 5 patients with lower eyelid retraction or ectropion after blepharoplasty, underwent secondary midface lifting after blepharoplasty. The preseptal space was separated under orbicularis oculi muscle by palpebral margin incision. The orbicularis retaining ligament and the tear trough ligament were severed through preperiosteal plane. The preseptal space was connected with premaxillary space and prezygomatic space. The malar fat pad and superficial fascia were vertically suspended and fixed on the periosteum of infraorbital ridge by selected medial, middle and lateral points. The orbicularis oculi muscle was suspended and superolaterally fixed at lateral orbital periosteum. Therefore, the midface could be lifted by multi-point, multi-level suspension and fixation.@*Results@#All incision healed in the first stage. Eyelid separation occurred to 1 patient, around 1 month after the operation. Tarsal strip lateral canthoplasty was performed for repair. Local protuberance of lateral lower eyelid occurred to another patient shortly after the operation, but improved after 3 months by lid massaging. No other complication was observed in the rest of the cases. All patients were followed up for 1 to 8 months and the results were satisfactory.@*Conclusions@#It is simple and practicable to utilize midfacial soft-tissue spaces. This method could benefit patient of less trauma, bleeding, and complications, and good clinical effect. It is a good choice for rejuvenation of the midface, especially for secondary midface rejuvenation after blepharoplasty, or complicated with lower eyelid retraction and ectropion.

Aberrant growth of the anterior cranial base relevant to severe midface hypoplasia of Apert syndrome / 대한악안면성형재건외과학회지

BACKGROUND: A 9-year-old male showed severe defects in midface structures, which resulted in maxillary hypoplasia, ocular hypertelorism, relative mandibular prognathism, and syndactyly. He had been diagnosed as having Apert syndrome and received a surgery of frontal calvaria distraction osteotomy to treat the steep forehead at 6 months old, and a surgery of digital separation to treat severe syndactyly of both hands at 6 years old. Nevertheless, he still showed a turribrachycephalic cranial profile with proptosis, a horizontal groove above supraorbital ridge, and a short nose with bulbous tip. METHODS: Fundamental aberrant growth may be associated with the cranial base structure in radiological observation. RESULTS: The Apert syndrome patient had a shorter and thinner nasal septum in panthomogram, PA view, and Waters’ view; shorter zygomatico-maxillary width (83.5 mm) in Waters’ view; shorter length between the sella and nasion (63.7 mm) on cephalogram; and bigger zygomatic axis angle of the cranial base (118.2°) in basal cranial view than a normal 9-year-old male (94.8 mm, 72.5 mm, 98.1°, respectively). On the other hand, the Apert syndrome patient showed interdigitating calcification of coronal suture similar to that of a normal 30-year-old male in a skull PA view. CONCLUSION: Taken together, the Apert syndrome patient, 9 years old, showed retarded growth of the anterior cranial base affecting severe midface hypoplasia, which resulted in a hypoplastic nasal septum axis, retruded zygomatic axes, and retarded growth of the maxilla and palate even after frontal calvaria distraction osteotomy 8 years ago. Therefore, it was suggested that the severe midface hypoplasia and dysostotic facial profile of the present Apert syndrome case are closely relevant to the aberrant growth of the anterior cranial base supporting the whole oro-facial and forebrain development.

Progress of midfacial fat compartments and related clinical applications / 中国修复重建外科杂志

Objective: To review the research progress of midfacial fat compartments, and to thoroughly understand its current state of the anatomy and the aging morphologic characters of midfacial fat compartments, as well as the current status of clinical applications.

Application of expanded polytetrafluoroethylene in filling nasal base / 中华医学美学美容杂志

Objective To investigate the effect and safety of PTFE filling on correction of nasal basal treatment of midfacial sag.Methods Top of the vestibular sulcus groove was cut along the alveolar mucosa under direct maxillary periosteum,and then face inward in depression and the side line of the scope of subperiosteal separation from near to the medial nasal spine and the lateral side of pyriform aperture;range of separation was the same of both sides as marked before operation,slightly larger than the prosthesis;then the arc being carved,which was consistent with the nasal triangle expansion,was implanted into sub-periosteal site along pear-shaped arc edge of the hole placement and the prosthesis was fixed;as the appearance was satisfied,the incision was sutured.Results Clinical follow-up lasted for 6 months,and all the wound healing was well in primary intention without infection,implant exposure and other complications and facial expressions were natural.The filled results were satisfactory.Conclusions For correction of midfacial depression without need of osteotomy,nasal application of PTFE filling is simple with good efficacy and it is worth promoting.

Hard- and soft-tissue profiles of the midface region in patients with skeletal Class III malocclusion using cone-beam computed tomography multiplanar-reconstructed image analysis / 대한치과교정학회지

OBJECTIVE: This study examined cone-beam computed tomography (CBCT)-derived multiplanar-reconstructed (MPR) cross-sections to clarify the salient characteristics of patients with skeletal class III malocclusion with midface deficiency (MD). METHODS: The horizontal and sagittal plane intersection points were identified for middle-third facial analysis in 40 patients in the MD or normal (N) groups. MPR images acquired parallel to each horizontal plane were used for length and angular measurements. RESULTS: A comparison of the MD and N groups revealed significant differences in the zygoma prominence among female patients. The convex zygomatic area in the N group was larger than that in the MD group, and the inferior part of the midface in the N group was smaller than that in the MD group for both male and female patients. A significant difference was observed in the concave middle maxillary area among male patients. CONCLUSIONS: This study was conducted to demonstrate the difference between MD and normal face through MPR images derived from CBCT. Male patients in the MD group had a more flattened face than did those in the N group. Female patients in the MD group showed a concave-shaped lower section of the zygoma, which tended to have more severe MD. These findings indicate that orthognathic surgery to improve skeletal discrepancy requires different approaches in male and female patients.

Alteraciones del tercio medio facial en la infancia como patogénesis del síndrome de apnea obstructiva del sueño / Midface alterations in childhood as pathogenesis of obstructive sleep apnea syndrome

Resumen: El inicio de la respiración nasal marca un impulso genéticamente determinado para airear las cavidades de la cara o senos paranasales, que a su vez inician su crecimiento y forman el espacio útil transitable desde el punto de vista respiratorio durante el desarrollo del tercio medio facial. Considerando la evidencia de que la obstrucción de la vía aérea superior tiene un rol primordial en la patogénesis de los trastornos respiratorios del sueño, cualquier patología que cause dificultad permanente al flujo aéreo nasal durante la respiración llevará a un hipodesarrollo de la amplitud requerida en esta vía, disminuyendo la estimulación del crecimiento de las cavidades sinusales y alterando el desarrollo del tercio medio facial en su conjunto.

Abstract: The onset of nasal breathing sets a genetically determined impulse to aerate the face cavities or paranasal sinuses, which in turn initiate its growth creating the useful trafficable space for air during the development of the midface. Considering the evidence that the upper airway obstruction has a primary role in the pathogenesis of respiratory sleep disorders, any condition that causes a permanent difficulty to the nasal airflow during breathing will cause hypo-development of the required amplitude in this airway, reducing the growth stimulation of the sinus cavities and altering the development of the midface as a whole.

Clinical efficacy of the correction of midface depression with structural fat grafting / 中华医学美学美容杂志

Objective To explore the clinical effects of correction the midface depression.Methods From Aug.2010 to Aug.2015,185 cases of midface depression (aged from 18 to 61 years)were corrected by using structural fat grafting technique,the site and volume were placed as follows,tear trough 1ml,lid cheek groove 2 ml,midcheek groove 0.5 ml,anteriorcheek 4-8 ml,nasolabial fold 1-2 ml,nasal alar base 1-2 ml,evator labii superioris 1-2 ml,zygomaticus major 1-2 ml,buccinator 1-3 ml,and upper lip 3-5 ml.Results 185 cases were followed from 3 months to 3 years and the results were evaluated by visual analogue scales;156 cases of midface depression were corrected after the first structural fat grafting,the midface changed from concave to convex,and acquired prominent malar became more attractive;satisfaction of visual analogue scales were more than 80 ％;only 12 cases needed a second grafting.Conclusions The midface concavity can be corrected effectively by using structural fat grafting technique,the results are satisfied and the technique is safe,simple and effective.

A Case of Cranial Fasciitis in Midface / 대한이비인후과학회지

Cranial fasciitis is an uncommon subset of nodular fasciitis composed of spindle cells and myxoid stroma. This is not considered as a true neoplasm, as it occurs mostly in the scalp as a rapidly growing mass accompanied by adjacent bony structure destruction. There are few cases of cranial fasciitis reported in the literature; however, we experienced a case of a 2-year-old girl with swelling of midface. Subtotal resection was performed and the final pathological result confirmed cranial fasciitis. We report this rare case with a review of the literature.

Treatment of midfacial hypoplasia in syndromic and cleft lip and palate patients by means of a rigid external distractor (RED)

INTRODUCTION: Distraction Osteogenesis (DO) became an alternative for the treatment of severe craniofacial skeletal dysplasias. The rigid external distraction device (RED) is successfully used to advance the maxilla and all the maxillary-orbital-frontal complex (monobloc) in children, adolescents and adults. This approach provides predictable and stable results, and it can be applied alone or with craniofacial orthognathic surgical procedures. OBJECTIVE: In the present article, the technical aspects relevant to an adequate application of the RED will be described, including the planning, surgical and orthodontic procedures.

INTRODUÇÃO: a Distração Osteogênica (DO) tornou-se uma alternativa para o tratamento das displasias craniofaciais esqueléticas severas. O aparelho distrator externo rígido (RED) é utilizado com êxito para avançar a maxila e todo o complexo maxilar-orbital-frontal (monobloco) em crianças, adolescentes e adultos. Essa abordagem proporciona resultados previsíveis e estáveis, podendo ser aplicada isoladamente ou junto a procedimentos cirúrgicos ortognáticos craniofaciais. OBJETIVO: no presente artigo, serão descritos os aspectos técnicos pertinentes a uma adequada aplicação do RED, incluindo o planejamento, procedimentos cirúrgicos e ortodônticos.

Apert syndrome (Acrocephalosyndactyly): a case report.

Apert syndrome is named for the French physician. Eugene Apert in 1906 described the syndrome acrocephalosyndactylia. It is a rare autosomal dominant disorder characterized by craniosynostosis, craniofacial anomalies, and severe symmetrical syndactyly of the hands and feet (i.e. cutaneous and bony fusion refers to webbing of fingers and toes). Apert syndrome is characterized by the premature fusion of certain skull bones (craniosynostosis). This early fusion prevents the skull from growing normally and affects the shape of the head and face. In addition, a varied number of fingers and toes are fused together (syndactyly). Most cases of Apert syndrome are sporadic, may result from new mutations in the gene. The purpose of this paper is to report a case of Apert syndrome in a female fetus of 30 weeks with asymmetrical skull confirmed by prenatal ultrasonography. Pregnancy was terminated and fetus was submitted for detailed autopsy in anatomy dissection hall. The findings and review of literature were presented in this article.

Blepharoplasty and periorbital surgical rejuvenation.

The periorbital region forms the epicenter of facial aging changes and receives highest attention from physicians and patients. The concern about visual function, clubbed with the need for hidden incisions, makes the periocular region a highly specialized surgical area, most appropriately handled by an ophthalmic plastic surgeon. The article provides an overview of cosmetic eyelid and facial surgery in the periocular region. Common aesthetic surgical procedures as well as ocular side-effects of commonly performed periocular injections are discussed from the dermatologist's point of view.

Comprehensive treatment for mid-inferior facial rejuvenation / 中华医学美学美容杂志

Objective To summarize the experience of the integrated treatment on mid-face ageing for better cosmetic results.Methods A total of 56 cases were treated.With using subciliary approaches,the orbicularis oculi was resected to expose the orbital septum,the orbital fat relieved and orbital septum reseted.Hyaluronic acid was injected to some patients with obvious nasolabial fold after operation.Results 56 cases were all followed up from 6 to 26 months (11.8 months on average) postoperatively.The flabby tissue had been tightened and all got better effects.Conclusions Various combination of technologies can be applied to reach the purpose of rejuvenation by correcting the volume abnormality,resetting the tissue,elevating reasonably,and filling the facial depression.

Periorbital changes with aging

As we face a rapidly aging population in the Republic of Korea, the number of patients with the need to prevent or alleviate periorbital changes due to aging will grow. The periorbital changes that occur with aging comprise a dynamic process involving the aging of facial tissue and bony structures. Epidermal thinning and decreases in collagen cause the skin to lose its elasticity. Loss of fat, coupled with gravity and muscle pull, leads to wrinkling and the formation of dynamic lines. The aging process has also been shown to affect the facial bones. Multiple studies suggest that aging of the orbit and midface bones occurs primarily due to contraction and morphologic changes. This loss of bony volume and projection may contribute to an aged appearance. The effort to understand each patient's individual involutional changes, which differ by age and gender, is mandatory. Identifying the patient's personal needs and selecting the appropriate treatment accordingly is crucial for achieving the best outcome both for the clinician and the patient.

Anatomical Review of Rectus Abdominis Muscle Free Flap for the Oral and Maxillofacial Reconstruction

One-Stage Reconstruction for Midfacial Defect after Radical Tumor Resection

A serious midface defect involving resection of squamous cell carcinoma originating from the hard palate was treated by an unusual reconstructive strategy. After tumor resection, surgical reconstruction was accomplished in one stage using one free flap with one distant and local flap: a radial forearm flap to reconstruct the upper lip, a forehead flap to reconstruct the external nose, a cantilever calvarial bone graft to replace the nasal skeleton and a nasolabial flap and split thickness skin graft to cover the internal nasal lining. The rationale for this one-stage reconstruction and the problems associated with midfacial reconstruction after wide tumor excision are discussed.

Reconstruction of Cheek Defect with Facial Artery Perforator Flap

PURPOSE: To reconstruct the midface, local flaps such as nasolabial flaps have been frequently used. These local flaps, however, have the shortcomings of requiring a secondary operation or limitations in the movement of the flap. Thus, new methods have been developed. This paper reports a case wherein the basal cell carcinoma on the cheek was resected and the skin and soft tissue defect was successfully treated using a facial artery perforator flap. METHODS: A 68-year-old female consulted the authors on the basal cell carcinoma that developed on her cheek. The mass was fully resected and revealed a 2.3 x 2.3 cm defective region. Using a Doppler ultrasonography, the facial artery path was traced, and using a loupe magnification, the facial artery perforator flap was elevated and the defective region was covered with the flap. RESULTS: The flap developed early venous congestion, but it disappeared without any treatment. Six months after the surgery, the patient was satisfied with the postoperative result. CONCLUSION: The facial artery perforator flap has a thin pedicle. It offers a big arc of the rotation that allows free movement and one-stage operation. These strengths make the method useful for the reconstruction of the midface among other procedures.