Caracterización de los pacientes con defectos bucomaxilofaciales atendidos en el Policlínico Universitario Julio Antonio Mella / Characterization of the patients with oral-maxillofacial defects attended in the University Polyclinic Julio Antonio Mella

Fundamento: las lesiones del macizo cráneofacial existen desde la evolución misma del hombre, lo que ha traído por consiguiente la necesidad de crear medios para enmascarar los defectos o restaurar el órgano perdido. Objetivo: caracterizar los pacientes con defectos bocomaxilofacial. Métodos: se realizó un estudio observacional, descriptivo y transversal desde enero del 2015 hasta mayo de 2018 en pacientes con defectos bucomaxilofaciales atendidos en el departamento de prótesis estomatológica del Policlínico Universitario Julio Antonio Mella de la Provincia Camagüey. El universo lo constituyeron 13 pacientes mayores de 20 años de edad de ambos sexos remitidos del Hospital Universitario Manuel Ascunce Domenech y Hospital Provincial Docente Oncológico María Curie.Resultados: respecto a los pacientes con defectos maxilofaciales rehabilitados según edad y sexo, fue más frecuente el sexo masculino y el grupo de 60 a 79 años. Según tipo y causa del defecto, fue superior el nasal y la oncológica con siete pacientes. Según tipo de defecto bucomaxilofacial y experiencia protésica, primó el defecto nasal, así como la experiencia protésica favorable en ocho pacientes. Conclusiones: los pacientes masculinos predominaron, junto con los mayores de 60 años. Los defectos bucomaxilofaciales más rehabilitados fueron los nasales y de origen oncológicos. Hubo un alto índice de experiencia favorable con el uso de las prótesis(AU)

Background: the injuries of the solid craniofacial exist from evolution of the man, which has brought therefore the need to create means to mask defects or to restore the lost organ. Objective: to characterize the patients with oral-maxillofacial defects.Methods: an observational, descriptive and transverse study was carried out from January, 2015 to May, 2018, in patients with oral-maxillofacial defects attended in the department dentistry prosthesis of the University Polyclinic Julio Antonio Mella of the city of Camagüey. The universe was constituted by 13 patients older than 20 years of age of both sexes cross-referred of the University Hospital Manuel Ascunce Domenech and Provincial University Oncological Hospital María Curie. Results: in relation to the patients with maxillofacial defects rehabilitated according to age and sex, prevailed the group 60 to 79 years, as well as the masculine sex. According to type and etiology of the defect, the nasal and the oncological was a superior with 7 patients respectively. According to type of defect oral-maxillofacial and prosthetic experience, the type of nasal defect had priority as well as the prosthetic favorable experience in 8 patients. Conclusions: the masculine patients predominated along with the older than 60 years old. The oral-maxillofacial defects most rehabilitated were the nasal and those of oncological causes. There was a tall index of favorable experience with the use of the prostheses (AU)

Aesthetic and Functional Rehabilitation of Postburn Maxillofacial Deformity.

Postburn maxillofacial deformities, although rare, are a significant cause of morbidity. Since contracture scars have a potential role in deforming the maxillofacial skeleton, the focus of treatment should be on growing patients, where scar contractures can drastically influence growth and morphology of the maxillofacial units. There are certain aspects of severe facial burns and deformities, especially of the jaws, that deserve sincere attention to overcome aesthetic and functional disabilities such as inability to masticate and incompetent lips. The purpose of this article, therefore, is to discuss these injuries, the mechanism of development of deformities of the jaws and associated structures and their management for better aesthetic, functional, and psychological health of patient. An interpretive clinical report is presented.

Maxillofacial morphological factors related to acceleration of maxillary growth attributed to facial mask treatment: a structural superimposition study.

BACKGROUND: Anatomical textbooks mention that the contact between the pterygoid process and the palatine's pyramidal process is not a "suture" but "conjugation.".The aim was to evaluate the maxillofacial morphological factor responding most to the orthopedic force of facial mask treatment, using the structural superimposition analysis. METHODS: Thirty-one girls with Angle Class III malocclusion treated using a facial mask (FM group) and 11 girls with pseudo-Class III malocclusion (pseudo-III group) were examined. Lateral cephalograms at pre- and posttreatment were analyzed to evaluate maxillofacial changes. Cephalometric structural superimposition analysis originating with Björk and Skieller was also performed. RESULTS: In the FM group, a multiple linear regression model showed that maxillary sutural growth was significantly associated with counter-clockwise rotation of the maxilla and treatment changes in the anteroposterior distance from the pterygomaxillary fissure to the maxillary anterior alveolus, not changes in the distance from the nasion to the maxillary anterior alveolus. CONCLUSIONS: Structural superimposition analysis showed that counter-clockwise rotation of the maxilla and changes in the distance from the pterygomaxillary fissure to the maxillary anterior alveolus responded most to the orthopedic force of facial mask treatment. The analysis implicated that the pterygoid fissure-palatine's pyramidal process conjugation responds most to facial mask treatment among maxillofacial sutures and conjugation, and that the difference in the response induces maxillary counter-clockwise rotation.

Dental and Maxillofacial Signs in Leri-Weill Dyschondrosteosis.

One of the most common causes of short stature is a defect of the short stature homeobox-containing (SHOX) gene, which is located in pseudoautosomal region 1 on the distal end of the short arm of chromosomes Xp22.33 and Yp11.32. More than 300 different mutations in the SHOX gene responsible for short stature syndrome have been described. The phenotypic expression of SHOX haploinsufficiency is remarkably varied. The 3 typical clinical presentations, from least to most severe, are idiopathic short stature without skeletal malformations, Leri-Weill dyschondrosteosis (LWD), and Langer mesomelic dysplasia, which is believed to represent the homozygous form of LWD. Despite a higher prevalence in women, suggesting the potentiating action of high estrogen levels on the effects of SHOX deficiency, the syndrome was initially believed to have an autosomal pattern of inheritance. In reality, heterozygous SHOX mutations can be transferred from the Y to the X chromosome and vice versa. This phenomenon is called "the jumping SHOX gene" and corresponds to a pseudoautosomal dominant inheritance. LWD is characterized by mesomelic short stature and Madelung deformity defined by an upward and medial displacement of the radial joint surface, which restricts range of motion. Less specific dysmorphic signs associated with LWD, such as short hands and feet, scoliosis, or muscular hypertrophy, have been described. When reviewing the dental and maxillofacial signs, only limited and summary data (micrognathia and high arched palate) have been published in the literature. This report presents a case of LWD that highlights many other noteworthy dental and maxillofacial signs that are important to clearly identify and appropriately treat.

Feasibility and Accuracy of Noncontact Three-Dimensional Digitizers for Geometric Facial Defects: An In Vitro Comparison.

PURPOSE: To evaluate the feasibility and accuracy of noncontact three-dimensional (3D) digitization systems for capturing facial defects. MATERIALS AND METHODS: A stone model of a facial defect was digitized using high-accuracy industrial computed tomography as a reference scan. The model was also scanned using four different types of noncontact 3D digitizers: a laser beam light-sectioning technology with camera system and three different stereophotogrammetry systems. All 3D images were reconstructed with corresponding software and saved as standard triangulated language (STL) files. The 3D datasets were geometrically evaluated and compared to the reference data using 3D evaluation software. Kruskal-Wallis H tests were performed to assess differences in absolute 3D deviations between scans, with statistical significance defined as P < .05. RESULTS: The four noncontact 3D digitization systems were feasible for digitizing the facial defect model, although the median 3D deviation of the four digitizers varied. There was a significant difference in accuracy among the digitizers (P < .001). CONCLUSION: Digitization of facial defect models using various noncontact 3D digitizers appears to be feasible and is most accurate with laser beam light-sectioning technology. Further investigations assessing digitization of facial defects among patients are required to clinically verify the results of this study.

[How I explore a dento-maxillofacial dysmorphosis]. / Comment j'explore une dysmorphose dento-maxillo-faciale.

Dento-maxillofacial dysmorphoses represent a considerable area of maxillofacial surgery. Their incidence has constantly been on the rise since the beginning of the century. This can be explained by variations in the food and society habits.We will first discuss the various causes (congenital and environnemental) and the pathophysiology of these disorders. Then, we will separate them according to the skeletal and Angle's classifications. We will examine the predictive criteria of these dysmorphoses from the youngest age and consider their clinical and cephalometrical diagnosis. We will envisage their functional and aesthetic consequences and expose the preventive options. Finally, we will describe the orthodontic and surgical treatment available today and conclude that surgery remains the gold standard by restoring the conventional axis.

Les dysmorphoses dento-maxillo-faciales (DDM) occupent un volet important de la chirurgie maxillo-faciale. Elles constituent un problème en constante progression depuis le début du siècle, s'expliquant notamment par des modifications des habitudes alimentaires et sociétales (1). Nous discuterons les diverses causes, congénitales et environnementales, ainsi que les aspects physiopathologiques de ces affections et ferons le point sur leur classification grâce à la classe squelettique et la classe dentaire d'Angle. Nous discuterons des critères prédictifs de ces dysmorphoses dès le plus jeune âge ainsi que de leur diagnostic clinique et céphalométrique. Nous détaillerons leurs conséquences esthétiques et fonctionnelles et exposerons les mesures préventives visant à éviter leur développement. Enfin, nous évoquerons la thérapeutique à la fois orthodontique et chirurgicale que nous pouvons actuellement proposer pour restaurer les fonctions masticatoires, phonétiques et respiratoires tout en harmonisant le tiers inférieur de la face.

Ellis-Van Creveld syndrome: dental management considerations and description of a new oral finding.

Ellis-Van Creveld is a rare syndrome with characteristic dental and orofacial findings. Dental management of patients with Ellis-Van Creveld syndrome can be complicated by the associated skeletal and cardiac abnormalities. Here, we present the dental and orofacial findings in a patient with Ellis-Van Creveld syndrome, describe a new oral finding, and discuss the dental management considerations.

Amniotic bands as a cause of congenital anterior staphyloma.

BACKGROUND: Congenital anterior staphyloma is a rare, complex malformation syndrome of the anterior segment. Only a few reports on associated systemic malformations have been published. We herein present a rare manifestation of congenital anterior staphyloma (CAS) combined with amniotic band disruption syndrome (ABS). PATIENT AND METHODS: Shortly after birth, a massive enlargement of the left eye was observed in a female child. Furthermore, an extensive bilateral congenital cleft lip and cleft alveolar ridge with oblique facial cleft extending into the left medial canthal region, coloboma(s) of the left eyelids, extensive adhesions between lids and eye bulb, as well as circumferential grooves, clubfeet, and terminal transverse defects in both hands and feet were present. Due to severe progression of eye bulb protrusion with thinning of the sclera, enucleation of the left eye was performed at the age of 3 years in order to prevent complications including perforation of the globe and with the aim of improving cosmetic aspects. RESULTS: Histopathological examination of the enucleated eye disclosed findings typical of congenital anterior staphyloma, including massive corneal staphylomatic deformation with superficial vascularization and elapsed corneoscleral margin, destruction of Bowman's layer, absence of Descemet's layer, corneal endothelium, and angle structures. The lens was only partially formed, and had mainly dissolved. The neural retina appeared normal. The optic nerve disc revealed a pronounced excavation. Facial clefts, lid colobomas, congenital constriction bands, and amputation of distal limbs match ABS. This malformation complex develops in early pregnancy, probably prior to 35 days post conception. CONCLUSION: This is the first report on an association of these two rare complex congenital malformations, congenital anterior staphyloma and amniotic band syndrome. The anterior staphyloma was unilateral, and related to facial clefts and lid coloboma in the area adjacent to the anterior staphyloma. Furthermore, the systemic deformities are clearly due to the amniotic bands, and the timing of the development of both complex malformations seems to be similar. All findings suggest that the presence of amniotic bands is a causative factor for all observed abnormalities including anterior staphyloma.

Binder phenotype in mothers affected with autoimmune disorders.

OBJECTIVE: To report four foetal cases of the Binder phenotype associated with maternal autoimmune disorders. PATIENTS AND METHODS: In three mothers with autoimmune diseases, 2D and 3D ultrasonographic measurements were made on four foetuses with the Binder profile, and were compared with postnatal phenotypes. RESULTS: The Binder phenotype can be detected in early pregnancy (14.5 WG). All foetuses had verticalized nasal bones and midfacial hypoplasia. Punctuate calcifications were found in almost all the cases. No specific maternal auto-antibody has been associated with foetal Binder phenotype. CONCLUSION: Since the Binder phenotype can be diagnosed at ultrasound examination during pregnancy, it is important to establish the underlying cause so as to assess the foetal prognosis. This study stresses the importance of systematic checks for maternal autoimmune disease in cases of prenatally diagnosed Binder phenotypes.

Oral rehabilitation with implant-based prostheses of two adult patients treated for childhood rhabdomyosarcoma.

BACKGROUND: Rhabdomyosarcoma is the most common malignant tumor in the nasal and paranasal sinus area at childhood. Multimodal treatment for this disorder has severe side effects due to normal tissue damage. As a result of this treatment, facial growth retardation and oral abnormalities such as malformation of teeth and microstomia can cause esthetic and functional problems. CASE REPORTS: Two cases are presented of patients with severe midfacial hypoplasia and reduced oral function as a result of treatment of rhabdomyosarcoma of the nasopharyngeal and nasal-tonsil region. With a combined surgical (osteotomy, distraction osteogenesis, implants) and prosthetic (implant-based overdenture) treatment, esthetics and function were improved.

Kocher Debre Semelaigne syndrome and associated orofacial aspects: report of a case.

Myopathy and muscular pseudohypertrophy associated with severe congenital hypothyroidism has been described as Kocher Debre Semelaigne syndrome, which is a rare disorder with only a few previously published reports. However, none of these reports have described the orofacial aspects of this syndrome. Here we report one such case, which may provide valuable information to specialists in oral healthcare regarding the associated orofacial aspects.

Goldenhar syndrome: clinical features with orofacial emphasis

OBJECTIVES: Goldenhar syndrome (GS) is a relatively common developmental disorder characterized by craniofacial anomalies in association with vertebral, cardiac, renal, and central nervous system defects. This paper describes GS features with special emphasis on oral characteristics. MATERIAL AND METHODS: The clinical features of 6 patients with GS aged 3 months to 12 years are described, and a brief review of the literature about this genetic disorder is presented. RESULTS: All patients demonstrated the classical triad of GS, including mandibular hypoplasia resulting in facial asymmetry, ear and/or eye malformation, and vertebral anomalies. In addition, renal and gastrointestinal abnormalities were observed in 2 patients. Regarding the oral involvement, 2 patients presented cleft lip and palate, and 1 patient had temporomandibular joint malformation. Malocclusion was found in all patients. CONCLUSION: Our orofacial findings correlate with the reported cases in the literature, and point out that after diagnosis GS patients need to be examined for systemic abnormalities.

Goldenhar syndrome: clinical features with orofacial emphasis.

OBJECTIVES: Goldenhar syndrome (GS) is a relatively common developmental disorder characterized by craniofacial anomalies in association with vertebral, cardiac, renal, and central nervous system defects. This paper describes GS features with special emphasis on oral characteristics. MATERIAL AND METHODS: The clinical features of 6 patients with GS aged 3 months to 12 years are described, and a brief review of the literature about this genetic disorder is presented. RESULTS: All patients demonstrated the classical triad of GS, including mandibular hypoplasia resulting in facial asymmetry, ear and/or eye malformation, and vertebral anomalies. In addition, renal and gastrointestinal abnormalities were observed in 2 patients. Regarding the oral involvement, 2 patients presented cleft lip and palate, and 1 patient had temporomandibular joint malformation. Malocclusion was found in all patients. CONCLUSION: Our orofacial findings correlate with the reported cases in the literature, and point out that after diagnosis GS patients need to be examined for systemic abnormalities.

Paciente con malformaciones en cara, boca y dedos: síndrome orofaciodigital tipo I o síndrome de Papillon-Léage Psaume / Patients With Malformations of the Face, Mouth, and Fingers: Type I Orofaciodigital or Papillon-Léage Psaume Syndrome

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Facial metrics in children with corticotrophin-producing pituitary adenomas suggest abnormalities in midface development.

BACKGROUND: Tumors of the hypothalamic-pituitary unit have been linked to genetic syndromes that are associated with midfacial abnormalities. AIM: We hypothesized that mutations of genes that affect the development of the face (and consequently of the anterior pituitary) may be present in children with ACTH-producing pituitary adenomas, and if this is true then facial measurements would be different from those predicted by parental features. METHODS: We studied 20 children with corticotropinomas and a control group and their parents. All facial measurements were expressed according to standard deviation scores. RESULTS: Significant differences were seen between the children with pituitary adenomas and their parents for vertical facial height measures: nasal length (p < 0.001), lower facial height (p < 0.03) and overall facial height (p < 0.01). CONCLUSION: We conclude that some of the indices of midline craniofacial development, in particular those affecting the vertical axis, are different in children with corticotroph adenomas producing ACTH.

Modulation of diabetes-induced palate defects by maternal immune stimulation.

Maternal diabetes can induce a number of developmental abnormalities in both laboratory animals and humans, including deformities of the face and palate. The incidence of birth defects in newborns of women with diabetes is approximately 3 to 5 times higher than among nondiabetics. In mice, nonspecific activation of the maternal immune system can reduce fetal abnormalities caused by various etiologies including hyperglycemia. This study was conducted to determine whether nonspecific maternal immune stimulation could reduce diabetes-induced palate defects and orofacial clefts. Female ICR mice were immune stimulated before induction of hyperglycemia with Freund's complete adjuvant (FCA), granulocyte-macrophage colony-stimulating factor (GM-CSF), or interferon-gamma (IFNgamma). Streptozocin was used to induce hyperglycemia (26-35 mmol blood glucose) in females before breeding. Fetuses from 12 to 18 litters per treatment group were collected on Day 17 of gestation. Palate width and length were measured, and the incidence of orofacial clefts was determined. Palate length and width were both decreased by maternal hyperglycemia. Maternal immune stimulation with GM-CSF or FCA limited the degree of palate shortening from the hyperglycemia. Each of the three immune stimulants attenuated significant narrowing of the palate. Rates of orofacial clefts were not significantly different between treatment groups. Palatogenesis is a complex process driven by cellular signals, which regulate cell growth and apoptosis. Dysregulation of cellular signals by maternal hyperglycemia can result in fetal malformations. Maternal immune stimulation may prevent dysregulation of these signaling pathways thus reducing fetal malformations and normalizing palate growth.

Bone and maxillofacial abnormalities in thalassemia: a review of the literature.

Thalassemia is an inherited blood disorder due to an imbalanced globin chain synthesis leading to anaemia that requires regular blood transfusions and iron-chelating therapy. Of all organ failures secondary to iron deposit, and all the complications, heart failure still represents the first cause of death. Osteopenia and osteoporosis can be considered important causes of morbidity in a population whose lifespan is getting longer, with a strong impact on their quality of life. Authors have reported mainly bone, oral and maxillofacial abnormalities and relative complications, especially in terms of traumatic risk, in patients affected by thalassemia. As examples, this study reports bone modifications in three clinical cases; one of these was also complicated with a femoral fracture, surgically treated with the same criteria of metastatic femoral bone disease. More research on this topic is necessary for the prevention of several complications caused by this disease, and to carefully plan dental or traumatologic operations.

Immunohistochemical identification of TGF-beta1 at the maxillaries in growing Sprague-Dawley rats and after muscle section.

Growth factors are currently being extensively studied in the literature to ascertain their role during maxillofacial development. Taking into account that few investigations refer to the functions of growth in the maxillaries, our aim was to identify the TGF-beta1 immunohistochemical expression pattern in the maxillaries of growing rats. A secondary aim was to identify this pattern after orofacial function inhibition by muscle section. In the palate and the mandibular symphysis and body, we found that bone was formed through an endomembranous pathway with intense TGF-beta1 staining inside chondroid cells during the maximum development stages. At the midpalatal suture and the mandibular symphysis and condyle, endochondral ossification was detected with an intense expression of TGF-beta1 inside the chondrocytes when major growth occurred. After the muscle had been sectioned, at the mandible the maturation process was accelerated, this change being transitory until muscular function was recovered. However, at the palate, the intervention caused a greater disturbance of the growing pattern, which did not recover normality.