Early Surgical Intervention of Congenital Syngnathia.

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

Diagnóstico precoz de la estrechez del maxilar superior en la dentición mixta / Early diagnosis of the maxillary´s narrowness in the mixed dentition

Las compresiones del maxilar superior son alteraciones transversales por discrepancia óseo-dentaria. .En la dentición mixta temprana puede establecerse la estrechez mediante índices que relacionan el tamaño dental con el ancho transversal del arco dentario. Se analizaron 33 modelos superiores de niños en el tercer período de desarrollo clínico según Barnett. Se estableció la estrechez realizando el análisis métrico del arco dental mediante el índice de Pont. Se describió la anomalía de posición anterior según las siguientes variables: A) Apiñamiento dentario: 1-Escalón de los dientes anterosuperiores; 2-Rotación mesial de los cuatro incisivos; 3-Rotación mesial de los incisivos centrales y rotación distal de los incisivos laterales y 4-Rotación distal de los incisivos centrales. B) Ausencia de apiñamiento: 1-Reabsorción atípica y 2- Ausencia de reabsorción atípica. Las anomalías de posición y/o reabsorciones atípicas anteriores en este período de desarrollo clínico se asocian a estrechez transversal del maxilar superior Siendo la malposición más frecuente la rotación mesial de los cuatro incisivos, siguiendo las reabsorciones atípicas y el escalón de los dientes anterosuperiores, luego la rotación mesial de los incisivos centrales y distal de los laterales y por último la rotación distal de los incisivos centrales (AU)

Inside the traverse alteration of the maxillary the compressions are described as uni or bilateral where an imbalance is settled down between the dental size and the size of the maxillary causing alterations in the position of the teeth. In the early mixed teething the narrowness of the maxillary can be settled by means of indexes that relate the dental size with the traverse width of the dental arch. Thirty models of the maxillary of children according to Barnett's third development period were analyzed. The metrical analysis of the dental arch form was carried out through Pont's index. The theoretic values were compared with the real ones establishing the deviations of the norm that is to say the narrow nest. Out of the 30 cases analyzed, 40% presented mesial rotation of the 4 incisors; 27% showed a stop of the front teeth; 27% atypical reabsorption; 20% mesial rotation of the central incisors and distal rotation of the lateral incisors and the 10% presented a distal rotation of the central incisors. With regard to the front atypical discrepancy 36.66% of the cases had a discrepancy above 6 mm and the 23.33% below 3 mm (AU)

Syngnathia-Congenital Maxillomandibular fusion: Case Report and Literature Review.

Congenital maxillomandibular fusion is a rare disorder of the craniofacial region. Maintenance of the airway and difficulty in feeding are the main concerns for such patients. We performed a PubMed search to identify reported cases of syngnathia. With reference to the site of fusion and corresponding anomalies, the available studies were reviewed. The maxillomandibular fusion can be anterior fusion, unilateral or bilateral fusion, and complete fusion. The condition can be an isolated problem or associated with a syndrome. The condition requires immediate attention because it can be fatal. A case of isolated unilateral syngnathia, with the detailed review of the published data, is reported.

Marcus Gunn Jaw-Winking Syndrome: A Comprehensive Review and Report of Four Novel Cases.

PURPOSE: To report 4 cases of Marcus Gunn jaw-winking synkinesis (MGJWS) in the absence of ptosis. METHODS: A retrospective review of patients with MGJWS and congenital ptosis was compiled from the public and private subspecialty adult and pediatric oculoplastic practices of the 2 senior authors (AAM, TGH). Clinical data collected on patients with MGJWS included visual acuity, stereopsis, ocular motility, side of jaw-wink, presence or absence of ptosis, levator function, clinical photographs and videos, and any management undertaken. RESULTS: A total of 848 cases of congenital ptosis were seen. Of these, there were 72 consecutive patients with MGJWS, of which 4 cases (5.6%) had no ptosis. One patient had bilateral MGJWS, with ptosis on one side only. The authors found the incidence of MGJWS in our study population to be 8.5% of all congenital ptosis cases. When the authors excluded syndromic, neurogenic, and myopathic causes of congenital ptosis, the incidence was 12.1%. In addition, the authors found a trend toward females with simple congenital ptosis and MGJWS being affected on the left side, however this was not statistically significant. CONCLUSIONS: The authors report the first case series of MGJWS in the absence of ptosis within the second largest series ever reported. These findings may help further our understanding of the etiology behind MGJWS.

Syngnathia Between the Palate and Mouth Floor, Cleft Palate, and Funnel Chest.

Syngnathia is a rare malformation involving soft tissue and/or bony adhesions between the maxilla and mandible. Less than 40 patients have been reported in the literature. Here the authors report a 6-month-old infant diagnosed as syngnathia of the palate and mouth floor combined with cleft palate and funnel chest in the Department of Oral and Maxillofacial Surgery at Henan Provincial People's Hospital in January 2015. The authors discussed and evaluated the diagnostic and treatment difficulties on surgical and anesthetic procedure. There is no standard treatment protocol, but early treatment is necessary to improve airway functions and infant feeding, and to support proper nutrition for the growth of maxillofacial region.

Changes in Soft Tissue Measurements After Mandibular Distraction Osteogenesis.

BACKGROUND: Mandibular distraction osteogenesis is the process of generating new bone in a gap between 2 bone segments in response to the application of graduated tensile stress across the bone gap. These distraction forces applied to bone also create tension in the surrounding soft tissues resulting in simultaneous expansion of the functional soft tissue matrix. AIM: To investigate and evaluate the changes in facial soft tissue following mandibular distraction osteogenesis and their relationship with the changes in bone movements. MATERIALS AND METHODS: Mandibular elongation of 13 patients was done by the use of bilateral distraction osteogenesis in 10 patients and unilateral distraction osteogenesis in 3 patients, soft tissue evaluation was done by measuring 4 facial lines (tragus-commissure of mouth, lateral canthus-commissure of mouth, medial canthus-commissure of mouth, and lower lip length). The measurements were done by the use of measurement tape preoperatively and after the consolidation period. RESULTS: Soft tissue changes were observed in all patients. There was highly significant increase in tragus-commissure line in all patients, significant increase in lateral canthus-commissure line in 10 patients, and significant increase in medial canthus-commissure line and lower lip length in 9 patients. The lines' relation with the overjet was highly significant in right side of tragus-commissure line, lateral canthus-commissure line, medial canthus-commissure line, and lower lip length, and significant in left side of tragus-commissure line, lateral canthus-commissure line, and medial canthus-commissure line CONCLUSION:: Distraction osteogenesis has recognizable effect on facial soft tissue profile in addition to its effect on skeletal structure.

Malposition of Teeth and Jaws in Patients with Congenital Superior Oblique Palsy.

BACKGROUND: Patients with congenital superior oblique palsy tend to adopt a head tilt to the contralateral side to maintain binocular single vision. It has long been recognised that facial asymmetries may be caused by a head tilt. The aim of this study was to describe the effect of habitual head tilt due to congenital superior oblique palsy on dental occlusion. PATIENTS AND METHODS: The study was designed as a descriptive cohort study. Ten patients with congenital superior oblique palsy (3 female, 7 male; mean age 51.7 (y) ± 15.8 SD, ranging from 19 to 69 (y)) underwent orthodontic examination. Orthodontic findings and values for vertical, torsional and horizontal deviation measured with the Harms tangent screen and stereopsis using a random dot test were compared. RESULTS: Three orthodontic parameters were found to correlate significantly or at least as trend with orthoptic parameters. Midline deviation of the upper jaw to the face (rho = 0.623; p = 0.054) and anterior positioning of upper first molar in the sagittal plane (rho = 0.594; p = 0.07) correlate with the vertical deviation; overbite correlates with horizontal deviation measured in the primary position (rho = 0.768; p = 0.016). CONCLUSIONS: In this small study, three orthodontic parameters correlated with orthoptic findings in patients with congenital superior oblique palsy. Further studies are needed to establish whether congenital superior oblique palsy is more frequent in patients exhibiting abnormal values of these orthodontic parameters.

Defeito ósseo de Stafne û relato de dois casos / Stafne bone defect û report of two cases

A cavidade óssea de Stafne, também conhecida como pseudocisto de Stafne ou cavidade óssea idiopática, tem gerado muitos conflitos nos diagnósticos entre os profissionais de Odontologia. A lesão supracitada faz parte de um conjunto de lesões radiolúcidas e assintomáticas descritas por Edward Stanfe (1942) e está topograficamente localizada entre o ângulo mandibular e o primeiro molar inferior, abaixo do canal alveolar inferior, sendo denominada de depressão óssea mandibular lingual posterior. O tratamento consiste apenas em acompanhamento radiográfico e tomografia...

The bone cavity Stafne, also known as pseudocyst of Stafne or idiopathic bone cavity, has generated many conflicts regarding diagnosis among dental professionals. The aforementioned lesion is part of a set of radiolucent and asymptomatic lesions described by Edward Stanfe in 1942 and is topographically located between the mandibular angle and the lower first molar, under the inferior alveolar channel, and it is called posterior lingual mandibular bone depression. Treatment consists only of radiographic follow-up and cone-beam tomography...

Keratocystic Odontogenic Tumour Mimicking Lateral Periodontal Cyst: A Diagnostic Dilemma.

The Keratocystic Odontogenic Tumor is a developmental cyst derived from the enamel organ or from the dental lamina. It is a benign, multicystic, intraosseous tumor of odontogenic origin, with a characteristic lining of parakeratinized stratified squamous epithelium and has a potential for aggressive, infiltrative behavior and recurrence. Keratocystic Odontogenic Tumors have a predilection for males and occurs mainly in the second and third decade of life, most commonly in the mandible, mostly in the posterior body, the angle and the ascending ramus. It extends in the intramedullary space making it difficult to diagnose at an early stage. It is regarded as a distinctive entity because of its characteristic histology, proliferation kinetics and behavior. Main in 1970 described, collateral variant of Keratocystic Odontogenic Tumor, which presents adjacent to the roots of the teeth usually in the mandibular premolar region and radiologically is indistinguishable from the lateral periodontal cyst and gingival cyst.

Severe Agnathia-Otocephaly Complex: Surgical Management and Longitudinal Follow-up From Birth Through Adulthood.

Agnathia-otocephaly complex (AOC) is characterized by mandibular hypo- or aplasia, ear abnormalities, microstomia, and microglossia. Though rare and often fatal, this is the first report detailing various reconstructive strategies beyond infancy as well as longitudinal follow-up into adulthood. All patients with AOC treated at our institution over a 30 year period were reviewed. Four patients were identified, one with agnathia, one with micrognathia. Two males with nanognathia (defined as a symphyseal remnant without body nor ramus) were also included. The mean follow-up was 17 years. All four underwent perinatal tracheostomy and gastrostomy-tube placement. Commissuroplasties were typically performed before 3 years of age and repeated as necessary to allow for oral hygiene. Mandibular reconstruction was most successful with rib between ages 3 and 8, after which time, free fibula transfer was utilized. Due to some resoprtion or extrusion, all patients underwent repeated bone grafting procedures. Tissue expansion of the neck was used to restore the lower third of the face, but was most successful in the teenage years. At last follow-up of the eldest patients, one was in college while another was pursuing graduate education. AOC need not be a fatal nor untreatable condition; a reasonable quality of life can be achieved. Although the lower-facial contour may be improved, and a stoma created, the lack of musculature make deglutition virtually impossible with current therapies. Just as transplantation has emerged as a modality for facial restoration following severe trauma, so too may it be a future option for congenital deformities.

A origem dos toros palatinos e mandibulares: bases para a sua interpretação clínica / Origin of torus palatinus and torus mandibularis: basis for clinical interpretation

Os toros palatinos e mandibulares são distúrbios do desenvolvimento do tipo anomalia de forma, com manifestação tardia no crescimento e maturação dos maxilares. Os casos familiares e a persistência dos toros com a idade e em desdentados lhe atribuem uma origem genética e dificultam a sua interpretação como uma resposta adaptativa à sobrecarga oclusal, ao bruxismo e outros fatores externos: os toros não são hiperplasias ou hipertrofias adaptativas. Os toros são protuberâncias ósseas sem cápsula fibrosa, o que os diferencia dos osteomas e lhes tira a natureza neoplásica, mesmo que benigna, especialmente porque também não apresentam crescimento contínuo e sem controle por parte do organismo. O tamanho dos toros se estabiliza no final do crescimento dos maxilares, ao redor dos 22 a 24 anos. Os toros são constituídos de osso normal, do ponto de vista funcional e estrutural, e podem ser utilizados como sítio de origem de transplante ósseo autógeno para outros locais, ou como sede de implantes osseointegráveis, se houver conveniências clínicas para tais procedimentos...

Torus palatinus and torus mandibularis are developmental anomalies of shape that become late manifest during growth and maturation of the jaws. Family history and the constant presence of tori with age and among edentulous patients attach a genetic origin to them and hinder their clinical interpretation as an adaptive response to occlusal overload, grinding and other external factors: tori are not a form of hyperplasia or adaptive hypertrophy. They are bone protuberances without a fibrous capsule, which differentiates them from osteomata and frees them from a neoplastic nature, albeit benign, especially because they do not grow continuously and uncontrollably in one’s organism. The size of tori stabilizes by the end of maxillary growth, at around the age of 22 to 24 years. They are composed of normal bone, from a functional and structural perspective, and might be used as autograft harvesting site or osseointegrated implant placement site within clinically acceptable conditions...

Incidence of bilateral Marcus Gunn jaw-wink.

This case report describes the unusual finding of bilateral Marcus Gunn jaw-wink. Only a few bilateral jaw-wink patients have been published as case reports. The authors dispute this notion as portrayed in the literature that a bilateral wink is a rare entity. A thorough review of prior case series on congenital ptosis and jaw-wink demonstrates that bilateral involvement is more common than previously thought. A supplemental video shows the classic action of lateral pterygoid contraction causing ipsilateral eyelid retraction.

Blepharoptosis.

Blepharoptosis of the upper eyelid is a common condition among patients presenting for oculoplastic surgery. Although there are many types of ptosis, the two most frequent clinical cases are simple congenital ptosis in young patients and senile ptosis in adults. Patient examination is important to distinguish these from other more infrequent types of ptosis, such as those neurogenic, myogenic and posttraumatic. These latter cases may require specific therapeutic strategies. Ptosis can usually be corrected surgically. The article discusses guidelines for the choice of intervention. Various different surgical techniques are also presented, together with results and complications.

Veracidad del ángulo ANB de Steiner en pacientes con clase II esqueletal, comparándolo con el análisis de Wits / Veracity of the Steiner´s ANB angle in patients with skeletal class II, compared with the Wits analysis

El objetivo de este estudio fue eterminar la veracidad del ángulo ANB en pacientes con clase II esqueletal, comparando este ángulo con el análisis de Wits. El trabajo se llevó a cabo sobre una población de 201 individuos de ambos sexos con dentición permanente y mixta, que asistieron al posgrado de la carrera de especialización de Ortodoncia de la Facultad de Odontología de La Plata, a quienes se les tomaron teleradiografías laterales de cráneo en oclusión habitual, sobre las cuales se realizaron cálculos y trazados cefalométricos. Se evaluaron el ángulo ANB de Steiner, donde se determinó la relación sagital de la base apical y del análisis de Wits, donde se determinó la relación maxilomandibular en el plano sagital. Los resultados obtenidos demostraron que no hay una coincidencia en el diagnóstico entre el ángulo ANB y la medición lineal de Wits.

Recurrent agnathia-otocephaly caused by DNA replication slippage in PRRX1.

Agnathia-otocephaly is a rare craniofacial malformation complex that is caused by de novo heterozygous and biallelic mutations in PRRX1 in two unrelated babies, respectively. We studied the PRRX1 gene in a non-consanguineous Indonesian female infant who was diagnosed prenatally with severe retrognathia (bilateral Pruzansky type III). Her older affected brother died shortly after birth and had agnathia-otocephaly. A c.266_269dupAAAA frameshift mutation in the poly A tract in PRRX1 was identified in the proband while her father only had an inframe duplication (c.267_269dupAAA) of the adenosine trinucleotide residue. Expression of both mutations in COS7 cells showed loss of function of the frame shift mutation only. Results of SNP genotyping coupled with recurrence of this novel mutation in this family are consistent with a paternally derived germline mosaicism rather than autosomal recessive inheritance as predicted by the family history. Severe retrognathia (bilateral Pruzansky III) and agnathia-otocephaly represent a spectrum of craniofacial malformations in this family.

Is the coexistence of intraoral synechia and cleft palate anomaly a sequence?

Intraoral synechia is a rarely seen intraoral anomaly. As a result of intraoral synechiae, fusion of the palatal shelves may be prevented because of the abnormal interposition of the tongue. Hence, cleft palate anomaly accompanies intraoral synechiae in many patients. The main problem for these patients is the inadequate oral opening for feeding. Flexible nasopharyngeal examination before intubation may help the anesthetist for simple excision of synechiae in the newborn period. In this article, a newborn who had a congenital synechia between the mandible and the maxilla has been presented, and the etiopathogenesis of intraoral synechia and the importance of flexible nasal endoscopy before endotracheal intubation are discussed.

[Use of comprehensive 3D-head model for diagnostics of anomalies of jaw-dental system].

Angular and linear parameters, characterizing symmetry of occlusional plane position from the left and the right sides with regard to poL-n-poR reference plane, and also relative to PL plane were determined and offered using complex "Head-Dentitions" 3D-model.

"See-saw" Marcus Gunn syndrome.

The authors report an unusual case of Marcus Gunn syndrome in an adult patient, characterized by classical Marcus Gunn on one side and inverse Marcus Gunn on the other side. The patient exhibited a "see-saw" kind of eyelid movement on opening and closing the mouth and on moving the jaw from side to side. This clinical entity has been reported very rarely. A similar but etiologically different acquired condition seen commonly in adults, Marin Amat syndrome, is also discussed.

[Prognostic factors and visual outcome for open globe injuries with intraocular foreign bodies]. / Prognostische Faktoren und klinische Ergebnisse in der Behandlung von offenen Augenverletzungen mit intraokularen Fremdkörpern.

AIMS: The aim of this study was to evaluate the clinical features as well as the visual results and determine the prognostic factors after the removal of retained intraocular foreign bodies (IOFBs). PATIENTS AND METHODS: At our centre we conducted a non-randomised, non-comparative retrospective analysis of records of 31 patients with intraocular foreign bodies treated using pars plana vitrectomy and other conventional surgical techniques during a 3-year period between January 2006 and December 2008. Using the OTS classification for ocular injuries (United States Eye Injury Registry [USEIR]), we evaluated the visual outcome and various pre-, intra- and postoperative clinical factors. RESULTS: Almost all patients (96.8 %) were male with a mean age of 32.29 years ± 13.38 SD (SD = standard deviation). The most common cause of ocular injury was hammering (58 %), in 27 cases the IOFB was magnetic. Follow-up ranged from 1 to 34 months with a mean of 4.5 months. In 30 eyes, the IOFBs could be removed during the first surgery. The average time between injury and surgery was 15.78 ± 23.45 SD hours for 27 patients (87.1 %). In 3 cases the intraocular foreign body (IOFB) was removed 7, 9, 14 days after the injury. In one case the IOFB was removed in a second surgery 40 days after the injury. In 23 cases we performed pars plana vitrectomy, for 8 patients we performed anterior segment surgery, in 21 cases the lens was also removed. At the time of surgery initial vitreous haemorrhage was present in 17 patients (54.8 %). OTS score for ocular injury was calculated as 2.45 ± 0.78 SD. Initial visual acuity was below 20 / 200 in 61.3 % of the cases. Subsequent to surgical treatment over a mean follow-up of 2.5 months, 25 patients (80.64 %) achieved a visual acuity of 20 / 200 or better (0.69 ± 0.35 SD). Best corrected visual acuity was significantly better for ocular injuries with a higher OTS score (p = 0.000095). Other significant factors for good visual outcome were good initial visual acuity (p = 0.002), the absence of vitreal haemorrhagies (p = 0.024) and small size of the IOFB (p = 0.043). Postoperative complications included retinal detachment (9.67 %), endophthalmitis (3.22 %) and PVR (3.22 %). CONCLUSION: Acceptable visual results could be achieved after the removal of posterior segment IOFBs by vitrectomy. A reliable classification of ocular injuries may provide significant information about the prognosis of injuries with intraocular foreign bodies.

Qualidade de vida em portadores de disfunção temporomandibular: um estudo transversal / Health-related quality of life in patients with temporomandibular: a cross sectional study

Introdução - A hiperatividade muscular pode desencadear dor e/ou cansaço muscular (fadiga) e estar associado à portadores de disfunção temporomandibular. O objetivo deste estudo foi classificar os portadores de disfunção temporomandibular (DTM) e correlacionar com o impacto na qualidade de vida (QV). Material e Métodos - Foi realizado um estudo transversal em uma amostragem de 302 participantes, com faixa etária entre 17 a 50 anos, que foram divididos em dois grupos (80 pacientes do sexo masculino e 80 do sexo feminino) e randomizados através de programa de geração de números aleatórios. O diagnóstico e classificação da DTM, em ambos os grupos, foi obtido pelo índice anamnético de Fonseca e a qualidade de vida através dos questionários da versão brasileira do SF36. Resultados - Os resultados mostraram que 38.75% dos participantes não apresentaram DTM sendo que a grande parte dos participantes analisados possuíram disfunção leve (41.87%) e moderada (14.37%) com prejuízo principalmente das características mentais como, Vitalidade e Aspectos Emocionais, sendo que a Capacidade Funcional foi o domínio que se demonstrou mais preservado dentro da amostra. Conclusão - Existe uma influência direta do grau de DTM com a qualidade de vida dos participantes sintomáticos.

Introduction - The muscle hyperactivity can be associated to temporomandibular disorders(TMDs) and cause pain and/or muscle fatigue. The aim of this study was to classify TMD patients and provide a correlation with quality of life (QL) impact. Material and Methods - A cross-section study was conducted on a 302-participant sample with age level from 17 to 50 years old. The participants were randomly divided into two groups (80 male subjects and 80 female subjects). TMD diagnosis and classification were obtained for both groups by means of the Fonseca anamnesic index while the quality of life was obtained by means of the Brazilian version of the SF-36. Results -The data collected demonstrated that 38.75% of the participants did not present TMD. Most of the participants assessed presented mild (41.87%) and moderate TMD (14.37%) with deficits observed mainly on the mental characteristics, such as Vitality and Emotional Aspects. Functional Capacity was the most preserved domain within the sample. Conclusion - There is a direct influence of the TMD level on the quality of life of symptomatic subjects.