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.

A 7-year old female with arthrogryposis multiplex congenita, Duane retraction syndrome, and Marcus Gunn phenomenon due to a ZC4H2 gene mutation: a clinical presentation of the Wieacker-Wolff syndrome.

Background: Duane retraction syndrome and arthrogryposis multiplex congenita have an incidence of approximately 1:1500-1:3000 live births. However, the association of these two entities with a Marcus-Gunn might be a rare and, until now, under-recognized clinical presentation of the Wieacker-Wolff Syndrome.Patient and methods: We report a 7-year-old female with dysmorphic features, global developmental delay, arthrogryposis multiplex congenita (AMC), Duane retraction syndrome (DRS), and unilateral Marcus Gunn jaw winking.Results: Whole Exome Sequencing showed a de novo premature stop codon in ZC4H2. Extensive genetic and metabolic work was negative otherwise and Brain MRI showed delayed non-specific myelination abnormalities. She continues to have significant delays but does not have regression, seizures or other neurological complications. She has required a multidisciplinary approach for the management of her multiple contractures.Conclusion: This case confirms ZC4H2 as a cause of syndromic DRS and extends the ZC4H2 phenotype to include Marcus Gunn jaw winking.

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)

Marcus Gunn Jaw-Winking Syndrome: a Case Report.

INTRODUCTION: Marcus Gunn syndrome is a rare phenomenon with very less number of cases reported in literature. It may be congenital or acquired. AIM: The aim of this case report was to report the clinical characteristics of Marcus Gunn patient from our Clinic. CASE REPORT: A comprehensive opthalmologic examination, CDVA (corrected distance visual acuity), fundus examination and photography, was conducted in Marcus Gunn patient. Clinical findings of patient presented as - chin positioned slightly upwards, extraocular motility normal on both eyes, cover test with normal findings, pupillary examination normal on both eyes. Left upper eyelid was in a lower position than the right one. On right eye, rima interpalpebrarum was 9 mm with upgaze of 13mm. On the left eye, rima interpalpebrarum was 5 mm with upgaze of 6 mm, and with open mouth, left rima interpalpebrarum was 10 mm. Visual acuity on both eyes was 1.0. Cycloplegic refraction on both eyes was +0,75 diopters (D), and Lang test was normal. In the differential diagnosis of patients with ptosis, Marcus Gunn jaw winking syndrome should be considered especially if it improves during feeding, sucking, chewing, smiling or any kind of mouth movement. In case of ptosis always do the jaw test. Have the infant bottle-feed. An older child can chew gum. Have the patient open the mouth, move the jaw from side to side, or protrude the jaw forward. CONCLUSION: Address first to treatment of any amblyopia if present - eyeglasses, patching etc., or strabismus. Think twice before deciding to operate.

Marcus Gunn Jaw-Winking Syndrome Associated with Morning Glory Disc Anomaly.

Marcus Gunn jaw-winking syndrome (MGJWS) is a rare form of congenital blepharoptosis and one of the congenital cranial dysinnervation disorders (CCDD). In addition, morning glory disc anomaly (MGDA) is a congenital optic disc anomaly of unknown etiology. The present report is the first to describe an association between MGJWS and MGDA in an otherwise healthy 7-year-old boy. He also had counting finger vision, anisometropia, esotropia, and monocular elevation deficiency in the same eye. In the literature, both MGJWS and MGDA have been reported to be associated with Duane retraction syndrome, a form of CCDD.

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.

Retrospective 25-year follow-up of treatment outcomes in Angle Class III patients : Success versus failure.

OBJECTIVES: Despite recommendations for early treatment of hereditary Angle Class III syndrome, late pubertal growth may cause a relapse requiring surgical intervention. This study was performed to identify predictors of successful Class III treatment. MATERIALS AND METHODS: Thirty-eight Class III patients treated with a chincup were retrospectively analyzed. Data were collected from the data archive, cephalograms, and casts, including pretreatment (T0) and posttreatment (T1) data, as well as long-term follow-up data collected approximately 25 years after treatment (T2). Each patient was assigned to a success or a failure group. Data were analyzed based on time (T0, T1, T2), deviations from normal (Class I), and prognathism types (true mandibular prognathism, maxillary retrognathism, combined pro- and retrognathism). RESULTS: Compared to Class I normal values, the data obtained in both groups yielded 11 significant parameters. The success group showed values closer to normal at all times (T0, T1, T2) and vertical parameters decreased from T0 to T2. The failure group showed higher values for vertical and horizontal mandibular growth, as well as dentally more protrusion of the lower anterior teeth and more negative overjet at all times. In adittion, total gonial and upper gonial angle were higher at T0 and T1. A prognostic score-yet to be evaluated in clinical practice-was developed from the results. The failure group showed greater amounts of horizontal development during the years between T1 and T2. Treatment of true mandibular prognathism achieved better outcomes in female patients. Cases of maxillary retrognathism were treated very successfully without gender difference. Failure was clearly more prevalent, again without gender difference, among the patients with combined mandibular prognathism and maxillary retrognathism. Crossbite situations were observed in 44% of cases at T0. Even though this finding had been resolved by T1, it relapsed in 16% of the cases by T2. CONCLUSION: The failure rate increased in cases of combined mandibular prognathism and maxillary retrognathism. Precisely in these combined Class III situations, it should be useful to apply the diagnostic and prognostic parameters identified in the present study and to provide the patients with specific information about the increased risk of failure.

Dexmedetomidine-based monitored conscious sedation combined local anesthesia for levator resection in a 10-year-old child with Marcus Gunn jaw-winking synkinesis: A case report.

RATIONALE: Levator resection has become a routine procedure for patients with severe Marcus Gunn jaw-winking synkinesis (MGJWS). To optimize the surgical outcome, adult patients need to be kept awake, or easily aroused and responsive to verbal commands during the operation. However, levator resection is commonly performed under general anesthesia in pediatric patients. In the present case, we described a successful anesthetic protocol of conscious sedation with local anesthesia for levator resection in a child. PATIENT CONCERNS: A 10-year-old boy with MGJWS was admitted to our hospital and scheduled for levator resection. The patient was born through a normal delivery and had no previous history of allergy, no comorbidity, and no history of receiving anesthesia or operation. The laboratory tests of the patient were unremarkable. DIAGNOSES: The diagnosis of MGJWS was made by two experienced ophthalmologists. INTERVENTIONS: A 10-year-old boy with MGJWS was admitted to our hospital and scheduled for levator resection. The levator resection was performed under monitored conscious sedation with dexmedetomidine and local anesthesia. OUTCOMES: Patient with spontaneous breathing responded normally to verbal commands throughout the operation, and no adverse events occurred. The patient and ophthalmologist reported high satisfaction with anesthesia management. LESSONS: Dexmedetomidine-based monitored conscious sedation with local anesthesia is a feasible alternative to general anesthesia for levator resection in collaborative patients.

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.