Lacrimal drainage anomalies in Tessier cleft 3 with unilateral anophthalmos.

Bilateral Tessier cleft types 3 and 4 are rare and commonly involve the lacrimal drainage system owing to their anatomical location. Such clefts commonly present with associated ocular anomalies and include colobomatous eyelids, hypertelorism, microphthalmia, punctal or canalicular agenesis, and nasolacrimal duct obstruction or exstrophy. The current report presents an 18-month-old baby with bilateral Tessier cleft 3 with a unilateral anophthalmos, symmetrical eyelid colobomas, and lacrimal drainage anomalies. The lacrimal anomalies noted include small lacrimal sac with inferior canaliculus on the right side and upper and lower punctal and canalicular agenesis on the left side. Computed tomographic dacryocystography demonstrated unilateral lacrimal sac and bilateral maldevelopment of the bony nasolacrimal duct.

The complementary role of imaging modalities in Binder phenotype. Can prognostic factors of neonatal respiratory distress be found?

OBJECTIVE: To evaluate the complementarity between prenatal ultrasound, computed tomography, and MRI scans for fetuses with Binder phenotype. METHODS: We carried out a retrospective study from January 1, 2009, to June 30, 2018, of fetuses with Binder phenotype. Prenatal ultrasound (US) data were collected. A systematic survey of the entire skeleton was performed to look for associated abnormalities such as calcifications, brachytelephalangy, and spinal stenosis. Parents were systematically offered fetal skeletal computed tomography (CT). RESULTS: Thirteen cases were included. Two cases of perinatal respiratory distress (18%) were observed. Chondrodysplasia punctata was diagnosed from the presence of calcifications, especially of the proximal femoral epiphyses and tarsal bones, in five cases (38%) by US and in 10 cases (83%) by CT. Calcifications of the hyoid bone were detected by CT in three cases (25%) one of which had respiratory distress. Polyhydramnios was associated with the Binder phenotype in four cases (30%) one of which had respiratory distress. One single fetus had combined polyhydramnios and laryngeal calcifications, and he suffered from perinatal respiratory distress. CONCLUSION: An antenatal diagnosis of Binder phenotype is often associated with chondrodysplasia punctata. We recommend the use of fetal CT as a complement to US in this condition.

A review of syndromes associated with blue sclera, with inclusion of malformations of the head and neck.

Blue sclera is attributed to a diversity of mechanisms, mostly arising in genetic syndromes and, to a lesser extent, in nongenetic disorders and may occur as a side effect of medication intake. A literature search was conducted to establish a database of blue scleral associations. This article represents the most comprehensive assemblage of etiopathologies coincidental with blue sclera, comprising 66 genetic syndromes, 8 disorders, and 4 pharmacologically induced pigmentations. To increase the knowledge regarding the clinical significance of blue sclera, summaries of the systemic and oral maxillofacial comorbidities are provided. Recognition of the presence of blue sclera is important as it could prompt a timelier and more thorough diagnostic evaluation of possible systemic and dental malformations, potentially improving clinical outcomes.

Three-dimensional assessment of the temporal bone and mandible deformations in patients with congenital aural atresia.

OBJECTIVE: To investigate the deformations of temporal bone and mandible combined with congenital aural atresia. METHODS: A total of 158 patients with congenital aural atresia were included in the study. The raw CT data of the temporal bone was imported into MIMICS v 12 and threshold dissection, region growing and three-dimensional (3D) calculation were used to calculate 3D models. The 3D characteristics of the temporal bone and upper part of mandible were assessed. RESULTS: The tympanic part of the temporal bone was all undeveloped. Of all the patients included, 14 patients were found to have severe maxillofacial malformations. Among them, 2 cases have floating arch, 4 cases have interrupted arch, 5 cases have mandibular processes hypoplasia and 3 cases have interrupted arch combined with severe maxillary malformation. Ten of the 14 patients were suffered from dysplasia of the mastoid part of the temporal bone as well. CONCLUSION: Maxillofacial malformations may sometimes coexist with congenital aural atresia. Otolaryngologists should not neglect the coexisted maxillofacial malformations and give timely referral to maxillofacial surgeons.

Anticentromere antibody positive Ackerman's Syndrome with Granulomatous Anterior Uveitis.

Ackerman's Syndrome or Intersticial Granulomatous Dermatitis with Arthritis has been an issue of increasing number of reports in the last decade which had focused its heterogeneous cutaneous and rheumatologic expression besides the initial manifestations reported by Ackerman and his group. Granulomatosis anterior uveitis has not been previously described. Some patients are reported to have positive autoantibodies but association with anticentromere antibodies has not been previously described as well, to our knowledge. We report a new case of Ackerman Syndrome with cutaneous, articular and ocular involvement with positive anticentromere antibodies successfully treated with systemic steroids, methotrexate, hydroxychloroquine and cyclosporine. The ocular involvement and the association of anticentromere antibodies lead us to hypothesize that constellation of symptoms and autoimmune mechanisms of this uncommon multisystemic syndrome are yet to be clarified.

Absceso alveolar agudo en pacientes mayores de 19 años. Municipio Unión de Reyes / Acute alveolar abscess in patients elder 19 years old. Municipality Union de Reyes

Introducción: frecuentemente las infecciones de la cavidad bucal son el resultado del deterioro que provoca la caries con exposición de la pulpa dental. Una higiene dental descuidada o inadecuada, y complicaciones asociadas a enfermedades periodontales, son otras de las causas, que pueden incidir en la aparición de procesos infecciosos. Objetivo: caracterizar el comportamiento del absceso alveolar agudo en los pacientes mayores de 19 años en la consulta de urgencia estomatológica del poblado de Alacranes, Municipio Unión de Reyes. Materiales y Métodos: se realizó un estudio observacional descriptivo en pacientes mayores de 19 años, del área de Alacranes municipio Unión de Reyes. El universo de estudio estuvo constituido por los pacientes que acudieron a la consulta de urgencia estomatológica, de enero de 2011 a enero 2012. Con previo consentimiento informado la muestra la constituyó 135 pacientes, con patológica de absceso alveolar agudo, teniendo en cuenta las variables: edad, sexo, etiología y complicaciones. Los datos obtenidos fueron registrados en una base de datos y se procesaron estadísticamente. Resultados: los grupos de 20-29 años y el sexo masculino fueron los más afectados, con 38 % y 54.8 %, respectivamente. La causa fundamental fue la caries dental con un 91.8 %. La celulitis facial resultó la complicación más frecuente. Conclusiones: la etiología más usual del absceso alveolar agudo fue la caries dental. Los pacientes afectados que no recibieron una puntual asistencia sufrieron complicaciones (AU).

Introduction: frequently, oral cavity infections are due to the deterioration caused by caries with dental pulp exposition. An inadequate and careless dental hygiene and complications associated to periodontal diseases are other reasons the may lead to the occurrence of infectious processes. Objective: Characterise the behaviour of the absceso alveolar acute in the greater patients of 19 years in the query of urgency estomatológica of the populated of Alacranes, municipality Union de Reyes. Materials and Methods: a descriptive, observational study was carried out in patients elder 19 years old, in the health area Alacranes, municipality Union de Reyes. The studied universe was formed by the patients assisting to the dentist emergency consultation from January 2011 to January 2012. With previous informed consent, the sample was formed by 135 patients with acute alveolar abscess, taking into account the following variables: age, sex, etiology and complications. The obtained data were recorded in the database and statistically processed. Results: the most affected groups were the 20-29 years-old group and the male sex, with 38 % and 54.8 % respectively. Dental caries was the main cause with 91.8 %. Facial cellulitis was the most frequent complication. Conclusions: dental caries was the most usual etiology of the acute alveolar abscess. The affected patients who did not receive opportune health care suffered complications (AU).

Absceso alveolar agudo en pacientes mayores de 19 años. Municipio Unión de Reyes / Acute alveolar abscess in patients elder 19 years old. Municipality Union de Reyes

Introducción: frecuentemente las infecciones de la cavidad bucal son el resultado del deterioro que provoca la caries con exposición de la pulpa dental. Una higiene dental descuidada o inadecuada, y complicaciones asociadas a enfermedades periodontales, son otras de las causas, que pueden incidir en la aparición de procesos infecciosos. Objetivo: caracterizar el comportamiento del absceso alveolar agudo en los pacientes mayores de 19 años en la consulta de urgencia estomatológica del poblado de Alacranes, Municipio Unión de Reyes. Materiales y Métodos: se realizó un estudio observacional descriptivo en pacientes mayores de 19 años, del área de Alacranes municipio Unión de Reyes. El universo de estudio estuvo constituido por los pacientes que acudieron a la consulta de urgencia estomatológica, de enero de 2011 a enero 2012. Con previo consentimiento informado la muestra la constituyó 135 pacientes, con patológica de absceso alveolar agudo, teniendo en cuenta las variables: edad, sexo, etiología y complicaciones. Los datos obtenidos fueron registrados en una base de datos y se procesaron estadísticamente. Resultados: los grupos de 20-29 años y el sexo masculino fueron los más afectados, con 38 % y 54.8 %, respectivamente. La causa fundamental fue la caries dental con un 91.8 %. La celulitis facial resultó la complicación más frecuente. Conclusiones: la etiología más usual del absceso alveolar agudo fue la caries dental. Los pacientes afectados que no recibieron una puntual asistencia sufrieron complicaciones (AU).

Introduction: frequently, oral cavity infections are due to the deterioration caused by caries with dental pulp exposition. An inadequate and careless dental hygiene and complications associated to periodontal diseases are other reasons the may lead to the occurrence of infectious processes. Objective: Characterise the behaviour of the absceso alveolar acute in the greater patients of 19 years in the query of urgency estomatológica of the populated of Alacranes, municipality Union de Reyes. Materials and Methods: a descriptive, observational study was carried out in patients elder 19 years old, in the health area Alacranes, municipality Union de Reyes. The studied universe was formed by the patients assisting to the dentist emergency consultation from January 2011 to January 2012. With previous informed consent, the sample was formed by 135 patients with acute alveolar abscess, taking into account the following variables: age, sex, etiology and complications. The obtained data were recorded in the database and statistically processed. Results: the most affected groups were the 20-29 years-old group and the male sex, with 38 % and 54.8 % respectively. Dental caries was the main cause with 91.8 %. Facial cellulitis was the most frequent complication. Conclusions: dental caries was the most usual etiology of the acute alveolar abscess. The affected patients who did not receive opportune health care suffered complications (AU).

Prenatally Diagnosed Cases of Binder Phenotype Complicated by Respiratory Distress in the Immediate Postnatal Period.

Binder phenotype, or maxillonasal dysostosis, is a distinctive pattern of facial development characterized by a short nose with a flat nasal bridge, an acute nasolabial angle, a short columella, a convex upper lip, and class III malocclusion. We report 3 cases of prenatally diagnosed Binder phenotype associated with perinatal respiratory impairment.

Temporomandibular Joint Disorders in Patients With Different Facial Morphology. A Systematic Review of the Literature.

PURPOSE: The present article aimed to review systematically the literature on the relation between facial skeletal structures and temporomandibular joint (TMJ) disorders. MATERIALS AND METHODS: A systematic search in the dental and medical literature was performed to identify all studies of humans assessing the relation between TMJ disorders and facial morphology. Articles were included based on study design, irrespective of TMJ disorder (eg, disc displacement, osteoarthrosis, or unspecified), skeletal features, diagnostic strategies (e.g., imaging techniques or clinical assessment), and population (eg, demographic features of participants) under investigation. The selected articles were assessed according to a format based on patients, problem, and population, intervention, comparison, and outcome and quality was evaluated based on the Newcastle-Ottawa Scale. RESULTS: Thirty-four articles were included in the review, 27 of which concerned adult samples and 7 concerned adolescent samples. Quality was generally moderate. The articles dealt with the relation between facial morphology and the following TMJ disorders, assessed clinically or by magnetic resonance (MR): disc displacement (n = 20), osteoarthritis or osteoarthrosis (n = 8), and temporomandibular disorder signs and symptoms (n = 6). The different approaches featuring the various investigations and the presence of some potential methodologic biases complicated a summary of the findings. Most studies reported that some features related to the vertical dimension of the face might help distinguish patients with potential TMJ disc displacement or MR-detected signs of osteoarthrosis from those without TMJ disorders. CONCLUSIONS: The quality of the available literature is not adequate to provide an evidence base on the topic. Despite the heterogeneity of design and findings of the reviewed articles, it seems reasonable to suggest that skeletal Class II profiles and hyperdivergent growth patterns are likely associated with an increased frequency of TMJ disc displacement and degenerative disorders.

El colgajo submental en reconstrucción de defectos orofaciales / Submental flap in reconstruction of orofacial defects

Objetivo. Las técnicas microquirúrgicas con colgajos libres constituyen el «Gold Standard» en la reconstrucción inmediata de defectos postoncológicos de cabeza y cuello. Sin embargo, son procedimientos complejos, que requieren un alto grado de especialización, no exentos de complicaciones y morbilidad. El colgajo submental constituye una alternativa reconstructiva en el territorio maxilofacial, en casos en los que la reconstrucción microquirúrgica no está indicada. El objetivo del trabajo es mostrar los beneficios del empleo del colgajo submental en la reconstrucción maxilofacial. Material y método. Presentamos la experiencia recogida en el Servicio de Cirugía Oral y Maxilofacial del H.U. Ramón y Cajal de Madrid desde 2009 hasta 2013, registrando un total de 20 reconstrucciones realizadas con colgajo submental pediculado en pacientes con procesos neoplásicos a nivel intra y extraoral. Resultados. Los resultados fueron satisfactorios en 19 pacientes intervenidos, atendiendo a los criterios de cobertura del defecto, estética y funcionalidad. Se realizaron 12 disecciones cervicales funcionales electivas, con resultado histológico N0. En ningún caso se detectó transferencia de enfermedad tumoral cervical al lecho receptor. Solo se ha evidenciado recurrencia local de la enfermedad en un paciente. Conclusiones. El colgajo submental constituye una alternativa válida para la reconstrucción de defectos orofaciales, especialmente en aquellos pacientes que por edad o estado general deteriorado requieren tratamientos poco agresivos y con tiempos quirúrgicos reducidos. Requiere descartar la presencia de enfermedad metastásica ganglionar cervical previamente a su realización. Su empleo es controvertido para la reparación de defectos tras resección de tumores con alta linfofilia (AU)

Objective. The microsurgical techniques with free flaps are the 'Gold Standard' in the immediate reconstruction of post-cancer defects of the head and neck. However, procedures are complex, requiring a high degree of specialization, and not exempt from complications and morbidity. The submental flap is an alternative reconstruction technique in the maxillofacial field in cases where the microsurgical reconstruction is not indicated. The objective of this work is to show the benefits of the use of the submental flap in the maxillofacial reconstruction. Material and method. The experience of the Department of Oral and Maxillofacial Surgery of the H. U. Ramón y Cajal of Madrid from 2009 to 2013 is described, using the records of a total of 20 reconstructions made with submental pedicled flap in patients with intra- and extra-oral cancers. Results. The results were satisfactory in the 19 patients who underwent surgery, according to the criteria for coverage of the defect, aesthetics and functionality. There were 12 elective functional neck dissections, with histological findings, N0. In no case was transfer of cervical tumor disease to the recipient bed detected. There was only local recurrence of the disease in 1 patient. Conclusions. The submental flap constitutes a valid alternative for the reconstruction of orofacial defects, especially in elderly patients or patients that, due to deteriorated general condition require less aggressive treatments and reduced surgical times. Requires rule out The presence of cervical lymph node metastatic disease needs to be ruled out prior to surgery. Its use is controversial for the repair of defects after resection of tumors with high levels of tumor-infiltrating lymphocytes (AU)

[Diagnosis and treatments of craniomaxillofacial deformities with OSDB].

Obstructive sleep disordered breathing (USDB) is a common diseases which caused by upper airway(UA) occlusion, muscle tone problems and collapse of upper airway etc. The article introduces how to select surgical treatment protocol. First, it is necessary of PSG and upper airway evaluation. Then, it is key to master surgical indications. For the children with hypertrophy of tonsillectomy or adenoidectomy, it may first step of tonsillectomy or adenoidectomy, they needed followed up by orthodontic doctor or orthognathic surgeon for tooth and maxillomandibular malformation. For the adult OSDB patients, there are two method, soft tissue reduction/mass excision around UA or maxillomandibular advancement(MMA), and MMA is recommended to the patients such as failure from UPPP and other surgical treatment, but for severe obesity patients, bariatric surgery is commended, it is very effective for soft tissue reduction around UA and weigh loss. For the OSDB patients with craniaomaxillomandibular deformities, such as craniosynostosis syndrome/micrognathia. Finally, it is important of the UA management for the surgical patients around perioperative period. All in all, it is necessary of the multidisciplinary cooperation for the OSDB patients, surgical treatment is useful method but it is not commend to all OSDB patients.

Ackerman syndrome: a rare cause of arthritis with dermatitis.

We present a case of a middle-aged woman who presented with acute onset of non-erosive oligoarthritis and cutaneous lesions. Her laboratory work up revealed mild anaemia with positive antinuclear antibody. Her skin biopsy confirmed the presence of interstitial granulomatous dermatitis. She was treated with a successful trial of non-steroidal anti-inflammatory agents. Interstitial granulomatous dermatitis with arthritis (IGDA), or Ackerman syndrome, is an under-recognised cause of arthritis with dermatitis. This is primarily due to the varied clinical presentation of the skin lesions and the non-specific laboratory findings. Our aim is to highlight the pivotal role of skin biopsy as part of the diagnostic assessment of patients who present with arthritis and concomitant skin lesions.

Características orofaciais e risco para apneia obstrutiva do sono em hipertensos / Caracteristicas orofaciales y riesgo de anea obstructiva del sueño em hipertensos / Orofacial characteristics and risk for obstructive sleep apnea in hypertensive individuals

Anormalidades da anatomia orofacial estão envolvidas na patogênese da Apnéia Obstrutiva do Sono porfacilitarem o fechamento das vias áreas. O estudo objetivou associar a presença de alterações orofaciais ao risco deApnéia Obstrutiva do Sono em hipertensos. 95 indivíduos (46% homens e 54% mulheres, 64 ± 10 anos) foram avaliadosquanto à distância tireomentoniana, Mallampati, palato ogival, grau de Angle, índice de massa corpórea, circunferênciasabdominal e pescoço. O risco para Apnéia Obstrutiva do Sono foi obtido pelo Questionário de Berlim.Foram observadosíndice de massa corpórea de 30 ± 5 Kg/m2, circunferência abdominal de 101 ± 12 cm e do pescoço 39 ± 3cm. Correlaçõespositivas foram observadas entre classificação de Mallampati, circunferências abdominal e do pescoço (p= 0,025 e 0,03,respectivamente). Pelo Questionário de Berlim, 46 indivíduos (48%) apresentavam alto risco, dos quais76% classificadoscomo Mallampati III/ IV, 69% distância tireomentoniana < 6 cm, 26% Angle II e 10% palato ogival. A prevalência dealterações orofaciais foi maior nos indivíduos comrisco elevado (93%) do que nos de baixo risco (50%). Alterações naanatomia orofacial, particularmente Mallampati, reiteram risco de Apnéia Obstrutiva do Sono e podem facilitar a triagemclínica como diagnóstico precoce.

[Congenital piriform aperture stenosis and odontogenic disorders]. / Sténose congénitale de l'orifice piriforme et troubles de l'odontogenèse.

PURPOSE: We had for objective to assess odontogenic disorders associated to a congenital piriform aperture stenosis and to study their various presentations. METHODS: Twelve patients presenting with a congenital piriform aperture stenosis, 1 week to 3 months of age, were retrospectively included from 1998 to 2008. All patients underwent an initial CT scan to evaluate the temporary dental germs. RESULTS: Deciduous dental germs were abnormal in 75% of the cases. Thirty-three percent had a single median maxillary central incisor. DISCUSSION: The concept of solitary median maxillary central incisor syndrome makes for a more pathophysiological approach of this type of disease, with various clinical presentations, corresponding to various levels of severity of a same pathological process.

Impact of cone-beam computed tomography on orthodontic diagnosis and treatment planning.

INTRODUCTION: In this study, we measured the impact of cone-beam computed tomography (CBCT) on orthodontic diagnosis and treatment planning. METHODS: Participant orthodontists shown traditional orthodontic records for 6 patients were asked to provide a diagnostic problem list, a hypothetical treatment plan, and a clinical certainty. They then evaluated a CBCT scan for each patient and noted any changes, confirmations, or enhancements to their diagnosis and treatment plan. RESULTS: The number of diagnosis and treatment plan changes varied widely by patient characteristics. The most frequently reported diagnosis and treatment plan changes occurred in patients with unerupted teeth, severe root resorption, or severe skeletal discrepancies. We found no benefit in terms of changes in treatment plan for patients when the reason for obtaining a CBCT scan was to examine for abnormalities of the temporomandibular joint or airway, or crowding. Orthodontic participants who own CBCT machines or use CBCT scans frequently in practice reported significantly more diagnosis and treatment plan changes and greater confidence after viewing the CBCT scans during the study. CONCLUSIONS: The results of this study support obtaining a CBCT scan before orthodontic diagnosis and treatment planning when a patient has an unerupted tooth with delayed eruption or a questionable location, severe root resorption as diagnosed with a periapical or panoramic radiograph, or a severe skeletal discrepancy. We propose that CBCT scans should be ordered only when there is clear, specific, individual clinical justification.

Multiple system morbidities associated with children with snore symptom.

OBJECTIVE: To exam the relationship between snoring and morbidities of multiple systems in children. STUDY DESIGN: Children with snoring were enrolled and divided into primary snorer (PS) group and obstructive sleep apnea hypopnea syndrome (OSAHS) group based on polysomnography. The healthy children served as the control group. The growth parameters, maxillofacial malformations, blood chemistry, electrocardiogram, and echocardiogram were recorded and intelligence testing was performed in the enrolled children who were ≥6 years old. RESULTS: The weight and height were similar in the control group (n = 60) and the PS group (n = 63), but lower in the OSAHS group (n = 89; P < 0.001). Occurrence of adenoidal face and dental malocclusion in the OSAHS and the PS group was significantly higher than that in the control group (P < 0.001). Compared with the control group, the OSAHS group had a lower serum high-density lipoprotein cholesterol level, higher low-density lipoprotein cholesterol level; and a possible higher pulmonary artery pressure based on the echocardiogram (P < 0.001). All the above parameters in the PS group were similar to those in the control group. Full-scale IQ and performance IQ of the OSAHS group was lower (P < 0.001), attention deficits were significantly higher in the OSAHS group (P < 0.001), but were similar in the PS group when compared to the control group. CONCLUSIONS: OSAHS in children is associated with delayed growth, maxillofacial malformations, impaired cognitive functions, abnormalities in lipid metabolism, and changes in pulmonary artery pressures. PS children also have higher incidence of maxillofacial malformations but have a normal growth and normal cognitive functions.

Clinical and radiological presentation of hemimaxillofacial dysplasia/segmental odontomaxillary dysplasia: critical analysis and report of a case.

OBJECTIVES: The purpose of the present study was to critically evaluate the literature, describe the clinical and radiographic features of HD-SOD (hemimaxillofacial dysplasia/segmental odontomaxillary dysplasia), and describe one new case, involving a 12-year-old boy, with detailed radiological, clinical, and histologic characteristics. METHODS: Thirty-six cases published between 1987 and 2010, together with the present case, were evaluated according to criteria that included gender, age, location of the lesion, findings, and symptoms. RESULTS: We found that the lesion is discovered mainly in the first decade of life (71%), and has a male predilection (64%, 23/64). The maxillary alveolar process was affected unilaterally in all cases, with gingival and bone enlargement and facial asymmetry being constant findings. Missing premolars and skin manifestations were found to be common features. CONCLUSIONS: New case reports should include clinical, radiographic, and histologic findings; follow-up reports; and treatment protocols to improve dentist and parent information regarding HD.

Duplicated, translocated maxilla and upper lip: a case report of a rare congenital anomaly.

A 3-month-old male patient presented to the maxillofacial department in Al-Amiri Dental Center, Kuwait, with a facial deformity. There was no family history of any systemic illness or abnormalities nor consanguinity. On examination; the patient had a transverse incomplete facial cleft on the right cheek, the maxilla and the upper lip were duplicated and translocated to the right lower jaw and lip, with all components of mucosa as well as a complete compliment of deciduous teeth. There was unilateral macrosomia as well as cleft of the secondary palate and triple uvulae. The mandible had restricted mobility. Photographs and a Computerized Axial tomogram (CAT scan) were taken and a complete work up to exclude congenital cardiac disease was also done. The surgical approach, and possible future operations were explained in detail and discussed with the parents. Their consent was taken.