Incidence and morphology of the incisive suture in CT scanning of young children and human fetuses.

PURPOSE: Incisive suture is a suture classically described on the oral face of the palate in fetuses and young children. The aim of our study was to describe the evolution of the incisive suture in human fetuses and to evaluate the incidence of this suture in a population of young children under 4 years, to determine if there is a possibility of improving the anterior growth of the maxilla, by stimulation of this suture. METHODS: One hundred and thirty CT scan images of patients aged from birth to 48 months have been studied and nine fetal palates aged from 18 to 26 weeks of development, have been scanned using high-resolution X-ray micro-computed tomography RESULTS: The CT scan images of patients showed that an incisive suture was present in 33/130 cases (25,4%). All the patients with a suture were under 2 years old. The fetal palate study showed that the suture was present in the inferior aspect of the palate (oral cavity) in all cases. The incisive suture increased from 18 to 24 weeks. At 26 weeks it stopped growing although the intercanine length increased. Considering the closure of the suture in a vertical plane, our study on fetuses has shown that the incisive suture is closing from its superior side (nasal side) to its inferior side. CONCLUSIONS: Considering all these results it appears to us that the incisive suture is partially ossified after birth, it cannot be stimulated by orthodontic appliances.

Bifid mandibular canals: CBCT assessment and macroscopic observation.

PURPOSE: The bifid mandibular canal is an anatomical variation, which causes anesthetic failure and surgical accidents occasionally. The purpose of this study is to observe the prevalence and anatomical location of bifid mandibular canals, providing clinical value in reducing the occurrence of surgical accidents and postoperative complications. METHODS: A total of 321 outpatients were selected in this study. Their CBCT images were observed, and the prevalence of bifid mandibular canals as well as the composition ratios of each branch type was evaluated according to the classification of Naitoh. The bifid mandibular canals and their branches' diameter, length, horizontal distance to the buccal/lingual wall of the mandible, and vertical distance to the mandibular alveolar ridge were measured. Furthermore, 194 dry adult mandibles from the Department of Oral Anatomy and Physiology of Tianjin Medical University were observed to evaluate the prevalence and the average diameter of retromolar foramina. RESULTS: Of all the 321 patients, 84 (26.17%) cases of bifid mandibular canals and 105 (16.36%) sides of unilateral bifurcation were observed. Based on Naitoh's classification, the retromolar canals were the most common types (46.67%), followed by the forward canals (40.00%), the dental canals (10.48%) and the buccolingual canals (2.86%). In 194 dry adult mandibles, 23 cases (11.86%) and 29 sides (7.47%) of retromolar foramina were found and the average diameter of retromolar foramina was 0.94 ± 0.30 mm. CONCLUSION: More than a quarter of the population has the bifid mandibular canal, which is a potential factor of the onset of surgery accidents. CBCT is an effective method to identify the branches of mandibular canals. Preoperative CBCT examination can help reduce various postoperative complications.

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)

Standardizing Terminology and Assessment for Orofacial Conditions in Juvenile Idiopathic Arthritis: International, Multidisciplinary Consensus-based Recommendations.

OBJECTIVE: To propose multidisciplinary, consensus-based, standardization of operational terminology and method of assessment for temporomandibular joint (TMJ) involvement in juvenile idiopathic arthritis (JIA). METHODS: Using a sequential expert group-defined terminology and methods-of-assessment approach by (1) establishment of task force, (2) item generation, (3) working group consensus, (4) external expert content validity testing, and (5) multidisciplinary group of experts final Delphi survey consensus. RESULTS: Seven standardized operational terms were defined: TMJ arthritis, TMJ involvement, TMJ arthritis management, dentofacial deformity, TMJ deformity, TMJ symptoms, and TMJ dysfunction. CONCLUSION: Definition of 7 operational standardized terms provides an optimal platform for communication across healthcare providers involved in JIA-TMJ arthritis management.

Efficacy of rhBMP-2 in Cleft Lip and Palate Defects: Systematic Review and Meta-analysis.

The aim of this study was to analyze the efficacy of using rhBMP-2 (recombinant human morphogenetic protein-2) in the treatment of patients with cleft lip and palate defects (CLPD). Seven databases were screened: PubMed (Medline), Lilacs, Ibecs, Web of Science, BBO, Scopus, and The Cochrane Library. Clinical trials that evaluated the use of bioactive treatment with rhBMP-2 in the treatment of patients with CLPD were included. Statistical analyses were performed by comparing the standardized mean difference of bone formation volume and bone filling percentage (p = 0.05). Ten studies compared the use of rhBMP-2 and iliac crest bone graft (ICBG). The global analysis for bone formation volume and bone filling percentage showed that bioactive materials were similar to ICBG with a standardized mean difference of respectively 0.07 (95% CI - 0.41 to 0.56) and 0.24 (95% CI - 0.32 to 0.80). The available literature suggested that use of rhBMP-2 presented similar bone formation results to those of ICBG in secondary alveolar bone grafting for patients with CLPD.

Anomalías dentomaxilofaciales de la atención primaria de salud en escolares de 6-12 años / Dentomaxillofacial anomalies of primary health care in school children aged 6-12 years

Introducción: Las anomalías dentomaxilofaciales correspondientes al nivel primario de atención estomatológica son muy frecuentes en la dentición mixta y su epidemiología ha sido muy poco abordada en las investigaciones ortodóncicas. Objetivo: determinar la prevalencia de las anomalías dentomaxilofaciales y caracterizar a los escolares seleccionados. Método: Se realizó estudio descriptivo, observacional y transversal en escolares de 6 a 12 años, pertenecientes a 4 escuelas primarias del área de salud del Policlínico Docente José Martí, de Santiago de Cuba, de mayo de 2015 hasta febrero de 2016. La muestra quedó constituida por 676 escolares con anomalías dentomaxilofaciales. Las variables estudiadas fueron la edad, el sexo, color de la piel, anomalías dentomaxilofaciales de la atención primaria de salud y etiología. Los datos se obtuvieron mediante el examen clínico y el interrogatorio en la labor de terreno y como medida de resumen se utilizó el porcentaje. Resultados: Entre los principales resultados sobresalió una alta prevalencia (64,6 por ciento) de las anomalías de la atención primaria en los niños estudiados, la más frecuente fue el síndrome de clase I con vestibuloversión y diastemas (40,4 por ciento), seguido de la oclusión invertida anterior simple (36,4 por ciento), el sexo más afectado fue el masculino (55,3 por ciento), la edad de 6 años (28,6 por ciento) y los niños de piel mestiza (64,3 por ciento). Los agentes etiológicos más frecuentes fueron los hábitos bucales deformantes (42,5 por ciento). Conclusiones: Se concluyó que estas entidades son frecuentes en la atención primaria; por lo que el estomatólogo debe estar capacitado para el diagnóstico precoz y tratamiento oportuno(AU)

Introduction: Dentomaxillofacial anomalies corresponding to the primary level of stomatological attention are very frequent in the mixed dentition and its epidemiology has been very little addressed in orthodontic investigations. Objective: to determine the prevalence of dentomaxillofacial anomalies and characterize the selected students. Method: A descriptive, observational and cross-sectional study was conducted among schoolchildren from 6 to 12 years old, belonging to 4 primary schools of the health area of the José Marti Teaching Polyclinic in Santiago de Cuba, from may 2015 to February 2016. The sample consisted of 676 schoolchildren with dentomaxillofacial anomalies. The variables studied were age, sex, skin color, dentomaxillofacial anomalies of the primary health care and etiology. The data was obtained through the clinical examination and the questioning in the field work and as a summary measure the percentage was used. Results: Among the main results, there was a high prevalence (64.6 percent) of the primary care anomalies in the children studied, the most frequent being Class I syndrome with vestibuloversion and diastema (40.4 percent), followed by simple anterior inverted occlusion (36.4 percent), the sex most affected was male (55.3 percent), the age of 6 years (28.6 percent) and children of mixed skin (64.3 percent). The most frequent etiological agents were deforming oral habits (42.5 percent). Conclusions: It was concluded that these entities are frequent in primary care; so the dentist must be trained for early diagnosis and timely treatment(EU)

Oral manifestations and dentofacial anomalies in ß-thalassemia major children in Dubai (UAE).

AIMS: The purpose of this study was to identify special oral and dentofacial manifestations of ß-thalassemia major (ΒTM) in Emarati children in Dubai (UAE). MATERIALS AND METHODS: A total of 38 (ΒTM) and 76 healthy Emirati children were recruited. An oral examination was conducted to determine dmft/DMFT indices, Simplified Oral Hygiene Index, occlusal anomalies, dentofacial, and soft-tissue abnormalities. RESULTS: ΒTM children had significantly higher DMFT compared to the healthy controls (p-value, 0.017). The Met Need Index (MNI) and the Restorative Index (RI) were lower in the ΒTM group but without statistical significance. Calculus Index (CI) was found to be significantly higher in ΒTM children (p-value, 0.002). The proportion of gingivitis was significantly lower in the ΒTM group (p-value, 0.009). ΒTM children had significantly higher proportion of retained primary teeth (p-value, 0.001) and gingival pigmentation compared with healthy controls (p-value, 0.001). BTM children did not have any statistically significant difference in the Angle molar classiication compared to the control. Interestigly, a significantly higher proportion of transverse cross bite was found in the control subjects compared to the BTM group (p-value, 0.004). CONCLUSIONS: Significantly higher DMFT, retained primary teeth, gingival pigmentation, and CI was found in the BTM group compared to the controls while significantly lower gingivitis index and transverse cross bite was seen in the BTM group.

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.

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).

Síndrome de Sturge-Weber: revisión / Sturge-Weber Syndrome: A Review

El síndrome de Sturge Weber es un trastorno neurocutáneo congénito, esporádico causado por una mutación somática activadora en el gen GNAQ, con una incidencia de uno de cada 20,000-50,000 nacidos. Se caracteriza por la presencia de una mancha en vino de Oporto facial, angiomatosis leptomeníngea y glaucoma. La manifestación neurológica más común son las convulsiones, que suelen comenzar en los primeros meses de vida. El glaucoma puede estar presente desde el nacimiento o desarrollarse posteriormente. Los estudios de neuroimagen permiten visualizar la angiomatosis leptomeníngea, ayudando al diagnóstico del síndrome de Sturge Weber. El tratamiento estándar incluye láser para la mancha en vino de Oporto facial, anticonvulsivantes y tratamiento médico o quirúrgico del glaucoma. El pronóstico de la enfermedad dependerá de la extensión de la malformación leptomeníngea y del grado de afectación ocular (AU)

Sturge-Weber syndrome is a sporadic congenital neurocutaneous disorder caused by a somatic activating mutation in GNAQ; it affects 1 in every 20,000 to 50,000 newborns. It is characterized by a facial Port-wine stain, leptomeningeal angiomatosis, and glaucoma. Seizures are the most common neurological manifestation and typically present in the first months of life. Glaucoma may be present at birth or develop later. Neuroimaging studies show leptomeningeal angiomatosis, supporting diagnosis. Standard treatment for Sturge-Weber syndrome includes laser treatment for the Port-wine stain, anticonvulsants, and medical or surgical treatment for the glaucoma. Prognosis depends on the extent of leptomeningeal involvement and the severity of the glaucoma (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).

[Orthodontic treatment need in school-age children in the Leningrad region].

Epidemiology of dentoalveolar anomalies is undoubtedly important, but in terms of the organization of orthodontic care, greater interest are data on the needs in this type of treatment. In a situation of limited manpower and resources for the provision of orthodontic care information about needs in orthodontic treatment allows you to define a group of patients with the primary need for orthodontic treatment, and to identify priorities to optimize the organization of orthodontic care in the region. Such data can be obtained by using the Dental Aesthetics Index (DAI) and Index of Orthodontic Treatment Need (IOTN). The aim of the study was to analyze the epidemiology of various forms of dentoalveolar anomalies school-age children of Kirishi district of Leningrad region, as well as their needs in orthodontic treatment in accordance with objective evaluation indices. The study involved 734 pupils of Kirishi lyceum №1 of Leningrad region. Analysis of the prevalence of dentoalveolar anomalies, as well as needs in the orthodontic treatment was conducted in three age groups: I mixed dentition period (6-9 years), II mixed dentition period (10-13 years), and permanent dentition (14-17). To determine the needs in the orthodontic treatment were used two most common international index (DAI and IOTN). In Kirishi district of Leningrad region dentoalveolar anomalies were found in 88.8% of children of school age, which is in accordance with the indices and IOTN DAI needs in orthodontic treatment is 38.8% and 54.5%, respectively. In order to reduce unnecessarily high load volume medical institutions orthodontic profile, optimize utilization of financial resources, as well as reducing social tension it is recommended to introduce the practice of doctors-orthodontists methodology for determining the needs in orthodontic treatment by objective indices.

ICD-10 coding in otorhinolaryngological malformations: Analyses of 2342 Q-diagnoses.

INTRODUCTION: The 10th revision of the International Statistical Classification of Diseases and Related Health Problems (ICD-10) includes more than 14,400 codes. The aim of this study was to study the prevalence and demographics of otorhinolaryngological congenital malformations in an outpatient clinic based of the ICD-10 Q-diagnoses used for congenital malformations, deformations and chromosomal abnormalities. MATERIALS AND METHODS: Electronic hospital records covering six years (2007-2013) were searched to identify all patients with ICD-10 Q-diagnosis. RESULTS: 2342 patients were identified. Malformations of the face and neck were most prevalent (30%). The gender distribution was equal except malformations of tongue, mouth and pharynx, where 70% of the patients were male. CONCLUSIONS: There seems to be a significant excess of ICD-10 codes for otorhinolaryngological malformations. Ten most common otorhinolaryngological malformation codes cover more than 94% of the diagnoses. In addition, the illogicalities and the possibility of coding by diagnosis, symptoms or clinical findings makes the coding suboptimal for the purposes it was originally created for. Malformations of the nose and larynx are rare compared to other anatomic localizations. The age at diagnosis of branchial cysts differs significantly from all other congenital malformations supporting the theory of cystic transformation of cervical lymph nodes.

[Naso-ethmoido-maxillary protrusion (NEMP): a specific dysmorphosis]. / La pro-naso-ethmoïdo-maxillie (PNEM) : une dysmorphose spécifique.

Naso-ethmoido-maxillary protrusion (NEMP) is a rare dental and facial dysmorphosis, with excessive growth of basicranium, ethmoid, maxillary, and nasal bones. The clinical presentation includes nasal and upper lip protrusion, telecanthus, a class 2 malocclusion with maxillary protrusion and exoclusion. The craniofacial field is increased in Delaire's analysis. Contrary to isolated maxillary protrusion secondary to membranous ossification dysfunction, NEMP is a constitutional anomaly resulting from an excessive primary growth of the chondrocranium. The therapeutic management of NEMP should take into account these specificities.

[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.

Binder syndrome.

UNLABELLED: Patients with chondrodysplasia punctata (CDP) usually present with Binder-type features, and often CDP is misdiagnosed as Binder syndrome. This study reviewed the management and outcome of patients with Binder syndrome and CDP in a multidisciplinary setting. METHODS: The notes and radiographs of the patients managed at the Australian Craniofacial Unit with a multidisciplinary setting since 1976 were reviewed, and data were collected on patient demographics, associated medical and surgical problems, subsequent management, and complications. RESULTS: Seventy-seven patients were treated over the 30-year period (5 patients were lost to follow-up); of the remaining 72 patients, 60 (83%) had Binder syndrome, and 12 (17%) were patients with CDP. Forty were males, and 32 were females, with an age range of 6 months to 47 years. Thirteen patients (18%) had a strong family history, and 65 patients (90%) have so far undergone surgical correction, and of those, 35 (54%) have completed their treatment, the longest follow-up time being 18 years. The mean number of surgical procedures was 2.4, and 18 patients (28%) had postoperative complications, which included partial necrosis of the maxilla, osteomyelitis of the mandible, facial nerve and inferior alveolar nerve neuropraxia, nasal bone graft exposure, and cellulitis. DISCUSSION: Because of the phenotypic characteristics shared by both Binder syndrome and CDP, it is most likely that Binder syndrome is not a syndrome, nor is it an entity, but most likely to be an "association." We would advocate that these patients should be managed in a multidisciplinary setting.

Outcomes of hypoplastic left heart syndrome in low-birth-weight patients.

The objective of this study was to assess outcomes of hypoplastic left heart syndrome (HLHS) patients weighing ≤ 2.5 kg throughout staged palliation. We performed a single-center retrospective review. Abstracted data included gestational age, birth weight, presence of noncardiac anomalies, and survival through Fontan. Fifty-two patients met inclusion criteria, with a median birth weight of 2.14 kg and gestational age of 36 weeks. Five patients received comfort care only. Of 47 patients who underwent initial surgical palliation, 51% survived to initial hospital discharge. Birth weight and gestational age (GA) were similar between survivors and nonsurvivors. Compared with survivors, risk factors for death prior to initial hospital discharge were as follows: small for GA (P = 0.005), noncardiac anomalies (P = 0.04), need for post-perative extracorporeal membrane oxygenation (P = 0.0004), and conversion from initial palliation to Sano shunt (n = 5, no survivors). Operative survival following Stage 2 palliation was 91% (21/23) and 94% after Fontan (17/18). Overall survival for palliated patients from birth through Fontan was 36%. Low-birth-weight neonates with HLHS have poor overall survival through the Fontan operation, with highest mortality following Stage 1 palliation. Being small for GA and the presence of noncardiac anomalies are important preoperative risk factors for early mortality.

Aplasia and agenesis of the frontal sinus in Turkish individuals: a retrospective study using dental volumetric tomography.

Agenesis of the paranasal sinuses is an uncommon clinical condition that appears mainly in the frontal (12%) and maxillary (5-6%) sinuses; in some populations, it appears at a higher proportion. This study investigated the prevalence of agenesis of the frontal sinuses using dental volumetric tomography (DVT) in Turkish individuals. The frontal sinuses of 410 patients were examined by DVT scans in the coronal planes for evidence of the absence of the frontal sinuses. A bilateral and unilateral absence of the frontal sinuses was seen in 0.73% and 1.22% of cases, respectively. In one case, both agenesis and aplasia of the frontal sinus was seen (0.24%). The low percentage of frontal sinus agenesis must be considered during pre-surgical planning related to the sinuses. DVT may be used as a diagnostic tool for the examination of frontal sinus aplasia.

Binder phenotype: associated findings and etiologic mechanisms.

Binder phenotype (BP), or maxillonasal dysostosis, consists of 6 characteristics: arhinoid face, abnormal position of nasal bones, intermaxillary hypoplasia/malocclusion, reduced/absent anterior nasal spine, atrophy of nasal mucosa, and absence of frontal sinus. The purposes of this study were (1) to review the characteristic facial findings, other malformations, and diagnoses in 8 patients with BP; (2) to compare these patients to those in the literature; and (3) to discuss developmental mechanisms, including genetic and environmental factors, involved in this facial defect. An initial 24 cases of BP were identified from the Iowa Registry of Congenital & Inherited Disorders during the period of 1998 to 2008. Chromosome analysis performed in all 24 cases revealed the following: trisomy 21, trisomy 18, and mosaic trisomy 18. Of the 24 patients, 8 met the specific diagnostic characteristics of BP. All 8 patients were evaluated in the genetics clinic at University of Iowa Children's Hospital, having diagnoses of vitamin K epoxide reductase deficiency, Xp22.3 deletion with chondrodysplasia punctata, Stickler syndrome, fetal warfarin syndrome, Robinow syndrome, and unknown etiology. This study, unlike those in the literature, ascertained cases through a population-based active surveillance registry and therefore may better represent the incidence of BP (∼1 per 18,000). Most cases were sporadic with a recognizable pattern of malformation, highlighting that chromosomal, genetic, and exogenous factors may cause BP. Of 8 cases remaining after exclusion of chromosome syndromes, 3 cases had in common the involvement of the vitamin K-dependent metabolic pathway, which likely represents a significant pathogenetic mechanism of BP. Clinical characterization of BP, as in these cases, may allow further understanding of other causative developmental mechanisms.