Displasia frontonasal / Frontonasal dysplasia

Los trastornos del desarrollo son aquellos padecimientos que se manifiestan por defectos en la embriogénesis de la región afectada. La cara del ser humano comienza su formación alrededor de la cuarta semana de vida intrauterina y se manifiesta gracias a la fusión de cinco prominencias: dos pares conocidas como maxilar y mandibular, y una impar conocida como frontonasal. Cuando esta fusión no se lleva a cabo de una forma óptima, aparecen numerosas alteraciones del desarrollo como el labio y paladar hendido, y la displasia frontonasal. La displasia frontonasal produce frecuentemente afecciones oculares, nasales y orales. Dentro de las manifestaciones orales destacan una forma atípica de hendidura labial o palatina, afecciones dentales y alteraciones en el crecimiento de la cara. Dada la gran relación que este padecimiento tiene con la cavidad oral resulta importante que el odontólogo conozca la etiología y las características clínicas de este trastorno (AU)

Developmental disorders are those conditions that are manifested by defects in the embryogenesis of the affected region. The human face begins its formation around the fourth week of intrauterine life and is manifested thanks to the fusion of five prominences: two pairs known as maxillary and mandibular and odd one known as frontonasal. When this fusion is not carried out in an optimal way, numerous developmental alterations appear, such as cleft lip and palate and frontonasal dysplasia. Frontonasal dysplasia frequently produces ocular, nasal and oral affections. Among the oral manifestations, and atypical form of clef lip and/or palate, dental affections and alterations in the growth of the face stand out. Given the great relationship that this condition has with the oral cavity, it is important for the dentist to know the etiology and clinical characteristics of this disorder (AU)

Nasal septum deviation and inferior nasal concha bone hypertrophy in class III facial deformity / Desviación del septum nasal e hipertrofia de la concha nasal inferior en deformidad facial clase III

SUMMARY: The aim of this research was to analyze the morphology of the nasal septum and inferior nasal concha bone in class III facial deformities prior to orthodontic treatment in orthognathic surgery candidates. 40 subjects were included in this research. The inclusion criteria were an Angle class III, negative overjet and SNA angle less than 80º. Patients with facial asymmetry, facial trauma or who had undergone maxillofacial or ENT procedures were excluded. CBCT images were obtained for all the patients and the nasal septum deviation, morphology of inferior nasal concha bone and ostium of the maxillary sinus were analyzed and related to the complexity of the facial deformity expressed by the ANB angle and dental relations. The measurement was standardized by ICC and the data was analyzed using a chi square test and Spearman's coefficient with a p value < 0.005 for statistical significance. Nasal septal deviation was observed in 77.5 %. The deviation angle was 13.28º (±4.68º) and the distance from the midline to the most deviated septum was 5.56 mm (±1.8 mm) with no statistical relation to the complexity of the facial deformity. The deviated nasal septum showed inferior nasal concha bone hypertrophy on the concave side of the nasal septum deviation (p=0.049). The open or closed condition of the maxillary sinus ostium was not related to any conditions in the septum or complexity of the facial deformity. Inferior nasal concha bone hypertrophy could be related to nasal septal deviation. The nasal condition in a class III facial deformity could not differ from the general population; careful in orthognathic surgery as to be assume in the Le Fort I Osteotomy and nasal approach related to nasal septum deviation and inferior nasal concha bone.

RESUMEN: El objetivo de esta investigación fue analizar la morfología del septum y la concha nasal inferior en sujetos con deformidad facial clase III previo al tratamiento de ortodoncia preparatorio para cirugía ortognática. Fueron incluidos 40 sujetos en esta investigación. Los criterios de inclusión fueron la de presentar una clase III de Angle, overjet negativo y ángulo SNA menor que 80º. Sujetos con asimetría facial, trauma facial o quienes presentaron algún tipo de procedimiento maxilofacial o de otorrinolaringología fueron excluidos. Tomografía computadorizada cone beam (CBCT) fueron obtenidas para todos los sujetos donde le morfología del septum nasal, morfología de la concha nasal inferior y el ostium del seno maxilar fueron analizados y relacionados con la complejidad de la deformidad facial expresada como ángulo ANB y relaciones dentales. Las medidas fueron estandarizadas por el ICC y los datos fueron analizados utilizando la prueba chi cuadrado y coeficiente de Spearman con un valor de p<0,05 para obtener relaciones significativas. La desviación del septum nasal se observó en el 77,5 %; el ángulo de desvío fue de 13,28º (±4,68º) y la distancia de desvío del septum desde la línea media fue de 5,56 mm (±1,8 mm) sin diferencias estadísticas en relación a la complejidad de la deformidad. El desvío de septum nasal demostró hipertrofia de la concha nsal inferior en el lado cóncavo del septum desviado (p=0,049). La condición de ostium abierto o cerrado no fue relacionado con ninguna condición del septum nasal o complejidad de la deformidad facial. La hipertrofia de la concha nasal inferior se relacionó con el desvío de septum nasal. La condición nasal en deformidad facial de clase III no es diferente de la observada en la población general; cuidados deben ser realizados en cirugía ortognática para el desarrollo de la osteotomía de Le Fort I y aproximación nasal en relación al desvío de septum y probable alteración de la concha nasal inferior.

Incisivo central único de la línea media del maxilar, holoprosencefalia y estenosis congènita del orificio nasal anterior en un recién nacido prematuro: a propósito de un caso / Solitary median maxillary central incisor, holoprosencephaly and congenital nasal pyriform aperture stenosis in a premature infant: case report

El síndrome del incisivo central único de la línea media del maxilar es un trastorno raro que implica anomalías de la línea media, como holoprosencefalia, anomalías de las fosas nasales, fisura palatina, labio leporino, hipotelorismo, microcefalia y panhipopituitarismo. La estenosis congénita del orificio nasal anterior es una causa mortal de dificultad respiratoria neonatal debido al estrechamiento del orificio nasal anterior, y podría confundirse con la atresia de coanas. En este informe, presentamos el caso de un recién nacido con síndrome del incisivo central único de la línea media del maxilar acompañado de otras anomalías, tales como holoprosencefalia, estenosis del orificio nasal anterior, microcefalia y panhipopituitarismo. El cariotipado mostró una deleción heterocigota en el gen SIX3 en la región 2p21, que produjo una forma más grave de holoprosencefalia.

Solitary median maxillary central incisor syndrome is a rare disorder involving midline abnormalities such as holoprosencephaly, nasal cavity anomalies, cleft palate-lip, hypotelorism, microcephaly, and panhypopituitarism. Congenital nasal pyriform aperture stenosis is a lethal cause of neonatal respiratory distress due to narrowing of the pyriform aperture anteriorly and it can be confused with choanal atresia. In this report, we present a newborn infant with solitary median maxillary central incisor syndrome accompanied by other abnormalities including holoprosencephaly, nasal pyriform aperture stenosis, microcephaly and panhypopituitarism. Chromosomal analysis showed heterozygous SIX3 gene deletion at 2p21 region resulting in a more severe form of holoprosencephaly.

Solitary median maxillary central incisor, holoprosencephaly and congenital nasal pyriform aperture stenosis in a premature infant: case report. / Incisivo central único de la línea media del maxilar, holoprosencefalia y estenosis congènita del orificio nasal anterior en un recién nacido prematuro: a propósito de un caso.

Solitary median maxillary central incisor syndrome is a rare disorder involving midline abnormalities such as holoprosencephaly, nasal cavity anomalies, cleft palate-lip, hypotelorism, microcephaly, and panhypopituitarism. Congenital nasal pyriform aperture stenosis is a lethal cause of neonatal respiratory distress due to narrowing of the pyriform aperture anteriorly and it can be confused with choanal atresia. In this report, we present a newborn infant with solitary median maxillary central incisor syndrome accompanied by other abnormalities including holoprosencephaly, nasal pyriform aperture stenosis, microcephaly and panhypopituitarism. Chromosomal analysis showed heterozygous SIX3 gene deletion at 2p21 region resulting in a more severe form of holoprosencephaly.

El síndrome del incisivo central único de la línea media del maxilar es un trastorno raro que implica anomalías de la línea media, como holoprosencefalia, anomalías de las fosas nasales, fisura palatina, labio leporino, hipotelorismo, microcefalia y panhipopituitarismo. La estenosis congénita del orificio nasal anterior es una causa mortal de dificultad respiratoria neonatal debido al estrechamiento del orificio nasal anterior, y podría confundirse con la atresia de coanas. En este informe, presentamos el caso de un recién nacido con síndrome del incisivo central único de la línea media del maxilar acompañado de otras anomalías, tales como holoprosencefalia, estenosis del orificio nasal anterior, microcefalia y panhipopituitarismo. El cariotipado mostró una deleción heterocigota en el gen SIX3 en la región 2p21, que produjo una forma más grave de holoprosencefalia.

Correção do dorso nasal baixo ou em sela com enxerto composto de cartilagem conchal fragmentada fixa ao pericôndrio envolvidos à fáscia da mastóide / Correction of low or saddle nasal dorsum with a composite graft of fragmented conchal cartilage fixed to the perichondrium wrapped in mastoid fascia

Introdução: A rinoplastia de aumento em muitos casos torna-se mais difícil que a rinoplastia de redução. Enxertos dorsais sólidos realizados com cartilagem costal têm sido muito utilizados para aumento dorsal, porém estão associados com altos índices de revisão, por isso, muitos autores passaram a utilizar cartilagem em cubos envoltos por fáscia. A fáscia da mastoide, conectada ao pericôndrio da cartilagem conchal auricular, pode formar um enxerto composto para o aumento do dorso nasal, sendo também uma opção de tratamento. O objetivo é demonstrar a possibilidade do uso de cartilagem da concha auricular fragmentada fixa ao seu pericôndrio, e envoltos na fáscia da mastoide, formando um enxerto composto para aumento do dorso nasal. Métodos: Tratase de um estudo retrospectivo de 9 pacientes operados entre 2012 e 2016 no Hospital de Base da Faculdade de Medicina de São José do Rio Preto, em que foi realizado aumento do dorso nasal com cartilagem conchal fragmentada fixa ao seu pericôndrio e envolto à fáscia da mastoide. Resultados: Os pacientes foram acompanhados de 6 a 48 meses. Foram questionados quanto à satisfação do procedimento nasal e sensibilidade auricular, com avaliação positiva dos pacientes e cirurgiões. Conclusão: A cartilagem conchal parece ser uma alternativa de grande valia para procedimentos de aumento de dorso nasal. Esta cartilagem envolta com fáscia da mastoide parece ser uma alternativa vantajosa em comparação ao uso de outras fáscias, com baixa morbidade e taxa de complicações, podendo ser uma grande opção para tratamento do nariz em sela.

Introduction: In many cases, augmentation rhinoplasty is more difficult than reduction rhinoplasty. Solid dorsal grafts performed with costal cartilage have been widely used for dorsal augmentation; however, they are associated with high rates of revision. Thus, many authors began to use cartilage cut into cubes wrapped in fascia. The mastoid fascia, connected to the perichondrium of the auricular conchal cartilage can form a composite graft to augment the nasal dorsum, which is also a treatment option. The objective is to demonstrate the possibility of using fragmented auricular conchal cartilage fixed to its perichondrium and wrapped in mastoid fascia to form a composite graft for augmentation of the nasal dorsum. Methods: This is a retrospective study of 9 patients who underwent operation between 2012 and 2016 at the Base Hospital of the Faculty of Medicine of São José do Rio Preto, in which the nasal dorsum was augmented with fragmented conchal cartilage fixed to its perichondrium and wrapped in the mastoid fascia. Results: The patients were followed up for up 6 to 48 months. They were questioned about their satisfaction with the nasal procedure and hearing sensitivity, and provided a positive evaluation of the surgeons. Conclusion: The conchal cartilage seems a highly valuable alternative graft for nasal dorsum augmentation procedures. The technique of using cartilage wrapped in mastoid fascia seems to be an advantageous alternative when compared with those using cartilage wrapped in other fasciae: it has low morbidity and complications rates and can be a great option for saddle nose treatment.

Importance of a multidisciplinary approach and monitoring in fetal warfarin syndrome.

Warfarin is a synthetic oral anticoagulant that crosses the placenta and can lead to a number of congenital abnormalities known as fetal warfarin syndrome. Our aim is to report on the follow-up from birth to age 8 years of a patient with fetal warfarin syndrome. He presented significant respiratory dysfunction, as well as dental and speech and language complications. The patient was the second child of a mother who took warfarin during pregnancy due to a metallic heart valve. The patient had respiratory dysfunction at birth. On physical examination, he had a hypoplastic nose, pectus excavatum, and clubbing of the fingers. Nasal fibrobronchoscopy showed upper airway obstruction due to narrowing of the nasal cavities. He underwent surgical correction with Max Pereira graft, zetaplasty, and osteotomies for the piriform aperture. At dental evaluation, he had caries and delayed eruption of the upper incisors. Speech and language assessment revealed high palate, mouth breathing, little nasal patency, and shortened upper lip. Auditory long latency and cognitive-related potential to auditory stimuli demonstrated functional changes in the cortical auditory pathways. We believe that the frequency of certain findings observed in our patient may be higher in fetal warfarin syndrome than is appreciated, since a significant number result in abortions, stillbirths, or children evaluated in the first year of life without a follow-up. Thus, a multidisciplinary approach and long-term monitoring of these patients may be necessary.

Tratamento estético da columela nasal: columela oculta e columela pendente / Aesthetic treatment of retracted and hanging nasal columellas

Os autores revelam, no presente trabalho, suas abordagens frente às alterações estéticas da columela nasal, mais observadas em sua prática clínica, a columela oculta e a columela pendente. Para a columela oculta os autores descrevem o uso de um retalho condro-mucopericondral do septo nasal como escolha preferencial. Para o tratamento da columela pendente os autores utilizam as abordagens, direta e indireta, descritas por Randall, nos casos leves a moderados, e para os casos mais acentuados utilizam uma associação de táticas além de uma modificação do que é descrito por Armstrong e Joseph. Suas opções de tratamento são confrontadas com o que há descrito na literatura da especialidade e seus resultados comparativos são apresentados.

This study discusses approaches for aesthetic correction of nasal columellas, i.e. retracted and hanging columellas, most commonly observed in clinical practice. We describe a chondral-mucoperichondrial nasal septal flap and direct or indirect approaches described by Randall as our preferred procedures to correct retracted and mild to moderate hanging columella, respectively. A combination of procedures is used in more severe cases, in addition to modified Armstrong and Joseph's approaches. These treatment options are compared to other literature reports on this specialty.

Absent mandibular gap in the retronasal triangle view: a clue to the diagnosis of micrognathia in the first trimester.

OBJECTIVE: To describe a new ultrasound technique that may be useful for the diagnosis of micrognathia in the first trimester of pregnancy. METHODS: The retronasal triangle (RNT) view is a technique that captures the coronal plane of the face in which the primary palate and the frontal processes of the maxilla are visualized simultaneously. Normal first-trimester fetuses display a characteristic gap between the right and left body of the mandible in this view (the 'mandibular gap'). The presence or absence of this gap was evaluated and measured prospectively during real-time scanning (n = 154) and retrospectively by analyzing three-dimensional (3D) datasets (n = 50) in normal first-trimester fetuses undergoing screening for aneuploidy at 11-13 weeks' gestation. 3D datasets from 12 fetuses with suspected micrognathia were also collected and examined retrospectively for the same features. RESULTS: The mandibular gap was identified in all 204 normal fetuses and increased linearly with increasing crown-rump length (y = 0.033x + 0.435; R(2) = 0.316), with no statistically significant differences between measurements obtained by two-dimensional ultrasound and 3D offline analysis. Among fetuses with suspected micrognathia, three 3D datasets were excluded from analysis because of poor image quality in one and the diagnosis of a normal chin in two. In the remaining nine fetuses, the mandibular gap was absent and was replaced by a bony structure representing the receding chin in seven (77.8%) cases and was not visualized due to severe retrognathia in the remaining two (22.2%) cases. All fetuses with micrognathia had associated anomalies, including seven with aneuploidy and two with skeletal dysplasia. CONCLUSIONS: The RNT view may be a helpful technique for detecting micrognathia in the first trimester. The absence of the mandibular gap or failure to identify the mandible in this view is highly suggestive of micrognathia and should prompt a targeted ultrasound scan to assess for other anomalies. Further research is needed to determine the false-positive and false-negative rates of this technique.

Nuchal translucency and nasal bone in first-trimester ultrasound screening for aneuploidy in multiple pregnancies.

OBJECTIVE: To review our experience with first-trimester screening of chromosomal abnormalities in multiple pregnancies using nuchal translucency (NT) thickness measurement and nasal bone assessment. METHODS: Cases of multiple pregnancy presenting for first-trimester ultrasound evaluation and with viable fetuses measuring between 45 and 84 mm were recruited for this study. Crown-rump length, NT thickness, the presence or absence of the nasal bone and chorionicity were determined and the information prospectively collected in a dedicated database. RESULTS: There were 206 twin pregnancies, eight sets of triplets, and one set of quadruplets, totalling 440 screened fetuses. Information on perinatal outcome was available in all cases. The NT thickness was measured in all cases and was found to be greater than the 95(th) percentile in six (8.6%) of the 70 monochorionic fetuses and in 10 (2.7%) of the 370 dichorionic fetuses (P < 0.05, Fisher's exact test). The nasal bone was successfully assessed in 421 of the 440 (95.7%) fetuses and found to be absent in only four cases, three of which were found to have aneuploidy. Chromosomal abnormalities were diagnosed in six fetuses from one monochorionic and four dichorionic twin pregnancies. Five of the six affected cases were associated with increased NT thickness (sensitivity 83.3%), whereas the nasal bone was absent in only three of the aneuploid fetuses (sensitivity 50%). CONCLUSIONS: First-trimester ultrasound screening for chromosomal abnormalities using NT thickness in multiple pregnancies is highly sensitive. However, nasal bone assessment is not only limited in sensitivity but also more challenging in multiple than in singleton pregnancies owing to difficulties in obtaining adequate views of the fetal face.

Clinical and radiographic presentation and preparation of the prototyping model for pre-surgical planning in Apert's syndrome.

Acrocephalosyndactyly, or Apert's syndrome, described nearly a century ago, is a craniofacial dysostosis, an autosomal dominant condition characterized by severe development disturbances of the craniofacial region including bilateral coronal synostosis associated with midface hypoplasia, exophthalmia, hypertelorism, symmetric syndactyly of the hands and feet, cone-shaped calvarium, pharyngeal attenuation and malocclusion. The aim of this study was to assess clinical and computed tomography (CT) imaging patterns of a non-operated patient with Apert's syndrome, correlating the cranium, face and the skull base bone abnormalities. Three-dimensional images were generated from spiral CT scans in order to produce a prototyping model in polyamide material. Clinical examination determined that syndactyly of the hands and feet, pseudocleft in the midline palate and midface hypoplasia were present. The surgical model allowed the analysis of some abnormalities regarding to calvaria morphology, nasal bones and maxilla, improving the criteria for a case diagnosis and surgical plan.

Screening de aneuploidías: presencia de hueso nasal entre las 11-14 semanas: una técnica del nuevo milenio / Screening for aneuploids: presence of nasal bone between weeks 11 and 14: a technique for the new millenium

El screening de aneupliodías antenatal durante el primer trimestre ha evolucionado radicalmente durante los últimos años, asociando métodos tanto bioquímicos como ecográficos. El screening bioquímico al utilizar Pregnancy-associated plasma protein-A (PAPP-A) y la subunidad _-HCG libre presenta una sensibilidad para trisomía 21 de un 40 por ciento y 35 por ciento respectivamente. Sin embargo presenta una tasa de falsos positivos de 5 por ciento. En la búsqueda de mejorar la sensibilidad y disminuir la tasa de falsos positivos han aparecido nuevas técnicas ecográficas. La medición de la translucencia retronucal entre las semanas 11 y 14, mejoró la sensibilidad a un 77 por ciento como marcador único, manteniendo la tasa de falsos positivos. Una nueva técnica es la presencia de hueso nasal entre las semanas 11 y 14, avalada por estudios anatomo-radiológicos. Esta técnica tiene una sensibilidad 60 a 80 por ciento para trisomía 21, con una tasa de falsos positivos de 0.25 a 2,8 por ciento y con la consiguiente disminución de procedimientos invasivos. La técnica es aplicable tanto vía abdominal como transvaginal con una rápida curva de aprendizaje.

Transmaxillary osteogenesis distraction combined with orthopedics and orthodontics in the correction of a severe retrusion of the upper maxilla.

A patient having adequate records and diagnosed as having Binder syndrome is presented. Nasomaxillary hypoplasia requires a definitive treatment, use of bone grafts, upper maxillary osteotomies, and advancement or a combination of both. Bone grafts can be reabsorbed, and complete maxillary advance modifies normal occlusion in a certain way, because the posterior sector is not compromised. The aim of this case treatment is to create a new osseous surface that makes the eruption of the permanent dental pieces easier and gives the correct skeletal position to the upper maxilla with distraction osteogenesis combined with an orthopedic appliance, transmaxillary segment osteotomy, and subsequent orthodontics. The absence of the osseous surface in the upper maxilla and the presence of supernumeraries in the anterior region determined the permanence of most of the temporary dental pieces, resulting in the impossibility of making the exchange to permanent dental pieces. The technique and the devices are simple and easy to manipulate. After the treatment, an adequate dental relation is restored as well as effective advance of the upper maxillary bone; thus, an excellent functional and stable esthetic result is achieved, avoiding any complication of velopharingeal incompetence.

Angular analysis of corpus callosum in 18 patients with frontonasal dysplasia.

Considering the rarity of the frontonasal dysplasia (FD) and the few reports about it in a large casuistry using magnetic resonance image (MRI), we describe the results of the angular analysis of the corpus callosum of 18 individuals with FD (7 male, 11 female), using an easily-reproductive method. Group I had 12 individuals with isolated form and Group II had 6 individuals with FD syndromic with unknown etiology. The results are presented in set. Comparing with the control group, patients with FD presented alpha angle increase and beta and gamma angles reduction (p<0.05). Alpha and gamma angles express the relationship between the anterior portion of corpus callosum and the floor of 4th ventricle. Considering the embryonary development, these findings would occur secondarily to failure during the development of nasal capsula. Thus, angular anomaly in corpus callosum would be a usual finding, and not fortuitous in patients with FD.

Angular analysis of corpus callosum in 18 patients with frontonasal dysplasia

Considerando a raridade da displasia frontonasal (DF) e os poucos estudos sobre esta condição clínica usando ressonância magnética (RM), descrevemos os resultados da análise angular do corpo caloso em 18 indivíduos com DF (7 homens, 11 mulheres), usando um método de fácil reprodução. O Grupo I foi formado por 12 indivíduos com DF isolada e o Grupo II, por 6 portadores de DF sindrômica de etiologia desconhecida. Não houve diferença entre os grupos, e os dados são apresentados em conjunto. Comparando com o grupo controle, houve aumento significativo do ângulo alfa e redução dos ângulos beta e gama (p<0,05) Os ângulos alfa e gama expressam a relação entre a porção anterior do corpo caloso e do piso do 4º ventrículo. Esses achados radiológicos poderiam ocorrer secundariamente à falência do desenvolvimento da cápsula nasal. Assim, as anomalias angulares no corpo caloso poderiam ser um achado usual, e não fortuito, na DF.

Association between first trimester absence of fetal nasal bone on ultrasound and Down syndrome.

OBJECTIVES: To evaluate the association between absence of nasal bone on ultrasound and Down syndrome in fetuses at 11-14 weeks of pregnancy. METHODS: One hundred and ninety-four consecutive fetuses from singleton pregnancies undergoing chorionic villi sampling (CVS) were evaluated by transabdominal ultrasound. A sagittal view of the fetal face was obtained and the absence or presence of nasal bone was recorded and correlated with the fetal karyotype. RESULTS: A successful view of the fetal profile was possible in 183 of 194 (94%) fetuses. The nasal bone was absent in three of five fetuses with Down syndrome, and in one of 175 (0.6%) chromosomally normal fetuses. CONCLUSION: Absence of nasal bone by first trimester ultrasound was significantly associated with Down syndrome. When a proper view of the fetal face was obtained, the nasal bone was visible in more than 99% of karyotypically normal fetuses.

Treatment of the nasal hump with preservation of the cartilaginous framework.

Classically, nasal hump reduction is based on the partial resection of the cartilages and bones of the nose, as it was described by Joseph almost a century ago. The cartilaginous portion of the hump consists of a single unity formed by the two upper lateral cartilages and the septal cartilage. During hump reduction in the classic rhinoplasty, this structure is slashed in three pieces, which is the main cause of irregularities, shadows, and pinchings over the long-term results. Late follow-ups of the classical hump removal often show an inverted V-shaped shadow on the dorsum secondary to the destruction of the osseous-cartilaginous transition. The angle and relation between the septal and upper lateral cartilages are reduced, which may compromise the functional aspect. The destruction of the unique anatomy of the cartilaginous hump is one of the main causes of this functional and aesthetic sequela. Here, we present a technique that preserves the cartilaginous framework of the nasal hump by lowering it through the resection of a strip of septum, avoiding the problems described above.

Síndrome de Binder con manifestaciones ortopédicas asociadas / Binders Syndrome with associated orthopedic malformations

El síndrome de Binder o Displasia Nasomaxilar es una curiosa enfermedad caracterizada por una hipoplasia del tercio medio facial que produce cambios mandibulares compensatorios que traen como resultado la aparición de una maloclusión clase III de Angle. Se han descrito anomalías raquídeas asociadas a esta enfermedad en las personas que la padecen. Por lo interesante que resulta el tema nos proponemos realizar una revisión de la literatura y mostrar una paciente afectada por esta enfermedad, en la que además, se ponen de manifiesto alteraciones asociadas en el raquis cervical y lumbar(AU)

Evaluación quirúrgica y evaluación de las septoplastias en el Hospital Base Goyeneche 1980 a 1995

El presente estudio se realizó con el objeto de evaluar a los pacientes intervenidos quirúrgicamente por desviación septal en el Servicio de Otorrinolaringología del Hospital Goyeneche de Arequipa entre los años 1980 a 1995. El compromiso del tabique nasal tuvo mayor incidencia en el sexo masculino, en su mayoría estudiantes, siendo la causa preponderante la traumática. El cuadro clínico se caracterizó por obstrucción nasal, además de presentar concominantemente deformidad de la pirámide nasal e hipertrofia de cornetes y, algunos casos acompañados de otra patología respiratoria. La desviación septal se localizó mayormente en el cartílago cuadrangular siendo el tipo de deformidad más frecuentemente la desviación. En el acto operatorio la anestesia más utilizada fue la local, al igual que las incisiones de Killian y Cottle en las septoplastías. la evolución fue favorable en su mayoría

Congenital absence of nasal bones.

Isolated congenital nasal malformation is rare; the isolated absence of any specific nasal structure is even rarer. They are related to craniofacial stenosis syndromes and to facial cleft, which are described in Tessier classification; also they can appear in 58 complex genetic syndromes. Nasal malformations may be acquired as a consequence of traumas, tumors, infectious diseases, or sequelae of aesthetic surgery. Gorham's syndrome is a rare disease that produces spontaneous and asymptomatic disappearance of any bone of the skeleton. In the world literature, there is no case of Gorham's syndrome with disappearance of the nasal bones. This case report is of a 20-year-old patient who sought correction of an aesthetic defect produced by a cartilaginous hump without the presence of the nasal bones. The absence of both nasal bones is produced by failure of the development of both centers of ossification. Through study of embryological development of the nasal structure, isolated absence of the nasal bones can be explained.

Síndrome CCC (cuello corto, campodactilia, cardiopatía) con variabilidad: nuevo síndrome autosómico dominante / Syndrome CCC (short neck, camptodactily, cardiopathy) with variability expression: a new autosomic dominant syndrome

Se presenta el cuadro clínico no descrito anteriormente de una familia en la que se encontraron 16 personas que mostraban, en forma constante, cuello corto y ancho, cardiopatía a expensas del ventrículo izquierdo y camptodactilia con hipoplasia de los pliegues de flexión. Todos tenían talla baja con variabilidad en su expresión. Otros datos no constantes fueron paladar alto, depresión del puente nasal, micrognatia, cúbito varo, posición dental anormal e hiperplasia gingival. Al tomar en cuenta las malformaciones constantes se le ha denominado "sindrome CCC", siglas que corresponden a cuello corto, cardiopatía y camptodactilia.