Variaciones anatómicas de los senos frontales en radiografías de cráneo de pacientes con cráneos braquicéfalos / Anatomical variations of frontal sinuses in skull radiographs of patients with brachycephalic skulls

Introducción: La anatomía ósea de las cavidades paranasales presenta múltiples variantes, que pueden ser causa o factor predisponente de enfermedad rinosinusal, entre las que se encuentran las asimetrías y las agenesias. Éstas pueden ser reparos de gran importancia en la cirugía endoscópica nasal y demandan atención médica con frecuencia. Objetivo: Determinar las variaciones anatómicas de los senos frontales en los pacientes con cráneos braquicéfalos y que no presentaron diagnóstico de enfermedad rinosinusal. Métodos: Se realizó un estudio observacional, descriptivo y transversal, donde fueron estudiados los diámetros transversales y longitudinales de los senos frontales a través de radiografías de cráneo AP en 28 pacientes con cráneos braquicéfalos, en el servicio de imagenología del Hospital Juan Bruno Zayas Alfonso en los meses de enero y febrero de 2019. Resultado: Se identificaron las principales variaciones de los senos frontales a través de radiografías de cráneo y su comportamiento según variables epidemiológicas. Conclusiones: Predominó los enfermos (9) en el rango etáreo de 19-29 años, lo que representa un 32,1 por ciento, hubo un ligero predominio de los hombres con respecto a las mujeres, 10 (35,7 por ciento) pacientes presentaron agenesia bilateral y 3 (10,7 por ciento) agenesia unilateral izquierda. Las magnitudes de las cavidades derechas fueron mayores que las izquierdas y los senos frontales estudiados son asimétricos por su tamaño y posición(AU)

Introduction: The bone anatomy of paranasal cavities presents a large number of variants which may be the cause of or predisposing factor for rhinosinus disease, among which are asymmetry and agenesis. These may be very important hurdles in nasal endoscopic surgery and often require medical care. Objective: Determine the anatomical variations in the frontal sinuses of patients with brachycephalic skulls not diagnosed with rhinosinus disease. Methods: A cross-sectional observational descriptive study was conducted of frontal sinus longitudinal and transverse diameters of frontal sinuses based on AP skull radiographs of 28 patients with brachycephalic skulls attending the imaging service of Juan Bruno Zayas Alfonso Hospital from January to February 2019. Result: Identification was made of the main frontal sinus variations in skull radiographs and their behavior according to epidemiological variables. Conclusions: The 19-29 years age group prevailed, with 9 patients (32.1 percent). There was a slight predominance of men versus women. Ten (35.7 percent) patients had bilateral agenesis and 3 (10.7 percent) had unilateral left agenesis. The magnitudes for right cavities were greater than those for left cavities. The frontal sinuses studied were asymmetrical in terms of size and position(AU)

Una revisión de la literatura acerca de las características neuropsicológicas de niños con craneosinostosis simple en diferentes edades / A review of the literature on the neuropsychological characteristics of children with single suture craniosynostosis at different ages

Introduction: Single suture craniosynostosis is the premature closure of one or more cranial sutures. It leads to abnormal skull growth and in some cases to structural and/or functional brain abnormalities. If these anomalies cause a psychomotor development lag and/or cognitive impairment is a controversial topic. This study aims to review the main findings related to psychomotor development and cognitive functioning in children with craniosynostosis, from birth to school age, dividing them into three stages: birth to 3 years, preschool and school. Method: An electronic search was made in PubMed from 2000 to 2015, using the following keywords: "craniosynostosis and neurodevelopment", "craniosynostosis and neuropsychological", "craniosynostosis and cognition". Results: In the early stage of life some children with craniosynostosis can have motor, language and cognition deficits; these impairments are also present in a variable percentage of preschool children, in some cases accompanied with behavior disorders; deficits in attention, executive functions and learning disorders, are reported in school years. Conclusion: Some neuropsychological alterations are found in a variable proportion of patients with craniosynostosis in all stages of development whichare revealed depending on the demands of each age. It is difficult to draw a cognitive profile for this population because of the heterogeneity related to characteristics of the sample, time of assessment, functions assessed and instrument used. It is important to make longitudinal neuropsychological assessments to know how children with craniosynostosis are developing in late stages and to develop consensus about cognitive functions and assessment instruments appropriate for each age.

Introducción: La craneosinostosis simple es el cierre prematuro de una o más suturas craneales que provoca crecimiento anormal del cráneo y en algunos casos anormalidades cerebrales estructurales y/o funcionales. Es un tema controversial si estas anormalidades causan el retraso psicomotor y/o cognitivo reportado en estos pacientes. Esta revisión tiene como objetivo abordar los hallazgos encontrados en el desarrollo psicomotor y cognitivo en niños con craneosinostosis, desde el nacimiento hasta la edad escolar, dividiéndolos en tres etapas: nacimiento a 3 años, etapa preescolar y escolar. Método: Se realizó una búsqueda en PubMed del 2000 al 2015, utilizando estas palabras claves: "craniosynostosis and neurodevelopment", "craniosynostosis and neuropsychological", "Craniosynostosis and cognition". Resultados: En las etapas tempranas de la infancia, los niños con craneosinostosis pueden tener deficiencias motoras, de lenguaje y cognitivas; estos déficits están presentes en un porcentaje variable en la etapa preescolar y pueden acompañarse de alteraciones conductuales; deficiencias atencionales, ejecutivas y de aprendizaje, se reportan en la edad escolar. Conclusión: Se reportan alteraciones neuropsicológicas en un porcentaje variable en estos pacientes en todas las etapas del desarrollo, que se van manifestando conforme las demandas del ambiente. Es difícil establecer un perfil neuropsicológico debido a la heterogeneidad de las características de las muestras en los estudios, el tiempo de la evaluación, las funciones estudiadas y los instrumentos utilizados. Es importante realizar un seguimiento longitudinal para conocer el desarrollo de estos niños, así como el hacer un consenso sobre las funciones cognitivas a evaluar e instrumentos a utilizar para cada edad.

Lip projection analysis in brachycephalic, dolichocephalic and mesocephalic individuals of a Mexican population / Análisis de la proyección labial en pacientes braquiocefálicos, mesocefálicos y dolicocefálicos de una población mexicana

The aim of this study was to examine the lip projection of brachycephalic, dolichocephalic and mesocephalic Mexican patients and to analyze the relationship between dental protrusion and the lip position, to identify if a labial soft tissue projection exists and to determine what features predominated in this study. A total of 120 lateral radiographs of the skull were randomly selected from patients aged 16-25 years. The linear and angular data were measured, the values of the upper and lower lip projection relative to Ricketts E-line, Steiner S-line and Arnett TV-line were collected. The angles to be analyzed were the nasola bial, mandibular plane and interincisal, and the mentolabial groove depth. The statistical sig nificance was determined by the Student t-test. A significant difference between mesocephalic and brachycephalic patients was observed measuring the angle between the Frankfurt plane and the mandibular plane (P<0.001). Between dolichocephalic and mesocephalic individuals significant differences were observed in the lower lip to E-line (P<0.031), lower lip to S-line (P<0.010), the interincisal angle (P<0.032) and the mandibular ́s plane (P<0.001). A statistical significant difference was shown between brachycephalic and dolichocephalic individuals: lower lip to E-line (P<0.001), upper lip to S-line (P<0.037), lower lip to S-line (P<0.001), interincisal angle (P<0.034) and the angle between the mandibular ́s plane (P<0.001). Lip soft tissue projection will depend on the population studied; we found some significant differences when compared with the cephalometric norms.

El objetivo del presente estudio fue examinar la proyección labial en pacientes braquicéfalos, mesocéfalos y dolicocéfalos mexicanos al igual que analizar la relación entre la protrusión dental y la posición labial, identificar si existe proyección labial de los tejidos blandos y determinar las características predominantes en el estudio. Un total de 120 radio grafías laterales de cráneo se seleccionaron de manera aleatoria de pacientes entre 16-25 años. Los datos angulares y lineales se identificaron, los valores de la proyección labial superior e inferior con respecto a línea línea-E de Ricketts, línea-S de Steiner y línea-VV de Arnett fueron recolectados, los valores angulares a analizar fueron el ángulo nasolabial, el plano mandibular y el ángulo interincisal al igual que el surco mentolabial. Se usó la prueba T de Student para obtener la significancia. Una diferencia significativa se observó entre pacientes mesocéfalos y braquicéfalos en el plano mandibular con Frankfort (P <0.001). Entre dolicocéfalos y meso céfalos diferencias significativas se observaron en el labio inferior a línea-E (P <0.031), labio inferior a S (P <0.010), ángulo interincisal (P <0.032) y el plano mandibular (P <0.001). Diferencias significativas se observaron entre pacientes braquicéfalos y dolicocéfalos: labio inferior a línea-E (P <0.001), labio superior a línea-S (P <0.037), labio inferior a línea-S (P <0.001), ángulo interincisal (P < 0.034) y el plano mandibular (P <0.001). La proyección labial depende de la población y se pueden observar algunas diferencias significativas al ser comparadas con la norma cefalométrica.

Chiari IV Malformation, the Lückenschädel and Sagittal Craniosynostosis Association: Case Report and Literature Review / Associação entre malformação de Chiari IV, Lückenschädel e craniossinostose sagital: relato de caso e revisão de literatura

The association between Lückenschädel and craniosynostosis is unusual and unknown. Genetic origin is a possibility, representing one of many possible phenotypes for mutation. To the best of our knowledge, the association of such anomalies in a type IV Chiari malformation has never been reported before. The authors present the case of a patient with obstructive hydrocephalus, diagnosed with Chiari IV malformation associated with Lückenschädel and sagittal craniosynostosis. The Lückenschädel is the bone abnormality least commonly associated with Chiari malformation. It consists of a defect in the bones ofmembranous origin that formthe cranial vault. This anomaly arises from periosteal dysplasia, and is characterized by rounded and irregular gaps in the skull, bound by bony ridges. Craniosynostosis is due to premature fusion of the cranial sutures and is sometimes associated with the Chiari complex of malformations. The diagnostic of Lückenschädel and craniosynostosis is done by imaging, through which the skull assumes the aspect of a hive, characteristic of Lückenschädel and it is possible to see the premature fusion of the sutures. The Lückenschädel usually does not require treatment because of spontaneous resolution; whereas, craniosynostosis warrants surgery for aesthetic and functional reasons. The Chiari malformation IV only requires treatment when it results in syringomyelia or hydrocephalus.

O Lückenschädel e a craniossinostose são uma associação incomum e pouco conhecida, com possível origem genética, o que representa um dos muitos fenótipos possíveis para uma mutação. Até onde sabemos, a associação destas anomalias em um caso de malformação de Chiari tipo IV não foi relatada antes. Os autores apresentam o caso de um paciente com hidrocefalia obstrutiva, que foi diagnosticado com malformação de Chiari tipo IV associada com Lückenschädel e craniossinostose sagital. O Lückenschädel é a anormalidade óssea menos comum associada com a malformação de Chiari. É constituída por umdefeito nos ossos de origem membranosa que formam a abóbada craniana. Esta anomalia surge de displasia do periósteo e é caracterizada por falhas arredondadas e irregulares no crânio, delimitadas por cristas ósseas. A craniossinostose se deve à fusão prematura das suturas cranianas e é por vezes associada com o complexo de malformações de Chiari. Os diagnósticos de Lückenschädel e craniossinostose são feitos por imagem, onde o crânio assume um aspecto de colmeia, característico de Lückenschädel, e é possível ver a fusão prematura das suturas. O Lückenschädel geralmente não requer tratamento por causa de resolução espontânea e craniosynostosis necessita de cirurgia para fins estéticos e funcionais. A malformação de Chiari IV só requer tratamento quando resulta em siringomielia ou hidrocefalia.

Comparison of periodontal parameters in individuals with syndromic craniosynostosis

Craniosynostosis syndromes are characterized by premature closure of one or more cranial sutures, associated with other malformations, the most frequent of which are the Crouzon and Apert syndromes. Few studies in the literature have addressed the oral health of these individuals. The purpose of this study was to compare the periodontal status of individuals with Apert, Crouzon, Pfeiffer and Saethre-Chotzen syndromes before toothbrushing and compare the efficiency of plaque removal before and after mechanical toothbrushing. The probing depth, plaque index (according to Lõe and O'Leary), clinical attachment level, gingival index (according to Silness and Lõe) and amount of keratinized mucosa were evaluated before toothbrushing, and the O'Leary plaque index was assessed before and immediately after toothbrushing, on the same day, in 27 individuals aged 11 to 36 years. There was statistically significant difference in the mean probing depth and clinical attachment level among regions (p=0.00; p=0.01, respectively). The gingival index did not reveal statistically significant differences. With regard to the plaque index, the left region exhibited higher plaque index values than the right and anterior regions. No significant results were found in the analysis of keratinized mucosa. Comparison of the O'Leary plaque index before and after toothbrushing revealed statistically significant difference for all syndromes except for the Pfeiffer syndrome (p<0.05). In conclusion, there was no difference in the periodontal status among individuals with syndromic craniosynostosis. The posterior region was more affected than the anterior region as to the presence of plaque, loss of insertion and probing depth. Individuals with Pfeiffer syndrome exhibited greater toothbrushing efficiency than individuals with the other craniosynostosis syndromes.

Síndromes de Apert e Crouzon: características de interesse odontológico e relato de casos clínicos / Apert and Crouzon syndromes: dental interest characteristics and case reports

As síndromes de Apert e Crouzon são doenças do grupo das craniosinostoses. São condições raras, de origem autossômica, que resultam em fechamento precoce das suturas cranianas, além de diversas anomalias, incluindo crânio em forma de torre, hipoplasia maxilar, maloclusão dentária, entre outras. A síndrome de Apert, no entanto, diferencia-se pela presença de sindactilias palmares e plantares. O objetivo deste trabalho é descrever as principais características dessas síndromes e descrever dois casos clínicos de pacientes em tratamento na Clínica de Ortodontia da PUC Minas.

Atypical presentation of Prader-Willi syndrome with Klinefelter (XXY karytype) and craniosynostosis

A síndrome de Prader-Willi é afecção genética de deficiência mental associada a hipogonadismo hipogonadotrófico, hiperfagia e obesidade. Descrevemos o caso de menino de 4 anos de idade, filho de casal consangüíneo, apresentando três condições clínicas não relacionadas: síndrome de Prader-Willi, cariótipo 47,XXY (compatível com síndrome de Klinefelter) e craniossinostose coronal. Ao nosso conhecimento, não foi relatado caso semelhante previamente na literatura.

Cerebral Salt Wasting Syndrome After Calvarial Remodeling in Craniosynostosis

Hyponatremia and increased urine output after calvarial remodeling have been noted in pediatric patients with craniosynostosis. If not treated properly, patients develop hypoosmotic conditions that can lead to cerebral edema, increased intracranial pressure, and collapsed circulation. Postoperative hyponatremia after central nervous system surgery is considered as the syndrome of inappropriate antidiuretic hormone (SIADH) secretion. Recently, however, cerebral salt wasting syndrome (CSWS) instead of SIADH has been reported frequently. CSWS is associated with a decreased serum sodium level, increased urinary sodium level, increased urine output, decreased ECF volume, increased atrial natriuretic peptide (ANP) level, and increased brain natriuretic peptide (BNP) level. We experienced nine patients with craniosynostosis who underwent calvarial remodeling. By postoperative day 1, the ANP and BNP levels increased by 3-6 folds compared with the preoperative levels. They returned to the normal levels by postoperative day 5. The ADH level was within the normal range even after operation. The urinary sodium level increased in all patients by postoperative day 1 and 3. But the serum sodium level, and serum and urine osmolarity were normal due to appropriate replacement of sodium and fluid. After calvarial remodeling, the potential development of CSWS should be considered and distinguished from SIADH. The patients with CSWS require normal saline resuscitation and should prophylactically receive normal saline.

Positional moulding in premature hydrocephalics.

Seven premature hydrocephalics presenting with lambdoid positional moulding (LPM) were reviewed. All were treated for hydrocephalus secondary to aqueductal stenosis, Dandy Walker Syndrome and infection. Parenchymal hemorrhage, intraventricular bleed, cortical atrophy, septal agenesis, cortical anomalies and subdural hygroma were the other common associations. These children did not show expected improvement in their higher mental functions at 6 months to 5.4 years of follow-up, following the management of hydrocephalus. It was not the LPM but associated intracranial anomalies, which were most probably responsible for their poor outcome. The differentiation from posterior plagiocephaly is also highlighted.

Dificuldade de intubaçäo traqueal em paciente com craniossinostose: relato de caso / Tracheal intubation difficulty in patient with craniosynostosis: case report

Justificativa e objetivos - Existem formas complexas de craniossinostose acompanhadas de malformações da face e das vias aéreas, que podem levar a dificuldade de intubação traqueal (IOT). O objetivo deste relato é apresentar um caso de intubação traqueal difícil, em criança submetida à cirurgia para correção da craniossinostose. Relato de caso - Criança com 57 dias, 3700 gramas, perímetro cefálico 39 cm, ASA II, com craniossinostose, retrognatia, macroglosia e exoftalmo bilateral, dificuldade inspiratória e retração intercostal leve. Após decúbito dorsal, coxim sob os ombros, pré-oxigenação e infusão de propofol (10 mg) e succinilcolina (5 mg), realizou-se laringoscopia (lâmina nº1 reta), sem visualização da epiglote. Foi feita ventilação assistida seguida de novas tentativas (4) de IOT, sem sucesso. Optou-se pela ventilação espontânea assistida e IOT, novamente sem sucesso. Pela capnografia não havia CO2 exalado. Com auxílio de fibroscópio intubou-se a traquéia, porém a sonda era estreita e foi trocada por outra com balonete. A SpO2 era de 98 por cento, porém com instabilidades, ás vezes com 60 por cento. A PetCO2 apresentava hipocapnia com morfologia irregular. Á auscultura mostrava ventilação pulmonar bilateral, por com entrada de ar no estômago. Suspeitou-se de fístula tráquo-esofágica traumática. Com endoscopia esofágica constatou-se que a sonda de intubação estava no esôfago; como o balonete foi insuflado na cavidade oral posterior, que estava edemaciada, impedia o vazamento de gás. O volume corrente ventilava o estômago e os pulmões simultaneamente. A sonda foi introduzida corretamente na traquéia com fibroscópio e realizou-se a cirurgia. Conclusões - É necessário distinguir craniossinostose simples daquelas acompanhadas de malformações faciais e de vias aéreas. E fundamental que se tenha equipamentos adequados para IOT e além da monitorização básica, a capnografia é de grande valor na confirmação da intubação traqueal

Dois casos de craniossinostoses e a importância do diagnóstico precoce / The two cases of craniosynostosis and the importance of early diagnosis

As craniossinostoses (CSO) ou, como säo classicamente denominadas, "cranioestenoses" säo entidades já bem conhecidas há mais de vinte séculos e que, se näo adequadamente manejadas, trazem inúmeras complicaçöes principalmente no que diz respeito à limitaçäo do espaço intracranial e deformidades craniais, hipertensäo intracraniana e retardo no desenvolvimento neuropsicomotor. Neste trabalho, os autores descrevem aspectos importantes sobre essa entidade no que se refere a: histórico, etiopatogenia, classificaçäo, tratamento cirúrgico e, ainda, através do relato de dois casos distintos de CSO, um de sutura sagital e outro de sutura bicoronal, demonstram a importância do diagnóstico do tratamento precoce de modo a otimizar os resultados cirúrgicos e prevenir as temidas complicaçöes.

The orbital development on patients with craniosynostosis

O desenvolvimento orbital embrionário é afetado pela incidência de craniossinostose devido a uma fusäo precoce das suturas cranianas. O desenvolvimento embriológico e o estudo do tratamento cirúrgico desta condiçäo é executado. A finalidade deste artigo foi permitir um melhor conhecimento para alcançar um diagnóstico precoce e preciso, que säo os instrumentos para obter bons resultados clínicos

Craneoestenosis asociada a síndrome de malformaciones múltiples / Craniosynostoses associated with multiple malformations syndrome

Esta revisión se hizo con el objeto de analizar la variabilidad clínica, los fundamentos para el diagnóstico y el manejo ofrecido a los pacientes con craneoestenosis. Se revisaron los expedientes de los niños que asistieron al Instituto Nacional de Pediatría en un período de 10 años (1971-1981) con este diagnóstico. El diagnóstico se basó en el cuadro clínico y se confirmó con estudio de gabinete. Hubo 27 pacientes con craneoestenosis asociada a un síndrome conocido. De éstos, 11 correspondieron al síndrome de Crouzon; 11 a síndrome de Apert; 4 tenían anomalía de cráneo en trébol; 2 síndrome de Pfeiffer y 1 síndrome de Saethre-Chotzen. Veinte pacientes fueron operados; de éstos 4 fallecieron por complicaciones postquirúrgicas