The Discovery of a Bone in the Anterior Fontanelle of the Skull of the Sheep (Ovis aries) / Descubrimiento de un Hueso en la Fontanela Anterior del Cráneo de la Oveja (Ovis aries)

SUMMARY: A comparative study of the skull morphology was conducted using 270 prenatal and 750 postnatal skull samples from three breeds of sheep in Nigeria namely, Balami, Uda and Yankasa. A unique bone peculiar to the Yankasa breeds of sheep was found consistently at the centre of the anterior fontanelle in the young (day-old to 1 year). At two years of age and above, the bone was fused and disappeared completely. This brings the total numbers of the neurocranium bones of the skull in the Yankasa breeds to 8, as against the 7 bones documented in the ovine species. Due to the fact that this bone has not been described in the literature, we venture to name it the anterio-fontanelle bone (of Atabo).

Se realizó un estudio comparativo de la morfología del cráneo utilizando 270 muestras de cráneos prenatales y 750 postnatales de tres razas de ovejas en Nigeria, Balami, Uda y Yankasa. Un hueso único peculiar de las razas de ovejas Yankasa se encontró consistentemente en el centro de la fontanela anterior en las crías (de un día a 1 año). A los dos años de edad o más, el hueso se fusionó y desapareció por completo. Esto eleva el número total de huesos del neurocráneo en el cráneo en las razas Yankasa a 8, frente a los 7 huesos documentados en la especie ovina. Debido a que este hueso no ha sido descrito en la literatura, nos aventuramos a denominarlo hueso antero-fontanela (de Atabo).

Evaluación tomográfica de la fontanela anterior de pacientes pediátricos en la ciudad de Quito / Tomographic evaluation of the anterior fontanelle in pediatric patients in the Quito city

INTRODUCCIÓN. La evaluación del tamaño y osificación de las suturas craneales pudo determinar alteraciones del desarrollo cerebral. El estudio realizó la evaluación tomográfica del tamaño de la fontanela anterior y determinó el porcentaje de osificación en función de la edad. MATERIALES Y MÉTODOS. Se realizó un estudio retrospectivo, observacional, en el que se evaluó 367 tomografías computarizadas de cráneo en niños y niñas hasta 2 años de edad, desde enero de 2012 hasta agosto de 2015, en cuatro centros de imagen de la ciudad de Quito, Ecuador. RESULTADOS. El tamaño promedio de la fontanela anterior medida en tomografía computarizada con la fórmula de Popich y Smith, fue de 48,43 mm SD (15,15mm) en hombres y 37,36mm SD (13,97mm) en mujeres, en el grupo de edad de 0 a 3 meses, sin diferencia estadística significativa (p=> 0,05). El 8,0% de los pacientes tuvo una fontanela osificada a la edad de 5 meses. La edad media de osificación de la fontanela fue 18 meses. En el grupo de edad de 21 a 24 meses el porcentaje de osificación fue de 82,0%. CONCLUSIÓN. Este estudio proporcionó valores de referencia que detallaron el porcentaje de tamaño y osificación de la fontanela anterior en función de la edad. Existió amplia variabilidad del tamaño medio de la fontanela anterior, lo cual sugiere que el cierre prematuro o tardío podría considerarse como variantes de la normalidad.

INTRODUCTION. The evaluation of the size and ossification of the cranial sutures could determine alterations in brain development. The study performed the tomographic evaluation of the size of the previous source and determined the percentage of ossification in the function of age. MATERIALS AND METHODS. A retrospective, observational study was conducted in which 367 cranial CT scans were evaluated in children up to 2 years of age, from January 2012 to August 2015, in four imaging centers in the city of Quito, Ecuador. RESULTS The average size of the previous source in computed tomography with the formula of Popich and Smith was 48,43 mm SD (1515 mm) in men and 37,36 mm SD (13,97 mm) in women, in the group of age from 0 to 3 months, without significant statistical difference (p=> 0.05). 8,0% of patients had a source after 5 months. The average age of the source of the source was 18 months. In the age group from 21 to 24 months, the percentage of ossification was 82,0%. CONCLUSIONS This study provides reference values that detail the percentage of size and the classification of the previous source in the function of age. There is wide variability of the average size of the previous source, which implies premature closure or possible delay as variants of normality.

Características clínicas, radiológicas y auxológicas de pacientes con displasia cleidocraneal seguidos en un hospital pediátrico de referencia en Argentina / Clinical, radiological, and auxological characteristics of patients with cleidocranial dysplasia followed in a pediatric referral hospital in Argentina

La displasia cleidocraneal es una displasia esquelética autosómica dominante causada por mutaciones en el gen RUNX2, con una prevalencia estimada de 1/1 000 000 de recién nacidos. Se presentan 37 pacientes (22 mujeres) evaluados entre 1992 y 2016 en las clínicas de displasias esqueléticas, Hospital Garrahan, Argentina. Hallazgos: 35% de antecedentes familiares positivos; edad mediana al momento del diagnóstico: 2,61 años; características radiológicas positivas en el cráneo y el pubis: 95%; en las clavículas: 100%. Las complicaciones dentales y auditivas fueron comunes. Auxología: mediana de estatura de -1,81 (-3,26-0,2) DE en los varones, -1,36 (-4,28-1,36) DE en las mujeres. Cinco de trece pacientes fueron bajos para la estatura parental. Estatura adulta (mediana): 162,8 cm y 149,2 cm en los varones y las mujeres. No fueron evidentes alteraciones en la proporción estatura sentada/estatura. Un paciente presentó macrocefalia real; 12 (32%), macrocefalia relativa. Se describe variabilidad intrafamiliar de estatura.

Cleidocranial dysplasia is an autosomal dominant skeletal dysplasia caused by mutations in the RUNX2 gene; its prevalence has been estimated at 1/1 000 000 newborn infants. This study presents 37 patients (22 girls) assessed between 1992 and 2016 at the Skeletal Dysplasias Multidisciplinary Clinics of Hospital Garrahan, Argentina. Findings: 35% of positive family history; median age at the time of diagnosis: 2.61 years old; positive radiological findings in the skull and pubis: 95%; in the clavicles: 100%. Dental and hearing complications were common. Auxology: boys had a median height of -1.81 SD (-3.26 to 0.2) and girls had a median height of -1.36 SD (-4.28 to 1.36). Five out of 13 patients were short for parental height. Adult height (median): 162.8 cm in boys and 149.2 cm in girls. No evident alterations were observed in the sitting height/height ratio. One patient had true macrocephaly; 12 (32%), relative macrocephaly. Intrafamily variability was described in terms of height.

Cleidocranial dysplasia- a case report and literature review

Cleidocranial dyspalsia is a rare congenital skeletal disorder, associated with hypoplasia or aplasia of clavicles, delayed closure of cranial sutures and fontanels, frontoparietal bossing, delayed exfoliation of primary dentition, delayed or failure of eruption of permanent teeth, and presence of multiple supernumerary teeth. The disorder is caused by mutation in the CBFA1 gene, on the short arm of chromosome 6p21. Estimated prevalence ofcleidocranial dysplasia is one per million, without gender or ethnic predilection. A multidisciplinary approach is often required for dental management of multiple supernumerary teeth. We report clinical and radiographic presentation and surgical management of multiple supernumerary teeth in an 11-year-old child with cleidocranial dysplasia.

Trombosis de senos venosos cerebrales en menores de dos meses de edad y sus secuelas / Cerebral sinovenous thrombosis in children under two months old and sequels

Antecedentes: La trombosis de senos venosos cerebrales ( TSVC) es un padecimiento infre- cuente que puede tener alta mortalidad y secuelas neurológicas severas, por su variada sintomatología puede ser mal diagnosticada. Objetivo: Determinar la presentación clínica, laboratorial y tomográfica, así como las secue- las en niños menores de 2 meses de edad con trombosis de senos venosos cerebral atendi- dos en el Hospital Nacional Mario Catarino Rivas (HNMCR) de enero del 2009 a febrero del 2013. Pacientes y Métodos: estudio descripti- vo con diseño longitudinal. La muestra fue de 14 niños menores de 2 meses de edad a quie- nes se diagnosticó TSVC y se obtuvieron datos clínicos, laboratoriales y tomográficos. Se les realizó seguimiento para determinar las secue- las psicomotoras. Resultados: las manifesta- ciones clínicas reportadas fueron fontanela tensa en todos los pacientes, convulsiones en el 86% y deshidratación en el 71%. Todos presentaron anemia con niveles de hemoglobi- na promedio de 6.7 g/dl. La tomografía cere- bral mostró afectación de los senos: sagital superior 100%, lateral izquierdo 78.6%, lateral derecho 71.4% y recto 50%. 7 niños tuvieron retraso psicomotor, 4 tuvieron desarrollo normal y 3 fallecieron. Conclusión: La TSVC sigue siendo una patología vascular poco com- prendida y posiblemente sub diagnosticada que amerita mayor atención del cuerpo médico...(AU)

Development of fontanelle and paranasal sinuses in the skull of prenatal buffalo (Bubalus bubalis) / 대한수의학회지

The maxillary sinus appeared first among the sinuses of the skull at 92 days of prenatal stage. The early formation of frontal sinus was observed at 157 days in the cranial most part of the frontal bone while the early formation of palatine sinus was observed at 170 days. A total of five fontanelles were observed in prenatal skulls of buffalo. The largest and unpaired fontanelle was anterior fontanelle. The mastoid and sphenoidal fontanelle were paired. The ossification of anterior fontanelle was first observed at 164 days. All the fontanelles were ossified completely prenatally.

Congenital inclusion dermoid cyst of the posterior fontanel: a case report

Background: Dermoid cysts are rare and benign tumors originating from failure of the normal embryologic development. Congenital inclusion dermoid cysts are usually located over the anterior fontanel. Surgical treatment is curative and provides good aesthetic results. The authors present a rare case of congenital inclusion dermoid cyst over the posterior fontanel and discuss the location, differential diagnosis, treatment and prognosis of this unusual pathological entity. Case description: A two-year old boy presented with a lesion on the posterior aspect of the head which had dated since birth. Imaging studies revealed an extracranial lesion over the posterior fontanel without intracranial extension. Surgical removal was performed and the histopathological examination confirmed the diagnosis of a dermoid cyst. The patient was discharged free of any medical complications. Conclusion: Although congenital inclusion dermoid cysts are more common over the anterior fontanel, they can occur over the posterior fontanel as well. Surgical treatment is curative in both locations with favorable aesthetic results.

Changes of anterior fontanel size in children aged 0 - 2 years / 中华儿科杂志

<p><b>OBJECTIVE</b>To study the development of anterior fontanel(AF) in children less than 2 years of age.</p><p><b>METHOD</b>The size of AF of the children under 2 years of age was measured. The criteria were: (1) All the children were singletons and term (37 weeks ≤ gestational age ≤ 40 weeks) at birth, birth weight > 2500 g. (3) Those with intracranial diseases (included trauma and asphyxia) and scalp hematoma were ruled out. (3) Healthy children (without intracranial disease, growth retardation, congenital syndrome or bone metabolic diseases such as rickets).</p><p><b>RESULT</b>(1) The mean value of AF in neonates was 1.5 (0.3 - 2.5) cm, and the average of the AF at 1 month after birth was 2.2 cm, which was the largest one. The size of AF was 1.0 (0.3 - 2.0) cm at age 12 months, and 0.5 (0.3 - 0.7) cm at 24 months. (2) The percentage for the closure of the AF was 3% at 6 months, 26.5% at 12 months, and 93.0% at 24 months. (3) There were no gender differences in the size of the AF (P > 0.05). And the size of AF was not correlated with the development levels of weight, length, and head circumference (P > 0.05).</p><p><b>CONCLUSION</b>(1) The size of AF at 1 month was maximum (2.2 cm), and then decreased by years. The AF was almost closed (93%) at 24 months. (2) There were no gender differences in anterior fontanel (P > 0.05). The size of AF was not correlated with the growth of weight, length, and head circumferences (P > 0.05). (3) The fontanel dimensions should be represented by oblique diameters of the fontanel in clinical pediatrics. (4) The AF closure time needs to be further evaluated in normal children.</p>

Evaluation of congenital hypothyroidism in Fars province, Iran

In Iran thyroid-stimulating hormone [TSH] based neonatal screening program is included in health care services from 2005 for detection of patients with primary congenital hypothyroidism [CH]. This study was performed for a critical evaluation of the screening program primary congenital hypothyroidism in Fars province, Iran. From November 2006 to September 2007, TSH serum concentrations of 63031 newborns, 3 to 5 days old born in Fars province, were measured by heel prick. The newborns with TSH >/= 5mIU/L were recalled for measurement of serumT4 and TSH in venous blood samples Of 127 recalled subjects, 43 were confirmed to be hypothyroid, showing a prevalence of 1:1465 with F:M ratio of 1.05:1. The most common clinical and radiological findings were prolonged jaundice [73%], large anterior fontanel [56%], wide posterior fontanel [55%], absence of distal femoral epiphysis [20%], and umbilical hernia [11%]. Scintigraphy of the thyroid with [99m]TC revealed eutopia [67.4%], hypoplasia [23.3%], agenesis [4.7%] and ectopia [2.3%]. It is concluded that a cut off value of TSH >/= 5mIU/L overestimates recalling the number of patients with CH. The most common cause of congenital hypothyroidism is not dysgenesis of the gland and perhaps dyshormonogenesis in Iran is more common than what is reported in other countries

Ultrasonografía craneal neonatal / Neonatal cranial ultrasonography

La neuroecografía está posicionada en la práctica clínica, de acuerdo a la literatura consultada, como un procedimiento con una especificidad y efectividad que permite dar un reporte diagnóstico con seguridad. Esta técnica tiene como ventajas la exploración en tiempo real, el carácter atóxico e inocuo del procedimiento y el corto tiempo en el que se realiza, según la experticia del realizador. Como herramienta diagnóstica en recién nacidos pretérminos una de las principales indicaciones para la solicitud del procedimiento es poder hacer la identificación de las alteraciones visibles en la anatomía macroscópica, teniendo en cuenta las ventajas proporcionadas por la ventana acústica utilizada en el estudio sonográfico, las cuales dependen principalmente de la proximidad del transductor a la neuroanatomía que se desea explorar. [Ustáriz CG, Redondo LC, Avendaño LC, Noguera EC. Ultrasonografía craneal neonatal, revisión de tema. MedUNAB 2012; 15(3): 167-174].

According to the literature, the neurosonography technique is positioned in clinical practice as a procedure with a specificity and effectiveness which allows a safe diagnostic report. This technique has the advantages of real-time scanning, the non-toxic and harmless character of the procedure, and the short time in which is performed according to the expertise of the physician. Used as a diagnostic tool in preterm newborns, one of its most significant applications is the identification of the visible changes in macroscopic anatomy, taking into account the advantages provided by the acoustic window used in the sonographic study, which in turn depends mainly on the proximity of the transducer to the neuroanatomy to be scanned. [Ustáriz CG, Redondo LC, Avendaño LC, Noguera EC. Neonatal cranial ultrasonography. MedUNAB 2012; 15(3):167-174].

Mysterious Foreign Body in Transverse Sinus

This case report describes a patient who had a foreign body in transverse sinus. A 35-year-old Korean-Chinese man visited the emergency room with lacerated wound in left eyelid and a foreign body which was stumbled upon in the skull. On examination, there was right side hemianopsia in his left eye. He did not complain any headache or show any abnormal neurological signs, but there was a foreign body at left transverse sinus in computed tomography which was taken at another hospital. There was no intracranial abnormality except the foreign body in computed tomography. Because of the financial problem, additional evaluations were not possible. We herein report a strange case in which the pathway of a foreign body to locate in transverse sinus was ambiguous, and suggest that the foreign body located in transverse sinus might have been the penetrated along the anterior fontanelle and passed through the superior sagittal sinus.

Anatomic Illustrations of Cranial Ultrasound Images Obtained Through the Mastoid Fontanelle in Neonates

PURPOSE: Neonatal cranial sonongraphy performed through the mastoid fontanelle is more useful to evaluate the peripheral structures at the convexity of the cerebral hemispheres and brainstem rather than that performed through the anterior fontanelle. The purpose of this study is to demonstrate the anatomy of the extracerebral CSF space and brainstem and to suggest appropriate scan planes for performing neonatal cranial sonography through the mastoid fontanelle using MRI and multiplanar reconstruction programs. MATERIALS AND METHODS: A neonate with normal features on ultrasonography and good image quality on MRI, including the 3D-SPGR axial scans, was selected. We made the reconstructed MR images corresponding to the sonongraphic planes and the anatomic models of the neonatal cranial sonographic images by using axial MRI as the standard reference on the same screen. We demonstrated the sonographic images at the levels of the body of the caudate nucleus and lentiform nucleus, the head of the caudate nucleus and thalamus, the third ventricle and midbrain, and the midbrain and cerebellar vermis on the oblique axial scans. Four oblique coronal images at the levels of the periventricular white matter, basal ganglia, thalamus and tentorium were also obtained. RESULTS: We illustrated the anatomic atlas with including four oblique axial scans and four oblique coronal scans that corresponded to the neonatal cranial sonographic images through the mastoid fontanelle. CONCLUSION: We objectively analyzed the anatomy of the extracerebral CSF space and brainstem by using MRI and multiplanar reconstruction programs and we provided the standardized sonographic scan planes through the mastoid fontanelle. This study will be very helpful for evaluating the abnormalities of the peripheral structures at the convexity of the cerebral hemispheres and brainstem.

Anatomic Illustrations of Cranial Ultrasound Images Through the Posterior Fontanelle in Neonates: Objective Analysis of Oblique Sonographic Scans using MRI and a Reconstruction Program

PURPOSE: Neonatal cranial sonongraphy through the posterior fontanelle is more useful than through the anterior approach for the evaluation of posterior brain structures. The aims of this study were to determine the appropriate neonatal cranial sonography scan planes through the posterior fontanelle, and to objectively evaluate the anatomy of neonatal cranial sonographic images. MATERIALS AND METHODS: Neonates who underwent cranial sonography and MRI including 3D-SPGR axial scans and showed normal features on both modalities were enrolled. We reconstructed MR images corresponding to sonongraphic planes, then constructed anatomic models of the neonatal cranial sonographic images using axial MRI as the standard reference on the same screen. RESULTS: We successfully created anatomic atlas that represents 8 oblique coronal and 4 oblique sagittal scans and planes that corresponded to the neonatal cranial sonographic images through the posterior fontanelle. CONCLUSION: The objective manner of this anatomic research provided standardized sonographic scan planes and created anatomic model through the posterior fontanelle. Cranial sonographic models through the posterior fontanelle using MRI and multi-planar reconstruction program will be helpful in the evaluation of sonographic anatomy and detection of abnormalities in the basal ganglia, thalamus and posterior part of the brain.

Hipotireoidismo congênito: perfil clínico dos recém-nascidos identificados pelo Programa de Triagem Neonatal de Minas Gerais / Congenital hypothyroidism: the clinical profile of affected newborns identified by the Newborn Screening Program of the State of Minas Gerais, Brazil

OBJETIVO: Avaliar o perfil clínico de recém-nascidos com hipotireoidismo congênito identificados pelo Programa Estadual de Triagem Neonatal de Minas Gerais entre 2000 e 2006. MÉTODOS: A frequência das manifestações clínicas de hipotireoidismo congênito foi analisada em relação aos níveis de hormônio estimulante da tireoide T4L (determinados por quimioluminescência, valores de referência de 0,3 a 5,0 µUI/mL e 0,8 a 1,8 ng/dL, respectivamente) e idades ao diagnóstico e tratamento. Estudou-se 443 crianças, sendo 55,8 por cento do sexo feminino e 95 por cento delas com menos 60 dias de vida. RESULTADOS: Os sinais clínicos mais prevalentes foram: hérnia umbilical (51 por cento), fontanela anterior ampla (50,3 por cento) e fontanela posterior aberta (47,2 por cento). Hipotonia, macroglossia e sucção débil foram os sinais mais associados com a gravidade bioquímica da doença. Em 32,1 por cento das crianças houve atraso na idade óssea ao diagnóstico. As medianas de TSH e T4L séricos foram 120 µUI/mL e 0,62 ng/dL, respectivamente. A mediana da idade de início de tratamento foi de 28 dias. CONCLUSÃO: Existem sinais clínicos precoces que sugerem o diagnóstico de hipotireoidismo congênito. Portanto, diante de uma criança com esses sinais, devem ser avaliados TSH e T4L séricos para confirmar ou excluir a doença, independentemente do resultado do teste de triagem. A idade de início de tratamento ainda foi elevada, mas o programa vem adotando estratégias para redução da mesma.

OBJECTIVE: To evaluate the clinical profile of newborns with congenital hypothyroidism identified by the Newborn Screening Program of the State of Minas Gerais, Brazil, between 2000 and 2006. METHODS: Analysis of factors involved in this profile, including: TSH and FT4 levels (determined by chemiluminescence, with limits of normality set at 0.3-5.0 µUI/mL and 0.8-1.8 ng/dL, respectively), age at diagnosis and age at treatment. The study sample consisted of 443 children, 55.8 percent were female and 95 percent were seen before completing 60 days of life. RESULTS: The most prevalent clinical signals were: umbilical hernia (51 percent), enlarged anterior fontanel (50.3 percent), and open posterior fontanel (47.2 percent). Hypotonia, macroglossia and feeding difficulties were the clinical signs most frequently associated with the biochemical severity of the disease. A delay in bone age was present in 32.1 percent of the children at diagnosis. The median of serum TSH and FT4 was 120 µUI/mL and 0.62 ng/dL, respectively. The median age at start of treatment was 28 days. CONCLUSION: There are some early clinical signs that suggest a diagnosis of congenital hypothyroidism. Therefore, when presented with a child exhibiting these signs, serum TSH and FT4 should be assayed in order to confirm or rule out the disease, irrespective of the result of screening. Age at start of treatment remains high, but strategies are being implemented to reduce it.

Congenital epidermoid cysts of the anterior fontanel

Two cases of a congenital epidermoid cyst located over the anterior fontanel are reported in Tunisian white boys, aged 10 and 12 months. In both cases, cyst had manifested at birth as a small soft mass over the head which gradually enlarged. Radiological examinations revealed no intracranial extension. The cysts were completely excised without complications with good outcome. Review of the literature demonstrates that epidermoid cysts at this location have many common features and would appear to form a district entity

Relationship between the depth of cingulate sulcus on neonatal high resolution cranial ultrasound and gestational age / 소아과

PURPOSE: It is critical that the exact gestational age of a newborn baby, especially premature baby, be determined to evaluate the status of a disease and its management and to estimate the prognosis of a patient. This study aimed to investigate an easy and accurate method to estimate gestational age on cranial ultrasound, requiring minimal additional time and equipment. METHODS: A high-resolution coronal sonographic image was obtained via the anterior fontanel with a 5-12 Mhz linear probe after the usual cranial sonographic examination. We measured the depth of cerebral hemisphere, thickness of corpus callosum, and depth of cingulate sulcus and obtained the correlations between these factors and gestational age. RESULTS: Depth of cingulate sulcus had the highest correlation coefficiency with gestational age (r=0.878, P=0.000). All the cases, except for 2 cases, had a gestational age of more than 37 weeks, when the depths of cingulate sulcus were more than 0.55 cm, and had a gestational age less than 34 weeks, when the depths of cingulate sulcus were less than 0.35 cm. CONCLUSION: Measurement of the depth of cingulate sulcus was a simple and accurate method to estimate the gestational age on cranial ultrasound. The gestational age is more than 37 weeks, when the depth of cingulate sulcus is more than 0.55 cm, and is less than 34 weeks, when the depth of cingulate sulcus is less than 0.35 cm.

Correction of Frontal Bone Defect in Cleidocranial Dysostosis with Porous Polyethylene(Medpor(R)): A Case Report

PURPOSE: Cleidocranial dysostosis is a rare hereditary disorder affecting bones that develop by intramembranous formation. The typical features include excessive growth of transverse diameter of the skull, hypoplastic clavicles, low height and characteristic facial features. METHODS: A 28-year-old female patient visited by frontal area depression. The diagnosis was performed by computed tomographic study and radiographic imaging. The patient had widely opened anterior fontanelle, partial fused metopic suture, multiple wormian bone and supernumenary impacted teeth. Under the coronal incision, we exposed depressed frontal area and corrected with Medpor block carving. RESULTS: Postoperatively frontoparietal skull was aestheticlly improved and satisfied the patient. CONCLUSION: Authors report a case of cleiodocranial dysostosis who underwent correction of abnormal skull shape by Medpor(R) insertion.

O uso da fontanela posterior no diagnóstico ultra-sonográfico das hemorragias periintraventriculares / Use of posterior fontanelle in the ultrasound diagnosis of intraventricular/periventricular hemorrhage

OBJETIVO: Avaliar o uso da fontanela posterior em recém-nascidos prematuros (< 37 semanas) e com peso de nascimento < 1.500 g no diagnóstico ultra-sonográfico da hemorragia periintraventricular e verificar se a utilização da fontanela anterior associada à posterior modifica a concordância entre examinadores. MÉTODOS: Estudo prospectivo, onde foram avaliados 85 recém-nascidos. O ultra-som foi realizado através da fontanela anterior e, a seguir, pela posterior. Para a análise da concordância entre a fontanela anterior isolada e em associação à posterior, foi considerado o diagnóstico consensual entre dois examinadores. Na falta de consenso, um terceiro examinador decidiu. RESULTADOS: A concordância entre os dois primeiros examinadores apresentou Kappa de 0,80 (IC95 por cento 0,76-0,84). Entretanto, na avaliação apenas da fontanela anterior, o Kappa foi de 0,74 (IC95 por cento 0,70-0,78). Trinta e sete hemisférios apresentaram hemorragia grau II; desses, o diagnóstico foi realizado pela fontanela anterior em 17 hemisférios (45,9 por cento); em 10 (27 por cento), o diagnóstico foi suspeitado pela fontanela anterior e confirmado pela posterior; e em 10 (27 por cento), o diagnóstico foi realizado exclusivamente pela posterior. Das 454 avaliações o uso da fontanela posterior permitiu que o diagnóstico de hemorragia periintraventricular fosse realizado em 20 (4,4 por cento) (< p 0,001). CONCLUSÕES: Neste estudo, a fontanela anterior associada à posterior foi superior à anterior isolada na detecção da hemorragia periintraventricular. A realização do ultra-som através da fontanela posterior permitiu o diagnóstico de hemorragia grau II não suspeitadas pela fontanela anterior. O uso da fontanela posterior também foi útil para o esclarecimento da presença de hemorragias nos exames inconclusivos pela anterior.

OBJECTIVE: To evaluate the use of the posterior fontanelle in premature neonates (< 37 weeks) with a birth weight < 1,500 g in the ultrasound diagnosis of intraventricular/periventricular hemorrhage and to assess whether the use of the anterior fontanelle associated with the posterior fontanelle changes the interrater agreement. METHODS: Eighty-five premature neonates were evaluated in this prospective study. Ultrasound was performed using the anterior fontanelle, and later, the posterior fontanelle. A consensus diagnosis between two raters was used to analyze the agreement between the anterior fontanelle alone and in association with the posterior fontanelle. If there was no consensus, a third observer was involved to decide. RESULTS: Agreement between the first two raters had a kappa of 0.80 (95 percentCI 0.76-0.84). However, when the anterior fontanelle was evaluated alone, kappa was 0.74 (95 percentCI 0.70-0.78). Thirty-seven hemispheres had grade II hemorrhage, of which 17 (45.9 percent) had their diagnosis performed using the anterior fontanelle; in 10 (27 percent), diagnosis was suspected by the anterior fontanelle and confirmed by the posterior fontanelle; and in 10 (27 percent), diagnosis was exclusively performed using the posterior fontanelle. Of the 454 hemispheres, in 20 (4.4 percent) the diagnosis of intraventricular hemorrhage was performed exclusively by the posterior fontanelle (p < 0.001). CONCLUSIONS: In this study, the anterior fontanelle associated with the posterior fontanelle was better than the use of the anterior fontanelle alone in the identification of intraventricular/periventricular hemorrhage. Ultrasound using the posterior fontanelle allowed diagnosis of unsuspected grade II hemorrhage by the anterior fontanelle. Use of the posterior fontanelle was also useful to clarify presence of hemorrhage in inconclusive examinations by the anterior fontanelle.

Schinzel-Giedion Syndrome and Psychomotor Retardation: A case report

We reported a girl diagnosed Schinzel-Giedion syndrome with severe psychomotor retardation and malformation that was characterized by mid face retraction, scoliosis, skull anomaly, wide cranial fontanels, brain cortical atropy, atrial septal defect, and hydronephrosis. Urinary tract infection, respiratory tract infection, and seizure were common. The neurodevelopment therapy was not effective and developmental stage was not improved. Spasticity was the only findings which was improved.