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Copyright of Acta Veterinaria Brasilica is the property of Acta Veterinaria Brasilica and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Objective: To evaluate radiological (gestational and perinatal) and neonatal signs of patients with Patau syndrome and semilobar holoprosencephaly, as well as to report the association of both pathologies. Case description: This case report is about a female infant, born at term with trisomy of the chromosome 13 and semilobar holoprosencephaly, with thalamic fusion and a single cerebral ventricle, in addition to several other changes that worsened the patient’s prognosis. Comments: Chromosome 13 trisomy is a genetic alteration that leads to the symptoms that determines Patau syndrome. In this syndrome, cardiovascular, urogenital, central nervous system, facial structure and intellectual impairment are common, in addition to problems in limb formation, such as decreased humerus and femur length, polydactyly, hypotelorism and low ear implantation. It is estimated, however, that holoprosencephaly is present in only 24 to 45% of the patients with trisomy 13. [ABSTRACT FROM AUTHOR]

Introduction: Syntelencephaly is a forebrain malformation, which is a variant of holoprosencephaly. Few cases have been published in the Latin American literature. Case presentation: Female newborn, daughter of a diabetic mother. Diagnosis of holoprosencephaly is made at 21 weeks' gestation. At birth, cleft lip and palate, clubfoot, bilateral pyelocaliceal ectasia, and complex congenital heart disease were also detected. Because of the latter, the patient died in the first week of life. Magnetic resonance images compatible with syntelencephaly were identified. Conclusions: Patients with syntelencephaly and other congenital multiples have a poor prognosis. Its management should be multidisciplinary, including Genetics specialists. [ABSTRACT FROM AUTHOR]

Holoprosencephaly (HPE) is a congenital malformation of the central nervous system (CNS) that is produced by the lack of segmentation of the prosencephalon in the two cerebral hemispheres. This defect occurs between the days 18th and 28th of gestation and affects both the telencephalon and the structures of the face. In 1964, De Myer classified holoprosencephaly according to the degree of severity into lobar, semilobar, allobar and mid-hemispheric. The etiology of HPE is heterogeneous. Chromosomal abnormalities are found in up to 60% of those affected, mainly trisomy of chromosome 13. The prognosis is bad, in most cases. Prenatal diagnosis of HPE is feasible during the three trimesters of pregnancy. In the first trimester it is performed through the finding of the fusion of the frontal horns of the lateral ventricles and the absence of the sign of the butterfly in a transthalamic axial section of the fetal head. From the second trimester, the findings show a single ventricle, absence of midbrain line and thalamus fused to the midbrain. In addition, other facial defects in the midline can be recognized. The aim of this work is to present a case of allobar HPE and to describe its main clinical features. Likewise, it is intended to show the relevance of recognizing the normal fetal anatomy by ultrasonography in order to detect early developmental alterations. This is a case of a 16 year-old, primigest patient, with a 27.1 week gestation, who consulted for polyhydramnios and fetal alterations evident in the last obstetric ultrasound. A new ultrasound scan is carried out which reports a single fetus with the following alterations: holoprosencephaly, cyclopia, proboscis, arrinia and absence of gastric chamber, findings compatible with trisomy 13. Finally, the pregnancy is terminated by cesarean section, obtaining a deceased newborn. Prenatal and early diagnosis, based on the recognition of normal and abnormal anatomical structures in an ultrasonographic study, allow progress to be made in the diagnostic algorithm and often leads to confirmatory tests (of trisomy, for example) through a prenatal genetic study such as the karyotype. This also makes it possible to carry out individualized attention in a Perinatal Health, High Obstetric Risk or Fetal Medicine unit and eventually make appropriate decisions, according to the legal framework, together with the family. [ABSTRACT FROM AUTHOR]

ABSTRACTOBJECTIVE: The bispectral index (BIS) is a parameter derived by electroencephalography (EEG) which provides a direct measurement of the effects of sedatives and anesthetics on the brain and offers guidance on the adequacy of anesthesia. The literature lacks studies on BIS monitoring in pediatric patients with congenital brain disease undergoing general anesthesia.CLINICAL FEATURES: A 13-year-old child weighing 32 kg, suffering from lobar holoprosencephaly, underwent surgery in which the bispectral index (BIS) monitoring the depth of anesthesia showed an abnormal response. Detailed analysis of the trends of BIS values in the different observation times demonstrated sudden falls and repetitive values of BIS likely related to repetitive epileptiform electrical activity caused by sevoflurane.CONCLUSION: The BIS is a very useful monitoring tool for assessing the degree of depth of anesthesia and to analyze the electroencephalographic variations of anesthetics. Particular attention should be given to patients with congenital disorders of the central nervous system in which the BIS may give abnormal responses that do not reflect an accurate assessment of the depth of anesthesia.

OBJECTIVE: Holoprosencephaly (HPE) is heterogeneous in pathogenesis, integrating genetic susceptibility with the influence of environmental factors. Submicroscopic aberrations may contribute to the etiology of HPE. Our aim was to report the molecular analysis of 4 fetuses with HPE and normal metaphase karyotype. METHOD: A whole genome BAC-array based Comparative Genomic Hybridization (array CGH) was carried out in fetal blood samples. All potential cytogenetic alterations detected on the arrays were matched against the known copy number variations databases. RESULTS: The array CGH analysis showed copy number gains and losses in all cases. We found a recurrent deletion in 15q14 (clone RP11-23J11) and in 15q22 (clone RP11-537k8) in 2 out 4 cases analyzed. We also observed submicroscopic gain in 6p21 in 3 out of 4 fetuses in nearby clones. All these regions were tested in known databases and no copy number variations have been described for them. CONCLUSION: This is the first report of molecular characterization through a whole genome microarray CGH of fetuses with HPE. Our results may contribute to verify the effectiveness and applicability of the molecular technique of array CGH for prenatal diagnosis purposes, and contributing to the knowledge of the submicroscopic genomic instability characterization of HPE fetuses.

Objetivos: determinar y analizar la prevalencia de ciclopía en el Hospital Universitario del Valle (HUV) y buscar en los casos los factores etiológicos descritos en la literatura. Metodología: el presente es un estudio de corte transversal analítico, ensamblado a partir del sistema de vigilancia epidemiológica de malformaciones congénitas en el HUV y bajo la metodología del Estudio Colaborativo Latinoamericano de Malformaciones Congénitas (ECLAMC), se detectaron los casos de ciclopía acontecidos entre diciembre de 2004 y abril de 2008, se obtuvo el número de nacimientos y se encontró la prevalencia de ciclopía en el HUV. Gracias a un formato de entrevista del ECLAMC, se buscaron en las pacientes los factores que según la literatura se asocian con la ocurrencia de ciclopía. Se tomó fotos de cada uno de los casos. Resultados: durante el período comprendido entre diciembre de 2004 y abril de 2008 se presentaron 18.224 nacimientos en el HUV. De éstos, 8 presentaron ciclopía con una prevalencia de 1 en 2.278 nacimientos (43,8 por 100.000). Todos los casos se clasificaron según Meyer como tipo I y 3 casos presentaron etmocefalea. Se encontró exposición a misoprostol, alcohol y marihuana en un caso; diabetes en otro, marihuana en otro; un caso presentó trisomia 13 y otro en que no se obtuvo cariotipo los hallazgos sugirieron trisomía 13. Conclusiones: La ciclopía en Cali y el Valle del Cauca es considerada un evento endémico. Por lo cual, se necesita un sistema de vigilancia epidemiológico de malformaciones congénitas, holoprosencefalia y ciclopía para Cali y el Valle del Cauca. Además, se necesitan estudios controlados sobre factores de riesgo individuales y colectivos.Objectives: identifying and analysing cyclopia prevalence in the Hospital Universitario del Valle and searching for the aetiological factors described in the literature in the cases so found. Methodology: this was a prospective study of cases and controls regarding cases of cyclopia occurring between December 2004 and April 2008 which were detected via HUV's congenital malformation epidemiological surveillance system, using Latin-American Collaborative Study of Congenital Malformations of births and cyclopy prevalence in the HUV were obtained. ECLAMC's interview form was used for searching for factors associated by the literature with cyclopy occurring. Photos were obtained in all cases. Results: there were 18,224 births in the HUV from between December 2004 and April 2008; 8 of these presented cyclopy, giving a 1 in 2,278 births prevalence (43.8 per 100,000). All cases were classified as being Meyer type I. Three cases suffered from etmocephaly. Exposure to misoprostol, alcohol and marijuana was found in one case; diabetes in another; and marijuana in a third one. A case of trisomy 13 was presented and another case in which findings suggested trisomy 13, the karyotype was not obtained. Conclusions: cyclopy in Cali and Valle del Cauca are endemic events. A congenital malformation, holoprosencephaly and cyclopy epidemiological surveillance system is needed for Cali and Valle del Cauca area.

A holoprosencefalia (HPE) é a mais freqüente das malformações craniofaciais descritas na literatura. Diversos genes já foram identificados como causadores desse tipo de anomalia, entre eles, o Sonic Hedgehog (SHH), ZIC2, SIX3 e TGIF. O objetivo deste estudo foi avaliar as habilidades neuropsicolingüísticas de um indivíduo com HPE e mutação no gene SHH, apresentando características fenotípicas do tipo Like. Os resultados evidenciaram que, apesar de se tratar de um grau leve de HPE (fenótipo Like), o paciente apresentou perdas significativas nas habilidades lingüísticas, com aspecto cognitivo dentro da normalidade. O exame de ressonância magnética do encéfalo revelou hipoplasia da comissura anterior e presença de cisto temporal à esquerda, achados aparentemente não relacionados à sintomatologia clínica.The holoprosencephaly (HPE) is the most frequent of the craniofacial malformations described in literature. Several genes have already been identified as responsible for this kind of anomaly, among them, the Sonic Hedgehog (SHH), ZIC2, SIX3 and TGIF. The aim of this study was to evaluate the neuropsycholinguistic abilities of an individual with the HPE and mutation of the SHH gene, presenting phenotypic characteristics of the Like type. The results evidenced that, in spite of the lightness of the degree of HPE (Like phenopype), the patient showed significant loss of the linguistic abilities, although the cognitive aspects were adequate. The magnetic resonance of the encephalon revealed hypoplasia of the anterior commissure and presence of a temporal cyst on the left side, findings apparently not related to the clinic symptomatology.

La ciclopía es una malformación congénita rara e incompatible con la vida, caracterizada por la presencia de un solo ojo en posición central, secundaria a holoprosencefalia alobar, de etiología heterogénea, con una prevalencia de 1-3 en 100.000 nacimientos. Presentamos cuatro casos de ciclopía nacidos en el Hospital Universitario del Valle (Cali, Colombia) entre el 4 de diciembre del 2004 y el 23 de mayo del 2005. En un período de 170 días, en el cual hubo 3.594 nacimientos, lo que correspondería a una prevalencia de 1,11 casos por 1.000 nacimientos para ese período, que es 50 veces lo reportado en la literatura.Cyclopia is a rare congenital malformation which is non-compatible with life. It has heterogeneous aetiology and is characterised by the presence of a single eye in a central position, being secondary to alobar holoprosencephaly and having 1-3 prevalence per 100,000 births. 4 cases of cyclopia occurred in 3,594 births at the Valle teaching hospital in Cali, Colombia, from Dec 4th 2004 to May 23rd 2005. This represents an incidence of 1.11 cases per 1,000 births, being 50 times greater than that reported in the literature.

Holoprosencefalia es la no segmentación del prosencéfalo, se caracteriza por defectos en la línea media. Se presenta con hipertelorismo, nariz corta ensilladura, labio, paladar hendido, filtrum amplio, microcefalia e hidrocefalia. Crisis convulsivas y anomalías endocrinológicas. Paciente masculino, 3meses de edad, madre, G1P1A0C0 15 años. Control-prenatal, completo. Embarazo normal. A las 34 semanas se diagnostica defecto del sistema nervioso central y retardo del crecimiento-intrauterino. Parto institucional, recién nacido pretérmino, 36 semanas, peso, 1900g. Cianótico, no llanto, requirió oxígeno. Se hospitaliza por 12 días, ecografía y TAC cerebral, holoprosencefalia, sin hipertensión-endocraneana. Al mes, se hospitaliza por síndrome convulsivo, neumonía, síndrome coqueluchoide, se sigue por consulta externa, paciente con fiebre ínter-diaria y episodios convulsivos durante un mes. Se hospitaliza, al ingreso tres episodios convulsivos tónico¿clónico-generalizados, irritable, hipertelorismo ocular, nariz de puente bajo ancho, labio y paladar hendido, hernia umbilical, hernia inguinoescrotal y micropene. Hipertonia, retardo del desarrollo psicomotor. Punción lumbar, resultado, meningitis bacteriana se inicia manejo antibiótico. Posterior, Na, 155.3 K,5.95 Cl,121, problemas de cortisol y Panhipopituitarismo, inicia reemplazo hormonal. Neurología refiere agenesia cerebral reacciones con tallo cerebral y cerebelo, mal pronóstico, hace paro-cardiorrespiratorio maniobras de reanimación cardiopulmonar, continúa en mal estado general, periodos de apnea, baja saturación, expansibilidad torácica disminuida. Hace 3 paros cardiorrespiratorios, en el tercero no hay respuesta. Fallece. La Holoprosencefalia es un defecto del SNC ligado a mal pronóstico. En este caso, el paciente reaccionaba con tallo-cerebral y cerebelo, además panhipopituitarismo alteraciones hormonales y meningitis bacteriana que complicó más el cuadro.

Objetivos: enfatizar a importância do diagnóstico precoce da holoprosencefalia, a abordagem dos casos nascidos vivos e o aconselhamento genético oferecido aos pais. Determinar o delineamento epidemiológico da família de cada caso afetado. Detectar a incidência desta malformação durante o período de estudo. Métodos: estudo retrospectivo, através de revisão de prontuários protocolados, com o questionário do Estudo Colaborativo Latino-Americano de Malformações Congênitas (ECLAMC), em todos os hospitais de nível terciário da cidade de Pelotas (RS). Todos os casos foram submetidos à avaliação judiciosa de um médico geneticista. Foi realizada revisão de literatura médica. Resultados: em 13 anos de coleta de dados (janeiro de 1990 a dezembro de 2002) ocorreram seis casos de holoprosencefalia. A incidência foi de 0,8:10.000 nascimentos. Detectamos a ocorrência de diabete gestacional e ingestão de ácido acetilsalicílico em dois casos isolados, associação esta, que já foi documentada na literatura revisada. Conclusão: a holoprosencefalia é uma grave malformação congênita, podendo ser precocemente diagnosticada para o adequado manejo dos casos nascidos vivos, estudo citogenético familiar e planejamento de gestações futuras.

O termo holoprosencefalia (HPC) é usado para o complexo de deformidades hemisféricas causadas por falha no desenvolvimento da vesícula prosencefálica. Este estudo tem como objetivo relatar o espectro morfológico de 12 casos de HPC, classificá-los e compará-los com a literatura pertinente. Avaliadas 5837 necrópsias realizadas entre 1960 e 1995, 12 apresentavam HPC. Dados como sexo, idade, e presenças de outras malformações associadas foram avaliados e comparados com as demais malformações e com o total de necrópsias. A maioria dos casos era de masculinos (66,66%) e neomortos (75%). A forma de HPC mais frequentemente encontrada foi a lobar (58,3%) e a alteração facial mais frequente foi a ciclopia (25%). Houve tendência estatística de que a HPC possa, dentro das malformações do sistema nervo central (SNC), acometer mais o sexo masculino e de que a neomortalidade seja maior na HPC do que nas outras malformações do SNC.

Resumo Objetivo O índice bispectral (BIS) é um parâmetro derivado por eletroencefalografia (EEG) que fornece uma medida direta dos efeitos de sedativos e anestésicos no cérebro e orientação sobre a adequação da anestesia. A literatura carece de estudos sobre a monitoração do BIS em pacientes pediátricos com doença cerebral congênita submetidos à anestesia geral. Características clínicas Criança de 13 anos, com 32 kg, com holoprosencefalia lobar, foi submetida a cirurgia em que a monitoração da profundidade da anestesia com o uso do BIS mostrou uma resposta anormal. A análise detalhada das tendências dos valores do BIS nos diferentes tempos de observação mostrou quedas súbitas e valores repetitivos do BIS, provavelmente relacionados à atividade elétrica epileptiforme repetitiva causada por sevoflurano. Conclusão O BIS é uma ferramenta de monitoração muito útil para avaliar o grau de profundidade da anestesia e as variações eletroencefalográficas dos anestésicos. Atenção especial deve ser dedicada aos pacientes com doenças congênitas do sistema nervoso central nos quais o BIS pode apresentar respostas anormais que não refletem a avaliação precisa da profundidade da anestesia. Objective The bispectral index (BIS) is a parameter derived by electroencephalography (EEG) which provides a direct measurement of the effects of sedatives and anesthetics on the brain and offers guidance on the adequacy of anesthesia. The literature lacks studies on BIS monitoring in pediatric patients with congenital brain disease undergoing general anesthesia. Clinical features A 13‐year‐old child weighing 32 kg, suffering from lobar holoprosencephaly, underwent surgery in which the bispectral index (BIS) monitoring the depth of anesthesia showed an abnormal response. Detailed analysis of the trends of BIS values in the different observation times demonstrated sudden falls and repetitive values of BIS likely related to repetitive epileptiform electrical activity caused by sevoflurane. Conclusion The BIS is a very useful monitoring tool for assessing the degree of depth of anesthesia and to analyze the electroencephalographic variations of anesthetics. Particular attention should be given to patients with congenital disorders of the central nervous system in which the BIS may give abnormal responses that do not reflect an accurate assessment of the depth of anesthesia. [ABSTRACT FROM AUTHOR]

We studied clinical and EEG features of 36 cases with congenital malformations of the CNS. Patients were followed at the outpatient clinic of Hospital Cayetano Heredia and of Hogar Clinica San Juan de Dios in Lima-Peru, from January 1984 to June 1992. Eighty percent of the patients had convulsive syndromes and mental retardation. The most frequent malformation was agenesis of corpus callosum, and it was not possible to find a "typical" EEG pattern. The second were porencephalic cysts, with a good clinical-EEG correlation. There were two typical cases of schizencephaly, one of hemimegalencephaly with good prognosis, and one of holoprosencephaly. The results are compared to those obtained for a series we previously reported. Data discussed take into account reports on the subject registered in the literature. It is concluded that EEG is an useful method to evaluate possible CNS malformations in developing countries.

Para a realização deste estudo, que tem por objetivo classificar e analisar o aspecto mascroscópico do cérebro e facies na holoprosencefalia, foram revisados seis casos examinados no Departamento de Patologia da FFFCMPA. Comparação dos dados observados neste estudo é feita com aqueles da literatura sobre o assunto.

The use of misoprostol is contraindicated in pregnancy because of its potential as an abortifacient and its relationship with malformations such as microcephaly, Möbius syndrome and clubfoot, among other complications. We present two cases of patients with malformations evident at term birth with a history of misoprostol intake by the mother in the first trimester. The use of misoprostol in countries where abortion is illegal increases its misuse with the consequent appearance of newborns with various congenital malformations. It is important to disseminate and understand the teratogenic effects of this drug, as well as the control of its use. [ABSTRACT FROM AUTHOR]

Congenital anomalies of the central nervous system comprise a wide spectrum of malformations associated with a wide variety of genetic syndromes and chromosomal anomalies, and they are among the principal causes of morbidity and mortality in infants. Among these anomalies, holoprosencephaly arises from the complete or partial failure of the brain to divide into the cerebral hemispheres. Imaging tests are fundamental for the prenatal diagnosis of holoprosencephaly; the diagnostic process usually starts with sonography and then the findings are refined with fetal MRI. Radiologists need to be familiar with the possible findings because the prognosis varies., (Copyright © 2021 SERAM. Published by Elsevier España, S.L.U. All rights reserved.)

Cyclopia is a rare congenital malformation incompatible with life; it is characterized by the presence of a single eye in the center position, secondary to alobar holoprosencephaly. Cyclopia etiology is heterogeneous, with a prevalence of 1.05 in 100,000 births. We report a case of cyclopia with sinoftalmfa in a fetus of 21 weeks where they use 3D multislice computed tomography as a complementary study. [ABSTRACT FROM AUTHOR]

Copyright of Biomédica: Revista del Instituto Nacional de Salud is the property of Instituto Nacional de Salud of Colombia and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Arquivos de Neuro-Psiquiatria is the property of Thieme Medical Publishing Inc. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Revista Clínica de Ortodontia Dental Press is the property of Dental Press International and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Objectives: identifying and analysing cyclopia prevalence in the Hospital Universitario del Valle and searching for the aetiological factors described in the literature in the cases so found. Methodology: this was a prospective study of cases and controls regarding cases of cyclopia occurring between December 2004 and April 2008 which were detected via HUV's congenital malformation epidemiological surveillance system, using Latin-American Collaborative Study of Congenital Malformations (ECLAMC) methodology. The number of births and cyclopy prevalence in the HUV were obtained. ECLAMC's interview form was used for searching for factors associated by the literature with cyclopy occurring. Photos were obtained in all cases. Results: there were 18,224 births in the HUV from between December 2004 and April 2008; 8 of these presented cyclopy, giving a 1 in 2,278 births prevalence (43.8 per 100,000). All cases were classified as being Meyer type I. Three cases suffered from etmocephaly. Exposure to misoprostol, alcohol and marijuana was found in one case; diabetes in another; and marijuana in a third one. A case of trisomy 13 was presented and another case in which findings suggested trisomy 13, the karyotype was not obtained. Conclusions: cyclopy in Cali and Valle del Cauca are endemic events. A congenital malformation, holoprosencephaly and cyclopy epidemiological surveillance system is needed for Cali and Valle del Cauca area. [ABSTRACT FROM AUTHOR]

A cyclopia case with prenatal diagnosis by two dimensional and three dimensional ecography is presented, chordocentesis was realized, the chariotype in fetal blood with G banding presented trisomy 13. Phenotypic characteristics prenatally found where confirmed with the physical examination of the newborn. A revision to the literature about cyclops associated with trisomy 13 was made, and important aspects in prenatal diagnosis were highlighted. [ABSTRACT FROM AUTHOR]

Copyright of MedULA is the property of Facultad de Medicina, Universidad de los Andes, Venezuela and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

En Colombia las malformaciones congénitas constituyen la tercera causa de mortalidadinfantil, en el caso de la holoprosencefalia esta entidad tiene su mayor incidencia en personasde estrato socioeconómico bajo y su etiología principal es de tipo poligénico-ambiental (Cohen1989). En este trabajo realizaremos la descripción de un caso de holoprosencefalia mediante caracterización genética y por imagen.

Congenital anomalies of the central nervous system comprise a wide spectrum of malformations associated with a wide variety of genetic syndromes and chromosomal anomalies, and they are among the principal causes of morbidity and mortality in infants. Among these anomalies, holoprosencephaly arises from the complete or partial failure of the brain to divide into the cerebral hemispheres. Imaging tests are fundamental for the prenatal diagnosis of holoprosencephaly; the diagnostic process usually starts with sonography and then the findings are refined with fetal MRI. Radiologists need to be familiar with the possible findings because the prognosis varies., (Copyright © 2021 SERAM. Publicado por Elsevier España, S.L.U. All rights reserved.)

A case report of the agnathia holoprosencephaly complex and a review of the literature related to the complex genetic and embryologic aetiology of this group of major birth defects of face and central nervous system are informed. The present clinical case is the first reported in Colombia. [ABSTRACT FROM AUTHOR]

Objective: The bispectral index (BIS) is a parameter derived by electroencephalography (EEG) which provides a direct measurement of the effects of sedatives and anesthetics on the brain and offers guidance on the adequacy of anesthesia. The literature lacks studies on BIS monitoring in pediatric patients with congenital brain disease undergoing general anesthesia., Clinical Features: A 13-year-old child weighing 32kg, suffering from lobar holoprosencephaly, underwent surgery in which the bispectral index (BIS) monitoring the depth of anesthesia showed an abnormal response. Detailed analysis of the trends of BIS values in the different observation times demonstrated sudden falls and repetitive values of BIS likely related to repetitive epileptiform electrical activity caused by sevoflurane., Conclusion: The BIS is a very useful monitoring tool for assessing the degree of depth of anesthesia and to analyze the electroencephalographic variations of anesthetics. Particular attention should be given to patients with congenital disorders of the central nervous system in which the BIS may give abnormal responses that do not reflect an accurate assessment of the depth of anesthesia., (Copyright © 2014 Sociedade Brasileira de Anestesiologia. Published by Elsevier Editora Ltda. All rights reserved.)