Nomogram for predicting post-traumatic hydrocephalus after decompressive craniectomy for traumatic brain injury

SUMMARY OBJECTIVE: This study aimed to develop and validate a practical nomogram to predict the occurrence of post-traumatic hydrocephalus in patients who have undergone decompressive craniectomy for traumatic brain injury. METHODS: A total of 516 cases were enrolled and divided into the training (n=364) and validation (n=152) cohorts. Optimal predictors were selected through least absolute shrinkage and selection operator regression analysis of the training cohort then used to develop a nomogram. Receiver operating characteristic, calibration plot, and decision curve analysis, respectively, were used to evaluate the discrimination, fitting performance, and clinical utility of the resulting nomogram in the validation cohort. RESULTS: Preoperative subarachnoid hemorrhage Fisher grade, type of decompressive craniectomy, transcalvarial herniation volume, subdural hygroma, and functional outcome were all identified as predictors and included in the predicting model. The nomogram exhibited good discrimination in the validation cohort and had an area under the receiver operating characteristic curve of 0.80 (95%CI 0.72-0.88). The calibration plot demonstrated goodness-of-fit between the nomogram's prediction and actual observation in the validation cohort. Finally, decision curve analysis indicated significant clinical adaptability. CONCLUSION: The present study developed and validated a model to predict post-traumatic hydrocephalus. The nomogram that had good discrimination, calibration, and clinical practicality can be useful for screening patients at a high risk of post-traumatic hydrocephalus. The nomogram can also be used in clinical practice to develop better therapeutic strategies.

Simultaneous Cranioplasty and External Ventricular Drain Implantation in Patients with Hydrocephalus: Case Series and Literature Review

Introduction The increase in intracranial pressure (ICP) is a neurological complication resulting from numerous pathologies that affect the brain and its compartments. Therefore, decompressive craniectomy (DC) is an alternative adopted to reduce ICP in emergencies, especially in cases refractory to clinical therapies, in favor of patient survival. However, DC is associated with several complications, including hydrocephalus (HC). The present study presents the results of an unusual intervention to this complication: the implantation of an external ventricular drain (EVD) in the intraoperative period of cranioplasty (CP). Methods Patients of both genders who presented with HC and externalization of the brain through the cranial vault after decompressive hemicraniectomy and underwent EVD implantation, to allow the CP procedure, in the same surgical procedure, were included. Results Five patients underwent DC due to a refractory increase in ICP, due to automobile accidents, firearm projectiles, falls from stairs, and ischemic strokes. All evolved with HC. There was no uniform time interval between DC and CP. The cerebrospinal fluid (CSF) was drained according to the need for correction of cerebral herniation in each patient, before undergoing cranioplasty. All patients progressed well, without neurological deficits in the immediate postoperative period. Conclusion There are still several uncertainties about the management of HC resulting from DC. In this context, other CP strategies simultaneous to the drainage of CSF, not necessarily related to ventriculoperitoneal shunt (VPS), should be considered and evaluated more deeply, in view of the verification of efficacy in procedures of this scope, such as the EVD addressed in this study.

Infecciones del sistema nervioso central asociadas a dispositivos de derivación de LCR en niños, en un centro neuro-quirúrgico de referencia nacional / Central nervous system infections associated with CSF shunt devices in children, at a national reference hospital

INTRODUCCIÓN: Las infecciones asociadas a dispositivos de derivación de LCR son una complicación frecuente en su utilización. Lo más habitual es la presencia de cocáceas grampositivas, como Staphylococcus coagulasa negativa (50% en algunas series) y Staphylococcus aureus. Esta complicación agrega morbimortalidad al paciente neuroquirúrgico, aumentando la estadía hospitalaria y los costos de tratamiento. OBJETIVO: Conocer la incidencia de infecciones asociadas a dispositivos de derivación de LCR en un centro de referencia nacional. METODOLOGÍA: Estudio descriptivo, retrospectivo. Se recolectó la información de los pacientes pediátricos (bajo 18 años) entre 2018 y 2019. Se realizó un análisis estadístico descriptivo e inferencial utilizando el lenguaje estadístico R 3.4.0 y RStudio 1.3.9. Se calculó la incidencia acumulada para cada procedimiento, evaluando si existe diferencias significativas entre ellas. Estudio aprobado por el Comité de Ética Pediátrico del SSMO. RESULTADOS: En el período estudiado se realizaron 175 cirugías. Encontramos 19 casos de ventriculitis asociada a derivativa ventriculo-peritoneal y 7 casos en derivativa ventricular-externa. Los agentes más frecuentes fueron las cocáceas grampositivas. No se logró identificar factores de riesgo significativos.

BACKGROUND: Infections associated with CSF shunt devices are a frequent complication in their use. The most common is the presence of gram positive coccaceae, such as coagulase negative Staphylococcus (50% in some series) and Staphylococcus aureus. This complication adds morbidity and mortality to the neurosurgical patient, increasing hospital stay and treatment costs. AIM: To determine the incidence of infections associated with CSF shunt devices in a national referral center. METHODS: Retrospective, descriptive study. Information was collected on pediatric patients between 2018 and 2019. A descriptive and inferential statistical analysis was performed using the statistical language R 3.4.0 and RStudio 1.3.9. The cumulative incidence for each procedure was calculated, evaluating whether there were significant differences between them. This study was approved by the Pediatric Ethics Committee of the SSMO. RESULTS: In the period studied, 175 surgeries were performed. We found 19 cases of ventriculitis associated with ventriculoperitoneal derivative and 7 cases in ventricular-external derivative. The most frequent agents were grampositive coccaceae. It was not possible to identify significant risk factors.

Effect of surgical treatment on prognosis in preterm infants with obstructive hydrocephalus / 中国当代儿科杂志

OBJECTIVES@#To study the effect of surgical treatment on prognosis in preterm infants with obstructive hydrocephalus.@*METHODS@#A retrospective analysis was performed on the medical data of 49 preterm infants with obstructive hydrocephalus. According to the treatment regimen, they were divided into two groups: surgical treatment (@*RESULTS@#Among the 49 preterm infants with obstructive hydrocephalus, severe intracranial hemorrhage (37 cases; 76%) and central nervous system infection (10 cases, 20%) were the main causes of hydrocephalus. There was no significant difference in the composition of etiology between the two groups (@*CONCLUSIONS@#Surgical treatment can improve the survival rate of preterm infants with obstructive hydrocephalus and the prognosis of preterm infants with severe intracranial hemorrhage.

Avaliação do tempo de permanência da derivação ventriculoperitoneal em adolescentes com hidrocefalia congênita / Evaluation of the length of stay of ventriculoperitoneal shunt in adolescents with congenital hydrocephalus

O tratamento das hidrocefalias corresponde a mais da metade do movimento anual de um serviço de neurocirurgia pediátrica. Ainda hoje a cirurgia para implantação das derivações ventrículoperitoneais é o tratamento de escolha, uma vez que proporciona um imediato controle da pressão intracraniana e possui técnica amplamente difundida entre os serviços de neurocirurgia. A maioria dos casos tem como origem a hidrocefalia congênita, com incidência estimada de cerca de 326 casos por 100.000 nascidos vivos na América Latina. Um grande desafio para o neurocirurgião é a falha do sistema de derivação ventriculoperitoneal, cuja incidência após os primeiros 15 anos de cirurgia pode ser superior a 80%. Dentre as causas de falha da drenagem, podemos identificar as mecânicas (obstruções, hiperdrenagem, migrações, fraturas ou desconexões dos cateteres) e as infecciosas. Pouco se conhece sobre os fatores relacionados ao sucesso cirúrgico naqueles que atingiram a adolescência livres de complicações e com um ótimo funcionamento do sistema, uma vez que um número pequeno de pacientes chega ao fim da adolescência sem a necessidade de revisões cirúrgicas. O presente estudo tem como fonte de dados de pesquisa duas grandes unidades públicas de saúde especializadas em pediatria no município do Rio de Janeiro: o Hospital Municipal Jesus e o Instituto Nacional de Saúde da Mulher, da Criança e do Adolescente Fernandes Figueira. Trata-se de um estudo de coorte retrospectivo dos adolescentes que foram submetidos à derivação ventriculoperitoneal para o tratamento de hidrocefalia congênita, sendo registradas as variáveis clínicas observadas no momento em que foram operados, os aspectos radiológicos e os relacionados à técnica cirúrgica. Os adolescentes que receberam apenas a primeira intervenção cirúrgica para colocação da derivação ventrículo peritoneal foram comparados com aqueles submetidos a pelo menos uma reintervenção para avaliar a associação entre fatores clínicos, radiológicos e da técnica cirúrgica com o tempo de permanência das derivações. O peso no momento da cirurgia e o tempo cirúrgico mostraram associação significativa com a longevidade, relacionados ao sucesso cirúrgico da derivação ventriculoperitoneal em adolescentes com diagnóstico de hidrocefalia congênita.

More than half of the annual movement of a pediatric neurosurgery service comprehend the treatment of hydrocephalus. Even today, the treatment of choice for hydrocephalus is the surgical implantation of ventriculoperitoneal shunt since it provides immediate control of intracranial pressure, and its technique is widely used among neurosurgery services. Most cases originate from congenital hydrocephalus, which has an estimated incidence of about 326 cases per 100,000 live births in Latin America. The major challenge for the neurosurgeon is the failure of the ventriculoperitoneal shunt system, which accounts for more than 80% incidence after the first 15 years of surgery. Among the causes of drainage failure, we can distinguish the mechanical ones (obstructions, hyperdrainage, migrations, fractures, or disconnections of the catheters) and the infectious ones. Little is known about the factors related to surgical success in those who reached adolescence free of complications and with an optimal functioning of the system, since a small number of patients reach the end of adolescence without the need for surgical revisions. The source of the research data for this study are two large public health pediatric hospitals in the city of Rio de Janeiro: The Hospital Municipal Jesus and the Instituto Nacional da Saúde da Mulher, da Criança e do Adolescente Fernandes Figueira. This is a retrospective cohort study of adolescents who underwent ventriculoperitoneal shunt for the treatment of congenital hydrocephalus, in which the clinical variables observed at the time of the first surgery, the radiological aspects and those related to the surgical technique used will be registered. Adolescents who received only the first surgical intervention for placement of the peritoneal ventricle shunt were compared with those who underwent at least one reintervention, to assess the association between clinical, radiological, and surgical technique factors with the length of stay of the shunts. The weight at the time of the surgery and the length of the procedure showed a significant association with longevity, related to the surgical success the peritoneal ventricle shunt in adolescents diagnosed as congenital hydrocephalus.

Ventricular-Peritoneal Shunt in Newborns in a High-Risk Maternity Unit: Case Series

Objective To identify factors related to ventricular-peritoneal shunt (VPS) complications in pediatric patients at a high-risk maternity hospital. Methods Prospective study, conducted between September/2018 and June/2019, with selected newborns without previous ventricular bypass who underwent neurosurgery for VPS placement in a high-risk maternity hospital in the state of Sergipe, Brazil. Diagnosis of hydrocephalus occurred by transfontanelle ultrasound. The variables were analyzed by Student t-test, adopting p < 0.05 as statistical significance. Results Seven newborns participated in the study, 3 male and 4 female. Folic acid supplementation during pregnancy was considered a positive influencing factor in the 1st minute Apgar.Hydrocephalus secondary to premature hemorrhagewas present inmost newborns. Prematurity, 1st minute Apgar score < 7, and birth weight< 2,500 g did not represent a significant negative risk factor for prolonged hospitalization after neurosurgery. One newborn had cerebrospinal fluid infection and was the only one with heart disease. Conclusion This is the first scientific research that associates the benefits of maternal use of folic acid during pregnancy to better newborn Apgar scores. Only one newborn developed complications after neurosurgery, the only one with an associated comorbidity. Further studies are needed to provide more evidence on risk factors related to complications of VPS implantation in newborns. This neurosurgical procedure in a highrisk maternity contributed to the early management of hydrocephalus.

Clinical performance of fixed-pressure Sphera Duo® hydrocephalus shunt / Performance clínica da válvula de pressão fixa Sphera Duo®

Abstract Introduction: Cerebral hydrodynamics complications in shunted patients are due to the malfunction of the system. The objective of this retrospective, single-center, single-arm cohort study is to confirm the safety and performance of Sphera® Duo when used in adult patients suffering from hydrocephalus, pseudotumor cerebri or arachnoid cysts. Methods: Data were generated by reviewing 112 adult patient's charts, who were submitted to a ventriculoperitoneal shunt surgery and followed for one year after surgery. Results: The results show us that 76% of patients had their neurological symptoms improved and that the reoperation rate was 15% in the first year following surgery. Discussion: Sphera Duo® shunt system is an applicable shunt option in routine neurosurgical management of hydrocephalus by several causes. It has presented good results while mitigating effects of overdrainage. Overdrainage is especially important in adults with non-hypertensive hydrocephalus and can cause functional shunt failure, which causes subnormal ICP (particularly in the upright position) and is associated with characteristic neurological symptoms, such as postural headache and nausea. Conclusion: Sphera Duo® shunt system is safe when used in adult patients suffering from hydrocephalus, pseudotumor cerebri or arachnoid cyst.

Resumo Introdução: As complicações da hidrodinâmica cerebral em pacientes com derivação ventriculoperitoneal são frequentemente relacionadas ao malfuncionamento do sistema. O objetivo deste estudo retrospectivo de coorte de centro único é avaliar a segurança e performance clínica do Sistema Sphera® Duo quando utilizado em adultos com hidrocefalia, pseudotumor cerebral ou cistos aracnoides. Métodos: Avaliamos os prontuários de 112 pacientes adultos submetidos a cirurgia de derivação ventriculoperitoneal e acompanhados por 1 ano após a cirurgia. Resultados: O resultado mostra que 76% dos pacientes melhoraram dos sintomas neurológicos e a taxa de reoperação foi de 15% no primeiro ano após a cirurgia. Discussão: O sistema de derivação Sphera Duo® é uma opção de shunt adequada a ser usada no tratamento neurocirúrgico da hidrocefalia por causas diversas. Ele demonstrou bons resultados clínicos enquanto reduziu riscos de hiperdrenagem. A hiperdrenagem é especialmente preocupante e mórbida em pacientes adultos com hidrocefalia não hipertensiva e pode levar a prejuízo clínico e disfunção da válvula, com sintomas de hipotensão craniana, como cefaléia ortostática e náuseas. Conclusão: O sistema de derivação Sphera Duo® é seguro para tratamento da hidrocefalia, pseudotumor cerebri ou cistos aracnóides em adultos.

Surgical Treatment of Cavernous Angiomas in the Pineal Gland ­ Case Report

Pineal cavernous angioma is a vascular malformation that has a prevalence lower than 1%. The etiology is debated. It is believed that it is originated from an autosomal dominant inheritance or from radiotherapeutic treatment. Complete resection enables the cure and prevents complications of the natural evolution of the lesion, mainly hemorrhagic events. A female patient, with 67 years of age, presented mental confusion and visual acuity deficit, which evolved to periods of psychomotor agitation. A magnetic resonance imaging (MRI) scan of the head showed a lobulated mass lesion in the pineal region with hypersignal on T1 and hyposignal on the susceptibility weighted imaging (SWI) sequence. Hydrocephalus was also noticed. The patient underwent a microsurgery for complete lesion resection though a supracerebellar/ supratentorial access. The anatomopathology revealed an arteriovenous malformation compatible with cavernous angioma. The pineal cavernous angioma is a rare malformation that should be considered in the differential diagnosis of expansive lesions of the pineal gland.

Cerebrospinal fluid drainage options for posthemorrhagic hydrocephalus in premature neonates / Opções de drenagem liquórica em neonatos prematuros com hidrocefalia pós hemorrágica

ABSTRACT Objective The literature describes various cerebrospinal fluid (CSF) drainage techniques to alleviate posthemorrhagic hydrocephalus in preterm newborns; however, consensus has not been reached. The scope of this study was describing a case series of premature neonates with posthemorrhagic hydrocephalus and assessing the outcomes of different approaches used for CSF diversion. Methods A consecutive review of the medical records of neonates with posthemorrhagic hydrocephalus treated with CSF drainage was conducted. Results Forty premature neonates were included. Serial lumbar puncture, ventriculosubgaleal shunt, and ventriculoperitoneal shunt were the treatments of choice in 25%, 37.5% and 37.5% of the cases, respectively. Conclusion Cerebrospinal fluid diversion should be tailored to each case with preference given to temporary CSF drainage in neonates with lower age and lower birth-weight, while the permanent ventriculoperitoneal shunt should be considered in healthier, higher birth-weight neonates born closer to term.

RESUMO Objetivo A literatura descreve várias opções de drenagem liquórica (DL) para alivio da hidrocefalia pós-hemorrágica (HPH) em neonatos prematuros; contudo, não existe um consenso sobre a melhor abordagem. O escopo deste estudo foi descrever uma série de casos de neonatos prematuros, portadores de HPH, verificando os resultados de diferentes técnicas utilizadas para DL. Métodos Revisão consecutiva dos prontuários de neonatos com diagnostico de HPH submetidos a DL. Resultados Quarenta recém-nascidos prematuros foram incluídos. A punção lombar seriada (PL), a derivação ventriculosubgaleal (VSG) e a derivação ventrículo peritoneal (VP) foram o tratamento escolhido em 25%, 37,5% e 37,5% dos casos, respectivamente. Conclusão As opções de DL devem ser avaliadas caso a caso, sendo dada preferência às drenagens temporária em prematuros com idade e peso mais baixos ao nascer, enquanto o shunt definitivo (derivação VP) pode ser considerado naqueles prematuros mais saudáveis, com idade e peso superiores.

Revisiting Retrograde Ventriculosinus Shunt as an Alternative for Treating Hydrocephalus in Children

Introduction Retrograde ventriculosinus shunt (RVSS) is a useful option in the daily routine of neurosurgeons dealing with hydrodynamics. The objective of this manuscript is to review the main data about RVSS. Methods We performed a critical review. The keywords used were hydrocephalus, shunt, venous sinus, ventriculosinus shunt, retrograde ventriculosinus shunt, and sagittal sinus. The search was performed in the Medline (Pubmed) and EMBASE databases. Results Van Canneyt et al (2008) and Pinto et al (2016) performed experimental studies confirming the effectiveness of RVSS. El Shafei et al (1985, 1987, 2001) authors reported several cases treated with efficacy and few complications. Oliveira et al (2015, 2016) compared RVSS and ventriculoperitoneal shunt (VPS) in the treatment of hydrocephalus after myelomeningocele repair in infants, with similar functional results. Oliveira et al (2015, 2016) also described the applicability of RVSS in situations when VPS is not feasible, or when the peritoneum is not useful. Discussion Retrograde ventriculosinus shunt is a safe and more physiological option, which requires the use of less prosthetic material. It is feasible and applicable. Especially in children, it generates a normotensive state after shunting, allowing centrifugal head growth, once there is no intracranial hypotension due to overdrainage, which may reflect in long-term better psychomotor development. Conclusions The surgical technique of RVSS is feasible. The clinical results are comparable with those of the VPS.

Introdução A derivação ventriculosinusal retrógrada (DVSR) deve ser uma opção na rotina de neurocirurgiões que trabalham com hidrodinâmica. O objetivo deste estudo é revisar os principais dados sobre DVSR. Métodos Realizamos uma revisão crítica. As palavras-chave usadas foram hydrocephalus shunt, venous sinus, ventriculosinus shunt, retrograde ventriculosinus shunt, e sagittal sinus. A busca foi feita nas bases de dados Medline (Pubmed) e EMBASE. Resultados Van Canneyt et al (2008) e Pinto et al (2016) realizaram estudos experimentais confirmando a eficiência do DVSR. El Shafei et al (1985, 1987, 2001) relataram vários casos tratados com eficiência e poucas complicações. Oliveira et al (2015, 2016) compararam DVSR e derivação ventriculoperitoneal (DVP) no tratamento de hidrocefalia após reparo de mielomeningocele em crianças, obtendo resultados funcionais similares. Oliveira et al (2015, 2016) também descreveram a aplicabilidade do DVSR em situações em que o DVP não é possível, ou o peritônio é hostil. Discussão A DVSR é uma opção segura e mais fisiológica, que requer menos material protético; é factível e aplicável. Particularmente em crianças, gera um estado normotenso após o shunt, permitindo crescimento cefálico centrífugo, uma vez que não há mais hipotensão intracraniana devido à hiperdrenagem, o que pode refletir em melhor desenvolvimento psicomotor em longo prazo. Conclusões A técnica cirúrgica DVSR é viável. Os resultados clínicos se são comparáveis à DVP.

Choroid plexus coagulation for pediatric hydrocephalus: review of historical aspects and rebirth

This study aims to review historical aspects and rebirth of the endoscopic choroid plexus coagulation (CPC) for pediatric hydrocephalus. The first CPC procedure goes back to early 1930s. After the development of other treatment methods and the understanding of CSF dynamics, the application of CPC dramatically decreased by 1970s. In 2000s, there was a rebirth of CPC in combination with endoscopic third ventriculostomy (ETV), and remains one of the options for the treatment of pediatric hydrocephalus in selected cases. CPC might provide a temporary reduction in CSF production to allow the further development of CSF absorption in infant, and adding to ETV for infants with communicating hydrocephalus may increase the shunt independent rate thus avoiding the consequence of late complication related to the shunt device. This is important for patients who are difficult to be followed up, due to geographical and/or socioeconomic difficulties. And also adding CPC to ETV for obstructive hydrocephalus in infants younger than 1 year of age may also increase the successful rate. Furthermore, CPC may be an option for cases with high chance of shunt complication such as multiloculated hydrocephalus, extreme hydrocephalus and hydranencephaly. In comparison with the traditional treatment of CSF shunting, the role of CPC needs to be further evaluated in particular concerning the neurocognitive development.

Surgical technique of retrograde ventricle-sinus shunt is an option for the treatment of hydrocephalus in infants after surgical repair of myelomeningocele / A derivação ventrículo-sinusal retrógrada é uma opção para o tratamento de hidrocefalia em lactentes após correção de mielomeningocele

ABSTRACT Introduction Treatment of hydrocephalus is accomplished primarily through a ventricular-peritoneal shunt (VPS). This study aims to describe the application of retrograde ventricle-sinus shunt (RVSS) in patients with hydrocephalus after surgical treatment of myelomeningocele. Method A prospective, randomized and controlled pilot study. We consecutively enrolled 9 patients with hydrocephalus after surgical repair of myelomeningocele from January 2010 to January 2012. These patients underwent elective RVSS or VPS. Five underwent RVSS and 4 underwent VPS. Patients were followed for one year with quarterly evaluations and application of transcranial Doppler. Results RVSS group showed outcomes similar to those of VPS group. Doppler revealed significant improvement when comparing preoperative to postoperative period. RVSS group had significantly higher cephalic perimeter than VPS group. Neuropsychomotor development, complications and subjective outcomes did not differ between groups. Conclusion RVSS shunt is viable; it is an alternative option for the treatment of hydrocephalus.

RESUMO O tratamento da hidrocefalia é realizado principalmente através de uma derivação ventrículo-peritoneal (DVP). Nosso objetivo é descrever a aplicação da derivação ventrículo-sinusal retrógrada (DVSR) em pacientes com hidrocefalia após o tratamento cirúrgico de mielomeningocele. Método Estudo prospectivo, randomizado e controlado. Selecionados consecutivamente 9 pacientes com hidrocefalia após correção cirúrgica de mielomeningocele de janeiro de 2010 a janeiro de 2012. Eles foram submetidos à DVSR ou DVP. Cinco foram submetidos à DVSR e 4 à DVP. Foram seguidos por 1 ano com realização trimestral de avaliações e aplicação do Doppler transcraniano. Resultados O grupo DVSR apresentou desfechos semelhantes ao grupo DVP. O Doppler mostrou melhora significativa quando comparado o pré-operatório com o pós-operatório. O grupo DVSR apresentou perímetro cefálico significativamente maior que o grupo DVP. O desenvolvimento neuropsicomotor e complicações não diferiram entre os grupos. Conclusão A derivação ventrículo-sinusal retrógrada é viável; ela é uma opção para o tratamento de hidrocefalia.

Cráneo en trébol y fisura facial bilateral / Cloverleaf skull and bilateral facial clefts

Introducción: El síndrome del cráneo en trébol, o síndrome de Kleeblattschädel, es una malformación rara en la cual el cráneo presenta un aspecto de trébol. Es causado por el cierre prematuro de varias suturas, evidenciándose desde antes del nacimiento. Objetivo: presentar nuestra experiencia en un caso de síndrome del cráneo en trébol, y actualizar la información de la literatura. Caso clínico: lactante de sexo femenino, 5 meses de edad, diagnósticos al nacimiento de Fisura labio-Palatina e Hidrocefalia. A los 30 días de vida se instaló válvula ventrículo peritoneal, y se procedió a enucleación ocular bilateral por proceso infeccioso. Se controla en Genética donde se confirma macrocefalia y craneosinostosis tipo cráneo en trébol. El estudio citogenético 46XX, Ecocardiografía normal, TAC de cerebro mostró anomalias múltiples asociadas a hidrocefalia y malformaciones inespecíficas. Conclusión: El cráneo en trébol puede presentarse aisladamente o asociado a otras anomalías congénitas, conformando varios síndromes de craneosinostosis, como Crouzon, Pfeiffer o Carpenter. También puede ser componente de la secuencia de rotura amniótica o de diversas displasias, como la campomélica, tanatofórica tipo ii, o la distrofia torácica asfixiante de Jeune. El caso descrito no cumple con todas las características necesarias para incluirlo dentro de un síndrome específico, y no habiendo antecedentes familiares que sugieran patrón de herencia ni anomalías cromosómicas se concluye que se trata de un caso de anomalías congénitas de presentación esporádica.

Introduction: Cloverleaf skull syndrome, or Kleeblattschädel syndrome, is a rare malformation in which the skull has a cloverleaf appearance. It is caused by the premature closure of several sutures, being evident before birth. Objective: To present our experience in a case of cloverleaf skull syndrome, and update the information from the literature. Clinical case: A female infant of 5 months of age, diagnosed at birth with cleft lip and palate and hydrocephaly. A peritoneal ventricle valve was implanted at 30 days of life, and an ocular enucleation was performed due to an infectious process. The patient was followed-up in Genetics, where it confirmed a macrocephaly and craniosynostosis type cloverleaf skull. The 46XX cytogenetic study and echocardiography were normal. The brain CT scan showed multiple anomalies associated with hydrocephaly and non-specific malformations. Conclusion: Cloverleaf skull may be present in isolated form or associated with other congenital abnormalities, leading to various craniosynostosis syndromes, such as Crouzon, Pfeiffer or Carpenter. It may also be a component of the amniotic rupture sequence or to different dysplasias, such as campomelic dysplasia, thanatophoric dysplasia type 2, or the asphyxiating thoracic dystrophy of Jeune. The case presented does not fulfil all the characteristics needed to be included within a specific syndrome, and on not having a family history that suggests a hereditary pattern or chromosome abnormalities, it is concluded that it is a case of a congenital anomaly of sporadic presentation.

Clinical and ultrasonographic criteria for using ventriculoperitoneal shunts in newborns with myelomeningocele / Critérios clínicos e ultrassonográficos para a indicação de derivação ventrículo peritoneal em neonatos portadores de mielomeningocele

Objective Hydrocephalus is one of the main complications associated with myelomeningocele (MM). This study aimed to identify clinical and ultrasonographic criteria for using ventriculoperitoneal (VP) shunts in this group of patients.Method A retrospective cohort study, based on established protocol for VP shunt implant in hydrocephalic children with MM. Parameters used to guide the indication of VP shunts included measurement of head circumference (HC), evaluation of fontanels, and measurement of lateral ventricular atrium (LVA) width by transcranial ultrasonography.Results 43 children were included in the analysis, of which 74% had hydrocephalus and required a VP shunt. These children had LVA width ≥ 15 mm, showed increased HC, or had bulging fontanels.Conclusion VP shunt is required in children with increased HC (≥ 2 standard deviation regarding age group), bulging fontanels, or LVA width of ≥ 15 mm after the closure of MM.

Objetivo Identificar os critérios clínicos e ultrassonográficos para a recomendação do implante de derivações ventrículo peritoneais (DVP) em neonatos portadores de mielomeningocele (MM).Método Estudo de coorte retrospectivo, com base no protocolo estabelecido para o implante de DVP em crianças com hidrocefalia associada a MM. Parâmetros utilizados para orientar a indicação de DVP incluíram a medida da circunferência craniana (CC), a avaliação das fontanelas e a medida da largura lateral do átrio ventricular (LAV), avaliado por ultrassonografia transcraniana.Resultados 43 crianças foram incluídas na análise, dos quais 74% tinham hidrocefalia com recomendações para uso de DVP.Conclusão O aumento da CC e o abaulamento de fontanelas foram os principais critérios para a indicação de DVP. A DVP é necessária em crianças com aumento da CC (≥ 2 desvios padrões para a idade), fontanelas abauladas, ou LAV ≥ 15 mm após o fechamento cirúrgico da MM.

Histoplasmosis del sistema nervioso central en un paciente inmunocompetente / Histoplasmosis of the central nervous system in an immunocompetent patient

La histoplasmosis es una afección polifacética producida por el hongo dimorfo Histoplasma capsulatum , cuyas esporas son inhaladas y llegan al pulmón, órgano primario de infección. La forma meníngea, considerada como una de las manifestaciones más graves de esta micosis, suele presentarse en individuos con alteraciones en la inmunidad celular: pacientes con síndrome de inmunodeficiencia humana adquirida, con lupus eritematoso sistémico o con trasplante de órgano sólido, así como en lactantes, debido a su inmadurez inmunológica. La forma de presentación más usual es de resolución espontánea y se observa en individuos inmunocompetentes que se han expuesto a altas concentraciones de conidias y fragmentos miceliares del hongo. En estas personas, la afección se manifiesta por trastornos pulmonares y por la posterior diseminación a otros órganos y sistemas. Se presenta un caso de histoplasmosis del sistema nervioso central en un niño inmunocompetente.

Histoplasmosis is a multifaceted condition caused by the dimorphic fungi Histoplasma capsulatum whose infective spores are inhaled and reach the lungs, the primary organ of infection. The meningeal form, considered one of the most serious manifestations of this mycosis, is usually seen in individuals with impaired cellular immunity such as patients with acquired immunodeficiency syndrome, systemic lupus erythematous or solid organ transplantation, and infants given their immunological immaturity. The most common presentation is self-limited and occurs in immunocompetent individuals who have been exposed to high concentrations of conidia and mycelia fragments of the fungi. In those people, the condition is manifested by pulmonary disorders and late dissemination to other organs and systems. We report a case of central nervous system histoplasmosis in an immunocompetent child.

Coagulação endoscópica do plexo coroide no tratamento primário da hidranencefalia e da hidrocefalia extrema / Endoscopic coagulation of the choroid plexus in the primary treatment of extremehydrocephalus and hydranencephaly

Objetivos: Descrever a técnica de coagulação neuroendoscópica do plexo coroide e divulgá-la como opção de tratamento primário da hidranencefalia e da hidrocefalia extrema. Mostrar a casuística de coagulação endoscópica de plexo coroide do Serviço de Neurocirurgia do Hospital da Baleia em BeloHorizonte, MG, Brasil. Métodos: Imagens tomográficas foram avaliadas por um neurocirurgião, sendo selecionadas crianças portadoras de hidranencefalia e hidrocefalia extrema com cavidade ventricular única. Os pacientes foram acompanhados por até três anos no pós-operatório. Resultados: Foram tratadas seis crianças com hidranencefalia e seis com hidrocefalia extrema. Um paciente faleceu na primeira semana de pós-operatório em função de choque hipovolêmico e hipernatremia graves e outro cursou com aumento progressivo do perímetro cefálico e necessidade de derivação ventriculoperitoneal. Um terceiro paciente apresentou óbito tardio não relacionado à cirurgia endoscópica. Os demais apresentaram evolução adequada no pós-operatório. Conclusão: A coagulação endoscópica do plexo coroide mostrou-se alternativa eficaz no tratamento da hidranencefalia e da hidrocefalia extrema.

Objectives: Describing the neuroendoscopic technique for coagulation of the choroid plexus and publicize it as an option for the primary treatment of extreme hydrocephalus and hydranencephaly. Display the series of endoscopic coagulation of the choroid plexus of the Neurosurgery Department of the Hospital da Baleia in Belo Horizonte, MG, Brazil. Methods: Tomographic images were used to select children with hydranencephaly and extreme hydrocephalus with single ventricular cavity. Patients were followed for up to three years postoperatively. Results: Six children with hydranencephaly and six with extreme hydrocephalus were treated. One patient died in the first week postoperatively due to hypovolemic shock and severe hypernatremia and another developed progressive increase in head circumference and the need for ventriculoperitoneal shunt. A third patient had late death unrelated to endoscopic surgery. The others had adequate postoperative evolution. Conclusion: The endoscopiccoagulation of the choroid plexus is an effective alternative in the treatment of extreme hydrocephalus and hydranencephaly

Endoscopic third ventriculostomy has no higher costs than ventriculoperitoneal shunt / Terceiro ventriculostomia endoscópica não apresenta custos mais elevados do que a derivação ventrículo peritoneal

Objective: To evaluate the cost of endoscopic third ventriculostomy (ETV) compared to ventriculoperitoneal shunt (VPS) in the treatment of hydrocephalus in children. Method: We studied 103 children with hydrocephalus, 52 of which were treated with ETV and 51 with VPS in a prospective cohort. Treatment costs were compared within the first year after surgery, including subsequent surgery or hospitalization. Results: Twenty (38.4%) of the 52 children treated with VPS needed another procedure due to shunt failure, compared to 11 (21.5%) of 51 children in the ETV group. The average costs per patient in the group treated with ETV was USD$ 2,177,66±517.73 compared to USD$ 2,890.68±2,835.02 for the VPS group. Conclusions: In this series there was no significant difference in costs between the ETV and VPS groups. .

Objetivo: Avaliar os custos da terceiro ventriculostomia endoscópica (TVE) comparada à derivação ventrículo peritoneal (DVP) no tratamento da hidrocefalia em crianças. Método: Foram estudadas 103 crianças com hidrocefalia, 52 das quais tratadas com TVE e 51 com DVP numa coorte prospectiva. Foram comparados os custos do tratamento no primeiro ano após a cirurgia, incluindo cirurgias ou internações subsequentes. Resultados: Vinte (38,4%) das 52 crianças tratadas com DVP necessitaram de outro procedimento por disfunção da válvula, em comparação a 11 (21,5%) das 51 crianças do grupo tratado com TVE. Os custos médios por paciente no grupo tratado com TVE foram de USD$ 2,177.66±517.73 comparados a USD$ 2.890,68±2.835,02 para o grupo DVP. Conclusões: Nesta série não houve diferença significativa de custos entre o grupo TVE e DVP. .

Agenesia sacra asociada a disrrafismo espinal e hidrocefalia / Sacra agenesia associated to spinal dysraphism and hydrocephaly

Introducción: la agenesia sacra es una malformación congénita rara que forma parte del síndrome de regresión caudal. Se caracteriza por un grupo de anomalías en las cuales la columna caudal está ausente. Esta enfermedad es la malformación más frecuente en los hijos de madres diabéticas, además se ha relacionado con otros factores predisponentes, como deficiencias de ácido fólico, de vitaminas, uso de insulina en el embarazo, e incluso, la hipoxia. Entre un 30-40 por ciento de pacientes con agenesia sacra completa, pueden tener asociado un mielomeningocele, y el desplazamiento de las raíces nerviosas empeora los trastornos neurológicos. En estos casos, la hidrocefalia, muchas veces también asociada a malformación Chiari tipo II, está ya presente al nacer. Caso clínico: se presenta el caso de un neonato con agenesia sacra asociada a disrrafismo espinal e hidrocefalia. La intervención quirúrgica fue precoz, se le colocó derivación ventrículo peritoneal y se realizó la reparación del defecto del tubo neural. La evolución posoperatoria fue favorable, aunque persistieron los déficits neurológicos preoperatorios. Conclusiones: no se hallaron factores predisponentes en este paciente y el análisis del cariotipo fue normal. Las anomalías óseas de miembros inferiores fueron las más llamativas, así como la presencia de hidrocefalia asociada a malformación Chiari tipo II y mielomeningocele. El tratamiento a estos casos requiere de un enfoque multidisciplinar, y la reparación quirúrgica del mielomeningocele debe ser precoz para conseguir una evolución favorable. Las formas graves pueden ocasionar una muerte temprana neonatal, en cambio, los niños que sobreviven, generalmente presentan inteligencia normal

Introduction: sacra agenesia is a rare congenital malformation as part of the caudal regression syndrome. It is characterized by a group of anomalies in which the caudal cord is absent. This disease is the most common malformation found in children from diabetic mothers but it has also been related to other predisposing factors such as folic acid deficiencies, vitamin deficiencies, use of insulin at pregnancy and even hypoxia. Thirty to forty percent of patients with complete sacra agenesia can also have myelomeningocele, and the displacement of nerve roots worsens the neurological disorders. In these cases, hydrocephaly, many times associated to Chiari malformation type II, is also present at birth. Clinical case: a neonate with sacra agenesia associated to spinal dysraphism and hydrocephaly. Surgical intervention was performed early, a peritoneal ventricular derivation was placed and the neural tube defect was repaired. The post-surgery evolution was favorable, but the preoperative neurological deficits persisted. Conclusions: there were no predisposing factors in this patient and the analysis of the cariotype was normal. The bone anomalies of the lower members were the most remarkable aspects as well as the hydrocephaly associated to Chiari malformation type II and myelomeningocele. The treatment of these cases requires multidisciplinary approach and surgical repair of the myelomeningocele at early phase to achieve favorable evolution. The most severe forms can cause early neonatal death; however, those surviving children generally present normal intelligence coefficient