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Migration of the catheter tip of a ventriculoperitoneal shunt (VPS) through a patent processus vaginalis (PV) has seldom being reported. In this series, we analyzed all cases of inguinoscrotal migration of an VPS catheter at our center. Between 1975 and 2015, 295 VPS insertions were performed. In seven infants (2.3%), migration of the catheter through a patent PV was demonstrated. Cases with hydroceles had associated VPS malfunction, but cases with hernias were not associated with it. The current recommendation is to delay a PV closure until 2 years of age in cases of infants with hydrocele without further radiological examinations, but this might not be the case in infants with VPS.

A 4-year-old boy with kaposiform lymphangiomatosis (KLA) developed progressive headaches and papilloedema and was diagnosed with pseudotumor cerebri initially treated with acetazolamide. Clinical deterioration prompted placement of a ventriculoperitoneal shunt. After the surgery, the child’s condition has markedly improved. A network of intracranial lymphatics is presently being investigated. Neuroimaging excluded KLA infiltration of the skull and/or meninges, leaving as the most plausible explanation for the child’s pseudotumor cerebri the existence of an increase in intracranial venous pressure by venous compression at the thorax. To our knowledge, our case constitutes the first report of pseudotumor cerebri occurring in the context of KLA.

Arachnoid cysts (ACs) produce symptoms (1) directly by compression of the adjacent brain, (2) indirectly by blockage of cerebrospinal fluid (CSF) pathways, or (3) by bleeding. Absolute indications for treatment of ACS include brain compression, increased intracranial pressure, and hydrocephalus. But not always can the presence of an AC be regarded as responsible for the patients’ presenting symptoms. Much controversy exists about the need for surgical treatment of these cysts. In addition, many ACs merely represent incidental findings. Basically, the current options for the treatment of intracranial ACs include cyst fenestration (by endoscopic or open surgery) and cyst shunting. Cyst fenestration is directed towards relieving the cyst pressure by establishing a communication from the cyst to the cisterns or subarachnoid spaces. This option may also include the partial or total excision of the cyst’s walls. Cyst shunting consists of a draining system that vents the cyst fluid to an intra- or extracranial compartment by using some sort of CSF derivation. In spite of the reported diversity of surgical options, the most important issue regarding AC management still arises from the indications on which and when ACs should be treated. Hydrocephalus may as well occur in association with ACs constituting a sound indication for surgery. Hydrocephalus usually originates from obstruction of the CSF pathways and is considered as being of obstructive type. However, some authors propose a common origin for both hydrocephalus and AC formation attributing this association to impairment in CSF absorption, in which case the hydrocephalus is regarded as communicating. This distinction is not only of academic interest but it also has practical consequences at the time of choosing the most appropriate treatment. The occurrence of hydrocephalus is closely related with the location and size of the cysts. Broadly speaking, hydrocephalus mainly occurs in midline and posterior fossa cysts, while it is infrequent in sylvian and convexity lesions. The introduction of endoscopic techniques has lead to a dramatic change in classic treatments as open fenestration and cystoperitoneal shunting. At present, many instances with hydrocephalus and ACs can be managed exclusively with neuroendoscopic procedures in a simultaneous way although some instances will still require CSF shunting. The ideal treatment seems to consist of performing the modality that offers a high success rate using a single procedure that simultaneously addresses the treatment of both the hydrocephalus and the AC.

At present, numerous patients are found to harbor an intracranial arachnoid cyst (AC). In the recent past, many of these cysts were treated with some type of surgery, such as cystoperitoneal shunting or cyst fenestration, based only on the presence of the cyst in neuroimaging studies. Indications for neurosurgical treatment of ACs include direct brain compression, distortion of intracranial structures, hydrocephalus, and intracranial hypertension. The ample use of new neuroimaging methods has led to the straightforward diagnosis of many cysts that apparently are producing no harm to the brain, which are known as incidental ACs. On the other hand, we have witnessed during the last decades the appearance of a large number of publications dealing with the complications derived from AC treatments, mostly after cyst shunting. This led many authors to advise restricting surgical treatment to cases that present clear clinical and laboratory features of brain dysfunction to prevent hazards derived from the surgeries. Even considering the low risks of surgical treatments, some complications seem to be avoidable utilizing stricter indications. This more conservative approach has motivated some authors to suggest that certain complications would not have occurred if many ACs had never been operated on. A main concern refers to “preventive surgery” directed at avoiding the eventual damage caused by the cysts on the developing brain of children. Another conflicting issue arises on surgical indications in patients with epilepsy, neurocognitive, and behavioral symptoms. Accordingly, many investigators have focused their research on the development of dependable diagnostic tests that may help in the decision-making process. In this chapter, we will deal with the value of single-photon emission computerized tomography on early detection of the possible brain damage that these cysts may cause.

Cerebrospinal (CSF) fluid overdrainage in shunted hydrocephalus is a well-known condition. Syndromes pertaining to excessive CSF drainage following cystoperitoneal shunting seem to be less recognized. CSF overdrainage presents under several conditions ranging from orthostatic headaches to shunt dependency, so-called slit-ventricle syndrome, intracranial hypertension (pseudotumor), posterior fossa crowding, and hindbrain herniation (acquired Chiari malformation). Several pathogenetic hypotheses have been put forward to explain the diverse clinical manifestations of arachnoid cyst overshunting, which probably reflects the lack of agreement of researchers and clinicians about their nature. In the same way, a variety of treatments have been utilized for the management of overdrained cysts. Logically, findings derived from the knowledge of overdrained hydrocephalus have greatly contributed to understand the facts concerning CSF overdrainage in arachnoid cyst shunting. In this chapter, we describe pathogenetic mechanisms, clinical features, diagnostic clues, and modalities of management of arachnoid cyst overshunting, by reviewing the current literature and referring to our own experience.

The role of interspinous devices (ISD) after lumbar herniated disc surgery for the prevention of postoperative back pain is controversial. The aim of this comparative prospective study was to determine outcomes in a selective cohort with L5–S1 disc herniation and degenerative disc changes after microdiscectomy with or without insertion of an ISD. One hundred and two consecutive patients underwent an L5–S1 microdiscectomy with or without implantation of an ISD. Group 1 consisted of 47 patients, with mild (n = 22), moderate (n = 14) or severe (n = 11) degenerative disc changes who had microdiscectomy alone. Group 2 comprised 45 patients with similar types of disc changes who underwent microdiscectomy with an ISD implant. The Visual Analogue Scale (VAS) was used to grade low-back pain and postoperative clinical status was rated according to the modified MacNab criteria. Mean VAS score for low-back pain improved significantly at 1 year follow-up from 7.3 at baseline to 2.75 (p < 0.001) in Group 1 and from 6.7 to 1.5 (p = 0.001) in Group 2. VAS score at 1 year showed significant improvements in 21 Group 1 patients versus 30 Group 2 patients (p = 0.001). Forty four percent of Group 1 patients and 80% of Group 2 patients showed improvement using the modified MacNab criteria. Patients in both groups reported significant improvement in sciatic pain and disability after microdiscectomy with or without an ISD implant. Patients with mild degenerative disc changes were more likely to achieve improvement of their low-back pain when treated with both microdiscectomy and ISD insertion.

Chiari type I malformation is a congenital disorder that is characterized by the caudal extension of the cerebellar tonsils through the foramen magnum into the cervical canal and by a reduced posterior fossa volume.We report our surgical technique of reposition, reduction, or resection of the cerebellar tonsils for the management of Chiari I malformation. The procedure was performed in 22 adult patients, in three different centers, with a mean age of 37 years. Clinical complaints included headaches, nuchalgia, vertigo, and upper-limb weakness or numbness. Seven patients had cervical syringomyelia. Symptoms developed within a mean time of 36 months (range, 12-70 months).The cerebellar tonsils were exposed through a dura mater-arachnoid incision at the atlanto-occipital space after a 0.5-cm rimming craniectomy of the occipital bone in all patients. In seven patients the tonsils were resected, in other seven were reduced by subpial coagulation and aspiration, and in the remaining eight patients the tonsils were repositioned after coagulating their surfaces. Three patients had also a posterior fossa arachnoid cyst that was fenestrated in two of them. All patients improved postoperatively. Syringomyelia was reduced in five of seven patients. The mean length of the follow-up period was 12 months.Selective reposition, reduction, or resection of herniated cerebellar tonsils may improve symptoms in adult patients with Chiari I malformation.

Objectives: The main aim of this review article is to discuss implant-supported overdentures (ISOs) as treatment in edentulous patients. Besides, we will try to discuss among the different treatment options in such patients and to analyze their validity when ISOs are compared with other clinical modalities. At the same time, we will try to suggest clinical guidelines supported by current clinical studies. Material and methods: We performed a Medline search and review of pertinent articles on the mentioned subject from 1986 to 2011. As a searching strategy, we used the following words: implant-supported overdentures, attachment systems, Locator attachment, cantilever, fixed prosthesis. Results and conclusions: Implant-supported overdentures constitute an accurate and predictable treatment option and achieve a higher patients’ satisfaction. This type of treatment constitutes a cheaper treatment than fixed prostheses and in some patients, with loss of lip support or with an interoclusal space larger than 15 mm, the choice of implant-supported overdentures seems to prevent future aesthetic or phonetic problems. Key words:Overdentures, implant occlusion, implant rehabilitation, total edentulous rehabilitation, fixed prosthesis.

Resumen: Introducción: Las deformidades craneales, que han existido desde siempre, recientemente han suscitado un gran interés. Varios estudios muestran un aumento llamativo de consultas por plagiocefalia occipital. En este trabajo se analizan los factores clínico-epidemiológicos relativos a esta deformación. Pacientes y métodos: Se revisaron las historias de 158 niños atendidos en nuestro hospital para evaluación de deformación craneal. Se recogieron datos clínicos y de neuroimagen, tratamientos y resultados, analizando especialmente los factores de riesgo. Resultados: Durante el estudio se apreció un aumento de visitas por esta causa. La media de edad al consultar fue de 9 meses, existiendo un claro predominio (68%) de varones. El tipo de deformación fue: plagiocefalia occipital unilateral (89,2%), bilateral (7,6%) y dolicocefalia (3,2%). Aparte de la posición para dormir, se encontraron diversos factores de riesgo, como colecciones pericerebrales de líquido cefalorraquídeo (LCR) en el 50% de los niños valorados mediante estudios de neuroimagen. El tratamiento fue conservador (posicional y/o fisioterapia) en la mayoría de niños y con cascos en 12, apreciándose mejoría en 136 pacientes. Discusión y conclusiones: Estos hallazgos concuerdan con los de la bibliografía. Un resultado original del estudio fue la elevada incidencia de colecciones pericerebrales de LCR. El aumento de plagiocefalia posicional coincidió con las indicaciones de la Academia Americana de Pediatría sobre la posición para dormir, con el fin de evitar la muerte súbita del lactante. Se destaca el papel del pediatra en la detección y el manejo inicial de estos pacientes. La instauración de medidas de prevención se considera más importante que las indicadas para su tratamiento. Abstract: Introduction: Cranial deformities have always existed, but it is only until recently that they provoked a greater interest. Several reports have demonstrated an increasing tendency for occipital plagiocephaly. This work presents an analysis of the clinical-epidemiological features of these deformities in our area. Patients and methods: The medical records and neuroimaging studies of 158 children seen at our hospital due to a skull deformation were analyzed, paying special attention to risk factors. Results: During the study period, an increase in the number of consultations for skull deformation of children was noted. The mean age for the patients at consultation was 9 months. There was a predominance of boys (68%) over girls. The deformities were: unilateral- (89.2%) or bilateral-posterior plagiocephaly (7.6%), and dolicochephaly (3.2%). In addition to the supine position for sleeping, there were several risk factors, among them pericerebral collections of cerebrospinal fluid found in 50% of children subjected to neuroimaging studies. Most children were managed conservatively, while 12 patients were given helmets, which led to an improvement in 136 cases. Discussion and conclusions: The present findings agree with those in the current literature. A unique result of this study was the high rate of pericerebral fluid collections found in children assessed with neuroimaging methods. The increase in positional plagiocephaly seems to parallel the application of the recommendations for supine sleeping position established by the American Academy of Pediatrics for preventing the sudden infant death syndrome. The role played by Pediatricians in the detection, initial management and referral of these children is highlighted. Prevention measures seem to be more important than those regarding treatment.

By means of some illustrations, the authors briefly report the effects of some accidental head injuries caused by diverse mechanisms occurring in children. Many of these accidents seem to be preventable, but others are completely unavoidable and escape prevention as the one that is depicted in the cover of this issue.

Resumen Fundamento y objetivo Justificar la importancia clinica de la determinacion del isoenzima de fosfatasa alcalina (PLAP-like) como marcador en tumores de celulas germinales (TCG). Pacientes y metodos Se documentan dos casos clinicos observando el comportamiento de este isoenzima: 1) Nino con germinoma intracraneal en region pineal, 75% de actividad de PLAP-like al inicio (valor normal: 0%), resto de marcadores tumorales negativos, y 2) Nina con teratoma inmaduro de ovario estadio I, elevacion de la alfafetoproteina (AFP), 197 ng/mL; PLAP-like 5,1% al inicio y 0,7% en recidiva tumoral tras dos anos. Se determina PLAP-like valorando la actividad remanente tras termodesnaturalizacion serica. Discusion y conclusiones PLAP-like se comporta como unico marcador especifico en el primer caso de tumor germinal pineal que justifica su utilidad junto a pruebas de imagen en el diagnostico de tumores de localizacion craneal. En el seguimiento, tambien es critico el analisis dado que constata la buena respuesta al tratamiento en el primer caso, y en el segundo caso clinico, es el unico marcador que complemente la valoracion de la recidiva. Concluimos en la relevancia del analisis de este isoenzima como marcador complementario en TCG.

Pediatric obesity has become a widespread problem of health in developed countries. Overweight in the pediatric population obeys to a variety of causes. A few of Carreño de Miranda's paintings show pathological conditions occurring in children of his epoch. We briefly illustrate the significance of Carreño's paintings that portray some of these diseases. Two of his best-known works constitute an artistic representation of childhood obesity.

The management of arachnoid cysts (AC) remains controversial. An additional problem derives from the management of hydrocephalus associated with an AC. In this work, we discuss existing procedures proposed in the current literature for their treatment.We reviewed selected reports on intracranial ACs placing special interest in those about the association of hydrocephalus and ACs. We also briefly surveyed data of our patients with this association.Hydrocephalus is often found in midline and posterior fossa ACs. Interhemispheric lesions may also evolve with ventriculomegaly, while middle fossa lesions rarely produce hydrocephalus. Patients' age, cyst location and size, and macrocephaly have all been related to the development of hydrocephalus. Some authors remark on the role played by hydrocephalus and hypothesize that some ACs would result from disturbed cerebrospinal fluid (CSF) dynamics. They also propose that ACs might represent a localized form of hydrocephalus. We also comment on hydrocephalus in relation to the diverse locations of ACs. Neuroendoscopic techniques have transformed previous ways of management as cystoperitoneal shunting and open fenestration.ACs may be pathogenetically related with hydrocephalus, and conversely, ACs may cause hydrocephalus. In some patients, aberrant CSF dynamics seems to play a major role in the development of both cyst and hydrocephalus. Hydrocephalus and ACs may be treated exclusively with neuroendoscopic procedures, although some patients will still require CSF shunting. The ideal option seems to consist of choosing the method that offers the highest success with a single procedure for treating the hydrocephalus and the AC simultaneously.

The aims of this study are the following: first, to analyze incidence, trends, and survival of nervous system tumors in children under the age of 15 in the Region of Murcia, Spain, during the years 1998–2009 and second, to evaluate if certain environmental exposures may be involved in the etiology of childhood nervous system tumors. The study was performed on the spatial and temporo-spatial distribution of the observed cases. The Environment and Pediatric Cancer in the Region of Murcia is an ongoing research project aimed at carefully collecting pediatric environmental history (PEH) and to use geographical information systems to map the incidence and to analyze the geographical distribution of pediatric cancer incidence in our region. Between 1998 and 2009, 125 patients were diagnosed with nervous system tumors. The spatial and temporal space clusters were evaluated using Kulldorff's statistics. Address at diagnosis was the main feature evaluated. The incidence (cases/million children) for central nervous system (CNS) tumors was 34.2, that for sympathetic nervous system tumors was 10.9, and that for retinoblastoma was 1.9. There was evidence of space clustering for medulloblastoma and space–time clustering for all tumors, CNS tumors, astrocytoma, and neuroblastoma. The incidence and survival for each type and subtype of nervous system tumors were within the reported values for the European region. There is evidence that spatial and spatial–temporal distribution in these cases is not random. The development of a careful PEH in these patients will help to reinforce geographical information system studies and to ascertain the importance of associated risk factors.

The authors report the case of a 23-year-old woman with café-au-lait spots and axillary and inguinal freckling who presented with a diploic chronic spontaneous hematoma of the left parietal bone. To the authors' knowledge, this case represents the first description of a diploic hematoma in a patient with stigmata of neurofibromatosis Type 1 unrelated to head trauma. Plain skull radiography showed an osteolytic lesion with well-circumscribed margins, corresponding to the hematoma, together with exuberant perilesional vascular markings. Angiography demonstrated an incidental aneurysm of the left supraclinoidal internal carotid artery and an unusual cortical venous drainage toward the diploic vessels. The blood flow of these vessels on the right hemicranium was sluggish and exhibited enlarged diploic venous lacunas. The authors hypothesize that the hematoma was formed by both an abnormal venous drainage toward the diploic vascular net, together with a vasculopathy that caused stenosis and obstruction of the normal drainage pathways from these vessels.

Se analiza el papel de la radiocirugía en un caso de meduloblastoma multirrecidivado del adulto tras dos intervenciones, radioterapia fraccionada, quimioterapia y dos autotrasplantes. Se resalta la precocidad de la respuesta clínico-patológica al tratamiento radioquirúrgico.

Resumen: Introducción: La presencia de un seno dérmico en la región dorsal de los niños constituye un motivo habitual de consulta en los servicios de neurocirugía pediátrica. Su frecuencia y posibles complicaciones hacen que su diagnóstico y tratamiento precoces sean importantes.En este trabajo revisamos los criterios sobre el tratamiento de los senos sacrocoxígeos para sugerir un manejo racional de acuerdo con los datos publicados para evitar, en lo posible, derivaciones y exploraciones costosas e innecesarias. Material y métodos: Se revisaron las historias de niños diagnosticados de sinus dérmico sacrocoxígeo vistos en nuestro hospital durante el período 2005–2009. Asimismo se realizó una búsqueda bibliográfica de los artículos más relevantes sobre el tema. Resultados: Durante estos años se vieron 32 pacientes con fosita coxígea. La mayoría de niños fueron remitidos por cirujanos pediátricos, pediatras de zona o pediatras de los servicios de urgencia de los hospitales de nuestra región. Diecisiete niños fueron estudiados con pruebas de neuroimagen y 15 fueron evaluados exclusivamente mediante exploración física. En ninguno de los pacientes se apreció penetración intraraquídea. Conclusiones: De acuerdo a nuestra experiencia y a la bibliografía consultada pensamos que la exploración clínica es suficiente para un diagnóstico y manejo correcto de estos senos, siendo el dato más importante la situación de la fosita coxígea dentro de los límites del repliegue interglúteo. En casos dudosos de conexión del seno con la duramadre se deberá realizar una ecografía como prueba de primera línea y, si es necesario, una resonancia magnética y consulta con Neurocirugía. Abstract: Introduction: The presence of a dermal sinus on a child's back is a common cause for paediatric neurosurgical consultation. The relative frequency of these lesions and fears on their possible clinical consequences enhances the importance of early diagnosis and treatment. With this work we aimed to search for the most relevant clinical features that might indicate an intradural penetration of the lesions and that may advise performing neuroimaging studies and a paediatric neurosurgical consultation. Patients and methods: The records pertaining to children diagnosed with sacro-coccygeal pits seen at our institution during the years 2005–2009 were reviewed. A Pub Med search of the most relevant articles on the subject was also performed. Results: Thirty-two children diagnosed with sacro-coccygeal pits were seen at our institution in the study period. Most cases had been sent for neurosurgical consultation by their paediatricians, paediatric surgeons or paediatricians of the emergency services in our region. Seventeen patients were submitted to some neuroimaging study and 15 were evaluated only by physical examination. In no patient was an intradural penetration of the lesion observed. The lack of reports on coccygeal sinuses probably reflects the unimportance given to these lesions in the absence of neurological involvement. Conclusions: A detailed physical examination can reduce the number of neuroimaging studies, an intergluteal localization of the sinus being the most important diagnostic clue. Doubtful cases of an intradural penetration of the lesion can be further evaluated by ultrasound as a preliminary screening tool and by magnetic resonance and neurosurgical consultation if deemed necessary.

Occult spinal dysraphism (OSD) encompasses various conditions. A dermal sinus tract (DST) consists of a duct communicating to the skin with deep structures that carries an important risk of infection. A different lesion consisting of a translucent skin opening and a fibrous tract that lacks a lumen can also be found in OSD. We termed this lesion pseudo-dermal sinus tract. We reviewed clinical features of 20 patients with spinal skin orifices. The patients were classified into two categories: group 1 comprised children with true DST and group 2 included patients with skin dimples resembling a DST. The aim of this study was to analyze differential features of patients in these two groups as they behaved dissimilarly in regard to clinical significance and outcomes, especially those concerning infectious risk. Children of group 1 (n = 8) presented with tiny skin orifices and with superficial or deep infection. In contrast, group 2 (n = 12) mostly manifested with neurological symptoms. No patient in group 2 developed an infection preoperatively. In both groups, magnetic resonance showed tracts that ended in different structures. Histopathology of the lesions were dissimilar, those of group 1 being hollow tubular structures lined by epithelium while those of group 2 being solid tracts of fibrous connective tissue. DST constitutes a clinicopathological diagnosis. Although sharing some common cutaneous and neuroimaging findings, both groups behaved differently. Patients of group 1 tended to show up with infection requiring urgent surgery. Patients of group 2 often presented with neurological manifestations and skin lesions, but needed no immediate operation.

Niikawa-Kuroki, or Kabuki syndrome (KS), is characterized by distinctive facial features, skeletal anomalies, persisting fingertip pads with dermatoglyphic abnormalities, short stature, and mental retardation. Neurological manifestations and CNS anomalies have been described in some patients with this condition. However, craniosynostosis has been documented in only 4 patients with KS who did not undergo operations. The authors report a case of KS with unicoronal synostosis that constitutes the first documented instance of a patient with this syndrome submitted to surgery. Previous reported instances of craniosynostosis occurring in KS are briefly reviewed. Although rarely documented, craniosynostosis might represent a relatively frequent feature of this syndrome. Kabuki syndrome should be considered at the time of evaluating children with craniosynostosis. The diagnosis of KS can be suspected from the patients' characteristic facial features. Kabuki syndrome appears to be an underdiagnosed condition in the craniosynostosis population. Given that most patients with this syndrome suffer from only mild to moderate mental retardation, surgical correction can be considered in instances of KS with craniosynostosis.

Since 1992, the Centers for Disease Control and Prevention recommends that women of childbearing age consume 400 µg of folic acid per day to reduce the risk of neural tube defects (NTD). It has been speculated that both NTD and nervous system tumors (NST) may share common mechanisms of altered development. It examines the association between folic acid supplementation and the risk for childhood NST.Incident cases of children with cancer in Spain registered between 2004 and 2006 were identified through the MACAPE Network Group. Tumors were classified as tumors derived from the neuroectoderm (cases) and those with a mesoderm origin (controls). In a second analysis, NST were further divided into central nervous system tumors (CNST) and sympathetic nervous system tumors (SNST). We compared folic acid supplementation between the groups.Overall, folic acid supplementation any time during pregnancy was similar between cases and controls (odds ratio (OR)=1.05; 95% confidence interval (CI) 0.92-1.20). However, supplementation before the 21st and 36th days of gestation resulted in significantly lower NST than in children with mesoderm tumors (OR=0.34; 95% CI 0.17-0.69 and OR=0.58; 95% CI 0.37-0.91, respectively). Preconceptional intakes of folic acid were also lower in NST although marginally nonsignificant (OR=0.44; 95% CI 0.10-1.02). When NST were divided into CNST and SNST, significant differences between tumors of mesoderm origin were only found for CNST.Our results support the hypothesis that folate supplementation reduces the risk of childhood NST, especially CNST. The specific mechanism and cellular role that folate may play in the development of CNST have yet to be elucidated.

Background The authors report the morphological and neuroimaging findings of an immature human fossil (Cranium 14) diagnosed with left lambdoid synostosis. Discussion The skull was recovered at the Sima de los Huesos site in Atapuerca (Burgos, Spain). Since the human fossil remains from this site have been dated to a minimum age of 530,000 years, this skull represents the earliest evidence of craniosynostosis occurring in a hominid. A brief historical review of craniosynostosis and cranial deformation is provided.

Objective: To analyze the association of diverse cerebral anomalies in a series of pediatric patients with cerebellar tonsillar ectopia. Methods: We reviewed the medical records of 60 children diagnosed with Chiari type 1 malformation (CM1), of these, 20 patients (11 boys and 9 girls; mean age 7.2 years, range 2–16 years) had an associated cerebral anomaly. Symptoms of tonsillar ectopia evolved over a mean of 12 months (range 3 months to 4 years). Syringomyelia was present in 5 cases. All patients underwent a posterior fossa decompression. Results: Disclosed anomalies included: congenital hydrocephalus (n = 11), cervicomedullary kinking (n = 5), focal cerebral heterotopia with epilepsy (n = 4), partial agenesis of the corpus callosum (n = 4), hypoplastic brain stem (n = 2), holoprosencephaly (n = 1), and subcortical dysplasia in the context of neurofibromatosis type 1 (n = 1). Other malformations included: subcortical hamartoma associated with neurofibromatosis type 1, craniofacial dysmorphism secondary to Noonan syndrome, congenital occipital plagiocephaly, os odontoideum, craniofacial cleft, juvenile rheumathoid arthritis with platybasia, and osteogenesis imperfecta with bathrocephaly and scoliosis. Conclusion: Craniocerebral anomalies in children treated for CM1 may be found consistently. The association of hydrocephalus, which was the most common anomaly in this cohort, with cerebellar tonsillar ectopia may contribute to a poor outcome in regard to tonsillar herniation symptoms.

Macrocephaly-cutis marmorata telangiectatica congenita (M-CMTC) is a newly described condition characterized by macrocephaly (megalencephaly), cutis marmorata telangiectatica congenita, macrosomia and/or asymmetric growth, central nervous system abnormalities and neurological manifestations.This condition is usually documented in the Genetics' literature but it has been scarcely disseminated among neurosurgeons. Sudden death of uncertain origin has been reported in children with M-CMTC. Hydrocephalus and tonsillar herniation often occur in this syndrome. The appearance of symptoms and signs of intracranial hypertension or of brain stem compression in M-CMTC patients may herald the development of irreversible neurological damage or death.We report two children diagnosed with M-CMTC and hydrocephalus who were given a ventriculo-peritoneal shunt. In addition, one of them exhibited tonsillar herniation.Given the high incidence of hydrocephalus and/or tonsillar herniation that occurs in M-CMTC, serial neuroimaging studies and neurosurgical referral seem to be warranted in children with this syndrome.

Several positions are currently utilized for operating patients with posterior fossa lesions. Each individual position has its own risks and benefits, and none has demonstrated its superiority. A dreaded, and probably underreported, complication of these procedures is cervical cord infarction with quadriplegia.We reviewed eight previous reported instances of this devastating complication aimed at ascertaining its pathogenesis to suggest preventive strategies. Several hypotheses have been put forward to explain the occurrence of this complication. Some factors involved in the production of cervical cord infarction include patient's position (seated or prone), hyperflexion of the neck, excessive spinal cord traction, canal stenosis, and systemic arterial hypotension. We hypothesize that spinal cord infarction in our patient might have resulted from compromised blood supply to the midcervical cord caused by tumor infiltration of the cervical leptomeninges in addition to a brief episode of arterial hypotension during venous air embolism.We treated an 8-year-old girl who developed quadriplegia after surgery for a fourth ventricular ependymoma. Postoperative magnetic resonance imaging demonstrated cervical cord infarction. Evoked potentials confirmed the diagnosis.With this report, we want to draw the attention of neurosurgeons to the possibility of the occurrence of this dreadful complication during posterior fossa procedures. Retrospectively, the only measures that might have helped to avoid this complication in our patient would have been using the prone position and intraoperative monitoring of evoked potentials.

Cerebrospinal fluid (CSF) overdrainage in hydrocephalus is well recognized. Overshunting following cysto-peritoneal (CP) drainage in patients with arachnoid cysts (AC) is rarely documented. We report five patients with acquired Chiari malformation I and three with posterior fossa overcrowding due to excessive CSF drainage in shunted intracranial ACs. We review our observations and discuss the current knowledge on the pathogenesis and management of this complication. The medical records of the eight patients were analyzed in regard to clinical manifestation, cyst and shunt characteristics, management, and outcomes. Mean age of the patients was 5.5 years. After an average interval of 5 years, five patients developed symptoms related to hindbrain herniation and three to severe shunt overdrainage following CP shunting. Several management modalities were utilized that achieved a good result in seven instances. Some shunted ACs may evolve with overdrainage syndromes. Posterior fossa overcrowding and tonsillar herniation constitute their most severe forms. CSF hypotension, bone changes, venous engorgement, and probably cerebral chronic edema at the posterior fossa constitute the main factors involved in the pathogenesis of this entity. We also review previous instances of acquired Chiari malformation originating after AC shunting. Posterior fossa overcrowding and acquired Chiari I malformation can develop after excessive CSF drainage of intracranial ACs. Overshunting manifestations require prompt recognition and management. Preventive measures consist of making a stringent selection of cases being considered for surgery, avoiding CP drainage, and placing of a programmable valve as initial treatment of intracranial ACs if shunting is considered.

Intra-ventricular haemorrhage (IVH) can occur spontaneously or during the surgical revision of ventricular cerebrospinal fluid (CSF) shunts.The aim of the study was to report the safety and efficacy of an original method for treatment of IVH that may occur at the time of valve revision aimed at maintaining the function of previously implanted CSF shunts.We reviewed the medical records of six patients who experienced an IVH in the presence of a previously placed ventriculoperitoneal (VP) shunt. Five of the haemorrhages occurred during ventricular catheter replacement and the remaining one in a child given a VP shunt who sustained a spontaneous intra-cerebral haemorrhage. We inserted an external ventricular drainage without removing the original shunt. Urokinase was administered via the ventricular drain during several days until blood clearance in the CSF. Disappearance of the ventricular clots was checked by a cranial computerised tomography scan, while CSF shunt function was verified by the children's evolution and/or by a reservoir tap.Follow-up evaluation of the six patients demonstrated that the existing VP shunts were functioning appropriately and that the treatment was safe.Patients with IVH complicating ventricular catheter replacement and patients with spontaneous bleeding who harbour a VP shunt can be treated by intra-ventricular urokinase to avoid the removal of the initial shunt. The technique has proven to be safe and utilises the ventricular drain placed for the acute management of the IVH. Shunt replacement will always be possible in case of failure of the technique we are reporting.

Increased intra-abdominal pressure has been reported to result in raised intracranial pressure in a variety of conditions such as obesity and pregnancy, and it also constitutes an infrequent cause of ventriculoperitoneal (VP) shunt malfunction. Patients with neurological deficits, as those with myelomeningocele or cerebral palsy, are prone to developing a neurogenic bowel and to suffer chronic constipation. Although previously recognized, VP shunt failure attributed to constipation has only recently been described. We briefly review the etiopathogenesis, diagnosis and management of severe constipation leading to VP shunt malfunction. Our aim is to draw the attention of pediatric neurosurgeons towards severe constipation as a possible cause of VP shunt failure thus avoiding unnecessary surgical valve revisions, to which children with hydrocephalus are often submitted to.We report two children that developed transient VP shunt failure because of intense constipation that caused exacerbation of previously shunted hydrocephalus. One of the patients constitutes the first description of this complication associated with an anteriorly placed anus and the other with intestinal paresis after ileostomy. Conservative treatment aimed at alleviating the increased intra-abdominal pressure resulted in complete resolution of the children's manifestations of VP shunt failure, without having to resort to surgical revision of the VP shunt.

Intracranial cerebrospinal fluid (CSF) volume depletion causes diverse clinical syndromes most of them constituting the manifestations of decreased intracranial pressure. Subdural collections or chronic subdural hematomas are the best-known consequences of persistent CSF leaks, especially in overshunted hydrocephalus. Continuous CSF escape also occurs after lumbar puncture, spinal anesthesia, and diverse spinal surgeries. A 6-year-old boy submitted to reoperation of spinal cord compression due to partial sacral agenesis complained of postoperative orthostatic headaches and vomiting initially attributed to CSF hypotension. There were neither subcutaneous fluid accumulations nor CSF leakage from the wound. The child was treated with strict bed rest and intravenous hydration for 5 days. On reassuming orthostatism, the patient had syncope but did not hit his head. A cranial computerized tomography scan showed an acute subdural hematoma that was managed conservatively with total recovery. A review of current literature showed scanty reports of acute intracranial bleeding occurring after CSF depletion following spinal surgical procedures. To our knowledge, our reported patient represents the second case of this occurrence following surgery for closed spinal dysraphism in a child. The authors briefly review documented instances of acute subdural hematoma following spinal procedures, advise about its diagnosis, and suggest preventive measures.

Two patients with intracranial arachnoid cysts, one with myelomeningocele-hydrocephalus and the other with a subdural fluid collection, were given a cerebrospinal (CSF) extracranial shunt. All four patients developed features of CSF overdrainage following shunting and were treated by cranial vault expanding procedures. Before undergoing decompressive craniotomy, the patients were treated by a variety of procedures, including changing of obstructed ventricular catheters (n=4), insertion or upgrading of programmable valves (n=3), and foramen magnum decompression (n=1). Clinical manifestations of these four patients were attributed to craniocerebral disproportion caused by chronic and progressive skull changes due to dampening of the CSF pulse pressure, which is necessary for maintaining normal cranial growth. On the basis of our previous experience with expanding craniotomies in cases of minimal forms of craniosynostosis, we treated these patients with bilateral parietal craniotomies, with satisfactory results. In conclusion, biparietal decompressive craniotomy constitutes a useful and safe procedure for relieving the clinical manifestations of some CSF overdrainage syndromes, especially in cases with slit-ventricle syndrome and craniocerebral disproportion that prove to be refractory to simpler management procedures.

Glutaric aciduria type 1 (GA-1) is an inborn error of metabolism that results from a deficiency of glutaryl-CoA dehydrogenase. This disorder mainly manifests in early childhood and most patients with this condition develop a dystonic-dyskinetic syndrome. We report the anesthetic management of two sisters with GA-1, aged 30 and 17 months respectively at the time of surgery, who presented with macrocephaly and psychomotor delay. The children required CSF shunting procedures for hydrocephalus and subdural fluid collections, which were performed under total intravenous anesthesia with propofol and remifentanil.

The authors report two unusual cases of extraspinal midline lumbosacral lesions that resembled the usual skin markers of occult spina bifida.The pathological diagnosis of the masses was plexiform neurofibroma and mastocytoma, respectively.Some lesions, as those we are reporting, although occurring at the lumbosacral midline, do not necessarily belong to occult spinal dysraphism, as was initially suspected. Plexiform neurofibroma and skin mastocytoma are very rare indeed in this spinal location.The two cases represent an important addition to the differential diagnosis of a congenital dorsal midline mass.

Hydrocephalus associated with intraspinal tumors constitutes a well-documented occurrence. The accepted mechanisms for this association seem to be well established. On the contrary, hydrocephalus in the context of intraspinal dermoids has been rarely recognized and its pathogenetic mechanism appears to be different.The authors report four pediatric cases of spinal dermoid tumors and dermal sinuses that developed hydrocephalus during the evolution of these congenital lesions of ectodermal origin.In two children, the mechanism leading to the development of hydrocephalus consisted of leptomeningeal inflammation due to bacterial meningitis or to spillage of dermoid cyst contents in the cerebrospinal fluid spaces. We hypothesize that ventricular dilatation in the other two might be the result of chemical meningitis occurring during intrauterine life. Two cases developed ventriculomegaly prior to the diagnosis of their spinal cord disease.The unusual evolution of these cases suggests that neuroimaging studies that include the spine should be performed in cases of childhood "unexplained hydrocephalus".

Background Accidental transmission of Creutzfeldt-Jakob disease (CJD) has been reported after neurosurgical interventions, use of intracerebral electrodes, corneal transplants, and after the administration of human-derived hormones. Acquired CJD has also been documented after dural grafting with tissues of human cadaver origin. At present, quinacrine and chlorpromazine are being investigated for the treatment of sporadic CJD, with the hope of offering an effective treatment of an otherwise fatal disease. Our objective was to report a case of iatrogenic CJD occurring 18 years after the implant of a dural graft of human origin and to inform on the results of the treatment with quinacrine and chlorpromazine. Case Description In 1984, a 46-year-old woman was given a Lyodura graft for decompression of Chiari I malformation and syringomyelia. The patient was diagnosed with CJD in 2002. In view of the scarce options for treatment of CJD and after reviewing the current literature, the patient was treated with quinacrine and chlorpromazine. She showed no clinical improvement and died 6 months after hospital admission. The iatrogenic origin of the disease in this patient is supported by the clinical features, laboratory data, and findings from the brain necropsy. Conclusions Patients who have received a dural graft of cadaveric origin may be at risk for developing CJD after very prolonged incubation periods. Treatment with quinacrine and chlorpromazine for acquired CJD was ineffective in our patient. A clinical trial on the use of antiprion agents is warranted.

Patients with craniosynostosis are readily diagnosed by clinical and neuroimaging findings. Surgical treatment is indicated for preventing neurological deficits and for correcting esthetically unacceptable head deformities. In recent years, we have witnessed a progressive number of neurosurgical consultations for abnormal head shapes unrelated to premature fusion of the cranial sutures, especially of positional plagiocephaly. There have been descriptions of abnormalities in the cerebrospinal fluid (CSF) spaces in children with craniosynostosis.The aim of the present study was to investigate the role of the changes of the CSF spaces in the development of positional skull deformities in children.The authors reviewed demographic, clinical, and neuroimaging data of 23 patients assessed for some form of nonsynostotic skull deformity (group A). The results were compared with those of a simultaneous group of nine infants diagnosed with benign extracerebral collections of fluid (group B).The study group was composed of 11 boys and 12 girls, aged 3 years or younger. Seventeen children had plagiocephaly, four scaphocephaly, and two brachycephaly. Sixteen children (15 with plagiocephaly and 1 with brachycephaly) exhibited enlarged subarachnoid CSF spaces. In group B, the boy/girl ratio was of 7:2. Infants in group A presented at an older age (mean 12.7 months) than group B (mean age at presentation of 7.17 months). Children with benign extracerebral fluid collections were born with a head circumference (HC) greater than infants with positional skull deformities (p=0.005). The percentile of the children's HC at consultation was also larger for children of group B (p=0.03). The form of clinical presentation differed between the two groups. Most infants of group A were seen because of a type of head deformity, and children in group B were studied for macrocephaly. Long-term follow-up assessment showed better outcomes for patients in group B than for children of group A in regard to regression of initial symptoms (p=0.03).Most positional head deformities appear to be related with the children's positioning for sleeping. We have not confirmed macrocephaly as a contributing factor for positional deformities. The distribution of extracerebral CSF and the presence of abnormal collections of fluid in children with positional head deformities do not seem to be related with the findings of pericerebral CSF encountered in children with benign extracerebral collections of fluid. In our view, brain pulsations, transmitted to these accumulations of CSF, play an important part in the development of the infants' skull deformities (p=0.02). The findings of enlarged CSF spaces in children with nonsynostotic skull deformation constituted an age-related event, as these collections tended to disappear as the children grew older (p=0.04).

Diverse materials have been used for dural closure, including grafts of cadaveric origin. Some 168 cases of Creutzfeldt-Jakob disease have been reported following the implant of human dura mater. A few publications have addressed the use of dural grafts in children, but none has analyzed the feasibility of autologous tissues for this purpose.Since 1994, the authors have utilized autologous tissues for dural grafting in children undergoing neurosurgical procedures, aimed at studying the feasibility of its use in this age group.We studied 56 children submitted to neurosurgical procedures who underwent an implant of a patch of autologous tissue for dural closure. Epidemiological, clinical, and neuroimaging data were processed using statistical methods.The study group was composed of 29 boys and 27 girls, with ages ranging from 1 month to 17 years (mean 7.6 years). The procedures corresponded to lesions at the posterior cranial fossa (n = 41), supratentorial cranial compartment (n = 12), and spine (n = 3). Seventy-five percent of the children had no complication. Four patients had adverse effects unrelated to dural grafting, while ten children had complications that might be associated with it. The most frequent complication was pseudomeningocele (n = 6), followed by infection (n = 3) and CSF leak (n = 1). Although hydrocephalus seemed to have an influence on the occurrence of complications, their incidence in patients without hydrocephalus did not differ statistically.Autologous grafts of fascia or pericranium can be safely used for dural reconstruction in children. Given the reported complications associated with the use of foreign materials for duraplasty, the authors recommend the use of autologous tissues for dural repair, reserving other types of dural grafts for cases in which its usage is unavoidable.

The case of a 14-year-old girl who presented with a 2-week history of raised intracranial pressure is reported. A left frontal extra-axial tumor was totally removed, whose histopathologic diagnosis was rhabdoid meningioma (RM). Rhabdoid meningiomas constitute a special malignant phenotype of meningioma that has been recently included in the WHO classification of tumors of the nervous system. Usually, RMs affect middle-aged and elderly individuals. We report the fourth case of a RM occurring in a child to illustrate that the diagnosis of this tumor subtype, given its prognostic implications, must also be considered in pediatric patients

Resumen El drenaje excesivo de las valvulas constituye una complicacion dificil, tanto de prevenir como de tratar. Los autores revisan una serie de 512 ninos operados mediante derivacion de liquido cefalorraquideo, de manera retrospectiva, para analizar las causas de esta complicacion. La causa de la hidrocefalia fue congenita (n= 172), debida a mielomeningocele (n= 123), posthe-morragica (n=103), tumoral (n= 64), postinfecciosa (n=40) y postraumatica (n= 10). Ochenta y ocho ninos (17,8%) presentaron alguna complicacion por funcionamiento excesivo del sistema valvular. Se analizo la etiologia de la hidrocefalia relacionandola con los cuadros de hiperdrenaje. La complicacion mas frecuente fue la obstruccion del extremo ventricular de la derivacion (n= 50), seguida del cuadro de colapso ventricular sintomatico (n= 19), de hematoma subdural (n= 10) y de cuarto ventriculo aislado (n=9). Las diversas complicaciones se distribuyeron de manera homogenea entre todos los grupos etiologicos, sin diferencia estadistica. El cuadro de colapso ventricular sintomatico (CVS) ocurrio en 19 ninos (3,71%), cifra menor que la descrita en la bibliografia. Solo dos grupos de pacientes con hidrocefalia adquirida (postinfecciosa y posthemorragica) tuvieron una mayor tasa de CVS (p=0,005), lo que se atribuyo a la mayor incidencia en estos ninos de procesos destructivos cerebrales. El tratamiento del CVS fue complejo y requirio diversos procedimientos, que se aplicaron de manera escalonada, incluyendo cinco craniectomias descompresivas. Los autores recomiendan evitar, en lo posible, el empleo de valvulas, utilizando en su lugar, y siempre que sea posible, tecnicas de neuroendoscopia, o valvulas de nueva tecnologia, que supuestamente disminuiran el numero de complicaciones de hiperfuncion valvular.

Object. Most craniocerebral injuries are caused by mechanisms of acceleration and/or deceleration. Traumatic injuries following progressive compression to the head are certainly unusual. The authors reviewed clinical and radiological features in a series of patients who had sustained a special type of cranial crush injury produced by the bilateral application of rather static forces to the temporal region. Their aim was to define the characteristic clinical features in this group of patients and to assess the mechanisms involved in the production of the cranial injuries and those of the associated cerebral and endocrine lesions found in this peculiar type of head injury. Methods. Clinical records of 11 patients were analyzed with regard to the state of consciousness, cranial nerve involvement, findings on neuroimaging studies, endocrine symptoms, and outcome. Furthermore, an experimental model of bitemporal crush injury was developed by compressing a dried skull with a carpenter's vice. Seven of the 11 patients were 16 years old or younger. All patients presented with a characteristic clinical picture consisting of no loss of consciousness (six patients), epistaxis (nine patients), otorrhagia (11 patients), peripheral paralysis of the sixth and/or seventh cranial nerves (10 patients), hearing loss (five patients), skull base fractures (11 patients), pneumocephalus (11 patients), and diabetes insipidus (seven patients). Ten patients survived the injury and most recovered neurological function. Conclusions. Static forces applied to the head in a transverse axis produce fractures in the skull base that cross the midline structures without producing significant cerebral damage. Stretching of cranial nerves at the skull base explains the nearly universal finding of paralysis of these structures, whereas an increase in the vertical diameter of the skull accounts for the occurrence of diabetes insipidus in the presence of an intact function of the anterior pituitary lobe. The association of clinical, endocrine, and neuroimaging findings encountered in this peculiar type of head injury supports the idea that this subset of injured patients has a distinctive clinical condition, namely the syndrome of bitemporal crush injury to the head.

Grisel's syndrome is a uni- or bilateral subluxation of the atlas on the axis associated with an inflammatory condition of the head or neck. This uncommon entity usually affects children. The condition seems to be secondary to hyperemia and laxity of the atlanto-axial joints. We report clinical and neuroimaging findings, treatment modalities, and outcomes in 4 children with atlanto-axial rotatory subluxation (AARS) associated with a previous upper respiratory tract infection treated at our Unit during the last 7 years. All children presented with neck pain, head tilt, and reduced neck motion. In 3 patients, the condition was secondary to otitis media and in the remaining patient to viral pharyngitis. The diagnosis was established by clinical signs and symptoms and confirmed by neuroimaging studies, especially by computerized tomography and 3-D reconstructions. Early detection and treatment resulted in a good outcome in all patients. None of the patients required surgical treatment. Grisel's syndrome must be suspected in children with painful torticollis associated with an upper tract respiratory infection. The best results are obtained with early conservative treatment, which prevents chronic changes leading to persistent neck pain and deformity, namely atlanto-axial rotatory fixation (AARF). We emphasize the fact that the diagnosis of Grisel's syndrome is mainly clinical, while the diagnostic confirmation of AARF can only be attained by special neuroimaging techniques.

This case study reports neuropsychological and structural magnetic resonance (MRI) studies of a 10-year-old girl with a left hemisphere lesion, caused by an underwater fishing harpoon penetrating her head when she was 6 years old. The patient showed a marked deficit in the acquisition of reading, writing and arithmetic, as well as an attentional deficit. Magnetic resonance images revealed left cortical lesions in the orbital region and the gyrus angularis, as well as in the caudate and putamen nuclei and longitudinal inferior fascicle. Neuropsychological assessment showed frontal and parietal lobe dysfunctions consistent with the lesional data. The structural data explain the neuropsychological impairment and suggest that, although the left lesion was early and relatively small, plasticity was incomplete.

Case report. We report the case of a newborn baby who presented with scalp aplasia cutis congenita and an underlying skull defect. Discussion.We discuss the diverse options for the management of this condition and the feasibility of early surgical repair in the light of the current literature. We also comment on the possible role of benzodiazepines in the genesis of this lesion.

A previous study by computational fluid dynamics (CFD) of the three-dimensional (3-D) flow in ventricular catheters (VC) disclosed that most of the total fluid mass flows through the catheter’s most proximal holes in commercially available VC. The aim of the present study is to investigate basic flow patterns in VC prototypes. The general procedure for the development of a CFD model calls for transforming the physical dimensions of the system to be studied into a virtual wire-frame model which provides the coordinates for the virtual space of a CFD mesh, in this case, a VC. The incompressible Navier–Stokes equations, a system of strongly coupled, nonlinear, partial differential conservation equations governing the motion of the flow field, are then solved numerically. New designs of VC, e.g., with novel hole configurations, can then be readily modeled, and the corresponding flow pattern computed in an automated way. Specially modified VCs were used for benchmark experimental testing. Three distinct types of flow pattern in prototype models of VC were obtained by varying specific parameters of the catheter design, like the number of holes in the drainage segments and the distance between them. Specifically, we show how to equalize and reverse the flow pattern through the different VC drainage segments by choosing appropriate parameters. The flow pattern in prototype catheters is determined by the number of holes, the hole diameter, the ratio hole/segment, and the distance between hole segments. The application of basic design principles of VC may help to develop new catheters with better flow circulation, thus reducing the possibility of becoming occluded.

The concept of iatrogenic complications is intimately associated with untoward problems related to the execution of a medical or surgical procedure. There is no medical act devoid of the possibility of an iatrogenic incident. However, many of them seem to be preventable and occur in spite of the willingness or experience of the treating physician. Therefore, iatrogenesis is apt to occur even when a good medical practice has been carried out. The line that distinguishes iatrogenic incidents from true complications seems to be very thin and difficult to draw.