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Case Report: 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., Discussion and Conclusions: 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.

Background: 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., Case Description: 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., Discussion and Conclusion: 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.

Object: 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., Methods: 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., Results: 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., Conclusions: 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 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., (Copyright © 2014 Elsevier Ltd. All rights reserved.)

Background: 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., Methods: 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)., Results: 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., Conclusions: Selective reposition, reduction, or resection of herniated cerebellar tonsils may improve symptoms in adult patients with Chiari I malformation.

Objectives: This study aims to describe clinical-epidemiological data regarding accidental fall injuries occurring during homecare activities among children up to 1 year of age and to compare their outcomes according to the type of trauma., Methods: We searched four different hospital databases on head injuries from 1999 to 2009. Patients recorded under the descriptors "accidental fall" and "home-related" in the subtext were selected. Patients were classified into two groups: those who flipped over and fell from a changing table (n = 253) and those who fell from the bed sustaining a direct impact from the floor (n = 483)., Results: There was no difference between both groups with respect to age, gender, and Glasgow Coma Scale score. However, children who suffered injuries after an accidental fall from the changing table were more likely to require surgery (26/483 vs. 57/253, p < 0.0001), had a mean longer length of stay (LOS, 4 vs. 1 day), and a higher incidence of depressed skull fractures (12/483 vs. 24/253, p < 0.0001). Children with a direct impact from the floor after falling off the bed were expected to suffer from simple linear skull fractures, while those who flipped over the changing table were more likely to present facial, soft tissue, or skeletal injuries., Conclusions: Children who flipped over a changing table during their homecare activities were more likely to require surgery, showed a higher morbidity, and showed a longer LOS than those who fell down from the bed. These results probably reflect the different impact energy according to each injury mechanism.

The authors illustrate the cases of two children with headaches, one diagnosed with Chiari type 1 malformation and the other with hydrocephalus, who played wind instruments. Both patients manifested that their headaches worsened with the efforts made during playing their musical instruments. We briefly comment on the probable role played by this activity on the patients' intracranial pressure and hypothesize that the headaches might be influenced by increases in their intracranial pressure related to Valsalva maneuvers. We had serious doubts on if we should advise our young patients about giving up playing their music instruments.

Background: Craniocervical distraction injuries, including atlanto-axial dislocation (AAD) and atlanto-ocipital dislocation (AOD), are often associated with severe spinal cord involvement with high morbidity and mortality rates. Many patients with these injuries die at the accident scene, but advances in emergency resuscitation and transport permit that many patients arrive alive to hospitals., Discussion: Children with craniocervical distraction injuries usually present with a severe cranioencephalic traumatism that is the most relevant lesion at admission. After resuscitation and hemodynamic stabilization, the spinal cord damage appears as the main lesion. Apnea and quadriparesis, or quadriplegia, are usually present at the onset. Early diagnosis and management perhaps decrease life-threatening manifestations of the spinal lesion. But even so, the primary spinal cord insult is often irreversible and precludes obtaining a satisfactory functional outcome., Patients and Methods: We report the findings of four children with craniocervical distraction injuries (AOD and AAD) who presented with severe spinal cord damage. All patients were admitted with respiratory distress or apnea together with significant brain injuries. The medical records pertaining to these patients are summarized in regard to clinical features, management, and outcome., Conclusions: In spite of timely and aggressive management, craniocervical injuries with spinal cord involvement continue to have a dismal prognosis. Outcome is closely related to the severity of the initial brain and spinal cord damage and is nearly always fatal in cases of complete spinal cord transection. Priority should be given to life-threatening complications. Ethic issues on indications for surgery deserve a detailed discussion with the children's parents.

Background: Head injuries constitute one of the leading causes of pediatric morbidity and mortality. Most injuries result from accidents involving an acceleration/deceleration mechanism. However, a special type of head injury occurs when the children sustain a traumatism whose main component is a static load in relation to a crushing mechanism with the head relatively immobile., Patients and Methods: We report a series of children who sustained a craniocerebral injury of variable severity produced by head crushing. We also analyze epidemiological and clinical data, and biomechanics in these injuries., Results: Mean age of the group (13 boys/6 girls) was 4.1 years. All patients showed external lesions (scalp wounds or hemorrhage from the nose, ears, or throat). Eleven children were initially unconscious. Six children presented cranial nerve deficits in addition to impaired hearing. Skull base fractures were seen in most cases with extension to the vault in 11 instances. Fourteen patients had an associated intracranial lesion, including two with diffuse axonal injury. Surgery was performed in three instances. Only seven patients were left with sequelae., Discussion and Conclusions: The observed skull, brain, and cranial nerve lesions corresponded to a mechanism of bilateral compression of the children's heads mainly occasioned by a static load, although an associated component of dynamic forces was also involved. The skull and its covering and the cranial nerves were the most severely affected structures while the brain seemed to be relatively well preserved. Most crush injuries appear to be preventable by the appropriate supervision of the children.

In 1899, the Spanish painter Joaquín Sorolla represented, in a large canvas, a group of children probably with sequels of poliomyelitis bathing at Valencia's beach. The title of this painting was Sad Legacy. This work contributed to the international diffusion of Sorolla's artistic creation. We briefly report some facts regarding the painter and his work referring to those portraits of children and especially of sick children.

The objective of this article is to assess the safety of intraspinal infusion of autologous bone marrow mononuclear cells (BMNCs) and, ultimately, to look for histopathological signs of cellular neurotrophism in amyotrophic lateral sclerosis (ALS) patients. We conducted an open single arm phase I trial. After 6 months observation, autologous BMNCs were infused into the posterior spinal cord funiculus. Safety was the primary endpoint and was defined as the absence of serious transplant-related adverse events. In addition, forced vital capacity (FVC), ALS-functional rating scale (ALS-FRS), Medical Research Council scale for assessment of muscle power (MRC), and Norris scales were assessed 6 and 3 months prior to the transplant and quarterly afterward for 1 year. Pathological studies were performed in case of death. Eleven patients were included. We did not observe any severe transplant-related adverse event, but there were 43 nonsevere events. Twenty-two (51%) resolved in ≤2 weeks and only four were still present at the end of follow-up. All were common terminology criteria for adverse events grade ≤2. No acceleration in the rate of decline of FVC, ALS-FRS, Norris, or MRC scales was observed. Four patients died on days 359, 378, 808, and 1,058 post-transplant for reasons unrelated to the procedure. Spinal cord pathological analysis showed a greater number of motoneurons in the treated segments compared with the untreated segments (4.2 ± 0.8 motoneurons per section [mns per sect] and 0.9 ± 0.3 mns per sect, respectively). In the treated segments, motoneurons were surrounded by CD90+ cells and did not show degenerative ubiquitin deposits. This clinical trial confirms not only the safety of intraspinal infusion of autologous BMNC in ALS patients but also provides evidence strongly suggesting their neurotrophic activity., (Copyright © 2012 AlphaMed Press.)

Aims: We aimed to assess the effects of exposure to tobacco smoke, alcohol and illegal drugs during early pregnancy on the head circumference (HC) at birth of otherwise healthy neonates., Methods: A follow-up study from the first trimester of pregnancy to birth was carried out in 419 neonates. An environmental reproductive health form was used to record data of substance exposure obtained during the first obstetric visit at the end of the first trimester. A multiple linear regression model was created for this purpose., Results: Alcohol intake during pregnancy and medical ionizing radiation exposure were the most significant predictors of HC. The mothers' alcohol consumption increased with the mothers' and fathers' education level, net family income and fathers' alcohol consumption. In contrast, maternal smoking decreased with increasing mothers' and fathers' education level and net family income. About 13% of the surveyed embryos were exposed to illegal drugs., Conclusions: Mild to moderate alcohol consumption diminishes the at-birth HC of theoretically healthy newborns in a linear form. There was no threshold dose. We perceived a need for increasing the awareness, and for training, of health care professionals and parents, in regard to risks of alcohol consumption and for recommending abstinence of these substances in both parents during pregnancy. It should also be remembered that medical ionizing radiation should be performed only during the first half of the cycle in fertile women. We think that our study has an important social impact as it affords data for implementing policies for promoting "healthy pregnancies".

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.

Aim: This study aims to report a patient with Chiari type 1 malformation (CM1) occurring in the context of pseudohypoparathyroidism type 1a (PHP-Ia) that we believe represents the first instance of this association in the current literature., Case Report: The authors describe the case of a 6-year-old girl diagnosed with PHP-Ia who presented an associated tonsillar descent. During the follow-up, the skull vault and the occipital squama became extremely thickened at the same time as the tonsillar herniation showed a marked regression., Discussion: Chronic tonsillar descent has been reported in diverse genetic and metabolic diseases of bone. A constant finding in PH-Ia consists of changes that mainly involve the bones of the patients' hands and feet. Cerebral anomalies have also been documented in PHP-Ia, especially cerebral calcifications, but in contrast involvement of the skull bones has seldom been described in this condition. The authors briefly discuss the probable role played by the observed skull changes in the origin and subsequent regression of the tonsillar descent in this child., Conclusions: We suggest that CM1 may develop in patients with PHP-Ia and that it should be actively sought, especially in individuals diagnosed with PHP-Ia presenting with neurological manifestations. Probably, the seeming rarity of chronic tonsillar descent in PHP-Ia is due to the fact that many patients with this condition are rarely investigated with magnetic resonance.

Aims: 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., Methods: 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., Results: 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., Conclusions: 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.

Aim: 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., Methods: 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., Results and Discussion: 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., Conclusions: 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 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.

Background: 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., Patients and Methods: 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., Aims: 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., Results: 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., Conclusions: 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.

Background: The standard procedure for the diagnosis of central nervous system (CNS) infections consists of cerebrospinal fluid (CSF) sampling, which is usually accomplished by a lumbar puncture. However, in some patients presenting with acute hydrocephalus submitted to immediate CSF drainage, the fluid is customarily obtained from the placed draining system. In addition, the CSF obtained from the ventricular and lumbar spaces in some cases may show unusual differences, both in physiological and pathological conditions., Illustrative Cases: We report two children who presented with confounding results in the initial studies of their ventricular and lumbar CSF who were subsequently diagnosed with tuberculous meningitis, causing delay in diagnosis and treatment. AIM. By reporting these cases, we wanted to alert the treating physician about the possibility of this discrepancy to avoid the delayed diagnosis and management of the affected patients., Discussion: We comment on the possible pathophysiological mechanisms that may result in this dissociation in ventricular and lumbar CSF composition. CONCLUSIONS; Normal results in CSF studies, especially those of the ventricular fluid, do not always rule out the presence of tuberculous meningitis. We suggest obtaining a CSF sample from the lumbar subarachnoid space in doubtful, or suspicious, cases of CNS infection even in the presence of a normal ventricular CSF.

Objective: To report preliminary experience in using Microporous Polysaccharide Hemospheres (MPH; Medafor, Inc, Minneapolis, Minnesota, USA) for cerebral and dural sinus hemostasis., Methods: Absorbable hemospheres for hemostasis were used in 10 patients (6 men, 4 women, mean age 56.2 years) undergoing cerebral procedures. The indication was corticosubcortical cerebral hemostasis after resection of meningiomas (n = 5) and gliomas (n = 5). In one case, absorbable hemospheres were applied for generalized oozing over the superior sagittal sinus. The surgical technique, time to bleeding control, and associated complications were recorded., Results: Effective hemostasis, defined as cessation of oozing bleeding, was achieved no later than 2 minutes after topical agent application in all patients except two, in whom the hemostatic application was repeated. Mean follow-up was 12 months. No patient developed allergic reactions or systemic complications in association with hemostatic absorbable hemospheres. There was no case of cerebral hematoma, swelling, or infection after surgery., Conclusions: In this preliminary study, the direct application of absorbable hemospheres helped to control superficial cerebral bleeding, reducing the use of bipolar coagulation and shortening surgical time. Although use of absorbable hemospheres seems to be safe and effective, further investigations and prospective studies with longer follow-up are strongly recommended to arrive at final conclusions., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Unlabelled: BACKGROUND AND CASE REPORT: Many surgical procedures require a rigid immobilization of the patients' head, which is usually achieved by using a pin-type head holder. We briefly illustrate the case of a 4-year-old girl who sustained a depressed skull fracture by penetration of a pin of the head holder. The fracture was noted at the end of the surgery performed for treatment of a cerebellar astrocytoma and was managed conservatively., Discussion: Several factors seem to be involved in the production of this complication as are faulty application of the pins, excessive pin pressure, skull thinning, young patient's age, and pathological conditions that evolve with long-standing raised intracranial pressure. Prevention and management measures are briefly discussed.

Purpose: 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., Methods: 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., Results: 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., Conclusions: 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 definite diagnosis of hydrocephalus valve infection is generally made by cerebrospinal fluid (CSF) sampling via the valve reservoir, which is considered to be more dependable than that of the CSF obtained by lumbar puncture., Case Report: We treated a 17-year-old boy with an intra-abdominal pseudocyst due to ventriculoperitoneal shunt infection caused by Staphylococcus warneri whose ventricular CSF, obtained via the valve reservoir, was repeatedly sterile thus causing a considerable delay in the management of the complication., Discussion and Conclusions: S. warneri constitutes an emergent contaminant of catheters and prostheses. We found only a detailed report of S. warneri infection of a ventriculoatrial shunt. If manifestations of peritoneal involvement in shunted patients would occur, the attention should be shifted to the distal component of the shunt hardware, even in the presence of a normal ventricular CSF as happened in our case to avoid unnecessary delay in diagnosis and management.

Dural arteriovenous fistulae (DAVFs) are infrequent lesions, the most common locations of which are the cavernous, sigmoid and transverse sinuses. The cribiform plate is one of the less frequent sites for DAVFs, where they entail a high hemorrhage risk. Feeding arteries for ethmoidal DAVFs can be uni- or bilateral. However, the draining fistulous system has classically been described as unilateral. The authors report the second case in literature of bilateral ethmoidal DAVF, which is defined as that with bilateral draining veins. The present case was diagnosed only after surgical exploration of both cribiform plates. No preoperative radiological test could detect the presence of a bilateral venous draining system from the ethmoidal DAVF. Possible reasons for that lack of presurgical diagnosis are discussed. Bilateral surgical exploration of the anterior cranial fossa is recommended when dealing with ethmoidal DAVFs, even when they seem to be unilateral on preoperative studies., (Copyright © 2010 Elsevier B.V. All rights reserved.)

Background: Shunt malfunction typically presents with headaches, vomiting, and impaired consciousness. Paraparesis has been rarely reported as the initial manifestation of valve dysfunction., Case Illustration: A 17-year-old boy was admitted with invalidating quadriparesis that after neuroimaging evaluation was found to be produced by communicating syringomyelia attributed to shunt malfunction., Discussion and Conclusion: Persistence of the central canal in association with communicating hydrocephalus may lead to the development of communicating syringomyelia. In this context, insufficient drainage of CSF produced by shunt dysfunction may evolve exclusively with symptoms and signs of spinal cord involvement in the absence of the usual cerebral manifestations related to shunt failure.

We report an original method for implanting bone marrow stem cells within the spinal cord parenchyma. This method was used for the experimental treatment of patients diagnosed with amyotrophic lateral sclerosis. The methodology is reproducible and devoid of major complications even in patients showing significant spinal atrophy. Therefore, this report describes a surgical procedure that could be used in other experimental treatments involving the intraspinal delivery of stem cells., (Copyright (c) 2010 Elsevier B.V. All rights reserved.)

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.

Background: Embryonal central nervous system (CNS) tumors are currently classified into three types: medulloblastoma, atypical rhabdoid/teratoid tumors, and primitive neuroectodermal tumor (PNET). A distinctive subtype of PNET called "embryonal tumor with abundant neuropil and true rosettes" (ETANTR) was reported in 2000., Discussion: ETANTR is a recently described variety of PNET that combines microscopic features of neuroblastoma and ependymoblastoma, demonstrating areas of fine fibrillary neuropil intermingled with cellular zones and ependymoblastic rosettes. It has been suggested that this neoplasm should be considered as a separate entity. ETANTR is an eminently pediatric tumor that has been reported exclusively in children younger than 4 years., Illustrative Cases: A 9-month-old girl underwent subtotal resection of a brainstem neoplasm. A 23-month-old girl was submitted to surgery for a frontoparietal tumor. In both instances, the histopathological diagnosis confirmed ETANTR. Both children were treated with chemotherapy and one with radiotherapy. Death in both patients occurred after a mean period of 10 months., Conclusions: By reporting these two new instances of ETANTR, we want to contribute to the knowledge of this highly malignant CNS embryonal neoplasm that occurs only in young children, given its present lethal prognosis, the scarcity of reported cases, and the lack of treatment guidelines.

A 2-month-old girl developed meningitis, ventriculitis and brain abscess in the course of Citrobacter koseri infection. She was successfully treated with the combined use of antibiotics, intra-cavitary urokinase and surgery, thus avoiding the development of hydrocephalus and of ventricular loculation. C. koseri is a Gram-negative pathogen with a strong predilection for the neonatal brain. Brain abscesses develop in roughly 77% of cases, causing severe neurological sequels in one-half and death in one-third of patients. The authors aim to report the role of neurosurgical treatment for managing the severe complications that may arise in the course of C. koseri brain infection and the use of urokinase for preventing the development of loculated hydrocephalus., (Copyright (c) 2009 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.)

Background: Mass lesions arising in the scalp or the cranial vault of children require a significant diagnostic work-up but their nature, in most instances, can only be clarified after histopathological study. Tumors of osseous consistency or merging from within cranial bones are seldom susceptible of fine needle aspiration biopsy and require excision. Ossified cephalhematoma is a rare entity that is usually related to perinatal or accidental trauma. Exceptionally, ossified cephalhematoma has a non-traumatic origin and arises from a different condition, as vascular tumor or malformation., Discussion: Cavernomas of the skull are infrequent and occur in middle-aged individuals. Skull cavernomas in infants lack the sunburst radiographic pattern seen in adults and may present as a bone-like tumor whose appearance is identical to that of ossified cephalhematoma. A literature search showed only two previous reports of patients younger than 1 year with skull cavernomas presenting with associated ossified cephalhematomas. We hypothesize that this type of skull cavernomas are congenital in origin and that they manifest precociously due to the occurrence of early bleeding., Case Illustration: We report a 12-month-old boy presenting with a hard tumor arising on his right parietal bone that was present since birth. After diagnostic work-up, the bone tumor and the associated intraosseous hematoma were removed. Interestingly, the source of the hematoma was a cavernous hemangioma originating in the skull., Conclusions: Calvarial cavernous hemangiomas may bleed precociously, even before birth, and manifest as large intraosseous hematomas. Differential diagnosis against ossified cephalhematoma can only be established through histopathological study.

The objective of this study was to analyze the association of cerebellar tonsillar descent and syringomyelia in patients with posterior fossa arachnoid cysts. We reviewed the medical records of ten patients (mean, age 33; range, 24-49 years) diagnosed with posterior fossa arachnoid cyst and tonsillar descent. Symptoms evolved over a mean of 12 months (range, 6 months to 3 years). Syringomyelia was present in six cases. Six patients underwent a suboccipital craniectomy, three cases underwent an additional C1 laminectomy, and a further case had a limited craniectomy and tonsillar reduction. Three patients were also treated for hydrocephalus: one with a ventriculoperitoneal shunt and two with endoscopic third ventriculostomy. Two patients had conservative treatment. The posterior fossa arachnoid cysts were located at the vermis-cisterna magna (n = 4), the cerebellar hemispheres (n = 2), the cerebellopontine angle (n = 3), and the quadrigeminal cistern (n = 1). A patient with achondroplasia showed features of platybasia. Associated malformations included craniofacial dysmorphism in a patient diagnosed of trichorhinophalangeal syndrome and a case with a primary temporal arachnoid cyst. After a mean follow-up of 2 years (range, 3 months to 5 years), four patients showed resolution of their neurological symptoms, and two exhibited persisting ocular findings. Headaches and nuchalgia improved in four cases and persisted in four. Syringomyelia was resolved in four patients and improved in two. Patients harboring a posterior fossa arachnoid cyst may evolve with acquired Chiari malformation and syringomyelia. Initial management should be directed to decompressing the foramen magnum and should include the resection of the arachnoid cyst's walls. A wait-and-see attitude can be implemented in selected cases. In our experience, hydrocephalus should be properly addressed before treating the arachnoid cyst.

Object: The aim of this study was to evaluate whether clinical improvement is noticeable after a minimally invasive procedure such as that used with the Aperius PercLID System in patients with degenerative lumbar spinal stenosis (DLSS) and neurogenic intermittent claudication (NIC)., Methods: The patients were treated with the aforementioned system at 3 different centers. The initial requirement to be included in the study was a minimum follow-up of 12 months. The authors studied 40 cases of DLSS in patients with NIC (age 72.7 +/- 8.08 years). Symptom severity, physical function, quality of life, and self-rated pain were assessed preoperatively and at the 12-month follow-up using the Zurich Claudication Questionnaire (ZCQ) and a visual analog scale. The procedure was conducted under spinal (35 patients) or local (5 patients) anesthesia, using biplanar fluoroscopy for visualization., Results: Single-level treatment was performed in 28 patients and 2-level treatment was performed in 12 patients. Based on time recordings in 24 cases, the mean procedural time was 19.9 +/- 5.0 minutes. The mean pain visual analog scale score improved significantly from 8.1 +/- 2.19 at baseline to 3.44 +/- 2.89 at the 1-year follow-up. The ZCQ score for patient satisfaction showed 90% of the patients being satisfied with the procedure. The mean rates of improvement in ZCQ score for symptom severity and physical function at 1 year were 38.7 +/- 33.3% and 33.8 +/- 29.7%, respectively, and both proved to be statistically significant. Most improvement was seen in mobility, pain/discomfort, and ability for self-care., Conclusions: In this preliminary study, the Aperius system provided clinically significant improvement after 1 year of follow-up in patients older than 65 years with DLSS and NIC.

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.

Background: Patients operated on for myelomeningocele (MMC) and lipomeningocele (LMC) can suffer from late functional worsening that, in many cases, is due to spinal cord tethering by the post-repair scarring process., Illustrative Cases: In this case-based update, we report two patients operated on for MMC and LMC, respectively, who presented the clinical manifestations of spinal cord tethering, which we attributed to severe foreign body reactions to the materials used at their primary back surgery. In the first case, the cause of the tethering was an intense fibrotic scar around the silk suture used at the initial MMC repair, while in the second one, it was due to a fibrotic mesh containing the lyophilized dural graft implanted for LMC surgery. After a search of the current literature, we were unable to find cases of spinal cord tethering related to excessive scarring after dural repair with foreign materials used for the surgical correction of spinal dysraphism., Discussion and Conclusions: The pathogenesis of spinal cord tethering in our instances closely relate with the histopathologically observed intense foreign body reaction around the materials used at the primary back surgery. We recommend avoiding as much as possible the use of foreign materials during the initial repair of spinal dysraphism.

Background: Originally described by Dahlin et al. in 1984, fibrocartilaginous mesenchymoma (FCM) constitutes a rare bone tumor of children and adolescents that mainly affects the long bones. A spinal location of this tumor is exceptional. Only two previous instances of vertebral FCM have been reported in the current literature, and both occurred in young adults., Report of the Case: We report the case of a 9-year-old boy with backache caused by a neoplasm that involved the Th12 vertebra treated with tumor excision. Histopathological diagnosis was confirmatory of FCM. During a follow-up period of 2 years, there was no evidence of disease progression. Our patient, thus, represents the first case of FCM occurring in a child., Conclusions: Given the rarity of spinal FCM, there are no guidelines about its management. However, treatment of this neoplasm seems to be mainly surgical aiming at total removal of the lesion, if feasible, as FCM may recur locally.

Background: 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., Discussion: 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., Illustrative Cases: 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., Conclusions: 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.

Massive hemorrhage is a very uncommon event among hemangioblastomas. Forty-four cases have been reported before this review. Thorough analysis of all reported cases on literature was accomplished. The majority presented as parenchymatous or subarachnoid bleedings. Subarachnoid hemorrhage was only associated with spinal hemangioblastomas, while parenchymatous bleedings were more, but not only, originated from cranial instances. Ventricular hemorrhage from a hemangioblastoma was exceptional, with only two previous cases bleeding directly into the ventricular compartment. To our knowledge, the illustrative case is the first one of pure tetraventricular hemorrhage from a hemangioblastoma. When hemangioblastoma bleeding occurs, it is usually the very first clinical presentation of a previously undetected tumor. Solid type, large size, and spinal-radicular locations are more frequently related to hemorrhage. Hemorrhage following hemangioblastoma embolization and the association of this tumor with other bleeding lesions, such as arteriovenous malformations and aneurysms, is also discussed.

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., (Copyright © 2011 S. Karger AG, Basel.)

Background: 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., Discussion: 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., Case Report: 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., Conclusions: 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.

Background: Cerebrospinal fluid (CSF) overdrainage in hydrocephalus is well recognized. Overshunting following cysto-peritoneal (CP) drainage in patients with arachnoid cysts (AC) is rarely documented., Aim: 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., Patients and Methods: The medical records of the eight patients were analyzed in regard to clinical manifestation, cyst and shunt characteristics, management, and outcomes., Results: 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., Discussion: 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., Conclusions: 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.

Background: Intra-ventricular haemorrhage (IVH) can occur spontaneously or during the surgical revision of ventricular cerebrospinal fluid (CSF) shunts., Aim: 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., Patients and Methods: 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., Results: Follow-up evaluation of the six patients demonstrated that the existing VP shunts were functioning appropriately and that the treatment was safe., Conclusions: 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.

Introduction: Several emerging clinical and neuroimaging conditions due to CSF intracranial hypotension have been documented. Recently, a few instances of a severe form of cervical myelopathy due to CSF overshunting have also been reported. Patients with this type of cervical myelopathy usually evolve with marked clinical manifestations of spinal cord involvement., Materials and Methods: In this article, we report a 20-year-old girl, previously given a ventriculoperitoneal shunt at age 6 years, who presented with neck pain after a whiplash injury., Results and Discussion: Magnetic resonance imaging revealed a cervical extramedullary mass that mimicked a cervical extradural hematoma. Further neuroimaging studies demonstrated that it corresponded to an engorged cervical epidural venous plexus that we attributed to chronic CSF overdrainage. After expectant management, the patient experienced a total recovery of her symptoms. To our knowledge, this is the first report of this condition occurring in an asymptomatic patient. Our aim in reporting this patient is twofold: (1) to warn the clinician about these neuroimaging findings that may mimic a cervical extradural hematoma and (2) to illustrate that engorged cervical epidural plexus may occur in patients in the absence of clinical manifestations of myelopathy. Recognition of this entity is important to distinguish it from a mass lesion in which a surgical intervention might be indicated.