Pseudotumor cerebri in kaposiform lymphangiomatosis: a case report and pathogenetic hypothesis.

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.

Basic cerebrospinal fluid flow patterns in ventricular catheters prototypes.

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.

Intracranial hemorrhage following surgery for occult spinal dysraphism: a case-based update.

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.

Accidental head trauma during care activities in the first year of life: a neurosurgical comparative study.

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.

Wind instruments and headaches.

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.

Severe spinal cord injury in craniocervical dislocation. Case-based update.

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.

"Triple R" tonsillar technique for the management of adult Chiari I malformation: surgical note.

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.

Pediatric diseases in Juan Carreño de Miranda's paintings.

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.

Portraying disease: Sorolla's Sad Legacy.

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.

Pediatric head injuries from earthquakes.

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.

Pediatric crushing head injury: biomechanics and clinical features of an uncommon type of craniocerebral trauma.

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.

Head circumference at birth and exposure to tobacco, alcohol and illegal drugs during early pregnancy.

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".

Depressed skull fracture by a three-pin head holder: a case illustration.

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.

Hydrocephalus and arachnoid cysts.

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.

Spinal dermal sinus and pseudo-dermal sinus tracts: two different entities.

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.

Chiari type 1 anomaly in pseudohypoparathyroidism type Ia: pathogenetic hypothesis.

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.

Space-time clustering in childhood nervous system tumors in the Region of Murcia, Spain, 1998-2009.

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.

Staphylococcus warneri ventriculoperitoneal shunt infection: failure of diagnosis by ventricular CSF sampling.

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.