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.

Migration of a ventriculoperitoneal shunt catheter through the processus vaginalis in infants: how serious inguinal hernia and hydrocele might be.

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.

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.

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.

Microdiscectomy with and without insertion of interspinous device for herniated disc at the L5-S1 level.

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.

"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.

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.

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.

Neurotrophic bone marrow cellular nests prevent spinal motoneuron degeneration in amyotrophic lateral sclerosis patients: a pilot safety study.

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.

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.

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".

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.

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.