Outcome in pediatric hydrocephalus: a comparison between previously used outcome measures and the hydrocephalus outcome questionnaire.

OBJECT: The objectives of this study were to assess, in a cohort of children with recently treated hydrocephalus, the correlation between scores on the Hydrocephalus Outcome Questionnaire (HOQ) and the children's type of schooling and motor functioning, and to assess the overall outcome of the children. METHODS: The health status of 142 pediatric patients (85 boys) with previous hydrocephalus, born between 1995 and 1999, was assessed. Outcomes were determined using the HOQ, type of schooling, and motor functioning. Data were obtained from parental interviews and patient medical records. RESULTS. Twelve patients died (8.5%). Responses to the HOQ were obtained from 107 patients (65 boys). The mean age of the patients was 7 years and 9 months +/- 1.42 years (range 6-10 years). The Physical Health score of the HOQ correlated well with the motor functioning score (r = 0.652) as did the Cognitive Health score with the type of schooling (r = 0.672). Fifty-nine percent of the patients were able to attend a school for students with normal intelligence. Disabling motor functioning was found in only 30% of patients. Epilepsy was present in 14%. CONCLUSIONS: The results show a good correlation between the type of schooling and the Cognitive HOQ score and between the Physical HOQ score and the motor functioning score. The HOQ is a simple and very useful measurement for determining outcome in pediatric hydrocephalus.

Ventriculoperitoneal shunt occlusion due to spontaneous intraabdominal knot formation in the catheter. Case report.

A male infant was born full term with a prenatally diagnosed lumbosacral meningomyelocele and dilated ventricles that required ventriculoperitoneal shunt treatment. Following shunt insertion, a dysfunction occurred and operative exploration of the distal catheter revealed a knot formation resulting in occlusion of the shunt lumen. After the knot was untangled, the symptoms of increased intracranial pressure diminished. The authors stress that any possible cause of mechanical shunt complication should be taken into account when shunt malfunction is expected.

Navigated laser-assisted endoscopic fenestration of a suprasellar arachnoid cyst in a 2-year-old child with bobble-head doll syndrome. Case report.

The authors present the case of a 2-year-old boy with bobble-head doll syndrome (BHDS) associated with a large suprasellar arachnoid cyst and enlarged ventricles, who was successfully treated with neuronavigated laser-assisted endoscopic ventriculocystocisternostomy. The clinical history, surgical treatment, and clinical follow up of the patient are described. A navigated laser-assisted endoscopic ventriculocystocisternostomy of the suprasellar arachnoid cyst led to cessation of the head bobbing, and notable reduction of the cyst and ventricles was visible on the postoperative magnetic resonance images. Caused by a suprasellar arachnoid cyst, BHDS can be successfully treated with navigated laser-assisted endoscopic ventriculocystocisternostomy. The advantages of this procedure are minimal invasiveness and facilitated guidance of the neuronavigation system to the target area when normal anatomical landmarks are not visible.

Robot-assisted thoracoscopic resection of a benign mediastinal neurogenic tumor: technical note.

OBJECTIVE: Robotic surgery systems were introduced recently with the objective of enhancing the dexterity and view during procedures that use a videoscope. The first case report of robot-assisted thoracoscopic removal of a benign neurogenic tumor in the thorax is presented. METHODS: A 46-year-old woman presented with a history of paravertebral pain. A chest x-ray revealed a left paravertebral mass. A magnetic resonance imaging scan revealed a well-encapsulated mass that was suspected to be a neuroma at the level of T8-T9, separate from vascular structures, without extension in the foramina, and without a spinal canal component. RESULTS: A left robot-assisted thoracoscopic resection of the tumor was performed. After placement of six trocars, the tumor was carefully dissected and removed through one of the trocar openings. The histopathological findings revealed an ancient schwannoma. CONCLUSION: This case report demonstrates the feasibility of robot-assisted thoracoscopic extirpation of a thoracic neurogenic tumor. Robot-assisted surgery may prove to be of additional value in challenging thoracoscopic surgery, such as the delicate surgical removal of benign neurogenic tumors, because of the support in manipulation and visualization during videoscopic interventions.

Treatment of hydrocephalus determined by the European Orbis Sigma Valve II survey: a multicenter prospective 5-year shunt survival study in children and adults in whom a flow-regulating shunt was used.

OBJECT: The goal of this study was to evaluate the long-term results of a flow-regulating shunt (Orbis Sigma Valve [OSV] II Smart Valve System; Integra NeuroSciences, Sophia Antipolis, France) in the treatment of hydrocephalus, whether it was a first insertion procedure or surgical revision of another type of shunt, in everyday clinical practice in a multicenter prospective study. METHODS: Patients of any age who had hydrocephalus underwent implantation of an OSV II system. The primary end point of the study was defined as any shunt-related surgery. The secondary end point was a mechanical complication (shunt obstruction, overdrainage, catheter misplacement, migration, or disconnection) or infection. The overall 5-year shunt survival rates and survival as it applied to different patient subgroups were assessed. Five hundred fifty-seven patients (48% of whom were adults and 52% of whom were children) were selected for OSV II shunt implantation; 196 patients reached the primary end point. Shunt obstruction occurred in 75 patients (13.5%), overdrainage in 10 patients (1.8%), and infection in 46 patients (8.2%). The probability of having experienced a shunt failure-free interval at 1 year was 71% and at 2 years it was 67%; thereafter the probability remained quite stable in following years (62% at the 5-year follow-up examination). No difference in shunt survival was observed between the overall pediatric (< or = 16 years of age) and adult populations. In the pediatric age group, however, there was a significantly lower rate of shunt survival in children younger than 6 months of age (55% at the 5-year follow-up examination). CONCLUSIONS: In this prospective study the authors demonstrate the effectiveness of flow regulation in the treatment of hydrocephalus both in children and in adults. Flow-regulating shunts limit the incidence of overdrainage and shunt-related complications. The overall 5-year shunt survival rate (62%) compares favorably with rates cited in other recently published series.

An 8-year-old boy with a Pott's puffy tumor.

An 8-year old boy with a history of trauma, sinusitis and a swelling of the frontal bone with somnolence was diagnosed with a Pott's puffy tumor (PPT). Minimal invasive surgical intervention was performed together with a strict regimen of antibiotic therapy. In this case debridement of the frontal bone was not necessary. Serial X-ray imaging of the skull showed complete ossification of the frontal bone lesion. Early diagnosis using thorough radiological evaluation is necessary to effectuate the proper therapeutic approach. For this reason, a patient with a forehead swelling and a history of trauma and/or sinusitis should be suspected for a PPT.

A bundle approach to reduce the incidence of external ventricular and lumbar drain-related infections.

OBJECT: An important complication of external CSF drainage is bacterial meningitis or ventriculitis, resulting in increased morbidity, mortality, and health care costs. In 2003, a high rate (37%) of probable drain-related infections was identified at the authors' hospital. A multidisciplinary working group was installed to reduce this incidence to < 10% within 1.5 years. METHODS: An intervention strategy based on 5 pillars (increased awareness, focused standard operating procedures, a diagnostic and therapeutic algorithm, timely administration of prophylaxis, and improvement of the drainage system) was designed and implemented from 2004 to 2006. During this period all patients with external CSF drainage were prospectively monitored. RESULTS: Between 2004 and 2006, there were 467 patients in whom 579 drains (external ventricular and external lumbar) had been placed. The overall incidence of drain-related infections was 16.2% in 2004, 8.9% in 2005, and 11.3% in 2006. For external lumbar drains the number of infections per 100 drain days was 2.4 in 2004, 0.6 in 2005, and 0.8 in 2006. For external ventricular drains these rates were 1.7, 1.0, and 1.2, respectively. Meanwhile, the causative noncutaneous microorganisms, indicative for systemic-contamination during manipulation, decreased. By retrospective analysis, the proportion of patients with a probable drain-related infection decreased from 37% in 2003 to 9% in 2005 and 2006. CONCLUSIONS: The authors' multidisciplinary approach in which different preventive measures were combined was associated with a significant reduction in the incidence of drain-related secondary meningitis, and thus provides an important improvement of patient safety.

New transcranial Doppler index in infants with hydrocephalus: transsystolic time in clinical practice.

Raised intracranial pressure (ICP) in infants with hydrocephalus may cause (ir)reversible damage to the brain parenchyma but can be present without clinical signs and/or symptoms. Therefore, new, favorably noninvasive, detection methods are needed to distinguish between compensated hydrocephalus with normal intracranial pressure and slowly progressive hydrocephalus with increased intracranial pressure. Because early ischemic changes in the brain parenchyma are associated with increased intracranial pressure, transcranial Doppler (TCD) indices may be useful to detect increased intracranial pressure in infants with hydrocephalus. Twenty-four infants with hydrocephalus underwent noninvasive ICP measurement, magnetic resonance imaging and TCD before and after cerebrospinal fluid (CSF) diversion. The TCD indices were paired to the anterior fontanelle pressure findings and compared for correlation. After CSF diversion, ICP decreased significantly from 21.8 cm H(2)O to 7.7 cm H(2)O (p<0.005). The transsystolic time (TST) as measured with TCD increased significantly from 176 to 221 ms (p<0.005), whereas the pulsatility index (PI) decreased significantly from 1.3 to 1.0 (p<0.05). The resistance index (RI) decreased significantly from 0.73 to 0.63 (p<0.05). Mean bloodflow velocity through the middle cerebral artery increased significantly from 55.5 to 75.8 cm/s (p<0.005). TST has a strong correlation with the ICP (p<0.005). Measuring TST with TCD can be helpful in the decision-making process about whether to perform CSF diversion in infants with hydrocephalus. Because TST is related solely to the relative changes in the flow velocity caused by intracranial physical properties, it has a closer relation to ICP than the PI and the RI.

Longitudinal diffusion-weighted imaging in infants with hydrocephalus: decrease in tissue water diffusion after cerebrospinal fluid diversion.

OBJECT: Progressive hydrocephalus may lead to edema of the periventricular white matter and to damage of the brain parenchyma because of compression, stretching, and ischemia. The aim of the present study was to investigate whether cerebral edema can be quantified using diffusion-weighted imaging in infants with hydrocephalus and whether CSF diversion could decrease cerebral edema. METHODS: Diffusion-weighted MR imaging was performed in 24 infants with progressive hydrocephalus before and after CSF diversion. Parametric images of the trace apparent diffusion coefficients (ADCs) were obtained. The ADCs of 5 different cortical and subcortical regions of interest were calculated pre- and postoperatively in each patient. The ADC values were compared with age-related normal values. Mean arterial blood pressure and anterior fontanel pressure were measured immediately after each MR imaging study. RESULTS: After CSF diversion, the mean ADC decreased from a preoperative value of 1209 +/- 116 x 10(-6) mm(2)/second to a postoperative value of 928 +/- 64 x 10(-6) mm(2)/second (p < 0.005). Differences between pre- and postoperative ADC values were most prominent in the periventricular white matter, supporting the existence of preoperative periventricular edema. Compared with age-related normal values, the preoperative ADC values were higher and the postoperative ADC values were lower, although within normal range. The decrease in ADC after CSF drainage was more rapid than the more gradual physiological decrease that is related to age. The preoperative ICP was elevated in all patients. After CSF diversion the ICP decreased significantly to within the normal range. A linear correlation between ADC values and ICP was found (correlation coefficient 0.496, p < 0.001). In all patients the mean arterial blood pressure was within physiological limits both pre- and postoperatively. CONCLUSIONS: This study shows a rapid and more extensive decrease in ADC values after CSF diversion than is to be expected from physiological ADC decrease solely due to increasing patient age. The preoperative ADC increase can be explained by interstitial edema caused by transependymal CSF leakage or by vasogenic edema caused by capillary compression and stretching of the brain parenchyma. This study population of infants with (early recognized) hydrocephalus did not suffer from cytotoxic edema. These findings may help to detect patients at risk for cerebral damage by differentiating between progressive and compensated hydrocephalus.

Nasal encephalocele in a child with Beckwith-Wiedemann syndrome.

Beckwith-Wiedemann syndrome (BWS) is a rare congenital syndrome characterized by gigantism, macroglossia, exophthalmos, postpartum hypoglycemia, and multiple midline defects such as omphalocele. The authors describe, to the best of their knowledge, the first case of a child in whom BWS was diagnosed and who was subsequently treated for a nasal encephalocele. Because the authors believe that this feature might not be an incidental finding in patients with BWS, intranasal masses in these patients should be carefully differentiated, as complications might be severe.

Laser-assisted endoscopic third ventriculostomy: long-term results in a series of 202 patients.

OBJECTIVE: Endoscopic third ventriculostomy is a well-known surgical option in the treatment of noncommunicating hydrocephalus. We studied complications and long-term success in 202 patients to demonstrate the safety and efficacy of laser-assisted endoscopic third ventriculostomy (LA-ETV) using a unique "black" fiber tip/diode laser combination for controlled tissue ablation. METHODS: We studied 213 LA-ETVs, which were performed in 202 patients. Patients' ages ranged from 2 days to 83 years (mean age, 27 yr). The mean follow-up period for all patients was 2.7 years (range, 2 d to 12 yr). Hydrocephalus was caused by aqueductal stenosis in 65 patients, tumors in 67 patients, hemorrhages in 24 patients, myelomeningoceles in 20 patients, cysts in 15 patients, and other causes in 11 patients. The long-term effectiveness of LA-ETV was studied with Kaplan-Meier analysis. RESULTS: Technically successful LA-ETVs were accomplished in 196 of the 202 patients (97%). The overall success rate for a functional LA-ETV was 68% at the 2-year follow-up evaluation. LA-ETV was more effective in patients aged 1 year and older (70% success rate) than in younger patients (59% success rate). Success rates were greater in patients with aqueductal stenosis or tumors as compared to other etiologies. Complications occurred in 22 procedures (10.3%). Only one patient (0.5%) experienced a major complication. No surgical mortalities or laser-related complications occurred. CONCLUSION: This study demonstrates that LA-ETV is a safe and effective procedure that is comparable to other techniques for ETV. LA-ETV is most effective in patients aged 1 year and older and in patients with aqueductal stenosis and tumors, with a low major complication rate.

Postoperative hemicerebellar inflammation mimicking recurrent tumor after resection of a medulloblastoma. Case report.

The authors present the case of a 4-year-old boy in whom a medulloblastoma in the left cerebellar hemisphere was successfully resected with no signs of residual tumor on the postoperative magnetic resonance (MR) images. A second MR imaging study performed 1 month after surgery demonstrated an extensive, contrast-enhancing lesion in the left cerebellar hemisphere, which simulated massive recurrent tumor, and repeated surgery was considered. A third postoperative MR imaging study, performed for evaluation of the craniospinal axis 10 days after the second postoperative study, still showed some contrast enhancement in the left cerebellar hemisphere, but the lesion had almost disappeared. Postoperative hemicerebellar inflammation seemed to be the most likely explanation. This case illustrates that early postoperative inflammation can mimic recurrent tumor on MR images obtained after resection of a medulloblastoma and caution should be taken in interpreting such images. Clinical history, neurological examination, laboratory findings, and repeated MR imaging studies can be helpful in evaluating the patient accurately.

Magnetic resonance imaging for quantitative flow measurement in infants with hydrocephalus: a prospective study.

OBJECT: Raised intracranial pressure (ICP) that is associated with hydrocephalus may lead to alterations in cerebral hemodynamics and ischemic changes in the brain. In infants with hydrocephalus, defining the right moment for surgical intervention based on clinical signs alone can sometimes be a difficult task. Clinical signs of raised ICP are known to be unreliable and sometimes even misleading. Furthermore, when sutures are closed, ICP does not always correlate with the size of the ventricles or with the clinical signs or symptoms. In this study the authors investigated whether cerebral blood flow (CBF) can be measured by using quantitative MR angiography in infants with progressive hydrocephalus. In addition, the authors investigated the relationship between CBF and ICP, before and after cerebrospinal fluid (CSF) diversion. METHODS: Fifteen infants with progressive hydrocephalus (age range 1 day-7 months) were examined. All patients underwent anterior fontanel pressure measurement, MR angiography, and mean arterial blood pressure measurements before and after CSF diversion. Brain volume was measured to compensate for the physiological increase in CBF during brain maturation in infants. RESULTS: The mean preoperative ICP was 19.1 +/- 8.4 cm H(2)O (+/- standard deviation). The mean postoperative ICP was 6.7 +/- 4.0 cm H(2)O (p < 0.005). The mean preoperative CBF was 25.7 +/- 11.3 ml/100 cm(3) brain/min. After CSF diversion, CBF increased to 50.1 +/- 12.1 ml/100 cm(3) brain/min (p < 0.005). The mean arterial blood pressure did not change after surgical intervention. CONCLUSIONS: Magnetic resonance imaging can be used to measure CBF in infants with hydrocephalus. Raised ICP was related to a decrease in CBF. After CSF diversion, CBF and ICP improved to values within the normal range.

Redislocation in a halo vest of an atlanto-occipital dislocation in a child: recommendations for treatment.

STUDY DESIGN: A case of a child with a traumatic atlanto-occipital dislocation (AOD), with unexpected secondary redislocation in a halo vest is presented. An extensive literature review was performed. OBJECTIVES: To show the difficulties in diagnosing AOD, to emphasize its intrinsic instability, and to make recommendations for treatment of this condition. SUMMARY OF BACKGROUND DATA: AOD is a rare condition that is difficult to diagnose. Because of the high neurologic morbidity, this injury often results in death. Different diagnostic approaches have been evaluated throughout the literature. Also, there are different opinions on how to treat AOD. There is no large trial that compares conservative treatment with surgery. METHODS: A 4-year-old child with a longitudinal distraction of the head is described. Because of his tenuous medical condition, initial treatment was in a halo vest. However, redislocation occurred inside the vest, so surgical stabilization was performed as soon as the patient's condition allowed. RESULTS: To diagnose AOD, different measuring techniques can be applied on a lateral cervical spine radiograph. Using a combination of these tools, the sensitivity of the examination can be increased. There are 3 types of dislocation. Type II, which is a longitudinal distraction, is most unstable. Once confirmed, AOD should be immobilized. This can temporarily be performed with a halo-frame, but our case shows that this does not necessarily provide a stable situation, and early surgical reconstruction should be considered. CONCLUSIONS: AOD can be an extremely unstable lesion and is difficult to diagnose. Although initial treatment in a halo vest is recommended, redislocation can occur even in the vest. We recommend urgent surgical stabilization.