Analysis of the potential risk of central intravenous lines and/or total parenteral nutrition with ventriculoatrial shunts.

OBJECT: The distal catheter of a ventriculoatrial (VA) cerebrospinal fluid shunt is potentially exposed to bacterial seeding from a subclavian central line. The risk of blood stream infections (BSIs) from central lines increases with administration of total parenteral nutrition (TPN). The potential risks of shunt malfunction or infection in patients with a VA shunt and a concurrent subclavian central line and/or TPN administration have not been studied. METHODS: A retrospective review of 49 pediatric patients with placement of a VA shunt was performed. Three outcome measures were studied: shunt malfunction, shunt infection, and bacteremia/fungemia requiring shunt removal. All outcomes were measured by 1 year after shunt insertion. We analyzed the following potential risk factors: age at shunt insertion, prior ventriculoperitoneal (VP) shunt, prior shunt infection, abdominal infection/necrotizing enterocolitis (NEC), concurrent subclavian central line, and administration of TPN. The association between each risk factor and outcome was evaluated using Fisher's exact test to generate the relative risk. Additionally, a logistic regression analysis was performed to evaluate the odds ratio of the outcomes to risk factors considering age as a covariate. RESULTS: The average age at shunt insertion was 6.3 ± 7.6 years. The most common diagnosis was posthemorrhagic hydrocephalus of prematurity (53.1 %). Fifteen patients (30.1 %) had a shunt malfunction within 1 year, 6 (12.2 %) had a shunt infection, and 3 (6.1 %) required removal of the shunt due to bacteremia/fungemia. The age at shunt insertion was not a statistically significant independent risk factor for any of the three outcomes. Prior shunt infection predicted an increased risk for both future shunt malfunction and infection in both the associative relative risk analysis and the age-dependent logistic regression analysis, although the correlation did not reach statistical significance. The presence of a subclavian central line or TPN administration did not statistically increase the risk over baseline for any of the outcomes in either analysis. CONCLUSIONS: The relatively small number of patients limits the power of the study. Considering this limitation, the data suggests that the presence of a concurrent subclavian central line or administration of TPN does not increase the risk of shunt malfunction or infection over the baseline of this high-risk cohort.

Parasagittal cranial fasciitis following infratemporal fossa rhabdomyosarcoma.

Cranial fasciitis is a rare lesion of young children characterized by proliferation of fibroblastic spindle cells. Most are scalp masses and are only rarely intracranial, where an association with radiation therapy is exceptional. We report a 32-month-old toddler with a facial rhabdomyosarcoma, diagnosed at 3 months of age, and treated with surgery, chemotherapy and brachytherapy. Brain MRI at 28 months revealed a large, left parasagittal, dural-based, T2 hyperintense and T1 hypointense enhancing mass with superior sagittal sinus compression and bony hyperostosis. The mass was completely resected during an open craniotomy. Histologically, the lesion was comprised of loosely and haphazardly arranged bland spindle cells embedded in a myxoid background. Thick hyalinized collagen bundles were especially prominent. The spindle cells reacted for vimentin but not SMA, myogenin, MyoD1 or EMA. A diagnosis of cranial fasciitis was rendered. The role of radiation therapy in the pathogenesis of intracranial cranial fasciitis is discussed.

Anaplastic astroblastoma presenting as massive, sudden-onset, intraparenchymal hemorrhage.

Astroblastoma is a rare primary glial tumor of children and young adults, typically located in the cerebral hemispheres and presenting as a well-circumscribed, nodular, often cystic enhancing mass. The existence of astroblastoma as a distinct clinicopathologic entity has long been debated but is recognized in the 2007 WHO classification of CNS tumors. The grading of these tumors remains unsettled. Currently, no grade has been established and there are no clearly defined diagnostic criteria for low- or high-grade tumors. Astroblastomas in general are thought to have a favorable outcome with low rates of recurrence following gross total resection. We report a case of astroblastoma with 'high-grade/anaplastic' histology in a 12-year-old, previously healthy girl. The patient experienced severe, sudden-onset headache, and quickly became comatose. Head CT showed a massive intraparenchymal hemorrhage in the right frontal lobe with surrounding edema. She underwent emergent posterior frontal craniotomy for decompression and hematoma evacuation. Pathologic examination revealed a sharply demarcated hemorrhagic heterogeneous glial tumor with focal papillary architecture, densely hyalinized blood vessels, and intensely GFAP-positive perivascular cells. The tumor showed unequivocal high-grade features including an elevated proliferative index. The diagnosis of anaplastic astroblastoma was rendered. The patient expired on postoperative day 30. This case illustrates the potential poor outcome of high-grade astroblastoma and highlights the morphologic heterogeneity of this rare neoplasm.

Chiari in the family: inheritance of the Chiari I malformation.

This report presents three families with Chiari malformation type I that became symptomatic during childhood: a mother and son; a set of monozygotic twins; and two half-siblings and their two maternal cousins. These children presented with various symptoms, including headache, stiff neck, and swallowing difficulty. A review of the relevant literature is presented, with an emphasis on familial examples and proposed inheritance. Less common presentations of Chiari malformation type I are discussed, as well as the possible pathogenesis of Chiari malformation type I and associated syringomyelia.

Efficacy of phosphorus-32 brachytherapy without external-beam radiation for long-term tumor control in patients with craniopharyngioma.

OBJECT Radioactive phosphorus-32 (P32) has been used as brachytherapy for craniopharyngiomas with the hope of providing local control of enlarging tumor cysts. Brachytherapy has commonly been used as an adjunct to the standard treatment of surgery and external-beam radiation (EBR). Historically, multimodal treatment, including EBR, has shown tumor control rates as high as 70% at 10 years after treatment. However, EBR is associated with significant long-term risks, including visual deficits, endocrine dysfunction, and cognitive decline. Theoretically, brachytherapy may provide focused local radiation that controls or shrinks a symptomatic cyst without exposing the patient to the risks of EBR. For this study, the authors reviewed their experiences with craniopharyngioma patients treated with P32 brachytherapy as the primary treatment without EBR. The authors reviewed these patients' records to evaluate whether this strategy effectively controls tumor growth, thus avoiding the need for further surgery or EBR. METHODS The authors performed a retrospective review of pediatric patients treated for craniopharyngioma between 1997 and 2004. This was the time period during which the authors' institution had a relatively high use of P32 for treatment of cystic craniopharyngioma. All patients who had surgery and injection of P32 without EBR were identified. The patient records were analyzed for complications, cyst control, need for further surgery, and need for future EBR. RESULTS Thirty-eight patients were treated for craniopharyngioma during the study period. Nine patients (23.7%) were identified who had surgery (resection or biopsy) with P32 brachytherapy but without initial EBR. These 9 patients represented the study group. For 1 patient (11.1%), there was a complication with the brachytherapy procedure. Five patients (55.5%) required subsequent surgery. Seven patients (77.7%) required subsequent EBR for tumor growth. The mean time between the injection of P32 and subsequent treatment was 1.67 ± 1.50 years (mean ± SD). CONCLUSIONS In this small but focused population, P32 treatment provided limited local control for cyst growth. Brachytherapy alone did not reliably avert the need for subsequent surgery or EBR.

Analysis of long-term (median 10.5 years) outcomes in children presenting with traumatic brain injury and an initial Glasgow Coma Scale score of 3 or 4.

OBJECT: Patients with traumatic brain injury (TBI) with low presenting Glasgow Coma Scale (GCS) scores have very high morbidity and mortality rates. Neurosurgeons may be faced with difficult decisions in managing the most severely injured (GCS scores of 3 or 4) patients. The situation may be considered hopeless, with little chance of a functional recovery. Long-term data are limited regarding the clinical outcome of children with severe head injury. The authors evaluate predictor variables and the clinical outcomes at discharge, 1 year, and long term (median 10.5 years) in a cohort of children with TBI presenting with postresuscitation GCS scores of 3 and 4. METHODS: A review of a prospectively collected trauma database was performed. Patients treated at Riley Hospital for Children (Indianapolis, Indiana) from 1988 to 2004 were reviewed. All children with initial GCS (modified for pediatric patients) scores of 3 or 4 were identified. Patients with a GCS score of 3 were compared with those with a GCS score of 4. The outcomes of all patients at the time of death or discharge and at 1-year and long-term follow-up were measured with a modified Glasgow Outcome Scale (GOS) that included a "normal" outcome. Long-term outcomes were evaluated by contacting surviving patients. Statistical "classification trees" were formed for survival and outcome, based on predictor variables. RESULTS: Sixty-seven patients with a GCS score of 3 or 4 were identified in a database of 1636 patients (4.1%). Three of the presenting factors differed between the GCS 3 patients (n = 44) and the GCS 4 patients (n = 23): presence of hypoxia, single seizure, and open basilar cisterns on CT scan. The clinical outcomes were statistically similar between the 2 groups. In total, 48 (71.6%) of 67 patients died, remained vegetative, or were severely disabled by 1 year. Eight patients (11.9%) were normal at 1 year. Ten of the 22 patients with long-term follow-up were either normal or had a GOS score of 5. Multiple clinical, historical, and radiological factors were analyzed for correlation with survival and clinical outcome. Classification trees were formed to stratify predictive factors. The pupillary response was the factor most predictive of both survival and outcome. Other factors that either positively or negatively correlated with survival included hypothermia, mechanism of injury (abuse), hypotension, major concurrent symptoms, and midline shift on CT scan. Other factors that either positively or negatively predicted long-term outcome included hypothermia, mechanism of injury, and the assessment of the fontanelle. CONCLUSIONS: In this cohort of 67 TBI patients with a presenting GCS score of 3 or 4, 56.6% died within 1 year. However, approximately 15% of patients had a good outcome at 10 or more years. Factors that correlated with survival and outcome included the pupillary response, hypothermia, and mechanism. The authors discuss factors that may help surgeons make critical decisions regarding their most serious pediatric trauma patients.

A pilot study of preirradiation chemotherapy and 1800 cGy craniospinal irradiation in young children with medulloblastoma.

PURPOSE: Craniospinal irradiation (CSI) is necessary in the treatment of medulloblastoma, although it results in significant long-term sequelae, particularly in young children. We prospectively evaluated the feasibility of giving preirradiation chemotherapy followed by 1800 cGy CSI to young children with localized medulloblastoma. METHODS AND MATERIALS: Between January 1993 and July 1997, 7 consecutive patients (age, 20-64 months) with M0 medulloblastoma were enrolled. After surgical resection, patients received 4 months of multiagent chemotherapy followed by 1800 cGy CSI and 5400 cGy to the posterior fossa. RESULTS: Median follow-up is 8.9 years. No patient developed progressive disease during chemotherapy. One patient developed widespread metastatic recurrence 2 months after completing radiation therapy and died. Two additional patients developed isolated frontal horn relapses 32 and 36 months after initial diagnosis and received further irradiation and chemotherapy. Both of these patients remain alive 7.1 and 3.6 years from the time of recurrence. Four of the six survivors have endocrine deficits. All of the survivors require special assistance in school. CONCLUSIONS: Craniospinal irradiation doses of 1800 cGy may not be adequate to prevent exoprimary recurrences. Despite the CSI dose reduction, neuroendocrine and neurocognitive sequelae are substantial.

Radiographic and clinical outcome of syringomyelia in patients treated for tethered cord syndrome without other significant imaging abnormalities.

OBJECT: The surgical management of patients with symptoms of tethered cord syndrome (TCS) who lack significant radiographic abnormalities is controversial. One potential MRI marker for TCS is a spinal cord syrinx or syringomyelia. Alternatively, a syrinx may be a benign and incidental finding. In this report the authors evaluated a highly selected cohort of patients with symptoms of TCS with minimal radiographic abnormalities other than syringomyelia. They analyzed clinical and radiographic outcomes after tethered cord release (TCR). METHODS: A retrospective review of data from 16 children who met the study inclusion criteria was performed. All patients had been surgically treated at Riley Hospital for Children in Indianapolis, Indiana, between 2006 and 2011. All children had clinical symptoms of TCS as well as available pre- and postoperative MRI data. RESULTS: The most common presentation (12 [75%] of 16 patients) was urinary dysfunction, defined as symptoms of urgency or incontinence with abnormal urodynamic studies. Clinical follow-up data were available in 11 of these 12 patients. All 11 had improvement in symptoms at an average follow-up of 17 months. Seven (87.5%) of 8 patients presenting with back or leg pain had improvement. Three patients had progressive scoliosis; 2 had stabilization of the curve or mild improvement, and 1 patient had worsening deformity. Radiographic follow-up data were obtained an average of 14.5 months after surgery. Twelve patients (75%) had stable syringomyelia after TCR. Four patients showed improvement, with 2 having complete radiographic resolution. CONCLUSIONS: Highly selected patients with symptoms of TCS did very well clinically. Patients with abnormal urodynamic studies, pain, and gait disturbances showed a high rate of symptomatic improvement. However, a smaller percentage of patients had radiographic improvement of the syrinx. Therefore, the authors suggest that the decision to perform TCR should be based on clinical symptoms in this population. Symptomatic improvement was not necessarily related to radiographic resolution of the syrinx.

Phosphorus-32 therapy for cystic craniopharyngiomas.

BACKGROUND AND PURPOSE: To examine control rates for predominantly cystic craniopharyngiomas treated with intracavitary phosphorus-32 (P-32). MATERIAL AND METHODS: 22 patients with predominantly cystic craniopharyngiomas were treated at Indiana University between October 1997 and December 2006. Nineteen patients with follow-up of at least 6 months were evaluated. The median patient age was 11 years, median cyst volume was 9 ml, a median dose of 300 Gy was prescribed to the cyst wall, and median follow-up was 62 months. RESULTS: Overall cyst control rate after the initial P-32 treatment was 67%. Complete tumor control after P-32 was 42%. Kaplan-Meier 1-, 3-, and 5-year initial freedom-from-progression rates were 68%, 49%, and 31%, respectively. Following salvage therapy, the Kaplan-Meier 1-, 3-, and 5-year ultimate freedom-from-progression rates were 95%, 95%, and 86%, respectively. All patients were alive at the last follow-up. Visual function was stable or improved in 81% when compared prior to P-32 therapy. Pituitary function remained stable in 74% of patients following P-32 therapy. CONCLUSIONS: Intracystic P-32 can be an effective and tolerable treatment for controlling cystic components of craniopharyngiomas as a primary treatment or after prior therapies, but frequently allows for progression of solid tumor components. Disease progression in the form of solid tumor progression, re-accumulation of cystic fluid, or development of new cysts may require further radiotherapy or surgical intervention for optimal long-term disease control.

Tumor bleeding from a de novo aneurysm associated with optic glioma.

Aneurysms in children are rare and potentially devastating lesions. The authors report the case of a 16-year-old girl with a complicated medical history related to a chiasmal glioma diagnosed at 18 months of age. She had previously received multiple modalities of radiation treatment, including external beam, proton therapy, and Gamma Knife. She presented with hemorrhage centered in the tumor and extending into the ventricular space. There was no subarachnoid blood. Magnetic resonance imaging demonstrated the hemorrhage and tumor anatomy. Magnetic resonance angiography revealed an aneurysm at the internal carotid artery bifurcation, but the lesion was more clearly delineated on CT angiography. A comparison MR imaging study obtained 6 months earlier, even in retrospect, did not show evidence of an aneurysm. This case illustrates the salient point that the clinician must search for vascular lesions in the patient with spontaneous "tumor bleeding," especially if that patient has risk factors for aneurysm formation. The authors also suggest that a CT angiogram is better at radiographically demonstrating an intratumoral aneurysm than an MR angiogram in this scenario.

Cyst-ventricle stent as primary or salvage treatment for posterior fossa arachnoid cysts.

OBJECT: The optimal treatment of symptomatic posterior fossa arachnoid cysts is controversial. Current options include open or endoscopic resection, fenestration, or cyst-peritoneal shunt placement. There are potential drawbacks with all options. Previous authors have described stenting a cyst into the ventricular system for supratentorial lesions. The current authors have used a similar strategy for posterior fossa cysts. METHODS: The authors performed a retrospective review of 79 consecutive patients (1993-2010) with surgically treated intracranial arachnoid cysts. RESULTS: The authors identified 3 patients who underwent placement of a stent from a posterior fossa arachnoid cyst to a supratentorial ventricle. In 2 patients the stent construct consisted of a catheter placed into a posterior fossa arachnoid cyst and connecting to a lateral ventricle catheter. Both patients underwent stent placement as a salvage procedure after failure of open surgical fenestration. In the third patient a single-catheter cyst-ventricle stent was stereotactically placed. All 3 patients improved clinically. Two patients remained asymptomatic, with radiographic stability in a follow-up period of 1 and 5 years, respectively. The third patient experienced initial symptom resolution with a demonstrable reduction of intracystic pressure. However, he developed recurrent headaches after 2 years. CONCLUSIONS: Posterior fossa cyst-ventricle stenting offers the benefits of ease of surgical technique and a low morbidity rate. It may also potentially reduce the incidence of shunt-related headaches by equalizing the pressure between the posterior fossa and the supratentorial compartments. While fenestration is considered the first-line therapy for most symptomatic arachnoid cysts, the authors consider cyst-ventricle stenting to be a valuable additional strategy in treating these rare and often difficult lesions.

Progression of cerebrospinal fluid cell count and differential over a treatment course of shunt infection.

OBJECT: The physiological reaction of CSF white blood cells (WBCs) over the course of treating a shunt infection is undefined. The authors speculated that the CSF WBC count varies with different infecting organisms in peak level and differential percentage of polymorphonuclear (PMN) leukocytes, lymphocytes, monocytes, and eosinophils. The authors hope to identify clinically useful trends in the progression of CSF WBCs by analyzing a large group of patients with successfully treated shunt infections. METHODS: The authors reviewed 105 successfully treated cases of shunt infections at Riley Hospital for Children. The study dates ranged from 2000 to 2004; this represented a period prior to the routine use of antibiotic-impregnated shunt catheters. They analyzed the following organisms: coagulase-negative staphylococci, Staphylococcus aureus, Propionibacterium acnes, Streptococcal species, and gram-negative organisms. The initial CSF sample at diagnosis was analyzed, as were levels over 14 days of treatment. Model fitting was performed to generate curves for the expected progression of the WBC counts and the differential PMN leukocytes, lymphocyte, monocyte, and eosinophil percentages. RESULTS: Gram-negative organisms resulted in a higher initial (p = 0.03) and peak WBC count with a greater differential of PMN leukocytes compared with other organisms. Propionibacterium acnes infections were associated with a significantly lower WBC count and PMN leukocytes percentage (p = 0.02) and higher eosinophil percentage (p = 0.002) than other organisms. The pattern progression of the CSF WBC count and differential percentages was consistent for all infections. There was an initial predominance of PMN leukocytes, followed by a delayed peak of lymphocytes, monocytes, and eosinophils over a 14-day course. All values trended toward zero over the treatment course. CONCLUSIONS: The initial and peak levels of CSF WBCs vary with the infecting organisms. The CSF cell counts showed a predictable pattern during the treatment of shunt infection. These trends may be useful to the physician in clinical decision making, although there is a wide range of variability.

Analysis of the risk of shunt failure or infection related to cerebrospinal fluid cell count, protein level, and glucose levels in low-birth-weight premature infants with posthemorrhagic hydrocephalus.

OBJECT: Premature, low-birth-weight infants with posthemorrhagic hydrocephalus have a high risk of shunt obstruction and infection. Established risk factors for shunt failure include grade of the hemorrhage and age at shunt insertion. There is anecdotal evidence that the amount of red blood cells or protein levels in the CSF may affect shunt performance. However, this has not been analyzed specifically for this cohort of high-risk patients. Therefore, the authors performed this study to examine whether any statistical relationship exists between the CSF constituents and the rate of shunt malfunction or infection in this population. METHODS: A retrospective cohort study was performed on premature infants born at Riley Hospital for Children from 2000 to 2009. Inclusion criteria were a CSF sample analyzed within 2 weeks prior to shunt insertion, low birth weight (< 1500 grams), prematurity (birth prior to 37 weeks estimated gestational age), and shunt insertion for posthemorrhagic hydrocephalus. Data points included the gestational age at birth and shunt insertion, weight at birth and shunt insertion, history of CNS infection prior to shunt insertion, shunt failure, shunt infection, and the levels of red blood cells, white blood cells, protein, and glucose in the CSF. Statistical analysis was performed to determine any association between shunt outcome and the CSF parameters. RESULTS: Fifty-eight patients met the study entry criteria. Ten patients (17.2%) had primary shunt failure within 3 months of insertion. Nine patients (15.5%) had shunt infection within 3 months. A previous CNS infection prior to shunt insertion was a statistical risk factor for shunt failure (p = 0.0290) but not for shunt infection. There was no statistical relationship between shunt malfunction or infection and the CSF levels of red blood cells, white blood cells, protein, or glucose before shunt insertion. CONCLUSIONS: Low-birth-weight premature infants with posthemorrhagic hydrocephalus have a high rate of shunt failure and infection. The authors did not find any association of shunt failure or infection with CSF cell count, protein level, or glucose level. Therefore, it may not be useful to base the timing of shunt insertion on CSF parameters.

Clear-cell ependymoma: a report of intracortical tumor with significant desmoplasia.

We report a case of clear-cell ependymoma in a 20-month-old girl who presented with seizures. The tumor had several uncommon features including a solid consistency, a purely intracortical location, and a remarkable degree of desmoplasia. Clinical, radiological, and pathological findings are presented. This case emphasizes the value of ultrastructural studies in pathological work-up of brain tumors.

Cerebrospinal fluid eosinophilia in children with ventricular shunts.

OBJECT: Eosinophils have been reported in children with cerebrospinal fluid (CSF) shunts. The goal of this study was to describe the risk factors, relationship to infection, and clinical significance of CSF eosinophilia in a large group of shunt-treated patients. METHODS: The authors performed a retrospective review of data obtained in all patients who underwent ventricular shunt placement or revision at the James Whitcomb Riley Hospital for Children between 2000 and 2004. RESULTS: Eosinophils were identified during a follow-up shunt evaluation in 93 (31%) of 300 patients after initial shunt placement. Eosinophilia was statistically related to CSF extravasation (p < 0.0001), shunt infection (p = 0.031), blood in CSF (p < 0.0001), younger age at shunt insertion (p = 0.030), and the diagnosis of posthemorrhagic hydrocephalus (p < 0.0001). Patients with CSF eosinophilia had a higher risk of subsequent shunt failure (p < 0.0001). Analysis was performed using data obtained in a cohort of patients with a total of 130 shunt infections. Cerebrospinal fluid eosinophils were identified in 118 infections (90.8%). The leukocytic and eosinophilic reactions were dependent on the infecting organism. Propionibacterium acnes had a statistically lower CSF leukocyte count but higher differential percentage of eosinophils than the other common pathogens. CONCLUSIONS: Cerebrospinal fluid eosinophilia is a relatively common finding in children with shunts. Patients with CSF eosinophilia had an increased risk of shunt malfunction in the present series. Eosinophilia is associated with infection, CSF extravasation, and blood in the CSF. Patients with P. acnes-induced shunt infections have higher eosinophil percentages than are found in infections associated with other common organisms. Therefore, in patients with eosinophilia, extended anaerobic culture studies should be performed with particular attention paid to searching for this pathogen.

Interaction of ventriculoperitoneal shunt and baclofen pump.

OBJECTIVE: The authors present three cases of children with shunted hydrocephalus and intrathecal delivery of baclofen via an implanted pump. Each case illustrates a potential interaction of these devices. MATERIALS AND METHODS: A review of the pertinent charts, operative notes, and discharge summaries was performed, along with examination of the pertinent literature. CONCLUSION: The first case illustrates that a change in the pharmacology or effect of intrathecal baclofen may be caused by a failure of a shunt affecting cerebrospinal fluid clearance. The second case illustrates that patients can have ventricular changes with any procedure that accesses the thecal space, even with a functioning shunt. The third case illustrates that ensuring that a patient's shunt is functional before implantation of a baclofen pump is imperative. As the potential pool of patients with both shunted hydrocephalus and need for intrathecal baclofen grows, clinicians should be aware of potential serious interactions.