Bradycardia Without Hypertension: Is It a Common Clinical Presentation of Ventriculoperitoneal Shunt Malfunction in Pediatric Patients?

BACKGROUND: Mechanical shunting of cerebrospinal fluid is an effective treatment for hydrocephalus. Some studies suggest that bradycardia without hypertension may also be observed in ventriculoperitoneal (VP) shunt malfunction; however, in our experience, this is not a common presenting sign. OBJECTIVE: The aim of this study was to evaluate whether bradycardia without hypertension was a common sign in patients presenting to the pediatric emergency department (ED) with a VP shunt malfunction. METHODS: A retrospective observational study, from May 2006 to April 2015, which included a random sample of children admitted to the ED with clinical features suggestive of possible VP shunt malfunction. Control patients were defined as those who arrived at our ED with suspected VP shunt malfunction that was later ruled out on further workup. RESULTS: A total of 65 patients were included in this study. A significantly greater number of patients with a confirmed shunt pathology presented with vomiting (P = 0.01) and lethargy/apathy (P = 0.01). In the control group, a significantly greater number of patients presented with fever (P = 0.004) and seizures (P = 0.02). The number of patients presenting with bradycardia was not significantly different between the shunt pathology and control groups (P > 0.05). CONCLUSIONS: Bradycardia is not a common presentation in patients with VP shunt malfunction. Bradycardia is often recognized as a significant sign; however, it is one of the last presenting signs. Educating patients about the early signs must be considered as part of the treatment for VP shunt malfunction.

Pediatric perimedullary arteriovenous fistula of the conus medullaris supplied by the artery of Desproges-Gotteron.

The cone artery, or artery of Desproges-Gotteron, is sometimes seen arising from the internal iliac artery. The authors describe a case of a symptomatic perimedullary arteriovenous fistula (AVF) of the conus medullaris in an 8-year-old boy who presented with a protracted history of urinary difficulty and severe sudden-onset right lumbosciatic pain that evolved to severe paraparesis with compromise of the sphincter muscles. The spinal AVF, which was supplied by the cone artery and a thoracic radiculomedullary artery that joined at the fistula site in a large partially thrombosed varix, was completely occluded with Onyx liquid embolic. The patient's clinical condition improved rapidly after embolization. As shown in this patient, urgent endovascular embolization of spinal AVFs can be very rewarding, even in patients with severe neurological presentation. The artery of Desproges-Gotteron appears to be a rare arterial variation. To the authors' knowledge, this is the first pediatric case of a conal AVF supplied by this artery.

Solitary juvenile xanthogranuloma mimicking intracranial tumor in children.

Juvenile xanthogranuloma (JXG) is primarily a benign cutaneous disorder of non-Langerhans hystiocytic proliferation. Systemic involvement occurs in 4% of patients; isolated central nervous system (CNS) lesions are rare. We report solitary CNS-JXG lesions in two patients. A 3.5-year-old boy with a parietal-occipital lesion underwent total resection with no surgical morbidity and no recurrence at 16-month follow-up. A 3.5-year-old girl underwent subtotal resection of a tumor extending from the left Meckel's cave and invading the cavernous sinus and left orbit with extensive cranial nerve involvement. Tumor regrowth with leptomeningeal spread at 9-month and 12-month follow-up was managed with steroids and chemotherapy (vinblastine and later cladribine). We present our experience and review the literature pertaining to rare reports of solitary CNS-JXG.

Surgical implications of the thickened pituitary stalk accompanied by central diabetes insipidus.

OBJECT: The authors discuss the indications for and timing of a diagnostic neurosurgical procedure in children with diabetes insipidus (DI) and a thickened pituitary stalk (TPS) on magnetic resonance (MR) imaging. METHODS: Seven children with a TPS who presented with DI eventually underwent surgery for diagnostic purposes. The ages at onset of DI were 6 to 16 years, and the follow-up period until surgery was 26.9 +/- 11.9 months. In four of seven children, the stalk appeared normal on the first MR image, but it was thickened and variably enhancing on later images in all instances. The reason for eventual surgery was endocrinological deterioration in two of seven children, radiological progression in two children, and a combination of the two in three children. Three children experienced visual disturbances and four children had optic nerve, chiasma, or hypothalamus involvement. All children suffered additional endocrinological abnormalities pursuant to the initial DI. A definitive diagnosis was achieved in six of seven children: germinomas in five and Langerhans cell histiocytosis in one. One child had lymphocytic infiltrate. None of the children deteriorated neurologically or endocrinologically after the operation. On follow up, vision deficit was irreversible in all children who demonstrated visual abnormalities before treatment. CONCLUSIONS: Surgery should be performed in children with a TPS and DI for early diagnosis and disease-oriented therapy when there is further endocrinological, radiological, or clinical deterioration. The complication rate is low in open biopsies, and histological diagnosis is achieved in most of the cases. All children who present with central DI must undergo head MR imaging, and even if results are normal, close radiological and clinical follow up is mandatory.

Epidermal growth factor receptor gene amplification and expression in disseminated pediatric low-grade gliomas.

OBJECT: Pediatric low-grade gliomas (LGGs) are the largest group of central nervous system neoplasms in children. Although these tumors are generally benign, 5 to 10% of patients with pediatric LGGs present with leptomeningeal dissemination. The genetic and biological nature of these tumors is poorly understood. The authors looked for certain molecular abnormalities that may differentiate disseminated gliomas from the other pediatric LGGs. METHODS: Comparative genomic hybridization (CGH) was applied to 18 pediatric LGGs. Six cases featuring disseminated pediatric LGGs were compared with 12 control cases involving nondisseminated pediatric LGGs. Fluorescence in situ hybridization (FISH) analysis and immunohistochemical analysis were used to highlight further specific genetic targets. The CGH revealed multiple chromosomal abnormalities in five of six cases with disseminated gliomas and in six of 12 control cases. No correlation was found between the number of chromosomal abnormalities and dissemination status. Amplification of chromosome 7 was noted in four of six cases with disseminated gliomas as opposed to one of 12 control cases (p = 0.02). The FISH analysis revealed epidermal growth factor receptor (EGFR) amplification in one case negative to chromosome 7 amplification by CGH, raising the amplification cases to five of six (p = 0.0038). Immunohistochemical analysis for EGFR was positive in six of six cases and in two of 12 control cases (p = 0.0015). At the end of a mean follow-up period of 7.2 years, all patients with disseminated gliomas are alive with variable but slow disease progression. CONCLUSIONS: The high rate of EGFR gene amplification and protein expression in disseminated pediatric LGGs is intriguing and may have implications for our understanding of the role of EGFR in glioma genesis. Targeted therapies may be available for these children. Larger-scale studies are needed to establish further these findings.

Skull base approaches in the pediatric population.

BACKGROUND: This study aimed to examine the surgical, oncologic, and developmental results of infants and children undergoing extirpation of skull base tumors. METHODS: Sixty-seven children aged 0.5 to 18 years (mean, 11 years) who were operated on during a 6-year period made up the study cohort. Eighteen cases (27%) involved malignant tumors, and 49 (73%) involved benign tumors. The most common benign tumors were craniopharyngioma (n = 10) and juvenile nasopharyngeal angiofibroma (n = 8). The most common malignant tumor was sarcoma (n = 5). Thirty-six tumors (55%) involved the anterior skull base, and the rest involved the lateral (n = 24) and posterior (n = 7) skull base. Subcranial, transfacial, and subfrontal approaches were used for extirpation of anterior skull base tumors. Voluminous or malignant tumors were excised by use of combined approaches (subcranial-transfacial, subcranial-degloving, or pterional-degloving). Twenty-two children underwent adjuvant therapy (chemotherapy, radiation, or both). Postoperative follow-up was 3 to 60 months. RESULTS: No severe postoperative complications (ie, meningitis, cerebrospinal fluid leak, tension pneumocephalus) and no perioperative mortality occurred. Two and a half years later, 54 of the children (80%) are alive and well. Five children, two with optic glioma and one each with squamous cell carcinoma, ependymoma, and germinoma, have died of their disease. The subcranial approach had no cosmetic impact on the craniofacial development of the patients. CONCLUSION: The extirpation of skull base tumors by use of conventional surgical techniques is feasible and safe among infants and children. The complication and mortality rates are lower than those in adults. The long-term cosmetic effect of the subcranial approach is negligible.

Low-dose aspirin prophylaxis and risk of intracranial hemorrhage in patients older than 60 years of age with mild or moderate head injury: a prospective study.

OBJECT: The goal of this paper was to investigate a possible relationship between the consumption of low-dose aspirin (LDA) and traumatic intracranial hemorrhage in an attempt to determine whether older patients receiving prophylactic LDA require special treatment following an incidence of mild-to-moderate head trauma. METHODS: Two hundred thirty-one patients older than 60 years of age, who arrived at the emergency department with a mild or moderate head injury (Glasgow Coma Scale [GCS] Scores 13-15 and 9-12, respectively), were included in the study. One hundred ten patients were receiving prophylactic LDA (100 mg/day) and these formed the aspirin-treated group. One hundred twenty-one patients were receiving no aspirin, and these formed the control group. There was no statistically significant difference between the two groups with respect to age, sex, mechanism of trauma, or GCS score on arrival at the emergency department. Most of the patients sustained the head injury from falls (88.2% of patients in the aspirin-treated group and 85.1% of patients in the control group), and had external signs of head trauma such as bruising or scalp laceration (80.9% of patients in the aspirin-treated group and 86.8% of patients in the control group). All patients underwent similar neurological examinations and computerized tomography (CT) scanning of the head. The CT scans revealed evidence of traumatic intracranial hemorrhage in 27 (24.5%) patients in the aspirin-treated group and in 31 patients (25.6%) in the control group. Surgical intervention was required for five patients in each group (4.5% of patients in the aspirin-treated group and 4.1% of patients in the control group). A surprising number of the patients who arrived with GCS Score 15 were found to have traumatic intracranial hemorrhage, as revealed by CT scanning (11.5% of patients in the aspirin-treated group and 16.5% of patients in the control group). Surgery, however, was not necessary for any of these patients. CONCLUSIONS: There was no statistically significant difference in the frequency or types of traumatic intracranial hemorrhage between patients who had received aspirin prophylaxis and those who had not. The authors conclude that LDA does not increase surgically relevant parenchymal or meningeal bleeding following moderate and minor head injury in patients older than 60 years of age.

Surgical treatment of spasticity by selective posterior rhizotomy: 30 years experience.

BACKGROUND: Spasticity is a common neurologic disorder with adverse effects on the patient's function. Conservative management is unsuccessful in a significant proportion of patients and neurosurgical intervention should be considered. The mainstay of surgical treatment of spasticity is selective posterior rhizotomy, i.e., section of sensory nerve roots of the cauda equina. OBJECTIVE: To report our experience with selective posterior rhizotomy in the treatment of spasticity. METHODS: We retrospectively reviewed our experience in 154 patients who underwent SPR during 30 years. The indication for surgery was spasticity that significantly hindered the patient's function or care and was resistant to conservative treatment. All patients were evaluated for spasticity in the lower and upper limbs, the presence or absence of painful spasms, and sphincter disturbances. The decision as to which roots to be sectioned, and to what extent, was based mainly on clinical muscle testing. RESULTS: Reduction of spasticity in the lower limbs was obtained in every case, with improvement in movements in 86% of cases. Painful spasms were alleviated in 80% of cases. Amelioration of neurogenic bladder was observed in 42%. A minority of the patients also showed improvement in speech and cognitive performance. There was no perioperative mortality or major complications. CONCLUSION: SPR is a safe and effective method for the treatment of spasticity with long-lasting beneficial effects. We suggest that this method be considered more frequently for patients with spasticity that interferes with their quality of life.

Survey of critical care management of severe traumatic head injury in Israel.

OBJECTIVE: To survey the current monitoring and treatment policies for patients with severe traumatic brain injury (STBI) in Israel, and to compare the management of pediatric and adult intensive care units (ICUs). DESIGN: Questionnaires were sent to the medical directors of all ICUs managing STBI patients. RESULTS: All 21 ICUs responded to the questionnaire. All of the units were within tertiary hospitals. An intracranial pressure (ICP) monitoring device was used in over 75% of the patients in 6 out of7 (86%) of the pediatric intensive care units (PICUs), compared with 11 out of 14 (79%) of the adults ICUs. Mannitol was used in all of the units for documented elevated ICP. Mild hyperventilation (4-4.6 kPa) was applied in 52% of the units. Mild hypothermia was routinely used in 4 out of 7 (57%) and hypertonic solutions (NaCI 3%) in 3 out of 7 (43%) of the PICUs, compared with only 2 out of 14 (14%) and none (0%) of the adults ICUs respectively. PICUs aimed for a lower ICP (< or =15 mm Hg) and cerebral perfusion pressure (> or =50 mm Hg) than adult ICUs (< or =20 mm Hg and > or =60 mmHg respectively). Barbiturates were used only in patients with refractory intracranial hypertension. CONCLUSION: This survey reveals a relatively high degree of homogeneity in the treatment of STBI patients in Israel. Most patients are treated in accordance with recently published literature. We attribute this uniformity to the fact that all patients are being treated within tertiary care, university-affiliated centers. PICUs are faster at implementing new modalities of treatment and tend to adopt more aggressive treatment strategies.