Cerebral haemorrhage in the presence of primary childhood central nervous system vasculitis--a review.

PURPOSE: The aim of the study was to evaluate frequency and clinical relevance of haemorrhagic events associated with primary angiitis of the central nervous system in childhood (cPACNS), a rare but increasingly recognized disease with varying clinical presentations. METHOD: A systematic literature review from 1990 onwards was conducted to identify reported cases of cPACNS. RESULTS: A total of 110 paediatric patients met the inclusion criteria. The median age was 9.5 years. Seven children (7/110, 6.4 %) demonstrated cerebral haemorrhage. Death occurred only in children with cerebral haemorrhage (4/110, 3.6 %); both a sudden and prolonged course of disease was observed. CONCLUSION: PACNS is a rare disease and the occurrence of haemorrhage with this condition is even rarer; however, the risk of an unfavourable outcome under these circumstances seems to be increased. PACNS adds to the list of differential diagnoses of intracerebral haemorrhage in the paediatric population.

Endoscopic rhino-neurosurgical approach for non-adenomatous sellar and skull base lesions.

OBJECTIVE: Since endoscopic endonasal transsphenoidal surgery requires skills of both neurosurgeons and otorhinolaryngologists, and the nose is the primary corridor of approach, we favour the term `endoscopic rhino-neurosurgery` and report on our interdisciplinary experience treating non-adenomatous lesions with skull base extension. METHODS: Between 2004 and 2009, 58 patients with 21 different disease patterns underwent endoscopic rhino-neurosurgical procedures. Mean age was 39.9 years, 50% were female. Seven had undergone prior surgery. Clinically, 34.5% presented with visual field deficits and with nerve palsies. Preoperatively, 62.1% showed a normal pituitary function. RESULTS: Mean follow-up was 13.1 months. The surgical goal depended on type of lesion; the intended extent of resection was achieved in 81%. Recovery from visual field deficits occurred in 80%. Neither deteriorated nor new cranial nerve palsies were observed. A normal endocrinological function could be maintained in 94.4%. Permanent diabetes insipidus occurred in 7 patients. Surgical complications included cerebrospinal fluid (CSF) leaks in 6 patients and meningitis in 4. All complications were managed successfully. There was no surgery-related mortality. CONCLUSION: The endoscopic rhino-neurosurgical approach is applicable for a wide variety of lesions comprising sella and skull base. As our data prove, this technique can be performed with satisfying results in non-adenomatous lesions as well.

Factors influencing the complication rate of subduroperitoneal shunt placement for the treatment of subdural hematomas in infants.

OBJECT: In this study the authors' goal was to identify the complication rate of subduroperitoneal (SDP) shunts for the treatment of subdural hematomas (SDHs) in infants and to determine the influences on and predictive factors for these complications. METHODS: The authors present a case series spanning the years 1994 to 2003 and include a statistical analysis of 161 children 2 years of age or younger with SDH who were treated using a unilateral valveless SDP shunt. The patient history, characteristics, and treatment methods including prior therapies, neuroimaging findings, and clinical outcomes were measures of evaluation. Thirty-six children (22.4%) suffered complications related to SDP shunts: obstruction in 27 (16.8%), infections in eight (5%), disconnection in four (2.5%), migration in three (1.9%), wound complications (leakage and skin ulceration) in two (1.2%), and symptomatic subdural rebleeding in one (0.6%) necessitating bur hole evacuation. Seventeen children (10.6%) underwent placement of a second SDP shunt because of ipsilateral or contralateral persistent fluid collections, or premature shunt removal. With the exception of 12 patients (7.4%), shunt removal was performed systematically and resulted in the following minor complications in 30 children (18.6%): an adherent proximal catheter in 16 (9.9%), transient symptoms of intracranial hypertension in six (3.7%), subcutaneous cerebrospinal fluid accumulation in four (2.5%), local infections in three (1.9%), and hydrocephalus requiring placement of a ventriculoperitoneal shunt in one (0.6%). Status epilepticus at presentation and neuroimaging findings such as areas of hyperdensity on computed tomography (CT) scans representing fresh blood in the subdural fluid collections before shunt insertion and at follow up were predictors of shunt-related complications. Correlations were also discovered for the following CT findings: ischemic lesions before shunt treatment, cerebral atrophy and ventricular dilation during the last follow up, and residual medium to large collections before shunt removal. Children who attained a good outcome were less affected by shunt-related complications, unlike those who presented with focal deficits and/or visual impairment. CONCLUSIONS: Subduroperitoneal shunt placement for the treatment of SDH in infants is--despite the complication rate--an effective and often inevitable treatment option, especially for most large and symptomatic SDHs; a certain number of complications could be reduced with careful and precise surgical techniques. Close observation for detection of risks is mandatory, and seizure control is essential to prevent further brain injury that may result in large subdural fluid collections that are difficult to treat.

Lymphocytic hypophysitis in the pediatric population.

BACKGROUND: Lymphocytic hypophysitis (LYH) is a rare inflammatory disease of the pituitary gland that usually affects women in their ante- or immediate postpartum period; males are affected less frequently than females. An autoimmune pathogenesis is suggested. Symptoms comprise anterior and/or posterior pituitary insufficiency of varying degrees. So far, specific characteristics of this rare disease in childhood are not well described. CASE HISTORY: We report the case of a 12-year-and-11-month-old boy with histologically confirmed LYH clinically presenting with diabetes insipidus. A high-dose steroid therapy was administered, in which the therapeutic effect was uncertain. His 6-year follow-up is presented. DISCUSSION: The literature is reviewed for children presenting with LYH; their characteristics, pituitary involvement, and clinical follow-up are listed and discussed. CONCLUSION: Even though magnetic resonance imaging can be highly suspicious for LYH, only surgical exploration can confirm the diagnosis. The efficacy of medical treatment is still controversial; a close follow-up is necessary to control and correct the endocrinological function, if required.

Risk reduction of subdural collections following endoscopic third ventriculostomy.

OBJECT: To evaluate the efficacy and safety using fibrin glue and absorbable hemostats for packing the endoscopic tract in a pediatric population in the prevention of subdural fluid collections after endoscopic third ventriculostomy. MATERIALS AND METHODS: Twenty-one endoscopic third ventriculostomies were performed in 20 hydrocephalic children with a mean age of 22 months over a 4-year period using uniformly this technique. Six children, with age ranged 6 days to 22 months (mean 9 months), had severe ventriculomegaly with thin brain mantle less than 10 mm. There was no mortality and no permanent morbidity related to the endoscopic procedure. One child developed an asymptomatic collection, which could be managed conservatively and was most likely attributed to the young age of the child and the assumed poor cerebrospinal fluid absorption ability. Lost compliance of the brain parenchyma may play an additional role, which was not related to the thickness of the brain mantle. No adverse effects regarding the material used for sealing were observed over a mean follow-up of 23 months. CONCLUSIONS: Subdural fluid collections could be prevented in 20 out of 21 procedures by using this technique regardless of the thickness of the brain mantle, the application of mixture of fibrin glue and hemostatic agents seems to be safe, and we consider this technique effective in reducing the risk of this potential complication.

Chiari I malformation and intra-cranial hypertension:a case-based review.

OBJECTIVE: To present clinical and morphological findings before and after surgery in a child with Chairi I malformation (CMI) and intra-cranial hypertension (IH). The literature is reviewed and pathophysiologic factors are discussed. CLINICAL PRESENTATION: A 13-year-old obese boy with a 3-week history of headaches, neck pain, torticollis and progressive visual deterioration was admitted. Bi-lateral chronic papilloedema and decrease in visual acuity were found in the presence of a previously diagnosed CMI. INTERVENTION AND FOLLOW-UP: Intra-cranial pressure monitoring demonstrating increased pressure levels was followed by a sub-occipital decompression, C1 laminectomy and duroplasty. Post-operatively, the boy improved markedly, the 6 months follow-up opthalmological examination demonstrated resolution of papilloedema, but consecutive bi-lateral optic nerve atrophy. CONCLUSION: IH with progressive visual deterioration represents one of the varying clinical presentations of CMI and may be classified as a secondary form of idiopathic IH. Neuro-ophthalmological examination in all patients with CMI is recommended to identify the real incidence of this presentation. Altered CSF dynamics, venous hypertension and obesity as co-factors may be causative pathophysiologic factors.

Hyperbaric oxygen therapy for the treatment of brain abscess in children.

INTRODUCTION: The treatment of brain abscess remains a challenging topic usually involving a multimodal concept. METHODS: We report our experience with hyperbaric oxygen (HBO) therapy in five children presenting with brain abscesses between 1995 and 2002 at the Department of Neurosurgery, Graz. Mean age was 14.8 (range 11-17 years). All abscesses were located supratentorially. One child had a single abscess and one had multilocated abscesses. Two other patients presented with both subdural empyema and brain abscess, one of them showing an epidural empyema as well. In another child, the brain abscess was associated with meningoencephalitis and subdural empyema. In all of them the underlying condition was spread of infection from the paranasal sinuses, except for one, who was immunocompromised due to cytotoxic chemotherapy for acute lymphocytic leukaemia. RESULTS: One single brain abscess and one of the multiple abscesses were drained. All subdural/epidural empyemas were treated surgically. Antibiotics were administered intravenously for 13 to 22 days (mean 22 days). All patients underwent HBO therapy; the number of treatments ranged from 26 to 45 "dives" (mean 30). Treatments were given once daily at 2.2 atmosphere absolutes for 60 min at 12 m. During the hospital stay all improved their clinical condition, with continued regression of abnormalities on magnetic resonance imaging (MRI). In the following weeks, other interventions were performed to treat the origin of the infections. At 6 months follow-up they were all in good clinical condition, either symptom free or with minor residual symptoms. MRI at this time showed no evidence of disease in three, a residual dural enhancement in one and a residual shrunken collection in the child with multilocated abscesses. No recurrence was observed during a mean follow-up of 21 months (range from 7 to 72 months). CONCLUSION: HBO therapy in children with brain abscesses seems to be safe and effective, even when they are associated with subdural or epidural empyemas. It provides a helpful adjuvant tool in the usual multimodal treatment of cerebral infections and may reduce the intravenous course of antibiotics and, consequently, the duration of hospitalization. Multidisciplinary management is recommended to optimize care for these critically ill children.

Pleomorphic xanthoastrocytoma of the cerebellopontine angle in a child.

CASE REPORT: The case of a 6-year-old girl with a pleomorphic xanthoastrocytoma (PXA) in the left cerebellopontine angle (CPA) is reported. The clinical, radiological, and histopathological findings are presented. DISCUSSION: To our knowledge, the presence of PXA in the CPA has not previously been reported. The rare infratentorial locations of PXA (frequently seen in association with ganglioglioma) are reviewed, as well as the other infrequent locations of this tumor. This report adds to the list of differential diagnoses of CPA tumors in children.

Gamma knife radiosurgery for callosotomy in children with drug-resistant epilepsy.

OBJECTIVES: Gamma knife radiosurgery as a noninvasive procedure is increasingly used as a treatment option in patients with intractable seizures. We investigated efficacy and safety of gamma knife radiosurgery (GKRS) for callosotomy in children. MATERIALS AND METHODS: Between 2000 and 2004 three children between 4 and 14 years (mean 8 years) underwent radiosurgical callosotomy. One child presented with Lennox-Gastaut syndrome, two with hemispheric cortical dysplasia. These two children underwent functional hemispherotomy before GKRS. GKRS was performed with a marginal dose of 55-60 Gy on the 50% isodose. RESULTS: Mean follow-up was 35 months. Radiosurgical callosotomy was ineffective in one child with the Lennox-Gastaut syndrome, whereas in the remaining two children, a 100% seizure reduction of generalized tonic-clonic seizures, 20-70% reduction of partial seizures, and a progress in mental and physical development was achieved. No postradiosurgical side effects were observed in all children. CONCLUSION: Radiosurgical callosotomy might be offered after hemispherotomy to complete callosal resection. However, larger number of patients and longer follow-ups are needed to draw final conclusions.

CSF shunts in children: endoscopically-assisted placement of the distal catheter.

OBJECTIVES: Ventriculoperitoneal shunting is the most common treatment for hydrocephalus. Repeated shunt revisions and other previous surgical procedures can complicate the placement of the distal catheter. Occasionally, when conventional sites like the abdominal cavity and the right atrium are used up or unavailable, a ventriculopleural shunt can be inserted. MATERIALS AND METHODS: Between April 2001 and August 2002, 7 children ranging in age from 5 months to 17 years underwent ten endoscopically-assisted cerebrospinal fluid shunt insertions. All patients had had previous multiple shunt revisions and other abdominal operations. In addition 4 children had suffered from severe peritonitis, 1 of them with a subsequent adhesion ileus. In 9 cases the terminal portion of the shunt was inserted with laparoscopic assistance and in 1 case with thoracoscopic assistance. The latter had previously undergone two conventional intercostal thoracotomies for shunt insertions. Placement of the shunt was performed in a suitable area under direct laparoscopic or thoracoscopic vision by a peel-off needle into the peritoneal or pleural cavity. In 2 children repeated endoscopic approaches were necessary for shunt replacement due to infections. RESULTS: We used this procedure successfully in each case without operation-related complications. Neither new adhesions nor difficulties in laparoscopic replacement of the shunt were observed. CONCLUSION: Laparoscopic and thoracoscopic assistance in the placement of the distal catheter has the benefit of allowing a safe and precise insertion with visual control of shunt function, even in patients with prior surgical interventions. Additionally, this minimally invasive technique appears to prevent new adhesions arising in the peritoneal cavity.

Somatosensory evoked potentials in children with severe head trauma.

UNLABELLED: We evaluated the predictive value of somatosensory evoked potentials (SEP) in a series of children with severe traumatic brain injury (TBI). The prospective clinical investigation was performed in a Level I paediatric trauma centre. We included 26 consecutive comatose paediatric patients aged from 1 month to 17 years (median age 11 years) following severe TBI (initial Glasgow Coma Score (GCS) 8 or below). Besides SEP recordings, the intracranial pressure and the results of an initial cranial CT scan were filed. The Glasgow Outcome Scale (GOS) was used to assess outcome at discharge. Thirteen children had normal SEP measurements, three patients had abnormal SEP recordings and a cortical response was bilaterally absent in ten children. Out of 26 children, 10 died whereas two remained in a persistent vegetative state. Only one child suffered from significant neurological deficits (GOS 3) at discharge. Seven patients survived with a GOS of 4 and six children survived without neurological impairment (GOS 5). Normal SEP indicated a favourable outcome in most children but did not rule out the occurrence of death, while absence of SEP was related to unfavourable outcome in all cases. CONCLUSION: Measurement of somatosensory evoked potentials provides valuable data for determining the prognosis at early coma stages. Our data show that an unfavourable outcome can be predicted with higher precision than a favourable outcome.