Mario Francesco Fraioli, Filiberto Contratti and Chiara Fraioli
Afroditi Despina Lalou, Marek Czosnyka, Joseph Donnelly, John D. Pickard, FMedSci, Eva Nabbanja, Nicole C. Keong, Matthew Garnett and Zofia H. Czosnyka
Normal pressure hydrocephalus is not simply the result of a disturbance in CSF circulation, but often includes cardiovascular comorbidity and abnormalities within the cerebral mantle. In this study, the authors have examined the relationship between the global autoregulation pressure reactivity index (PRx), the profile of disturbed CSF circulation and pressure-volume compensation, and their possible effects on outcome after surgery.
The authors studied a cohort of 131 patients in whom a clinical suspicion of normal pressure hydrocephalus was investigated. Parameters describing CSF compensation and circulation were calculated during the CSF infusion test, and PRx was calculated from CSF pressure and mean arterial blood pressure (MAP) recordings. A simple scale was used to mark the patients’ outcome 6 months after surgery (improvement, temporary improvement, and no improvement).
The PRx was negatively correlated with resistance to CSF outflow (R = −0.18; p = 0.044); patients with normal CSF circulation tended to have worse autoregulation. The correlation for patients who were surgically treated (n = 83) was R = −0.28; p = 0.01, and it was stronger in patients who experienced sustained improvement after surgery (n = 48, R = −0.43; p = 0.002). In patients who did not improve, the correlation was not significantly different from zero (n = 19, R = −0.07; p = 0.97). There was a trend toward higher values for PRx in nonresponders than in responders (0.16 ± 0.04 vs 0.09 ± 0.02, respectively; p = 0.061), associated with higher MAP values (107.2 ± 8.2 in nonresponders vs 89.5 ± 3.5 in responders; p = 0.195). The product of MAP × (1 + PRx), which was proposed as a measure of combined arterial hypertension and deranged autoregulation, showed a significant association with outcome (greater value in nonresponders; p = 0.013).
Autoregulation proves to associate with CSF circulation and appears strongest in shunt responders. Outcome following CSF diversion is possibly most favorable when CSF outflow resistance is increased and global cerebral autoregulation is intact, in combination with arterial normotension.
Adam M. H. Young, Mathew R. Guilfoyle, Helen Fernandes, Matthew R. Garnett, Shruti Agrawal and Peter J. Hutchinson
There is increasing interest in the use of predictive models of outcome in adult head injury. Two international models have been identified to be reliable modalities for predicting outcome: the Corticosteroid Randomisation After Significant Head Injury (CRASH) model, and the International Mission on Prognosis and Analysis of randomized Controlled Trials in TBI (IMPACT) model. However, these models are designed only to identify outcomes in adult populations.
A retrospective analysis was performed on pediatric patients with severe traumatic brain injury (TBI) admitted to the pediatric intensive care unit (PICU) of Addenbrooke's Hospital between January 2009 and December 2013. The individual risk of 14-day mortality was calculated using the CRASH-Basic and -CT models, and the risk of 6-month mortality calculated using the IMPACT-Core and -Extended (including CT findings) models. Model accuracy was determined by standardized mortality ratio (SMtR; observed/expected deaths), discrimination was evaluated as the area under the receiver operating curve (AUROC), and calibration assessed using the Hosmer-Lemeshow χ2 test.
Ninety-four patients with an average age of 7.3 years were admitted to the PICU with a TBI. The mortality rate was 12.7% at 14 days and at 6 months. For the CRASH-Basic model, the SMtR was 1.42 and both calibration (χ2 = 6.1, p = 0.64) and discrimination (AUROC = 0.92) were good. For the IMPACT-Core model, the SMtR was 1.03 and the model was also well calibrated (χ2 = 8.99, p = 0.34) and had good discrimination (AUROC = 0.85). Poor outcome was observed in 17% of the cohort and identified with the CRASH-Basic and IMPACT-Core models to varying degrees: standardized morbidity ratio = 0.89 vs 0.67, respectively; calibration = 6.5 (χ2) and 0.59 (p value) versus 8.52 (χ2) and 0.38 (p value), respectively; and discrimination (AUROC) = 0.92 versus 0.83, respectively.
Adult head injury models may be applied with sufficient accuracy to identify predictors of morbidity and mortality in pediatric TBI.
Federico Di Rocco, Matthew R. Garnett, Stephanie Puget, Francisco Pueyerredon, Thomas Roujeau, Francis Jaubert and Christian Sainte-Rose
✓Rosai–Dorfman disease (RDD) is a rare idiopathic histiocytic disorder that only occasionally involves the central nervous system (CNS). Previous cases of RDD involving the CNS were generally seen in adults. Pediatric cases of RDD are rare, and the disease in these cases typically has an indolent clinical course. In this report, the authors describe a pediatric case of intracranial RDD with rapid clinical and radiological progression.
A previously healthy 13-year-old girl presented with a 15-day history of progressive left-sided headaches, vomiting, and fever. On examination she was pyrexial but otherwise normal. Neuroimaging results demonstrated an extraaxial left frontal lesion with peripheral enhancement. A bur hole was drilled over the lesion to obtain a tissue sample and de-bulk the lesion. The initial histological results showed a nonspecific inflammatory lesion. Postoperatively, the patient was asymptomatic, and neuroimaging results confirmed a significant reduction in the size of the lesion. Repeated neuroimaging 3 months later, however, revealed a large recurrence of the lesion, which was removed macroscopically by a craniotomy. Histological analysis of the tissue confirmed the RDD diagnosis. At the latest follow-up (12 months) the patient had remained asymptomatic with no evidence of recurrence on neuroimaging. This is the first reported case of intracranial RDD with an aggressive clinical course.
Bertrand Baussard, Federico Di Rocco, Matthew R. Garnett, Nathalie Boddaert, Arielle Lellouch-Tubiana, Jacques Grill, Stephanie Puget, Thomas Roujeau, Michel Zerah and Christian Sainte-Rose
The aim of this study was to retrospectively review the clinical presentation, the roles of surgery and adjuvant therapy, and the treatment-related morbidity in children with a ganglioglioma in the posterior fossa and to try and determine the prognostic factors.
Between 1991 and 2006, 10 children were treated for a posterior fossa ganglioglioma at the authors' institution. The mean age of the children, the duration of symptoms prior to diagnosis, and the follow-up were 8.2, 2.4, and 3.9 years, respectively. Nine of the children presented with symptoms of raised intracranial pressure. Preoperative imaging showed enhancement in all patients; in eight it was in a patchy distribution. The operations consisted of radical resection (> 75%) in seven children, biopsy in two, and a total macroscopic excision in one.
The surgical procedure did not cause deterioration in the neurological condition in any of the children. There was no recurrence in the child who underwent total macroscopic excision of the tumor, and there has been no tumor progression in three children, two of whom have had no evidence of enhancement of the postoperative residual tumor. The tumor has progressed in six children, requiring further surgery in three, chemotherapy in four, and radiotherapy and second-line chemotherapy in one child to control the tumor.
The imaging of gangliogliomas in the posterior fossa showed patchy enhancement. The patients in whom it was possible to achieve a radical resection, aimed at removing at least the enhancing portion of the tumor, have not required further treatment. A second excision, for progressive tumors, is an effective adjuvant therapy.
Stéphanie Puget, Matthew Garnett, Alison Wray, Jacques Grill, Jean-Louis Habrand, Nathalie Bodaert, Michel Zerah, Mercia Bezerra, Dominique Renier, Alain Pierre-Kahn and Christian Sainte-Rose
The current treatment of craniopharyngiomas is evolving into one of a multimodal approach in which the aim is disease control and improved preservation of quality of life (QOL). To date, an appropriate classification system with which to individualize treatment is absent. The objectives of this study were to identify preoperative prognostic factors in patients with craniopharyngiomas and to develop a risk-based treatment algorithm.
The authors reviewed data obtained in a retrospective cohort of 66 children (mean age 7.4 years, mean follow-up period 7 years) who underwent resection between 1984 and 2001. Postoperative recurrence rates, vision status, and endocrine function were consistent with those reported in the literature. The postoperative morbidity was related to hypothalamic dysfunction. The preoperative magnetic resonance imaging grade, clinically assessed hypothalamic function, and the sugeon's operative experience (p = 0.007, p = 0.047, p = 0.035, respectively) significantly predicted poor outcome. Preoperative hypothalamic grading was used in a prospective cohort of 22 children (mean age 8 years, mean follow-up period 1.2 years) treated between 2002 and 2004 to stratify patients according to whether they underwent gross-total resection (GTR) (20%), complete resection avoiding the hypothalamus (40%), or subtotal resection (STR) (40%). In cases in which residual disease was present, the patient underwent radiotherapy. There have been no new cases of postoperative hyperphagia, morbid obesity, or behavioral dysfunction in this prospective cohort.
For many children with craniopharyngiomas, the cost of resection is hypothalamic dysfunction and a poor QOL. By using a preoperative classification system to grade hypothalamic involvement and stratify treatment, the authors were able to minimize devastating morbidity. This was achieved by identifying subgroups in which complete resection or STR, performed by an experienced craniopharyngioma surgeon and with postoperative radiotherapy when necessary, yielded better overall results than the traditional GTR.
Marie Bourgeois, Darach William Crimmins, Ricardo Santos De Oliveira, Alexis Arzimanoglou, Matthew Garnett, Thomas Roujeau, Federico Di Rocco and Christian Sainte-Rose
The authors sought to analyze the success rate of surgery in the management of medically intractable epilepsy in children with Sturge–Weber syndrome and to determine whether the extent and timing of surgery affected seizure and developmental outcomes.
The authors performed a retrospective review of 27 children who underwent surgery at their institution for medically resistant epilepsy, and they examined the outcomes with regard to epilepsy control and neuropsychological development.
Seventeen children (63%) experienced onset of their epilepsy when they were younger than 1 year of age. These patients were significantly more likely to have hemiparesis (p ≤ 0.001) and status epilepticus (p ≤ 0.001) and be developmentally delayed (p ≤ 0.025) than children whose epilepsy started later in life. Eight patients underwent a hemispherectomy (either anatomical or functional), and complete resolution of epilepsy was noted in all. Of the 19 patients in whom a focal resection was performed, 11 (58%) became seizure free. The 10 children in whom there was residual disease were more likely to have continuing epilepsy than the nine whose lesions were completely excised (p ≤ 0.05). Seventeen children exhibited improvement in their developmental status following surgery. This improvement was significantly affected by completeness of resection (p ≤ 0.05) and age at surgery (p ≤ 0.009). Seizure freedom per se was not affected by the timing of surgery.
Medically intractable epilepsy in children can be treated effectively by surgery. The degree of resection or disconnection of diseased tissue, but not patient age at the time of surgery, is an important factor in achieving epilepsy control. Early surgery is more likely to improve developmental outcome.
Thomas Santarius, Srikanth Dakoji, Fardad T. Afshari, Frances L. Raymond, Helen V. Firth, Helen M. Fernandes and Matthew R. Garnett
The authors report a case of an isolated schwannoma of left hypoglossal nerve in a 9-year-old girl. To the authors' knowledge, this is the first case report of hypoglossal nerve schwannoma in the pediatric population in the absence of neurofibromatosis Type 2.
The patient presented with a 2-month history of morning nausea and vomiting with occasional daytime headaches. Magnetic resonance imaging and subsequent CT scanning revealed a dumbbell tumor with a belly in the lower third of the posterior fossa and head underneath the left jugular foramen. Its neck protruded through an expanded hypoglossal canal. Although the lesion bore radiological characteristics of a hypoglossal schwannoma, the absence of hypoglossal palsy and the apparent lack of such tumors in the pediatric population the preoperative diagnosis was not certain.
The tumor was approached via a midline suboccipital craniotomy, and gross-total resection was achieved. Pathological examination confirmed the diagnosis of schwannoma. Blood and tumor tests for mutations in the NF2 gene were negative. Postoperative mild hypoglossal palsy recovered by the 3-month follow-up, and an MRI study obtained at 1 year did not show recurrence.
Stephanie Puget, Darach W. Crimmins, Matthew R. Garnett, Jacques Grill, Ricardo Oliveira, Nathalie Boddaert, Alison Wray, Arielle Lelouch-Tubiana, Thomas Roujeau, Federico Di Rocco, Michel Zerah and Christian Sainte-Rose
Two to five percent of pediatric brain tumors are located in the thalamus. The optimal management for these tumors remains unclear. The aim of this study was to determine whether clinical and neuroimaging features could guide treatment, and to what extent these features, together with histological diagnosis and treatment modalities, influenced survival.
The records of 69 children who presented with a thalamic tumor between 1989 and 2003 were retrospectively reviewed. Three groups of tumors were analyzed separately: 1) unilateral thalamic tumors (54 lesions); 2) thalamopeduncular tumors (six); and 3) bilateral thalamic tumors (nine).
In the patients in whom a unilateral thalamic tumor was diagnosed, 33 had an astrocytic tumor. Of the 54 patients, 32 had a low-grade and 22 had a high-grade tumor. The survival rate was significantly better for patients with the following characteristics: symptom duration longer than 2 months (p < 0.001), lesions with low-grade histological features (p = 0.003), and tumor excision greater than 90% at surgery (p = 0.04). The perioperative morbidity and mortality rates were 37 and 4%, respectively. Fifty-four percent of the patients in this group had a long-term and independent survival. The thalamopeduncular tumors were mostly pilocytic astrocytomas, which had a good prognosis following surgery. The bilateral thalamic tumors in this series were mainly low-grade astrocytic lesions, and more than half of the children attained long-term survival (mean follow-up duration 4.5 years).
The majority of tumors arising in the thalamus are astrocytic, of which less than half are high-grade lesions. Histological evaluations should be performed in all patients in whom resection is being considered for discrete lesions. Long-term survival is possible in patients with these tumors.
Report of 2 cases
Stéphanie Puget, Matthew R. Garnett, Delphine Leclercq, Graziella Pinto-Primard, Dinane Samara-Boustani, Christian Sainte-Rose, David Genevieve, Michel Polak, Pascale De Lonlay, Francis Brunelle, Alim Louis Benabid and Nathalie Boddaert
The association between hypothalamic dysfunction and obesity is well documented in both clinical and experimental models. The authors describe 2 children who developed obesity that could not be explained by endocrinological, genetic, or eating disorders. In both cases, cranial MR imaging revealed the typical appearance of a lipoma in the paramedian hypothalamus. In the absence of other etiologies, the authors hypothesized that in these 2 children obesity was caused by their hypothalamic lipomas. To the authors' knowledge, these are the first cases of hypothalamic lipomas likely to be causing obesity that have been described in children. These cases highlight the importance of performing cranial MR imaging in children with otherwise unexplained obesity.