Mario Francesco Fraioli, Filiberto Contratti and Chiara Fraioli
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.
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.
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.
Thomas Roujeau, Guilherme Machado, Matthew R. Garnett, Catherine Miquel, Stephanie Puget, Birgit Geoerger, Jacques Grill, Nathalie Boddaert, Federico Di Rocco, Michel Zerah and Christian Sainte-Rose
Empirical radiotherapy is the current treatment for children with diffuse pontine lesions that have imaging characteristics of an infiltrative malignant astrocytoma. The use of chemotherapeutic agents is, however, currently under investigation in the treatment of these tumors. To be included into a trial, patients need a definitive histological diagnosis. The authors present their prospective study of the stereotactic biopsy of these lesions during a 4-year period.
A suboccipital, transcerebellar approach was used to obtain biopsy samples in 24 children.
Two patients suffered deficits. Both had a transient (< 2 months) new cranial nerve palsy; one of these patients also experienced an exacerbation of a preoperative hemiparesis. No patient died during the perioperative period. A histological diagnosis was made in all 24 patients as follows: 22 had a malignant infiltrative astrocytoma, one had a low-grade astrocytoma, and one had a pilocytic astrocytoma. The diagnosis of the latter two patients affected the initial treatment after the biopsy.
The findings of this study imply that stereotactic biopsy sampling of a diffuse pontine tumor is a safe procedure, is associated with minimal morbidity, and has a high diagnostic yield. A nonmalignant tumor was identified in two of the 24 patients in whom the imaging findings were characteristic of a malignant infiltrative astrocytoma. With the advent of new treatment protocols, stereotactic biopsy sampling, which would allow specific tumor characterization of diffuse pontine lesions, may become standard.
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.
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.