✓ The authors report a case of infra- and supratentorial intracerebral hemorrhage complicating the postoperative course of a patient who had undergone surgical removal of a cervical schwannoma with an hourglass configuration. To their knowledge, this is the first case in which this neurosurgical procedure was followed by such a complication. Possible mechanisms are discussed; however, pathological events leading to this complication are unclear. The development of new neurological deficits not attributable to the surgical procedure should suggest this possibility.
Xavier Morandi, Laurent Riffaud, Beatrice Carsin-Nicol, and Yvon Guegan
Aurore Bussat, Maia Proisy, Bertrand Bruneau, Guillaume Bouzillé, Céline Chappé, and Laurent Riffaud
Tumor-related edema of the optic tract (EOT) corresponds to a preferential posterior distribution of peritumoral edema along the white matter tract of the visual system. To date, the consequences of EOT have never been evaluated specifically in the pediatric population. In this study, the authors attempted to identify clinical and radiological features associated with the development of EOT and the specific influence of this edema on visual function.
A retrospective review was performed of data collected from patients younger than 18 years who underwent surgery for a tumor in the sellar region at the authors’ institution between January 2005 and January 2016. Data were collected on patient characteristics, ophthalmological evaluations, and neuroimaging findings. To evaluate and compare visual function impairment, ophthalmological data were converted to a global visual function score, which took into account visual acuity, visual field evaluations, and laterality deficiencies. The visual acuity score was defined according to the International Classification of Diseases, 10th Revision. Visual field deficiencies were converted to a score of 0–2. Two opposing groups were then distinguished according to the presence or absence of EOT. Visual acuity, visual field results, and global scores were compared between groups before and after treatment.
Twenty-six patients were included in the study: 17 patients with craniopharyngioma, 3 patients with pilocytic astrocytoma, 2 patients with ganglioglioma, 2 patients with germ cell tumor, 1 patient with macroprolactinoma, and 1 patient with Rathke’s cleft cyst. There were 11 children in the group with edema and 15 children in the group without edema. None of the following criteria were statistically different between the 2 groups: age, sex, clinical symptoms at presentation (endocrine deficiency or intracranial hypertension signs), incidence of hydrocephalus, compression of the optic tracts and mass effect on the optic chiasm, tumor size and localization, presence of intratumoral cysts, treatment, type of tumor, or recurrence. The median global visual function and visual acuity scores were not significantly different between the groups either at presentation or at final evaluation. The visual field score was lower (i.e., more deficiency) in the group with edema than in the group without edema (p < 0.05); 89% of the patients with edema had severe or mild visual field impairment versus only 40% of the patients without edema. At the final examination after treatment, the visual field scores were not different between the 2 groups. Although not significant, the number of patients with optic disc pallor was greater in the group without edema both at diagnosis and at final examination.
This study confirms that EOT in the context of sellar region tumor in children is not necessarily associated with a less-favorable visual prognosis.
Xavier Morandi, Benoit Godey, Laurent Riffaud, Nathalie Heresbach, and Gilles Brassier
Claire Haegelen, Laurent Riffaud, Marc Bernard, Beatrice Carsin-Nicol, and Xavier Morandi
✓The authors describe the case of a 72-year-old woman with dural plasmacytoma revealing an immunoglobulin (Ig) G-kappa multiple myeloma (MM). She presented with headaches and left hemiparesis. Magnetic resonance imaging demonstrated a right frontal extraaxial lesion arising from the dura mater, and biological studies revealed hypercalcemia, hyperproteinemia, and a serum gamma globulin peak. A diagnosis of IgG-kappa MM was based on microscopic examination and immunohistochemical analysis of the dural plasmacytoma as well as on signs of systemic myeloma after surgery. The patient died 3 years after the first symptoms of MM despite systemic chemotherapy and no recurrence of the dural plasmacytoma.
Myelomatous involvement of the dura mater is a rare occurrence given that only three cases have been reported to date. Nevertheless, this pathological entity should be differentiated from solitary dural plasmacytoma (SDP) because the prognosis is radically different. Progression seems to be correlated with systemic disease in contrast to the long-term survival associated with SDP. Careful systemic evaluation should be made in such a presentation to rule out MM, which would require different management and has a different prognosis.
Laurent Riffaud, Stephan Saikali, Emmanuelle Leray, Abderrahmane Hamlat, Claire Haegelen, Elodie Vauleon, and Thierry Lesimple
In this article, the authors report their experience in the management of adult patients with medulloblastoma at their institution to identify prognostic factors important for survival and disease control.
Between 1977 and 2005, 27 patients who were ≥ 16 years old and had medulloblastoma were treated consecutively. There were 16 women and 11 men with a median age of 21 years (range 16–54 years). Gross-total resection was performed in 21 patients, subtotal (≥ 90%) in 2, incomplete in 1, and biopsy in 3 patients. Six patients had the desmoplastic variant, and 21 patients presented with classic medulloblastoma. Staging according to the Chang classification showed 4 patients with tumors invading the brainstem (2 with Stage T3b and 2 with Stage T4), 3 patients with metastases (2 with Stage M2 and 1 with Stage M3), and 1 patient in whom the stage was unknown (Stage MX) who died 10 days postoperatively. Twenty patients were assigned to the standard-risk group and 7 to the high-risk group. All patients except the one whose status was classified as Stage MX underwent craniospinal radiotherapy at our institution. Seven patients received chemotherapy before radiotherapy.
The 5- and 10-year overall survival rates for the present study were 81 and 62%, respectively. The median overall survival time was 17.7 years. The 5- and 10-year event-free survival rates were 72 and 57%, respectively. The median event-free survival time was 17.9 years. Univariate analysis showed that survival was significantly correlated with sex (women had a better prognosis than men) and M stage (patients without metastases had a better outcome). Patient age, duration of symptoms, Karnofsky Performance Scale score at presentation, hydrocephalus, tumor location, brainstem invasion, extent of resection, histological subtype, preradiotherapy chemotherapy, risk group, and period of presentation were not significant variables. Multivariate analysis identified sex and M stage as well as the period of presentation as independent prognostic factors for overall and event-free survival times. Eleven patients suffered tumor recurrence within a median time of 4.2 years. The posterior fossa was not the most common site of recurrence, and delayed recurrence was not rare. All patients in whom the tumor recurred have died despite aggressive treatments. The median survival time after diagnosis of recurrence was 2.5 years. Questionnaires on quality of life and cognition showed high scores in favor of limited negative effects in the perception of mental and physical health after treatment. The authors observed 1 supposed second malignancy (thyroid carcinoma) and no evidence of pituitary dysfunction.
Long-term survival is possible in adults treated for medulloblastoma. Although rare, metastasis seeding at presentation is a poor prognostic factor. The possibility of delayed recurrence necessitates close follow-up of all patients. Tumor recurrences should be treated with aggressive therapies as some patients may have sustained response. Adjuvant chemotherapy should be given to high-risk patients, but its role in reducing recurrences, particularly distant ones, remains unclear in the standard-risk group.
Gabriel Eustache, Nicolas Nardi, Chloé Rousseau, Sandra Aouaissia, Sylvie Aillet, Severine Delahaye Larralde, Eric Wodey, and Laurent Riffaud
Tranexamic acid (TXA) is an antifibrinolytic drug that has achieved significant reduction in perioperative blood loss and the quantity of blood transfused in many pediatric surgical procedures, without morbidity. Despite the accumulation of evidence regarding its effectiveness in craniosynostosis repair surgery, TXA is not unanimously employed by pediatric neurosurgery teams. The purpose of this study was to evaluate the impact of the routine use of TXA in a homogeneous population of children who underwent open surgery for monosutural craniosynostosis.
The authors retrospectively reviewed 3 cohorts of patients that were subdivided from 2 cohorts surrounding the initiation of TXA (group 1, TXA− [2008–2011] and group 2, TXA+ [2011–2013]) and a third cohort of more recent patients (group 3, TXA+ [2016–2017]). TXA was administered using the same protocol comprising a loading dose of 10 mg/kg over 15 minutes after induction of general anesthesia followed by a 10 mg/kg per hour infusion until skin closure. Patients in all 3 groups underwent similar standardized procedures for scaphocephaly, trigonocephaly, and unicoronal craniosynostosis by the same pediatric neurosurgeon.
Overall, 102 infants were included in the study: 32 infants in group 1, 36 in group 2, and 34 in group 3. Significant reductions in transfusion of packed erythrocytes (PE) and fresh-frozen plasma (FFP) were observed between the TXA− and TXA+ time periods. The median volume of PE transfusion was reduced by > 50% with the use of TXA (42.8 mL/kg in the TXA− group vs 20.0 in the TXA+ groups, p < 0.0001). Reduction in PE transfusion was 100% postoperatively in the TXA+ groups (20.0 mL/kg in the TXA− group vs 0.0 in the TXA+ groups, p < 0.0001). The median volume of FFP transfusion was reduced by 100% with the use of TXA (12.8 mL/kg in the TXA− group vs 0.0 in the TXA+ groups, p < 0.0001). All children in group 1 received a transfusion, whereas 3 children (8%) and 7 children (20%) in groups 2 and 3, respectively, did not. Significant reductions in postoperative drain output were also noted between the TXA− and TXA+ time periods. The total hospital length of stay was significantly lower in the TXA+ groups (p < 0.0001).
Use of TXA reduced blood loss and the need for transfusions but also decreased the hospital length of stay and, thus, minimized overall medical care costs. Intraoperative administration of TXA in craniosynostosis repair surgery should be routinely used in all centers that practice these procedures.