Tuberous sclerosis complex (TSC) is associated with the potential development of benign hamartomas, including subependymal giant cell astrocytomas (SEGAs). Intracranial hypertension can be caused by SEGAs due to their propensity to block the foramen of Monro. The traditional management approach is to monitor SEGAs with periodic neuroimaging and to resect those that exhibit serial growth and/or cause clinical signs of intracranial hypertension. Recent observations suggest that rapamycin therapy may induce partial regression of SEGAs, therefore providing a potential alternative to resection. The authors present the case of an 8-year-old girl with bilateral SEGAs that led to progressive hydrocephaly and incipient signs of papilledema. Three months after initiating rapamycin therapy, the SEGAs exhibited significant reduction in size (82.6% on the left and 46.7% on the right), and the lesions remained stable 5 months later. Compared with previous case reports, similar or even greater antitumor efficacy was achieved with much lower trough levels of rapamycin (10–15 compared with 3.3–4.5 ng/ml, respectively). The authors discuss various aspects of rapamycin therapy and address unresolved issues that highlight the need for further prospective clinical trials.
Ala Birca, Claude Mercier, and Philippe Major
Adil Harroud, Alexander G. Weil, Jean Turgeon, Claude Mercier, and Louis Crevier
A major challenge in sagittal craniosynostosis surgery is the high transfusion rate (50%–100%) related to blood loss in small pediatric patients. Several approaches have been proposed to prevent packed red blood cell (PRBC) transfusion, including endoscopic surgery, erythropoietin ortranexamic acid administration, and preoperative hemodilution. The authors hypothesized that a significant proportion of postoperative anemia observed in pediatric patients is actually dilutional. Consequently, since 2005, at CHU Sainte-Justine, furosemide has been administered to correct the volemic status and prevent PRBC transfusion. The purpose of this study was to evaluate the impact of postoperative furosemide administration on PRBC transfusion rates.
This was a retrospective study of 96 consecutive patients with sagittal synostosis who underwent surgery at CHU Sainte-Justine between January 2000 and May 2012. The mean age at surgery was 4.9 ± 1.5 months (range 2.8–8.7 months). Patients who had surgery before 2005 constituted the control group. Those who had surgery in 2005 or 2006 were considered part of an implementation phase because furosemide administration was not routine. Patients who had surgery after 2006 were part of the experimental (or furosemide) group. Transfusion rates among the 3 groups were compared. The impact of furosemide administration on transfusion requirement was also measured while accounting for other variables of interest in a multiple logistic regression model.
The total transfusion rate was significantly reduced in the furosemide group compared with the control group (31.3% vs 62.5%, respectively; p = 0.009), mirroring the decrease in the postoperative transfusion rate between the groups (18.3% vs 50.0%, respectively; p = 0.003). The postoperative transfusion threshold remained similar throughout the study (mean hemoglobin 56.0 g/dl vs 60.9 g/dl for control and furosemide groups, respectively; p = 0.085). The proportion of nontransfused patients with recorded hemoglobin below 70 g/dl did not differ between the control and furosemide groups (41.7% vs 28.6%, respectively; p = 0.489). Surgical procedure, preoperative hemoglobin level, estimated blood loss, and furosemide administration significantly affected the risk of receiving a postoperative PRBC transfusion. When these variables were analyzed in a multiple logistic regression model, furosemide administration remained strongly associated with a reduced risk of being exposed to a blood transfusion (OR 0.196, p = 0.005). There were no complications related to furosemide administration.
A significant part of the postoperative anemia observed in patients who underwent sagittal craniosynostosis surgery was due to hypervolemic hemodilution. Correction of the volemic status with furosemide administration significantly reduces postoperative PRBC transfusion requirements in these patients.
Didier Scavarda, Philippe Major, Anne Lortie, Claude Mercier, and Lionel Carmant
Ischemic cerebral vascular accidents (CVAs) in children result in epilepsy in 25% of patients, which is refractory in 7% of cases. Repeated seizures worsen the global and cognitive prognosis of these patients. To evaluate the prognosis of epilepsy and cognitive development in children with refractory seizures following a CVA, the authors retrospectively studied the effectiveness of periinsular hemispherotomy in the treatment of these patients.
Between March 1995 and November 2007, 8 children who suffered from stroke-induced refractory epilepsy underwent a periinsular hemispherotomy. All patients' charts were reviewed in a retrospective manner. Age at the time of the CVA, imaging studies, cause of the ischemic event, onset of the first seizure, patient's handedness, the extent of the parenchymal damage, electroencephalography findings, type of epileptic seizures, number of seizures per day, number of antiepileptic medications, preoperative neuropsychological evaluation, and surgical outcome with regard to the patient's seizure activity were analyzed.
There were 7 boys and 1 girl in this study. The mean age at stroke was 23 months (range birth–5 years). The mean age at onset of epilepsy was 22 months (range 0–60 months). The mean age at the time of the hemispherotomy was 7 years (range 54–130 months). The average delay prior to the hemispherotomy was 5 years and 3 months (range 23–115 months). Prior to surgery, the average number of seizures per day was 35 (range 5–100). The average number of antiepileptic medications introduced before the hemispherotomy was 8 (range 6–12). Six patients required only 1 surgical intervention and 2 necessitated 2 separate operations:1 underwent a 2-staged hemispherotomy and the other underwent a prior callosotomy. There were no reported surgical complications in this series. Seven children are seizure free. However, the remaining child, after a 3.5-year disease-free interval, has recently started having seizures. No child demonstrated an improvement in neuropsychological evaluation.
The periinsular hemispherotomy must be considered an alternative in the therapeutic approach to stroke-induced pediatric refractory epilepsy. It is effective in controlling seizure activity. The authors believe the delay before hemispherotomy must be shortened in children with post-CVA refractory epilepsy.
Alain Roux, Claude Mercier, Albert Larbrisseau, Louis-Jacques Dube, Céline Dupuis, and Raquel Del Carpio
✓ Epidermoid cysts are tumors familiar to neurosurgeons, but intramedullary epidermoid cysts are rare. The authors report the case of a 6-year-old girl presenting with progressive paraparesis. A midthoracic intramedullary mass was revealed on myelography and magnetic resonance (MR) imaging and confirmed as an intramedullary epidermoid cyst at surgery, at which time the cyst was removed. This is the fourth report documenting a purely intramedullary epidermoid cyst occurring in a child. The pathology and etiology, epidemiology, clinical features, radiology (including MR image characteristics), and surgical treatment of such rare intramedullary benign tumors are discussed. Magnetic resonance imaging reduces the delay in diagnosis of spinal cord tumors but should be guided by clinical judgment.
Ann Mansur, Benjamin Morgan, Alexandre Lavigne, Nicolas Phaneuf-Garand, Jocelyne Diabira, Han Yan, Unni G. Narayanan, Darcy Fehlings, Golda Milo-Manson, Blythe Dalziel, Sara Breitbart, Claude Mercier, Dominic Venne, Pierre Marois, Alexander G. Weil, Jeffrey S. Raskin, Sruthi P. Thomas, and George M. Ibrahim
In nonambulatory children with predominantly spastic cerebral palsy (CP), the authors compared care needs, symptom burden, and complications after surgical treatment with either intrathecal baclofen (ITB) pump insertion or selective dorsal rhizotomy (SDR). The patients were treated at two Canadian centers with variability in practice pertaining to these surgical options.
The authors performed a retrospective analysis of nonambulatory children with predominantly spastic quadriplegic or diplegic CP who underwent treatment with ITB or SDR. These two strategies were retrospectively assessed by comparing patient data from the two treatment groups for demographic characteristics, outcomes, and complications. A partial least-squares analysis was performed to identify patient phenotypes associated with outcomes.
Thirty patients who underwent ITB and 30 patients who underwent SDR were included for analysis. Patients in the ITB group were older and had lower baseline functional status, with greater burdens of spasticity, dystonia, pain, deformity, bladder dysfunction, and epilepsy than patients in the SDR group. In addition, children who underwent SDR had lower Gross Motor Function Classification System (GMFCS) levels and were less likely to experience complications than those who underwent ITB. However, children treated with SDR had fewer improvements in pain than children treated with ITB. A single significant latent variable explaining 88% of the variance in the data was identified.
Considerable baseline differences exist within this pediatric CP patient population. Factors specific to individual children must be taken into account when determining whether ITB or SDR is the appropriate treatment.