The authors report the case of a 25-month-old boy who underwent endoscopic third ventriculostomy (ETV) for hydrocephalus resulting from aqueductal stenosis. The patient's recovery was monitored longitudinally and prospectively using MR diffusion tensor imaging (DTI) and formal neuropsychological testing. Despite minimal change in ventricle size, improvement in the DTI characteristics and neurodevelopmental trajectory was observed following ETV. These data support the use of DTI as a biomarker to assess therapeutic response in children undergoing surgical treatment for hydrocephalus. In the patient featured in this report, DTI appeared to provide more information regarding postoperative neurodevelopmental outcome than ventricle size alone.
Robert T. Buckley, Weihong Yuan, Francesco T. Mangano, Jannel M. Phillips, Stephanie Powell, Robert C. McKinstry, Akila Rajagopal, Blaise V. Jones, Scott Holland and David D. Limbrick Jr.
Robert T. Buckley, Tiffany Morgan, Russell P. Saneto, Jason Barber, Richard G. Ellenbogen and Jeffrey G. Ojemann
Functional hemispherectomy is a well-recognized surgical option for the treatment of unihemispheric medically intractable epilepsy. While the resultant motor deficits are a well-known and expected consequence of the procedure, the impact on other cortical functions has been less well defined. As the cortical control of swallowing would appear to be threatened after hemispherectomy, the authors retrospectively studied a pediatric population that underwent functional hemispherectomy for medically intractable epilepsy to characterize the incidence and severity of dysphagia after surgery.
A retrospective cohort (n = 39) of pediatric patients who underwent hemispherectomy at a single institution was identified, and available clinical records were reviewed. Additionally, the authors examined available MR images for integrity of the thalamus and basal ganglia before and after hemispherectomy. Clinical and video fluoroscopic assessments of speech pathology were reviewed, and the presence, type, and duration of pre- and postoperative dysphagia were recorded.
New-onset, transient dysphagia occurred in 26% of patients after hemispherectomy along with worsening of preexisting dysphagia noted in an additional 15%. Clinical symptoms lasted a median of 19 days. Increased duration of symptoms was seen with late (> 14 days postoperative) pharyngeal swallow dysfunction when compared with oral dysphagia alone. Neonatal stroke as a cause for seizures decreased the likelihood of postoperative dysphagia. There was no association with seizure freedom or postoperative hydrocephalus.
New-onset dysphagia is a frequent and clinically significant consequence of hemispherectomy for intractable epilepsy in pediatric patients. This dysphagia was always self-limited except in those patients in whom preexisting dysphagia was noted.
Matthew R. Reynolds, Robert T. Buckley, Santoshi S. Indrakanti, Ali H. Turkmani, Gerald Oh, Emanuela Crobeddu, Kyle M. Fargen, Tarek Y. El Ahmadieh, Andrew M. Naidech, Sepideh Amin-Hanjani, Giuseppe Lanzino, Brian L. Hoh, Bernard R. Bendok and Gregory J. Zipfel
Vasopressor-induced hypertension (VIH) is an established treatment for patients with aneurysmal subarachnoid hemorrhage (SAH) who develop vasospasm and delayed cerebral ischemia (DCI). However, the safety of VIH in patients with coincident, unruptured, unprotected intracranial aneurysms is uncertain.
This retrospective multiinstitutional study identified 1) patients with aneurysmal SAH and 1 or more unruptured, unprotected aneurysms who required VIH therapy (VIH group), and 2) patients with aneurysmal SAH and 1 or more unruptured, unprotected aneurysms who did not require VIH therapy (non-VIH group). All patients had previously undergone surgical or endovascular treatment for the presumed ruptured aneurysm. Comparisons between the VIH and non-VIH patients were made in terms of the patient characteristics, clinical and radiographic severity of SAH, total number of aneurysms, number of ruptured/unruptured aneurysms, aneurysm location/size, number of unruptured and unprotected aneurysms during VIH, severity of vasospasm, degree of hypervolemia, and degree and duration of VIH therapy.
For the VIH group (n = 176), 484 aneurysms were diagnosed, 231 aneurysms were treated, and 253 unruptured aneurysms were left unprotected during 1293 total days of VIH therapy (5.12 total years of VIH therapy for unruptured, unprotected aneurysms). For the non-VIH group (n = 73), 207 aneurysms were diagnosed, 93 aneurysms were treated, and 114 unruptured aneurysms were left unprotected. For the VIH and non-VIH groups, the mean sizes of the ruptured (7.2 ± 0.3 vs 7.8 ± 0.6 mm, respectively; p = 0.27) and unruptured (3.4 ± 0.2 vs 3.2 ± 0.2 mm, respectively; p = 0.40) aneurysms did not differ. The authors observed 1 new SAH from a previously unruptured, unprotected aneurysm in each group (1 of 176 vs 1 of 73 patients; p = 0.50). Baseline patient characteristics and comorbidities were similar between groups. While the degree of hypervolemia was similar between the VIH and non-VIH patients (fluid balance over the first 10 days of therapy: 3146.2 ± 296.4 vs 2910.5 ± 450.7 ml, respectively; p = 0.67), VIH resulted in a significant increase in mean arterial pressure (mean increase over the first 10 days of therapy relative to baseline: 125.1% ± 1.0% vs 98.2% ± 1.2%, respectively; p < 0.01) and systolic blood pressure (125.6% ± 1.1% vs. 104.1% ± 5.2%, respectively; p < 0.01).
For small, unruptured, unprotected intracranial aneurysms in SAH patients, the frequency of aneurysm rupture during VIH therapy is rare. The authors do not recommend withholding VIH therapy from these patients.
Robert T. Buckley, Anthony C. Wang, John W. Miller, Edward J. Novotny and Jeffrey G. Ojemann
Laser ablation is a novel, minimally invasive procedure that utilizes MRI-guided thermal energy to treat epileptogenic and other brain lesions. In addition to treatment of mesial temporal lobe epilepsy, laser ablation is increasingly being used to target deep or inoperable lesions, including hypothalamic hamartoma (HH), subependymal giant cell astrocytoma (SEGA), and exophytic intrinsic hypothalamic/third ventricular tumors. The authors reviewed their early institutional experience with these patients to characterize clinical outcomes in patients undergoing this procedure.
A retrospective cohort (n = 12) of patients undergoing laser ablation at a single institution was identified, and clinical and radiographic records were reviewed.
Laser ablation was successfully performed in all patients. No permanent neurological or endocrine complications occurred; 2 (17%) patients developed acute obstructive hydrocephalus or shunt malfunction following treatment. Laser ablation of HH resulted in seizure freedom (Engel Class I) in 67%, with the remaining patients having a clinically significant reduction in seizure frequency of greater than 90% compared with preoperative baseline (Engel Class IIB). Treatment of SEGAs resulted in durable clinical and radiographic tumor control in 2 of 3 cases, with one patient receiving adjuvant everolimus and the other receiving no additional therapy. Palliative ablation of hypothalamic/third ventricular tumors resulted in partial tumor control in 1 of 3 patients.
Early experience suggests that laser ablation is a generally safe, durable, and effective treatment for patients harboring HHs. It also appears effective for local control of SEGAs, especially in combination therapy with everolimus. Its use as a palliative treatment for intrinsic hypothalamic/deep intraventricular tumors was less successful and associated with a higher risk of serious complications. Additional experience and long-term follow-up will be beneficial in further characterizing the effectiveness and risk profile of laser ablation in treating these lesions in comparison with conventional resective surgery or stereotactic radiosurgery.
Anthony C. Wang, George M. Ibrahim, Andrew V. Poliakov, Page I. Wang, Aria Fallah, Gary W. Mathern, Robert T. Buckley, Kelly Collins, Alexander G. Weil, Hillary A. Shurtleff, Molly H. Warner, Francisco A. Perez, Dennis W. Shaw, Jason N. Wright, Russell P. Saneto, Edward J. Novotny, Amy Lee, Samuel R. Browd and Jeffrey G. Ojemann
The potential loss of motor function after cerebral hemispherectomy is a common cause of anguish for patients, their families, and their physicians. The deficits these patients face are individually unique, but as a whole they provide a framework to understand the mechanisms underlying cortical reorganization of motor function. This study investigated whether preoperative functional MRI (fMRI) and diffusion tensor imaging (DTI) could predict the postoperative preservation of hand motor function.
Thirteen independent reviewers analyzed sensorimotor fMRI and colored fractional anisotropy (CoFA)–DTI maps in 25 patients undergoing functional hemispherectomy for treatment of intractable seizures. Pre- and postoperative gross hand motor function were categorized and correlated with fMRI and DTI findings, specifically, abnormally located motor activation on fMRI and corticospinal tract atrophy on DTI.
Normal sensorimotor cortical activation on preoperative fMRI was significantly associated with severe decline in postoperative motor function, demonstrating 92.9% sensitivity (95% CI 0.661–0.998) and 100% specificity (95% CI 0.715–1.00). Bilaterally robust, symmetric corticospinal tracts on CoFA-DTI maps were significantly associated with severe postoperative motor decline, demonstrating 85.7% sensitivity (95% CI 0.572–0.982) and 100% specificity (95% CI 0.715–1.00). Interpreting the fMR images, the reviewers achieved a Fleiss’ kappa coefficient (κ) for interrater agreement of κ = 0.69, indicating good agreement (p < 0.01). When interpreting the CoFA-DTI maps, the reviewers achieved κ = 0.64, again indicating good agreement (p < 0.01).
Functional hemispherectomy offers a high potential for seizure freedom without debilitating functional deficits in certain instances. Patients likely to retain preoperative motor function can be identified prior to hemispherectomy, where fMRI or DTI suggests that cortical reorganization of motor function has occurred prior to the operation.