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Jeremy Steinberger, Dominic A. Nistal, and Saadi Ghatan

Infantile hemangiomas (IHs) are the most common benign neoplasm of the neonatal and newborn period, affecting approximately 5% of infants. However, true IHs presenting in the neuraxis are quite rare with only 15 documented cases in the literature. Management of IH consists of utilizing steroids and immunomodulatory therapies to reduce the size of the tumor and surgery to remove the tumor to decrease symptoms and the risk of bleeding. Operative management of epidural and intradural extramedullary spinal hemangiomas has been described; however, management of intradural intramedullary IH has not been detailed in the literature. In this report, the authors describe the case of a 3-year-old girl who presented with multiple hemangiomas involving the liver, lung, and spine, with one component of the tumor involving the posterior intramedullary aspect of the spinal cord at the level of T3. After medical therapies had failed, the patient underwent endovascular embolization of the spinal hemangioma followed by resection of the tumor. While there is extensive literature on IH throughout many organ systems, only a handful of cases involving the neuraxis have been described. Operative management of refractory IH seems to allow for the reduction of tumor burden and the prevention of hemorrhagic injury.

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Jason A. Ellis, Paul C. McCormick II, Neil A. Feldstein, and Saadi Ghatan

OBJECT

Cystic lesions in the atrium (trigone) of the lateral ventricle may become symptomatic due to obstruction of physiological CSF circulation and/or from mass effect on adjacent structures. A minimally invasive approach that not only allows for straightforward access to multiple regions of the atrial cyst wall, but also enables direct inspection of the entire lateral ventricular system, has not been elaborated. In this paper the authors describe their experience with the endoscopic transoccipital horn approach for treating cystic lesions in the atrium of the lateral ventricle.

METHODS

A retrospective review was performed of all patients who underwent endoscopic surgical treatment for cysts in the atrium of the lateral ventricle between 1999 and 2014.

RESULTS

The cohort consisted of 13 consecutive patients who presented with symptomatic lateral ventricular entrapment due to the presence of an atrial cyst. There were 9 male and 4 female patients, with a median age of 5 years. Headache was the most common complaint at presentation. The transoccipital horn approach facilitated successful cyst reduction and fenestration in all cases. Temporal and occipital horn entrapment was reversed in all cases, with reestablishment of a physiological CSF flow pattern throughout the ventricles. Hydrocephalus was also reversed in all patients presenting with this neuroimaging finding at presentation. No cyst or ventricular entrapment was noted to recur during a mean follow-up period of 36 months. No patient in the study cohort required repeat surgery or permanent CSF diversion postoperatively.

CONCLUSIONS

The endoscopic transoccipital horn approach represents a safe and effective treatment strategy for patients with symptomatic atrial cysts of the lateral ventricle. Using this minimally invasive technique, all poles of the lateral ventricular system can be visualized and the unobstructed flow of CSF can be confirmed after cyst resection obviating the need for additional diversion.

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Mitchel S. Berger, Saadi Ghatan, Michael M. Haglund, Jill Dobbins, and George A. Ojemann

✓ Adults and children with low-grade gliomas often present with medically refractory epilepsy. Currently, controversy exists regarding the need for intraoperative electrocorticography (ECoG) to identify and, separately, resect seizure foci versus tumor removal alone to yield maximum seizure control in this patient population. Forty-five patients with low-grade gliomas and intractable epilepsy were retrospectively analyzed with respect to preoperative seizure frequency and duration, number of antiepileptic drugs, intraoperative ECoG data (single versus multiple foci), histology of resected seizure foci, and postoperative control of seizures with or without antiepileptic drugs.

Multiple versus single seizure foci were more likely to be associated with a longer preoperative duration of epilepsy. Of the 45 patients studied, 24 were no longer taking antiepileptic drugs and were seizure-free (mean follow-up interval 54 months). Seventeen patients, who all had complete control of their seizures, remained on antiepileptic drugs at lower doses (mean follow-up interval 44 months); seven of these patients were seizure-free postoperatively, yet the referring physician was reluctant to taper the antiepileptic drugs. Four patients continued to have seizures while receiving antiepileptic drugs, although at a reduced frequency and severity. In this series 41% of the adults versus 85% of the children were seizure-free while no longer receiving antiepileptic drugs, with mean postoperative follow-up periods of 50 and 56 months, respectively. This difference was statistically significant (p = 0.016). Therefore, based on this experience and in comparison with numerous retrospective studies involving similar patients, ECoG is advocated, especially in children and in any patient with a long-standing seizure disorder, to maximize seizure control while minimizing or abolishing the need for postoperative antiepileptic drugs.

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Jason A. Ellis, Richard C. E. Anderson, Jonathan O'Hanlon, Robert R. Goodman, Neil A. Feldstein, and Saadi Ghatan

Object

Idiopathic intracranial hypertension (IIH) may be refractory to available medical and surgical therapies. Patients with this condition may suffer from intractable headaches, experience visual deterioration, or have other symptoms related to elevated intracranial pressure. Internal cranial expansion (ICE) is a novel surgical procedure that the authors have developed for the treatment of patients with this condition. Here, they describe ICE and present their initial experience in using this surgical procedure for the treatment of patients with refractory IIH.

Methods

The authors conducted a retrospective review of 10 consecutive patients who underwent ICE for the treatment of IIH during a 5-year period. Preoperative and postoperative clinical parameters including patient symptoms, presence of papilledema, and available ICP or CSF opening pressures were compared. Procedural details and complications were noted. Intracranial volume increases were calculated using available pre- and postoperative CT scans.

Results

Follow-up for the 10 patients in this series ranged from 1 to 39.6 months (mean 15.5 months). Technically successful ICE was performed in all patients within the cohort. Surgical complications included a single postoperative seizure in one patient and a sagittal sinus tear with no clinical sequelae in another patient. At the time of last follow-up, 7 (70%) of 10 patients were either symptomatically improved or asymptomatic. Six (67%) of 9 patients with preoperative headaches had reduction or resolution of this symptom, and all patients (4 of 4) with preoperative papilledema had a reduction in or complete resolution of this sign. Postoperative ICP or CSF opening pressures were normal in all patients (4 of 4) tested. Postoperative intracranial volume expansion ranged between 3.8% and 12%.

Conclusions

Internal cranial expansion is a safe and effective surgery for the treatment of patients with refractory IIH. This surgery expands the intracranial volume and thus promotes ICP normalization, which may lead to the reduction or complete resolution of the signs and symptoms of IIH. Internal cranial expansion may be used as part of a multidisciplinary management approach in the treatment of refractory IIH.

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Fedor Panov, Sara Ganaha, Jennifer Haskell, Madeline Fields, Maite La Vega-Talbott, Steven Wolf, Patricia McGoldrick, Lara Marcuse, and Saadi Ghatan

OBJECTIVE

Approximately 75% of pediatric patients who suffer from epilepsy are successfully treated with antiepileptic drugs, while the disease is drug resistant in the remaining patients, who continue to have seizures. Patients with drug-resistant epilepsy (DRE) may have options to undergo invasive treatment such as resection, laser ablation of the epileptogenic focus, or vagus nerve stimulation. To date, treatment with responsive neurostimulation (RNS) has not been sufficiently studied in the pediatric population because the FDA has not approved the RNS device for patients younger than 18 years of age. Here, the authors sought to investigate the safety of RNS in pediatric patients.

METHODS

The authors performed a retrospective single-center study of consecutive patients with DRE who had undergone RNS system implantation from September 2015 to December 2019. Patients were followed up postoperatively to evaluate seizure freedom and complications.

RESULTS

Of the 27 patients studied, 3 developed infections and were treated with antibiotics. Of these 3 patients, one required partial removal and salvaging of a functioning system, and one required complete removal of the RNS device. No other complications, such as intracranial hemorrhage, stroke, or device malfunction, were seen. The average follow-up period was 22 months. All patients showed improvement in seizure frequency.

CONCLUSIONS

The authors demonstrated the safety and efficacy of RNS in pediatric patients, with infections being the main complication.

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Malgosia A. Kokoszka, Fedor Panov, Maite La Vega-Talbott, Patricia E. McGoldrick, Steven M. Wolf, and Saadi Ghatan

The responsive neurostimulation (RNS) system, an adjunctive treatment for pharmacoresistant partial-onset seizures with 1 or 2 foci, has been available to patients aged 18 years or older since the device’s FDA approval in 2013. Herein, the authors describe their off-label application of this technology in 2 pediatric patients and the consequent therapeutic benefit without surgical complications or treatment side effects. A 14-year-old nonambulatory, nonverbal male with severe developmental delay was considered for RNS therapy for medically and surgically refractory epilepsy with bilateral seizure onsets in the setting of a normal radiological examination and a known neuropathological diagnosis of type I cortical dysplasia. The RNS system was implanted with strip electrodes placed on the left lateral frontal and right lateral temporal neocortex. At 19 months’ follow-up, cortical stimulation resulted in sustained reduction in both seizure frequency—3 seizures per day down from 15 to 30 per day—and seizure severity. The patient subsequently underwent a trial of corticothalamic stimulation with a right temporal cortical strip and a left thalamic depth electrode, which resulted in a further 50% reduction in seizure frequency. In a second case, a 9-year-old right-handed female with radiological evidence of a small watershed infarct on the left and medically refractory seizures was referred for presurgical evaluation. Invasive monitoring revealed an unresectable seizure focus in the eloquent cortex of the left posterior frontal and parietal lobes. The RNS device was implanted with cortical leads placed at the putative seizure focus. At 21 months after surgery, the patient had been seizure free for 4 months, following a 17-month period in which the seizure frequency had decreased from 12 per month to 2 per month, with associated functional and behavioral improvement. The authors’ results suggest that RNS may be a palliative option for children with intractable seizures whose condition warrants off-label use of the surgical device. The improved therapeutic effect noted with time and sustained RNS treatment points to a possible neuromodulatory effect.

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Michael M. Haglund, Mitchel S. Berger, Dennis D. Kunkel, JoAnn E. Franck, Saadi Ghatan, and George A. Ojemann

✓ The role of specific neuronal populations in epileptic foci was studied by comparing epileptic and nonepileptic cortex removed from patients with low-grade gliomas. Epileptic and nearby (within 1 to 2 cm) nonepileptic temporal lobe neocortex was identified using electrocorticography. Cortical specimens taken from four patients identified as epileptic and nonepileptic were all void of tumor infiltration. Somatostatin- and γ-aminobutyric acid (GABAergic)-immunoreactive neurons were identified and counted. Although there was no significant difference in the overall cell count, the authors found a significant decrease in both somatostatin- and GABAergic-immunoreactive neurons (74% and 51 %, respectively) in the epileptic cortex compared to that in nonepileptic cortex from the same patient. It is suggested that these findings demonstrate changes in neuronal subpopulations that may account for the onset and propagation of epileptiform activity in patients with low-grade gliomas.

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Saadi Ghatan, David W. Newell, M. Sean Grady, Sohail K. Mirza, Jens R. Chapman, Frederick A. Mann, and Richard G. Ellenbogen

✓ Children younger than 3 years of age represent a distinct subpopulation of patients at particular risk for high cervical and craniovertebral injuries. There are few descriptions of survivors of severe craniocervical trauma among the very young, and scarce data exist regarding management after initial emergency stabilization.

The authors describe three children, age 1 to 32 months, who presented with craniocervical junction injuries. Variable neurological findings were observed at presentation (cranial nerve deficits, obtundation, and moderate-to-severe quadriparesis). All three were treated with prolonged immobilization and have recovered with minimal to no neurological deficit.

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Malgosia A. Kokoszka, Patricia E. McGoldrick, Maite La Vega-Talbott, Hillary Raynes, Christina A. Palmese, Steven M. Wolf, Cynthia L. Harden, and Saadi Ghatan

OBJECTIVE

The purpose of this study was to report outcomes of epilepsy surgery in 56 consecutive patients with autism spectrum disorder.

METHODS

Medical records of 56 consecutive patients with autism who underwent epilepsy surgery were reviewed with regard to clinical characteristics, surgical management, postoperative seizure control, and behavioral changes.

RESULTS

Of the 56 patients with autism, 39 were male, 45 were severely autistic, 27 had a history of clinically significant levels of aggression and other disruptive behaviors, and 30 were considered nonverbal at baseline. Etiology of the epilepsy was known in 32 cases, and included structural lesions, medical history, and developmental and genetic factors. Twenty-nine patients underwent resective treatments (in 8 cases combined with palliative procedures), 24 patients had only palliative treatments, and 3 patients had only subdural electroencephalography. Eighteen of the 56 patients had more than one operation. The mean age at surgery was 11 ± 6.5 years (range 1.5–35 years). At a mean follow-up of 47 ± 30 months (range 2–117 months), seizure outcomes included 20 Engel Class I, 12 Engel Class II, 18 Engel Class III, and 3 Engel Class IV cases. The age and follow-up times are stated as the mean ± SD. Three patients were able to discontinue all antiepileptic drugs (AEDs). Aggression and other aberrant behaviors observed in the clinical setting improved in 24 patients. According to caregivers, most patients also experienced some degree of improvement in daily social and cognitive function. Three patients had no functional or behavioral changes associated with seizure reduction, and 2 patients experienced worsening of seizures and behavioral symptoms.

CONCLUSIONS

Epilepsy surgery in patients with autism is feasible, with no indication that the comorbidity of autism should preclude a good outcome. Resective and palliative treatments brought seizure freedom or seizure reduction to the majority of patients, although one-third of the patients in this study required more than one procedure to achieve worthwhile improvement in the long term, and few patients were able to discontinue all AEDs. The number of palliative procedures performed, the need for multiple interventions, and continued use of AEDs highlight the complex etiology of epilepsy in patients with autism spectrum disorder. These considerations underscore the need for continued analysis, review, and reporting of surgical outcomes in patients with autism, which may aid in better identification and management of surgical candidates. The reduction in aberrant behaviors observed in this series suggests that some behaviors previously attributed to autism may be associated with intractable epilepsy, and further highlights the need for systematic evaluation of the relationship between the symptoms of autism and refractory seizures.

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Saadi Ghatan, Patricia McGoldrick, Christina Palmese, Maite La Vega-Talbott, Harriet Kang, Malgosia A. Kokoszka, Robert R. Goodman, and Steven M. Wolf

Object

The risk of developing epilepsy after perinatal stroke, hypoxic/ischemic injury, and intracerebral hemorrhage is significant, and seizures may become medically refractory in approximately 25% of these patients. Surgical management can be difficult due to multilobar or bilateral cortical injury, nonfocal or poorly lateralizing video electroencephalography (EEG) findings, and limited functional reserve. In this study the authors describe the surgical approaches, seizure outcomes, and complications in patients with epilepsy due to vascular etiologies in the perinatal period and early infancy.

Methods

The records were analyzed of 19 consecutive children and adults with medically refractory epilepsy and evidence of perinatal arterial branch occlusions, hypoxic/ischemic insult, or hemorrhagic strokes, who underwent surgery at the Comprehensive Epilepsy Center of Beth Israel Medical Center and St. Luke's-Roosevelt Hospital Center. Preoperative findings including MRI, video EEG, functional MRI, and neuropsychological testing were analyzed. The majority of patients underwent staged operations with invasive mapping, and all patients had either extra- or intraoperative functional mapping.

Results

In 7 patients with large porencephalic cysts due to major arterial branch occlusions, periinsular functional hemispherotomy was performed in 4 children, and in 3 patients, multilobar resections/disconnections were performed, with 1 patient undergoing additional resections 3 years after initial surgery due to recurrence of seizures. All of these patients have been seizure free (Engel Class IA) after a mean 4.5-year follow-up (range 15–77 months). Another 8 patients had intervascular border-zone ischemic infarcts and encephalomalacia, and in this cohort 2 hemispherotomies, 5 multilobar resections/disconnections, and 1 focal cortical resection were performed. Seven of these patients remain seizure free (Engel Class IA) after a mean 4.5-year follow-up (range 9–94 months), and 1 patient suffered a single seizure after 2.5 years of seizure freedom (Engel Class IB, 33-month follow-up). In the final 4 patients with vascular malformation-associated hemorrhagic or ischemic infarction in the perinatal period, a hemispherotomy was performed in 1 case, multilobar resections in 2 cases, and in 1 patient a partial temporal lobectomy was performed, followed 6 months later by a complete temporal and occipital lobectomy due to ongoing seizures. All of these patients have had seizure freedom (Engel Class IA) with a mean follow-up of 4.5 years (range 10–80 months). Complications included transient monoparesis or hemiparesis in 3 patients, transient mutism in 1 patient, infection in 1 patient, and a single case of permanent distal lower-extremity weakness. Transient mood disorders (depression and anxiety) were observed in 2 patients and required medical/therapeutic intervention.

Conclusions

Epilepsy surgery is effective in controlling medically intractable seizures after perinatal vascular insults. Seizure foci tend to be widespread and rarely limited to the area of injury identified through neuroimaging, with invasive monitoring directing multilobar resections in many cases. Long-term functional outcomes have been good in these patients, with significant improvements in independence, quality of life, cognitive development, and motor skills, despite transient postoperative monoparesis or hemiparesis and occasional mood disorders.