Hemispherectomy for Sturge-Weber Syndrome

Hoffman, Harold J.; Hendrick, E B; Dennis, Maureen; Armstrong, Dallas

doi:10.1159/000119821

Cited by 68 publications

(60 citation statements)

References 10 publications

(15 reference statements)

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Ramirez de Arellano 16 in 1961, found that monkeys subjected to hemidecortication at age 6 days had excellent preservation of function in the contralateral limbs and face as compared to monkeys who had their decortication at age 19 months. Hoffman et al 17 in an earlier report found that early decortication helps preservation of function of the normal remaining hemisphere, cessation of seizures, less severe of hemiparesis, and maintenance of reasonably good intelligence.…”

Section: Discussionmentioning

confidence: 98%

Sturge-Weber-Dimitri Disease: Role of Hemispherectomy in Prognosis

Ogunmekan

Hwang

Hoffman

1989

Can. j. neurol. sci.

Self Cite

View full text Add to dashboard Cite

We report 12 patients with Sturge-Weber-Dimitri disease treated surgically between January 1975 and December 1987. Hemispherectomy was performed on ten, two others underwent occipital lobectomy for intractable seizures. All operations were performed between the ages of 3 months and 20 months, except in two at age 8 and 9 years. The onset of seizures in all was between 2 and 8 months of age, except for two at 15 months. There were no postoperative deaths. Postoperative shunt procedures were required in 3 out of 12 (25%). Postoperative seizure control for one year or more was achieved in 11 out of 12 patients. The remaining patient is on medication with no seizures, but follow-up is less than a year. Intellectual deterioration was not seen after surgery except in 2 patients who had late operations. We conclude that patients with Sturge-Weber-Dimitri syndrome who have intractable seizures in the first 6 months of life and unilateral hemispheric involvement should be considered for early resection of the involved hemisphere. RESUME: Le role de I'hemispherectomie dans le prognostic de la maladie de Sturge-Weber-Dimitri Nous rapportons les cas de 12 patients atteints de la maladie de Sturge-Weber-Dimitri qui ont ete traites chirurgicalement entre Janvier 1975 et decembre 1987. Une hemispherectomie a ete effectuee chez 10 patients et 2 autres ont subi une lobectomie occipitale pour epilepsie refractaire au traitement. Toutes les interventions ont ete realisees entre Page de 3 et 20 mois, sauf chez deux patients ages de 8 et 9 ans respectivement. Le debut des crises convulsives se situait entre 2 et 8 mois chez tous les patients sauf chez deux qui avaient commence a 15 mois. II n'y a eu aucun deces en post-operatoire. On a du avoir recours a un shunt post-operatoire chez 3 des 12 patients (25%). On a obtenu un controle des crises pendant un an ou plus apres 1'intervention chez 11 des 12 patients. L'autre patient n'a pas eu de crise sous medication apres un suivi de moins de 12 mois. Nous n'avons pas observe de deterioration intellectuelle apres la chirurgie sauf chez 2 patients operes tardivement. Nous concluons que, chez les patients atteints du syndrome de Sturge-WeberDimitri qui presentent des crises convulsives refractaires au traitement dans les premiers six mois apres la naissance et dont Patteint hemispherique est unilateral, une resection precoce de Phemisphere atteint doit etre consideree.

show abstract

Section: Discussionmentioning

confidence: 98%

Sturge-Weber-Dimitri Disease: Role of Hemispherectomy in Prognosis

Ogunmekan

Hwang

Hoffman

1989

Can. j. neurol. sci.

Self Cite

View full text Add to dashboard Cite

show abstract

“…In this group of patients, one can expect a 70%-85% chance of seizure freedom following hemispherectomy. 3,5,9,12,14,15,18,19,27,30,31 The technique of hemispherectomy has evolved over the past 30 years to include approaches that primarily disconnect the affected hemisphere from the contralateral side, and that have become more minimally invasive, requiring less cerebral tissue to be removed. Al- though these advancements have led to expedited patient recovery and diminished length of stay in hospital, just as with anatomical hemispherectomy, there are instances of seizure recurrences or failures that arise after PIH or other functional disconnection procedures.…”

Section: Discussionmentioning

confidence: 99%

“…The ventricle is not routinely exposed, and mesiotemporal structures are not resected in cases in which HD is used. 9,17,26 For PIH, the frontoparietal opercular cortex is resected down to the insular pial bank, creating the suprainsular window; the infrainsular window is created by resecting the temporal opercular cortex down to the pia overlying the insula. 31,32 Typically, an anterolateral temporal lobectomy including mesial structures is performed in most cases.…”

Section: Methods Index Hemispheric Procedures Performed For Intractabmentioning

confidence: 99%

Utility of diffusion tensor imaging studies linked to neuronavigation and other modalities in repeat hemispherotomy for intractable epilepsy

Kiehna

Widjaja

Holowka

et al. 2016

PED

View full text Add to dashboard Cite

OBJECT Hemispherectomy for unilateral, medically refractory epilepsy is associated with excellent long-term seizure control. However, for patients with recurrent seizures following disconnection, workup and investigation can be challenging, and surgical options may be limited. Few studies have examined the role of repeat hemispherotomy in these patients. The authors hypothesized that residual fiber connections between the hemispheres could be the underlying cause of recurrent epilepsy in these patients. Diffusion tensor imaging (DTI) was used to test this hypothesis, and to target residual connections at reoperation using neuronavigation. METHODS The authors identified 8 patients with recurrent seizures following hemispherectomy who underwent surgery between 1995 and 2012. Prolonged video electroencephalography recordings documented persistent seizures arising from the affected hemisphere. In all patients, DTI demonstrated residual white matter association fibers connecting the hemispheres. A repeat craniotomy and neuronavigation-guided targeted disconnection of these residual fibers was performed. Engel class was used to determine outcome after surgery at a minimum of 2 years of follow-up. RESULTS Two patients underwent initial hemidecortication and 6 had periinsular hemispherotomy as their first procedures at a median age of 9.7 months. Initial pathologies included hemimegalencephaly (n = 4), multilobar cortical dysplasia (n = 3), and Rasmussen's encephalitis (n = 1). The mean duration of seizure freedom for the group after the initial procedure was 32.5 months (range 6–77 months). In all patients, DTI showed limited but definite residual connections between the 2 hemispheres, primarily across the rostrum/genu of the corpus callosum. The median age at reoperation was 6.8 years (range 1.3–14 years). The average time taken for reoperation was 3 hours (range 1.8–4.3 hours), with a mean blood loss of 150 ml (range 50–250 ml). One patient required a blood transfusion. Five patients are seizure free, and the remaining 3 patients are Engel Class II, with a minimum follow-up of 24 months for the group. CONCLUSIONS Repeat hemispherotomy is an option for consideration in patients with recurrent intractable epilepsy following failed surgery for catastrophic epilepsy. In conjunction with other modalities to establish seizure onset zones, advanced MRI and DTI sequences may be of value in identifying patients with residual connectivity between the affected and unaffected hemispheres. Targeted disconnection of these residual areas of connectivity using neuronavigation may result in improved seizure outcomes, with minimal and acceptable morbidity.

show abstract

“…In fact, it has been suggested that thrombotic pial events, stagnation of venous blood and hypoxemia can cause gliosis and gradual tissue loss. 10 Hemispherectomy outcomes are usually good, with up to 100% of patients becoming seizure-free after the procedure. [3][4] Some authors have reported periods of seizure freedom as long as 17 years post-hemispherectomy in patients with SWS 11 .…”

Section: Discussionmentioning

confidence: 99%

Seizure Recurrence 29 Years After Hemispherectomy for Sturge Weber Syndrome

Andrade

McAndrews

Hamani

et al. 2010

Can. j. neurol. sci.

View full text Add to dashboard Cite

Sturge-Weber syndrome (SWS) is a rare congenital disease associated with venous angiomatosis of the leptomeninges and ipsilateral, cutaneous facial port-wine stain (facial angiomatosis). Seizures are seen in more than 80% of cases, cognitive impairment in 50% and frequently glaucoma. 1-2 Seizures are the result of leptomeningeal angioma and underlying calcifications. These abnormalities are usually considered to be limited to one hemisphere. Frequently, seizures in SWS are refractory to medical treatment. In those cases surgical resection of the affected areas is recommended. Some form of hemispherectomy is usually performed for SWS-associated intractable epilepsy and most patients become seizure-free after surgery. 3-5 Another possible benefit of hemispherectomy is a better cognitive profile in patients that became seizure-free after such a procedure. However, very long follow-ups addressing seizure control and cognitive deficits in hemispherectomized patients are rare and they usually show good outcomes. Here we report on a SWS patient who become seizure-free after hemispherectomy performed in the first year of life, however, seizures recurred 29 years later without any identifiable cause. She also had cognitive decline before the onset of seizures. To the best of our knowledge this is the longest seizure-free interval between hemispherectomy and seizure recurrence in SWS.

show abstract

Hemispherectomy for Sturge-Weber Syndrome

Cited by 68 publications

References 10 publications

Sturge-Weber-Dimitri Disease: Role of Hemispherectomy in Prognosis

Sturge-Weber-Dimitri Disease: Role of Hemispherectomy in Prognosis

Utility of diffusion tensor imaging studies linked to neuronavigation and other modalities in repeat hemispherotomy for intractable epilepsy

Seizure Recurrence 29 Years After Hemispherectomy for Sturge Weber Syndrome

Contact Info

Product

Resources

About