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Hemispherotomy in an infant with hemimegalencephaly.

Kulkarni SD, Deopujari CE, Patil VA, Sayed RJ - J Pediatr Neurosci (2015 Apr-Jun)

Bottom Line: Hemispheric disconnection has been reported to be efficient in seizure control and prevents further cognitive injury and developmental delay.She had more than 90% reduction of seizures with good developmental outcome on follow-up.Thus, despite risks of the procedure, early surgery should be preferred in infants with HME.

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatric Neurosciences, Bai Jerbai Wadia Hospital, Parel, Mumbai, Maharashtra, India.

ABSTRACT
Hemimegalencephaly (HME) is a rare hamartomatous congenital malformation of the brain. The epilepsy pattern in HME can be partial seizures or may present as spasms as in epileptic encephalopathy. Epilepsy associated with HME is usually resistant to antiepileptic drugs and requires surgical intervention. Hemispheric disconnection has been reported to be efficient in seizure control and prevents further cognitive injury and developmental delay. We report a case of HME, who underwent a two-stage hemispherotomy due to complications in the first surgery. She had more than 90% reduction of seizures with good developmental outcome on follow-up. Thus, despite risks of the procedure, early surgery should be preferred in infants with HME.

No MeSH data available.


Related in: MedlinePlus

(a) Computed tomography brain (axial view) after first surgery showing intraventricular hemorrhage (b) Magnetic resonance imaging brain (T1-weighted images) after first surgery showing incomplete disconnection between the two hemispheres. (c) Electroencephalogram (post first surgery) showing asymmetry between two hemispheres without any epileptiform discharges
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Figure 2: (a) Computed tomography brain (axial view) after first surgery showing intraventricular hemorrhage (b) Magnetic resonance imaging brain (T1-weighted images) after first surgery showing incomplete disconnection between the two hemispheres. (c) Electroencephalogram (post first surgery) showing asymmetry between two hemispheres without any epileptiform discharges

Mentions: In view of resistant epilepsy, due to a structural etiology, a decision of hemispherotomy was taken. She underwent surgery (right temporal lobectomy, callosotomy followed by deafferentation) at 9 months of age. However, during the surgery, she developed complication in the form of intraventricular hemorrhage (IVH). Complete functional hemispherotomy was abandoned in view of hemodynamic instability. An intraventricular drain was inserted. Postoperatively, her computed tomography brain showed IVH [Figure 2a]. In the immediate postoperative period, she was hemodynamically stable and did not have any seizures. MRI brain study done 6 weeks postoperatively showed incomplete disconnection between the two hemispheres [Figure 2b]. Her EEG 6 weeks postoperatively showed gross asymmetry without any epileptiform discharges [Figure 2c]. By this time, her spasms had stopped and she started attaining milestones but her left side paucity of movements was marked. Eventually, she achieved head holding and started socializing. Her seizure frequency decreased over next 6 months to occasional partial seizures.


Hemispherotomy in an infant with hemimegalencephaly.

Kulkarni SD, Deopujari CE, Patil VA, Sayed RJ - J Pediatr Neurosci (2015 Apr-Jun)

(a) Computed tomography brain (axial view) after first surgery showing intraventricular hemorrhage (b) Magnetic resonance imaging brain (T1-weighted images) after first surgery showing incomplete disconnection between the two hemispheres. (c) Electroencephalogram (post first surgery) showing asymmetry between two hemispheres without any epileptiform discharges
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4489073&req=5

Figure 2: (a) Computed tomography brain (axial view) after first surgery showing intraventricular hemorrhage (b) Magnetic resonance imaging brain (T1-weighted images) after first surgery showing incomplete disconnection between the two hemispheres. (c) Electroencephalogram (post first surgery) showing asymmetry between two hemispheres without any epileptiform discharges
Mentions: In view of resistant epilepsy, due to a structural etiology, a decision of hemispherotomy was taken. She underwent surgery (right temporal lobectomy, callosotomy followed by deafferentation) at 9 months of age. However, during the surgery, she developed complication in the form of intraventricular hemorrhage (IVH). Complete functional hemispherotomy was abandoned in view of hemodynamic instability. An intraventricular drain was inserted. Postoperatively, her computed tomography brain showed IVH [Figure 2a]. In the immediate postoperative period, she was hemodynamically stable and did not have any seizures. MRI brain study done 6 weeks postoperatively showed incomplete disconnection between the two hemispheres [Figure 2b]. Her EEG 6 weeks postoperatively showed gross asymmetry without any epileptiform discharges [Figure 2c]. By this time, her spasms had stopped and she started attaining milestones but her left side paucity of movements was marked. Eventually, she achieved head holding and started socializing. Her seizure frequency decreased over next 6 months to occasional partial seizures.

Bottom Line: Hemispheric disconnection has been reported to be efficient in seizure control and prevents further cognitive injury and developmental delay.She had more than 90% reduction of seizures with good developmental outcome on follow-up.Thus, despite risks of the procedure, early surgery should be preferred in infants with HME.

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatric Neurosciences, Bai Jerbai Wadia Hospital, Parel, Mumbai, Maharashtra, India.

ABSTRACT
Hemimegalencephaly (HME) is a rare hamartomatous congenital malformation of the brain. The epilepsy pattern in HME can be partial seizures or may present as spasms as in epileptic encephalopathy. Epilepsy associated with HME is usually resistant to antiepileptic drugs and requires surgical intervention. Hemispheric disconnection has been reported to be efficient in seizure control and prevents further cognitive injury and developmental delay. We report a case of HME, who underwent a two-stage hemispherotomy due to complications in the first surgery. She had more than 90% reduction of seizures with good developmental outcome on follow-up. Thus, despite risks of the procedure, early surgery should be preferred in infants with HME.

No MeSH data available.


Related in: MedlinePlus