Modified hemispherectomy for infantile hemiparesis and epilepsy

Abstract

Objective: To investigate the effect and medical imaging of modified hemispherectomy on patients with infantile hemiparesis and medically refractory epilepsy.

Patients and methods: Forty-three patients with infantile hemiparesis and refractory epilepsy who underwent hemispherectomy were enrolled. The treatment effect and medical imaging were analyzed.

Results: Anatomical hemispherectomy was successfully performed in all patients (100%). In all patients, the muscular tension decreased and the contracted limbs relaxed. In the pathological examination of the resected brain tissue, secondary cicatricial gyri with concomitant cortical dysplasia was present in 36 cases and polycerebellar gyrus malformation and porencephalia in the other 7 cases. Followed up for 7-15 years (mean 11.3), all patients were alive without a long-term sequela. Epilepsy was satisfactorily controlled, with complete seizure relief in 39 cases (91%) classified as Engel I and basic control in the other 4 (9%) defined as Engel II. The posthemispherectomy medical imaging demonstrated that the intracranial space on the operative side shrank, and the healthy cerebral hemisphere shifted markedly toward the hemispherectomy side, with expanded lateral ventricle on the healthy side and thickened skull and enlarged frontal sinus on the operative side. After 4-5 years, the intracranial space on the operative side disappeared in 75% of the patients, demonstrating enlarged cerebral peduncle on the healthy side.

Conclusion: Further modified hemispherectomy in patients with infantile hemiparesis and medically refractory epilepsy demonstrated markedly ameliorated effects on epilepsy control and the prevention of superficial cerebral hemosiderosis in the long-term follow-up.

Keywords: complications; hemispherectomy; infantile hemiparesis; medical imaging; refractory epilepsy.

Figure 1

A 16-year-old girl had medically refractory epilepsy and underwent hemispherectomy. (a–c) Before the hemispherectomy, magnetic resonance imaging of the brain revealed compensatory enlargement of the left cerebral peduncle but atrophic right cerebral peduncle (arrow in A) and atrophic brain tissue (arrow in B and C). (d–f) Computed tomographic scan (CTS) at 7 days posthemispherectomy demonstrated enlarged cerebral peduncle (arrows in D) on the left side and a residual intracranial space (arrow in E and F) on the right side with accumulation of air and fluid. (g–i) CTS 3 years later showed enlarged cerebral peduncle on the left side (double arrows in G), right-shifted center line (double arrows in H and I), and shrank epidural space (bigger arrow in H and I) under the skull. Calcification (arrow head in G–I) occurred on the dura mater bordering the shrank epidural space and the left hemisphere, and this calcified dura mater gave a support to the left hemisphere.

Figure 2

An 8-year-old girl with infantile hemiparesis and medically intractable epilepsy had undergone anatomical hemispherectomy. (a–c) Before the hemispherectomy, magnetic resonance imaging of the brain revealed compensatory enlargement (double arrows in A) of the right cerebral peduncle, atrophic left cerebral peduncle (single arrow in A), and atrophic brain tissue (double arrows in B and C). (d–f) CTS at 10 days posthemispherectomy demonstrated left-shifted midline structures and the residual intracranial space (double arrows in B and C) with accumulated air and fluid. (g–i) CTS 3 years later revealed enlarged cerebral peduncle on the right (double arrows in G), further left-shifted midline structures and shrank epidural space (double arrows in H and I) under the thickened skull. The arrow head indicates the dura mater separating the residual epidural space and the brain tissue on the right.

Figure 3

A 21-year-old girl had anatomical hemispherectomy because of cerebral hemorrhage at 1 month after birth and medically intractable epilepsy for 16 years. (a–c) Before the hemispherectomy, magnetic resonance imaging of the brain revealed compensatory enlargement (double arrows in A) of the left cerebral peduncle, atrophic right cerebral peduncle (single arrow in A), and atrophic brain tissue on the right (double arrows in B and C). (d–f) CTS at 8 days posthemispherectomy demonstrated right-shifted midline structures and the residual intracranial space (double arrows) with accumulated air and fluid. (g–i) CTS 3 years later revealed enlarged cerebral peduncle on the left (double arrows in G), further right-shifted midline structures and shrank epidural space (double arrows in H and I) under the thickened skull. The arrow head indicates calcification of the dura mater separating the residual epidural space and the brain tissue on the left.

Figure 4

This patient is the same as in Figure 2. (a) The head was marked for operation. (b) The convex surface of the excised cerebral hemisphere was shown. (c) The internal surface of the excised cerebral hemisphere was shown. (d) The basal ganglia and thalamus were preserved after hemispherectomy. (e) The dura mater was folded and sutured to reduce the subdural space on the operative side. (f) Follow-up 3 years later.

Figure 5

A 9-year-old boy who had medically intractable epilepsy was treated with anatomical hemispherectomy. (a) The head was marked for operation. (b) The convex surface of the excised cerebral hemisphere was shown. (c) The internal surface of the excised cerebral hemisphere was shown. (d) The basal ganglia and thalamus were preserved after hemispherectomy. (e) The dura mater was folded and closed with suturing to reduce the subdural space on the operative side. (f) Follow-up 2 years later.