Surgical Management of Tuberous Sclerosis

Abstract

Tuberous sclerosis is a genetic disorder with incidence of 1 into 6000 birth. It is a multi-systemic disorder. Seizures associated with tuberous sclerosis (TS) can be difficult to control with medical therapy.
Objective: To determine the role of surgery for the management of seizures and other symptoms in Tuberous Sclerosis.
Material and Method: Four patients with TSC who underwent surgery were admitted in the Department of Neurosurgery Sheikh Zayed Hospital, Rahim Yar Khan and Department of Neurosurgery, PGMI / Lahore General Hospital, Lahore.
Results: We admitted 5 cases, 3 males and 2 females. All presenters with seizers other presenting feature were headache and vomiting in all 5 cases, vomiting in 5 cases. All cases were operated were craniotomy and removal of tumour. Surgery were V.P. Shunt was performed in 3 cases. All 5 cases revealed excellent outcome and seizures well controlled with anticonvulsant postoperatively which were poorly controlled preoperatively. All 5 cases were discharged in satisfactory condition within 2 – 3 weeks. Histopathology the histopathology of all 5 cases were subependymal giant cell astrocytoma (SEGA).
Outcome: All 5 cases revealed excellent outcome and the seizures were well controlled post-operatively with anticonvulsants. While pre-operative fits, were poorly controlled. All 5 cases were discharged in satisfactory condition with 2 – 3 weeks.
Conclusion: The surgery had excellent outcome for tuberous sclerosis provided timely decision is taken to treat the tumor and associated hydrocephalus. Most of these patients will lead ventriculoperitoneal shunt or EVD as an emergency step to save the life.