Hypothalamic hamartomas are rare lesions for which different classification schemes have been proposed. The authors report on an exceptionally large solid-cystic hamartoma that led to hydrocephalus, precocious puberty, and intractable gelastic seizures. They discuss potential mechanisms of the development of hypothalamic hamartomas.
Christian Dorfer, Gregor Kasprian, Angelika Mühlebner and Thomas Czech
Christian Dorfer, Thomas Czech, Angelika Mühlebner-Fahrngruber, Aygül Mert, Gudrun Gröppel, Klaus Novak, Anastasia Dressler, Edith Reiter-Fink, Tatjana Traub-Weidinger and Martha Feucht
Outcomes following functional hemispherotomy in patients with drug-resistant epilepsy have been well described. However, studies reporting long-term longitudinal outcomes after subhemispheric disconnective epilepsy surgery are still limited.
The authors conducted a retrospective review of prospectively collected data of 10 children who underwent temporoparietooccipital (TPO) disconnective surgery at the Vienna Pediatric Epilepsy Center.
There were 3 males and 7 females (median age 8.7 years; range 4.2–22.1 years). The affected hemisphere was the left in 3 patients and the right in 7. The patients' median age at seizure onset was 3.0 years (range 0.2–8.3 years). The median duration of epilepsy before surgery was 5.2 years (range 1.3–17.2 years). The underlying pathology was TPO malformation of cortical development in 5 patients, and venous infarction, posterior hemispheric quadrant atrophy, Sturge-Weber syndrome, cortical involvement of a systemic lupus erythematosus, and gliosis after cerebral tumor treatment in 1 each. In 6 children, a pure TPO disconnection was performed; in 2 patients, the temporal lobe was resected and parietooccipital disconnection was performed. The 2 remaining patients had had previous epilepsy surgery that was extended to a TPO disconnection: disconnection of the occipital lobe (n = 1) and resection of the temporal lobe (n = 1). The authors encountered no complications while performing surgery. No patient needed blood replacement therapy. No patient developed CSF disturbances that warranted treatment. Nine of 10 patients are currently seizure free since surgery (Wieser Class 1a) at a median follow-up time of 2.1 years (range 4 months to 8.1 years).
Temporoparietooccipital disconnection is a safe and effective motor-sparing epilepsy surgery in selected cases. Technical adjuncts facilitate a better intraoperative visualization and orientation, thereby enabling a less invasive approach than previously suggested.