Transcallosal endoscopic resection of hypothalamic hamartoma in a case with Pallister-Hall syndrome

Dericioglu N., SAYGI S., Akalan N.

Epileptic Disorders, vol.13, no.2, pp.209-213, 2011 (SCI-Expanded) identifier identifier

  • Publication Type: Article / Review
  • Volume: 13 Issue: 2
  • Publication Date: 2011
  • Doi Number: 10.1684/epd.2011.0440
  • Journal Name: Epileptic Disorders
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Page Numbers: pp.209-213
  • Keywords: gelastic seizures, hypothalamic hamartoma, Pallister-Hall syndrome, polydactyly, surgery
  • Ankara University Affiliated: Yes

Abstract

Pallister-Hall syndrome (PHS) is a very rare syndrome characterized by hypothalamic hamartoma (HH), polydactyly, panhypopituitarism, imperforate anus and other visceral anomalies. Contrary to patients with isolated HH, neurological dysfunction and precocious puberty are uncommon and seizures are usually well controlled with anticonvulsant medication. Therefore, conservative management of HH is advised. To the best of our knowledge, seven cases of PHS with surgical resection of the HH have so far been reported. Five patients were either seizure-free or had >90% seizure reduction postoperatively. Here, we present a case of PHS of a patient who also underwent transcallosal endoscopic resection of the HH with a subsequent 70% reduction in seizure frequency. © 2011 John Libbey Eurotext and Springer.