title: Posterior quadrant disconnection surgery for Sturge-Weber syndrome.
abstract: OBJECTIVE
  Some patients with Sturge-Weber syndrome (SWS) need epilepsy surgery for adequate seizure control and prevention of psychomotor deterioration. The majority of patients with SWS have leptomeningeal angioma located over the temporal, parietal, and occipital lobes. We applied posterior quadrant disconnection surgery for this type of SWS with intractable seizure. We evaluated the efficacy of this procedure in seizure control and psychomotor development.
  
  
  METHODS
  Ten patients who were surgically treated using the posterior quadrantectomy (PQT) were enrolled in this study. Surgical outcome was analyzed as seizure-free or not at 2 years after surgery. Psychomotor development was evaluated by the scores of mental developmental index (MDI) and psychomotor developmental index (PDI) in the Bayley Scales of Infant Development II preoperatively, and at 6 and 12 months after the PQT.
  
  
  RESULTS
  Eight of 10 patients were seizure-free. Patients without complete elimination of the angiomatous areas had residual seizures. Average MDI and PDI scores before the surgery were 64.8 and 71.6, respectively. Scores of MDI at 6 and 12 months after the PQT in seizure-free patients were 80.5 and 84.5, respectively (p < 0.01). PDI scores at these postoperative intervals were 87.3 and 86.4, respectively (p < 0.05). Patients with residual seizures did not improve in either MDI or PDI.
  
  
  CONCLUSIONS
  The PQT achieved good seizure control and improved psychomotor development in patients with SWS. The complete deafferentation of angiomatous areas is required for seizure-free results and psychomotor developmental improvement.
authors.affiliation: Department of Neurosurgery, Juntendo University, Tokyo, Japan.
authors.forename: Hidenori
authors.initials: H
authors.lastname: Sugano
authors.affiliation: Department of Neurosurgery, Juntendo University, Tokyo, Japan.
authors.forename: Hajime
authors.initials: H
authors.lastname: Nakanishi
authors.affiliation: Department of Neurosurgery, Juntendo University, Tokyo, Japan.
authors.forename: Madoka
authors.initials: M
authors.lastname: Nakajima
authors.affiliation: Department of Neurosurgery, Juntendo University, Tokyo, Japan.
authors.forename: Takuma
authors.initials: T
authors.lastname: Higo
authors.affiliation: Department of Neurosurgery, Juntendo University, Tokyo, Japan.
authors.forename: Yasushi
authors.initials: Y
authors.lastname: Iimura
authors.affiliation: Department of Pediatrics, Juntendo University, Tokyo, Japan.
authors.forename: Kyoko
authors.initials: K
authors.lastname: Tanaka
authors.affiliation: Department of Pediatrics, Juntendo University, Tokyo, Japan.
authors.forename: Mariko
authors.initials: M
authors.lastname: Hosozawa
authors.affiliation: Department of Pediatrics, Juntendo University, Tokyo, Japan.
authors.forename: Shinichi
authors.initials: S
authors.lastname: Niijima
authors.affiliation: Department of Neurosurgery, Juntendo University, Tokyo, Japan.
authors.forename: Hajime
authors.initials: H
authors.lastname: Arai
mesh_terms: D003337:Corpus Callosum / Q000601:surgery; D003399:Craniotomy / Q000379:methods*; D004830:Epilepsy, Tonic-Clonic / Q000175:diagnosis / Q000601:surgery*; D006391:Hemangioma / Q000175:diagnosis / Q000601:surgery; D006801:Humans; D007089:Image Enhancement; D008279:Magnetic Resonance Imaging; D008577:Meningeal Neoplasms / Q000601:surgery; D009434:Neural Pathways / Q000601:surgery*; D038361:Neuronavigation / Q000379:methods*; D009778:Occipital Lobe / Q000601:surgery*; D010296:Parietal Lobe / Q000601:surgery*; D011596:Psychomotor Disorders / Q000175:diagnosis / Q000601:surgery; D013341:Sturge-Weber Syndrome / Q000175:diagnosis / Q000601:surgery*; D013702:Temporal Lobe / Q000601:surgery*
keywords: Epilepsy surgery; Posterior quadrantectomy; Psychomotor development; Seizure outcome; Sturge-Weber syndrome