Nobuyuki Akutsu1, Mai Azumi2, Junji Koyama2, Atsufumi Kawamura2, Masaaki Taniguchi3, Eiji Kohmura3. 1. Department of Neurosurgery, Hyogo Prefectural Kobe Children's Hospital, 1-6-7 Minatojima-minamimachi, Chuo-ku, Kobe, 650-0047, Japan. akutsu_kch@hp.pref.hyogo.jp. 2. Department of Neurosurgery, Hyogo Prefectural Kobe Children's Hospital, 1-6-7 Minatojima-minamimachi, Chuo-ku, Kobe, 650-0047, Japan. 3. Department of Neurosurgery, Kobe University Graduate School of Medicine, Kobe, Japan.
Abstract
PURPOSE: Hydranencephaly is a rare condition that occurs during embryogenesis after neurogenesis and is characterized by the near complete absence of the cerebral hemispheres. In general, patients with hydranencephaly have been considered to have a markedly reduced life expectancy. We present 4 patients with hydranencephaly who have survived for over 5 years. The management and problems encountered in these cases are discussed. METHODS: A retrospective review was conducted at our institution. Medical charts and radiographic studies were reviewed. Data including age at follow-up, sex, clinical complications, and surgical procedures were recorded. RESULTS: Six patients were radiologically diagnosed with hydranencephaly during the period from January 2000 to December 2012. Two patients were excluded from our study: one because of death from pneumonia at 1 year of age and another because of transfer to another hospital. Four patients (3 males and 1 female) were included in the analysis. All 4 patients underwent ventriculoperitoneal shunt (VPS) placement and shunt revision. VPS infection occurred in 3 of 4 cases, and bloody cerebrospinal fluid (CSF) was observed in 2 of 4 cases. One patient underwent successful choroid plexus cauterization (CPC) and shunt removal after shunt infection. CONCLUSIONS: Prolonged survival with hydranencephaly is not unusual in the modern treatment era. CSF shunt problems, such as recurring shunt malfunction and shunt infection, represent one of the major problems, and avoiding CSF shunt with CPC is particularly desirable in patients with hydranencephaly.
PURPOSE:Hydranencephaly is a rare condition that occurs during embryogenesis after neurogenesis and is characterized by the near complete absence of the cerebral hemispheres. In general, patients with hydranencephaly have been considered to have a markedly reduced life expectancy. We present 4 patients with hydranencephaly who have survived for over 5 years. The management and problems encountered in these cases are discussed. METHODS: A retrospective review was conducted at our institution. Medical charts and radiographic studies were reviewed. Data including age at follow-up, sex, clinical complications, and surgical procedures were recorded. RESULTS: Six patients were radiologically diagnosed with hydranencephaly during the period from January 2000 to December 2012. Two patients were excluded from our study: one because of death from pneumonia at 1 year of age and another because of transfer to another hospital. Four patients (3 males and 1 female) were included in the analysis. All 4 patients underwent ventriculoperitoneal shunt (VPS) placement and shunt revision. VPS infection occurred in 3 of 4 cases, and bloody cerebrospinal fluid (CSF) was observed in 2 of 4 cases. One patient underwent successful choroid plexus cauterization (CPC) and shunt removal after shunt infection. CONCLUSIONS: Prolonged survival with hydranencephaly is not unusual in the modern treatment era. CSF shunt problems, such as recurring shunt malfunction and shunt infection, represent one of the major problems, and avoiding CSF shunt with CPC is particularly desirable in patients with hydranencephaly.