Kazuyoshi Morimoto

Tethered Cord Associated with Anorectal Malformation

Ten children with a tethered cord and also an anorectal malformation are reported in this document. The anorectal malformations comprised 5 vesicointestinal fissures, 2 cloacal exstrophies, 2 rectovesical fistulas and 1 rectobulbar fistula. All of the patients underwent colostomy in advance of surgery for untethering of the spinal cord. Although their neurologic deficits had previously been considered static, they were subjected to radiographic examination of the caudal spine and found to have a tethered cord. These 10 children were among 55 children with a tethered cord surgically treated at the Division of Neurosurgery of the Osaka Medical Center and Research Institute for Maternal and Child Health during the last 11 years. Data were obtained for these 10 children (6 boys and 4 girls, mean age 1.7 years) who underwent surgical untethering. Several hypotheses are offered to explain this association. Anorectal malformations are related to underlying spinal cord anomalies, which may be amenable to neurosurgical correction. Eight of our patients had no skin stigma of the lumbosacral region, in contrast to an ordinary tethered cord. Spinal cord imaging is necessary to closely scrutinize these children.

Neurodevelopmental outcome in children with fetal hydrocephalus

Thirty-eight children with fetal hydrocephalus treated surgically during the neonatal period were analyzed concerning their neurodevelopmental outcome by means of a follow-up study. The mean gestation at diagnosis was 31.1 weeks, and the mean postconceptual age at the first operation was 36.3 weeks. The ages of the patients at the last follow-up ranged from 3.8 to 11.8 years (mean = 7.4 years of age). During the follow-up period, they underwent neurologic examinations and were also evaluated to determine the developmental quotient or intelligence quotient. The relationships between age at diagnosis or the first operation for hydrocephalus and neurodevelopmental outcome, and between the lateral ventricular width/hemispheral width ratio at diagnosis and age at diagnosis or the first operation, and neurodevelopmental outcome were statistically evaluated. The neurodevelopmental outcome in the patients was normal in three patients, borderline intelligence in one patient, mental retardation in seven patients, and motor disturbance in 27 patients (five of whom were intellectually normal). The etiology was a major determinant of the outcome in the children with early postnatal surgical treatment of fetal hydrocephalus. Furthermore, an early onset and a high lateral ventricular width/hemispheral width ratio at diagnosis of hydrocephalus were significantly correlated with a poor intellectual outcome.

Spinal Lipomas in Children – Surgical Management and Long-Term Follow-Up

Since its introduction, MR imaging has been easy to perform on all children with lumbosacral cutaneous stigmata, and has enabled the phenomenal refinement of spinal pathology. We investigated the overall outcomes of children with spinal lipomas at the Osaka Medical Center and Research Institute for Maternal and Child Health in Osaka, Japan. Between 1991 and 2003, 76 children with a tethered cord underwent a total of 90 surgical procedures at our institutes. Of this cohort, 67 cases had spinal lipomas. The mean age of patients at first operation for asymptomatic lipoma was 22.4 months (range: 1 month to 16 years, trim mean: 10.1 months, mode: 5 months), except 4 cases. The mean total follow-up for the cohort since the first surgical procedure was 7.2 years (trim mean: 7.9 years). Since the introduction of MR imaging, the reoperation rate for symptoms or signs in our series was 16.4% (11 cases). Regarding the 13 subsequent reoperations (2 patients had 2 operations), 3 patients were reoperated on due to multiplication of the lipomas after untethering, 3 due to urologic symptoms and 5 due to orthopedic signs. There were 2 cases who also had to undergo reoperation early due to CSF leakage. Filum and conus lipomas have similar tethering pathologies, but differ in the outcome following surgery. Filum lipomas are benign, and therefore surgery is safe and effective. Conus lipomas are more difficult to manage.

Cellular level of purine compounds in ischemic gerbil brain by high performance liquid chromatography.

Abstract: The cellular level of ATP and related compounds in ischemic gerbil brain was investigated by high performance liquid chromatography (HPLC). Brain samples were obtained in situ following ligation of the common carotid artery. AMP and ADP peaks in the brain extracts in the ischemic group became much larger whereas the ATP peak decreased dramatically. The most striking finding was an extensive increase of adenosine: 50–100 fold. The levels of inosine and hypoxanthine also increased greatly in typical symptomatic gerbil.

Serial IMP-SPECT and EEG studies in an infant with hemimegalencephaly

An 8-month-old girl of hypomelanosis of Ito associated with hemimegalencephaly had frequent seizures beginning 44 h after birth. The seizures were secondarily generalized or unilateral initially, followed by infantile spasms at about 1.5 months of age. Frequent partial seizures appeared at 4 months of age. [123I]N-Isopropyl-p-iodoamphetamine (IMP) single photon emission computed tomography (SPECT) was performed serially during an interictal period at 1, 3 and 7 months of age. At 1 and 3 months, IMP-SPECT showed a marked increase of IMP uptake in the pathological left hemisphere and electroencephalography (EEG) revealed left-sided dominant hypsarrhythmia. At 7 months of age, a reversal was seen, there being decreased uptake on SPECT in the pathological hemisphere and abundant high amplitude background activity mingled with epileptic discharges on EEG in the non-pathological hemisphere. These serial changes of IMP uptake on SPECT seemed to reflect either changes in epileptic activity or maturational changes in cerebral perfusion in hemimegalencephaly.

Fahr’s Disease Associated with Cystic Astrocytoma

The authors present a case of Fahr’s disease associated with a cystic astrocytoma. This is the first patient with both entities to be reported.

Pathology of brain parenchyna in meningeal carcinomatosis; Immunohistochemical study with astroprotein (GFAP) and tubulin

Effects of leptomeningeal tumor on the brain parenchyma was studied by the immunohistochemical method with astroprotein (GFAP) and tubulin in a rat model of meningeal carcinomatosis. Thickening of subpial glial lining (external glial layer) and hypertrophy of subpial astrocytes, detected by the antiserum to GFAP, was the early sign of parenchymal involvement. The glial lining was continuous as far as the tumor cells were confined to the subarachnoid space, however, penetration of tumor cells into subpial brain was associated with disruption of the glial lining. Speculative role of this lining in preventing the tumor cell to infiltrate into brain tissue was discussed. In contrast to the prominent immunohistochemical changes in astrocytes, neuronal tubulin immunoreactivity was not altered even in the late stage of the disease. The present study demonstrated that the leptomeningeal dissemination of tumor cells did cause pathologic change in brain parenchyma as was evidenced by the reactive change of astrocytes. However, the preserved immunoreaction for tubulin suggested that the nerve cell damage was not severe even at the advanced stage of the disease.

Bilateral intracranial granulomas as a complication of infected subdural peritoneal shunt

Intracranial granuloma in childhood is considered to be a very rare disorder. A three-year-old child is described who had bilateral intracranial granulomas originating from the ends of bilateral subdural-peritoneal shunts. Serial computerized tomographic examinations revealed variable and peculiar findings. Since subdural-peritoneal shunt is used in neonate, intracranial granuloma must be considered as a potential complication of unremoved subdural-peritoneal shunt.

High-grade spinal cord tumor with cerebellar and retroperitoneal extension.

Accessible online at: www.karger.com/pne A 4-month-old girl presented with a 3-week history of fecal incontinence and a 1-week history of paraparesis. She exhibited no voluntary motion of the lower extremities. Spinal magnetic resonance imaging revealed an intradural intramedullary mass under the T9 region that exhibited a swelling cord with an area of high intensity on T1-weighted imaging (fig. 1a). A preoperative brain CT scan 2 weeks later revealed another multiple cerebellar enhanced lesion, although the initial CT scan had not revealed any lesion (fig. 1B). The patient underwent laminotomy and posterior fossa craniectomy, and then partial excision of the spinal and cerebellar tumors. Examination of formalin-fixed, paraffinembedded sections of the cerebellar masses showed poorly differentiated, pleomorphic cells with marked nuclear atypia and brisk mitotic activity (fig. 1C). However, the histology of the spinal tumor was not verified. Although craniospinal irradiation (50 Gy) in addition to chemotherapy was performed for 3 months, abdominal and back distension appeared. An axial body CT scan revealed an enormous retroperitoneal extension (fig. 1D). She died 2 weeks later. At autopsy, the spinal cord tumor was found to extend through the intervertebral foramen to the retro-

Dandy-Walker cyst associated with occipital meningocele

Dandy-Walker cyst associated with occipital meningocele is very rare. Only 12 patients have been reported. We report a female infant with Dandy-Walker cyst and occipital meningocele whose diagnosis was suspected antenatally by in utero ultrasonography. At birth, head circumference was normal for 37 weeks gestation. She underwent surgical repair of the occipital meningocele immediately after birth. The post-operative course was uneventful until the sixth day of life when progressive enlargement of the head with progressive ventricular dilatation was recognized. Communication between the posterior fossa cyst and the occipital meningocele was confirmed neuroradiologically; the occipital meningocele may have compensated for the increased intracranial pressure in fetal life.