✓Although the Codman-Hakim programmable valve is popular, several problems arising from its design have been described. The authors report an additional cause of shunt obstruction in the system. A 6-year-old girl who had received a ventriculoperitoneal shunt with the Codman-Hakim programmable valve system presented with worsening consciousness. The valve proved hard to flush, and emergency revision of the valve was performed. Examination of the extracted valve revealed that the pressure control cam had migrated into the outlet of the valve, thus causing the obstruction. A crack in the plastic housing surrounding the cam suggesting a past impact to the system was also revealed. These factors should thus be kept in mind as potential sources of obstruction of the valve system, especially in patients susceptible to episodes of head impact.
Satoru Shimizu, Satoshi Utsuki, Sachio Suzuki, Hidehiro Oka and Kiyotaka Fujii
Satoshi Utsuki, Hidehiro Oka, Takao Sagiuchi, Satoru Shimizu, Sachio Suzuki and Kiyotaka Fujii
✓ The recurrence of intracranial mature teratomas as germ cell tumors of different histological types is rarely reported. The authors describe the first case of the malignant transformation of an intracranial mature teratoma into a yolk sac tumor in a 16-year-old boy who presented with a 1-month history of anorexia and somnolence. Seven years prior to this presentation, the boy had undergone surgery for extirpation of a mature pineal teratoma. Computed tomography images obtained at his second presentation revealed a homogeneously enhanced mass within the third ventricle. The tumor was resected and the results of a histological examination were consistent with a yolk sac tumor. After resection, the patient underwent radiation therapy followed by chemotherapy with cisplatin and etoposide but died of tumor progression 15 months after his second hospitalization.
Satoshi Utsuki, Satoshi Tanaka, Hidehiro Oka, Kazuhisa Iwamoto, Takao Sagiuchi and Kiyotaka Fujii
✓ Extracranial bone metastasis from glioblastoma multiforme (GBM) has rarely been reported in the literature, and most metastatic GBMs are multiple bone metastases. The authors describe the first case of a GBM with metastasis only to the axis. This 42-year-old man presented with a 2-month history of headache, nausea, vomiting, and disorientation. Magnetic resonance imaging demonstrated a right temporal tumor, which was diagnosed as a GBM based on tumor resection. The patient was treated using radiation (6000 cGy) and the intravenous administration of nimustine hydrochloride. Eighteen months thereafter, he experienced the sudden onset of neck pain. Magnetic resonance studies revealed a tumor in the axis that was diagnosed as GBM based on biopsy procedure.
Satoshi Utsuki, Hidehiro Oka, Kimitoshi Sato, Satoru Shimizu, Sachio Suzuki and Kiyotaka Fujii
Peritumoral hemangioblastoma cysts are usually composed of fibrous tissue without tumor cells. The authors describe the first case in which fluorescence with 5-aminolevulinic acid (5-ALA) was used to diagnose a hemangioblastoma tumor in a peritumoral cyst wall. A 27-year-old woman with a homogeneous, enhanced nodular lesion in the right hemisphere of the cerebellum underwent surgical treatment. After the nodular lesion was removed, the cyst region was observed with the aid of a semiconductor laser with a peak wavelength of 405 ± 1 nm, which was powered using a fiberoptic cable. The cyst region was visualized with strong fluorescence, which disappeared after tissue removal. The fluorescent cyst consisted of tumor cells. The authors conclude that fluorescence diagnosis performed using 5-ALA can inform the choice of removing hemangioblastoma cysts.
Kimitoshi Sato, Satoru Shimizu, Satoshi Utsuki, Sachio Suzuki, Hidehiro Oka and Kiyotaka Fujii
✓ Although the Codman Hakim programmable valve (CHPV) system is popular, several problems arising from its design have been described. The authors report an additional cause of shunt failure associated with the use of the CHPV system. A 7-year-old girl who had previously undergone placement of a ventriculoperitoneal shunt with the CHPV system was admitted in a comatose state. Radiography did not demonstrate any breakage or change in the opening pressure of the valve. Manual pumping of the valve resulted in neurological improvement and shrinkage of the girl’s ventricles. However, ventriculomegaly was again observed several hours later, and an emergency valve revision was performed.
The manufacturer’s examination of the extracted valve revealed a crack on the surface of the hard plastic housing covering the valve chamber, which appeared to be suggestive of some type of blunt trauma. In addition, the flat spring that transmitted resistance from the pressure control cam to the valve ball was deformed in such a manner that it caused excessive pressure against the valve ball. Because of this deformity, the actual opening pressure in this patient had reached a maximum level of 226 mm H2O instead of the adjusted pressure of 60 mm H2O. In patients with the CHPV system whose medical history is difficult to ascertain, the possibility of damage to the valve spring and the need for a prompt revision surgery should be considered. The authors conclude that placement of the CHPV system may be contraindicated in patients with a known habit of head banging.
Successful stent placement for cervical artery dissection associated with the Ehlers—Danlos syndrome
Case report and review of the literature
Akira Kurata, Hidehiro Oka, Taketomo Ohmomo, Hitoshi Ozawa, Sachio Suzuki, Kiyotaka Fujii, Shinichi Kan, Yoshio Miyasaka and Harue Arai
✓ This 44-year-old man with Ehlers—Danlos syndrome (EDS) Type IV presented with hemiparesis and the Gerstmann syndrome. Left carotid artery (CA) angiography revealed a dissecting aneurysm with severe stenosis located in the common CA; the lesion was successfully treated with a stent graft. The patient's clinical course after endovascular surgery was uneventful, without occurrence of megacolon. The literature for spontaneous CA dissection in EDS Type IV cases is reviewed and points for investigation and treatment are discussed.
Junko Takahashi-Fujigasaki, Masayoshi Matumoto, Issei Kan, Hidehiro Oka and Masaharu Yasue
Atypical teratoid/rhabdoid tumor (AT/RT) is an aggressive embryonic brain tumor predominantly seen in young children. The authors report an unusual case of a fourth ventricle AT/RT in an infant who survived for 26 years. The tumor was resected when the patient was 6 months of age, and radiation therapy (40-Gy total dose) was performed thereafter. The patient was free from the disease for 26 years until a recurrent tumor was found in the spinal cord. The spinal cord neoplasm was a “collision tumor” with 2 components: benign schwannoma and recurrent AT/RT. The patient died of dissemination of the recurrent tumor 5 months after it was excised. This is the longest survival of a patient with AT/RT ever reported and indicates that long-term survival, more than 20 years, can be achieved in infantile-onset AT/RT. Despite intensive treatment, the prognosis for AT/RT is very poor, especially in children younger than 3 years of age. The benefits of upfront radiation therapy for AT/RT should be carefully assessed with respect to its inevitable toxicity in very young children. However, early upfront radiation therapy may be of therapeutic interest to prevent aggressive progression of the disease.
Satoru Shimizu, Satoshi Utsuki, Kimitoshi Sato, Hidehiro Oka, Kiyotaka Fujii and Kimihiko Mii
Satoshi Utsuki, Hidehiro Oka, Sumito Sato, Sachio Suzuki, Satoru Shimizu, Satoshi Tanaka and Kiyotaka Fujii
✓The response of nonfluorescing infiltrating tumors that had been exposed to 5–aminolevulinic acid and irradiated using a laser at a wavelength of 405 nm was analyzed intraoperatively using spectroscopy. Histological analyses demonstrated that neoplastic cells were present in the tissue region that displayed a peak at 636 nm, whereas no neoplastic cells were present in the region that exhibited only the excitation light peak. The authors conclude that the intraoperative use of laser spectroscopy can allow the diagnosis of infiltrating tumor and the detection of boundaries of the infiltrate when standard fluorescence techniques fail.
Satoshi Tanaka, Jiro Akimoto, Yoshitaka Narita, Hidehiro Oka and Takashi Tashiro
Methylation of O6-methylguanine-DNA methyltransferase (MGMT) has been reported to be a good prognostic factor for patients with glioblastoma multiforme (GBM). To determine whether the absolute value of MGMT messenger RNA (mRNA) might be a prognostic factor and useful for predicting the therapeutic effectiveness of temozolomide, especially with regard to GBMs, the authors measured the absolute value of MGMT mRNA in gliomas by using real-time reverse-transcription polymerase chain reaction (RT-PCR).
MGMT mRNA was measured in 140 newly diagnosed gliomas by real-time RT-PCR using the Taq-Man probe. Among 73 GBMs, 45 had been initially treated with temozolomide and radiation.
The mean MGMT mRNA value was significantly lower in oligodendroglial tumors than in other tumors. In the 73 GBMs, a significant prognostic factor for progression-free survival was fewer than 1000 copies/ μgRNA of MGMT mRNA (p = 0.0150). Of 45 patients with GBMs that had been treated with temozolomide and radiation, progression-free survival was significantly longer for those whose GMB had fewer than 1000 copies/μgRNA of MGMT mRNA than for those whose GBM had more than 1000 copies/μgRNA (p = 0.0090). In 32 patients with GBMs treated by temozolomide and radiation whose age was younger than 75 years and whose Karnofsky Performance Scale score was more than 70, progression-free and overall survival times were longer for those with GBMs of fewer than 5000 copies/μgRNA of MGMT mRNA than for those with GBMs of more than 5000 copies/μgRNA (p = 0.0365 and p = 0.0312).
MGMT mRNA might be useful as a prognostic factor and for predicting the results of therapy for GBMs treated by temozolomide. New individual adjuvant therapy based on the results of MGMT mRNA quantitation has been proposed.