Current and Future Perspectives in Craniosynostosis

Craniosynostosis has a varied clinical spectrum, ranging from isolated single suture involvement to multi-sutural fusions. Greater understanding of the pathogenesis of craniosynostosis has led to the development of practical treatment protocols. Three stages of growth have determined the approach to managing craniosynostosis : the early period, up to 12 months; the intermediate period, from 1 to 10 years; and the late period, beginning at 10 years. This review discusses current surgical management and future perspectives in craniosynostosis.

Foraminoplastic Superior Vertebral Notch Approach with Reamers in Percutaneous Endoscopic Lumbar Discectomy : Technical Note and Clinical Outcome in Limited Indications of Percutaneous Endoscopic Lumbar Discectomy

To describe the details of the foraminoplastic superior vertebral notch approach (FSVNA) with reamers in percutaneous endoscopic lumbar discectomy (PELD) and to demonstrate the clinical outcomes in limited indications of PELD. Retrospective data were collected from 64 patients who underwent PELD with FSVNA from August 2012 to April 2014. Inclusion criteria were high grade migrated disc, high canal compromised disc, and disc protrusion combined with foraminal stenosis. The clinical outcomes were assessed using by the visual analogue scale (VAS), Oswestry Disability Index (ODI) and modified MacNab criteria. Complications related to the surgery were reviewed. The procedure used a unique approach, using the superior vertebral notch as the target and performing foraminoplasty with only reamers under C-arm control. The mean age of the 55 female and 32 male patients was 52.73 years. The mean F/U period was 12.2+/-4.2 months. Preoperative VAS (8.24+/-1.25) and ODI (67.8+/-15.4) score improved significantly at the last follow-up (VAS, 1.93+/-1.78; ODI, 17.14+/-15.7). Based on the modified MacNab criteria, excellent or good results were obtained in 95.3% of the patients. Postoperative transient dysthesia (n=2) and reoperation (n=1) due to recurred disc were reported. PELD with FSVNA could be a good method for treating lumbar disc herniation. This procedure may offer safe and efficacious results, especially in the relatively limited indications for PELD.

Radicular Compression by Intraspinal Epidural Gas Bubble Occurred in Distant Two Levels after Lumbar Microdiscectomy

The authors report a case of symptomatic epidural gas accumulation 2 weeks after the multi-level lumbar surgery, causing postoperative recurrent radiculopathy. The accumulation of epidural gas compressing the dural sac and nerve root was demonstrated by CT and MRI at the distant two levels, L3-4 and L5-S1, where vacuum in disc space was observed preoperatively and both laminectomy and discectomy had been done. However, postoperative air was not identified at L4-5 level where only laminectomy had been done in same surgical field, which suggested the relationship between postoperative epidural gas and the manipulation of disc structure. Conservative treatment and needle aspiration was performed, but not effective to relieve patient's symptoms. The patient underwent revision surgery to remove the gaseous cyst. Her leg pain was improved after the second operation.

Surgical Management and Outcome of Tethered Cord Syndrome in School-Aged Children, Adolescents, and Young Adults

OBJECTIVE: The adolescent presentation of tethered cord syndrome (TCS) is well-recognized, but continues to pose significant diagnostic and management controversies. The authors conducted a retrospective study of clinical outcomes after surgical intervention in 24 school-aged children, adolescents, and young adults with TCS. METHODS: All 83 patients with a lipomyelomeningocele (LMMC) underwent untethering surgery for caudal cord tethering between 1987 and 2007. The clinical charts and follow-up data were reviewed. Of these patients, 24 school-aged children, adolescents, and young adults with TCS were studied with respect to the clinical, radiologic, pathologic features, and surgical outcomes. RESULTS: Untethering procedures were performed in 24 patients (age range, 7-25 years) for TCS of various origins (lipoma, lipomyelomeningocele, and tight filum terminale). Specific circumstances involving additional tugging of the already tight conus, and direct trauma to the back precipitated the onset of symptom in 50% of the patients. Diffuse and non-dermatomal leg pain, often referred to the anorectal region, was the most common presenting symptom. Progressive sensorimotor deficits in the lower extremities, as well as bladder and bowel dysfunction, were also common findings, but progressive foot and spinal deformities were noted less frequently. The most common tethered lesions were intradural lipomas, thickened filum and fibrous band adhesions into the placode sac. The surgical outcome was gratifying in relation to pain and motor weakness, but disappointing with respect to resolution of bowel and bladder dysfunction. Of the 24 patients with TCS, pre-operative deficits improved after surgery in 14 (58.3%), remained stable in 8 (33.4%), and worsened in 2 (8.3%). CONCLUSION: The pathologic lesions of tethered cord syndrome in school-aged children, adolescents, and young adults, are mostly intradural lipomas and tight filum. It is suggested that the degree of cord traction results in neurologic dysfunction in late life due to abnormal tension, aggravated by trauma or repeated tugging of the conus during exercise. Early diagnosis and adequate surgical release might be the keys to the successful outcome in school-aged children, adolescents, and young adults with TCS.

Tethered Cord Syndrome ; Surgical Indication, Technique and Outcome

OBJECTIVE: The authors tried to reveal some unique features of lipomeningomyelocele (LMMC), including clinical presentation, factors precipitating onset of symptoms, pathologic entities of LMMC associated with tethered cord syndrome, and surgical outcome in LMMC patients. METHODS: Seventy-five patients with LMMC were enrolled in this study. Neuro-imaging and intraoperative findings allowed classification of LMMC into three Types. The patients were divided into two groups by age : A (51 patients), from birth to 3 years, and B (24 patients), from 3 to 24 years. For prevention of retethering of the cord, a mega-dural sac rebuilding procedure was performed in 15 patients. RESULTS: During a mean postoperative follow-up period of 4 years, the surgical outcome was satisfactory in terms of improved pain and motor weakness, but disappointing with reference to the resolution of bowel and bladder dysfunction. Among these 75 patients with LMMC, preoperative deficits were improved after surgery in 29 (39%), remained stable in 28 (37%), changed slightly in 13 (17%), and worsened in 5 (7%). Patients in group A achieved better outcomes than those in group B. Depending on the type of lesion, patients with types I and II LMMC have better outcomes than those with type III LMMC. Finally, retethering of the cord with neurological deterioration occurred in 4 (5.3%) of the 75 patients, but no retethering was found in the 15 patients who were recently treated with a mega-dural sac rebuilding procedure. CONCLUSION: Our data continue to support the opinion that early diagnosis and optimal surgery are still essential for the treatment of patients with LMMC, since there is a high likelihood of residual neurological functions that can be preserved. Based on our surgical experience of untethering and decompression of lipomas, a mega-dural sac repair is useful to prevent retethering of the cord.

Temozolomide Chemotherapy in Patients with Recurrent Malignant Gliomas

Numerous studies have demonstrated the clinical activity of temozolomide, a second-generation alkylating agent, against malignant brain tumors, however, its activity has not been reported in an Asian population. This study analyzed the efficacy and toxicity of temozolomide in 25 adult patients with recurrent or progressive malignant gliomas after surgery and standard radiation therapy with or without chemotherapy, enrolled in our institution since July 2000. Sixteen patients had glioblastoma multiforme (GBM), six with anaplastic astrocytoma, and three with anaplastic oligodendroglioma. Of the 25 patients, 3 (12%) achieved a complete response (CR), 8 (32%) achieved a partial response (PR), 6 (24%) had stable disease (SD), and 8 (32%) had progressive disease (PD). Two patients achieved a CR, 4 patients achieved a PR, 3 patients had SD and 7 patients had PD in GBM, and 1 patient achieved a CR, 4 patients achieved a PR, 3 patients had SD, 1 patient had PD in the non-GBM patients. Median progression free survival was 8 weeks in GBM and 22 weeks in the non-GBM patients. The median overall survival of each group was 17 weeks and 28 weeks. Temozolomide demonstrated moderate activity in recurrent and progressive malignant gliomas without serious toxicity.

Minimally Invasive Neuroendoscopic Surgery for Pituitary Apoplexy Diagnosed During Pregnancy / 대한산부인과학회지

Pituitary apoplexy is a life-threatening condition resulting from hemorrhage or necrosis of a pituitary tumor with subsequent compression of the optic nerves and cavernous sinuses. This is the first case report of a patient who experienced pituitary apoplexy due to hemorrhage of pituitary adenoma, which was initially recognized during pregnancy, and submitted to minimally invasive neuroendoscopic transnasal transsphenoidal approach in the third trimester of pregnancy.

Experiences of Bifrontal Interhemispheric Approach in Craniopharyngioma Surgery

OBJECTIVE: The purpose of this study is to evaluate the surgical outcomes of bifrontal interhemispheric(BIH) approach and compare them to those of the pterional approach for the treatment of craniopharyngioma. METHODS: Seventeen patients had their first operation for the resection of craniopharyngiomas between 2000 and 2004 at our medical center. Eleven patients who had the pterional approach and 6 with the BIH approach were enrolled. The age range at the time of surgery was 5 to 80 years (mean age 35.6 years old). The presenting symptoms were visual disturbance in 12 patients and signs of increased intracranial pressure in 5 patients. RESULTS: The tumors were totally removed in 3(27 %) and subtotally in 8(73 %) patients with the pterional approach. Total tumor removal was achieved in 5 out of 6(83%) patients by the BIH approach, except 1 patient with a subchiasmatic lesion. Vision was improved in 4(36 %) patients treated with the pterional approach and in all patients treated by the BIH approach CONCLUSION: The BIH approach for craniopharyngioma surgery may be an effective and safe approach for tumors that extend outside of the sellar-suprasellar region with acceptable outcomes.

Unusual Clinical Course of Giant Vertebral Artery Aneurysm after Proximal Artery Embolization: Case Report / 대한뇌혈관외과학회지

The authors present the case of a patient who suffered from cerebellar dysfunction, radiographically documented brainstem compression, and neurological deterioration after proximal artery occlusion in a giant vertebral artery aneurysm. Symptom resolution was achieved after complete embolization of remained lumen of aneurysm. The cause of neurological deterioration is brainstem compression due to mass effect of the aneurysm. In order to avoid this fatal complication, it is necessary to determine appropriate therapy for the vertebral artery giant aneurysm by evaluating cerebral blood flow and other factors about growth of aneurysm.

Analysis of Angiographic Findings and Clinical Impact of Anterior Clinoidectomy in Internal Carotid-Posterior Communicating Artery Aneurysm Surgery - Clinical Research - / 대한뇌혈관외과학회지

OBJECTIVE: In the case of internal carotid-posterior communicating (ICPCom) artery aneurysm it is possible to successfully clip the aneurysmal neck without any difficulty. However, if the aneurysmal neck is wide, the aneurysmal sac is giant, the aneurysmal sac is hidden by the anterior clinoid process (ACP), or its dome is located in ventral portion or low-lying ICPCom aneurysm, it is difficult to open the proximal aneurysmal neck and we encounter a barrier in controlling bleeding in case of premature rupture of the aneurysm. They need to be resected the ACP for successful aneurysmal clipping. We propose angiographic criteria for predicting necessity of resection of the ACP before clipping of the ICPCom artery aneurysm. METHODS: Between 1999 and 2003, 16 patients with ICPCom artery aneurysm were treated with the resection of the ACP prior to applying the clip on the neck of the aneurysm. We retrospectively analyzed the preoperative cerebral angiographies, and the clinical and operative findings. We measured various radiometric parameters to reveal the angiographic characteristics. RESULTS: The mean value of the radiographic measurement in case of the cerebral angiography in 16 patients is as follows: angle A (the angle between the midline of the skull and the axis of the C1 segment on A-P view) ranged from 15 to 80 degrees (mean+/-SD, 42+/-5 degrees), angle B (the angle between the axes of the C1 and C2 segments on A-P view) ranged from 70 to 150 degrees (mean+/-SD, 110+/-15 degrees), and distance C (the distance between the tip of the ACP and the most proximal portion of the aneurysmal neck on the lateral view) ranged from 2 to 9 mm (mean+/-SD, 4.5+/-1 mm). CONCLUSION: We have resected the ACP in 16 of the 40 ICPCom aneurysms. The mean values of angle A, angle B, and distance C is 42+/-5 degrees, 110+/-15 degrees, and 4.5+/-1 mm, respectively. We did not encounter any difficulty in clipping in all the cases in which there was no premature rupture of the aneurysm. Most of cases had a good outcome.

Primary Yolk Sac Tumor of the Cerebellar Hemisphere: Case Report

Primary intracranial yolk sac tumor usually arises in the pineal or suprasellar area and shows poor prognosis. Several children with primary yolk sac tumor in the cerebellum have been reported in the literature, and none of these survived beyond 30 months. We report a case of primary yolk sac tumor in the cerebellum showing longer survival with intensive multimodal combined therapy using surgery, preirradiation chemotherapy, and irradiation.

Surgical Treatment of Middle Cranial Fossa Arachnoid Cyst by Wide Excision and Fenestration

OBJECTIVE: The results of the excision of both outer and inner membranes with fenestration to the basal and parasellar cisterns for symptomatic primary middle cranial fossa arachnoid cysts are presented. METHODS: Twenty-three symptomatic cases of middle cranial fossa arachnoid cyst treated by excision with fenestration from 1993 to 2001 at our hospital were analyzed retrospectively. RESULTS: There was no significant morbidity and mortality after surgery and no recurrence of cyst during the follow-up period(mean 40.8 months). We observed reduction of the cyst with expansion of the surrounding brain and clinical improvement in most of the patients. All cases of type III by Galassi classification, 83% of type II cases and half of type I cases were belonged to the excellent group(reduction of the cyst size over 50% during follow-up period). Seventeen cases(74%) were belonged to the excellent group and 6 cases(26%) were the good group(reduction of the cyst size under 50% during follow-up period). CONCLUSION: The results of this study suggest that the excision and fenestration procedure may be considered as the primary shunt-independent procedure in patients with symptomatic middle cranial fossa arachnoid cyst.

Stereotactic Endoscopic Evacuation of Intraventricular Hematomas

Over the last decades, the use of endoscope in neurosurgery gradually gained importance. Although the major indication of neuroendoscopic procedure is intraventricular procedures, the experience of neuroendoscopic intervention of intraventricular hemathoma is rather small. The authors present our experience of four patients with acute ventricular dilatation with intraventricular hematoma through frame-based stereotactic guidance. Through neuroendoscopic intervention, the clot was removed more than 80% each procedure. After the procedure, the silastic catheter was left for continuous intraventricular pressure monitoring. Neither thrombolytic agent nor hyperosmolar treatment was needed postoperatively. There was no mortality or morbidity directly related to endoscopic procedure itself. Brief overview of this technique is given.

Clinical Analysis of Spontaneous Intracranial Hypotension

OBJECTIVE: Spontaneous intracranial hypotension(SIH) is a rare condition presented with cardinal postural headache and low CSF pressure. The authors describe the characteristic clinical features, imaging findings, and treatment methods in the patients with spontaneous intracranial hypotension. METHODS: Between May 1994 and Aug. 2001, eight patients were diagnosed with spontaneous intracranial hypotension based on characteristic radiologic and clinical findings in our department. All cases were shown diffuse meningeal enhancement(DME) on magnetic resonance(MR) image. Two cases had subdural hematoma. RESULTS: The most characteristic feature on Brain MR image is DME with/without subdural fluid collection. Radionuclide image reveals direct leaking point and rapid appearance of urinary bladder activity. Direct leaking points were detected in all four cases who performed radioisotope study. Autologous epidural blood patches were applied to four cases, and all of them were improved without complication. Among four patients treated with conservative method, three were improved, but one patient was died of spontaneous chronic subdural hematoma. CONCLUSION: We suggest that 1) meticulous studies need to be performed aggressively if SIH is diagnosed clinically and radiologically, 2) the most effective study may be radioisotope study, and 3) if leaking point is found, aggressive management such as autologous epidural blood patches is recommended.

Treatment of High-Grade Basilar Artery Stenosis by Primary Stenting

Patients with symptomatic stenosis of the basilar artery have a poor prognosis and the treatment options are limited. Surgical bypasses are technically difficult and there is no proven benefit. Percutaneous angioplasty shows significant complications because of dissection, restenosis secondary to elastic recoil and embolic phenomena. The medical treatment with aspirin and wafarin showed the annual risk of 20% for symptomatic basilar artery stenosis as repoted in WASID(Warfarin-Aspirin Symptomatic Intracranial Disease)study. We report a patient with symptomatic high grade stenosis of the basilar artery refractory to appropriate maximal medical therapy, in whom endovascular stenting was performed successfully without preliminary balloon angioplasty. Excellent angiographic result was achieved and there were no procedural and periprocedural complications. The patient was asymptomatic except preprocedural mild dysarthria and had no neurological symptoms during clinical follow-up of 2 months at outpatient department. Primary stenting of basilar artery stenosis may be an alternative to balloon angioplasty for patient with symptomatic lesions refractory to medical therapy or in whom anticoagulation is contraindicated.

The Efficiency of Radiation Therapy in the Treatment of Intracranial Oligodendrogliomas : Factors Influencing the Prognosis / 대한방사선종양학회지

PURPOSE: Oligodendrogliomas (ODG) are a rare, slow growing, tumor in the brain, which can be cured by complete surgical resection, but as yet it is not known if postoperative adjuvant radiation therapy (RT) is essential. We analyzed the treatment results of patients with irradiated ODG to investigate the efficacy of RT in terms of survival rates and other influencing prognostic factors. METHODS AND MATERIALS: Between March 1983 and December 1997, 42 patients with ODG were treated with RT at our hospital. The RT was performed daily at a dose of 1.8~2.0 Gy, at 5 fractions per week, to a total dose of between 39.6 Gy and 64.8 Gy (mean 53.3 Gy). The ages of the patients ranged between 5 and 62 years, with a median age of 39 years. The mean follow-up period was 63.4 months (8-152 months). The Kaplan-Meier method was used to assess the survival, and 5 year survival rates (5-YSR). Log rank tests and Cox regression analyses were used to define the significance of prognostic factors. RESULTS: The majority of ODG in this study were located in the cerebral hemisphere (83.3%). ODG are slightly more common in men than women, and commonly occurs in middle age, between the 3rd and 4th decades. It has been recommended that RT is commenced within 4 weeks following surgery (5-YSR; 86% vs. 49%; p0.05). CONCLUSION: A local involved field irradiation with conventional fractionation, commencing within 4 weeks following surgical excision of the tumor, was beneficial for the 5-YSR, but a total radiation dose exceeding 60 Gy did not improve the 5-YSR.

Takayasu's Arteritis Presented with Subarachnoid Hemorrhage: Report of Two Cases

Takayasu's arteritis is a chronic inflammatory disease that produces a narrowing of the aorta and its major branches. Fibrosis and thickening of the arterial wall often occur in later stages, resulting in a cerebrovascular accident. The authors report two young women patients who presented with subarachnoid hemorrhage (SAH) and occlusive cerebrovasular disease associated with Takayasu's arteritis. Both patients had sudden headache and hemiparesis. Physical examination showed weak radial pulse, carotid bruit, and asymmetrical blood pressure. Erythrocyte sedimentation rate (ESR) was elevated in both patients. SAH was confirmed by brain computerized tomography (CT) or lumbar puncture. Occlusive cerebrovascular disease was diagnosed by brain magnetic resonance imaging (MRI), brain magnetic resonance angiography (MRA), and cerebral angiography. The findings of aortography and cerebral angiography were compatible with Takayasu's arteritis, but intracranial aneurysm was not found in either patient.

Effect of Vascular Endothelial Growth Factor (VEGF) on Neuronal and Glial Response Following Transient Global Ischemia in Rats

OBJECTIVE: The authors present the effect of VEGF upon neuronal and glial response following transient global ischemia of the rat METHODS: We studied the effect of VEGF in 36 rats subjected to 15 minutes of transient global ischemia. Animals were devided into control group(transient global ischemia only: day-3, day-7, day-14, respectively n=6) and VEGF-treated group(transient global ischemia with intraventricular injection of 100 micro gram VEGF: day-3, day-7, day-14, respectively n=6). These animals were sacrificed at 3 days, 7 days and 14 days after induction of ischemia. Nissle stain and immunohistochemistry of GFAP(glial fibrillary acidic protein), OX-42, and ED1 were done for assessment of neuronal and glial responses. RESULTS: In the CA1 hippocampus, there was a significant reduction of pyramidal cell damage in VEGF-treated group as compared with control group in post-ischemia 3, 7, 14 days(p0.05). In the assessment of CA1 hippocampus with GFAP stained areas, there was significant reduction of reactivity in post-ischemia 3, 7 days(p0.05). In the CA3 hippocampus, reduction of GFAP reactivity was significant in post-ischemia 3, 7 days(p0.05). In the assessment of CA1 hippocampus with OX-42 stained areas, there was significant reduction of reactivity in post-ischemia 3, 7, 14 days(p<0.05). But in the CA3 hippocampus, the difference was not significant in post-ischemia 3, 7 days(p<0.05). In the assessment of of CA1 hippocampus with ED1 stained areas, there was significant reduction of reactivity in post-ischemia 3, 7, 14 days(p<0.05). But in the CA3 hippocampus, the difference was significant in post-ischemia 3 days only(p<0.05). CONCLUSION: These results suggest that VEGF can reduce neuronal damage in transient global ischemia, thus have protective effect on ischemic brain injury. In our experiment, the reduction of glial response with VEGF seems to be related to the secondary neuroprotective effect of VEGF. However, the proliferation of endothelial cells and new vessel formation take days to months, the thus neuroprotective effect of VEGF against ischemia seems to related to a certain mechanism rather than angiogenesis.

Syringostomy Using Myringostomy Tube in Syringomyelia Associated Type I Chiari Malformation

We describe a case of syringomyelia associated with type I Chiari malformation treated with syringostomy using myringostomy tube. The syrinx was found at C2 to C5 level, and the patient presented with quadriparesis and both shoulder pain. We performed extensive suboccipital craniectomy, C1 laminectomy, duroplasty, and then syringostomy using myringostomy tube. Postoperatively, the clinical and neurological improvement was noted and MRI showed reduced size of syrinx.

Effects of Sense and Antisense Expression of Protein Kinase Calpha on the Proliferation and Radiosensitization of U-87 Human Glioblastoma Cells

OBJECTIVE: It has been suggested that protein kinase C(PKC) may be one of a number of important regulatory enzymes influencing the tumor cell proliferation and intracellular sensitivity to irradiation. In this study, authors investigate the role of PKC in the growth and radiosensitization of glial brain tumors. METHODS: Human glioblastoma cell line U-87 was stably transfected with sense and antisense complementary deoxyribonucleic acid(cDNA) encoding PKCalpha. The effect of sense and antisense PKCalpha cDNA transfection on PKCalpha expression, PKC activity, cell proliferation, and radiosensitivity of tumor cells was determined. RESULTS: There was no significant difference in cell proliferation between control cells and cDNA(sense and antisense) transfected cells on thiazolyl blue(microculture tetrazolium, MTT) assay. PKC activity was increased by 68% in cells transfected with the sense PKCalpha cDNA(U-87/sPKCalpha), and was reduced by 32% in cells transfected with the antisense PKCalpha cDNA(U-87/aPKCalpha) compared to control cells(p<0.05). Western blotting with a polyclonal antibody against PKCalpha and scanning densitometric analysis of autoradiograms revealed that PKCalpha expression was enhanced by about 5 times of that of control cells in U-87/sPKCalpha cells and was suppressed by more than 30% of that of control cells in U-87/aPKCalpha cells. After exposure to 6 Gy irradiation, cell viability on MTT assay was increased by 43.7% in U-87/sPKCalpha cells and was reduced by 24.3% in U-87/aPKCalpha cells compared to control cells(p<0.001). CONCLUSION: The results of this study demonstrate that PKCalpha overexpression confers a relative radior-esistance and PKCalpha suppression enhances a radiosensitivity on U-87 cells. These observations suggest that PKCalpha plays an important role in regulating cell response to irradiation and a specific modulation of PKCalpha expression in malignant gliomas may influence the radiosensitivity of them.