Intracystic bleomycin (ICB) administration is known to be effective in the treatment of cystic craniopharyngiomas (CRPs) and rarely causes serious complications. The authors report a case of vasculopathy after ICB injection for a recurrent cystic CRP. A 5-year-old boy presented with the cystic recurrence of a CRP. A catheter and Ommaya system were inserted into the cyst, followed by a leakage test 2 weeks later. Bleomycin was delivered at 3 mg per week for 14 weeks (total dose 42 mg). Four months later, the patient's activity decreased and right hemiparesis occurred. Magnetic resonance imaging and cerebral angiography demonstrated a recurrent multicystic mass and infarction at the left middle cerebral artery territory with stenosis/occlusion of the left distal internal carotid artery and posterior cerebral artery. During the operation to remove the recurrent cystic mass, fibrotic stenoocclusion of the left intracranial arteries was identified. The cyst was totally removed. However, additional multiple border zone infarctions occurred in the left hemisphere, and the patient became wheelchair bound. Leakage of ICB was considered as the most probable cause of the cerebral vasculopathy and corresponding infarction. A leakage test may not always guarantee a leakage-free status even if the test is negative. Direct bypass surgery should have been considered to prevent a high risk of postoperative infarction in this case of a recurrent CRP with confirmed vasculopathy.
Won-Sang Cho, Seung-Ki Kim, Kyu-Chang Wang, Ji Hoon Phi, and Byung-Kyu Cho
Hyunho Choi, Ji Yeoun Lee, Ji Hoon Phi, Seung-Ki Kim, Byung-Kyu Cho, and Kyu-Chang Wang
Postoperative epidural hematoma (EDH), a blood collection between the inserted galeal flap and the overlying skull flap (epigaleal flap hematoma), is a frustrating complication of the surgical treatment of moyamoya disease (MMD) in pediatric patients. The symptoms of postoperative EDH are often similar to those of postoperative cerebral ischemia, and may cause confusion during clinical decision making. The authors designed this study to evaluate the incidence, clinical presentation, risk factors, and treatment outcomes of postoperative EDH in pediatric patients with MMD.
A retrospective review of 148 pediatric patients with 250 craniotomies who underwent indirect bypass revascularization surgery between January 2002 and December 2006 was performed. This group consisted of 60 male and 88 female patients, and the mean age at surgery was 7.5 years (range 1–18 years).
Of the 250 craniotomies, postoperative EDH was detected in 32 cases. In 12 cases of EDH, surgical treatment was necessary (4.8% of 250 craniotomies). During the same period, 743 non-MMD craniotomy operations were performed. In 6 of these 743 operations, patients developed postoperative EDH that required surgical treatment, significantly less than the percentage of EDH requiring postoperative treatment in patients with MMD (0.8%, p < 0.001). The average interval between craniotomy surgery and the detection of EDH was 1.8 days (range 0–5 days) in patients with MMD and 0.5 days (range 0–2 days; p = 0.018) in the non-MMD craniotomy group.
Postoperative EDH was observed in significantly fewer cases (17 of 191) when a subcutaneous drain (SCD) was inserted over the bone flap than in cases in which an SCD was not inserted (14 of 55; p = 0.001). The mean international normalized ratio of prothrombin time in the immediate postoperative blood test was 1.27 ± 0.17 in the EDH group and 1.20 ± 0.11 in the non-EDH group (values are expressed as the mean ± SD; p = 0.008). Central galeal flap tenting suture and immediate postoperative platelet count were not related to the incidence of postoperative EDH following pediatric MMD surgery.
Postoperative EDH is more likely to be found following craniotomy in patients with MMD than in those without MMD, and may occur in a delayed fashion. Insertion of an SCD and immediate correction of an abnormal value for international normalized ratio of prothrombin time can decrease the incidence of postoperative EDH following surgery for pediatric MMD.
Kyung Hyun Kim, Ji Yeoun Lee, Ji Hoon Phi, Seung-Ki Kim, Byung-Kyu Cho, and Kyu-Chang Wang
The surgical indications for some arachnoid cysts (ACs) are controversial. While surgical procedures can be effective when an AC is a definite cause of hydrocephalus or papilledema, most ACs do not cause any symptoms or signs. Some surgeons perform several procedures to treat ACs because of their large size. The purpose of this study was to compare the long-term outcomes of Galassi type III ACs between surgery and nonsurgery groups.
The medical records of 60 patients diagnosed with sylvian ACs (Galassi type III) who visited Seoul National University Children’s Hospital from July 1990 to March 2018 were analyzed. The authors compared the outcomes between those treated with surgery and those not treated with surgery.
Of the 60 patients, 27 patients had no symptoms, 19 patients had vague symptoms and signs associated with ACs, and the remaining 14 patients had definite AC-related symptoms and signs. Thirty-eight patients underwent surgery, and 22 patients underwent observation. Some operations were accompanied by complications. Among the 33 patients in the surgery group, excluding 5 with hydrocephalus or papilledema, 8 patients needed 18 additional operations. However, there were no patients in the nonsurgery group who needed surgical intervention during the follow-up period (mean 67.5 months), although the size of the AC increased in 2 patients. Changes in AC size were not correlated with symptom relief.
When patients with hydrocephalus or papilledema were excluded, there was no difference in the outcomes between the surgery and nonsurgery groups regardless of the size of the sylvian AC. Surgeons should be cautious when deciding whether to operate.
Ho Jun Seol, Kyu-Chang Wang, Seung-Ki Kim, Yong-Seung Hwang, Ki Joong Kim, and Byung-Kyu Cho
Patients with moyamoya disease complain of headache before surgery, after surgery, or in both periods. To clarify the pathophysiological features of these headaches which are one of the major symptoms in moyamoya disease after indirect bypass surgery, the authors reviewed data obtained in patients with moyamoya disease who underwent such surgery at their institute.
The authors surveyed 204 children with moyamoya disease younger than 17 years of age who underwent indirect bypass surgery consecutively between 1988 and 2000. If headache and associated symptoms disturbed their daily activity, required rest and/or medication, and occurred at least once a month, the headache was regarded as significant and the patient was included in the study. A postoperative headache was defined as the presence of significant headache 12 months after the operation or later. Preoperative headache was documented in 44 patients. Postoperative headache was observed in 63% (28 of 44) of the patients with preoperative headache and in 6.3% (10 of 160) of those without preoperative headache. Aggravation on postoperative magnetic resonance images or single-photon emission computerized tomography scans did not correlate with this symptom (p = 0.729 and 0.203, respectively). The clinical course had the following features: 1) a coexisting stage of headache and transient ischemic attack; 2) a second stage of headache only; and 3) a final stage of improvement or disappearance of headache.
The authors demonstrated that headaches can persist or develop after indirect bypass surgery despite successful prevention of cerebral ischemia. In addition to decreased cerebral blood flow, progressive recruitment and redistribution of blood flow should be considered as a cause of headaches in patients with moyamoya disease.
Ji Hoon Phi, Seung-Ki Kim, Sung-Hye Park, Seok Ho Hong, Kyu-Chang Wang, and Byung-Kyu Cho
Immature teratomas of the central nervous system (CNS) are rare neoplasms. Although adjuvant therapy is generally recommended after resection, the exact role of each therapeutic modality is not yet established. The purpose of this study was to analyze the clinicopathological correlation and the role of resection to define the optimal treatment modalities for immature teratomas of the CNS.
Between 1987 and 2002, eight patients underwent radical surgery for a lesion diagnosed as a CNS immature teratoma at the authors' institution. The clinical courses of these patients and the pathological features of their tumors were retrospectively reviewed.
Gross-total resection (GTR) was achieved in six patients at the initial operation. The mean follow-up period was 75 months. Two patients received postoperative adjuvant therapies and two patients did not, against medical advice. None of the four patients experienced recurrence after long-term follow up. Another four patients, all of whom underwent GTR of the tumor, did not receive adjuvant therapy as part of a prospective treatment scheme. One of them exhibited early recurrence and metastasis. The tumor had pathological features denoting a high-grade (Norris Grade III) lesion and neurocytomatous differentiation.
Aggressive resection seems to be of utmost importance in the treatment of immature teratomas of the CNS. Adjuvant chemotherapy and radiotherapy can be deferred if GTR is achieved in low-grade, immature teratomas, but adjuvant therapies may be warranted for high-grade ones.
Ji-Woong Kwon, Byung-Kyu Cho, Eui Chong Kim, Kyu-Chang Wang, and Seung-Ki Kim
A 13-year-old girl exhibited rapid deterioration in mental status 15 days after surgery for craniopharyngioma. Serial CT scanning detected progression of a low-density lesion on the left frontotemporal lobe. The serum level of C-reactive protein was elevated and polymerase chain reaction identified herpes simplex virus DNA in the cerebrospinal fluid. Antiviral therapy with high-dose acyclovir (10 mg/kg 3 times daily) was begun. She recovered and could speak short sentences, but dysphasia and right hemiparesis remained. Early diagnosis and active treatment of herpes simplex encephalitis is essential for improving prognosis and saving lives.
Chang Sub Lee, Ji Hoon Phi, Seung-Ki Kim, Byung-Kyu Cho, and Kyu-Chang Wang
Congenital dermal sinus (CDS) usually develops in the midline of the body as a single tract. To date, only a few patients with multiple CDS tracts and ostia have been reported. The authors analyzed the clinical features of patients with spinal CDS and multiple ostia and proposed a novel hypothesis for the pathogenesis of the atypical CDS.
Five patients with spinal CDS and multiple ostia were included. The clinical, radiological, and operative features of these patients were reviewed retrospectively.
Three patients demonstrated bilateral paramedian ostia at the same or a very similar spinal level. One patient showed a paramedian and a midline ostium. One patient had 2 unilateral paramedian ostia at different spinal levels. The layers of the internal ending of CDS tracts were diverse. Complete removal of the tracts was possible in all patients. Two patients had dermoid tumors. All patients had an associated anomaly, such as a lumbosacral lipoma or the Currarino triad. The authors propose a “zipping error” hypothesis for the formation of dual ostia located at the spinal level of primary neurulation. An associated anomaly such as a lumbosacral lipoma may contribute to the formation of dual ostia.
Unilateral or bilateral dual ostia may be present in patients with CDS, especially when an associated anomaly is present. The atypical CDS may develop from aberrant neural tube closure.
Won-Sang Cho, Seung-Ki Kim, Sung-Hye Park, and Byung-Kyu Cho
Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor of infancy and early childhood that occurs mainly in soft tissue and the retroperitoneum. The pathological characteristics of a KHE are fascicles of spindleshaped endothelial cells and slitlike vascular channels with irregular tumor margins. In spite of benign features such as rare mitoses and a low MIB-1 labeling index, a KHE is categorized as an intermediate malignancy because of local invasiveness into adjacent lymph nodes or organs. Resection is the treatment of choice for this type of tumor and adjuvant medical therapy is prepared for the tumor remnants and the associated Kasabach-Merritt syndrome. In this paper the authors report a case of a KHE without Kasabach-Merritt syndrome arising from the right tentorium cerebelli in a 13-month-old male child. The biological behavior of the tumor, including its pathological traits and clinical course, was malignant in nature. This case may be not only the first intracranial extraaxial KHE, but also the first truly malignant variant. Kaposiform hemangioendothelioma should be considered in the differential diagnosis of intracranial and extraaxial tumors.
New prognostic factors for adjacent-segment degeneration after one-stage 360° fixation for spondylolytic spondylolisthesis: special reference to the usefulness of pelvic incidence angle
Invited submission from the Joint Section Meeting on Disorders of the Spine and Peripheral Nerves, March 2007
Jeong Yoon Park, Yong Eun Cho, Sung Uk Kuh, Jun Hyung Cho, Dong Kyu Chin, Byung Ho Jin, and Keun Su Kim
The purpose of this study was to evaluate the correlation between adjacent-segment degeneration (ASD) and pelvic parameters in the patients with spondylolytic spondylolisthesis. Sagittal balance is the most important risk and prognostic factor in the development of ASD. The pelvic incidence angle (PIA) is an important anatomical parameter in determining the sagittal curvature of the spine and in the individual variability of the sacral slope and the lordotic curve. Thus, the authors evaluated the relationship between the pelvic parameters and the ASD.
Among 132 patients with spondylolytic spondylolisthesis who underwent surgery at their institution, the authors selected patients in whom a one-stage, single-level, 360° fixation procedure was performed for Grade I spondylolisthesis and who underwent follow-up for more than 1 year. Parameters in 34 patients satisfied these conditions. Of the 34 patients, seven had ASD (Group 1) and 27 patients did not have ASD (Group 2). The investigators measured degree of spondylolisthesis, lordotic angle, sacral slope angle (SSA), pelvic tilt angle (PTA), PIA, and additional parameters pre-and postoperatively. The radiographic data were reviewed retrospectively.
The population consisted of nine men and 25 women whose mean age was 48.9 ± 9 years (± standard deviation) (range 28–65 years). Seven patients developed ASD after undergoing fusion. Of all the parameters, pre-and postoperative degree of spondylolisthesis, segmental lordosis, lordotic angle, SSA, preoperative PTA, and pre-operative PIA did not differ significantly between the two groups; only postoperative PTA and PIA were significantly different.
The development of ASD is closely related to postoperative PIA and PTA, not preoperative PIA and PTA. The measurement of postoperative PIA can be used as a new indirect method to predict the ASD.
So-Hyang Im, Chang Wan Oh, O-Ki Kwon, Byung-Kyu Cho, Young-Seob Chung, and Dae Hee Han
Object. Involuntary movement is an uncommon manifestation of a transient ischemic attack. It may be induced by cerebral hemodynamic insufficiency, which is associated with several cerebral ischemic diseases. The authors present three cases of limb shaking due to moyamoya disease (MMD) or radiation-induced middle cerebral artery stenosis, and three additional cases of choreic movement due to MMD. Neuroimaging studies and surgical outcomes in these patients were retrospectively analyzed to investigate the pathological mechanism underlying the symptoms and to provide guidance for the management of involuntary movement disorders in cases of ischemic cerebral disease.
Methods. The patient population included two children and four adults with ages at presentation ranging between 7 and 50 years. The initial presenting symptoms were involuntary movements in all six cases. A magnetic resonance imaging finding common in all cases was a small infarct in the frontal corona radiata, which did not extend to the cortex or basal ganglia. A perfusion defect in the frontoparietal cortical and subcortical regions was demonstrated by singlephoton emission computerized tomography in all patients. Improved hemodynamic circulation in the frontoparietal cortical and subcortical regions occurred in parallel with clinical improvement following indirect or direct bypass surgery.
Conclusions. Ischemic dysfunction of the frontal cortical and subcortical motor pathways rather than that of the basal ganglia was suspected to be the cause of the observed contralateral involuntary movements. Direct and indirect bypass surgery can be used effectively to treat involuntary movements in patients with cerebral ischemic diseases such as MMD and in those with stenosis of an intracranial major artery.