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Background: Achondroplasia is an autosomal dominant disorder mainly affecting bony growth, typically resulting in markedly short stature. From a neurosurgical viewpoint, patients sometimes develop spinal cord compression at the narrowed foramen magnum and hydrocephalus. This study aims to construct growth references for height, weight, and head circumference (HC) of young achondroplasia patients in Korea and to evaluate the predictability of the necessity and timing of neurosurgical procedures through growth patterns., Methods: Growth data were collected from achondroplasia patients who visited our institution between January 2002 and August 2022. First, we constructed percentile growth curves of height, weight, and HC for the patients under 3 years of age with the generalized additive model for location, scale, and shape (GAMLSS). Second, the growth patterns of the patients with hydrocephalus who underwent neurosurgical procedures such as foramen magnum decompression (FMD) and ventriculoperitoneal (VP) shunt were analyzed., Results: There were 125 achondroplasia patients, including 67 males and 58 females. Among 125 patients, 46 underwent FMD, and 5 underwent VP shunt. As short stature and macrocephaly were typical characteristics of achondroplasia, the height of achondroplasia was lower than that of the general population, and HC in achondroplasia showed accelerated growth postnatally. There were no significant changes in HC in hydrocephalus patients before they underwent neurosurgical procedures. The influence of hydrocephalus on the growth patterns of HC in achondroplasia seemed insignificant., Conclusion: Growth references for height, weight, and HC in young achondroplasia patients were constructed. It is the first report of growth patterns of achondroplasia in Korea. Unlike other pediatric patients, the diagnosis of hydrocephalus and the necessity of neurosurgical procedures are hard to be predicted with HC in achondroplasia. Neuroimaging should be considered for achondroplasia patients with neurological symptoms., (© 2023. Institut National de la Santé et de la Recherche Médicale (INSERM).)

Object: Intracranial germ cell tumors (GCTs) frequently present with bifocal lesions in both the suprasellar and pineal areas. The pathogenesis of these bifocal GCTs has been the subject of controversy. Bifocal GCTs may be caused by synchronous tumors or by metastatic spread of tumor cells from one site to the other. The prognosis associated with bifocal GCTs has also been a cause of concern., Methods: The authors constructed a single-institution patient cohort comprising 181 patients with intracranial GCTs. The clinical characteristics of bifocal GCTs were compared with those of suprasellar and pineal GCTs., Results: Bifocal GCTs were observed in 23 patients (12.8%). Eighteen patients presented with bifocal GCTs that were diagnosed as germinomas, but 5 patients exhibited mixed GCTs. Analyses of age distributions and comparisons of tumor sizes were compatible with a model of a metastatic origin of bifocal GCTs. Eleven patients (47.8%) presenting with bifocal GCTs exhibited tumor seeding at presentation. Tumor seeding was significantly associated with bifocal lesions (p < 0.001). Patients with bifocal germinomas showed significantly shorter event-free survival and overall survival than did those presenting with germinomas from a single site of origin., Conclusions: Bifocal GCTs are not restricted to germinomas, as had been previously reported, but do include mixed GCTs. The authors hypothesize that bifocal GCTs may result from the metastatic spread of suprasellar or pineal GCTs. The bifocal presentation of germinomas may be a poor prognostic sign and should alert clinicians to the possibility of a disseminated disease.

Objective: To evaluate the chronologic trends in brain tumor surgery at a single institution over 53 years (1957-2009)., Methods: Data were collected from medical records and documents (eg, daily records, surgical notes, or electronic databases) preserved in the library of the Department of Neurosurgery, Seoul National University College of Medicine., Results: During the period 1957-2009, 10,009 brain tumors were surgically treated. Glial tumor (25.2%) was the most frequently reported histologic category. Meningioma and pituitary adenoma accounted for 17.5% and 17.1% of the histologies. In children <20 years old, astrocytoma (16.7%), embryonal tumor (16.2%), and germ cell tumor (11.9%) were the most common histologies. The chronologic trend for changes in proportions of individual brain tumor surgeries showed that cases of glioma have gradually increased since the 1980s, whereas cases of benign extra-axial tumors, including meningioma, sellar tumor, and schwannoma, have decreased. This trend implies that the presurgical diagnosis of glioma has increased owing to modern imaging technologies, and alternative treatment options, such as radiosurgery or observation, have been more frequently applied over time to benign extra-axial tumors. Simultaneously, the surgical management of lymphoma and metastatic tumor has increased gradually., Conclusions: This article describes the largest series of chronologic analysis of brain tumor surgeries conducted at a single institution and reflects the longitudinal trends by treatment option in incidences of selected brain tumors., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Established in 1957, the Department of Neurosurgery at Seoul National University College of Medicine is the one of the oldest neurosurgical departments in Korea. The seven past Chairmen (Bo Sung Sim, Kil Soo Choi, Dae Hee Han, Byung-Kyu Cho, Hyun Jib Kim, Hee-Won Jung, and Dong Gyu Kim) have devoted themselves to the development of the department. The current chair, Chun Kee Chung, assumed the position in July 2010. The current department comprises several clinical programs that encompass the entire spectrum of neurosurgical disorders, with 29 specialized faculty members and care teams in three hospitals: Seoul National University Hospital (SNUH), Boramae Medical Center (BMC), and Seoul National University Bundang Hospital (SNUBH). The remarkable growth of the department during the last half century made it possible to perform 5,666 operations (3,299 at SNUH, 411 at BMC and 1,860 at SNUBH) during 2009. A total of 1,201 articles authored by faculty members were published in scientific journals between 1958 and 2009, approximately 32% of which were published in international journals. The department is regarded as the "Mecca" of neurosurgery in Korea because of its outstanding achievement and the many distinguished alumni with leadership roles in the academic field. This article traces the clinical, academic, and scientific development of the department, its present activities, and its future direction., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Background: Clinical research on moyamoya disease (MMD) has focused on symptomatic outcome such as transient ischemic attacks. Neurocognitive function in children critically affects social outcome and is closely related to quality of life. This study is the first to analyze the neurocognitive profiles of children with MMD before and after surgery., Methods: Sixty-five patients were selected out of 137 who underwent surgery for MMD between 2006 and 2008. The preoperative and postoperative neurocognitive function was tested using the Korean version of the Wechsler Intelligence Scale for Children-Revised (KEDI-WISC-R) and the Bender Gestalt Test (BGT). Pre- and postoperative profiles of patients with or without major infarctions were compared. Patients with borderzone infarctions were analyzed as well., Results: Preoperatively, patients had age-appropriate full-scale intelligence quotient (FSIQ) and verbal IQ (VIQ) scores, which were maintained after surgery. There was significant improvement in performance IQ (PIQ) (p = 0.01) and BGT scores postoperatively (p < 0.01). Among the subtests, Coding showed significant improvement postoperatively (p < 0.01). Preoperatively, patients with major infarctions had significantly lower FSIQ (p < 0.01), VIQ (p = 0.01) and PIQ (p < 0.01) scores compared with those without infarctions. The pre- and postoperative neurocognitive profiles of the patients with borderzone infarctions fell between those of patients with absolutely no infarctions and those of patients with major infarctions., Conclusions: Considering the natural history of MMD, which leads to a drastic decline in neurocognitive functions, the present findings indicate a role for early active surgery to save the intellectual abilities of children with MMD., (Copyright © 2010 S. Karger AG, Basel.)