• Journal of Yeungnam Medical Science
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• v.22 no.2
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• pp.259-265
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• 2005

Central diabetes insipidus (DI) is a syndrome characterized by thirst, polydipsia and polyuria. Langerhans cell histiocytosis is one of the etiologies of DI. Recently we experienced a central DI associated with Langerhans cell histiocytosis. The 44 years old female patient complained right hip pain, polydipsia and polyuria. We carried out water deprivation test. After vasopressin injection, urine osmotic pressure was increased from 109 mOsmol/kg to 327 mOsmol/kg (300%). Brain MRI showed a thickened pituitary stalk and air bubble like lesions sized with 5cm, 7cm was shown on fifth L-spine and right hip bone at hip bone CT. CT guided biopsy revealed abnormal histiocytes proliferation and abundant lymphocytes. The final diagnosis was central DI associated with systemic Langerhans cell histiocytosis invading hip bone, L-spine and pituitary stalk. Desmopressin and etoposide chemotherapy were performed to the patient.

• Clinical and Experimental Pediatrics
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• v.58 no.7
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• pp.270-273
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• 2015

Legg-Calve-Perthes (LCP) disease is characterized by idiopathic avascular osteonecrosis of the epiphysis of the femur head. The main factor that plays a role in the etiology of the disease is decreased blood flow to the epiphysis. Many predisposing factors have been suggested in the etiology of LCP disease, and most have varying degrees of effects. Here we present the case of a boy aged 4 years and 10 months with complaints of short stature and a diagnosis of multiple hypophyseal hormone deficiency, in whom LCP disease and difficult birth-related pituitary stalk interruption syndrome were identified by anamnesis. The present case revealed that LCP disease and hypophyseal hormone deficiency could be secondary to difficult birth and that LCP disease could be secondary to insulin-like growth factor 1 deficiency. Additionally, to the best of our knowledge there is no published case on the relation between LCP disease and insulin-like growth factor 1 deficiency. Therefore, we believe that this case is worthy of presentation.

• Clinical and Experimental Pediatrics
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• v.50 no.11
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• pp.1110-1115
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• 2007

Purpose : Idiopathic central diabetes insipidus (CDI) is defined in CDI patients without definite etiology. Some patients initially diagnosed as idiopathic CDI progressed to organic causes. We reviewed clinical, endocrinological, and radiological courses of 20 patients who was initially diagnosed as idiopathic CDI, to assess the predicting factors for progression to brain tumors. Methods : We reviewed the medical data and followed up their clinical courses in 20 CDI patients who had no definite organic etiology, such as malformation, tumor, at the time of diagnosis. Results : Our study included 15 males and 5 females. Mean age of CDI diagnosis was $7.8{\pm}3.6$ (2.1-14.7) years. Mean follow-up duration was $8.6{\pm}5.1$ (1.5-18) years. Six (30%) patients were diagnosed as brain tumor during follow-up. Ten (50%) of 20 patients had growth hormone deficiency. Multiple pituitary hormone deficiencies were found more frequently in brain tumor patients than idiopathic patients (60% vs 7%, P=0.037). Pituitary stalk thickening (PST) and loss of posterior pituitary signal were observed in 9 patients (47%), respectively. The newly development of PST was observed in patients diagnosed as brain tumor. Conclusion : About 30% of idiopathic CDI patients progress to organic disease such as germ cell tumor or histiocytosis. If there are multiple anterior pituitary hormone deficiency or newly development of PST, more close and careful follow-up is needed.

• Journal of Korean Neurosurgical Society
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• v.30 no.5
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• pp.561-566
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• 2001

Objective : The purpose of this study are to evaluate the effectiveness of Gamma Knife radiosurgery(GKS) as a treatment of craniopharyngioma and to investigate the proper dose planning technique in GKS for craniopharyngioma. Method : Between May 1992 and March 1999, seven Gamma Knife radiosurgical procedures were done for residual tumor mass of 6 patients with craniopharyngioma after microsurgical resection. Conventional radiation therapy was not performed. In this study, their clinical, radiological and radiosurgical data were analyzed and the radiation dosage to the optic pathway, hypothalamus, pituitary stalk, and cavernous sinus were calculated and correlation with clinical outcome was evaluated. The mean follow-up period was 33.5 months(12.3-55.2 months). Result : The mean tumor volume was 4.4cc(0.4-18.0cc) and the maximum radiation dose ranged from 14 to 32 Gy(mean 20.9Gy). The radiation was given with isodose curve, 50-90% and the marginal dose varied within 8-22.4Gy(mean 12.7Gy). The mean number of isocenter was 4.3(1-12). The tumor was well controlled in all cases. In 5 of 7 cases, the size of tumor decreased to 10-50% of pre-GKS volume and remaining two showed no volume change. The mean dose to optic pathway was 5.7Gy(5.1-11.2Gy) and there were no complications. Conclusion : GKS seems to be effective for control of craniopharyngioma as an adjuvant treatment after microsurgical resection and even suboptimal dose for tumor margin is considered to be enough for tumor control. It is safe with careful dose planning to protect surrounding important structures, especially optic pathway. We believe conventional radiation therapy should be avoided because it has limitation for dose planning of additional treatments such as radiosurgery or intracystic instillation of radioisotope in case of recurrence.

• Journal of Korean Neurosurgical Society
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• v.39 no.5
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• pp.329-334
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• 2006

Objective : In selected cases, the transsphenoidal approach[TSA] can be extended anteriorly to the tuberculum sellae, chiasmatic sulcus, and planum sphenoidale to obtain direct exposure of the suprasellar cisterns and its contents. We applied this modification of the TSA to various lesions of the presellar and suprasellar areas. We evaluate our clinical experience of this technique and review the related literature. Methods : From 1999 to 2004, we used the transsphenoidal supradiaphragmatic intradural approachs[TSIAs] in 9 patients who had various lesions at the pre- and suprasellar regions. Concomitant presellar extension of the bone window was performed with the sublabial or transnasal transseptal transphenoidal techniques. After removal of the lesions, sellar or anterior cranial floor was repaired with silicone plate substitute. Results : The TSIAs have been applied in the following cases : four tuberculum sellae meningiomas, two craniopharyngiomas, two Rathke's cleft cysts, and one non-functioning macroadenoma. The complications were one case of visual acuity decrease and one cerebrospinal fluid rhinorrhea. Conclusion : The TSIA is easily applicable through a minor modification of the standard TSA. It is suitable for removing lesions located in the presellar and suprasellar area adjacent to the pituitary stalk with minimal brain manipulation and decreased morbidity.

• Journal of Korean Neurosurgical Society
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• v.30 no.7
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• pp.849-854
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• 2001

Delayed cerebral infarction after resection of craniopharyngioma is a uncommon entity, but leads to high morbidity and mortality. We present 3 such cases and discuss the etiology and pathogenesis with review of pertinent literatures. In our cases, delayed deterioration of consciousness was observed in all cases. All of them expired. The cause may be multifactorial and the surgical approach may contribute to the pathogenesis of delayed cerebral infarction. We suspect vasospasm might be the major mechanism of pathogenesis. Vessels were primed to spasm during operation due to blood in the cistern or mechanical injury. Vasoactive materials may have been liberated from the pituitary stalk or injured hypothalamus, either at the time of surgery, or later, after portions of tumor have undergone necrosis. The high degree of suspicion to detect vasospasm should be done in the case of the delayed deterioration of mental status at an early stage of craniopharyngioma surgery. Possible mechanism underlying this delayed cerebral infarction are discussed.