Effectiveness of C1 Laminectomy for Chiari Malformation Type 1: Posterior Fossa Volume Expansion and Syrinx-Volume Decrease Rate

Purpose@#This study aimed to analyze the effect of foramen magnum decompression with C1 laminectomy (C1L) for Chiari malformation type 1 (CM-1) in terms of improving clinical symptoms, expanding posterior fossa volume, and decreasing syrinx volume. @*Materials and Methods@#Between January 2007 and June 2019, 107 patients with CM-1 were included. The median patient age was 13±13 years (range: 9 months–60 years), female-to-male ratio was 1:1, and average length of tonsil herniation was 13±5 mm (range: 5–24 mm). Surgical techniques were divided into four groups based on duraplasty or C1L usage. Among the study subjects, 38 patients underwent duraplasty and had their syrinx volumes measured separately on serial magnetic resonance imaging. A three-dimensional visualization software was used to evaluate the syrinx-volume decrease rate. @*Results@#Bony decompression exhibited a mere 20% volume expansion of the lower-half posterior fossa. C1L offered a 3% additional volume expansion, which rose to 5% when duraplasty was added (p=0.029). There were no significant differences in complication rate when C1L was combined with duraplasty (p=0.526). Syrinx volumes were analyzed in 38 patients who had undergone duraplasty. Among them, 28 patients who had undergone duraplasty without C1L demonstrated a 5.9% monthly decrease in syrinx volume, which was 7.5% in the remaining 10 patients with C1L (p=0.040). @*Conclusion@#C1L was effective in increasing posterior fossa volume expansion, both with and without duraplasty. A more rapid decrease in syrinx volume occurred when C1L was combined with duraplasty.

From Resection to Disconnection for Seizure Control in Pediatric Epilepsy Children

Epilepsy surgery revealed dramatically improved seizure outcomes over medical therapy in drug-resistant epilepsy patients. Children with epilepsy, however, have multiple epileptic focuses which require multilobar resection for better seizure outcome. Multilobar resection has not only the several severe surgical complications, such as hydrocephalus and shunt-related craniosynostosis, due to intracranial volume reduction. Isolation method (disconnection surgery) was progressively studied over epileptic focus removal (resective surgery) for seizure control. This concept was first introduced for functional hemispherotomy, and its primary principle is to preserve the vital vascularized brain that is functionally disconnected from the contralateral healthy brain. Currently in most epilepsy centers, the predominant disconnection surgical methods, including functional hemispherotomy, are continually being refined and are showing excellent results. They allow the functional isolation of the hemisphere or multi-lobe, affected by severe epilepsy. This review describes recent findings concerning the indication, surgical technique, seizure outcome and complications in several disconnection surgeries including the functional hemispherotomy for refractory pediatric epilepsy.

From Resection to Disconnection for Seizure Control in Pediatric Epilepsy Children

Epilepsy surgery revealed dramatically improved seizure outcomes over medical therapy in drug-resistant epilepsy patients. Children with epilepsy, however, have multiple epileptic focuses which require multilobar resection for better seizure outcome. Multilobar resection has not only the several severe surgical complications, such as hydrocephalus and shunt-related craniosynostosis, due to intracranial volume reduction. Isolation method (disconnection surgery) was progressively studied over epileptic focus removal (resective surgery) for seizure control. This concept was first introduced for functional hemispherotomy, and its primary principle is to preserve the vital vascularized brain that is functionally disconnected from the contralateral healthy brain. Currently in most epilepsy centers, the predominant disconnection surgical methods, including functional hemispherotomy, are continually being refined and are showing excellent results. They allow the functional isolation of the hemisphere or multi-lobe, affected by severe epilepsy. This review describes recent findings concerning the indication, surgical technique, seizure outcome and complications in several disconnection surgeries including the functional hemispherotomy for refractory pediatric epilepsy.

Late-Developing Metastatic Malignant Melanoma in the Thoracic Spine Originating from Choroidal Melanoma

A 54-year-old woman visited Gangnam Severance Hospital for left side flank pain. She had a history of total removal of malignant melanoma on the left eye ball 20 years prior. No evaluation had been performed since then. A paravertebral mass at thoracic ninth level (T9) was discovered on spinal magnetic resonance imaging, and pathology confirmed malignant melanoma. Following positron emission tomography-computed tomography, no other metastasis was discovered. After removal of the paravertebral mass, palliative chemotherapy (dacabarzine + tamoxifene) was administered in 3 cycles over 2 months. Radiotherapy with simultaneous integrated boost technique was performed at 4,350 cGy total over 15 days, 290 cGy per delivery, and was administered with the first cycle of palliative chemotherapy. Despite this treatment, multiple metastases developed throughout her body 7 months later, and the patient is continuing chemotherapy.

The Relationship of Serum Angiotensin Converting Enzyme (ACE), Angiotensin II and Clinical Markers in the Idiopathic Interstitial Pneumonia / 결핵

BACKGROUND: There have been several studies showing that angiotensin II and the angiotensin convertingenzyme (ACE) contribute to the activation of fibroblast including the pulmonary fibrosis, and apoptosis of the al veolar epithelium in idiopathic intersititial pneumonia. This study was performed to identify the relationship between the serum angiotensin II. ACE and the pulmonary function test (PFT), the dyspnea score, and the cell fraction of the bronchoalveolar lavage fluid (BALF). METHODS: Twenty three patients with idiopathic interstitial pneumonia from March, 1999 to October, 2001 at Gachon medical school were enrolled in this study. They were divided into IPF(UIP) (16) and NSIP (7) group. Twelve of the idiopathic interstitial pneumonia patients (UIP : 5, NSIP: 7) were diagnosed by an open lung biopsy, 11 of IPF patients were diagnosed by the American Thoracic Society (ATS) diagnostic criteria. The PFT values, dyspnea score, serum ACE and angiotensin II were measured, and a bronchoscopy was performed to obtain the BALF. RESULTS: Of all the patients, 7 were in the normal range and 14 showed an increase in the serum level of angiotensin II. In terms of the serum ACE level, 14 patients had an increased level. The DLCO% of the angiotensin II. increased group was significantly lower than the not-increased group (p=0.021). Other factors did not correlate with the serum ACE or the angiotensin II increased group and not-increased group. CONCLUSION: These results suggest that an increased angiotensinII serum level may be associated with in crease in the of alveolar capillary block in the progression of pulmonary fibrosis in idiopathic interstitial pneumonia.

Two Cases of Microscopic Polyangiitis with Honeycomb Lung / 결핵

Microscopic polyangiitis is a systemic small-vessel vasculitis that is associated primarily with necrotizing glomerulonephritis and pulmonary capillaritis. A recurrent and diffuse alveolar hemorrhage due to pulmonary capi llaritis is the main clinical manifestation of lung involvement. Recently, an interstitial lung disease that mimics idiopathic pulmonary fibrosis was reported to be rarely associated with microscopic polyangiitis. Here we report two patients with microscopic polyangiitis who showed a honeycomb lung at the time of the initial diagnosis with a brief review of relevant literature.

A Case Intestinal Mucormycosis Healing of Ulcer after Only Amphotericin B Treatment / 대한소화기내시경학회지

Mucormycosis is an uncommon, frequently fatal, opportunistic fungal infection and usually occur in immunocompromised patients. Rhinocerebral and pulmonary disease are the common forms but intestinal infection is an extremely rare form of the disease. Invasive intestinal mucormycosis is usually fatal, therefore, few cases reported survival after early diagnosis and surgical resection combined with antifungal treatment. Because of high mortality after surgery, the healing of ulcers caused by intestinal mucormycosis has not previously observed in immunocompromised host. We reported a case of the intestinal mucormycosis that had been healed the ulcer and symptomatic improvement after only amphotericin B.

A Study of Clinical Investigations of Pulmonary Tuberculoma / 결핵

BACKGROUND: A pulmonary tuberculoma is one of the most common causes of a solitary pulmonary lesion. Treating a tuberculoma is still controversial and there are few reports on antituberculosis chemotherapy. In this study, the clinical findings and changes in the size of tuberculomas on a radiograph after completing antiuberculosis chemotherapy was investigated. METHODS: The medical records, an chest radiographs of 18 pulmonary tuberculoma patients who were admitted to the Gachon medical school, Ghil medical center between April 1998 and August 2001, were reviewed. The symptomatic changes were recorded and the size of the tuberculomasl following treatment were compared. To compare the size, the long distance of each tuberculoma on the chest radiographs were measured and the additional radiological findings of calcification, satellite nodules and cavities were investigated. RESULTS: Fifteen patients were men and 3 were women. The median age was 46(24-74). Among these 18 patients, 14 patients had clinical symptoms. The other 4 patients were diagnosed incidentally as during a routine chest radiograph. The mean size of the tuberculomas on the initial plain chest film was 4.3+/-2.3cm(range : 1.7-10cm)and after 6 months treatment, it had decreased to 1.68+/-2.00cm(range : 1.5-6.5cm)(P<0.05). At least 6 months of antituberculosis chemotherapy resulted in the findings of a tuberculoma with a disappearance in 9, a decreased size in 4, and no change in 5 on the chest radiograph. Calcifications were found in 3 patients on the initial chest film and the chest CT and all calcified tuberculomas had disappeared after treatment. CONCLUSIONS: A lthough a pulmonary tuberculoma can remain as an inactive lesion for a long time, if it is confirmed by pathological or bacteriological methods, antituberculosis chemotherapy will be beneficial despite the presence of calcification.

Effect of Inhaled Fluticasone Propionate on Hypothalamic-Pituitary-Adrenal Axis in Bronchial Asthma Patient / 결핵

BACKGROUND: Inhaled glucocorticoids are the medical treatment of choice in asthma patients. Fluticasone propionate is one of the most effective inhaled corticosteroids and has been reported to have minimal effect on the hypothalamic-pituitary-adrenal axis at the recommended dose. However, reports of long-term trials characterizing their systemic safety with chronic use are rare. This study was designed to evaluate the long-term safety of inhaled fluticasone propionate to the hypothalamic-pituitary-adrenal axis. METHOD: This study was conducted on 21 patients to evaluate the adrenal response to rapid ACTH stimulation test after 6 months of treatment with fluticasone propionate from 200 µg to 750 µg daily. The serum cortisol levels was measured to assess its effect on the hypothalamic-pituitary-adrenal axis just prior to the injection, at 30 minutes and 60 minutes after an intramuscular injection of synthetic ACTH. RESULT: The mean dose of inhaled fluticasone propionate was 355 µg per day(SD=174 µg, range=200 µg to 750 µg). The mean serum cortisol levels of the patients was 11.0 µg/dl(SD=6.4 µg/dl) prior to the injection, 20.0 µg/dl(SD=7.7 µg/dl) after 30 minutes, and 23.0 µg/dl(SD=6.3 µg/dl) after 60 minutes. Sixteen patients of the 21 patients had a normal response(>18 µg/dl), and 5 out of the 21 patients had serum cortisol levels below the normal range after the rapid ACTH stimulation test. CONCLUSION: Adrenal suppression occurred in 5 out of 21 patients with 6 months treatment with inhaled fluticasone propionate.