Familial Occurrence of Moyamoya Disease: Report of Four Patients in Two Families

Moyamoya disease is an occlusive cerebrovascular disease characterized by stenosis or occlusion of main cerebral arteries. The etiology of moyamoya disease is still unclear, but the frequent familial occurrence suggests that some genetic factors may contribute to its etiology. In Korea, though the moyamoya disease is not infrequent, its familial occurrence has not been reported yet. We report four female patients(in two families) of moyamoya disease with its familial occurrence. Two patients are siblings and the other two are mother and daughter. The ages of symptom onset were thirties in three patients and sixties in one patient. Three patients had intracerebral hemorrhage including intraventricular hemorrhage(recurrent in two). We got cerebral angiography in three patients and magnetic resonance angiography (MRA) in one patient. Full laboratory evaluations were done in three patients, including HLA typing in two patients(mother and daughter). All the patients did not have any hereditary disease or any other disease which are known to cause moyamoya-like changes. Considering the reported familial incidence in Japan, careful search for the family members would reveal much more familial cases in Korea also.

Abdominal Burkitt Lymphoma in Children: CT Finding

PURPOSE: To evaluate the CT findings of Burkitt's lymphoma involving the abdomen in children. MATERIALS AND METHODS: We retrospectively analyzed the abdominal CT of ten children who presented with abdominal symptom. Theywere confirmed by operation in two cases and by fine needle aspiration biopsy in eight to be suffering from Burkitt's lymphoma. We also abdominal ultrasonography(USG) (n=10) and carried out small bowel follow-through examination(SBS) (n=5). Analyses focused on features of the abdominal mass : bowel wall thickening, ascites, lymphadenopathy, and the involvement of intra-abdominal solid organ. RESULTS: Abdominal CT at the time ofpresentation showed a huge conglomerated mass encasing segments of small bowel and also peripherally displacingbowel loops (n=9), bowel wall thickening (n=10), and ascites (n=10). In three of these cases, we were able to see tumor necrosis and cavity formation. Extensive infiltration into mesenteric fat and obliteration of tissue planemade it impossible to identify on CT the margin of the tumor and the presence of mesenteric lymphadenopathy. Infour patients, sonography showed enlarged mesenteric lymph nodes(15-20mm), and in three, retroperitoneal lymphnodes(5mm, 10mm, 12mm in long dimension) were detected on CT and USG. CONCLUSION: Abdominal CT can reveal the characteristic imaging features of Burkitt's lymphoma in children. These are a huge conglomerate mass with or without cavity formation, that encases the small bowel and infiltrates the mesentery, ascites, and the relatively spared retroperitoneal lymph nodes.

Growth Hormone-Secreting Pituitary Adenoma: Clinical and MR Imaging Findings

PURPOSE: To describe clinical and MRI findings of growth hormone-secreting pituitary adenoma, to determine ifthere are any characteristic MRI findings different from those of other pituitary adenomas, to evaluate the relationship between tumor size and serum growth hormone level, and to assess the results of immunohistochemical study. MATERIALS AND METHODS: We retrospectively analysed clinical and MRI findings of 29 patients with growth hormone-secreting pituitary adenoma confirmed by serum growth hormone level and surgery. We also evaluated the relationship between the tumor volume and serum growth hormone level, and the results of immunohistochemical study. Coronal and sagittal T1-weighted MR images in all patients and gadolinium enhanced T1-weighted MR images in28 patients were obtained with 2.0 T(24 cases) and 0.5 T(5 cases) MR imagers. The images were analyzed in terms oftumor size, signal intensity, degree of contrast enhancement, extent of tumor growth and the presence or absenceof cystic change, hemorrhage and calcification. RESULTS: Clinical manifestations included facial feature changeand soft tissue swelling of hands and feet(n=29), headache(n=12), impaired visual acuity(n=9), symptoms of hyperprolactinemia(n=8), visual field defect(n=5), and others(n=6). On MR images, all of the 29 cases were seen tobe macroadenomas and the size of the tumors averaged 2.2cm(1-5.2 cm). Supra- and infrasellar extensions were seenin 21 and 22 patients, respectively. Cavernous sinus invasion was noted in seven, and in one this was bilateral.Signal intensity was isointense with cortical grey matter in 26 cases(90%). Cystic change or necrosis was seen ineight cases(28%), hemorrhage in four(14%), and calcification in two(7%). After enhancement, most(25/28) of the tumors enhaned less than mormal pituitary in degree. There was no correlation between serum growth hormone level and tumor size. Immunohistochemical study showed positive growth hormone-secreting cells in only 69%(11/16). CONCLUSION: Clinical findings in patients with growth hormone-secreting pituitary adenomas were various and included acromegaly, headache, visual impairment, and symptoms of hyperprolactinemia, for example. On T1-weightedMR images, all tumor were macroadenoma, and there were no characteristic findings different from those of other pituitary tumors. The volume of a tumor did not correlate with serum hormone level. Immunohistochemical study showed positive growth hormone-secreting cells in a limited number of cases.

Masticator Space Lesions: MRI and CT Findings

PURPOSE: We evaluated the MR and CT findings of the masticator space lesions in order to identify the differences among the malignant and benign tumors and infectious conditions. MATERIALS AND METHODS: MR and CT findings in 46 cases with proven masticator space lesions were reviewed retrospectively. We analysed the involvement of masticator muscles, adjacent spaces, orbit and intracrahium, homogeneity, necrosis, cystic changes, growth patterns, calcifications, enhancement patterns, MR signal intensity, and CT attenuation. RESULTS: Among the 29 cases of malignant tumors, seven cases were mandibular tumors including four chondrosarcomas, and 22 cases were extramandibular tumors. Malignant tumors of mandibular origin showed large masses with severe bone destruction and epicenter of mandible. Extramandiblular malignant tumors showed the epicenter out of the mandible and less severe bone destruction than mandibular tumors: Among the nine benign tumors, four cases were ameloblastomas which showed the well-defined masses and the expansion of the mandible, and four cases were extramandibular tumors which showed well-marginated extramandibular masses with no bone destruction. Among the eight infectious conditions, five cases were mandibular osteomyelitis with or without abscess formations, and the other three cases were infections from adjacent soft tissue or limited to the soft tissue. CONCLUSION: By careful observations of growth patterns, involvement of the masticator and adjacent spaces, bone changes, and epicenter of the lesions, one can discriminate a mandibular lesion from an extramandibular lesion. With this approach, it is thought to be easier to suggest a dignosis among a wide spectrum of masticator lesions.

Cushing's Disease: The Diagnostic Value of Radiologic Studies

PURPOSE: To evaluate the diagnostic value of radiologic studies in the Cushing's disease MATERIALS AND METHODS: We analyzed retrospectively the CT(n=19, dynamic CT was not perfomed), MR (n=23, dynamic MR in 2 cases), and the results of inferior petrosal sinus sampling(IPSS)(n=13) in 25 patients(19 women, 6men, age range 16-58, mean 32.2) with pathologically-confirmed ACTH secreting pituitary adenoma. We analyzed the radiologic findings and determined how accurately each method could detect and lateralize the lesion within the pituitary gland. The results were compared with the surgical findings and with each other. RESULTS: Seven patients(28%) out of 25 had macroadenomas and the lesions were detected and correctly diagnosed by CT and MR in all. Eighteen patients(72%) had microadenoma. In patients with microadenoma the sensitivity was 38.5%(5/13) in CT, 64.7%(11/17) in MR, 90.9%(10/11) in IPSS. In two patients with dynamic MR, in whom the conventional Gd-enhanced MR showed false negative results, the lesions were detected as low signal intensity mass and correctly lateralized. Considering the results of both MR and IPSS, the sensitivity increased up to 94.1%(17/18). CONCLUSION: Considering the invasiveness of IPSS, Gd-enhanced MR, preferably dynamic, seems to be an imaging modality of choice in Cushing's disease. Combined use of IPSS in selected patients further increases the accuracy of diagnosis and lateralization.

Incidence and Significance of Pleural Effusion after Hepatoma Surgery

PURPOSE:We performed this study to evaluate the clinical significance and temporal changes of pleural effusion developed after the resection of hepatoma. MATERIALS AND METHODS: We reviewed retrospectively follow-up chest radiographs of 97 patients who had undergone operation for hepatoma and had no radiologically demonstrable postoperative complications. The duration of pleural effusion was classified into five groups and the amount of pleural effusion at one week after operation was graded into four groups. Statistical significance of the relationship between the duration, amount of pleural effusion and five factors, which are location and size of tumor, age of the patients, methods of operation, and preoperative liver function, was studied respectively. RESULTS:Pleural effusion was developed in 63.9% (62/97) and the mean duration was 2.5 weeks. In 92% (52/56), pleural effusion disappeared spontaneously within four weeks. Patients who had hepatoma in upper portion of the right lobe developed more frequent pleural effusion which persisted longer, and was larger in amount at one week after operation(p<0.05). There were no statistically significant differences between pleural effusion and the other four factors. CONCLUSION:Pleural effusion following hepatoma surgery should not be regarded as a sign of post-operative complication, as it invariably disappears spontaneously within four weeks. Development of pleural effusion is considered to be caused by local irritation and disturbance of lymphatic flow at the diaphragm.

MR Findings of Thoracic and Abdominal Aortic Aneurysms: Comparison with Anglographic and Surgical Findings

PURPOSE: To assess the utility of spin-echo magnetic resonance(MR) imaging in the evaluation of thoracic and abdominal aortic aneurysm. MATERIALS AND METHODS: The spin-echo MR images of 27 aortic aneurysms in 22 patients were analyzed and correlated with angiography and/or operative findings retrospectively. Evaluations included location, type, and maximum diameter of the aneuyusm, mural thrombus, major branch involvement, and relationship with adjacent organ. RESULTS: The location of aneurysms was ascending thoracic aorta in seven cases, ascending thoracic aorta and aortic arch in one, descending thoracic aorta in six, thoracoabdominal aorta in three, and abdominal aorta in eight. Nineteen were fusiform, and eight were saccular. The mean of maximum diameters of the aneurysms was 7.9cm (4-10cm) on MR and 7.3cm (3-10cm) on angiography. Mural thrombus were noted in 13 cases on MR imaging and seven cases on angiograhpy. Angiography also underestimated the amount of mural thrombus. Eight cases involved major aortic branches. Although MR imaging and angiography were equal in the assessment of major abdominal aortic branches, MR imaging could not clearly demonstrate arch vessels, especially left subclavian artery, in aortic arch aneurysms. Among seven ascending thoracic aneurysms, six had aortic regurgitation. MR imaging showed left ventricular enlargement in all six cases. There was pericardial effusion in four cases which were noted only on MR imaging. MR imaging demonstrated hydronephrosis and renal atrophy in two cases of abdominal aortic aneurysms respectively. CONCLUSION: In the assessment of size of the aneurysm, mural thrombus, and relation with adjacent organs, MR imaging was better than angiography. MR and anglographic findings were equal in the assessment of the location and type of the aneurysm. Angiography was better than MR imaging in the assessment of major branch involvement, especially left subclavian artery.