Mucopolysaccharidosis: Abnormal Findings on Abdomen and Chest Excluding Musculoskeletal System

PURPOSE: Mucopolysaccharidosis (MPS) is a lysosomal storage disease that causes tissue distortion and dysfunction due to the infiltration of mucopolysaccharide in connective tissue. The purpose of this study was to evaluate the characteristic findings of abdominal CT and plain chest radiography in patients with MPS. MATERIALS AND METHODS: Sixty-two children with MPS diagnosed by urine analysis were involved in this study; 24 of these underwent abdominal CT and the findings were reviewed by two radiologists, who reached a consensus. Organomegaly was classified as severe, moderate or mild. On chest PA radiographs of 42 of the children, the transverse diameter of the trachea was measured and compared with that of 42 normal controls. Student's t test was used for statistical analysis. RESUTLS: At abdominal CT, hepatomegaly was observed in 22 patients (92%; 2 severe, 15 moderate and 5 mild); and splenomegaly was present in 18 (75%; 2 severe, 4 moderate and 12 mild). Among eight patients (33%) with pancreatic enlargement, one had a severly enlarged pancreas, while in the remaining seven, enlargement was mild. Also present were inguinal hernia (n=15), umbilical hernia (n=12), undulation with thickening of the diaphragmatic crura (n=10), abnormalities related to the male genitalia (n=5) and vascular anomaly (n=3). In MPS patients, the mid-point diameter of the trachea (range, 5.6-9 mm; mean, 6.9 mm) was significantly less than in normal controls (range, 8-14 mm; mean, 10.8 mm) (p<0.001). CONCLUSION: An awareness of the characteristic abnormalities observed at abdominal CT and chest PA radiography can lead to a better understanding of MPS in children.

Radiological Changes Following High Frequency Oscillatory Ventilation Therapy in Very Low Birth Weight Infants with Respiratory Distress Syndrome

PURPOSE: High frequency oscillatory ventilation (HFOV) is used to support infants with severe respiratory failure unresponsive to conventional ventilation (CV). We reviewed chest radiographs before and after HFOV with clinical correlation in infants with respiratory distress syndrome (RDS). METHODS: Eighteen very low birth weight infants with RDS who had HFOV were included in this study. All patients were diagnosed as having RDS clinically and radiologically. Mean gestational age of infants was 27 weeks (range : 24-31 weeks). The mean duration of HFOV was 3 days (range : 14 hours-9 days). The chest radiographs of these infants within 3 hours before and after application of HFOV were retrospectively reviewed. Radiological findings based on aeration and parenchymal densities were classified into improved, no change, and progressed. Medical records were reviewed for FiO2 levels, clinical outcomes, complications, and causes of death. RESULTS: In 15 of 18 infants, aeration and parenchymal densities were improved and FiO2 levels were also improved after HFOV. Four of these 15 infants who showed improvement of radiological findings developed pneumothorax, sepsis, pulmonary or intestinal bleeding, and subsequently died. In remaining 3 infants in whom chest radiographs after HFOV showed no interval change or progression, oxygenation was also worsened and all died. CONCLUSION: Chest radiographs of HFOV-treated, very low birth weight infants showed improvement of aeration and parenchymal densities in most cases. Clinical outcome was good in infants who showed improvement on chest radiographs compared to those of progression group as far as there was no associated complication. Knowledge of radiological changes after HFOV will help in interpretation of chest radiographs in those HFOV-treated infants.

Neurodevelopmental Outcome in Infants with Increased Subarachnoid Cerebrospinal Fluid Accumulation / 대한소아신경학회지

Since the advent of ultrasonography, brain imaging evaluation by this method became very popular in pediatric population due to its easy access and noninvasiveness. As more and more ultrasonographic head imagines were performed, the enlargement of subarachnoid corebrospinal fluid (CSF) space was found to be a common finding. Enlargement of subarachnoid CSF spaces, especially extra-axial CSF space is a specific radiologic pattern but its pathogenesis and significance have not been established. Widening of the subarachnoid CSF space on cranial imaging has been related to a wide spectrum of conditions including genetic syndromes, prematurity, previous intraventricular or subarachnoid hemorrhage, meningitis, cerebral atrophy or subdural collections, and it may occur in pathologic or nonpathologic conditions. Various terms have been used to describe this finding . external hydrocephalus, benign subdural effusion, benign extra-axial collections, subdural hygroma, benign subarachnoid fluid collection. Since there is no clear study regarding the neurodevelopmental outcomes of these population, many physicians have performed follow-up imaging studies and have had difficulty in explaining to parents about this finding. Our goals in the present study were to provide further clinical and developmental information concerning enlargement of the subarachnoid space by the prospective follow-up of a group of infants identified by specific head ultrasonographic criteria and to propose a plan for future management.

Imaging Findings of Neonatal Adrenal Disorders

In newborn infants, normal adrenal glands are characterized by a relatively thin echogenic center surroundedby a thick, hypoechoic cortical rim as seen on ultrasound (US). Various disorders involving the neonatal adrenalgland include adrenal hemorrhage, hyperplasia, cyst, Wolman's disease, and congenital neuroblastoma. Adrenalhemorrhage is the most common cause of an adrenal mass in the neonate, though differentiation between adrenalhemorrhage and neuroblastoma is in many cases difficult. We describe characteristic US, CT and MR imaging findingsin neonates with various adrenal disorders.

Imaging Findings of Neonatal Adrenal Disorders

In newborn infants, normal adrenal glands are characterized by a relatively thin echogenic center surroundedby a thick, hypoechoic cortical rim as seen on ultrasound (US). Various disorders involving the neonatal adrenalgland include adrenal hemorrhage, hyperplasia, cyst, Wolman's disease, and congenital neuroblastoma. Adrenalhemorrhage is the most common cause of an adrenal mass in the neonate, though differentiation between adrenalhemorrhage and neuroblastoma is in many cases difficult. We describe characteristic US, CT and MR imaging findingsin neonates with various adrenal disorders.

Interventional Recanalization of Artificial Arteriovenous Fistula and Graft for Hemodialysis: Angioplasty andPulsed-Spray Thrombolysis with Urokinase

PURPOSE: To evaluate the effectiveness of percutaneous transluminal angioplasty (PTA) and pulsed-spraypharmacomechanical thrombolysis (PSPMT) using urokinase for the management of insufficient hemodialysis access. MATERIALS AND METHODS: Between September 1996 and May 1998, 21 insufficient hemodialysis accesses were treated in16 patients (3 artificial arteriovenous fistulae, AVF ; and 13 arteriovenous graft, AVG). PTA and PSPMT were performed in 6 and 15 and 15 cases, respectively, and success and long-term patency rates were evaluated. RESULTS: The overall success rate of PTA and PSPMT for insufficient hemodialysis access was 76.2%(16/21). The success rates of PTA and PSPMT were 83.3%(5/6) and 73.3%(11/15), respectively. the primary patency rates of PSPMT were 69+/-12.8% at 6 months and 38+/-18.6% at 12 months. One of the two initially successful PTAs had been patent for 7months, and the second PTA was performed at that time due to venous stenosis. The other was patent for 15 months throughout the follow-up period. CONCLUSION: PTA and PSPMT are effective primary methods for the treatment of insufficient hemodialysis access ; success and patency rates were high, and the procedures can be performed repeatedly.

Phalangeal and Metacarpal Metastases from Clear Cell Sarcoma of the Kidney: A Case Report

Clear cell sarcoma of the kidney(CCSK) is rare, and was previously thought to be a variant of Wilms' tumor. Incontrast to patients with Wilms' tumor, bone metastasis is common in patients with CCSK, in which because of itsskeletal metastasis, prognosis is worse. We describe the radiographic, MR imaging, and bone scintigraphy findings,and include a review of the literature.

Radiologic Findings of Sinus Pericranii

Sinus pericranii is a rare vascular anomaly consisting of abnormal venous communication between intra- andextracranial circulation. We report one case, confirmed by surgery, and describe the radiological findings ofDoppler ultrasonography, CT and MR imaging.

Radiologic Findings of Various Disorders Related to Chemotherapy in Children

Because available therapy cannot always distinguish between malignant and nonmalignant cells, the toxicity ofchemotherapeutic agents to normal tissue remains a troublesome issue. Various chemotherapeutic agents such asbleomycin, doxorubicin, cyclophosphamide and L-asparaginase, which cause pulmonary fibrosis, cardiomyopathy,pancreatitis, and hemorrhagic cystitis, respectively, are familiar to radiologists. The purpose of this report isto describe the radiologic findings of various organ abnormalities related to chemotherapy.

Mediastinal Interfaces and Lines: Clinical Significance and Radiographic-CT Correlation

Mediastinal interfaces on a chest radiograph result from contact between mediastinal structures and the adjacent lung, while mediastinal lines result from contact between the two lungs across the midline. A variation of mediastinal interface is mediastinal stripe, a narrow band produced by contact of both sides of a mediastinal structure with the lungs. Alterations in mediastinal interfaces and lines may be due to variations in normal anatomy, or may reflect the presence of abnormalities within the mediastinum. Familiarity with the various normal mediastinal interfaces and lines, and the changes that occur with disease is important for the interpretation of the chest radiograph and in the diagnosis of mediastinal abnormalities. The purpose of this pictorial essay is to illustrate the most important normal and abnormal interfaces and lines and also to correlate radiographic and CT findings.

Primary Epiploic Appendagitis: 4 Case Report

Primary epiploic appendagitis of the colon is a rare cause of abdominal pain and is a self-limiting disease. Double contrast barium enema showed a smooth extrinsic compression located anterolateraly to the involved colon. Ultrasonography revealed a non-compressible echogenic ovoid mass attached to the colon wall. On abdominal CT, all lesions were seen as pedunculated ovel fatty masses with surrounding streaky densities connected to the serosal surface of the adjacent colon. Follow up CT performed 13-25(mean 18) days later showed that during this intrval, the volume of the mass and extent of surrounding infiltrations had decreased.

Adenosquamous Carcinoma of the Liver: A Case Report

A case of primary adenosquamous carcinoma of the liver is reported. A large cystic mass with irregular enhancing wall was seen in the left lobe of the liver; multiple hepatic cysts and metastatic nodules were scattered in both lobes. Sonoguided needle biopsy was performed and on histopathology, adenosquamous carcinoma was diagnosed. The radiologic and pathologic relationships of this unusual neoplasm are discussed.

Endodermal Sinus Tumor Arising from Resected Benign Cystic Sacrococcygeal Teratoma

Endodermal sinus tumors are an uncommon malignancy in children and occur most often in the gonads and sacrococcygeal region. In these locations, thy are found as primary tumors ; in teratoma they form part of the malignant component. We present CT and MR imaging findings in two cases of endodermal sinus tumor occurring after the resection of a mature benign cystic sacrococcygeal teratoma during the neonatal period. Awareness of the transformation of a benign cystic teratoma to a different type of malignant germ cell tumor may be helpful in the evaluation of sacrococcygeal masses.

Endodermal Sinus Tumor Arising from Resected Benign Cystic Sacrococcygeal Teratoma

Endodermal sinus tumors are an uncommon malignancy in children and occur most often in the gonads and sacrococcygeal region. In these locations, thy are found as primary tumors ; in teratoma they form part of the malignant component. We present CT and MR imaging findings in two cases of endodermal sinus tumor occurring after the resection of a mature benign cystic sacrococcygeal teratoma during the neonatal period. Awareness of the transformation of a benign cystic teratoma to a different type of malignant germ cell tumor may be helpful in the evaluation of sacrococcygeal masses.

Radiologic Findings of Neonatal Sepsis

PURPOSE: To review the simple radiographic and sonographic findings in infants with neonatal sepsis. MATERIALS AND METHODS: We retrospectively analyzed simple chest and abdominal radiographs, and brain sonograms in 36 newborn infants (preterm: term=23:13). With neonatal sepsis diagnosed by blood culture and clinical manifestations. RESULTS: Pulmonary parenchymal infiltrate excluding respiratory distress syndrome and pulmonary edema or atelectasis was found in 22 infants (61%). Paralytic ileus, hepatosplenomegaly, and necrotizing enterocolitis were present in 18 (50%), 9 (25%), and 1 (3%) infants, respectively, while skeletal changes suggesting osteomyelitis were found in three. Brain sonography was performed in 29 infants and in four, abnormalities were seen ; these comprised three germinal matrix hemorrhages and one intraparenchymal hemorrhage. In six patients (17%) radiologic examinations revealed no abnormality. CONCLUSION: In patients with neonatal sepsis, pulmonary infiltrates and paralytic ileus were common abnormalities. Although these were nonspecific, radiologic findings may be used to supplement clinical and laboratory findings in diagnosing neonatal sepsis and planning its treatment.

T1 Lung Cancer: Role of Mediastinoscopy and CT in the Diagnosis of Mediastinal Adenopathy

PURPOSE: To evaluate the role of mediastinoscopy and CT in the preoperative nodal evaluation in patients with T1 lung cancer. MATERIALS AND METHODS: Between November 1994 and July 1996, 125 patients underwent thoracotomy and/or mediastinoscopy for surgical treatment of lung cancer. Among them, 35 patients had T1 lung cancer(peripheral lung cancer less than 3cm in diameter) on CT. One patient finally proved to have T4 lung cancer with pleural seeding at thoracotomy. In the remaining 34 patients, pathologic evaluation of mediastinal lymph nodemetastasis was feasible and the results were correlated with CT findings. On CT, nodes larger than 10mm in short-axis diameter were regarded as abnormal. RESULTS: The patients had adenocarcinoma in 12, squamous cellcarcinoma in 11, bronchioloalveolar carcinoma (BAC) in 10, and large cell carcinoma in one. Fifteen among total 478 sampled lymph nodes contained malignant tumor. Six (three with adenocarcinoma, two with squamous cell carcinoma, and one with large cell carcinoma) of 34 patients (18%) had nodal metastasis. With 112 sampled nodes, BAC did not show any nodal metastasis. Sensitivity and specificity of CT for nodal detection were 0% and 100% for2R, 0% and 100% for 4R, 100% and 97 % for 5, 50% and 100% for 7 and 0% and 100% for 10R, respectively. CONCLUSION: T1 lung cancer shows relatively high (18%) prevalence of mediastinal lymph node metastasis. Because small nodesless than 10mm in diameter contain malignancy and CT is insensitive in detection of metastatic nodes,mediastinoscopy is still needed for preoperative nodal evaluation except BAC.

Constipation in Children-Colonic Transit Time and the Barr-Score

PURPOSE: We have assessed the clinical usefulness of solid maker transit technique and Barr-score in 31 patients with idiopathic constipation (male:female; 17:14, mean age; 6.7 years, range 1.4-12 years). METHODS: All patients underwent full history taking and physical examination including rectal examination. On first visit to gastroenterology clinic a plain abdominal film was taken for Barr-score which evaluated by two observers without the information of the patients. Kappa score was calculated for inter or intra observer variability. Sitz marker were given orally at 9 am on days 1, 2 and 3, and another plain abdominal film was taken on day 5. Total remained solid marker were counted for the degree of retention. We calulated the residual sitz marker. Each film was divided into right colon, left colon and rectosigmoid areas, using bony landmarks, and the marker content of each area counted. RESULTS: 2 patients were excluded because they resisted to take sitz maker. Transit times were normal, mild, moderate and severe delay in 12 patients, 2 patients, 3 patients and 12 patients respectively on day 5 film. The correlation coefficiency between the degree of transit delay and clinical severity was 0.89. Among 14 patients with transit delay, 12 patients has outlet obstruction, 1 patient right colonic delay and 1 patients left colonic delay. The Kappa for inter and intra-personal variability were 4.13 and 4.18 respectively (moderate consistency each). The correlation coefficiency between Barr-score and solid marker colonic transit time was 0.603 (P=0.0008). CONCLUSIONS: This results showed that the solid marker colonic transit time and Barr score were useful in evaluation of patients with constipation.