Neuronal Intestinal Dysplasia in an Infant: Case Report

Neuronal intestinal dysplasia in pediatric patients has similar clinical symptoms and often similar radiologic findings to those of Hirschsprung's disease. Yet neuronal intestinal dysplasia shows hyperplasia of the myenteric plexus for the pathology, and it requires different treatment compared with Hirschsprung disease. This disease has been reported many times in Europe but, to date, only one case has been reported in the radiologic literatures in Korea. We report here on a case of neuronal intestinal dysplasia that involved the entire colon in a two-month-old boy, and we include the radiographic findings.

Neuronal Intestinal Dysplasia in an Infant: Case Report

Neuronal intestinal dysplasia in pediatric patients has similar clinical symptoms and often similar radiologic findings to those of Hirschsprung's disease. Yet neuronal intestinal dysplasia shows hyperplasia of the myenteric plexus for the pathology, and it requires different treatment compared with Hirschsprung disease. This disease has been reported many times in Europe but, to date, only one case has been reported in the radiologic literatures in Korea. We report here on a case of neuronal intestinal dysplasia that involved the entire colon in a two-month-old boy, and we include the radiographic findings.

Sonography-guided Gastrografin Enema for Meconium Plug Syndrome in Premature Newborns: Preliminary Results

PURPOSE: To evaluate the usefulness of sonography-guided Gastrografin enema for the diagnosis and treatment of meconium plug syndrome in premature newborns in a neonatal intensive care unit (NICU). MATERIALS AND METHODS: Fifteen premature newborns underwent 23 sonography-guided Gastrografin enemas on the 8th day of life on average (range: 3 days-21 days). The gestational age and birth weight (mean+/-standard deviation) of the patients were 29+/-2 weeks and 999+/-148 g, respectively. The diagnosis of meconium plug syndrome was based on relevant clinical and radiological findings. Diluted Gastrografin (1:3, 17-45 ml) was administered through a rectal tube under the guidance of sonography until it reached to the cecum. Thereafter, immediate post-procedure and delayed portable abdominal radiographs were taken. All the procedures were done within the NICU. We reviewed the dates of meconium evacuation and the first feeding after the last enema from the clinical data. Radiologically, the timing of the radiographic improvement after the last enema was checked. In cases of distended distal ileum with meconium on pre-enema sonography, follow-up sonography was performed to determine the interval change after gastrografin enema. RESULTS: None of the sonography-guided Gastrografin enemas performed within the NICU caused procedure-related complications. Meconium was evacuated within one day in all patients. In 14 patients, on average, feeding was started on the 7th day and radiographic improvement was observed on the 3rd day. Four of the five patients showing a distended distal ileum with meconium revealed a decrease in ileal distension on follow-up sonography. On the other hand, the other patient, who did not show such a decrease on follow-up sonography, was found to have ileal stenosis during subsequent surgery. CONCLUSION: Sonography-guided Gastrografin enema is a safe and effective bedside procedure, when performed in the NICU for the diagnosis and treatment of meconium plug syndrome.

Usefulness of MR Cholangiography in the Evaluation of Neonatal Cholestasis: Comparison with 99mTc DISIDA Scan

PURPOSE: To evaluate the diagnostic validity of MR cholangiography as a second-line imaging tool following sonography in the evaluation of neonatal cholestasis, we compared MR cholangiography with 99mTc DISIDA scan. MATERIALS AND METHODS: We retrospectively evaluated sonography, 99mTc DISIDA scan and MR cholangiography in twenty-two neonates and infants (age range, 23-103 days; mean age, 57 days) presenting with conjugated hyperbilirubinemia. Of the 22 patients, 15 were diagnosed as biliary atresia by operative cholangiography and liver biopsy and six as neonatal hepatitis by imaging finding and clinical data. Remaining one patient was diagnosed as neonatal hepatitis by operative cholangiography and liver biopsy. Two independent observers for each study were assigned to review the images of 99mTc DISIDA scan and MR cholangiography without giving the final diagnosis or other clinical data. Diagnostic accuracy and interobserver variability for each study were evaluated. RESULTS: On 99mTc DISIDA scan, biliary atresia was mistaken for neonatal hepatitis in eight patients and vice versa in four patients. On MR cholangiography, it was mistaken biliary atresia as neonatal hepatitis and vice versa in each two patients. Sensitivity, specificity, accuracy, positive and negative predictive values of 99mTc DISIDA scan were 48%, 47%, 48%, 66% and 30%, respectively, and those of MR cholangiography were 90%, 71%, 84%, 87% and 81%, respectively. Interobserver variabilities for 99mTc DISIDA scan and MR cholangiography were 0.62 and 0.85, respectively. CONCLUSION: In the evaluation of patients with neonatal cholestasis, it would be advisable to use MR cholangiography, having superior diagnostic accuracy to 99mTc DISIDA scan, as a second-line imaging tool following sonography.

Nodular Regenerative Hyperplasia of the Liver in an Infant: Case Report

Nodular regenerative hyperplasia (NRH) of the liver is an uncommon disease entity, especially in the pediatricage group. A few cases have been reported in the radiologic literature, but follow-up imaging studies are rare. We describe a case of NRH, diagnosed by ultrasound-guided needle biopsy, in a seven-month-old infant with cri-du-chat syndrome. Initial ultrasound revealed several small hypoechogenic nodules in the liver, but CT and MR failed to demonstrate their presence. Two follow-up sonographic examinations were performed 7 and 20 months later, revealing increases in the size and number of the nodules.

Design and Clinical Application of Safe Air-Pressure Reduction System for Intussusception

PURPOSE: This study was performed to design a safe air-pressure reduction system which can absorb rapidlyrising intraluminal pressure during intussusception, and comparison with other reduction systems to test itsclinical availability. MATERIALS AND METHODS: The air-pressure reduction system consisted of a pressure gauge,air insufflators, a pressure controller, buffers, and rapid exhaustion devices, and to determine itsabsorbability, it was connected with a bowel model. By using it in 20 infants with intussusception, we comparedthe absorbability of our air-pressure reduction system with preexisting systems. RESULTS: While extraluminalpressure was applied to the bowel model in which baseline intraluminal pressure was set to 120mmHg, this rose to176mmHg (56mmHg high to standard, 100%) in the direct infusion system, but to only 130 mmHg (10mmHg high tostandard, 17.9%) in a system connected to a large buffer of 10,500 mL capacity. Immediately after the applicationof extraluminal pressure for less than 1 sec, this air-pressure reduction system showed better absorbability thanthe hydrostatic reduction system. Applying this system to 20 infants with intussusception, this was successfullyreduced in 19 cases(95%), without complications. CONCLUSION: In this experiment, it was proved that the systemabsorbed rapid intraluminal pressure elevation. Its use would help prevent bowel perforation during air reductionoccurring during intussusception.

Diagnosis of Hirschsprung's Disease: Accuracy of Barium Enema Findings

PURPOSE: To determine the relative accuracy of barium enema findings of Hirschsprung's disease (HD) and to calculate a level of probability for three signs combined. MATERIAL AND METHODS: Barium enema findings in 45 patients who had undergone rectal biopsy to prove or exclude the diagnosis of HD were retrospectively analyzed by evaluating the presence of a transition zone, irregular contractions and delayed evacuation of barium. Seventeen were neonates (group 1) and the other 28 were infants and children (group 2). The sensitivity, specificity, and positive and negative predictive values of the findings were compared. RESULTS: In visualization of a transition zone, sensitivity, specificity and positive predictive value were 76.5%, 72.7% and 89.7%, respectively. Sensitivity for irregular contractions and delayed evacuation of barium was 76.5% and 91.7%, respectively, whereas for specificity, the corresponding values were 63.6% and 40%. Sensitivities for radiologic signs were higher in group 1 than in group 2, but, the specificities were lower. If two or three findings were positive, the level of probability was 85 - 100%. If two findings were negative, however, the corresponding value was 30%. CONCLUSION: We conclude that the most reliable HD finding is the presence of a transition zone. Irregular contractions and the delayed evacuation of barium are not specific. Two or three positive findings may suggest a higher probability of HD than any single positive finding alone.