• Advances in pediatric surgery
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• v.10 no.2
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• pp.99-106
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• 2004

Intestinal atresia is a frequent cause of intestinal obstruction in the newborn. We reviewed the clinical presentation, associated anomalies, types of atresias, operative managements, and early postoperative complications in 36 cases of intestinal atresia treated at the Department of Surgery, Kyungpook National University Hospital between January 1994 and February 2003. Location of the lesion was duodenum in 17 patients, jejunum in 11 patients and ileum in 8 patients. The male to female ratio was 1:1.4 in duodenal atresia (DA), 2.7:1 in jejunal atresia (JA) and 7:1 in ileal atresia (IA). The most common type was type III (41.1 %) in DA, and type I (52.6 %) in JA and IA. The most common presenting symptoms was vomiting(88.2 %) in DA, but in jejunoileal atresia, vomiting(89.4 %) and abdominal distension(89.4 %) were the most common sign and symptom. All cases of DA were diagnosed by plain abdominal radiography. There were 6 cases of DA with congenital heart disease, 3 cases of DA with Down syndrome and 3 cases of JA with meconium peritonitis. Segmental resection was performed in 13 cases, duodenoduodenostomy in 11 cases, membrane excision in 7 cases, jejunojejunostomy in 2 cases, gastroduodenostomy in 2 cases and ileocolic anastomosis in 1 case. There were 9 postoperative complications including 3 each of anastomotic leakage, wound infection, and intestinal obstruction 3 cases. The mortality rate for DA was 11.8 %(2/17). Both deaths in DA were attributed to congenital heart disease. The mortality rate for JA was 18% (2/11). Both cases died with sepsis and short bowel syndrome.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.3 no.1
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• pp.63-67
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• 2000

Purpose: Biliary atresia, one of the major causes of neonatal cholestais, is an idiopathic, serious disorder, affecting the newborn that results in complete obstruction of biliary tract. Successful reestablishment of bile flow is dependent on early surgical intervention, early diagnosis is imperative. The authors evaluate the utility of Tc-99m-labeled diisoprpyliminodiacetic acid (DISIDA) hepatobiliary scintigraphy in the diagnosis of biliary atresia. Methods: From January, 1995 to August, 1999, total 60 patients with neonatal cholestasis underwent Tc-99m DISIDA hepatobiliary scintigraphy at Asan Medical Center. Results: The undelying causes of neonatal cholestasis were biliary atresia in 14, neonatal hepatitis in 33, intrahepatic bile duct paucity in 9, and total parenteral nutrition induced cholestasis in 4. All patient with biliary atresia were interpreted correctely in DISIDA hepatobiliary scintigraphy, showing 100% sensitivity. Of the 46 patients with neonatal hepatitis and other causes, 37 patients had intestinal radioactivity showing 80% specificity. Conclusion: Visualization of DISIDA in the intestinal tract indicates patency of the biliary ducts and excludes the diagnosis of biliary atresia. But the absence of intestinal excretion on the DISIDA hepatobiliary scintigraphy dose not necessarily indicate biliary atresia.

• Advances in pediatric surgery
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• v.3 no.1
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• pp.6-14
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• 1997

To differentiate biliary atresia from intraheaptic cholestasis, Tc-99m DlSIDA hepatobiliary scintigraphies and percutaneous needle biopsies of 60 consecutive infants were evaluated retrospectively. Twenty three patients had biliary atresia and 37 patients intraheaptic cholestasis(neonatal hepatitis 34, TPN induced jaundice 2 and Dubin-Johnson syndrome 1). All sixty patients underwent Tc-99m DlSIDA hepatobiliary scintigraphy with phenobarbital pretreatment. Of 23 patients with biliary atresia, 22 were correctly interpreted showing 96% sensitivity while of 37 patients with intraheaptic cholestasis, only 12 had intestinal excretion of radionuclide showing 32% specificity. Among the forty needle biopsies, 17 of biliary atresia and 23 of intrahepatic cholestasis, 37 were correctly interpreted as either having biliary atresia or intrahepatic cholestasis. The overall diagnostic accuracy was 93%. Of 3 misdiagnosed cases, the histologic findings of two patients with biliary atresia(aged 43 days and 54 days at the first needle biopsy) were essentially the same as those of neonatal hepatitis. Follow-up biopsies, however, showed findings consistent with biliary atresia. The third one(VLBW premie with history of 8 weeks of TPN) showed mild ductal proliferation and portal fibrosis. This was interpreted as suspicious for biliary atresia. Jaundice resolved gradually. In summary, patients who have intestinal excretion of radionuclide on Tc-99m DlSIDA hepatobiliary scintigraphy, biliary atresia can be ruled out. But the patients who do not have intestinal excretion of radionuclide should have further investigation by needle biopsy. Judicious use of Tc-99m DISIDA hepatobiliary scintigraphy and percutaneous needle biopsy can give a diagnostic accuracy of 95% or more in cases of infantile cholestasis.

• The Korean Journal of Nuclear Medicine
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• v.28 no.3
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• pp.357-363
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• 1994

We evaluated the diagnostic accuracy of $^{99m}Tc$-DISIDA scintigrauhy as a mean of differentiating biliary atresia from neonatal hepatitis. $^{99m}Tc$-DISIDA scintigraphy was visually interpreted by assessing the presence or absence of radioactivity in the intestine or gall bladder. In patients without intestinal radioactivity, we measured the hepatic retention index and the hepatic uptake index. The hepatic retention Index was expressed as the amount of change of liver activity from 5 minutes to 30 minutes postinjection. The hepatic uptake Index was graded visually with 5 minute images using the following scoring scheme : grade 0(normal hepatic uptake), grade 1(decreased hepatic uptake), grade 2(hepatic uptake equal to cardiac uptake), and grade 3(hepatic uptake less than cardiac uptake). Age, total bilirubin, and hepatic uptake index were compared between the biliary atresia and the neonatal hepatitis group, between neonatal hepatitis patients with and without intestinal radioactivity, and between the biliary atresia and neonatal hepatitis patients with absent intestinal radioactivity. The results were as follows ; 1) None of the 30 biliary atresia patients showed intestinal radioactivity, while 31/40 neonatal hepatitis patients showed intestinal radioactivity. The sensitivity, specificity, and accuracy of the presence of intestinal radioactivity ?or the diagnosis of biliary atresia was 100%, 78%, and 87%, respectively. 2) In patients with absent intestinal radioactivity the mean hepatic retention index was $1.5{\pm}0.6$ in the 16 biliary atresia patients, and $1.1{\pm}0.2$ in the 7 neonatal hepatitis patients(p<0.01). All 7 patients with hepatic retention index over 1.5 had biliary atresia. But there were 9 patients with biliary atresia below 1.5. 3) No significant differences were found in age, total bilirubin, or hepatic uptake Index between biliary atresia and neonatal hepatitis patients. However there were differences in age, total bilirubin, and hepatic uptake index between neonatal hepatitis patients with and without intestinal radioactivity. The hepatic uptake index was significantly lower, age was old, and total bilirubin was low in the group with intestinal radioactivity compared the group without intestinal radioactivity(p<0.05). Relation between total bilirubin and the hepatic uptake index was that total bilirubin was relatively low at normal hepatic uptake index in biliary atresia and neonatal hepatitis patients. 4) When hepatic uptake index and hepatic retention index were high it suggest that biliary atresia is more likely, considered relation between hepatic uptake Index and the hepatic retention index. Thus, we conclude that $^{99m}Tc$-DISIDA scintigraphy is accurate in the differential diagnosis of biliary atresia and neonatal hepatitis. In patients without intestinal radioactivity, the hepatic retention index and hepatic uptake index, along with the patient's age and total bilirubin level may supplement diagnosis and improve diagnostic accuracy.

• Journal of Chest Surgery
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• v.20 no.4
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• pp.751-759
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• 1987

We have experienced 4 cases of modified Fontan operation for Tricuspid atresia type III from February 1983 to May 1987 at the department of Thoracic 8 Cardiovascular Surgery, Seoul National University Hospital. There were 3 cases with left juxtaposition of right atrial auricle. The operatic mortality rate was 25% [one case death].

• Journal of Chest Surgery
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• v.15 no.4
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• pp.394-401
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• 1982

Recently, 4 cases underwent successful surgical correction of tetralogy of Fallot combined with pulmonary atresia in 2 cases, with abnormal coronary artery in another 2 cases. The operation consisted of a patch repair to the ventricular septal defect. The pulmonary atresia and stenosis were corrected with the method of external connection, from the right ventricle to the pulmonary artery using the valved conduits.

• Proceedings of the KOR-BRONCHOESO Conference
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• 1972.03a
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• pp.17.3-17
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• 1972

We experienced a case of congenital atresia of external auditory canal bilaterally which was corrected surgical with good hearing results.

• Advances in pediatric surgery
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• v.17 no.2
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• pp.170-178
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• 2011

Hiatal hernia is a very rare disease in the pediatric population. However information from our esophageal atresia postoperative follow-up program has hypotheses; "Hiatal hernia may more frequently occur in postoperative esophageal atresia patients (EA group) than in the general pediatric population (GP group)" and "The tension on the esophagus after esophageal anastomosis may be an important etiologic factor of hiatal hernia in EA group". To prove the first hypotheses, we compared the incidence of hiatal hernia in the GP group with the incidence in the EA group. The Incidence in the GP group was obtained from national statistic data from Statistics Korea and Health Insurance Review and Assessment Service of Korea. The incidence in the EA group was obtained from the medical record and the imaging studies of our esophageal atresia postoperative follow-up program. To prove the second hypothesis, the presumptive risk factors for the development of hiatal hernia in EA group, such as the type of esophageal atresia, degree of esophageal gap, the stage operation and the redo-operation with resection and re-anastomosis of esophagus were analyzed statistically. The total number of patients in the EA group was ninety-nine and there were 5 hiatus hernias. The incidence of EA group (5 %) is significantly higher than incidence of GP group (0.024 %). (p=0.0001) The statistical analysis of the presumptive risk factors for hiatal hernia development in EA group failed to show any evidence of correlation between postoperative esophageal tension and the hiatal hernia. This study shows that the postoperative patients with esophageal atresia have high occurrence of hiatal hernia and should be followed up carefully to detect hiatal hernia.

• Advances in pediatric surgery
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• v.11 no.2
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• pp.157-164
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• 2005

Esophageal atresia without tracheoesophageal fistula accounts for 7-11 % of all types of esophageal atresia and is very difficult to treat. In our hospital from 1990 to 2005, we operated upon 40 patients with esophageal atresia, and 6 had pure atresia. The preoperative characteristics, operative findings and post operative course of the six patients with pure atresia were analysed. Immediate gastrostomy was performed in all 6 patients. One patient had simultaneous cervical esophagostomy. Esophageal reconstruction procedures were transhiatal gastric pull up in 3 patients, esophagocologastrostomy utilizing left colon in 1, and transthoracic esophagoseophagostomy with esophageal bougination in 2. Postoperative complications were pneumonia, anastomosis leakage, and gastroesophageal reflux symptom. Conservative management was effective in all patients. A larger series of cases would be required to demonstrate the most effective treatment for this particular anomalous condition.

• Journal of the Korean Society of Radiology
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• v.83 no.5
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• pp.991-1002
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• 2022

Biliary atresia is a rare but significant cause of neonatal cholestasis. An early and accurate diagnosis is important for proper management and prognosis. To diagnose biliary atresia, various imaging studies using ultrasonography, MRI, hepatobiliary scans, and cholangiography can be performed, although ultrasonography is more important for initial imaging studies. In this article, we review the findings of biliary atresia from various imaging modalities, including ultrasonography, MRI, hepatobiliary scans, and cholangiography. The known key imaging features include abnormal gallbladder size and shape, periportal thickening visible as a 'triangular cord' sign, invisible common bile duct, increased hepatic arterial flow, and combined anomalies. Aside from the imaging findings of biliary atresia, we also reviewed the diagnostic difficulty in the early neonatal period and the role of imaging in predicting hepatic fibrosis. We hope that this review will aid in the diagnosis of biliary atresia.