Soon-Ok Choi

A new diagnostic approach to biliary atresia with emphasis on the ultrasonographic triangular cord sign: Comparison of ultrasonography, hepatobiliary scintigraphy, and liver needle biopsy in the evaluation of infantile cholestasis

BACKGROUND/PURPOSE The authors evaluated prospectively the utility of ultrasonography, Tc-99m-DISIDA hepatobiliary scintigraphy, and liver needle biopsy in differentiating biliary atresia (BA) from intrahepatic cholestasis in 73 consecutive infants who had cholestasis. METHODS Sixty three ultrasonographic examinations of 61 infants with 7.0-MHz transducer were carried out, focusing on the fibrous tissue at the porta hepatis. The authors defined the triangular cord (TC) as visualization of a triangular or tubular shaped echogenic density just cranial to the portal vein bifurcation on a transverse or longitudinal scan. RESULTS Although 17 of 20 ultrasonographic examinations from infants who had BA denoted TC, 43 ultrasonographic examinations from infants with either neonatal hepatitis (NH) or other causes of cholestasis denoted no TC, showing a diagnostic accuracy of 95% with 85% sensitivity and 100% specificity. Investigation with Tc-99m-DISIDA hepatobiliary scintigraphy showed that 24 of 25 infants who had BA had no gut excretion, and 16 of 46 infants who had either NH or other causes of cholestasis had gut excretion, showing a diagnostic accuracy of 56% with 96% sensitivity and 35% specificity. Therefore, gut excretion of tracer excluded BA, but no gut excretion of tracer needed further investigations as liver needle biopsy. Forty-four liver needle biopsies were carried out in 19 infants who had BA and 24 infants who had either NH or other causes of cholestasis. Although 18 of 20 biopsy findings in infants who had BA were correctly interpreted as having BA, 23 of 24 biopsy results in infants who had either NH or other causes of cholestasis were correctly diagnosed, showing a diagnostic accuracy of 93% with 90% sensitivity and 96% specificity. CONCLUSIONS Since the introduction of ultrasonographic TC sign in the diagnosis of BA by our institution, we have found that it seemed to be a simple, time-saving, highly reliable, and non-invasive tool in the diagnosis of BA from other causes of cholestasis. The authors propose a new diagnostic strategy in the evaluation of infantile cholestasis with emphasis on ultrasonographic TC sign as first priority of investigations. When the TC is visualized, prompt exploratory laparotomy is mandatory without further investigations. When the TC is not visualized, hepatobiliary scintigraphy is the next step. Excretion of tracer into the small bowel actually rules out BA. Liver needle biopsy is reserved only for the infants with no excretion of tracer. The authors believe that a correct decision regarding the need for surgery can be made in almost all cases with infantile cholestasis by this multidisciplinary approach.

Gastrointestinal phytobezoars in childhood

We reviewed 33 children under the age of 15 with gastrointestinal phytobezoars. Twenty were boys and 13 were girls. Nearly all patients were observed in late fall and winter. In 22 patients, symptoms developed following ingestion of several ripe or dried persimmons with seeds but none had a history of unripe persimmon ingestion. All patients but three underwent enterotomy, gastrotomy, or enterotomy combined with gastrotomy for bezoar removal. The remaining three had small bowel resection due to strangulation. There was a single location of the bezoar in 85% and multiple locations in 15% at operation. Emergency laparotomy was performed on 25 patients who had typical mechanical intestinal obstruction revealed the plain abdominal films. It is not surprising that persimmon is the most common cause of bezoar formation in Korea, because it is a favorite fruit among Koreans, either in hard, soft, or dried variety.

Cystic rectal duplication : a rare cause of neonatal bladder-outlet obstruction and hydronephrosis

Abstract A case of cystic rectal duplication (RD) is presented. A 7-day-old female was admitted with acute urinary retention, voiding difficulty, and abdominal distention since she was 4 days of age. Ultrasound and abdominal computed tomography (CT) demonstrated a huge, cystic mass in the pelvis and abdomen that resulted in acute urinary retention and bilateral hydronephrosis. CT-guided drainage of the lesion followed by transabdominal surgical excision resulted in a cure. Pathologic examination demonstrated a RD lined by respiratory epithelium.

Late complications and current status of long-term survivals over 10 years after Kasai portoenterostomy.

Purpose Whereas the Kasai portoenterostomy (KPE) is an accepted first line of surgery for bile drainage in infants with biliary atresia, its long-term effectiveness is not clear because its etiology and pathogenesis remains unknown. This study was aimed to investigate the late complications occurring in long-term survivors and the current status of living patients who survived over 10 years after KPE. Methods A retrospective analysis of the medical records of 32 patients who underwent KPE from 1990 to 2000 was done. We analyzed 10-year survival rates with the Kaplan-Meier method and the current status of the long-term survivors. Results The overall 10-year survival rate by Kaplan-Meier method after KPE was 76.2%. Eight (25%) patients had died, including 4 who were transplanted. Nineteen (59.4%) patients survived over 10 years. Among them, 6 (31.6%) patients had portal hypertension, and 5 (26.3%) had episodes of cholangitis. Two had intrahepatic cyst and 2 had intestinal obstruction. Six (31.6%) patients have been well without any complications. Conclusion The long-term survival rate of biliary atresia is slightly improving. However, two thirds of patients suffer from various complications. One-third of survivors go on without any complication. As biliary atresia is known as a progressive inflammatory disease, careful life-long follow- up is needed in long-term survivals after KPE.

Haddad Syndrome with PHOX2B Gene Mutation in a Korean Infant

Congenital central hypoventilation syndrome with Hirschsprungs disease, also known as Haddad syndrome, is an extremely rare disorder with variable symptoms. Recent studies described that congenital central hypoventilation syndrome had deep relation to the mutation of the PHOX2B gene in its diagnosis and phenotype. We report a newborn male infant with clinical manifestations of recurrent hypoventilation with hypercapnea and bowel obstruction. These clinical manifestations were compatible with congenital central hypoventilation syndrome and Hirschsprungs disease, and polyalanine 26 repeats in the PHOX2B gene supported the diagnosis of congenital central hypoventilation. We described a first case of Haddad syndrome in Korean and its clinical and genetic characteristics were discussed.

Primary segmental volvulus of the ileum mimicking meconium plug syndrome

Primary segmental volvulus (PSV) of the ileum in neonate occurs rarely but shows an aggressive clinical course. Thus, early diagnosis is important to prevent necrosis and perforation of the intestine. We report a case of PSV of the ileum in a 2-day-old female neonate whose clinical features and radiologic findings appeared to be meconium plug syndrome.

An unusual urachal sinus with external opening in the midline suprapubic area.

Urachal sinuses usually manifest as umbilical discharge, but the authors experienced a case of unusual urachal sinus with external opening in the midline suprapubic area. The distal end of the sinus tract was found to extend directly to the median umbilical ligament. The histology shows a sinus tract lined with a modified transitional epithelium with surrounding inflammatory infiltrates. The authors believe that this is the first report of a urachal sinus with external opening other than the umbilicus in the English-language literature.

Appendicoumbilical fistula as a sequela of perforated appendicitis

This is the first report, we believe, of a 5-year-old boy who developed appendicoumbilical fistula as a sequela of perforated appendicitis. We discuss a proposed explanation of the mechanism in its formation.

Purulent umbilical drainage from infection of left umbilical artery associated with open umbilicoperitoneal communication

This is a case report of a 20-day-old girl with persistent umbilical drainage due to infection of the left umbilical artery that ruptured at the base of the umbilicus and formed an open umbilicoperitoneal communication.

Successful endoscopic submucosal dissection of a giant polyp in a 21-month-old female

Endoscopic submucosal dissection (ESD) is now recognized as the preferred treatment modality for gastrointestinal epithelial lesions. A 21-month-old female was admitted with a giant hyperplastic polyp causing a gastric outlet obstruction. Successful ESD was performed with caution. The post-procedural course was uneventful without a bleeding episode. Although further study of the feasibility of ESD in early children is necessary, ESD could be applied to avoid laparotomy even in young children.