Eunyoung Jung

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.

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.

Mesenteric suture granuloma caused by retained fragments of suture material in a girl who had a laparotomy 12 years previously

The authors report a case of a mesenteric suture granuloma in a 12 year-old-girl who had a small bowel resection for a complicated intussusception at the age of 5 months. At later exploration a whitish round tumor located on the anti-mesenteric side of the intestine was found. Several small intestinal loops also abutted on the tumor. Pathologic examination showed fibrosis and a granuloma containing linear colored braided suture material with multinucleated giant cell. As mesenteric suture granulomas have a complex appearance and mimic a soft tissue tumor during imaging, it is important for a surgeon to know about this condition and to consider the history of previous surgery when evaluating the images of patients presenting with an abdominal or pelvic mass. Suture granulomas separate from previous suture sites have not been described in the literature.

Diagnostic Accuracy of Combined Acetylcholinesterase Histochemistry and Calretinin Immunohistochemistry of Rectal Biopsy Specimens in Hirschsprung’s Disease

Background. Acetylcholinesterase (AchE) histochemistry has been established as an accurate diagnostic tool for Hirschsprung’s disease (HD). In addition, calretinin immunohistochemistry is also reported as a reliable and adjunctive method to diagnose HD. We investigated the diagnostic value of combined AchE histochemistry and calretinin immunohistochemistry in rectal suction biopsies from HD and non-HD patients. Methods. We retrospectively reviewed 99 rectal suction biopsy specimens including 4 repeat biopsies from 95 patients (34 HD and 61 non-HD). Each specimen was evaluated with hematoxylin-eosin, AchE histochemistry, and calretinin immunohistochemistry. Results. Of 95 patients, only 21 (22.1%) showed some ganglion cells. All 61 non-HD cases revealed no abnormal AchE-positive fibers. Of 34 HD patients, 32 exhibited abnormal AchE fibers, but 2 showed no stained fibers. None of the tissues from the HD patients exhibited calretinin immunoreactivity. Test sensitivity and specificity of AchE histochemistry alone were 93.5% and 100.0%, respectively, while calretinin immunohistochemistry were 100.0% and 85.2%, respectively. Conclusions. AchE histochemistry is a good diagnostic method for HD, if feasible, and a combination of AchE histochemistry and calretinin immunohistochemistry will help increase the accuracy of the diagnosis of HD.