Congenital stenosis of the hepatic duct at the porta hepatis in children with choledochal cyst.

BACKGROUND: The intrahepatic gallstone is known as one of the complications of choledochal cyst. Stenosis of the hepatic duct may contribute to the formation of the stone. This report describes concurrent congenital stenosis of the hepatic duct identified during the primary operation of choledochal cyst. METHODS: Fourteen children were operated on for choledochal cyst. To identify the concurrent stenosis, cholangiography and inspection of the hepatic duct were performed during the operation. In those patients with identified membranous stenosis, the membrane was resected. In those with nonmembranous stenosis, the wide hilar hepaticojejunostomy was performed after hepatic duct plasty. RESULTS: Eleven sites of hepatic duct stenosis were identified, 8 membranous stenoses and 3 relative stenoses without membrane. The membranous stenoses were classified into 4 subtypes as follows: a small central opening, a marginal opening, 2 openings, and a bridgelike structure. At the primary surgery on choledochal cyst, those stenoses were released, and the formation of the gallstone has not been observed in 4 to 11 years in all cases.

Matrix metalloproteinase-9 expression in congenital diaphragmatic hernia during mechanical ventilation.

PURPOSE: Matrix metalloproteinase-9 (MMP-9) is detected in lung tissues subjected to ventilator-induced injury and is involved in the process of lung injury. We investigated the immunohistochemical expression of MMP-9 in the bilateral lungs of newborns with congenital diaphragmatic hernia (CDH) during mechanical ventilation and evaluated the degree of damage based on MMP-9 expression. METHODS: Lung tissue samples were obtained during autopsy from six newborns with CDH. Control lung tissue samples were obtained from two of these newborns; one who died of persistent pulmonary hypertension after being ventilated for 2 days, but whose bilateral lungs were not subjected to the compressions of herniation, and one who died of bilateral diaphragmatic hernias soon after birth, but was not subjected to artificial ventilation. The other four newborns with CDH had unilateral Bochdalek hernias. Immunohistochemical detection of MMP-9 expression was done using a wet autoclave antigen retrieval method on sections from formalin-fixed and paraffin-embedded lung tissue. RESULTS: The reaction and distribution of MMP-9 was strongly positive in the alveolar macrophages in thickened alveolar septi and ducts, and in the inflammatory cells around the parenchymal hemorrhage and intra-alveolar spaces. Only the four patients with unilateral Bochdalek hernia had widely positive MMP-9 immunoreactivity in the unaffected side, as well as the affected side. CONCLUSIONS: Based on MMP-9 expression, the lungs of newborns with CDH were damaged bilaterally during mechanical ventilation.

A case of pediatric ovarian dysgerminoma associated with high serum levels and positive immunohistochemical staining of neuron-specific enolase.

A 5-year-old girl presented with a painful abdominal mass. Abdominal magnetic resonance imaging (MRI) showed 3 separate masses. Tumor markers including lactate dehydrogenase (LDH), cancer antigen-125 (CA-125), beta-subunit of human chorionic gonadotropin (beta-hCG) and neuron-specific enolase (NSE) were elevated. At operation, the main tumor arose from the left ovary and was associated with torsion, whereas the other lesions were lymph node metastases. A salpingo-oophorectomy was performed. Histopathologic examination indicated that the tumor was a dysgerminoma. Immunohistochemicallly, the cells were positive for NSE and placental alkaline phosphatase (PALP) but were negative for CA-125, beta-hCG, S-100, glial fibrillary acidic protein, and vimentin. The elevated serum levels of tumor markers improved dramatically after the operation and chemotherapy.