Hideyuki Uotani

A case of congenital midline cervical cleft with congenital heart disease

A congenital midline cervical cleft (CMCC) is a rare developmental anomaly. It may represent failure of the branchial arches to fuse in the midline and presents at birth with a ventral midline defect of the skin of the neck. Congenital heart disease along with CMCC is rarer, and most of the cases reported are associated with chest wall defects or thoracic ectopia cordis. The authors report a case of a 5-month-old girl with CMCC and an atrial septal defect (ASD) and discuss the clinical presentation, embryologic development, and treatment.

A case of resection under the IVC-atrial venovenous bypass of a hepatoblastoma after intraarterial chemotherapy

An 11-month-old infant with a huge hepatoblastoma occupying almost the entire liver was admitted to the hospital. Serum alpha fetoprotein (AFP) level was elevated to 685,120 ng/mL. Combination chemotherapy with continuous arterial infusion of tetrahydropyranyl Adriamycin (THP-Adriamycin) and cisplatin based on the 91B1 protocol of the Japanese Study Group For Pediatric Liver Tumor (JPLT) was administered as the adjuvant chemotherapy. The tumor responded to three courses of chemotherapy and shrank in size, although venocavography showed that the inferior vena cava (IVC) was completely occluded below the entry of the hepatic veins. Right hepatic trisegmentectomy was performed with an IVC-atrial venovenous bypass that prevented massive bleeding. In this case, it was recognized that the IVC-atrial venovenous bypass was advantageous in an infant. The procedure is very simple and the blood flow obtained by the bypass was sufficient. Two weeks postoperatively, three courses of chemotherapy were initiated after the protocol. The patient remains well without signs of recurrence 39 months postoperatively, and the AFP value has remained within 10 ng/mL.

Ileoileal Intussusception and Ileal Stricture Associated with Necrotizing Enterocolitis in a Premature Infant : Report of a Case

Abstract A 630-g male infant developed presumed necrotizing enterocolitis (NEC) after fungal enteritis that resulted in subsequent fungal septicemia associated with pneumoperitoneum on the 9th day of life. Urgent percutaneous Penrose drainage was required. During the operation on the 14th day, an ileoileal intussusception and an ileal stricture were found with perforations on each oral side of the lesions. The distinction between NEC and intussusception in premature infants when they coexist can be difficult, as these conditions share common symptoms. The relation between ischemic injury and dysmotility of the intestine is also discussed. The similarity of intrauterine fetal distress and NEC in premature infants, as seen in the present case of the intussusception associated with focal NEC, suggests that mesenteric and enteric vascular ischemia could be one trigger of intussusception in neonates.

Expression of GLUT-1 and GLUT‐3 in Xanthogranulomatous Cholecystitis Induced a Positive Result on 18F-FDG PET: Report of a Case

Although several reports have revealed that fluorine-18 fluorodeoxyglucose positron emission tomography ((18)F-FDG PET) is useful for differentiating between benign and malignant lesions in the gallbladder, the positive results of (18)F-FDG PET are not specific for malignancy because (18)F-FDG is also accumulated in inflammatory lesions. It is known that the most important pathway for (18)F-FDG to enter the cell body is mediated by the facilitative glucose transporter-1 (GLUT-1) through GLUT-3. We herein present a case of xanthogranulomatous cholecystitis (XGC) with a positive result on (18)F-FDG PET. In this case, GLUT-1 and GLUT-3 were both positively expressed in inflammatory cells at the gallbladder wall of XGC and this is the first report to reveal GLUT expression in XGC. This report reveals that surgeons should carefully consider the appropriate treatment of gallbladder tumor, even with a positive result on (18)F-FDG PET.

Non-Hodgkin's Lymphoma of the Ascending Colon in a Patient with Becker Muscular Dystrophy: Report of a Case

Abstract We herein present the findings of a 10-year-old boy with non-Hodgkins lymphoma of the ascending colon which caused intussusception and intestinal bleeding. He had a history of Becker muscular dystrophy. However, he had neither hypertrophic calves nor cardiomyopathy, and his serum creatine kinase (CK) level always exceeded 2 000 IU/l. Preoperatively, a laboratory examination revealed high serum levels of CK (2 038 IU/l), aspartate aminotransferase (AST), alanine aminotransferase (ALT), and lactate dehydrogenase (LDH), and the blood hemoglobin level was 7.0 g/dl. A barium enema examination revealed an intussusception in his ascending colon, which was found to be a highly vascular tumor on Doppler ultrasound scans. A right hemicolectomy was performed. Macroscopically, the 5 × 6 × 8-cm solid tumor of the ascending colon resembled a submucosal tumor and had two ulcerous lesions at the tip. The tumor was histologically diagnosed to be a diffuse large B-cell lymphoma of the ascending colon. General examinations revealed no involvement of lymphoma postoperatively. At 13 months after surgery, the CK (3 786 IU/l), AST (110 IU/l), ALT (138 IU/l), and LDH (420 IU/l) levels are still high, and the patient is doing well without any signs of recurrence.