Hiroshi Ohgami

Different genomic and metabolic patterns between mass screening-positive and mass screening-negative later-presenting neuroblastomas

The mass screening of neuroblastoma has been undertaken in Japan by measuring urinary vanillylmandelic acid (VMA) and homovanillic acid (HVA) in all infants at the age of 6 months. This program may not only improve the prognosis but also provide important insights into the biology and evolution of human neuroblastoma. The authors studied and discuss the clinical significance of the N‐myc oncogene, catecholamine metabolism, and other tumor markers in 43 patients with neuroblastoma who underwent the urinary screening test at 6 months of age. Thirty patients were found by the screening, and 13 were negative at the screening but later had a tumor. In the former group, the tumors were mostly in early stages (Stage I, 12; Stage II, 11; Stage III, seven), no amplification of N‐myc was observed, and all patients are alive without disease. Although two patients whose urine at the screening showed elevated VMA and HVA levels and accidentally were not treated for 13 and 17 months, there was no change in the values of VMA and HVA during that time. However, in the latter group, the tumors were mostly in advanced stages (Stage I, one; Stage III, four; Stage IV, eight), and N‐myc amplification was observed in seven of 13. Only two of these 13 are alive without disease. The age at diagnosis of the screening‐negative group was 23 months compared with 8 months in the patients identified by screening, and the pattern of catecholamine metabolites in the screening‐negative group tended to be dopaminergic with a low VMA–HVA ratio, especially in cases with N‐myc amplification. These data suggest that the screening‐positive patients with neuroblastoma may have favorable characteristics, and the biology of these tumors may be different from that of screening‐negative later‐presenting tumors. They also suggest that there may be at least two distinct subsets of neuroblastoma. For the early detection of the poor prognostic neuroblastomas, the measurement of urinary dopamine with VMA and HVA at later ages, such as 1 to 2 years, should be considered.

Mucoepidermoid tumor of the bronchus in an infant

This is a rare case of mucoepidermoid tumor of the bronchus in a 4-yr-old boy who was treated by lobectomy of left lower lung.

Complete disruption of the main pancreatic duct: A case successfully managed by percutaneous drainage

The authors report on a 7-year-old boy who sustained blunt abdominal trauma on a bicycle handlebar. A large traumatic pancreatic pseudocyst developed, for which percutaneous external drainage under ultrasound guidance was performed. Both the catheter fistulogram and endoscopic retrograde cholangiopancreatogram showed complete disruption of the main pancreatic duct. During continuous external drainage, the pseudocyst disappeared. The drainage flow decreased gradually and ceased. The patient is well, with normal endocrine and exocrine pancreatic functions, 2 years after discharge.

Remnants of urachus in infants and children —The problems of diagnosis and treatment—

A total 17 cases of remnants of urachus were examined between 1981 and 1989, including 2 cases of patent urachus, 5 of urachal cyst, 9 of urachal sinus, and 1 of urachal diverticulum. A long urachal sinus was considered to be caused by the self-destruction of a cyst. While patent urachus was diagnosed in neonates with urine discharge from the umbilicus, cysts or sinuses accompanied by infection were more often found in older children. Imaging of the burrows was diagnostic in 8 out of 11 cases, ultrasound examination in 9 out of 12, and CT examination in all of 3 cases. Since this condition is frequently accompanied by malformations of the urinary system, attention must be paid to locating the accompanying anomalies. Although primary extraction was performed in 13 cases, the primary treatment of cases with highly infected cysts should be drainage, followed by secondary extraction. Furthermore, caution must be exercised to avoid hemorrhage in cases with dilation of the umbilical artery.

Management of colorectal polyps in children.

Forty‐two patients each with a colorectal polyp have been treated in our hospital over the past 10 years. In twenty‐two cases the polyps were noted in the rectum, nine in the sigmoid colon, seven in the descending colon and four in the transverse colon. Each patient had only one polyp. Hematochezia was the main symptom in 29 patients, prolapse of the polyp from the anus in 10, abdominal pain due to intussusception in two and no symptoms were observed in one. Auto‐amputation of the polyp was considered to have occurred in five patients. Twelve rectal polyps were resected from a transanal operation, and another 25 polyps were removed endoscopically with electric cautery. We have had no experience of endoscopic complications such as bleeding or perforation. A histological examination revealed an adenoma in one patient. Other polyps were non‐neoplastic and were classified as juvenile, inflammatory and hyperplastic in 30, two and four patients, respectively. There have been no recurrences of polyps to date.

An infantile case of infiltrating lipoma in the buttock

This paper presents a rare case of infantile infiltrating lipoma in the buttock. The usefulness of magnetic resonance imaging of the tumor in the follow-up study is discussed.

Management of traumatic pancreatic pseudocysts in children

Eight children with pancreatic injuries after blunt abdominal trauma are presented. One patient with pancreatitis without complications was treated successfully with medication; seven (87.5%) developed pseudocysts, which resolved spontaneously with conservative treatment in four cases (57.1%). Non-resolving pseudocysts in 3 patients finally required surgical intervention. Pseudocysts that resolve spontaneously and those that require surgical intervention can be distinguished. The two groups showed different time sequences in both serum amylase values and pseudocyst size. Analyses of these clinical data revealed three factors with significant predictive value. On the basis of these findings, a new management strategy for traumatic pancreatic pseudocysts in children is proposed. The cyst is likely to resolve spontaneously in cases showing any two or more of the following factors: (1) cyst presentation after day 6; (2) maximum serum amylase below 1,600 IU/l; and (3) serum amylase below 600 IU/l on day 20. However, early surgical intervention is required in the presence of two or more of the following: (1) cyst presentation before day 4; (2) maximum serum amylase above 1,900 IU/l; or (3) serum amylase above 1,200 IU/l on day 20.

Simultaneous occurrence of Henoch-Schnlein purpura and acute appendicitis

We report a case of simultaneous occurrence of Henoch-Schönlein purpura and acute appendicitis in a 6-year-old girl. Her complaints and the findings on physical examination were typical of acute appendicitis. Ultrasonic examinations showed a swollen appendix with fluid collection. Emergency laparotomy demonstrated perforating appendicitis with abscess formation. Melena had started the day before the operation, and skin purpura developed on the 4th postoperative day. The serum factor XIII level was reduced to 48%. The administration of both steroids and factor XIII concentrate alleviated the associated symptoms. Immunohistochemical examination of the excised appendix revealed deposits of immunoglobulin A on the vascular wall.

Bleeding tendency in children with biliary atresia

Sixty-eight childrenwith biliary atresia (BA) were admitted to the Department of Pediatric Surgery, Kyushu University Faculty of Medicine and fukuoka Municipal Childrens Hospital, from 1971 to 1986. In 11 (16.2%) there was a tendency toward bleeding associated with obstructive jaundice and breast feeding. In 4 of the 11 cases, vitamin K deficiency (VKD) was diagnosed by coagulation studies. The bleeding tendency improved after administrationn of vitamin K in all 4 cases; the other 7 were also suspected of having VKD. The usefulness of the Normotest [7] for the diagnosis of VKD and for mass screening of infantile hepatobiliary disorders, particularly BA, is discussed.