No abstract

A 17-month-old girl who had been followed up as an extremely-low-birth-weight infant presented with hepatoblastoma in the right lobe of her liver. Preoperative angiography revealed an absence of the portal vein, and the visceral venous return was through the left renal vein into the inferior vena cava. No liver dysfunction and no jaundice were found; however, a marked elevation of the alpha-fetoprotein level was noted. She underwent a typical right hepatic lobectomy successfully after chemotherapy and has no evidence of recurrence 6 months after surgery.

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We used an Applied Alexis (Applied Medical Resources Corp, USA) wound protector and retractor (WPAR) instead of a "spring loaded silo" (SLS) for gastroschisis reduction because SLS is currently unavailable in Japan. Our patient was a 1,707 g female diagnosed prenatally with gastroschisis born at 35 weeks gestation by spontaneous vaginal delivery. Attempted primary closure failed and a WPAR was used as a silo. A piece of the bottom ring of the WPAR was cut out and a smaller ring was made from it, so it would fit into the patient's abdomen. Eviscerated organs were inserted into the retractor through the bottom ring, and the bottom ring was placed into the abdominal cavity through the gastroschisis defect without suturing to complete the silo. Operating time was 20 minutes. Three days later, all eviscerated organs had been reduced into the peritoneal cavity, the silo was removed, and the gastroschisis defect closed without difficulty. Postoperative recovery was uneventful and the WPAR was an excellent alternative for making a silo.

Japan has a nationwide mass-screening program for neuroblastoma in 6-month-old infants. Neuroblastoma can regress spontaneously, and some institutions observe selected cases. We evaluated the management of screened neuroblastoma at our hospital since 1997 when an observation program was introduced. Criteria for the observation program were stage-I, stage-II, or stage-IVs tumors, urinary vanillylmandelic acid (VMA) and homovanillic acid (HVA) levels <40 microg/mg creatinine, tumor <5 cm in diameter, no invasion to the intraspinal canal or great vessels, and parental consent to participate. Patients who did not meet observation criteria underwent surgery or mild chemotherapy according to the location of the tumor. If patients met observation criteria after chemotherapy, surgical intervention was no longer performed. Thirty-six patients attended our hospital for screened neuroblastoma from 1997 to 2002. Thirty-three patients who were managed at our hospital participated in this study. Ten subjects met observation criteria. Tumors regressed in 7 patients (mean follow-up period 36.3 months) with corresponding decreases in VMA and HVA levels (group A). Three underwent surgery (group B) because of increasing VMA and HVA levels, increase in tumor size, or guardian's request. Twenty-three subjects did not meet observation criteria. Four patients underwent primary surgery (group C), and 19 patients had chemotherapy initially. Fourteen patients met observation criteria after chemotherapy and two are still having chemotherapy (group D). Three patients required surgery due to insufficient regression of their tumors (group E). Fourteen subjects in group D had marked decreases in VMA and HVA levels and tumor size (mean follow-up period 29.1 months), and tumors were not detected using imaging techniques in 8 patients. Histological examination of all resected specimens during the study period showed favorable histology and no N-myc amplification. There was no evidence of unfavorable prognosis in any of the 33 subjects, although 1 patient who underwent primary surgery had a vanishing kidney 1 year later and 1 patient had multiple bony metastases after complete resection of tumor, which was treated by chemotherapy. Until the real significance of mass screening for neuroblastoma as a public health measure is confirmed, observation with careful follow-up should be adopted more extensively because it has a favorable outcome in many cases, and is associated with minimal therapeutic complications.