Early diagnosis and differentiation of CBA from neonatal hepatitis is very essential. Many investigations including labolatory, radiographic and radioisotopic tests have been practised. Ultrasonographic examination of the liver and biliary tree is a recent addittion to these tests. Twenty-one cases of intants with hyperbilirubinemia who had US examinations and other tests have been studied and reviewed. In seven patients gall bladder could not be identified by US examination and preoperatively diagnosed as CBA. One case of CBA had small, round, atrophic gall bladder. Cystic dilatation of extrahepatic biliary tree were found in some special cases of CBA. All patients with neonatal hepatititis and congenital biliary dilatation had normal gall bladder which shrinked after Ceosnin injection. However the gall bladddr in infants with CBA did not change in shape and size after Ceosnin administration. US examination offer many usuful informations in patients with suspected CBA for differentiation of obstractive jaundice.
A histopathological study of the gallbladder, particularly a comparative study to the fetal gallbladder, in fifty-two biliary atresia patients, was done who had undergone the operation at Juntendo University Hospital. In cases of so-called uncorrectable type, very hypoplastic gallbladder which was similar to the fetal gallbladder at two to three monthsgestation was buried in narrow fissure of liver parenchyma. On the other hand, in correctable type, the gallbladder was well developed and appeared outside of liver parenchyma. Histological findings of the gallbladder in biliary atresia patients were divided into three groups. Group I (25%) showed almost normal. Group II (56%) showed flattened epithelium and disappeared folds. Group III (19%) showed no lumen and disappeared epithelium. The histological change of the gallbladder was severer, the size of the bile duct in the remnant of extra-hepatic bile ducts at porta hepatis was smaller (p<0.05). However, liver fibrosis and postoperative bile flow did not correlate with histological changes of gallbladder in biliary atresia.
The therapeutic usefulness of cisplatinum (CDDP) as an anticancer agent has been well documented, but the serious nephrotoxicity remains as major hazard in clinical use. Liposome-encapsulated cisplatinum (CDDP-liposome) was employed to arrest the aberrant growth of cultured Human neuroblastoma cells IMR-32, and further to reduce the side effect of CDDP for its clinical use. CDDP-liposome was prepared by the modified Forssen's method (Cancer Rec. 43, 546-550, 1983), i.e. ,phosphatidylcholine (PC) : phosphatidylserine (PS): cholesterol =6: 2: 3 on molar ratio. After sonication CDDP-liposome was separated from free drug by gelfiltration (Sephadex G-50). CDDP-liposome dose-dependently inhibited the cell growth of IMR-32 in a similar fashion to free CDDP. While 2.0μg/ml of free CDDP induced 50% inhibition of DNA synthesis (ID50) of IMR-32, only 0.7μg/ml of CDDP encapsulated in liposome, approximately 1/3 concentration as high as free CDDP, did ID50. In contrast, CDDP-liposome showed a lower inhibitory effect on normal mouse fibroblast 3T3 (ID50: above 10.0μg/ml) than free CDDP, but on rat normal glioblast required almost same drug-dose as free CDDP. CDDP-liposome gave rise to 3-fold higher incorporation (1.4μg CDDP/mg protein) into IMR-32 than that of free CDDP (0.5μg CDDP/mg protein) at the same CDDP concentration (30.0μg/m/). These results suggest that CDDP-liposome, showing an effective growth inhibition through the selective drug-incorporation, is applicable to clinical use for neuroblastoma in the future.
Bone mineral content (BMC) and bone width (BW) of the radius were measured by photon absorptiometry in 120 children from neonate to 14-year-old and 10 patients with congenital biliary atresia (CBA) living more than 3 years after operation. Though the BMC and the BMC/BW values were well correlated with aging, the BMC/BW value was more appropriate than the BMC value as an index of bone mineralization. Some parameters which were obtained from microdensitometry with X-ray films of the metacarpal bone in 43 normal children were also correlated with aging and the BMC/BW values measured by photon absorptiometry. Ten patients with CBA were classified into 2 groups according to their postoperative clinical courses. The group A included 5 patients who had neither jaundice nor portal hypertension, while the groug B included 5 patients with various degree of liver dysfunction, hepatosplenomegaly and portal hypertention. The BMC/BW values in the both groups were distributed within normal range and the values in the group A were increasing with aging as well as in normal children, while in the group B such increases of the BMC/BW values associated with aging were not found, but only slight increases or even gradual decreases were often found. BM-Age was calculated from a measured BMC/BW value in a patient with CBA and the regression equation between the BMC/BM and age in normal children. Age Difference (A.D.) between the BM-Age and the chronological age was a useful indicator for evaluating bone mineralization in postoperative patientss with CBA. Especially changes of the A.D. assorciated with aging had a good relationship with the postoperative courses and the average values of the A.D. in the group B indicated a significant retardation in bone mineralization compared to the group A. Serum 25-OH-vitamin D and calcium also correlated with the changes of A.D. and postoperative courses of the patients with CBA.
During the past three years period, we used 34 Broviac catherters for long-term total parental nutrition in infants and children. The mean duration of a single catheter could be plolonged for 147 days, because accidental removal of the Broviac catheter was not occured. The incidence of removal of the catheter for total complications was one per 255 days, and the incidence of removal for sepsis was one per 291 days. Leaks or cracks of the catheters were seen in four tsmes. For further plolongation of catheter's life-span, the prevention of catheter damage should be considered.
The causes of postoperative prolongation of jaundice were investigated in 18 patients who showed prolonged jaundice after definitive operation for biliary atresia. Following results were obtained: 1) Some patients had abnormal high levels of serum transaminase, alkaline phosphatase, and γ-GTP preoperatively. 2) Portal pressure at the time of definitive operation was comparatively high, more than 200mmH_2O, in most of the patients whose serum bilirubin level was more than 5mg/dl. On the other hand, the portal pressure in patients whose bilirubin level was less than 5mg/dl, was mostly under 200mmH_2O. There was, however, no significant difference between them and non-interic patients. 3) Although it is not the leading factor, ascending cholangitis is one of the significant cause of nrolnncfped jaundice. 4) Almost all of the patients who showed prolonged jaundice showed a tendency toward elevation of the γ-globulin and/or TTT and/or γ-GTP in the two or three months period after the operation. When these tendencies are seen postoperatively, the patients should be treated more carefully.
A mass screening system of neuroblastoma by means of a vanillylmandelic acid (VMA) in 6-month-old infants has now became common in almost all areas of Japan. Nagoya city started this system in 1977 (the 2nd earliest in Japan), and Aichi prefecture in 1981. By March 1986, 231,971 infants were examined, and eleven asymptomatic neuroblastoma were discovered. The incidence of neuroblastoma by this system was one out of 21,000 infants. Eight patients including three disseminated cases were treated at our institution. All of them have completed their treatment and are alive without any evidence of the disease. Their disease-free intervals have lasted from 15 months to 6 years. The mass screening system contributes to the improvement of treatment of neuroblastoma. Careful attention, however, should be paid to those whose results were negative, as neuroblastoma could occur later.
A case of patent urachus was presented in this paper and briefly discussed regarding to the diagnostic method and treatment. A 1-month-old female infant was admitted to our hospital because of granulomatous mass and serous discharge from the umbilicus. As a result of fistulography, the fistula was found from the umbilicus toward the apex of the bladder. The complete excision of the urachus was performed for the therapy. Pathological features of the fistula showed granulation tissue with the inflammatory response characterized by cellular infiltration, fluid exudation and degeneration. The tubulal structures lined by transitional epithelium, which were suspected to be originated from the urachus, were seen in the deep portion.
Corrective surgery was performed in three cases of cloacal anomaly and one case of possible cloacal anomaly. All of them had underwent rectal pull-through previously. Vagino-plasties were performed at the age of 4 and 5 years. The problems of difficulty for correction of vagina at the separated operation from rectal pull-through were dicussed. In case 1, perineal skin flap vaginoplasty was performed. In case 2, abdomino-perineo-sacral vagino-rectal pull-through was performed after removal of previously pulled through rectum. In case 3, which was associated with prune belly syndrome, abdomino-perineal vaginal pull-through was performed. In case 4, the right sided vagina was anastomosed to the left sided uterus and the left sided vagina was pulled through down to the perineum. This case had a tiny opening at the tip of clitoris that might be cloacal opening.
An 8-year-old girl with dermatomyositis was reffered because of right ureteral perforation. Initially, a tube nephrostomy was performed, but resulted in loss of the long segment ureter. After the complete clinical improvement of dermatomyositis, she was operated to provide internal contuinity of the right ureter. The vermiform appendix was utilized as a conduit from the right renal pelvis to the lower ureter with satisfactory function after one year and 6 months. Our satisfactory experience with one patient may show the efficacy of the appendix ureter-oplasty when the kidney has little mobility and the appendix has the required caliber and length.
Superior mesenteric artery (SMA) syndrome in caused by a mechanical compression of the 3rd portion of the duodenum due to the superior mesenteric artery, and results in a duodenal obstruction with bile stained vomitus. Three children with SMA syndrome are presented, and their clinical features were reviewed in comparison with 186 cases in the Japanese literatures. All of the 3 children were treated successfully by total parenteral nutrition, postural changes and nasogastric drainage under starvation for several days. The male-female ratio of the 186 cases nas 1.7: 1. Although the average age at the onset of the disease was 23 years old, the peak of ages was in teens. Most children with SMA syndrome were often caused by rapid growth, while patients over 16 years of age were mainly due to malnutrition developed after major surgery. The average value of weight for height at the onset was 83.7±12.5%. SMA syndrome can be often managed by conservative treatments with favorable results.
A 5-day-old female newborn presenting a large abdominal mass was admitted to our institute. Ultrasonographic study revealed a cystic mass in her lower abdomen. A vaginal bulge due to imperforate hymen was observed. Hydrometrocolpos was diagnosed preoperatively and operation was performed with perineal approach. Imperforate hymen was insized and 155ml of light pink cloudy fluid was obtained, and then the abdominal mass was diminished. Postoperative course is satisfactory. A collection of 13 cases of hydrometrocolpos in Japanese literatures suggests that in any case of female neonate or infant with an abdominal mass, the perineal resion should be carefully examined to prevent inadequate lapalotomy.
A 14-year-old girl having hepatocellular carcinoma (liver cell carcinoma) associated with positive hepatitis B surface antigen (HB_s-Ag) was reported. She had been suffering from type B hepatitis when she was 9 years old. The tumor of 13cm in diameter was located in the left hepatic lobe. Left hepatic lobectomy was performed. HB_s-Ag was demonstrated in serological examination. Histologically Orcein-stain-positive cells were present in non-neoplastic liver tissues. She was suspected to be a case of maternal transmission because her mother and brother were HB_s-Ag positive carries. There was no cirrhosis in the liver tissue. Nine months after the hepatectomy, lung mstastasis was found out. The chemotherapy was not effective, and 14 months after the first surgery, the right lower lobectomy was done. However she died 8 days after the second operation. Review of the Japanese literatures disclosed 10 other cases of HB_s-Ag positive hepatocellular carcinoma in childhood. They may imply the significant relationship between HB virus and hepatocellular carcinoma not only in adult but also in childhood.
Two-stage arterial switch operation was successfully performed in a 14-month-old patient with Taussig Bing anomaly complicated with coarctation of the aorta. Preductal coarctation with hypoplastic distal portion of aortic arch was reconstructed using end to end anastmosis and with carotid flap aortoplasty. Pulmonary artery was banded 27mm in circumference at the age of 18 days. Definitive operation was carried out at the age of 14 months due to the progression of heart failure and cyanosis. D-shaped intraventricular buffle was applied between subpulmonic VSD and the origin of pulmonary artery through right ventriculotomy and great atreries were switched according to Lecompte's maneuver. Cardiac catherization and angiography on the 38th postoperative day showed neither evidence of aortic regurgitation nor stenosis at the site of anastmosis. The patient is doing quite well and shows excellent weight gain 29 months postoperatively.
We operated on a 5-year-old girl with adenomatous goiter and hyperthyroidism. The patient had delayed growth and a large goiter since the age of 6 monthsold, when the serum level of thyroid iodine was high, and the diagnosis of hyperthyroidism was made. Treatment with propylthiouracil (PTU) was started. Circulating thyroid antibodies and long-acting thyroid stimulator (LATS) were not detected. The patient continued to require PTU in order to remain euthyroid. At the age of 5 yearsold, the goiter enlarged and became nodular, so subtotal thyroidectomy was done. The diagnosis of adenomatous goiter was made. The postoperative course was uneventful. One year and 10 months after the operation, the patient remains euthyroid without medication. The majority of patients with adenomatous goiter are adults and are clinically euthyroid. Infants with hyperthyroidism associated with adenomatous goiter are very rare.
A case of long-gap esophageal atresia (Gross A type) was presented, in which an end-to-end anastomosis was successfully established by a combination of esophageal elongation technique and "spiral esophagomyotomy". Advantages from this spiral myotomy are as follows: 1) In circular myotomy the amuscular segment often becomes dilates with time, forming diverticulum. In spiral myotomy, on the contrary, the diameter of the myotomized portion becomes smaller due to a torsion effect along the spiral incision caused by a downward stretching of the upper pouch, preventing diverticular dilatation. 2) By addition of the torsion, the diameter of the dilated upper pouch diminishes, making an anastomosis between the upper and the lower pouch more feasible. 3) Networks of nervous and vascular systems do not seem to be impaired so much in the spiral incision as in circular myotomy.
We experienced a rare case of tricuspid valve obstruction secondary to Candida endocarditis. The neonate on the forth day of age was admitted to our hospital for vomitting and abdominal distension. Operative findings suggested total colon agangliosis with severe enterocolitis. She felt in a critical state of panperitonitis following multiple perforation of the intestine, sepsis and DIC after the operation. She recovered from her septic state with massive resection of intestine, drainage operation, broad spectrum antibiotics therapy, total parenteral nutrition via a central venous catheter and exchange blood transfusion. But on the 58th day of age, she developed severe hypoxemia and positive blood culture of Candida. Two dimensional echocardiography indicated tricuspid valve obstruction with large ball-like lesion and severe right-to-left shunt at the atrial level. Autopsy findings revealed Candida endocarditis on the tricuspid valve. In this case report, diagnosis and treatment of mycotic endocarditis of critically ill children were also discussed.
One hundred and eleven cases of rhabdomyosarcoma were analysed on prognosis in this report. In the latter five years, the two-year tumor-free survival rate was significantly better. That was 45.7% (21/46) versus 25.5% (12/47). On operation, total resection, sub-total resection and other operative methods were compared in relation to age and site of tumor occurence. Between 1 and 3 years of age, total resection was better than other operative methods. In the totally resected group, the age between 1 and 3 was better than other age groups. On radiotherapy, the tumor-free two-year survival rates were compared among without radiation, under 3,500rad and over 3,500rad, in relation to stages. Only in the group over 3,500rad, Stage I was better than other stages significantly. On chemotherapy, VAC therapy was used only in 21.1% in the former 5 years, while it was used in 57.5% in the latter 5 years with or without use of Adriamycin. VAC therapy used over 6 months gave significantly better prognosis. VAC therapy did not give better results in any stages, when studied statistically. The problem was shortage of case number in this study. The analysed results were stored for future comparison in the same basis.
In 1986, 674 pediatric malignant solid tumors were registered to the Japanese Society of Pediatric Surgeons under the support of the Children's Cancer Association of Japan. Among these, there were 180 neuroblastoma group tumors, 54 malignant renal tumors, 52 liver tumors, 106 teratomas, 26 rhabdomyosarcomas and 256 other tumors. In 180 neuroblastomas group tumors, there were 110 neuroblastomas, 55 ganglioneuroblastomas and 15 ganglioneuromas. Twenty-nine neuroblastomas and 10 ganglioneuroblastomas were discovered through the VMA mass screening system in Japan. Total primary tumor resection was perfored in 118 cases, but metastatic tumor resection was not successful in 51 cases of them. There were 54 Wilms' tumors, including 3 bilateral tumors. Total resection of tumor was done in 44 cases. Postoperative chemotherapy was done in all except 2 cases. Among the liver tumors, there were 41 hepatoblastomas, 7 hepatocarcinomas and one un-differentiated sarcoma. In macroscopic findings, 36 were massive and 8 were multi-nodular in type. In stage distribution, 55% were in stage III and 22% were in stage IV. Liver resection was performed in 39 (75.6%), but radical resection was in 32 cases. In teratoma group tumors, 58 mature and 9 immature teratomas were included, besides 39 malignant teratomas. Among these, there were 9 testicular, 3 ovarial and 15 sacrococcygeal embryonal carcinomas. In 32 cases tumor was totally resected and in 4 subtotally resected. In 31 cases chemotherapy was given in various combination. In rhabdomyosarcoma, 11 were in pelvis, 6 were in head and neck region, 4 in abdomen and 3 in thorax.