N-myc oncogene of 29 neuroblastic tumors was measured and compared with known prognostic factors. The number of patients with the N-myc amplification of more than 3 copies was 17 (59%), and that of more than 11 copies was 11 (38%). All the fatal cases had more than 11 copies of N-myc. The amplified N-myc was observed in cases with advanced stages, suprarenal origin and the age of 1 to 4 years. However, the correlation with histology could not be found. The patients who had received preoperative chemotherapy but whose tumor could not be completely removed had a tendency to have an amplified N-myc. Thus, the genomic amplification of N-myc seemed to be a new clinically useful prognostic factor of neuroblastoma in children.
In order to clarify a developmental maturation of anorectal sphincterie mechanism, 96 normal children including 10 newborn-infants and 27 infants younger than one year of age were manometrically studied, using a perfusion system with open-tip catheter. Furthermore, 17 patients with rectal prolapse, of whom 13 (76%) were 2-year-old, were studied to elucidate pathophysiology of rectal prolapse in childhood and following results were obtained. 1) In normal infants and children, anal-canal pressure and square of high pressure zone have rapidly increased during infancy. It increased again from the end of pre-school age after a temporary cease in 2 to 3 years of age. Rectal pressure did not eiffer among various age groups. 2) Length of anal canal has rapidly increased in infancy and attained to level of adult before pre-school age. Frequency of rhythmical waves in anal-canal has gradually increased till pre-school age with the age. 3) Manometric studies were performed before and after therapy for rectal prolapse and it was revealed that there were characteristic findings such as an over-contraction of anal-canal after reflexive relaxation. The anal-canal pressure showed higher pressure after distension of the rectal ampulla than before. Resting pressure in anal-canal and square of high pressure zone were higher than those of normal children. It is concluded from these results that anorectal sphincterie mechanism may attain to maturity before 2 years of age and rectal prolapse in childhood may result from a contractive imbalance between levator muscles and external sphincter as its maldevelopment.
In 97 neonates receiving tatal parenteral nutrition, clinical assessment was made for a newly devised amino acid solution from the standpoint of plasma amino acid profile and nutritional effect. Correlation between plasma amino acid profiles and the dose of each amino acid administered was investigated, from which minimum, standard and maximum effective doses for each amino acid was determined. Further study is necessary to develop an optimal amino acid solution for total parenteral nutrition in neonates. From the above data described, a currently recommendable amino acid solution for total parenteral nutrition in neonates was proposed.
It is anticipated that various problems of digestion and absorption occur after digestive surgery on neonates and infants, Among them, maldigestion and malabsorption of fats is most troublesome, but only a few reports provide concrete descriptions. In order to study digestion and absorption of fats, purified 131^I-triolein test was newly developed by us and performed in pediatric patients who underwent abdominal surgery. In this test, the "Digestion-Absorption Index" (triglyceride/non-esterified fatty acic of fecal fat) was calculated to differentiate fat maldigestion resulting from fat malabsorption. Exocrine function of the pancreas was also studied by pancreatic function diagnostant (PFD). Following results were obtained: 1) Difference in the digestibility and absorbability of fats occurred depending on the age of patients; in particular, the infants under the age of two months showed marked fat maldigestion and malabsorption. 2) In congenital biliary atresia (CBA) very severe fat maldigestion and malabsorption were seen, but the exocrine function of the pancreas was normal. 3) Infants with pancreatitis, either non-complicated or complicated, showed pancreatic exocrine disorder and fat maldigestion and malabsorption. 4) Among surgical patients with congenital intestinal atresia, all with a short small in-testine left behind, those without ileocecal valve showed marked fat maldigestion and malabs-orption, and a poor result of PFD test. 5) Among various cases of enterostomy, patients with ileostomy showed marked fat maldigestion and malabsorption, and a poor result of PFD test.
Seven patients with imperforate anus (3 high type and 4 intermediate types) have under-gone posterior sagittal anorectoplasty. Two patients with high type required laparotomy for fistula closure, Clininical results in postoperative period are satisfactory. Further careful evaluation for longer periods is needed.
A new sclerosing therapy with OK-432, streptococcal preparation, was performed in 9 patients with cystic hygroma. Localized inflammatory reactions at the site of injections occured for 2〜3 days immediately after injections in all patients. Complete regression of cystic hy-groma was noted in 8 and marked regression in one out of 9 patients. No significant side effect was noted except for fever for 1〜2 days following the injections. In our exprience, one or two injections of OK-432 were enough for cure of cystic hygroma.
Plasma levels of amino acids and rapid turnover proteins (RTPs) and serum level of albumin were analyzed in four postoperative patients with biliary atresia. These patients presented decreased levels of branched chain amino acids and increased levels of aromatic amino acids in plasma. Though administration of branched chain amino acid-enriched diet (Elemental Diet for Hepatic Disease: ED-H) normalized plasma levels of amino acids, no significant increase in both of plasma RTPs and serum albumin were observed. As possible complications of this treatment, the decreased level of tyrosine and watery diarrhea were noted in one patient. Further trial should be required to assess the effect of this diet.
During the last 5 years, 18 pediatric surgical patients with septicemia were treated by exchange transfusion. The procedure was repeated in a total of 46 times until clinical symptoms and laboratory data improved. Thirteen patients recovered from septicemia. There were no complication due to exchange transfusion. We conclude that, in addition to removal of the infectious focus and conventional medical therapy, repeated exchange transfusion is very effective in treating septicemia, if carried out in its early stage.
Reconstruction of male urethra was performed with free bladder mucosa graft in 5 patients. Each patient had received previous unsuccessful hypospadias repair and had no usable redundant preputial skin. Technical points were 1) meticulous suture with fine suture material and fine instrument, 2) enlargement of anastomotic plane as far as possible. As a complication, urinary extravasation from the anastomotic portion was occurred temporarily in one patient, but no other particular complications such as urinary fistula or stenosis were experienced. This technique is available to urethral reconstruction not only for failed hypospadias repair but various situation of male urethral abnormalities.
A surgical case of interruption of the aortic arch (type A) in a 12-month-old boy weighting 4,610 g was reported. He was associated with a subarterial infundibular septal defect, severe mitral regurgitation and pulmonary hypertention without a patent ductus arteriosus. Surgical procedures were interposition of a ducron graft between left subclavian artery and the descending aortic stump, patch closure of the ventricular septal defect, and mitral valve replacement. His immediate postoperative condition was satisfactory but he expired on the following day of the operation because of cardiac dysfunction after reoperation.
Three female cases of conjoined thoracopagus twins are reported. All of these cases had conjoined hearts with complicated cardiovascular anomalies, fused livers, intestinal conjunctions and some other anomalies. Case 1 and 2 died of pneumothorax and gastric perforation respectively within three days after birth. The third case also died of cardiac insufficiency at the age of 57 days. Autopsies of these cases revealed that they could not had been separated because of shared common hearts. The embryology, incidence, diagnosis and treatment of thoracopagus twins were discussed.
A 1-year-old girl with bilateral metachronous Wilms tumor was reported. Because the initial clinical and perioperative examinations disclosed unilateral Wilms tumor of the right nephrectomy was done first. Despite of the administration of AMD and VCR, a lelt abdomi-nal mass appeared 1 month after the operation. Intravenous pyelography, aortography and CT disclosed the Wilms tumor of the left kidney. Total resection of the tumor of the left kidney was done on the 44th day after the initial operation. She was received chemotherapy of AMD, VCR and ADR for 48 weeks after the second operation. She is in good health without any signs of recurrence or metastasis for 3 years and 4 months after the second operation.
Rectal cancer in children is extermely rare. In this paper, we reported a 13-year-old boy underwent abdominoperineal resection and he is now doing well with no evidence of disease 10 years after operation. We collected 64 cases of patients younger than 20-year-old in the world and discussed with regard to a distinctive feature and prognosis. There is a 2: 1 male: female ratio. According to histologic study, 74% were what is called the mucinsecreting adenocarcinoma in-cluding signet-ring formation. Formerly, delay in early diagnosis was emphasised as cause of poor prognosis but the duration untill operation from onset was shorter than adult. Consquently, prognosis depends upon the charactristics of carcinoma.
A 51-day-old male infant with a volvulus of the sigmoid colon was admitted to Niigata University Hospital. The diagnosis of the volvulus was confirmed by means of plain X-ray of the abdomen and barium enema, and successfully reduced with hydrostatic pressure by barium enema. It is not so difficult to obtain precise diagnosis of the volvulus of the sigmoid colon by means of plain X-ray of the abdomen and barium enema study. The volvulus can be demon-strated by barium enema as a term "bird's beak sign" or "twisted taper sign". In adult cases, surgical resection of the affected bowel is recommended, but in infants and children conservative treatment may be more preferable because the incidence of recurrence is not so high in infants and children.
We experienced a typical case of Beckwith-Wiedemann syndrome. Prenatal ultrasonography demonstrated a bulging of fetal umbilicus and hydramnion at 23 weeks gestation. The neonate was delivered by cesarean section for rupture of amnionic bag to a gravida II, para I, 26-year-old mother at 34 weeks of gestation. Physical examination of the newborn revealed a birth weight of 2,630g and an estimated gestational age of 37 weeks. The tongue was prominent and a few indentations were present in the bilateral ear lobes. A nevus flammeus on forehead extended to the nose and eyelids. Hernia into the umbilical cord, 4X3.5X11.5cm, and clitoromegaly were present. There was no asymmetry of the body. Hernia into the umbilical cord was repaired on the first day. Two hours after the birth, blood glucose level was 19 mg/dl. Hypoglycemia was treated with an intravenous glucose infusion. Hypoglycemic state lasted for 6 days after the birth. Chromosomal study was normal. At 21-day-old, ultrasonography demonstrated bilateral mild renal hypertrophy. But renal function was normal. She is now l-year-3-month-old, and appropriate in growth for her age. Large tongue and ear lobe indentations are present. A nevus flammeus becomes more light in color. There are 70 case reports of Beckwith-Wiedemann syndrome in Japan. Our case had exomphalos, macroglossia, ear lobe indentations, nevus flammeus, hypoglycemia, and viscer omegaly. Such a typical case is very rare. Beckwith-Wiedemann syndrome needs to be followed up by paying careful attension to respiratory failure by macroglossia, hypoglycemia, and malignancy.
Reversed rotation of the midgut is extremely rare among anomalies of intestinal rotation. A 22-day-old premature boy presenting with abdominal distention, vomiting, and melena was emergently admitted to this hospital. A diagnosis of the perforation of gastrointestinal tract was made by the finding of abdominal plain film. Emergency laparotomy revealed the liver and entire colon ipsilateral type according to the Estrada' classification of reversed rotation, combined with a midgut volvulus of 810 degrees in a counterclockwise fashion and a perforation of the upper jejunum. A volvulus of the midgut was untwisted, and Ladd's operation with additional appendectomy were performed. Then partial resection of the jejunum followed by an end to end anastomosis was performed. Postoperatively, Douglas' abscess and subsequently sepsis developed. He died of sepsis and the bleeding of trachea on the 46th postoperative day.
A female infant weighing 3,800g was born at 41 weeks of gestation in a satisfactory cource to a healthy 23-year-old primipara. The infant had a parasite conjoined with her sacrococcygeal region. The autosite had no other anomalies. The asymmetrical conjoined twins were easily separated by operation 36 hours after birth. The parasite was 730g in weight. It had a femur, a tibia, a fibula, seven phalanger and hypoplastic pelvis. Vertebral structures were not present. There was no connection of viscerae between the parasite and the autosite. It had well develoed intestines, multilocular cyst, unilocular cyst, and lower limb. Histlogically, the wall of multilocular cyst consists of tissues of three germ layers that has differentiated to the various degrees. It was diagnosed, there-fore, as the Sacral Parasite. After the operation, her growth and development are favorable. Reviewing the Japanese literatures, the authors could find only 2 cases of sacrococcygeal parasite. The pathogenesis and diagnotic criteria of this anomaly were discussed.
A 2-year-old boy had been experiencing repeated episodes of intussusception for five month. In one of these attacks, the exploratory laparotomy was performed. The mass was not palpable at the leading point of the intussusception. On December 19, 1984, he was readmitted to our university hospital with abdominal pain and vomiting. A barium enema examination revealed an intussusception in the splenic flexure, but hydrostatic reduction was imcomplete. The emergency laparotomy was performed. The intussucception was reduced with Hutchinson's method. About 30cm proximal to the ileocecal valve at the antimesenteric side, a mass, measuring 2.5 by 2.0 by 2.0cm, was found. Consequently a llcm segment of ileum resected and end-to-end anastomosis was made. Microscopic examination revealed gastric mucosa including chief and parietal cells with rugose type. The patient was discharged on the seventeenth day after operation without any significant complication. Heterotopic gastric mucoca in the small bowel, other than in the Meckel's diverticulum or other congenital anomalies, is exceedingly rare. To our knowledge, sixteen such cases in children have been reported in English and Japanese literature. The recurrent intussusception would be worth while searching such local causes including heterotopic gastric mucosa.
We encountered a 5-month-old male infant with biliary peritonitis due to perforation of the common bile duct, which was treated by intraperitoneal drainage. A postoperative cholangiogram revealed a thum-tip sized cyst at the duodenal opening of the common bile duct (Alonso-Lej III-typed choledochal cyst, or choledochocele). Transduodenal resection of the cyst was achieved 30 days after the first operation. The postoperative course was uneventful. The incidence of Alonso-Lej III-typed choledochal cyst is very low and moreover the presented case appears to be the youngest one in the literature. Pathogenesis, classification and treatment of the lesion were also discussed on the basis of 31 collected cases.
A 10-year-old boy suffering from von Recklinghausen's disease complained of chest pain with common cold symptoms. Detailed examinations revealed dumbbell-type tumor located in the left upper mediatinum. There were no neurologic symptoms. Transthoracic excision of the tumor was successfully achieved. The tumor possibly origi-nated from sympathic trunk intruding through the 3rd and 4th thoracic intervertebral foramina. Histological examination disclosed neurofibroma. Post-operative course was uneventful. Mediastinal dumbbell-type neurofibroma is very rare affection of von Recklinghausen's disease and only 5 cases were found in the literature. In this paper it is emphasized that early diagnosis and treatment are invariably necessary in order to avoid irreversible neurologic disorders secondary to dumbbell-type tumor, although its affection is rare in von Reckling-hausen's disease.
A one-year-old female infant developed chronic respiratory failure due to congenital central alveolar hypoventilation. We implanted a diaphragm pacemaker (Glenn's radio-frequency type) to the patient, and her respiratory status was improved. Although this apparatus devised by Glenn has been widely used in the united states, clinical applications in Japan were rarely reported. Diaphragm pacing might be one of the effective treatments to the patient with chronic respiratory failure. We discussed the indication, mechanical subject and surgical approach concerning diaphragm pacemaker implantation.