Thirty-three pediatric burn patients were managed by a newly planned initial hydration formula modified from Shriner's formula. In this new formula, the maintenance fluid volume was increased to 2,400ml per body surface area (m2) from 2,000ml of Shriner's. The patients were divided into two groupes, in Group 1 (16 patients) 4.5% glucose was added, while in Group 2 (17 patients) no glucose was added in initial hydration in order to clarify the disadvantages of glucose addition into resuscitation fluid for burn shock. The results of this study are summarized as follows : 1) All patients were resuscitated well from burn shock without any more volume increment, and no critical cardiopulmonary complication occurred in refilling period. 2) In Group 1 (4.5% glucose added) hyperglycemia, glucosuria, polyuria, and other metabolic adverse reactions were prominent in comparing to Group 2. 3) Although albumin was added in the same volume as Shriner's formula, serum protein and colloid osmotic pressure fell into profoundly low level. But it's addition was considered to contribute to maintaining the circulating blood volume without any more fluid increment. In conclusion, this newly planned initial hydration formula is very proper for pediatric burn resuscitation, but no glucose should be added in it.
As antireflux operation for vesicoureteric reflux, Politano-Leadbetter procedure was performed in 54 infants and children (88 ureters) during 10 years until 1983. Two infants with IVth degree refluxing megaureter presented a stricture at ureterovesical anastomotic junction which required a secondary repair. We found this procedure is rather difficult and complicated one especially in young infants. Since 1984, Cohen technique was performed in 13 infants and children (24 ureters) and satisfactory results were obtained except one ureter which showed persistant reflux postoperatively in a patient with severe neurogenic bladder. From our experience, the Cohen technique is simpler and safer than Politano-Leadbetter procedure. We recommend, therefore, this technique for children with vesicoureteric reflux, particularly in young infants.
Periproctal abscess and anal fistula are common disease in pediatric surgery. During the last 21 years a total 470 cases have been experienced. 463 (98.9%) were male and 7 were female, showing an outstanding predominance in male. Age at time of onset is within three months after birth in about two-thirds of the cases. The lesions were found at the two lateral directions of the anus in 68.5% of cases, while at the anterior or/and posterior direction in 2%. In female cases, about a half occurred at two lateral directions, and the other half occurred at the anterior posterior directions. The cases with birth weight over 3,000g were 91% of all cases, and the patients with birth weight over 3,500 g were 39%. This disease shows the trend of high incidence in the well developed infant. The results of treatment showed that 47 patients were treated by surgery. Fistulotomy was done in 31 cases, while fistulectomy was done in 16 cases. Thirty-six cases (78%) were cured within 30 days and 4cases had repeated surgery. In 120 cases in which the fistula remains unhealed after a period of three months, some characteristic features were presented. Multiple fistulas were apt to remain unhealed. The later the treatment is begun, the cure is more prolonged. Anorectal manometory with perfused side opening catheter was carried out in 12 obstinate cases with conservative treatment. As a result of'the measurement, the decrease of the anal canal resting pressure, the basal rhythmic contraction number of anal and the increase of the rectal resting pressure were recorded. These data may be arised from the damage of internal and external sphincteric muscle. Therefore, in these cases, it may be best to do the surgical procedure.
Long-term follow-up studies are necessary to evaluate the results of corrective surgery for biliary atresia and understand the residual problems in long-term survivors. This is the first report presenting the results of follow-up studies in the adolescents and adults who have survived for more than ten years after successful surgery for biliary atresia. Twenty-six cases, 12 males and 14 females, ranging in age from 10 to 29 years were examined. Twenty-one cases had hepatic portojejunostomy and 5 had hepatico-jejunostomy. All cases except one who died suddenly of the rupture of esophageal varices at the age of 16 years are leading normal lives. The height and weight of all the cases except two were comparable to those of normal Japanese. Hepatomegaly still remained in 5 of 22 cases (23%) and splenomegaly in 8 of 22 cases (34%). Of these 8 cases, 3 have undergone splenectomy during their postoperative courses. Although esophagoscopy demonstrated the esophageal varices in 9 of the 19 cases examined (47%), episodes of variceal bleeding were noted in only 4 cases. Liver function tests disclosed a moderate degree of abnormality in half of the cases. The majority of abnormal findings mentioned above was related with complications such as severe cholangitis during their early postoperative courses.
Since monitoring arterial blood gasses (PaO2, PaCO2, pH) and blood pressure is important in the management of severly ill newborns, the arterial lines must be kept open for days and weeks by continuous infusion with either MEDI-QUIC and Intraflo or a syringe pump. However, these currently available systems have some disadvantages when they are used in newborns.The volume of infusion may not be countable and large enough to carry the risk of overhydration when MEDI-QUIC is used. Although the flow is constant and countable when a syringe pump is employed, the system does not respond to quick change in the pressure without a moment of delay because of relatively high compliance of the system. A new system which does not have these disadvantage was reported. It was constructed in a way that an Intraflo was connected to a syringe pump. An Intraflo was used for increase in pressure in the syringe so that the compliance in the system decreased. The syringe pump could control the flow constant and minimum.
Primary excision of a choledochal cyst has recently become the operation of choice. A choledochal cyst is usually dissected in its wall. This commonly used approach could carry the risk of leaving the intrapancreatic portion of a choledochal cyst (IPCC) and of a considerable amount of hemorrhage. Complete excision of the IPCC is strongly recommended in order to avoid the stasis of pancreatic juice, the formation of caliculi, and the development of carcinoma of the IPCC. The operation technique for complete excision of the IPCC for choledochal cyst patients was described in detail; Bile duct arterial branch of the posterior superior pancreaticoduodenal artery was ligated and divided at first. The IPCC was dissected from the pancreas on the outer layer of the epicholedochal plexus in the cystic wall. The IPCC including a narrow segment beneath the cyst was resected. The cyst was lifted upward to expose the portal vein in the hepatoduodenal ligament and dissected up to the hepatic hilum. Between 1982 and 1985 January complete excision of the IPCC was performed in 11 patients with satisfactory results; 4 males and 7 females, whose age range from 4 month to 13 year old. No injury of the vascular, pancreatic and biliary ductal structures was encountered. Average blood loss was 133 g.
A 2-year-old boy with rhabdomyosarcoma of the prostate, extended to the urinary bladder, was represented. The patient presented with dysuria. He received combination chemotherapy of vincristine, actinomycin-D, cyclophosphamide and adriamycin, and Linac irradiation following by total retropubic prostatectomy with segmental resection of the bladder. There was no residual sarcomatous cell in the surgical specimen. However, he returned to hospital suffering from vomiting and lower abdominal mass 4 months after surgery. The relapsing decreased 95% in size by PVP therapy involving cis-platinum, vinblastine and pepleomycin. He is alive with small calcificated shadow on CT 4 months after the chemotherapy. Progresses on the treatment for this type of sarcoma were also discussed.
Congenital epulis is a rare benign tumor of the newborn. A newborn baby girl was noted to have a mass, measuring 15×10×8 mm, arose from the right mandibular alveolus close to the midline. The presence of the tumor interfered directly with feeding and she was fed by spoon. The tumor was excised at the 15th day after birth. After excision of the tumor, she was able to feed satisfactorily from the breast. The tumor was diagnosed histologically as benign granular cell tumor.
Many cases of familial occurrence of congenital malformations have been reported in previous literatures. However, the etiology of congenital malformations has been controversial and unknown. The relationship between occurrences of different congenital malformations in siblings and heredity have not been clarified, too. Two cases with different congenital malformation in siblings, ruptured omphalocele in an elder sister and anal atresia in a younger brother, were presented in this paper. Four such cases of different congenital malformations in siblings have been reported previously in Japan.
A male neonate 2 days after birth was admitted to this hospital presenting with frequent vomiting, which improved within a few days. Passage of G. I. tract seemed to be good by the initial gastrografin meal examination. Vomiting, however, continued intermittently for about two months, and the repeated upper gastrointestinal series revealed markedly delayed emptying of the gastric contents, associated with bird's beak sign, and laparotomy was done. A clockwise volvulus of the midgut through 360° was reduced. The splenic flexure and descending colon were normally located, but the cecum into which the terminal ileum emptied from the cranial was situated in the right flank with the colon passing transversely and thereafter running to the cranial. No hepatic flexure was present, and the greater omentum was scanty without attachment to the colon. The duodenum and jejunum were buried in the abnormal adhesion between those and the mesentery of the right colon, passing towards the left across the midline, then turning to the right, and consequently reappearing cut of the abnormal adhesion. The small intestine occupied the right side of the abdomen. The superior mesenteric artery was situated behined the duodenum. This condition seemed to be the prearterial right-sided cecum, according to the classification of reversed rotation by Amir-Jahed. Bill's procedure in addition to Ladd's operation was performed, and postoperative recovery was uneventful. The reversed rotation is extremely rare, and up to date about 50 cases have been found in the oversea literature, and only about 20 cases have been reported in Japan.
Congenital superior mesenteric arteriovenous malformation is rare lesion. Our review of the literature revealed only two cases, a 31-year-old male and a 40-year-old male. A male infant, weighing 2,480 gr, was born at 37 weeks and 6 days by vaginal delivery after uncomplicated pregnancy. He developed the disseminated intravascular coagulation syndrome soon after birth and treated with exchange transfusion. He was transferred to our hospital for abdominal tumor at fourth day of his life. A diagnosis of congenital superior mesenteric arteriovenous malformation was made by abdominal CT scan and aortography. This is the first reported case of this disease seen in newborn infant.
A 8-year-old girl with the complaints of fever, upper abdominal pain, nausea and diarrhea was admitted at Niigata University Hospital on June 24, 1984. Diagnosis of congenital choledochal cyst was confirmed by ultrasonography and computed tomography. Endoscopic retrograde cholangiopancreatography through the major papilla vateri under general anesthesia, revealed cystic dilatation of the choledochus without showing pancreatic duct. Endoscopic retrograde pancreatography through the orfice of the minor papilla disclosed the Santorini duct and dorsal pancreatic duct without communication to the dilated choledochus. This evaluation led to the diagnosis of congenital choledochal cyst with pancreatic malfusion. Primary excision of the choledochal cyst and Roux-en-Y hepaticojejunostomy were performed on June 29, 1984. Postoperative course was uneventful and she was discharged on the 15th postopsrative day. In this case, congenital stenosis of the distal choledochus, malformation of the choledocopancreatic duct system and inflammation are etiologically suggested to be the cause of the congenital choledochal cyst.
Dysgerminoma is divided into two types, i.e., pure type and mixed type. Mixed type is associated with other various elements of germ cell tumors. We experienced a case of mixed type with high serum concentration of both human chorionic ganadotropin (=HCG) and α-Fetoprotein (=AFP). The patient is a 12-year-old girl who was admitted in Osaka University hospital because of abdominal distension and elevated serum HCG and a-Fetoprotein. Tumor was originated from right ovary. Histopathological diagnosis was Dysgerminama. The prognosis and the therapy of mixed type Dysgerminoma was discussed.
A daughter and her mother with congenital biliary ductal dilatation (CBD) were presented. The daughter underwent resection of large choledochal cyst and hepatic portojejunostomy when she was 11 year old. Her mother, 48 year old, had biliary ductal dilatation with cancer. She had pancreato-duodenectomy with resection of the choledochal cyst. She had anomalous arrangement of pancreatico-biliary ductal system. Three familial occurrence of CBD has been reported in Japanese literature including our case. The combinations were father-daughter, two sisters, and mother-daughter. All except one that was not examined had anomalous arrangement of pancreatico-biliary ductal system. We discussed the possibility of heredity in CBD as follows. 1) x-linked dominant inheritance, 2) autosomal dominant inheritance with low penetrance in male.
An 1-year-4-month-old girl presenting with anemia and drowsiness was admitted to theCenter. A fist-sized tumor was palpated at the lower abdomen. An emergency laparotomy revealed hemoperitoneum caused by intraabdominal rupture of the tumor of the abdominal wall. The tumor was resected en-mass, and is suspected to have urachal origin. Urachal sarcoma reported in the English and Japanese literature were 17 cases. It differs extraordinarilly from urachal carcinoma or any other neoplasms. The main symptoms are abdominal mass and abdominal pain. Two out of 17 cases reported survived more than two years after operation. Four basic criteria to satisfy the identification of urachal sarcoma, regarding it's localization, extention and histology were proposed and discussed.
Japanese Society of Pediatric Surgeons started the Registry in 1971 and continued to have nation-wide registration on pediatric surgical tumors. Seven District Registration Centers worked effectively for the purpose. Follow-up studies on the cases in the first ten years of registration were analysed in this report. Two-year tumor-free survival rate was used for this study. Originally, the numbers of five main tumors, registered from 1971 through 1980 were 2,436 including 1,036 neuroblastomas, 495 nephroblastemas, 294 liver tumors, 405 teratoma group tumors and 206 rhabdomyosarcomas. However, the cases followed-up in this study were rather smaller in number. Tumor-freer 2-year survival rates were as follows; 33.6% (196/584) in neuroblastoma, 60.2% (163/271) in nephroblastoma, 41.8% (46/110) iu hepatic malignant tumors, 59. 3% (67/113) and 35. 8% (35/99) rhabdomyosarcoma. In those 5 tumors, 2-year tumor-free survival rates were studied in relation to the year of treatment, sex, age, histological types and related histopathological factors, original site of occurrence, stage of disease, and interrelationship of each item. The Pediatric Tumor Registry has been supported by the Children's Cancer Association of Japan.