Cardiac surgery in the very young continues to be a challenge. Operative mortality is consistently higher than in the older child. Improved diagnostic, anesthetic and operative techniques as well as postoperative care have progressively lowered the age of highest risk (1-8). Consequently, more infants are referred earlier for palliation or corrective surgery. However, there remains an age limit below which operative risk is increased. To illustrate the problem of age related risk I reviewed all infants less than age 31 days who underwent cardiac surgery during past 3 years at Hospital for Sick Children, Toronto. The first month of life was selected arbitrarilly, the exact age of higher risk being rather vague. Three hundred and fifty four infants under 1 month of age underwent a total of 366 cardiac operations during this 3 year period of 1980, 81 and 82, inclusive. Operative mortality is 23.8% in this group, significantly higher than the 3.7% mortality for the 1,568 patients over the age of 1 month (P=.0001). While the infants under 1 month constitute only 19% of all cardiovascular operations in our institution, they contribute 61% of the total mortalily. Clearly, if we are to improve future results in congenital heart surgery the newborn group must be the focus of our efforts. Why is surgery in the newborn period risky? Does age alone contriburte to increased mortality? Is the severity of the cardiac defect significantly worse? Is there a metabolic immaturity in the infant which precludes a successful outcome for surgical interventation? Are there more associated non-cardiac diseases contributing to the newborn's illness or are there other still un-recognized factors affecting the newborn? All of these factors may be involved in any one newborn and progress will depend on controlling their various negative influences. The range of diagnosis in this group of newborns in very large (Table 1). The only common factor is that all of these infants were acutely ill requiring urgent or emergency surgical treat-ment. Many lessons can be learned from examining each of these groups in detail. Time and space does not permit elaboration about each group and I will limit my comments to the more frequently encountered lesions. The first 4 diagnoses (Table 1) encompass 76% of the infants. Notice too that the commonly encountered atrial and ventricular septal defects (VSD) seen in older infants and children in-frequently cause surgical intervention in the newborn.

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Vitamin status (B, B, Folic acid, Biotin, A, D and E) in pediatric surgical patients under-going intravenous hyperalimentation (IVH) over 4 weeks was studied and following results were obtained. 1. 20 patients aged between 8 months and 3 years with no evidence of liver damage nor abnormal loss of intestinal fluid were divided into two groups; one with vitamin supplementation during IVH and the other without vitamin supplementation. The blood vitamin status in patients without vitamin supplementation decreased gradually by the 4th week of IVH, while the other group with vitamin remained in normal range. However, the blood levels of vitamin A and E in this group showed gradual increase during IVH indicating that the dose of vitamin A and E should be reduced when IVH is prolonged. 2. To ascertain the vitamin status in patients with impaired liver function and excessive loss of intestinal fluid, blood levels of vitamins in four patients with congenital biliary atresia and one with high jejunostomy were measured during IVH. In patients with biliary atresia, the blood levels of fat soluble vitamins were low, although vitamins in standard dose had been given during IVH. A 3-month-old male with high jejunostomy developed vitamin D deficiency. This baby had been on IVH since his 2nd day of life. Bottle feeding was simultaneously carried out with IVH. At the age of 3 months, blood level of vitamin D was less than 5 ng/ml and bone x-ray of the extremities showed characteristic findings of vitamin D deficiency which was cured by increased vitamin D supplementation for 2 months. Our results indicate that it is important not only to include vitamins in the IVH solution, but to administer them in appropriate amount to prevent deficiency and intoxication.

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Urinary excretion of 3MH, NPN, and creatinine was measured in 48 pediatric surgical patients for the first 8 days after the operation. The normal values of 3MH excreted in the urine were; 0.409±0.051mg/kg/day in 2 days old newborns (n=10, M±SD), 0.483±0.065mg/kg/day in 7 days to 1 month old infants (n=30), 0.472±0.059mg/kg/day in 1 month to 6 months old infants (n=15), and 0.463±0.049mg/kg/day in 6 months to 1 year old infants (n=12). Urinary excretion of 3MH was significantly low in 2 days old newborns, when compared with those in the other age groups. Marked increase of urinary excretion of 3MH an NPN was observed shortly after the operation and the amount of 3MH and NPN excreted in the urine gradually decreased thereafter. After the fifth postoperative day, no significance was found between pre- and postoperative urinary excretion of 3MH and NPN. Urinary excretion of 3MH and NPN seems to be dependent upon the extent of surgical invation. There was a close correlation between the duration of operation and urinary excretion of both 3MH and NPN on the first two postoperative days. Urinary excretion of 3MH and NPN increased in whom intake of calories was below 50 Cal/kg/day or the nitrogen balance was negative. Increase of urinary excretion of 3MH and NPN after the operation was more significant in neonates within 6 days after birth than in infants of the other age groups. It was suggested that the urinary excretion of 3MH is dependent upon the degree of muscle damage. There was a close correlation between 3MH/Cr and 3MH excretion. The results of the present studies disclosed that the urinary excretion of 3MH and NPN is a useful indicator for evaluation of the postoperative protein metabolism.

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Postoperative anal continence after surgical correction of anorectal anomalies was evaluated with clinical and objective assessments. Clinical assessments was done with a modification of the system developed by Kelly, and objective assessment was based on both manometric and radiologic studies. In radiologic studies, attention was paid to the presence of leakage of the contrast medium and of anterior angulation of the rectum. Manometric studies were performed by an open-tip method with a continuous infusion system. The probe was withdrawn at a constant speed and an anorectal pressure profile was recorded. The manometric data were analyzed with regard to three factors: 1. amplitude of ano-rectal pressure difference : 2. length of high pressure zone: and 3. presence of anorectal reflex. Patients with translevator anomalies treated with perineal anoplasty were excellent both in clinical and objective scores. But discrepacies between these scores were observed in patients with supralevator anomalies. It was shown that they had high clinical scores in spite of low scores by objective means, and a serial analysis of these cases with clinical assessment showed that fecal continence improved in a time course of follow up and was established finally after the patients went through puberty. In the case of supralevator anomalies, patients treated with sacroperineal pull-through showed better results with respect to objective assessment than those treated with abdomino-perineal or abdomino-sacroperineal pull-through. It was concluded that objective assessment, consisting of manometric and radiologic studies, would be more valuable than clinical assessment in evaluating postoperative anorectal functions and operative procedures.

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Distribution of gut hormones (vasoactive intesinal polypeptide, substance P, motilin and somatostatin) in the bowels was immunohistochemically studied in patients of Hirschsprung's disease, normal human control and Congenital Aganglionosis Rats. In the colon of normal subject and the ganglionic bowel segment of Hirschsprung's disease patients, VIP and substance P were detected in the cytoplasma of the ganglion cells and their sur-roundings. The nerve fibers in the muscle layer and submucosa of the bowel were immunohistochemically stained. The endocrine cells in the mucosal epithelium were also strongly stained. Motilin and somatostatin were detected in the cytoplasma of ganglion cells and endocrine cells. In the aganglionic segment of Hirschsprung's disease patients', these gut hormones have been diminished or decreased. Distribution of VIP was greater at the transitional segment that at the normoganglionic segment. The endocrine cells in the aganglionic segment were same in number as in the normoganglionic segment. These cells were immunohistochemically stained equally. Electron microscopically, synaptic large cored vesicles (200-300nm) was decreased on the aganglionic segment. This findings suggest reduction of peptidergic nerve ending. The results lead a conclusion that the aganglionic segment in Hirschsprung's disease does not have peptidergic innervation (nonadrenergic inhibitory neuron) in the bowel. Genesis of the gut endocrine cell is different from that of the enteric nervous system.

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Endoscopic findings in 5 cases of hiatus hernia, who were treated during the period from 1980 through 1982, were reported and pathophysiology of hiatus hernia in infancy and childhood was discussed. In the typical case, esophago-cardiac junction and esophageal hiatus of diaphragma are divided apart each other. Corresponding to this anatomical change, two narrowings were observed endoscopically. In the case in which two narrowings were tightly closed, biphased lower esophageal sphincter pressure was obtained in pressure study. On the contrary, in the case the narrowings were loosely closed, LESP were flattened and widened. Inflammatory changes were well documented only by endoscopy. Histological changes of esophagitis, which include the infiltration of neutophils, have well corresponded to the endoscopic findings, especially to Lugor stain. Postoperative work up reviealed that the fold made by fudoplication had an important role in antireflux mechanism.

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A case with hiatus hernia associated with esophageal stricture and secondary short esophagus, was reported. The patient was twelve years old male and in highly disturbed nutritional condition, because of persistent vomiting started shortly after birth. The diagnosis of hiatus hernia with esophageal stricture was made by X-ray study, manometry, pH measurement and fiberscopy. The operation was done after his general condition was improved by the adequate nutritional support. There was a firm and long stricture at the lower esophagus which was densely adhered to the surrounding tissues. Even if the esophagus was dissected up to the aortic arch, the proper length of intra-abdominal esophagus could not be made. So it was considered that this patient had also secondary short esophagus caused by severe reflux esophagitis. The anti-reflux operation was performed by Boerema-Filler procedure, and the re-established cardia could be repositioned below the diaphragm by forceful traction of the esophagus downwards combined with pushing the hiatal region of diaphragm upwards, inspite of the short esophagus. After the operation, the esophageal stricture was managed by conservative dilatation. The patient is doing well 3 years after the operation.

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Congenital duodenal obstruction is known to occur most frequently at the second portion of duodenum where biliary tract and pancreatic duct terminate, and also known that membranous obstruction is common. In our series of eleven cases of congenital duodenal membranous stenosis, orifice of the ampulla opened just on the membrane in 4 cases and immediately above or below the membrane in 2 cases. In surgical correction of this disorder, we consider, exicsion of the membrane combined with duodenoplasty is coming to an usual procedure. Then, the recognition of the intimate reration-ship between the membrane and the ampulla may prevent inadvertent injuries to the ampulla. In one case, we were obliged to perform papilloplasty because of the injury of the ampulla at the resection of the membrane. Intravenous secretin injection, which induces an excretion of pancreatic juice from the ampulla, should be recommended as an alternative and effective trial for the recognition of the ampulla.

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The Mullerian Duct Cyst (MDC) is formed by the enlarged proststic utricle which is the remnant of the Mullerian duct in male presenting urinary retention, dysuria, urinary infection, hematuria, VUR and erectil impotency. A 5 month old male infant was admitted to our clinic with an abdominal mass and urinary infection. He presented with Jarcho-Levin syndrome (Spondylothoracic Dysplasia) which was recognized shortly after birth. The intravenous pyelogram and voiding cystourethrogram revealed a large cyst between the bladder and the rectum communicating to the vermontenum in the urethra. By these findings, the diagnosis of MDC was achieved. The cyst measuring 5×4×4cm was successfully removed by transabdominal approach. The postoperative outcome was satisfactory. Pathological examination of the excised specimen revealed the stratified squamous epithelium lining the inner surface and the developed musle layer resembling to the vagina.

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A 10-year-old girl presented with fatigue, lameness, lower leg pain, constipation, polyuria, headache, vomiting, and abdominal pain. Diagnosis of primary hyperparathyroidism due to solitary tumor was made by scintigraphy with Tl-Tc subtraction method. The parathyroid gland containing tumor was extirpated. Histological diagnosis was chief cell adenoma. Postoperative transient hypocalcemia was treated by administration of calcium gulconate, and maintenance dose of calcium lactate, 1-D_3 and Vit. D. were continued for 6 months after the operation. Subperiosteal resorption was improved completely by the 6 th postoperative month and cortical defect of general bones was reconstructed gradually thereafter. She has been growing normally without any symptom. The authors collected 16 pediatric patients with primary hyperparathyroidism from Japanese literatures. The age ranged from newborn to 14 years. Changes of bones observed in 13 patients and nephrolithiasis was observed in 2 out of these 13 patients. The remaining 2 patients showed chemical type of the disease and one patient died of hypercalcemic crisis. Ulceration of the alimentary tract or pancreatitis was not observed among these 16 patients. Pathology was single adenoma in 9, hyperplasia of the gland in 3, and not described in 4 patients.

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We have experienced a 6 year-old boy with recurrent rupture of esophageal varices due to splenic and left portal vein obstruction as well as right portal vein stenosis. We successfully treated this patient with esophageal transaction with using SPTU Auto-stapler, splenectrmy and parietal cell vagotoiny. It is easy and time saving procedure. Presently this patient is doing very well at 10 months post-operative period. Since any type of shunt operation is thought to be contraindication before the age 10, it is worth while to consider these procedures as the first choice. The long term follow up is mandatory to establish them as a routine use.

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A case of 1 month old girl of chylousascites associated with malrotation of intestine was reported. The association of malrotation with chylousascites is very rare. This is second case in Japan as far as known. She was urgently sent to our hospital complaining of dyspnea with abdominal distention and loss of appetite. By the laboratory examinations leucocytosis and hypoproteinemia were presented. A diagnostic abdominal paracentesis obtained milky ascites and many fat globules and chylo-micron were found microscopically. The diagnosis of chylousascites was made. The ingredients of the ascites were protein 5.5g/dl, totallipid 2,180mg/dl, triglyceride 2,420mg/dl, phospholipid 122mg/dl and β-lipoprotein 1,005mg/dl. The Goldon's test showed 2.0% rouling out the proteinloosing entelopathy. Patient was treated by five times paracentesis and the low fat milk (Meiji No.810: protein 13.3g/100g, fat 3.5g/100g) for 100 days. Her course was uneventful and patient left the hospital after 18 weeks. It could be pointed out that in addition to the malrotation, the hypoplasia of the lymphatic system seems to play a part in this case. Low fat milk administration was very effective for this case.

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The authors present here a case of congenital abdominal wall defect associated with vitelline vascular remnant. It suggests a hint to embryogenesis of gastroschisis. A new-born male, weiging 3,000g, was transferred to the authors' hospital because of abdominal wall defect (gastroschisis). On operation, the abdominal wall defect was 28mm in diameter. The umbilical cord attached normally on the left margin of the defect, and an abnormal string was found between eviscerated ileal mesentery and the right margin of the defect. The string was diagnosed as vitelline vascular remnant by its histological findings and its connecting position. The authors concluded that the abdominal wall defect occurred in the umbilical cord itself, because the vitelline artery and vein had to attach the umbilical cord. This suggests that gastroschisis is probably a result of umbilical cord rupture in the uterin.

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A case of teratoma occurling in an intraabdominal testicle in a 4mo. old infant was reported. The baby was admitted with left abdominal mass. He had been noticed of left undescended testicle from birth. Investigation after admission proved left abdominal tumor with calcification.Emergency laparotomy was performed at 4mo. 7d. of age. An intraabdominal 8×7×4.8cm tumor was removed. It was well capsulated, fed with left spermatic vessels and the left sper-matic cord was apparently going into the tumor. Histopathological examination of the removed tumor revealed mature teratoma containing bone, cartilage, fibrous tissue, fat, intestine, lymph-nodes, bronchus and nerve tissue. There was an atrophic tubular tissue of left testis attached to the tumor, suggesting that the tumor have occured from the left testis. This case may be the forth report of mature teratoma in undescended testes in children.

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