Electron microscopic studies of the hepatic tissues biopsied at hepatic porto-enterostomy or porto-cholecystostomy were performed in 14 patients of biliary atresia. The intrahepatic bile ducts showed the evidences of degenerative changes in 11 out of 13 patients; abnormal increase or decrease in electron density of the cystoplasm and the nuclei, cytoplasmic accumulation of the electron dense materials, cytoplasmic vacuoles and disrupted cell membrans, cystic dilatation of the smooth surface endplasmic reticulum, and dilatation of the intercellular spaces. In many degenerative bile ducts their lumens were found to be narrowed by edematous projection of the cytoplasm (blebs) or by accumlation of the fragments of the degenerative epitherial cells and rarely by bile thrombi. The ductal lumens were not obstructed in patients who completely recovered from jaundice after hepatic porto-enterostomy or hepatic porto-cholecystostomy.
Fiftythree patients with congenital neurogenic vesical dysfunction due to myelodysplasia who have been seen at the Hokkaido University Hospital from October 1959 to December 1980, were subjected for an analysis of the clinical, urographic change and urodynamic findings. In 12 children who were initially examined at the age less than 6 months, intravenous pyelogram was normal. In 7 of those who had undergone cystourethrography there was no ureteral reflux. The follow-up studies (5 months to 21 years) were available in 37 patients. Excepting 6 cases who had been seen before the advent of current conservative regimen, 31 patients have been managed either by Crede alone (17 pts) or clean intermittent self-catheterization (14 pts). These 31 patients showed no signs of clinical or urographic deterioration, but rather marked improvement. Significant renal deterioration was noted onty in 2 patients who belonged to our old seriese and had undergone hasty urinary diversion long time previously. Of various urodynamic parameters, the electromyographic sphincter dyssynergia to carrelate best with voiding dysfunction, since it was absent in the group of patients who had been managed naturally by Crede voiding while it was present in those who could empty the bladder with the aid of clean intermittent self-catheterization.
Fifteen cases of splenectomized children under age of 15 years during the past 15 years were reviewed. The basic disease for indication of splenectomy were hemolytic anemia, chronic idiopathic thrombocytopenic purpura (chronic ITP), portal hypertension and histiocytosis. Two patients developed postsplenectomy sepsis and 4 and 5 months postoperatively. In these two cases, lymphocyte and neutrophil concentrations in blood tended to decrease before the onset of postsplenectomy sepsis. The death occured within 24 hours after the onset of symptoms. Even though the risk of sepsis might fade by advance of age, continuous prophylaxis with antibiotics should be given for at least 2 years after splenectomy.
Bi-directional ano-rectal manometric studies using a double lumen tube were performed in 13 patients with Hirchsprung's disease who had undergone Z-shaped anastomosis. After Z-shaped anastmosis, the anterior wall of the rectum is aganglionic, but the posterior wall is ganglionic. Therefore, there may be a significant difference anatomically and physiologically between the anterior and the posterior wall. Using a bi-directional double lumen tube, resting pressure, resting anal canal pressure and anal canal contractile wave were measured but there was no statistically significant difference. But 80% of the postoperative cases showed dissociation of resting pressure profile. Recto-anal reflex was found in 6 postoperative cases. Reflex was clear in the posterior wall, but unclear or none in the anterior wall. These facts suggested that the mechanism of the recto-anal reflex after Z-shaped anastomosis for Hirschsprung's disease was due to the peristalsis of the posterior normal colon. Further investigation will be necessary to elucidate the postoperative anorectal movement.
Eightyfive patients with severe pulmonary hypertention (Pp/Ps over 0.75) underwent various open-heart surgeries at the department of Thoracic and Cardiovascular Surgery, Sapporo Medical College and Hospital, during the period from July 1, 1973 through July 31, 1981. The cardiac diseases included were as follows (deaths in parentheses) ; ventricular septal defect (VSD + PH) 59 cases (11); complete transposition of the great arteries (TGA + VSD) 15 cases (10); coarctation of the aorta (CoA complex) 8 cases (5) ; interruption of the aortic arch (IAA + VSD) 3 cases (2). The overall mortality was 32.9%. In VSD with severe PH, the operative mortality was 18.0%. On the other hand, there was no death in 15 cases of VSD with moderate PH (Pp/PsO.6 - 0.75). of 15 cases with TGA + VSD, Mustard's operation was performed in 10 (7), Senning's operation In 3 (1), Jatene's operation in 1 (1), and Kaye-Stansel's operation in 1 (1). Of 8 cases with CoA complex, primary total correction was performed in 2 (2) and staged operation in 6 (2). In cases with IAA+VSD, a primary total correction of Muraoka's operation was successfully performed in a 2 year old boy. With use of combined hypothermia (surface and perfusion cooling) and cardiac arrest, results of primary total correction have significantly improved in the group older than 3 months of age.
Post-operative function after surgical treatment of ano-rectal malformation was evaluated in 46 cases. The following conclusions were made by follow up studies in 32 cases. 1) Answers to our questionnaire were received in all cases. 2) Severe associate anomalies and infection especially pneumonia were the main cause of death in the follow-up period. 3) Immediate postoperative results at discharge were poor in high type cases while almost normal in low type cases. 4) Our previous long-term follow up data were compared with the immediate postoperative results at discharge, revealing improvements in most cases but no improvements were noted in several cases. 5) Present follow up study indicated general tendency for gradual improvement in most cases but unsatisfactory results were still remaining in some cases. 6) Cases with anal mucosal prolapse proved to show other unsatisfactory signs as well. 7) In cases with mental retardation, no ano-rectal functional improvement was found. For these children, an approach for mental disorders should be considered before evaluation of the surgical result.
Thromboembolic complications after angiography and their influence in subsequent limb growth were studied in a consecutive series of 71 infants. They were subjected to photoplethysmography, measurement of ankle systolic pressure, the studies of limb growth and ischemic symptoms. Among 10 patients of thromboembolic complications, 5 died of malignant tumors. The follow-up study was done in the remaining 5 caces. Of these five patients, one showed contracture of the ankle, one successful thrombectomy, two claudication and one defective limb growth respectively. Thromboembolic complications following angiography in infants should be treated at the early phase to prevent subsequent defective limb growth.
For the purpose of investigating the possibility to establish an experimental animal model, the anorectal malformations of swine was studiea The questionaires were sent to 404 swineries in order to know the actual occurrence of imperforate anus in swines. The statistical analysis on their reports revealed that an imperforate anus was indemnified per 566.6 swine deliveries (the incidence was 0.81%), and that those anomalies had predominant tendency in hereditary occurrence. Close similarities were also demonstrated between swine and human being in the types of anorectal malformations. In conclusion, those swine with imperforate anus can be used as an appropriate experimental animal model and they are considered to be very useful material for studying the pathogenesis and for devising the treatment method of human anorectal malformations.
Fistura formation at the penoscrotal junction is the commonest complication of the urethroplasty for proximal hypospadias. We performed urethroplasties on 7 cases using scrotal flap method to prevent fistula at this point. The fistula at the penoscrotal junction was not observed in all cases, but a transient fistula at the distal part and a meatal stenosis were seen in two cases. This methodis surely useful to prevent fistula formation at the penoscrotal junction.
This paper reports 3 cases of the infected urachal cyst communicated with the bladder. The first case was a 5 year-7 month-old girl with a lower abdominal mass and pyuria. The cystogram showed conical projection upward the umbilicus that suggested a communication to the cyst. The second case was an 8 month-old girl with an umbilical fistula. Microscopic examination revealed a small channel to the bladder. The interior of this cyst was lined by transitional cells. The third case was a 2 year-8 month-old boy with complaints of an abdominal mass and pollakiuria. Infection of the cyst made the bladder mucosa hyperemic and caused symptoms of urocystitis without presence of bacteria. Ultrasonography was usefull to demonstrate the size of the cyst and to determine the site of skin incision. The infected urachal cyst should be excised completely including the duct which communicates with the bladder.
Four cases of extremely premature infants with esophageal atresia (Gross C type) were managed by esophageal banding using a silastic tape. Three cases died. In one case who died on the 44th postoperative day no histological changes were found at the banded portion of the esophagus at autopsy. A female infant with birth weight of 1,190g was successfully managed by esophageal banding for a period of 10 months until anastomosis of the esophagus and removal of the banding tape were safely performed. At present she is 2 yrs old. An esophagogram shows no significant narrowing at the site of esophageal banding.
Two high risk cases of esophageal atresia with distal tracheoesophageal fistula (group C of Waterston's classification) were successfully treated by silastic banding of the distal esophagus. This procedure is thought to be safer than the Holder's original extrapleural fistula division and useful in the management of premature or critically ill babies with esophageal atresia. Unfortunately both patients died of sepsis three months after operation. Autopsies showed esophagea stricture and weakness of the esophogeal wall at the banding site. Histological studies showed dissection of the external longitudinal muscle layer and proliferation of the collagen fibers at the banding site. It was suspected that the factors providing pathological changes at the banded esophageal wall are related to tightness and duration and the materials used for banding. It is concluded that a loose banding is safer and satisfactory for prevention of gastric reflux.
Two cases of gasless Gross type C esophageal atresia were presented. Both were diagnosed of esophageal atresia without tracheoesophageal fistula (TEF) since no gas could be found in the gastrointestinal tract. Case 1. A 3 day old male neonate developed excessive salivation and cyanotic spell shortly after birth. Roentgen studies showed coil-up sign and airless abdomen. On the basis of this study, the preoperative diagnosis was esophageal atresia without tracheoesophageal fistula. Gastrostomy was performed at the age of four days. The stomach was relatively small. Two days after operation, feeding via gastrostomy was started. The patient did very well. Any complication such as pneumonia was not noted. At twenty-eighth day of age, a metal prove was inserted into the distal esophagus through gastrostomy for the purpose of measuring the gap between the upper and lower esophagi. The prove was found in the trachea. Immediately the ligation of TEF and esophage-esophagostomy was performed by extrapleural approach. The location of TEF was slightly cephalad of bifurcation. Stenosis or plugging in the TEF was not found. Postoperative course was uneventful. Case 2. A female neonate was born after pregnancy complicated by hydramnios. Routine nasogastric tubing had found esophageal atresia. Roentgen studies five hours after birth revealed airless abdomen. On the next day, gastrostomy was performed. The stomach was relatively small. When gastrostomy feeding was initiated, she became cyanotic and vomited saliva with coughing. On the eighth day of birth, TEF ligation was performed. Postoperative course was complicated with respiratory distress. She died of pneumonia with mediastinitis on the twelveth postoperative day. In our survey of Japanese cases, the incidence of such type TEF cases was only 5.6% (6 of 108 Cases). For the accurate diagnosis of this condition, the lower esophageal pouch should be examined by air or contrast media through gastrostomy before gastrostomy feeding is initiated.
Congenital biliary atresia associated with preduodenal portal vein in a 81-day-old girl is reported. Preduodenal location of the portal vein is a rare congenital anomaly which is frequently associated with various visceral anomalies, but its association with biliary atresia is relatively rare.Twenty-five cases of this combination were found in eighty-four preduodenal portal veins reported in the world literature including our case. In the case of preduodenal portal vein, anatomical relationship between the portal vein and the extrahepatic biliary tract in the porta hepatis might be changed; the biliary tract frequently locates posteriorly to the portal vein and enters the liver in the posterior position of the porta hepatis. A careful investigation and dissection of the proper porta hepatis should be necessary being kept in mind of this possibility in congenital biliary atresia associated with preduodenal portal
A case of mediastinal giant hemangioma was reported. The patient was a 13 years old female. She was asymptomatic, but an abnormal cardiac silhouette was pointed out in a chest X-Ray film and the patient was transferred to our hospital. At operation, a polycystic giant anterior mediastinal mass was resected, that was diagnosed as hemangioma. The tumor was 16x11x7 cm in size and weighted 694 gram. Mediastinal hemangioma is very rare. Most patients are asymptomatic. It is often found by a radiological examination. All the cases reported in Japan are histologically benign, but some malignant cases have been reported in foreign countries. So, whenever the patient is suspectecd to have a mediastiaal tumor or mediastinal hemangioma, the tumor must be resected as soon as possible.
A two year old girl with an intestinal obstruction due to mucocutaneous lymphnode syndromd (MCLS) was admitted to our hospital with vomiting, abdominal distension and MCLS. MCLS was diagnosed by typical skin eruptions, lymphnode swelling and fever. Intestinal obstruction was diagnosed by barium enema as a mechanical obstruction which would require surgery. The operative finding showed the intestinal obstruction was caused by a kinked adhesion between the inflamed lymphnode and jejunum. Intestinal decompression and adhesiolysis were done. In post-operative course, obstruction recurred 8 days after the. surgery. In spite of medical treatment, the intestinal obstruction was not released. Twelve days after the previous operation, a second laparotomy was done. Severe adhesion was found in the abdominal organ. After adhesiolysis of the entire intestine, an internal intestinal splint catheter was inserted through gastrostomy into the intestine and the colon. Although posjt-operative intestinal paralysis was severe, the patients course was uneventfull. Only 5 cases of intestinal obstruction due to MCLS has been reported in literature. All these cases had weak intestinal peristalsis. This paralytic intestinal obstruction is a typical sign of MCLS ileus.