Four infants with congenital subglottic stenosis underwent anterior cricoid split (ACS) successfully. The age of the patients ranged from 1 month to 8 months. They pressented with stridor since birth. Bronchoscopic findings were round-shaped stenosis at cricoid ring site without/with granulation tissue due to previous endotracheal intubation. One of the patients had underwent a cartilage graft prior to ACS, which was failed. ACS is a simple technique and can be used as an alternative procedure to tracheotomy for a small infant or even neonate and dose not preclude subsequent more extensive laryngotracheoplasty if subglottic stenosis is not resolved. This paper summarized the operative technique and our experiences of postoperative management of ACS.
Treatment of the urologic anomalies associate with anorectal malformation and urologic complications after surgery for various anorectal malformations are discussed. Thirty six patients with major urologic anomalies were found among 228 patients with imperforate anus. Urologic anomalies included undescended testis in 12 cases, hypospadia in 8, phimosis in 3, renal dysplasia in 6, hydroureter in 6, vesicoureteric reflux (VUR) in 12 and so on. Orchiopexy was done in 8 patients at the age of 8 months to 9 years. Two patients with hypospadias were operated on when they were 4 years old. Nephrectomy was performed in 3 patients for unilateral dysgenesis of the kidney with hydroureter at the age of 2 months to 6 years. Antireflux operation was done in 3 patients with VUR when they were between 3 and 6 years old. VUR disappeared spontaneously after the anoplasty in 5 patients under 2 years old. Postoperative complications were counted in 14 patients. Operative treatment was performed in 8 patients. One re-opening of rectourethral fistula was treated by Soave's opetration. Three urethral injuries were cured by simply suturing of the urethra and indwelling of Foley catheter. Postoperative urinary incontinence in a patient with cloacal type was treated with the construction of ileal conduit. Two urethral strictures were treated with vesicostomy and urethral bougienage. One urethral diverticulum was resected and repaired. Eight patients with neurogenic bladder (NB) were treated with vesicostomy or urethral catheterization. All of these patients with NB recovered from urinary disturbance within 7 years.
To make a guidance for closure of fistula after definitive surgery with external biliary fistula for biliary atresia, 84 cases were analysed retrospectively. The rate of postoperative complications was high in patients who had the fistula closed within one and half a year after surgery. The following results were obtained from this study. 1) Optimal time for closure of the external fistula in from 2 to two and half a year after definitive surgery. 2) The history of postoperative complications, especially chronological changes of bile flow and the history of discharge of foods from external fistula, should be checked carefully. Closure of the fistula should be scheduled at more than 1 year after the episode of the postoperative complication. Lysis of the adhesion should be considered if the patient had a history of the food excretion from the external fistula. 3) In patients with jaundice, surgery for closure of the external fistula should be limited to patients with stable liver function, and the operation should be performed at more than 3 years after definitive surgery.
Thirteen children who had both severe mental and severe physical retardation, underwent Nissen fundoplication for gastroesophageal reflux. Most patients suffered from intractable vomiting, hematemesis and failure to thrive. Diagnostic study revealed large hiatus hernia by barium swallow, severe reflux esophagitis by endoscopy, and high pH score in 24-hour esophageal pH monitoring. Candidates for surgery were suggested by pediatric neurologists from medicosocial viewpoints. After surgery, the symptoms of GER disappeared and nutritive state was improved in all patients except one in whom GER recurred six months after surgery and was repaired again. Relaparotomy for postoperative intestinal obstruction was perfromed on three children. Three children have died at the time of mean duration of 2.5 years after surgery, none from causes related to the surgical procedure. Follow-up study of ranged from one to nine years after surgery was revealed that eight patients stay home with their families in good condition, one patient is admitted in an institution for severely retarded children, and another one lost to follow-up. Our standard technique of antireflux surgery for severely retarded children consists of complete fundoplication, crural repair, posterior gastropexy, pyloroplasty and gastrostomy for feeding purpose. Posterior gastropexy was performed with anchoring of the fundoplicated distal esophagus to the median arcuate ligament because this procedure seems necessary to prevent recurrent hernia. Quality of their individual life improved after surgery.
In the field of pediatric surgery, there are many children whose chief complaint is vomiting. Upper gastrointestinography constitutes an useful exploratory approach to detect the cause of vomiting. In this report, we analyzed clinical data on 87 cases of obvious vomiting and abdominal swelling, which have been diagnosed as gastric volvulus (GV) by upper gastrointestinography. We also examined the axis of GV in experimental monkeys. The results obtained are as folows : (1)Organoaxial volvulus is an indispensable factor for typical GV. (2)Newborns and infants presenting vomiting and abdominal swelling should be examined using abdominal plain X-P and gastrography, to check for GV. (3)Diaphragmatic eventration and esophageal hiatal hernia may be complicated by the GV necessitation sufficient observation and appropriate treatment. (4)Hypertrophic pyrolic stenosis, gastroesophagial reflux and intestinal malrotation may also be complicated by the GV, which should, therefore, be considered in the diagnosis of patients with continual vomiting. (5)Surgical corrections is preferable in secondary GV. In idiopathic GV, however, conservative therapy is selected first, and surgical correction, gastropexy, is preferable when conservative therapy has proved not to be effective.
We started to use extracorporeal membrane oxygenation (ECMO) in 1986. Since then, ten critical cases of congenital diaphragmatic hernia (CDH) have been managed with ECMO. A veno-arterial bypass was employed in all cases. The average age of starting was 34.3 hours old, and the average ECMO time was 96.3 hours. Seven out of 10 infants survived. We believe ECMO is most beneficial in the management of critically ill patients with CDH, particularly to overcome the condition of persistent fetal circulation.
The case is boy aged 6 years and 4 months, who had fever and abdominal pain, and was introduced to our hospital for suspected appendicitis. On admission, a tumor with tenderness was palpated at the right hypogastric region. Because the tumor still persisted after the antibiotic therapy, it was very difficult to make its diagnosis. Thus, laparatomy and biopsy was performed. Pathohistological findings confirmed the diagnose of mesenteric necrotizing lymphadenitis. The presented disorder has been rarely reported, and in review of the literature, this seems to be the first case reported in Japan.
The first case of an inflammatory fibroid polyp (IFP) of the small intestine in a Japanese infant, one-year-old boy, is reported. This is the 10th and the youngest case of an IFP of gastrointestinal tract among 10 patients less than 15 years of age reported in English or Japanese literature. In this case, an exploratory laparotomy following the frequent recurrence of intussusception led to the diagnosis of an IFP of the ileum. IFPs of the gastrointestinal tract are rare. They are mostly found in the stomach, and have occasionally been reported in the small intestine and colon. The majority of IFPs have been found in adult patients more than 45 years of age, and very few in children. IFPs of the small intestine are found mostly, as in our patient, following recurrent intussusception. IFPs are benign and recurrence is rare. The etiology of IFP still remains unknown.
A 7 year-old boy with a history of anemia was hospitalized because of massive gastrointestinal bleeding. Emergency laparotomy was performed under the presumptive diagnosis of Meckel's diverticulum, which was suggested by ^<99m>Tc scintigraphy. Microscopic examination confirmed the pathological diagnosis of ileal duplication with bleeding ulcer due to the ectopic gastric mucosa. The postoperative course was uneventful and the patient was discharged on the 14th postoperative day. Duplication of the alimentary tract is an unusual congenital anomaly that is often not suspected preoperatively. ^<99m>Tc scintigraphy was considered to be helpful in this case. It is important to differentiate alimentary tract duplication from Meckel's diverticulum because ^<99m>Tc scintigraphy could be positive for both of these cases. Alimentary tract duplication could be correctly diagnosed based on clinicopathological findings. The cases of alimentary duplication initially presented with gastrointestinal (G-I) bleeding were reviewed among the Japanese literature. The most frequent symptoms are abdominal pain, intussusception, and small bowel obstruction, but the massive gastrointestinal bleeding is thought to be rare in Japan.
A 6-year-old boy with pulmonary atresia and ventricular septal defect was admitted to our institute with hemoptysis and an enlarging mass in the right upper chest. He previously underwent systemic-to-pulmonary shunts in 2 occasions, between aortic arch and right pulmonary artery at the age of 2 months and between left subclavian artery and left pulmonary artery at the age of 3 years. A diagnosis of false aneurysm of the right sided shunt was made and a semiemergent surgical repair was successfully performed with the aid of normothermic cardiopulmonary bypass. Seventy seven days after this procedure, the patient underwent complete correction of the cardiac defect without any difficulty. False aneurysm is a rare complication of systemicto-pulmonary shunt constructed with polytetrafluoroethylene. Since the aneurysm tends to grow rapidly and rupture, surgical treatment is urgently required on an emergency basis.
We encountered a 4-month-old infant who had arrhythmia during 4 months of total pareteral nutrition (TPN) following ileocolostomy for congenital ileal atresia. Supraventricular and ventricular premature contractions were monitored on ECG. The arrhythmias continued despite administration of antiarrhythmic drugs. In addition, the plasma selenium level was 0.9μg/dl, which was very low for an infant. Arrhythmia due to selenium deficiency was suspected, and selenious acid was added to TPN. Both the electrocardiographic abnormalities and plasma selenium level normalized in 5 days after the administration of selenious acid. The plasma selenium level increased to 6.0μg/dl. In patients receiving TPN, prophylactic selenium supplementation is necessary and plasma selenium levels should be determined regular inervals.
Tow cases of cholecysto and choledocholithiasis in infants are reported. Cholelithiasis in childhood is rare, especially in infants. The first case was a 7-month-old infant who had recieved TPN for 33 days on NPO for 20 days after the operation for ileal atresia. The second case was 5-month-old infant with Hirschsprung disease who had received TPN for 16 days on NPO 20 days due to intestinal stagnation after colostomy. Both cases received cholecystectomy and choledochotomy as the operation for cholelithiasis. They are healthy and dealing normal life now. The causes of cholelithiasis in infants are discussed.
Four cases with congenital atrio-ventricular block were experienced in the past 7 years. They were diagnosed prenatally and required the external pacemaking therapy within 12 hours after birth because of the cardiac failure. Two patients were able to be eventually weaned from the external, one (case 1) required the pacemaker (VDD mode) implantation at 10 months after birth because of critical bradycardia and the other (case 2) was lost to follow-up after the age 3 months. Two other patients, case 3 and 4, were unable to be weaned from external pacing and required implantation of the pacemaker (VVI mode) at 40 days and 74 days of age. Based on these experiences, we concluded that permanent pacemaker implantation during the neonatal period could be avoided because the temporary external pacing was stablely performed even in neonates with low birth weight. Median sternotomy was usefull in the young infants to select the optimal place for myocardial electrode and to implant a generator under rectus abdominis in the epigastric region.
Most fetal ureters have folds and kinks which occur as invaginations of the musculature and mucosa, but they usually disappear with growth. Presense of folds and kinks after birth is called persistent fetal ureter (PFU) and rarely causes a true obstruction. We report here a case of a newborn male infant with bilateral hydronephrosis due to PFU. Routine prenatal ultrasound detected a cystic mass in his abdomen. After birth CT and ultrasound revealed bilateral hydronephrosis. Because of the marked dilatation of the right pelvis, a ultrasonically guided percutaneous nephrostomy was performed at the day of birth. After that serial antegrade pyelogram via the nephrostomy and intravenous urogram revealed bilateral PFU. Passage of the right ureter improved gradually and the nephrostomy was removed. He has been observed for 15 months and the bilateral ureteral folds have disappeared gradually and the bilateral hydronephrosis also has diminished. We discuss the management of this lesion. In infants, ureteral folds disappear with growth and hydronephrosis diminishes, so that careful obsevation is more desirable than surgery. However, in older children disappearance of ureteral folds can not be expected, so that surgical therapy is required.
A newborn male baby was brought to our hospital because of a thin, flaccid abdominal wall. Clinical examination revealed multiple anomalies such as high imperforate anus, malrotation of the intestine, patent urachus, omphalocele, megacystis, urethral stenosis and bilateral undescended testis, so that colostomy, repair of malrotation, excision of the urachus, one stage repair of the abdominal wall and vesicostomy was performed at the day of birth. Further examination of urinary tract after the first operation revealed agenesis of the right kidney and bilateral vesicoureteric reflux (VUR). He is unable to expel urine per urethra at the age of two years and bouginage of the urethra is done for the purpose of intermittent catheterization and antireflux operation for VUR is scheduled to be done in the near future. A review of the literature to date in Japan has brought to light an additional 110 cases of the prune belly syndrome, and discussion was made on these cases. High mortality rate (40.5%) was observed and the causes of death were chiefly pulmonary failure, sepsis and renal failure. Therefore more proper treatment should be given to serve good renal function and to prevent urinary and respiratory tract infection.
A 13-month-old boy is presented of intrathoracic desmoid tumor which was developed after resection of teratoma from right upper mediastinum. The desmoid tumor was totally removed except for the small part where the desmoid tumor had invaded into the chest wall at the apex. No chemotherapy and radiotherapy were done after operation. One month later, the desmoid tumor recurred from the apex of the right thorax into the thoracic cavity. Fortunately the patient has no respiratorysymptom until now. The authers discuss the origin of the desmoid tumor and the strategy against the recurrent desmoid tumor in children.
Segmental dilatation of the colon is a rare entity. Two boys associated with low type rectoanal anomalies who showed the segmental dilatation of the rectosigmoid colon are reported. The dilatation of the colon caused progressive constipation, though no obstructive signs were detected on anal digital examination. The resection of the dilated colon with modified Soave's operation cured constipation. Aganglionosis was ruled out in both cases. The morphological findings, such as an abrupt dilation in proximal caliber change, and the clinical characteristics in our cases resembled the segmental dilatation of the colon that was first reported by Swenson. Though the etiology of this entity is unknown, adequate diagnosis and resection of the dilated intestine are necessary to obtain good prognosis.
Torsion of the vermiform appendix in a 2 1/3-year-old boy, who appears to be the youngest patient with this disease in the world literature, is reported. He had no previous history of abdominal pain. He began to complain of abdominal pain about 30 minites after standing on his hands for the first time in his life. Two days later, he was admitted to our hospital because of severe intermittent abdominal pain. Tenderness of his right lower abdomen associated with a WBC count of 23100/cmm and an enlarged appendix as seen in the ultrasonogram led us to perform a laparotomy under the diagnosis of acute appendicitis. The vermiform appendix showed clockwise torsion of 360°at its root and a gangrenous appearance. There was no adhension of the appendix to the surrounding tissue, and so release of the torsion and an appendectomy were easily performed. No fecalith or inflammation of the appendix was observed. The postoperative course of the patient was uneventful. Although anatomical or pathological causes of torsion of the appendix are still unknown, STANDING ON HIS HANDS was suggested as an important etiological factor in this case.
Cecal diverticulitis is quite rare among children. It is frequently misdiagnosed as acute appendicitis preoperatively, because its clinical presentation is difficult to distinguish from that of acute appendicitis. It is important, therefore, to have cecal diverticulitis in mind as one of the differential diagnoses for acute appendicitis at the time of examination and operation. We report a 13-year-old boy with cecal diverticulitis whose preoperative diagnosis was acute appendicitis. Laparotomy revealed a moderately inflammed cecal diverticulum, which was resected wedgewise in addition to an incidental appendectomy. It was a simple and false diverticulum. In the literature, there are only two reports of cecal diverticulitis in a child, an 11-year-old girl by Suzuki, 1984 in Japan and a 2-year-old boy by McPherson, 1985 in England. We believe that this is the third report of cecal diverticulitis in a child. It is quite difficult to distinguish cecal diverticulitis from acute appendicitis. However, when we suspect cecal diverticulitis from present history and symptoms, we should try to detect a diverticulum using ultrasonography, barium enema and so on to avoid unnecessary laparotomy.
A case of biliary atresia (BA) with meconium peritonitis is reported, and its pathogenesis is discussed. The operative findings of meconium peritonitis suggested that bile juice was excreted until the late perinatal phase. After operation, the serum bilirubin was elevated and a second operation was performed for hyperbilirubinemia. An intraoperative cholangiogram revealed type IlIa BA and the main fibrotic remnant connected to the liver which was slightly shifted to the right from the porta hepatis. As the length of the residual small bowel was only 70cm, a hepaticoportocholecystostomy was performed. The structure of this remnant may indicate that the common hepatic duct had had some dysplasia originally. The complete obstruction of the extrahepatic bile duct was thought to have occurred secondary to the influence of meconium peritonitis.