The association between neuroblastoma and the subunit B of creatine kinase (CK-B) was investigated to assess the possibility of the role of CK-B as a tumor marker for neuroblastoma. With the use of a sensitive solid-phase sandwich enzyme immumoassay system, the cocentration of serum CK-B was measured in 47 patients with inguinal hernia under 10 years old for normal controls, and in 29 patients with neuroblastoma. The CK-B concentration was elevated on 66% of the neuroblastoma patients. The relationship between the prognosis of neuroblastoma patients and the serum CK-B level was found statistically significant. These data suggest that CK-B is an useful serum marker for prediction of prognosis, diagnosis, and monitoring the effectiveness of therapy.
This study was conducted to evaluate the motility of colon and anorectum in Hirschsprung's disease. Forty-two patients with Hirschsprung's disease and 42 control infants were supplied to this study. By means of VTR-endoscopy which was consisted of a fiberscope; a color camera; a video tape recorder, simple observation of anorectum, the observation of reflex movement to the pressure stimulation to the rectum, and the observation of the peristalsis at the transitional zone were performed. The reflex movements were also analyzed using a computed image analyzer and expressed numerically. In Hirschsprung's disease, a cave like appearance of the anorectum and the lack of the reflex relaxation were of diagnostic value, and the peristaltic waves faded out at the transitional zone just like waves surging on a beach. After definitive operation mainly by Ikeda Z-plasty, the reflex relaxation of anal canal which appeared to be concentric in most of the cases were observed in 27 patients out of 38 (71.7%). These results suggest that the aganglionic segment is not spastic and the reflex ability of internal sphincter muscle can be obtained after the definitive operation.
Acute urinary retention in newborns and infants is uncommon. We presented 12 cases of urinary retention with palpably distended bladder whom we treated during the last 14 years. The etiologic disorders included 7 posterior urethral valves and 2 bladder diverticula. Another three cases consisted of an ectopic ureterocele, a hydrometrocolpos and functional vesical dysfunction in a newborn associated with intestinal hypoperistalsis. In about half of the cases, urinary tract infection seemed to be the predisposing factor to acute retention. In general, patients with the worse degree of obstruction present the symptom at the earlier times, occasionally even in the newborn period. Two cases have been diagnosed by the prenatal ultrasonography. The reliable methods to demonstrate underlying causing disorders are micturition cystourethrography and ultrasonography. In patients with bladder diverticula, the extent of the diverticulum during voiding together with the existence of reflux must be monitored under fluoroscopic control. Although temporary vesical drainage through a small urethral catheter is an effective treatment, a diversion of the upper urinary tract is mandatory in cases with prolonged urinary tract infection or poor recovery of the renal function. Follow-up studies of patients revealed impaired renal function in 6 (5 with posterior urethra lvalve and one with ureterocele). Urinary incontinence continued in patients with posterior urethral valve and urogenital sinus anomaly, and it has become the main problem after they reached the school age.
Rectal mucosal prolapse is a frequent complication observed following operation for imperforate anus, especially in those with high type anorectal malformation. These patients suffer from soiling of mucus, bleeding, and pain. Regarding the treatment of this complication, simple resection of redundant mucosa usually gives unsatisfactory results and leads to recurrences of the prolapse. Recently, we have performed four-leaf-clover shaped circumferential sliding skin graft (SSG) in addion to the resection of redundant mucosa for this complication. In this paper, incidence of postoperative rectal mucosal prolapse and results of surgical treatment for prolapse were discussed. Incidence of prolapse following operation for imperforate anus was 18% (33/188) in all case, 58% (25/45) in high type, 18% (4/22) in intermediate type, 3% (4/123) in low type malformation, respectively. Particularly, high incidence of prolapse was observed in patients who underwent abdominoperineal pull-through operation (59% : 24/41 in high type, and 31% : 4/13 in intermediate type, respectively). All over recurrence rate following surgical treatment of prolapse was 42% (14/33). Each operative procedure had the following recurrecce rate, 14% (2/14) in circumferential SSG, 60% (3/5) in partial SSG, 62% (8/13) in simple mucosal resecton, and 100% (1/1) in Gant-Miwa's procedure, respectively. The longer the period between the primary anoplasty and the operation for prolapse, the lower was the recurrence rate of the prolapse.
Incidence of the postoperative complications in patients with low imperforate anus, and surgical managements following primary procedure of anoplasty are discussed. In 123 patients with low imperforate anus underwent primary anoplasty, 18 patients were performed secondary anal plastic surgery because of postoperative complications-anal stenosis, prolapse of rectal mucosa, recurrent fistula, and anterior anal site. Among the 18 male patients without fistula orifice who underwent perineal anoplasty (14;covered anus complete, and 4;anocutaneous fistula), second operation was necessary in the 4 patients with anal stenosis or remnant of fistulous tract. Cut back procedure was done in 49 patients (36;anocutaneous fistula, 6;anovestibular fistula, 6;anovulvar fistula, and one;anterior anus). In 12 female (6;anovestibular, and 6;anovulvar fistula), 3 patients required second operation for the anterior site of anus or perineal fistula. Z-type perineoplasty as a rectovaginal separation was effective for the female patient with anterior anal site following the cut back procedure. Potts' procedure was done in 50 patients (32;anovestibular, 10;anovulvar, and 8;anocutaneous fistula). In 5 patients with recurrent anovestibular fistula, 3 patients with rectal mucosal prolapse, and 3 patients with anal stenosis, second operations of various type were required. For the treatment of recurrent anovestibular fistula, recently, fistulectomy without colostomy became feasible by the aid of intravenous hyperalimentation and colon preparaion.
The purpose of this paper is to study by means of intraoperative manometrics how to create the lower esophageal high pressure zone (HPZ). Sixteen patients with severe gastroesophageal reflux (two months to 15 years old) were selected and their esophagogastric junction were reconstructed by Boerema-Filler procedure. The lower esophageal HPZ were measured seven times before and during the operation, including after mobilizing the lower esophagus, after tying the right crus of the diaphragm, after fixing the lower esophagus to the anterior edge of the esophageal hiatus, after creation of His angle by Filler procedure, and so on. The following results were obtained: The tone of HPZ was mainly created by the mobilization of the lower esophagus and tying the right crus, and the lenght of HPZ was solely created by the mobilization and fixation of the lower esophagus.
A case of Wiedemann-Beckwith syndrome (W-B syndrome) was reported. A female baby was born after 34 weeks of gestation, with a birth weight of 4254g. Prenatal echogram highly suspected W-B syndrome because of disproportionally large fetus and omphalocele. At birth, omphalocele and macroglossia were noted. Omphalocele was treated just immediately after birth. The baby showed severe hypoglycemia (10-20mg/dl) accompanying hyperinsulinemia (200μU/ml) , which was treatedby parenteral nutrition and she also showed persistent high levels of Alpha-fetoproteim (AFP), over 30×10^4nglml. Histological findings revealed nesidioblastosis and neonatal hepatitis. On the other hand, she developed hypertrophic obstructive cardiomyopathy (HOCM) approximately at age of 4 mouths which was treated with Ca antagonist successfully. We speculated that the HOCM corresponded to the prolonged hyperinsulinemia in the neonatal period. W-B syndrome is characterized by omphalocele, macroglossia and gigantism and it shows various clinical features and may present several critical problems, such as severe neonatal hypoglycemia. Therefore, perinatal intensive care is indispensable and longterm management is also necessary for this syndrome.
A 4-month-old female with a bronchogenic cyst which communicates with the membranous part of the trachea was presented. She had an acute onset of progressive dyspnea and was transferred to our hospital after intubation of a trachreal tube. A cystic legion in the mediastinum was recognized by the CT-scan and the contrast esophagogram. The content of the cyst was strongly suspected as air by CT-scan. Exploring by the right thoracotomy, we resected almost all the part of the cyst adheded to the membranous part of the trachea. The small defect of the trachea formed at surgery was sutured by 5-0 Ticron and was covered with the pleura and the wall of the esophagus. Three months later, she needed intratracheal intubation and respiratory control for a week because of the stricture of the membranous part of the trachea where the cyst adheded. Except this episode she had an uneventful postoperative course.
A 4-year-old boy with pancreatoblastoma was reported. He was admitted to our hospital with complaints of abdominal pain and abdominal distension. On admission hard mass was palpated below the left costal margin. Labolatory examinations revealed elevated serumAFP (1437.9ng/ml) .X-CT and US showed abnormal mass occupying left upper portion of the abdomen. This case was diagnosed preoperatively as malignant teratoma arising from the retroperitoneal cavity, and operation was performed. The tumor was successfully extirpated with the tail of the pancreas to which it closely adhered. The pathological specimen showed squamoid corpuscle surrounded by acinar like arrangement, so called organoid structure. According to the histological findings, the final diagnosis of pancreatoblastoma was made. Sixteen cases of pancreatoblastoma in children were reviewed from Japanese literatures. Cases with complete resection of the tumor without metastasis had good prognosis. Serum AFP was useful in many cases as a tumor marker for making diagnosis and following up patients in post operative course.
Anti-reflux surgery for two infants with unilateral vesico-ureteral reflux (VUR) was performed to prevent the development of reflux nephropathy. The first case, two-day-old male, was admitted to our hospital for the purpose of operation for atresia ani. At the age of 9 day-old, it was recognized that his renal function was sevesely impaired. By voiding cystourethrogram, the diagnosis of VUR (grade III) on the right side with left renal hypoplasia was made. To promote the normal growth of the right kidney, anti-reflux surgery according to Cohen's method was performed at the age of 97 day-old. His total renal function has remained stable after the surgical intervention. The second case was a 3 day-old male and admitted to our hospital because of poor sucking. Investigation revealed depressed renal function. With further examination, the diagnosis of left VUR (grade V) and right renal hypoplasia was made. Low protein diet (1.5-2.0g/kg/day) has been started to lower the osmolar loading. For the same reason as the first case, an anti-reflux surgery according to Cohen's method was performed at the age of 74 day-old. His renal function improved dramatically after the surgery. And he was discharged from our hospital at the age of 116 day-old. These cases might suggest that an early surgical intervention and low protein diet lead to excellent recovery of renal function in patients with congenital urinary tract anomalies.
A twenty-month-old boy with congenital cystic adenomatoid malformation is presented. He had a two-month history of unresolving pulmonary infiltration following a respiratory infection. Chest X-ray after bronchographic examination showed multiple lucent lesions occupying most of the right middle and lower lung fields, with contralateral shift of the mediastinum. At thoracotomy, multicystic lesion involving the right lower lobe was found. A lobectomy was performed and the histologic diagnosis was cystic adenomatoid malformation.
Renal abscess is uncommon in children and extremely rare in infants. We report here a case of 18-day-old boy. He was admitted with high fever and a mass in the right quadrant of the abdomen. Sonography and CT demonstrated the abnormal fluid collection around right kidney. Operation revealed not only a perirenal abscess but also a collection of lymph. Right nephrectomy and drainage were performed for uncontrollable bleeding from the kidney. Histological examination revealed small and multiple renal abscesses with congenital renal cyst. Staphylococcus hominis were cultured form the abscess and blood. After operation retrograde cystography demonstrated residual right ureter and ipsilateral VUR. In the diagnosis of renal and perirenal abscesses, sonography and CT are not invasive and valuable. Though aggressive antibiotic therapy is recommended as initial treatment, surgical management is mandatory if there is an evidence of perinephric extention or no improvement by conservative therapy.
Mesenchymal hamartoma of the liver is a rare tumor in childhood. Moreover, the recurrence after surgical intervention has not been reported in Japanese literatures. We experienced a case of mesenchymal hamartoma of the liver which recurred 2 months after excision. Two-year-old female was admitted to our hospital for the evaluation of abdominal distension. CT and abdominal ultrasonography revealed multicystic lesions of the liver but laboratory and angiographic findings denied a malignant tumor of the liver. A benign cystic tumor was the most probable diagnosis. Operation was performed on July 24, 1984. The cyst in the right lobe of the liver was enucleated and the middle lobe which contained small cyst was partialy resected. Histology of the tumor was cystic mesenchymal hamartoma of the liver. Recurrence of cystic lesion was observed by CT scan and abdominal ultrasonography after 2 months. The patient was readmitted for reoperation. CT and ultrasonography revealed nearly the same findings. Extended right lobectomy was performed on July 25, 1985. The postoperative course was uneventful. No recurrence has been detected after 3 years. Only one case of recurrence was reported in English literatures. We consider that surgical treatment of cystic mesenchymal hamartoma of the liver should be lobectomy of the liver.