Flow cytometric DNA content analyses were performed on samples of 72 patients with neuroblastoma. Nuclear suspensions obtained from paraffin-embedded samples were prepared using the method described by Schutte et al. DNA ploidy could be determined in 62 out of 72 patients. DNA diploidy was detected in 25 (40.3%) and DNA aneuploidy was in 37 (59.7%) of the 62 patients. High incidence of DNA aneuploidy was observed in patients with prognostically favorable indicators such as age (under one year old), clinical stage (I, II, IVS) and primary site (extraadrenal sites). DNA aneuploidy was associated with age (p<0.0l), clinical stage (p<0.0l) and survival rate (p<0.01). It is of great interest that the distribution of patients differs in clinical stage between DNA diploidy and aneuploidy. Almost all patients with DNA diploidy were observed in advanced clinical stages, whereas patients with DNA aneuploidy were observed in all stages. As our research on samples obtained at different periods from the same patients revealed no changes in DNA ploidy, biological behavior may differ between diploidy and aneuploidy. In patients with diploidy, tumor growth might be too rapid to be diagnosed at an early clinical stage. In contrast, in patients with aneuploidy, tumor growth might be slow. It is concluded that DNA ploidy is not only a significant prognostic factor but also an indicator of biological behavior of neuroblastoma.
To determine whether lithocholic acid (LCA) contributes to the occurrence of intrahepatic cholestasis associated with total parenteral nutrition (TPN-IHC) in neonates. we investigated the serum bile acid fraction of neonates on TPN. Twenty-five surgical neonates who received TPN for more than 2 weeks without enteral nutrition were studied. TPN-IHC was defined by having a serum direct bilirubin level of over 2.0mg/dl. Serum bile acid fraction was analyzed by HPLC using 3α-hydroxy steroid dehydrogenase. Eleven patients developed TPN-IHC during the first month of life, and 8 (32%; IHC group) had TPN-IHC when bile acid was analyzed. Serum total bile acid levels for both groups were 14.4 and 76.1nmol/ml, respectively. Glycine and taurine conjugated cholic and chenodeoxy cholic acid were detected in both the non-IHC and IHC groups, but only traces of unconjugated and secondary bile acids (deoxycholic and lithocholic acid) were found. In conclusion, LCA is unlikely to be a causative factor of TPN-IHC in neonates.
During the period between 1973 and 1989, 26 patients with left sided congenital diaphragmatic hernia (CDH) were operated upon within 24 hours after birth at Kobe Children's Hospital. The stomach was located in the chest (group A) in 18 patients, whereas the stomach in the abdomen in 8 patients (group B). We compared the mortality, associated anomalies, the time requiring endotracheal intubation, incidentce of persistent fetal circulation (PFC), and postoperative PaC0_2/Ventilation Index between the two groups. The mortality rate was 44.4% in group A, and 16.7% in group B. Each of the dead cases revealed severe pathological conditions, eg. congenital heart diseases, TEF, tracheomalacia, or congenital cytomegalovirus infection, etc. The time requiring respiratory management ranged from 0 day to 39 days (mean 15days) in group A, and 2 days to 7 days (mean 4 days) in group B. PFC occurred in 75% in group A, but none in group B (p<0.01). According to PaC0_2/Ventilation Index, seven of 15 cases belonged to high-risk group in group A, and only one of 8 cases was seen in group B. The stomach in the chest in CDH is a significant sign of poor outcome.
We reviewed the cases which needed prolonged ventilatory support over a month after cardiovascular surgery for congenital heart diseases. We have experienced 12 such cases among 458 surgical cases between November, 1981 and December, 1988 at our institution. The cases were classified into 3 groups as follows. Group I (n : 6) was unable to be extubated due to heart failure. Group II (n : 2) was due to heart failure combined with other anomaly. Group III (n : 4) was due to tracheo-bronchial abnormality. 4 cases in Group I were able to be extubated after recovery from heart failure. All cases in Group II were extubated after repair of the combined anomalies. But all cases in Group III were unable to be extubated due to complicated tracheobronchomalacia and 2 of them have been placed under very long-term ventilatory support with CPAP.
The normal position of the anus is not defined objectively. Using a simple technique, the anal position index was designed to define the position of anus in neonates and infants as follows: the ratio of the distance between the scrotum and the anus to the distance between the scrotum and the coccyx for males and the distance between the vagina and the anus to the distance between the vagina and the coccyx for females. The anal position index in neonates was not affected by body weight. In male infants, the anal position index was not affected by age. But in female infants, the anal position index decreased from neonatal period to three months of age but no significant difference was demonstrated from three months of age to twelve months of age. We think that the anal position index is useful as an objective parameter to assess the localization of the anus.
The case of a nine year-old boy with achaiasia of the cardia was reported. Three main features of achaiasia including esophageal aperistalsis, incomplete relaxation of the lower esophageal sphincter (LES), and elevated resting LES pressure were clearly demonstrated by manometric studies. The patient was treated by pneumatic dilatation six times over two years without completely relieving the obstructive symptoms. A modified Heller cardiomyotomy was thus performed and Thal cardioplasty with fundic patch was added in order to prevent postoperative gastroesophageal reflux. The manometric studies of esophageal achalasia were discussed.
We have performed splenectomy in 26 children with chronic idiopathic thrombocytopenic purpura (ITP). Immediately before splencetomy, high dose intravenous gammaglobulin (HDIVG) was given in 8 patients (IVG group) and HDIVG was not given in 18 patients (N-IVG group). The age of IVG group was 11.4 ± 3.2 years and older children were more frequently found in this group (vs 7.9 ± 3.6 years in N-IVG group) (p<0.025). Platelet count (x10^4/mm^3) was 18.0 ± 11.4 in IVG group and 3.2 ± 3.5 in N-IVG group. In IVG group, significant rise in platelet count was seen (p<0.005). Experience of the surgeons in IVG group was 7.4 ±1.7 years and 9.6±7.0 years in N-IVG group. There was no significant difference. Blood loss (ml/kg), however, was 1.9 ±1.6 in IVG group and 9.7 ± 5.2 in N-IVG group. Fewer blood loss was recognized in IVG group (p<0.005). In treatment for ITP HDIVG is effective not only to defer or reduce needs of splenectomy but also to hematologically prepare before splenectomy and leads us an easy control of blood loss in operation. The side effects of HDIVG are deniable but the therapy costs. HIDIVG therapy have altered splenectomy for ITP in both quality and quantity.
Feminizing genitoplasty was performed in 13 patients with ambiguous genitalia. Of these 13 children, ranging from 2 to 16 years old, 9 had congenital adrenal hyperplasia (21-hydroxylase deficiency: solt-losing type was in 6, simple virilizing type in 3), the others were diagnosed as male pseudohermaphroditism in 2, and mixed gonadal dysgenesis in 2. Preoperative genitography and endoscopy were routinely done for evaluation of the level of vaginal entry (low or high) and demonstration of the uterus. In 12 children, clitoroplasty with saving of the dorsal neuro-vascular bundle and M-shaped-flap vaginoplasty were performed successfully. In 6 of them labioplasty was simultaneously done at the operation. In one child with high vaginal entry new vagina was reconstruted with bilateral perineal skin flaps, but the vaginal cavity was insufficient in length. We conclude that in female children with ambiguous genitalia, except no vagina, one-staged feminizing genitoplasty can be done at the age of 2-3 years old. And vaginal bougienage in helpful in preventing vaginal stenosis, and an adequate hormonal treatment is important to keep vagina wide in these patients.
A case with duplication of the duodenum found in the neonatal period is reported. A nine-day-old girl was referred with a history of frequent bilous vomitings since 7th day of life. The upper gastrointestinal X-ray, duodenal ultrasound and computed tomographic findings demonstrated severe narrowing of the second portion of the duodenum produced by an extraluminal cystic lesion. At operation, the duplication cyst was found to arise from the anti-mesenteric side of the second portion of the duodenum. The cyst was 3.5cm X 2cm X 2cm in size and projected against the true duodenal lumen, but no communication was present. The yellowish serous fluide was aspirated from the cyst. After the cyst was diagnosed as a duplication cyst, excision of the common wall between the duplication and the adjacent intestine was performed. Pathologic study showed that the musculature of the cyst was connected with the smooth musculature of the duodenal wall, The common wall was formed by the smooth musculature and the intestinal mucosal membrane. The patinet showed no other anomalies. Post operative course was uneventful and the patient was discharged at 37th day of life. Duplication cyst of the duodenum is a rare anomaly, representing only 10% of all duplications in the alimentary tract. According to the literature in Japan, 28 cases with duodenal duplication had been reported. Among these, only 6 cases were found in the neonatal period. The Bremer's theory is considered the best explanation of the development of the duplication in this case.
A four-day-old boy was transfered to Tsukuba University Hospital with abdominal distension due to perforation of the gastrointestinal tract. Chest and abdominal X-ray showed gastric perforation but there were several interesting findings. The stomach-bubble shadow was right sided with the gastric impending rupture. Three small mass shadows within intraabdominal free air were seen in the right region, indicating polysplenia. The cardiac shadow was in normal position suggesting left isomerism. The operative findings were just as suggested by preoperative diagnosis and simultaneously disclosed an annular pancreas causing duodenal stenosis, preduodenal portal vein and reversed rotation of the intestine. Direct suture closure of the gastric wall and duodenoduodenostomy were performed, and postoperative course was uneventful. Ultrasonography and MR images revealed PDA, VSD, IVC interruption, and azygos and hemiazygos continuation.
We experienced a case of 884g female infant with intrauterin growth retardation, who had abdominal distention and bilous vomiting. At operation, a caliber change was observed in ileum which was full of tenacious meconium. A tube ileostomy was performed and the obstruction was successfully relieved. This case was considered to correspond to meconium disease named by Rickham. Half cases of meconium disease were suffered from intrauterin growth retardation in the review of former reports. Thus, possible etiology of the condition may be related to intrauterin growth retardation.
A nine-month-old boy was admitted to our hospital with hepatomegaly and precocious puberty. Ultrasonography, CT-scan and DSA-angiography demonstrated a huge tumor in the right lobe of the liver. Serum AFP was 877400ng/ml and Human Chorionic Gonadotropin (HCG) level in serum and urine were 36IU/ml and 66MIU/ml. The patient underwent a right hepatic lobectomy. Histology of the resected tumor revealed embryonal type hepatoblastoma and HCG in the multinucleated giant cells in the immunohistochemically (PAP method). HCG in serum normalized at day 8 after surgery, and serum AFP fell progressively to a 14ng/ml. The patient underwent treatment of chemotherapy during the following 3 months. The boy is asymptomatic with two years follow-up and no tumoral recurrence.
A 2-year-old boy with bilateral cervical fistulae, bilateral pre-auricular fistulae and hearing impairment was reported. His father had the same symptoms and his elder sister had also bilateral pre-auricular fistulae. These symptoms were considered to fit in Branchio-Oto-Renal (BOR) syndrome defined by Melnick et al, although renal abnormality was not demonstrated in this case. This is the forth case of BOR syndrome reported in Japan.
A 10-year-old Japanese boy with chronic pancreatitis was treated. Ultrasonography and computerized tomography showed a markedly dilated pancreatic duct. Intraoperative pancreaticography showed a rare type of fusion of the dorsal and the ventral pancreatic ducts and the dilatation of the pancreatic duct which extended distally from the site of the fusion of the ducts at the embryonic stage. The patient underwent ductoplasty of the minor papilla. He is well and asymptomatic 4 months after the operation. This case suggests that partial obstruction of the pancreatic duct due to incomplete fusion of the two pancreatic ducts may have predisposed this child to the development of chronic pancreatitis.
A girl of two years and 9 months was addmitied to our hospital with disturbance ol eyesight. Close examinations revealed dumbbell type-neuroblastoma with metastasis to the head and femur bone, sphenoid bone, and abdominal skin (Stage 4A). After the chemotherapy of the A1 protocol and radiation therapy for the metastatic lesion of the head bone, intraspinal and metastatic lessions disappeared on imaging studies. And then resection of the primary tumor at the retroperitoneal cavity was performed. Microscopic examination revealed a well differentiated type of ganglioneuroblastoma in the primary tumor. Auto bone marrow transplantation was then performed. Recurrent tumor in the spinal canal was detected on CT scan two years and 8 months after initiation of treatment. Laminotomy and resection of intraspinal tumor were performed immediately. Microscopic examination of the spinal tumor revealed ganglioneuroma, Neuroblastoma is notable for its ability to spontaneously regress and in some instances, to differentiate to less malignant ganglioneuromas. This is sometimes found after chemotherapy or radiation therapy. We speculated that in this case, ganglioneuroblastoma differentiated into a mature ganglioneuroma in the spinal canal by chemotherapy.
A case of giant cyst formation in the ureter was diagnosed in the 36th week of gestation by ultrasono-graphic examination. The patient was born at 40 weeks of gestation in a normal delivery. Two giant cysts which occupied the area between the kidney and the bladder were found by US and CT. Preoperative diagnoses of a hypoplastic kidney on the right and hydronephrosis were made, but distal ureteral atresia associated with paravesical cyst was found at operation and later confirmed by histological examination. Distal ureteral atresia is a very rare deformity of the ureter. The literatures was reviewed.
We have a case of rectal membraneous atresia in a 1-day-old male. His anus looked normal, but the rectum was closed by a membrane about 5 mm proximal to the pectineal line. The membrane was excised through the anus and bougienage had continued for about 3 months after operation. There is no functional problem in his evcuation. Rectal membraneous atresia is not described in The International Classification (1970). It was first named by Japanese study group pf anorectal anomalies. Rectal atresia is classified in the high deformities (supralevator). The definition of the term "rectal atresia" in 1970 was that the proximal and distal pouches were connected with a cord like tissue. But several patients whose rectums were separated with a membrane were rarely reported in Japan. They are clearly in the low deformities. In the Workshop at Wingspread in U.S.A., rectal membraneous atresia was not classified. We hope the anomalies like this case will classified clearly.
A two-year eight-month-old girl was admitted due to vomiting and abdominal distension. Supine radiography of the abdomen revealed a stomach markedly distended with air. We suspected acute gastric volvulus and performed conservative therapy. On the next day an upper gastrointestinal series with a nasogastric tube confirmed the presence of mesenteroaxial gastric volvulus and demonstrated no gastrographin passing through the Pyloric outlet. Because the patinet's condition not relieved An Operation was performed. We recognized gastric perforation of the major flexureand mesenteroaxial gastric volvulus, furthermore the ligature between a stomach and a transverse colon. We thought that the ligature was cause of acute gastric volvulus. Gastropexy and suture of perforation was performed. We discussed the cause and treatment of acute gastric volvulus with gastric perforation in childhood.