An ileostomy at the aganglionic ileum for total colon aganglionosis.

For the initial management of total colon aganglionosis (TCA), an ileostomy is usually created at the distal end of the ganglionic bowel, which is associated with profuse diarrhea resulting in a water and electrolyte imbalance as well as malnutrition during the ileostomy period. An ileostomy at the aganglionic ileum several centimeters toward the anal side of the transition for TCA is thus expected to cause bowel retention without any bowel obstruction and to facilitate water reabsorption in the small bowel. We performed this procedure in 2 babies with TCA. The length of the aganglionic ileum was 50 cm and 10 cm, respectively. The ileostomies were made 5 cm distal to the transition at 1 month and 1 day after birth, respectively. Oral feedings were well tolerated, resulting in adequate weight gain and the stools were semisolid during the ileostomy periods. Definitive operations using the Duhamel procedure with a right colon patch graft without the creation of a new ileostomy were successfully performed at 7 and 3 months of age, respectively. The postoperative courses were satisfactory. An ileostomy at the aganglionic ileum appears to be the method of choice as initial management for patients with TCA.

A case of biliary atresia with cystic dilatation of the extrahepatic bile duct and polysplenia syndrome.

A 79-day-old girl presented with jaundice and acholia. Laboratory findings disclosed elevated levels of bilirubin, transaminases, and gamma-glutamyl transferase. A chest X-ray film showed dextrocardia. A computed tomographic scan revealed a cystic mass at the porta hepatis, multiple spleens in the right side of the abdomen, and absence of the inferior vena cava. Under the diagnosis of biliary atresia (BA) (I-cyst) associated with polysplenia syndrome, a hepatic portojejunostomy was performed at 80 days of age. The postoperative course was uneventful, and the jaundice cleared. Although the occurrence of associated anomalies in BA is rare, polysplenia is most commonly seen, and its incidence is reported to be 2% to 10%. Patients with BA and polysplenia usually have a poor prognosis, however, this patient may have a good prognosis due to uneventful recovery from the jaundice.

Novel breakpoints of the EWS gene and the WT1 gene in a desmoplastic small round cell tumor.

We report here a 15-year-old boy with an intraabdominal desmoplastic small round cell tumor (DSRCT). Cytogenetic analysis of the tumor cells showed the chromosomal translocation (11;22). Reverse-transcriptase polymerase chain reaction and sequencing analysis revealed a chimeric transcriptional message of the EWS gene exon 10 fused to the WT1 gene exon 8. The typical chimeric transcript seen in DSRCT is an in-frame fusion of EWS exon 7 to WT1 exon 8. The tumor in this case had a novel and longer chimeric transcript, which should be a potent transcription factor. Genetic analysis is a very powerful and specific aid in the differential diagnosis of small round cell tumors.

Prenatally detected cystic neuroblastoma.

A case of adrenal cystic neuroblastoma (NB) detected by prenatal ultrasonography (US) is presented. The suprarenal mass initially showed pure cystic features on a variety of imaging studies such as US, computed tomography, and magnetic resonance imaging. Tumor markers were negative. The mass was suspected to be an adrenal hemorrhage rather than a NB. Three months later, although the diameter was unchanged, the thickness of the cyst wall seemed to have slightly increased. Surgical exploration revealed an adrenal cystic tumor and histology showed a NB in situ. Forty-five infants with prenatally detected NB were found in the English literature; about one-half of them were cystic NBs, and most had a favorable outcome.

Heterotopic pancreas of the esophagus associated with a rare type of esophageal atresia.

An infant with a rare type of esophageal/tracheal anomaly associated with heterotopic pancreas of the esophagus is herein reported. The upper pouch containing heterotopic pancreas reached 1.5 cm below the tracheal carina, and the distal esophagus connected to the trachea 2 cm above the tracheal carina and thus formed a partial duplication of the esophagus. Heterotopic pancreas of the esophagus is extremely rare, with only 7 cases previously reported. Here we report the combination of heterotopic pancreas and esophageal atresia with tracheoesophageal fistula.

Chromosomal aberration in lipoblastoma: a case with 46,XX,ins(8;6)(q11.2;q13q27).

Chromosomal aberrations involving 8q11.2 have been reported in lipoblastoma. We report here a case of lipoblastoma with new chromosomal aberration. 46,XX,ins(8;6)(q11.2;q13q27). Cytogenetic analysis would facilitate the clinical differentiation between myxoid liposarcoma and the pathologically similar lipoblastoma and the identification of genetic loci related to cellular growth.

Severe hypertension and cardiac failure associated with neuroblastoma: a case report.

The authors report on 3-year-old-girl with neuroblastoma complicated by severe hypertension and cardiac failure. She had cardiomegaly and pleural and pericardial effusions. Echocardiogram showed left ventricular hypertrophy and decrease of the left ventricular ejection fraction to 0.36 (normal > .40). Abdominal computed tomographic scan indicated a 7 x 7-cm tumor in the left suprarenal area. There was a marked increase in catecholamines and metabolites in her body fluids. After hypertension was controlled with doxazosin (a long-acting alpha 1 adrenergic blocker), her cardiac function gradually improved. A tumor was surgically removed and diagnosed as a poorly differentiated ganglioneuroblastoma. Preoperative differentiation between neuroblastoma and pheochromocytoma was not possible on the basis of catecholamine analysis or imaging studies including computed tomography scan and magnetic resonance imaging. It is important to control hypertension quickly in the patients with catecholamine-induced cardiomyopathy to facilitate surgical intervention for diagnosis and treatment.

Pre-operative time course changes in liver function tests in biliary atresia: its usefulness in the discrimination of biliary atresia in early infancy.

In order to investigate the possibility of early discrimination of extrahepatic biliary atresia from other cholestatic diseases, a series of results of liver function tests in infants with cholestatic diseases were reviewed. The results of routine liver function tests (LFT) recorded in patients' charts were reviewed within 12 weeks after birth in 47 infants with extrahepatic biliary atresia (BA), 10 infants with neonatal hepatitis (NH) and 130 age-matched control infants (CO) without cholestatic diseases. The mean of each test value for each week after birth was derived from the actual data examined in each infant. No differences were observed between BA and CO in the levels of aminotransferases within 2 weeks after birth. Total bilirubin and direct bilirubin levels were significantly different between BA and CO within 1 week after birth (16.1 +/- 3.2 mg/dL vs 11.1 +/- 4.5 mg/dL, 4.6 +/- 2.6 mg/dL vs 0.7 +/- 0.3 mg/dL, respectively). The direct bilirubin-total bilirubin ratio exceeded 25% within the first week in BA. The individual values of direct bilirubin (DB) exceeded 2 mg/dL within the first week in all infants with BA, while none of the individual values exceeded 1.6 mg/dL in CO. Gamma-glutamyl transpeptidase levels were significantly different between BA and CO at 4 weeks (432 +/- 272 IU/L vs 79 +/- 43 IU/L) and thereafter; and were significantly different between BA and NH at 6 weeks (314 +/- 232 IU/I vs 69 +/- 58 IU/L) and thereafter. These data suggest that the determination of direct bilirubin within 1 week after birth can detect extrahepatic biliary atresia patients from those with physiologic jaundice, and gamma-glutamyl transpeptidase levels may discriminate BA from NH at no later than 6 weeks of age.

A case of prenatally detected bilateral paraureteral diverticula associated with neurofibromatosis.

Although paraureteral diverticulum is thought to be a congenital anomaly, no prenatally detected cases have been reported. Herein, we report a case of bilateral paraureteral diverticula prenatally detected by maternal ultrasonography. This case also had dysplastic kidneys in association with neurofibromatosis.

Early definitive surgery for total colon aganglionosis: Duhamel procedure with right colon patch graft using GIA stapler.

A report is presented of an infant with total colon aganglionosis (TCA) with ileal involvement in whom a modified Duhamel procedure was used accompanied by a right colon patch graft 12 cm in length using a GIA-90 stapler at 3 months of age. The patient with TCA is usually treated by an initial diverting ileostomy followed by a definitive operation performed several months later. However, the management during the ileostomy period is associated with a variety of problems. In to minimize the resulting complications, we developed a procedure to allow a much earlier definitive operation.

Intravenous digital subtraction angiography for the evaluation of renal artery blood flow following the removal of a neuroblastoma.

There have been several reports of acute renal failure following the resection of an abdominal neuroblastoma combined with ipsilateral nephrectomy as well as the atrophy or disappearance of an unresected kidney after tumor resection. Spasms or thrombosis of the renal artery during tumor excision are considered to be the major cause. Since 1989, intravenous digital subtraction angiography (IVDSA) has been used to evaluate the renal artery blood flow immediately following surgery in seven patients with abdominal neuroblastomas. IVDSA was performed using a central venous catheter inserted prior to surgery. In all seven patients, IVDSA provided clear images for the evaluation of renal artery blood flow. In one of the two patients whose kidneys briefly became cyanosed during tumor excision, IVDSA demonstrated an occlusion of the renal artery and prompt measures could be taken to reestablish the blood flow. No complications of IVDSA occurred in any of the seven patients. IVDSA using a central venous catheter was thus considered to be useful for evaluating the renal artery blood flow in patients with a suspected renal artery blood flow disturbance without any risk of complications, and this modality obviated the need for intraarterial angiography.

Clinico-pathological studies on a transitional type between extrahepatic biliary atresia and paucity of the interlobular bile ducts.

Among the neonatal and infantile cases of obstructive jaundice seen at Niigata and Yamagata University Hospitals between 1976 and 1990, extrahepatic bile ducts were visualized in 19 cases by either preoperative endoscopic retrograde cholangiopancreaticography (ERCP) or intraoperative cholangiography. Neonatal hepatitis was diagnosed in 3 of these cases by clear images of the bile duct system extending from the common bile duct to the intrahepatic bile duct. In 7 cases, the common bile duct was able to be seen, while the common hepatic duct was only slightly visualized. Four of these 7 cases were consistent with paucity of the interlobular bile ducts (PILBD) based on hepatic histology, while the remaining 3 showed fibrosis, bile ductular proliferation, and many bile plugs in the bile ductuli of the portal areas, concurrent with histological changes in extrahepatic biliary atresia (EHBA), not PILBD. In 9 cases, only the common bile duct was visualized while the common hepatic duct was not seen, 7 of these 9 cases being consistent with type III-a1 EHBA. In 2 cases, neither fibrosis nor proliferation of the bile ductuli was observed in the portal areas, and portal areas without any bile ductuli were also seen, in accordance with findings for PILBD. Three cases which showed similar hepatic histological findings to EHBA despite the presence of patent extrahepatic bile ducts, and 2 cases which had obstructed extrahepatic bile ducts and hepatic histological findings similar to PILBD, were thought to be of a transitional type between EHBA and PILBD.(ABSTRACT TRUNCATED AT 250 WORDS)

[Obstructive jaundice from benign hepatic cysts].

Obstructive jaundice secondary to benign, non-parasitic hepatic cysts is rare, since there are only 19 previously reported cases. We reported a case of polycystic disease of the liver and kidneys in which relief of developing jaundice was obtained by hepatic lobectomy and fenestration procedure for the multiple cysts. Immense occupancy and porta hepatis proximity of the cysts were triggers for developing jaundice. Urgent resection of the major cysts is recommended for extremely progressing jaundice.

[Cefotiam concentration in the peritoneum in infants during surgery].

1. Supposing the hernia sac to be peritoneum, intraperitoneal transition of cefotiam (CTM) was examined on 57 cases of infantile inguinal hernia subdivided into 7 groups. 2. After one shot intravenous injection, CTM showed rapid transition into the blood and the peritoneal tissue and even at a dose of 40 mg/kg and 120 minutes later. CTM exhibited an excellent antibacterial activity considering of MIC80 against clinically isolated bacterial strains. 3. Healing by first intention of operative wounds was obtained in all of the cases given CTM without any case suggestive of postoperative infection. No clinical side effect was observed in any one of the cases.

Intestinal pseudoobstruction in a neonate caused by idiopathic muscular hypertrophy of the entire small intestine.

We report a case of intestinal obstruction in a neonate caused by massive muscular hypertrophy of the entire small intestine. Furthermore, the muscularis propria consisted of three layers, in most part. The pathogenesis of this apparently unique lesion is obscure.

[Cholecystolithiasis causing Mirizzi's syndrome with a rare anomaly of the extrahepatic bile duct in a child. Report of a case].

A 14 year-old girl, who was admitted to our hospital due to increasing jaundice, intermittent right upper quadrant pain and fever, underwent operation based on the diagnosis of the Mirizzi's syndrome. Calculi filled the cystic duct and compressed the right hepatic duct. The right and left hepatic ducts lay closer to the duodenum than usual. The operation was limited to cholecystectomy alone. The calculi showed laminar cut surfaces and were composed of bilirubin lime. In addition to reporting our case, the literature dealing with this particular entity is reviewed.

Case report of biliary atresia associated with preduodenal portal vein, ventricular septal defect and bilobed spleen.

A case report of biliary atresia associated with preduodenal portal vein is presented with a review of 27 similar cases previously reported. The occurrence of associated anomalies in these 28 cases has a much higher frequency (82%) than coincidental association. They were associated with cardiovascular anomalies in 71%, polysplenia in 68%, malrotation of the intestine in 61%, situs inversus in 22% and duodenal atresia in 9%. Developmental anomaly is considered to participate strongly in the aetiology of biliary atresia combined with preduodenal portal vein.

A case report of intrahepatic biliary atresia showing an unusual liver histology.

The extrahepatic biliary tract of a male infant, including both the left and right hepatic ducts, was proven to be patent up until his time of death, however, histologic examination of the liver revealed severe fibrosis of the portal areas with ductular proliferation, scattered bile thrombi, and subsequent biliary cirrhosis. In this case, since the obstructive lesion occurred at the secondary branching of the bilateral hepatic duct and because fewer changes were present in the interlobular bile ducts, it seemed possible to consider that the liver histology revealed findings similar to those of extrahepatic biliary atresia.

A case of congenital colonic atresia associated with atresia ani.

The association of atresia ani with congenital colonic atresia is extremely rare, with only one such case having been reported up to date. We have recently treated a female infant with atresia ani (covered anus complete) accompanied by atresia of the sigmoid colon, who was managed by three stage surgery.