Biliary tract involvement in children with histiocytosis X.
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Biomedical subjects
Publications and source records attributed to F Brunelle.
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The authors present the first report of a pigment gallstone of the common bile duct in a 2 month-old infant treated in a non-surgical manner. The gallstone extraction was percutaneous, by a small catheter introduced in the biliary tract under ultrasound control. The clinical and ultrasonographic follow-up (19 months) confirmed complete recovery. This new therapeutic modality might avoid surgery for primary common bile duct cholelithiasis.
Between 1978 and 1985, 11 girls and one boy underwent an elective operation for a congenital choledochal dilatation associated with an anomalous biliopancreatic junction. In 10 out of these 12 cases the children suffered several episodes of abdominal pain, and the diagnosis was missed since a jaundice appeared. The ultrasonographic examination demonstrated in all cases a dilatation of both extra- and intrahepatic bile ducts. The preoperative diagnosis was always established by the mean of a transhepatic cholangiography (8 cases) or a percutaneous cholecystography (4 cases), which showed in every case a dilated choledochus, and a common biliopancreatic channel, 15 to 35 mm long. A high amylase level was found in the bile in 10/10 cases when it was measured. A cholecystokinin test was performed in 4 cases, resulting in each case in a considerable increase of amylase and lipase levels in bile. All children were treated by excision of the dilated choledochus and gallbladder, followed by an hepaticojejunostomy with a Roux en Y loop. The follow-up is 6 months to 5 years for 9 children: 8 are cured, and on girl, who had a major dilatation of the left intrahepatic bile ducts, suffered from episodic abdominal pain and an episode od cholangitis 6 years after the operation. The role of such a common channel in the pathogeny of congenital choledochal cysts, acute pancreatitis in children, and biliary carcinomas in young adults is discussed according to the literatures of the last 10 years.
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Seventy-one children with portal hypertension and a portocaval anastomosis were studied by ultrasound. The patency of the shunt may be inferred when it directly visualised. There are four indirect signs of patency: (1) decrease of the thickness of the lesser omentum; (2) decrease of the gastro-oesophageal collaterals; (3) decrease of the portal vein size; and (4) increase of the diameter of the inferior vena cava, as compared to pre-operative ultrasound.
A case of successful electrocoagulation of an internal mammary artery with a bipolar electrode and alternating current is reported in a boy with cystic fibrosis of the lung and hemoptysis.
The authors report a case of hemobilia in a ten-year-old boy after abdominal trauma. An intrahepatic false aneurysm was successfully embolized during the diagnostic angiogram. This is, to their knowledge, the first pediatric case successfully embolized with posttraumatic hemobilia.
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The authors describe a remote control feeding pacifier for easy realisation of upper G.I. series in children.
The authors report the ultrasound and angiographic features of adenomas occurring in children with glycogen storage disease. Seven cases from 83 patients were diagnosed either by ultrasound, preoperative angiography or during surgery. The lesions appear on ultrasound as multiple rounded intrahepatic masses. Their degree of echogenicity as well of vascularity on angiography is highly variable. Ultrasound is the modality of choice in detecting adenomas. No malignant degeneration was observed.
The authors studied 21 hepatic tumors in children using B-scan and real-time ultrasound as well as angiography. The final diagnosis was established surgically. Cystic tumors and hemangioendotheliomas were excluded. In all but one malignant tumor (15/16), ultrasound showed absence of one branch of the portal vein in the area of the tumor. Partial amputation of intrahepatic portal branches was confirmed angiographically in all cases. In all 6 benign tumors, ultrasound demonstrated a patent portal system despite compression by tumor. These findings were confirmed angiographically. No specific echo pattern allowing differentiation between benign and malignant tumors was found. The authors conclude that ultrasonic differentiation between malignant and benign hepatic tumors in children is possible, provided that special attention is given to the intrahepatic vessels.
The gallbladder, the cystic and the choledocal ducts are patent in one fifth of biliary atresia cases, and can be used for the corrective operation. The main interest of this hepatoportocholecystostomy (HPC) is to prevent the cholangitis episodes which are the most severe complication of successful hepatoportoenterostomies (HPE). However a singular complication of HPC has been noticed by previous authors: the choleperitonitis. We report a recent case in which this complication was diagnosed by a routine ultrasonography at the 3rd week of the uneventful postoperative course of a HPC performed in an 1 1/2 month old infant. An early reoperation (HPE) had a good result with restoration of a satisfactory bile flow. The child is anicteric with a follow-up of one year. In our experience with 208 corrective operations between 1969 and 1981, HPC was used in 38 cases, with restoration of bile flow in 17 cases. A choleperitonitis was noticed in 4 cases in this series, and in a 5th additional recent case. Three children were not suitable for reoperation because of very poor condition and died within 3 to 17 months after HPC. Two children were reoperated upon early with a good result. We advocate the HPC procedure despite the risk of choleperitonitis and emphasize the interest of early postoperative routine ultrasonography, especially when stools remain acholic at the 4th postoperative week.
The need for surgery in pancreatic pseudocysts in children is directly related to the status of the duct of Wirsung. Clinical deterioration was often the factor leading to surgery. The retrospective study of ten cases of pancreatic pseudocyst pointed to the value of ultrasound as an indicator of pancreatic duct involvement. CT was also of help in one case. ERCP, performed only in one patient before surgery, may be indicated in difficult cases.
Three cases of embolization of the liver in children with a specially designed micro-coil are presented. The coils can be delivered through 4.1 or 3.7 F catheter and so this technique may be employed in the very young child.