ABSTRACT
The recto-sigmoid index on barium enema may aid in the diagnosis of Hirschsprung's disease. However, data on its reliability in different age groups are sparse. The recto-sigmoid index and transitional zone were evaluated blindly in 107 patients with diagnostic rectal suction biopsies. Patients were divided into 3 groups: neonates, infants older than 1 month, and children. The recto-sigmoid index and transitional zone agreed with the histopathologic diagnosis in 79% and 87% of the cases, respectively. Their negative predictive values reached clinical significance in infants and children but not in neonates. Their positive predictive values were not significant in any age group. The recto-sigmoid index identified 4 patients with recto-sigmoid Hirschsprung's disease whose diagnosis was missed by evaluating the transitional zone alone.
Subject(s)
Barium Sulfate , Enema , Hirschsprung Disease/diagnostic imaging , Adolescent , Adult , Biopsy , Child , Child, Preschool , Colon, Sigmoid/diagnostic imaging , Colon, Sigmoid/pathology , Confidence Intervals , Contrast Media , Diagnosis, Differential , Female , Hirschsprung Disease/pathology , Humans , Infant , Infant, Newborn , Male , Predictive Value of Tests , Radiography , Rectum/diagnostic imaging , Rectum/pathology , Retrospective Studies , Sensitivity and Specificity , SuctionABSTRACT
BACKGROUND: Radiologic assessment of pancreaticobiliary ductal disease (PBDD) in children currently consists of physiologic tests (radionuclide examinations) or invasive anatomic studies (ERCP and PTC). An accurate noninvasive and reproducible examination that can direct the subsequent need for more invasive studies would be helpful in this patient group. OBJECTIVE: To determine the effectiveness of MRCP as a screening tool for PBBD in the pediatric population. MATERIALS AND METHODS: Over the last year, 33 patients ranging from 7 months to 20 years of age were prospectively evaluated with MRCP on a 1.5 T magnet. One patient was examined twice, several months apart. Thirteen patients had liver transplants. Coronal SPGR and heavily T-2W FSE cross-sectional images were obtained. Standard and oblique 2- to 6-cm-thick slab SSFSE (single-shot fast spin echo) acquisition and 3D MIP reconstruction of 2D FSE images were obtained in the planes of the CBD and pancreatic duct. Nine studies were performed with the patient under sedation with chloral hydrate or nembutal and fentanyl with quiet respiration, and the non-sedated patients were assessed with single breath hold or quiet respiration. Three patients received secretin. MRCP results were correlated with ERCP (9), PTC (7), liver biopsy (13), clinical information (6), surgery (3), and autopsy (2). RESULTS: All 34 studies performed were considered diagnostic. Periportal fluid, proximal bowel fluid, and gallbladder distention did not significantly diminish the diagnostic information in any cases. Motion artifact did not cause serious degradation in image quality. MRCP depicted abnormalities including stones, stricture, intraductal tumor, and extrinsic compression, all of which were confirmed at ERCP, PTC ( two unsuccessful in patients with non-dilated ducts by MRCP), surgery, liver biopsy, and autopsy. There were no false-negative examinations. Normal pancreatic studies performed to exclude pancreas divisum were followed without additional clinical or laboratory evidence of pancreatitis. Secretin administration increased the conspicuity of the pancreatic duct in two of three patients. CONCLUSION: MRCP is a fast non-invasive method of evaluating the pancreatic duct and biliary tree in children. A normal MRCP may obviate the need for PTC or ERCP. Abnormalities detected on MRCP can direct the type of intervention.
Subject(s)
Biliary Tract Diseases/diagnosis , Magnetic Resonance Imaging/methods , Pancreatic Diseases/diagnosis , Adolescent , Adult , Child , Child, Preschool , Female , Humans , Infant , Male , Prospective StudiesABSTRACT
OBJECTIVE: To determine the effectiveness of a new multidisciplinary approach using neoadjuvant intrahepatic chemoembolization (IHCE) and liver transplant (OLTx) in patients with unresectable hepatic tumors who have failed systemic chemotherapy. MATERIALS AND METHODS: From November 1989 to April 1998, 14 children (2-15 years old) were treated with 50 courses of intra-arterial chemotherapy. Baseline and post-treatment contrast-enhanced CT and alpha-fetoprotein levels were performed. Seven had hepatoblastoma, and 7 had hepatocellular carcinoma (1 fibrolamellar variant). All patients had subselective hepatic angiography and infusion of cisplatin and/or adriamycin (36 courses were followed by gelfoam embolization). The procedure was repeated every 3-4 weeks based on hepatic function and patency of the hepatic artery. RESULTS: Six of 14 children received orthotopic liver transplants (31 courses of IHC). Pretransplant, 3 of 6 showed a significant decrease in alpha-fetoprotein, while only 1 demonstrated a significant further reduction in tumor size). Three of 6 patients are disease free at this time. Three of 6 patients died of metastatic tumor 6, 38, and 58 months, respectively post-transplant. One of 14 is currently undergoing treatment, has demonstrated a positive response, and is awaiting OLTx. Three of 14 withdrew from the program and died. Four of 14 patients developed an increase in tumor size, developed metastatic disease, and were not transplant candidates. Two hepatic arteries thrombosed, and one child had a small sealed-off gastric ulcer as complications of intrahepatic chemoembolization. CONCLUSION: The results of intrahepatic chemoembolization are promising and suggest that some children who do not respond to systemic therapy can be eventually cured by a combination of intrahepatic chemoembolization orthotopic liver transplant. Alpha-fetoprotein and cross-sectional imaging appear to be complementary in evaluating tumor response. IHCE does not appear to convert an anatomically unresectable lesion to a candidate for partial hepatectomy.
Subject(s)
Carcinoma, Hepatocellular/therapy , Chemoembolization, Therapeutic/methods , Hepatoblastoma/therapy , Liver Neoplasms/therapy , Adolescent , Antineoplastic Agents/administration & dosage , Child , Child, Preschool , Cisplatin/administration & dosage , Doxorubicin/administration & dosage , Female , Humans , Infusions, Intra-Arterial , Liver Transplantation , Male , Treatment Outcome , alpha-Fetoproteins/metabolismABSTRACT
BACKGROUND: To evaluate the effect of intrahepatic arterial chemotherapy (IAC) on children with primary hepatic malignancies. METHOD: A nonrandomized inception cohort of 11 pediatric patients was referred for treatment of advanced primary hepatic malignancies at Children's Hospital of Pittsburgh. None of the patients was a candidate for resection before the initiation of IAC. Tumor response to treatment was observed by determining serum alpha-fetoprotein (AFP) levels and by abdominal computed tomographic scan. The patients received hepatic artery infusions of cisplatin and/or doxorubicin. The last five also received gelfoam embolization. RESULTS: Eight of 11 patients had multiple IAC treatments. Eight patients had AFP-producing tumors, and five of the eight had dramatic reductions in serum levels after IAC treatment. Five of the 11 patients underwent successful orthotopic liver transplantation after receiving IAC therapy, and the five explanted specimens showed varying degrees of tumor necrosis. One-year survival in patients in the authors' center is 67% for those with hepatoblastoma and 40% for those with hepatocellular carcinoma. Three-year survival is 60% and 30% for patients with hepatoblastoma and hepatocellular carcinoma, respectively. CONCLUSION: Intrahepatic arterial chemotherapy therapy can halt the progression and possibly down-stage advanced pediatric hepatic malignancies. This therapy can also be used as a successful adjunct in altering a patient's chance for successful liver transplantation.
