A Pediatric Case of Well-Functioning Supernumerary Kidney: Relative Renal Function Assessment Using Radionuclide Imaging.

We present an interesting image of a well-functioning supernumerary kidney evaluated with DMSA (dimercaptosuccinic acid) renal scintigraphy in a 14-year-old girl. At 2 years of age, the patient had a diagnosis of supernumerary kidney. She remained asymptomatic up to childhood age, and then a DMSA study was required to guide the following adequate surveillance strategy. DMSA study provided a clear imaging of supernumerary kidney in the left side of the abdomen showing a regular uptake and a normal function in relation to its own size.

Pancreaticoduodenectomy for malignancies in children.

PURPOSE: Malignant tumors of the common bile duct or of the pancreas head are uncommon in childhood [Perez EA, Gutierrez JC, Koniaris LG, Neville HL, Thompson WR, Sola JE. Malignant pancreatic tumors: incidence and outcome in 58 pediatric patients. J Pediatr Surg. 2009; Jan; 44 (1): 197-203]. With radical surgery being the standard cure for nonmetastatic diseases, pancreaticoduodenectomy (PD) is the best choice when the tumor is localized in the head of the pancreas, or in the lower portion of the common bile duct. The purpose of the present study is to describe five consecutive children managed by PD, and reviewing the particular aspects and results of this rare procedure in children. METHODS: Between 2007 and 2010, five patients (median age: 7 years) underwent PD for nonmetastatic malignant tumors. In two cases, PD was performed en bloc with a right hepatectomy in order to achieve the radical resection of a recurrent biliary sarcoma. Four patients benefited from a "pylorus-preserving" PD procedure. In two patients, resection of the portal vein and vascular reconstruction was performed, and in one case, an extended resection of the biliary ductal system was necessary. RESULTS: All resection margins were clear. The postoperative course was uneventful, with no pancreatic or biliary leakage in all of the patients. Oral refeeding was achieved by the eighth postoperative day. In two cases, a late revision of pancreatic-jejunal anastomosis was performed because of mild steatorrea and a suspected anastomotic stricture. Two of the patients, who were subsequently operated on second hand, for biliary sarcoma, died from the recurrence; while three of the others, with pancreatic malignancies, are alive and well, with a good functional outcome. CONCLUSIONS: Surgical resection is the treatment of choice for tumors of the pancreatic head area. In the absence of regional or metastatic extension, the radicality of primary intervention is associated with favorable outcomes. Good functionality results were observed after the PD was limited to the head of the pancreas and subject to pylorus-preserving techniques.

Meso-Rex bypass as an alternative technique for portal vein reconstruction at or after liver transplantation in children: review and perspectives.

Direct portal revascularization can be achieved by interposing a vascular graft between the SMV and the Rex recessus (left portal vein system): the MRB. To review indications and results of the procedure in the setting of pediatric liver transplantation, reports were selected from the English literature. Previously reported series were updated to analyze long-term outcome. A new series was added and analyzed as a complementary set of cases. A total of 51 cases were analyzed. With a 96% overall patient survival rate and a 100% long-term patency rate when the IJV is used for the bypass, MRB achieves a very successful physiologic cure of chronic portal hypertension and restores the portal flow into and through the liver graft. It also has been used successfully for primary revascularization of liver grafts, as well as for managing early acute portal vein thrombosis episodes. The use of this procedure in conjunction with other strategies and techniques might be of interest for transplant surgeons, particularly those caring for children.

Transient elastography for assessment of fibrosis in paediatric liver disease.

The prognosis and management of chronic liver diseases in children largely depend on the extent and progression of liver fibrosis, which is often the most important predictor of disease outcome, and thus influences the indication for potential therapy. Unfortunately, liver biopsy continues to be the gold standard for the staging and grading of fibrosis. Liver biopsy is an invasive and painful technique with several limitations. These limitations have led to the development of alternative noninvasive methods for the accurate assessment of fibrosis and for the maintenance of an acceptable risk/benefit ratio. In the last decades, transient elastography (TE) has received increasing consideration as a means of evaluating disease progression in paediatric chronic liver disease. TE is an accurate and reproducible methodology for identifying subjects without fibrosis or significant fibrosis, or with advanced fibrosis. In this review, we provide an outline of liver fibrosis in paediatric liver diseases, including fibrogenesis, and noninvasive techniques for the diagnosis and follow-up of fibrosis, and then focus on the characteristics of TE and on its strength in the assessment of liver fibrosis, paying particular attention to studies conducted in children.

Unresectable multifocal omental and peritoneal inflammatory myofibroblastic tumor in a child: revisiting the role of adjuvant therapy.

Inflammatory myofibroblastic tumor is an uncommon lesion, also called pseudotumor, with a variable natural course from benign with spontaneous regression to mimicking malignant tumors. We report a case of diffuse peritoneal and omental pseudotumor in a 10-year-old boy characterized by aggressive behavior at the onset followed by stability after subtotal resection and chemotherapy. Total excision was not possible because of the tumor dissemination over the whole peritoneal surface. Adjuvant antiinflammatory drug (ketorolac tromethamine) and chemotherapy (methotrexate-vinblastine followed by ifosfamide-adriamycin and ifosfamide alone) were helpful to obtain rapidly complete resolution of clinical symptoms and anatomic stability of the residual lesions. Long-term evolution, in the absence of continued therapy, has been characterized by progressive involution and reduction of the residual masses.

DWI and MR-spectroscopy in the differential diagnosis of focal brain lesions.

The case of a patient with a previous history of cerebral infarction, shown to be positive for a brain neoformation on control CT, is presented. Subsequent MRI for an in-depth diagnostic study was completed with DWI and MR spectroscopy to define the nature of the lesion. The differential diagnosis of the lesion is discussed. In conclusion, the major diagnostic role of combined standard MRI with DWI sequences and MR-spectroscopy in the radiologic study of focal brain lesions, is stressed.