Thoracoscopic Resection of Lung Nodules following CT-Guided Labeling in Children and Adolescents with Solid Tumors.

Resection of lung metastases in children with solid tumors is regularly hampered by limited intraoperative detectability and relevant operative trauma of the open surgical access. The aim of this study was to analyze thoracoscopic resection of lung metastases in children following CT-guided labeling with coil wires. We retrospectively analyzed data of children and adolescents undergoing this approach at our institution between 2010 and 2022 with regard to technical aspects as well as surgical and oncological data. Within this period, we performed this procedure on 12 patients wherein we resected 18 lesions (1-5 per patient). The median age of patients was 178 months (51-265). The median duration of coil wire placement was 41 min (30-173) and the median surgery time was 53 min (11-157). No conversions were necessary and no intraoperative complications occurred. Complete microscopic resection (R0) was achieved in all labeled lesions and malignant tumor components were found in 5/12 patients. Our study shows that with a careful patient selection, thoracoscopic resection of lung metastases after coil wire labeling is a safe and reproducible procedure in children. Using this approach, lesions that are expected to have a reduced intraoperative detectability during open surgery become resectable. Patients benefit from the minimally invasive surgical access and reduced operative trauma.

Translational immunoPET imaging using a radiolabeled GD2-specific antibody in neuroblastoma.

Background: Antibodies targeting surface expressed disialoganglioside GD2 are increasingly used in neuroblastoma immunotherapy and might also have potential for use in radioimmunotherapy. As such targeted treatments might benefit from a dedicated theranostic approach, we studied the influence of radiolabeling on the binding characteristics of ch14.18 antibodies produced by Chinese hamster ovary (CHO) cells and evaluated the benefit of GD2-ImmunoPET as a potential tool for therapy planning. Methods:64Cu was used to reduce radiation burden, which is of high importance especially in a pediatric patient population. 64Cu-labeling was accomplished using the chelators NOTA- or DOTAGA-NCS. Radiolabeled antibodies were characterized in vitro. [64Cu]Cu-DOTAGA-ch14.18/CHO was studied in a neuroblastoma mouse model (subcutaneous CHP-134 xenografts). In vivo PET and MR images were acquired at 3 h, 24 h, and 48 h p.i. The specificity of binding was verified using GD2-negative tumors (HEK-293 xenografts), a control antibody and in vivo blocking. A first translational application was performed by PET/MRI in a patient with metastasized neuroblastoma. Results: Radiolabeling at an antibody-to-chelator ratio ≥1:10 yielded a product with a radiochemical purity of ≥90% and a specific activity of 0.2-1.0 MBq/µg. Radiochelation was stable over 48 h in PBS, mouse serum or EDTA, and 50.8 ± 3.5% and 50.8 ± 2.0% of the radiolabeled conjugates, prepared at antibody-to-chelator ratios of 1:10 or 1:15, were immunoreactive. In vivo, highly specific accumulation (31.6 ± 5.8% ID/g) in neuroblastoma was shown preclinically. Clinical PET/MR scans using [64Cu]Cu-NOTA-ch14.18/CHO (NOTA used for safety reasons) could visualize neuroblastoma metastases. Conclusions:In vivo,64Cu-labeled ch14.18/CHO is suitable for specific identification of neuroblastoma in PET. A first patient PET indicated the feasibility of the method for clinical translation and the potential utility in image-guided therapy.

Interventional closure of a bronchopleural fistula in a 2 year old child with detachable coils.

BACKGROUND: Bronchopleural fistula (BPF) is a severe complication following pneumonia or pulmonary surgery, resulting in persistent air leakage (PAL) and pneumothorax. Surgical options include resection, coverage of the fistula by video-assisted thoracoscopic surgery (VATS), or pleurodesis. Interventional bronchoscopy is preferred in complex cases and involves the use of sclerosants, sealants and occlusive valve devices. CASE PRESENTATION: A 2.5-year-old girl was admitted to our hospital with persistent fever, cough and dyspnoea. Clinical and radiological examination revealed right-sided pneumonia and pleural effusion. The child was started on antibiotics, and the effusion was drained by pleural drainage. Following removal of the chest tube, the child developed tension pneumothorax. Despite insertion of a new drain, the air leak persisted. Thoracoscopic debridement with placement of another new drain was performed after 4 weeks, without abolishment of the air leak. Bronchoscopy with bronchography revealed a BPF in right lung segment 3 (right upper-lobe anterior bronchus). We opted for an interventional approach that was performed under general anaesthesia during repeat bronchoscopy. Following bronchographic visualisation of the fistula, a 2.7 French microcatheter was placed in right lung segment 3 (upper lobe), allowing occlusion of the fistula by successive implantation of 4 detachable high-density packing volume coils, which were placed into the fistula. Subsequent bronchography revealed no evidence of residual leakage, and the chest tube was removed 2 days later. The chest X-ray findings normalized, and follow-up over 4 years was uneventful. CONCLUSIONS: Bronchoscopic superselective occlusion of BPF using detachable high-density packing large-volume coils was a successful minimally invasive therapeutic intervention performed with minimal trauma in this child and has not been reported thus far. In our small patient, the short interventional time, localized intervention and minimal damage in the lung seemed superior to the corresponding outcomes of surgical lobectomy or pleurodesis in a young growing lung, enabling normal development of the surrounding tissue. Follow-up over 4 years did not show any side effects and was uneventful, with normal lung-function test results to date.

Reduction of radiation exposure in scoliosis monitoring using flat detector and pulsed fluoroscopy technology.

A new flat detector and pulsed fluoroscopy technology is available to further reduce radiation exposure in radiological monitoring during scoliosis treatment in children and adolescents. The aim of this study is to compare different settings of the system (opening area(OA) and image quality settings (IQS)) in order to find the optimal parameters with high image quality and the lowest possible radiation exposure. Therefore, we examined four cadaver spines (T1 to sacrum) with the flat detector technique using digital pulsed fluoroscopy and simulated the abdominal soft tissues. The images were merged and evaluated by three different investigators using an established scoring system. For comparison, we used digital radiography images of the cadaver spines. The values for the DAP increased from the small OA (33% ; 0.56 µGy·m²) to the maximum OA (100% ; 0.82 µGy·m²) by 45% (p = .003) and from the low image quality setting (0.57 µGy·m²) to the high setting (0.84 µGy·m²) by 48% (p = .028). Despite the low DAP, the setting 33% OA achieved the best point values for image quality, therefore this setting is clearly preferred. Using a digital fluoroscopy system allows a significant reduction of radiation exposure by a factor of 7.5 (3.88µGy·m² to 0.5µGy·m²) compared to slot- scanning x-ray (EOS). Due to this success, the flat detector and pulsed fluoroscopy technology can be an alternative to established methods such as X-ray and EOS in clinical use.

123Iodine-metaiodobenzylguanidine scintigraphy versus whole-body magnetic resonance imaging with diffusion-weighted imaging in children with high-risk neuroblastoma - pilot study.

BACKGROUND: The prognostic value of the International Society of Paediatric Oncology European Neuroblastoma Research Network (SIOPEN) skeletal score using 123iodine-metaiodobenzylguanidine (MIBG) has been confirmed for people with high-risk neuroblastoma. Whole-body MRI with diffusion-weighted imaging is used increasingly. OBJECTIVE: To compare the original SIOPEN score and its adaption by diffusion-weighted imaging in high-risk stage 4 neuroblastoma and to evaluate any consequences of score differences on overall survival. MATERIALS AND METHODS: This retrospective observational study included pediatric patients who underwent MIBG scintigraphy and whole-body MRI, including diffusion-weighted imaging, between 2010 and 2015. Semi-quantitative skeletal scores for each exam were calculated independently. A difference of two or more points was defined as clinically relevant and counted as M+ (more in diffusion-weighted imaging) or S+ (more in MIBG). In cases of a negative result in one of the studies, residual disease of 1 point was also rated as relevant. We tested correlation and differences on an exam basis and also grouped by different therapeutic conditions. Overall survival was used to evaluate prognostic relevance. RESULTS: Seventeen children with 25 paired examinations were evaluated. Median MIBG scintigraphy score was 0 (interquartile range [IQR] 0-4, range 0-25) vs. a median whole-body MRI score of 1 (IQR 0-5.5, range 0-35) (P=0.018). A relevant difference between whole-body MRI and MIBG scintigraphy was noted in 14 of the 25 paired examinations (M+: n=9; S+: n=5). After treatment, the median survival of cases with M+ was 14 months (IQR 4-59, range 1-74 months), while S+ cases showed a median survival of 49 months (IQR 36-52, range 36-52 months) (P=0.413). CONCLUSION: The SIOPEN scoring system is feasible for whole-body MRI but might result in slightly higher scores, probably because of MRI's superior spatial resolution. Further studies are necessary to validate any impact on prognosis.

Retrospective accuracy analysis of MRI based lesion size measurement in neuroblastic tumors: which sequence should we choose?

BACKGROUND: MR imaging of neuroblastic tumors is widely used for assessing the effect of chemotherapy on tumor size. However, there are some concerns that MRI might falsely estimate lesion diameters due to calcification and fibrosis. Therefore, the aim of our study was to compare neuroblastic tumor size based on MRI measurements to histopathology measurements of the resected specimens as standard of reference. METHODS: Inclusion criteria were diagnosis of a neuroblastic tumor, MR imaging within 100 days to surgery and gross total resection without fragmentation of the tumor between 2008 and 2019. Lesion diameters were measured by two radiologists according to RECIST 1.1 in axial plane in T2w turbo spin echo (TSE), diffusion-weighted imaging (DWI), and in T1w pre- and postcontrast sequences. Furthermore, the largest lesion size in three-dimensions was noted. The largest diameter of histopathology measurements of each specimen was used for comparison with MRI. RESULTS: Thirty-seven patients (mean age: 5 ± 4 years) with 38 lesions (neuroblastoma: n = 17; ganglioneuroblastoma: n = 11; ganglioneuroma: n = 10) were included in this retrospective study. There was excellent intra-class correlation coefficient between both readers for all sequences (> 0.9) Tumor dimensions of reader 1 based on axial MRI measurements were significantly smaller with the following median differences (cm): T1w precontrast - 1.4 (interquartile range (IQR): 1.8), T1w postcontrast - 1.0 (IQR: 1.9), T2w TSE: -1.0 (IQR: 1.6), and DWI -1.3 (IQR: 2.2) (p < 0.001 for all sequences). However, the evaluation revealed no significant differences between the three-dimensional measurements and histopathology measurements of the resected specimens regardless of the applied MRI sequence. CONCLUSIONS: Axial MRI based lesion size measurements are significantly smaller than histopathological measurements. However, there was no significant difference between three-dimensional measurements and histopathology measurements of the resected specimens. T2w TSE and T1w postcontrast images provided the lowest deviation and might consequently be preferred for measurements.

Correction to: Allogeneic hematopoietic stem cell transplantation in two brothers with DNA ligase IV deficiency: a case report and review of the literature.

After publication of the original article (1), it was brought to our attention that references 24 and 31 are inappropriately cited in the article.

Allogeneic hematopoietic stem cell transplantation in two brothers with DNA ligase IV deficiency: a case report and review of the literature.

BACKGROUND: DNA ligase IV deficiency is a rare autosomal recessive disorder caused by hypomorphic mutations in the DNA ligase IV (LIG4) gene. DNA ligase IV is an essential protein for the development of a healthy immune system as well as for the protection of genomic integrity. Apart from typical stigmata, patients with DNA ligase IV deficiency are characterized by progressive bone marrow failure and a predisposition to malignancy. To our knowledge this reported case is the first description of two brothers with ligase IV deficiency who are treated with different hematopoietic stem cell transplantation (HSCT) regimens resulting in vastly divergent outcomes. CASE PRESENTATION: The cases of two brothers suffering from severe recurrent infections and growth retardation are described. The laboratory findings showed pancytopenia with significant lymphopenia. The two boys were diagnosed with DNA ligase IV deficiency, associated with severe combined immunodeficiency (SCID). Both patients received HSCT from two different matched unrelated donors (MUD) at the age of 33 and 18 months. The older brother succumbed post-transplant due to fatal side-effects 143 days after allogeneic HSCT. The younger brother - conditioned with a different regimen - received a T cell depleted graft 4 months later. No severe side-effects occurred, neither post-transplant nor in the following years. Ten years after HSCT the patient is well off, living a normal life and attending a regular high school. His immune system is fully reconstituted, resulting in a maximum of T cell receptor (TCR) diversity, which is a prerequisite for immune competence. However, he still suffers from microcephaly, dwarfism and dystrophy. CONCLUSIONS: This case report gives an example of a successful HSCT as a treatment option in a genetic disorder such as ligase IV deficiency, using a rather mild conditioning regimen. Further studies are required to determine the viability and efficacy of this treatment option.

Radiation Dose Optimization in Pediatric Chest CT: Major Indicators of Dose Exposure in 1695 CT Scans over Seven Years.

PURPOSE: To analyze possible influencing factors on radiation exposure in pediatric chest CT using different approaches for radiation dose optimization and to determine major indicators for dose development. MATERIALS AND METHODS: In this retrospective study at a clinic with maximum care facilities including pediatric radiology, 1695 chest CT examinations in 768 patients (median age: 10 years; range: 2 days to 17.9 years) were analyzed. Volume CT dose indices, effective dose, size-specific dose estimate, automatic dose modulation (AEC), and high-pitch protocols (pitch ≥ 3.0) were evaluated by univariate analysis. The image quality of low-dose examinations was compared to higher dose protocols by non-inferiority testing. RESULTS: Median dose-specific values annually decreased by an average of 12 %. High-pitch mode (n = 414) resulted in lower dose parameters (p < 0.001). In unenhanced CT, AEC delivered higher dose values compared to scans with fixed parameters (p < 0.001). In contrast-enhanced CT, the use of AEC yielded a significantly lower radiation dose only in patients older than 16 years (p = 0.04). In the age group 6 to 15 years, the values were higher (p < 0.001). The diagnostic image quality of low-dose scans was non-inferior to high-dose scans (2.18 vs. 2.14). CONCLUSION: Radiation dose of chest CT was reduced without loss of image quality in the last decade. High-pitch scanning was an independent factor in this context. Dose reduction by AEC was limited and only relevant for patients over 16 years. KEY POINTS: · The radiation dose of pediatric chest CT was reduced in the last decade.. · High-pitch scanning is an independent factor of dose optimization.. · Dose reduction by AEC is limited and only relevant for older children.. CITATION FORMAT: · Esser M, Hess S, Teufel M et al. Radiation Dose Optimization in Pediatric Chest CT: Major Indicators of Dose Exposure in 1695 CT Scans over Seven Years. Fortschr Röntgenstr 2018; 190: 1131 - 1140.

Fibrolipomatous hamartomas of the median nerve in infancy and early childhood-imaging hallmarks, symptomatology, and treatment.

A fibrolipomatous hamartoma (FLH) is a rare lesion leading to an enlargement of the affected nerve and commonly manifests at the median nerve. Symptomatic patients are mostly adolescents or adults. In children below 10 years, this entity is rather unknown and likely to be misdiagnosed. We report three children with FLH, two severely and one mildly symptomatic, all below 4 years of age at the time of first presentation. Two of three children were initially misdiagnosed. We provide a review of the pertinent clinical and radiological findings of the entity. Two patients had a characteristic macrodactyly. The two symptomatic children underwent surgical carpal tunnel decompression. The intervention relived their symptoms with a long-lasting effect. Surgical reduction of the hamartoma mass is not indicated and medical treatment non-existent. CONCLUSION: A symptomatic FLH of the median nerve is rare in children below the age of 5 years but has to be kept in mind as differential diagnosis in case of wrist and/or palm swelling, macrodactyly, and pain in hand or forearm. MRI is diagnostic, with very characteristic features, which can also be identified in high-resolution nerve ultrasound. This article aims to increase the knowledge about the entity including the diagnostic features and the management options. What is Known: • Fibrolipomatous hamartomas (FLHs) of the median nerve are rare, possibly associated with macrodactyly and tissue growth at the wrist and thenar side of the palm. • An associated carpal tunnel syndrome typically occurs, if at all, in adulthood. What is New: • We describe two children below 4 years with symptomatic carpal tunnel syndrome, experiencing a long-lasting favorable outcome after carpal tunnel decompression. In this age group, only one other child undergoing surgery has been published so far. • MRI and high-resolution ultrasound demonstrate the characteristic features of FLHs and are the diagnostic modalities of choice. Biopsy is not recommended.

A semiquantitative MRI-Score can predict loss of lung function in patients with cystic fibrosis: Preliminary results.

OBJECTIVES: To evaluate the applicability of a semiquantitative MRI scoring system (MR-CF-S) as a prognostic marker for clinical course of cystic fibrosis (CF) lung disease. METHODS: This observational study of a single-centre CF cohort included a group of 61 patients (mean age 12.9 ± 4.7 years) receiving morphological and functional pulmonary MRI, pulmonary function testing (PFT) and follow-up of 2 years. MRI was analysed by three raters using MR-CF-S. The inter-rater agreement, correlation of score categories with forced expiratory volume in 1 s (FEV1) at baseline, and the predictive value of clinical parameters, and score categories was assessed for the whole cohort and a subgroup of 40 patients with moderately impaired lung function. RESULTS: The inter-rater agreement of MR-CF-S was sufficient (mean intraclass correlation coefficient 0.92). MR-CF-S (-0.62; p < 0.05) and most of the categories significantly correlated with FEV1. Differences between patients with relevant loss of FEV1 (>3%/year) and normal course were only significant for MR-CF-S (p < 0.05) but not for clinical parameters. Centrilobular opacity (CO) was the most promising score category for prediction of a decline of FEV1 (area under curve: whole cohort 0.69; subgroup 0.86). CONCLUSIONS: MR-CF-S is promising to predict a loss of lung function. CO seems to be a particular finding in CF patients with an abnormal course. KEY POINTS: • Lung imaging is essential in the diagnostic work-up of CF patients • MRI serves as a powerful, radiation-free modality in paediatric CF patients • Observational single-centre study showed significant correlation of MR-CF score and FEV 1 • MR-CF score is promising in predicting a loss of lung function.

Computer-assisted surgery planning in children with complex liver tumors identifies variability of the classical Couinaud classification.

BACKGROUND: In complex malignant pediatric liver tumors there is an ongoing discussion regarding surgical strategy; for example, primary organ transplantation versus extended resection in hepatoblastoma involving 3 or 4 sectors of the liver. We evaluated the possible role of computer-assisted surgery planning in children with complex hepatic tumors. METHODS: Between May 2004 and March 2016, 24 Children with complex liver tumors underwent standard multislice helical CT scan or MRI scan at our institution. Imaging data were processed using the software assistant LiverAnalyzer (Fraunhofer Institute for Medical Image Computing MEVIS, Bremen, Germany). Results were provided as Portable Document Format (PDF) with embedded interactive 3-dimensional surface mesh models. RESULTS: Median age of patients was 33months. Diagnoses were hepatoblastoma (n=14), sarcoma (n=3), benign parenchyma alteration (n=2), as well as hepatocellular carcinoma, rhabdoid tumor, focal nodular hyperplasia, hemangioendothelioma, or multiple hepatic metastases of a pancreas carcinoma (each n=1). Volumetry of liver segments identified remarkable variations and substantial aberrances from the Couinaud classification. Computer-assisted surgery planning was used to determine surgical strategies in 20/24 children; this was especially relevant in tumors affecting 3 or 4 liver sectors. Primary liver transplantation could be avoided in 12 of 14 hepaoblastoma patients who theoretically were candidates for this approach. CONCLUSIONS: Computer-assisted surgery planning substantially contributed to the decision for surgical strategies in children with complex hepatic tumors. This tool possibly allows determination of specific surgical procedures such as extended surgical resection instead of primary transplantation in certain conditions.

Comparison between high-resolution CT and MRI using a very short echo time in patients with cystic fibrosis with extra focus on mosaic attenuation.

BACKGROUND: It would be beneficial to establish pulmonary MRI as a complementary approach to CT for direct visualization of mosaic perfusion, bullae, and emphysema in patients with cystic fibrosis. OBJECTIVES: The purpose of this study was to compare both modalities, CT and MRI, using the Helbich-Bhalla score with a special focus on reliable detection of a mosaic pattern. METHODS: Out of 51 patients examined by MRI on a 1.5-Tesla system during a period of 2 years, 19 patients were scheduled for additional low-dose CT in a clinical context. The MRI protocol comprised a gradient echo (GRE) sequence with a very short echo time (TE = 0.8 ms) in inspiration and expiration, a 3-D GRE sequence in breath hold, and a fast spin echo sequence with respiration and ECG triggering. MDCT was carried out in inspiration and adapted to body weight using 100 or 120 kV, 30-60 mA, 1- and 3-mm slice thicknesses, as well as low and high kernels. Additionally incremental slices in 3 positions were recorded in expiration for distinct detection of air trapping. CT and MRI analyses were performed by two radiologic readers in consensus unaware of the clinical parameters. The Helbich-Bhalla score of both examinations was correlated. Mean difference and accordance were assessed in each category. RESULTS: There was a strong correlation between CT and MRI (R = 0.87, p < 0.01). The mean Helbich-Bhalla score for CT was 12.2 (range 1-18) and for MRI it was 11.7 (range 2-19). The mean difference was 0.5 points. Besides this strong correlation for findings (bronchiectasis, mucus plugging, peribronchial thickening, and consolidation) with a prolonged T2 TE in MRI, we could also state a qualitative agreement of 95-100% in the categories with short T2 and low signal intensity in MRI as emphysema, bullae, and mosaic perfusion. CONCLUSIONS: These results suggest that in our patient group none of the relevant findings were missed by MR imaging and reading.

Percutaneous revascularization of reoccluded meso-Rex shunts in extrahepatic portal vein obstruction.

Extrahepatic portal vein (PV) obstruction (EHPVO) is a rare disorder in early childhood with unknown incidence and mostly unknown etiology. In children with EHPVO, the hepatopedal flow of the mesenteric venous blood is hindered by an obstruction of the PV resulting in prehepatic portal hypertension. The curative treatment with a meso-Rex shunt connects the superior mesenteric vein to the left PV by a venous autograft. Despite good primary patency, reocclusion of a meso-Rex bypass and its treatment can be challenging. We present 2 patients with EHPVO with subtotal functional occlusion of a meso-Rex shunt treated by percutaneous interventions with short- and mid-term follow-up.

Molecular imaging and photodynamic therapy in hepatoblastoma.

Molecular imaging is a novel field in cancer research combining various in vivo imaging modalities with molecular biology. Different techniques such as magnetic resonance tomography (MRI), positron emission tomography (PET), optical imaging methods (bioluminescence, fluorescence), or combination of these are used in basic research as well as in patients in different tumor entities. In hepatoblastoma (HB), there are only few reports on molecular imaging methods in a preclinical (optical imaging) and clinical setting (PET, PET-CT). Unimprovable treatment outcomes of patients in advanced tumor stages require novel treatment approaches. Photodynamic diagnosis (PDD) and photodynamic therapy (PDT) are novel diagnostic and therapeutic tools. Photodynamic diagnosis allows in vitro and in vivo detection of tumor cells using their fluorescending behaviour. PDT is a novel anticancer treatment approach leading to tumor cell destruction via apoptosis. In hepatoblastoma, there are only few reports on in vitro and in vivo studies using this treatment modality. First results seem to be promising and further studies will be required to further evaluate these techniques and to transfer them into clinical settings. This paper reviews different modalities of molecular imaging, photodynamic diagnosis and photodynamic therapy in childhood hepatoblastoma.

Single kidney and ureteral atresia in a newborn girl: a treatment concept.

OBJECTIVE: To demonstrate a rare case of urological pathology, we report a combination of a single kidney and ureteral atresia. The treatment concept and outcome are outlined. PATIENT AND METHOD: Antenatal ultrasound had revealed urinary ascites which lead to caesarean section in the 34th gestational week. Persisting anuria was confirmed postnatally and peritoneal dialysis started on the second day of life. Subsequent laparotomy revealed ureteral atresia after 3 cm of patent ureter. We created an ileum conduit after discussing various other therapeutic options. RESULT AND CONCLUSION: A follow up of 12 months has shown steady function of the stoma with stable renal parameters. An ileal conduit represents a good option if high drainage is necessary in early childhood.

Vascular encasement as element of risk stratification in abdominal neuroblastoma.

BACKGROUND: Vascular encasement of major vessels has been introduced as element of image defined risk factors (IDRF) for stratification of abdominal neuroblastoma. Some subgroups of this tumor entity are still subject of discussion regarding surgical approach and radicality. Aim of this study was to analyse a cohort of related patients. PATIENTS AND METHODS: Children operated on for neuroblastoma with encasement of major abdominal vessels (April 2002-April 2009) were retrospectively evaluated regarding surgical procedures, intra- and postoperative complications, and outcome. RESULTS: There were 18 patients with abdominal NB and encasement of major vessels. Mean age at operation was 43.5 months (2.5-113), mean operation time was 228 minutes (157-428). Complete macroscopic tumor resection was realised in 14 children. Vascular reconstruction was necessary in 5 patients. Tumor progression/relapses requiring further operation occurred in 3 patients. Major postoperative complications were 1 loss of unilateral renal function with subsequent nephrectomy, 1 renal vein thrombosis (operative revision), 1 renal artery embolism (operative revision), and 1 ureteral obstruction (stenting). Mean follow up was 34.8 months (2-78). CONCLUSIONS: Vascular encasement as part of IDRF is a valuable tool for stratification of abdominal NB. Surgery of NB with vascular encasement includes divers and complex procedures. Children seem to benefit from complete tumor resection or at least relevant tumor reduction although operations can mean a relevant strain for the patients.

Magnetic resonance nephrography for planning of laparoscopic partial nephrectomy in a pediatric case of ureteral triplication.

We present the case of ureteral triplication with vesicoureteral reflux into the lowest and middle pole in a 5-year-old girl. Magnetic resonance (MR) nephrography depicted loss of function of the lowest pole, which could not be assessed through MAG3 renal scan. Morphologic analyses revealed organ structure and vascular anatomy in superior quality. A laparoscopic partial nephroureterectomy of the lower pole was performed. Intraoperative findings correlated exactly with morphologic data obtained through MR nephrography. Dynamic MR nephrography should be considered as diagnostic tool of choice for selected kidney anomalies before surgery.

Laparoscopic Roux-en-Y hepaticojejunostomy in children with long common pancreaticobiliary channel: surgical technique and functional outcome.

BACKGROUND: In patients with maljunction of pancreatic and common bile duct (CBD)-defined as a long common pancreaticobiliary channel (LCPC)-a resection of the CBD and a hepaticojejunostomy is recommended. To date, this operation is usually performed through an open approach. In this article, we report on our experience with minimally invasive surgery (MIS) for LCPC in children. PATIENTS AND METHODS: From 2004 to 2008, 7 children underwent MIS for LCPC. Mean age at operation was 46 months. Two patients had a choledochal cyst (Todani type IV) additionally. Diagnosis was made preoperatively by magnetic resonance cholangiopancreaticography, in 5 children confirmed by endoscopic retrograde cholangio-pancreaticography. A four-trocar technique was used for the laparoscopic approach. Follow-up examinations included laboratory tests, ultrasound, and scintigraphy. RESULTS: A Roux-en-Y hepaticojejunostomy was performed in all patients-in 6 children completely by laparoscopy. In 1 child, the operation was converted to open after CBD diversion due to a large, vulnerable liver. In 2 children with extended choledochal cyst, additionally, a reconstruction of the separated hepatic ducts was performed. Reresection of a CBD stump was carried out by laparoscopy in another patient. A leakage of the anastomosis occurred in 1 child. CONCLUSIONS: The laparoscopic approach for pathology of pancreaticobiliary ducts might be a new alternative for surgical treatment in infants and children. It can also be performed in cases with choledochal cyst involving the hepatic ducts and for reresection of remnants of CBD.

Hirschsprung disease associated with total colonic agenesis and imperforate anus--case report and review of the literature.

We report a case of Hirschsprung disease associated with total colonic agenesis and high-type imperforate anus in a newborn girl. The patient also presented with uterus bicornis and a single kidney. The treatment procedure is presented together with a review of literature. The presented combination of pathologies has never been described before.