Publications by authors named "Oliver Rompel"

30 Publications

  • Page 1 of 1

Prognostic Value of CTA-Derived Left Ventricular Mass in Neonates with Congenital Heart Disease.

Diagnostics (Basel) 2021 Jul 6;11(7). Epub 2021 Jul 6.

Department of Radiology, University Hospital Erlangen, Maximiliansplatz 3, 91054 Erlangen, Germany.

For therapeutic decisions regarding uni- or biventricular surgical repair in congenital heart disease (CHD), left ventricular mass (LVM) is an important factor. The aim of this retrospective study was to determine the LVM of infants with CHD in thoracic computed tomography angiographies (CTAs) and to evaluate its usefulness as a prognostic parameter, with special attention paid to hypoplastic left heart (HLH) patients. Manual segmentation of the left ventricular endo- and epicardial volumes was performed in CTAs of 132 infants. LVMs were determined from these volumes and normalized to body surface area. LVMs of patients with different types of CHD were compared to each other using analyses of variances (ANOVA). An LVM cutoff for discrimination between uni- and biventricular repair was determined using receiver operating characteristics. Survival rates were calculated using Kaplan-Meier statistics. Patients with a clinical diagnosis of an HLH had significantly lower mean LVM (21.88 g/m) compared to patients without applicable disease (50.22 g/m; < 0.0001) and compared to other CHDs, including persistent truncus arteriosus, left ventricular outflow tract obstruction, transposition of the great arteries, pulmonary artery stenosis or atresia, and double-outlet right ventricle (all, < 0.05). The LVM cutoff for uni- vs. biventricular surgery was 33.9 g/m (sensitivity: 82.3%; specificity: 73.7%; PPV: 94.9%). In a subanalysis of HLH patients, a sensitivity of 50.0%, specificity of 100%, PPV of 100%, and NPV of 83.3% was determined. Patient survival was not significantly different between the surgical approaches or between patients with LVM above or below the cutoff. LVM can be measured in chest CTA of newborns with CHD and can be used as a prognostic factor.
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http://dx.doi.org/10.3390/diagnostics11071215DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8303678PMC
July 2021

Blunt renal trauma-induced hypertension in pediatric patients: a single-center experience.

J Pediatr Urol 2021 Jun 30. Epub 2021 Jun 30.

Clinic of Urology and Pediatric Urology, University Hospital Erlangen, Germany. Electronic address:

Purpose: Children have a greater chance of sustaining a renal injury than adults and higher odds of having a high-grade renal injury. Hypertension is a rare complication of blunt renal trauma, with risk being higher in cases of major renal trauma. We reviewed the cases of pediatric blunt renal trauma-induced hypertension in our tertiary referral center in an attempt to better understand this rare condition.

Study Design: A retrospective evaluation of children under the age of 18 who were admitted to our department during the last 20 years and were diagnosed with blunt renal trauma.

Results: Twenty-three children presented with blunt renal trauma, one of whom was treated with emergency nephrectomy. Four children (18%) developed post-traumatic hypertension. All four cases were associated with a reduction in blood flow to the kidney, either through injury to the renal artery (in three cases) or through extrinsic compression of the kidney by a large perirenal hematoma (Page kidney; in one case). The Page kidney case developed hypertension during the initial hospitalization, and it resolved spontaneously after five months through the gradual resorption of the perirenal hematoma. Among the three cases of renal artery injury, hypertension during the initial hospitalization was only observed in one case, with hypertension in the other two cases manifesting after two months and four years, respectively. All three cases of renal artery injury resulted in a complete loss of function of the injured kidney, and two cases were treated with nephrectomy. Following nephrectomy, the blood pressure level returned to normal within a few days.

Discussion: Development of hypertension following a blunt renal trauma can be heterogenous, with the time of manifestation stretching between days after the accident and years thereafter. Children have a higher risk of renal trauma and, according to published data out of the National Trauma Data Bank, a 20-times higher risk of renal artery injury in comparison to the adult population. Large multicenter studies are required to answer the question of whether children are therefore more prone to blunt renal trauma-induced hypertension than adults.

Conclusions: Our study highlights the importance of blood pressure monitoring in children following blunt renal trauma, as post-traumatic hypertension can develop even years after the accident. In cases of a poorly functioning kidney, nephrectomy may be regarded as a curative therapy.
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http://dx.doi.org/10.1016/j.jpurol.2021.06.026DOI Listing
June 2021

Cinematic Rendering in Mixed-Reality Holograms: A New 3D Preoperative Planning Tool in Pediatric Heart Surgery.

Front Cardiovasc Med 2021 9;8:633611. Epub 2021 Feb 9.

Department of Pediatric Cardiology, University Hospital Erlangen, Friedrich-Alexander University Erlangen-Nürnberg (FAU), Erlangen, Germany.

Cinematic rendering (CR) is based on a new algorithm that creates a photo-realistic three-dimensional (3D) picture from cross-sectional images. Previous studies have shown its positive impact on preoperative planning. To date, CR presentation has only been possible on 2D screens which limited natural 3D perception. To depict CR-hearts spatially, we used mixed-reality technology and mapped corresponding hearts as holograms in 3D space. Our aim was to assess the benefits of CR-holograms in the preoperative planning of cardiac surgery. Including 3D prints allowed a direct comparison of two spatially resolved display methods. Twenty-six patients were recruited between February and September 2019. CT or MRI was used to visualize the patient's heart preoperatively. The surgeon was shown the anatomy in cross-sections on a 2D screen, followed by spatial representations as a 3D print and as a high-resolution hologram. The holographic representation was carried out using mixed-reality glasses (HoloLens®). To create the 3D prints, corresponding structures were segmented to create STL files which were printed out of resin. In 22 questions, divided in 5 categories (3D-imaging effect, representation of pathology, structure resolution, cost/benefit ratio, influence on surgery), the surgeons compared each spatial representation with the 2D method, using a five-level Likert scale. The surgical preparation time was assessed by comparing retrospectively matched patient pairs, using a paired -test. CR-holograms surpassed 2D-monitor imaging in all categories. CR-holograms were superior to 3D prints in all categories (mean Likert scale 4.4 ± 1.0 vs. 3.7 ± 1.3, < 0.05). Compared to 3D prints it especially improved the depth perception (4.7 ± 0.7 vs. 3.7 ± 1.2) and the representation of the pathology (4.4 ± 0.9 vs. 3.6 ± 1.2). 3D imaging reduced the intraoperative preparation time ( = 24, 59 ± 23 min vs. 73 ± 43 min, < 0.05). In conclusion, the combination of an extremely photo-realistic presentation via cinematic rendering and the spatial presentation in 3D space via mixed-reality technology allows a previously unattained level of comprehension of anatomy and pathology in preoperative planning.
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http://dx.doi.org/10.3389/fcvm.2021.633611DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7900175PMC
February 2021

Potential for Radiation Dose Reduction in Dual-Source Computed Tomography of the Lung in the Pediatric and Adolescent Population Compared to Digital Radiography.

Diagnostics (Basel) 2021 Feb 10;11(2). Epub 2021 Feb 10.

Department of Radiology, University Hospital Erlangen, 91054 Erlangen, Germany.

Low-dose dual-source computed tomography (DSCT) protocols for the evaluation of lung diseases in children and adolescents are of importance since this age group is particularly prone to radiation damage. The aim of this study was to evaluate image quality of low-dose DSCT of the lung and to assess the potential of radiation dose reduction compared to digital radiographs (DR). Three groups, each consisting of 19 patients, were examined with different DSCT protocols using tin prefiltration (Sn96/64/32 ref. mAs at 100 kV). Different strengths of iterative reconstruction were applied (ADMIRE 2/3/4). DSCT groups were compared to 19 matched patients examined with posterior-anterior DR. Diagnostic confidence, detectability of anatomical structures and small lung lesions were evaluated on a 4-point Likert scale (LS 1 = unacceptable, 4 = fully acceptable; a value ≥ 3 was considered acceptable). Effective dose (ED) was 31-/21-/9-fold higher in Sn96/Sn64/Sn32 compared to DR. Diagnostic confidence was sufficient in Sn96/Sn64 (LS 3.4/3.2), reduced in Sn32 (LS 2.7) and the worst in DR (LS 2.4). In DSCT, detectability of small anatomical structures was always superior to DR ( < 0.05). Mean lesion size ranged from 5.1-7 mm; detectability was acceptable in all DSCT groups (LS 3.0-3.4) and superior to DR (LS 1.9; < 0.05). Substantial dose lowering in DSCT of the pediatric lung enables acceptable detectability of small lung lesions with a radiation dose being about 10-fold higher compared to DR.
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http://dx.doi.org/10.3390/diagnostics11020270DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7916398PMC
February 2021

[Sudden Blindness after Enterocystoplasty – A Case Report].

Klin Padiatr 2021 03 4;233(2):88-90. Epub 2021 Jan 4.

Cnopfsche Kinderklinik, Department of Pediatric Surgery and Pediatric Urology, Nuremberg, Germany.

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http://dx.doi.org/10.1055/a-1301-1906DOI Listing
March 2021

Association of Lymphatic Abnormalities with Early Complications after Fontan Operation.

Thorac Cardiovasc Surg 2021 12 31;69(S 03):e1-e9. Epub 2020 Dec 31.

Department of Pediatric Cardiology, Friedrich-Alexander-Universitat Erlangen-Nürnberg, Erlangen, Germany.

Background: Increased central venous pressure is inherent in Fontan circulation but not strongly related to Fontan complication. Abnormalities of the lymphatic circulation may play a crucial role in early Fontan complications.

Methods: This was a retrospective, single-center study of patients undergoing Fontan operation from 2008 to 2015. The primary outcome was significant early Fontan complication defined as secondary in-hospital treatment due to peripheral edema, ascites, pleural effusions, protein-losing enteropathy, or plastic bronchitis. All patients received T2-weighted magnetic resonance images to assess abdominal and thoracic lymphatic perfusion pattern 6 months after Fontan completion with respect to localization, distribution, and extension of lymphatic perfusion pattern (type 1-4) and with application of an area score (0-12 points).

Results: Nine out of 42 patients developed early Fontan complication. Patients with complication had longer chest tube drainage (mean 28 [interquartile range [IQR]: 13-60] vs. 13 [IQR: 2-22] days,  = 0.01) and more often obstructions in the Fontan circuit 6 months after surgery (56 vs. 15%,  = 0.02). Twelve patients showed little or no abnormalities of lymphatic perfusion (lymphatic perfusion pattern type 1). Most frequently magnetic resonance imaging showed lymphatic congestion in the supraclavicular region (24/42 patients). Paramesenteric lymphatic congestion was observed in eight patients. Patients with early Fontan complications presented with higher lymphatic area score (6 [min-max: 2-10] vs. 2 [min-max: 0-8]),  = 0.001) and greater distribution and extension of thoracic lymphatic congestion (type 3-4:  = 5/9 vs.  = 1/33,  = 0.001).

Conclusion: Early Fontan complication is related to hemodynamic factors such as circuit obstruction and to the occurrence and extent of lymphatic congestion.
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http://dx.doi.org/10.1055/s-0040-1722178DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7909602PMC
December 2021

Bioabsorbable Unsintered Hydroxyapatite/Poly-l-Lactic Acid Pin Fixation of Osteochondritis Dissecans in Adolescents: Initial Experiences.

Z Orthop Unfall 2020 Nov 24. Epub 2020 Nov 24.

Department of Radiology, University Hospital Erlangen, Erlangen, Germany.

Background: This study sought to retrospectively evaluate the clinical and magnetic resonance imaging (MRI) outcomes of u-HA/PLLA pin (u-HA/PLLA: hydroxyapatite/poly-L-lactic acid) pin fixation of unstable osteochondritis dissecans (OCD) lesions of the knee.

Methods: Seven adolescent patients (four females and three males) with arthroscopically unstable OCD lesions of the knee were included. The mean age at diagnosis was 13.1 years. Clinical results were evaluated preoperatively and during follow-up using the Ogilvie-Harris score (0 - 15 points). MRI scans were performed preoperatively and during follow-up, with results evaluated using the Dipaola classification (grades 1 - 4). Mean follow-up time was 29 months.

Results: The median Ogilvie-Harris score improved from 13 points (range: 10 - 14 points) to 15 points (range: 13 - 15 points). Separately, the median Dipaola score improved from 3 points (range: 2 - 4 points) to 1 point (range: 1 - 4 points). No complications such as infection, synovitis, or intra-articular adhesion were observed.

Conclusions: Initial experiences using bioabsorbable u-HA/PLLA pins for the refixation of unstable OCD lesions in adolescents in the knee are promising, and MRI provides excellent monitoring of healing.
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http://dx.doi.org/10.1055/a-1289-0733DOI Listing
November 2020

Dual-source computed tomography of the lung with spectral shaping and advanced iterative reconstruction: potential for maximum radiation dose reduction.

Pediatr Radiol 2020 08 17;50(9):1240-1248. Epub 2020 Jun 17.

Department of Radiology, University Hospital Erlangen, Erlangen, Germany.

Background: Radiation dose at CT should be as low as possible without compromising diagnostic quality.

Objective: To assess the potential for maximum dose reduction of pediatric lung dual-source CT with spectral shaping and advanced iterative reconstruction (ADMIRE).

Materials And Methods: We retrospectively analyzed dual-source CT acquisitions in a full-dose group (FD: 100 kV, 64 reference mAs) and in three groups with spectral shaping and differing reference mAs values (Sn: 100 kV, 96/64/32 reference mAs), each group consisting of 16 patients (age mean 11.5 years, standard deviation 4.8 years, median 12.8 years, range 1.3-18 years). Advanced iterative reconstruction of images was performed with different strengths (FD: ADMIRE Level 2; Sn: ADMIRE Levels 2, 3 and 4). We analyzed dose parameters and measured noise. Diagnostic confidence and detectability of lung lesions as well as anatomical structures were assessed using a Likert scale (from 1 [unacceptable] to 4 [fully acceptable]).

Results: Compared to full dose, effective dose was reduced to 16.7% in the Sn 96 group, 11.1% in Sn64, and 5.5% in Sn32 (P<0.001). Noise values of Sn64 did not statistically differ from those in FD (45.7 vs. 38.9 Hounsfield units [HU]; P=0.132), whereas noise was significantly higher in Sn32 compared to Sn64 (61.5 HU; P<0.001). A Likert score >3 was reached in Sn64 regarding diagnostic confidence (3.2) and detectability of lung lesions (3.3). For detectability of most anatomical structures, no significant differences were found between FD and Sn64 (P≥0.05).

Conclusion: In pediatric lung dual-source CT, spectral shaping together with ADMIRE 4 enable radiation dose reduction to about 10% of a full-dose protocol while maintaining an acceptable diagnostic quality.
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http://dx.doi.org/10.1007/s00247-020-04714-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7398955PMC
August 2020

Small direct right ventricular cardiac metastasis of osteosarcoma in a 10-year-old boy affirmed by cardiac MRI.

Radiol Case Rep 2020 Jun 13;15(6):761-764. Epub 2020 Apr 13.

Department of Radiology, Friedrich-Alexander University Erlangen-Nürnberg, Erlangen, Germany.

Background: Metastatic osteosarcoma with direct cardiac involvement is an exceptionally rare finding. Reliable detection of cardiac metastases is known to be crucial for patients therapy and prognosis.

Case Summary: In a 10-year-old boy affected by osteosarcoma of the left femur, a baseline Fluorine-18-fluorodeoxy-glucose positron emission tomography/computed tomography (F-FDG PET/CT) was performed to assess the full extent of disease. Whole-body scan detected numerous bone metastases together with a single pulmonary metastasis. Moreover, increased tracer uptake was observed in the intracavitary right cardiac ventricle in the position of a subtle spot of calcification. Because of nondetectability of a cavitary lesion on echocardiography, cardiac magnetic resonance imaging (CMRI) examination was performed to evaluate cardiac F-FDG PET/CT finding. CMRI revealed a small nodule in the right ventricle attached to the trabeculae, highly suspicious of a direct cardiac metastasis. After 4 cycles of chemotherapy, complete regression of tracer uptake of the lesion was observed on a follow-up F-FDG PET/CT scan.

Conclusion: CMRI is able to detect even small, clinically asymptomatic cardiac metastases in young patients affected by osteosarcoma.
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http://dx.doi.org/10.1016/j.radcr.2020.03.019DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7160384PMC
June 2020

Myelin Oligodendrocyte Glycoprotein Antibody-Associated Neuromyelitis Optica Spectrum Disorder in a 6-Year-Old Boy.

Klin Padiatr 2019 07 7;231(4):220-223. Epub 2019 May 7.

Department of Pediatrics, University of Erlangen, Erlangen.

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http://dx.doi.org/10.1055/a-0860-5590DOI Listing
July 2019

Evaluation of ventricular septal defects using high pitch computed tomography angiography of the chest in children with complex congenital heart defects below one year of age.

J Cardiovasc Comput Tomogr 2019 Jul - Aug;13(4):226-233. Epub 2019 Feb 1.

University Hospital Erlangen, Department of Radiology, Maximiliansplatz 1, 91054, Erlangen, Germany; Imaging Science Institute Erlangen, Ulmenweg 18, 91054, Erlangen, Germany. Electronic address:

Background: Aim of this study was to assess the accuracy of ventricular septal defects (VSD) using high pitch computed tomography angiography (CTA) of the chest in children below 1 year of age, compared to the intraoperative findings and echocardiography.

Methods: Out of 154 patients that underwent Dual-Source CTA of the chest using a high-pitch protocol at low tube voltages (70-80 kV), 55 underwent surgical repair of a VSD (median age 8 days, range 1-348 days). The margins of the VSDs and their relation to the surrounding structures were reproduced by en-face views using multiplanar reformations (MPR). Absolute diameter, normalized area and relative area compared to the aortic valve annulus were used for discrimination between restrictive and non-restrictive defects. Localization was classified into four subtypes. The results were compared to two-dimensional echocardiography and intraoperative findings.

Results: Median absolute size of VSDs did not differ significantly between CTA-measurements (10.8 mm, range 2.8-18.1 mm) and intraoperative findings (12.0 mm, 3.0-25.0 mm, p = 0.09). Echocardiographic values were significantly lower (9.6 mm, 3.0-18.5 mm, both p < 0.01). The classification of the location and orientation matched the intraoperative situs in 96.4% of all cases using CT and in 87.3% using echocardiography. Echocardiography missed the relation to valves in 11% of all cases. Pre-interventional sensitivity and specificity for detection of a VSD were 97.2/98.9% compared to echocardiography. Median radiation dose was 0.32 mSv (range 0.12-2.00 mSv) and differed significantly between second and third generation Dual-Source CT (0.43 vs. 0.22 mSv, p = 0.003).

Conclusion: Size and subtype of VSDs can be accurately assessed by CTA of the chest in patients with complex congenital heart defects at a very low radiation dose.
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http://dx.doi.org/10.1016/j.jcct.2019.01.023DOI Listing
February 2020

Improved visualization of peripherally inserted central catheters on chest radiographs of neonates using fractional multiscale image processing.

BMC Med Imaging 2019 01 7;19(1). Epub 2019 Jan 7.

Department of Radiology, University Hospital Erlangen, Friedrich-Alexander-Universität (FAU) Erlangen-Nürnberg, Maximiliansplatz 3, 91054, Erlangen, Germany.

Background: Peripherally inserted central catheters (PICCs) provide secure intravenous access for the delivery of life-sustaining medications and nutrition. They are commonly used in pediatrics. Confirmation of correct central catheter tip position is crucial. Verification is usually done by a radiograph. The aim of this study is to evaluate the ability of Fractional Multiscale image Processing (FMP) to detect PICC tips on the digital chest radiographs of neonates.

Methods: A total of 94 radiographs of 47 patients were included in the study. 29 patients were male, 18 were female. The mean age of all examined children was 9.2 days (range 0-99 days). In total, six readers (two radiologists, two residents in radiology, one last year medical student, one neonatologist) evaluated 94 unprocessed and catheter-enhanced radiographs using a 5-point Likert scale (1 = poor catheter tip visualization, 5 = excellent catheter tip visualization). Additionally, the two radiologists evaluated the diagnostic confidence for chest pathologies using a 5-point Likert scale (1 = poor diagnostic confidence, 5 = excellent diagnostic confidence). Radiographs were evaluated on a dedicated workstation.

Results: In all cases, the catheter-enhanced radiograph rated higher than (n = 471), or equal (n = 93) to, the unprocessed radiograph when visualizing catheter tips. 87% of the catheter-enhanced radiographs obtained a rating of 4 or higher, while only 42% of unprocessed radiographs received 4 or more points. Regarding diagnostic confidence for chest pathologies one radiologist rated two catheter-enhanced radiographs higher than the unprocessed radiographs, while all other 186 evaluations rated the catheter-enhanced radiographs equal to (n = 78) or lower than (n = 108) the unprocessed radiographs. Only 60% of the catheter-enhanced radiographs yielded a diagnostic confidence of 4 or higher, while 90% of the unprocessed images received 4 or more points.

Conclusion: Catheter-enhanced digital chest radiographs demonstrate improved visualization of low contrast PICC tips in neonates compared to unprocessed radiographs. Furthermore, they enable detection of accompanying chest pathologies. However, definitive diagnosis of chest pathologies should be made on unprocessed radiographs.
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http://dx.doi.org/10.1186/s12880-018-0302-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6322299PMC
January 2019

Asymmetric Omphalopagus in a Triplet after In Vitro Fertilization: A Rare Case of Conjoined Twinning.

Case Rep Pediatr 2018 23;2018:9349606. Epub 2018 Aug 23.

Institute of Pathology, Faculty of Medicine, Friedrich-Alexander-University Erlangen-Nürnberg, Erlangen, Germany.

Introduction: Asymmetric omphalopagus is a rare situation of conjoined twinning, in which a grossly defective twin is attached to the thorax and upper abdomen of the main twin. We describe a case of an asymmetric omphalopagus accompanied by a normal triplet after assisted reproductive technology (ART) and tried to further characterize the all aspects of the conjoined twins. : Perioperative diagnostic imaging was carried out followed by an autopsy to evaluate all aspects of the parasite accompanied by histological, immunohistochemical, and molecular biological evaluation. The parasite had well-developed lower extremities as well as upper extremities with a cleft hand syndrome. The sex was nondeterminable, but DNA fingerprinting revealed that both parasite and autosite are monozygotic, so are females. There was no sign of any axial skeleton or central nervous system. We found a rudimentary rectum with a nonpervious anus, a kidney, ureter, urinary bladder, and a blind-ending urethra. The blood supply of the parasite was connected to the vessel system of the autosite.

Conclusions: To our knowledge, only two cases of parasitic omphalopagus after ART have been described to date. Altogether, 52 cases have been reported, and in most of them, the parasites were successfully separated.
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http://dx.doi.org/10.1155/2018/9349606DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6126085PMC
August 2018

Prenatal Correction of X-Linked Hypohidrotic Ectodermal Dysplasia.

N Engl J Med 2018 04;378(17):1604-1610

From the Departments of Pediatrics (H.S., I.K., S.W., A.D., M.W., W.R.), Obstetrics and Gynecology (F.F., M.W.B.), and Radiology (O.R.), University of Erlangen-Nürnberg, Erlangen, and Radiology Nienburg, Nienburg/Weser (C.T.) - both in Germany; the Department of Biochemistry, University of Lausanne, Epalinges, Switzerland (S.S.-M., C.K.-Q., M.V., P.S.); and Edimer Pharmaceuticals, Andover, MA (N.K.).

Genetic deficiency of ectodysplasin A (EDA) causes X-linked hypohidrotic ectodermal dysplasia (XLHED), in which the development of sweat glands is irreversibly impaired, an condition that can lead to life-threatening hyperthermia. We observed normal development of mouse fetuses with Eda mutations after they had been exposed in utero to a recombinant protein that includes the receptor-binding domain of EDA. We administered this protein intraamniotically to two affected human twins at gestational weeks 26 and 31 and to a single affected human fetus at gestational week 26; the infants, born in week 33 (twins) and week 39 (singleton), were able to sweat normally, and XLHED-related illness had not developed by 14 to 22 months of age. (Funded by Edimer Pharmaceuticals and others.).
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http://dx.doi.org/10.1056/NEJMoa1714322DOI Listing
April 2018

A new approach of three-dimensional guidance in paediatric cath lab: segmented and tessellated heart models for cardiovascular interventions in CHD.

Cardiol Young 2018 May 18;28(5):661-667. Epub 2018 Jan 18.

1Department of Pediatric Cardiology,University Hospital Erlangen,Friedrich-Alexander University Erlangen-Nuremberg,Erlangen,Germany.

Background: Optimal imaging is essential for catheter-based interventions in CHD. The three-dimensional models in volume-rendering technique currently in use are not standardised. This paper investigates the feasibility and impact of novel three-dimensional guidance with segmented and tessellated three-dimensional heart models in catheterisation of CHD. In addition, a nearly radiation-free two- to three-dimensional registration and a biplane overlay were used.Methods and resultsWe analysed 60 consecutive cases in which segmented tessellated three-dimensional heart models were merged with live fluoroscopy images and aligned using the tracheal bifurcation as a fiducial mark. The models were generated from previous MRI or CT by dedicated medical software. We chose the stereo-lithography format, as this promises advantage over volume-rendering-technique models regarding visualisation. Prospects, potential benefits, and accuracy of the two- to three-dimensional registration were rated separately by two paediatric interventionalists on a five-point Likert scale. Fluoroscopy time, radiation dose, and contrast dye consumption were evaluated. Over a 10-month study period, two- to three-dimensional image fusion was applied to 60 out of 354 cases. Of the 60 catheterisations, 73.3% were performed in the context of interventions. The accuracy of two- to three-dimensional registration was sufficient in all cases. Three-dimensional guidance was rated superior to conventional biplane imaging in all 60 cases. We registered significantly smaller amounts of used contrast dye (p<0.01), lower levels of radiation dose (p<0.02), and less fluoroscopy time (p<0.01) during interventions concerning the aortic arch compared with a control group.

Conclusions: Two- to three-dimensional image fusion can be applied successfully in most catheter-based interventions of CHD. Meshes in stereo-lithography format are accurate and base for standardised and reproducible three-dimensional models.
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http://dx.doi.org/10.1017/S1047951117002840DOI Listing
May 2018

Histopathological proof of the pathogenicity of a rare GFAP mutation in a patient with flaccid paraparesis.

Brain Dev 2018 Apr 27;40(4):330-333. Epub 2017 Nov 27.

Department of Pediatrics, Neuropediatrics, Friedrich-Alexander University of Erlangen-Nürnberg, Erlangen, Germany.

Infantile Alexander disease is a rare progressive leukodystrophy caused by autosomal dominant mutations in the (GFAP) gene typically presenting with psychomotor retardation, progressive macrocephaly and refractory epilepsy. Neuroradiological hallmarks are extensive white matter lesions with frontal preponderance as well as signal intensity changes of basal ganglia and medulla oblongata with variable contrast enhancement. Here, we report an atypical manifestation in a 21-month-old boy presenting with flaccid paraparesis and areflexia. Cognitive, visual as well as fine motor skills and muscular strength of the upper extremities were appropriate for age. Weight and height as well as head circumference were within normal range. Clinical or electroencephalographic signs of seizures were absent. Cranial MRI demonstrated bifrontal cystic tumorous lesions with partial contrast rims, as well as space-occupying focal lesions of the caudate nuclei. Spinal MRI revealed swelling of the lumbar and cervical spinal cord. CSF and blood chemistry showed normal results. Histopathology of a subcortical lesion showed large amounts of Rosenthal fibers and protein droplets characteristic of Alexander disease. Sequencing detected a heterozygous mutation of the GFAP gene (c.205G > A; p.(Glu69Lys)) that has been reported before as probably pathogenetic in another case of lower spinal involvement. This well documented case draws attention to atypical spinal manifestations of Alexander disease and gives histopathological proof of the pathogenetic role of a rare GFAP mutation with marked spinal involvement.
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http://dx.doi.org/10.1016/j.braindev.2017.11.005DOI Listing
April 2018

Compound heterozygous RYR1 mutations in a preterm with arthrogryposis multiplex congenita and prenatal CNS bleeding.

Neuromuscul Disord 2018 01 28;28(1):54-58. Epub 2017 Sep 28.

Department of Pediatrics, Neuropediatrics, Friedrich-Alexander-University of Erlangen-Nürnberg, Erlangen, Federal Republic of Germany.

RYR1 mutations, the most common cause of non-dystrophic neuromuscular disorders, are associated with the malignant hyperthermia susceptibility (MHS) trait as well as congenital myopathies with widely variable clinical and histopathological manifestations. Recently, bleeding anomalies have been reported in association with certain RYR1 mutations. Here we report a preterm infant born at 32 weeks gestation with arthrogryposis multiplex congenita due to compound heterozygous, previously MHS-associated RYR1 mutations, with additional signs of prenatal hemorrhage. The patient presented at birth with multiple joint contractures, scoliosis, severe thoracic rigidity and respiratory failure. He continued to depend on mechanical ventilation and tube feeding. Muscle histopathology showed a marked myopathic pattern with eccentric cores. Interestingly, the patient had additional unusual prenatal intraventricular hemorrhage, resulting in post-hemorrhagic hydrocephalus as well as epidural hemorrhage affecting the spinal cord. This report adds to the phenotypic variability associated with RYR1 mutations, and highlights possible bleeding complications in affected individuals.
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http://dx.doi.org/10.1016/j.nmd.2017.09.009DOI Listing
January 2018

Accuracy and Specific Value of Cardiovascular 3D-Models in Pediatric CT-Angiography.

Pediatr Cardiol 2017 Dec 31;38(8):1540-1547. Epub 2017 Jul 31.

Department of Pediatric Cardiology, University Hospital Erlangen, Friedrich-Alexander University Erlangen-Nuernberg, Loschgestrasse 15, 91054, Erlangen, Germany.

Computed tomography (CT)-angiography is routinely performed prior to catheter-based and surgical treatment in congenital heart disease. To date, little is known about the accuracy and advantage of different 3D-reconstructions in CT-data. Exact anatomical information is crucial. We analyzed 35 consecutive CT-angiographies of infants with congenital heart disease. All datasets are reconstructed three-dimensionally using volume rendering technique (VRT) and threshold-based segmentation (stereolithographic model, STL). Additionally, the two-dimensional maximum intensity projection (MIP) reconstructs two-dimensional data. In each dataset and resulting image, measurements of vascular diameters for four different vessels were estimated and compared to the reference standard, measured via multiplanar reformation (MPR). The resulting measurements obtained via the STL-images, MIP-images, and the VRT-images were compared with the reference standard. There was a significant difference (p < 0.05) between measurements. The mean difference was 0.0 for STL-images, -0.1 for MIP-images, and -0.3 for VRT-images. The range of the differences was -0.7 to 1.0 mm for STL-images, -0.6 to 0.5 mm for MIP-images and -1.1 to 0.7 mm for VRT-images. There was an excellent correlation between the STL-, MIP-, VRT-measurements, and the reference standard. Inter-reader reliability was excellent (p < 0.01). STL-models of cardiovascular structures are more accurate than the traditional VRT-models. Additionally, they can be standardized and are reproducible.
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http://dx.doi.org/10.1007/s00246-017-1693-7DOI Listing
December 2017

Diffuse Encephalopathy Associated with Isolated Cerebral Langerhans Cell Histiocytosis.

Pediatr Neurol 2016 09 11;62:62-5. Epub 2016 May 11.

Department of Pediatrics, Friedrich-Alexander University Erlangen-Nürnberg, Erlangen, Germany.

Background: Langerhans cell histiocytosis is a rare disease of the monocyte-macrophage system. Abnormalities of the hypothalamic-pituitary region are common in individuals with central nervous system involvement.

Patient Description: This six-year-old boy developed rapidly progressive aggressive behavior, central diabetes insipidus, and repeated complex partial seizures. Magnetic resonance imaging revealed a diffuse leukoencephalopathy-like pattern and numerous infratentorial and supratentorial granulomatous nodules in the brain parenchyma along with infundibular and hypothalamic mass lesions. Stereotactic serial biopsies enabled histopathologic and immunohistochemical diagnosis of Langerhans cell histiocytosis.

Conclusions: Similar MRI findings have rarely been described in the literature. These findings represent part of the broad neuroradiological spectrum of Langerhans cell histiocytosis of the nervous system in children.
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http://dx.doi.org/10.1016/j.pediatrneurol.2016.05.006DOI Listing
September 2016

Paediatric and adult soft tissue sarcomas with NTRK1 gene fusions: a subset of spindle cell sarcomas unified by a prominent myopericytic/haemangiopericytic pattern.

J Pathol 2016 Apr;238(5):700-10

Institute of Pathology, University Hospital, Friedrich-Alexander University Erlangen-Nuremberg, Erlangen, Germany.

Neoplasms with a myopericytomatous pattern represent a morphological spectrum of lesions encompassing myopericytoma of the skin and soft tissue, angioleiomyoma, myofibromatosis/infantile haemangiopericytoma and putative neoplasms reported as malignant myopericytoma. Lack of reproducible phenotypic and genetic features of malignant myopericytic neoplasms have prevented the establishment of myopericytic sarcoma as an acceptable diagnostic category. Following detection of a LMNA-NTRK1 gene fusion in an index case of paediatric haemangiopericytoma-like sarcoma by combined whole-genome and RNA sequencing, we identified three additional sarcomas harbouring NTRK1 gene fusions, termed 'spindle cell sarcoma, NOS with myo/haemangiopericytic growth pattern'. The patients were two children aged 11 months and 2 years and two adults aged 51 and 80 years. While the tumours of the adults were strikingly myopericytoma-like, but with clear-cut atypical features, the paediatric cases were more akin to infantile myofibromatosis/haemangiopericytoma. All cases contained numerous thick-walled dysplastic-like vessels with segmental or diffuse nodular myxohyaline myo-intimal proliferations of smooth muscle actin-positive cells, occasionally associated with thrombosis. Immunohistochemistry showed variable expression of smooth muscle actin and CD34, but other mesenchymal markers, including STAT6, were negative. This study showed a novel variant of myo/haemangiopericytic sarcoma with recurrent NTRK1 gene fusions. Given the recent introduction of a novel therapeutic approach targeting NTRK fusion-positive neoplasms, recognition of this rare but likely under-reported sarcoma variant is strongly encouraged.
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http://dx.doi.org/10.1002/path.4701DOI Listing
April 2016

Third-generation dual-source 70-kVp chest CT angiography with advanced iterative reconstruction in young children: image quality and radiation dose reduction.

Pediatr Radiol 2016 Apr 6;46(4):462-72. Epub 2016 Jan 6.

Department of Radiology, University Hospital Erlangen, Maximiliansplatz 1, 91054, Erlangen, Germany.

Background: Many technical updates have been made in multi-detector CT.

Objective: To evaluate image quality and radiation dose of high-pitch second- and third-generation dual-source chest CT angiography and to assess the effects of different levels of advanced modeled iterative reconstruction (ADMIRE) in newborns and children.

Materials And Methods: Chest CT angiography (70 kVp) was performed in 42 children (age 158 ± 267 days, range 1-1,194 days). We evaluated subjective and objective image quality, and radiation dose with filtered back projection (FBP) and different strength levels of ADMIRE. For comparison were 42 matched controls examined with a second-generation 128-slice dual-source CT-scanner (80 kVp).

Results: ADMIRE demonstrated improved objective and subjective image quality (P < .01). Mean signal/noise, contrast/noise and subjective image quality were 11.9, 10.0 and 1.9, respectively, for the 80 kVp mode and 11.2, 10.0 and 1.9 for the 70 kVp mode. With ADMIRE, the corresponding values for the 70 kVp mode were 13.7, 12.1 and 1.4 at strength level 2 and 17.6, 15.6 and 1.2 at strength level 4. Mean CTDIvol, DLP and effective dose were significantly lower with the 70-kVp mode (0.31 mGy, 5.33 mGy*cm, 0.36 mSv) compared to the 80-kVp mode (0.46 mGy, 9.17 mGy*cm, 0.62 mSv; P < .01).

Conclusion: The third-generation dual-source CT at 70 kVp provided good objective and subjective image quality at lower radiation exposure. ADMIRE improved objective and subjective image quality.
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http://dx.doi.org/10.1007/s00247-015-3510-xDOI Listing
April 2016

TALPID3 controls centrosome and cell polarity and the human ortholog KIAA0586 is mutated in Joubert syndrome (JBTS23).

Elife 2015 Sep 19;4. Epub 2015 Sep 19.

Institute of Human Genetics, University Hospital of Cologne, Cologne, Germany.

Joubert syndrome (JBTS) is a severe recessive neurodevelopmental ciliopathy which can affect several organ systems. Mutations in known JBTS genes account for approximately half of the cases. By homozygosity mapping and whole-exome sequencing, we identified a novel locus, JBTS23, with a homozygous splice site mutation in KIAA0586 (alias TALPID3), a known lethal ciliopathy locus in model organisms. Truncating KIAA0586 mutations were identified in two additional patients with JBTS. One mutation, c.428delG (p.Arg143Lysfs*4), is unexpectedly common in the general population and may be a major contributor to JBTS. We demonstrate KIAA0586 protein localization at the basal body in human and mouse photoreceptors, as is common for JBTS proteins, and also in pericentriolar locations. We show that loss of TALPID3 (KIAA0586) function in animal models causes abnormal tissue polarity, centrosome length and orientation, and centriolar satellites. We propose that JBTS and other ciliopathies may in part result from cell polarity defects.
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http://dx.doi.org/10.7554/eLife.08077DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4641851PMC
September 2015

Pediatric cardiac MRI: automated left-ventricular volumes and function analysis and effects of manual adjustments.

Pediatr Radiol 2015 Apr 19;45(5):651-7. Epub 2014 Nov 19.

Department of Radiology, University Hospital Erlangen, Maximiliansplatz 1, 91054, Erlangen, Germany,

Background: Cardiac MRI is an accurate and reproducible technique for the assessment of left ventricular volumes and function. The accuracy of automated segmentation and the effects of manual adjustments have not been determined in children.

Objective: To evaluate automated segmentation and the effects of manual adjustments for left ventricular parameter quantification in pediatric cardiac MR images.

Materials And Methods: Left ventricular parameters were evaluated in 45 children with suspected myocarditis (age 13.4 ± 3.5 years, range 4-17 years) who underwent cardiac MRI. Dedicated software was used to automatically segment and adjust the parameters. Results of end-diastolic volume, end-systolic volume, stroke volume, myocardial mass, and ejection fraction were documented before and after apex/base adjustment and after apex/base/myocardial contour adjustment.

Results: The software successfully detected the left ventricle in 42 of 45 (93.3%) children; failures occurred in the smallest and youngest children. Of those 42 children, automatically segmented end-diastolic volume (EDV) was 151 ± 47 ml, and after apex/base adjustment it was 146 ± 45 ml, after apex/base/myocardial contour adjustment 146 ± 45 ml. The corresponding results for end-systolic volume (ESV) were 66 ± 32 ml, 63 ± 29 ml and 64 ± 28 ml; for stroke volume (SV) they were 85 ± 25 ml, 83 ± 23 ml and 83 ± 23 ml; for ejection fracture (EF) they were 57 ± 10%, 58 ± 9% and 58 ± 9%, and for myocardial mass (MM) they were 104 ± 31 g, 95 ± 31 g and 94 ± 30 g. Statistically significant differences were found when comparing the EDV/ESV/MM results, the EF results after apex/base adjustment and after apex/base/myocardial contour adjustment and the SV results (except for comparing the SVs after apex/base adjustment and after apex/base/myocardial contour adjustment).

Conclusion: Automated segmentation for the evaluation of left ventricular parameters in pediatric MR images proved to be feasible. Automated segmentation + apex/base adjustment provided clinically acceptable parameters for the majority of cases.
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http://dx.doi.org/10.1007/s00247-014-3219-2DOI Listing
April 2015

Contrast medium application in pediatric high-pitch cardiovascular CT angiography: manual or power injection?

J Cardiovasc Comput Tomogr 2014 Jul-Aug;8(4):315-22. Epub 2014 May 22.

Department of Radiology, University of Erlangen-Nuremberg, Maximiliansplatz 1, Erlangen 91054, Germany.

Background: Dual-source CT offers accurate depiction of cardiac structures in children with congenital heart disease. For cardiac CT, optimal enhancement of the cardiovascular structures is essential. There is considerable controversy about the administration of contrast medium (CM) in infants and small children, with either a power injector or a manual (hand) injection.

Objective: The aim of this study was to compare image quality with power injection of CM (study group) and manual injection (control group).

Methods: Thirty-four patients (study group, 6.8 ± 9.6 months and control group, 4.6 ± 8.9 months, nonrandomized) underwent dual-source CT angiography of the chest using a prospective electrocardiography-triggered high-pitch spiral mode (pitch, 3.4; 80 kV). In the study group (17 patients), a power injector was used, and in the control group (17 patients, historical group), manual CM injection had been performed. To assess image quality, both subjective and objective parameters were evaluated independently by 2 experienced radiologists.

Results: Subjective overall image quality, signal-to-noise ratio, and contrast-to-noise ratio were significantly higher using power injection compared with manual injection (P < .05). However, depiction of cardiovascular structures did not differ significantly between both groups in all evaluated regions except the superior vena cava and the coronary arteries.

Conclusion: In infants and small children with congenital heart disease, both manual and power injector protocols allowed for diagnostic imaging of cardiac and extracardiac structures. However, image quality and vascular attenuation were superior using a power injector.
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http://dx.doi.org/10.1016/j.jcct.2014.05.002DOI Listing
May 2015

Preoperative assessment of the aortic arch in children younger than 1 year with congenital heart disease: utility of low-dose high-pitch dual-source computed tomography. A single-centre, retrospective analysis of 62 cases.

Eur J Cardiothorac Surg 2014 Jun 3;45(6):1060-5. Epub 2013 Dec 3.

Department of Radiology, University Hospital Erlangen, Erlangen, Germany.

Objectives: To evaluate the feasibility, image quality and impact of 3D imaging in low-dose high-pitch dual-source computed tomography (DSCT) to assess arbitrary anatomical malformations of the aortic arch in children <1 year of age with congenital heart disease (CHD).

Methods: Between January 2010 and May 2013, DSCT was performed to assess the aortic arch anatomy in a total of 62 consecutive patients with CHD (aged 0-348 days). DSCT was used whenever conventional echocardiography was not sufficient to display the complex anatomy entirely. Image data acquisition was realized within a single cardiac cycle using prospective ECG triggering. 3D reconstruction for surgical planning was performed. Image quality was assessed retrospectively, using a 4-point scale from '1 = no artefacts' to '4 = uninterpretable'. The accuracy and impact of the 3D reconstructions was compared with intraoperative findings using a 5-point scale (from '1 = essential' to '5 = misleading'). Administered radiation exposure was evaluated.

Results: Imaging was successful in all patients, image quality was rated 1.34 on the 4-point scale and the impact of the 3D reconstructions for surgical planning was 2.05 on the 5-point scale. Mean dose-length product was 6.8 ± 2.6 mGy cm, and the effective dose was 0.45 ± 0.13 mSv (0.21-0.74).

Conclusions: DSCT is a fast and appropriate imaging modality in the preoperative assessment of the aortic arch for surgical planning in CHD.
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http://dx.doi.org/10.1093/ejcts/ezt537DOI Listing
June 2014

Dual source computed tomography in patients with congenital heart disease.

Thorac Cardiovasc Surg 2014 Apr 29;62(3):203-10. Epub 2013 Aug 29.

Department of Pediatric Cardiology, University Hospital Erlangen, Erlangen, Germany.

Objectives: The objective of this study was to review our early experience with the dual source computed tomography (DSCT), a recently available scanner technique equipped with two X-ray tubes and two detectors, in the context of congenital cardiac malformations.

Patients And Methods: We reviewed 40 pediatric patients with congenital heart disease (CHD) who underwent DSCT between September 2009 and December 2011 as diagnostic imaging tool for surgical procedures.

Results: The median age was 0.36 years (range: 3 days to 44 years). Great vessels (n = 13), cardiac anatomy (n = 13), trachea and vascular rings (n = 7), pulmonary veins (n = 4), and coronary arteries (n = 3) were focused on, which revealed important information for surgery. Scanning quality was affected in only two cases (metal artifacts and tachycardia). Overall median age-specific dose was 1.47 mSv. In patients younger than 1 year (n = 26), median dose was 1.28 mSv.

Conclusion: DSCT allows a very rapid scan speed, examinations are performed in spontaneously breathing patients, and the radiation exposure is relatively low. It is very valuable in the setting of complex surgery by revealing the position of anatomical structures in their relation to each other. Missing information can be acquired less invasively in addition to echocardiography and might replace cardiac catheterization for several morphological indications.
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http://dx.doi.org/10.1055/s-0033-1349791DOI Listing
April 2014

A novel interstitial deletion of 10q24.2q24.32 in a patient with renal coloboma syndrome.

Eur J Med Genet 2012 Mar 31;55(3):211-5. Epub 2012 Jan 31.

Center for Human Genetics and Laboratory Medicine Dr. Klein and Dr. Rost, Martinsried, Germany.

Renal coloboma syndrome (RCS) is considered to be a rare autosomal dominant inherited disorder characterized by renal malformations and optic disc coloboma. Ocular anomalies range from asymptomatic abnormalities in retinal blood vessel patterning to large excavations of the optic nerve associated with reduced visual acuity. Commonly observed manifestations of the kidney are renal hypoplasia and vesicoureteric reflux leading to end-stage renal disease. Mutations in the PAX2 gene on chromosome 10 have been identified in patients with RCS. Up to date, nucleotide substitutions, insertions, small deletions, one de novo translocation, and one 240 kb deletion of the coding region of the PAX2 gene have been described to be responsible for RCS. We report here a new case of a patient with RCS due to a deletion of 3.8 Mb on chromosome 10q. Deletions on the long arm of chromosome 10 harboring the PAX2 gene seem to be a rare cause for RCS. Nevertheless, array-CGH testing should represent an important and valuable addition to PAX2 gene sequencing in diagnostic of RCS.
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http://dx.doi.org/10.1016/j.ejmg.2012.01.011DOI Listing
March 2012

Ultrasound of the bowel in children: how we do it.

Pediatr Radiol 2010 Apr;40(4):528-36

Department of Radiology, The Children's Hospital of Philadelphia, University of Pennsylvania, Philadelphia, PA, USA.

Advances in US like high-resolution transducers, harmonic imaging and panoramic modality have overcome some of the obstacles for sonography of the small and large bowel that existed in the past. In a number of centers, bowel US is an established routine method both in adults in children. Bowel US has the distinct advantages of being widely available, relatively cheap, free of radiation and easy to perform. In addition, US of the bowel demonstrates both mural and extramural pathological changes. Patients with inflammatory bowel disease have frequent imaging studies due to disease recurrences and need of follow-up after treatment. Thus this group of pediatric patients benefits most from an optimized and standardized bowel US. This review provides a comprehensive step-by-step approach how to perform US of the bowel in children with emphasis on imaging inflammatory bowel changes. It is meant to serve like a recipe and facilitate the routine performance of US of the bowel in children.
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http://dx.doi.org/10.1007/s00247-010-1550-9DOI Listing
April 2010

Harmonic US imaging of appendicitis in children.

Pediatr Radiol 2006 Dec 20;36(12):1257-64. Epub 2006 Sep 20.

Division of Paediatric Radiology, Institute of Diagnostic and Interventional Radiology, Klinikum Nürnberg Süd, Nuremberg, Germany.

Background: Harmonic imaging (HI), a relatively new ultrasound modality, was initially reported to be of use only in obese adult patients. HI increases the contrast and spatial resolution resulting in artefact-free images, and has been shown in adults to significantly improve abdominal sonography. Regarding its application in paediatric patients, just a handful reports exist and these do not encompass its use in intestinal sonography.

Objective: To compare the sonomorphological image quality of HI and fundamental imaging (FI, conventional grey-scale imaging) in the diagnosis of histologically confirmed appendicitis in children.

Materials And Methods: For this prospective comparative study, 50 children (male/female 25/25; mean age 9.9 years) suspected of having appendicitis were recruited. In all patients US examination of the appendix and periappendiceal region was performed preoperatively and appendectomy carried out. The final diagnosis was based on histological examination of the appendix. Both FI and HI were used in the US examination (tissue harmonic imaging, THI; Sonoline Elegra, Siemens; 7.5 MHz linear transducer). A detailed comparison of the images from FI and HI was performed using a scoring system. The parameters compared included delineation of the appendiceal contour, wall, mucosa, contents of the appendix and surrounding tissues. Furthermore, periappendiceal findings such as mesenteric echogenicity, free fluid, lymph nodes and adjacent bowel wall thickening were compared.

Results: In 43 children (86%) acute appendicitis was histologically confirmed. The inflamed appendix could be depicted in the HI and FI modes in 93% and 86%, respectively. HI was found to be significantly better for the depiction of the outer contour, wall, mucosa and contents of the appendix (P<0.01). This was also true for the demonstration of free fluid, mesenteric lymph nodes, adjacent bowel walls and mesenteric echogenicity.

Conclusion: HI should be the preferred modality for scanning the right lower abdomen in suspected acute appendicitis. The diagnosis of acute appendicitis can then be more definitely ascertained.
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http://dx.doi.org/10.1007/s00247-006-0313-0DOI Listing
December 2006

Aortocaval fistula after stent-graft repair.

J Endovasc Ther 2006 Feb;13(1):117-20

Department of Vascular and Endovascular Surgery, Klinikum Nürnberg, Germany.

Purpose: To report an aortocaval fistula after stent-graft repair and the feasibility of interventional treatment.

Case Report: A 78-year-old man with a 61-mm infrarenal aortic aneurysm (AA) was treated successfully with a Zenith bifurcated stent-graft. Three years later, the patient presented with deteriorating renal function and acute bronchial obstruction. Computed tomography showed an aortic diameter increased to 90 mm, dilatation of the inferior vena cava, and a distal type I endoleak. The patient's condition quickly deteriorated, and emergent imaging found a fistula with brisk flow between the aneurysm sac and the left iliac vein within a distal type I endoleak. During emergency endovascular repair, iliac extensions were implanted in the right common iliac artery and left external iliac artery. The left hypogastric artery was coil embolized to exclude flow into the aneurysm sac. After positioning the extensions, cardiac function improved, and the fistula was no longer palpable. The cardiac indices and renal function normalized, and he was discharged 20 days after admission.

Conclusion: Aortocaval fistulas are a rare complication of AA stent-graft repair and may be successfully treated by interventional means.
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http://dx.doi.org/10.1583/05-1558MR.1DOI Listing
February 2006
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