Dana Loana Dumitriu

Living Donor Liver Transplantation in Children: Surgical and Immunological Results in 250 Recipients at Université Catholique de Louvain.

Objectives: To evaluate the outcome of pediatric living donor liver transplantation (LDLT) regarding portal vein (PV) reconstruction, ABO compatibility, and impact of maternal donation on graft acceptance. Background: LDLT and ABO-mismatched transplantation constitute feasible options to alleviate organ shortage in children. Vascular complications of portal hypoplasia in biliary atresia (BA) and acute rejection (AR) are still major concerns in this field. Methods: Data from 250 pediatric LDLT recipients, performed at Cliniques Universitaires Saint-Luc between July 1993 and June 2012, were collected retrospectively. Results were analyzed according to ABO matching and PV complications. Uni- and multivariate analyses were performed to study the impact of immunosuppression, sex matching, and maternal donation on AR rate. Results: Overall, the 10-year patient survival rate was 93.2%. Neither patient or graft loss nor vascular rejection, nor hemolysis, was encountered in the ABO nonidentical patients (n = 58), provided pretransplant levels of relevant isoagglutinins were below 1/16. In BA recipients, the rate of PV complications was lower after portoplasty (4.6%) than after truncal PV anastomosis (9.8%) and to jump graft interposition (26.9%; P = 0.027). In parental donation, maternal grafts were associated with higher 1-year AR-free survival (55.2%) than paternal grafts (39.8%; P = 0.041), but only in BA patients. Conclusions: LDLT, including ABO-mismatched transplantation, constitutes a safe and efficient therapy for liver failure in children. In BA patients with PV hypoplasia, portoplasty seems to constitute the best technique for PV reconstruction. Maternal donation might be a protective factor for AR.

Performance of chest ultrasound in pediatric pneumonia

OBJECTIVE The objective of this study was to evaluate the performance of ultrasound in detecting lung consolidation in children suspected of pneumonia, in comparison to the current gold standard, chest X-rays. MATERIALS AND METHODS From September 2013 to June 2014, a monocentric prospective study was performed on all children between 0 and 16 years-old, referred for chest X-ray for suspected pneumonia. Each child was examined by chest ultrasound by an examiner blinded to the chest X-ray. The presence or absence of areas of consolidation, their number and location were noted for each technique. The size of the consolidations identified only on ultrasound was compared with that of consolidations visible on both techniques. RESULTS 143 children (mean age 3 years; limits between 8days and 14 years) were included. Ultrasound detected at least one area of consolidation in 44 out of 45 patients with positive X-rays. Of the 59 areas of consolidation on X-ray, ultrasound identified 54. In the 8 patients with negative X-ray, ultrasound revealed 17 areas of consolidation. The mean size of consolidations visible only on ultrasound was 9.4mm; for consolidations visible on both techniques the mean size was 26mm (p<0.0001). The sensitivity and specificity of ultrasound were calculated at 98% and 92%. PPV and NPV were 85% and 99%, respectively. CONCLUSION Chest ultrasound is a fast, non-ionizing and feasible technique. With its high negative predictive value, it can replace X-rays in order to exclude lung consolidation in children, thus reducing radiation exposure in this population.

Postendoscopic duodenal hematoma in children: Ultrasound diagnosis and follow‐up

Intramural duodenal hematomas have most frequently been reported in children in a traumatic setting. We present two cases of duodenal hematoma that occurred after upper gastrointestinal tract endoscopy with biopsy in children without significant prior medical history. The diagnosis was made by ultrasound, in correlation with the clinical presentation. Because the patients were hemodynamically stable, they were treated conservatively and the regression of the hematoma was followed up with ultrasound until its complete resolution. These cases demonstrate the risks of endoscopy, which are not to be neglected even in children without impaired coagulation, and the manner in which ultrasound can provide the correct diagnosis and follow‐up.

Reply to Dr. B. Karmazyn regarding ‘Duodenum between the aorta and the SMA does not exclude malrotation’

Sir, In response to Dr. Karmazyn’s letter [1], we would like to clarify the following: We disagree with the statement that the study evaluated patients with suspected malrotation, since this was not the case. The population, as stated in the materials and methods segment of the text, included asymptomatic patients explored for recurring respiratory symptoms [2]. Moreover, in all cases, US demonstrated the absence of whirlpool and the parallel position of the mesenteric vessels, excluding (unexpected) midgut volvulus. We agree with the title of Dr. Karmazyn’s letter, given that “the presence of the duodenum between the SMA and the aorta does not exclude malrotation,” complicated by midgut volvulus. If the duodenum twists around the axis of the mesenteric vessels, as it does in midgut volvulus, this clockwise rotation can position a portion of it behind the mesenteric vessels, as shown in Figs. 3 and 4 in his letter [1]. However, in these cases, the authors did not show parallel orientation of the superior mesenteric vessels. The embryology of intestinal rotation demonstrates the importance of the relationship between the duodenum and the mesenteric vessels. Upper gastrointestinal fluoroscopy defines the normal duodenal position in relation to bone structures. The advantage of US therefore resides in its ability to visualize the direct relationship between the mesenteric vessels and the duodenum and to identify those situations in particular in which the duodenum is not located in the aorto-mesenteric angle. In conclusion, retromesenteric D3 excludes uncomplicated malrotation, and parallel orientation of the mesenteric vessels excludes midgut volvulus. Only cross-sectional imaging as a whole and US in particular can achieve both objectives in the same setting and in the same study, not UGI.

Liver and systemic hemodynamics in children with cirrhosis: Impact on the surgical management in pediatric living donor liver transplantation.

Cirrhosis in adults is associated with modifications of systemic and liver hemodynamics, whereas little is known about the pediatric population. The aim of this work was to investigate whether alterations of hepatic and systemic hemodynamics were correlated with cirrhosis severity in children. The impact of hemodynamic findings on surgical management in pediatric living donor liver transplantation (LT) was evaluated. Liver and systemic hemodynamics were studied prospectively in 52 children (median age, 1 year; 33 with biliary atresia [BA]). The hemodynamics of native liver were studied preoperatively by Doppler ultrasound and intraoperatively using invasive flowmetry. Portosystemic gradient was invasively measured. Systemic hemodynamics were studied preoperatively by Doppler transthoracic echocardiography and intraoperatively by using transpulmonary thermodilution. Hemodynamic parameters were correlated with Pediatric End‐Stage Liver Disease (PELD) score and the histological degree of fibrosis (collagen proportionate area [CPA]). Cirrhosis was associated with a 60% reduction of pretransplant total liver flow (n = 46; median, 36 mL/minute/100 g of liver) compared with noncirrhotic livers (n = 6; median, 86 mL/minute/100 g; P = 0.002). Total blood flow into the native liver was negatively correlated with PELD (P < 0.001) and liver CPA (P = 0.005). Median portosystemic gradient was 14.5 mm Hg in children with cirrhosis and positively correlated with PELD (P < 0.001). Portal vein (PV) hypoplasia was observed mainly in children with BA (P = 0.02). Systemic hemodynamics were not altered in our children with cirrhosis. Twenty‐one children met the intraoperative criteria for PV reconstruction using a portoplasty technique during the LT procedure and had a smaller PV diameter at pretransplant Doppler ultrasound (median = 3.4 mm; P < 0.001). Cirrhosis in children appears also as a hemodynamic disease of the liver, correlated with cirrhosis severity. Surgical technique for PV reconstruction during LT was adapted accordingly. Liver Transplantation 23 1440–1450 2017 AASLD.

Ultrasound of the duodenum in children.

Ultrasound is well suited for examining the pediatric duodenum, given the small size of the patients, the lack of ionizing radiation and high-resolution imaging potential. Technical considerations, normal anatomy, congenital and acquired pathology of the duodenum, and the advantages and limitations of US are discussed and illustrated in this review.

Unusual association between lysinuric protein intolerance and moyamoya vasculopathy

INTRODUCTION Lysinuric protein intolerance (LPI) is a form of inherited aminoaciduria caused by a deficiency in the cationic amino acid transport process on the basolateral membrane of enterocytes and renal tubular cells. Clinical signs include gastrointestinal symptoms, failure to thrive, hepatosplenomegaly, osteoporosis, episodes of coma, intellectual deficiency, lung and renal involvement, bone marrow abnormalities, as well as altered immune response. Moyamoya disease is a cerebrovascular disorder predisposing sufferers to stroke through progressive stenosis of the intracranial internal carotid arteries and their proximal branches. Patients with characteristic moyamoya vasculopathy who also exhibit well-recognized associated conditions, such as Down syndrome or sickle-cell disease, are diagnosed with moyamoya syndrome, whereas those with no known associated risk factors are said to suffer from moyamoya disease. CASE STUDY A 5-year-old girl exhibiting aversion to protein-rich food and splenomegaly presented with a history of recurrent ischemic strokes. Cerebral angiography confirmed moyamoya vasculopathy. Metabolic investigation revealed abnormalities characteristic of LPI. This diagnosis was confirmed by the detection of a mutation within the SLC7A7 gene upon molecular investigation. CONCLUSION To the best of our knowledge, this is the first reported case of an association between moyamoya vasculopathy and LPI. While the question of association or coincidence cannot yet be answered, several pathophysiological consequences of LPI can be defined as separate, such as links between the impact of low arginine levels on the function of vascular endothelium and brain nitric oxide metabolism, as well as hemophagocytic syndrome associated with the risk of vasculitis, thus accounting for the development of moyamoya vasculopathy.

Pnematosis Intestinalis and Portal Venous Gas in Pediatric Liver Transplant Recipient.

FIGURE 1. Abdominal ultrasound, transverse scan of the epigastric region displaying the transplanted liver. A, Air bubbles are present in the portal vein (arrow), its branches, and diffusely throughout the liver parenchyma. B, Pulsed Doppler waveform of the portal flow FIGURE 2. Abdominal ultrasound, transverse scan of the left flank: pneumatosis intestinalis in the wall of the left colon (arrows); ( 1⁄4 air in the bowel lumen).

Pitfalls in the diagnosis of common benign bone tumours in children

AbstractBenign bone tumours in children are frequent lesions, often with a typical and very identifiable radiological presentation. However, their natural evolution and complications may be the source of variations and errors in interpretation. It is therefore important to understand the possible sources of change in the radiological aspect and to be familiar with common pseudotumoral lesions. The main aim of this review is to review typical aspects of the most common benign bone tumours in children, as well as less frequent variants of these tumours. Teaching points • Benign bone tumours in children may have atypical radiological presentations. • Some normal variants are commonly misinterpreted as tumours. • X-ray is the main imaging tool for focal bone lesions. • Depending on the X-ray, complementary imaging examinations and biopsy may be necessary.