Stereotactic radiofrequency ablation of a variety of liver masses in children.

BACKGROUND AND AIMS: Surgical resection is currently the cornerstone of liver tumor treatment in children. In adults radiofrequency ablation (RFA) is an established minimally invasive treatment option for small focal liver tumors. Multiprobe stereotactic RFA (SRFA) with intraoperative image fusion to confirm ablation margins allows treatment for large lesions. We describe our experience with SRFA in children with liver masses. METHODS: SRFA was performed in 10 patients with a median age of 14 years (range 0.5-17.0 years) suffering from liver adenoma (n = 3), hepatocellular carcinoma (n = 1), hepatoblastoma (n = 2), myofibroblastic tumor (n = 1), hepatic metastases of extrahepatic tumors (n = 2) and infiltrative hepatic cysts associated with alveolar echinococcosis (n = 1). Overall, 15 lesions with a mean lesion size of 2.6 cm (range 0.7-9.5 cm) were treated in 11 sessions. RESULTS: The technical success rate was 100%, as was the survival rate. No transient adverse effects higher than grade II (Clavien and Dindo) were encountered after interventions. The median hospital stay was 5 d (range 2-33 d). In two patients who subsequently underwent transplant hepatectomy complete ablation was histologically confirmed. Follow-up imaging studies (median 55 months, range 18-129 months) revealed no local or distant recurrence of disease in any patient. CONCLUSIONS: SRFA is an effective minimal-invasive treatment option in pediatric patients with liver tumors of different etiologies.

Needle detachment in a slim and physically active child with insulin pump treatment.

Insulin pump therapy (CSII) is well established in pediatric patients with type 1 diabetes. In childhood diabetes, insulin pump treatment shows considerable advantages such as fewer injections, increased flexibility, fewer hypoglycemic events and lower HbA1c levels. Side effects such as catheter obstruction, technical pump failure, and dermatological complications have been observed, but are rarely reported. The reported patient is a physically very active and slim 10-year-old boy with reduced subcutaneous fatty tissue. After strong muscular activity an accidental rupture of the infusion set and needle detachment occurred in October 2013. X-ray and ultrasound imaging localized the needle in the musculus rectus femoris dexter. The needle was kept in situ and oral antibiotic treatment to prevent inflammatory reaction was prescribed. Repeated ultrasound measurements documented that the needles position had remained unchanged. Steel needle catheters (Sure-T infusion set, 6 mm) positioned in a thin layer of subcutaneous fat tissue of the thigh, combined with intense sports activity can result in a needle rupture and penetration into the muscle. Careful monitoring provides an alternative to surgery and lowers the risk of muscular necrosis. Because of differences in the distribution of subcutaneous fat tissue, an individualized catheter selection is necessary in pump treatment for children and adolescents, requiring a variety of different catheter sets.

Efforts of the human immune system to maintain the peripheral CD8+ T cell compartment after childhood thymectomy.

BACKGROUND: Homeostatic mechanisms to maintain the T cell compartment diversity indicate an ongoing process of thymic activity and peripheral T cell renewal during human life. These processes are expected to be accelerated after childhood thymectomy and by the influence of cytomegalovirus (CMV) inducing a prematurely aged immune system. The study aimed to investigate proportional changes and replicative history of CD8+ T cells, of recent thymic emigrants (RTEs) and CD103+ T cells (mostly gut-experienced) and the role of Interleukin-(IL)-7 and IL-7 receptor (CD127)-expressing T cells in thymectomized patients compared to young and old healthy controls. RESULTS: Decreased proportions of naive and CD31 + CD8+ T cells were demonstrated after thymectomy, with higher proliferative activity of CD127-expressing T cells and significantly shorter relative telomere lengths (RTLs) and lower T cell receptor excision circles (TRECs). Increased circulating CD103+ T cells and a skewed T cell receptor (TCR) repertoire were found after thymectomy similar to elderly persons. Naive T cells were influenced by age at thymectomy and further decreased by CMV. CONCLUSIONS: After childhood thymectomy, the immune system demonstrated constant efforts of the peripheral CD8+ T cell compartment to maintain homeostasis. Supposedly it tries to fill the void of RTEs by peripheral T cell proliferation, by at least partly IL-7-mediated mechanisms and by proportional increase of circulating CD103+ T cells, reminiscent of immune aging in elderly. Although other findings were less significant compared to healthy elderly, early thymectomy demonstrated immunological alterations of CD8+ T cells which mimic features of premature immunosenescence in humans.

Tracking Lung Clearance Index and chest CT in mild cystic fibrosis lung disease over a period of three years.

INTRODUCTION: Lung disease remains the main cause of morbidity and mortality in patients with Cystic Fibrosis (CF). To detect lung disease before clinical symptoms become apparent, sensitive tools are essential. Spirometry is used for monitoring, but the FEV1 remains frequently normal throughout childhood. The Lung Clearance Index (LCI) calculated from Multiple Breath Washout (MBW) was introduced at the CF centre Innsbruck in 2007 for assessing ventilation inhomogeneity in patients with mild lung disease. We hypothesized that LCIs in 2007 are of prognostic value for the presence or absence of structural lung changes in later years. METHODS: Between 2007 and 2010 MBW, spirometry and ultra-low-dose HR-CT were prospectively tracked in 36 patients (6-53 years) with a mean FEV1 ≥ 80% predicted in 2007. RESULTS: At study start the majority of patients had abnormal CT scores and LCI results. While CT and spirometry remained largely stable throughout the study, LCI results slightly improved but still correlated with CT scores in 2010. LCI results in 2007 correlated with CT scores in 2010 while FEV1 did not. In 86% the LCI value in 2007 was indicative for the presence or absence of structural lung changes in 2010. CONCLUSION: The LCI is a sensitive tool for detecting and tracking pulmonary changes. Extended structural changes are unlikely if the LCI is normal. The LCI has the potential to be used for monitoring the progression of early CF lung disease and assessing the effect of treatment in both clinical care and research settings.

Sensitivity of Lung Clearance Index and chest computed tomography in early CF lung disease.

It is widely accepted that CF lung disease starts before clinical symptoms become apparent or spirometry deteriorates. Computed chest tomography (CT) is the reference method for identifying structural changes in CF; however, radiation exposure limits its use as a monitoring tool. It has been suggested that the Lung Clearance Index (LCI) measured by Multiple Breath Washout (MBW) for assessing ventilation inhomogeneity is a more sensitive surrogate marker than spirometry allowing non-invasive monitoring of CF lung disease. The aim of this study was to prospectively investigate the diagnostic accuracy of the LCI in comparison to CT in CF patients with early lung disease and normal FEV(1) (>80% pred.). MBW and ultra-low-dose CT were performed in 34 patients (6-26 years). LCI was abnormal in 76.5% subjects. LCI and CT correlated significantly in 82.3%. LCI was related to presence and extent of structural lung changes observed on CT with a sensitivity of 88%. Diagnostic accuracy of the LCI for detecting CF lung disease in patients with normal FEV(1) was good when compared to CT. Results indicate that structural changes are unlikely if a normal LCI is measured. We speculate that serial measurements of the LCI for assessing ventilation inhomogeneity may help to identify early structural lung disease and help to reduce the individual cumulative radiation dose. The LCI may be a suitable surrogate marker for monitoring progression of CF lung disease and effect of treatment in both, clinical care and research settings.

Segmental bowel-wall thickening on abdominal ultrasonography: an additional diagnostic sign in Kawasaki disease.

Abdominal complaints are a frequent early feature of Kawasaki disease, an acute systemic febrile vasculitis of childhood. The only well known correlating morphological finding on abdominal US is gallbladder hydrops. We report two boys with Kawasaki disease who showed segmental thickening of the small-bowel wall on abdominal US. These changes are thought to result from bowel-wall oedema due to vasculitis of the supplying vessels. We conclude that in an acutely ill febrile child with the finding of segmental bowel-wall thickening on abdominal US, Kawasaki disease should be considered in the differential diagnosis. The search for gallbladder hydrops and segmental bowel-wall thickening on abdominal US may serve as an important diagnostic tool in Kawasaki disease, supporting early recognition and prompt treatment.

Fluid-debris level in follicular cysts: a pathognomonic sign of ovarian torsion.

BACKGROUND: Early diagnosis of ovarian torsion is mandatory to avoid common complications. Sonography is the imaging modality of choice, but diagnosis is still a challenge. OBJECTIVE: To report an additional sonographic feature of ovarian torsion in order to improve diagnostic accuracy. MATERIALS AND METHODS: Grey-scale and colour Doppler pelvic sonography was performed in 12 patients with acute onset of intermittent lower abdominal pain and in 1 asymptomatic patient with a palpable mass in the labium majus. Patient ages ranged from 4 months to 17 years. RESULTS: In all patients an enlarged ovary with peripheral cysts was demonstrated. High-resolution sonography detected fluid-debris levels within these cystic structures in 11 of the 13 patients (sensitivity 85%) and provided an additional diagnostic sign. Six patients had cystic teratoma. CONCLUSIONS: We confirm the fluid-debris level as a pathognomonic sonographic sign of ovarian torsion with or without associated teratoma.

Dural ectasia in children with Marfan syndrome: a prospective, multicenter, patient-control study.

UNLABELLED: The clinical diagnosis of Marfan syndrome in childhood is difficult, because symptoms may not have developed to their full expression until adulthood. The Ghent nosology for the diagnosis of Marfan syndrome classifies dural ectasia as a major diagnostic criterion. More than two thirds of adult patients with Marfan syndrome show dural ectasia, while the frequency in childhood is unknown. This prospective multicenter observational patient-control study was performed to identify pathologic changes of the lumbosacral spine in young patients with Marfan syndrome. DESIGN: Prospective clinical trial, multicentric, cross-sectional. SETTING: MRI of the lumbosacral spine. PATIENTS: Twenty patients with proven Marfan syndrome, 20 patients suspicious for Marfan syndrome and 38 healthy controls. OUTCOME MEASURES: Vertebral body diameter (VBD) from L1 to S1, dural sac diameter (DSD) from L1 to S1, dural sac ratio (DSR), qualitative assessment of the lumbosacral spine. RESULTS: DSD and VBD in different age groups were higher in patients with proven or suspected Marfan syndrome than in healthy controls (DSD: L1, 6-8 years, P < 0.05). VBD related to body height showed a similar growth related increase in patients with proven or suspected Marfan syndrome and controls. DSD related to body height was elevated in patients with proven or suspected Marfan syndrome at different levels of the lumbar spine. DSD at levels L1, L5, and S1, and DSR at levels L5 and S1 of patients with proven Marfan syndrome were significantly higher (P < 0.05) than in controls. CONCLUSION: Even during childhood pathologic changes inside the lumbosacral spine of patients with Marfan syndrome can be observed. Dural ectasia, which occurs at different levels of the lumbar spine, can be detected at levels L5 and S1 in up to 40% of patients with Marfan syndrome.

Cerebrospinal fluid leakage after lumbar puncture in neonates: incidence and sonographic appearance.

OBJECTIVE: The purpose of this article is to review the incidence and sonographic appearance of cerebrospinal fluid leakage after lumbar puncture in the neonatal period. Thirty-three neonates underwent spinal sonography after diagnostic lumbar puncture. A total of 21 of these patients showed cerebrospinal fluid leakage into the epidural space extending from the level of the cauda equina to the lumbar (n = 9), the thoracic (n = 8), or the cervical (n = 4) region. In eight patients, the subarachnoid space was markedly compressed by the epidural fluid collection. CONCLUSION: Cerebrospinal fluid leakage into the epidural space is a frequent complication of lumbar puncture in neonates and has a characteristic appearance on sonograms. Leakage after lumbar puncture must be differentiated from cerebrospinal fluid leakage due to perinatal meningeal injury. If cerebrospinal fluid leakage at the puncture site compresses the subarachnoid space, sonography assists in the performance of subsequent lumbar puncture.