Search

Testicular torsion is a surgical emergency. Early diagnosis and surgical treatment are vital in order to preserve the affected gonad. Current surgical teaching emphasizes sudden, severe, persistent, unilateral scrotal pain as a cardinal symptom of testicular torsion. We present the case of unilateral testicular torsion in a 14-year-old patient who presented with the absence of severe pain. Despite a delayed presentation to the emergency department, the gonad could be salvaged successfully. Literature on the topic of testicular torsion presenting with minimal pain is limited. Nevertheless, pediatric surgeons might be faced with cases similar to the one we describe. Underestimating this phenomenon might lead to a delay of treatment. In such cases, ultrasound can be a beneficial addition in the diagnosis and accelerate definitive operative treatment. The presented case clearly demonstrates that, although we do not include testicular torsion without severe pain in our surgical teaching algorithms, we might encounter it in our clinical practice.

Sialoblastoma is a rare congenital malignant tumor of the salivary glands. A case of a submandibular sialoblastoma in a 1.5-year-old child is presented. A comparative analysis on 79 pediatric cases reported in the literature suggests a less aggressive behavior for submandibular sialoblastoma in comparison with other sites. Classically, diagnosis is confirmed by open biopsy, but fine-needle aspiration may offer an alternative with reduced morbidity. Expression of AFP and high levels of Ki-67 have been associated with poor prognosis. Whilst early surgical resection with negative margins is widely accepted as first-line treatment, there is no consensus on therapy of recurrence and follow-up. MRI and sonography represent valid tools for the follow-up, which is usually restricted to 3-5 years.Conclusion: Submandibular sialoblastomas may have a different biological profile in comparison with parotid tumors with the absence of metastasis and much lower rate of recurrence. Comprehensive diagnostics should include additional options such as fine-needle aspiration and markers to assess cell proliferation and AFP. Literature suggests that surgery alone is sufficient for the treatment of tumors with low malignancy. Follow-up should be tailored according to the tumor site and might be limited to 3-5 years. What is Known: • Sialoblastoma is a rare congenital malignant tumor with an unpredictable clinical outcome. What is New: • Sialoblastoma of submandibular origin seems to have a less aggressive behavior in comparison with other sites. • Fine-needle aspiration and markers to assess proliferation index (i.e., suggestive of potential more aggressive course/malignancy) should be strongly considered in the diagnostic work-up. • Radical surgery as first-line therapy and a 3-5-year follow-up are acceptable for tumors with a low malignancy.

Given the complexity of care for children with a CNS tumor a multidisciplinary team approach seems to be the gold standard. Since January 2017 the pediatric brain and spine tumor center has been established, compromised of a multidisciplinary team including neuropediatrics, pediatric neurosurgery, pediatric radiology, radiation oncology, pediatric endocrinology, physio - and occupational therapy, social work and nursing under the leadership of the pediatric neuro-oncologist. All newly diagnosed pediatric CNS tumors as well as all follow up care independent of the treatment received are done for by this team. The multidisciplinary team approach also includes a preoperative discussion and planning for each individual patient as well as a jointly disclosure. A multidisciplinary follow up outpatient clinic takes place twice a month with a tumor board prior to the clinic to discuss all the patients and their different issues. All patients are seen by the needed disciplines. In total 62 patients were seen over a period of 12 months in this clinic with clear improvement of care as well as satisfaction with the care from patients and parents. The center also offers second opinions for patients and parents as well as advice for colleagues from other centers especially in the entity of low grade gliomas. The center also serves as a research platform for clinical as well as basic research projects. The structured implementation of a multidisciplinary team improves significantly the care of pediatric brain tumor patients and close collaboration between medical experts even in a university children’s hospital.

A 2-year-old girl fell off a bunk bed onto a parquet floor. She immediately reported neck pain and presented with muscle spasm and limited motion of the cervical spine (C-spine). Plain X-rays of the C-spine showed no osseous lesion. Owing to persisting pain and limited motion in the neck, MRI of the C-spine was obtained which revealed intact ligaments and cervical spinal cord, as well as soft tissue swelling in front of the anterior arch of the atlas. Subsequent CT of the C-spine confirmed a complete, undisplaced fracture of the anterior arch of the atlas (Gehweiler type I fracture). A Minerva cast was applied for 2 months, followed by a soft cervical collar. Persistent neck pain and limited range of motion of the neck after a fall may be indicative of atlas fracture that should be ruled out by CT.

Because of its subcutaneous location, the sternum can be examined sonographically using a linear scanner. We report about two children who experienced blunt chest trauma. Anterior–posterior chest X-rays were normal. Ultrasonic imaging confirmed a fracture of the sternum with dorsal displacement of the distal fragment (by 0.97 cm) in the first child and a transverse fracture of the body of the sternum without displacement in the second child. In both children, pericardial effusion was excluded by sonography. The displaced fracture of the sternum was confirmed by magnetic resonance imaging (MRI), which ruled out concomitant injuries of the soft tissues adjacent to the sternum. Both sternum fractures were managed nonoperatively. These cases serve to emphasize the importance of sonography that represents an ionizing radiation free, noninvasive, efficient, and safe imaging modality to diagnose fractures of the sternum in children while also enabling the assessment of the pericardium.

Aim: To investigate the effect of fasting times for clear fluids and solids/non-clear fluids on gastric content volume using magnetic resonance imaging (MRI). Methods: Pediatric patients undergoing diagnostic MRI under deep propofol sedation, with the stomach located within the area of diagnostic study, were included in this clinical observational study. According to standard institutional guidelines, children were allowed to eat/drink until 4 h and to drink clear fluids until 2 h before scheduled induction time of anesthesia. Gastric content volume per kg body weight (GCVw) was determined using MRI and compared with actual fasting times prior to induction. Results: Overall 68 patients aged from 0.3 to 19.6 (2.8) years were investigated. Fasting time for clear fluids ranged from 1.1 to 15.5 (5.5) h, for non-clear fluids/solids from 4.0 to 20.2 (6.7) h. GCVw ranged from 0.2 to 6.3 (0.75) ml·kg−1 and showed no significant negative correlation to fasting times for clear fluids (r = −0.07, P = 0.60) and non-clear fluids/solids (r = −0.08, P = 0.51). Conclusions: Based on this preliminary data, GCVw showed considerable variation but did not correlate with fasting times in children and adolescent patients. Recommended fasting times were often exceeded.

Haemolytic uraemic syndrome (HUS) is the primary diagnosis of 4.5% of children on chronic renal replacement therapy. Approximately 5% of all HUS cases have an "atypical" or recurrent course. Atypical HUS is an inadequate term that applies to a heterogeneous group of conditions. We describe this group as non-diarrhoeal (D-) ), non-EHEC (EHEC - ) HUS. Patients in the non-diarrhoeal, non-EHEC, relapsing group are much more likely to exhibit severe hypertension, histological findings of arterial as well as arteriolar disease, chronic and end-stage renal failure. In general, these patients have an alarmingly high risk of graft loss from disease recurrence or thrombosis ranging from 60-100%. Family history is crucial, and where family members have relapsing disease, transplantation is a very high risk procedure (recurrence 100%). Patients with (D-)HUS need very careful consideration before transplantation, including molecular investigation of complement regulators (and von Willebrandt protease (ADAMTS13) activity, although this goes beyond the scope of this review). Guidelines are accessible under http://www.espn.ucwm.ac.uk . On no account should live related donation take place unless the risks of graft loss are understood. International collaboration to identify safer ways of transplanting these challenging patients is urgently needed.

Item does not contain fulltext Hemolytic uremic syndrome (HUS) includes a heterogeneous group of hemolytic disorders. Among the identified causes of HUS are infections, particularly infections with Shiga toxin-producing ESCHERICHIA COLI (STEC), complement disorders, and disorders interfering with the degradation of von Willebrand factor (VWF). Other causes for atypical HUS include the cobalamin metabolism; pregnancy/hemolysis, elevated liver enzymes, and low platelets syndrome (HELLP); drugs; and other disorders (e.g., systemic diseases appearing as HUS, such as systemic lupus erythematosus and rejection after transplantation). The group not related to STEC is often also called atypical HUS. Most of the occurrences of infectious HUS have only one episode. Recurrent episodes (recurrent HUS) have strong relationships to diseases of the complement system. In these two subgroups the prognosis is poor, with severe renal insufficiency, together with the need for renal replacement therapy. Severe arterial hypertension is common. Treatment options are limited. To better define this group of patients, the European Society for Pediatric Nephrology supported an initiative to develop a European HUS registry. In this registry, 167 patients were acquired; 73 were female (43.8%). The year of onset of the disease ranged from 1974 to 2005. The prevalence of atypical HUS/recurrent HUS can be calculated as 3.3 per million child population (< 18 years). Underlying disorders included factor H, factor I, MCP-1, pneumococci, and von Willebrand factor disturbances. In 33 patients at least one renal transplantation was performed (total, 55 kidneys); 18% were successful and 73% demonstrated recurrence or thrombosis. Treatment options were plasma substitution or plasmapheresis. Despite continued efforts, transplantation is not recommended at present for these patients. Living-related transplantation should be abandoned. New therapeutic strategies are urgently needed.

To investigate the effect of fasting times for clear fluids and solids/non-clear fluids on gastric content volume using magnetic resonance imaging (MRI).Pediatric patients undergoing diagnostic MRI under deep propofol sedation, with the stomach located within the area of diagnostic study, were included in this clinical observational study. According to standard institutional guidelines, children were allowed to eat/drink until 4 h and to drink clear fluids until 2 h before scheduled induction time of anesthesia. Gastric content volume per kg body weight (GCV(w)) was determined using MRI and compared with actual fasting times prior to induction.Overall 68 patients aged from 0.3 to 19.6 (2.8) years were investigated. Fasting time for clear fluids ranged from 1.1 to 15.5 (5.5) h, for non-clear fluids/solids from 4.0 to 20.2 (6.7) h. GCV(w) ranged from 0.2 to 6.3 (0.75) ml·kg(-1) and showed no significant negative correlation to fasting times for clear fluids (r = -0.07, P = 0.60) and non-clear fluids/solids (r = -0.08, P = 0.51).Based on this preliminary data, GCV(w) showed considerable variation but did not correlate with fasting times in children and adolescent patients. Recommended fasting times were often exceeded.

Recurrent hemolytic uremic syndrome (recHUS) is a heterogeneous group of disorders. The pathogenesis of recHUS is not fully understood. recHUS has a high risk of development of terminal renal insufficiency and other sequelae. Abnormalities in complement factor H or in membrane-bound complement inhibitors with consecutive complement activation can be found in approximately 30 to 50% of the patients. Starting in 2001, we evaluated 42 patients with recHUS from five European countries (Germany, Austria, Hungary, Switzerland, and the Czech Republic). We measured the terminal complement complex (TCC) by an enzyme-linked immunosorbent assay using a neoepitope-specific anti-C9 antibody in 17 patients in plasma (native complement activation), serum (after coagulation), and zymosan-activated serum (Z-serum; after stimulation of coagulation). We compared the results to those of 16 healthy persons. In patients with recHUS (eight males, nine females) with a median age of 10.8 years, the TCC values were higher in plasma (0.57 versus 0.48 microg/mL; P = 0.04) and serum (3.1 versus 2.2 microg/mL) than in those of the control group, with a median age of 28.6 years (six males, 10 females) The TCC values in patients with low C3 compared with patients with normal C3 levels were even higher in plasma and serum, and the ratio was much lower. Children with recHUS have higher concentrations of TCC in plasma and serum. The ratio of Z-serum to serum showed significantly lower values in children with recHUS (96.01 versus 150.3; P = 0.01). These findings indicate a higher grade of complement activation and consumption in recHUS, suggesting that TCC may mediate cell toxicity. This may play an important role in the inferior outcome of these patients. The isolated substitution of factor H, or other complement inhibitors to block TCC formation, may represent useful therapies for these patients.

A 12-year-old Turkish girl suffered from abdominal pain located in the right lower abdomen for 3 weeks. Ultrasound revealed palisade-like swelling of the mucosa in the ileum. Gastrointestinal biopsy showed incipient granulomas in the stomach and moderate fibrosis of the terminal ileum. Subsequently, bilateral hilar adenopathy and an abnormal level of serum angiotensin-converting enzyme were detected. The relevance of paediatric sarcoidosis mimicking Crohn's disease is discussed.

Reports on von Willebrand factor (vWF) in hemolytic-uremic syndrome (HUS) are not unequivocal. Because of potential pathogenic implications, we examined the ability of vWF to bind to collagen in vitro, which reflects its function. Plasma vWF antigen (vWF:Ag) and collagen-binding activity (vWF:CBA) were measured by enzyme-linked immunosorbent assay in children with (1) diarrhea-associated (D+) HUS (n = 27), (2) chronic renal insufficiency (CRI) (n = 8), (3) gastroenteritis (GE) not associated with HUS (n = 15), (4) immune thrombocytopenia (ITP) (n = 40) and from controls (n = 35). Structural vWF was evaluated by multimer analysis. Children with D+ HUS had vWF:Ag of 2.53 and vWF:CBA of 1.98 U/mL. The corresponding values for patients with ITP were 1.35 and 1.82 U/mL, with CRI 1.55 and 1.55 U/mL, and with GE 1.68 and 2.10 U/mL; all values were higher than in controls (1.04 and 1.16 U/mL). The mean ratio of vWF:CBA to vWF:Ag ratio in controls was 1.13; only children with HUS had a dysfunctional vWF, as indicated by a low ratio of 0.78; the ratio was elevated in children with ITP (1.36) and GE (1.27) and was normal in those with CRI (1.06). No ultralarge molecular multimers of vWF were detected in any group, including HUS. The very high concentration of plasma vWF:Ag in HUS probably reflects endothelial cell damage or irritation. In contrast to all other groups, only children with HUS had a dysfunctional vWF, caused either by a primary (due to enterohemorrhagic Escherichia coli) or secondary (due to consumption of functionally active vWF) process. This abnormality was not obvious as structural anomaly by multimer analysis.