Thekla von Kalle

Sonography of acute appendicitis in children: 7 years experience.

Objective. The objective of this prospective study was to determine the accuracy in diagnosing appendicitis in children by using high-resolution graded compression sonography. Materials and methods. During a 7-year period, 3859 children (mean age 9.9 years) with clinically suspected appendicitis were evaluated by sonography with the graded compression technique. The ultrasound results were correlated with the intraoperative and histopathological findings or clinical outcome. Results. High-resolution sonography was performed in 3859 children. Of the 610 patients who underwent a laparotomy, 494 had histologically proven acute or perforated appendicitis (prevalence 13 %). In these children, sensitivity, specificity and overall accuracy of sonography were 90 %, 97 % and 96 %, respectively. The reasons for false-negative and false-positive results are discussed. Conclusion. Although the use of ultrasound to diagnose acute appendicitis in children has excellent results, the decision for surgery remains a clinical one because of the continuing false-negative and false-positive results from sonography.

Contrast-enhanced MRI of normal temporomandibular joints in children—is there enhancement or not?

OBJECTIVES To determine the degree of contrast enhancement of normal TM joint (TMJ) in children and to provide reference data for patients with JIA. METHODS A total of 100 dynamic contrast-enhanced MRI of 46 children from 1.6 to 18 years (median 9.1 years), who underwent the examinations for reasons other than TMJ disease, were retrospectively analysed in accordance with our local ethics committee. Time-intensity curves of the mandibular condyle and the soft tissue of the joint were assessed in relation to pre-contrast images and to muscle tissue. RESULTS After contrast injection the soft tissue of all joints showed a rapid initial increase of signal intensity (SI) followed by a slow increase, with the maximum after 6 min (end of the scan). Maximum SI in the joint tissue was on average 73% higher than pre-contrast, 53% higher than in the condyle and 20% higher than in an adjacent muscle. However, in 15% of measurements post-contrast SI of the muscle was higher than that of the joint tissue. The enhancement curve of the bone marrow in the condyle showed a peak by 2 min post-contrast. CONCLUSION Contrast enhancement is a normal finding in the soft tissue and the condyle of the TMJ in children and adolescents. This should be taken into account when assessing the TMJ of children with JIA. As the relative enhancement of joint in comparison with muscle tissue proved to be variable, subtraction analysis of pre- and post-contrast T1 weighted images seems to provide a better basis for visual assessment.

Cervical spine involvement in patients with juvenile idiopathic arthritis - MRI follow-up study

BackgroundTo describe MRI and clinical findings in patients with juvenile idiopathic arthritis with cervical spine involvement at onset and follow-up under therapy.Methods13 patients with signs of cervical spine involvement in juvenile idiopathic arthritis with a median disease duration of 1.7 years were included in the study. Clinical records and MR images were retrospectively analyzed according to symptoms and findings concerning the cervical spine.ResultsAt the onset of cervical spine involvement all patients showed limited range of motion, whereas only 5 of them complained of pain. In MR images joint hyperintensity, contrast enhancement, malalignment, ankylosis, erosion and narrowing of the spinal canal at cranio-cervical junction were found at 28, 32, 15, 2, 2 and 3 sites in 12 (93%), 13 (100%), 8 (62%), 2 (15%), 2 and 3 (20%) patients respectively. 3 of the 5 patients with pain (60%) showed ankylosis, erosions or narrowing of the spinal canal at cranio-cervical junction on MRI. At follow-up - after a median disease duration of cervical spine arthritis of 2.1 years and a variable duration of treatment with methotrexate (all patients) and biological agents (12 patients) - joint hyperintensity, enhancement and malalignment decreased to 15, 19 and 6 sites in 10 (77%), 11 (85%) and 3 (20%) patients respectively whereas ankylosis, erosion and narrowing of the spinal canal at cranio-cervical junction increased to 7, 6 and 4 sites in 3 (20%), 4 (31%) and 4 patients respectively. Pain was no longer reported, but 9 of 13 (69%) patients still had a limited range of motion with 6 of them (46%) showing skeletal changes on MRI.ConclusionsThis first MRI based follow-up study shows that cervical spine arthritis can follow a severe disease course in juvenile arthritis. While malalignments and inflammation sites decreased osseous changes with erosions, ankylosis, and narrowing of the spinal canal increased under treatment despite only minor subjective complaints. Therefore close MRI monitoring of these patients appears to be reasonable.

Towards Establishing a Standardized Magnetic Resonance Imaging Scoring System for Temporomandibular Joints in Juvenile Idiopathic Arthritis

The temporomandibular joints (TMJs) are frequently affected in children with juvenile idiopathic arthritis (JIA). Early detection is challenging, as major variation is present in scoring TMJ pathology on magnetic resonance imaging (MRI). Consensus‐driven development and validation of an MRI scoring system for TMJs has important clinical utility in timely improvement of diagnosis and serving as an outcome measure. We report on a multi‐institutional collaboration toward developing a TMJ MRI scoring system for JIA.

Does diffusion-weighted MRI (DW-MRI) change treatment strategy in pediatric cholesteatoma?

Abstract Conclusion: This review highlights the currently limited and contradictory evidence on the role of diffusion-weighted magnetic resonance imaging (DW-MRI) in the management of pediatric cholesteatoma patients. Available data confirm the need for prospective DW-MRI studies of a larger number of pediatric patients. Factors such as patient compliance, adhesion to follow-up or local logistic circumstances should also be considered. Objectives: This systematic review aimed to collect and analyze the body of evidence on the usefulness of DW-MRI in first-stage tympanoplasty and in replacing second-look surgery in children with cholesteatoma. Methods: A comprehensive search of medical databases was conducted. The keywords used were pediatric/paediatric, magnetic resonance imaging, MRI, DW-MRI, cholesteatoma, child, and all meaningful combinations of them. Results: Only one study with 17 patients primarily fulfilled all criteria. Sensitivity, specificity, and positive and negative predictive values were 62%, 88%, 89%, and 58%, respectively. A second study retrospectively analyzed a subset of younger patients (n = 21) of a larger population. Sensitivity, specificity, and positive and negative predictive values were 100%. One additional study described 1 child and 11 adolescents among 32 cases. In all cases the correlation between imaging and surgery findings was 100%. There were no randomized controlled trials and power calculations were not performed.

Inflammatory myofibroblastic tumors—A retrospective analysis of the Cooperative Weichteilsarkom Studiengruppe

Inflammatory myofibroblastic tumors (IMTs) are a rare subgroup of soft tissue tumors. The outcome of patients with IMT has been reported as favorable when the tumor is completely resected. If surgical resection is not possible, systemic therapy has to be considered. However, the best systemic treatment and response rates are currently unclear.

Multiple Metachronous Osteosarcomas in a Patient with Li-Fraumeni Syndrome

A patient of ours suffered a total of four osteosarcomas, all confirmed by reference pathology, over a period of 9 years. She had osteoblastic osteosarcoma of the left proximal fibula at age 5, then chondroblastic osteosarcoma of the right proximal humerus and the left 5th rib at age 12, and osteoblastic osteosarcoma of the right clavicle at age 14. There were no signs for pulmonary or other metastases. She received preoperative chemotherapy with standard or modified osteosarcoma regimens for all but the last manifestation and histologic response was good (<10% viable tumor) in all evaluable lesions (Table 1). These lesions were also removed with wide margins, while the clavicle osteosarcoma required revision surgery as histology of first resection revealed involved margins. Postoperative chemotherapy was administered upon all occasions (Table 1). The patient had a family history of cancer with bilateral breast cancer and pelvic chondrosarcoma of her mother, both in the 4th decade of life, and met the 2009 Chompret criteria for TP53 mutation screening [1]. Genetic evaluation of TP53 revealed a novel mutation, namely heterozygous deletions in exons 6, 7, and 8, confirming presence of the Li-Fraumeni syndrome (LFS) in the girl. Currently, 15 months after the last disease episode, the patient is free of disease as well as in good clinical condition and attends school regularly. Long-term follow-up will consider follow-up recommendations for patients with osteosarcoma [2] and individuals with LFS [3, 4].

EURO-B.O.S.S.: A European study on chemotherapy in bone-sarcoma patients aged over 40: Outcome in primary high-grade osteosarcoma

Introduction: The EUROpean Bone Over 40 Sarcoma Study (EURO-B.O.S.S.) was the first prospective international study for patients 41-65 years old with high-grade bone sarcoma treated with an intensive chemotherapy regimen derived from protocols for younger patients with high-grade skeletal osteosarcoma. Methods: Chemotherapy based on doxorubicin, cisplatin, ifosfamide, and methotrexate was suggested, but patients treated with other regimens at the investigators’ choice were also eligible for the study. Results: The present report focuses on the subgroup of 218 patients with primary high-grade osteosarcoma. With a median follow-up of 47 months, the 5-year probability of overall survival (OS) was 66% in patients with localized disease and 22% in case of synchronous metastases. The 5-year OS in patients with localized disease was 29% in pelvic tumors, and 70% and 73% for extremity or craniofacial locations, respectively. In primary chemotherapy, tumor necrosis ≥90% was reported in 21% of the patients. There were no toxic deaths; however, hematological toxicity was considerable with 32% of patients experiencing 1 or more episodes of neutropenic fever. The incidence of nephrotoxicity and neurotoxicity (mainly peripheral) was 28% and 24%, respectively. After methotrexate, 23% of patients experienced delayed excretion, in 4 cases with nephrotoxicity. Conclusions: In patients over 40 years of age with primary high-grade osteosarcoma, an aggressive approach with chemotherapy and surgery can offer the probability of survival similar to that achieved in younger patients. Chemotherapy-related toxicity is significant and generally higher than that reported in younger cohorts of osteosarcoma patients treated with more intensive regimens.

The prognostic value of early radiographic response in children and adolescents with embryonal rhabdomyosarcoma stage IV, metastases confined to the lungs: A report from the Cooperative Weichteilsarkom Studiengruppe (CWS): Sparber-Sauer et al.

Patients with metastatic rhabdomyosarcoma (RMS) have a poor prognosis apart from children with embryonal RMS whose metastases are confined to the lungs (PRME). The prognostic significance of response in patients with metastatic disease is still unknown and optimal treatment remains to be defined.

High-Grade Osteosarcoma of the Foot: Presentation, Treatment, Prognostic Factors, and Outcome of 23 Cooperative Osteosarcoma Study Group COSS Patients

Osteosarcoma of the foot is a very rare presentation of a rare tumor entity. In a retrospective analysis, we investigated tumor- and treatment-related variables and outcome of patients registered in the Cooperative Osteosarcoma Study Group (COSS) database between January 1980 and April 2016 who suffered from primary high-grade osteosarcoma of the foot. Among the 23 eligible patients, median age was 32 years (range: 6–58 years), 10 were female, and 13 were male. The tarsus was the most commonly affected site (n=16). Three patients had primary metastases. All patients were operated: 5 underwent primary surgery and 18 received surgery following preoperative chemotherapy. In 21 of the 23 patients, complete surgical remission was achieved. In 4 of 17 patients, a poor response to neoadjuvant chemotherapy was observed in the resected primary tumors. Median follow-up was 4.2 years (range: 0.4–18.5). At the last follow-up, 15 of the 23 patients were alive and 8 had died. Five-year overall and event-free survival estimates were 64% (standard error (SE) 12%) and 54% (SE 13%), which is similar to that observed for osteosarcoma in general. Event-free and overall survival correlated with primary metastatic status and completeness of surgery. Our findings show that high-grade osteosarcoma in the foot has a similar outcome as osteosarcoma of other sites.