[Pediatric developmental disorders of the musculoskeletal system]. / Pädiatrische Entwicklungsstörungen des muskuloskeletalen Systems.

CLINICAL/METHODICAL ISSUE: In pediatric musculoskeletal disorders, a distinction is made between normal and abnormal development. STANDARD RADIOLOGICAL METHODS: X­ray examination is the standard diagnostic procedure. METHODOLOGICAL INNOVATIONS: Cross-sectional imaging, such as magnetic resonance imaging (MRI), computed tomography (CT), and ultrasonography (US) are increasingly used. PERFORMANCE: X­ray imaging is still the examination method of choice but can be supplemented by cross-sectional imaging depending on clinical situation. ACHIEVEMENTS: Cross-sectional imaging is helpful and necessary in many cases but significantly more time-consuming and costly than x­ray examinations. PRACTICAL RECOMMENDATIONS: Initially standard x­rays should be taken, which can then be supplemented with cross-sectional imaging depending on the result and clinical question (MRI and US are preferred as there is no radiation).

Chronic non-bacterial osteomyelitis (CNO) in childhood and adolescence - a disease with many faces. / Die Chronische Nicht Bakterielle Osteomyelitis (CNO) im Kindes- und Jugendalter ­ eine Erkrankung mit vielen Gesichtern.

BACKGROUND: Chronic non-bacterial osteomyelitis (CNO) is a chronic inflammatory skeletal disease that affects particularly children and adolescents and is often diagnosed with a delay. With whole-body MRI, early diagnosis of this disease is possible in many cases. Since children and adolescents frequently present with non-specific complaints for outpatient radiological diagnosis, every radiologist should have basic knowledge of this complex clinical picture. MATERIALS: In this review the basics and current findings regarding the disease are discussed. Unusual courses are also presented. RESULTS AND CONCLUSION: With knowledge of the radiographic and MR tomographic characteristics of the mostly multifocal bone lesions, the diagnosis of CNO can be reliably made in many cases. In particular, the early use of whole-body MRI plays an important role. Thus, therapeutic delays and resulting complications and the number of unnecessary biopsies for diagnosis can be reduced.

[Radiological diagnosis of apophyseal injuries of the pelvic region]. / Radiologische Diagnostik von Apophysenverletzungen der Beckenregion.

Avulsion injuries of the pelvis are common sports-related findings in skeletally immature adolescent patients. They usually present as an avulsion of the unfused apophysis, resulting from forced muscular contraction during typical athletic movements or from chronic traction injuries. Acute apophyseal avulsion injuries are usually easily detected with radiography. Occasionally, advanced imaging such as ultrasound or magnetic resonance imaging is required in order to detect or fully assess the extent of injury. Chronic injuries can mimic aggressive entities, such as neoplasms and may thus cause difficulties in differential diagnosis. Profound knowledge of typical locations of apophyseal tendon attachment sites at the osseous pelvis and correct interpretation of imaging findings can facilitate accurate diagnosis. This article discusses the clinical and radiological findings of pelvic apophyseal avulsion injuries in pediatric patients and highlights potential complications and differential diagnoses.

Cough-induced chylothorax in a two-year-old boy - case report and review of the literature.

BACKGROUND: Chylothorax is a very rare form of pleural effusion in children, especially after the neonatal period, and predominantly occurs secondary to cardiothoracic surgery. It can lead to significant respiratory distress, immunodeficiency, and malnutrition. Effective treatment strategies are therefore required to reduce morbidity. CASE PRESENTATION: A previously healthy two-year old boy was admitted with history of heavy coughing followed by progressive dyspnea. The chest X-ray showed an extensive opacification of the right lung. Ultrasound studies revealed a large pleural effusion of the right hemithorax. Pleural fluid analysis delivered the unusual diagnosis of chylothorax, most likely induced by preceded excessive coughing. After an unsuccessful treatment attempt with a fat-free diet and continuous pleural drainage for two weeks, therapy with octreotide was initiated. This led to complete and permanent resolution of his pleural effusion within 15 days, without any side effects. CONCLUSIONS: Severe cough may be a rare cause of chylothorax in young children. Octreotide seems to be an effective and safe treatment of spontaneous or traumatic chylothorax in children. There is, however, a lack of comprehensive studies for chylothorax in children and many issues concerning diagnostic strategies and treatment algorithms remain.

Acute care diagnostics in children for general radiologists - not alone in the hospital at night. / Akutdiagnostik Kinder für AllgemeinradiologInnen ­ nachts nicht allein im Krankenhaus.

BACKGROUND: Radiologic care for children and adolescents during night and weekend shifts is challenging. This is especially true when a dedicated pediatric radiology front or background service is not available. METHODS: The purpose of this review is to present the approach, the most relevant diagnoses, and their differential diagnoses based on four common example cases - abdominal pain, respiratory/chest pain, headache, and refusal to walk. Essentials such as clinical classification (e. g., disease dynamics) and practical instructions (e. g., necessity of acute cross-sectional imaging) are presented. RESULTS AND CONCLUSION: For the abdomen, appendicitis ranks first among acute diseases. Other important diseases are intussusception and volvulus. Far more frequently, however, gastroenteritis is the cause of abdominal pain. Usually no imaging is required in this case. In unclear clinical situations, ultrasound may be indicated. In suspected pulmonary infections, chest imaging is limited to inconclusive cases and suspicion of complications such as pleural empyema. Major emergencies include (spontaneous) pneumothorax and aspiration. Headache is a common symptom. Immediate imaging is only necessary in cases of suspected acute inflammatory (meningitis/encephalitis) or vascular disease (e. g., hemorrhage due to vascular malformations). MRI is the primary imaging modality in these cases. Restricted walking/refusal to walk is a classic nonspecific sign, particularly of acute musculoskeletal disease, especially in younger children. Clinical examination is essential to narrow down the field of investigation. Besides the frequent and symptomatic coxitis fugax, the rare but serious (septic) arthritis/osteomyelitis must not be overlooked. KEY POINTS: · Radiological care of children and adolescents is challenging, especially during night and weekend shifts.. · However, in close cooperation with the referring colleagues/clinics, the appropriate approach can be effectively determined even if the symptoms are not clear.. · The selection of the optimal imaging method is based on guidance and guidelines, but also on the condition of the child/adolescent.. · A silent or whimpering child is cause for alarm.. CITATION FORMAT: · Beer M, Mentzel H, Steinborn M et al. Acute care diagnostics in children for general radiologists - not alone at night in the hospital. Fortschr Röntgenstr 2023; 195: 205 - 216.

CT-like MR-derived Images for the Assessment of Craniosynostosis and other Pathologies of the Pediatric Skull.

PURPOSE: To evaluate the diagnostic value of CT-like images based on a 3D T1-weighted spoiled gradient echo-based sequence (T1SGRE) for the visualization of the pediatric skull and the identification of pathologies, such as craniosynostosis or fractures. METHODS: In this prospective study, 20 patients with suspected craniosynostosis (mean age 1.26 ± 1.38 years, 10 females) underwent MR imaging including the T1SGRE sequence and 2 more patients were included who presented with skull fractures (0.5 and 6.3 years, both male). Additionally, the skull of all patients was assessed using radiography or CT in combination with ultrasound. Two radiologists, blinded to the clinical information, evaluated the CT-like images. The results were compared to the diagnosis derived from the other imaging modalities and intraoperative findings. Intrarater and interrater agreement was calculated using Cohen's κ. RESULTS: Of the 22 patients 8 had a metopic, 4 a coronal and 2 a sagittal craniosynostosis and 2 patients showed a complex combination of craniosynostoses. The agreement between the diagnosis based on the T1SGRE and the final diagnosis was substantial (Cohen's κ = 0.92, 95% confidence interval (CI) 0.77-1.00 for radiologist 1 and κ = 0.76, CI 0.51-1.00 for radiologist 2). Of the patients with fractures, one presented with a ping pong fracture and one with a fracture of the temporal bone. Both radiologists could identify the fractures using the T1SGRE. CONCLUSION: The visualization of the pediatric skull and the assessment of sutures using a CT-like T1SGRE MR-sequence is feasible and comparable to other imaging modalities, and thus may help to reduce radiation exposure in pediatric patients. The technique may also be a promising imaging tool for other pathologies, such as fractures.

Two-Phase Deep Learning Algorithm for Detection and Differentiation of Ewing Sarcoma and Acute Osteomyelitis in Paediatric Radiographs.

BACKGROUND/AIM: Ewing sarcoma is a highly malignant tumour predominantly found in children. The radiological signs of this malignancy can be mistaken for acute osteomyelitis. These entities require profoundly different treatments and result in completely different prognoses. The purpose of this study was to develop an artificial intelligence algorithm, which can determine imaging features in a common radiograph to distinguish osteomyelitis from Ewing sarcoma. MATERIALS AND METHODS: A total of 182 radiographs from our Sarcoma Centre (118 healthy, 44 Ewing, 20 osteomyelitis) from 58 different paediatric (≤18 years) patients were collected. All localisations were taken into consideration. Cases of acute, acute on chronic osteomyelitis and intraosseous Ewing sarcoma were included. Chronic osteomyelitis, extra-skeletal Ewing sarcoma, malignant small cell tumour and soft tissue-based primitive neuroectodermal tumours were excluded. The algorithm development was split into two phases and two different classifiers were built and combined with a Transfer Learning approach to cope with the very limited amount of data. In phase 1, pathological findings were differentiated from healthy findings. In phase 2, osteomyelitis was distinguished from Ewing sarcoma. Data augmentation and median frequency balancing were implemented. A data split of 70%, 15%, 15% for training, validation and hold-out testing was applied, respectively. RESULTS: The algorithm achieved an accuracy of 94.4% on validation and 90.6% on test data in phase 1. In phase 2, an accuracy of 90.3% on validation and 86.7% on test data was achieved. Grad-CAM results revealed regions, which were significant for the algorithms decision making. CONCLUSION: Our AI algorithm can become a valuable support for any physician involved in treating musculoskeletal lesions to support the diagnostic process of detection and differentiation of osteomyelitis from Ewing sarcoma. Through a Transfer Learning approach, the algorithm was able to cope with very limited data. However, a systematic and structured data acquisition is necessary to further develop the algorithm and increase results to clinical relevance.

Treatment of Unspecific Back Pain in Children and Adolescents: Results of an Evidence-Based Interdisciplinary Guideline.

Using a structured approach and expert consensus, we developed an evidence-based guideline on the treatment and prevention of non-specific back pain in children and adolescents. A comprehensive and systematic literature search identified relevant guidelines and studies. Based on the findings of this literature search, recommendations on treatment and prevention were formulated and voted on by experts in a structured consensus-building process. Physical therapy (particularly physical activity) and psychotherapy (particularly cognitive behavioral therapy) are recommended for treating pediatric non-specific back pain. Intensive interdisciplinary treatment programs should be provided for chronic and severe pain. Drug therapy should not be applied in children and adolescents. Further research on non-specific back pain in childhood and adolescence is strongly needed to reduce the imbalance between the high burden of non-specific back pain in childhood and adolescence and the low research activity in this field.

Etiology, Risk Factors, and Diagnosis of Back Pain in Children and Adolescents: Evidence- and Consensus-Based Interdisciplinary Recommendations.

Using a structured approach and expert consensus, we developed an evidence-based guideline on the diagnosis of back pain and the treatment of non-specific back pain in children and adolescents. The first part comprises etiology, risk factors, and diagnosis. The second part, published in the same issue, includes treatment and prevention. A comprehensive and systematic literature search was conducted to identify relevant guidelines and studies. Based on the findings of this literature search, recommendations on risk factors and diagnosis were formulated and voted on by experts in a structured consensus-building process. Notable red flags for specific back pain and evidence-based risk factors for non-specific back pain in children and adolescents were identified. Only three evidence-based recommendations could be formulated for causes, red flags, and risk factors for back pain, while two recommendations are based on expert consensus. Regarding diagnostics, eight expert consensus recommendations and one evidence-based recommendation could be provided. Despite the importance of adequate diagnosis for the treatment of back pain in children and adolescents, results of this work confirm the deficit in research investment in this area.

Surgical treatment of a spontaneous spinal epidural hematoma under antithrombotic treatment of a multisystem inflammatory syndrome in children associated with SARS-COV-2.

Multisystem inflammatory syndrome in children (MIS-C) is a novel syndrome of multisystemic inflammation affecting children. This case report documents an exceptional and severe complication of an epidural hematoma in a 3-year-old boy under the treatment of MIS-C. During the course of the disease, the patient suffered from a hypocoagulable state and an extensive multisegmental epidural hematoma in the cervical spinal canal. This led to severe anterior spinal cord compression and tetraparesis. Extensive emergency surgery had to be carried out to reverse rapid clinical deterioration.

The color Doppler twinkling artifact of implants after endoscopic treatment of vesicoureteral reflux in children: A common finding with high potential for misdiagnosis.

BACKGROUND: Endoscopic treatment of vesicoureteral reflux (VUR) is a common therapeutic procedure in children. Over the last years several studies reported on calcified deflux implants that were misinterpreted as ureteral stones leading to unnecessary diagnostic and therapeutic procedures. OBJECTIVE: Based on an own case, where a calcified implant with a strong twinkling artifact was misdiagnosed as a ureteral stone, the purpose of our study was to evaluate the sonographic imaging appearance of implants after endoscopic VUR repair with special emphasis on the color twinkling artifact. MATERIAL AND METHODS: In 40 children (mean age 9.5 years) with 62 treated ureteral units follow-up sonography was performed after a mean time interval of 48.8 months after surgery. The injected deposit was evaluated with B-mode sonography and color Doppler sonography and deposit volume, posterior acoustic shadowing and the appearance and extension of the twinkling artifact were evaluated. RESULTS: 47 of 62 injected units (75.8%) could be identified on follow-up sonography. In 13 of 47 units (27.7%) posterior acoustic shadowing was noted. On color Doppler sonography a twinkling artifact appeared in 26 of the 47 visible cases (55.3%). There was a statistically significant correlation between a positive twinkling sign and the deposit age. CONCLUSION: In conclusion our study shows that the twinkling artifact is a common finding in follow-up sonography of children after endoscopic treatment of VUR. As the twinkling artifact is a sensitive imaging sign for the detection of ureteral calculi the risk of misinterpretation and mistreatment is given.

Normal Variations and Pathologic Disorders of Chondrification and Ossification of the Foot and Related Diseases.

Formation of the skeletal elements of the foot involves different stages of development. Failure in segmentation of the cartilaginous plate is supposed to be the underlying mechanism leading to tarsal coalition. Variants or disorders in ossification might result in harmless osseous anomalies or symptomatic disease. When the ossification is almost completed, several secondary ossification centers start to ossify. They are usually incidental findings. In symptomatic patients they have to be differentiated from fractures or can be the source of complaints by themselves.

Whole-Body MRI in Children and Adolescents - S1 Guideline. / Ganzkörper-Magnetresonanztomografie im Kindes- und Jugendalter ­ S1-Leitlinie.

Whole-body MRI is an imaging method that uses advanced modern MRI equipment to provide high-resolution images of the entire body. The goal of these guidelines is to specify the indications for which whole-body MRI can be recommended in children and adolescents and to describe the necessary technical requirements. CITATION FORMAT: · Schaefer JF, Berthold LD, Hahn G et al. Whole-Body MRI in Children and Adolescents - S1 Guidelines. Fortschr Röntgenstr 2019; 191: 618 - 625.

Impact of Pathological Fractures on the Prognosis of Primary Malignant Bone Sarcoma in Children and Adults: A Single-Center Retrospective Study of 205 Patients.

BACKGROUND: The purpose of this study was to investigate whether pathological fractures (PF) influence the prognosis of patients with osteosarcoma (OS) or Ewing tumor (ET) regarding 5-year survival, occurrence of metastases, and local recurrence. METHODS: We retrospectively analyzed 205 patients with metastatic and nonmetastatic OS or ET. Survival analysis was performed for all patients and differentiated for patients with OS (n = 127) and ET (n = 78) as well as for adults (n = 101) and children (n = 104). RESULTS: Patients with PF showed survival rates of 64% compared to 83% for those without PF (p = 0.023). Local recurrence occurred in 7% of the patients without and in 24% of those with PF (p = 0.023). In patients with ET and in children, survival analysis showed no significant difference between patients with and without PF in survival and local recurrence rates. In patients with OS, survival rate decreased from 83 to 59% (p = 0.024) and local recurrence rate increased from 13 to 30% (p = 0.042). In adults, survival rate decreased from 78 to 51% (p = 0.004) and local recurrence rate increased from 13 to 42% (p < 0.001). In multivariate analysis, age and PF were associated with inferior survival. CONCLUSION: This study suggests that the occurrence of PF has a negative impact on survival and implicates an increased risk of local recurrence. In children and in patients with ET, PF did not have a prognostic impact.

Bone marrow involvement identifies a subgroup of advanced Ewing sarcoma patients with fatal outcome irrespective of therapy in contrast to curable patients with multiple bone metastases but unaffected marrow.

PURPOSE: Advanced Ewing sarcomas have poor prognosis. They are defined by early relapse (<24 months after diagnosis) and/or by metastasis to multiple bones or bone marrow (BM). We analyzed risk factors, toxicity and survival in advanced Ewing sarcoma patients treated with the MetaEICESS vs. EICESS92 protocols. DESIGN: Of 44 patients, 18 patients were enrolled into two subsequent MetaEICESS protocols between 1992 and 2014, and compared to outcomes of 26 advanced Ewing sarcoma patients treated with EICESS 1992 between 1992 and 1996. MetaEICESS 1992 consisted of induction chemotherapy, whole body imaging directed radiotherapy to the primary tumor and metastases, tandem high-dose chemotherapy and autologous rescue. In MetaEICESS 2007 this treatment was complemented by allogeneic stem cell transplantation. EICESS 1992 comprised induction chemotherapy, local therapy to the primary tumor only followed by consolidation chemotherapy. RESULTS: In MetaEICESS 8/18 patients survived in complete remission vs. 2/26 in EICESS 1992 (p<0.05). Survival did not differ between MetaEICESS 2007 and MetaEICESS 1992. Three MetaEICESS patients died of complications, all in MetaEICESS 1992. After exclusion of patients succumbing to treatment related complications (n=3), 7/10 patients survived without BM involvement, in contrast to 0/5 patients with BM involvement. This was confirmed in a multivariate analysis. There was no correlation between BM involvement and the number of metastases at diagnosis. CONCLUSION: The MetaEICESS protocols yield long-term disease-free survival in patients with advanced Ewing sarcoma. Allogeneic stem cell transplantation was not associated with increased death of complications. Bone marrow involvement is a risk factor distinct from multiple bone metastases.

High resolution transbulbar sonography in children with suspicion of increased intracranial pressure.

PURPOSE: To evaluate the accuracy of high resolution transbulbar sonography for the estimation of intracranial pressure (ICP) in children. METHODS: In children and adolescents with acute neurologic symptoms of various origin, transbulbar sonography was performed. Besides measurement of the optic nerve sheath diameter (ONSD), the ultrastructure of the subarachnoid space of the optic nerve sheath was evaluated. The results of transbulbar sonography were correlated with clinical data based on cross-sectional imaging, ICP measurement, and ophthalmologic examination. RESULTS: Eighty-one patients (age 3-17.8 years, mean 11.7 years) were included. In 25 children, cross-sectional imaging and ICP measurement revealed increased intracranial pressure. The mean ONSD was 6.85 ± 0.81 mm. Twenty patients (20/25, 80 %) had a microcystic appearance of the subarachnoid space of the optic nerve. In 56 children without evidence of increased intracranial pressure, the mean ONSD was 5.77 ± 0.48 mm. Forty-nine patients (49/56, 87.5 %) had a normal homogenous appearance of the subarachnoid space. The ONSD in children with increased intracranial pressure was significantly higher than in patients without (p < 0.001). CONCLUSION: High resolution transbulbar sonography of the optic nerve is a useful technique for the rapid and non-invasive estimation of intracranial pressure in children. Besides measurement of the optic nerve sheath diameter, evaluation of the ultrastructure of the subarachnoid space of the optic nerve is a helpful parameter.

Severe pretreatment cerebral edema in newly diagnosed type 1 diabetes.

INTRODUCTION: Cerebral edema (CE) is a rare and dangerous complication of diabetic ketoacidosis. In typical cases, it may develop during several hours after the beginning of ketoacidosis therapy. Nevertheless, CE sometimes occurs before the start of any therapy - as for the patient in this report here. CASE REPORT: We describe a 12-year-old girl with newly diagnosed type 1 diabetes, presenting with severe headache and disorientation. Diabetes-related symptoms were not reported by the family. Clinical investigation revealed signs of meningeal irritation and Kussmaul breathing. In the laboratory, severe ketoacidosis (pH 6.95) and hyperglycemia (blood glucose 20.9 mmmol/l) were found. Cranial computed tomography showed CE. The patient was treated with a very cautious fluid and insulin therapy and recovered within 3 days. MRI after recovery showed normal findings without residuals of CE. CONCLUSION: CE before any treatment of ketoacidosis is a very rare complication of type 1 diabetes. Early diagnosis and effective treatment are extremely important for the patient's outcome and prognosis.

Visual loss without headache in children with pseudotumor cerebri and growth hormone treatment.

We report on two prepubescent girls with visual loss due to idiopathic intracranial hypertension (IIH), or pseudotumor cerebri, both treated with recombinant human growth hormone for growth failure. The interval from starting hormone therapy to diagnosis of IIH was 3 and 18 months, respectively. Both girls did not complain of headache and nausea. They were neither obese nor did they suffer from renal insufficiency. In both patients, we observed bilateral optic disc edema with visual loss and elevated cerebrospinal fluid (CSF) pressures. Other causes of IIH were excluded with neuroimaging and CSF examination. Cessation of drug administration is often sufficient for symptom resolution in cases of hormone therapy-associated IIH. However, visual field defects in one girl remained unchanged during follow-up of 8 months. In children with IIH, the spectrum of neurologic and visual manifestations might be variable and unspecific. Diagnosis and management of IIH can be difficult in the absence of headache. Blurred or double vision due to cranial nerve palsy might be the only symptom rather than complaints about reduced visual acuity. Therefore, regular clinical monitoring of visual function and fundus appearance is essential for early diagnosis, efficient management, and improvement of visual outcome in children receiving recombinant human growth hormone.

Lumbar disc herniation in three patients with cystic fibrosis: a case series.

INTRODUCTION: To date, lumbar disc herniation has not been reported in the context of cystic fibrosis even though back pain and musculoskeletal problems are very common in patients with cystic fibrosis. CASE PRESENTATION: We report on three patients with cystic fibrosis who experienced lumbar disc herniation in the course of their disease at ages 19 to 21 years (a 22-year-old Caucasian man, a 23-year-old Caucasian man, and a 21-year-old Caucasian woman). Our third patient eventually died because of her deteriorated pulmonary situation, which was influenced by the lumbar disc herniation as it was not possible for her to perform pulmonary drainage techniques properly because of the pain. CONCLUSIONS: Lumbar disc herniation can lead to a vicious cycle for patients with cystic fibrosis as it may promote pulmonary infections. This report highlights the need to investigate patients correctly.