High reliability of scrotal ultrasonography in the management of childhood primary testicular neoplasms.

BACKGROUND: The role for testis-sparing surgery in the treatment of primary intratesticular lesions in childhood is growing. The reliability of scrotal ultrasonograpy (US) in the management of these lesions is still controversial. PATIENTS: Between 1991 and 2007, 383 children and adolescents presented with testicular abnormalities. Ultrasound results and records of patients with primary testicular neoplasms were analyzed. RESULTS: 12 of 383 patients (3.1%) had a histologically proven primary intratesticular neoplasm. Scrotal US was highly sensitive for the detection of these lesions. Patients' mean age at initial US was 6 years (9 prepubertal, 3 juvenile patients). The most frequent symptom was a painless unilateral scrotal mass (75%). Tumor markers or testosterone were elevated in 6/12 boys. Histology was intratesticular germ cell in 7, sexcord stromal tumor in 4 and capillary hemangioma in 1 patient. US correctly distinguished between benign and malignant lesions in all cases. When combined with clinical symptoms, US predicted 75% of histologies. After including hormone and tumor marker levels, a correct preoperative diagnosis was made for all boys with germ cell, and for 75% of boys with sexcord stromal tumor. CONCLUSION: Scrotal US is highly sensitive for the detection of childhood primary intratesticular tumors and, when combined with clinical data, highly reliable for differential diagnosis. It may help clinicians to decide when to opt for testis-sparing surgery.

[Subcutaneous annular granuloma: a differential diagnosis not to be forgotten in expansive lower leg (soft-tissue) lesions of young children]. / Subkutanes Granuloma annulare: eine nicht zu vergessende Differenzialdiagnose bei expansiven (Weichteil-) Läsionen an den unteren Extremitäten von jungen Kindern.

PURPOSE: Expansive lower leg lesions in children include numerous, even malignant, entities. Good differential diagnostic knowledge is extremely important in those conditions in order to prevent unnecessary biopsy. Therefore, our own observations of a not particularly rare pseudotumorous soft-tissue disease are presented and the value of ultrasonography is emphasized. MATERIALS AND METHODS: The imaging material (sonograms in all, MRI and radiograms in the first 4 and 3 patients, respectively) and the clinical charts of 7 young children (4 females, 3 males; aged 28 - 65 months) were analyzed retrospectively. RESULTS: All lesions were pretibial, firm, indolent, non-movable, and without inflammatory signs. They had developed spontaneously. Laboratory tests were normal. Ultrasound demonstrated all lesions located subcutaneously, homogeneously echopoor with indistinct borders and only little increased perfusion. On MRI they presented with low signal in T 1, high in T 2 and marked contrast enhancement. No intraosseous changes or calcifications were found. Histological proof in the first 2 patients disclosed subcutaneous annular granuloma. Based on an identical clinical and sonographic initial presentation, biopsy and additional imaging were dispensed in the later patients. Their clinical course with only sonographic controls was as expected. CONCLUSION: When dealing with expansive lower leg soft-tissue lesions in young children, the presented typical clinical and sonographic findings should give rise to consideration of the benign entity of subcutaneous annular granuloma and primary monitoring of those patients with only ultrasound.

[Infradiaphragmatic extralobar pulmonary sequestration]. / Infradiaphragmale extralobäre pulmonale Sequestration.

PURPOSE: To demonstrate characteristic imaging findings in infradiaphragmatic extralobar pulmonary sequestration (IEPS) with special emphasis on ultrasound (US). MATERIALS AND METHODS: The imaging material (pre- and postnatal US in all cases, magnetic resonance imaging (MRI) in 2 cases) for 4 infants (3 girls, 1 boy) was reviewed. 2 patients underwent surgery (after birth and at 4 months of age, respectively) and the diagnosis of IEPS was confirmed. The other 2 patients were monitored conservatively using US for up to 15 months. RESULTS: All 4 left-side suprarenal masses exhibited the same characteristic sono-morphology, leading to the suspected diagnosis of IEPS. The masses were small (max. 10 ml), hyperechoic with cystic components and without calcifications, well demarcated and separate from the normal kidney and the suprarenal gland, and without any change in prenatal and directly postnatal size. Doppler US showed low-grade perfusion in all cases and an aberrant systemic artery originating from the abdominal aorta in 2 cases. MRI did not add any fundamental information. Despite the suspected imaging diagnosis of IEPS and negative urinalyses for neuroblastoma, 2 patients underwent surgery for histological confirmation. The lesions in the other 2 patients were monitored via US. A complete disappearance after 4 months in one patient and a continuous decrease in size over 15 months in the other patient were documented. CONCLUSION: Based on the characteristic findings of prenatal and postnatal US, IEPS can be diagnosed reliably. With the knowledge of its benign spontaneously regressing behavior, suspected suprarenal lesions should be treated conservatively via US monitoring.

Parvovirus B19 infection associated with unilateral cervical lymphadenopathy, apoptotic sinus histiocytosis, and prolonged fatigue.

This report describes the case of a 16 year old girl with a history of high fever, prolonged fatigue, and cervical lymphadenopathy of the right side. In addition, the patient showed neutropenia, thrombopenia, and pronounced reticulopenia. Cervical ultrasound showed unilateral hypoechoic lymph nodes up to 23 mm in diameter suspicious for malignant lymphoma. Histology of a cervical lymph node specimen revealed massive nodular histiocytic proliferation and prominent apoptosis without necrosis. Parvovirus B19 was detected by polymerase chain reaction and immunohistochemistry in the lymph node. In summary, this case is an unusual presentation of parvovirus B19 infection. The virus was identified as the potential causative agent of unilateral cervical lymphoma and apoptotic sinus histocytosis, thus broadening the clinicopathological spectrum of parvovirus B19 induced diseases.

Use of non-ionic contrast media for tracheobronchography in neonates and young infants.

The paper describes the experience gained with tracheobronchography in 11 neonates and young infants with severe pulmonary disease--most of them on artificial respiration--using isotonic solutions of iopromide (17 series) and iotrolan (14 series). Both contrast agents were tolerated without any obvious problems when instilled into the lungs. Because of its higher content of iodine and greater viscosity, iotrolan was superior to iopromide as regards both the demonstration even of the more peripheral bronchial morphology and the duration of the contrast coating.

[Comparative x-ray and nuclear medical studies of osteosarcomas to evaluate the effectiveness of preoperative chemotherapy]. / Vergleichende röntgenologische und nuklearmedizinische Untersuchungen beim Osteosarkom zur Beurteilung der Effektivität einer präoperativen Chemotherapie.

In 16 patients with a long bone osteosarcoma treated after the therapy regimen of COSS 80/82 (Cooperative Osteosarcoma Study) the tumour response to preoperative chemotherapy was assessed by conventional roentgenograms (plain films, tomograms, angiograms). The histological grade of regression determined in the operative specimen was used as a reference for the extent of tumour devitalisation. By their different typical roentgenological course the osteosarcomas with a very good and those with a rather poor response could be correctly identified (100%). On following the clinically used differentiation between "good responders" (histological grades I to III) and "poor responders" (grades IV to VI) the corresponding classification of these tumours by roentgenograms and functional scintigraphic imaging was found to be correct in 94% of the cases. The coincidence of the correct as well as the false results in both radiological methods was as high as 100% if those osteosarcomas showing only a "medium response" in their roentgenograms were added to the clinical group of good responders. There were no fundamentally discrepant results obtained by each of the two radiological techniques. With the combined workup of the roentgenological and the scintigraphic material any incorrect preoperative estimation of tumour regression with a harmful influence on therapy (date and type of surgery, mode of postoperative chemotherapy) could be avoided.

[Comparison of x-ray and histological findings in osteosarcomas following preoperative chemotherapy]. / Vergleich röntgenologischer und histologischer Befunde bei Osteosarkomen nach präoperativer Chemotherapie.

Since the introduction of pre-operative chemotherapy, osteosarcomas have shown a more favourable prognosis. Reaction of the tumour due to chemotherapy is judged pre-operative primarily by radiology (plain films, angiography, CT, scintigraphy). There is little evidence concerning the radiological appearances after pre-operative chemotherapy and morphological changes, particularly in respect of tumour regression. Specific radiological changes were therefore compared with pathological findings following chemotherapy and operation in 17 patients with osteosarcomas. Tumours were examined which showed radiological evidence of intra-and extra-osseous sclerosis or lysis and which still were classified as vital tumour tissue. Tumour planes were reconstructed from large histological sections of the operative specimen and compared with the radiological appearances. Sclerosis was found to be due to reactive new bone formation or to mineralisation of the osteosarcomatous tissue. Lysis correlated with persistent vital tumour, or in connective tissue. Nine out of ten cases, regarded as vital on radiological evidence, showed vital tumour cells on histological section. Lyses and scleroses were not reliable indications of the pre-operative state of the osteosarcoma following chemotherapy. On the other hand, combined qualitative radiological criteria for assessing tumour vitality, proved to be helpful.

[Comparison of x-ray plain films, x-ray tomograms and computed tomograms in lung nodules in children and adolescents]. / Vergleich von Röntgenübersichtsaufnahmen, Röntgenschichtaufnahmen und Computer-tomogrammen bei pulmonalen Rundherden im Kindes- und Jugendalter.

A comparison was carried out of the value of plain radiographs, tomography and CT in 37 patients aged between five and 24 years. The majority of these patients had an osteosarcoma. Thirty-five CT examinations were performed on 16 patients and about 300 foci were demonstrated. Plain radiographs only showed 50%, and tomograms only 70% of these lesions. Surgery was carried out in 34 patients and there was agreement between the findings at thoracotomy and radiology in about half the cases. Frequently, more metastases were found at operation than could be demonstrated radiologically, in a few cases there were fewer.

[Experiences with a new film-screen system in pediatric thoracic radiography]. / Erfahrungen mit einem neuen Film-Folien-System in der pädiatrischen Thoraxdiagnostik.

PURPOSE: To compare the x-ray images made by the Kodak InSight Pediatric Imaging System (InSight P) with conventional film-screen systems in pediatric chest radiography. MATERIAL AND METHODS: The comparison involved chest radiographs made using Quanta-Fast-Detail/Cronex 4 (DuPont), Trimax 16/XDA (3 M) and DuPont UVR/UVL systems. The image quality of critical structures and the physical parameters of quantum interference, contrast and resolution were assessed. The energy path of the system was assessed by preparing density curves. Test conditions were in accordance with the latest guidelines of the Bundesärztekammer (German Physician's Association). RESULTS: The mediastinal area, retrocardiac and paravertebral spaces and the peripheral vessels of the lung were all displayed more distinctly using InSight P. The reason for this seems to be a lower degree of quantum interference associated with this system. With tube voltages between 60 and 80 kV, InSight P displayed a relatively low degree of sensitivity. CONCLUSION: InSight P can be used to produce predominantly high quality chest radiographs of infants between one and five years of age. However, this system has limited sensitivity in the tube voltage range recommended by the German Physicians' Association.

[Currarino syndrome: variability of imaging findings in 22 molecular-genetically identified (HLXB9 mutation) patients from five families]. / Currarino-Syndrom: Variabilität der bildgebenden Befunde bei 22 molekulargenetisch identifizierten (HLXB9-Mutation) Patienten aus fünf Familien.

PURPOSE: The imaging documents, obtained in connection with a primarily molecular genetic study on Currarino syndrome, should be evaluated with special respect to the constancy resp. the variability of findings in patients with proven HLBX9 mutations. METHODS: In five female non-related index patients with clinico-radiologically diagnosed Currarino syndrome and further 53 members of their families, changes of the homeobox gene HLXB9 had been analysed. Variable mutations of this gene were found in a total of 23 individuals including the five index patients. In 22 of them the preexisting radiological documents could be collected as well as further imaging (plain sacrococcygeal radiography and/or lumbosacral MRI at least) initiated. This was followed by a detailed evaluation of pathological findings in the os sacrum/coccyx as well as in the presacral, the intraspinal, the anorectal, and the urogenital region, finally. RESULTS: Imaging investigations revealed concomitant phenotypic abnormalities in all and even nine clinically asymptomatic individuals with proven HLXB9 mutations. A sacrococcygeal defect of varying intensity was depicted in every case. Complete Currarino triad (i. e. sacrococcygeal defect, presacral mass = anterior meningocele and/or tumor, anorectal malformation) was only found in the five index patients and three further relatives. In all other cases, one or more of the following anomalies were detected with variable combination and with decreasing frequency: anterior meningocele (12), presacral tumor (11), tethered cord (10), intraspinal lipoma (8), anorectal stenosis/atresia (8), syringocele (5), rectal fistula (3), urogenital (2). CONCLUSION: Currarino syndrome should be considered as a differential diagnosis in all patients with chronic constipation since early infancy and its imaging index finding, i. e. a sacrococcygeal defect, should be looked for with plain radiography, first. In positive cases or other phenotypic suspicious constellations molecular genetic analysis for HLBX9 mutations should be the next step. If positive again, this should be followed by complete adequate imaging in the patient as well as by plain sacrococcygeal radiography in, at least, symptomatic family members.

[Skull injury in childhood: comparison of ultrasonography with conventional X-rays and computerized tomography]. / Schädeltrauma im Kindesalter: Vergleich der Sonographie mit konventionellen Röntgenaufnahmen und der Computertomographie.

PURPOSE: The purpose of our study was to compare the value of ultrasound, conventional x-ray diagnosis and CT in detecting skull fractures and intracranial haemorrhage in children suffering from a head injury. MATERIAL AND METHODS: We examined 210 children who had a head injury. In all cases the calvarium was investigated by ultrasound using a 7.0 MHz linear transducer. In children with an open fontanel (n = 190) the cerebrum was screened additionally by ultrasound following a standard protocol. The sonographic findings were correlated to the x-ray examination (n = 21) and CT (n = 13). RESULTS: Ultrasound enabled diagnosis of linear calvarial fractures (n = 29), depressed fractures (n = 6) and intracranial haemorrhage (n = 8). X-Ray and CT examination confirmed the diagnosis of linear calvarial fractures in 16 cases, of depressed fractures in 6 cases. CT confirmed the sonographic diagnosis of intracranial haemorrhage in 8 cases. CONCLUSION: Ultrasound as a primary method can replace the conventional x-ray in detecting calvarial fracture and posttraumatic sequelae. Additional CT examination depends on the sonographic and neurological status.

[A rare location for extratruncular vascular malformation]. / Eine seltene Lokalisation einer extratrunkulären vaskulären Malformation.

We report on an undefined tumor of the right ulnar side of the elbow in a 15-year-old boy. The clinical examination showed painful swelling in the area of the ulnar nerve at the right elbow as well as sensory and motor deficits of the right hand corresponding to distribution of the ulnar nerve. Ultrasound examination and magnetic resonance imaging showed a tumor along the nerve. Subsequent surgical therapy included tumor decompression by division of the fascia. The histological examination demonstrated a capillary hemangioma that infiltrated the ulnar nerve.After surgery, oral glucocorticoid therapy with cortisone (5 mg/kg per day) was administered over a period of 4 weeks, alternating between 1 week of therapy and 1 week without medication. The result of this combined therapy was a rapid diminution of the tumor and an almost complete restitution of the neural function.

Ultrasound in abdominal and soft-tissue childhood PTLD (post-transplant lymphoproliferative disease).

AIM: Post-transplant lymphoproliferative disease (PTLD) is a rare condition, which should be well known to all paediatric medical facilities dealing with bone marrow and solid organ transplantation. The spectrum and the primary detecting modality of the initial imaging findings in paediatric transplant recipients with abdominal and soft-tissue PTLD should be studied retrospectively. METHOD: 7 children/adolescents (female: 4, male: 3; age: 3 - 19 yrs.; study period: 7 yrs.) after heart (5), kidney (1) or liver (1) transplantation were evaluated regarding their initial clinical and imaging findings of PTLD. RESULTS: 6 patients had a latent Epstein-Barr virus (EBV) infection. PTLD presented with clinical symptoms in only 5 patients (ileus: 2, soft-tissue swelling: 2, intussusception: 1) and was detected on routine abdominal ultrasound (US) controls in the remaining patients. US was the primary imaging modality in all children and led to suspecting PTLD in 6 patients. In the seventh case, US had been misinterpreted first. Compared to US, additional magnetic resonance imaging (MRI) and/or computed tomography (CT) better demonstrated the extent of the disease in 3 children, but were even inferior in another 3. There was no completely false-negative US examination during the study period. CONCLUSION: US is reliable for detecting as well as excluding abdominal and soft-tissue PTLD in paediatric patients after solid-organ transplantation and might even be superior to MRI/CT. Therefore, all patients with an increased risk of developing PTLD should be closely monitored by ultrasound. MRI/CT may be reserved for supplementary imaging in cases incomplete or equivocal on US, but are primarily essential in all patients with a localisation of PTLD not accessible by US.

[DeBarsy-Moens-Dierckx-syndrome (author's transl)]. / DeBarsy-Moens-Dierckx-Syndrom: Beobachtung bei Geschwistern

The symptoms of DeBarsy-Moens-Dierckx-syndrome previously described only in single patients, have been observed in a brother and a sister. Both children showed dwarfism, dystrophy, oligophrenia, an old looking face, and a typically lax skin and cloudy cornea as signs of degeneration of the elastic tissue. Moderate pathological changes were also found in the electroencephalogram, the echoencephalogram, and the X-ray films of the wrists. The analysis of the chromosomes and many other laboratory data were normal. The family-history gave no clue as far as the heredity mode is concerned. As far as we know this is the first description of this syndrome in siblings.