Susanna Vogt

Comparison of whole-body STIR-MRI and 99mTc-methylene-diphosphonate scintigraphy in children with suspected multifocal bone lesions.

The study was performed to compare whole-body short time inversion recovery (STIR) MR imaging and 99mTc-methylene diphosphonate planar scintigraphy in the examination of children with suspected multifocal skeletal malignant lesions. Sixteen patients with known or suspected malignant skeletal disease underwent both whole-body STIR MR imaging and bone scintigraphy. The lesions were described and numbered according to scintigraphic evaluation criteria. Thus, 16 regions were analyzed in each patient for the comparison between the two modalities. Histology was proven in the primary malignant regions. Follow-up MRIs were registered. Scintigraphy and MRI follow-up were evaluated as gold standard. A total of 139 different lesions was observed by both modalities. Baseline whole-body MRI revealed 119 bone lesions in 256 possible sites (46.5%); scintigraphy revealed only 58 lesions (22.6%). Congruence was observed in only four patients (25%). According to the location of the lesion, correlation was observed in 39/139 lesions (28%). In all, 57.5% of the lesions were detected only by MRI and 14.5% of the lesions were detected only by scintigraphy. Whole-body MRI was more sensitive (P<0.001). Of all lesions numbered which could be separated in the initial MRI, whole-body MRI detected 178 lesions in the patients. The results suggest that whole-body MRI using a STIR sequence is an effective radiation free method for examination of children with suspected multifocal bone lesions. MRI showed more lesions than conventional 99mTc-methylene diphosphonate scintigraphy. Therefore, whole-body MRI may be feasible as a screening modality for metastatic and skip lesions in osteosarcoma, PNET, Ewing sarcoma and Langerhans cell histiocytosis in children.

Pediatric brain MRI in neurofibromatosis type I

Neurofibromatosis (NF) is the most common of the phakomatoses, with a prevalence of 1 in 3–4,000. Many organ systems can be affected. In addition to multiple peripheral neurofibromas, NF I predisposed to CNS tumors including optic glioma, astrocytoma and plexiform neurofibroma. The purpose of this pictorial review is to illustrate characteristic brain MR imaging lesions in children with NF I and to give some recommendations about diagnostic imaging procedures in children suffering from NF I. Typical findings in brain MRI are hyperintense lesion on T2-weighted images, so-called unknown bright objects, which may be useful as an additional imaging criterion for NF I. Contrast administration is necessary in MR studies to maximize tumor detection and characterization, to add confidence to the diagnosis of benign probable myelin vacuolization, and to document stability of neoplasm on follow-up examinations. We recommend to perform serial MR imaging in children every 12 months. The frequency of follow-up in children with known brain tumors will vary with the tumor grade, biological activity and treatment.

Voiding urosonography with ultrasonography contrast medium in children

Voiding urosonography (VUS) with ultrasonography contrast medium is a new modality in the detection of vesicoureteral reflux (VUR) in children. The purpose of this study was to evaluate the diagnostic accuracy of VUS compared with voiding cystourethrography (VCUG) in the detection of VUR. One hundred and eighteen patients, aged 3 weeks to 16 years, with 234 ureterorenal units, were investigated by VUS with ultrasonography contrast medium and radiographic VCUG in one session. The indications were predominantly urinary tract infection and follow-up of previously detected VUR. During the sonographic examination, the bladder was filled with saline solution and an ultrasonography contrast medium (Levovist) was administered through a catheter. Reflux was diagnosed when echogenic bubbles were observed in the ureter or in the renal pelvis. Afterwards, conventional VCUG was performed. Concordant findings were obtained in 210 of 224 ureterorenal units (93.7%). Reflux was excluded by both methods in 174 units (77.7%). With the VCUG as the reference, the sensitivity of VUS was 90%, the specificity 94.6%, the positive predictive value 78.3%, and the negative predictive value 97.8%. The accuracy was 93.7%. In conclusion, VUS with ultrasonography contrast medium is a reliable diagnostic tool for the detection of VUR in children.

US and MRI of gastrointestinal graft-versus-host disease

Abstract. Abdominal problems often complicate the clinical course after bone marrow transplantation. Graft-versus-host disease occurs as a complication of allogenic bone marrow transplantation. In this report, the findings of intestinal involvement are described and correlated with histopathological findings. Increased bowel-wall thickness and increased vascularity were shown by US. MRI demonstrated generalised increased bowel-wall thickness associated with bowel-wall enhancement after administration of IV gadolinium.

Vascular complications (splenic and hepatic artery aneurysms) in the occipital horn syndrome: report of a patient and review of the literature

Abstract We report an 18-year-old boy with occipital horn syndrome who developed aneurysms of the splenic and hepatic arteries. Occipital horn syndrome, also called X-linked cutis laxa or Ehlers-Danlos syndrome (EDS) type IX, is characterised by a skeletal dysplasia which includes occipital horns, broad clavicles, deformed radii, ulnae and humeri, narrow rib cage, undercalcified long bones and coxa valga. Distinctive features common to all patients are unusual facial appearance, hypermobility of finger joints, limitation of extension of elbows, chronic diarrhoea and genitourinary abnormalities. In this case report we describe the difficulties encountered in the diagnostic management of patients with EDS-related vascular lesions.

Intra-abdominal lymphangioma in a newborn.

We report the case of a male newborn infant with an intra‐abdominal cavernous lymphangioma that produced acute abdominal symptoms. Sonography showed a cystic mass with thin septations, which caused intestinal obstruction and stenosis of the iliac artery. The histologic findings after surgical resection reflected the sonographic appearance of the mass. The differential diagnosis of cystic abdominal lesions is discussed. Sonography is the preferred method of diagnosis.

High-flow priapism in acute lymphatic leukaemia

Priapism is defined as prolonged and persistent erection of the penis without sexual stimulation. It is associated with excessive hyperleukocytosis (e.g. in acute or chronic leukaemia); however, this complication is rarely seen in the pediatric population. We report a 12-year-old boy suffering from acute leukaemia presenting with, at first intermittent, but increasingly persistent erection. Doppler US revealed signs of high-flow priapism. MRI excluded intrapelvic tumour masses, and three-dimensional contrast-enhanced MR angiography could not demonstrate an arteriovenous fistula or thrombosis. Cavernosal blood-gas measurement was in agreement with high-flow priapism. On the basis of the imaging findings, invasive therapeutic management was avoided in our patient with a successful outcome.

X-linked forms of chondrodysplasia punctata: Re-description of two cases originally reported by Professor Erich Hässler in 1940 and the knowledge six decades later

Six decades ago, Professor Erich Hassler, one of the oldest still living European pediatricians who celebrated his 103 t h birthday in April 2002, described two patients with chondrodysplasia punctata. He performed profound clinical, radiological and histological studies of a female neonate with severe X-chromosomal dominant chondrodysplasia punctata type Conradi-Hunermann and investigated an 8-year-old male with a probably X-linked recessive form of chondrodysplasia punctata. He compared these patients with 7 previously described cases also accompanied by stippled epiphyses. To honor the personality of Professor Hassler, both cases were repeated again with his consent. These case reports are connected with the recent knowledge about the molecular background of chondrodysplasia punctata. The X-linked types of chondrodysplasia have been distinguished from other diseases also characterized by stippled epiphyses.

Büschelartiges Hämangiom (“tufted angioma”) des Unterarms Fallbericht und Literaturübersicht

ZusammenfassungHintergrund: Das büschelartige Hämangiom (“tufted angioma”) ist ein benigner vaskulärer Tumor der Haut, dessen Kenntnis in der Differenzialdiagnose zu aggressiveren und malignen Tumoren wichtig ist. Fallbericht. Es wird über ein 8 Monate altes Mädchen mit diesem Tumor im Unterarmbereich berichtet. Sonographie und Magnetresonanztomographie zeigten einen gut vaskularisierten Weichgewebetumor, bei dem aufgrund interseptaler Ausbreitung ein maligner Prozess nicht ausgeschlossen werden konnte. Die Histologie der Inzisionsbiopsie ergab ein zellreiches kapillares Hämangiom, dessen morphologische Veränderungen eine Einordnung als ein seltenes büschelartiges Hämangiom erlaubten. Diskussion. Eine Abgrenzung des büschelartigen Hämangioms gegenüber aggressiveren und malignen vaskulären Läsionen der Haut ist aufgrund des typischen histologischen Bilds möglich, während eine Differenzierung mit Hilfe bildgebender Verfahren nicht gelingt.AbstractBackground: Tufted angioma represents a benign vascular lesion of the skin. The knowledge of this distinctive entity is important in distinction of clinically more aggressive and malignant neoplasms respectively. Case report. We report a 8-months-old girl who developed a deep seated tufted haemangioma on her right forearm. Sonography and MRI showed a good vascularized tumor with interseptal spreading. Histological examination of the incisional biopsy revealed a cellular capillary haemangioma with morphological features of rare tufted haemangioma. Discussion. Characteristic morphological features of tufted haemangioma allow accurate diagnosis and differential diagnosis to clinically more aggressive and malignant neoplasms of skin and soft tissues, wheras radiological methods are rather unspecific and do not distinguish reliably between these vascular lesions. It is of special clinical importance that tufted haemangioma represents an enlarging but biologically benign vascular neoplasm.

Klinik, Radiologie, Genetik und Therapie hereditärer Skeletterkrankungen Ein historischer Überblick anläßlich des 100. Geburtstages von Professor Erich Häßler

In diesem Beitrag werden klinische Symptomatik, Radiologie, Genetik und die Therapie spezieller Skeletterkrankungen, soweit möglich unter Bezugnahme auf eigene Patienten, diskutiert. Ausgewählt sind hier die Mucopolysaccharidose Typ I-S (Scheie-Syndrom), das Okzipitalhorn-Syndrom und die autosomal-rezessiv erbliche Osteopetrose. Die Arbeit ist Herrn Professor Erich Häßler gewidmet, der 1999 seinen 100.Geburtstag beging. Professor Häßler war von 1953 bis 1965 Direktor der Jenaer Kinderklinik und beschäftigte sich wissenschaftlich mit verschiedenen Knochenerkrankungen wie der Osteopetrose, der Chondrodysplasia punctata und der Mucopolysaccharidose I-H (Hurler- Syndrom).