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Biomedical subjects

F M Vanhoenacker

Publications and source records attributed to F M Vanhoenacker.

At least 19 recordsLinked to original sources
PubMed2003

Camurati-Engelmann disease. Review of radioclinical features.

PURPOSE: To present a retrospective overview of the clinical and radiological features of Camurati-Engelmann disease (CED) in a large family with genetically proven CED. MATERIAL AND METHODS: Clinical features and imaging studies were available in 8 affected patients out of a large Jewish-Iraqi family with 21 affected members in four generations. The patients' ages ranged between 7 and 44 years. RESULTS AND CONCLUSIONS: The most frequent symptoms were pain and muscle weakness accompanied by waddling gait. Two patients were asymptomatic. Radiologically, the disease can be classified as a craniotubular hyperostosis. Typically, fusiform thickening of the diaphyseal portions of the long bones was seen in all 8 patients, but in 1 patient, metaphyseal involvement was observed as well. Radioclinical abnormalities were most often detected before the age of 30, and were usually more extensive at older age. Radiological abnormalities may precede the clinical signs. Concomitant broadening of the diaphyses of long bones and narrowing of the medullary canal suggest that both an excessive periosteal apposition of bone and a defective resorption of bone at the endosteal side of the long bones exist.

Adolescent↗
PubMed2002

Imaging of pulmonary tuberculosis.

Tuberculosis, more than any other infectious disease, has always been a challenge, since it has been responsible for a great amount of morbidity and mortality in humans. After a steady decline in the number of new cases during the twentieth century, due to improved social and environmental conditions, early diagnosis, and the development of antituberculous medication, a stagnation and even an increase in the number of new cases was noted in the mid-1980s. The epidemiological alteration is multifactorial: global increase in developing countries; minority groups (HIV and other immunocompromised patients); and elderly patients due to an altered immune status. Other factors that may be responsible are a delayed diagnosis, especially in elderly patients, incomplete or inadequate therapy, and the emergence of multidrug-resistant tuberculosis. The course of the disease and its corresponding clinicoradiological pattern depends on the interaction between the organism and the host response. Classically, pulmonary tuberculosis has been classified in primary tuberculosis, which occurred previously in children, and postprimary tuberculosis, occurring in adult patients. In industrialized countries, however, there seems to be a shift of primary tuberculosis towards adults. Furthermore, due to an altered immunological response in certain groups, such as immunocompromised and elderly patients, an atypical radioclinical pattern may occur. The changing landscape, in which tuberculosis occurs, as well as the global resurgence, and the changed spectrum of the clinical and radiological presentation, justify a renewed interest of radiologists for the imaging features of pulmonary tuberculosis. This article deals with the usual imaging features of pulmonary tuberculosis as well as the atypical patterns encountered in immunodepressed and elderly patients.

Adult↗
PubMed2002

Tuberculosis of the central nervous system: overview of neuroradiological findings.

This article presents the range of manifestations of tuberculosis (TB) of the craniospinal axis. Central nervous system (CNS) infection with Mycobacterium tuberculosis occurs either in a diffuse form as basal exudative leptomeningitis or in a localized form as tuberculoma, abscess, or cerebritis. In addition to an extensive review of computed tomography and magnetic resonance features, the pathogenesis and the relevant clinical setting are discussed. Modern imaging is a cornerstone in the early diagnosis of CNS tuberculosis and may prevent unnecessary morbidity and mortality. Contrast-enhanced MR imaging is generally considered as the modality of choice in the detection and assessment of CNS tuberculosis.

Humans↗
PubMed2001

Hepatosplenic antracosilicosis: a rare cause of splenic calcifications.

A case of a 62-year-old man with known longstanding pulmonary antracosilicosis, with associated hepatosplenic antracosilicosis, is presented. A CT scan of the upper abdomen revealed multiple calcifications within the spleen, and to a lesser degree within the subcapsular region of the liver, as well as "egg-shell" calcifications of abdominal lymph nodes, most noticeable at the splenic hilum. Although histopathologically not proven, the similar appearance of the calcified hepatosplenic nodules to the small round calcifications scattered throughout the lungs, as well as the typical "egg-shell" morphology of the calcified abdominal lymph nodes, should raise the suspicion of hepatosplenic antracosilicosis.

Anthracosilicosis↗
PubMed2001

Thyroid acropachy: correlation of imaging and pathology.

Thyroid acropachy is a rare manifestation of autoimmune thyroid disease, in the form of soft tissue swelling of the hands and feet with insidious onset, associated with clubbing and characteristic periosteal reactions. It is usually part of a syndrome consisting of a typical triad of thyroid acropachy, exophthalmos, and pretibial myxedema. The purpose of this case report is to demonstrate the imaging features of this typical triad in a 65-year-old-woman. This case is the first in which the MRI features of thyroid dermopathy are documented.

Aged↗
PubMed2001

Congenital skeletal abnormalities: an introduction to the radiological semiology.

Despite the recent advances in the molecular diagnosis of congenital abnormalities, the initial identification and the decision to refer a patient for further molecular analysis and expensive genetic tests still relies frequently on clinical and radiological criteria. The radiological identification of syndromes, dwarfs and dysplasias is a difficult task, because there are so many findings to consider and so many syndromes to remember that the problem is overwhelming. There is a definite need for an easy and systematic analysis system, in order to try to categorize a skeletal dysplasia in a certain group. In this brief review, we suggest an approach to the evaluation of skeletal syndromes, based on the analysis of cardinal criteria, from which the most useful information is derived, and additional criteria, making further differentiation possible. Generally, cardinal information is derived from analysis of the long bones, hands, pelvis and the spine, whereas the analysis of other skeletal elements, like the skull, feet, and other flat bones is of additional value.

Bone Diseases, Developmental↗
PubMed2001

Hereditary multiple exostoses: from genetics to clinical syndrome and complications.

OBJECTIVE: To give an overview of genetic, clinical and radiological aspects in two families over four generations with known hereditary multiple exostoses (HME). METHODS AND MATERIAL: After linkage analysis in both families to localize the defective gene, mutation analysis was performed in these genes to identify the underlying mutation. In the 31 affected individuals, location, number and morphology and evolution of exostosis, evolution of remodeling defects at the metaphysis, and the extent of possible complications were evaluated on clinical and imaging (plain radiography, computed tomography (CT), and magnetic resonance imaging (MRI)) data over a lifetime period. RESULTS AND CONCLUSIONS: Both families demonstrate the gene defect in the same EXT-2 gene locus on chromosome 11p. Exostoses are preferentially located in the lower extremity (hip, knee and lower leg), humerus, and forearm. Any other bone may be involved, except for the calvaria of the skull and the mandible. Exostoses are rather sessile than pedunculated. Exostosis is rarely present at birth but develops gradually and may persist to grow slowly after closure of the growth plates. Preferential expression of the remodeling defect was seen in the hip, distal femur (trumpet-shaped metaphysis) and forearm (shortening of the ulna with secondary bowing of the radius and development of a pseudo-Madelung deformity). These radiological manifestations start at the age of 4-5 years and become more obvious as the enchondral bone formation progresses with age. Reported complications in these families consist of local entrapment phenomenons (vessel, tendon, nerve), frictional bursitis, and sarcomatous transformation. MRI was able to suggest these complications and is the imaging technique of choice in the evaluation of symptomatic exostoses.

Chromosome Mapping↗
PubMed2001

Heterotaxy syndrome in an adult, with polysplenia, visceral and cardiovascular malposition.

A rare case of an asymptomatic heterotaxy syndrome associated with dextrocardia is presented in a 45-year-old woman. This anomaly was incidentally discovered on computed tomography and ultrasound of the upper abdomen. Besides the presence of multiple abnormally positioned spleens, right-sided stomach, left-sided liver, short pancreas and venous anomalies, like interrupted left-sided inferior vena cava with azygos continuation, and dextrocardia were seen. Although cardiac abnormalities are frequently associated with heterotaxy syndromes with polysplenia in children, this is far less frequent in adults because of early decease in children with cardiac abnormalities. To the best of our knowledge, this is the third reported case of heterotaxy with polysplenia associated with dextrocardia in an asymptomatic adult patient.

Female↗
PubMed2001

MR imaging anatomy of the knee.

MRI of the knee joint is one of the most common and important clinical applications in most MRI units today. Thorough knowledge of the detailed anatomy is a prerequisite for the understanding of knee pathology. This article reviews the MR anatomy of menisci, the cruciate ligaments, the medial and lateral supporting structures, the extensor mechanism, synovial plicae around the joint and some pitfalls in the interpretation.

Humans↗
PubMed2000

Proteus syndrome: MRI characteristics of plantar cerebriform hyperplasia.

Proteus syndrome is a rare congenital hamartomatous syndrome with a variety of abnormalities. It shares many features with other congenital hamartomatous disorders, but cerebriform hyperplasia of the soles and the palms is known as a quite distinctive characteristic in the dermatologic literature. The purpose of this case report is to demonstrate the MRI features of plantar cerebriform hyperplasia in a 9-year-old boy with known Proteus syndrome.

Child↗
PubMed2000

Desmoplastic fibroma of bone: MRI features.

Desmoplastic fibroma of bone is a very rare benign tumor, which may be locally aggressive. In contrast to the well-documented radiological appearance, the literature on MR imaging features of this tumor is scarce. The MR imaging characteristics in our case are compared to those previously reported. Although there is a considerable overlap in the MR imaging features with other bone tumors, an interesting MR feature of desmoplastic fibroma is the presence of low to intermediate signal intensity foci on T2-weighted images, which radiographically does not correspond to calcifications. This feature may help narrow the differential diagnosis.

Adult↗
PubMed2000

Sclerosing bone dysplasias: genetic and radioclinical features.

Although knowledge of basic genetics in the field of sclerosing bone dysplasias is progressing, the radiologist still plays a pivotal role in the diagnosis of this relatively poorly understood group of disorders. Based on a target site approach, these anomalies are classified into three groups. Within each group, further differentiation can be made by distinctive clinical findings and by mode of inheritance: (a) dysplasias of endochondral bone formation: osteopetrosis (Albers-Schönberg disease), pycnodysostosis, enostosis, osteopoikilosis, osteopathia striata (Voorhoeve disease); (b) dysplasias of intramembranous bone formation: progressive diaphyseal dysplasia (Camurati-Engelmann disease) and variants, hyperostosis corticalis generalisata (Van Buchem disease) and variants; and (c) mixed sclerosing dysplasias: melorheostosis (Leri disease) and overlap syndromes.

Bone Diseases, Developmental↗