Total intravenous hyperalimentation (TIH) complications in childhood: a radiological survey

Sixty consecutive patients, except for one 7-year-old patient, their ages varying from 1 day to 12 months received total intravenous hyperalimentation (TIH). It was possible to observe non-pathological catheter malpositions and, in 14 patients, other complications. The most serious complications observed included the following: thrombus and pericatheter thrombus calcification; superior vena cava thrombotic occlusion; hydrothorax; mediastinal effusion; generalized septic arthritis; venous transfixion with flooding of the soft tissues of the neck; vein wall lesions; collateral vertebral circulation; and a catheter tip blocked in vein. A brief comment is given on the use of this procedure and what may happen. This paper was given at the 18^th E.S.P.R. Meeting in Oslo, on May 22nd, 1981     

Diagnosis of hypochondroplasia: the role of radiological interpretation

Background. Hypochondroplasia is characterised by phenotypic and genetic heterogeneity. Differentiation from other conditions with disproportionate short stature is often difficult. Objective. To determine the reliability of radiological interpretation in the diagnosis of hypochondroplasia and to evaluate the most typical skeletal abnormalities. These data were correlated with molecular findings. Materials and methods. We enrolled 21 patients with suspected hypochondroplasia based on the radiological criteria most often reported in the literature on this disease. Height, sitting height and head circumference were measured in all patients. Radiographs of the lumbar spine, left leg, pelvis and left hand were obtained. The presence of the N540K mutation in the fibroblast growth factor receptor 3 (FGFR3) gene was verified by restriction enzyme digestion. All radiographs which enabled the selection of patients were reviewed a second time by two paediatric radiologists in a blinded examination. Their results were compared. Results. Both radiologists confirmed the diagnosis in 10 out of 21 patients, while in the other 52 % of cases they excluded the disease, were uncertain or they did not agree on the final interpretation of the data. The best agreement rate was obtained in the evaluation of the lumbar spine and the legs. The radiological features of the nine patients (43 %) carrying the N540K substitution were not remarkably different from the ones reported in the patients without this mutation. Conclusion. Our study shows that the crucial skeletal regions on which to focus the diagnosis of hypochondroplasia are the lumbar spine and legs, while the pelvis and hands seem to be less characteristic. To reduce the risk of misdiagnosis, accurate radiological and clinical evaluation is needed, especially in cases without a defined genetic defect. Received: 15 November 1999 Accepted: 8 May 2000     

Acute appendicitis in a premature baby

A case of acute appendicitis in a premature baby in whom diagnosis was suggested on plain films of the abdomen is presented. In this baby air in a hollow viscus suspected of being an enlarged appendix was the clue to diagnosis. The diagnostic dilemma of this rare and life-threatening condition in premature babies and newborns is underlined. The relevance of different imaging modalities and of different findings in this age group is discussed. Awareness of this rare condition and possible differential diagnosis in newborns and premature babies is stressed.     

A review of the actual knowledge of the processes governing growth and development of long bones

Autoptic samples of human bones (from 8 weeks of gestation to 12 years of age) and a second group of serial, skeletal x-rays (required for pathologies not related to bone dysplasia in children from 4 months to 17 years of age) provided the material for the analysis of the physes normal growth mechanism presented in this review. Before the appearance of the ossification centers epiphyseal growth rests exclusively on chondrocytes proliferation (interstitial growth), without any detectable differentiated cellular organization. When endochondral ossification starts a defined spatial disposition of chondrocytes and a corresponding organization of the intercellular matrix is set up, so that it is possible to identify a growth vector corresponding to the columns of piled chondrocytes with direction from hypertrophic toward the proliferative cell layers. The complexity of the tubular bones growth process is well represented by the spatial arrangement of the growth vectors. In the late epiphyseal growth another mechanism is active in addition to endochondral ossification, namely, articular cartilage interstitial growth and subchondral remodelling. The knowledge of the normal mode of organization of the physis and its temporal sequence can help to better understand of the deviaton from the normal development of metaphyseal and epiphyseal dysplasias.