Biomedical subjects
Publications and source records attributed to B Desprechins.
A case of a functioning double aortic arch is presented in a 4-month-old boy with recurrent respiratory infections and poor feeding. An esophagogram, Doppler echocardiography and aortography were performed. We describe the typical findings of this rare disorder.
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We report a successful endoscopic obliteration of a large recurrent tracheoesophageal fistula (diameter 0.6 cm, length 2.0 cm) in a 12-year-old girl, using a combination of Histoacryl (n-butyl-z-cyanoacrylate) and Aethoxysclerol injected through a polyethylene catheter. The severe pulmonary infection, which rendered surgery potentially life threatening, disappeared after the endoscopic closure. Since the obliteration, now over 12 months ago, the girl is asymptomatic. Endoscopic obliteration is a worthwhile technique and should be considered as an alternative to surgery in patients presenting with a complicated recurrent tracheoesophageal fistula.
Infected right atrial thrombosis is an uncommon but severe complication of the use of central indwelling catheters in children. Massive thrombi around a right atrial catheter were seen in two critically ill premature infants after 3 weeks of parenteral nutrition. A catheter-related sepsis had previously occurred and had been treated by antibiotics. Subsequently, protracted thrombocytopenia, fluctuating hepatomegaly, and increasing respiratory distress were found in both cases. A right atrial mass was detected by 2 dimensional echocardiography. Cardiotomies for removal of the infected thrombus were performed with success in both cases. In one infant, weighing only 900 gm, surgical removal was accomplished with the aid of inflow stasis. Premature infants with catheter-related sepsis appear at greater risk for intracardiac thrombosis and should undergo echocardiography as part of their evaluation. Infected atrial thrombi can be safely removed by cardiotomy even in the very small premature infant.
We report a case of ossifying fibroma of the long bones (OFLB). This lesion, occurring mainly during the first decade of life, typically affects the tibial diaphysis and clinically presents as a painless bony enlargement. Radiographic features include an osteolytic cortical expansion, containing diffuse calcifications, and separated from the medullary by a thick cortical rim. CT appears to be the examination of choice to define these signs and permits differentiation from monostotic fibrous dysplasia and from adamantinoma (by demonstrating the presence or absence of diffuse high attenuation values). The importance of making the differential diagnosis is stressed, as prognosis and management of OFLB, fibrous dysplasia and adamantinoma may vary considerably.
In recent years, *I-MIBG (*I-metaiodobenzylguanidine), which is transported and stored in the chromaffin cells, has been shown to allow good visualization of neuroblastomas in children. This paper deals with 30 *I-MIBG-scans performed in 20 children: 16 with neuroblastoma, 3 with retinoblastoma, and 1 with a malignant paraganglioma. A high detection rate was found for both primary and secondary sites of neuroblastoma. *I-MIBG was generally superior to 99mTc-MDP bone scintigraphy in the detection of bone metastases. Our experience illustrates the unique place of *I-MIBG-scintigraphy compared with other imaging techniques: it makes it possible to define the nature of the tumour, particularly in cases with normal catecholamine levels; to establish how extensive the lesions are at the time of diagnosis; and to confirm complete remission. No abnormal *I-MIBG uptake was noted in the 3 cases of retinoblastoma.
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CT-scan was used to examine rhinosinusitis in the developing sinuses; 196 children aged from 3 to 14 years were selected on the base of their chronic rhinorrhea, nasal congestion and cough. The patients were subdivided into six age groups (3-4, 5-6, 7-8, 9-10, 11-12 and 13-14 years). In the youngest age group, the authors noted maxillary involvement in 63%, ethmoidal involvement in 58%, and even sphenoidal sinus involvement in 29% of the children. Involvement decreased gradually with age, with 10% of ethmoidal and 0% of sphenoidal involvement in the 13-14 years age group. Maxillary sinusitis, however, persisted very frequently in the oldest age group (65%). Frontal involvement seems to become significant at the age of 7-8 years (7%) but it never exceeds 15% (11-12 age group). Septal deviations occurred in 16% of the youngest up to 72% in the oldest age group. The prevalence of bullous conchae increased with age too, although less prominently.
Since the early eighties, *I-MIBG (= *I-metaiodobenzylguanidine), which is stored in the neurotransmitter storage granules of chromaffin cells, has been increasingly used for the detection of pheochromocytomas and allied sympathoadrenal pathologies. The aim of this paper is to illustrate, by means of clinical examples, the role of *I-MIBG-scintigraphy in child neuroblastoma and to underline its original place, compared with other imaging techniques, in determining the neuro-ectodermal origin of a tumor as well as in establishing the extension of the lesions at the time of diagnosis and during follow-up.
Cortical, subcortical and subependymal tubers were more completely and more clearly depicted by MR than by CT and US in a 3-week-old infant. These lesions were best shown on T1-weighted images as areas of high signal intensity.
CT-scan was used to examine rhinosinusitis in the developing sinuses; 196 children aged from 3 to 14 years were selected on the base of their chronic rhinorrhea, nasal congestion and cough. The patient group was subdivided into six age groups (3-4, 5-6, 7-8, 9-10, 11-12 and 13-14 years). In the youngest age group, the authors noted maxillary involvement in 63%, ethmoidal involvement in 58%, and even sphenoidal sinus involvement in 29% of the children. Involvement decreased gradually with age, with 10% of ethmoidal and 0% of sphenoidal involvement in the 13-14 years age group. Maxillary sinusitis, however, persisted very frequently in the oldest age group (65%). Frontal involvement seems to become significant at the age of 7-8 years (7%) but it never exceeds 15% (11-12 age group). Septal deviations occurred in 16% of the youngest up to 72% in the oldest age group. A prevalence of bullous conchae increased with age too, although less prominently.
In this study the potential of thoracic subtraction fluoroscopy has been evaluated on the basis of our experience with this functional imaging modality in patients presenting with cystic fibrosis. The method, which consists of subtracting dynamic digital fluoroscopic images of the breathing chest in the time interval difference (TID) mode, applies to the study of the lucency variations during the respiratory cycle and provides dynamic functional information about ventilation and/or perfusion and diaphragmatic kinetics, without any injection and with minimal irradiation. To assess the validity of the TID procedure, our results were compared with standard roentgenography, clinical data, and scintigraphic findings, with emphasis on the latter. The limitations and advantages of TID compared with scintigraphy are discussed. Three groups of discrepancies emerge which do not discredit TID. TID offers rather the advantage of providing anatomic pictures and unique dynamic information, which is emphasized.
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Two unusual patients with sensory-induced seizures are presented. The clinical approach and evolution of seizures induced by bathing in patients with and without accompanying epilepsy are discussed.
Synovial sarcoma of the retropharynx is an extremely rare neoplasm. To date, only 10 cases have been reported in patients less than 15 years old. Our 15-month-old patient is, to our knowledge, the youngest ever reported with a retropharyngeal synovial sarcoma in the world literature. In the past, treatment usually consisted of surgery followed by radiotherapy or adjuvant chemotherapy, or both. In the case reported here, combination chemotherapy (ifosfamide, vincristine, and actinomycin D), used as the initial treatment modality, induced a dramatic decrease of the synovial sarcoma.
The purpose of this work was to evaluate bone marrow abnormalities in the lower limbs of patients with cystic fibrosis by means of MR imaging. Eight patients with cystic fibrosis ranging in age from 16 to 35 years (average age 25.1 years) were evaluated with MR imaging of the lower extremities. T1 weighted spin echo sequences were obtained in all patients. Pelvis, femora and tibia were imaged in the coronal plane whereas the feet were imaged in the sagittal plane. The studies were independently evaluated by two musculoskeletal radiologists. The images were not mixed with disease-free images, but the readers were completely unaware of clinical data. Distribution of haematopoietic and fatty marrow was graded on a 5-point scale. In five patients, both observers considered marrow distribution as probably or definitely abnormal relative to their age. Although our findings are very preliminary, our investigation suggests that cystic fibrosis may be added to the list of disorders in which bone marrow abnormalities may be observed.