Biomedical subjects
Publications and source records attributed to M Panuel.
Splenic hemangioma is a very rare neoplasm in children. We report three cases of splenic hemangioma, two of solitary hemangioma and one of hemangiomatosis. Two patients had hematologic disorders related to hemangioma. Imaging findings of this entity are presented and discussed with emphasis on pathologic correlation.
The results of MRI and MIBG scintigraphy performed on the spine of 14 children with neuroblastoma are reported. In 6 cases of diffuse spinal bone marrow tumor infiltration, diagnosis is easier with MIBG scintigraphy than with MRI. In 5 cases, MRI detected hyposignal of the vertebral body without any spinal abnormality on MIBG scintigraphy. A discussion of the reasons for negative MIBG scintigraphy is presented and in these 5 cases, it is suggested that a lateral view of MIBG scintigraphy and HMDP-Tc99m scintigraphy may be performed, even vertebral body biopsy in order to assess bone marrow tumoral infiltration.
Three pediatric cases of malignant rhabdoid tumor of the neck are described. Clinical data and imaging findings (US, CT and MRI) are stressed. The mass was well defined, containing punctate calcifications in two cases and encasing the vessels in two other cases. Two patients were treated with a chemotherapy regimen according to MMT 89 SIOP protocol, one had chemotherapy and radiotherapy. Two children died of progressive disease; the remaining child who had complete surgical removal of the tumor is on remission 17 months after diagnosis.
An open-label noncomparative clinical study was conducted to evaluate the efficacy and tolerance of ioversol (Optiray 300), a new low-osmolality non ionic contrast medium, in body contrast-enhanced CT scanning in infants and children. Fourty pediatric patients who required contrast-enhanced CT scanning for evaluation of chest, abdominal or pelvic masses participated in the study. The vascular enhancement was judged to be excellent or good in 72.5% of the cases and the CT scans were judged to be diagnostic in 95% of the cases. Ioversol was well tolerated in the study with only two mild adverse reactions (nausea and vomiting, and metallic taste).
The diagnosis of Ewing's malignant tumor in the young still raises major problems, either from a clinical point of view because of its rarity, its pluri-potentiality and various symptoms, or on imaging because of its numerous pitfalls. Accordingly the disease is often misdiagnosed as osteomyelitis. Only a high quality biopsy can determine histological diagnosis of undifferentiated small round cell tumor. The chromosomic study shows a specific (11;22)(q24;q12) translocation, and immunocytochemistry and molecular biology show the tumor's neuroectodermal origin. For 20 years, therapy tended toward first line tumor chemoreduction, followed, when appropriate, by complete resection and orthopedic reconstruction of the bone. Radiotherapy, which is responsible for long-term sequelae is now increasingly restricted to inaccessible or incompletely excised tumors. More intensive chemotherapy is being examined in patients with poor prognosis factors such as a negative response to induction chemotherapy, a significant mass or metastases. As a result of new strategies, disease-free survival rate is now between 60-70%. The management of this disease is highly multidisciplinary and patients will now be included in multicentric controlled therapeutic trials. Long term follow-up has to be carried out following completion of treatment.
The authors report one case of Drash syndrome: association of Wilms' tumor, nephropathy and genital abnormalities. The definition, prognosis and origin of this triad are discussed.
Three pediatric cases of hydatic cyst of the kidney are reported. This is a very rare condition in children. Usually, the presentation is a cystic space-occupying lesion of the kidney. Several diagnostic methods are available but it is stressed that ultrasonography may be sufficient. Surgical treatment is still necessary.
A new case of duodenal atresia with gas below the obstruction is reported. This apparently contradictory feature is due to a bifid termination of the common bile duct in the duodenum, air by-passing the atretic area. The anomaly was proven by operative cholangiography.
Between 1985 and 1990, we treated 46 cases of esophageal atresia in the pediatric surgery department in Marseille. In 17 of these patients atresia was associated with tracheomalacia. The most common respiratory manifestations were acute apneic attacks, cyanotic spells and recurring pneumopathy. The underlying cause was arterial compression of the airways by the innominate artery in 6 cases, the aortic arch in 2 cases, and a common origin of the innominate and left carotid arteries in 2 cases. Eleven patients underwent surgery to relieve arterial compression: suspension of the aorta from the under surface of the sternum (10 cases) and reimplantation of the innominate artery (1 case). Results in terms of tracheal caliber and symptomatic relief were excellent in 10 cases including 1 case requiring redo, and poor in 1 case. For diagnosis, our experience shows the importance of endoscopy, nuclear magnetic resonance and recognition of gastroesophageal reflux which is often associated (9/11). The role of tracheomalacia is discussed. On the basis of our results and those previously reported, aortopexy appears to be an effective surgical technique in patients with esophageal atresia presenting respiratory complications due to arterial compression.
The MR aspects of spondylodiscitis in pediatric patients were studied with a 0.5-Tesla supraconductive magnet: 12 patients were divided in two groups; acute disease (9) and period of sequelae (3). The first group was divided into two subgroups according to the age and the clinical findings: infants (2) and children (7). In infants, MR gave good visualization of the destruction of vertebrae and discs. Presuppurative abscesses appeared as light signals, their extent and position relative to the cord being clearly visualized in frontal and sagittal planes in T1-weighted images. In older children, there were a low vertebral signal and disappearance of the disc-vertebra borders on T1-weighted images and a high vertebral signal with a decreased and flattened disc signal on T2-weighted images. Frontal and sagittal planes were used. During the first month of antibiotic therapy, the follow-up scans showed no changes, and even disclosed a spread of the abnormal signal in the vertebral body. At the stage of sequelae, on T1 the spinal signal was normal with a decreased disc width and on T2 the disc signal was low while the vertebrae appeared normal.
16 cases of primary sacral bone tumours in children are reported. These include 13 patients with Ewing's sarcoma and 3 with very rare primary sacral bone tumours in childhood--chordoma, haemangiopericytoma and osteoblastoma. All sacral bone tumours, with the exception of Ewing's sarcoma are very rare in childhood. The possibility of a sacral tumour should be considered in a child with radiculopathy. CT and MR make the diagnosis of primary sacral bone tumours much easier with the added possibility of recognition of the true nature of the lesion in many instances. Reports of primary sacral bone tumours in children are scarce. Most of the patients are incorporated in adult series which do not specify the age of the child and the site of the tumour. The purpose of this paper is to describe 16 children with primary sacral bone tumours.
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Five cases of uterus didelphys, unilateral imperforate vagina and ipsilateral renal agenesis are reported with a follow-up from 1 to 5 years. Diagnosis included typical revelation at puberty (2 cases) but also in the first months of life (2 cases). One case was only diagnosed at the age of 6 years, and the presence of an ectopic ureter was noted in the imperforate vagina. The authors point out the necessity of precise evaluation and careful management in order to maintain an intact reproductory tract. Simple excision of the septum is recommended which can most of the time be done by the vaginal approach.
High level ano-rectal anomalies were evaluated by Magnetic Resonance Imaging (MRI) in 15 patients. One neonate and two infants were evaluated preoperatively. Sagittal slices identified properly the blind rectal end in the 3 cases. Associated anomalies (tethered spinal cord, right ectopic kidney in 1 case, recto-urinary fistula in another case, upper excretory dilatation in 2 cases) were found during the same examination. In the post-operative period (12 cases), major data were obtained on axial transverse slices. When pulled-through intestine is outside the levator sling, re-do-surgery can be indicated. MRI appears as an accurate tool to assess these anomalies.
Two cases of calcified thrombus of inferior vena cava in children are reported and the relevant literature reviewed. Although the physiopathology of this lesion is poorly understood and its detection is usually fortuitous, its presentation is univocal.
Technical aspects and indications for the use of digital subtraction angiography of thorax in 109 children are discussed, and the interest of this exploratory method emphasized in chronic respiratory disease in children. Results obtained are compared with other investigations for screening of bronchopulmonary dysplasia. Although generally reliable for exploration of thoracic aorta anomalies (coarctation and abnormal vascular arch) it is considered to be incompletely effective for investigation of congenital heart disease.
Predisposing factors in two children with urinary tract candidiasis included: neonatal period, uropathy with malformation, massive antibiotic therapy. Ultrasound images showed dilated excretory cavities of variable echo intensities and without posterior shadow cone. Diagnosis was confirmed by urine culture and a satisfactory outcome obtained by specific therapy and surgery for malformations.