Biomedical subjects
Publications and source records attributed to F Diard.
Having found three cases of ectasia of the umbilical recess in the antenatal period we decided to review the embryology and the ultrasound of the anatomy of the portal system. We consider that this is a normal anatomic variant of the portal system because the lesion is asymptomatic and regresses spontaneously.
Metaphyseal sclerotic bone changes associated with benign phaeochromocytoma are very rare in childhood. We report four cases, in each of which the radiographic changes returned to normal after removal of the tumour.
A 5-month-old boy presented with a right flank abdominal mass. Plain film of the abdomen showed a vertebral column within the mass. Surgery confirmed the diagnosis of fetus in fetu developed within an ectopic intraperitoneal right testicle. Fetus in fetu differs from teratoma by the presence of axial skeleton and organogenesis. When intratumoral axial skeleton is present, plain films are sufficient for the diagnosis.
Achalasia of the esophagus is rare in children. The authors report eight cases and emphasize two atypical clinical records: The first one is a 3 month-old infant with respiratory distress and "near miss" Sudden Death Infant Syndrome. The second one is a ten year-old boy with association of achalasia of the esophagus, ACTH insensibility and alacryma. Theses cases are discussed.
Pelizaeus-Merzbacher disease is a rare sex-linked leukodystrophy. Its metabolic basis has not been identified yet and diagnosis in mainly based on clinical findings and pathological investigation. Cerebral biopsy is not always performed because of lack of available therapy. Genetic counseling can be provided if the diagnosis is secured. Four cases are reported, in children with characteristic signs, who underwent magnetic resonance (MR) Imaging. MR Imaging appears to be more sensitive than computed tomography to visualize white matter disease: T1 weighted images suggest hypomyelination, in conformity with abnormal myelination. T2 weighted images show a non specific high signal in the white matter.
Twelve cases of Mucolipidosis II (I-cell disease) with a wide range of severity of skeletal involvement were studied. Pathological findings in two cases provided helpful information in understanding the radiographic features of dysostosis multiplex. Inhibition of the growth plate cartilage calcification and rickets-like lesions were observed in the metaphyses. Enhanced subperiosteal remodelling and paratrabecular fibrosis were also evident in the diaphyses. High levels of parathormone were found in one case. This finding supports the hypothesis that bone lesions may be secondary, at least in part, to damage in such viscera as the kidney and/or the liver and that they are mediated by vitamin D and parathormone.
The authors report a case of subdural hematoma of the posterior fossa in a full-term neonate. The lack of specific symptoms is frequent. Diagnosis relies on CT scan and NMR data. In cases with neurologic involvement, good results depend on rapid neurosurgical treatment, in order to avoid sequelae.
We retrospectively studied the radiographic documents of seventy girls with bony age varying from nine to eighteen years old and divided in nine equal groups and seventy boys with bony age from ten to nineteen years old. We analyzed the AP pelvic X ray of each child focusing on cartilages and ossification centers of both pelvic bone and upper femoral extremity. The purpose of this study was to assess the dates of apparition and ossification of these ossification centers and the dates of fusion of cartilages. The iliac ossification center (epiphysaris nucleus ilii) appears lately at bony age of thirteen years and half for girls and fourteen years and half for boys. The triradiate cartilage closes the earliest from its medial to its lateral portion before the apparition of the iliac ossification center. This closing date corresponds to the beginning of growth acceleration. Therefore, the period of growth acceleration can be identified by an accurate analyse of the triradiate cartilage closure on a single AP pelvic X ray.
Based on reports of 9 surgically proven cases, the authors stress the contribution of high-resolution sonography in the work-up of omphalovesical midline anomalies in children. Sonography (US) proved useful, especially in disorders of urachal patency (cystic mass and sinus type of the malformation). In the cystic-type mass (3 cases), a midabdominal echogenic cystic mass was demonstrated. The echogenic content resulted from infectious complication. In the sinus type, an echogenic, thickened, tubular omphalovesical tract (8-15 mm) was visualized. This tubular configuration results from the normal omphalovesical anatomy, as can be demonstrated by high-resolution US. With infection, the fascia surrounding the urachal remnants seems to limit the infection. Differential diagnosis should include vesical duplications anomalies, dystrophic calcifications of the umbilical arteries remnants, and, in case of a solid mass, urachal carcinoma. Ultrasound should be part of the work-up of any suspected urachal or other midline anomaly.
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We recently treated one female infant and two young girls with coincidence of three anomalies: --hypoplastic ectopic kidney; --single vaginal ectopic ureter; --ipsilateral genital abnormality. Clinical presentation was lifelong wetting, "multicystic" kidney, urinary tract infection, or abdominal pain. The diagnostic studies include ultrasonography, excretory urography, cystography and vaginoscopic examination with retrograde catheterization, rarely radionuclide scanning. This study failed in two cases, with non visualization of the hypoplastic and dysplastic kidney, and diagnosis was done at laparotomy. Genital anomalies were unilateral hydrocolpos with uterus didelphys in one case, dilated or cystic Gärtner's duct in the other two. The anomaly may be caused by a maldevelopment of the wolffian and müllerian ducts in early fetal life, between 5 and 6 weeks of gestation. A review of the literature revealed only 6 similar instances in childhood. The combination of lifelong wetting, pelvic mass or pain in girl with "solitary" kidney on excretory urography and ultrasonography should alert to this syndrome. Meticulous search for the hypoplastic kidney, the genital abnormality and the ectopic ureteral orifice are keys to early accurate diagnosis and treatment.
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A case of arteriovenous malformation of the spinal canal in a 8 year-old girl is reported. This child complained of night abdominal pain for several months, without diagnosis. Plain X-ray showed an enlargement of the lumbar spinal canal. Magnetic resonance imaging diagnosed a spinal vascular malformation, which was confirmed by angiography. Interest of MRI in such a case is emphasized.
External epiphysiolysis with valgus slip of the femoral epiphysis is rare. The authors report two such chronic cases, one unilateral and the other bilateral. This disorder complicates a coxa valga with a reversed cephalic growth plate, downward and externally. This coxa valga which appears constitutional is sometimes associated with an increase in femoral antetorsion. Treatment is the same as classical varus slip of the femoral epiphysis.
The advantages, complications or sequelae of surgical treatment of primary vesico-ureteral reflux in children are discussed in the light of 382 cases operated upon between 1971 and 1984. Changes in ideas on the fate of primary reflux and its consequences make it necessary to modify our therapeutic strategy, as shown by a study of our records and an exhaustive review of the literature. As a result, we suggest that reflux into a non-dilated ureter should not be operated, whereas reflux into a widely dilated ureter requires surgery. The operation should take place at an early stage in cases with severe renal lesions with pronounced radiological and biochemical alterations. In children with reflux of medium severity, the decision to operate or treat medically must rest on such criteria as age and severity, frequency and resistance of episodes of urinary infection, radiological and endoscopic data and signs of renal tissue functional damage.