Biomedical subjects
Publications and source records attributed to D R Roth.
A 10-year-old boy had intermittent ureteropelvic junction obstruction that proved to be owing to benign fibroepithelial polyps. The clinical features and management of this rare lesion are discussed.
Nephroblastoma, or Wilms tumor, is the most common renal neoplasm in children and accounts for approximately a fifth of all malignant growths in this age group. However, the incidence of Wilms tumor in adults is much less common, with less than 200 cases having been reported in the literature. In addition, since there are at least 53 synonyms for this tumor an adequate computer search for articles relating to this subject is difficult. Because of the scarcity of this disease in adults definite treatment modalities have not been accepted thoroughly until recently. The rationale for the present treatment modalities is based largely on the National Wilms Tumor Study groups 1 to 3. We present radiologic and histologic findings of this tumor in 2 additional cases. The current treatment modalities, consisting of chemotherapy with or without adjunctive radiotherapy, are discussed.
A total of 11 children with chronic meningomyeloceles underwent a clinical neurological, urodynamic and neurophysiological evaluation to define further the level and type of lesion present in the lumbosacral spinal area. The neurophysiological evaluation was correlated with detrusor activity. Detrusor hyperreflexia was present in 45 per cent of the patients and functional activity of the somatic sacral reflex arc was demonstrated in 4. Absolute interruption of the somatic sacral reflex arc was found in the remaining patients with detrusor areflexia. All patients had evidence of a lower motor neuron lesion on clinical neurological evaluation, with weak or absent myotatic and musculocutaneous reflexes. Electromyographic evaluation revealed signs of severe partial to complete denervation of the affected muscle groups. However, increased motor unit potentials were observed in 5 patients after coughing and stimulation of the perianal skin. The bulbocavernosus reflex was absent clinically and electrophysiologically in all 6 patients tested. However, this finding did not indicate absolute interruption of the reflex arc, since further temporal and spatial stimuli resulted in an increase in motor unit potentials. Somatosensory cortical evoked potentials were useful in demonstrating an intact afferent input system to the spinal cord and brain area. However, lumbosacral evoked potentials appeared to be the least useful neurophysiological method to demonstrate partial preservation of the input to the spinal cord level. Therefore, detrusor and external urethral sphincter function may be documented on urodynamic and neurophysiological evaluation when the clinical examination has revealed a complete lower motor neuron lesion. The complexity of the lesion caused by the malformation in patients with meningomyelocele is shown.
The frequent use of fetal ultrasound is allowing early (prenatal) diagnosis of numerous uropathies previously delayed until the child either became symptomatic or had a palpable mass. We would anticipate an increasing number of neonates presenting for repair of obstructions, especially ureteropelvic junction obstruction. To evaluate our experience with this disorder in infants we reviewed our experience with ureteropelvic junction obstruction during the last 6 years in 16 infants less than 1 year old. Principles of evaluation, surgical techniques and results are presented. To date, no secondary procedures have been necessary and most neonates have shown dramatic improvement in the parenchymal mass and intrarenal anatomy.