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[Bone abnormalities. Muscular dystrophy and lithiasis: lithogenic factors and therapeutic difficulties]
R Montero Rubio1, J J Pérez Pérez, I Jiménez Jiménez
1Cátedra y Servicio de Urología, Fundación Jiménez Díaz, Universidad Autónoma, Madrid.
Unlabelled:
Duchenne's muscular dystrophy with kypho-scoliosis, progressive muscle weakness and abnormal fatigue of the muscles results in an immobilisation syndrome with increased bone resorption and hypercalciuria. The accompanying chest deformity alters the respiratory capacity, causing pulmonary insufficiency, acidosis and acid urine. Dorso-lumbar kypho-scoliosis, occasionally very serious, alters the status of the upper urinary tract affecting urine transportation (stasis). Thus, hypercalciuria, urinary acidosis, stasis and infection will determine the formation of urinary lithiasis that can take place in these patients.
Material And Methods:
15 patients with a variety of myopathies (Duchenne's disease, Myasthenia gravis,...) or serious skeletal deformities with metabolic renal lithiasis (pyelic or calyceal) were seen by our group. Other patients presented post-traumatic (paraplegia, hemiplegia,...) or poliomyelitic skeletal sequels or Pott's disease, with septic lithiasis. After evaluating all likely approaches including ESWL, the latter was chosen being the least aggressive. Conventional surgery, either percutaneous or endoscopic, foretells technical problems in terms of lithiasis approach. Both the case introducing the subject, Duchenne's muscular dystrophy, with bilateral renal lithiasis and the others are a reflection of complexity of finding the righ approach for these patients, including ESWL.
Results:
Scoliosis was not a technical obstacle, since patients could be placed in lateral/oblique position to situate the stone in the right spot for lithotrity. Debris removal was easy, with no obstructive complications, in spite of the significant immobilisation of these patients.
Conclusion:
Immobilisation syndrome, acidosis, stasis and infection could jointly determine the lithogenesis mechanism in patients with muscle diseases or serious skeletal deformities and with renal lithiasis. ESWL has an opportunity in serious cases, where other techniques including surgery have major difficulties.