RESUMO

We report an unusual case of a patient with sporadic visceral myopathy and involvement of the entire gastrointestinal and urinary tract. Visceral myopathy is a form of chronic idiophatic intestinal pseudo-obstruction characterized by vacuolar degeneration, atrophy and fibrosis of the intestinal propia muscle layer without inflammatory cells. It can be found in childhood and adolescence affecting the gastrointestinal and urinary visceral muscle. The familial occurrence can be found in about 30% of cases and the mode of transmission is autosomal recessive in most families. It is crucial to exclude secondary forms of chronic intestinal pseudo-obstruction and to obtain full thickness intestinal biopsy for the diagnosis. Surgical treatment is only beneficial in cases with isolated segmental involvement of the gastrointestinal tract.

Assuntos

Pseudo-Obstrução Intestinal/etiologia , Doenças Musculares/complicações , Adulto , Dilatação Patológica/diagnóstico , Dilatação Patológica/etiologia , Feminino , Humanos , Pseudo-Obstrução Intestinal/diagnóstico , Intestino Delgado/patologia , Doenças Musculares/diagnóstico , Doenças Ureterais/patologia , Doenças Urológicas/diagnóstico , Doenças Urológicas/etiologia

RESUMO

We report an unusual case of a patient with sporadic visceral myopathy and involvement of the entire gastrointestinal and urinary tract. Visceral myopathy is a form of chronic idiophatic intestinal pseudo-obstruction characterized by vacuolar degeneration, atrophy and fibrosis of the intestinal propia muscle layer without inflammatory cells. It can be found in childhood and adolescence affecting the gastrointestinal and urinary visceral muscle. The familial occurrence can be found in about 30

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of cases and the mode of transmission is autosomal recessive in most families. It is crucial to exclude secondary forms of chronic intestinal pseudo-obstruction and to obtain full thickness intestinal biopsy for the diagnosis. Surgical treatment is only beneficial in cases with isolated segmental involvement of the gastrointestinal tract.