Causes

Causes

IPO is a rare disease. Approximately half of cases arise shortly after birth or in infancy , caused by a number of very rare enteric neuropathies and myopathies, including genetic and familial, inflammatory and degenerative forms. Other cases arise later in life when a secondary aetiology is more common. In some patients, a cause is not found and these are termed idiopathic. The full list of causes is given in Summary box 73.5 . Summary box 73.5 Causes of intestinal pseudo-obstruction /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF /uni25CF Carlos Justiniano Ribeiro Chagas , 1879–1934, Director of the Oswaldo Cruz Institute and Professor of Tropical Medicine, University of Rio de Janeiro, Brazil. - IPO presents clinically with the symptoms and signs of small bowel obstruction with pain, distension and vomiting. After clinical evaluation and plain radiology , a degree of suspicion is helpful to avoid unnecessary and potentially harmful surgery . Such suspicion is merited when there is no obvious cause for - mechanical obstruction, i.e. no known bowel disease, previous surgery or hernia, and on the length of history . Here, knowing the list of secondary causes becomes helpful. For instance, in someone who is a smoker with finger clubbing, a small cell carcinoma of the lung may be the cause of paraneoplastic pseudo-obstruction; alternatively , the patient may have clinical signs of scleroderma. Axial imaging is essential to exclude mechanical obstruction. Adjunctive blood and imaging tests may help define a cause and these can include MRI of the brain and skeletal muscle biopsy for rare diagnoses such as mitochondrial myopathies. Primary neuropathies and myopathies can be diagnosed histologically , but this requires full-thickness tissue and a vari - - ety of special stains (available only in specialist centres). Since laparotomy and bowel resections are best a voided, a laparo - scopic or minilaparotomy full-thickness biopsy may be war - ranted for diagnosis ( Figures 73.6 and 73.7 ).

Primary Several very rare enteric myopathies and neuropathies Unknown (termed ‘idiopathic’) Secondary Connective tissue disease, especially scleroderma Radiation injury Amyloidosis Autonomic neuropathies including diabetes and paraneoplasia Infections: Chagas’ disease (South American trypanosomiasis)