Hollow Visceral Myopathy: Diagnosis and Management

The aetiology of hollow visceral myopathy remains poorly understood despite the review of histology and is often misdiagnosed. It is characterized by impaired intestinal function and motility in absence of mechanical obstruction, which may be familial (transmitted by a dominant or recessive gene) or sporadic. Familial visceral myopathy is a rare hereditary myopathic degeneration of both gastrointestinal (and urinary tracts) that causes chronic intestinal obstruction. Typically, it presents after the first decade of life with symptoms of abdominal distension, abdominal pain, vomiting, constipation, and diarrhoea. It is, therefore, a rare cause of chronic intestinal pseudo-obstruction. Secondary chronic intestinal pseudo-obstruction is typically managed by treating the underlying condition, however, decompression by colostomy or tube gastrostomy/enterostomy/caecostomy may be performed; this also allows for feeding and irrigation of the alimentary tract. This case study emphasizes the atypical presentation of hollow visceral myopathy and the importance of thorough investigations, the various findings during surgery (including a para-duodenal volvulus), and the different approaches to decompression of the intestinal tract that may be considered for such a patient.