Congenital abnormality or acquired disease may affect any of these components to produce dysfunction that results in a gut motility disorder. The disorders may be restricted to only part of the gut such as gastro-oesophageal reflux or may diffusely affect the gut and the bladder as in Hollow Visceral Myopathy. The latter are due to a heterogeneous group of disorders in which developmental abnormality of the enteric nerves and muscle coats occurs. The commonest of these is Hirschsprung's disease. Other conditions which may be inherited such as hollow visceral myopathy or congenital absence of argyophil neurons are extremely rare and only 2 - 20 cases in the UK are known.
Diagnosis requires specialist paediatric gastroenterological and histopathological investigation.
Treatment is both pharmacological and surgical and may require nutritional support either enterally or parenterally. Surgery should only be carried out in centres experienced in the field. A register of patients with intestinal pseudo-obstruction is maintained in the Department of Gastroenterology and Histopathology at the Great Ormond Street Hospital for Children in London. In children the majority of conditions are congenital and some are familial. None at present.