Published online Jun 14, 2015. doi: 10.3748/wjg.v21.i22.7059
Peer-review started: January 8, 2015
First decision: January 22, 2015
Revised: March 9, 2015
Accepted: March 30, 2015
Article in press: March 31, 2015
Published online: June 14, 2015
Visceral myopathy is one of the causes of chronic intestinal pseudo-obstruction. Most cases pathologically reveal degenerative changes of myocytes or muscularis propia atrophy and fibrosis. Abnormal layering of muscularis propria is extremely rare. We report a case of a 9-mo-old Thai male baby who presented with chronic intestinal pseudo-obstruction. Histologic findings showed abnormal layering of small intestinal muscularis propria with an additional oblique layer and aberrant muscularization in serosa. The patient also had a short small bowel without malrotation, brachydactyly, and absence of the 2nd to 4th middle phalanges of both hands. The patient was treated with cisapride and combined parenteral and enteral nutritional support. He had gradual clinical improvement and gained body weight. Subsequently, the parenteral nutrition was discontinued. The previously reported cases are reviewed and discussed.
Core tip: This report describes the case of a 9-mo-old boy who presented with chronic intestinal pseudo-obstruction. Full-thickness small bowel biopsy showed abnormal layering of muscularis propria (additional oblique layer) and serosal aberrant muscularization. There have been only eight previously reported abnormal layering cases and only one case with an additional oblique layer. The patient also had a short small bowel without malrotation, brachydactyly, and absence of the 2nd to 4th middle phalanges of both hands. The patient showed clinical improvement with medical treatment and nutritional support.