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Annals of Clinical & Laboratory Science 38:143-148 (2008)
© 2008 Association of Clinical Scientists

Case Report

Unusual Type of Colonic Neuromuscular Disorder with Extensive Vacuolization

Sandhya Sundaram1, Robert E. Brown2 and Ping L. Zhang3
1 Department of Pathology, Sri Ramachandra Medical College, Porur, Chennai, India, 2 Department of Pathology, University of Texas Health Sciences Center, Houston, Texas, and 3 Department of Anatomic Pathology, William Beaumont Hospital, Royal Oak, Michigan

Address correspondence to Ping L. Zhang, M.D., Ph.D., Department of Anatomic Pathology, William Beaumont Hospital, 3601 West 13 Mile Road, Royal Oak, MI 48073, USA; tel 248 898 9990; fax 248 898 8020; e-mail ping.zhang{at}beaumont.edu.

Various pathological abnormalities of smooth muscle and innervation result in clinical syndromes with disordered motility of the small intestine and colon. Although these abnormalities have been extensively reported clinically, their pathologic changes and pathophysiologic mechanisms have not been well elucidated. We report a case of visceral neuropathy with secondary muscle changes in a 7-yr-old ventilator dependent, mentally retarded child who presented with a history of chronic constipation and symptoms of intestinal obstruction. The muscle layer of the colectomy specimen showed extensive infiltration of vacuolated cells that were positive for S-100 and synaptophysin but negative for glial fibrillary acidic protein (GFAP) and neural filament protein (NFP). Calretinin positivity was preserved in submucosal ganglion cells but was absent in vacuolated nerve branches. Masson’s trichrome stain showed evidence of fibrosis, indicative of muscle damage. There was a reduced number of intestinal cells of Cajal in the muscularis propria, as indicated by CD117 (c-kit) immunostaining. This disorder is most likely a sporadic visceral neuropathy, secondarily affecting muscular function, that causes colonic pseudo-obstruction.

Keywords: colon, neuromuscular disorder, calretinin, vacuolization






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