ABSTRACT
Bezoars are conglomerates of food or fiber in the alimentary tract of humans and certain animals, mainly ruminants. A trichobezoar represents a mass of accumulated hair. Trichobezoars may present as an isolated gastric mass, as an extension into the small intestine, or as an independent fragmented mass in the small intestine. The presence of discrete coexisting gastric and ileal trichobezoars has been reported only rarely in the literature. This is a case report of a 10-year-old girl presenting with small-bowel obstruction secondary to synchronous trichobezoars in the stomach and ileum. The case highlights the role of imaging and importance of complete evaluation of the gastrointestinal tract at the time of surgical evacuation.
ABSTRACT
Mucopolysaccharidoses are a type of lysosomal storage disorders characterized by defect in the degradation of Mucopolysaccharides due to deficiency of specific lysosomal enzymes leading to their accumulation in various tissues. MPS -VI (Maroteaux-Lamy Syndrome) is an autosomal recessive syndrome due to deficiency of enzyme Aryl- Sulfatase -B, and is characterized by characteristic facies, normal intelligence, Dysostosis multiplex, organomegaly, joint stiffness, corneal clouding and striking inclusions in peripheral blood leucocytes. We present an 8-year-old male child with MPS-VI syndrome, confirmed by enzyme assay.