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A 12 year old boy with Proteus syndrome (macrodactyly, plantar/palmar cerebriform hyperplasia, lipomata, verroucous naevi, macrocephaly, and/or vascular abnormalities) presented with gross abdominal distension and severe intractable constipation. Magnetic resonance imaging (MRI) scan showed a uniformly hyperintense T1 signal characteristic of adipose tissue widely spread throughout the abdomen and pelvis (fig 1). Laparotomy revealed a huge infiltrating lipomatosis mass encasing the left colon, including the rectum.
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