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Lipomatosis of the colon complicating Proteus syndrome
  1. G Mackay1,
  2. L Spitz1,
  3. K McHugh2
  1. 1Department of Paediatric Surgery, Great Ormond Street Hospital for Children NHS Trust, Great Ormond Street, London WC1N 3JH, UK; l.spitz{at}ich.ucl.ac.uk
  2. 2Department of Radiology, Great Ormond Street Hospital for Children NHS Trust

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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.

Figure 1

Axial T1 weighted MRI showing diffuse hyperintense signal tissue typical of fat surrounding and separating bowel loops (note similar signal from the diffuse lipomatous abdominal lesion and subcutaneous fat). The lesion extends posteriorly on the left into the paraspinal musculature, displacing psoas anteriorly.

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