Implications of Peutz-Jeghers Syndrome in Children and Adolescents

Excerpt

Pigmentation tends to arise in infancy, occurring around the mouth, nostrils, perianal area, fingers and toes, and the dorsal and volar aspects of hands and feet (Figure 1). They may fade after puberty but tend to persist in the buccal mucosa. The primary concern to the paediatrician is the risk of small bowel intussusception causing intestinal obstruction, vomiting, and pain. In addition, intestinal bleeding leading to anaemia can occur.

The management of a young child with mid-gut PJS polyps is controversial. In a retrospective review, 68% of children had undergone a laparotomy for bowel obstruction by the age of 18 years, and many of these proceeded to a second laparotomy within 5 years (1). There is a high re-operation rate after initial laparotomy for small bowel obstruction.