Abstract
The clinical and pathological features in 12 children with symptomatic gastrointestinal polyposis are presented. Five children with Peutz-Jeghers syndrome all presented with intussusception. Three children with Gardner syndrome presented with multiple large epidermoid cysts of the scalp. The other 4 with juvenile polyposis all presented with anaemia, often associated with growth retardation and extragastrointestinal abnormalities. One of these 4, as well as her mother, had Osler disease with pulmonary arteriovenous malformation. Some degree for overlap in the histology was noted between the polyps in the three conditions, and confusion may arise if only small areas are examined. The histological evolution of small lesions in juvenile polyposis and the surgical observation of friability of adjacent non-polypoid mucosa and association with multiple disorders, local as well as generalised, mechanical as well as inflammatory, suggest these to be the result of non-specific ulceration followed by granulation tissue formation, inflammatory infiltration, and irregular epithelial regeneration.
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Chow, C.W., Taylor, R.G., Stokes, K.B. et al. Gastrointestinal polyposis in infancy and childhood. Pediatr Surg Int 4, 27–34 (1988). https://doi.org/10.1007/BF00173080
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DOI: https://doi.org/10.1007/BF00173080