Peutz - Jeghers syndrome is an autosomal dominant disease, with an incidence of 1/15,000 characterised by hamartomatous polyps of the gastrointestinal tract, melanin deposits in the lips, buccal mucosa, perioral area and/or facial skin and a family history of PJS. The author reports a case of PJS in a 9 year old child admitted to a super specialty hospital in India, who presented with complaints of recurrent cramping in abdomen, abdominal pain, vomiting, loss of appetite and oral lesions. The USG and CT scan abdomen revealed multiple polyps in the small intestine. Laprotomy was done, but the child had recurrent admission in hospital due to constipation and vomiting and burning sensation in oral cavity with oral lesions. This report describes about this rare condition.
Samruddhi S Bhakare