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Peutz-Jeghers Syndrome Presented as Jejunojejunal Intussusception

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Abstract

Peutz-Jeghers syndrome (PJS) is an autosomal dominant disorder characterized by intestinal hamartomatous polyps in association with skin and mucosal macular melanin deposition. There is a lifetime chance of 93% of cancers when all cancers are combined and the incidence of the disease is variable between 1 in 8300 and 1 in 280,000 live births (Daniell et al., Familial Cancer 17(3):421-427, 2018). Here, we present a 14-year-old Indian boy presenting to surgical emergency with intestinal obstruction secondary to jejunojejunal intussusception for which resection and anastomosis were done. Post-operative histopathological examination showed a single polyp leading to intussusception. He classically showed mucocutaneous pigmentation on general examination. Early identification of Peutz-Jeghers syndrome on a routine visit to healthcare can decrease complications like intestinal obstruction and help in starting early surveillance for cancers.

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Authors and Affiliations

  1. Department of General Surgery, Lady Hardinge Medical College, 110001, New Delhi, India

    Vasudeva Lam, Ankit Sachan & Ashok Kumar

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  1. Vasudeva Lam
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  2. Ankit Sachan
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  3. Ashok Kumar
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Correspondence to Vasudeva Lam.

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Lam, V., Sachan, A. & Kumar, A. Peutz-Jeghers Syndrome Presented as Jejunojejunal Intussusception. Indian J Surg 83 (Suppl 3), 804–806 (2021). https://doi.org/10.1007/s12262-021-02947-3

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  • DOI: https://doi.org/10.1007/s12262-021-02947-3

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