Atıl ÇAKMAK, İlknur KEPENEKÇİ, Erkinbek OROZAKUNOV, Onur KIRIMKER, Ali Ercüment KUTERDEM

Tekrarlayan intussussepsiyon ile tanısı konan bir yetişkin Peutz-Jeghers sendromu olgusu

Peutz-Jeghers sendromu; deri ve mukozalarda hiperpigmente lezyonlar, intestinal ve ekstraintestinal çoklu hamartomatöz poliplerle karakterize otozomal dominant geçişli kalıtsal bir hastalıktır. İntestinal polipler; gastrointestinal kanamaya bağlı demir eksikliği anemisi ve intussussepsiyon ile intestinal obstruksiyon nedeni olabilirler. Peutz-Jeghers Sendromlu hastalarda gastrointestinal ve ekstragastrointestinal malignite riski belirgin olarak artmıştır. 48 yaşında erkek hastaya tekrarlayan intussussepsiyonlar nedeniyle laparatomi uygulandı. İntussussepsiyonun sebebi jejenumda hamartamatöz polip olarak tespit edildi ve hastaya erişkin yaşta Peutz-Jeghers sendromu tanısı konuldu. Peutz-Jeghers sendromunun prekanseröz bir sendrom olduğu, erişkinlerde de görülebileceği ve intestinal poliplere bağlı multiple laparatomilerden korunmak amaçlı sendromun takibinin önemini hatırlatmak amaçla vakamızı sunmaktayız.

Peutz-jeghers syndrome presenting with recurrent intussuseption: A case report

Peutz-Jeghers syndrome is an autosomal dominantly inherited disorder which is characterized with hyperpigmented skin and mucosa lesions, intestinal and extraintestinal multiple hamartomatous polips. Intestinal polips may cause iron deficiency anemia due to gastrointestinal bleeding and intestinal obstruction due to intusseption. Gastrointestinal and extragastrointestinal malignancy risk is remarkably increased in patients with Peutz-Jeghers syndrome. Laparatomy was performed to a 48-year-old man because of recurrent intusseptions. The reason of intussuseption was found as hamartomatous polyp in the jejenum and the patient was diagnosed as adult Peutz-Jeghers syndrome. We present our case to remind that Peutz-Jeghers syndrome is a precancerous syndrome, can be seen at adult and all the patients should be followed up to avoid multiple laparatomies due to intestinal polips.

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