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Pediatrics Conference 2018
Journal of Pediatric Care
ISSN: 2471-805X
Page 54
March 26-27, 2018
Edinburgh, Scotland
2
nd
Edition of International Congress on
Pediatrics
Introduction & Aim:
Peutz-Jeghers syndrome (PJS) is
an autosomal dominant inherited disorder characterized
by intestinal hamartomatous polyps in association with
mucocutaneous pigmentations. Although, intussusception has
been reported as a well-known complication of PJS, recurrent
intussusception as an alarming finding in a patient with normal
gastrointestinal endoscopy is uncommon. A 7-year-old boy
who had recurrent intussusception episodes and diagnosed as
PJS with histopathologically after surgical excision of involved
bowel segments is presented to discuss the clinical features and
treatment options of recurrent intussusception as a presenting
finding of PJS. We aimed to present this case to emphasize
that patients with PJS may present with intussusception and
hamartomatous polyps might be precancerous, in this syndrome.
Case Report:
A 7-year-old boy was admitted to emergency
with complaints of abdominal pain and vomiting lasting three
days. He had a history of recurrent colicky abdominal pain for
approximately six months. There was no history of hematemesis
or melena. He had undergone upper gastrointestinal endoscopy
and colonoscopy because of multiple hyperpigmented macules
over only the lipsand it is revealednormal endoscopicfindings. His
physical examination showed slight distension with generalized
tenderness. According to physical examination findings and
bowel obstruction, he underwent explorative laparotomy. A jejuno-
jejunal intussusception was found 5 cm distal of the ligament
of Treitz during surgical exploration. Intussusception could not
reduce manually and a polypoid mass in the jejunal lumen was
the leading point. The intussuscepting segment including the
polyp was excised by small bowel resection, and end-to-end
anastomosis was performed. Histopathological examination of
the specimen revealed hamartomatous polyp and Peutz-Jeghers
syndrome was confirmed histologically with low-grade dysplasia.
Conclusion:
Peutz-Jeghers syndrome is a hereditary disease
characterized with gastrointestinal polyposis and skin or mucosal
pigmentation. Polypoid lesions are almost hamartomatous and
are not usually precancerous. Our patient underwent upper and
lower endoscopy with presumptive diagnosis of PJS before
the clinical findings of intussusception. Endoscopic evaluation
revealed normal findings without any polypoid lesion. Although
the association of PJS and intussusception is well-known, this
complication is rarely presenting finding for PJS. Patients with
hyperpigmentous lip lesions with recurrent intussusception
episodesmay requiresurgical explorationsnot only relievingsmall
bowel obstruction but also diagnose the underlying polypoid
lesions. In conclusion, patients with PJS should be regularly and
closely monitored in order to reduce the number of laparotomies
and due to the increased risk of cancer.
dr.sinankilic@yahoo.comPeutz-Jeghers syndrome: an unusual cause of recurrent
intussusception in a 7-year-old boy
Sinan Kili
Hospital Clinic of Pediatric Surgery, Turkey
J Pediatr Care, Volume 4
DOI: 10.21767/2471-805X-C1-006