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Case Reports
. 2024 May 28;2024(5):rjae335.
doi: 10.1093/jscr/rjae335. eCollection 2024 May.

Uncommon manifestation of Peutz-Jeghers syndrome: a case of jejuno-jejunal intussusception and volvulus leading to small bowel obstruction

Hesham Barhmji  1   2 , Abduraboh Alsalehi  2   3 , Ahmad Kammasha  4 , Ahmad Alkheder  4   5
Affiliations
Case Reports

Uncommon manifestation of Peutz-Jeghers syndrome: a case of jejuno-jejunal intussusception and volvulus leading to small bowel obstruction

Hesham Barhmji et al. J Surg Case Rep. .

Abstract

Peutz-Jeghers syndrome (PJS) is a rare genetic disorder causing gastrointestinal polyps and skin pigmentation. Our case report highlights a unique instance of jejuno-jejunal intussusception associated with PJS in a 28-year-old female patient who presented to the emergency department with colicky abdominal pain, tachycardia, and gastrointestinal symptoms. Physical examination revealed mucocutaneous hyperpigmentation. Imaging studies showed a U-shaped distension in the jejunum with thickening and pneumatosis. Laparotomy revealed a jejuno-jejunal volvulus with intussusception. Surgical resection successfully addressed gangrenous jejunal tissue and ileal polyps. Histopathology confirmed PJS polyps. Postoperatively, the patient recovered well and was discharged. Family history revealed similar skin lesions in her uncle. Our case highlights the need for prompt surgical intervention to address complications associated with PJS and elucidates a unique presentation of PJS involving jejuno-jejunal intussusception and volvulus leading to complete small bowel obstruction. We aim to deepen understanding and prompt discussions on optimal therapeutic strategies.

Keywords: PJS; Peutz-Jeghers syndrome; bowel obstruction; emergency; intussusception; volvulus.

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Conflict of interest statement

None declared.

Figures

Figure 1
Figure 1
Mucocutaneous pigmentation on the buccal mucosa and lips.
Figure 2
Figure 2
Abdominal X-rays in erect position reveals five air fluid levels.
Figure 3
Figure 3
View during the surgery: intussusception jejuno-jejunal and hamartomatous polyps of the ileum.
Figure 4
Figure 4
Colonoscopy with multiple polyps.
Figure 5
Figure 5
Histopathological study: showing an edematous appearance of the intestinal mucosa with inflammatory lymphocytes and histiocytes.
See this image and copyright information in PMC

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