001SB Peutz Jeghers Small Bowel Polyps and Intusussception

A 10 year old girl presents with abdominal pain

An AI generated image shows a freckled face involving the forehead, nose cheeks and upper lip

Peutz-Jeghers Syndrome with Facial Freckling
An -year-old girl with a history of small bowel polyps presents with facial freckles, particularly on her face, upper lip, buccal mucosa, and fingertips, consistent with Peutz-Jeghers syndrome.
Comment:
Peutz-Jeghers syndrome is characterized by mucocutaneous pigmentation and multiple hamartomatous polyps, often involving the small bowel. The pigmentation, particularly on the lips and fingertips, is a hallmark feature, while the polyps can predispose to complications such as intussusception.
Ashley Davidoff MD TheCommonVein.net (139511) (001SB).

SBFT Spot film shows

Peutz-Jeghers Syndrome with Jejuno-Jejunal Intussusception
A 10-year-old girl presents with acute abdominal pain. Clinical examination reveals freckles on her face and upper lip, freckles in her buccal mucosa, and on her fingertips, consistent with Peutz-Jeghers syndrome.
Small bowel follow-through reveals evidence of jejuno-jejunal intussusception with telescoping of the bowel loops. The contrast-filled intussusceptum is seen within the dilated, mostly air-filled intussuscipiens. A coiled spring sign is noted indicative of contrast outlining the interfaces between the telescoped bowel loops.
Comment:
The coiled spring sign is a classic radiologic finding in intussusception, characterized by contrast outlining the interfaces of the telescoped bowel loops. This appearance results from contrast material trapped between layers of the bowel wall, emphasizing the complex folding pattern created during bowel telescoping. Its recognition is essential for diagnosing intussusception, as it indicates bowel invagination and potential obstruction requiring prompt medical intervention.
Ashley Davidoff MD TheCommonVein.net (00514.2.8) (001SB).

 

A second spot film shows

Peutz-Jeghers Syndrome with Jejuno-Jejunal Intussusception
A 10-year-old girl presents with acute abdominal pain. Clinical examination reveals freckles on her face and upper lip, freckles in her buccal mucosa, and on her fingertips, consistent with Peutz-Jeghers syndrome.
Small bowel follow-through reveals evidence of jejuno-jejunal intussusception with telescoping of the bowel loops. The contrast-filled intussusceptum is seen within the dilated, mostly air-filled intussuscipiens. A coiled spring sign is noted indicative of contrast outlining the interfaces between the telescoped bowel loops.
Comment:
The coiled spring sign is a classic radiologic finding in intussusception, characterized by contrast outlining the interfaces of the telescoped bowel loops. This appearance results from contrast material trapped between layers of the bowel wall, emphasizing the complex folding pattern created during bowel telescoping. Its recognition is essential for diagnosing intussusception, as it indicates bowel invagination and potential obstruction requiring prompt medical intervention.
Ashley Davidoff MD TheCommonVein.net (00512) (001SB).

 

Abdominal Plain Film

Peutz-Jeghers Syndrome with Jejuno-Jejunal Intussusception
A 10-year-old girl presents with acute abdominal pain.
Clinical examination reveals freckles on her face and upper lip, freckles in her buccal mucosa, and on her fingertips, consistent with Peutz-Jeghers syndrome.
Small bowel follow-through reveals multiple filling defects within a dilated jejunum in the left upper quadrant, consistent with small bowel polyps. The findings are characteristic of Peutz-Jeghers syndrome, . Ashley Davidoff MD (00513) (001SB).

 

Peutz-Jeghers Syndrome with Facial Freckling
An 8-year-old girl with a history of small bowel polyps presents with facial freckles, particularly on her face, upper lip, buccal mucosa, and fingertips, consistent with Peutz-Jeghers syndrome.
Comment:
Peutz-Jeghers syndrome is characterized by mucocutaneous pigmentation and multiple hamartomatous polyps, often involving the small bowel. The pigmentation, particularly on the lips and fingertips, is a hallmark feature, while the polyps can predispose to complications such as intussusception.
Ashley Davidoff MD TheCommonVein.net (139511) (001SB).

 

Peutz-Jeghers Syndrome with Jejuno-Jejunal Intussusception
A 10-year-old girl presents with acute abdominal pain. Clinical examination reveals freckles on her face and upper lip, freckles in her buccal mucosa, and on her fingertips, consistent with Peutz-Jeghers syndrome.
Small bowel follow-through reveals evidence of jejuno-jejunal intussusception with telescoping of the bowel loops. The contrast-filled intussusceptum (b, asterisk) is seen within the dilated, mostly air-filled intussuscipiens (b, red arrowheads). A coiled spring sign is noted (b, white arrowheads), indicative of contrast outlining the interfaces between the telescoped bowel loops.
Comment:
The coiled spring sign is a classic radiologic finding in intussusception, characterized by contrast outlining the interfaces of the telescoped bowel loops. This appearance results from contrast material trapped between layers of the bowel wall, emphasizing the complex folding pattern created during bowel telescoping. Its recognition is essential for diagnosing intussusception, as it indicates bowel invagination and potential obstruction requiring prompt medical intervention.
Ashley Davidoff MD TheCommonVein.net (00514.2CL.1k) (001SB).

 

1.

Clinics in Diagnostic Imaging. 159. Jejunal Intussusception Due to Peutz-Jeghers Syndrome. Krishnan V, Chawla A, Wee E, Peh WC. Singapore Medical Journal. 2015;56(2):81-5; quiz 86.

Wang H, Luo T, Liu WQ, et al. Journal of Gastrointestinal Surgery : Official Journal of the Society for Surgery of the Alimentary Tract. 2011;15(12):2218-25.

4. Peutz-Jeghers Syndrome. McGarrity TJ, Amos CI, Baker MJ GeneReviews® [Internet]. Updated 2021 Sep 2.

5. Clinical Presentations and Surgical Approach of Acute Intussusception Caused by Peutz-Jeghers Syndrome in Adults. Wang H, Luo T, Liu WQ, et al. Journal of Gastrointestinal Surgery : Official Journal of the Society for Surgery of the Alimentary Tract. 2011;15(12):2218-25.