Peutz Jeghers Syndrome
Peutz Jeghers Syndrome

Video Endoscopic Sequence 1 of 19.

Peutz Jeghers Syndrome

A 32 year-old female, who suffers from Peutz-Jeghers syndrome, as an interesting fact had been surgically operated on a intestinal volvulus at 13 years old, and an ovarian teratoma at age 25, had had several polypectomies before the endoscopies presented here.

Peutz Jeghers syndrome (PJS) is an autosomal dominant genetic condition characterized by multiple benign polyps called hamartomas on the mucous lining in the gastrointestinal system. These polyps occur most often in the small intestine but also occur in the stomach and large intestine. Affected individuals also have dark skin discoloration, especially around the eyes, nostrils, mucous membranes of the mouth, perianal area and inside the mouth. Affected individuals have an increased risk for intestinal and other cancers.

PJS is characterized by multiple benign polyps called hamartomas on the mucous lining in the gastrointestinal system. These polyps occur most often in the small intestine but also occur in the stomach and large intestine and may cause chronic rectal bleeding, anemia and bowel collapse or obstruction.

Affected individuals also have dark skin discoloration, especially around the eyes, nostrils, mucous membranes of the mouth, perianal area and inside the mouth and on the fingers. Hyperpigmentation may fade in adolescent or adulthood.

Affected females have an increased risk for a benign ovarian tumor called SCTAT (sex cord tumors with annular tumors) for which symptoms may include irregular or heavy periods or early puberty. Affected males can develop a tumor in the testes that secretes estrogen and can lead to breast development (gynecomastia). Individuals with PJS have an increased risk for intestinal and other cancers.

 

 

 

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Sindrome Peutz Jeghers

Video Endoscopic Sequence 2 of 19.

Peutz Jeghers Syndrome

The primary description of PJS was published by Dutch physician Peutz in 1921 in one Dutch family in three young children. His observations in this family led to the definition of what is now known as Peutz–Jeghers syndrome(the Harrisburg family) as a gastrointestinal familial polyposis with pigmentations. Simultaneously occurring nasal polyposis was described in the original report by Peutz]. The pedigree of this original Dutch family continues to be followed. Jeghers specified the description in 10 cases from different families in his work in 1949, and defined the relations between pigmented lesions, gastrointestinal polyposis and increased risk of carcinoma; approximately half of his patients suffered from gastrointestinal malignancy.

 

 

 

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Peutz Jeghers Syndrome

Video Endoscopic Sequence 3 of 19.

Only tiny polyps were found in the stomach

 

 

 

 

 

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Peutz Jeghers Syndrome

Video Endoscopic Sequence 4 of 19.

Multiple polyps of the duodenum, some with pedicled

 

Peutz Jeghers Syndrome

Video Endoscopic Sequence 5 of 19.

Retroflection image observing part of the body and antrum fundus and few tiny polyps.

 

 

Peutz Jeghers Syndrome

Video Endoscopic Sequence 6 of 19.

Close up to a tiny polyp in the antrum

Peutz Jeghers Syndrome

Video Endoscopic Sequence 7 of 19.

Pedunculated polyp of of the Duodenum.

 

Peutz Jeghers Syndrome

Video Endoscopic Sequence 8 of 19.

Another Pedunculated polyp of the duodenum

 

Peutz Jeghers Syndrome

Video Endoscopic Sequence 9 of 19.

Two Polyps of the Duodenum

Peutz Jeghers Syndrome

Video Endoscopic Sequence 10 of 19.

Hamartoma in duodenum.

Large lobulated polyp with a long stalk in distal duodenum

 

 

Peutz Jeghers Syndrome

Video Endoscopic Sequence 11 of 19.

Biopsies of the largest polyp found in the duodenum are acquired.

Peutz Jeghers Syndrome

Video Endoscopic Sequence 12 of 19.

More polyp of the duodenum

 

Peutz Jeghers Syndrome

Video Endoscopic Sequence 13 of 19.

Polypectomy of polyps of the colon was performed, some had already been removed in another institution.

 

Peutz Jeghers Syndrome

Video Endoscopic Sequence 14 of 19.

Cecum polyp

 

Peutz Jeghers Syndrome

Video Endoscopic Sequence 15 of 19.

Terminal ïlium

No polyp of the Ilium Terminal was observed

 

 

 

 

Peutz Jeghers Syndrome

Video Endoscopic Sequence 16 of 19.

Polypectomy of Polyp of the cecum is carry out

 

Peutz Jeghers Syndrome

Video Endoscopic Sequence 17 of 19.

Polypectomy of Long Pedicle Polyp

Peutz Jeghers Syndrome

Video Endoscopic Sequence 18 of 19.

 

 

 

 

 

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Peutz Jeghers Syndrome

Video Endoscopic Sequence 19 of 19.

 

 

 

 

 

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