Double intussusception in a teenage child with Peutz-Jeghers syndrome: A case report

Chiew, Junloong; Sambanthan, Sekkapan Thannimalai; Mahendran, Hans Alexander

doi:10.12998/wjcc.v9.i23.6804

Cited by 5 publications

(3 citation statements)

References 14 publications

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“…PJS can be diagnosed by the presence of microscopically confirmed hamartomatous polyps and a minimum of two of the following clinical criteria: Family history, mucocutaneous melanotic spots, and small bowel polyps with bleeding per rectally. [ 6 ] In our series, we established the diagnosis using above-mentioned criteria.…”

Section: Discussionmentioning

confidence: 99%

Peutz–Jeghers Syndrome

Shukla,

Tiwari,

Dariya

et al. 2023

Journal of Indian Association of Pediatric Surgeons

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Introduction: Peutz–Jeghers Syndrome (PJS) is an autosomal dominant disease presenting with hamartomatous polyps in the gastrointestinal tract and hyperpigmented macules on lips and oral mucosa. The incidence of this syndrome is approximately 1 in 1,20,000 births. Materials and Methods: In this article, we are presenting 11 cases of PJS which were misdiagnosed and patients were compelled to visit hospital repeatedly. All these cases were diagnosed based on clinical suspicion, family history, and histopathological examination of specimens. Most of the cases presented with intussusception and required emergency surgical management. Results: PJS can be diagnosed by the presence of microscopically confirmed hamartomatous polyps and a minimum of two of the following clinical criteria: Family history, mucocutaneous melanotic spots, and small bowel polyps with bleeding per rectally. The diagnosis can be missed if the melanotic spots on the face are missed. Routine investigations, imaging, and endoscopy were done in all cases. PJS patients need regular follow-up due to chance of recurrence of symptoms and susceptibility to cancer. Conclusion: PJS needs a high index of suspicion for diagnosis in cases of recurrent abdominal pain with bleeding per rectum. Proper family history and meticulous clinical examination for melanosis are very important to prevent the misdiagnosis of these cases.

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Section: Discussionmentioning

confidence: 99%

Peutz–Jeghers Syndrome

Shukla,

Tiwari,

Dariya

et al. 2023

Journal of Indian Association of Pediatric Surgeons

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“…2 Patients have an increased chance of complications like intussusception that can lead to intestinal obstruction. 3 They also have a predisposition to both gastrointestinal and non-gastrointestinal malignancies. 4 So active surveillance is essential in all cases.…”

Section: Introductionmentioning

confidence: 99%

Peutz Jeghers syndrome with multiple intussusceptions

Giri

Saju²,

Rahul³

2022

Int Surg J

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Peutz Jegher’s syndrome is a rare autosomal dominant disorder characterized by the development of hamartomatous polyps and mucocutaneous melanin pigmentation. This case report shows the case of an adult Indian female with intestinal obstruction due to multiple intussusceptions that were caused by hamartomatous polyp of Peutz Jegher’s syndrome. A 32-year-old female presented to the surgical emergency with complaints of colicky abdominal pain, vomiting and melena and with a positive family history for Peutz Jegher’s syndrome. On abdominal examination, a mass was palpable in her right lumbar area and per rectal examination showed ballooning and blood staining. She was provisionally diagnosed with Peutz Jegher’s syndrome with intussusception and her ultrasound imaging of the abdomen also showed the features suggestive of intussusception. She underwent an emergency laparotomy with resection and anastomosis of the involved segment of the jejunum and limited right hemicolectomy. Intraoperative findings showed multiple intussusceptions in her both large and small bowels along with multiple polyps which was seen to be hamartomatous polyps in the histopathological studies. The postoperative period was uneventful and during the review she was healthy. Intussusception is the major complication of Peutz Jegher’s syndrome and it can be prevented with regular surveillance in known cases. Intussusception should be kept in mind as the first differential diagnosis when these patients present with acute abdomen. The investigation of choice that’s preferred is a cross sectional imaging of the gastrointestinal tract to confirm with the diagnosis. Periodic surveillance is essential in those patients with known history or those with the mutation of STK11 (LKB1) gene.

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“…9,10 ConclusionsPeutz-Jeghers syndrome is less common in children and usually presents with hematemesis, melena, or recurrent intussusception. Gastroduodenal intussusception is a very rare complication causing gastric outlet obstruction.…”

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confidence: 99%

Peutz-Jeghers Syndrome Causing Gastroduodenal Intussusception : A Rare Case Report

Chaudhary,

Agrawal,

Situ

et al. 2023

J. Nepal Paedtr. Soc.

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Peutz-Jeghers syndrome (PJS) is a rare disease characterized by melanotic macular lesions over the pulp of fingers, in oral cavity mucosa, lips, and hamartomatous gastric and intestinal polyps. They usually present with intestinal intussusception or intestinal bleeding; gastroduodenal intussusception is an infrequent presentation. Endoscopic resection or complete surgical excision of the polyp is the treatment of choice. We here present a case of a seven year male with multiple gastric polyps presenting as gastric outlet obstruction.

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Double intussusception in a teenage child with Peutz-Jeghers syndrome: A case report

Cited by 5 publications

References 14 publications

Peutz–Jeghers Syndrome

Peutz–Jeghers Syndrome

Peutz Jeghers syndrome with multiple intussusceptions

Peutz-Jeghers Syndrome Causing Gastroduodenal Intussusception : A Rare Case Report

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