Journal of Indian Association of Pediatric Surgeons
Journal of Indian Association of Pediatric Surgeons
                                                   Official journal of the Indian Association of Pediatric Surgeons                           
Year : 2023  |  Volume : 28  |  Issue : 3  |  Page : 250--252

Small bowel intussusception due to solitary Peutz–Jeghers Jejunal polyp: A rare entity

Bharat Kamath, Samprathi Doddamallappa, Arpit Roy, Sudhir Dhobale 
 Department of Surgery, HBT Medical College and RN Cooper Hospital, Mumbai, Maharashtra, India

Correspondence Address:
Samprathi Doddamallappa
Department of Surgery, HBT Medical College and RN Cooper Hospital, 3rd Floor, “D” Wing, U 15, Bhaktivedanta Swami Road, JVPD Scheme, Juhu, Mumbai - 400 056, Maharashtra


Peutz–Jeghers syndrome (PJS) is characterized by mucocutaneous pigmentation with multiple hamartomatous polyps of the gastrointestinal tract and/or a familial history with autosomal dominant inheritance pattern having incomplete penetrance, with some cases arising from spontaneous mutations. We present this case of a 12-year-old female who presented with jejunojejunal intussusception and on exploration revealed a polypoidal mass around 50 cm from duodenojejunal flexure acting as a lead point. A segmental jejunal resection with anastomosis was performed and histopathology confirmed a solitary Peutz–Jeghers (PJ) hamartomatous polyp. She neither had mucocutaneous pigmentation nor family history of PJS or any other polyps anywhere in the gut on further evaluation by endoscopies. A solitary PJ polyp in the jejunum is a rare entity, and to the best of our knowledge, only around 13 cases have been reported in world literature so far. Regular follow-up is essential in young children, so that future manifestations of the PJS are not missed.

How to cite this article:
Kamath B, Doddamallappa S, Roy A, Dhobale S. Small bowel intussusception due to solitary Peutz–Jeghers Jejunal polyp: A rare entity.J Indian Assoc Pediatr Surg 2023;28:250-252

How to cite this URL:
Kamath B, Doddamallappa S, Roy A, Dhobale S. Small bowel intussusception due to solitary Peutz–Jeghers Jejunal polyp: A rare entity. J Indian Assoc Pediatr Surg [serial online] 2023 [cited 2023 Sep 30 ];28:250-252
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Full Text


Peutz–Jeghers syndrome (PJS) is a rare autosomal dominant genetic disease characterized by hamartomatous gastrointestinal (GI) polyposis (nearly 90% of patients) most commonly in the small bowel followed by colon, stomach, and rectum along with mucocutaneous pigmentation characterized by dark blue or brown macules most commonly seen at the vermillion border of the lips, buccal mucosa, hands, and feet and a familial history. PJS patients have a 90% lifetime risk of cancer, including colorectal (most common), gastric, pancreatic, lung, breast, uterine, cervical, testicular, and ovarian.[1]

Peutz–Jeghers (PJ) polyp is defined as a unique hamartomatous polyp having the same histological features as PJS polyps without associated mucocutaneous pigmentation or family history of PJS. As per literature, its most common site is duodenum (around 30 cases), followed by stomach (around 14 cases reported), jejunum (around 13 cases reported), colon (around 5 cases reported), and rectum. It is a rare condition where the incidence, prevalence, genetic, and hereditary predisposition and familial history have not been evaluated due to inadequate data.

 Case Report

A 12-year-old female presented to the emergency department with complaints of colicky periumbilical pain, nonbilious vomiting, and mild abdominal distension of 3 days' duration. The patient had mild tachycardia with a soft, mildly distended nontender abdomen. Ultrasound of the abdomen was suggestive of jejunojejunal intussusception with classical target sign.

The patient was taken up for exploratory laparotomy. A near completely reduced intussusception was seen intraoperatively with a polypoidal mass acting as a lead point approximately 50 cm from the duodenojejunal flexure. Rest of the bowel looked healthy. Segmental resection and jejunojejunal anastomosis was done. The cut open specimen revealed a solitary polyp with narrow base and tree-like appearance. [Figure 1]. Postoperative course was uneventful. Contrast-enhanced computed tomography of the abdomen and both upper and lower GI endoscopy were done to rule out polyps or growth elsewhere in the GI tract. Histopathological examination revealed solitary PJ hamartomatous polyp with characteristic leaf-like branching. [Figure 2]. The patient is on regular follow-up over a period of 14 months and is asymptomatic.{Figure 1}{Figure 2}


Jeghers et al. first described PJS which is a rare autosomal dominant genetic disease characterized by mucocutaneous pigmentation of mouth, lips, hands, and feet and familial GI polyposis. GI polyps are classified into hyperplastic, inflammatory, adenomatous, and hamartomatous polyps. Hamartomatous polyps are extremely rare and mainly occur in PJS. There were some cases where hamartomatous polyps were confirmed by pathology in the absence of mucocutaneous pigmentation or a family history of digestive tract diseases.[2] Since they lack the characteristics of PJS, they are known as solitary PJ polyp.[3]

The PJ polyp is a rare entity and the cases that were documented were only after the patient was symptomatic, and hence a large population of patients with solitary PJ polyps remain undiagnosed.[4] It is unclear whether the presence of a solitary PJ polyp in the intestine is considered an incomplete form of PJS or a separate entity, because of the absence of associated clinical features and positive family history as seen in PJS.

The pathological feature of solitary PJ polyp is the presence of smooth muscle bundles from the muscularis mucosae extending into the polyp to form a typical branch-like structure covered by almost normal mucosa. In the previously reported cases in world literature, the age group ranges from 3 months to 43 years. Our patient here presented with neither mucocutaneous pigmentation nor positive family history and was therefore characterized as a case of solitary PJ polyp without a specific chromosomal analysis.

The malignant potential of solitary PJ polyp remains unclear although 2 cases have been documented in the world literature.[5] So far, only 13 cases of the solitary jejunal PJ polyp have been reported in the indexed world literature and our case appears to be the fourteenth case of solitary PJ hamartomatous polyp.


The probable diagnosis of a solitary PJ polyp comes from a characteristic hamartomatous polyp without familial history and mucocutaneous pigmentation which differentiates it from PJS. A solitary PJ polyp should be kept in mind as a differential diagnosis in intestinal polyps causing small bowel intussusception. A regular follow-up is essential in young children so that future manifestations of the PJS are not missed.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.


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