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Year : 2017  |  Volume : 22  |  Issue : 4  |  Page : 245-247

Solitary Peutz–Jeghers polyp of jejunum: A rare cause of childhood intussusception

1 Department of Pediatric Surgery, Postgraduate Institute of Medical Education and Research, Chandigarh, India
2 Department of Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
3 Department of Pediatric Gastroenterology, Postgraduate Institute of Medical Education and Research, Chandigarh, India

Date of Web Publication12-Sep-2017

Correspondence Address:
Prema Menon
Department of Pediatric Surgery, Advanced Pediatric Centre, P.G.I.M.E.R., Chandigarh - 160 012
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0971-9261.214442

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An extremely rare case of solitary jejunal Peutz–Jeghers polyp causing intussusception in an 8-year-old boy is reported. The polyp was excised by laparoscopic-assisted surgery. This appears to be only the fourth and the youngest patient with such a polyp reported in the indexed English language literature.

Keywords: Child, intussusception, jejunum, Peutz–Jeghers syndrome, solitary Peutz–Jeghers polyp

How to cite this article:
Kalavant AB, Menon P, Mitra S, Thapa BR, Narasimha Rao KL. Solitary Peutz–Jeghers polyp of jejunum: A rare cause of childhood intussusception. J Indian Assoc Pediatr Surg 2017;22:245-7

How to cite this URL:
Kalavant AB, Menon P, Mitra S, Thapa BR, Narasimha Rao KL. Solitary Peutz–Jeghers polyp of jejunum: A rare cause of childhood intussusception. J Indian Assoc Pediatr Surg [serial online] 2017 [cited 2022 Sep 26];22:245-7. Available from: https://www.jiaps.com/text.asp?2017/22/4/245/214442

   Introduction Top

Small intestinal polyps are rare in children and are usually a part of polyposis syndromes, including Peutz–Jeghers (PJ) syndrome.[1] In 1962, a solitary polyp with a similar histology as that seen in PJ syndrome was reported without the other clinical features of the syndrome and was then termed as a solitary or isolated PJ polyp.[2] We report such a case in a young boy who presented with jejunal intussusception.

   Case Report Top

An 8-year-old boy was referred with four episodes of melena over the past 10 months, fatigability and refractory anemia. There was no history of abdominal pain, loss of weight/appetite, or drug intake. There was no history suggestive of bleeding dyscrasia or family history suggestive of PJ syndrome. On clinical examination, pallor was noted without perioral mucocutaneous pigmentation. Abdominal examination was normal. All hematological and biochemical investigations were within normal limits, except for hemoglobin of 6.5 g/dL, and stool examination positive for occult blood. Ultrasonography of the abdomen, upper and lower gastrointestinal (GI) endoscopy, and Tc99m Meckel's isotope scan were normal. Capsule endoscopy visualized an approximately 2 cm × 2 cm, single, sessile, submucosal polypoidal lesion in the small bowel. Contrast-enhanced computerized tomography (CECT) enteroclysis done a few days later showed small bowel intussusceptions. On laparoscopy, a 20 cm long jejunojejunal intussusception starting 10–15 cm from the duodenojejunal flexure was seen. This was reduced laparoscopically. An intraluminal mass could be appreciated in this segment. No other abnormality was detected. The involved bowel was delivered through the umbilical camera port incision [Figure 1] and resection anastomosis performed. The histopathological examination of the polyp was suggestive of a PJ polyp [Figure 2]. There were areas of pseudoinvasion in the lamina, submucosa, and muscularis propria.
Figure 1: Intraoperative photograph shows the jejunal loop delivered through the umbilical incision with indentation on the serosal surface. Resected cut open segment of jejunum shows a sessile, 2 cm × 2.1 cm × 1.3 cm, horseshoe-shaped polyp with irregular surface extending up to the serosal surface

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Figure 2: Scanner-view photomicrograph of the jejunal polyp (H and E) shows villiform surface projections traversed by arborescent smooth muscle fibers (

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He had an uneventful postoperative period. Although we could not do a specific chromosomal analysis on the patient for PJ syndrome, a family screening was negative. There was no family history of cancers in the GI tract or elsewhere. At 3-year follow-up, he remains asymptomatic with no manifestations of PJ syndrome.

   Discussion Top

Intestinal polyposis in children is associated with several genetic and nongenetically linked syndromes although the most common intestinal polyp is the juvenile “inflammatory” polyp occurring in the large intestine.[1] PJ syndrome is associated with polyposis in the large and small intestine with nearly 25%–50% patients being diagnosed in childhood or adolescence. The WHO criteria include more than three histologically typical intestinal polyps, i.e., hamartomatous polyps with epithelial elements in the submucosa, muscularis propria, and subserosa, frequently surrounded by mucin-filled spaces and branching core of smooth muscle with a lining of normal intestinal epithelial cells, mucocutaneous hyperpigmentation, and a positive family history.[1],[3] It has an autosomal dominant inheritance and usually presents with anemia or colicky pain by adolescence.[1]

Isolated or solitary PJ polyp, usually noted in older individuals, may present with intestinal obstruction including intussusception, especially in the duodenum and distal colon.[3],[4],[5] The youngest patients reported with a solitary PJ polyp causing duodenal and ileal intussusception appears to be a 3-year-old girl and a 13-year-old girl, respectively.[6],[7] Our case appears to be the youngest patient with a solitary jejunal PJ polyp in the indexed English language literature [Table 1].[3],[8],[9] Laparoscopic reduction of intussusception and excision of the lesion through a small periumbilical incision has also not been previously reported.
Table 1: Details of reported cases of solitary Peutz-Jeghers jejunal polyp

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It is unclear whether the solitary polyp is a separate entity or a subset of PJ syndrome. Mutation of the STK11 (LKB1), a tumor suppressor gene seen in up to 70% patients in the syndrome, has so far not been seen in isolated polyps.[7],[10] Although mostly considered benign, Sekino et al. after reviewing 27 cases aged 22–84 years with solitary duodenal PJ polyp reported malignant transformation in four (14.8%) cases and suggested screening for malignancy and excision of all polyps.[3],[5] A unique feature of a PJ syndrome polyp is pseudoinvasion which mimics malignant invasion, and patients can be mistakenly treated for small intestine cancer.[1] This epithelial invasion of the wall of the intestine was noted in our patient also.

The exact incidence of solitary PJ polyps is unknown as only symptomatic patients seek medical attention. The majority, being located in the duodenum or colon, can be diagnosed and removed by endoscopy. Investigating a small intestine polyp is, however, a challenging task. Upper GI contrast study with follow through and CECT with enteroclysis is useful. Capsule endoscopy as seen in our case helped in not only identifying the lesion but also confirmed the absence of other GI tract polyps.

A solitary PJ polyp though very rare should be kept in mind as a differential diagnosis in intestinal polyps causing small bowel intussusception. They should be excised not only to prevent future intestinal obstruction and bleeding but also for a rare possibility of malignant transformation. Regular follow-up is essential in young children so that future manifestations of the PJ syndrome are not missed.

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There are no conflicts of interest.

   References Top

Adolph VR, Bernabe K. Polyps in children. Clin Colon Rectal Surg 2008;21:280-5.  Back to cited text no. 1
Gannon P, Dahlin D, Bartholomew L, Beahrs O. Polypoid glandular tumors of the small intestine. Surg Gynecol Obstet 1962;114:666-72.  Back to cited text no. 2
Burkart AL, Sheridan T, Lewin M, Fenton H, Ali NJ, Montgomery E. Do sporadic Peutz-Jeghers polyps exist? Experience of a large teaching hospital. Am J Surg Pathol 2007;31:1209-14.  Back to cited text no. 3
Oncel M, Remzi FH, Church JM, Goldblum JR, Zutshi M, Fazio VW. Course and follow-up of solitary Peutz-Jeghers polyps: A case series. Int J Colorectal Dis 2003;18:33-5.  Back to cited text no. 4
Sekino Y, Inamori M, Hirai M, Suzuki K, Tsuzawa K, Akimoto K, et al. Solitary Peutz-Jeghers type hamartomatous polyps in the duodenum are not always associated with a low risk of cancer: Two case reports. J Med Case Rep 2011;5:240.  Back to cited text no. 5
Ho CA, Chin F, Gunn A, Alhagi VM, Sanmugam A. Duodeno-jejunal intussusception due to a solitary Peutz-Jeghers polyp in a 3-year-old Malaysian girl. J Pediatr Surg Case Rep 2013;1:254-7.  Back to cited text no. 6
Retrosi G, Nanni L, Vecchio FM, Manzoni C, Canali R, Busato G, et al. Solitary Peutz-Jeghers polyp in a paediatric patient. Case Rep Gastroenterol 2010;4:452-6.  Back to cited text no. 7
Sone Y, Nakano S, Takeda I, Kumada T, Kiriyama S, Hisanaga Y. Solitary hamartomatous polyp of Peutz-Jeghers type in the jejunum resected endoscopically. Gastrointest Endosc 2000;51:620-2.  Back to cited text no. 8
Ter Borg PP, Westenend PP, Hesp FW, van der Straaten FF, van de Vrie WW, Honkoop PP. A solitary Peutz-Jeghers type polyp in the jejunum of a 19 year-old male. Cases J 2008;1:68.  Back to cited text no. 9
Jenne DE, Reimann H, Nezu J, Friedel W, Loff S, Jeschke R, et al. Peutz-Jeghers syndrome is caused by mutations in a novel serine threonine kinase. Nat Genet 1998;18:38-43.  Back to cited text no. 10


  [Figure 1], [Figure 2]

  [Table 1]

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