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Idiopathic jejunojejunal intussusception at three sites in a 5 Year-old child

Patoulias Ioannis, Prodromou Katerina, Feidantsis Thomas, Koutsoumis Georgios, Kallergis Konstantinos
Aristotle University of Thessaloniki, 1st Pediatric Surgery Department, Thessaloniki, Greece



Katerina Prodromou, MD
Aristotle University of Thessaloniki
1st Pediatric Surgery Department
Thessaloniki, Greece
E-mail: This email address is being protected from spambots. You need JavaScript enabled to view it. .



Intussusception is the invagination of the intestine into its lumen. Jejunojejunal intussusception is one of the rarest forms of intussusception in childhood, as it occurs in less than 1% of all cases. It usually concerns children older than the age of three and it is often secondary. In this study, we present the case of a 5 year-old boy with an idiopathic jejunojejunal intussusception at 3 sites. Reviewing literature, the rarity of this type of intussusception emerged, as to our knowledge no similar case has been reported.

Keywords: jejunojejunal intussusception, child, idiopathic, secondary



Intussusception is the second most frequent cause of intestinal obstruction preceded only by pyloric stenosis. The incidence in the USA is 56 cases /100.000 children annually. About 67% of all intussusceptions occur in infancy and it is idiopathic in 94-95% of the cases [1]. The incidence of intussusception in children older than 1 year increases to 35% and in 20-25% of children older than 3 years a lead point is present [2, 3]. The most frequent type of intussusception in children younger than the age of 3 is ileocolic- in the case of ileocecal intussusception the appendix is part of the intussusceptum. The next in frequency is ileocolic, followed by colocolic, jejunoileal and ileoileal types. Duodenojujenal and jejunojujenal intussusceptions are extremely rare. In general, small intestine intussusception occurs in children older than 3 years old [3].

After careful overview of literature, no reported study concerning a jejunojejunal intussusception occurring at three different sites of the intestine in a child has come to our attention [3, 4, 5].

Case report

A 5 year old male child presented to our clinic’s emergency department with an intermittent colicky, cramping abdominal pain of acute onset. He had a disease free personal history and cystic fibrosis was excluded during neonatal period. Ten days prior to this incident, the patient presented with fever and cough and was diagnosed with an upper respiratory system infection. He was treated with Cefprozil per os (50 mg/kg/d). The patient appeared severely ill and restless and had acute abdomen on examination. There was neither a palpable abdominal mass nor evidence of blood at rectal examination. Blood test results revealed an increase in blood cell count (WBC 32630/μl, NE 85.6%) and an elevated CRP (CRP: 6 mg/dl, normal values under 5). The rest of the laboratory findings were within normal limits. Abdominal x-ray showed a non-obstructive ileus of the small intestine and the existence of gas in the ascending colon (fig. 1). On US scan two target signs on the bowel were detected, indicating intussusception at more than one site (fig. 2).

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Figure 1. Abdominal plain

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Figure 2. Ultrasonography

Preoperatively the child was administrated intravenous fluids (Ringer’s Lactate solution) and Cefuroxime at a dosage of 50mg/kg. He underwent laparotomy through a right lower quadrant incision. During open exploration of the abdomen, jejunojejunal intussusception was found at 3 different sites: the first was situated at 70 cm from the duodenojejunal curve, it was “loose” and the intussuscepted mass was 3cm long. The second was at 55cm, “tight” and 4cm long (fig. 3) and the third intussusception was found at 40 cm from the duodenojejunal curve, 2 cm long and “loose” (fig. 4).

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Figure 3. Jejunojejunal

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Figure 4. Third intussusception

The serosa had obvious venous congestion but no ischemic lesions. All three intussusceptions were reduced by manipulation. The “freed” (reduced) intestine did not appear ischemic. Careful “manual” exploration for a palpable lead point on the intestine mucosa or on the mesentery was performed with no abnormal findings. Meckel’s diverticulum was not present. Enlarged mesenteric lymph nodes were present near the ileocecal valve, whereas no enlarged lymph nodes of the jejunal mesentery were noticed. Appendectomy was performed. Postoperative period was uneventful and the young patient was released from the clinic on the 7th postoperative day. Discussion A seasonal increase in incidence of idiopathic intussusception has been documented, with peaks during spring, summer and mid-winter, possibly due to the increased incidence of upper respiratory and gastrointestinal tracts’ infections during the same time periods [6]. It often occurs after an adenovirus (type 3) infection of the upper respiratory tract, which causes swelling of the mesenteric lymphoid tissue and hypertrophy of Payer’s patches within the intestinal wall. These may be responsible for the onset of intussusception through alterations of the intestinal mobility by either the invagination of the part of the intestine that has increased mobility into the lumen of the following part or the local “stretching” of the intestinal wall due to the enlargement of the lymphoid tissue and Payer’s patches.

In a study by Spiro et al. [7] it is pointed out that the administration of cephalosporine increases the risk for intussusception by 20 times.

Possible lead points in secondary intussusception are:

• Meckel’s diverticulum

• Submucosal hemorrhages in Henoch-Schönlein purpura

• Submucosal hematoma due to injury [3] or bleeding disorder [8]

• Intestinal duplications • Polyps (Juvenile or Peutz-Jeghers syndrome) [9]

• Benign or malignant tumors (leiomyoma, fibromyoma, schwannoma, lymphoma). A general rule that must always be kept in mind is that in every child over six years old, that presents with intussusception, lymphoma should always be excluded [2, 10]

• Mesenteric cyst

• Cystic fibrosis (inspissated secretions and thick fecal matter in the intestinal lumen may act as a lead point)

• Hemangioma or lymphangioma.1.4-2.4% of childhood tumors that are located on the small bowel wall are lymphangiomas [11].

• Appendix Multiple lead points relate to cystic fibrosis or Henoch-Schönlein purpura [1].

The classic clinical manifestation of intussusception is present in half of the cases and it includes acute intermittent abdominal pain (98% of cases), vomiting (70%), fever (12%) and “currant jelly” stools (37%) [12]. Physical examination reveals an abdominal mass (32%), blood mixture in stool at rectal examination and intestinal paresis (12%) [12]. Our patient’s main symptom was intense abdominal pain. We believe that full presentation of the disease’s symptoms was not present because diagnosis was set six hours after the onset of the symptoms. There were no palpable abdominal masses as the intussusceptions were located in the left upper abdominal quadrant and were short in length. Ultrasonography is the basic screening tool for the diagnosis of intussusception.

It has 98-100% reported sensitivity, 88-199% specificity and a 100% negative prognostic value [13, 14]. The “multiple concentric “ring sign” or “target sign” in a transverse sonographic image of the bowel, as well as the “doughnut lesion” or “pseudokidney sign” on a longitudinal section, are characteristic findings of this condition [13, 14]. In addition, Doppler US gives information about the blood flow of the intussuscepted intestine. The absence of blood flow as well as the presence of trapped peritoneal fluid within an intussusception is indicative of intestinal ischemia [13, 14]. In our patient’s case, 2 out of 3 target signs were present on the US without any signs of intestinal ischemia. Once a second site of intussusception is recognized, a lead point is discovered in 66% of cases and a precise diagnosis is set in 48% of cases [2, 3, 13, 14]. During surgery, after the reduction of all 3 intussusceptions, a careful exploration of the intestine was performed and no lead points were found.

The authors of this report found this case challenging, not only because of its rarity but also because of the atypical presentation of intestinal intussusception as acute abdomen. Furthermore, idiopathic jejunojejunal intussusception at multiple sites in childhood has not been reported, to our knowledge, so far.


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