Intussusception of the small bowel associated with nephrotic syndrome

Abstract

We report the case of a 2-year-old boy who developed a small bowel intussusception during treatment failure of his first episode of nephrotic syndrome. Despite the absence of typical symptoms other than abdominal pain, the intussusception was diagnosed by ultrasonography and computed tomography and successfully reduced by air enema. No pathological lead point was discovered, and no symptoms of Henoch-Schönlein purpura developed later. Intussusception should be considered in the differential diagnosis of abdominal pain in patients with nephrotic syndrome, especially in patients exhibiting prolonged edema. Ultrasonography or computed tomography should be performed, even in the absence of other typical symptoms suggestive of intussusception. We should also bear in mind that the intussusception associated with nephrotic syndrome might occur at regions other than the typical ileocolic region, such as within the small intestine.

A Child With Nephrotic Syndrome and Abdominal Pain

“…The clinical differences compared to classic, idiopathic intussusception include atypical locations (ie, small bowel), absence of a palpable mass due ascites, older age at presentation, and absence of the classic triad of symptoms. 2 The first confirmed case of intussusception associated with nephrotic syndrome was diagnosed at autopsy in 1975, and subsequently a few case reports have been described in the literature. 3 …”

“…2,6–8 The precise mechanism for the development of intussusception in nephrotic syndrome is incompletely elucidated, but may involve a combination of generalized bowel wall edema as well as local factors such as intramural lymphoid hyperplasia in the presence of ascites or infection. In a review of published cases of intussusception associated with nephrotic syndrome, Asai et al 2 noted that intussusception occurred most commonly when generalized edema had been present for 2 or more weeks. This was the case in our patient, who developed acute abdominal pain after approximately 3 weeks of edema.…”

The reversal of intussusception associated with nephrotic syndrome by infusion of albumin

“…We have read with great interest the article by Asai et al in Pediatric Nephrology [1] in which the authors describe a 2-year-old boy who developed a small bowel intussusception during the treatment of nephrotic syndrome. Based on their observations, they suggest that intussusception should be considered in the differential diagnosis of abdominal pain in such patients and that intussusception may occur in regions other than the typical ileocolic region.…”

“…In general, treatment with air can be performed first, but if this fails, manual treatment with laparotomy should be performed. Although the intussusception was successfully reduced by air enema by Asai et al [1], small bowel intussusception usually requires surgery as liquids or air are unlikely to maintain their pressure after having passed the ileocecal valve; consequently, nonoperative reduction modalities are usually unsuccessful in such cases [5]. In our case, it was very difficult to decide the treatment modality because it was thought that surgery would be effective, but more dangerous due to SBP, and that nonoperative reduction would not be an effective treatment.…”

“…Reymond proposed a 'pivot (lead point) and torque' theory for the development of this condition [2]. The most popular hypothesis is that a combination of patches of bowel wall edema and peristaltic incoordination could provide the pivot and torque in patients with nephrotic syndrome [1,3]. In addition, peritonitis combined with nephrotic syndrome can induce intramural lymphoid hyperplasia, thereby increasing the risk of lead point formation [4].…”