Intussusception on Small Bowel Examinations in Children

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1 299 Alan Daneman1 Bernard J. Reilly1 MemI de Silva2 Patrick OIutola1 Received December 1 8, ; accepted after revision April 1 9, Department of Radiology, Hospital for Sick Children, 555 University Ave., Toronto, Ontario M5G 1 X8. Address reprint requests to A. Daneman. 2 Department of Radiology, Royal Alexandra Hospital for Children, Sydney, Australia. AJR 1 39: , August X/82/ $00.00 American Roentgen Ray Society Intussusception on Small Bowel Examinations in Children Six children in whom an intussusception was diagnosed on a small bowel followthrough examination are presented. The radiographic signs of intussusception on such a study include: (1 ) a narrow channel of barium representing the compressed lumen of the intussusceptum, (2) a soft-tissue mass on either side of this channel due to hypertrophy and edema of the walls of the intussusceptum and intussuscipiens, (3) a coil spring appearance around the narrow channel, and (4) a mass lesion at the distal end of the narrow channel. Not all of these signs are present in each case. Intussusception is a dynamic process and the value of delayed films and frequent fluoroscopy during small bowel follow-through studies in these cases is stressed. All six cases had a demonstrable lesion as a lead point. Jejunal intussusceptions are usually caused by benign lesions-malignancy being found almost exclusively in distal intussusceptions. Intussusception in children is usually ileocolic or ileoileocolic in nature and is usually confirmed or diagnosed by barium enema study. Its radiographic appeamances have been well described and illustrated. Finding an intussusception on a small bowel follow-through study is an unusual event. Few descriptions and illustrations are found in the literature [1-7]. In a review of 233 intussusceptions at the Hospital for Sick Children, Toronto, Humphry and Reilly [8] found 28 small bowel into small bowel intussusceptionsnone of these had a follow-through study. The purpose of this paper is to describe and illustrate the radiographic findings in six children in whom an intussusception was diagnosed on a small bowel follow-through examination. Five of these cases were seen at the Hospital for Sick Children, Toronto, Canada, and the sixth at the Royal Alexandra Hospital for Children, Sydney, Australia. Case Case 1 Reports A 1 0-year-old boy with Peutz-Jegher syndrome was seen at the Royal Alexandra Hospital for Children, Sydney, with chronic intermittent abdominal pain. A small bowel follow-through study revealed a partial obstruction in the proximal jejunum where barium passed through a long narrowed channel of barium to the right of the midline (fig. 1 A). A soft-tissue mass was noted on either side of this channel. This was interpreted as being a jejunojejunal intussusception. The follow-through study was repeated and again revealed a high-grade obstruction in the proximal jejunum with the same narrowed channel passing, this time, to the left of the midline and into the pelvis (fig. 1 B). The mass effect was not as evident at this examination. In delayed films the barium was noted to have passed completely through the narrowed segment into dilated distal loops. At operation, a long segment jejunojejunal intussusception was noted and the bowel wall associated with this intussusception was markedly thickened-probably related to its chronicity. A large hamartomatous polyp was present at the lead point of this intussusception.

2 . 300 DANEMAN El AL. AJR:139, August 1982 I A Case 2., A 32-month-old girl was admitted with colicky abdominal pain and bilious vomiting. Plain films of the abdomen revealed gas in the stomach and in a single loop of proximal jejunum, which was markedly dilated. A barium small bowel follow-through examination revealed complete obstruction at the distal end of the dilated jejunal loop (fig. 2A). No polyp or intussusception was evident initially, but delayed films showed that barium had passed distally through a narrowed channel, which was surrounded by a mass effect, and at the distal end of this channel there was a suggestion of a polyp (fig. 2B). At operation, a jejunojejunal intussusception was present. The polyp at the lead point was resected and was found to be consistent with the type seen in Peutz-Jegher syndrome. Case 3 A 1 0-year-old girl with Peutz-Jegher syndrome was admitted with a history of recurrent abdominal pain and nausea. A small bowel follow-through study revealed a large polyp in the proximal jejunum (fig. 3A). During fluoroscopy of this area, this polyp was noted to intussuscept intermittently (fig. 3B). Further areas of intussusception were noted to occur intermittently in the more distal parts of the small bowel as well. At laparotomy, multiple hamartomatous polyps were removed from the small bowel. Case 4 A 1 6-year-old boy with Schbnlein-Henoch purpura complained of severe abdominal pain and a large amount of blood loss in the.. \.-, -t , rnr..... Fig. 1 -Case 1. A, Follow-through study. Narrow channel in jejunum to right of midline (arrows) with surrounding mass effect. (Barium in left colon is from previous enema at another hospital.) B, Repeat examination. Markedly dilated jejunal loops with abrupt caliber change where this enters intussusception, this time passing to left (arrow). Diagnosis: long jejunojejunal intussusception due to hamartoma in Peutz- Jegher syndrome. stool. A small bowel follow-through study revealed multiple areas of hemorrhage, edema, and narrowing in the upper small bowel. In the mid-small bowel, the barium passed through a narrowed channel, and this was surrounded by a coil spring appearance (fig. 4). There was no obstruction at the site of this intussusception. Delayed films revealed no evidence of the intussusception, which must have reduced spontaneously. Case 5 A 7-year-old girl had bilious vomiting and central abdominal pain for 1 week. A right upper quadrant mass was palpable. Sonography suggested the presence of a bowel lesion in the right upper quadrant. A small bowel examination showed some dilated loops in the distal ileum. In the region of the terminal ileum, the barium passed through a markedly narrowed channel and then filled the large bowel in the region of the hepatic flexure (fig. 5). The narrowed channel was surrounded by a large irregular mass, and there was a suggestion of a coil spring appearance but the mucosa of this was very irregular (fig. SB). At operation, a large histiocytic lymphoma of the terminal ileum was found to be the lead point of an ileocecal intussusception. Case 6 A 4-year-old boy had 4 months of abdominal pain. A right upper quadrant mass was palpable. A small bowel follow-through examination revealed a narrowed channel of barium in the right upper quadrant where barium passed from the terminal ileum into the

3 AJR:139. August 1982 INTUSSUSCEPTION IN CHILDREN 301 B A Fig. 3.-Case 3. A, Barium follow-through study. Polyp (arrows) in prox- became dilated. and coil spring appearance was noted. Diagnosis: hamarimal jejunum. B, During fluoroscopy, intussusception was noted intermittently toma, Peutz-Je9her syndrome. at this site. Polyp was noted to intussuscept, more proximal loop of jejunum B

4 302 DANEMAN El AL. AJR:139, August 1982 transverse colon (fig. 6). This channel was surrounded by an irregular mass with distortion of the mucosa. At laparotomy, a cecocolic intussusception was present, and the lead point was a large histiocytic lymphoma of the cecum. Discussion Fig. 4.-Case 4. A, Barium follow-through study. Barium passes through narrowed channel surrounded by coil spring appearance in mid-small bowel. No obstruction. Diagnosis: mid-small bowel intussusception due to Schdnlein-Henoch purpura. Findings on a small bowel follow-through study that mdicate the presence of an intussusception include: (1 ) a narrow channel of barium representing the compressed lumen of the intussusception, seen in five of our cases (figs. 1, 2, 4-6); (2) a mass on either side of this narrow channel due to hypertmophy and edema ofthe walls ofthe intussusceptum and intussuscipiens, well visualized in four of our cases (figs. 1, 2, 5, 6); (3) a coil spring appearance around the narrow channel due to barium lining the valvulae of the opposing walls of the intussuscipiens and intussusceptum, noted in three of our cases (figs. 3-5); and (4) barium outlining a mass at the distal end of the narrow channel, seen with some certainty in only three of our cases (figs. 2, 5, 6). It can be seen that not all of the above signs are present in each case. If the intussusception is relatively fixed and tight due to edema or hypertrophy of the involved bowel wall, the coil spring appearance may not be present. In this situation, only the central narrow channel and surrounding mass effect may be visualized (figs. 1 and 2). The narrow Fig. 5.-Case 5. Barium follow-through study. Barium passes through narrowed channel (arrows) in region of terminal ileum into hepatic flexure. Channel surrounded by irregular mass; apparent coil spring appearance (arrowheads) shows marked mucosal distortion. Diagnosis: ileocolic intussusception due to histiocytic lymphoma of terminal ileum.

5 AJR:139, August 1982 INTUSSUSCEPTION IN CHILDREN 303 Lead Points Fig. 6.-Case 6. Small bowel study. Barium passes through narrowed channel into colon (arrows), surrounded by irregular mass and distortion of mucosal pattern. Dignosis: cecocolic intussusception due to histiocytic lymphoma of cecum. channel extends from the central axis of the dilated proximal lumen, which usually tapers sharply and symmetrically at the point of entry into the intussusception [2]. The coil spring appearance is more likely to be seen if the intussusception is loose and barium passing through the narrow channel is able to coat the opposing mucosal surfaces of the intussusceptum and intussuscipiens (figs. 3 and 4). In addition, an intussusception is a dynamic process and the appearance may change with time. Early films may just reveal an intestinal mass lesion (fig. 3A) or may show an obstruction with nonspecific appearances (fig. 2A). The true nature of the problem, that is, the typical appearances of the intussusception, may only become evident on delayed films (fig. 26) or at fluomoscopy (fig. 3B). Rarely transient, nonobstructive i ntussusceptions may be seen in the small bowel [1, 3, 5, 7]. The significance of these is debatable because these children may have no abdominal discomfort [1 ]. These intussusceptions may be multiple or single, usually involve short segments of bowel, and may disappear in seconds to minutes. Demonstrable lead points are not usually present. Cases 3 and 4 were noted to have transient intussusceptions during the small bowel study. The degree of obstruction and, hence, dilatation of bowel proximal to the small bowel intussusception varies and appears to be greater in the more proximal intussusceptions. A demonstrable lesion acting as a lead point has been found in only 5% of all cases of intussusception occurring in the pediatric age group [9]. The incidence of demonstrable lead points increases dramatically in intussusceptions in the proximal small bowel, in those occurring in the neonatal period [1 0], and in children older than 2 years [6, 9]. All of our six cases had a pathologic lesion as a lead point. At the time of presentation, one of the cases was 2 years and 8 months, one was 4 years, and the ages of the other four were years. A jejunojejunal intussusception was noted in three cases due to polyps associated with the Peutz-Jegher syndrome. A review of 1 3 large series of pediatric intussusceptions, which included 3,000 cases, revealed six cases of jejunal intussusception [6]. Five of these had no lead point and one had a duplication as the lead point. Other causes of jejunal intussusception in children include polyposis syndromes, isolated adenomatous polyps, and a single case report of a polypoid adenocarcinoma [6]. Case 4 is an example of a mid-small bowel intussusception due to Sch#{246}nlein-Henoch pumpura. Lindenauer and Tank [1 1 ] found SO cases of intussusceptions in SchOnlein- Henoch pumpuma recorded in the literature up until 1966; 50% were ileoileal intussusceptions. Glasier et al. [1 2] found an intussusception in three of 22 patients with Schbnlein- Henoch pumpuma; one was diagnosed on a small bowel follow-through ACKNOWLEDGMENTS study. Most ileocolic and ileoileocolic intussusceptions are diagnosed by barium enema studies. Recently, Humphry et ai. [4] reported two cases with idiopathic ileocolic intussusception seen at the Hospital for Sick Children, Toronto. Both had atypical clinical findings, and the intussusception was first diagnosed by a barium small bowel follow-through study. A similar case was reported by Rees and Lan [13]. The child had prolonged symptoms and the intussusception was diagnosed on a follow-through study. No demonstrable lead point was found. In a review of over 800 children with an intussusception seen at the Hospital for Sick Children, Toronto, over 27 years, nine were found to have a lymphoma as a lead point of an ileocolic intussusception (S. H. Em, personal communication). This represents 1 7% of all cases with a pathologic lesion. Cases S and 6 are examples from this group. Finding an intussusception on a small bowel followthrough study is an unusual event, but it is probably much more common than the paucity of illustrations in the litematume might suggest. These cases are usually associated with a demonstrable lead point that in the jejunum is most likely benign. Malignant lesions occur almost exclusively in the more distal intussusceptions. The children usually present at an older age than in idiopathic ileocolic intussusception and tend to run a more chronic course. This paper has illustrated the radiographic findings in six such cases. We thank S. H. Em, Department of Surgery, Hospital for Sick Children, Toronto, for providing information regarding previous

6 304 DANEMAN ET AL. AJR:139, August 1982 cases seen at this Hospital and Gladys Clarke and Linda Gibbs for manuscript preparation. REFERENCES 1. Caffey J. Pediatric x-ray diagnosis, 7th ed. Chicago: Year Book Medical, 1978: Carlson HC. Small intestinal intussusception: an easily misunderstood sign. AJR 1970;i 10: Franken EA. Gastrointestinal radiology in pediatrics, 1 st ed. New York: Harper & Row, 1975: Humphry A, Alton DJ, McKendry JBJ. Atypical ileocolic intussusception dignosed by barium follow-through. Pediatr Radio! (in press) 5. Singleton EB, Wagner ML, Dutton AV. Radiology of the alimentary tract in infants and children, 2d ed. Philadelphia: Saunders, 1977: Stone DN, Kangarloo H, Graviss ER, Danis AK, Silberstein MJ. Jejunal intussusception in children. Pediatr Radio! 1 980;9 : Teitelbaum MD, Arenson N. Recurrent small intestinal intussusception. AJR 1 950;63 : Humphry A, Reilly BJ. Small bowel into small bowel intussusception. J Can Assoc Radio! 1973;24: Em SH. Leading points in childhood intussusception. J Pediatr Surg 1976;1 1 : Patriquin HB, Afshani E, Effman E, et al. Neonatal intussusception. Radiology 1977; Lindenauer SM, Tank ES. Surgical aspects of Henoch-Schbnlein purpura. Surgery 1966;59: Glasier CM, Siegel MJ, McAlister WH, Shackelford GD. Henoch-Schbnlein syndrome in children: gastrointestinal manifestations. AJR 1980;136: Rees BI, Lan J. Chronic intussusception in children. Br J Surg 1 976;63 :33-35

7 This article has been cited by: 1. Paul M. Columbani, Stefan ScholzIntussusception [CrossRef] 2. Artur Bijoś, Artur Mazur, Mieczysława Czerwionka-Szaflarska Rola badania ultrasonograficznego w wybranych chorobach przewodu pokarmowego u dzieci. Pediatria Polska 82:9, [CrossRef] 3. Sigmund H. Ein, Alan DanemanIntussusception [CrossRef] 4. Luis A. Martinez-Frontanilla, Gerald M. Haase, Joel A. Ernster, Wm. Carl Bailey Surgical complications in Henoch- Schönlein purpura. Journal of Pediatric Surgery 19:4, [CrossRef]

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