PEDIATRICS Vol. 111 No. 3 March 2003, pp. 682-685
EXPERIENCE AND REASON |
Medical Treatment of Recurrent Intussusception Associated With Intestinal Lymphoid Hyperplasia
* Departments of Pediatrics
Radiology
Pediatric Surgery
Hadassah Medical Center and the Faculty of Medicine
The Hebrew University
Jerusalem 91120 Israel
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ABSTRACT |
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 TOP ABSTRACT INTRODUCTIONCASE REPORTSDISCUSSIONCONCLUSIONREFERENCES |
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Intestinal lymphoid hyperplasia (ILH) is an uncommon cause of recurrent intussusception in infants and young children. Surgical treatment has been suggested in the management of this disorder.
We report 2 cases in which recurrent intussusception was associated with ILH. A short course of steroids resulted in resolution of both symptoms and hyperplasia.
We conclude that when recurrent intussusception occurs in association with ILH, and no other lead point can be identified, it is important that treatment with steroids is considered before resorting to a more radical surgical approach.
Key Words: intestinal nodular lymphoid hyperplasia • recurrent intussusception • steroids
Abbreviations: ILH, intestinal lymphoid hyperplasia • GI, gastrointestinal
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INTRODUCTION |
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TOPABSTRACTINTRODUCTIONCASE REPORTSDISCUSSIONCONCLUSIONREFERENCES |
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Intussusception is a major cause of intestinal obstruction among infants under the age of 2 years. In most cases no obvious predisposing lesion or lead point is evident. Hypertrophied Peyer’s patches caused by viral infection, most often enteric adenovirus,1 may act as a lead point in some cases. In contrast, recurrent intussusception is often caused by an underlying leading point protruding from the intestinal wall into the lumen, such as an intestinal polyp or intestinal mucosal edema as in Henoch-Schonlein purpura. One of the less common causes of recurrent intussusception is intestinal lymphoid hyperplasia (ILH).2
ILH (or lymphoid nodular hyperplasia or intestinal nodular hyperplasia) is a benign condition in which lymph follicles in the intestine enlarge and cause grossly visible protrusions of the mucosa. It may be asymptomatic3 or may be associated with abdominal pain, gastrointestinal (GI) hemorrhage, and intussusception.4,5
We describe 2 cases of recurrent intussusception associated with ILH in which the hyperplasia resolved and symptoms have not recurred after oral steroid treatment.
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CASE REPORTS |
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TOPABSTRACTINTRODUCTIONCASE REPORTSDISCUSSIONCONCLUSIONREFERENCES |
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Case 1
A 10-month-old infant girl was referred to the emergency department because of abdominal pain, vomiting, and bloody stools. Physical examination revealed, on rectal examination, current jelly stool, but was otherwise unremarkable. The infant had had 2 previous episodes of intussusception, at the age of 7 months and 8 months, which were diagnosed by ultrasound and reduced by air enema. A leading point was not identified at these events. An upper GI series with small bowel follow-through performed after the second episode demonstrated multiple small, round filling defects along the distal small bowel and the colon. A Meckel’s scan was negative.
In the current episode a barium enema demonstrated a midtransverse colon intussusception, which was completely reduced by air enema. A double-contrast barium enema, performed 4 days later for the investigation of a possible leading point, demonstrated multiple small, round filling defects along the right transverse colon and the distal small intestine, consistent with ILH (Fig 1). No other leading point was identified. The parents were informed regarding the different treatment alternatives, and the patient was treated with oral prednisone, 1 mg/kg/d, for 2 weeks, with gradual tapering during a additional 4-week period. A double-contrast barium enema preformed 2 months later showed marked decrease of ILH (Fig 2). There was no recurrence of intussusception during a 2-year follow-up period.
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Case 2
A 5-year-old boy presented with severe abdominal pain. Physical examination revealed tenderness and fullness at the right lower quadrant, but was otherwise normal. Abdominal ultrasound demonstrated a midtransverse colon intussusception, which was reduced by air enema. A leading point was not identified by either of these studies. The boy had had 2 previous episodes of intussusception. The first one was at the age of 18 months, when surgical reduction was performed after failure of radiologic reduction. A leading point was not identified. The second episode was at the age of 4 years, and the intussusception was then reduced by air enema. A Meckel’s scan was negative. An upper GI series with small bowel follow-through demonstrated nodular filling defects consistent with ILH in the terminal ileum and cecum.
During the current episode, 12 hours after the radiologic reduction, the abdominal pain recurred. Abdominal ultrasound confirmed the clinical suspicion of recurrent intussusception, which was again reduced by air enema. An upper GI series with small bowel follow-through again demonstrated small, round filling defects in the terminal ileum and in the cecum, consistent with ILH (Fig 3). No other intraluminal mass or narrowing of the intestinal lumen was identified. The parents were informed regarding the different treatment alternatives, and the child was treated with oral prednisone 1 mg/kg/d for 2 weeks, with gradual tapering over a 4-week period. No recurrence of the intussusception has occurred during 18 months of follow-up.
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DISCUSSION |
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TOPABSTRACTINTRODUCTIONCASE REPORTSDISCUSSIONCONCLUSIONREFERENCES |
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ILH is an uncommon but well-recognized cause of recurrent intussusception in infants and young children. It is characterized by numerous hyperplastic lymphoid nodules underneath the surface epithelium, within the lamina propria and the submucosa of the gut.5,6 These nodules form polyp-like lesions that protrude from the intestinal wall into the lumen and can be detected both on endoscopy,5–7 and by double-contrast barium enema.2,3,8
The cause of ILH is still uncertain. It has been reported in association with viral, bacterial, or parasitic infections, as well as an allergic response to various foods.4,5,9,10 In adults, more frequently then in the children, it has been reported to occur in association with immune deficiencies such as acquired idiopathic hypogammaglobulinemia11,12 or human immunodeficiency virus infection.13
There is a wide spectrum of clinical features attributed to ILH. Laufer and deSa3 advocated that ILH is a normal feature of the pediatric colon, as they had observed ILH in 50% of children undergoing double-contrast barium enema, and could not find a correlation with clinical symptoms. In contrast, Colon et al5 reported 147 children with symptomatic ILH in the small intestine (43%) or in the colon (57%), who presented with abdominal pain (58%) or hematochezia (32%). Additional, less common complaints included low-grade fever, chronic diarrhea, and recurrent sinopulmonary symptoms. In 25 children in their study, ILH was associated with juvenile inflammatory polyps (6 patients), chronic esophageal reflux (5 patients) or chronic inflammatory bowel disease (13 patients); 1 child had an intussusception.5 Juda et al14 reported 12 children with symptomatic ILH, with either acute or chronic presentations, who all required surgical management; these children presented with abdominal pain, vomiting, GI bleeding, and recurrent intussusception.14
The radiologic appearance of multiple small, round filling defects detected in the distal small bowel or in the colon in an upper GI series with small bowel follow-through or in a double-contrast barium enema include conditions other than ILH. Miller et al15 indicated that the presence of such findings is affected by technical factors (the kind of barium used), and found no correlation between radiologic and clinical findings. As mentioned, Laufer and deSa3 considered these findings as a normal appearance of the pediatric colon. However, the presence of a central umbilication, the size (1–3 mm), the uniformity, and the age of the patient can all support the diagnosis of ILH.5,8 Radiologically, ILH has also been reported as part of a spectrum of diseases that include lymphoma. In both our cases, however, this possibility cannot be supported. Lymphoma is exceedingly rare under the age of 1 year, as was our first patient at the last episode of intussusception. At this time she had already been followed for 3 months, during which there was no other clinical, laboratory, or radiologic evidence for a systemic or lymphoproliferative disorder. In the second case, there was no clinical, laboratory, or radiologic evidence for lymphoma during a 1-year follow-up period between the second and the third episodes of intussusception, at the ages of 4 and 5 years. In this age group, those cases in which lymphoma is the cause of intussusception usually present with a large solid mass, rather than with multiple small lesions, as seen in our cases. In addition, in both our cases, no other manifestations of lymphoma were found during the follow-up period of 18 to 24 months.
There is no consensus regarding the management of patients with ILH. Laufer and deSa3 support the "watchful observation approach" whereas others advocated surgical treatment. In the series described by Colon et al,5 patients were treated with antihistamine, with clearance of symptoms within 14 to 21 days; a minority of these patients, who did not respond, were treated with steroids for 3 to 6 weeks. In the series reported by Juda et al,14 the impression was that surgical resection was indicated in most cases of symptomatic ILH. In most of the children with the acute form of presentation the appendix and the terminal ileum were involved, hence appendectomy was performed, while in 2 of the children who had recurrent intussusception, terminal ileal resection and right hemicolectomy were performed. Additional support for the surgical approach can be found in the case reported by Hasegawa et al.16 They described a boy with recurrent intussusception who had ILH of the terminal ileum, and underwent ileocecal resection without any subsequent recurrence of the intussusception.
Steroid treatment for ILH is not novel therapy. It had been suggested more than 5 decades ago.5,17–19 Yet, it is surprising that no mention of this therapeutic modality appears in current pediatric or pediatric gastroenterology textbooks.20,21 Indeed, a recently published case report22 highlighted the option of steroid treatment in an infant with diarrhea, bloody stools, and severe intestinal nodular lymphoid hyperplasia. The mechanism by which steroid treatment affects ILH remains unclear. In a recent study, Kokkonen and Karttunen9 speculated that ILH can represent a long-standing upregulation of intestinal immunologic activity as a response to luminal food antigens. If this is true, a lymphopenic effect of steroid therapy may be the reason for decrease in ILH after steroid treatment. The 2 cases presented demonstrate a plausible causative association between ILH and recurrent intussusception. Being a common intestinal finding on imaging studies in infants and children, ILH might indeed have been an incidental finding, that had resolved spontaneously. However, in both cases, radiologic evidence of ILH was documented during 2 separate episodes of intussusception, and in both cases a clinical and radiologic response to steroid treatment was undoubtedly evident. In the first case an almost normal intestinal mucosa was demonstrated after a short course of steroid treatment (Fig 2), and there was no recurrence of the intussusception during a 2-year follow-up. In the second case, as an additional episode of intussusception had not occurred, we did not find the performance of the follow-up barium enema justified. The uneventful clinical course suggested that clinically significant lymphoid hyperplasia was no longer present. We thus propose that the immediate initiation of steroid treatment had contributed to the rapid resolution of symptoms.
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CONCLUSION |
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TOPABSTRACTINTRODUCTIONCASE REPORTSDISCUSSIONCONCLUSIONREFERENCES |
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In both our patients no other possible reason for ILH, such as intestinal infections or hypogamaglobulinemia, could be found. Therefore, other than steroids, no other medical treatment could be offered to these children. Surgical intervention may be performed at any stage of treatment. Successful management with a short course of steroids thus enabled us to avoid unnecessary surgical interventions. We therefore conclude that when recurrent intussusception occurs in association with ILH, and no other leading point can be identified, it is important to consider a trial of steroid therapy before committing the child either repeated attempts of pneumatic or hydrostatic reduction or a more radical surgical resection approach.
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FOOTNOTES |
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Received for publication Oct 19, 2001; Accepted Jul 22, 2002.
Address correspondence to Benjamin Z. Koplewitz, MD, Department of Radiology, Hadassah Medical Center, Box 12000, Jerusalem 91120, Israel. E-mail: ben_kop{at}hotmail.com
Dr Shteyer’s current address is: Division of Pediatric Gastroenterology, St Louis Children’s Hospital, One Children’s Place, St Louis, MO 63110.
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REFERENCES |
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TOPABSTRACTINTRODUCTIONCASE REPORTSDISCUSSIONCONCLUSIONREFERENCES |
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PEDIATRICS (ISSN 1098-4275). ©2003 by the American Academy of Pediatrics
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