INSTRUCTIVE CASE

The role of steroids in children with multiple recurrencesof intussusceptionjpc_2406 180..181

Robert Lopez,1 Spencer W Beasley,1 Russell Blakelock,1 Kiki Maoate,1 Craig A McBride,5 Charlotte Slade,2

Andrew S Day3,4 and Sebastian K King1

Departments of 1Paediatric Surgery and 2Immunology and 3Paediatrics, Christchurch Hospital, and 4Department of Paediatrics, University of Otago,

Christchurch, New Zealand and 5Department of Paediatric Surgery, Royal Children’s Hospital, Herston, Queensland, Australia

Abstract: We describe the case of a boy who had 9 recurrences of intussusception, for which no pathological lesion at the leadpoint wasidentified. A contrast follow-through study revealed a follicular/nodular mucosal pattern, particularly prominent in the terminal ileum andcaecum. Patients with multiple recurrences usually have an identifiable lesion at the leadpoint, but sometimes recurrences may be due tolymphoid hyperplasia, as presumed in our case. No further recurrences occurred after a two month tapering course of oral prednisolone.

Key words: intussusception; laparoscopy; paediatric; recurrence; steroid.

Case

A previously well 11-month-old boy presented with a 3-dayhistory of abdominal pain, associated with blood and mucus inhis stools. An ileo-ileocolic intussusception that extended intothe transverse colon was successfully reduced by gas enema. Animmediate post-reduction ultrasound examination, performedbecause of a suspected lesion seen on fluoroscopy, demonstratedan indeterminate mass at the ileo-caecal valve with associatedlymphadenopathy, suggestive of a duplication cyst. However,repeat ultrasonography the following day was normal.

In the subsequent 4 months, the child presented to the hos-pital a further 10 times with similar symptoms of abdominalpain but without blood in his stools. On nine of the occasions,he had recurrence of the intussusception, demonstrated onultrasonography, and reduced by gas enema. The previouslysuspected mass was not identified on any of the subsequentultrasound examinations.

After the fifth recurrence was reduced by gas enema, the childunderwent a diagnostic laparoscopy, which demonstrated mod-

erate mesenteric lymphadenopathy and evidence of scarring atthe ileo-caecal valve. A lymph node biopsy was not performed.After the seventh recurrence, a contrast follow-throughrevealed ‘a prominent follicular/nodular mucosal patternthroughout the small bowel and visualised colon, particularly inthe region of the terminal ileum and ileo-caecal valve’. Fullblood count and urea and electrolytes were normal. Infectiouscauses were excluded, including Giardia and Cryptosporidium byfaecal antigen testing, Yersinia by serology, and Epstein Barrvirus by PCR on whole blood. Testing for coeliac disease byanti-gliadin IgG and IgA and anti-tissue transglutaminase (tTg)IgA was negative. Total serum IgA was concurrently measured,by a sensitive assay, at 0.19 g/L, a level at which false-negativeresults for anti-tTG IgA and anti-gliadin IgA are unlikely. Afterthe eighth recurrence, the child was commenced on oral pred-nisone (10 mg once daily) in an attempt to reduce the risk ofrecurrence, on the presumption that it was secondary to lym-phoid hyperplasia. A final recurrence occurred 1 day after theprednisone therapy was commenced.

Initial assessment of the immune system revealed panhy-pogammaglobulinaemia (IgG 0.5 g/L (5.3–8.0), IgA 0.1 g/L(0.2–0.6), IgM 0.2 (0.5–1.2)). However, immunoglobulin levelsnormalised after 2 months. Vaccine responses to tetanus toxoid,pneumovax and Haemophilus influenzae B were within normalreference ranges. Lymphocyte subsets, including class-switchedmemory B cells, were within age-matched reference ranges.The child had a 2-month course of prednisone (10 mg daily for2 weeks, reducing over the following 6 weeks), and has hadno further recurrence of intussusception in the subsequent18 months.

Discussion

In older children with intussusception, and those with recurrentdisease, a pathological lesion at the lead point must be

Key Points

1 Multiple recurrences of intussusception, in the absence of anidentifiable pathological lesion at the lead point, are relativelyrare.

2 Lymphoid hyperplasia is a recognised cause of recurrentintussusception.

3 Oral prednisone may be employed in the management ofrecurrent intussusception secondary to lymphoid hyperplasia.

Correspondence: Professor Spencer Beasley, Department of PaediatricSurgery, Christchurch Hospital, Private Bag 4710, Christchurch 8011, NewZealand. Fax: +64 3364 1584; email: spencer.beasley@cdhb.govt.nz

Accepted for publication 30 November 2011.

doi:10.1111/j.1440-1754.2011.02406.x

Journal of Paediatrics and Child Health 48 (2012) 180–181© 2012 The Authors

Journal of Paediatrics and Child Health © 2012 Paediatrics and Child Health Division (Royal Australasian College of Physicians)

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excluded.1,2 Our patient had recurrences of ileo-ileocolic intus-susception without evidence of a pathological lesion, apart fromlymphoid hyperplasia.

Lymphoid hyperplasia in the terminal ileum is a recognisedcause of intussusception.1,3 Non-specific in nature, it may becaused by viral or parasitic infections. Evidence for either wasnot found in this patient, although exhaustive viral serologywas not performed due to the finding of panhypogammaglo-bulinaemia.1,3 Intestinal lymphoid hyperplasia has also beenreported in association with immunodeficiency. Although notyet subjected to a formal trial, there are reports of decreasedhyperplasia in response to steroid therapy.4,5 The use of post-reduction steroids has been shown to decrease the early recur-rence rate in intussusception. Steroids have also been used toabrogate multiple recurrences of intussusception. Although ourpatient had marked panhypogammaglobulinaemia at the timeof diagnosis, the normalisation of immunoglobulin levels, withnormal vaccine responses, would make a primary immunode-ficiency unlikely.

Lymphoid hyperplasia is generally considered self-limiting inthis context. In children with multiple recurrences, both diag-nosis by histological analysis of colonoscopic biopsies and sur-gical resection have been advocated. Due to the extent of thehyperplasia seen radiologically in this child, surgical resectionwas not entertained. In light of that decision, and given the priorfindings both radiologically and immunologically, colonoscopywas unlikely to alter management and was therefore not per-formed. In the absence of an identifiable lead point, surgicalresection is rarely required in the management of recurrentintussusception.2,6 The difficulty is determining how manyrecurrences are permissible before operating, knowing thatthere is a significant chance in these patients of not finding anoperable lesion at operation.2 Our patient had multiple recur-rences requiring hospitalisation and treatment with gas enema,and a negative laparoscopy. He also had lymphoid hyperplasia,and no other identified candidate to explain these recurrences.Steroids were used in an attempt to decrease the hyperplasiaand lessen the likelihood of hyperplastic areas being picked upby a peristaltic wave and intussuscepted. While the introductionof steroids may have coincided with spontaneous resolution ofdisease in this patient, there is a biological plausibility to steroidssuccessfully decreasing lymphoid hyperplasia and preventingfurther recurrences.

Multiple Choice Questions

1 The most common cause of intussusception in infants is:a. Meckel’s diverticulumb. ileal polypc. idiopathicd. Henoch–Schonlein purpurae. lymphoma

2 Hypogammaglobulinaemia is not associated with:a. anti-epileptic medicationsb. common variable immunodeficiencyc. renal protein lossd. bacterial infectione. gastrointestinal protein loss

3 A gas reduction enema should not be used in which clinicalsituation:a. recurrent intussusceptionb. generalised peritonitisc. patients under the age of 3 monthsd. long duration of symptomse. blood stained stools (red currant jelly stools)

Multiple Choice Answers

1(C) Most cases of intussusception in infants are idiopathic.Although the majority of pathological lead points (e.g.Meckel’s diverticulum, ileal polyp, Henoch–Schonleinpurpura, lymphoma) appear in the infant age group, theoverwhelming majority of intussusceptions in patientsunder one year remains ‘idiopathic’.

2(D) Bacterial infection will typically lead to a rise in immu-noglobulin levels. All of the other answers may be asso-ciated with hypogammaglobulinaemia.

3(B) Generalised peritonitis is a contraindication for gas reduc-tion enema. Patients with generalised peritonitis second-ary to intussusception require resuscitation and operativemanagement. On the other hand, gas reduction enemasare appropriate for recurrent disease, in infants with longduration of symptoms (in the absence of peritonitis) andat any age. Those with ‘red currant’ stools should not beexcluded from a gas reduction enema.

References

1 Hasegawa T, Ueda S, Tazuke Y et al. Colonoscopic diagnosis oflymphoid hyperplasia causing recurrent intussusception. Surg. Today1998; 28: 301–4.

2 Blakelock RT, Beasley SW. The clinical implications of non-idiopathicintussusception. Pediatr. Surg. Int. 1998; 28: 913–9.

3 Shteyer E, Koplewitz BZ, Gross E et al. Medical treatment of recurrentintussusception associated with intestinal lymphoid hyperplasia.Pediatrics 2003; 111: 682–5.

4 Atwell JD, Burge D, Wright D. Nodular lymphoid hyperplasia of theintestinal tract in infancy and childhood. J. Pediatr. Surg. 1985; 20:25–9.

5 Kohler PF, Cook RD, Brown WR et al. Common variablehypogammaglobulinaemia with T-cell lymphoid interstitial pneumonitisand B-cell nodular lymphoid hyperplasia: different lymphocytepopulations with a similar response to prednisone therapy. J. AllergyClin. Immunol. 1982; 70: 299–305.

6 van der Laan M, Bax NM, van der Zee DC et al. The role of laparoscopyin the management of childhood intussusception. Surg. Endosc. 2001;15: 373–6.

R Lopez et al. Steroids in recurrent intussusception

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