- Email: [email protected]
Colonic stricture as a complication of haemolytic uraemic syndrome
Accepted Manuscript Colonic stricture as a complication of haemolytic uraemic syndrome Megan Grinlinton, Stephen Evans, Tonya Kara PII:
S2213-5766(17)30173-2
DOI:
10.1016/j.epsc.2017.09.011
Reference:
EPSC 829
To appear in:
Journal of Pediatric Surgery Case Reports
Received Date: 13 July 2017 Revised Date:
7 September 2017
Accepted Date: 9 September 2017
Please cite this article as: Grinlinton M, Evans S, Kara T, Colonic stricture as a complication of haemolytic uraemic syndrome, Journal of Pediatric Surgery Case Reports (2017), doi: 10.1016/ j.epsc.2017.09.011. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT Colonic Strictures: A Rare Complication of Haemolytic Uraemic Syndrome Megan Grinlintona, Stephen Evansa, Tonya Karaa a
Department of Paediatric Surgery, Starship Children’s Health, Auckland, New Zealand.
Corresponding author: Dr Megan Grinlinton Email: [email protected]
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Address: 222 Kepa Road, Mission Bay, Auckland 1071
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[email protected], [email protected], [email protected]
ACCEPTED MANUSCRIPT ABSTRACT Haemolytic uraemic syndrome (HUS) is an infectious disease that can rapidly become lifethreatening in the paediatric population. A number of long-term complications may arise from HUS including the rare development of colonic strictures. In this case report, we present
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two cases with similar presentations of colonic strictures following HUS. Case 1 is a 17month-old female who developed HUS and multiple complications including a sigmoid
colonic stricture. Once the stricture was resected, her recovery was rapid and complete. Case
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2 is a 3-year-old male who developed severe HUS requiring dialysis. After developing a small bowel obstruction, a laparotomy demonstrated caecal disease, pan-colonic
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inflammation, a calcified appendix and a strictured descending colon. A second operation revealed strictures at the transverse, descending and sigmoid colon. Once the diseased part of bowel had been removed his recovery was complete. This report demonstrates the diagnostic difficulty and patient morbidity that may arise from post-HUS colonic strictures. A contrast
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study is the recommended investigation of choice in patients presenting with ongoing gastrointestinal symptoms after an acute infection with HUS.
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STEC, shiga toxin
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KEY WORDS: HUS, haemolytic uraemic syndrome, colonic stricture, complication, colitis,
ABBREVIATIONS: Haemolytic uraemic syndrome (HUS), Shiga-toxin-producing Escherichia coli (STEC), gastrointestinal (GI), computed tomography (CT)
Haemolytic uraemic syndrome (HUS) is a leading cause of acute kidney injury in children, and most commonly results from infection with Shiga-toxin-producing Escherichia coli (STEC). It typically presents with a diarrhoeal prodrome, acute renal failure and haemolytic anaemia. A rare complication is colonic stricture, which may present months to years after
ACCEPTED MANUSCRIPT the initial infection has resolved. The rarity of this occurrence can lead to delays in diagnosis and management, especially as the patient’s symptoms are frequently atypical. In this case report, we present two children with HUS who developed severe colonic strictures requiring operative management. These cases illustrate the significance of delayed complications
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arising from HUS, as these may present a diagnostic conundrum to both physicians and
surgeons. We postulate that severe hypovolaemia with resultant bowel ischaemia in the acute
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setting of HUS may contribute to the formation of colonic strictures.
1. CASE REPORT 1
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A 17-month-old female presented to a regional hospital in March 2016 with a two-day history of diarrhoea and haematochezia. Initial laboratory results were indicative of HUS: haemoglobin of 69g/L; platelet count of 33 E+9/L; creatinine of 146umol/L; and urea of 14.2mmol/L. A stool culture tested positive for STEC H7:O157. She was transferred to
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Starship Children’s Health Paediatric Intensive Care (PICU) unit with severe colitis, and required large volumes of fluid resuscitation and peritoneal dialysis. She was discharged
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improved.
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three weeks later, once her renal function, albumin, blood pressure and nutrition were
She was readmitted one week after discharge with a small bowel obstruction. This was managed conservatively. Three weeks later she represented and a plain abdominal x-ray was suggestive of a partial small bowel obstruction. A contrast meal and follow-through study was performed but no strictures were demonstrated. A laparoscopy showed no adhesions, strictures or change in bowel calibre. She later developed diarrhoea and a stool sample was positive for Clostridium Difficile, which was treated with antibiotics. Her symptoms were attributed to this infection, and she was discharged seven weeks after admission.
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In the community, she continued to experience vomiting, abdominal distension and pain, and six weeks later she represented to hospital with these symptoms. An abdominal x-ray and a barium contrast study were repeated (Fig. 1, Fig. 2). These revealed a dilated, featureless
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transverse colon with a splenic flexure stricture. Fig. 1. Abdominal Plain Film
Fig. 2. Upper GI Contrast Study
Red arrows: Dilated transverse colon
Star: Dilated left hemicolon
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Yellow arrow: Site of stricture
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Green arrow: Collapse descending colon
She proceeded to laparotomy and was found to have a dilated small bowel and caecum, a dilated featureless transverse colon with a clear transition point proximal to the splenic flexure, and a healthy ascending, descending and sigmoid colon (Fig. 3.). A transverse
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colectomy was performed with primary anastomosis. Histopathology revealed evidence of chronic colitis with stricture formation (Fig. 4.).
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Fig. 3. Operative Findings
Fig. 4. Histological Findings Colonic wall with submucosal, mural and
White arrow: Omentum fused to stricture
extramural fibrosis and patchy chronic
Diamond: Decompressed distal transverse
inflammation.
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Star: Dilated proximal transverse colon
colon.
She was discharged nine days post-operatively, and has been well since, with good weight gain and full oral feed tolerance.
2. CASE REPORT 2
ACCEPTED MANUSCRIPT A 3-year-old male from a rural community presented in July 2016 with haematemesis, haematochezia, and a compensated metabolic acidosis secondary to severe dehydration, following ingestion of farm trough water. Laboratory results were consistent with HUS: haemoglobin of 87 g/L; platelet count 28 E+9/L; creatinine of 166 umol/L; and urea of
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15.0mmol/L. Stool cultures were positive for Giardia, Cryptosporidium and STEC. He was transferred to Starship Children’s Health PICU unit, where he required aggressive fluid resuscitation, ionotropic support, and antibiotics. He was managed with continuous
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venovenous haemofiltration initially, and progressed to intermittent haemodialysis.
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Several weeks into his admission, he developed abdominal distension, pain and vomiting. A plain abdominal x-ray demonstrated a dilated transverse colon with partial bowel obstruction. A contrast enema confirmed a stricture at the splenic flexure, a dilated transverse colon and marked luminal narrowing at the junction of the descending and sigmoid colon (Fig. 5). A
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computed tomography (CT) scan of the abdomen showed a transition point near the splenic flexure, with a narrow calibre thick-walled bowel beyond this likely representing a long stricture (Fig. 6).
Fig. 6. CT Abdomen
White arrow: Sigmoid colonic
White Star: Dilated transverse colon
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Fig. 5. Lower GI Contrast Study
stricture
Red Arrow: Colonic stricture Diamond: Liver
A laparotomy revealed chronic caecal, transverse and descending colonic inflammation, with dense adhesions between the transverse colon, stomach and jejunum. The appendix was heavily calcified and the descending colon was severely strictured. One short segment of jejunum was resected and anastomosed where there was a suspicion of a small bowel fistula
ACCEPTED MANUSCRIPT (Fig. 7). An appendicectomy was performed (Fig. 8), and a defunctioning ileostomy was fashioned, rather than resecting the colonic disease because of suspicion of ongoing active inflammation.
Fig. 8. Operative Findings
White arrow: Small bowel adhesions
Inflamed appendix
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Fig. 7. Operative Findings
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Histological findings described oedema and vascular congestion of the small bowel without
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evidence of fistulation, and active mucosal inflammation and ulceration of the appendix.
He returned five weeks later for a definitive laparotomy. A pre-operative lower GI contrast study confirmed a high-grade tapering stricture in the distal transverse colon (Fig. 9). Operative findings included extensive flimsy adhesions, a dilated ascending colon, and
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strictures at the transverse and proximal descending colon. An extended right hemicolectomy, closure of ileostomy, and adhesiolysis were performed. Histopathology revealed a colonic
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stricture, with fibrosis, ulceration and inflamed granulation tissue (Fig. 10).
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Fig. 9. Lower GI Contrast Study Prior
Fig. 10. Histological Findings
to Second Operation.
Inflamed portion of bowel with neutrophil
White arrow: Distal transverse colonic
infiltration.
stricture
He was discharged a week after his operation and has been well since, with no further hospital admissions at the time of publication.
ACCEPTED MANUSCRIPT 3. DISCUSSION 3.1. Background Shiga-toxin-producing Escherichia Coli (STEC) is transmitted to humans via contact with infected humans or animals, or ingestion of contaminated food or water1. Shiga toxin causes
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microvascular injury to glomerular and afferent capillary lumens, apoptosis, acute tubular necrosis and cortical necrosis in the kidney2. The connection between STEC and post-
diarrhoeal HUS was first reported in a study by Karmali et al in 19833. HUS is characterised
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by the pathognomonic triad of microangiopathic haemolytic anaemia, thrombocytopenia, and renal injury5. Masumoto et al13 state that shiga-toxin induced microangiopathy is postulated
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to be the main cause of ischaemia, and the resulting ischaemic change results in stricture.
Patients commonly present with abdominal pain, diarrhoea and haematochezia8. Serious sequelae include acute renal failure, with approximately 50% of patients requiring dialysis4,1
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in the acute phase. Long-term sequelae include proteinuria (15-30%), hypertension (5-15%), chronic kidney disease (9-18%) and end-stage renal disease (3-5%) 5, 9. The two cases in our series presented with unusually severe colitis and hypovolaemia. The combination of an acute
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kidney injury, hypovolaemia and acute colitis, as well as the fact that strictures formed within
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watershed areas of the bowel, may have led to the development of this rare pathology.
3.2. Gastrointestinal sequelae A wide variety of multisystem sequelae have been reported in the literature, encompassing renal, respiratory, cardiac, and neurological systems4, 3. The most common site of extra-renal involvement is the gastrointestinal tract8,10. Gastrointestinal sequelae may include pancreatic or colonic necrosis, intussusception, rectal prolapse, and infarction of organs supplied by the coeliac axis8. Colonic strictures as a late complication are rare11, 12, with only 22 cases
ACCEPTED MANUSCRIPT described over the last 40 years11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 28, including the two in this article (Table 1). Colonic strictures are estimated to occur in 1-3% of patients with HUS11, 12, but the actual numbers reported in the literature suggest that this is an overestimation.
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Any part of the colon may be affected by HUS colitis24. A retrospective analysis of 54
children found that the transverse and ascending colon were the most affected sites in HUSassociated colitis25. It has been stated that in STEC HUS, the left hemicolon is predominantly
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affected26. Previous studies suggest that late-presenting colonic strictures appear to affect the left colon more than the right. Both patients in our case series had strictures in the distal
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transverse colon proximal to the splenic flexure. This is known as a watershed region, as it receives dual blood supply from the most distal branches of the superior and inferior mesenteric artery. It is biologically plausible that hypotension may cause ischaemic injury to
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this area, where the blood supply is most vulnerable to low blood pressure.
The 3-year-old male in our case report had severe caecal and appendiceal disease, which is highly unusual given the typical pathological distribution for colonic strictures from HUS.
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All other authors report a predilection for left-sided colonic disease. In the literature, four patients had transverse colonic strictures, five patients had descending colonic strictures, and
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seven patients had sigmoid colonic strictures13. Maguire et al described a case where a fistula developed between the caecum and the sigmoid colon, but the patient primarily had sigmoidal disease23. De La Hunt et al described the only case of an oesophageal stricture resulting from HUS in 199127. Our second case therefore describes a rare and unique complication that has not previously been reported.
ACCEPTED MANUSCRIPT Segmental colonic ischaemia without necrosis may heal by fibrosis, leading to a reduced luminal diameter. The histology of both cases in this report showed acute and chronic inflammation, with ulceration and stricture formation. The majority of cases of HUS-related colonic strictures in the literature required operative management, except for two cases with
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mild strictures, who were managed conservatively14, 17. Most cases appeared to progress well after surgical resection, with only one report of ongoing issues following operative
management28. In this study, the patient had a sigmoid colectomy, but developed small bowel
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neuropathic and myopathic involvement post-operatively. Both of our cases required
operative management due to symptomatic colonic strictures, and both were considered
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surgically cured once these strictures were resected. We suggest that a contrast study be considered as the primary investigation for patients presenting in this manner. This aligns with recommendations made by Masumoto et al13.
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3.3 Postulated risk factors for development of colonic strictures after HUS Risk factors that may lead to formation of chronic colonic strictures after an acute episode of HUS have not been defined thus far. On review of the literature, the incidence of females
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developing this complication was slightly higher than males (59% vs. 41%), and there was an
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increased risk at a younger age, with the majority of patients being age 3 or under at diagnosis (59%), excluding cases in which age and sex was not specified.
It is possible that a severe initial presentation of HUS, or multiple ‘hits’ to the system could contribute towards colonic strictures. For example, in Case 1, the patient developed a Clostridium Difficile infection during her admission, which may have further impacted on already compromised bowel. Similarly, the second case had concurrent Giardia, Cryptosporidium and STEC infections on admission. In this case report, we believe that the
ACCEPTED MANUSCRIPT severity of both patient’s initial illness, including the presence of hypovolaemia, sepsis, multi-organ failure, and multiple-organism bacteraemia, could increase the risk of long-term colonic strictures.
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Spinale et al9 explored prognostic factors for long-term sequelae of HUS, and determined that the most important risk factor is the severity of the initial renal injury, in particular, anuria for over 10 days. The need for dialysis was also listed as a risk factor for long-term sequelae.
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Both of our patients required dialysis during their initial admissions. Although particular risk factors for the development of colonic strictures have not been explored, it follows that these
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indicators of severity are likely to increase the risk of all complications, including colonic strictures.
4. CONCLUSION
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Colonic stricture formation as a result of HUS is a rare but clinically important complication. The presentation can be insidious and may occur months to years after the initial insult. Surgical resection of colonic strictures appears to have favourable results but longer follow-
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up is needed. Our article adds significant information to the sparse body of material available
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thus far. Our first case illustrates the diagnostic conundrum that may occur in patients presenting with intermittent symptomatology. Our second patient describes the first case of right-sided colonic disease ever reported, and is the only publication from Australasia on this topic to date.
In both cases in this article, severe hypovolaemia on presentation may have contributed to the development of colonic strictures. The pathological formation of strictures may be attributable to both decreased blood flow to watershed areas of bowel, as well as colitis-
ACCEPTED MANUSCRIPT related microangiopathy. We therefore recommend aggressive fluid resuscitation and careful management of circulatory volume in hypovolaemic patients presenting with HUS. This may mitigate against serious complications such as stricture formation and the need for surgical
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intervention at a later date.
ACKNOWLEDGEMENTS: Thank you to the Paediatric Surgery Department at Starship Children’s Health, and to the families of the patients included in this study, for your support
REFERENCES
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and cooperation.
1. Mead PS, Griffin PM. Escherichia coli O157: H7. The Lancet. 1998 Oct 10;352(9135):1207-12.
2. Garg AX, Suri RS, Barrowman N, Rehman F, Matsell D, Rosas-Arellano MP,
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Salvadori M, Haynes RB, Clark WF. Long-term renal prognosis of diarrhoeaassociated hemolytic uremic syndrome: a systematic review, meta-analysis, and metaregression. Jama. 2003 Sep 10;290(10):1360-70.
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3. Karmali M, Petric M, Steele B, Lim C. Sporadic cases of haemolytic-uraemic
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syndrome associated with faecal cytotoxin and cytotoxin-producing Escherichia coli in stools. The Lancet. 1983 Mar 19;321(8325):619-20.
4. Siegler R, Oakes R. Hemolytic uremic syndrome; pathogenesis, treatment, and outcome. Current opinion in pediatrics. 2005 Apr 1;17(2):200-4.
5. Brandt JR, Joseph MW, Fouser LS, Tarr PI, Zelikovic I, McDonald RA, Avner ED, McAfee NG, Watkins SL. Cholelithiasis following Escherichia coli O157: H7associated hemolytic uremic syndrome. Pediatric Nephrology. 1998 Apr 4;12(3):2225.
ACCEPTED MANUSCRIPT 6. Russo P, Ruchelli ED, Piccoli DA, editors. Pathology of pediatric gastrointestinal and liver disease. Springer; 2014 Jun 5. 7. Miller DJ, Skucas J. The radiological examination of the colon: practical diagnosis. Springer Science & Business Media; 2012 Dec 6.
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8. McCarthy DW, Mutabagani K, Mahan JD, Caniano DA, Cooney DR. Infarction of the choledochus, liver, gallbladder, and pancreas: A unique complication of the
hemolytic uremic syndrome. Journal of pediatric surgery. 2000 Mar 31;35(3):502-4.
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9. Spinale JM, Ruebner RL, Copelovitch L, Kaplan BS. Long-term outcomes of Shiga toxin hemolytic uremic syndrome. Pediatric Nephrology. 2013 Nov 1;28(11):2097-
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105.
10. Scheiring J, Andreoli SP, Zimmerhackl LB. Treatment and outcome of Shiga-toxinassociated hemolytic uremic syndrome (HUS). Pediatric nephrology. 2008 Oct 1;23(10):1749.
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11. Grodinsky S, Telmesani A, Robson WL, Pick G, Scott RB. Gastrointestinal manifestations of hemolytic uremic syndrome: recognition of pancreatitis. Journal of pediatric gastroenterology and nutrition. 1990 Nov 1;11(4):518-24.
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12. Tapper D, Tarr P, Avner E, Brandt J, Waldhausen J. Lessons learned in the
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management of hemolytic uremic syndrome in children. Journal of pediatric surgery. 1995 Feb 1;30(2):158-63.
13. Masumoto K, Nishimoto Y, Taguchi T, Tsutsumi Y, Kanemitsu S, Hara T, Suita S. Colonic stricture secondary to hemolytic uremic syndrome caused by Escherichia coli O-157. Pediatric nephrology. 2005 Oct 1;20(10):1496-9. 14. Peterson RB, Meseroll WP, Shrago GG, Gooding CA. Radiographic features of colitis associated with the hemolytic-uremic syndrome. Radiology. 1976 Mar;118(3):667-71.
ACCEPTED MANUSCRIPT 15. Sawaf H, Sharp MJ, Youn KJ, Jewell PA, Rabbani A. Ischemic colitis and stricture after hemolytic-uremic syndrome. Pediatrics. 1978 Feb 1;61(2):315-7. 16. Bax RP, Reeves DS, White LO, Holt A, Bywater M. Case records of the Massachusetts general hospital. Weekly clinicopathological exercises. Case 12-1981.
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N Eng J Med 304: 715-22.
17. Kirks DR. The radiology of enteritis due to hemolytic-uremic syndrome. Pediatric radiology. 1982 Jul 1;12(4):179-83.
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18. Crabbe DC, Broklebank JT, Spicer RD. Gastrointestinal complications of the
1;83(12):773-5.
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haemolytic uraemic syndrome. Journal of the Royal Society of Medicine. 1990 Dec
19. Gordon AC, Cundall DB, Spicer RD, Mortensen NJ. Colonic stricture secondary to haemolytic uraemic syndrome. The European journal of surgery= Acta chirurgica. 1994 Dec;160(12):707.
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20. Babaian ME, Chow DC, Soloway GN, Taubin HJ, Roberts IM. Colonic ischemic stricture presenting as a late complication of the hemolytic uremic syndrome. Journal of clinical gastroenterology. 1997 Oct 1;25(3):555-6.
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21. Sebbag H, Lemelle JL, Moller C, Schmitt M. Colonic stenosis after hemolytic-uremic
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syndrome. European journal of pediatric surgery. 1999 Apr;9(02):119-20. 22. Gerios AJ, Hupertz VF. Colonic stricture and perforation after hemolytic-uremic syndrome in a pediatric patient. The American Journal of Gastroenterology. 2002 Sep 30;97(9):S221.
23. Maguire K, Keys C, Kronfli R, Penman D, Haddock G. Colonic stricture and fistula following haemolytic uraemic syndrome with toxic megacolon. European journal of pediatric surgery. 2010 May;20(03):210-2.
ACCEPTED MANUSCRIPT 24. Baud C, Saguintaah M, Veyrac C, Couture A, Ferran JL, Barnéon G, Veyrac M. Sonographic diagnosis of colitis in children. European radiology. 2004 Nov 1;14(11):2105-19. 25. Rahman RC, Cobeñas CJ, Drut R, Amoreo OR, Ruscasso JD, Spizzirri AP, Suarez
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AD, Zalba JH, Ferrari C, Gatti MC. Hemorrhagic colitis in postdiarrheal hemolytic uremic syndrome: retrospective analysis of 54 children. Pediatric Nephrology. 2012 Feb 1;27(2):229-33.
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26. Ilnyckyj AL, Greenberg HO, Bernstein CN. Escherichia coli O157: H7 infection mimicking Crohn's disease. Gastroenterology. 1997 Mar 31;112(3):995-9.
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27. De la Hunt MN, Rangecroft L, Morris KP, Coulthard MG. Oesophageal and severe gut involvement in the haemolytic uraemic syndrome. British journal of surgery. 1991 Dec 1;78(12):1469-72.
28. Bowles C, Ancker M, Triadafilopoulos G. Gastrointestinal dysfunction following
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hemolytic uremic syndrome. Digestive diseases and sciences. 2011 Aug 1;56(8):2241.
ACCEPTED MANUSCRIPT Tables
Table 1: Literature Review of HUS-Related Colonic Strictures
Brandt et
Yea
Site of Stricture
Age at
r
HUS onset
1998 ND
ND
Grodinsk
1990 ND
ND
1990 ND
ND
2 months
ND
ND
ND
ND
ND
ND
ND
5
F
3 months
5
M
3 weeks
2
M
5 months
y et al Grodinsk
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[11]
y et al [12]
Tapper et 1995 Sigmoid colon al
Masumot 2004 Sigmoid colon o et al
[14]
Peterson
[15]
Sawaf et
1978 Descending colon
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al
1976 Descending colon
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et al
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[13]
Duration
ND
al [11]
Sex
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[5]
Author
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Ref.
[16]
Bax et al
1981 Descending colon
7
F
ND
[17]
Kirks et
1982 Transverse colon
3
M
6 weeks
1982 ND
ND
ND
10 weeks
1990 Transverse colon
5
F
1 year
al [17]
Kirks et al
[18]
Crabbe
ACCEPTED MANUSCRIPT et al [18]
Crabbe
1990 Descending colon
2
F
6 weeks
1994 Descending colon
3
F
1 month
1994 Transverse colon
3
[19]
Gordon et al
[19]
Gordon et al
Babian et 1997 Sigmoid colon
32
al Sebbag
5 years
M
14 months
2010 Sigmoid colon +
4
M
1.5 years
3
M
5 weeks
2011 Sigmoid colon
10
F
5 years
Our case
2016 Transverse colon
1
F
5 months
Our case
2016 Transverse /
3
M
4 weeks
caeco-sigmoid fistula
1991 Oesophagus
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De La
al
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Maguire
Hunt et
[28]
F
2
et al [27]
4 years
2002 Sigmoid colon
Gerios et al
[23]
F
1
et al [22]
1 year
1999 Transverse colon
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[21]
F
SC
[20]
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et al
Bowles et al
-
descending / sigmoid colon + appendix
Fig. 1. Abdominal Plain Film Red arrows: Dilated transverse colon
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ACCEPTED MANUSCRIPT
Fig. 2. Upper GI Contrast Study Star: Dilated left hemicolon Yellow arrow: Site of stricture
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Green arrow: Collapse descending colon
Fig. 3. Operative Findings Star: Dilated proximal transverse colon Fig. 4. Histological Findings White arrow: Inflamed omentum fused to Colonic wall showing submucosal, mural stricture and extramural fibrosis and patchy chronic Diamond: Decompressed distal transverse
ACCEPTED MANUSCRIPT inflammation.
Fig. 6. CT Abdomen
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Fig. 5. Lower GI Contrast Study
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colon
White Star: Dilated transverse colon
stricture
Red Arrow: Colonic stricture
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White arrow: Sigmoid colonic
Diamond: Liver
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ACCEPTED MANUSCRIPT
Fig 8. Operative Findings
White arrow: Small bowel adhesions
Inflamed appendix
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Fig. 7. Operative Findings
Fig. 9. Lower GI Contrast Study Prior
Fig. 10. Histological Findings
to Second Operation.
Inflamed portion of bowel with neutrophil
White arrow: Distal transverse colonic
infiltration.
stricture
ACCEPTED MANUSCRIPT Highlights •
A combination of microangiopathy and hypovolaemic bowel ischaemia in the acute setting of HUS may lead to colonic strictures Colonic strictures may be difficult to diagnose and may present months to years after the initial episode of HUS
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•
•
A contrast study is the initial investigation of choice to identify colonic strictures
•
Aggressive fluid resuscitation and careful management of circulatory volume is
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M AN U
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crucial in managing critically unwell patients with HUS
S2213-5766(17)30173-2
DOI:
10.1016/j.epsc.2017.09.011
Reference:
EPSC 829
To appear in:
Journal of Pediatric Surgery Case Reports
Received Date: 13 July 2017 Revised Date:
7 September 2017
Accepted Date: 9 September 2017
Please cite this article as: Grinlinton M, Evans S, Kara T, Colonic stricture as a complication of haemolytic uraemic syndrome, Journal of Pediatric Surgery Case Reports (2017), doi: 10.1016/ j.epsc.2017.09.011. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
ACCEPTED MANUSCRIPT Colonic Strictures: A Rare Complication of Haemolytic Uraemic Syndrome Megan Grinlintona, Stephen Evansa, Tonya Karaa a
Department of Paediatric Surgery, Starship Children’s Health, Auckland, New Zealand.
Corresponding author: Dr Megan Grinlinton Email: [email protected]
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Address: 222 Kepa Road, Mission Bay, Auckland 1071
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[email protected], [email protected], [email protected]
ACCEPTED MANUSCRIPT ABSTRACT Haemolytic uraemic syndrome (HUS) is an infectious disease that can rapidly become lifethreatening in the paediatric population. A number of long-term complications may arise from HUS including the rare development of colonic strictures. In this case report, we present
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two cases with similar presentations of colonic strictures following HUS. Case 1 is a 17month-old female who developed HUS and multiple complications including a sigmoid
colonic stricture. Once the stricture was resected, her recovery was rapid and complete. Case
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2 is a 3-year-old male who developed severe HUS requiring dialysis. After developing a small bowel obstruction, a laparotomy demonstrated caecal disease, pan-colonic
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inflammation, a calcified appendix and a strictured descending colon. A second operation revealed strictures at the transverse, descending and sigmoid colon. Once the diseased part of bowel had been removed his recovery was complete. This report demonstrates the diagnostic difficulty and patient morbidity that may arise from post-HUS colonic strictures. A contrast
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study is the recommended investigation of choice in patients presenting with ongoing gastrointestinal symptoms after an acute infection with HUS.
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STEC, shiga toxin
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KEY WORDS: HUS, haemolytic uraemic syndrome, colonic stricture, complication, colitis,
ABBREVIATIONS: Haemolytic uraemic syndrome (HUS), Shiga-toxin-producing Escherichia coli (STEC), gastrointestinal (GI), computed tomography (CT)
Haemolytic uraemic syndrome (HUS) is a leading cause of acute kidney injury in children, and most commonly results from infection with Shiga-toxin-producing Escherichia coli (STEC). It typically presents with a diarrhoeal prodrome, acute renal failure and haemolytic anaemia. A rare complication is colonic stricture, which may present months to years after
ACCEPTED MANUSCRIPT the initial infection has resolved. The rarity of this occurrence can lead to delays in diagnosis and management, especially as the patient’s symptoms are frequently atypical. In this case report, we present two children with HUS who developed severe colonic strictures requiring operative management. These cases illustrate the significance of delayed complications
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arising from HUS, as these may present a diagnostic conundrum to both physicians and
surgeons. We postulate that severe hypovolaemia with resultant bowel ischaemia in the acute
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setting of HUS may contribute to the formation of colonic strictures.
1. CASE REPORT 1
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A 17-month-old female presented to a regional hospital in March 2016 with a two-day history of diarrhoea and haematochezia. Initial laboratory results were indicative of HUS: haemoglobin of 69g/L; platelet count of 33 E+9/L; creatinine of 146umol/L; and urea of 14.2mmol/L. A stool culture tested positive for STEC H7:O157. She was transferred to
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Starship Children’s Health Paediatric Intensive Care (PICU) unit with severe colitis, and required large volumes of fluid resuscitation and peritoneal dialysis. She was discharged
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improved.
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three weeks later, once her renal function, albumin, blood pressure and nutrition were
She was readmitted one week after discharge with a small bowel obstruction. This was managed conservatively. Three weeks later she represented and a plain abdominal x-ray was suggestive of a partial small bowel obstruction. A contrast meal and follow-through study was performed but no strictures were demonstrated. A laparoscopy showed no adhesions, strictures or change in bowel calibre. She later developed diarrhoea and a stool sample was positive for Clostridium Difficile, which was treated with antibiotics. Her symptoms were attributed to this infection, and she was discharged seven weeks after admission.
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In the community, she continued to experience vomiting, abdominal distension and pain, and six weeks later she represented to hospital with these symptoms. An abdominal x-ray and a barium contrast study were repeated (Fig. 1, Fig. 2). These revealed a dilated, featureless
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transverse colon with a splenic flexure stricture. Fig. 1. Abdominal Plain Film
Fig. 2. Upper GI Contrast Study
Red arrows: Dilated transverse colon
Star: Dilated left hemicolon
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Yellow arrow: Site of stricture
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Green arrow: Collapse descending colon
She proceeded to laparotomy and was found to have a dilated small bowel and caecum, a dilated featureless transverse colon with a clear transition point proximal to the splenic flexure, and a healthy ascending, descending and sigmoid colon (Fig. 3.). A transverse
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colectomy was performed with primary anastomosis. Histopathology revealed evidence of chronic colitis with stricture formation (Fig. 4.).
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Fig. 3. Operative Findings
Fig. 4. Histological Findings Colonic wall with submucosal, mural and
White arrow: Omentum fused to stricture
extramural fibrosis and patchy chronic
Diamond: Decompressed distal transverse
inflammation.
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Star: Dilated proximal transverse colon
colon.
She was discharged nine days post-operatively, and has been well since, with good weight gain and full oral feed tolerance.
2. CASE REPORT 2
ACCEPTED MANUSCRIPT A 3-year-old male from a rural community presented in July 2016 with haematemesis, haematochezia, and a compensated metabolic acidosis secondary to severe dehydration, following ingestion of farm trough water. Laboratory results were consistent with HUS: haemoglobin of 87 g/L; platelet count 28 E+9/L; creatinine of 166 umol/L; and urea of
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15.0mmol/L. Stool cultures were positive for Giardia, Cryptosporidium and STEC. He was transferred to Starship Children’s Health PICU unit, where he required aggressive fluid resuscitation, ionotropic support, and antibiotics. He was managed with continuous
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venovenous haemofiltration initially, and progressed to intermittent haemodialysis.
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Several weeks into his admission, he developed abdominal distension, pain and vomiting. A plain abdominal x-ray demonstrated a dilated transverse colon with partial bowel obstruction. A contrast enema confirmed a stricture at the splenic flexure, a dilated transverse colon and marked luminal narrowing at the junction of the descending and sigmoid colon (Fig. 5). A
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computed tomography (CT) scan of the abdomen showed a transition point near the splenic flexure, with a narrow calibre thick-walled bowel beyond this likely representing a long stricture (Fig. 6).
Fig. 6. CT Abdomen
White arrow: Sigmoid colonic
White Star: Dilated transverse colon
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Fig. 5. Lower GI Contrast Study
stricture
Red Arrow: Colonic stricture Diamond: Liver
A laparotomy revealed chronic caecal, transverse and descending colonic inflammation, with dense adhesions between the transverse colon, stomach and jejunum. The appendix was heavily calcified and the descending colon was severely strictured. One short segment of jejunum was resected and anastomosed where there was a suspicion of a small bowel fistula
ACCEPTED MANUSCRIPT (Fig. 7). An appendicectomy was performed (Fig. 8), and a defunctioning ileostomy was fashioned, rather than resecting the colonic disease because of suspicion of ongoing active inflammation.
Fig. 8. Operative Findings
White arrow: Small bowel adhesions
Inflamed appendix
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Fig. 7. Operative Findings
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Histological findings described oedema and vascular congestion of the small bowel without
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evidence of fistulation, and active mucosal inflammation and ulceration of the appendix.
He returned five weeks later for a definitive laparotomy. A pre-operative lower GI contrast study confirmed a high-grade tapering stricture in the distal transverse colon (Fig. 9). Operative findings included extensive flimsy adhesions, a dilated ascending colon, and
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strictures at the transverse and proximal descending colon. An extended right hemicolectomy, closure of ileostomy, and adhesiolysis were performed. Histopathology revealed a colonic
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stricture, with fibrosis, ulceration and inflamed granulation tissue (Fig. 10).
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Fig. 9. Lower GI Contrast Study Prior
Fig. 10. Histological Findings
to Second Operation.
Inflamed portion of bowel with neutrophil
White arrow: Distal transverse colonic
infiltration.
stricture
He was discharged a week after his operation and has been well since, with no further hospital admissions at the time of publication.
ACCEPTED MANUSCRIPT 3. DISCUSSION 3.1. Background Shiga-toxin-producing Escherichia Coli (STEC) is transmitted to humans via contact with infected humans or animals, or ingestion of contaminated food or water1. Shiga toxin causes
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microvascular injury to glomerular and afferent capillary lumens, apoptosis, acute tubular necrosis and cortical necrosis in the kidney2. The connection between STEC and post-
diarrhoeal HUS was first reported in a study by Karmali et al in 19833. HUS is characterised
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by the pathognomonic triad of microangiopathic haemolytic anaemia, thrombocytopenia, and renal injury5. Masumoto et al13 state that shiga-toxin induced microangiopathy is postulated
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to be the main cause of ischaemia, and the resulting ischaemic change results in stricture.
Patients commonly present with abdominal pain, diarrhoea and haematochezia8. Serious sequelae include acute renal failure, with approximately 50% of patients requiring dialysis4,1
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in the acute phase. Long-term sequelae include proteinuria (15-30%), hypertension (5-15%), chronic kidney disease (9-18%) and end-stage renal disease (3-5%) 5, 9. The two cases in our series presented with unusually severe colitis and hypovolaemia. The combination of an acute
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kidney injury, hypovolaemia and acute colitis, as well as the fact that strictures formed within
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watershed areas of the bowel, may have led to the development of this rare pathology.
3.2. Gastrointestinal sequelae A wide variety of multisystem sequelae have been reported in the literature, encompassing renal, respiratory, cardiac, and neurological systems4, 3. The most common site of extra-renal involvement is the gastrointestinal tract8,10. Gastrointestinal sequelae may include pancreatic or colonic necrosis, intussusception, rectal prolapse, and infarction of organs supplied by the coeliac axis8. Colonic strictures as a late complication are rare11, 12, with only 22 cases
ACCEPTED MANUSCRIPT described over the last 40 years11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 28, including the two in this article (Table 1). Colonic strictures are estimated to occur in 1-3% of patients with HUS11, 12, but the actual numbers reported in the literature suggest that this is an overestimation.
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Any part of the colon may be affected by HUS colitis24. A retrospective analysis of 54
children found that the transverse and ascending colon were the most affected sites in HUSassociated colitis25. It has been stated that in STEC HUS, the left hemicolon is predominantly
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affected26. Previous studies suggest that late-presenting colonic strictures appear to affect the left colon more than the right. Both patients in our case series had strictures in the distal
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transverse colon proximal to the splenic flexure. This is known as a watershed region, as it receives dual blood supply from the most distal branches of the superior and inferior mesenteric artery. It is biologically plausible that hypotension may cause ischaemic injury to
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this area, where the blood supply is most vulnerable to low blood pressure.
The 3-year-old male in our case report had severe caecal and appendiceal disease, which is highly unusual given the typical pathological distribution for colonic strictures from HUS.
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All other authors report a predilection for left-sided colonic disease. In the literature, four patients had transverse colonic strictures, five patients had descending colonic strictures, and
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seven patients had sigmoid colonic strictures13. Maguire et al described a case where a fistula developed between the caecum and the sigmoid colon, but the patient primarily had sigmoidal disease23. De La Hunt et al described the only case of an oesophageal stricture resulting from HUS in 199127. Our second case therefore describes a rare and unique complication that has not previously been reported.
ACCEPTED MANUSCRIPT Segmental colonic ischaemia without necrosis may heal by fibrosis, leading to a reduced luminal diameter. The histology of both cases in this report showed acute and chronic inflammation, with ulceration and stricture formation. The majority of cases of HUS-related colonic strictures in the literature required operative management, except for two cases with
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mild strictures, who were managed conservatively14, 17. Most cases appeared to progress well after surgical resection, with only one report of ongoing issues following operative
management28. In this study, the patient had a sigmoid colectomy, but developed small bowel
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neuropathic and myopathic involvement post-operatively. Both of our cases required
operative management due to symptomatic colonic strictures, and both were considered
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surgically cured once these strictures were resected. We suggest that a contrast study be considered as the primary investigation for patients presenting in this manner. This aligns with recommendations made by Masumoto et al13.
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3.3 Postulated risk factors for development of colonic strictures after HUS Risk factors that may lead to formation of chronic colonic strictures after an acute episode of HUS have not been defined thus far. On review of the literature, the incidence of females
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developing this complication was slightly higher than males (59% vs. 41%), and there was an
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increased risk at a younger age, with the majority of patients being age 3 or under at diagnosis (59%), excluding cases in which age and sex was not specified.
It is possible that a severe initial presentation of HUS, or multiple ‘hits’ to the system could contribute towards colonic strictures. For example, in Case 1, the patient developed a Clostridium Difficile infection during her admission, which may have further impacted on already compromised bowel. Similarly, the second case had concurrent Giardia, Cryptosporidium and STEC infections on admission. In this case report, we believe that the
ACCEPTED MANUSCRIPT severity of both patient’s initial illness, including the presence of hypovolaemia, sepsis, multi-organ failure, and multiple-organism bacteraemia, could increase the risk of long-term colonic strictures.
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Spinale et al9 explored prognostic factors for long-term sequelae of HUS, and determined that the most important risk factor is the severity of the initial renal injury, in particular, anuria for over 10 days. The need for dialysis was also listed as a risk factor for long-term sequelae.
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Both of our patients required dialysis during their initial admissions. Although particular risk factors for the development of colonic strictures have not been explored, it follows that these
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indicators of severity are likely to increase the risk of all complications, including colonic strictures.
4. CONCLUSION
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Colonic stricture formation as a result of HUS is a rare but clinically important complication. The presentation can be insidious and may occur months to years after the initial insult. Surgical resection of colonic strictures appears to have favourable results but longer follow-
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up is needed. Our article adds significant information to the sparse body of material available
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thus far. Our first case illustrates the diagnostic conundrum that may occur in patients presenting with intermittent symptomatology. Our second patient describes the first case of right-sided colonic disease ever reported, and is the only publication from Australasia on this topic to date.
In both cases in this article, severe hypovolaemia on presentation may have contributed to the development of colonic strictures. The pathological formation of strictures may be attributable to both decreased blood flow to watershed areas of bowel, as well as colitis-
ACCEPTED MANUSCRIPT related microangiopathy. We therefore recommend aggressive fluid resuscitation and careful management of circulatory volume in hypovolaemic patients presenting with HUS. This may mitigate against serious complications such as stricture formation and the need for surgical
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intervention at a later date.
ACKNOWLEDGEMENTS: Thank you to the Paediatric Surgery Department at Starship Children’s Health, and to the families of the patients included in this study, for your support
REFERENCES
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and cooperation.
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2. Garg AX, Suri RS, Barrowman N, Rehman F, Matsell D, Rosas-Arellano MP,
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Salvadori M, Haynes RB, Clark WF. Long-term renal prognosis of diarrhoeaassociated hemolytic uremic syndrome: a systematic review, meta-analysis, and metaregression. Jama. 2003 Sep 10;290(10):1360-70.
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3. Karmali M, Petric M, Steele B, Lim C. Sporadic cases of haemolytic-uraemic
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syndrome associated with faecal cytotoxin and cytotoxin-producing Escherichia coli in stools. The Lancet. 1983 Mar 19;321(8325):619-20.
4. Siegler R, Oakes R. Hemolytic uremic syndrome; pathogenesis, treatment, and outcome. Current opinion in pediatrics. 2005 Apr 1;17(2):200-4.
5. Brandt JR, Joseph MW, Fouser LS, Tarr PI, Zelikovic I, McDonald RA, Avner ED, McAfee NG, Watkins SL. Cholelithiasis following Escherichia coli O157: H7associated hemolytic uremic syndrome. Pediatric Nephrology. 1998 Apr 4;12(3):2225.
ACCEPTED MANUSCRIPT 6. Russo P, Ruchelli ED, Piccoli DA, editors. Pathology of pediatric gastrointestinal and liver disease. Springer; 2014 Jun 5. 7. Miller DJ, Skucas J. The radiological examination of the colon: practical diagnosis. Springer Science & Business Media; 2012 Dec 6.
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8. McCarthy DW, Mutabagani K, Mahan JD, Caniano DA, Cooney DR. Infarction of the choledochus, liver, gallbladder, and pancreas: A unique complication of the
hemolytic uremic syndrome. Journal of pediatric surgery. 2000 Mar 31;35(3):502-4.
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9. Spinale JM, Ruebner RL, Copelovitch L, Kaplan BS. Long-term outcomes of Shiga toxin hemolytic uremic syndrome. Pediatric Nephrology. 2013 Nov 1;28(11):2097-
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10. Scheiring J, Andreoli SP, Zimmerhackl LB. Treatment and outcome of Shiga-toxinassociated hemolytic uremic syndrome (HUS). Pediatric nephrology. 2008 Oct 1;23(10):1749.
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11. Grodinsky S, Telmesani A, Robson WL, Pick G, Scott RB. Gastrointestinal manifestations of hemolytic uremic syndrome: recognition of pancreatitis. Journal of pediatric gastroenterology and nutrition. 1990 Nov 1;11(4):518-24.
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12. Tapper D, Tarr P, Avner E, Brandt J, Waldhausen J. Lessons learned in the
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management of hemolytic uremic syndrome in children. Journal of pediatric surgery. 1995 Feb 1;30(2):158-63.
13. Masumoto K, Nishimoto Y, Taguchi T, Tsutsumi Y, Kanemitsu S, Hara T, Suita S. Colonic stricture secondary to hemolytic uremic syndrome caused by Escherichia coli O-157. Pediatric nephrology. 2005 Oct 1;20(10):1496-9. 14. Peterson RB, Meseroll WP, Shrago GG, Gooding CA. Radiographic features of colitis associated with the hemolytic-uremic syndrome. Radiology. 1976 Mar;118(3):667-71.
ACCEPTED MANUSCRIPT 15. Sawaf H, Sharp MJ, Youn KJ, Jewell PA, Rabbani A. Ischemic colitis and stricture after hemolytic-uremic syndrome. Pediatrics. 1978 Feb 1;61(2):315-7. 16. Bax RP, Reeves DS, White LO, Holt A, Bywater M. Case records of the Massachusetts general hospital. Weekly clinicopathological exercises. Case 12-1981.
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N Eng J Med 304: 715-22.
17. Kirks DR. The radiology of enteritis due to hemolytic-uremic syndrome. Pediatric radiology. 1982 Jul 1;12(4):179-83.
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18. Crabbe DC, Broklebank JT, Spicer RD. Gastrointestinal complications of the
1;83(12):773-5.
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haemolytic uraemic syndrome. Journal of the Royal Society of Medicine. 1990 Dec
19. Gordon AC, Cundall DB, Spicer RD, Mortensen NJ. Colonic stricture secondary to haemolytic uraemic syndrome. The European journal of surgery= Acta chirurgica. 1994 Dec;160(12):707.
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20. Babaian ME, Chow DC, Soloway GN, Taubin HJ, Roberts IM. Colonic ischemic stricture presenting as a late complication of the hemolytic uremic syndrome. Journal of clinical gastroenterology. 1997 Oct 1;25(3):555-6.
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21. Sebbag H, Lemelle JL, Moller C, Schmitt M. Colonic stenosis after hemolytic-uremic
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syndrome. European journal of pediatric surgery. 1999 Apr;9(02):119-20. 22. Gerios AJ, Hupertz VF. Colonic stricture and perforation after hemolytic-uremic syndrome in a pediatric patient. The American Journal of Gastroenterology. 2002 Sep 30;97(9):S221.
23. Maguire K, Keys C, Kronfli R, Penman D, Haddock G. Colonic stricture and fistula following haemolytic uraemic syndrome with toxic megacolon. European journal of pediatric surgery. 2010 May;20(03):210-2.
ACCEPTED MANUSCRIPT 24. Baud C, Saguintaah M, Veyrac C, Couture A, Ferran JL, Barnéon G, Veyrac M. Sonographic diagnosis of colitis in children. European radiology. 2004 Nov 1;14(11):2105-19. 25. Rahman RC, Cobeñas CJ, Drut R, Amoreo OR, Ruscasso JD, Spizzirri AP, Suarez
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AD, Zalba JH, Ferrari C, Gatti MC. Hemorrhagic colitis in postdiarrheal hemolytic uremic syndrome: retrospective analysis of 54 children. Pediatric Nephrology. 2012 Feb 1;27(2):229-33.
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26. Ilnyckyj AL, Greenberg HO, Bernstein CN. Escherichia coli O157: H7 infection mimicking Crohn's disease. Gastroenterology. 1997 Mar 31;112(3):995-9.
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27. De la Hunt MN, Rangecroft L, Morris KP, Coulthard MG. Oesophageal and severe gut involvement in the haemolytic uraemic syndrome. British journal of surgery. 1991 Dec 1;78(12):1469-72.
28. Bowles C, Ancker M, Triadafilopoulos G. Gastrointestinal dysfunction following
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ACCEPTED MANUSCRIPT Tables
Table 1: Literature Review of HUS-Related Colonic Strictures
Brandt et
Yea
Site of Stricture
Age at
r
HUS onset
1998 ND
ND
Grodinsk
1990 ND
ND
1990 ND
ND
2 months
ND
ND
ND
ND
ND
ND
ND
5
F
3 months
5
M
3 weeks
2
M
5 months
y et al Grodinsk
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[11]
y et al [12]
Tapper et 1995 Sigmoid colon al
Masumot 2004 Sigmoid colon o et al
[14]
Peterson
[15]
Sawaf et
1978 Descending colon
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al
1976 Descending colon
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et al
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[13]
Duration
ND
al [11]
Sex
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[5]
Author
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Ref.
[16]
Bax et al
1981 Descending colon
7
F
ND
[17]
Kirks et
1982 Transverse colon
3
M
6 weeks
1982 ND
ND
ND
10 weeks
1990 Transverse colon
5
F
1 year
al [17]
Kirks et al
[18]
Crabbe
ACCEPTED MANUSCRIPT et al [18]
Crabbe
1990 Descending colon
2
F
6 weeks
1994 Descending colon
3
F
1 month
1994 Transverse colon
3
[19]
Gordon et al
[19]
Gordon et al
Babian et 1997 Sigmoid colon
32
al Sebbag
5 years
M
14 months
2010 Sigmoid colon +
4
M
1.5 years
3
M
5 weeks
2011 Sigmoid colon
10
F
5 years
Our case
2016 Transverse colon
1
F
5 months
Our case
2016 Transverse /
3
M
4 weeks
caeco-sigmoid fistula
1991 Oesophagus
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De La
al
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Maguire
Hunt et
[28]
F
2
et al [27]
4 years
2002 Sigmoid colon
Gerios et al
[23]
F
1
et al [22]
1 year
1999 Transverse colon
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[21]
F
SC
[20]
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et al
Bowles et al
-
descending / sigmoid colon + appendix
Fig. 1. Abdominal Plain Film Red arrows: Dilated transverse colon
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ACCEPTED MANUSCRIPT
Fig. 2. Upper GI Contrast Study Star: Dilated left hemicolon Yellow arrow: Site of stricture
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Green arrow: Collapse descending colon
Fig. 3. Operative Findings Star: Dilated proximal transverse colon Fig. 4. Histological Findings White arrow: Inflamed omentum fused to Colonic wall showing submucosal, mural stricture and extramural fibrosis and patchy chronic Diamond: Decompressed distal transverse
ACCEPTED MANUSCRIPT inflammation.
Fig. 6. CT Abdomen
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Fig. 5. Lower GI Contrast Study
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colon
White Star: Dilated transverse colon
stricture
Red Arrow: Colonic stricture
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White arrow: Sigmoid colonic
Diamond: Liver
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Fig 8. Operative Findings
White arrow: Small bowel adhesions
Inflamed appendix
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Fig. 7. Operative Findings
Fig. 9. Lower GI Contrast Study Prior
Fig. 10. Histological Findings
to Second Operation.
Inflamed portion of bowel with neutrophil
White arrow: Distal transverse colonic
infiltration.
stricture
ACCEPTED MANUSCRIPT Highlights •
A combination of microangiopathy and hypovolaemic bowel ischaemia in the acute setting of HUS may lead to colonic strictures Colonic strictures may be difficult to diagnose and may present months to years after the initial episode of HUS
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•
•
A contrast study is the initial investigation of choice to identify colonic strictures
•
Aggressive fluid resuscitation and careful management of circulatory volume is
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crucial in managing critically unwell patients with HUS