Original Article
51
Ulcerative Ileitis after Proctocolectomy in Children: A Complication of Ulcerative Colitis or a Disease in Itself after Ileoanal Pullthrough? Alba Sánchez-Galán1 Ane M. Andres1 Beatriz Fernández-Caamaño2 Alejandra Vilanova1 Eva Dominguez1 Ruben Ortiz1 Gerardo Prieto2 Manuel Lopez-Santamaria1 J. A. Tovar1 1 Department of Pediatric Surgery, Universitary Hospital La Paz,
Madrid, Spain 2 Pediatric Gastroenterology Service, Universitary Hospital La Paz, Madrid, Spain
Address for correspondence Alba Sánchez-Galán, MD, Department of Pediatric Surgery, Hospital Universitario La Paz, Paseo de la Castellana 261 Madrid 28046, Spain (e-mail: [email protected]).
Abstract
Keywords
► ► ► ►
ulcerative ileitis prepouch ileitis pouchitis restorative proctocolectomy ► children
received May 15, 2014 accepted June 23, 2014 published online August 21, 2014
Ulcerative ileitis (UI) after restorative proctocolectomy (RPC) and ileoanal pullthrough procedure (IAPP) is a rare condition described as inflammation of the terminal ileum proximal to the anastomosis. It is mostly observed in ulcerative colitis (UC) and is designated as prepouch ileitis; sometimes with pouchitis, but not necessarily all the time. Its incidence in adults is less than 5%, but the information in children is limited. Pathogenesis is unknown, it has been considered as a disease by itself, independently on the underlying disease. It involves additional morbidity and impacts negatively on quality of life because there is no effective treatment so far. This study aims to review the cases of UI in our institution and to summarize their clinical features and management. A descriptive retrospective study of UI patients between 1990 and 2013 was conducted. Children with RPC and IAPP with clinical and pathological diagnosis of UI were included. Sociodemographic and clinical data, diagnostic, and therapeutic procedures were collected through medical records. UI was diagnosed in eight patients (six males) after RCP; four had UC, two had total colonic aganglionosis (TCA), and two had complex anorectal malformations (one cloacal exstrophy and one omphalopagus twin with bladder exstrophy). Different surgical techniques were used in each case: UC patients underwent IAPP, 50% with J-reservoir and 50% without it; posterior sagittal pullthrough was performed in those with anorectal malformations; one Soave and one Swenson procedure in those with TCA. In summary, three patients had reservoir and five did not. The median age at the IAPP and ostomy closure was 6 years (range 2 months–8 years) and 7 years (range 6 months–9 years), respectively. UI was found after a median of 23 months (range 1–48 months), all of them after digestive tract continuity was reestablished. The leading symptom was lower GI or gastrointestinal, both of them bleeding with abdominal pain, followed by abdominopelvic abscesses and malabsorption with weight loss. Pathology showed nonspecific inflammatory changes. Treatment included antibiotics, corticosteroids and/or immunosuppressive agents with variable response, requiring a new ileostomy in five cases. Ileitis disappeared after diversion. In
© 2015 Georg Thieme Verlag KG Stuttgart · New York
DOI http://dx.doi.org/ 10.1055/s-0034-1386643. ISSN 0939-7248.
Downloaded by: Queen's University. Copyrighted material.
Eur J Pediatr Surg 2015;25:51–55.
Ulcerative Ileitis after Proctocolectomy in Children
Sánchez Galán et al.
our experience, UI after colectomy is not an exclusive feature of UC as has been previously described. Although it appears with pouchitis, the presence of a reservoir is not a must, suggesting that this is a different entity. No medical treatment has been really effective in our patients and diversion above this level stopped the process. Further studies on its pathogenesis and treatment strategies are necessary.
Introduction Colectomy is necessary in the treatment of several diseases. Several techniques for restoring the continuity of the bowel and/or to divert it have been described, such as ileostomy, ileorectal or ileoanal anastomosis with or without pouch. All of them force the ileum to contract against a nonphysiological pressure environment that may cause damage to the ileum as it has been known for several decades.1–5 Inflammation of the ileum after a colectomy has been called in different ways over time: prestomal ileitis, postcolectomy ileitis, terminal ileitis, and prepouch ileitis.1,5–7 We chose the term ulcerative ileitis (UI) to designate this entity. Several pathogenetic factors have been proposed (infectious, mechanical, immunological/genetic, and ischemic) but the origin of the inflammation remains unknown. It is unclear whether it is an entity by itself or if it depends on the underlying disease. The UI has been mostly observed after restorative proctocolectomy (RPC) and ileoanal pullthrough procedure (IAPP) in cases of ulcerative colitis (UC).6–10 Incidence in adults has been estimated to be less than 5%, but the information available is limited in children.6,7,10 In these cases, it is important to differentiate it from “backwash ileitis,” the condition of the terminal ileum in UC. At first it was thought, like a reaction to the reflux of colonic contents into the terminal ileum, hence its name11; but its origin may be more complex and it is possible that UC with ileitis represents a distinct disease-specific phenomenon.12 In fact, while backwash ileitis always develops when
the affected colon is in place and disappears after RPC, on the contrary UI may appear at a variable time after colectomy, sometimes even years.1,6,7 UI can be asymptomatic and go unnoticed or mean additional morbidity and impact negatively on quality of life, particularly because no effective treatment has been implemented so far.9,10,13–15 The aim of this study was to review the cases of UI in our institution and to recognize their clinical features and management.
Materials and Methods We reviewed retrospectively the records of pediatric patients with UI undergoing RCP and IAPP between 1990 and 2013. Sociodemographic and clinical data, as well as diagnostic and therapeutic procedures were collected. Surgical procedures/ details were reported. The UI was defined as inflammation of the new terminal ileum after RPC and in all cases information on symptoms, endoscopy, and pathology of the lesions was collected. Numerical results were expressed as median and range.
Results UI was diagnosed in eight patients (six males and two females) after RPC; with a median age at IAPP of 6 years (range 2 months–8 years). In all cases, the IAPP was accompanied by a diverting ileostomy that was subsequently closed.
Table 1 Patient characteristics No.
Gender
Colectomy age
Ostomy closure age
Diagnosis
Surgery
Intervala (mo)
1
M
2y
na
UC
J-Pouch
na
2
M
5y
6y
UC
J-Pouch
na
3
F
7y
7y
UC
IAPP
4
4
M
8y
8y
UC
IAPP
1
5
M
2 mo
6 mo
TCA
Soave
6
6
M
6y
6y
TCA
Swenson
48
7
F
2y
3y
ARM
PSARP
40
8
M
8y
9v
ARM
PSARP
48
Abbreviations: ARM, anorectal malformation; F, female; IAPP, ileoanal pullthrough procedure; M, male; na, not available; PSARP, posterior sagittal anorectoplasty pullthrough; TCA, total colonic aganglionosis; UC, ulcerative colitis. a Interval is the time between ostomy closure and the development of ileitis. European Journal of Pediatric Surgery
Vol. 25
No. 1/2015
Downloaded by: Queen's University. Copyrighted material.
52
Ulcerative Ileitis after Proctocolectomy in Children
53
severe UI was identified that did not respond to any of the treatments applied. Finally, temporary or permanent bowel diversion was performed with complete recovery and resolution of the lesions. Overall, the median time between ostomy closure and the development of UI was 23 months (range 1–48 months). The most frequent symptom was lower gastrointestinal bleeding with abdominal pain, followed by abdominopelvic abscesses and malabsorptive diarrhea with weight loss. Endoscopic findings were similar in all patients and consisted of an erythematous and friable mucosa, spontaneous bleeding, complete loss of vascular pattern with large scattered ulcerations, and sometimes pseudopolyps formation (►Fig. 1a, b). Extension of affected ileum ranged from a few centimeters above the anal margin (including pouchitis) to 40 cm
Downloaded by: Queen's University. Copyrighted material.
Median age at the ostomy closure was 7 years (range 6 months–9 years). Half of the patients4 had UC and four had the other: two total colonic aganglionosis (TCA) and two complex anorectal malformations (one cloacal exstrophy and one omphalopagus twin with bladder exstrophy) (►Table 1). Different surgical techniques were used in each case. Patients with UC underwent IAPP, 50% with J-reservoir and 50% without a reservoir. UI always appeared after the ileostomy closure, and it was also associated with pouchitis in patients with reservoir. In one patient without a reservoir, early symptoms appeared with the onset of pelvic abscesses, fever, and even an enterovesical fistula. This behavior is rare in cases of UC, and it was mandatory to rule out Crohn disease. However, granulomatous reaction was never found along multiple biopsies that were made throughout the evolution of the patient. In those with TCA, one Soave and one Swenson procedure were performed. UI was remarkable here; the first by intense inflammatory activity causing abscesses and fistulization to the perineum, bleeding, and secondary anemia; the second by a profuse and chronic diarrhea that resulted in dehydration and failure to thrive. It was again necessary to rule out celiac disease and Crohn disease in the second case, but the diagnostic tests were inconclusive and all treatment attempts failed. Both patients required a new ileostomy to alleviate the symptoms. Last, both patients with anorectal malformation underwent a posterior sagittal pullthrough procedure. The child with cloacal exstrophy initially had small intestine, ileocecal valve and approximately 20 cm of the colon. The first intervention consisted of an intestinal-bladder plate closure and terminal colostomy. A few years later the colon was descended by a posterior sagittal anorectoplasty and anterior approach along and a protective ileostomy was performed. Two years after the ileostomy takes down, the patient developed a UI that affected 30 cm of ileum between the end-toend anastomosis and the ileocecal valve. Refractory anemia caused by UI forced to perform a new ileostomy and complete recovery was achieved 1 year later. Finally, bowel continuity was again restored resecting part of the ileum, but unfortunately the UI has kept on appearing in endoscopic controls. Regarding the omphalopagus twins, both sisters were separated the first days of life. Both twins had no anus, a high urogenital junction with Mullerian duplication and a single bladder plate under an omphalocele with two umbilical cords. In the center of the bladder plate ended one single colon. To distribute the colon during the separation of the twins, our patient received the distal part which was anastomosed end-to-end to the ileum above the ileocecal valve. The other twin received the valve, cecum, appendix, and the proximal half of the colon. In the first surgery, digestive tract was diverted. Later the posterior sagittal anorectoplasty was performed and finally the stoma was closed. About 40 months after the ileostomy closure, the patient began to suffer from lower gastrointestinal bleeding causing anemia and need for parenteral iron therapy and transfusions, in addition to the increase in bowel movements and fever. Endoscopically a
Sánchez Galán et al.
Fig. 1 (a) A patient diagnosed with ulcerative colitis, the endoscopic appearance of the small intestine above the reservoir showed a friable and edematous mucosa, thickening of folds, with superficial ulcers whose background consists of fibrin. (b) Endoscopic appearance of the reservoir with pouchitis and presence of an inflammatory polyp. European Journal of Pediatric Surgery
Vol. 25
No. 1/2015
Ulcerative Ileitis after Proctocolectomy in Children
Sánchez Galán et al.
proximally. In those cases with colon interposed between the outside and the terminal ileum, the UI was located in the ileum proximal to the ileocolic junction and involvement of the colon was variable. Histology showed a small bowel mucosa with multiple and superficial ulcerations and unspecified areas of acute and chronic inflammation (►Fig. 2). In all cases Crohn disease or infectious gastroenteritis was ruled out. Antibiotics were the treatment most commonly used (metronidazole), although probiotics, steroids, 5-ASA, azathioprine, tacrolimus, and infliximab were also used with an irregular response. Improvement of symptoms was observed with metronidazole, but the lesions did not disappear and patients became dependent to treatment. Only bowel disconnection achieved the complete remission of symptoms and lesions, taking place in 5/8 patients (62%) (►Fig. 3a, b).
Discussion Colon may be affected by different diseases requiring resection. Occasionally, the terminal ileum is secondarily affected after colectomy as in “backwash ileitis.” After removal of the colon, bowel continuity must be restored so new terminal ileum is placed in a nonphysiological area and an unnatural environment for it. Pressure conditions, characteristics of the bolus and the bacteriological environment change. Sometimes, this can result in a diseased ileum.1–5 Over time “postcolectomy ileitis” has been reported in different ways in the literature,1,5 but this entity has been recently described related to UC, defined as “prepouch ileitis.” Initially this was associated with pouchitis, caused by the same possible pathogenetic factors: fecal stasis, bacterial overgrowth, and inflammatory response to it.8,10,16 However, Bell described it in the absence of pouchitis and prepouch ileitis was proposed as a UC variant itself.6 In this study, we reviewed UC patients but also other diseases that led to the partial or total removal of the colon (TCA, anorectal malformation), and it has also been reported after colectomy for carcinomas and diverticulosis.1 Inflam-
Fig. 2 Histology of ulcerative ileitis patient showing an intestinal mucosa with an ulcer, disruption of the epithelium (arrows) and mucous content output with inflammatory infiltrate. European Journal of Pediatric Surgery
Vol. 25
No. 1/2015
Fig. 3 (a) Endoscopy of a patient with TCA that after colectomy developed a UI with multiple aphthae and erythematous mucosa. (b) The lesions completely disappeared after bowel diversion. TCA, total colonic aganglionosis; UI, ulcerative ileitis.
mation of the “new terminal ileum” appeared in all of them after the stoma closure at a variable time. Thus, UI is not related to the underlying disease and the presence of reservoir. The appearance of lesions after the stoma closure and its improvement after the diversion showed that it is probably an entity by itself and that its pathogenesis is related to local factors. When the colon is partially or completely removed, several conditions change: in the first place, the distance between the ileum and the outside is shorter; furthermore the ileocecal valve and the lymphoid structures of the terminal ileum are missing; moreover the absorptive capacity of the bowel changes and so does the fecal content (different composition, number and consistency of the stools); and finally a phenomenon of disbacteriosis or bacterial overgrowth can contribute. All of these factors trigger an acute and chronic nonspecific inflammatory reaction that we have called UI. Of course, we must be careful not to confuse it with other diseases as infectious processes, backwash ileitis or Crohn disease. In our review, we have a case where the behavior of
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54
Ulcerative Ileitis after Proctocolectomy in Children
Conflict of Interest None.
6 Bell AJ, Price AB, Forbes A, Ciclitira PJ, Groves C, Nicholls RJ. Pre-
7
8
9
10
11
12
13
References 1 Hallak A, Baratz M, Santo M, et al. Ileitis after colectomy for
ulcerative colitis or carcinoma. Gut 1994;35(3):373–376
14
2 Warren R, McKittrick LS. Ileostomy for ulcerative colitis; tech-
nique, complications, and management. Surg Gynecol Obstet 1951;93(5):555–567 3 Rogers AG, Bargen JA, Black BM. Chronic ulcerative colitis: early and late experiences of 124 patients with ileac stomas. Gastroenterology 1954;27(4):383–398 4 Lyons AS, Garlock JH. The complications of ileostomy. Surgery 1954;36(4):784–789 5 Thayer WR, Spiro HM. Ileitis after ileostomy: prestomal ileitis. Gastroenterology 1962;42(5):547–554
55
15
16
pouch ileitis: a disease of the ileum in ulcerative colitis after restorative proctocolectomy. Colorectal Dis 2006;8(5):402–410 Slatter C, Girgis S, Huynh H, El-Matary W. Pre-pouch ileitis after colectomy in paediatric ulcerative colitis. Acta Paediatr 2008; 97(3):381–383 Iwata T, Yamamoto T, Umegae S, Matsumoto K. Pouchitis and prepouch ileitis developed after restorative proctocolectomy for ulcerative colitis: a case report. World J Gastroenterol 2007; 13(4):643–646 Ugolini G, Rosati G, Montroni I, et al. Prepouch ileitis, myth or reality? The first case with acute abdomen. Inflamm Bowel Dis 2010;16(1):12–14 McLaughlin SD, Clark SK, Bell AJ, Tekkis PP, Ciclitira PJ, Nicholls RJ. Incidence and short-term implications of prepouch ileitis following restorative proctocolectomy with ileal pouch-anal anastomosis for ulcerative colitis. Dis Colon Rectum 2009;52(5):879–883 McCready FJ, Bargen JA, Dockerty MB, et al. Involvement of the ileum in chronic ulcerative colitis. N Engl J Med 1949;240(4): 119–127 Abdelrazeq AS, Wilson TR, Leitch DL, Lund JN, Leveson SH. Ileitis in ulcerative colitis: is it a backwash? Dis Colon Rectum 2005;48(11): 2038–2046 McLaughlin SD, Clark SK, Bell AJ, Tekkis PP, Ciclitira PJ, Nicholls RJ. An open study of antibiotics for the treatment of pre-pouch ileitis following restorative proctocolectomy with ileal pouch-anal anastomosis. Aliment Pharmacol Ther 2009;29(1):69–74 McLaughlin SD, Clark SK, Roberts CH, et al. Extended spectrum beta-lactamase-producing bacteria and Clostridium difficile in patients with pouchitis. Aliment Pharmacol Ther 2010;32(5): 664–669 McLaughlin SD, Clark SK, Tekkis PP, Ciclitira PJ, Nicholls RJ. An open study of maintenance antibiotic therapy for chronic antibioticdependent pouchitis: efficacy, complications and outcome. Colorectal Dis 2011;13(4):438–444 Kuisma J, Järvinen H, Kahri A, Färkkilä M. Factors associated with disease activity of pouchitis after surgery for ulcerative colitis. Scand J Gastroenterol 2004;39(6):544–548
This article has been updated as per the erratum published on October 26, 2014; doi: 10.1055/s-0034-1394279. The author names have been corrected. The correct listing of author names is as follows: Alba Sánchez-Galán, Ane M. Andres, Beatriz Fernández-Caamaño, Alejandra Vilanova, Eva Dominguez, Ruben Ortiz, Gerardo Prieto, Manuel Lopez-Santamaria, and J. A. Tovar.
European Journal of Pediatric Surgery
Vol. 25
No. 1/2015
Downloaded by: Queen's University. Copyrighted material.
the disease is typical of Crohn disease, although we were never able to demonstrate the presence of granulomas in biopsies or surgical specimens. Perianal activity was so intense that forced to resect the pulled through ileum with diversion. The reasons why the UI occurs only after a few colectomies and is not expressed with the same intensity in all patients are unknown. What we know is that like pouchitis, it responds poorly to treatments applied.1,6,10,14,15 Only bowel diversion resolved the symptoms in our series. However, it is clear that an ileostomy is not the best treatment or the preferred by the patient, family, and surgeon; moreover ileostomy involves other complications and morbidities. Therefore, it is necessary to further investigate this condition and its pathogenesis to offer better treatment options in the future.
Sánchez Galán et al.
51
Ulcerative Ileitis after Proctocolectomy in Children: A Complication of Ulcerative Colitis or a Disease in Itself after Ileoanal Pullthrough? Alba Sánchez-Galán1 Ane M. Andres1 Beatriz Fernández-Caamaño2 Alejandra Vilanova1 Eva Dominguez1 Ruben Ortiz1 Gerardo Prieto2 Manuel Lopez-Santamaria1 J. A. Tovar1 1 Department of Pediatric Surgery, Universitary Hospital La Paz,
Madrid, Spain 2 Pediatric Gastroenterology Service, Universitary Hospital La Paz, Madrid, Spain
Address for correspondence Alba Sánchez-Galán, MD, Department of Pediatric Surgery, Hospital Universitario La Paz, Paseo de la Castellana 261 Madrid 28046, Spain (e-mail: [email protected]).
Abstract
Keywords
► ► ► ►
ulcerative ileitis prepouch ileitis pouchitis restorative proctocolectomy ► children
received May 15, 2014 accepted June 23, 2014 published online August 21, 2014
Ulcerative ileitis (UI) after restorative proctocolectomy (RPC) and ileoanal pullthrough procedure (IAPP) is a rare condition described as inflammation of the terminal ileum proximal to the anastomosis. It is mostly observed in ulcerative colitis (UC) and is designated as prepouch ileitis; sometimes with pouchitis, but not necessarily all the time. Its incidence in adults is less than 5%, but the information in children is limited. Pathogenesis is unknown, it has been considered as a disease by itself, independently on the underlying disease. It involves additional morbidity and impacts negatively on quality of life because there is no effective treatment so far. This study aims to review the cases of UI in our institution and to summarize their clinical features and management. A descriptive retrospective study of UI patients between 1990 and 2013 was conducted. Children with RPC and IAPP with clinical and pathological diagnosis of UI were included. Sociodemographic and clinical data, diagnostic, and therapeutic procedures were collected through medical records. UI was diagnosed in eight patients (six males) after RCP; four had UC, two had total colonic aganglionosis (TCA), and two had complex anorectal malformations (one cloacal exstrophy and one omphalopagus twin with bladder exstrophy). Different surgical techniques were used in each case: UC patients underwent IAPP, 50% with J-reservoir and 50% without it; posterior sagittal pullthrough was performed in those with anorectal malformations; one Soave and one Swenson procedure in those with TCA. In summary, three patients had reservoir and five did not. The median age at the IAPP and ostomy closure was 6 years (range 2 months–8 years) and 7 years (range 6 months–9 years), respectively. UI was found after a median of 23 months (range 1–48 months), all of them after digestive tract continuity was reestablished. The leading symptom was lower GI or gastrointestinal, both of them bleeding with abdominal pain, followed by abdominopelvic abscesses and malabsorption with weight loss. Pathology showed nonspecific inflammatory changes. Treatment included antibiotics, corticosteroids and/or immunosuppressive agents with variable response, requiring a new ileostomy in five cases. Ileitis disappeared after diversion. In
© 2015 Georg Thieme Verlag KG Stuttgart · New York
DOI http://dx.doi.org/ 10.1055/s-0034-1386643. ISSN 0939-7248.
Downloaded by: Queen's University. Copyrighted material.
Eur J Pediatr Surg 2015;25:51–55.
Ulcerative Ileitis after Proctocolectomy in Children
Sánchez Galán et al.
our experience, UI after colectomy is not an exclusive feature of UC as has been previously described. Although it appears with pouchitis, the presence of a reservoir is not a must, suggesting that this is a different entity. No medical treatment has been really effective in our patients and diversion above this level stopped the process. Further studies on its pathogenesis and treatment strategies are necessary.
Introduction Colectomy is necessary in the treatment of several diseases. Several techniques for restoring the continuity of the bowel and/or to divert it have been described, such as ileostomy, ileorectal or ileoanal anastomosis with or without pouch. All of them force the ileum to contract against a nonphysiological pressure environment that may cause damage to the ileum as it has been known for several decades.1–5 Inflammation of the ileum after a colectomy has been called in different ways over time: prestomal ileitis, postcolectomy ileitis, terminal ileitis, and prepouch ileitis.1,5–7 We chose the term ulcerative ileitis (UI) to designate this entity. Several pathogenetic factors have been proposed (infectious, mechanical, immunological/genetic, and ischemic) but the origin of the inflammation remains unknown. It is unclear whether it is an entity by itself or if it depends on the underlying disease. The UI has been mostly observed after restorative proctocolectomy (RPC) and ileoanal pullthrough procedure (IAPP) in cases of ulcerative colitis (UC).6–10 Incidence in adults has been estimated to be less than 5%, but the information available is limited in children.6,7,10 In these cases, it is important to differentiate it from “backwash ileitis,” the condition of the terminal ileum in UC. At first it was thought, like a reaction to the reflux of colonic contents into the terminal ileum, hence its name11; but its origin may be more complex and it is possible that UC with ileitis represents a distinct disease-specific phenomenon.12 In fact, while backwash ileitis always develops when
the affected colon is in place and disappears after RPC, on the contrary UI may appear at a variable time after colectomy, sometimes even years.1,6,7 UI can be asymptomatic and go unnoticed or mean additional morbidity and impact negatively on quality of life, particularly because no effective treatment has been implemented so far.9,10,13–15 The aim of this study was to review the cases of UI in our institution and to recognize their clinical features and management.
Materials and Methods We reviewed retrospectively the records of pediatric patients with UI undergoing RCP and IAPP between 1990 and 2013. Sociodemographic and clinical data, as well as diagnostic and therapeutic procedures were collected. Surgical procedures/ details were reported. The UI was defined as inflammation of the new terminal ileum after RPC and in all cases information on symptoms, endoscopy, and pathology of the lesions was collected. Numerical results were expressed as median and range.
Results UI was diagnosed in eight patients (six males and two females) after RPC; with a median age at IAPP of 6 years (range 2 months–8 years). In all cases, the IAPP was accompanied by a diverting ileostomy that was subsequently closed.
Table 1 Patient characteristics No.
Gender
Colectomy age
Ostomy closure age
Diagnosis
Surgery
Intervala (mo)
1
M
2y
na
UC
J-Pouch
na
2
M
5y
6y
UC
J-Pouch
na
3
F
7y
7y
UC
IAPP
4
4
M
8y
8y
UC
IAPP
1
5
M
2 mo
6 mo
TCA
Soave
6
6
M
6y
6y
TCA
Swenson
48
7
F
2y
3y
ARM
PSARP
40
8
M
8y
9v
ARM
PSARP
48
Abbreviations: ARM, anorectal malformation; F, female; IAPP, ileoanal pullthrough procedure; M, male; na, not available; PSARP, posterior sagittal anorectoplasty pullthrough; TCA, total colonic aganglionosis; UC, ulcerative colitis. a Interval is the time between ostomy closure and the development of ileitis. European Journal of Pediatric Surgery
Vol. 25
No. 1/2015
Downloaded by: Queen's University. Copyrighted material.
52
Ulcerative Ileitis after Proctocolectomy in Children
53
severe UI was identified that did not respond to any of the treatments applied. Finally, temporary or permanent bowel diversion was performed with complete recovery and resolution of the lesions. Overall, the median time between ostomy closure and the development of UI was 23 months (range 1–48 months). The most frequent symptom was lower gastrointestinal bleeding with abdominal pain, followed by abdominopelvic abscesses and malabsorptive diarrhea with weight loss. Endoscopic findings were similar in all patients and consisted of an erythematous and friable mucosa, spontaneous bleeding, complete loss of vascular pattern with large scattered ulcerations, and sometimes pseudopolyps formation (►Fig. 1a, b). Extension of affected ileum ranged from a few centimeters above the anal margin (including pouchitis) to 40 cm
Downloaded by: Queen's University. Copyrighted material.
Median age at the ostomy closure was 7 years (range 6 months–9 years). Half of the patients4 had UC and four had the other: two total colonic aganglionosis (TCA) and two complex anorectal malformations (one cloacal exstrophy and one omphalopagus twin with bladder exstrophy) (►Table 1). Different surgical techniques were used in each case. Patients with UC underwent IAPP, 50% with J-reservoir and 50% without a reservoir. UI always appeared after the ileostomy closure, and it was also associated with pouchitis in patients with reservoir. In one patient without a reservoir, early symptoms appeared with the onset of pelvic abscesses, fever, and even an enterovesical fistula. This behavior is rare in cases of UC, and it was mandatory to rule out Crohn disease. However, granulomatous reaction was never found along multiple biopsies that were made throughout the evolution of the patient. In those with TCA, one Soave and one Swenson procedure were performed. UI was remarkable here; the first by intense inflammatory activity causing abscesses and fistulization to the perineum, bleeding, and secondary anemia; the second by a profuse and chronic diarrhea that resulted in dehydration and failure to thrive. It was again necessary to rule out celiac disease and Crohn disease in the second case, but the diagnostic tests were inconclusive and all treatment attempts failed. Both patients required a new ileostomy to alleviate the symptoms. Last, both patients with anorectal malformation underwent a posterior sagittal pullthrough procedure. The child with cloacal exstrophy initially had small intestine, ileocecal valve and approximately 20 cm of the colon. The first intervention consisted of an intestinal-bladder plate closure and terminal colostomy. A few years later the colon was descended by a posterior sagittal anorectoplasty and anterior approach along and a protective ileostomy was performed. Two years after the ileostomy takes down, the patient developed a UI that affected 30 cm of ileum between the end-toend anastomosis and the ileocecal valve. Refractory anemia caused by UI forced to perform a new ileostomy and complete recovery was achieved 1 year later. Finally, bowel continuity was again restored resecting part of the ileum, but unfortunately the UI has kept on appearing in endoscopic controls. Regarding the omphalopagus twins, both sisters were separated the first days of life. Both twins had no anus, a high urogenital junction with Mullerian duplication and a single bladder plate under an omphalocele with two umbilical cords. In the center of the bladder plate ended one single colon. To distribute the colon during the separation of the twins, our patient received the distal part which was anastomosed end-to-end to the ileum above the ileocecal valve. The other twin received the valve, cecum, appendix, and the proximal half of the colon. In the first surgery, digestive tract was diverted. Later the posterior sagittal anorectoplasty was performed and finally the stoma was closed. About 40 months after the ileostomy closure, the patient began to suffer from lower gastrointestinal bleeding causing anemia and need for parenteral iron therapy and transfusions, in addition to the increase in bowel movements and fever. Endoscopically a
Sánchez Galán et al.
Fig. 1 (a) A patient diagnosed with ulcerative colitis, the endoscopic appearance of the small intestine above the reservoir showed a friable and edematous mucosa, thickening of folds, with superficial ulcers whose background consists of fibrin. (b) Endoscopic appearance of the reservoir with pouchitis and presence of an inflammatory polyp. European Journal of Pediatric Surgery
Vol. 25
No. 1/2015
Ulcerative Ileitis after Proctocolectomy in Children
Sánchez Galán et al.
proximally. In those cases with colon interposed between the outside and the terminal ileum, the UI was located in the ileum proximal to the ileocolic junction and involvement of the colon was variable. Histology showed a small bowel mucosa with multiple and superficial ulcerations and unspecified areas of acute and chronic inflammation (►Fig. 2). In all cases Crohn disease or infectious gastroenteritis was ruled out. Antibiotics were the treatment most commonly used (metronidazole), although probiotics, steroids, 5-ASA, azathioprine, tacrolimus, and infliximab were also used with an irregular response. Improvement of symptoms was observed with metronidazole, but the lesions did not disappear and patients became dependent to treatment. Only bowel disconnection achieved the complete remission of symptoms and lesions, taking place in 5/8 patients (62%) (►Fig. 3a, b).
Discussion Colon may be affected by different diseases requiring resection. Occasionally, the terminal ileum is secondarily affected after colectomy as in “backwash ileitis.” After removal of the colon, bowel continuity must be restored so new terminal ileum is placed in a nonphysiological area and an unnatural environment for it. Pressure conditions, characteristics of the bolus and the bacteriological environment change. Sometimes, this can result in a diseased ileum.1–5 Over time “postcolectomy ileitis” has been reported in different ways in the literature,1,5 but this entity has been recently described related to UC, defined as “prepouch ileitis.” Initially this was associated with pouchitis, caused by the same possible pathogenetic factors: fecal stasis, bacterial overgrowth, and inflammatory response to it.8,10,16 However, Bell described it in the absence of pouchitis and prepouch ileitis was proposed as a UC variant itself.6 In this study, we reviewed UC patients but also other diseases that led to the partial or total removal of the colon (TCA, anorectal malformation), and it has also been reported after colectomy for carcinomas and diverticulosis.1 Inflam-
Fig. 2 Histology of ulcerative ileitis patient showing an intestinal mucosa with an ulcer, disruption of the epithelium (arrows) and mucous content output with inflammatory infiltrate. European Journal of Pediatric Surgery
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Fig. 3 (a) Endoscopy of a patient with TCA that after colectomy developed a UI with multiple aphthae and erythematous mucosa. (b) The lesions completely disappeared after bowel diversion. TCA, total colonic aganglionosis; UI, ulcerative ileitis.
mation of the “new terminal ileum” appeared in all of them after the stoma closure at a variable time. Thus, UI is not related to the underlying disease and the presence of reservoir. The appearance of lesions after the stoma closure and its improvement after the diversion showed that it is probably an entity by itself and that its pathogenesis is related to local factors. When the colon is partially or completely removed, several conditions change: in the first place, the distance between the ileum and the outside is shorter; furthermore the ileocecal valve and the lymphoid structures of the terminal ileum are missing; moreover the absorptive capacity of the bowel changes and so does the fecal content (different composition, number and consistency of the stools); and finally a phenomenon of disbacteriosis or bacterial overgrowth can contribute. All of these factors trigger an acute and chronic nonspecific inflammatory reaction that we have called UI. Of course, we must be careful not to confuse it with other diseases as infectious processes, backwash ileitis or Crohn disease. In our review, we have a case where the behavior of
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Ulcerative Ileitis after Proctocolectomy in Children
Conflict of Interest None.
6 Bell AJ, Price AB, Forbes A, Ciclitira PJ, Groves C, Nicholls RJ. Pre-
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References 1 Hallak A, Baratz M, Santo M, et al. Ileitis after colectomy for
ulcerative colitis or carcinoma. Gut 1994;35(3):373–376
14
2 Warren R, McKittrick LS. Ileostomy for ulcerative colitis; tech-
nique, complications, and management. Surg Gynecol Obstet 1951;93(5):555–567 3 Rogers AG, Bargen JA, Black BM. Chronic ulcerative colitis: early and late experiences of 124 patients with ileac stomas. Gastroenterology 1954;27(4):383–398 4 Lyons AS, Garlock JH. The complications of ileostomy. Surgery 1954;36(4):784–789 5 Thayer WR, Spiro HM. Ileitis after ileostomy: prestomal ileitis. Gastroenterology 1962;42(5):547–554
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pouch ileitis: a disease of the ileum in ulcerative colitis after restorative proctocolectomy. Colorectal Dis 2006;8(5):402–410 Slatter C, Girgis S, Huynh H, El-Matary W. Pre-pouch ileitis after colectomy in paediatric ulcerative colitis. Acta Paediatr 2008; 97(3):381–383 Iwata T, Yamamoto T, Umegae S, Matsumoto K. Pouchitis and prepouch ileitis developed after restorative proctocolectomy for ulcerative colitis: a case report. World J Gastroenterol 2007; 13(4):643–646 Ugolini G, Rosati G, Montroni I, et al. Prepouch ileitis, myth or reality? The first case with acute abdomen. Inflamm Bowel Dis 2010;16(1):12–14 McLaughlin SD, Clark SK, Bell AJ, Tekkis PP, Ciclitira PJ, Nicholls RJ. Incidence and short-term implications of prepouch ileitis following restorative proctocolectomy with ileal pouch-anal anastomosis for ulcerative colitis. Dis Colon Rectum 2009;52(5):879–883 McCready FJ, Bargen JA, Dockerty MB, et al. Involvement of the ileum in chronic ulcerative colitis. N Engl J Med 1949;240(4): 119–127 Abdelrazeq AS, Wilson TR, Leitch DL, Lund JN, Leveson SH. Ileitis in ulcerative colitis: is it a backwash? Dis Colon Rectum 2005;48(11): 2038–2046 McLaughlin SD, Clark SK, Bell AJ, Tekkis PP, Ciclitira PJ, Nicholls RJ. An open study of antibiotics for the treatment of pre-pouch ileitis following restorative proctocolectomy with ileal pouch-anal anastomosis. Aliment Pharmacol Ther 2009;29(1):69–74 McLaughlin SD, Clark SK, Roberts CH, et al. Extended spectrum beta-lactamase-producing bacteria and Clostridium difficile in patients with pouchitis. Aliment Pharmacol Ther 2010;32(5): 664–669 McLaughlin SD, Clark SK, Tekkis PP, Ciclitira PJ, Nicholls RJ. An open study of maintenance antibiotic therapy for chronic antibioticdependent pouchitis: efficacy, complications and outcome. Colorectal Dis 2011;13(4):438–444 Kuisma J, Järvinen H, Kahri A, Färkkilä M. Factors associated with disease activity of pouchitis after surgery for ulcerative colitis. Scand J Gastroenterol 2004;39(6):544–548
This article has been updated as per the erratum published on October 26, 2014; doi: 10.1055/s-0034-1394279. The author names have been corrected. The correct listing of author names is as follows: Alba Sánchez-Galán, Ane M. Andres, Beatriz Fernández-Caamaño, Alejandra Vilanova, Eva Dominguez, Ruben Ortiz, Gerardo Prieto, Manuel Lopez-Santamaria, and J. A. Tovar.
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the disease is typical of Crohn disease, although we were never able to demonstrate the presence of granulomas in biopsies or surgical specimens. Perianal activity was so intense that forced to resect the pulled through ileum with diversion. The reasons why the UI occurs only after a few colectomies and is not expressed with the same intensity in all patients are unknown. What we know is that like pouchitis, it responds poorly to treatments applied.1,6,10,14,15 Only bowel diversion resolved the symptoms in our series. However, it is clear that an ileostomy is not the best treatment or the preferred by the patient, family, and surgeon; moreover ileostomy involves other complications and morbidities. Therefore, it is necessary to further investigate this condition and its pathogenesis to offer better treatment options in the future.
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