Chronic intestinal pseudo-obstruction in children (review)
DOI:
https://doi.org/10.15574/PS.2024.82.72Keywords:
children, chronic intestinal pseudo-obstruction, diagnostic, treatmentAbstract
Hirschsprung disease and chronic intestinal pseudo-obstruction (CIPO) in children are the most common and severe disorders of gastro-intestinal tract motility. While the problems connected with the Hirschsprung disease widely coverage in native literature, the publications that devoted the CIPO absent.
Aim of the study was to analyze the literature data that devoted to the diagnosis and treatment of children with CIPO.
Intestinal pseudo-obstruction refers to heterogeneous groups of disorders with a similar phenotypic presentation characterized by obstructive intestinal symptoms in the absence of true anatomical obstruction. CIPO classification based on the primary dysfunction: affected enteral nervous system (neuropathy), affected smooth muscle (myopathy) or interstitial cells Cajal (mesenchymopathy). Besides that, the intestinal pseudo-obstruction is divided into primary and secondary, congenital and acquired, total and segmentary.
The main clinical features, methods of investigation in suspected CIPO such as radiological (roentgenography and roentgenoscopy, computed tomography, cine magnetic resonance), endoscopic, manometry, pathohistological, etc. were listed.
The main principles of nutrition support, conventional therapy, and surgical management were presented.
Conclusions. CIPO is a severe disorder of gastrointestinal motility that caused by various factors, which can be congenital and/or acquired. Diagnosis of CIPO based on the results of clinical, instrumental, and pathohistological investigation. The management of children with CIPO should include nutritional support, conventional therapy, and, for the clear indices, surgical correction.
No conflict of interests was declared by the authors.
References
Ahlfors F, Linander H, Lindstrom M et al. (2011). Familial intestinal degenerative neuropathy associated with chronic intestinal pseudo-obstruction. Neurogastroenterol Motil. 23(4): 347-355. e159. https://doi.org/10.1111/j.1365-2982.2010.01638.x; PMid:21122031
Amiot A, Cazals-Hatem D, Joly F et al. (2009). The role of immunohistochemistry in idiopathic chronic intestinal pseudoobstruction (CIPO): a case-control study. Am J Surg Pathol. 33(5): 749-758. https://doi.org/10.1097/PAS.0b013e31819b381a; PMid:19252433
Amiot A, Joly F, Alves A, et al. (2009). Long-term outcome of chronic intestinal pseudo-obstruction adult patients requiring home parenteral nutrition. Am J Gastroenterol. 104(5): 1262-1270. https://doi.org/10.1038/ajg.2009.58; PMid:19367271
Andersson S, Lönroth H, Simren M, et al. (2006). Gastric electrical stimulation for intractable vomiting in patients with chronic intestinal pseudoobstruction. Neurogastroenterol Motil. 18(9): 823-830. https://doi.org/10.1111/j.1365-2982.2006.00801.x; PMid:16918761
Ashworth M, Rampling D. (2015). Paediatric gastrointestinal motility disorders. Diagnostic Histopathology. 21(6): 223-231. https://doi.org/10.1016/j.mpdhp.2015.06.008
Bagwell CE, Filler RM, Cutz E et al. (1984). Neonatal intestinal pseudoobstruction. J Pediatr Surg. 19(6): 732-739. https://doi.org/10.1016/S0022-3468(84)80360-7; PMid:6440967
Billiauws L, Cohen M, Cazals-Hatem D, Joly F. (2022). Small intestine motility disorders: Chronic intestinal pseudo-obstruction. J Visc Surg. 159 (1): S22-S27. https://doi.org/10.1016/j.jviscsurg.2022.01.001; PMid:35131150
Chanpong A, Borrelli O, Thapar N. (2022). Hirschsprung disease and paediatric intestinal pseudo-obstruction. Best Pract Res Clin Gastroenterol. 56-57: 101765. https://doi.org/10.1016/j.bpg.2021.101765; PMid:35331399
Chini P, Toskes PP, Waseem S, et al. (2012). Effect of azithromycin on small bowel motility in patients with gastrointestinal dysmotility. Scand J Gastroenterol. 47(4): 422-427. https://doi.org/10.3109/00365521.2012.654402; PMid:22364597
Cogliandro RF, De Giorgio R, Barbara G, et al. (2007). Chronic intestinal pseudo-obstruction. Best Pract Res Clin Gastroenterol. 21(4): 657-669. https://doi.org/10.1016/j.bpg.2007.03.002; PMid:17643907
Coletta R, Khalil BA, Morabito A. (2014). Short bowel syndrome in children: surgical and medical perspectives. Semin Pediatr Surg. 23(5): 291-297. https://doi.org/10.1053/j.sempedsurg.2014.09.010; PMid:25459014
Connor FL, Di Lorenzo C. (2006). Chronic intestinal pseudo-obstruction: assessment and management. Gastroenterology. 130 (2; 1): S29-S36. https://doi.org/10.1053/j.gastro.2005.06.081; PMid:16473068
Cserni T, Biszku B, Guthy I et al. (2014). The first clinical application of the spiral intestinal lengthening and tailoring (Silt) in extreme short bowel syndrome. J Gastrointest Surg. 18 (10): 1852-1857. https://doi.org/10.1007/s11605-014-2577-2; PMid:24957255
Cucchiara S, Borrelli O. (2009). Nutritional challenge in pseudo-obstruction: the bridge between motility and nutrition. J Pediatr Gastroenterol Nutr. 48 (2): S83-S85. https://doi.org/10.1097/MPG.0b013e3181a15bfe
D'Antiga L, Goulet O. (2013). Intestinal failure in children: the European view. J Pediatr Gastroenterol Nutr. 56(2): 118-126. https://doi.org/10.1097/MPG.0b013e318268a9e3; PMid:22820123
De Giorgio R, Cogliandro RF, Barbara G et al. (2011). Chronic intestinal pseudo-obstruction: clinical features, diagnosis, and therapy. Gastroenterol Clin North Am. 40(4): 787-807. https://doi.org/10.1016/j.gtc.2011.09.005; PMid:22100118
De Giorgio, Guerrini S, Barbara G et al. (2004). Inflammatory neuropathies of the enteric nervous system. Gastroenterology. 126(7): 1872-1883. https://doi.org/10.1053/j.gastro.2004.02.024; PMid:15188182
Di Lorenzo C. (1999). Pseudo-obstruction: current approaches. Gastroenterology. 116(4): 980-987. https://doi.org/10.1016/S0016-5085(99)70082-X; PMid:10092321
Di Lorenzo C. (2005). Surgery in intestinal pseudo-obstruction: pro. J Pediatr Gastroenterol Nutr. 41 (1): S64-S65. https://doi.org/10.1097/01.scs.0000180311.55417.55
Di Lorenzo C, Flores AF, Buie T, Hyman PE. (1995). Intestinal motility and jejunal feeding in children with chronic intestinal pseudo-obstruction. Gastroenterology. 108(5):1379-1385. https://doi.org/10.1016/0016-5085(95)90685-1; PMid:7729629
Di Lorenzo C, Lucanto C, Flores AF et al. (1999). Effect of sequential erythromycin and octreotide on antroduodenal manometry. J Pediatr Gastroenterol Nutr. 29(3): 293-296. https://doi.org/10.1097/00005176-199909000-00010; PMid:10467994
Di Lorenzo C, Youssef NN. (2010). Diagnosis and management of intestinal motility disorders. Semin Pediatr Surg. 19(1): 50-58. https://doi.org/10.1053/j.sempedsurg.2009.11.006; PMid:20123274
Di Nardo G, Di Lorenzo C, Lauro A et al. (2017). Chronic intestinal pseudo-obstruction in children and adults: diagnosis and therapeutic options. Neurogastroenterol Motil. 29: 1. https://doi.org/10.1111/nmo.12945; PMid:27683196
Di Nardo G, Karunaratne TB, Frediani S, De Giorgio R. (2017). Chronic intestinal pseudo-obstruction: progress in management? Neurogastroenterol Motil. 29: 12. https://doi.org/10.1111/nmo.13231; PMid:29143474
Dudley HA, Sinclair IS, McLaren IF et al. (1958). Intestinal pseudo-obstruction. J R Coll Surg Edinb. 3(3): 206-217.
El-Chammas K, Sood MR. (2018). Chronic intestinal pseudo-obstruction. Clin Colon Rectal Surg. 31(2): 99-107. https://doi.org/10.1055/s-0037-1609024; PMid:29487492 PMCid:PMC5825855
Faulk DL, Anuras S, Christensen J. (1978). Chronic intestinal pseudoobstruction. Gastroenterology. 74(5; 1): 922-931. https://doi.org/10.1016/0016-5085(78)90156-7; PMid:346432
Faure C, Goulet O, Ategbo S et al. (1999). Chronic intestinal pseudoobstruction syndrome: clinical analysis, outcome, and prognosis in 105 children. French-Speaking Group of Pediatric Gastroenterology. Dig Dis Sci. 44(5): 953-959. https://doi.org/10.1023/A:1026656513463; PMid:10235603
Fell JM, Smith VV, Milla PJ. (1996). Infantile chronic idiopathic intestinal pseudo-obstruction: the role of small intestinal manometry as a diagnostic tool and prognostic indicator. Gut. 39(2): 306-311. https://doi.org/10.1136/gut.39.2.306; PMid:8977348 PMCid:PMC1383316
Gariepy CE, Mousa H. (2009). Clinical management of motility disorders in children. Semin Pediatr Surg. 18(4): 224-238. https://doi.org/10.1053/j.sempedsurg.2009.07.004; PMid:19782304
Goulet O, Sauvat F, Jan D. (2005). Surgery for pediatric patients with chronic intestinal pseudo-obstruction syndrome. J Pediatr Gastroenterol Nutr. 41 (1): S66-S68. https://doi.org/10.1097/01.scs.0000180312.55417.8e
Granata C, Puri P. (1997). Megacystis-microcolon-intestinal hypoperistalsis syndrome. J Pediatr Gastroenterol Nutr. 25(1): 12-19. https://doi.org/10.1097/00005176-199707000-00002; PMid:9226521
Gugliemi FW, Boggio-Bertinet D, Federico A et al. (2006). Total parenteral nutrition-related gastroenterological complications. Dig Liver Dis. 38(9): 623-642. https://doi.org/10.1016/j.dld.2006.04.002; PMid:16766237
Hashizume N, Yagi M, Ushijima K et al. (2017). Pharmacotherapy for pediatric chronic intestinal pseudo-obstruction: Nationwide survey in Japan. Pediatr Int. 59(4):467-472. https://doi.org/10.1111/ped.13201; PMid:27862707
Heneyke S, Smith VV, Spitz L et al. (1998). Chronic intestinal pseudoobstruction: treatment and long term follow up of 44 patients. Arch Dis Child. 81(1): 21-27. https://doi.org/10.1136/adc.81.1.21; PMid:10373127 PMCid:PMC1717974
Hubball A, Martin JE, Lang B et al. (2009). The role of humoral autoimmunity in gastrointestinal neuromuscular diseases. Prog Neurobiol. 87(1): 10-20. https://doi.org/10.1016/j.pneurobio.2008.09.011; PMid:18929621
Hussain SZ, Di Lorenzo C. (2002). Motility disorders. Diagnosis and treatment for the pediatric patient. Pediatr Clin North Am. 49(1): 27-51. https://doi.org/10.1016/S0031-3955(03)00107-X; PMid:11826807
Hyman P. (2011). Chronic intestinal pseudo-obstruction. In: Pediatric gastrointestinal and liver disease. Wyllie R, Hyams J, Kay M, [Eds.]. Philadelphia: Elsevier: 505-511. https://doi.org/10.1016/B978-1-4377-0774-8.10046-6
Iida H, Ohkubo H, Inamori M et al. (2013). Epidemiology and clinical experience of chronic intestinal pseudo-obstruction in Japan: a nationwide epidemiologic survey. J Epidemiol. 23(4): 288-294. https://doi.org/10.2188/jea.JE20120173; PMid:23831693 PMCid:PMC3709546
Kapur RP. (2010). Pathology of intestinal motor disorders in children. Surg Pathol Clin. 3(3): 711-741. https://doi.org/10.1016/j.path.2010.06.005; PMid:26839228
Keller J, Bassotti G, Clarke J et al. (2018). Expert consensus document: advances in the diagnosis and classification of gastric and intestinal motility disorders. Nat Rev Gastroenterol Hepatol. 15(5): 291-308. https://doi.org/10.1038/nrgastro.2018.7; PMid:29622808 PMCid:PMC6646879
Kellow JE. (2002). Small intestine: normal function and clinical disorders. Manometry. In: Schuster atlas of gastrointestinal motility in health and disease. Schuster MM, Crowell MD, Koch KL, [Eds.]. Hamilton-London: BC Decker: 219-236.
Khalaf A, Nowak A, Menys A, et al. (2019). Cine MRI assessment of motility in the unprepared small bowel in the fasting and fed state: Beyond the breath-hold. Neurogastroenterol Motil. 31(1): e13466. https://doi.org/10.1111/nmo.13466; PMid:30230099
Kim H-Y, Kim J-H, Jung S-E et al. (2005). Surgical treatment and prognosis of chronic intestinal pseudo-obstruction in children. J Pediatr Surg. 40(11): 1753-1759. https://doi.org/10.1016/j.jpedsurg.2005.07.046; PMid:16291165
Knowles CH, De Giorgio R, Kapur RP et al. (2010). The London Classification of gastrointestinal neuromuscular pathology: report on behalf of the Gastro 2009 International Working Group. Gut. 59(7): 882-887. https://doi.org/10.1136/gut.2009.200444; PMid:20581236
Ko D, Yang HB, Youn J, Kim HY. (2021). Clinical outcomes of pediatric chronic intestinal pseudo-obstruction. J Clin Med. 10(11): 2376. https://doi.org/10.3390/jcm10112376; PMid:34071279 PMCid:PMC8198288
Lapointe R. (2010). Chronic idiopathic intestinal pseudo-obstruction treated by near total small bowel resection: a 20-year experience. J Gastrointest Surg. 14(12): 1937-1942. https://doi.org/10.1007/s11605-010-1295-7; PMid:20872085
Lee JW, Bang KW, Jang PS et al. (2010). Neostigmine for the treatment of acute colonic pseudo-obstruction (ACPO) in pediatric hematologic malignancies. Korean J Hematol. 45(1): 62-65. https://doi.org/10.5045/kjh.2010.45.1.62; PMid:21120165 PMCid:PMC2983008
Lindberg G, Iwarzon M, Tornblom H. (2009). Clinical features and long-term survival in chronic intestinal pseudo-obstruction and enteric dysmotility. Scand J Gastroenterol. 44(6): 692-699. https://doi.org/10.1080/00365520902839642; PMid:19308797
Lindberg G, Tornblom H, Iwarzon M et al. (2009). Full-thickness biopsy fifindings in chronic intestinal pseudo-obstruction and enteric dysmotility. Gut. 58(8): 1084-1090. https://doi.org/10.1136/gut.2008.148296; PMid:19136514
Lu W, Xiao Y, Huang J et al. (2018). Causes and prognosis of chronic intestinal pseudo-obstruction in 48 subjects: a 10-year retrospective case series. Medicine (Baltim). 97(36): e12150-e. https://doi.org/10.1097/MD.0000000000012150; PMid:30200110 PMCid:PMC6133590
Lyford G, Foxx-Orenstein A. (2004). Chronic intestinal pseudo-obstruction. Curr Treat Options Gastroenterol. 7(4): 317-325. https://doi.org/10.1007/s11938-004-0018-0; PMid:15238207
Mallick S, Prasenjit D, Prateek K et al. (2014). Chronic intestinal pseudoobstruction: systematic histopathological approach can clinch vital clues. Virchows Arch. 464(5): 529-537. https://doi.org/10.1007/s00428-014-1565-y; PMid:24663670
Merlin A, Soyer P, Boudiaf M et al. (2008). Chronic intestinal pseudo-obstruction in adult patients: multidetector row helical CT features. Eur Radiol. 18(8): 1587-1595. https://doi.org/10.1007/s00330-008-0913-1; PMid:18357454
Mousa H, Hyman PE, Cocjin J et al. (2002). Long-term outcome of congenital intestinal pseudoobstruction. Dig Dis Sci. 47(10): 2298-2305. https://doi.org/10.1023/A:1020199614102; PMid:12395903
Muto M, Matsufuji H, Tomomas T et al. (2014). Pediatric chronic intestinal pseudo-obstruction is a rare, serious, and intractable disease: a report of a nationwide survey in Japan. J Pediatr Surg. 49(12): 1799-1803. https://doi.org/10.1016/j.jpedsurg.2014.09.025; PMid:25487487
Ohkubo H, Kessoku T, Fuyuki A et al. (2013). Assessment of small bowel motility in patients with chronic intestinal pseudo-obstruction using cine-MRI. Am J Gastroenterol. 108(7): 1130-1139. https://doi.org/10.1038/ajg.2013.57; PMid:23511458
Pakarinen MP, Koivusalo AI, Rintala RJ. (2009). Outcomes of intestinal failure - a comparison between children with short bowel and dysmotile intestine. J Pediatr Surg. 44(11): 2139-2144. https://doi.org/10.1016/j.jpedsurg.2009.05.002; PMid:19944223
Pakarinen MP, Kurvinen A, Koivusalo AI et al. (2013). Surgical treatment and outcomes of severe pediatric intestinal motility disorders requiring parenteral nutrition. J Pediatr Surg. 48(2): 333-338. https://doi.org/10.1016/j.jpedsurg.2012.11.010; PMid:23414861
Pimentel M. (2009). Review of rifaximin as treatment for SIBO and IBS. Expert Opin Investig Drugs. 18(3): 349-358. https://doi.org/10.1517/13543780902780175; PMid:19243285
Prapaitrakool S, Hollmann MW, Wartenberg HC et al. (2012). Use of buprenorphine in children with chronic pseudoobstruction syndrome: case series and review of literature. Clin J Pain. 28(8): 722-725. https://doi.org/10.1097/AJP.0b013e318243f609; PMid:22688601
Puri P, Gosemann JH. (2012). Variants of Hirschsprung disease. Semin Pediatr Surg. 21(4): 310-318. https://doi.org/10.1053/j.sempedsurg.2012.07.005; PMid:22985836
Raphael BP, Nurko S, Jiang H, et al. (2011). Cisapride improves enteral tolerance in pediatric short-bowel syndrome with dysmotility. Pediatr Gastroenterol Nutr. 52(5): 590-594. https://doi.org/10.1097/MPG.0b013e3181fe2d7a; PMid:21502831 PMCid:PMC3079853
Rosen R, Garza JM, Tipnis N, Nurko S. (2018). An ANMS-NASPGHAN consensus document on esophageal and antroduodenal manometry in children. Neuro Gastroenterol Motil. 30(3): 10.1111/nmo.13239. https://doi.org/10.1111/nmo.13239; PMid:29178261 PMCid:PMC5823717
Rosner H, Rubin L, Kestenbaum A. (1996). Gabapentin adjunctive therapy in neuropathic pain states. Clin J Pain. 12(1): 56-58. https://doi.org/10.1097/00002508-199603000-00010; PMid:8722736
Rudolph CD, Hyman PE, Altschuler SM et al. (1997). Diagnosis and treatment of chronic intestinal pseudo-obstruction in children: report of consensus workshop. J Pediatr Gastroenterol Nutr. 24(1): 102-112. https://doi.org/10.1097/00005176-199701000-00021; PMid:9093995
Sogawa H, Costa G, Armanyous S et al. (2021). Twenty years of gut transplantation for chronic intestinal pseudo-obstruction: technical innovation, long-term outcome, quality of life, and disease recurrence. Ann Surg. 273 (2): 325-333. https://doi.org/10.1097/SLA.0000000000003265; PMid:31274659
Sorhaug S, Steinshamn SL, Waldum HL. (2005). Octreotide treatment for paraneoplastic intestinal pseudo-obstruction complicating SCLC. Lung Cancer. 48(1): 137-140. https://doi.org/10.1016/j.lungcan.2004.09.008; PMid:15777981
Stanghellini V, Cogliandro RF, De Giorgio R et al. (2007). Chronic intestinal pseudo-obstruction: manifestations, natural history and management. Neurogastroenterol Motil. 19(6): 440-452. https://doi.org/10.1111/j.1365-2982.2007.00902.x; PMid:17564625
Stanghellini V, Cogliandro RF, De Giorgio R et al. (2005). Natural history of chronic idiopathic intestinal pseudo-obstruction in adults: a single center study. Clin Gastroent Hepatol. 3(5): 449-458. https://doi.org/10.1016/S1542-3565(04)00675-5; PMid:15880314
Stanley JD, Bartlett JG, Dart BW, Ashcraft JH. (2013). Clostridium diffificile infection. Curr Probl Surg. 50(7): 302-337. https://doi.org/10.1067/j.cpsurg.2013.02.004; PMid:23764494
Sumiyama K, Gostout CJ. (2011). Clinical applications of submucosal endoscopy. Curr Opin Gastroenterol. 27(5): 412-417. https://doi.org/10.1097/MOG.0b013e328349cf8e; PMid:21844750
Tanaka K, Ohkubo H, Yamamoto A et al. (2023). Natural history of chronic intestinal pseudo-obstruction and need for palliative care. J Neurogastroenterol Motil. 29(3): 378-387. https://doi.org/10.5056/jnm22152; PMid:37417265 PMCid:PMC10334206
Teich S, Mousa HM, Punati J et al. (2013). Effificacy of permanent gastric electrical stimulation for the treatment of gastroparesis and functional dyspepsia in children and adolescents. J Pediatr Surg. 48 (1): 178-183. https://doi.org/10.1016/j.jpedsurg.2012.10.038; PMid:23331812
Thapar N, Borrelli O. (2018). Anorectal manometry for the diagnosis of Hirschsprung disease: new heights for the balloon or just hot air? J Pediatr Gastroenterol Nutr. 67(3): 311-312. https://doi.org/10.1097/MPG.0000000000002076; PMid:29927866
Thapar N, Saliakellis E, Benninga MA et al. (2018). Paediatric intestinal pseudo-obstruction: evidence and consensus-based recommendations from an ESPGHAN-led expert group. J Pediatric Gastroenterol Nutrit. 66(6): 991-1019. https://doi.org/10.1097/MPG.0000000000001982; PMid:29570554
Trimarchi R, Visalli C, Quartararo C et al. (2021). Radiological evaluation of a case of chronic intestinal pseudo-obstruction (CIPO). Radiol Case Rep. 16(3): 651-655. https://doi.org/10.1016/j.radcr.2020.12.061; PMid:33488892 PMCid:PMC7806544
Vasile I, Vilcea D, Mogos D et al. (2003). Functional secondary megaduodenum. Chirurgia (Bucur). 98(6): 553-560.
Viti F, De Giorgio R, Ceccherini I et al. (2023). Multi-disciplinary insights from the First European Forum on Visceral Myopathy 2022 Meeting. Dig Dis Sci. 68(10): 3857-3871. https://doi.org/10.1007/s10620-023-08066-1; PMid:37650948 PMCid:PMC10517037
Yadak R, Breur M, Bugiani M. (2019). Gastrointestinal dysmotility in MNGIE: from thymidine phosphorylase enzyme deficiency to altered interstitial cells of Cajal. Orphanet J Rare Dis. 14(1): 33. https://doi.org/10.1186/s13023-019-1016-6; PMid:30736844 PMCid:PMC6368792
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