Chronic Megacolon in Young Patients: Epidemiological, Clinical, and Manometric Characteristics
DOI:
https://doi.org/10.52787/agl.v52i3.177Keywords:
Megacolon, idiopathic, young patientsAbstract
Introduction. Chronic megacolon manifests clinically as a motility disorder. In the vast majority of cases it causes constipation that is hard to treat. Based on its etiology, it can be congenital, acquired or idiopathic. Objective. To describe the epidemiological, clinical and manometric characteristics in a population under 40 years old with chronic megacolon and chronic constipation.
Materials and methods. Descriptive cross-sectional study. 23 patients treated at Hospital de Gastroenterología Dr. Bonorino Udaondo from March 2019 to March 2021 were enrolled in the study. The diagnosis of chronic megacolon was made by barium enema, and the diagnosis of chronic constipation was based on Rome IV criteria. Epidemiological and clinical data were collected. Anorectal functional outcomes were obtained by conventional manometry. In all cases, medical treatment for constipation was indicated.
Results. Thirteen female patients, median age: 21 (19-30); age at diagnosis: 16 (13-19). 7 patients (30.4%) had surgery. Causes: 14/23 idiopathic (60.9%), 6/23 probable Hirschsprung disease (2 patients with diagnosis confirmed by biopsy) (26.1%), and 3/23 secondary (13%). Extent: 6/21 megasigmoid (28.6%), 5/21 megarectum (23.8%), and 10/21 generalized (47.6%). Manometric data allowed to identify a prevalence of pelvic floor dyssynergia (14/22 patients; 64%), and rectal hyposensitivity (13/22 patients; 59%). Five patients (21.7%) showed no rectoanal inhibitory reflex. Complications: volvulus, 7/23 patients (30.4%), and fecaloma, 12/23 patients (52.2%). Treatment: 14/23 polyethylene glycol (66.7%), 5/23 linaclotide (23.8%), 13/23 prucalopride (61.9%), 7/23 Murphy drips (31.8%), and 18/23 combined treatments (81.8%). The risk of fecaloma was higher in patients with megarectum than in patients with megacolon (5/5 vs. 3/10) respectively [p < 0.03]). Hyposensitivity and dyssynergia were not associated with risk of fecaloma (p=NS). At the time of this study, only one patient will undergo surgery because she is not responding to medical treatment.
Conclusions. All patients showed at least one abnormal result in the manometry. More than half of the patients showed at least one fecalom.
References
-1. O'Dwyer RH, Acosta A, Camilleri M, Burton D, Busciglio I, Bharucha AE. Clinical Features and Colonic Motor Disturbances in Chronic Megacolon in Adults. Digestive Disease and Sciences 2015;60:2398-2407.
-2. Martucciello G, Pini Prato A, Puri P et al. Controversies concerning diagnostic guidelines for anomalies of the enteric nervous system: a report from the fourth International Symposium on Hirschsprung's disease and related neurocristopathies. J PediatrSurg 2005;40:1527-1531.
-3. Amiel J Sarnacki S et al. Hirschsprung disease, associated syndromes and genetics: a review. J Med Genet 2008;45:1-14.
-4. Holschneider A. M, Puri P. Hirschsprung´s Disease and Allied Disorders. Springer, 2008. https://drive.google.com/open?id=-1fI-_6B-crgTSvxqi_gK5cBYud7WBiiP5
-5. Enfermedades infecciosas. Chagas. Guía para el equipo de salud. Atención del paciente con enfermedad de Chagas. Ministerio de Salud de la Nación, 2006.
-6. Ohkubo H, Masaki T, Matsuhashi N, Kawahara H, Yokoyama T, Nakajima A, Ohkura Y. Histopathologic findings in patients with idiopathic megacolon: a comparison between dilated and non-dilated loops. Neurogastroenterol Motil 2014;26:571-580.
-7. Constantin, A.; Achim, F.; Spinu, D.; Socea, B.; Predescu, D. Idiopathic Megacolon-Short Review. Diagnostics 2021,11,2112. https://doi.org/10.3390/diagnostics11112112
-8. Graziano a y Ramirez Rojas; Megacolon. Cirugía Digestiva, F. Galindo. www.sacd.org.ar, 2009
-9. Marc A. Gladman, MRCOG, MRCS (Eng), S. Mark Scott, PhD, Peter J. Lunniss, MS, FRCS, and Norman S. Williams, MS, FRCS, FMedSci Systematic Review of Surgical Options for Idiopathic Megarectum and Megacolon. Ann Surg 2005;241:562-574.
-10. Lacy BE, Mearín F, Chang L, Chey WD, Lembo AJ, Simren M, Spiller R. Bowel Disorders. Gastroenterology 2016;150:1393-1407.
-11. Karim A, Tang CS and Tam PKH (2021) The Emerging Genetic Landscape of Hirschsprung Disease and Its Potential Clinical Applications. Front. Pediatr. 9:638093. DOI: 10.3389/fped.2021.638093
-12. Wang XJ, Camilleri M. Chronic Megacolon Presenting in Adolescents or Adults: Clinical Manifestations, Diagnosis, and Genetic Associations. Dig Dis Sci. 2019;64(10):2750-2756. DOI: 10.1007/s10620-019-05605-7
-13. Ehrenpreis T. Megacolon and megarectum in older children and young adults. Classification and terminology. Proc R Soc Med. 1967;60(8):799-801.
-14. Lombana LJ, Domínguez LC Cirugía en la enfermedad de Hirschsprung del adulto. Rev Col Gastroenterol 2007;22:231-237.
Published
How to Cite
Issue
Section
License
Copyright (c) 2022 Daniela Milito, Juan Pablo Stefanolo, Irina Riccobene, Agostina Pascual Russo, Marina Furia, Vanina Forestier, María Paz Lacuadra, Miguel Lumi, Horacio Vázquez, Lucila Facio
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.
