Chronic Intestinal Pseudo-Obstruction Associated with Type A Intestinal Neuronal Dysplasia
DOI:
https://doi.org/10.52787/agl.v53i3.317Keywords:
Pseudo-obstruction, aorto-mesenteric compass syndrome, Wilkie syndrome, intestinal neuronal dysplasia, hypo-ganglionosisAbstract
Introduction. Chronic intestinal pseudo-obstruction is a clinical entity with multiple causes. One of them is intestinal neuronal dysplasia, a rare disease with two subtypes: A and B.
Objective. To present a clinical case of a patient diagnosed with idiopathic gastroparesis associated with type A intestinal neuronal dysplasia.
Clinical case. A 30-year-old female patient with a diagnosis of idiopathic gastroparesis of a long evolution, with multiple hospitalizations and significant weight loss. She developed a aorto-mesenteric compass syndrome that was treated surgically with partial and temporary improvement. A jejunostomy was performed for feeding when symptoms recurred. During this surgical procedure, gastric and jejunal biopsies were performed and a diagnosis of intestinal neuronal dysplasia type A was made.
Conclusion. In the presence of symptoms of intestinal pseudo-obstruction or a diagnosis of idiopathic gastroparesis, it is necessary to consider the involvement of the gastrointestinal plexus. Obtaining an adequate tissue sample and evaluating it with appropriate techniques is of paramount importance to diagnosing the neurogenic etiology of the entity.
References
-1. Egal A, Jouët P, Lamarque D. Pseudo-obstruction intestinale chronique de l’adulte. Rev Med Internet. 2018;39(10):792-799. DOI: 10.1016/j.revmed.2018.03.010
-2. Rajalakshmi, T., Makhija, P., Babu, M.K. et al. Intestinal neuronal dysplasia type A. Indian J Pediatr. 2003;70,839-841. https://doi.org/10.1007/BF02723812
-3. Terra SA, Gonçalves AC, Lourenção PLTA, Rodrigues MAM. Challenges in the diagnosis of intestinal neuronal dysplasia type B: A look beyond the number of ganglion cells. World J Gastroenterol. 2021;27(44):7649-7660. DOI: 10.3748/wjg.v27.i44.7649
-4. Terra SA, de Arruda Lourenção PL, G Silva M, A Miot H, Rodrigues MAM. A critical appraisal of the morphological criteria for diagnosing intestinal neuronal dysplasia type B. Mod Pathol. 2017;30(7):978-985. DOI: 10.1038/modpathol.2017.4
-5. Cendón G, López M, Jimenez M, Alonso L, Asencio De A. Displasia neuronal intestinal asociada a otras malfomaciones congénitas. Cir. Pediátr. 2007;20(3). https://pesquisa.bvsalud.org/portal/resource/pt/ibc-056264
-6. Salas S, E. Jiménez T, JP. Manejo del Síndrome de Arteria Mesentérica Superior, Current Treatment of Superior Artery Mesenteric Syndrome. ciencia y salud [internet]. 2021;4(6) https://doi.org/10.34192/cienciaysalud.v4i6.227
-7. Galiano-Gil J, Hernández-Ortíz Y, Oharriz-Gallo Á, Hernández-Gil R. Síndrome de Wilkie. Revista Cubana de Cirugía. 2022;61(1). http://www.revcirugia.sld.cu/index.php/cir/article/view/1112
-8. García-Frade-Ruiz LF, Mundo-Gallardo LF, Solís-Ayala E. Síndrome de Wilkie. Med. interna Méx. 2017;33(2):254-258.
-9. Boschini Paéz M, Vargas Hernández MP, Miranda Solis L. Enfermedad de Hirschsprung: actualización en diagnóstico y tratamiento. Rev.méd.sinerg.2020;5(7):e406.
-10. Liu YR, Ba F, Cheng LJ, Li X, Zhang SW, Zhang SC. Efficacy of Sox10 Promoter Methylation in the Diagnosis of Intestinal Neuronal Dysplasia From the Peripheral Blood. Clin Transl Gastroenterol. 2019;10(12):e00093. DOI: 10.14309/ctg.0000000000000093. PMID: 31789936; PMCID: PMC6970557.
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Copyright (c) 2023 Josué Vázquez Arizmendi, Ulises Palacios Morejón, Brenda Martínez Luna, Carlos Domínguez Alvarez, Orlando Zamora Santana, Daniela Ariza Acuña
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