Hirschprung`s disease, a practical approach.

Main Article Content

Nicolás Zuluaga Arbeláez
Santiago Posada

Abstract

Initially, Hirschsprung's disease was


Hirschsprung's disease was described by the Danish pediatrician Harald Hirschsprung in 1888. It is the most common congenital enteric neuropathy, with a lack of relaxation of the enteric muscle, with subsequent intestinal obstruction. Its etiopathogenesis is given by aganglionosis in the digestive tract, mainly affecting the rectosigmoid region. Thus, keeping the internal anal sphincter contracted. It is classified into four types: short segment, the most common; long segment, ultrashort segment, and aganglionosis coli. The clinical presentation frequently begins in the neonatal period. The classic triad consists of abdominal distention, absence of meconium in the first 24 to 48 hours of life, and bilious vomiting with intolerance to the oral route. The diagnostic approach is performed by abdominal X-rays, contrast enema, or rectal manometry. The gold standard test is the rectal biopsy, which is considered positive if there are no ganglion cells in the myenteric and submucosal plexus of the colon. The initial management of the disease is based on intravenous fluid resuscitation, broad-spectrum antibiotics to prevent enterocolitis and bacterial translocation, decompression with a nasogastric tube, and colonic lavage. The definitive treatment is colonic resection of the affected segment. The main postoperative complications are perianal excoriation, constipation, dirt, diarrhea, fecal incontinence, and enterocolitis.

Downloads

Download data is not yet available.

Article Details

How to Cite
Zuluaga Arbeláez, N., & Posada, S. (2022). Hirschprung`s disease, a practical approach. Pediatría, 55(2), 91–97. https://doi.org/10.14295/rp.v55i2.312
Section
Review topics

References

Schäppi MG, Staiano A, Milla PJ, Smith VV, Dias JA, Heuschkel R, et al. A Practical Guide for the Diagnosis of Primary Enteric Nervous System Disorders: J Pediatr Gastroenterol Nutr. noviembre de 2013;57(5):677-86. DOI: https://doi.org/10.1097/MPG.0b013e3182a8bb50

Tabbers MM, DiLorenzo C, Berger MY, Faure C, Langendam MW, Nurko S, et al. Evaluation and treatment of functional constipation in infants and children: evidence-based recommendations from ESPGHAN and NASPGHAN. J Pediatr Gastroenterol Nutr. 2014;58(2):258–74. DOI: https://doi.org/10.1097/MPG.0000000000000266

Moore SW. Advances in understanding functional variations in the Hirschsprung disease spectrum (variant Hirschsprung disease). Pediatr Surg Int. 2017;33(3):285-98. DOI: https://doi.org/10.1007/s00383-016-4038-3

Torre-Mondragón L. Enfermedad de Hirschsprung. Mitos y realidades a 120 años de su descripción. Acta Pediatr Mex. 2008;29(3):139-146.

Carro Gabriela, Ormaechea Martín, Silva Ema Da, Juambeltz Carlos. Enfermedad de Hirschsprung: resultados del tratamiento quirúrgico en el Hospital Pediátrico Pereira Rossell. Arch. Pediatr. Urug. 89( 3 ): 158-164.

Sajjad N, Hilal K, Khandwala K, Arshad M, Uddin N. Usefulness of delayed films of contrast enema for detecting Hirschsprung’s disease. Cureus. 2019;11(12):e6339. DOI: https://doi.org/10.7759/cureus.6339

Butler Tjaden NE, Trainor PA. The developmental etiology and pathogenesis of Hirschsprung disease. Transl Res. 2013;162(1):1-15. DOI: https://doi.org/10.1016/j.trsl.2013.03.001

Amiel J, Lyonnet S. Hirschsprung disease, associated syndromes, and genetics: a review. J Med Genet. 2001;38(11):729–39. DOI: https://doi.org/10.1136/jmg.38.11.729

Kroll-Wheeler L, Wilson AM. Educational Case: Hirschsprung Disease. Acad Pathol. 1 de enero de 2019;6:237428951989308. DOI: https://doi.org/10.1177/2374289519893088

Hackam D, Grikscheit T, Wang K, Upperman J, Ford H. Chapter 39: Pediatric Surgery. In: Andersen D, Dunn D, Hunter J, ed. by. Schwartz’s principles of surgery 11th ed. Mc Gram Hill; 2019.

Moore SW. Total colonic aganglionosis in Hirschsprung disease. Semin Pediatr Surg. 2012;21(4):302-9. DOI: https://doi.org/10.1053/j.sempedsurg.2012.07.004

Raveenthiran V. Knowledge of ancient Hindu surgeons on Hirschsprung disease: evidence from Sushruta Samhita of circa 1200-600 bc. J Pediatr Surg. 2011;46(11):2204-8. DOI: https://doi.org/10.1016/j.jpedsurg.2011.07.007

Das K, Mohanty S. Hirschsprung Disease — Current Diagnosis and Management. Indian J Pediatr. 2017;84(8):618-23. DOI: https://doi.org/10.1007/s12098-017-2371-8

Mc Laughlin D, Puri P. Familial hirschsprung’s disease: a systematic review. Pediatr Surg Int. 2015;31(8):695-700. DOI: https://doi.org/10.1007/s00383-015-3730-z

Moore SW. Advances in understanding the association between Down syndrome and Hirschsprung disease (DS–HSCR). Pediatr Surg Int. 2018;34(11):1127-37. DOI: https://doi.org/10.1007/s00383-018-4344-z

Villota D VA, Saldarriaga G W, Gómez C JF. Síndrome de Mowat-Wilson: caso clínico. Rev Chil Pediatría. 2012;83(4):371-6. DOI: https://doi.org/10.4067/S0370-41062012000400008

Coyle D, Puri P. Hirschsprung’s disease in children with Mowat–Wilson syndrome. Pediatr Surg Int. 2015;31(8):711-7. DOI: https://doi.org/10.1007/s00383-015-3732-x

Moore SW, Maluleke T, El Hosny AA. Is Hirschsprung disease a purely neurological condition? A study of the Actin G2 smooth muscle gene in Hirschsprung disease. J Pediatr Surg. 2019;54(10):2028-31. DOI: https://doi.org/10.1016/j.jpedsurg.2019.01.069

Jiménez, J. and Fernandez, L., n.d. Enfermedad de Hirschsprung. AEPED, pp.47-52.

Wetherill C, Sutcliffe J. Hirschsprung disease and anorectal malformation. Early Hum Dev. 2014;90(12):927-32. DOI: https://doi.org/10.1016/j.earlhumdev.2014.09.016

Muise ED, Cowles RA. Rectal biopsy for Hirschsprung’s disease: a review of techniques, pathology, and complications. World J Pediatr. 2016;12(2):135-41. DOI: https://doi.org/10.1007/s12519-015-0068-5

Tam PKH, Chung PHY, St Peter SD, Gayer CP, Ford HR, Tam GCH, et al. Advances in paediatric gastroenterology. The Lancet. 2017;390(10099):1072-82. DOI: https://doi.org/10.1016/S0140-6736(17)32284-5

Cathia Selman; Constanza Alzola Internas 7° año, Facultad de Medicina, Universidad de Chile. Enfermedad de Hirschprung: Avances en el diagnóstico. Rev. Ped. Elec. 2016;13(2):13-18.

Westfal ML, Goldstein AM. Pediatric enteric neuropathies: diagnosis and current management. Curr Opin Pediatr. 2017;29(3):347-53. DOI: https://doi.org/10.1097/MOP.0000000000000486

Massardo V Teresa, Jaimovich F Rodrigo, Rodríguez T J. Carlos, Azolas A Cristián, Saavedra S José, Lillo G Ricardo et al . Tromboembolismo pulmonar en lactante menor, portadora de enfermedad de Hirschsprung: caso clínico. Rev. chil. radiol. 2005;11(1): 32-35. DOI: https://doi.org/10.4067/S0717-93082005000100008

Alberto Peña. Enfermedad de Hirschsprung Los avances y las preguntas no contestadas. Cir. Pediatr. 2002; 15: 46-47.

Suita S, Taguchi T, Ieiri S, Nakatsuji T. Hirschsprung’s disease in Japan: analysis of 3852 patients based on a nationwide survey in 30 years. J Pediatr Surg. enero de 2005;40(1):197-202. DOI: https://doi.org/10.1016/j.jpedsurg.2004.09.052

PÉREZ BILLI LUIS E, BENEDICTTI JUAN L, GUTIÉRREZ CARMEN, GALIANA HORACIO GUTIÉRREZ. Enfermedad de Hirschsprung con afectación total del colon: primer caso nacional con la técnica quirúrgica de Lester Martin modificada. Arch. Pediatr. Urug. 2001;72(1): 34-37.

Nakamura H, Lim T, Puri P. Inflammatory bowel disease in patients with Hirschsprung’s disease: a systematic review and meta-analysis. Pediatr Surg Int. 2018;34(2):149-54. DOI: https://doi.org/10.1007/s00383-017-4182-4

Green HL, Rizzolo D, Austin M. Surgical management for Hirschsprung disease: A review for primary care providers. J Am Acad Physician Assist. 2016;29(4):249. DOI: https://doi.org/10.1097/01.JAA.0000481397.68475.41

Ciriza-de-los-Ríos, Constanza, Mínguez, Miguel, Remes-Troche, José-María, & Lacima, Glòria. Manometría anorrectal de alta resolución y de alta definición: redescubriendo la función anorrectal. Revista Española de Enfermedades Digestivas. 2018;110(12):794-805. DOI: https://doi.org/10.17235/reed.2018.5705/2018

Similar Articles

<< < 3 4 5 6 7 8 9 10 11 12 > >> 

You may also start an advanced similarity search for this article.