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2016, Number 620

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Rev Med Cos Cen 2016; 73 (620)

Enfermedad de hirschsprung: Diagnóstico y manejo en niños y adultos

Delgado MA
Full text How to cite this article

Language: Spanish
References: 10
Page: 687-691
PDF size: 154.02 Kb.


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ABSTRACT

Hirschsprung Disease (HD) or Congenital Megacolon is an anomaly that is characterized mainly for the absence of ganglion cells in the myenteric plexus and submucosal in a variable segment of the intestine. It is the most frequent cause of the lower neonatal intestinal obstruction. However the real cause isn´t established yet. The Congenital Megacolon presents agonglionosis in different lengths of the intestine; many times associated to other congenital anomalies. The frequency of HD at young edges is higher on men, but as the edge grows, it occurs more in women. The clinical presentation changes between children and adults. In the pediatric population a constipation history since the first month of life, the delay on the elimination of meconium beyond the 24 hours, abdominal distension is a suspicion of Congenital Megacolon. The adults consult for a chronic constipation history, abdominal distension, use of laxatives and enemas since childhood. Hirschsprung disease is certainly diagnosed after birth. The most important exam to diagnose this disease is the rectal biopsy of the complete stenotic segment, which shows the absence of ganglion cells, nerve hypertrophy presence and incresement of the activity of acetycholinesterase. Agonglinosis is pathgnomonic for HD. The diagnostic is supported with studies of the barium enema, anorectal manometry and inmunohistochemestry. Nowadays the best treatment for HD is the transanal surgical descent, it takes the form of the “Pull-through” procedure in which the closest intestinal segment, the ganglionar intestine, is pulled down until it joins to the anus. The advantage of this process is that it isn´t much invasive; it is less complicated and reduces the days of staying in the hospital. After using a few surgical techniques for a time, it can implement a surgery that takes a few phases in just one surgery, one surgical time, how it was described in 1998, by the “Torre- Mondragon “and “Ortega-Salgado”. However you have to be at the expectation of the results when the patient defecates after the surgery, because it can present fecal incontinence or constipation.


REFERENCES

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  2. Casal M, García J, Martinez T, Rodríguez E. Tratamiento rehabilitador integral de megacolon agangliónico. Reporte de caso. Revista Cubana de Medicina Física y Rehabilitación. 2014, Vol. 6(1), pp 72-76.

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Rev Med Cos Cen. 2016;73