Hirschsprung's disease (HD, congenital aganglionic megacolon) is a motility disorder of the bowel that happens because of the absence of parasympathetic ganglion cells in the large intestine and colon. Therefore, preventing peristalsis, which causes feces to build up, then leading to bowel obstruction. It is most common among infants, yet it can happen in infants and children. Male children are more affected by this disorder. Reasons to contracting this disorder are inherited predisposition or congenital anomalies ( imperforate anus, urinary tract abnormalities, cardiac defects, seizure disorders, Down syndrome), or genetic mutations (RET proto-oncogene: encodes a receptor tyrosine kinase for members of the glial cell line-derived neurotrophic factor [GDNF] family of extracellular signaling molecules). Since these cells are …show more content…
In infants and children the signs and symptoms are chronic constipation, abdominal distention, incidence of forceful stool passing, vomiting, and one may be able to palpate a fecal mass easily. A comlication of inflammation of the small intestine could occur (enterocolitis), which results in foul smelling diarrhea, abdominal distention, and fever. To confirm diagnosis a rectal biopsy is used to determine the absence of ganglionic cells. Treatment is removing the abnormal area of the colon (two-stage process), or a laparoscopic-assisted pull-through. The nurse should maintain proper fluid and electrolyte balance of the pediatric patient prior to surgery. After surgery the nurse would maintain open airway of the NG tube, assess for abdominal distention, incision site care, and monitor the return of bowel sounds. The nurse would also provide family teaching on how to care for the temporary colostomy bag, signs and symptoms of complications ( enterocolitis, leaks, strictures at the area of anastomosis, caring for the families concerns, and advising them that with proper management their child will be able to return to a normal