The Hirschsprung’s Disease, is a disease of the large intestines or colon. It is a developmental disorder of the enteric nervous system that is characterized by the absence of ganglion cells in the last part of the colon, which is the rectum. It is worth mentioning that the, ganglion cells are nerve cells which control muscle movement thus aiding the shift of stool along the colon. Without these cells, muscles cannot push stool along the colon, resulting in the build up of the same and may eventually lead to a bacterial infection.
However, the precise mechanism that brings about hirschsprung’s disease is unknown. The disease occurs in one out of every 5000 children and is most common in males than females (North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, n. d). Diognosis of hirschsprung’s disease is usually made among the newborns due to their failure to pass meconium within 24-48 hours after birth. Other symptoms include difficulty in bowel movement, vomiting,watery diarrhoea, poor feeding and weight gain as well as infrequent but explosive stools.
It bears mentioning that clinical pathways are planned multidisciplinary procedures of care designed to support the implementation of clinical guideline and practice. They provide clinical guidance for the management of a patient and their aim is to improve the connection and co-ordination of patients’ care across different clinical sectors. Untreated hirchsprung’s disease may result in a mortality rate of approximately 80% in infants and therefore a lot of care has to be taken when dealing with this disease (Lee, 2010).
The management and treatment of hirchsprung’s disease depends on proper diagnosis which includes performing various rectal tests on the patient to either rule out or detect its presence. These tests include physical examination, abdominal xray, anal manometry, canoscopy, barium enema X-ray and finally rectal biopsy. Careful physical examination is first done at the lower rectum where by the doctor uses a gloved lubricated finger to check for abnormalities. This test is important as it determines the presence of any other associated defects such as anorectal anomaly.
Such an abnormality of the anus and rectum at birth refers to an infant being born without a normal rectal opening. The physical examination is used to rule out such cases but will not determine the presence of hirschsprug’s disease. The next stage after this is an X-ray which is done on the abdomen of the patient. This test is used to find out if the bowel is blocked. If hirschsprung’s is present, the X-ray will reveal decreased air in the colon or the areas where the colon has stretched longer than normal as stool backs up.
Other findings of abdominal X-ray include abdominal masses, intestinal blockage, build up of fluid in the abdomen, hole in the stomach or intestines or even injury to the abdominal tissues (U. S Department of Health and Human Services, 2010). However, the test will only allow the physician to suspect the diagnosis but not definitely diagnose it. A more valuable radiological study is the barium enema X-ray. A barium enema X-ray is an X-ray of the abdomen after a contrast, such as barium, is put in to the colon.
The function of the contrast is employed to increase the visibility of the colon on X-ray. Hirschprung’s disease will be characterised by a narrow colon where the ganglions are missing. A follow up after 24 hours may show remaining barium in the colon. This is a further indication of the hirschsprung’s disease. However, the doctor will need further tests to confirm this. The enema X-ray may nevertheless help to examine the length of the affected colon (Cincinnati Children’s Hospital Medical Center, 2010).
A procedure that will finally establish the diagnosis of the hirschsprung’s disease is the rectal biopsy. A sample of cells from the rectum is taken and then viewed under a microscope. Confirmation of the hirschsprung’s disease is based on the absence of ganglion cells in the biopsy segment. Instead, there will be nonmylinated cells in the sample. There are two types of biopsy which are rectal suction biopsy and also full thickness biopsy. In rectal suction biopsy, cells from the mucus lining of the colon are taken through suction and then observed under a microscope.
On the other hand, full thickness biopsy involves more tissues being removed from deeper layers of the colon either surgically or through a rectal suction. Absence of ganglia cells will indicate hirschsprung’s disease. Apart from the physical anal examination, there are other non-radiology areas that are used in the diagnosis of hirschsprung’s disease and these are manometry as well as canoscopy. Manometry involves insertion of an inflated balloon in the rectum and if the anal muscle does not relax, then hirschprung’s disease might be the cause.
Moreover, canoscopy entails the examination of the colon interior lining using a thin, lighted tube called canoscope. Hirschsprung’s disease is treated with surgery which is in two stages. The first stage involves the removal of the intestinal obstruction by performing a colostomy. The removal entails opening of the colon, into the abdomen through which the bowel can be drained into a wastebag. Several months later, surgeons get rid of the affected colon and then connect the healthy colon to the anus after obtaining the same from the colostomy (Mayo Foundation for Medical Education and Research, 2008).
Clinical pathways are therefore important as they help in the management of a disease from the time it is detected to the time it is treated. This ensures correct diagnosis of diseases and therefore proper treatment. Moreover, it guarantees quality care and also provides a means of continuous quality improvement. Besides, it offers a step by step procedures for decisions to be made with respect to a particular patient and the care to be given for any medical condition.