Hisprung disease (Hirschsprung) is a congenital disorder causing impaired intestinal passage (Mansjoer Ariff, et al. 2000). First introduced by Hirschsprung in 1886. Zuelser and Wilson, 1948 suggests that the narrowing of the intestinal wall is not found parasympathetic ganglion.
Hisprung disease called congenital aganglionic megacolon. This disease is a state of the large intestine (colon) that has no innervation (aganglionic). So, because there are parts of the large intestine (from the anus towards the top) that has no nerves (ganglion), then there is "paralysis" in running the colon so that bowel function becomes dilated (megacolon). The length of the colon affected different for each individual.
Hisprung
Disease Etiology Hisprung
The disease is caused by aganglionosis Meissner and Aurbach in the intestinal wall lining, ranging from spingter ani internus to the proximal, 70% rektosigmoid limited in the area, 10% to the entire colon and around 5% to the entire colon to the pylorus.
Thought to occur because of genetic factors common in children with Down syndrome, a failure during embryonic neural cells in the intestinal wall, failed existence, kranio caudal to myentrik and sub-mucosal plexus wall.
Symptoms Hisprung
As a result of bowel paralysis in carrying out its functions, the stool can not get out. Usually newborns will issue its first stool (meconium) in the first 24 hours. But in babies who suffer Hisprung, feces will come out late or even not able to get out at all. In addition it will also be visible baby belly bulging, accompanied by vomiting. If left longer, the baby weight will increase and there will be growth disorders.
Pathophysiology of Disease Hisprung
The term congenital aganglionic Mega Colon describing the primary damage in the absence of ganglion cells in the distal colonic mucosa wall sub. Aganglionic segment is almost always present in the proximal part of the rectum and colon. This raises the absence of abnormality or absence of movement propulsion (peristalsis) and the absence of spontaneous bowel evacuation and rectal spinkter can not relax thus preventing the escape of stool normally causes the accumulation in the intestine and distention of the gastrointestinal tract. The proximal to the damaged part in the Mega Colon (Betz, Cecily & Sowden, 2002:197).
All the intramural ganglion plexus in the intestine is useful to control the contraction and relaxation of normal peristalsis.
Fill in intestines pushed into aganglionic segments and feces collected in that region, causing the proximal colon terdilatasinya part of the area is due to obstruction and cause Colon section is widened (Price, S & Wilson, 1995: 141).
Additional examination of the disease Hisprung
Digital rectal examination to assess any relaxation of anal muscles.
Additional tests that may be done is abdominal x-ray, barium enema and rectal biopsy. Roentgen stomach aimed to see if there is enlargement / widening of the intestine is filled by stool or gas. Barium enema, which incorporates a radioactive liquid through the anus, so that later can be seen clearly on the x-ray up to what extent the colon affected by this disease. Biopsy (taking a tissue sample with a needle colon) through the anus may indicate a definite lack of innervation of the colon. A biopsy is usually performed when the large intestine affected by this disease long enough or barium enema examinations are less able to describe the extent of the affected bowel.
Hisprung Disease Complications
Necrotizing enterocolitis, intestinal pneumatosis, perikolon abscess, perforation and septicemia.
Management of clients with Hisprung
1. Conservative. In neonates performed sonde installation stomach and rectal tube to remove meconium and air.
2. Surgery while. Colostomy in neonates, late diagnosis, severe enterocolitis and poor general condition.
3. Definitive surgery. Mereseksi part intestinal aganglionosis and making anastomosis.
Nursing care in clients with Hisprung
A. Assessment.
1. Identity.
The disease is mostly found in infants and is a single disorder. Rarely in premature infants or in conjunction with other congenital abnormalities. In this segment from the anus to the sigmoid aganglionosis is more common in boys than girls. While abnormalities that exceeds even the entire sigmoid colon or small intestine are found as much in boys and girls (Ngastiyah, 1997).
2. History of Nursing.
a. The main complaint.
Obstipasi is the hallmark and the newborn. Trias is often found is a slow exit meconium (more than 24 hours after birth), flatulence and vomiting green. Other symptoms are vomiting and diarrhea.
b. History of present illness.
It is a congenital abnormality that is functional intestinal obstruction. Total obstruction at birth with vomiting, abdominal distension and absence of meconium evacuation. Babies often suffer from constipation, vomiting and dehydration. Mild symptoms of constipation for several weeks or months followed by acute intestinal obstruction. But there is also a mild constipation, enterocolitis with diarrhea, abdominal distension, and fever. Fetid diarrhea may occur.
c. History of the disease first.
No previous illnesses that affect the occurrence of Hirschsprung disease.
d. Family medical history.
No family suffered from the disease passed down to his son.
e. History of environmental health.
No association with environmental health.
f. Immunization.
There is no immunization for infants or children with Hirschsprung disease.
g. History of growth and development.
h. Nutrition.
3. Physical examination.
a. Cardiovascular system.
No abnormalities.
b. Respiratory system.
Shortness of breath, respiratory distress.
c. The digestive system.
Generally obstipasi. Abdominal bloating / abdominal strain, vomiting green. In older children there are chronic diarrhea. In anal plug will feel pinching fingers and when withdrawn will be followed by the release of air and meconium or feces spraying.
d. Genitourinarius system.
e. Nervous system.
No abnormalities.
f. Locomotor system / musculoskeletal.
Impaired sense of comfort.
g. The endocrine system.
No abnormalities.
h. Integumentary system.
Akral warm.
i. Auditory system.
No abnormalities.
4. Diagnostic tests and results.
a. Photos will appear upright plain abdominal intestines dilated or there is a picture
low intestinal obstruction.
b. With barium enema examination found the transition area, picture intestinal contractions
irregular in the narrowed, dilated segments enterocolitis and
there is retention of barium after 24-48 hours.
c. Suction biopsy, looking for ganglion cells in the sub mucosa.
d. Rectal muscle biopsy, which is taking rectal muscle layer.
e. Examination of acetylcholine esterase enzyme activity where there is increased activity
enzyme acetylcholine eseterase.
C. Nursing Diagnosis in Askep Hisprung
1. Elimination Disorders CHAPTER: obstipasi associated with spastic colon and absence of thrust.
2. Alteration in nutrition less than body requirements related to the inadekuat intake.
3. Lack of body fluids associated vomiting and diarrhea.
4. Impaired sense of comfort associated with the presence of abdominal distension.
5. Ineffective family coping related to the state of children's health status.
D. Planning Nursing at Askep Hisprung
1. Elimination Disorders CHAPTER: obstipasi associated with spastic colon and absence of thrust.
Objective: clients do not experience disruption to normal bowel elimination criteria, no abdominal distension.
Intervention:
Monitor the fluid out of the colostomy. Rationale: Knowing the color and consistency of the feces and determine the next plan
Monitor the amount of fluid colostomy. Rationale: The amount of fluid that comes out can be considered for replacement fluid
Monitor the effect of diet on bowel habit. Rational: To know that diet affects bowel habit disturbed.
2. Alteration in nutrition less than body requirements related to the inadekuat intake.
Objective: Nutritional needs are met with the criteria can tolerate as needed parenteal diet or oral.
Intervention:
Give parenteral nutrition as needed. Rationale: Meeting the needs of nutrition and fluids
Monitor your food intake during the treatment. Rationale: Knowing the nutritional balance as required 1300-3400 calories
Monitor or weights. Rationale: To determine the change in weight
3. Lack of body fluids associated vomiting and diarrhea.
Objective: The need for body fluids met the criteria do not become dehydrated, normal skin turgor.
Intervention:
Monitor for signs of dehydration. Rationale: Knowing the conditions and determine next steps
Monitor incoming and outgoing fluids. Rationale: To determine the body's water balance
Give caiaran as needed and the diprograrmkan. Rationale: Preventing dehydration
4. Impaired sense of comfort associated with the presence of abdominal distension.
Objective: The need for a sense of comfort criteria are met with calm, not crying, not disturbed sleep patterns.
Intervention:
Assess for signs of pain. Rationale: Knowing the level of pain and determine next steps
Provide comfort measures: holding, smooth voice, calm. Rationale: Efforts to distraction may reduce pain
Provide appropriate analgesic drug program. Rationale: Reducing the perception of pain whice works on the central nervous system
Bibliography
Kuzemko, Jan, 1995, Son of Clinical Investigation, Peter Andrianto interpreter, prints III, EGC, Jakarta.
Lyke, Evelyn Merchant, 1992, Assesing for Nursing Diagnosis; A Human Needs Approach, JB Lippincott Company, London.
Mansjoer, et al. 2000, Capita Selekta Medicine, ed.3, Media Aesculapius, Jakarta.
Ngastiyah, 1997, Child Care Hospital, EGC, Jakarta.
Hisprung disease called congenital aganglionic megacolon. This disease is a state of the large intestine (colon) that has no innervation (aganglionic). So, because there are parts of the large intestine (from the anus towards the top) that has no nerves (ganglion), then there is "paralysis" in running the colon so that bowel function becomes dilated (megacolon). The length of the colon affected different for each individual.
Hisprung
Disease Etiology Hisprung
The disease is caused by aganglionosis Meissner and Aurbach in the intestinal wall lining, ranging from spingter ani internus to the proximal, 70% rektosigmoid limited in the area, 10% to the entire colon and around 5% to the entire colon to the pylorus.
Thought to occur because of genetic factors common in children with Down syndrome, a failure during embryonic neural cells in the intestinal wall, failed existence, kranio caudal to myentrik and sub-mucosal plexus wall.
Symptoms Hisprung
As a result of bowel paralysis in carrying out its functions, the stool can not get out. Usually newborns will issue its first stool (meconium) in the first 24 hours. But in babies who suffer Hisprung, feces will come out late or even not able to get out at all. In addition it will also be visible baby belly bulging, accompanied by vomiting. If left longer, the baby weight will increase and there will be growth disorders.
Pathophysiology of Disease Hisprung
The term congenital aganglionic Mega Colon describing the primary damage in the absence of ganglion cells in the distal colonic mucosa wall sub. Aganglionic segment is almost always present in the proximal part of the rectum and colon. This raises the absence of abnormality or absence of movement propulsion (peristalsis) and the absence of spontaneous bowel evacuation and rectal spinkter can not relax thus preventing the escape of stool normally causes the accumulation in the intestine and distention of the gastrointestinal tract. The proximal to the damaged part in the Mega Colon (Betz, Cecily & Sowden, 2002:197).
All the intramural ganglion plexus in the intestine is useful to control the contraction and relaxation of normal peristalsis.
Fill in intestines pushed into aganglionic segments and feces collected in that region, causing the proximal colon terdilatasinya part of the area is due to obstruction and cause Colon section is widened (Price, S & Wilson, 1995: 141).
Additional examination of the disease Hisprung
Digital rectal examination to assess any relaxation of anal muscles.
Additional tests that may be done is abdominal x-ray, barium enema and rectal biopsy. Roentgen stomach aimed to see if there is enlargement / widening of the intestine is filled by stool or gas. Barium enema, which incorporates a radioactive liquid through the anus, so that later can be seen clearly on the x-ray up to what extent the colon affected by this disease. Biopsy (taking a tissue sample with a needle colon) through the anus may indicate a definite lack of innervation of the colon. A biopsy is usually performed when the large intestine affected by this disease long enough or barium enema examinations are less able to describe the extent of the affected bowel.
Hisprung Disease Complications
Necrotizing enterocolitis, intestinal pneumatosis, perikolon abscess, perforation and septicemia.
Management of clients with Hisprung
1. Conservative. In neonates performed sonde installation stomach and rectal tube to remove meconium and air.
2. Surgery while. Colostomy in neonates, late diagnosis, severe enterocolitis and poor general condition.
3. Definitive surgery. Mereseksi part intestinal aganglionosis and making anastomosis.
Nursing care in clients with Hisprung
A. Assessment.
1. Identity.
The disease is mostly found in infants and is a single disorder. Rarely in premature infants or in conjunction with other congenital abnormalities. In this segment from the anus to the sigmoid aganglionosis is more common in boys than girls. While abnormalities that exceeds even the entire sigmoid colon or small intestine are found as much in boys and girls (Ngastiyah, 1997).
2. History of Nursing.
a. The main complaint.
Obstipasi is the hallmark and the newborn. Trias is often found is a slow exit meconium (more than 24 hours after birth), flatulence and vomiting green. Other symptoms are vomiting and diarrhea.
b. History of present illness.
It is a congenital abnormality that is functional intestinal obstruction. Total obstruction at birth with vomiting, abdominal distension and absence of meconium evacuation. Babies often suffer from constipation, vomiting and dehydration. Mild symptoms of constipation for several weeks or months followed by acute intestinal obstruction. But there is also a mild constipation, enterocolitis with diarrhea, abdominal distension, and fever. Fetid diarrhea may occur.
c. History of the disease first.
No previous illnesses that affect the occurrence of Hirschsprung disease.
d. Family medical history.
No family suffered from the disease passed down to his son.
e. History of environmental health.
No association with environmental health.
f. Immunization.
There is no immunization for infants or children with Hirschsprung disease.
g. History of growth and development.
h. Nutrition.
3. Physical examination.
a. Cardiovascular system.
No abnormalities.
b. Respiratory system.
Shortness of breath, respiratory distress.
c. The digestive system.
Generally obstipasi. Abdominal bloating / abdominal strain, vomiting green. In older children there are chronic diarrhea. In anal plug will feel pinching fingers and when withdrawn will be followed by the release of air and meconium or feces spraying.
d. Genitourinarius system.
e. Nervous system.
No abnormalities.
f. Locomotor system / musculoskeletal.
Impaired sense of comfort.
g. The endocrine system.
No abnormalities.
h. Integumentary system.
Akral warm.
i. Auditory system.
No abnormalities.
4. Diagnostic tests and results.
a. Photos will appear upright plain abdominal intestines dilated or there is a picture
low intestinal obstruction.
b. With barium enema examination found the transition area, picture intestinal contractions
irregular in the narrowed, dilated segments enterocolitis and
there is retention of barium after 24-48 hours.
c. Suction biopsy, looking for ganglion cells in the sub mucosa.
d. Rectal muscle biopsy, which is taking rectal muscle layer.
e. Examination of acetylcholine esterase enzyme activity where there is increased activity
enzyme acetylcholine eseterase.
C. Nursing Diagnosis in Askep Hisprung
1. Elimination Disorders CHAPTER: obstipasi associated with spastic colon and absence of thrust.
2. Alteration in nutrition less than body requirements related to the inadekuat intake.
3. Lack of body fluids associated vomiting and diarrhea.
4. Impaired sense of comfort associated with the presence of abdominal distension.
5. Ineffective family coping related to the state of children's health status.
D. Planning Nursing at Askep Hisprung
1. Elimination Disorders CHAPTER: obstipasi associated with spastic colon and absence of thrust.
Objective: clients do not experience disruption to normal bowel elimination criteria, no abdominal distension.
Intervention:
Monitor the fluid out of the colostomy. Rationale: Knowing the color and consistency of the feces and determine the next plan
Monitor the amount of fluid colostomy. Rationale: The amount of fluid that comes out can be considered for replacement fluid
Monitor the effect of diet on bowel habit. Rational: To know that diet affects bowel habit disturbed.
2. Alteration in nutrition less than body requirements related to the inadekuat intake.
Objective: Nutritional needs are met with the criteria can tolerate as needed parenteal diet or oral.
Intervention:
Give parenteral nutrition as needed. Rationale: Meeting the needs of nutrition and fluids
Monitor your food intake during the treatment. Rationale: Knowing the nutritional balance as required 1300-3400 calories
Monitor or weights. Rationale: To determine the change in weight
3. Lack of body fluids associated vomiting and diarrhea.
Objective: The need for body fluids met the criteria do not become dehydrated, normal skin turgor.
Intervention:
Monitor for signs of dehydration. Rationale: Knowing the conditions and determine next steps
Monitor incoming and outgoing fluids. Rationale: To determine the body's water balance
Give caiaran as needed and the diprograrmkan. Rationale: Preventing dehydration
4. Impaired sense of comfort associated with the presence of abdominal distension.
Objective: The need for a sense of comfort criteria are met with calm, not crying, not disturbed sleep patterns.
Intervention:
Assess for signs of pain. Rationale: Knowing the level of pain and determine next steps
Provide comfort measures: holding, smooth voice, calm. Rationale: Efforts to distraction may reduce pain
Provide appropriate analgesic drug program. Rationale: Reducing the perception of pain whice works on the central nervous system
Bibliography
Kuzemko, Jan, 1995, Son of Clinical Investigation, Peter Andrianto interpreter, prints III, EGC, Jakarta.
Lyke, Evelyn Merchant, 1992, Assesing for Nursing Diagnosis; A Human Needs Approach, JB Lippincott Company, London.
Mansjoer, et al. 2000, Capita Selekta Medicine, ed.3, Media Aesculapius, Jakarta.
Ngastiyah, 1997, Child Care Hospital, EGC, Jakarta.
