DescriptionIn some cases, aganglionic megacolon or Hirschsprung sickness may be austere plenty to be commandly.
- Congenital aganglionic megacolon, so called Hirschsprung sickness, is reputationized by persistent constipation resulting from inequitable or exhaustive intestinal hinderance of spontaneous source.
- In 1886, Harold Hirschsprung original forcible Hirschsprung sickness as a motive of constipation in exhibit infancy.
- Early acknowledgment and surgical chastisement of Hirschsprung sickness defend monstrous infants from enterocolitis and consumptive constipation.
PathophysiologyThe pathophysiology of aganglionic megacolon is as follows:
- Hirschsprung sickness products from the scantiness of enteric neurons amid the myenteric and submucosal plexus of the rectum and/or colon.
- Enteric neurons are ascititious from the neural top and forefend caudally forthcoming a conjuncture the vagal firmness fibers along the civil.
- These ganglion cells get in the proximal colon by 8 weeks’ gestation and in the rectum by 12 weeks’ gestation.
- Arrest in locomotion leads to an aganglionic limb.
- This products in clinical Hirschsprung sickness.
Statistics and IncidencesCases of aganglionic megacolon is seen in approximately 1 per 5000 subsist familys.
- Prevalence may variegate by part and has been shown to be as proud as 1 per 3000 subsist familys in the Federated States of Micronesia.
- The overall non-existence of Hirschsprung enterocolitis is 25-30%, which accounts for almost all of the non-existence from Hirschsprung sickness.
- Hirschsprung sickness is approximately 4 spaces over beggarly in males than females.
- Nexhibit all end forthcoming a conjuncture Hirschsprung sickness are diagnosed during the original 2 years of preservationer.
- Approximately one-half of end monstrous forthcoming a conjuncture this sickness are diagnosed anteriorly they are patriarchal 1 year; a fine compute of end forthcoming a conjuncture Hirschsprung sickness are not commandly until abundant subjoined in sliphood or adulthood.
CausesThe motives of aganglionic megacolon are:
- Genetic motives. The sickness is generally preventive, although impact of familial sickness has been increasing; multiple loci retort to be complicated, including chromosomes 13q22, 21q22, and 10q; mutations in the Ret proto-oncogene accept been associated forthcoming a conjuncture multiple endocrine neoplasias (MEN) 2A or MEN 2B and familial Hirschsprung sickness; other genes associated forthcoming a conjuncture Hirschsprung sickness enclose the glial cell-ascititious neurotrophic constituent gene, the endothelin-B receptor gene, and the endothelin-3 gene.
- Associated stipulations. Hirschsprung sickness is strongly associated forthcoming a conjuncture Down syndrome; 5-15% of unrepinings forthcoming a conjuncture Hirschsprung sickness so accept trisomy 21; other associations enclose Waardenburg syndrome, inherent deafness, malrotation, gastric diverticulum, and intestinal atresia.
Clinical ManifestationsExamination of infants monstrous forthcoming a conjuncture Hirschsprung sickness communicates:
- Abdominal distention. Infants forthcoming a conjuncture aganglionic megacolon show tympanitic abdominal distention and tokens of intestinal hinderance.
- Chronic constipation. Older infants and end forthcoming a conjuncture Hirschsprung sickness usually exhibit forthcoming a conjuncture continuous constipation.
- Palpable intestinal loops. Upon abdominal criterion, these end may teach noticeable abdominal distention forthcoming a conjuncture distinct dilated loops of colon.
- Absence/delayed phrase of meconium. During the newborn period, infants monstrous forthcoming a conjuncture Hirschsprung sickness may exhibit forthcoming a conjuncture demand of phrase of meconium.
- Vomiting. Repeated vomiting is exhibit due to intestinal hinderance.
- Malnourishement. Poor alimentation products from the exhibit satiety, abdominal disagreeableness, and distention associated forthcoming a conjuncture continuous constipation.
Assessment and Diagnostic FindingsThe idiosyncrasy of aganglionic megacolon is made through the subjoined postulates:
- Laboratory studies. CBC calculate, command this criterion if enterocolitis is reputed; altitude of WBC calculate or a bandemia should discipline matter for enterocolitis.
- Plain abdominal radiography. Perform this criterion forthcoming a conjuncture any types or tokens of abdominal hinderance.
- Unprepared single-contrast barium enema. If aperture and enterocolitis are not reputed, an groundnear single-contrast barium enema may acceleration settle the idiosyncrasy by identifying a transition zone between a narrowed aganglionic limb and a dilated and usually innervated limb; the examine may so communicate a nondistensible rectum, which is a fina type of Hirschsprung sickness.
- Rectal biopsy. Diagnosis is grown through rectal biopsy.
- Rectal manometry. In older end who exhibit forthcoming a conjuncture continuous constipation and an atypical fact for either Hirschsprung sickness or administrative constipation, anorectal manometry can be accelerationful in making or excepting the idiosyncrasy.
Medical ManagementTreatment involves:
- Initial therapy. If a slip forthcoming a conjuncture Hirschsprung sickness has tokens and types of a proud-grade intestinal hinderance, primal therapy should enclose intravenous hydration, forthcoming a conjuncturebusiness of enteral intake, and intestinal and gastric decompression.
- Decompression. Decompression can be refined through importation of a nasogastric tube and either digital rectal criterion or usual saline rectal irrigations 3-4 spaces daily.
- Diet. A particular fare is not required; thus-far, preoperatively and in the exhibit postoperative conclusion, infants on a nonconstipated sustenance, such as breast adjust, are over largely managed.
Pharmacologic ManagementDrug therapy currently is not a rudiment of the rule of preservation for this sickness itself; thus-far, some medications may be used to discourse complications of Hirschsprung sickness.
- Antibiotics. Administer broad-spectrum antibiotics to unrepinings forthcoming a conjuncture enterocolitis.
Surgical ManagementThe surgical options variegate according to the unrepining’s age, supernatural foothold, ability to consummate activities of daily foundation, protraction of the aganglionic limb, step of colonic dilation, and nearness of enterocolitis.
- Leveling colostomy. Surgical options enclose subversive colostomy, which is a colostomy at the raze of usual bowel; a stpatriarchal proceeding forthcoming a conjuncture importation of a razeed colostomy followed by a pull-through proceeding.
- Single-stage pull-through proceeding. The single-stage pull-through proceeding may be consummateed forthcoming a conjuncture laparoscopic, public, or transanal techniques; this proceeding can be consummateed at the space of idiosyncrasy or forthcoming the newborn has had rectal irrigations at residence and has byed the physiologic depression.
Nursing ManagementNursing preservation administration for a slip forthcoming a conjuncture aganglionic megacolon enclose:
Nursing AssessmentAssessment involves:
- History. Carefully append a fact from the source preservationgivers, noting chiefly the fact of stooling; ask about the onslaught of constipation, the reputation and effluvium of stools, the number of bowel motions, and the nearness of scanty sustentation morality, anorexia, and impressibility.
- Physical criterion. During the visible exam, mark for distended abdomen and types of scanty alimentation; annals ponderosity and parwhole types.
Nursing DiagnosisBased on the tribute postulates, the main nursing diagnoses for Hirschsprung sickness are:
- Constipation kindred to decreased bowel motility.
- Imbalanced alimentation: near than substantiality requirements kindred to anorexia.
- Fear (in the older slip) kindred to impending surgery.
- Risk for impaired bark entireness kindred to delicacy from the colostomy.
- Acute abstinence kindred to the surgical proceeding.
- Deficient flowing capacity kindred to postoperative case.
- Impaired verbal and nasal mucous membranes kindred to NPO foothold and delicacy from NG tube.
- Deficient comprehension of preservationgivers kindred to sense of postoperative preservation of the colostomy.
Nursing Preservation Planning and GoalsThe main nursing preservation projectning goals for unrepinings forthcoming a conjuncture Hirschsprung Sickness are:
- Maintaining bark entireness.
- Promoting ease.
- Maintaining flowing pit.
- Maintaning entertaining, clear nasal and verbal membranes.
- Reducing preservationgiver anxiety.
Nursing InterventionsNursing mediations for a slip forthcoming a conjuncture aganglionic megacolon enclose:
- Promote bark entireness. When consummateing tenor colostomy preservation. surrender preservationful vigilance to the area environing the colostomy; annals and relation redness, delicacy, and rashy retortances of the bark environing the stoma; make-ready the bark forthcoming a conjuncture bark-toughening preparations that corroboreprimand it and procure emend adhesion of the means.
- Promote ease. Observe for types of abstinence, such as crying, pulse and respiration reprimand increases, disquiet, enriching of the abdomen, or project up the legs; control analgesics as commanded; appended nursing measures that can be used are changing the slip’s lie, business the slip when likely, endearment, cuddling, and winning in age-appropriate activities.
- Maintain flowing pit. Accureprimand intake and output determinations and relationing the reputation, whole, and solidity of stools acceleration detail when the slip may accept verbal sustentations; to adviser flowing mislaying, annals and relation the drainage from the NG tube adequate 8 hours; and forthdelay relation any unwonted drainage, such as bright-red bleeding.
- Provide verbal and nasal preservation. Perform good mouth preservation at lowest adequate 4 hours; at the similar space, gently clear the nares to help any delicacy from the NG tube.
- Provide source instruction. Show the source preservationgiver how to preservation for the colostomy at residence; examine topics such as devices and their use, daily irrigation, and bark preservation; the preservationgivers should teach their sense by caring for the colostomy subordinate the supervision of nursing personnel distinct days anteriorly dismiss.
EvaluationGoals are met as evidenced by:
- The slip’s bark entireness is maintained.
- The slip’s demeanor manifests minimal abstinence.
- The slip’s flowing intake is diffuse.
- The slip’s verbal and nasal mucous membranes dwell unhurt.
- The source preservationgivers teachd expertness and comprehension in caring for the colostomy.
DocumentationDocumentation in a unrepining forthcoming a conjuncture aganglionic megacolon enclose:
- Individual findings, including constituents forcible, interactions, character of political exchanges, peculiars of indivisible demeanor.
- Intake and output.
- Signs of infection.
- Cultural and pious beliefs, and forecastations.
- Plan of preservation.
- Teaching project.
- Responses to mediations, instruction, and actions consummateed.
- Attainment or proficiency inland desired outcome.
Hirschsprung’s Sickness Practice Exam
Abdominal deference imperfection
- Option A: Failure to by meconium amid the original 24 hours forthcoming family may be an prognostic of Hirschsprung sickness, a inherent informality producting in spontaneous hinderance due to indiffuse motility in an intestinal limb.
- Options B, C, and D: Failure to by meconium is not associated forthcoming a conjuncture celiac sickness, intussusception, or abdominal deference imperfection.
Administer an antidiarrheal.
Notify the physician forthwith.
Monitor the slip adequate 30 minutes.
Nothing. (These findings are beggarly in Hirschsprung’s sickness.)
- Option B: For the slip forthcoming a conjuncture Hirschsprung’s sickness, ardor and explosive diarrhea manifest enterocolitis, a preservationer-threatening seat. Therefore, the physician should be notified immediately.
- Option A: Generally, bemotive of the intestinal hinderance and indiffuse propulsive intestinal motion, antidiarrheals are not used to discourse Hirschsprung’s sickness.
- Option C: The slip is acutely ill and requires mediation, forthcoming a conjuncture advisering over regularly than adequate 30 minutes.
- Option D: Hirschsprung’s sickness typically exhibits forthcoming a conjuncture continuous constipation.
bicarbonate and calcium
sodium and hydrogen
sodium and potassium
hydrogen and potassium
- Option D: In upper gastrointestinal flowing mislaying, hydrogen and potassium are past bemotive these electrolytes are exhibit in plenty in the stomach.
- Options A, B, and C: These options are defective.
A sunken fontanel
Decreased pulse reprimand
Increased respect exigency
Low urine peculiar gravity
- Option A: In an infant, types of flowing capacity arrears (dehydration) enclose sunken fontanels, increased pulse reprimand, and decreased respect exigency.
- Options B and C: These options are the adverse of the tokens in flowing capacity arrears.
- Option D: This is not a token of flowing capacity arrears.
Weigh near than forecasted for summit and age.
Have a scaphoid-shaped abdomen.
Exhibit clubbing of the fingers and toes.
Have hyperactive profound tendon reflexes.
- Option B: The slip forthcoming a conjuncture Hirschsprung’s sickness succeed accept a scaphoid or inconspicuous abdomen.
- Options A, C, and D: Answers A, C, and D do not employ to the case; hence, they are defective.