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Hirschsprung Disease (Aganglionic Megacolon)

Janice has been worried encircling her son, Jay. He has been constipated for closely a month; they moderately intention it was all accordingly of his formula subside. However, Jay has working vomiting for 3 days already. As Janice was dressing her son, she accidentally deranged a portio of Jay’s abdomen, which seems to possess gross loops. Sacred for her slip’s shieldion, Janice brought her swiftly to the pediatrician. They told her gently that Jay has ingrained aganglionic megacolon. 

Description


In some cases, aganglionic megacolon or Hirschsprung ailment may be critical ample to be ordinary. 
  • Congenital aganglionic megacolon, so denominated Hirschsprung ailment, is symbolized by perpetual constipation fruiting from portioial or refined intestinal hinderance of habitual account.
  • In 1886, Harold Hirschsprung original illustrative Hirschsprung ailment as a accompute of constipation in forthcoming infancy.
  • Early memory and surgical discipline of Hirschsprung ailment shield unnatural infants from enterocolitis and debilitating constipation.

Hirschsprung Ailment |Pathophysiology


The pathophysiology of aganglionic megacolon is as follows:
  • Hirschsprung ailment fruits from the absence of enteric neurons among the myenteric and submucosal plexus of the rectum and/or colon.
  • Enteric neurons are conservative from the neural bead and avoid caudally delay the vagal resolution fibers along the interior.
  • These ganglion cells get in the proximal colon by 8 weeks’ gestation and in the rectum by 12 weeks’ gestation.
  • Arrest in travel leads to an aganglionic member.
  • This fruits in clinical Hirschsprung ailment.

Statistics and Incidences


Cases of aganglionic megacolon is seen in almost 1 per 5000 speed familys.
  • Prevalence may alter by part and has been shown to be as eminent as 1 per 3000 speed familys in the Federated States of Micronesia.
  • The overall portraiture of Hirschsprung enterocolitis is 25-30%, which accounts for closely all of the portraiture from Hirschsprung ailment.
  • Hirschsprung ailment is almost 4 spaces past contemptible in males than females.
  • Nforthcoming all consequence delay Hirschsprung ailment are diagnosed during the original 2 years of disposition.
  • Approximately one-half of consequence unnatural delay this ailment are diagnosed anteriorly they are patriarchal 1 year; a slender sum of consequence delay Hirschsprung ailment are not ordinary until considerable subjoined in sliphood or adulthood.

Causes


The accounts of aganglionic megacolon are:
  • Genetic accounts. The ailment is generally antipathetic, although impact of familial ailment has been increasing; multiple loci show to be confused, including chromosomes 13q22, 21q22, and 10q; mutations in the Ret proto-oncogene possess been associated delay multiple endocrine neoplasias (MEN) 2A or MEN 2B and familial Hirschsprung ailment; other genes associated delay Hirschsprung ailment emwhole the glial cell-conservative neurotrophic factor gene, the endothelin-B receptor gene, and the endothelin-3 gene.
  • Associated provisions. Hirschsprung ailment is strongly associated delay Down syndrome; 5-15% of resigneds delay Hirschsprung ailment so possess trisomy 21; other associations emwhole Waardenburg syndrome, ingrained deafness, malrotation, gastric diverticulum, and intestinal atresia.

Clinical Manifestations


Examination of infants unnatural delay Hirschsprung ailment discovers:
  • Abdominal distention. Infants delay aganglionic megacolon show tympanitic abdominal distention and demonstrations of intestinal hinderance.
  • Chronic constipation. Older infants and consequence delay Hirschsprung ailment usually confer-upon delay continuous constipation.
  • Palpable intestinal loops.  Upon abdominal Nursing essay, these consequence may teach remarkable abdominal distention delay gross dilated loops of colon.
  • Absence/delayed road of meconiumDuring the newborn epoch, infants unnatural delay Hirschsprung ailment may confer-upon delay insufficiency of road of meconium.
  • Vomiting. Repeated vomiting is confer-upon due to intestinal hinderance.
  • Malnourishement. Poor alimentation fruits from the forthcoming satiety, abdominal vexation, and distention associated delay continuous constipation.

Assessment and Diagnostic Findings


The personality of aganglionic megacolon is made through the subjoined grounds:
  • Laboratory studies. CBC compute, adjust this trial if enterocolitis is reputed; rise of WBC compute or a bandemia should construct regret for enterocolitis.
  • Plain abdominal radiography. Enact this trial delay any prefigurations or demonstrations of abdominal hinderance.
  • Unprepared sole-contrast barium enema. If aperture and enterocolitis are not reputed, an uninitiated sole-contrast barium enema may succor intentt the personality by identifying a transition zone betwixt a narrowed aganglionic member and a dilated and ordinaryly innervated member; the examine may so discover a nondistensible rectum, which is a fashionable prefiguration of Hirschsprung ailment.
  • Rectal biopsy. Personality is developed through rectal biopsy.
  • Rectal manometry. In older consequence who confer-upon delay continuous constipation and an atypical narrative for either Hirschsprung ailment or functional constipation, anorectal manometry can be succorful in making or save the personality.

Medical Management


Treatment involves:
  • Initial therapy. If a slip delay Hirschsprung ailment has demonstrations and prefigurations of a eminent-grade intestinal hinderance, modeblame therapy should emwhole intravenous hydration, delaypursuit of enteral intake, and intestinal and gastric decompression.
  • Decompression. Decompression can be wellbred through arrival of a nasogastric tube and either digital rectal Nursing essay or ordinary saline rectal irrigations 3-4 spaces daily.
  • Diet. A particular sustenance is not required; eventually, preoperatively and in the forthcoming postoperative epoch, infants on a nonconstipated nutriment, such as breast subside, are past easily managed.

Pharmacologic Management

Drug therapy currently is not a constituent of the measure of anxiety for this ailment itself; eventually, some medications may be used to entertain complications of Hirschsprung ailment.
  • Antibiotics. Administer broad-spectrum antibiotics to resigneds delay enterocolitis.

Surgical Management


The surgical options alter according to the resigned’s age, immaterial proviso, power to enact activities of daily food, extension of the aganglionic member, standing of colonic dilation, and nearness of enterocolitis.
  • Leveling colostomy. Surgical options emwhole subversive colostomy, which is a colostomy at the intente of ordinary bowel; a stpatriarchal act delay arrival of a intenteed colostomy followed by a pull-through act.
  • Single-stage pull-through act. The sole-stage pull-through act may be enacted delay laparoscopic, unconcealed, or transanal techniques; this act can be enacted at the space of personality or succeeding the newborn has had rectal irrigations at home and has byed the physiologic base.

Nursing Management


Nursing anxiety address for a slip delay aganglionic megacolon embody:

Nursing Assessment

Assessment involves:
  • History. Carefully subjoin a narrative from the race anxietygivers, noting distinctly the narrative of stooling; ask encircling the aggression of constipation, the symbol and trail of stools, the opulence of bowel motions, and the nearness of inconsiderable feeding manners, anorexia, and irritability.
  • Physical Nursing essay. During the tangible exam, note for high-flown abdomen and prefigurations of inconsiderable alimentation; archives moment and partotality prefigurations.

Nursing Diagnosis

Based on the tribute grounds, the important nursing diagnoses for Hirschsprung ailment are:

Nursing Anxiety Planning and Goals

The important nursing anxiety intentning goals for resigneds delay Hirschsprung Ailment are:
  • Maintaining bark refinedness.
  • Promoting ease.
  • Maintaining spirithither adjust.
  • Maintaning entertaining, upright nasal and vocal membranes.
  • Reducing anxietygiver anxiety.

Nursing Interventions

Nursing mediations for a slip delay aganglionic megacolon embody:
  • Promote bark refinedness. When enacting sequence colostomy anxiety. furnish anxietyful notice to the area about the colostomy; archives and description redness, impatience, and rashy showances of the bark about the stoma; fit the bark delay bark-toughening preparations that establish it and arrange amelioblame adhesion of the means.
  • Promote ease. Note for prefigurations of aversion, such as crying, pulse and respiration blame increases, perturbation, indemnifying of the abdomen, or artfulness up the legs; direct analgesics as adjusted; appended nursing measures that can be used are changing the slip’s standing, pursuit the slip when likely, endearment, cuddling, and winning in age-appropriate activities.
  • Maintain spirithither adjust. Accublame intake and output determinations and descriptioning the symbol, totality, and proportion of stools succor indivisibleize when the slip may possess vocal feedings; to warner spirithither dropping, archives and description the drainage from the NG tube perfect 8 hours; and quickly description any remarkable drainage, such as bright-red bleeding.
  • Provide vocal and nasal anxiety. Enact amiable mouth anxiety at smallest perfect 4 hours; at the similar space, gently upright the nares to succor any impatience from the NG tube.
  • Provide race education. Show the race anxietygiver how to anxiety for the colostomy at home; examine topics such as devices and their use, daily irrigation, and bark anxiety; the anxietygivers should teach their construction by caring for the colostomy beneath the supervision of nursing personnel divers days anteriorly empty.

Evaluation

Goals are met as evidenced by:
  • The slip’s bark refinedness is maintained.
  • The slip’s demeanor denotes minimal aversion.
  • The slip’s spirithither intake is extensive.
  • The slip’s vocal and nasal mucous membranes hold scatiness.
  • The race anxietygivers teachd expertness and acquaintance in caring for the colostomy.

Documentation

Documentation in a resigned delay aganglionic megacolon embody:
  • Individual findings, including factors affecting, interactions, affection of collective exchanges, biaseds of indivisible demeanor.
  • Intake and output.
  • Signs of infection.
  • Cultural and godly beliefs, and awaitations.
  • Plan of anxiety.
  • Teaching intent.
  • Responses to mediations, education, and actions enacted.
  • Attainment or motion internal desired termination.

Practice Quiz: Aganglionic Megacolon


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1. The nurse is assessing an infant delay Hirschsprung’s ailment. The nurture can ahold the infant to: A. Weigh close than awaited for top and age. B. Possess a scaphoid-shaped abdomen. C. Exhibit clubbing of the fingers and toes. D. Possess hyperactive designing tendon reflexes. 1. Answer: B. Possess a scaphoid-shaped abdomen.
  • Option B:  The slip delay Hirschsprung’s ailment get possess a scaphoid or secondary abdomen.
  • Options A, C, and D: Answers A, C, and D do not exercise to the proviso; accordingly, they are loose.
2. Dustin who was diagnosed delay Hirschsprung’s ailment has a fever and spirithither explosive diarrhea. Which of the subjoined would Nurture Joyce do original? A. Direct an antidiarrheal. B. Notify the physician quickly. C. Warner the slip perfect 30 minutes. D. Nothing. (These findings are contemptible in Hirschsprung’s ailment.) 2. Answer: B. Notify the physician quickly.
  • Option B: For the slip delay Hirschsprung’s ailment, fervor and explosive diarrhea denote enterocolitis, a disposition-threatening office. Therefore, the physician should be notified quickly.
  • Option A: Generally, accordingly of the intestinal hinderance and inextensive propulsive intestinal motion, antidiarrheals are not used to entertain Hirschsprung’s ailment.
  • Option C: The slip is acutely ill and requires mediation, delay warnering past regularly than perfect 30 minutes.
  • Option D: Hirschsprung’s ailment typically confer-upons delay continuous constipation.
3. Parents import their infant to the clinic, seeking entertainment for vomiting and diarrhea that has lasted for 2 days. On tribute, the nurture in inculpate detects dry mucous membranes and torpor. What other findings intimate a spirithither quantity nonpayment? A. A sunken fontanel B. Decreased pulse blame C. Increased blood constraining D. Low urine biased gravity 3. Answer: A. A sunken fontanel.
  • Option A: In an infant, prefigurations of spirithither quantity nonpayment (dehydration) emwhole sunken fontanels, increased pulse blame, and decreased respect constraining.
  • Options B  and C: These options are the counter of the demonstrations in spirithither quantity nonpayment.
  • Option D: This is not a demonstration of spirithither quantity nonpayment.
4. A newborn’s insufficiency to by meconium among the original 24 hours succeeding family may denote which of the subjoined? A. Hirschsprung ailment B. Celiac ailment C. Intussusception D. Abdominal mole shortcoming 4. Answer: A. Hirschsprung ailment.
  • Option A: Failure to by meconium among the original 24 hours succeeding family may be an demonstration of Hirschsprung ailment, a ingrained irregularity fruiting in habitual hinderance due to inextensive motility in an intestinal member.
  • Options B, C, and D: Failure to by meconium is not associated delay celiac ailment, intussusception, or abdominal mole shortcoming.
5. Which of the subjoined electrolytes are obsolete as a fruit of vomiting? A. bicarbonate and calcium B. sodium and hydrogen C. sodium and potassium D. hydrogen and potassium 5. Answer: D. hydrogen and potassium.
  • Option D: In excellent gastrointestinal spirithither dropping, hydrogen and potassium are obsolete accordingly these electrolytes are confer-upon in opulence in the stomach.
  • Options A, B, and C: These options are loose.

See Also


Related topics to this examine guide:

Further Reading


Recommended resources and books for pediatric nursing:
  1. PedsNotes: Nurse's Clinical Pocket Guide (Nurse's Clinical Pocket Guides)
  2. Pediatric Nursing Made Incredibly Easy
  3. Wong's Essentials of Pediatric Nursing
  4. Pediatric Nursing: The Critical Components of Nursing Care

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