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Hirschsprung's Disease .pdf
- 1. PRESENTED BY :MS. PREETI M. BAGUL NURSING LECTURER
- 3. INTRODUCTION Hirschsprung disease isan intestinal disorder characterized by the absence of nerves in parts of the intestine. This condition occurs when the nerves in the intestine (enteric nerves) do not form properly during development before birth (embryonic development).
- 4. DEFINITION Hirschsprung disease isa congenital malformation of large intestine caused due to congenital absence of ganglion cells in the submucosal and myentric plexus of intestine.
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- 6. • The abilityof the GI tract to respond to the state of the lumen and gut wall by activating peristalsis, controlling blood flow and secretions and thus maintain proper physiological balance depends on the enteric nervous system (ENS) Enteric Nervous System (ENS) Myenteric (Auerbach) Plexus Submucosal (Meissner) Plexus
- 8. INCIDENCES • 1/4th ofall cases of neonatal Intestinal obstruction. • 1 in 5000 live births • It is more common in males than female.
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- 10. CONGENITAL • Ediopathic • Interactionbetween 2 proteins encoded by 2 genes RET Proto- oncogene ( Chromosome 10) EDNRB gene ( Chromosome 13)
- 11. ACQUIRED Vascular causes • afterpull through procedure due to ischemia & tension. Non vascular causes • Vitamin B1 deficiency, Chronic infection( TB)
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- 15. Physical Examination Abdominal Tenderness Tightanal sphincter Rectal Impaction Enterocolitis Tachycardia Hypotension
- 18. Goal • To treatthe complications of unrecognized or untreated Hirschsrung’s disease. • To institute temporary measures until definitive reconstructive surgery can takes place • To manage bowel function after reconstructive surgery
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- 21. Inj Ampicilin 25mg/kg IV q6h Inj Gentamicin 2.5mg/kg IV q8h Inj. Metronidazole 7.5mg/kg IV q6h Inj Botulinum toxin type A >12 years 1.25-2.5 U IM <12 years 0.25-1U IM
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- 23. Swenson Procedure (1948) •The aganglionic segment is resected down to the sigmoid colon and rectum, and an oblique anastomosis is performed between the normal colon and the low rectum.
- 24. Duhamel Procedure (1956) •A rectoanal approach is used and a significant portion of aganglionic rectum is retained. • The aganglionic bowel is resected down to the rectum and the rectum is over sewn. • The proximal bowel is then brought through the retrorectal space and an end to end side anastomosis is performed on the remaining rectum.
- 25. Soave ( endorectal) procedure (1960) • In this there will be removing of mucosa and sub mucosa of the rectum ad pulling the ganglionic bowel through the aganglionic muscular cuff of the rectum. • It includes the primary anastomosis of anus.
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- 27. ASSESSMENT GENERAL • Feeding Habits •Fussiness • Irritability • Distended abdomen • Signs of under nutrition BOWEL HABIT • Frequency • Characteristics of stool • Onset of constipation • Medical history
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- 29. • Constipation relatedto decreased bowel motility. • Imbalanced nutrition: less than body requirements related to anorexia. • Altered Bowel Elimination related to hypertrophy and distention of the proximal colon • Imbalanced nutrition less than body requirement related to intake less.
- 30. • Acute painrelated to the surgical procedure. • Deficient fluid volume related to postoperative condition. • Impaired oral and nasal mucous membranes related to NPO status and irritation from NG tube. • Deficient knowledge of caregivers related to understanding of postoperative care of the colostomy. • Risk for impaired skin integrity related to irritation from the colostomy.
- 31. ASSESSMENT NURSING DIAGNOSIS EXPEXTED OUTCOME GOAL INTERVENTION RATIONALE Patientis not able to pass motion and may have pain in abdomen Constipation related to decreased bowel motility. Patient will be able to pass motion Patient maintain s normal bowel pattern • Assess the bowel pattern • Revise client’s dietary regimen and include fire rich diet • Increase fluid intake • Provide laxatives • Encourage the child for active exercises through play therapy • To get baseline data • To increase intestinal mobility • To provide ease to the stool to pass through lumen • To soften the stool • To increase the peristalsis
- 32. ASSESSMENT NURSING DIAGNOSIS EXPEXTED OUTCOME GOAL INTERVENTIONRATIONALE Child is not able to take food Child may cry Imbalanced nutrition: less than body requirements related to anorexia secondary to abdominal pain and distension Weight Loss: child will be able to have food and increasing the weight Child’s weight gains appropriat e to age • Assess the weight • Provide low residual diet • Increase fluid intake • Change the meal pattern and menu • Provide small but frequent meal • To get baseline data • To increase the digestibility • To provide ease to the stool to pass through lumen • To soften the stool • To increase intake • To met nutritional requirement
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- 34. To maintainintegrity of stoma and peristomal skin To prevent infection To promote general comfort To provide clean ostomy pouch for fecal evacuation To reduce odor from over used pouch OBJECTIVES OF COLOSTOMY CARE
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- 36. • Post operativeenterocolitis Swenson procedure • Constipation Duhamel repair • Diarrhea and incontinence with the Soave pull through procedure • Anastomosis leakage and stricture formation • Intestinal obstruction • Pelvic abscess • Wound infection
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- 38. PROGNOSIS • As expectedpatients with associated trisomy 21 tend to have poorer clinical outcome. • In general more than 90% of patient with Hirschsprung disease have satisfactory outcomes • Although many patients many have disturbances of bowel function for several years before developing normal continence.
- 39. CONCLUSION Hirschsprung disease isan congenital abnormality which is constructive and non life threatening
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- 41. RECAPITULATION • Introduction ofHirschsprung disease • Anatomy and physiology of Hirschsprung disease • Incidences • Pathophysiology of Hirschsprung disease • Clinical manifestations of Hirschsprung disease • Diagnostic Evaluations • Management of Hirschsprung disease Medical Surgical Nursing • Complications
- 42. ASSIGNMENT As per thenursing assessment enlist the nursing diagnosis and write down the nursing care-plan (any 2)
- 43. BIBLIOGRAPHY Wong D.L etal.Essentials of Paediatric Nursing. 6th edition. Missouri: Mosby; 2001 Marlow D.R. Redding B. Textbook of Paediatric nursing. 1st edition.Singapore: Harwourt Brace & company; 1998 Dr.Chaudari KC. Indian Journa of Paediatrics. Nov22 2007 Parthasarathy IAP textbook of Paediatrics. 2nd edition. jaypee: New Delhi; 2002