hirsch

• HIRSCHSPRUNG DISEASE

  "Congenital Aganglionic Megacolon"

  Mechanical obstruction from inadequate motility of intestine

  • ¼ neonatal obstruction; may not be diagnosed until later in infancy/childhood

  • Incidence: 1 in 5000 live births; more common in males and in Down Syndrome

  • Acute, life threatening / Chronic

  • Absence of ganglion cells in colon
• Pathophysiology hirsch

  • Absence of ganglion cells in one or more segments of the colon

  • Aganglion in distal portion GIT; rectosigmoid (75%)

  • Aganglionic segment beginning at internal anal sphincter, extending proximally blending into normal colon

  • Absence peristalsis → stool accumulates → distended bowel proximal to defect → MEGACOLON
• PATHO CONTINUATION hirsch

  Almost always includes rectum and proximal portion of the large intestine

  • “Skip segments”

  • Internal anal sphincter fail to relax → obstruction → prevent evacuation solid / gas / liquids

  • Intestinal distention and ischemia → Enterocolitis (leading cause of death)

  • Mostly diagnosed on 1st few months of life
• Clinical Manifestation hirsch

  • Aganglionic segment usually includes the rectum and proximal colon

  • Accumulation of stool with distension

  • Failure of internal anal sphincter to relax

  • Enterocolitis may occur

  • NB failure to pass meconium within 24-48 hours of life – Refusal to feed – Bilious vomiting – Abdominal distension
• Clinical Manifestations hirsch

  • Infancy – Failure to thrive – Constipation – Abdominal distension – Episodes of diarrhea and vomiting – Signs of enterocolitis

  • Explosive watery diarrhea

  • Fever

  • Appears significantly ill
• Clinical Manifestations Hirsch

  • Childhood (symptoms appear more chronic)

  – Constipation

  – Ribbon-like foul smelling stool

  – Abdominal distension

  – Visible peristalsis

  – Easily palpable fecal mass

  – Undernourished anemic appearance
• Diagnostic Evaluation

  X-ray, barium enema

  • Anorectal manometric exam (differential diagnosis) • Confirm diagnosis with rectal biopsy

  • Absence of ganglion cells in myenteric and submucosal sphincter
• Therapeutic Management

  • Surgery

  – Remove aganglion portion of bowel → relieve obstruction and restore normal bowel motility and function of internal anal sphincter

  • Two stages

  – Temporary ostomy

  • Proximal to aganglionic segment – Second stage “pull-through” procedure

  • Complete corrective surgery – when child weighs 9kg
• Preoperative Care

  • Malnourished
  • Symptomatic treatment with enemas
  • Low-fiber, high-calorie, high-protein diet
  • TPN
  • Preop preparation not needed by NB (sterile bowel)
  • Older children: empty bowel with repeated saline enemas and systemic antibiotics (decrease bacterial fluids)
  • Enterocolitis - most serious complication
  • Emergency preop care - frequent monitoring of v/s, BP
  • Monitor fluid and electrolyte imbalances, blood replacements
  • Symptoms of bowel perforation: fever, increased abdominal distension, vomiting, increased tenderness, irritability, dyspnea, cyanosis
• Nursing Consideration hursch

  • Measure abdominal circumference

  • Psychologic preparation

  • Postoperative care

  – Same as any post op care

  – Colostomy

  – stoma care

  – Diaper pinned below dressing

  – to prevent contamination of abdominal wound with urine – Foley catheter

  – divert urine flow away from abdomen
• Discharge Care

  • Instruct parents of proper colostomy care

  • Roll up colostomy pouch after it is emptied

  • Applying barrier preparations to surrounding skin
• Nursing Management

  • Monitoring for infection

  • Managing pain

  • Maintain hydration

  • Measuring abdominal circumference to detect any distention

  • Provide support to the child and family

  • Treatment for Enterocolitis associated with Hirschsprung:

  – Rectal irrigations

  – Antibiotics