Congenital Midline Anomalies

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Congenital Midline Anomalies

• Summer 2009

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Midline Anomalies

• Cleft lip and palate
• Tracheo-esophageal fistula
• Congenital heart defects
• Neural tube defects Spina bifida
• Omphalocele and Gastroschisis
• Anorectal malformations
• Extrophy of the bladder

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Omphalocele

• Intraabdominal contents herniate through
  umbilical cord.
• Fail to return to abdomen in 10th week of
  gestation. (intestines and liver)
• Protrusion covered by translucent membrane.
• 1.25/5000 live births (intestines only)
• Associated with other congenital abnormalities

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Gastroschisis

• Defect of the abdominal wall with herniation of
  abdominal viscera outside the abdominal cavity
  through defect to the side (usually the right) of
  the umbilicus.
• Small intestine and ascending colon
• 1/10,000 live births

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Care of child with omphalocele or gastroschisis

• Cover sac or contents with sterile saline gauze
  and sterile plastic.
• Monitor vital signs, especially temperature.
• NPO with IV maintenance of fluid and electrolyte
  status.
• Monitor for infection.
• Surgical correction silo for gastroschisis.
• Attainment of bowel motility and function.

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Anorectal malformations

• 1/4000 to 5000 live births (minor to major)
• Anal stenosis
• Anal atresia
• With or without colovesicular fistula
• Medical management with dilations
• Reconstructive surgery with or without a
  temporary ostomy

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9 years
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Bladder Exstrophy

• The bladder wall extrudes through the abdominal
  wall.

p. 1630
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Exstrophy of the Bladder

• Treatment Surgical Reconstruction
• Usually done 24 - 48 hours after birth
• Goals
• Bladder and abdominal wall closure
• Preserve urinary function and continence
• Creation of normal appearing genitalia
• Improvement of sexual functioning

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Exstrophy of the Bladder

• Pre-op Nursing Care
• Prevent infection and trauma to bladder
• Post-operative nursing care
• Care of the surgical site
• Positioning / alignment
• Neurovascular assessment of lower extremities
• Monitor renal function
• Promote comfort
• Discharge teaching

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Urologic surgical procedures

• Vesicostomy
• Ureterostomy
• Mitrafanoff catheterizable stoma
• Malone Antegrade Colonic Enema stoma (MACE or ACE)

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Post-op nursing care

• Care of stoma
• Skin protection
• Care of stents, tubes, drains
• Signs and symptoms of problems

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Spina Bifida/Myelomeningocele

• Neurogenic bladder and bowel
• High incidence of VUR especially if spastic
  bladder
• Increase incidence of PUV and UPJ obstruction
• Early management is essential

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Nursing Interventions

• Prevent damage to upper tracts
• Clean intermittent catheterization
• Vesicostomy or ureterostomies if Grade IV reflux
• Prevent constipation
• Prevent skin breakdown
• Promote self-care

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Mitrafanoffappendiceal stoma

• Creation of catheterizable channel from skin to
  bladder
• Channel is created from reversed appendix that is
  attached to bladder that has usually been
  augmented (made bigger). End of appendix brought
  to skin has nipple valve created and is usually
  place in the umbilicus.

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Post-op care for Mitrofanoff

• Stoma with stents and catheter protruding from
  it.
• Keep skin clean, dry and protected.
• Discharged home with stents and catheter in
  place.
• Teach care, prevention of infection, when to
  call, return visit.

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Teaching for Mitranoffusually done as outpatient

• Clean intermittent catheterization using long
  vinyl coude tipped catheter, usually a size 12
• Must catheterize or will go into renal failure
• Bladder neck is either closed or suspended to
  prevent leakage

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Malone Antegrade Colonic Enema

• Creation of catheterizable channel from ascending
  colon to skin of abdomen for purpose of giving
  colonic irrigation every other day
• Renders the child bowel continent
• Channel is fashioned from piece of small
  intestine and brought to skin in nipple valve

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Post-op care for MACE

• Stoma with catheter protruding from it.
• Keep skin clean, dry and protected.
• Discharged home with catheter in place.
• Teach care, prevention of infection, when to
  call, return visit.

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Teaching for MACE

• Must irrigate every other day to maintain
  continence
• Use mild enema solution
• Maintain schedule for frequency
• Allow time for evacuation

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Nursing assessment

• Ask child where stomas are and which one is which
• Allow child to do procedure as at home with usual
  ritual
• Must be done even if child is ill with unrelated
  disorder

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My Mom has Spina Bifida . . .