Congenital Midline Anomalies Summer 2009. Midline Anomalies Cleft lip and palate Tracheo-esophageal...

Congenital Midline Congenital Midline AnomaliesAnomalies

Summer 2009Summer 2009

Midline AnomaliesMidline 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

OmphaloceleOmphalocele

• 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

GastroschisisGastroschisis

• 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

Care of child with omphalocele or Care of child with omphalocele or gastroschisisgastroschisis

• 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.

Anorectal malformationsAnorectal 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

9 years9 years

The bladder wall extrudes The bladder wall extrudes through the abdominal through the abdominal

wall. wall.

p. 1630

Exstrophy of the BladderExstrophy 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

Exstrophy of the BladderExstrophy 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

Urologic surgical proceduresUrologic surgical procedures

• Vesicostomy• Ureterostomy• Mitrafanoff catheterizable stoma• Malone Antegrade Colonic Enema

stoma (MACE or ACE)

Post-op nursing carePost-op nursing care

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

Spina Spina Bifida/MyelomeningoceleBifida/Myelomeningocele

• Neurogenic bladder and bowel• High incidence of VUR especially if

spastic bladder• Increase incidence of PUV and UPJ

obstruction

• Early management is essential

Nursing InterventionsNursing Interventions

• Prevent damage to upper tracts– Clean intermittent catheterization– Vesicostomy or ureterostomies if Grade

IV reflux

• Prevent constipation• Prevent skin breakdown• Promote self-care

MitrafanoffMitrafanoffappendiceal stomaappendiceal 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.

Post-op care for MitrofanoffPost-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.

Teaching for MitranoffTeaching for Mitranoffusually done as outpatientusually 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

Malone Antegrade Colonic Malone Antegrade Colonic EnemaEnema

• 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

Post-op care for MACEPost-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.

Teaching for MACETeaching for MACE

• Must irrigate every other day to maintain continence

• Use mild enema solution• Maintain schedule for frequency• Allow time for evacuation

Nursing assessmentNursing 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

My Mom has Spina My Mom has Spina Bifida . . .Bifida . . .