Congenital Midline Anomalies Summer 2009
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
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
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
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.
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
The bladder wall extrudes through the abdominal wall. Bladder Exstrophy p. 1630
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
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
Urologic surgical procedures Vesicostomy Ureterostomy Mitrafanoff catheterizable stoma Malone Antegrade Colonic Enema stoma (MACE or ACE)
Post-op nursing care Care of stoma Skin protection Care of stents, tubes, drains Signs and symptoms of problems
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
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
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.
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.
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
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
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.
Teaching for MACE Must irrigate every other day to maintain continence Use mild enema solution Maintain schedule for frequency Allow time for evacuation
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