1. Congenital Midline Anomalies
Summer 2009
All of these anomalies occur prior to the 4th to 10th week of embryonic development.
Many occur together! If you see one, look for another!

2. 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

3. 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

4. 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

5. 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.

6. 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
Anal stenosis can be managed with dilations using stainless steel dilators. Weekly visits to nurse run clinic (CWOCN) or MD office; size of dilator is gradually increased. The baby HATES it! Mom needs LOTS of support to do it every day…and sometimes more frequently than daily.
Anatresia – stool can pass out fistula to bladder if there is one…sometimes there is a fistula to skin = colocutaneous fistula but colovesicular is more common. Stool is in urine! Risk for infection!

7. 9 years
Child with anorectal malformation. Required two stage surgery, temporary colostomy. The remaining segment was so short that the colostomy was left in place until the reconstruction “grew”. She had 3 surgeries although the take-down of the ostomy was fairly quick and simple. Continence can be achieved if there is enough sphincter. Goal is continence! If could not achieve continence used to have permanent diverting colostomy. Now they too can have the Malone ACE procedure that will be discussed later in lecture.
She was able to achieve continence although later that normal and is now 9 years old and very active and normal in every way.

8. The bladder wall extrudes through the abdominal wall.
Bladder Exstrophy
The bladder wall extrudes through the abdominal wall.
Many also have epispadius.
p. 1630

9. Exstrophy of the Bladder
Treatment – Surgical Reconstruction
Usually done hours after birth
Goals
Bladder and abdominal wall closure
Preserve urinary function and continence
Creation of normal appearing genitalia
Improvement of sexual functioning
Everything on slide

10. 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
Wound and pelvis have to be immobilized. Osteotomy to rotate innominate bones to close symphysis pubis. Ureterostomies done to divert urine to diaper or pouch.
Epispadius repair at 1-2 years of age.
In first stage bladder is closed and innominate bone rotated. Internal and/or external fixators used. Avoid abduction of legs. Bladder needs time to grow to accommodate normal amount of urine.
Second stage to repair bladder neck and reattach ureters.
Bladder tissue is abnormal and has high incidence of developing malignancy…start screening at about 20 yrs of age.
Continence may not be achieved. May need ACE or diversion.

11. Urologic surgical procedures
Vesicostomy
Ureterostomy
Mitrafanoff catheterizable stoma
Malone Antegrade Colonic Enema stoma (MACE or ACE)
Vesicostomy – bladder brought to abdominal wall and opened just above symphysis.
Ureterostomy. Both ureters can be brought out together or separately.
Mitrafanoff and MACE describe shortly.

12. Post-op nursing care Care of stoma Skin protection
Care of stents, tubes, drains
Signs and symptoms of problems
Vesicostomy and ureterostomy drain into diaper. Rarely do parent accept pouch unless it is needed to protect skin.
Mitrifanoff and MACE= Child is discharged with a catheter in the stoma as well as stents.

13. 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
Everything on slide. Emphasize that renal failure secondary to neurogenic bladder used to be the cause of early death of this population. Now their lifespan approaches that of normal adults.
High incidence of Posterior Ureteral valves (PUV) make catheterization difficult even with a coude tip catheter. Ureteral Pelvic Junction obstruction cause stasis of urine above the stenotic opening. Incompetent UPJs cause vesicoureteral reflux (VUR). Stents are placed for UPJs until stenotic portion can be removed. Incompetent UPJs are reconstructed to prevent reflux.
These defects can occur without spina bifida.

14. 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
Everything on slide.
Recurent UTIs used to cause staghorn calculi that filled the kidney pelvis leading to renal failure.
Constipation can cause stasis of urine as ureters cross constipated bowel leading to bladder which is in the pelvis.
Self-care and self concept are important for normal development. This lead to exploring a way to make intermittent catheterization easier that trying to reach the perineum in individuals with varying degrees of paralysis and trunk instability. It works so well that it is being used for many other diagnoses that require regular intermittent catheterization.
The MACE was developed after the Mitrofanoff as a way to completely empty the colon periodically so that it is empty and therefore incontinence can not occur.
Next slides describe procedure, pre and post op care and teaching.

15. Mitrafanoff appendiceal 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.
Everything on slide.

16. 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.
Everything on slide.

17. Teaching for Mitranoff usually 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
Bladder Must be catheterized regulary to prevent stasis which is the primary reason these individuals develop UTIs. It is NOT frequent caths that cause frequent UTIs, it is stasis of urine that breaksdown and become a perfect medium for bacterial growth.

18. 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
First used on children with incompetent anal sphincters – paralyzed open. Always have a smear of stool in their underwear! Gravity pushes it out as they walk or sit!

19. 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.
A diverting colostomy was originally done to divert stool while stoma heals. I have heard that some surgeons have had success without it! I don’t know any surgeon that does it without the colostomy.

20. Teaching for MACE Must irrigate every other day to maintain continence
Use mild enema solution
Maintain schedule for frequency
Allow time for evacuation
Most use saline to irrigate. Some who are very prone to constipation use a softening agent.
It can take several hours to evacuate. Remember the bowel is neurogenic – it doesn’t work normally!
They have to plan activities around the irrigation. Otherwise they will be incontinent.

21. 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
It is hard to identify where the stomas are. Scarred abdomen from other surgeries??? Mitrafanoff is usually hidden in the umbilicus. MACE is usually on the right side of the abdomen but…ASK! If you don’t know how to get the specimen, ask the child. He does it all the time.
Child should be able to do his own cath by time he enters school. I’ve taught boys as young as 3 and girls as young as 5.

22. Elizabeth is gorgeous. She was born in Mexico
Elizabeth is gorgeous. She was born in Mexico. We “found” her when her brother came to Shriner’s LA to have his clubfeet repaired. He was three and a half and not potty trained. I’d taught my clinic assistant to ask about siblings and she told me that his 6 year old sister wasn’t potty trained either. We immediately put mom on a bus to go back and bring the sister back with her the next day. IVP revealed Grade 4 VUR so bad that the kidney pelvis seemed to be continuous all the way to the bladder on one side and almost as bad on the other. She was immediately taken to surgery where a vesicostomy was performed before lunchtime. It took 3 months for her to reliable catheterize herself. Once she was reliable, we look down the vesicostomy and she has continued to perform self-cath. Both she and her brother have lipomeningoceles that are skin covered. Eventually they both needed bladder augmentations and bladder nexk reconstruction. They have moved to California because of all the medical care they required to get both of them bowel and bladder continent. She is now in nursing school.

23. My Mom has Spina Bifida . . .
Katanya’s mom was one of my “kids” at the Shriner’s myelo clinic. Her mom was totally incontinent with both sphincters widely open. (Obviously that prevents UTIs and constipation in infancy but is a nightmare when trying to achieve continence. She was a social outcast and had joined a gang. Shriner’s allowed me to make her my “clinic assistant” the first summer I was there. She was almost 14 at the time. She went on rounds with me and helped translate. She also helped translate and illustrate some of the clinic teaching materials. (The first ones I had here do were on kidney damage and catheterization. Next we did prevention of constipation.) A year later, I had began to address sexuality but too late. She disappeared for just over 6 months and came back to clinic pregnant. She was followed at a high risk pregnancy clinic and delivered a full term infant vaginally. She is now the mother of 4 healthy children, is a volunteer at Shriner’s, and has finally gotten here GED and is attending LA Community College.