Anorectal malformations Flashcards
Explain what is meant by VACTERL acronym
V: Vertebral defects - scoliosis / hemivertebrae / spina bifida
A: Anorectal malformation / Atresias
C: Cardiac anomalies – any combination (eg ASD/VSD/PDA)
TE: Tracheal-oesophageal fistula + oesophageal atresia
R: Renal - aplasia of a kidney, hydronephrosis etc
L: Limb defects - polydactyly, absence of radius, club feet
List the different anatomical classifications of anorectal malformation
Perineal fistula
Rectovestibular fistula
Rectourethral fistula
Rectovesical fistula
No fistula
Cloaca
What is the most common fistulas in boys and girls
Boys:
- Recto-urinary fistula in 95%
- Perineal inspection: no fistula
Girls:
- Recto-vestibular fistula in 95%
- Perineal inspection: fistula to perineum or vestibulum
What are the clinical signs of bowel obstruction in anorectal malformations in boys and girls
Boys:
- Develop bowel obstruction after 1 – 2 days
- Tiny or absent recto-urinary fistula does not allow sufficient amount of stools to be passed
- Abdominal distension, late sign: vomiting
Girls:
- Pass sufficient amount of stools through recto-vestibular fistula
- Will not develop bowel obstruction
What is the initial management of anorectal malformations
Treat as bowel obstruction:
* Hydration
* NPO, NG tube on free drainage
* Keep warm
Send to Paediatric Surgeon
What is the workup for a patient with no anus
Clinical evaluation of perineum / buttocks
Babygram
Screening sonar
* Renal Sonar
* Heart sonar
Invertogram: to confirm level of lesion - baby has to be atleast 24 hours old to allow air to pass through the system
What does the surgical management of anorectal malformations entail
No visible fistula: 3 stage procedure
* Colostomy
* Posterior sagittal ano-rectoplasty (PSARP) 4 wks after colostomy
* Anal dilatation – if needed
* Colostomy closure
Visible fistula:
* Mostly no colostomy necessary
* Anoplasty or ano-rectoplasty
