Four-Year-Old Female Patient with Sacrum Hypoplasia

• Four-year-old female patient presented with a previous diagnosis of cloaca.
• Colostomy was placed at birth and at 1 year a vesicoureteral reimplantation was performed.
• Patient was noted to have sacral hypoplasia, single right kidney, and normal cardiac anatomy.
• Colostomy was identified to be in the LLQ with two separate stomas very close together.
• Perineal exam showed single orifice that drained urine.
• Studies that should be performed prior to surgical repair include renal function panel, renal ultrasound, distal colostogram, cystoscopy, CT scan with 3D reconstruction and complete blood count.
• CT scan with 3D reconstruction was performed rather than distal colostogram.
• Complete blood count, coagulation panel, renal function panel, and renal ultrasound were normal.
• Cystoscopy was performed with a size 8 scope. Orifice was in front of the pubis. The scope was difficult to pass due to a 90-degree angle.
• Common channel was identified to be 7 cm, bladder capacity was 200 mL, normal urethral meatus, rectal fistula to the bladder neck, and no vagina.
• Guide wire was used to place a bladder catheter and a catheter was placed into the distal stoma to allow injection of contract for 3D reconstruction.
• At the time of the reconstruction, the surgeon was unable to pass a catheter into the common channel. She suspects that the initial cystoscope caused trauma to the channel and subsequent stenosis.
• Sphincter had a poor contraction when stimulated.
• Sagittal approach with laparotomy was used for surgical reconstruction.
• Recto-bladder fistula was divided, and the distal segment was very short. This was used for vaginal replacement.
• Because there was limited space between the pubis and the muscle complex, the vagina had to be placed into the muscle complex.
• In the pelvis, two ovaries were identified. The right ovary did not have a fallopian tube. The left ovary had a large fallopian tube and a hemiuterus.
• Mitrofanoff was performed using the appendix and a neo-Malone was created with a tubularized cecal pouch.

Takeaways

• Important to evaluate future prognosis for bowel control prior to deciding which segment of bowel will be used for vaginal replacement. From a technical standpoint, it easier to use the rectum for vaginal replacement, but this should only be used in patients with poor prognosis for bowel control. The patient presented is a perfect example of an instance of when the rectum can be used for vaginal replacement. This patient had long common channel, short sacrum and flat buttocks indicating a poor prognosis for bowel control.
• Typically, the common channel can be used as urethra but in this case most of the urine was coming out of the mucous fistula which is a sign that it is not draining well.