Browse THP Patient Cases

Early Cloacal Reconstruction Case: Evolution of Treament Strategies (Hendren Patient Case)

  • Neonate treated in 1975--early in experience with cloacal reconstruction
  • Sigmoid colostomy was done, but transverse colostomy now preferred
  • Demonstrated repair in lithotomy position to be possible, although today a posterior sagittal approach might be used
  • Hendren's first cloacal repair in 1962 had the vagina exteriorized with a perineal flap, and the rectum was repaired by a posterior sagittal approach and pullthrough

Infant Female Patient with Wide Urogenital Sinus Opening with Bladder Neck at Upper End, Double Diverging Vaginas, and Rectal Fistula Anterior to Vaginas (Hendren Patient Case)

  • Infant female referred in 1988 at 2 months after transverse colostomy elsewhere
  • Wide urogenital sinus opening with bladder neck at upper end, double diverging vaginas, and rectal fistula anterior to vaginas
  • A complex case that required unraveling the anatomy by radiology and cystoscopy to formulate a plan to reconstruct it

Urinary Tract Reconstruction Patient 7: Strictured Ileal Loop

This 14-year-old boy was referred with an ileal loop in 1981.

At age 8 years, two bilateral reimplantation operations had been performed elsewhere and failed.

The kidneys and ureters were only moderately dilated. The bladder was small, being defunctioned for 6 years. The loop was strictured.

Our initial surgery was to stretch his small bladder by inserting a temporary suprapubic catheter embarking on a 13-hour reconstruction operation 6 months later when the bladder had increased to 200 ml, a volume which he could hold as well as void.

The strictured bowel was removed and discarded in order to not make a lower ureter of bowel and risk restricturing. Despite pexing the kidneys downward it was decided to autotransplant the right kidney based on the iliac vessels. A tension free long tunnel reimplant was done on the right and a transureteropyelostomy was performed on the left ureter. The right kidney was perfused with cold Lactated Ringer’s solution and Manitol. Slush ice was used for further cooling. The patient was hypotensive when the clamps were removed which was quickly remedied with more transfused blood. A psoas hitch was used to stabilize the right reimplant. Fluid replacement during the operation was enormous: requiring 13 liters of normal saline, 6 units of packed red cells, 250 ml of D5W, and 750 ml of albumen solution.

Anatomy (Select Image for High-quality Version). (Top) Before operation. (Bottom) After undiversion.

X-rays (Select Image for High-quality Version). (Left) Preop. (Right) Soon postop.

X-rays (Select Image for High-quality Version). 1 year postop.

Urinary Tract Reconstruction Patient 6: Prune Belly Syndrome

This 15-year-old male was referred in 1975 with the Prune Belly Syndrome. At age 2 weeks, bilateral nephrostomies had been performed and thereafter an ileal loop which was unfortunately placed in backwards and did not drain well. A second ileal loop was later performed but it drained poorly also.

The long defunctioned bladder would hold only 100 ml at age 15, even filling with a water pressure 4 feet above his bladder level. Cystoscopy disclosed a type 3 urethral valve which was opened endoscopically in 4 quadrants.

The bowel segment was usuable after reversing its orientation to flow left to right! There was ureteropelvic junction obstruction on both sides. The left colic artery was placed behind the new renal pelvic junction. Both renal pelves were trimmed. The loop was tapered. A psoas hitch was performed to stabilize the new “ureter” reimplant.

Operative time was 11 hours. Blood replacement was with washed red cells to minimize sensitization in view of future renal transplantation.

At 1.5 years after his undiversion surgery, his chemistries were: BUN 40, creatinine 2.0, CO2 19, creatinine clearance 61 liters.

Anatomy (Select Image for High-quality Version). (Top) Preoperative anatomy. (Bottom) Postoperative anatomy.

X-rays (Select Image for High-quality Version). (Left) Antegrade nephrostogram via needle in left kidney. Note small,smooth walled capacity bladder not in use since infancy. (Right) Anategrade study via left kidney 2 years after undiversion at age 15 years. Note now normal bladder size.

Urinary Tract Reconstruction Patient 5: Undiversion by Removing an Ileal Loop Done for Failed Ureteral Reimplants

This 13-year-old boy was referred in 1976 for reconstructive surgery of an ileal loop done after ureteral reimplantation had failed in two prior hospitals 5 years previously. As an infant, he had been treated successfully for HIrschsprung’s Disease by colon resection and pull through. It was presumed that he had a neuropathic bladder.

Preoperative evaluation showed a 100-ml bladder, with good sensation and ability to empty the bladder when it was filled with saline.

Several useful maneuvers are illustrated in this operation. First, is the use of transureteroureterostomy, which we have used extensively. Care must be taken to avoid wedging the crossing ureter beneath a mesenteric vessel, especially the left colic artery. The loop was tapered to substitute for loss of right ureter.

Comment from Dr. Hendren: Patients with small bowel in the urinary tract very commonly will develop one or more well defined strictures which may need dilatation or even cutting as he did. This will require careful long-term observation.

Anatomy (Select Image for High-quality Version). (Top) Before and (Bottom) after reconfiguring his urinary tract.

X-ray (Select Image for High-quality Version). Antegrade study via left kidney. Note discrete narrowing of tapered bowel loop just prior to its entry to bladder. He is allergic to I.V. contrast. Subsequent evaluations have been done by ultrasonography.