A unique defect - persistent posterior cloaca: An example of staged genito-urinary and digestive tract reconstruction with an alternative vaginal creation using the urinary bladder wall.

We present a case of a girl with an extremely rare, posterior type of persistent cloaca, which was associated with other abnormalities, including an undeveloped vulva and vagina, agenesis of the right kidney, secondary obstructive megaureter, unicornate uterus, persisted tailgut, sacral bone hypoplasia, and pubic symphysis hypertrophy. An operative approach was as follows: (i) colostomy and ureterocutaneostomy; (ii) creation of an ileal conduit with antirefluxing uretero-ileal anastomosis, and then creation of a continent catheterizable ileal reservoir; (iii) anastomosis of sigmoid colon to rectal stump; and (iv) vaginal and external genital reconstruction. Because of abnormal anatomical conditions where the uterus was situated adjacent to the open, incompetent bladder neck, we decided to create a vagina using the bladder wall instead of the bowel segment.

Reconstructive option after radical mutilating surgery in children with genitourinary rhabdomyosarcoma: When sparing the bladder is not an option.

OBJECTIVES: To present versatile surgical reconstructive techniques and their outcomes in pediatric patients with genitourinary rhabdomyosarcoma. METHODS: We retrospectively analyzed the oncological and urological outcomes of seven patients treated between 1992 and 2014 according to the Cooperative Weichteilsarkom Studiengruppe protocols. Intergroup Rhabdomyosarcoma Study staging: local, six patients; and IV, one patient. HISTOLOGY: embryonal, five patients; unclassified, one patient; triton tumor one patient. Surgical treatment included: cystectomy, uterectomy and partial vaginectomy, one patient; radical cystectomy, two patients; cystectomy, one patient; cystectomy with partial prostatectomy, one patient; partial cystectomy, one patient; and partial prostatectomy, one patient. RESULTS: All patients were alive in complete remission at last follow up. In four cases, ileal conduit with ureteral reimplantation with serous-lined extramural tunnel (Abol-Enein technique) was carried out, which was followed by conversion into ileal continent bladder with continent appendiceal stoma for clean intermittent catheterization in three patients. In one boy, partial cystectomy and continent reconstruction was carried out during a single surgical procedure. One child with incontinent urinary diversion is still awaiting a continence solution. One child after partial prostatectomy is continent without any voiding disturbances. CONCLUSIONS: The timing and extent of radical surgery for treatment of genitourinary rhabdomyosarcoma depend on the local anatomical conditions, and the response to previous chemo- and radiotherapy. Cystectomy followed by various reconstructive techniques still remains an important option in the local treatment.

Laparoscopic single port surgery nephrectomy in a child - initial experience.

The aim of the stucdy was to present technical aspects and possible intraoperative complications based on the first single port surgery (SPS) laparoscopic nephrectomy performed in our center. The SPS laparoscopic nephrectomy of the right kidney was performed using a transperitoneal access in a 4-year-old child due to a small nonfunctional kidney complicated by hypertension. The intraoperative course was complicated by rupture of the gall bladder wall after grasper removal without leakage of its contents into the peritoneal cavity. No postoperative complications occurred. The SPS nephrectomy appears to be an advantageous method in children which offers a good cosmetic effect. Other benefits require confirmation in further studies. Fixation of the gall bladder is a very helpful but risky maneuver.