EN
TÜRKÇE
Poster - 108
Lost in transition
Maximiliane Minderjahn, Anja Lingnau
Charité Universitätsmedizin Berlin
We report on a now 34-year-old patient with congenital cloacal exstrophy, who has been continuously cared for in our pediatric surgical center after the primary closure of the bladder and pull-through operation in early childhood.
In 2004 and 2005, a bladder augmentation through ileal pouch and the creation of a continent bladder stoma (Mitrofanoff) were performed, along with vaginal reconstruction. In 2008, vulvoplasty and labial reconstruction were carried out, as well as a repeat sphincter dilation for recurrent anal stenosis. Upon discharge from the pediatric clinic, the patient performed bowel irrigations every 2 days and clean intermittent catheterization (CIC) 6-8 times per day via the Mitrofanoff stoma, maintaining social continence.
Since 2008, there have been recurring hospitalizations in the departments of gynecology, urology, nephrology, gastroenterology, and general surgery. Long-term complications included recurrent urosepsis leading to emergency left nephrectomy, severe recurring episodes of urolithiasis requiring multiple surgical removals of bladder stones via high-section, and a persistent bowel emptying disorder with massive coproliths requiring regular surgical intervention over months. Colleagues in gynecology have been conducting regular bougienage and revision surgeries on the stenosed and scarred neovagina since 2010, as well as recurring surgical procedures for abscesses in the internal and external genitalia.
Currently, the vagina and anus are at most fingertip-accessible, with ongoing regular dilations. Neither vaginal nor anal intercourse is possible. The Mitrofanoff stoma, following the last Deflux injection in 2020, is currently continent, and CIC is uncomplicated with normal creatinine levels.
A structured transition between pediatric and adult medicine, along with care in a competence center involving interdisciplinary collaboration of all specialists for patients with rare congenital malformations beyond childhood, must be a shared goal to ensure the best possible medical care and quality of life for patients beyond primary reconstruction.