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- Clementson Kockum, Christina
(författare)
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Bladder exstrophy : Clinical and experimental aspects
- 1998
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Doktorsavhandling (övrigt vetenskapligt/konstnärligt)abstract
- Bladder exstrophy is a complex malformation seen in 1/30 000. Most children are subject to repeated operations, including pelvic osteotomy, genital and bladder neck reconstruction and sometimes bladder augmentation or a urinary reservoir, to achieve continence and a normal genital function. A retrospective study of all Swedish children born 1970-89 showed a low continence rate, 17% , and a high rate of wound dehiscence. The results led to concentration of the treatment to fewer pediatric urologists and adoption of a national treatment policy. A prospective study of bladder augmentation and reservoir showed these operations to result in continence and a large bladder volume without major complications. Albumin leakage in the urine and increase rates for skeletal mineralisation need further evaluation. However, intestinal mucosa in the urinary tract is known to imply a risk of malignancy and metabolic complications over time. Therefore, an animal model for bladder augmentation in growing subjects was created. Theoretically, an intestinal patch without mucosa would diminish the risks but was in the pig model shown to shrink to such extent that the bladder volume decreased. A bladder augmented with a full thickness intestinal patch grew faster than the normal bladder which suggests the use of small intestinal patches in a growing child. A child with bladder exstrophy has a wide symphyseal diastasis leading to an outward rotation of the hips and a waddling gait. The fate of this malformation was studied using CT. The rotation of the pelvis was balanced by a torsion within the femur and an initial toeing-out was normalised over time. Incontinence, multiple operations and odd looking genitals implies a risk of psychological problems. However, tests and interviews showed a good self-esteem and a normal behaviour in most cases despite restrictions in social life and feelings of being different. Male teenagers seem to be a risk group and the parents are at risk for sustained psychological problems. Early support to parents and discussion groups for teenagers are suggested.
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