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Published on: November 30, 2010
Long-term outcome of Fowler-Stephens orchiopexy in boys with prune-belly syndrome
K K Patil1, P G Duffy, C R J Woodhouse
1Department of Paediatric Urology, Great Ormond Street Hospital for Children, London, United Kingdom. kalpanakp@aol.com
Insights
Orchiopexy for prune-belly syndrome in childhood leads to satisfactory adult sexual function and puberty. Most boys achieve normal hormonal profiles and sexual function after surgical correction of intra-abdominal testes.
Area of Science:
- Pediatric Surgery
- Urology
- Endocrinology
Background:
- Prune-belly syndrome is a congenital disorder affecting the abdominal wall, urinary tract, and testes.
- Intra-abdominal testes in boys with prune-belly syndrome are typically managed with orchiopexy.
- Conventional orchiopexy involves division of the gonadal vessels, with varying surgical approaches (1-stage or 2-stage).
Purpose of the Study:
- To evaluate the long-term morphological and functional outcomes of childhood orchiopexy in adults with prune-belly syndrome.
- To assess the spontaneous onset of puberty, hormonal profiles, and sexual function following orchiopexy.
- To compare outcomes based on different orchiopexy techniques (1-stage vs. 2-stage).
Main Methods:
- Retrospective review of 41 adult patients with prune-belly syndrome.
- Patients categorized into three groups based on orchiopexy type: bilateral 1-stage (n=20), unilateral 1-stage/contralateral 2-stage (n=10), and bilateral 2-stage (n=11).
- Assessment of testicular position, hormonal status, puberty achievement, and sexual function.
Main Results:
- Good scrotal testes were achieved in a majority of patients across all groups (90% bilateral in group 1, 60% bilateral in group 2, 70% bilateral in group 3).
- Most patients experienced spontaneous puberty and maintained good sexual function (18/20 in group 1, all in group 2, 10/11 in group 3).
- Only two patients required testosterone supplementation.
Conclusions:
- Orchiopexy with division of gonadal vessels is a successful treatment for intra-abdominal testes in prune-belly syndrome.
- Testicular function is generally sufficient to support spontaneous puberty and satisfactory sexual function in adulthood.
- The surgical approach (1-stage vs. 2-stage) did not significantly impede long-term functional outcomes.
Purpose:
Intra-abdominal testes in boys with prune-belly syndrome have been conventionally managed by 1 or 2-stage orchiopexy with division of the gonadal vessels. We reviewed a series of adults with prune-belly syndrome to assess the morphological and functional outcome of orchiopexy in childhood with specific reference to the spontaneous onset of puberty, hormonal profiles and sexual function.
Materials And Methods:
A total of 41 boys were divided into 3 groups depending on the type of orchiopexy performed, namely group 1-20 with bilateral 1-stage orchiopexy, group 2-10 with unilateral 1-stage and contralateral 2-stage orchiopexy, and group 3-11 with bilateral 2-stage orchiopexy.
Results:
In group 1 9 of 20 patients had good scrotal testes bilaterally, 6 had a good scrotal testis on 1 side and 3 had small testes on each side. Two boys required testosterone supplementation but 18 had normal hormonal and sexual function. In group 2 6 of 10 patients had good scrotal testes bilaterally and 4 had a good scrotal testis on 1 side. All patients underwent spontaneous puberty with good sexual function. In group 3 7 of 11 boys had good scrotal testes bilaterally and 3 had 1 good testis with normal puberty and sexual function. These 10 patients underwent spontaneous puberty with good sexual function.
Conclusions:
The majority of boys with prune-belly syndrome had a satisfactory outcome after orchiopexy with division of the gonadal vessels with testicular function sufficient to induce puberty and maintain satisfactory sexual function in adult life.
