Summary
Introduction
Prader–Willi syndrome (PWS) is a rare (1:20.000) genetic condition affecting both
males and females. Among other features, in boys, the syndrome is characterized by
cryptorchidism in 86–100% of cases, hypogonadism, delayed puberty and infertility.
The aim of the present study is to appraise the results of orchidopexy in this selected
population of children.
Study design
A follow-up study of children with PWS treated for undescended testes at a single
institution over a 20-year period was performed. Patients were identified from a National
PWS registry and reviewed at a special follow-up clinic. Data were collected from
electronic and hard copies records and reported as median (range).
Results
Thirty-three children (1–17 years) were identified. Co-morbidities were present in
22 (66%) and 15 (45%) were on growth-hormone therapy. Six patients (19%) had normal
testes palpable in the scrotum; twenty-seven (81%) had undescended testes and required
orchidopexy. Thirteen (48%) underwent a bilateral procedure for a total of 40 procedures.
A 2-stage Fowler-Stephens orchidopexy was required in 2 (7%) testes. At surgery hypotrophic
testes were documented in 6 (22%) patients. Age at orchidopexy was 1.4 years (0.5–5.5).
Age at F U was 7.2 years (1.7–17). Length of follow-up is 3.5 years (0.4–14). At follow-up
16 (40%) testes were of normal size and palpable in the scrotum; 7 (17.5%) testes
required redo-orchidopexy. All patients (6/33) over 16 years of age that had testosterone
levels tested had values below normal limits after successful orchidopexy.
Conclusions
Tabled
1
| Findings at follow-up (40 testes) | Number (%) |
|---|---|
| Normal testes in scrotum | 16 (40) |
| Hypotrophic testes in scrotum | 8 (20) |
| Testes in inguinal canal | 10 (25) |
| Non-palpable testes | 4 (10) |
| Atrophic testes in scrotum | 2 (5) |
| Redo-orchidopexy | 7 (17.5) |
Keywords
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Article info
Publication history
Published online: October 15, 2017
Accepted:
October 5,
2017
Received:
March 18,
2017
Identification
Copyright
© 2017 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.
