Outcome of orchidopexy in the Prader-Willi syndrome: an 18-year single centre experience
Rosie Cresner, BMBS, MRCS1, Carmen S. Chacon, MD2, Alexander Macdonald, FRCS2, Nicola Bridges, FRCPCH2, Marie-Klaire Farrugia, MD FRCS(Ed)2.
1University Hospital Southampton, Southampton, United Kingdom, 2Chelsea & Westminster Hospital NHS Trust, London, United Kingdom.
BACKGROUND: Prader-Willi Syndrome (PWS) is a rare genetic disorder associated with a range of complex medical and behavioural issues. Up to 90% of affected males have cryptorchidism, and 100% of these patients require induction of puberty due to gonadal failure, and what is more there have been no recorded cases of paternity. We reviewed our 18-year single centre experience to determine the rate of testicular atrophy and complications associated with orchidopexy in this cohort. Our aim is to improve the evidence underlying surgical intervention in this particular patient cohort.
METHODS: Single centre retrospective review of all patients with PWS who underwent orchidopexy over an 18-year period (June 2003 - June 2021, inclusive). Important outcomes were collected including; medical comorbidities, pre-operative imaging, pre-operative human chorionic gonadotropin (HCG) use, age at first surgery, need for staged orchidopexy, anaesthetic complications, testicular atrophy rate. Unless otherwise stated results are reported as median [range]. RESULTS: 43 patients with PWS referred for undescended testis were identified from a prospectively maintained database. A total of 19 patients underwent an orchidopexy at our centre, on 34 testes at a median age of 35 [18 - 143] months. Median age at referral was 13 [6- 134] months. 13 patients (68.4%) had a significant airway or respiratory co-morbidity with potential to complicate anesthesia. 5 patients had pre-operative ultrasound to assess the position of the testes where they were not clinically palpable due to body habitus. 7 patients (36.8%) with impalpable undescended testes received pre-operative HCG.8 testes underwent a 2-stage orchidopexy, 26 testes underwent a single-stage orchidopexy. 1 patient is still awaiting a second-stage procedure. No patient had more than 2 anesthetics. 1 patient had anaesthetic complication of desaturation at induction, and the anesthetic was reversed and surgery postponed.The first post-operative follow-up review was undertaken at 3 [0 - 6] months. There were no early (<30 days) complications. Total follow-up was 60 months [0-60]. 5 out of 34 operated testes atrophied which gives an atrophy rate 14.7%.
CONCLUSIONS: Our study has revealed a 14.7% atrophy rate following orchidopexy in patients with PWS. This is much higher than expected in other patient populations in whom early surgery makes a difference to long-term outcome of gonadal function - this is not the case for the PWS population. Delaying surgery to school-age may reduce anesthetic risk due to respiratory comorbidities and difficult airway, although this should be balanced with fluctuations in the patient's weight.
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