Long-term Clinical and Biochemical Outcomes in Adult Mucopolysaccharidosis Type I Hurler Scheie and Scheie Patients

In testicular torsion (TT), delayed emergency scrotal exploration (ESE) increases the risk of orchidectomy. Transfer of a patient with suspected TT from a district general hospital (DGH) to a paediatric surgical centre (PSC) delays ESE and potentially puts them at increased risk of testicular loss.Prior to 1st January 2017, all boys under aged <16 years presenting to a DGH within the East Midlands Clinical Network (EMCN) would be referred to the PSC. From this date, it was agreed within the EMCN that boys aged ≥5 years with suspected TT presenting to a network DGH would be managed locally, barring exceptional circumstances. Boys aged <5 years would be referred to the PSC for management.This study aimed to assess the impact of decentralisation of ESE for suspected TT on orchidectomy rates in the EMCN.All patients who underwent ESE under the care of paediatric surgery in the PSC, and all patients <16 years old who underwent ESE in 4 EMCN DGHs between January 2017 and December 2019 were identified. Neonatal cases and inpatient referrals were excluded. Comparison was made with published data on ESE performed in the PSC over the 9 years 2008–2016 prior to decentralisation.In the 9 years prior to decentralisation, there were 110 cases of TT in the PSC. In the subsequent 3 years, there were 40 in the PSC and 37 in the DGHs. The orchidectomy rate of boys with TT presenting to DGHs and undergoing exploration locally (16%, 6/37) contrasts with the rate in those transferred from DGHs to the PSC for exploration (58%, 15/26). The difference is highly significant (p = 0.00059, RR 0.28 [95% CI 0.13–0.63]) and indicates that in the EMCN, avoiding hospital transfer and performing ESE at the presenting DGH reduces the risk of orchidectomy by 72%.Decentralisation of the provision of ESE in boys with TT has resulted in a significantly lower orchidectomy rate in boys undergoing ESE in the presenting hospital than when transferred to the PSC. This study reinforces existing literature that demonstrates the effect of delayed ESE on orchidectomy rate, and supports the recommendations of national guidelines in the UK that transfer of boys to a PSC for ESE should only occur in exceptional circumstances.