Undescended Testes in Medically Complex Children: Evaluating A Common Urologic Condition Through a Multi-Disciplinary Lens

INTRODUCTION: Undescended testis (UDT) is a common genitourinary anomaly in boys. Orchiopexy before 18 months of age is recommended to preserve fertility and reduce testicular cancer risk. However, these recommendations are based on results of outcome studies for otherwise healthy boys and may not be readily applicable for those with developmental and/or genetic disorders for whom anesthetic and surgical risks may be increased and intervention may offer limited benefit. UDT management in boys with complex medical conditions warrants unique considerations, and data-driven guidelines are lacking. In this review, we explore this challenging clinical dilemma through a multi-disciplinary lens. METHODS: A narrative literature review was performed by specialists in pediatric anesthesia, endocrinology, genetics, oncology, urology, and development-behavioral pediatrics. Iteratively refined structured literature searches of the MEDLINE database, GeneReviews®, and online books were performed via PubMed. Anesthetic and surgical risks, fertility potential, testicular cancer risk, and ability to perform self-testicular exams as dictated by the patient's expected cognitive development were evaluated for healthy boys and boys with Down syndrome (DS), Prader Willi syndrome (PWS), Cerebral Palsy (CP), Fragile X, Neurofibromatosis Type 1, Williams syndrome, 22q11.2 deletion syndrome, Rett syndrome, Angelman syndrome, Tuberous sclerosis, and Smith-Magenis syndrome. The results were then evaluated by a bioethicist. RESULTS: For children with complex medical conditions, potential harms of intervention are not theoretical. Surgery may expose the child to substantial anesthetic and surgical risks for which their underlying conditions make them especially vulnerable. The ethical threshold for intervention may therefore be higher especially if certain benefits such as fertility remain uncertain. CONCLUSION: Management of UDT for medically complex boys is nuanced and warrants a multidisciplinary approach to thoroughly assess the risks and benefits of proceeding with, deferring, or declining orchiopexy. Multidisciplinary input will best facilitate individualized care that focuses on the specific needs of each child and appropriately respects parental discretion in determining management that is most in line with the child's long-term quality of life.