Von Hippel-Lindau [VHL] syndrome is a hereditary multisystem cancer syndrome characterized by a wide range of benign and malignant tumours. Renal neoplasms are the commonest solid organ malignancies associated with VHL seen in 25-45% of patients. Management of renal tumours is determined by multiple tumour related and patient-related factors and decision making is often difficult due to its multifocal nature and bilateral involvement. Its tendency for frequent recurrences further complicates the clinical picture. We review the existing literature on the management of renal malignancies in VHL syndrome focussing on observational strategies, minimally invasive approaches, nephron-sparing surgery and radical surgery. Furthermore, we provide the Sri Lanka perspective in the management of renal malignancies in VHL syndrome. Surgeons that manage such patients should be cognizant of extra-renal manifestations of VHL and the importance of a multidisciplinary team and referral pathways. Proper follow-up and selecting patients for suitable as well as minimal interventions such as minimally invasive techniques and nephron-sparing approaches is crucial in the management to provide acceptable cancer control while preserving renal functions. In Sri Lanka, establishing proper referral pathways to dedicated centres with a multidisciplinary team equipped with facilities to deal with renal as well as extra-renal manifestations would improve expertise, quality of care and reduce patient discomfort and default rate.

Emphysematous pyelonephritis(EPN) is a potentially fatal manifestation of necrotizing infection of renal parenchymal tissues and perirenal tissues with gas formation in renal system. Its clinical significance due to its diagnostic difficulty on clinical grounds, rapid progression and the demand for the invasive management strategies in short term necessitates the need for a more vigilant approach in vulnerable population. Risk population includes female diabetic patients, obstructed infected urinary system, immunosuppression. Since its initial reporting from 19th century the treatment strategies are more towards extensive invasive approaches due to its rapid progression into fatal condition. Fortunately, with the advent of modern imaging facilities, the diagnostic difficulties were somewhat alleviated which helps in staging the disease. Thence the treatment strategy evolved over time which includes various options in treatment regime. Currently the treatment for emphysematous pyelonephritis includes resuscitation with fluid electrolyte management, intravenous antibiotics to cover gram negative bacteria, risk factor management, minimally invasive approaches towards decompression and in worst case scenario immediate or delayed nephrectomy. Here, we discuss the clinical features,aetiopathogenesis, diagnostic approaches and the treatment strategies of this uncommon but potentially fatal condition.

No abstract