A Review of Von Hippel-Lindau Syndrome.

Von Hippel-Lindau syndrome (VHL) is a familial neoplastic condition seen in approximately 1 in 36,000 live births. It is caused by germline mutations of the tumor suppressor gene VHL, located on the short arm of chromosome 3. While the majority of the affected individuals have a positive family history, up to 20% of cases arise from de novo mutations. VHL syndrome is characterized by the presence of benign and malignant tumors affecting the central nervous system, kidneys, adrenals, pancreas, and reproductive organs. Common manifestations include hemangioblastomas of the brain, spinal cord, and retina; pheochromocytoma and paraganglioma; renal cell carcinoma; pancreatic cysts and neuroendocrine tumors; and endolymphatic sac tumors. Diagnosis of VHL is prompted by clinical suspicion and confirmed by molecular testing. Management of VHL patients is complex and multidisciplinary. Routine genetic testing and surveillance using various diagnostic techniques are used to help monitor disease progression and implement treatment options. Despite recent advances in clinical diagnosis and management, life expectancy for VHL patients remains low at 40-52 years. This article provides an overview of the major clinical, histological, and radiological findings, as well as treatment modalities.

Present Status of Autonomy in Surgical Residency--a Program Director's Perspective.

As surgery residents graduate and begin their careers as junior attending surgeons, the question of whether a surgeon can complete a case alone still lingers. Allowing autonomy during residency answers this question. The purpose of this study was to gather input from general surgery residency program directors on how they achieve autonomy for residents in their programs. An online survey of 18 questions was sent to all general surgery residency program directors in the United States between April and June of 2013 via e-mail. Questions were asked regarding classification of autonomy, percentage of case completed by the resident independently, and in what area a resident worked with minimal supervision. Of the 202 delivered, 85 program directors were responded (42%). Seventy-eight per cent of programs classified a resident as surgeon junior whether the resident completed more than 50 per cent of the case. Most classified autonomy as either the resident completing >75 per cent of a case (41%) or completing the critical steps of a surgery (41%). Eighty-eight per cent stated that chief residents completed the majority of cases under supervision, whereas only 12 per cent stated the chief had autonomy in the operating room and also acted as teaching assistant. While, 60 per cent stated their chief residents did not work in any area of the hospital independently. Despite differences in how autonomy is defined among programs, most program directors feel that their chief residents do not achieve complete autonomy. Programs should allow their residents to work in a progressive responsibility as they progress into their fourth and fifth years of residency to achieve autonomy.