Laparoscopic Left Partial Adrenalectomy in a Child with Von Hippel–Lindau and Recurrent Pheochromocytoma

Abstract

Introduction: Patients with Von Hippel–Lindau are at high risk of developing recurrent pheochromocytoma. In this 13-year-old patient status post right adrenalectomy with recurrence on the left, we hypothesized that a laparoscopic left partial adrenalectomy would be safe and effective at removing the tumor while preserving native adrenal function.1,2 This video includes a description of the patient's case and illustrates the key portions of his procedure, including complete removal of the tumor and preservation of the renal and adrenal blood supply. Methods: Although asymptomatic, surveillance abdominal ultrasound demonstrated enlarged paraaortic lymph nodes. Follow-up serum catecholamine was elevated, and abdominal computed tomography demonstrated a mass arising from the inferior aspect of the left adrenal gland. Preoperative alpha- and beta-blockade were undertaken. He presented electively on the day of surgery and underwent a laparoscopic left partial adrenalectomy. Key portions of the procedure include mobilization of the splenic flexure, circumferential dissection of the tumor with preservation of the renal vein, renal artery, and the adrenal vein, and separation of the tumor from normal residual adrenal gland.3 Results: Final pathology demonstrated complete resection and was consistent with a 2.6×2.3×1.9 cm pheochromocytoma with intact capsule. Postoperatively, the patient recovered well, with no complications and no requirement for cortisol replacement. He was discharged home on postoperative day 2. He will continue to be followed by his endocrinologist for annual screening. Conclusions: The technique for laparoscopic partial left adrenalectomy described here has utility in the pediatric population to preserve adrenal function during years of growth. Patients with Von Hippel–Lindau are at high risk of recurrence and need continued surveillance.4 This patient will continue to benefit from preserved native adrenal function for months to years before potential recurrence. The authors have nothing to disclose. There are no potential conflicts exist with any of the authors with this publication. Runtime of video: 4 mins 43 secs