Paediatric surgical management of primary pigmented nodular adrenocortical disease: a single tertiary centre experience and review of literature

Primary pigmented nodular adrenocortical disease (PPNAD) is a rare form of adrenocortical hyperplasia. Definitive management is bilateral adrenalectomy. However, there is no surgical consensus on the approach. We aimed to review the management of PPNAD cases utilising the minimally invasive approach. Retrospective, single tertiary centre, case series reviewing all cases of PPNAD managed between 2004 and 2018. Five adrenalectomies were performed in four PPNAD patients: One unilateral transperitoneal adrenalectomy (TPA) with subsequent contralateral TPA due to disease recurrence. One bilateral TPA. Two bilateral retroperitoneoscopic adrenalectomies (RPA). Median age at surgery: 7.45 years (range 3.34–10.16). Median operation length: 6.4 h for RPA and 3 h for TPA. Time to discharge post-operatively: median 9 days for RPA and 7 days for TPA. Oral intake median 1.5 days in RPA and 4 days post-operatively in TPA. There were no intra/post-operative complications. Our experience demonstrates the different surgical approaches used successfully in PPNAD. Although there are only four patients in our series, there are no reports of paediatric experience of RPA in PPNAD. RPA seems to be most favourable due to ease of access to adrenal glands, a faster return to enteral intake and normal activities. Local experience and expertise need to be considered when determining surgical approach. Level 3.