We present a case of 39y old male who underwent adrenalectomy and aortic lymph node dissection for neuroendocrine tumour in context of underlying VHL disease and previous adrenalectomy. Iatrogenic injury to his duodenum resulted in prolonged, multiple intraabdominal infections and development of enterocutaneous fistula. He had to remain NBM, relied on high dose iv hydrocortisone administration and TPN feed for several months. Conversion back to oral steroids was considered unreliable (tablets observed in fistula drainage bag) and resulted in adrenal crises. To facilitate self-care and discharge for home convalescence prior to stage 2 surgery to close EC fistula, continuous hydrocortisone delivery via sc pump was commenced using a Medtronic 640G insulin pump with mio infusion set. The pump reservoir was filled with 50 mg/ml hydrocortisone solution (100 mg Solucortef powder diluted with water for injection). 3 ml reservoir contained 150 mg of Hydrocortisone (1 pump unit of solution contained 0.5 mg of Hydrocortisone). The pump was set to delivery 60mg of hydrocortisone/24 h in immediate recovery and the dose was gradually reduced to 32 mg/24 h. This dose had to be increased further to 48.5 mg/24 h which ensured serum cortisol concentrations between 226 and 427 nmol/l during 2-hourly cortisol profiling. Separate Basal 2 profile (double the routine dose= 97 mg/24 h) was set for period of illness.
Discussion: The main challenge was complexity of reservoir filling. After intensive training the patient managed this, and the method was well received. In these exceptional circumstances, sc continuous hydrocortisone delivery proved to be reliable, leading to reduction in dose and resolution of signs of steroid over-treatment. It allowed a very complex patient to be discharged. The plan is to proceed to stage 2 surgery to close fistula, which will hopefully allow return to normal bowel functioning and return to oral hydrocortisone administration.