Peri-operative management for excision of plexiform neurofibromatosis complicated by massive blood loss

Abstract

Background: Neurofibromatosis is a hereditary disease characterized by formation of multiple fibromatous tumours and patches of skin pigmentation. Excision of these very vascular tumours is often delayed until they attain huge sizes. The surgical procedure is therefore usually lengthy and blood loss can be massive.
Aim: To report the successful peri-operative management for surgical excision of a plexiform neurofibroma on the left thigh complicated by a massive blood loss
Case report: A 28-year-old female weighing 78 kilograms presented at the University of Port Harcourt Teaching Hospital (UPTH) with a huge left thigh mass, nodules and brownish skin patches (café-au-lait spots) all over her body. Plexiform neurofibromatosis was diagnosed. The mass was subsequently excised under epidural anaesthesia and it weighed 15.4kg. Blood loss within the first three hours of surgery exceeded 3.5 litres. Six units of fresh whole blood were rapidly transfused. Five litres of normal saline were infused while intravenous ephedrine was administered.  Post-operative analgesia in the intensive care unit (ICU) was achieved with epidural bupivacaine and fentanyl. Patient remained in stable condition, had satisfactory post-operative analgesia and was discharged home two months after surgery for follow-up at the out-patient clinic.
Conclusion: Successful peri-operative management for neurofibromatosis excision complicated by a massive blood  loss requires that bleeding be promptly arrested, that blood volume and haemoglobin be rapidly restored so as to maintain tissue perfusion and oxygenation. Epidural anaesthesia can contribute to a successful outcome.

Keywords: Plexiform neurofibromatosis, Excision, Massive blood loss, Epidural anaesthesia