ABSTRACT
Von Recklinghausen’s disease is characterized by skin pigmentation, multiple neurofibromatosis, and osseous changes. In the management of anesthesia, a variety of pathologies need to be taken into account. This case describes the perioperative management of a patient with Recklinghausen’s disease suspected difficulty in airway management.
Keywords: Von Recklinghausen’s disease, Awake intubation, Airway management
Introduction
Von Recklinghausen’s disease, which was first reported by Friedrich Daniel von Recklinghausen in 1882, is characterized by skin pigmentation, multiple neurofibromatosis, and osseous changes.1 The disease occurs in 1 out of 3000–4000 individuals.2 Problems in anesthesia management include hypertension caused by renal artery stenosis and circulation instability and major bleeding caused by pheochromocytoma3–5; airway obstruction caused by a tumor in the mouth, pharynx, or airways 6; and an aberrant response to muscle relaxants.7,8 Here, we report the perioperative management of a patient with suspected difficulty in airway management because of cutaneous laxity caused by neurofibromatosis of Recklinghausen’s disease and trismus resulting from pathological fractures associated with mandibular osteomyelitis. The patient provided written informed consent.
Case Report
A 74-year-old woman (height, 151 cm; weight, 58 kg) was scheduled for tooth extraction and anti-inflammatory surgery under general anesthesia for right mandibular osteomyelitis. She had been diagnosed with von Recklinghausen’s disease; her mother also had the same disease. Her past medical history included surgery for appendicitis when she was 50 years of age. Her preoperative examination findings, including blood tests, chest radiography, electrocardiogram, and vital signs, were uneventful, except for mild hypertension noted by an internist. In addition, airway management was predicted to be difficult based on the following preoperative airway findings: cutaneous laxity (Figure 1, left) and mandibular micrognathia (Figure 1, right) caused by neurofibromatosis of von Recklinghausen’s disease and trismus resulting from pathological fractures associated with mandibular osteomyelitis (Figure 2).