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).