Anesthetic Course
Considering the airway findings, mask-to-face ventilation after
anesthesia induction was deemed inapplicable; therefore, we chose
fiberoptic intubation while maintaining spontaneous ventilation under
sedation. When the patient entered the operating room, standard monitors
were attached. No major problems were noted with her vital signs, except
for the systolic blood pressure of between 180 and 190. After
establishing an intravenous line in the left forearm and adequate
oxygenation at 6 L/min, we administered 2 mg of midazolam and 50 µg of
fentanyl. Once sedated, her systolic blood pressure decreased to
120–130. After confirming sufficient sedation and spontaneous
breathing, we performed cricothyroid cartilage puncture under ultrasound
guidance using 4% xylocaine. After puncture, awake intubation was
performed using a fiberscope from the right nasal cavity. The intubation
was successful. Thereafter, anesthesia was induced with 50 mg of
propofol and 40 mg of rocuronium. Intraoperatively, anesthesia was
maintained using oxygen, air, desflurane, fentanyl, and remifentanil.
Postoperatively, the patient recovered from the muscle relaxants, as
confirmed by neuromuscular monitoring. Then, 150 mg of sugammadex, a
rocuronium antagonist, was administered. After body movement, eye
opening, adequate spontaneous breathing, and obedience were confirmed,
she was extubated. Subsequently, the systolic blood pressure increased
to 180–190. Other vital signs remained within the normal range. The
operation lasted for 1 hour and 4 minutes, with 2 hours and 12 minutes
of anesthesia. The amount of blood loss was 30 g.
Discussion
As described above, various problems are associated with anesthesia
management in patients with von Recklinghausen’s disease. In the present
case, the worst problem in anesthesia management was the difficulty in
establishing an intact airway. Generally, mask-to-face ventilation is
difficult or impossible in patients with upper-airway deformity because
of several factors, including tumor, abscess, radiation history, and
surgery history9,10; thus, awake intubation is often
indicated. Although awake intubation is safe because it is performed
while maintaining the patient’s spontaneous breathing, it must be chosen
after careful consideration because it can be painful if the
anestesiologist is not skilled. The procedure cannot be performed
without patient cooperation. In addition, persistent airway injuries
have been reported.11 In a previous case report, a
patient with airway difficulty was anesthetized using remimazolam, which
has become clinically available recently in Japan, but was later
awakened because mask-to-face ventilation was
impossible.12 In our case, mask-to-face ventilation
was also impossible because of cutaneous laxity caused by
neurofibromatosis, which is a characteristic of von Recklinghausen’s
disease. Therefore, we had to choose awake intubation. The use of
midazolam, fentanyl, and a local anesthetic agent to reduce the
patient’s pain allowed an uneventful awake intubation. As a
precautionary measure, a tube is inserted from the nasal cavity. The
tube’s tip is placed close to the glottis, serving as an airway if
oxygenation is reduced due to insufficient spontaneous ventilation
during tracheostomy under sedation. Fortunately, such a tube was not
needed in the present case. A sedative agent with minimal respiratory
depression, such as dexmedetomidine, may be a better choice when the
impossibility of mask-to-face ventilation is suspected.
Another problem we encountered during anesthesia management was the
increase in systolic blood pressure, which is similar to a previous
report.13 Previous reports have documented cases of
massive bleeding caused by blood vessel fragility, which is a
characteristic of von Willebrand disease, in addition to difficulty in
intraoperative blood pressure management.13 In our
case, the systolic blood pressure was elevated to 180–190 owing to
tension upon entering the operating room and stimulation during
extubation. Thus, the possibility that the patient had hypertension
concomitantly cannot be ruled out. The patient’s blood pressure was
slightly high before the operation, which was noted by an internist.
Therefore, preoperative interventions, such as the administration of
antihypertensive medication, should have been performed. Although no
major circulatory changes were noted intraoperatively, adequate
consideration of the circulatory system may be necessary even if the
patient did not report any problems.
Furthermore, the effects of muscle relaxants persist in some cases. In
our case, the muscle relaxant effect subsided although it was monitored
continuously by neuromuscular monitoring during the operation. To ensure
safety, we reversed the muscle relaxant before awakening the patient
from anesthesia. In the case we experienced, there was no residual
muscle relaxant, but since the tracheal intubation was performed under
conscious intubation without muscle relaxants, it may not have been
necessary to use muscle relaxants intraoperatively and risk their
prolongation.
Conclusion
We experienced 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.
Although we were able to secure the airway by conscious intubation
without any problems, there were some points to be considered regarding
intercirculatory management and the use of muscle relaxants.