To the Editor:
A 20-year-old male patient with the diagnosis of neurofibromatosis was scheduled for excision of a recurrent tumor on the dorsum of his left foot. He was previously operated at the age of 2 for left metatarsophalangeal amputation and neurofibroma excision from thoracolumbar region. In the postoperative period, he had a cardiopulmonary arrest responding to immediate resuscitation, but aspiration of gastrointestinal content delayed his discharge to the 30th postoperative day. Recently, his soft tissue growth on his left foot troubled his walking ability. He was scheduled for an operation under spinal anesthesia, as he and his family rejected general anesthesia strictly because of their previous experience.
The 44-kg and 168-cm high patient, premedicated with midazolam 1.5 mg IV, was monitored with ECG, noninvasive blood pressure, and pulse oximetry. After administration of 1000 mL lactated Ringer’s solution, spinal anesthesia was performed on sitting position with a 27-gauge atraumatic spinal needle (Atraucan, Braun, Germany), with 2 mL 0.5 % hyperbaric bupivacaine between lumbar 4 and 5 intervertebral spaces. The level of the sensorial block was on thoracal 7, and the patient tolerated the surgery very well without additional hypnotics. The duration of the uneventful surgery was 1 h. No postoperative complications were observed and he was discharged from the hospital on the 2nd postoperative day.
The choice of anesthetic technique in patients with neurofibromatosis deserves careful systemic evaluation. Factors influencing airway management, respiratory and cardiovascular problems, central nervous system involvement, and vertebral anomalies make the choice of either general or regional anesthesia complex. For these patients who require surgery, general anesthesia has been the preferred method, as coexisting cranial or spinal neuromas might worsen the neurological status of even the asymptomatic patients during regional anesthesia (1).
In our patient, the presence of intracranial and spinal lesions has been ruled out by craniothoracolumbar MRI. Additionally a 27-gauge atraumatic spinal needle was used in order to prevent cerebrospinal fluid leakage that might possibly deteriorate neurological status. The majority of neurofibromatosis patients have a very benign disease, as in our case (2). A recent radiologic evaluation of the regional anesthesia site may suffice for the safety of the technique.
Patients’ requests and consent is also an important issue. The elements of informed consent are adequate information, adequate understanding, and voluntary decision (3). Because his family suspected general anesthesia as reason for the cardiopulmonary arrest, our patient inflexibly rejected sleeping and eagerly chose spinal anesthesia for his operation.
Spinal block may be extremely difficult in a patient with neurofibromatosis, as kyphoscoliosis or neurofibromas close to the needle puncture site may limit the safety of the procedure. Although some residual scars from his previous operation were present, they were not near the midline, so we did not meet any technical difficulties. Additionally, fluoroscopic guiding through the spinal block led us to an easy approach to the correct intervertebral space.
In conclusion, spinal anesthesia may be a safe practice in thoroughly evaluated patients with neurofibromatosis. Special caution must be taken to avoid neurological sequelae and uneventful anesthesia.
Altan Sahin, MD
Ulku Aypar, MD
1. Crochetiére C. Epidural analgesia for labour in a parturient with neurofibromatosis [commentary]. Can J Anaesth 1995; 42: 422–3.
2. Muir H. Epidural analgesia for labour in a parturient with neurofibromatosis [commentary]. Can J Anaesth 1995; 42: 423–4.
3. Chervnak FA, McCullough LB. Clinical guide to preventing ethical conflicts between pregnant women and their physicians. Am J Obstet Gynecol 1990; 162: 303–7.