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Regional anesthesia for a patient with hereditary neuropathy with liability to pressure palsies

Massimiliano Carassiti, MD^*, D. John Doyle, MD PhD FRCPC, Rita Cataldo, MD^*, Alessia Mattei, MD^*, Benedetta Gallì, MD^* and Felice E. Agrò, MD^*

^* University School of Medicine Campus Bio-Medico, Rome, Italy,
Cleveland Clinic Foundation, Cleveland, USA, E-mail: f.agro{at}unicampus.it

To the Editor:

Hereditary neuropathy with liability to pressure palsies (HNPP) is a focal, recurrent, hereditary, demyelinating neuromuscular disorder characterized by weakness and paresthesia following apparently trivial compression injury.¹ The most commonly affected sites are the peroneal nerve (from compression against the fibular head), the ulnar nerve (from prolonged resting on the elbow) and the radial nerve (from compression at the spiral groove in humerus). The brachial plexus is also frequently affected.

Hereditary neuropathy with liability to pressure palsies was first described by De Long, who studied a family of three generations suffering from recurrent peroneal neuropathy. Sausage-shaped swellings of the myelin sheath ("tomacula") are found on biopsy.¹ In most cases, the genetic anomaly is a deletion of 1.5 million base pairs in chromosomal region 17p11.2, which contains the gene coding for peripheral myelin protein 22 (PMP-22).¹ Despite such knowledge, implications for anesthesia in these patients remain provisional, and few reports on anesthetic management have been published, two being obstetric cases^2,3 and one case diagnosed following breast surgery.⁴

We recently provided anesthesia to a 27-yr-old male with HNPP who underwent surgery for arthroscopic anterior cruciate ligament reconstruction. The diagnosis of HNPP was made eight years previously when the patient suffered peroneal nerve injury with weakness and numbness in the left leg after prolonged sitting with his legs crossed. Electrophysiological studies demonstrated blocked peroneal nerve conduction at the level of the fibular head and signs of peripheral diffuse neuropathy at other sites. A Southern blot study identified a characteristic deletion on chromosome 17. The patient was initially treated with betamethasone, cobalamin and electrostimulation, with substantial recovery. At the time of the patient’s hospital admission, the anesthesiologist, neurologist and orthopedic surgeon met to plan perioperative management; little helpful literature was found.

We decided to employ regional anesthesia with the goal of avoiding the prolonged immobility with general anesthesia that presumably might increase the risk of a pressure palsy. We performed a L2–L3 spinal anesthetic using a Sprotte needle, and administered 12 mg of hyperbaric bupivacaine with the patient in the sitting position. The patient was then turned to the left lateral position to obtain a unilateral block. For surgery, he was positioned supine with his arms abducted to an angle under 90°. In addition, pads were positioned under both legs, and especially under the popliteal fossa of the operated leg, with the knees flexed slightly. We also encouraged the patient to move his arms and his right leg to maintain comfort. Another surgical precaution was to avoid using a tourniquet. The surgery was uneventful, lasting 90 min; after three hours the block completely regressed. There were no neurological complications and no complaints of pressure palsy. Neurological examinations performed six and 12 hr postoperatively did not reveal any abnormalities. The patient was discharged on the third postoperative day without event. Neurological examinations repeated after a week and after three and six months were negative.

Although there are no existing reports discussing the effect of surgical positioning in patients with HNPP, there are a number of potential causes of nerve palsy in these patients. Consequently, special effort was made to avoid any nerve stretching on the non-operated leg while the surgeon positioned the operated leg so as to avoid any pressure on the peroneal nerve at the fibular head. As noted earlier, we also avoided the use of a tourniquet, which is usually applied to reduce bleeding and provide better surgical conditions, to avoid direct extrinsic pressure on any nerves. In conclusion, patients with HNNP present special surgical positioning challenges for which regional anesthesia may provide specific advantages.

Footnotes

Accepted for publication January 8, 2007.

References

1 Chua SY, Lim YW, Lam KS, Low CO. Hereditary neuropathy with liability to pressure palsies. Singapore Med J 2006; 47: 625–6.[Medline]

2 Lepski GR, Alderson JD. Epidural analgesia in labour for a patient with hereditary neuropathy with liability to pressure palsy. Int J Obstet Anesth 2001; 10: 198– 201.[Medline]

3 Berdai S, Benhamou D, Equipe SOS-ALR. Regional anaesthesia for labor and delivery in a parturient with neuropathy with liability to pressure palsy (tomaculous neuropathy) (French). Ann Fr Anesth Reanim 2004; 23: 1011–4.[Medline]

4 Wijayasiri L, Batas D, Quiney N. Hereditary neuropathy with liability to pressure palsies and anaesthesia: peri-operative nerve injury. Anaesthesia 2006; 61: 1004–6.[Medline]

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