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Crow–Fukase syndrome is a multisystem disorder with anaesthetic risks including prolonged duration of action of neuromuscular blocking drugs. The case of a patient with this syndrome who underwent six separate elective procedures and one urgent surgical procedure is reported. Avoidance of neuromuscular blocking drugs and use of regional anaesthetic techniques are recommended.
Crow–Fukase syndrome is a unique combination of polyneuropathy, organomegaly, endocrinopathy, presence of M proteins and skin changes, also known as POEMS (for ‘polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes’) syndrome,1 and is strongly associated with plasma cell dyscrasia.1,2 There is little published work on anaesthesia for patients with this syndrome, but prolonged duration of action of neuromuscular blocking drugs3 and cardiomyopathy4,5 have both been described. Successful management of epidural anaesthesia has been reported,6 but in one report4 of combined general and epidural anaesthesia continuous infusions of dopamine and dobutamine were required. I believe this to be the first report of a series of anaesthetics using different techniques alone and in combination in a patient with this syndrome.
A 53-year-old man with Crow–Fukase syndrome presented for repair of an anterior abdominal wall hernia. He had widespread subcutaneous lipoatrophy and multiple hernias of the anterior abdominal wall, but it was planned to repair only the uppermost of them. He was in remission from the effects of the syndrome, but no information on whether this would alter the expected prolonged duration of action of neuromuscular blocking drugs could be obtained.
The patient was 1.83 m tall and weighed 109.3 kg. His neck movements, in all directions, were severely restricted by arthritis. He had no other comorbidities. His regular medication was prednisolone, tramadol, allopurinol and omeprazole. He had no known allergies. Shoulder surgery 3 years earlier, before the onset of the syndrome, was reported to have been uneventful. Routine preoperative investigations were normal apart from a white blood cell count of 14.4×109 cells/litre (with neutrophils 10.5×109 cells/litre and no eosinophils), alkaline phosphatase (ALP) of 106 units/litre and thinning of the mitral valve leaflets shown on echocardiogram.
In case postoperative respiratory support proved necessary a bed was booked in the Critical Care Unit. However, it was planned to try to reduce the likelihood of this risk by avoiding neuromuscular blocking drugs and using an epidural for postoperative analgesia. The risks and possible complications were explained and in consequence the patient decided not to proceed.
A year later he returned, now prepared to proceed. His general condition was unchanged. In the anaesthetics room his blood pressure (BP) was 165/95 mm Hg, with a heart rate of 80 beats/min and SpO2 98% breathing air. An infusion of 1 litre of Gelofusine was given through a 16-gauge cannula in the dorsum of his left hand. A lumbar epidural catheter was inserted using full aseptic precautions in the L1–2 intervertebral space and a test dose of 3 ml of levobupivacaine 5 mg/ml was given. After 5 min without adverse effects, anaesthesia was induced with propofol 200 mg and fentanyl 100 μg and anaesthesia was maintained by spontaneous ventilation with sevoflurane 8% in oxygen. When anaesthesia was sufficiently deep, laryngoscopy revealed a grade III view of the larynx, which was converted to a grade II view by using cricoid pressure, and tracheal intubation with a size 9.0 cuffed plastic tube was easily accomplished. He was transferred to the operating theatre where full monitoring was continued, anaesthesia was maintained with sevoflurane 2.5% in oxygen 0.5 litres/min and air 0.5 litres/min delivered into a circle breathing system, and a further 27 ml of levobupivacaine 5 mg/ml in divided doses were given into the epidural catheter. A warm air blanket was used to reduce thermal losses and Hartmann’s solution (1 litre) given during the 40 min of uneventful surgery and anaesthesia. After extubation he was transferred to the Critical Care Unit, where epidural analgesia was continued for 24 h before transfer to a general surgery ward.
A year later he presented for repair of a massive left inguinal hernia and orchidectomy. He continued to be in remission and his medication was unchanged. Routine preoperative investigations were normal apart from the haemoglobin, which was 11.4 g/dl, and the ALP that had increased to 191 units/litre. Anaesthesia using epidural only was planned and a critical care bed was booked. After intravenous cannulation an epidural catheter was inserted using full aseptic precautions in the L2–3 intervertebral space and a test dose of 3 ml lidocaine 2% was given. After 5 min without adverse effects 30 ml of levobupivacaine 5 mg/ml in divided doses were given. Another 10 ml of levobupivacaine 5 mg/ml 1 h later and fentanyl 100 μg a further 20 min later were given into the epidural catheter. A total of 3 litres of colloids and crystalloids were infused during the 2 h of surgery. His core temperature was maintained at greater than 36°C using a warm air blanket. Epidural analgesia was continued for 24 h in the Critical Care Unit before transfer to a general surgery ward.
A further 6 months later he presented for repair of the lower anterior abdominal wall and right inguinal hernias. It was planned to again use epidural only for anaesthesia and analgesia, and he was prepared as before. Following a bloody tap at the L2–3 intervertebral space, the epidural was sited at the L1–2 intervertebral space and test and initial doses were given as for the second operation. Unfortunately the block was inadequate, so the patient agreed to general anaesthesia with local anaesthetic infiltration. Anaesthesia was induced with propofol 200 mg, ketorolac 10 mg, papaveretum 15.4 mg and granisetron 1 mg and maintained with sevoflurane in oxygen-enriched air breathed spontaneously through a size 5 laryngeal mask. A total of 3 litres of colloids and crystalloids were infused and core temperature maintained at 36.8°C using a warm air blanket. Because the booked critical care bed was withdrawn after induction of anaesthesia he was returned to a general surgery ward with outreach support after extended recovery.
A further 6 months later he presented for repair of a lower anterior abdominal wall recurrence. Subarachnoid block at the L1–2 intervertebral space using heavy marcaine 3 ml was effective and without complications.
A further year later he presented for repair of a right inguinal recurrence. Subarachnoid block at the L1–2 intervertebral space using heavy marcaine 2.5 ml was effective and without complications.
At 2 weeks later he presented for drainage of a serous collection from the right flank; this had been aspirated under local anaesthesia but had recurred. Given the choice of subarachnoid or general anaesthesia he chose the latter. Anaesthesia was induced with propofol 200 mg, ketorolac 10 mg, fentanyl 100 µg and granisetron 1 mg and maintained with sevoflurane in oxygen-enriched air breathed spontaneously through a size 5 laryngeal mask.
A further 8 months later he returned with a further recurrence of the right inguinal hernia. He expressed a preference for general anaesthesia; he was given propofol 200 mg, ketorolac 10 mg, papaveretum 15.4 mg and ondansetron 8 mg, and maintenance was with sevoflurane in oxygen-enriched air breathed spontaneously through a size 5 laryngeal mask. Local anaesthetic infiltration using levobupivacaine 5 mg/ml was administered by the surgeon.
Respiratory support was not needed during or after any of the anaesthetics.
At 9 weeks after the last of these procedures he was admitted with a left hemiplegia. Investigation showed a right frontoparietal infarction, normal right carotid artery and no new changes on echocardiography. With physiotherapy and occupational therapy support he made a good recovery over a few months, though this was complicated by degenerative disease of both shoulder joints, postural hypotension and iron deficiency anaemia. Although he remains in remission from the Crow–Fukase syndrome, his mobility has been gradually deteriorating.
Crow–Fukase syndrome was first reported in 19687 (although a similar case had been reported in 19568). It has a high prevalence in Japan, with an incidence in men twice that in women and onset usually in the fifth decade.9 The clinical and pathological findings have been reviewed previously.9–12 The sensorimotor polyneuropathy is distal with gradual proximal spread, with more muscle weakness than sensory disturbance, and with the cranial and autonomic nerves usually uninvolved. Peripheral nerves show axonal degeneration and segmental demyelination with endoneurial oedema. Various multisystem endocrinopathies are common; gonadal and thyroid dysfunction are frequent, and adrenocortical insufficiency and glucose intolerance have both been described. Organomegaly usually involves the liver and often the lymph nodes and the spleen. Most patients have osteosclerotic bone lesions that often have an excess of monoclonal plasma cells. Many other associated features have been described, including cardiomyopathy, haematological abnormalities and pleural effusions. The presence of all five components of the syndrome are not necessary, but neuropathy is usually the main presenting feature.
The pathogenesis is unknown but probably immune mediated;10 high serum levels of vascular endothelial growth factor are found, and M protein, interleukin 1β and tumour necrosis factor α have been implicated.12
Treatment involves chemotherapy, surgery, radiotherapy and/or immunotherapy, and plasmapheresis if other treatments fail, and is more likely to be successful in patients with solitary bone lesions.12 The 5-year survival is 60% in patients with plasma cell dyscrasia and 20% in those with multiple myeloma.10
Competing interests: None.
Patient consent: Patient/guardian consent was obtained for publication.