Klippel-Feil syndrome is an inherited condition that was first described in 1912 and is characterized by the classic triad of shortness of the neck, reduced neck movements resulting from fusion of cervical vertebrae and a low posterior hairline (1). A wide constellation of associated features has been described and the anatomical and clinical expressions of the syndrome range from mild cosmetic deformity to severe disability (2). Other cervical abnormalities may complicate fusion of the vertebrae including spondylosis, hemivertebrae, hypermobility of unfused segments, and abnormalities of the atlantooccipital junction (3). These may lead to neurological deficits arising either spontaneously or as a result of minor trauma during direct laryngoscopy and tracheal intubation (4–7). Cardiac and renal anomalies occur frequently and kyphoscoliosis is present in up to 60% of patients and may cause difficulties with regional anesthetic techniques (3).
A MEDLINE review from 1966 to the present revealed only five case reports of the anesthetic management of these patients (7–11). Although the use of spinal anesthesia has been reported, this is the first reported use of thoracic epidural anesthesia in a patient with this condition (11).
A 26 yr-old female presented for bilateral reduction mammoplasty. She had a history of Klippel-Feil syndrome associated with kyphosis, unilateral deafness and synkinesia (mirror movements), a peculiar clinical finding in which the patient is unable to move one hand without producing similar reciprocal motion in the other hand. Despite a number of apparently uneventful general anesthetics in the remote past, her recent history included a failed fiberoptic intubation at another institution a year previously, leading to cancellation of the proposed surgery. On a separate occasion, an attempted gas induction was complicated by failure to intubate under direct laryngoscopy and loss of airway control. This life-threatening situation was ultimately overcome by the insertion of a laryngeal mask (LMA). The patient was fully aware of these past complications but was determined to have her surgery. Although this procedure is normally performed for cosmetic reasons, our patient was experiencing great difficulty in exercising because of her large breast mass and her reason for wanting surgery was primarily functional. Her previous anesthetic experience was so traumatic, however, that she was unwilling to consent to awake intubation.
At examination, she was of short stature (1.43 m) and weighed 68 kg. There was obvious deformity of the neck, which was characteristically short with virtually no flexion or extension (Fig. 1). Inter-incisor distance was 25 mm and examination of the pharynx revealed a grade IV airway (12). There was a moderate degree of kyphosis but the lower thoracic spinous processes were palpable without undue difficulty. The remainder of the examination was unremarkable, and in particular there was no focal neurological deficit.
Preoperative investigations included a lateral radiograph of the cervical spine (Fig. 2). This showed fusion of the upper cervical vertebrae, the roentgenographic hallmark of the syndrome, as well as fusion of the atlantooccipital joint. Magnetic resonance imaging suggested normal anatomy below the midthoracic region, and we decided to proceed with the case using low thoracic epidural anesthesia. In the event that it became necessary to convert to a general anesthetic, our plan was to secure the airway using a LMA, which had been successful in the past.
A 19-gauge epidural catheter (Arrow Flexitip plus; Arrow International, Reading, PA) was sited without difficulty at T11-12 using a 17-gauge Tuohy needle. Using the epidural confirmation technique involving electrical stimulation previously described, an electrocardiogram adapter (Arrow-Johans ECG adapter) was connected to the epidural catheter and the assembly was filled with saline (13,14). A nerve stimulator set at a frequency of 1 Hz with a pulse width of 200 ms was connected to the adapter. The stimulation test showed contraction of the T6-7 intercostal muscles at 2 mA, thus confirming catheter location. A standard test dose of 3 mL lidocaine 1.5% with epinephrine 1:200,000 was then given with negative response. Incremental boluses of ropivacaine (0.75% initially, followed by 0.5% to increase volume but limit the dose) were used to establish the block to the T4 dermatomal level as determined by loss of cold sensation. Total dose of ropivacaine was 225 mg administered over 3 h. This was supplemented by a further 150 mg bupivacaine with 2 mg epinephrine made up to 500 mL with normal saline given intra-operatively by local infiltration.
The procedure was well tolerated by the patient, who remained hemodynamically stable and required only minimal sedation throughout. The epidural was not used for postoperative analgesia because our patient was to remain overnight in a 24-h unit where epidural analgesia is not permitted by hospital policy. Postoperative recovery was uneventful and the patient was discharged on the next day.
The choice of anesthetic technique in this case was influenced by the relatively unusual history of failure to intubate on two separate occasions by very different means. On the first occasion, a LMA was required when airway control was temporarily lost after inhaled induction and attempted intubation by direct laryngoscopy. Although our patient suffered no neurological deficits as a result of this episode, the potential for neck instability associated with this condition is considerable. This is illustrated in a retrospective review of 21 patients with the Klippel-Feil syndrome, 43% of whom suffered neurological deficits either spontaneously or after minor trauma (15). On the second occasion, the proposed surgery was cancelled because of a failed awake fiberoptic intubation. This technique has been reported in a patient with the Klippel-Feil syndrome and would have presented a viable option, except that the patient was adamant in her refusal to undergo this procedure.
In patients with normal spinal anatomy, paravertebral blockade is a suitable technique for breast surgery (16). However, in a patient with marked cervical spinal anomalies we considered that the probability of failure with a technique requiring bilateral paravertebral injections to a level of T4 was unacceptable. Epidural anesthesia can be difficult to perform in the presence of kyphosis. In addition, the spread of local anesthetic solutions within the epidural space may be less predictable (11). We discussed the various options with both patient and surgeon at length and concluded that a low thoracic epidural with skin infiltration was the best approach. The result was successful and the patient was very satisfied with the outcome.
An electrical stimulation test was used to confirm proper epidural catheter placement before surgery (13,14). Using this test, a positive segmental motor response (trunk or limb movement) to electrical stimulation (1–10 mA) indicates that the catheter is in the epidural space. In this case, contraction of the T6-7 intercostal muscles was observed at a current of 2 mA, suggesting that the catheter was placed at the T6-7 level within the epidural space.
The dose of epidural ropivacaine used was 225 mg (3.3 mgkg−1). The dose of bupivacaine for skin infiltration was 150 mg (2.2 mgkg−1) plus epinephrine 2 mg (29 μgkg−1). We considered that the coadministration of epidural ropivacaine and subcutaneous bupivacaine was unlikely to pose a significant danger of toxicity to our patient for the following reasons: the dose of ropivacaine was well below the dose of up to 5 mgkg−1 that was used without adverse effects in a previous study, absorption of subcutaneous bupivacaine would be very slow in the presence of epinephrine, and all drugs were administered in incremental boluses over a 3-hour period, thus reducing the risk (17). Plasma levels of local anesthetic were not measured, as there were no symptoms or signs of systemic toxicity. In the unlikely event of a seizure taking place, our plan for airway control was to use an LMA.
The volume of local anesthetic solution required to extend the block to the T4 dermatome was in part because of the choice of the T11-12 interspace. The choice of a low thoracic approach was influenced by the difficulty in palpating the spinous processes of the midthoracic vertebrae. Pooling of local anesthetic solution in the midthoracic area secondary to kyphosis may also have increased volume requirements.
A further point of interest concerns the stability of the patient’s blood pressure even though the block extended to the upper thoracic dermatomes. Many studies have shown that reabsorption of small-dose epinephrine produces activation of β-adrenoceptors leading to hypotension and tachycardia. As the dose is increased, the effects that are seen depend on the ratio of α to β responses in different vascular beds. Systemic absorption of amounts larger than 1.5 mg epinephrine given subcutaneously is likely to produce an α-adrenoceptor effect, which tended to maintain blood pressure in this case (18).
It is important to stress that our patient presented very cogent reasons for having surgery. Given the nature of her anesthetic history, we would not have felt justified in proceeding with the case if the procedure had been purely cosmetic. As a result of her large breast mass, however, our patient’s exercise program—her principal interest outside of her work—was severely curtailed. Surgery was intended to restore this activity, and hence this operation had a major functional component. Finally, we wish to emphasize the importance of the team approach when dealing with challenging anesthetic problems of this nature.
1. Klippel M, Feil A. Un cas d’absence des vertebres cervicales avec cage thoracique remontant jusqu’a la bas du crane. Nouvelle Iconographie de la Salpetierre 1912; 25: 223–50.
2. McBride WZ. Klippel-Feil syndrome. Am Fam Physician 1992; 45: 633–5.
3. Hensinger RN, Lang JE, MacEwen GD. Klippel-Feil syndrome; a constellation of associated anomalies. J Bone Joint Surg Am 1974; 56: 1246–53.
4. Elster AD. Quadriplegia after minor trauma in the Klippel-Feil syndrome. A case report and review of the literature. J Bone Joint Surg Am 1984; 66: 1473–74.
5. David DJ, Edwards RM. Klippel-Feil syndrome [letter]. Anaesth Intensive Care 1995; 23: 752.
6. Louw JA, Albertse H. Traumatic quadriplegia after minor trauma in the Klippel-Feil syndrome [letter]. S Afr Med J 1987; 72: 889–90.
7. Naguib M, Farag H, Ibrahim Ae. Anaesthetic considerations in Klippel-Feil syndrome. Can Anaesth Soc J 1986; 33: 66–70.
8. Brechner VL. Unusual problems in the management of airways: I. Flexion-extension mobility of the cervical vertebrae. Anesth Analg 1968; 47: 362–73.
9. Daum RE, Jones DJ. Fibreoptic intubation in Klippel-Feil syndrome. Anaesthesia 1988; 43: 18–21.
10. Burns AM, Dorje P, Lawes EG, Nielsen MS. Anaesthetic management of caesarean section for a mother with pre-eclampsia, the Klippel-Feil syndrome and congenital hydrocephalus. Br J Anaesth 1988; 61: 350–4.
11. Dresner MR, Maclean AR. Anaesthesia for caesarean section in a patient with Klippel-Feil syndrome: the use of a microspinal catheter. Anaesthesia 1995; 50: 807–9.
12. Samsoon GL, Young JR. Difficult tracheal intubation: a retrospective study. Anaesthesia 1987; 42: 487–90.
13. Tsui BC, Gupta S, Finucane B. Confirmation of epidural catheter placement using nerve stimulation. Can J Anaesth 1998; 45: 640–4.
14. Tsui BC, Seal R, Entwistle L. Thoracic epidural analgesia via the caudal approach using nerve stimulation in an infant with CATCH22. Can J Anaesth 1999; 46: 1138–42.
15. Nagib MG, Maxwell RE, Chou SN. Identification and management of high-risk patients with Klippel-Feil syndrome. J Neurosurg 1984; 61: 523–30.
16. Greengrass R, O’Brien F, Lyerly K, et al. Paravertebral block for breast cancer surgery. Can J Anaesth 1996; 43: 858–61.
17. Finucane BT, Sandler AN, McKenna J, et al. A double-blind comparison of ropivacaine 0.5%, 0.75%, 1.0% and bupivacaine 0.5%, injected epidurally, in patients undergoing abdominal hysterectomy. Can J Anaesth 1996; 43: 442–9.
18. Hoffman BB, Lefkowitz RJ. Catecholamines, sympathomimetic drugs and adrenergic receptor antagonists. In: Hardman JG, Limbird LE, Molinoff PB, et al., eds. Goodman and Gilman’s: The pharmacological basis of therapeutics. 9th ed. New York: McGraw-Hill, 1996: 199–248.