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Year : 2014  |  Volume : 58  |  Issue : 3  |  Page : 341-343  

Klippel-Feil syndrome and neuraxial anaesthesia

Department of Anaesthesiology and Critical Care, Maulana Azad Medical College and Associated Lok Nayak Hospital, New Delhi, India

Date of Web Publication23-Jun-2014

Correspondence Address:
Dr. Sukhyanti Kerai
H. No. 53, Village Sultanpur Majra, Sultanpuri, New Delhi - 110 086
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0019-5049.135081

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How to cite this article:
Kerai S, Saxena K N, Taneja B. Klippel-Feil syndrome and neuraxial anaesthesia. Indian J Anaesth 2014;58:341-3

How to cite this URL:
Kerai S, Saxena K N, Taneja B. Klippel-Feil syndrome and neuraxial anaesthesia. Indian J Anaesth [serial online] 2014 [cited 2021 Jun 25];58:341-3. Available from: https://www.ijaweb.org/text.asp?2014/58/3/341/135081

   Introduction Top

Klippel-Feil syndrome (KFS) is an uncommon congenital disorder characterised by fusion of two or more cervical vertebrae. The perioperative anaesthetic management in these patients is complicated by anatomical changes, which presupposes the presence of difficult airway and by presence of multiple associated congenital anomalies. Literature search for anaesthetic management of KFS patient showed paucity of reports employing neuraxial technique.

We report successful management of a 57-year-old male of KFS for transurethral resection of prostrate (TURP) under spinal anaesthesia.

   Case Report Top

A 57-year-old male with benign prostrate hyperplasia presented for TURP. Airway examination revealed very short neck with limited flexion-extension and sideways neck movement. Mouth opening was adequate with a modified Mallampati Class 4 airway. He also had a low posterior hairline and scoliosis in the upper thoracic spine with increased lumbar lordosis. The clinical findings of short neck, limited neck movement, low posterior hair line and associated scoliosis led to suspicion of KFS [Figure 1] and X-rays of cervical spine-lateral and anterior-posterior views were obtained, which revealed complete fusion of C1-C2, C6-C7 and incomplete fusion of C4-C5 vertebrae [Figure 2]. The rest of systemic examination and investigations were normal. The surgery was planned under combined spinal epidural (CSE) anaesthesia. Standard preoperative preparations were carried out and a difficult airway cart was kept ready. With patient in the sitting position and under all aseptic precautions, CSE was attempted through midline approach initially by senior resident and then by senior anaesthesiologist, first in L3-L4 and then L4-L5 interspaces using CSE kit,(BD Durasafe TM CSE mini-kit) but was unsuccessful. Subsequent attempt was made by paramedian approach and epidural space was located at a depth of 5 cm in L4-L5 interspace. Subarachnoid block was then achieved with 1.8 mL of 0.5% hyperbaric bupivacaine using 27G BD TM Whitacre spinal needle. The epidural catheter was fixed at 8 cm at skin and a sensory block of T10 level was achieved within 15 min.
Figure 1: Patient of Klippel-Feil syndrome showing short neck and low posterior hairline

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Figure 2: Lateral view X-ray of lumbar spines

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The intra- and post-operative period was uneventful. The epidural catheter was removed the next day. X-ray lumbar spine was obtained in view of encountered difficulty in CSE through mid-line approach, which revealed complete fusion of L1-L2 and incomplete fusion of L2-L3 vertebrae [Figure 3]. The patient was discharged after 3 days with no neurological sequelae.
Figure 3: Lateral and anterior-posterior view of cervical spines

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   Discussion Top

Klippel-Feil syndrome is an inherited autosomal dominant disorder characterised by a triad of short neck, severe restriction of neck movements and a low posterior hairline. There are three different types of KFS depending on location and amount of fusion of vertebrae; [1] Type 1 - massive cervical fusion, which may involve upper thoracic spines, Type 2 - fusion of cervical vertebrae at 1 or 2 interspaces and may be associated other vertebral anomalies and Type 3 - lumbar or thoracic spine fusion associated with Type 1 or 2 as seen in our case.

Klippel-Feil syndrome is commonly associated with various other congenital anomalies: scoliosis, sprengel deformity, renal abnormalities, deafness, synkinesis, and congenital heart diseases. [2]

The major anaesthetic considerations while managing a patient with KFS are spinal deformities, which can lead to anticipated difficult airway due to fused cervical vertebrae. [3] Majority of the cases reported have received general anaesthesia with awake fibreoptic intubation for airway management [3],[4] consequent to limited neck movement.

There are few case reports [5],[6],[7] of KFS patients undergoing surgery under regional anaesthesia (RA).

There are many concerns regarding neuraxial anaesthesia in patients of KFS. First, the patient positioning achieved to perform anaesthetic technique can be suboptimal due to fusion of cervical and/or thoracic/lumbar vertebrae and associated scoliosis. The exaggerated lumbar lordosis in our patient caused difficulty in optimal positioning. Second, these patients are at increased risk of sustaining transient neurological deficit after minor trauma. [8] This is due to the fused segments and the resultant altered mechanical force transfer that makes the adjacent non-fused segments excessively mobile. Therefore, they need to be positioned very carefully avoiding excess force at neck and back during spinal/epidural block. Third, the associated spine anomalies like kyphosis and exaggerated lumbar lordosis make the intrathecal spread of drug unpredictable. Fourth, the patients of KFS Type 3 have fused lumbar/thoracic vertebrae, which can pose difficulty in administrating neuraxial techniques.

There are reports of encountering difficulties in administrating neuraxial anaesthesia in these patients. Hsu et al. [5] planned CSE for caesarean section in a patient of Type 3 KFS, but managed to obtain epidural anaesthesia only. Smith and Ray [7] encountered a dural puncture while performing lumbar epidural catheter insertion for caesarean section and reported success in second attempt. O'Connor et al. [6] have reported epidural space confirmation for catheter placement by electrical stimulation using an electrocardiogram adapter.

In our case, the decision of proceeding with CSE anaesthesia was made taking into consideration both nature of surgery and acceptance of patient. RA is technique of choice for TURP; it offers several advantages over general anaesthesia. In addition, our patient had associated scoliosis and by choosing RA, general anaesthesia with tracheal intubation and interference with lung dynamics, was avoided. The awake fibreoptic guided intubation would have been safest technique for managing this patient. Its success largely depends on patient's cooperation, availability of instruments and our patient was not comfortable in undergoing intubation while awake so we decided to proceed with CSE. Even though a careful examination of back should be performed in all patients in whom RA is contemplated and in KFS, a preoperative X-ray of thoracic and lumbar spines is helpful in selecting best intervertebral space for RA, especially Type 3 KFS patients. When available, ultrasound imaging would be preferable for identification of the epidural space.

As with any other case of anticipated difficult airway, RA for a patient of KFS may require emergent airway management in situations of high spinal anaesthesia or inadequate block when these cases are performed under RA. In emergency situations, airway may be secured with intubating laryngeal mask airway or other supraglottic devices which require less manipulation of cervical joints.

   Conclusion Top

Regional anaesthesia in a case of KFS can be safely performed in surgeries where low level block is required. This report highlights the need for careful preoperative clinical and radiological spine evaluation to identify patients with potential difficulty and planning to manage the anticipated problems in performing RA.

   References Top

1.Kaplan KM, Spivak JM, Bendo JA. Embryology of the spine and associated congenital abnormalities. Spine J 2005;5:564-76.  Back to cited text no. 1
2.Mahirogullari M, Ozkan H, Yildirim N, Cilli F, Güdemez E. Klippel-Feil syndrome and associated congenital abnormalities: Evaluation of 23 cases. Acta Orthop Traumatol Turc 2006;40:234-9.  Back to cited text no. 2
3.Khawaja OM, Reed JT, Shaefi S, Chitilian HV, Sandberg WS. Crisis resource management of the airway in a patient with Klippel-Feil syndrome, congenital deafness, and aortic dissection. Anesth Analg 2009;108:1220-5.  Back to cited text no. 3
4.Singh M, Prasad R, Jacob R. Anaesthetic challenges in a patient with Klippel-Feil syndrome undergoing surgery. Indian J Anaesth 2005;49:511-4.  Back to cited text no. 4
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5.Hsu G, Manabat E, Huffnagle S, Huffnagle HJ. Anesthetic management of a parturient with type III Klippel-Feil syndrome. Int J Obstet Anesth 2011;20:82-5.  Back to cited text no. 5
6.O'Connor PJ, Moysa GL, Finucane BT. Thoracic epidural anesthesia for bilateral reduction mammoplasty in a patient with Klippel-Feil syndrome. Anesth Analg 2001;92:514-6.  Back to cited text no. 6
7.Smith KA, Ray AP. Epidural anesthesia for repeat cesarean delivery in a parturient with Klippel-Feil syndrome. J Anaesthesiol Clin Pharmacol 2011;27:377-9.  Back to cited text no. 7
[PUBMED]  Medknow Journal  
8.Strax TE, Baran E. Traumatic quadriplegia associated with Klippel-Feil syndrome: Discussion and case reports. Arch Phys Med Rehabil 1975;56:363-5  Back to cited text no. 8


  [Figure 1], [Figure 2], [Figure 3]


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