Case report: Utilisation of the paramedian approach to epidural insertion in a case of klippel-feil syndrome

James R Skelly; Siaghal MacColgáin

doi:10.4103/JOACC.JOACC_72_21

CASE REPORT

Year : 2022 | Volume : 12 | Issue : 1 | Page : 56-58

Case report: Utilisation of the paramedian approach to epidural insertion in a case of klippel-feil syndrome

James R Skelly, Siaghal MacColgáin
Department of Anaesthetics, The National Maternity Hospital, Holles St., Dublin 2, Ireland

Date of Submission	16-Aug-2021
Date of Acceptance	12-Sep-2021
Date of Web Publication	14-Mar-2022

Correspondence Address:
Dr. James R Skelly
Department of Anaesthetics, The National Maternity Hospital, Holles St., Dublin 2
Ireland

Source of Support: None, Conflict of Interest: None

Check

DOI: 10.4103/JOACC.JOACC_72_21

Abstract

Klippel–Feil Syndrome (KFS) is a complex heterogeneous entity that can result in cervical spondylosis and thoracolumbar vertebral fusion. Combined, these features contribute to both a difficult airway and neuraxial anaesthesia. Previously, these patients required general anaesthesia in the obstetric setting, incorporating advanced airway techniques as the first line. Herein, we describe the novel use of the paramedian approach to epidural anaesthesia in a primigravid woman, with a background of KFS. The patient had a vaginal septum and double cervix and was considered at higher risk of obstetric complications. Antenatal assessment and forward planning within the multidisciplinary team setting were vital in the formulation and provision of safe care for this patient. Neuraxial ultrasound (US), undertaken at preassessment, yielded adequate views of the posterior complex in the paramedian sagittal oblique plane only. An anaesthetic plan with emphasis on early paramedian epidural insertion was thus formulated. The patient was admitted to early labour by the obstetric team. Neuraxial US replicated the previously attained windows and in keeping with the plan, the paramedian approach was utilised with successful insertion on the first attempt. Patient-controlled epidural anaesthesia infusion was utilised over her 6-hour labour with instrumental delivery. Epidural analgesia was maintained with a bilateral T6 sensory block with the patient comfortable throughout. This case study outlines the novel use of the paramedian approach to epidural anaesthesia in a patient with KFS. We also believe it exemplifies the need to pre-assess patients with abnormal spinal anatomy and utilises the neuraxial US to formulate an anaesthetic plan.

Keywords: Difficult airway, neuraxial analgesia, neuraxial ultrasound, obstetric anaesthesia, vertebral fusion

How to cite this article:
Skelly JR, MacColgáin S. Case report: Utilisation of the paramedian approach to epidural insertion in a case of klippel-feil syndrome. J Obstet Anaesth Crit Care 2022;12:56-8

How to cite this URL:
Skelly JR, MacColgáin S. Case report: Utilisation of the paramedian approach to epidural insertion in a case of klippel-feil syndrome. J Obstet Anaesth Crit Care [serial online] 2022 [cited 2022 May 19];12:56-8. Available from: https://www.joacc.com/text.asp?2022/12/1/56/339548

Introduction

Klippel–Feil syndrome (KFS) is a complex disorder with cervical spondylosis resulting in a limited range of motion and fusion of lumbar and thoracic vertebrae. These features lead to difficult airway and neuraxial techniques. Neuraxial anaesthesia has historically been avoided in these patients. This case outlines the novel use of the paramedian approach to epidural anaesthesia in a patient with KFS and abnormal spinal anatomy. In addition, it highlights the need to pre-assess these patients with the addition of neuraxial ultrasound (US) to formulate an anaesthetic plan for delivery.

Case Report

A 36-year-old primigravid woman, with KFS, was admitted to our institution on presentation with pains at 38 + 0 gestation.

She was previously assessed in the high-risk obstetric anaesthesia clinic. History, examination, and the neuraxial US were undertaken. The patient displayed micrognathia, a thyromental distance of 3 cm [Figure 1], Mallampati III, limited neck extension, thoracic kyphoscoliosis, marked lumbar lordosis with potential lumbar fusion and limited spinal mobility.

Figure 1: Original image. Lateral view of patient's cervical spine showing micrognathia and reduced thyromental distance

Click here to view

She underwent general anaesthesia (GA) in childhood, for nephrectomy, and was unaware of airway complications. Anaesthetic notes were unavailable upon request. Nil else was noted from her medical history.

On US, the median sagittal [Figure 2]a and transverse [Figure 2]b views of the posterior complex (PC) were unsatisfactory despite various attempts at image optimisation. The PC in the sagittal paramedian oblique plane was easily identified [Figure 2]c.

Figure 2: Original Image. US images of the lumbar spine in the seated position taken on post delivery day 1. Images were the best obtained after multiple attempts. PC (posterior complex), AC (anterior complex) in the (a)median sagittal, (b) median transverse and (c) paramedian sagittal oblique planes

Click here to view

An antenatal multidisciplinary team (MDT) meeting was undertaken. The patient was considered high risk for obstetric complications including caesarean section, due to a vaginal septum and double cervix. Considering her difficult spinal anatomy and described airway complications, she was at high risk for both failed neuraxial anaesthesia and airway complications.

Based on her pre-assessment and MDT discussions, an anaesthetic plan for labour emphasising early epidural insertion was formulated. The paramedian approach would be the first line based on her neuraxial US using a pre-puncture technique. If unsuccessful, the cathether would be placed under direct vision. Repeated epidural insertion attempts are associated with increased complications.^[1] In this case, the midline approach was potentially deleterious and likely unsuccessful. If unsuccessful, a remifentanil PCA would be commenced for labour analgesia. If the caesarean section was required, spinal anaesthesia was to be attempted, if there was no working epidural catheter in situ. If GA was required, awake fibreoptic intubation was Plan A.

Following the MDT meeting, it was deemed the patient would labour with a low threshold for a caesarean section. She was induced and transferred to the labour ward for an early epidural once labour was established.

On admission, neuraxial US replicated the previously attained images, in keeping with the plan, the paramedian approach was utilised marking the insertion point pre-puncture. The epidural space was identified and cathetherised on the first attempt. Epidural anaesthesia was maintained with a levobupivacaine/fentanyl patient-controlled epidural analgesia with a background infusion. Following a 6-hour labour, an instrumental delivery was performed. A bilateral T6 sensory block was maintained throughout.

Discussion

KFS is a rare but important syndrome possessing risk of both neuraxial failure and airway complications.

This case highlights the importance of neuraxial US in identifying the correct approach to neuraxial techniques in patients with KFS and other conditions with potential lumbar vertebral fusion.

We have described the care of a parturient with KFS and the novel utilisation of the paramedian approach to US visualisation of the PC and epidural insertion which has not been described in this cohort previously. This approach has previously been described in the setting of a bamboo spine.^[2] The successful paramedian approach, after failed neuraxial attempts in the midline, in the setting of ankylosing spondylitis, with a known difficult airway, has been described.^[3] These studies guided our care in this case.

Few case reports describing KFS in the obstetric setting exist. GA as first-line anaesthetic for caesarean section is the most described technique. This difficult airway cohort has been managed with awake fibreoptic intubations^[4],[5] and video laryngoscopy^[6] when undergoing GA for caesarean section.

These patients may also exhibit thoracolumbar vertebral fusion, along with cervical spine fusion and exaggeration of the spinal curvatures, all of which impede neuraxial techniques. Case reports in which spinal anaesthesia has been described for a parturient with KFS have been published^[7] adding to previously published work describing the use of epidural analgesia in this cohort.^[8] The use of combined spinal-epidural has been described for the elective section, but in this instance, the anaesthetic was inadequate.^[9] This concurs with previous reports which indicate that the degree of lumbar vertebral fusion varies significantly between patients,^[10] warranting early individual assessment.

A previous review also highlights the increased risk of spinal haematoma in ankylosing spondylitis, which is of relevance due to the shared characteristics of lumbar spinal fusion, and highlights the risk of repeated attempts.^[1] This exemplifies the necessity for the neuraxial US in preassessment in formulating a labour analgesic plan in those with difficult spinal anatomy. This will minimise repeated attempts in anatomically difficult planes and potentially haematomas.

Our strategy, of using the paramedian approach, was implemented based on our US imaging results at pre-assessment, this is in keeping with previous studies validating neuraxial anaesthesia in this cohort. This technique requires specialist skills.

The commonly held belief that these patients require GA incorporating advanced airway techniques as a first line in the obstetric setting can be challenged and should be considered on a case-by-case basis. These cases draw attention to the fact that complex patients need to be seen in the early antenatal period with a robust plan for their care devised.

This case study outlines the novel use of the paramedian approach to epidural anaesthesia in a patient with KFS. We also believe it exemplifies the importance of pre-assessing patients with abnormal spinal anatomy while utilising the neuraxial US to formulate an anaesthetic plan. Given our successful application of epidural analgesia via the paramedian approach, we advocate that a carefully titrated epidural is a viable initial step in the anaesthetic care of the labouring KFS patient as well as those undergoing caesarean section.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1.	Wulf H. Epidural anaesthesia and spinal haematoma. Can J Anaesth 1996;43:1260-71.
2.	Goyal R, Singh S, Shukla RN, Singhal A. Management of a case of ankylosing spondylitis for total hip replacement surgery with the use of ultrasound-assisted central neuraxial blockade. Indian J Anaesth 2013;57:69-71. [PUBMED] [Full text]
3.	Kumar CM, Mehta M. Ankylosing spondylitis: Lateral approach to spinal anaesthesia for lower limb surgery. Can J Anaesth 1995;42:73-6.
4.	Hase Y, Kamekura N, Fujisawa T, Fukushima K. Repeated anesthetic management for a patient with Klippel-Feil syndrome. Anesth Prog 2014;61:103-6.
5.	Broomhead CJ, Davies W, Higgins D. Awake oral fibreoptic intubation for caesarean section. Int J Obstet Anesth 1995;4:172-4.
6.	Sirico A, Maruotti G, Martinelli P, Lanna M, Anfora R, Setaro A, et al. Airway management with McGrath series 5 video laryngoscope in a woman with Klippel-Feil syndrome requiring urgent caesarean section. Int J Obstet Anesth 2015;24:286-8.
7.	Santonastaso DP, de Chiara A, Addis A, Pini R, Agnoletti V. Spinal anesthesia with a low dosage of local anesthetic for urgent cesarean delivery in a parturient with Klippel-Feil syndrome. J Clin Anesth 2019;52:78-9.
8.	Singh D, Mills GH, Caunt JA, Alderson JD. Anaesthetic management of labour in two patients with Klippel-Feil syndrome. Int J Obstet Anesth 1996;5:198-201.
9.	Kavanagh T, Jee R, Kilpatrick N, Douglas J. Elective cesarean delivery in a parturient with Klippel-Feil syndrome. Int J Obstet Anesth 2013;22:343-8.
10.	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.