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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 11  |  Issue : 2  |  Page : 115-117

Relapsing polychondritis in a primiparous women for elective lower segment caesarean section


Department of Anaesthesia, Singapore General Hospital, Singapore

Date of Submission22-Mar-2021
Date of Acceptance22-May-2021
Date of Web Publication01-Oct-2021

Correspondence Address:
Dr. Chi Ho Chan
Outram Rd - 169608
Singapore
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/JOACC.JOACC_24_21

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  Abstract 


Relapsing polychondritis is a rare multisystem autoimmune disorder characterized by recurrent, progressive inflammation and destruction of cartilaginous tissue. Respiratory involvement is the major cause of morbidity and mortality. Airway management during anaesthesia in these patients can be challenging and may result in failed oxygenation and death. Increased physiological demand during pregnancy further complicates anaesthesia planning. Collaborative management under a multidisciplinary team of obstetricians, obstetric anaesthetists, rheumatologist, and pulmonologist is essential. We report a case of a parturient with relapsing polychondritis and severe respiratory involvement for caesarean section under combined epidural-spinal anaesthesia and discuss the anaesthetic management based on current literature.

Keywords: Anaesthesia, caesarean, obstetric, parturient, relapsing polychondritis


How to cite this article:
Chan CH, Lu A, Mok MU. Relapsing polychondritis in a primiparous women for elective lower segment caesarean section. J Obstet Anaesth Crit Care 2021;11:115-7

How to cite this URL:
Chan CH, Lu A, Mok MU. Relapsing polychondritis in a primiparous women for elective lower segment caesarean section. J Obstet Anaesth Crit Care [serial online] 2021 [cited 2021 Dec 9];11:115-7. Available from: https://www.joacc.com/text.asp?2021/11/2/115/327403




  Introduction Top


Relapsing polychondritis (RP) is a rare multisystem autoimmune disorder characterized by recurrent, progressive inflammation and destruction of cartilaginous tissue. The disease involves multiple organs and poses significant challenges to anaesthesiologist during anaesthesia. Respiratory involvement may result in severe tracheal stenosis and tracheomalacia with airway collapse. Cardiovascular complications such as coronary artery diseases may also add complexity to hemodynamic control during anaesthesia. Anaesthesia considerations are further complicated by the increased physiological demand during pregnancy. We present a case and discuss the anaesthetic management of a patient with RP undergoing an elective lower segment caesarean section (LSCS).


  Case History Top


We present a case of a 40-year-old primiparous woman, 169 cm tall, and weighing 68.7 kg with a background of RP and significant respiratory involvement, who underwent a planned pregnancy and LSCS.

She was diagnosed with RP seven years prior with right pinna inflammation, tinnitus, hearing impairment, nose bridge inflammation, and severe airway obstruction. She had breathlessness on mild exertion but with no evidence of joint involvements or vasculitis. A pulmonary function test (PFT) [Figure 1] performed as part of her investigation for her breathlessness revealed severe obstructive lung disease with FEV1 of 1.17L (36% predicted), FVC of 1.8L (46% predicted) and FEV1/FVC of 65% (79% predicted). She was treated with Prednisolone, Azathioprine and Tacilizumab with good response. Tacilizumab was stopped before her planned pregnancy; Prednisolone and Azathioprine were continued throughout pregnancy. No advanced imaging was done. Transthoracic echocardiogram prior to pregnancy was unremarkable.
Figure 1: Flow-volume loop and Volume-time graph from pulmonary function test done prior to pregnancy showing severe airway obstruction with FEV1 of 1.17L (36% predicted), FVC of 1.8L (46% predicted) and FEV1/FVC of 65% (79% predicted), and a post-bronchodilator FEV1 of 1.14L, a -2% difference

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She was managed by a multidisciplinary team (MDT) of rheumatologist, obstetricians, respiratory physicians, and anaesthesiologist during her pregnancy. Her pregnancy was uneventful with no significant deterioration of respiratory function. In-view of maternal request and possible poor maternal effort associated with her obstructive lung disease during second stage of labour, MDT decided for elective LSCS at 37 weeks. The anaesthesia plan was to perform a combined spinal-epidural (CSE) anaesthesia which allowed fine titration of central neuraxial blockade level. If CSE was unsuccessful, a general anaesthesia (GA) via awake intubation technique will be performed. Cardiothoracic surgical (CTS) team was also notified in case of a need for extracorporeal membranous oxygenation (ECMO).

Surgery and anaesthesia were uneventful. CSE was performed at L3/4 using intrathecal hyperbaric bupivacaine 10 mg and fentanyl 15 mcg. Sensory level was at T8 after 10 min, however, rosed to T5 after Trendelenburg positioning and administration of 10 ml of epidural saline. Estimated blood loss was 500 mL and baby had APGAR score of eight and nine at one and five minutes, respectively. Intra-operative blood pressure was 100-130/50-65 mmHg, heart rate was 75-100 beats per minute, and oxygen saturation was above 98% throughout with no respiratory distress. Post-operatively, patient was closely monitored in the high dependency unit for hemodynamic and respiratory complications. Her post-operative recovery was unremarkable.


  Discussion Top


RP is a rare multisystem autoimmune disorder of unknown etiology, resulting in recurrent and progressive inflammation and destruction of the cartilaginous tissue of the body. The incidence of RP from population-based study is estimated to be 0.71-2.1 per million person-years.[1] In our local context, Singapore General Hospital reported only 10 cases of RP between 2005 and 2013.[2] The empirical diagnostic criteria based on clinical features, histology, and response to immunosuppressive therapy were described by McAdam et al., in 1976 and expanded by Damiani et al., in 1979. Diagnosis is challenging and delayed diagnosis is common.[3]

Patients with RP going for surgery can be challenging for anaesthetists. Vasculitis may cause coronary artery stenosis, valvular heart disease, aortic aneurysm, acute pericarditis, and arrhythmias, therefore invasive blood pressure monitoring and hemodynamic stable anaesthesia is a prerequisite.[3] Respiratory involvement includes airway stenosis, tracheomalacia, and recurrent pulmonary infections due to chronic inflammation and scarring resulting in destruction of the cartilaginous rings of larynx, trachea and bronchus, and disruption of mucociliary escalators. Case reports have described good laryngeal view and uneventful endotracheal intubations,[4],[5] however passing of the endotracheal tube (ETT) into the trachea can be difficult in severe diseases and tracheal stenosis.[6],[7] Local, regional anaesthesia, and epidural anaesthesia is considered safe in patients with RP and if not contraindicated, is the preferred choice. Kim et al.,[8] reported a case of open wedge resection of stomach under epidural anaesthesia which was uneventful. For patient that requires GA, a thorough pre-operative assessment delineates the severity of respiratory and cardiovascular involvement of RP. Pre-operative PFT identifies functional severity and computed tomography (CT) identifies anatomical tracheobronchial involvement. Awake intubation with fibreoptic bronchoscopy may be beneficial as it avoids airway collapse during induction of anaesthesia and ensures ETT is in the trachea before GA.

Furthermore, the extent of tracheobronchial involvement and tracheal narrowing can be assessed with bronchoscopy. Depending on the severity of the tracheal stenosis, the use of a smaller size ETT may be required. Our patient had poor PFT and no antenatal CT, hence we were uncertain of her RP severity and could only plan her as if she had severe respiratory involvement. In the event of GA and unable to intubate the trachea due to severe narrowing, CTS support was available for ECMO. However, she had reasonable respiratory function and the risk of needing ECMO was very low.

Management of patients with RP during pregnancy adds further complexity and must be managed by MDT. Early anaesthetic evaluation prior to delivery is necessary regardless of vaginal delivery or LSCS as time is needed for planning and preparation. There is no preferred mode of delivery in patients with RP and should be based on obstetric considerations. In a 25-year retrospective study by Papo, Wechsler et al.,[9] most of the patients underwent natural delivery uneventfully but none of their patients have severe respiratory tract involvement. The expected increase in respiratory and cardiovascular requirements and hormonal changes during pregnancy and labour may complicate and exacerbates the disease. In patients with RP going for normal delivery, epidural analgesia has benefit in reducing stress of labour as this could compromise cardiac output, work of breathing, and increase oxygen consumption. When LSCS is indicated, central neuraxial anaesthesia is the preferred mode of anaesthesia as it avoids airway manipulation, especially for RP patients with severe respiratory involvement.

Acknowledgement

Nil.

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 Top

1.
Hazra N, Dregan A, Charlton J, Gulliford MC, D'Cruz DP. Incidence and mortality of relapsing polychondritis in the UK: A population-based cohort study. Rheumatology (Oxford) 2015;54:2181-7.  Back to cited text no. 1
    
2.
Chuah TY, Lui NL. Relapsing polychondritis in Singapore: A case series and review of literature. Singapore Med J 2017;58:201-5.  Back to cited text no. 2
    
3.
Damiani JM, Levine HL. Relapsing polychondritis--report of ten cases. Laryngoscope 1979;89(6 Pt 1):929-46.  Back to cited text no. 3
    
4.
McAdam LP, O'Hanlan MA, Bluestone R, Pearson CM. Relapsing polychondritis: prospective study of 23 patients and a review of the literature. Medicine (Baltimore) 1976;55:193-215.  Back to cited text no. 4
    
5.
Tanaka TT, Furutani HF, Harioka TH. Anaesthetic management of a patient with relapsing polychondritis undergoing laparoscopic surgery. Anaesth Intensive Care 2006;34:372-4.  Back to cited text no. 5
    
6.
Fitzmaurice BG, Brodsky JB, Kee ST, Foppiano LE, McNutt J. Anaesthetic management of a patient with relapsing polychondritis. J Cardiothorac Vasc Anaesth 1999;13:309-11.  Back to cited text no. 6
    
7.
Hayward AW, al-Shaikh B. Relapsing polychondritis and the anaesthetist. Anaesthesia 1988;43:573-7.  Back to cited text no. 7
    
8.
Kim IK, Kim MS, Choi YS, Shin YS. Anaesthetic experience of a patient with relapsing polychondritis -A case report. Korean J Anaesthesiol 2012;63:465-8.  Back to cited text no. 8
    
9.
Papo T, Wechsler B, Bletry O, Piette AM, Godeau P, Piette JC. Pregnancy in relapsing polychondritis: Twenty-five pregnancies in eleven patients. Arthritis Rheum 1997;40:1245-9.  Back to cited text no. 9
    


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