Indian Journal of Anaesthesia  
About us | Editorial board | Search | Ahead of print | Current Issue | Past Issues | Instructions
Home | Login  | Users Online: 335  Print this pageEmail this pageSmall font sizeDefault font sizeIncrease font size    




 
 Table of Contents    
CASE REPORT
Year : 2020  |  Volume : 64  |  Issue : 6  |  Page : 524-526  

Anaesthesia for emergency caesarean section in a patient with Gitelman syndrome


Department of Anaesthesia and Critical Care, Amrita Institute of Medical Sciences, Kochi, Kerala, India

Date of Submission11-Jan-2020
Date of Decision06-Apr-2020
Date of Acceptance18-Apr-2020
Date of Web Publication01-Jun-2020

Correspondence Address:
Dr. Nitu Puthenveettil
Department of Anaesthesia and Critical Care, Amrita Vishwa Vidyapeetham, Kochi - 682 024, Kerala
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ija.IJA_40_20

Rights and Permissions
 


Gitelman syndrome is a rare autosomal recessive salt-losing disorder of renal tubules, which results in hypokalemic metabolic alkalosis. Associated hypomagnesaemia and hypocalcaemia are also seen. Gitelman syndrome is a variant of renal Bartter's syndrome. Anaesthetic management of these obstetric patients is challenging and requires a careful multidisciplinary approach. Close monitoring of potassium and magnesium levels and their supplementation is required to avoid complications to mother and baby.

Keywords: Anaesthesia, Gitelman syndrome, hypokalemia, obstetrics


How to cite this article:
Venugopalan S, Puthenveettil N, Rajan S, Paul J. Anaesthesia for emergency caesarean section in a patient with Gitelman syndrome. Indian J Anaesth 2020;64:524-6

How to cite this URL:
Venugopalan S, Puthenveettil N, Rajan S, Paul J. Anaesthesia for emergency caesarean section in a patient with Gitelman syndrome. Indian J Anaesth [serial online] 2020 [cited 2020 Jul 7];64:524-6. Available from: http://www.ijaweb.org/text.asp?2020/64/6/524/285530




   Introduction Top


Gitelman syndrome is a rare autosomal recessive salt-wasting disorder of renal tubules with an incidence of 1 in 40,000.[1] It is associated with hypomagnesaemia, hypocalcaemia and hypokalemic metabolic alkalosis. The defect is in thiazide-sensitive Na+ Cl co-transporter located in the distal convoluted tubules.[2] Gitelman syndrome is a variant of renal Bartter's syndrome. Bartter's syndrome results from mutation of transporters of ascending loop of Henle,[3] whereas Gitelman syndrome occurs due to a mutation in the solute carrier family 12, member 3, SLC12A3 gene, which encodes the co-transporter.[2]


   Case History Top


23-year-old G2A1, parturient at 35 weeks of gestation, a known case of Gitelman syndrome came to Obstetric out-patient department with a history of raised blood pressure. Two years back she was diagnosed with Gitelman syndrome by a consultant nephrologist. Since then she was on oral electrolyte supplementation and eplerenone 25 mg twice daily. Her investigations on admission showed potassium of 3.1 and magnesium of 1.2 meq L-1 respectively. After consulting with nephrologist oral potassium and magnesium correction was given. Five days after admission she started developing labour pain. Her investigations showed normal potassium and magnesium. Electrocardiogram (ECG) did not show any QT prologation. She was shifted to labour room and on examination was found to have cord prolapse. She was immediately taken up for caesarean section. As it was an emergency, a brief history and examination was done and general anaesthesia with rapid sequence induction was planned. On arrival in the operating room, monitoring was done with a 5-lead ECG, non-invasive blood pressure, and pulse oximetry. After securing IV access, rapid sequence induction was performed with 5 mg kg-1 thiopentone and 0.6 mg kg-1 of succinyl choline. She was intubated with 7 mm size endotracheal tube. Anaesthesia was maintained with oxygen, air and isoflurane and atracurium. Intraoperatively an arterial blood gas was sent, which showed pH of 7.35 with normal gas exchange, sodium of 132 mmol L-1, potassium of 3.4 mmolL-1, chloride of 106 mmol/L and calcium of 0.95 mmolL-1. Live baby was extracted, oxytocin 3U IV bolus followed by 3U/h infusion was started as per our institutional protocol. IV fentanyl 2μgkg-1 and anti-emetic ondansetron 4 mg was given in the operation theatre. Procedure was uneventful and patient was reversed and extubated on table. Postoperatively she was monitored for 6 h in the recovery room. She had an uneventful postoperative period and was discharged on third postoperative day.


   Discussion Top


Gitelman syndrome is a rare autosomal recessive renal tubular disease characterized by hypokalaemia, hypomagnesaemia, metabolic alkalosis and low urinary calcium excretion.[4] Presentation is usually seen in late childhood with parasthesia and generalized weakness.[2] There are case reports of chondrocalcinosis developing in these patients from severe hypomagnesaemia.[3] Other presenting symptoms include ataxia, vertigo, and blurred vision.[1],[2] Electrolyte disturbances make these patients prone to ventricular arrhythmias. QT prolongation is seen in approximately 50% of these patients.[2],[3] Even though function of renin-angiotensin-aldosterone is affected, blood pressure usually remains normal.[5]

Gitelman syndrome can affect women of child bearing age group.[5] Increased renin-angiotensin-aldosterone system activity associated with normal pregnancy causes loss of potassium and magnesium.[2] Increased foetal demands, hyper-emesis, diarrhoea during pregnancy may tilt the balance in pregnant patients with Gitelman syndrome.[4],[5] In a case series of five patients with Gitelman syndrome by Mascetti et al., four of them had uneventful pregnancy but one was complicated by fatigue and tetanic seizures.[6] Seizure could be due to hypomagnesemia and metabolic alkalosis.[7]

Multidisciplinary approach is required in the management of a parturient with Gitelman syndrome. Serial monitoring of potassium and magnesium levels have to be performed.[8] Preoperative ECG is necessary to rule out arrhythmias and QT prolongation. Oral supplementation of electrolytes during pregnancy may be required to ensure that these patients are asymptomatic. In some patients, correction of electrolyte levels cannot be achieved inspite of supplementation.[5],[9] Normalisation of electrolyte values is not recommended.[10] Magnesium depletion alone is less likely to cause arrhythmias but may exacerbate arrhythmias caused by hypokalemia. Intravenous supplementation may be required in labour and during caesarean section.[5] Treatment with aldosterone antagonists during pregnancy is controversial but sometimes used. Potassium-sparing drugs such as amiloride and eplerenone is preferred to spironolactone in pregnancy.[11]

There are no clear guidelines in the anaesthetic management of Gitelman syndrome. Regional anaesthetic techniques may be preferable over general anaesthesia to avoid polypharmacy and risk of failed intubation and aspiration, and it also provides better post-operative analgesia.[12] Epidural or combined spinal epidural anaesthesia could be used. Uneventful spinal anaesthesia for caesarean delivery in pregnant women with Gitelman syndrome have been reported.[2] We used general anaesthesia as our patient did not have QT prolongation and there was severe foetal distress. In patients with QT prolongation, anaesthetic drugs have to be selected carefully. Supine hypotension has to be avoided by the use of wedge under hip.[13] Thiopentone and propofol are considered safe. Ketamine is better avoided. Halogenated inhalational agents can prolong QT interval. Sefoflurane has to be avoided, but isoflurane has been reported to be safe. Succinyl choline can prolong QT, but this effect could be reversed by using alfentanyl along with it. Among muscle relaxants rocuronium, vecuronium and atracurium are safe, but pancuronium needs to be avoided. Antiemetic ondansetron has to be avoided. During positive pressure ventilation high airway pressures and Valsalva maneuver are to be avoided.[14] Complications that may occur peri-operatively include laryngeal spasm, stridor, paraesthesia, tetany, convulsions, and ventricular arrhythmias.[14] Hypotension during surgery usually responds to fluid resuscitation, electrolyte replacement and vasopressors.[6],[7] Stable parturient with near normal electrolytes posted for caesarean section does not require invasive lines; a baseline arterial blood gas (ABG) prior to procedure may be useful. Invasive lines and ICU care should be considered in patients with unstable potassium levels, symptomatic patients and high risk pregnancies. Continuous ECG monitoring during labour could be a safe strategy to follow since up to 50% of these patients are reported to have prolonged QT syndrome.

In conclusion, multidisciplinary team including obstetricians, endocrinologists, nephrologists, anaesthetists and neonatologists are required for good obstetric and neonatal outcome. Anaesthetic management of these parturients requires a detailed preoperative assessment, optimisation, serial monitoring of electrolytes and awareness of potential complications. In uncomplicated pregnancy with asymptomatic patients, near normal level of potassium and magnesium are considered acceptable.

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.
Scholl UI, Lifton RP. Molecular genetics of Gitelman's and Bartter's syndromes and their implications for blood pressure variation. In: Genetic Diseases of the Kidney. Vol13. 2009. p. 227-47.  Back to cited text no. 1
    
2.
Shanbhag S, Neil J, Howell C. Anaesthesia for caesarean section in a patient with Gitelman's syndrome. Int J Obstet Anesth 2010;19:451-3.  Back to cited text no. 2
    
3.
Knoers NV, Levtchenko EN. Gitelman syndrome. Orphanet J Rare Dis2008;3:22.  Back to cited text no. 3
    
4.
Çetik S, Calik NC, Ozisik L, Gul Oz S, Arici M. Gitelman syndrome diagnosed in a woman in the second trimester of pregnancy. EJCRIM 2019;6:001100. doi: 10.12890/2019_001100.  Back to cited text no. 4
    
5.
de Haan J, Geers T, Berghout A. Gitelman syndrome in pregnancy. Int J Gynaecol Obstet 2008;103:69-71.  Back to cited text no. 5
    
6.
Mascetti L, Bettinelli A, Simonetti GD, Tagliabue A, Syrén ML, Nordio F,et al. Pregnancy in inherited hypokalemic salt-losing renal tubular disorder. Obstet Gynecol 2011;117:512-6.  Back to cited text no. 6
    
7.
Shahzad MA, Mukhtar M, Ahmed A, Ullah W, Saeed R, Hamid M. Gitelman syndrome: A rare cause of seizure disorder and a systematic review. Case Rep Med 2019;2019:4204907. doi: 10.1155/2019/4204907.  Back to cited text no. 7
    
8.
Ferrandis CP, Gargallo MC, Granell GM. Anesthesia for cesarean section in Gitelman's syndrome. Rev Esp Anestesiol Reanim 2002;49:437-8.  Back to cited text no. 8
    
9.
Basu A, Dillon DS, Taylor R, Davison JM, Marshall SM. Is normalisation of serum potassium and magnesium always necessary in Gitelmansyndrome for a successful obstetric outcome? BJOG 2004;111:630-4.  Back to cited text no. 9
    
10.
Elbouajaji K, Blanchier D, Pourrat O, Sarreau M. Management of Gitelman syndrome during pregnancy reporting 12 cases. Nephrol Ther 2018;14:536-43.  Back to cited text no. 10
    
11.
Wu WF, Pan MJ. The outcome of two pregnancies in a patient with Gitelman syndrome: Case report and review of the literature. Matern Fetal Neonatal Med 2019;28:1-3.  Back to cited text no. 11
    
12.
Bhatia P, Chhabra S. Physiological and anatomical changes of pregnancy: Implications for anaesthesia. Indian J Anaesth 2018;62:651-7.  Back to cited text no. 12
[PUBMED]  [Full text]  
13.
Upadya M, Saneesh PJ. Anaesthesia for non-obstetric surgery during pregnancy. Indian J Anaesth 2016;60:234-41.  Back to cited text no. 13
[PUBMED]  [Full text]  
14.
Fazio G, Vernuccio F, Grutta G, Lo Re G. Drugs to be avoided in patients with long QT syndrome: Focus on the anaesthesiological management. World J Cardiol 2013;5:87-93.  Back to cited text no. 14
    




 

Top
 
  Search
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

 
  In this article
    Abstract
   Introduction
   Case History
   Discussion
    References

 Article Access Statistics
    Viewed357    
    Printed0    
    Emailed0    
    PDF Downloaded108    
    Comments [Add]    

Recommend this journal