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Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 15  |  Issue : 1  |  Page : 146-148  

Peripartum management of gitelman syndrome for vaginal delivery: A case report and review of literature


1 Department of Anaesthesiology and Pain Medicine, “Elena Venizelou” General Hospital of Athens, Athens, Greece
2 Department of Obstetrics/Gynecology, “Elena Venizelou” General Hospital of Athens, Athens, Greece

Date of Submission06-Jun-2021
Date of Acceptance04-Jul-2021
Date of Web Publication30-Aug-2021

Correspondence Address:
Dr. Konstantina Kalopita
Department of Anaesthesiology and Pain Medicine, “Helena Venizelou” General Hospital, 2 Helena Venizelou Square, GR 11521, Athens
Greece
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/aer.aer_82_21

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   Abstract 

We describe the anesthetic management of a spontaneous vaginal delivery at 38 weeks' gestation in a 36-year-old patient with Gitelman syndrome (GS). GS is a rare autosomal recessive renal tubulopathy characterized by hypomagnesemia, hypocalciuria, and secondary aldosteronism, which results in hypokalemia and metabolic alkalosis. To minimize any increase in catecholamine levels and consequent risk of ventricular arrhythmias, a labor epidural analgesia was administered using ropivacaine and fentanyl, along with intravenous magnesium and potassium supplementation. Ropivacaine was substituted for routine bupivacaine to decrease the risk of drug-induced cardiotoxicity. In the event of a cesarean section, the anesthetic plan was to continue with top-up epidural anesthesia and in case of failure, to convert to general anesthesia using propofol and rocuronium for induction. Delivery outcome was successful and uneventful.

Keywords: Epidural analgesia, Gitelman syndrome, hypokalemia, hypomagnesemia, pregnancy


How to cite this article:
Micha G, Kalopita K, Theodorou S, Stroumpoulis K. Peripartum management of gitelman syndrome for vaginal delivery: A case report and review of literature. Anesth Essays Res 2021;15:146-8

How to cite this URL:
Micha G, Kalopita K, Theodorou S, Stroumpoulis K. Peripartum management of gitelman syndrome for vaginal delivery: A case report and review of literature. Anesth Essays Res [serial online] 2021 [cited 2021 Nov 30];15:146-8. Available from: https://www.aeronline.org/text.asp?2021/15/1/146/325035


   Introduction Top


Gitelman syndrome (GS) is a rare autosomal recessive renal tubulopathy characterized by hypomagnesemia, hypocalciuria, and secondary aldosteronism, resulting in hypokalemia and metabolic alkalosis.[1] It is a variant of a group of renal diseases termed Bartter syndrome.[1] The syndrome is recessively inherited, caused by inactivating mutations in the SLC12A3 gene that encodes the thiazide-sensitive sodium chloride cotransporter expressed in the apical membrane of cells lining the distal convoluted tubule.[1] Sudden cardiac arrest has been described and long QT syndrome may be present.[2]

Several cases of anesthetic management of GS have been reported, but none in a parturient in active labor.[3],[4],[5],[6]


   Case Report Top


A 36-year-old primigravida (height 160 cm; weight 78 kg; BMI 30.5 kgm− 2) previously diagnosed with GS based on genetic testing presented in active labor at 38 weeks of gestation.

One month prior to delivery, she was evaluated at the obstetric anesthesia high-risk clinic. After discussion between the anesthetist, cardiologist, nephrologist, and obstetrician, it was decided it was reasonable to proceed with labor epidural analgesia to reduce stress.

On preanesthetic examination, no other medical history was recorded. Cardiopulmonary auscultation revealed no abnormality and airway examination was normal. Although she described paresthesia in the face in the past, the diagnosis was incidental. She had regular antenatal care from the nephrologist and oral supplementation with magnesium, potassium, and chloride kept her symptom free.

On admission to the labor ward, continuous cardiotocography, electrocardiography (ECG), oxygen saturation, and respiratory rate monitoring were commenced and continued throughout her stay. An arterial line was inserted to facilitate continuous blood pressure monitoring and arterial blood gas (ABG) sampling.

The obstetrician examined the patient and found her cervix was dilated 3–4 cm, fully effaced; the decision was made to place an epidural catheter. The epidural space was located at L2-3 vertebral interspace using an 18-gauge Tuohy needle with loss of resistance to saline. An epidural catheter was threaded 5 cm into the epidural space. After a negative test dose of lidocaine 40 mg, 12 mL of 0.2% ropivacaine and fentanyl 2 mcg.mL−1 were injected incrementally over a 20-min interval. Effective analgesia extended bilaterally to T10 and was maintained during labor with five further top-ups.

She was hemodynamically stable with minimal changes in blood pressure throughout labor. Four hours later, a healthy neonate weighing 3.2 kg was delivered spontaneously with Apgar scores of 9 and 10 at 1 and 5 min. She received a total of 1500 mL Hartmann's solution intraoperatively for a total estimated blood loss of 500 mL. Postoperatively, she was monitored for 6 h in the recovery room. An ABG sample demonstrated pH of 7.48 with normal gas exchange and electrolyte values within the normal range apart from potassium (3.2 mmol.L−1). She received supplemental intravenous potassium and had her serum electrolyte concentrations evaluated three times a day. A postdelivery ECG revealed no arrhythmias. Given that she was stable, the uterus well contracted, and her renal function normal, analgesia was optimized using regular analgesics. She was discharged home 72 h postdelivery.


   Discussion Top


GS was first reported by Gitelman in 1966, characterized by hypokalemia, hypomagnesemia, hypocalciuria, and metabolic alkalosis.[1] GS incidence is about ~25 per million.[1] Clinical manifestations include muscle cramps, tiredness, tetany, nausea, vomiting, polyuria, nocturnal diuresis, and heart abnormalities.[7]

Our review of the literature revealed several cases of parturients with effective obstetric management and neonatal outcome.[3],[7] Mascetti et al. in a case series reviewed five parturients with GS, with only the fifth pregnancy complicated by tiredness and tetanic seizures.[8] The latter could be attributed to hypomagnesemia and metabolic alkalosis.[8] According to a recent literature review by Yu et al.,[3] there were 57 reported pregnancies in 41 women affected with GS, among which 49 resulted in uneventful live births. Among them, only one parturient developed ventricular fibrillation on day 9 of hospitalization, which unfortunately was complicated by fetal demise and a refractory cardiac arrest to advanced cardiac life support protocols.[9]

The timing and mode of delivery are determined by obstetric factors. There are no contraindications for vaginal delivery in parturients with well-controlled serum level of potassium and magnesium.[7] Close monitoring of electrolytes is imperative and even more in the first trimester when their renal function undergoes significant changes. Despite normalization of serum levels of potassium and magnesium being under debate, the goal should focus on levels that allow patients to be asymptomatic.[10] Low serum magnesium levels may exacerbate arrhythmias caused by hypokalemia, so careful intravenous supplementation should be administered during labor. Potassium-sparing drugs such as amiloride and eplerenone are considered safe and are commonly preferred to spironolactone antenatally.[10],[11]

Anesthetic management should be focused at preoperative correction of electrolytes and acid–base disturbances as well as avoidance of trigger factors that prolong QTc interval.[2],[12] Continuous ECG monitoring during labor as well as baseline ABGs could be a safe strategy to follow.[4] Thus, regional anesthetic techniques may be preferable in these cases to avoid multidrug administration,[5] with meticulous attention to preserving normotension due to the implication of GS to blood pressure variation.[7],[13] In the present case, regular epidural top-ups were administered in order to ensure that the anesthetic block can be easily extended should the need for an urgent cesarean section arise. Ropivacaine was substituted for routine racemic bupivacaine to decrease the risk of low blood pressure and irregular heart rate.[14] Had general anesthesia been required, necessary precautions were made to avoid exposure to trigger agents that prolong QTc interval. Halogenated inhalational agents and especially sevoflurane should be avoided,[12] but isoflurane has been reported to be safe.[5] Thiopentone is considered safe in contrast to ketamine.[6] Succinylcholine can prolong QTc interval as well as pancuronium.[12] Antiemetic ondansetron should also be avoided.[12] In our case, total intravenous anesthesia was considered acceptable and rocuronium was our first-choice neuromuscular blocking drug. Long-acting uterotonics, such as carbetocin, should also be considered to reduce the risk of postpartum bleeding.[7]

Owing to the rarity of these high-risk pregnancies, a multidisciplinary approach is essential. In view of the anesthetic considerations of GS, a cesarean delivery might be considered safe, while vaginal birth should not be avoided in well-controlled GS parturients.

Informed consent

Written informed consent was obtained from the patient for publication of this case report and use of the patient data for research and educational purposes.

Declaration of patient consent

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

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
   References Top

1.
Knoers NV, Levtchenko EN. Gitelman syndrome. Orphanet J Rare Dis 2008;3:22.  Back to cited text no. 1
    
2.
Farmer JD, Vasdev GM, Martin DP. Perioperative considerations in patients with Gitelman syndrome: A case series. J Clin Anesth 2012;24:14-8.  Back to cited text no. 2
    
3.
Yu H, Liao H, Wang X, Tong Y. Outcome of twin pregnancy in a patient with Gitelman syndrome: A case report and literature review. Ann Palliat Med 2020;9:2361-6.  Back to cited text no. 3
    
4.
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. 4
    
5.
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.  Back to cited text no. 5
  [Full text]  
6.
Shah RB, Shah VR, Parikh GP, Vora KS. Anesthesia in a patient with gitelman syndrome. J Anaesthesiol Clin Pharmacol 2016;32:405-6.  Back to cited text no. 6
[PUBMED]  [Full text]  
7.
Zhang J, Liu F, Tu J. Gitelman syndrome in pregnancy: A case series. J Matern Fetal Neonatal Med 2020:1-6.  Back to cited text no. 7
    
8.
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. 8
    
9.
Elkoundi A, Kartite N, Bensghir M, Doghmi N, Lalaoui SJ. Gitelman syndrome: A rare life-threatening case of hypokalemic paralysis mimicking Guillain-Barré syndrome during pregnancy and review of the literature. Clin Case Rep 2017;5:1597-603.  Back to cited text no. 9
    
10.
Basu A, Dillon RD, Taylor R, Davison JM, Marshall SM. Is normalisation of serum potassium and magnesium always necessary in Gitelman Syndrome for a successful obstetric outcome? BJOG 2004;111:630-4.  Back to cited text no. 10
    
11.
de Haan J, Geers T, Berghout A. Gitelman syndrome in pregnancy. Int J Gynaecol Obstet 2008;103:69-71.  Back to cited text no. 11
    
12.
Fazio G, Vernuccio F, Grutta G, Re GL. 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. 12
    
13.
Scholl UI, Lifton RP. Molecular genetics of Gitelman's and Bartter's syndromes and their implication for blood pressure variation. In: Lifton RP, Somlo S, Giebisch GH, Seldin DW, editors. Genetic Diseases of the Kidney. New York, NY: Elsevier; 2009. p. 229-47.  Back to cited text no. 13
    
14.
Gao Y, Chen B, Zhang X, Yang R, Hua Q, Li B. The anesthetic bupivacaine induces cardiotoxicity by targeting L-type voltage-dependent calcium channels. J Int Med Res 2020;48:1-11.  Back to cited text no. 14
    




 

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