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LETTERS TO EDITOR
Year : 2019  |  Volume : 67  |  Issue : 3  |  Page : 898-899

Gitelman syndrome with Arnold–Chiari malformation for neurosurgery


Division of Neuroanesthesia, Department of Anesthesiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala, India

Date of Web Publication23-Jul-2019

Correspondence Address:
Dr. Ajay P Hrishi
Division of Neuroanesthesia, Department of Anesthesiology, 4th Floor, C Block, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum - 695 011, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.263189

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How to cite this article:
Nagmoti SV, Sethuraman M, Hrishi AP. Gitelman syndrome with Arnold–Chiari malformation for neurosurgery. Neurol India 2019;67:898-9

How to cite this URL:
Nagmoti SV, Sethuraman M, Hrishi AP. Gitelman syndrome with Arnold–Chiari malformation for neurosurgery. Neurol India [serial online] 2019 [cited 2019 Dec 15];67:898-9. Available from: http://www.neurologyindia.com/text.asp?2019/67/3/898/263189




Sir,

We present the anesthetic challenges in the management of a 22-year old male patient with Gitleman syndrome and Arnold– Chiari malformation More Details (ACM) who underwent foramen magnum decompression (FMD).

Gitelman syndrome is a rare autosomal recessive renal disorder in which thiazide-sensitive sodium chloride cotransporters and magnesium channels in the distal convoluted tubule are affected, resulting in hypokalemic metabolic alkalosis, hypomagnesemia, and hypocalcinuria.[1] The patients present with symptoms and signs such as muscle weakness, cramps, easy fatigability, abdominal pain, vomiting, chondrocalcinosis, and paraesthesia of face.[1] They are prone to ventricular tachyarrythmias and sudden cardiac death, especially in patients presenting with QT prolongation on electrocardiography (ECG).[1] ACM is a developmental anomaly which is characterized by downward displacement of cerebellar tonsils through the foramen magnum [Figure 1].
Figure 1: T2 weighted MRI sagittal section of posterior fossa and spine showing the herniating cerebellar tonsils and holocord syrinx

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It is associated with basilar invagination, platybasia, and syringomyelia.[2] Patients usually manifest with headache, neck pain, and paraesthesia of the limb.[2] Our search of literature did not reveal the anesthetic management of a patient with Gitelman syndrome and ACM, who is usually anesthetized for FMD to be perfomed in a prone position.

A 22-year old male patient presented with occipital headache and paraesthesia of right upper and lower limbs since 2 years, with magnetic resonance imaging of the brain and spine revealing Chiari malformation and holocord syrinx from the level of C2-T12 [Figure 1]. The surgical team proposed a FMD and C1 arch excision to relieve the symptomatology and prevent further neurological worsening. Laboratory investigations revealed a serum potassium of 1.8 mEq/L, serum magnesium of 1.4 mg/dL, and other electrolytes were within normal limits. A blood gas analysis was done, which showed severe uncompensated metabolic alkalosis. ECG revealed QTc prolongation with a baseline heart rate of 110 beats/min. No abnormalities were detected on preoperative echocardiography, ultrasound abdomen, and pulmonary function tests.

The attempt to optimize potassium and magnesium levels and to correct metabolic alkalosis before surgery with intravenous Inj. potassium chloride and Inj. magnesium sulfate along with Tab. spironolactone did not yield any significant change in serum levels. Thus, a nephrology consultation was asked for and a diagnosis of Gitelman syndrome was made. Clearance for surgery was given under elevated risk as neurological symptoms would have worsened without surgical intervention. It was proposed to avoid any large fluid shifts and to supplement intravenous potassium chloride and magnesium in the perioperative period.

On the day of surgery, after attaching the standard monitors, that is, the five lead ECGs, noninvasive blood pressure monitoring, and pulse oximetry, the patient was induced with Inj. thiopentone sodium 5 mg/kg, Inj. fentanyl 4 μg/kg, and Inj. vecuronium 0.1 mg/kg, given to facilitate tracheal intubation. The airway was secured with a 8.0 cuffed reinforced endotracheal tube aided by a C-MAC video laryngoscope. The left radial artery was cannulated for a beat-to-beat monitoring of blood pressure, and a peripherally inserted central line was secured through the left cubital vein. The patient was placed in a prone position keeping the head and neck in neutral position to avoid any movement of the cervical cord. Anesthesia was maintained with total intravenous anesthesia (TIVA) with Inj. propofol (100–200 μg/kg/h), Inj. fentanyl 1 μg/kg/h, and Inj. atracurium 0.01 mg/kg/h. Bispectral index monitoring was done to titrate TIVA; neuromuscular monitoring was done for assessing the neuromuscular blockade; and, forced air warmer was used to maintain normothermia. The mean blood pressure was maintained between 70 and 80 mmHg and end tidal (Et) CO2 between 35 and 40 mmHg. The surgery lasted for 4 h with a urine output of 800 mL and a blood loss of 400 mL, which was within the allowable blood loss limit. 20 mEq of potassium chloride and 1 g of magnesium sulphate were administered intravenously in the intraoperative period. At the end of surgery, the patient was made supine, neuromuscular blockade was reversed with Inj. glycopyrrolate 0.01 mg/kg and Inj. neostigmine 0.05 mg/kg, and the patient was extubated after return of spontaneous, adequate, and regular respiration at a train of four (TOF) ratio of 0.9.

The occurrence of two syndromes in this patient required us to rule out systemic anomalies that might add to the anesthetic challenge. Anesthetic concerns included associated airway problems, electrolyte imbalance, increased sensitivity to neuromuscular blockers due to hypomagnesemia, preoperative ECG with QTc prolongation, risk of intraoperative arrhythmias, adverse cardiac events due to hypotension, and occult autonomic dysfunction associated with ACM.[2],[3] The goals of our anesthetic management were avoidance of drugs that prolong the QT interval such as sevoflurane and ondansetron, prevention of hypotension, avoidance of hyperventilation, and thus respiratory alkalosis. Other concerns included minimizing neck extension during intubation which can aggravate tonsillar herniation and brain stem compression using a C-Mac video laryngoscope, and also the prevention of complications of a prone position.[3] Perioperative supplementation of potassium and magnesium was required, as any further decline in their levels would not be tolerated by the patient, and may have resulted in tachyarrythmias and cardiac arrest. There is a very narrow therapeutic margin for maintaing serum potassium and magnesium levels, and rapid correction to normokalemia should be avoided as these normokalemic levels present as relative hyperkalemia for these patients and manifest as bradyarrythmia and asystole. Drugs which will cause prolongation of QT interval such as sevoflurane and ondansetron should be avoided in these patients as they can trigger arrythmias with existing QTc prolongation as seen in Gitelman syndrome. Perioperative blood transfusion, use of drugs such as succinylcholine, salbutamol, and calcium, which could alter the levels of potassium, should be administered cautiously. Adequate postoperative analgesia and any stimulus which results in hyperventilation have to be avoided as this will worsen the existing metabolic alkalosis in the absence of renal compensation in patients with Gitelman syndrome.

In addition, neuronal symptoms in Gitelman syndrome can overlap and are similar to symptoms of complications after Chiari malformation surgery. A careful review of the clinical history, a comprehensive preanesthetic evaluation, and an appropriate perioperative management will help in overcoming the challenges presented by patients with ACM and Gitelman syndrome.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
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. 1
    
2.
Anand LK, Malhotra A, Saroa R, Kazal S, Sarna R. Anesthetic management of a patient with syringomyelia and Arnold-Chiari malformation type I with autonomic dysfunction. Anaesth Pain Intensive Care 2015;19:408-11.  Back to cited text no. 2
    
3.
Aminoff MJ, Wilcox CS. Autonomic dysfunction in syringomyelia. Postgrad Med J 1972;48:113-5.  Back to cited text no. 3
    


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