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Anesthesia for percutaneous nephrolithotomy in a case of Kartagener’s syndrome

Bhosale, Guruprasad P; Shah, Veena R

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Journal of Anaesthesiology Clinical Pharmacology: Apr–Jun 2013 - Volume 29 - Issue 2 - p 280-282
doi: 10.4103/0970-9185.111741
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Dear Editor,

Kartagener’s syndrome is an autosomal recessive disorder characterized by clinical triad of situs inversus including dextrocardia, bronchiectasis, and sinusitis.[12] We report the anesthetic management of a patient with Kartagener’s syndrome posted for percutaneous nephrolithotomy (PCNL) under general anesthesia.

A 25-year-old male was admitted to the hospital with pain in the left flank and burning micturition. He also had history of repeated respiratory infections, chronic sinus infections, and bronchospasm. Chest X-ray showed dextrocardia, cystic bronchiectasis predominantly in left base and stomach bubble on right side. Ultrasound abdomen showed situs inversus. Both X-ray Kidney Ureter Bladder film and ultrasound abdomen revealed a staghorn calculus in left kidney with moderate hydronephrosis. Electrocardiogram (ECG) demonstrated sinus rhythm with lead I displaying inversion of P wave, deep Q wave and inversion of T wave. V1 to V6 leads demonstrated progressively decreasing amplitude of R wave. The ECG was normal when all the leads were reversed and the 2D echocardiography was also normal. Pulmonary function tests revealed FEV1 (Forced Expiratory Volume in one second) of 65% of predicted, FVC (Forced Vital Capacity) of 91% of predicted and FEV1/FVC ratio of 73%. Spirometry showed improvement after bronchodilator therapy. Pre-operative arterial blood gas analysis showed pH 7.33, PaO2 87 mm Hg, PaCO2 48 mm Hg and HCO3 22 mmol/L. Based on the presence of classical triad, Kartagener’s syndrome was diagnosed during workup for the surgery. During pre-anesthetic visit, patient had cough with whitish viscous expectoration for which he received vigorous chest physiotherapy and postural drainage. He was also given nebulization with salbutamol and fluticasone twice a day. Parenteral antibiotics and incentive spirometry were started pre-operatively.

The patient was shifted to operation theatre for left PCNL under general anesthesia. Intraoperative monitoring included ECG with all leads reversed, pulse oximetry, noninvasive blood pressure, temperature, capnography and neuromuscular monitoring. Midazolam 2 mg and inj. fentanyl 2 μg/kg IV were given as premedication. He was pre-oxygenated and anesthesia induced with propofol 2.5 mg/kg IV. Two minutes before intubation, intravenous lignocaine was given in order to suppress the airway reflexes. After muscle relaxation with succinylcholine, the trachea was intubated. Anesthesia was maintained with oxygen, air and isoflurane with vecuronium as neuromuscular blocker. The end-tidal CO2 was maintained between 30 mm Hg and 35 mm Hg with the tidal volume of 10-12 ml/kg, Respiratory Rate of 10/min with I: E ratio of 1:2. Airway pressures were lower than 25 mm Hg and oxygen saturation was 100%. Humidification was maintained by heat and moisture exchanger. After intubation, patient was placed in prone position for surgery. Good hydration was maintained with crystalloids (Normal saline and ringer lactate solution) 2.2 L. Total duration of surgery was 2 h and 20 min and consisted of two kidney punctures including one supracostal puncture. At the end of surgery, two nephrostomy tubes were placed and analgesia was given with infiltration of nephrostomy tract with bupivacaine 0.5% 20 ml solution. Neurouscular blockade was reversed with neostigmine 0.05 mg/kg and atropine 0.6 mg IV. Immediate post-extubation, oxygen saturation dropped to 93% which improved with throat suctioning with the help of laryngoscope. Paracetamol and ondansetron were given once in 8 h post-operatively.

Incentive spirometry and chest physiotherapy was started in the postanesthesia care unit (PACU). On 2nd post-operative day, patient developed an episode of bronchospasm with no new changes in repeat X-ray chest, which was treated with intravenous theophylline. Patient improved, shifted to the ward and was discharged on 5th post-operative day after removal of nephrostomy tubes.

Kartagener’s syndrome is a variant of immotile cilia syndrome which is an autosomal recessive disorder of microtubules of ciliated epithelial cells.[1] Because of the impairment of ciliary motility, patient with Kartagener syndrome can present with respiratory difficulty in the peri-operative period. Primary goals in the management would be assessment of cardiopulmonary function and prevention of pulmonary complications.[1] Our patient presented with repeated chest infections. Pre-operatively, thorough chest physiotherapy, postural drainage, inhaled bronchodilators, inhaled steroids and antibiotics helped. Patients with dextrocardia reveal a sinus rhythm when ECG leads are reversed as in our patient. When dextrocardia occurs with situs inversus, heart is mostly normal, without any evidence of congenital heart disease as in our case.[1]

In patients with history of bronchospasm, the incidence of wheezing is higher when thiopental is given for induction than in those given propofol.[3] The mechanism of the relative bronchodilating effect of propofol is not known. In our patient, we used propofol for intubation and to suppress airway reflexes prior to intubation intravenous lidocaine was given 2 min before tracheal intubation. Humidification was done with heat and moisture exchanger and good hydration was maintained to decrease the viscid secretions.

It is important to prevent lung complications by prevention of post-operative nausea and vomiting and good perioperative analgesia, in addition to proper suctioning in the perioperative period.[2] Whenever possible local or regional anesthesia is to be preferred over general anesthesia.[4] In our patient, because of the staghorn calculus in the renal pelvis, general anesthesia was planned. Furthermore, there is nasal cavity narrowing by chronic sinusitis in Kartagener’s syndrome, hence oropharyngeal airway is preferred over the nasal airway as was used in our case post-operatively.

Ventilatory parameters with large tidal volumes (10-15 ml/kg) combined with slow inspiratory flow rates as used in our case minimize the likelihood of turbulent airflow and help maintain optimal ventilation-to-perfusion matching.[5] Slow respiratory rates (6-10 breaths/min) provide sufficient time for complete exhalation to occur.

In summary, the anesthesiologist should be aware of the peri-operative complications and implications of cardiopulmonary inversion in patients of Kartagener’s syndrome.

References

1. Reidy J, Sischy S, Barrow V. Anaesthesia for Kartagener’s syndrome Br J Anaesth. 2000;85:919–21
2. Andoh T, Momota Y, Murata K, Kotani J. Difficult airway management for general anesthesia in two patients with Kartagener syndrome Masui. 2010;59:610–3
3. Wu RS, Wu KC, Sum DC, Bishop MJ. Comparative effects of thiopentone and propofol on respiratory resistance after tracheal intubation Br J Anaesth. 1996;77:735–8
4. Mathew PJ, Sadera GS, Sharafuddin S, Pandit B. Anaesthetic considerations in Kartagener’s syndrome: A case report Acta Anaesthesiol Scand. 2004;48:518–20
5. Al-Ruzzeh S, Kurup VHines RL, Marschall KE. Respiratory diseases Stoelting’s Anesthesia and Co-Existing Disease. 20126th ed Philadelphia Elsevier Saunders:181–217
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