Anaesthesia for Kartagener’s syndrome

J. Reidy*,1, S. Sischy2 and V. Barrow3

1Dunkeld Anaesthetic Practice PO Box 41203, Craighall 2024, Johannesburg, South Africa. 2Cardiothoracic ICU Morningside Mediclinic, Private Bag X6, Bryanston, South Africa. 3Sandton Mediclinic, Private Bag X1, Bryanston, South Africa*Corresponding author

Accepted for publication: June 5, 2000


    Abstract
 Top
 Abstract
 Introduction
 Case report
 Discussion
 References
 
Kartagener’s syndrome is a hereditary syndrome involving a combination of dextrocardia (situs inversus), bronchiectasis and sinusitis, transmitted as an autosomal recessive trait. We describe a patient who had three anaesthetics over a period of a few months. Discussion relates to anaesthetic considerations in the syndrome and to recent findings relating to the molecular mechanisms of left-right development.

Br J Anaesth 2000; 85: 919–21

Keywords: complications, Kartagener’s syndrome; complications situs inversus; anaesthesia, general


    Introduction
 Top
 Abstract
 Introduction
 Case report
 Discussion
 References
 
A 27-yr-old female presented for surgery three times. She had Kartagener’s syndrome.1 The first operation was a left middle lobectomy for bronchiectasis. Some months later she complained of abdominal pain and her second operation revealed an ovarian adenocarcinoma. This operation was complicated by an episode of postoperative pneumonia. Two weeks later she had a total abdominal hysterectomy with bilateral salpingo-oophorectomy.


    Case report
 Top
 Abstract
 Introduction
 Case report
 Discussion
 References
 
The first operation—left middle lobectomy
The patient’s main complaint was one of recurrent episodes of pneumonia. She also admitted to chronic sinusitis and volunteered the information that ‘her heart was on the wrong side but normal’. Examination revealed no clubbing; her apex beat was on the right side; auscultation of the heart sounds was normal and there were some crepitations and rhonchi mainly in the left axilla.

Chest x-ray revealed dextrocardia and a right-sided stomach bubble. ECG demonstrated sinus rhythm and inversion of the P wave in lead 1 with a deep Q wave and inversion of the T wave. CT scan showed bronchiectasis that was most prominent in the left middle lobe. Lung function tests were normal and it was not considered necessary to do a blood gas study. She was admitted to the clinic for physiotherapy and scheduled for left middle lobectomy.

Anaesthesia for thoracotomy and lobectomy was unremarkable apart from it seemed logical to use a right-sided double lumen tube as she had three lobes on the left and two on the right with the longer main bronchus on the right. General anaesthesia was induced with propofol and maintained with isoflurane and sufentanil. For neuromuscular block we used vecuronium. Routine monitoring was enhanced by inserting a radial arterial line. The trachea was intubated with a right-sided 37 FG Mallinkrodt double-lumen tube and the lungs ventilated with a Servo Elema 900C ventilator.

After surgery, the trachea was extubated and the patient returned to the ward. Her postoperative course was uneventful and she was sent home after a few days.

The second operation—diagnostic laparoscopy
A few months later after the lobectomy the patient presented to a gynaecologist with abdominal pain. We were not involved with her management at this time. She had a general anaesthetic for laparoscopy that revealed adenocarcinoma of the ovary. Her postoperative course was complicated by an episode of left lower lobe pneumonia.

The third operation—total abdominal hysterectomy and bilateral salpingo-oophorectomy
Two weeks after the laparoscopic examination, the patient presented for total abdominal hysterectomy and bilateral salpingo-oophorectomy. In view of the fact that her last procedure had been complicated by pneumonia, spinal anaesthesia was chosen. She was sedated with midazolam 5 mg i.v. Spinal anaesthesia was given through a 25-gauge pencil point needle using 12.5 µg of hyperbaric bupivacaine. A continuous propofol infusion was used for sedation. Surgery was performed through a Pfannenstiel incision. The operation and postoperative period was uneventful.


    Discussion
 Top
 Abstract
 Introduction
 Case report
 Discussion
 References
 
Kartagener’s syndrome is a variant of the immotile cilia syndrome.2 This has also been called primary ciliary dyskinesia and is a result of an autosomal recessive disorder of the microtubules of ciliated cells. Symptoms include male sterility, chronic or recurrent respiratory tract infection, and bronchiectasis because of the absence of mucociliary clearance. In 50% of patients situs inversus occurs and hence Kartagener’s syndrome.

It may become possible to link the occurrence of abnormal ciliary function and abnormal position of the body organs. It seems that genes determine the structure, function, and time of appearance of proteins in the embryo that influence normal development and siting of the internal organs asymmetrically (situs solitus). On the left side of the embryo, near Hensen’s node, a variety of proteins are secreted (Sonic Hedgehog, Nodal, Lefty and Pitx2). On the right side are others (Activin betaB, Snail and Fibroblast Growth factor-8).3 These normally influence rotations, that lead to a left-sided heart and the usual position and structure of the lungs and abdominal organs. Abnormal genes and so abnormal proteins would lead to malpositions. Normal ciliary motion in the mouse node is anticlockwise and conceivably influences the flow of the proteins to their correct sites in the embryo. In the immotile cilia syndrome it is possible that abnormal distribution of the proteins occurs. Is this the reason that the dextrocardia of Kartagener’s syndrome links with the immotile cilia syndrome, and also situs inversus?

The anaesthetic implications of Kartagener’s syndrome are varied. The anaesthetist might be involved with patients who have sinus surgery, pulmonary surgery, infertility investigations or possibly cardiac surgery. Of primary importance will be assessment of pulmonary and cardiac structure and function, and also prevention of pulmonary complications in the bronchiectatic patient.

Physiotherapy, postural drainage, antibiotics, bronchodilators and incentive spirometry all have a role perioperatively. Where possible, local or regional anaesthesia is to be preferred to general anaesthesia. In thoracic surgery, the anatomy of the bronchi should be considered before selecting a double lumen tube.

Knowledge of the position of the abdominal organs and of the branching pattern of the main stem bronchi is important in categorizing malpositions; and these must be borne in mind. When dextrocardia occurs with situs inversus, the heart is most frequently normal. When dextrocardia occurs without situs inversus, when the visceral situs is indeterminate (situs ambiguus), or if isolated levocardia is present, associated, often complex, multiple anomalies are usually present.4 Dextrocardia with complete situs inversus occurs in approximately 2 per 10 000 births. The incidence of congenital heart disease is low being about 3%. Kartagener’s syndrome will occur in about 20% of patients.5 Thus, the incidence being approximately 1 in 50 000 births. Our patient did not have an echocardiogram and on clinical grounds was assumed to have a totally normal heart.

In contrast, dextrocardia with situs solitus or situs ambiguus is less common (1 per 20 000 births) and the incidence of congenital heart disease is extremely high, probably 90% or greater. Dextrocardia with situs solitus usually, although not invariably, associates with severe complex cardiac abnormalities. They are most commonly transposition of the great arteries, double outlet right ventricle, ventricular septal defect, single ventricle and pulmonary stenosis or artresia.5 In patients with dextrocardia and situs ambiguus, polysplenia or asplenia may be present in association with complex multiple cardiac abnormalities. These include a combination of systemic and pulmonary venous abnormalities, defects in the ventricular and atrial septa and endocardial cushion defects.

There may be pulmonary artery obstruction and maldevelopment of the great arteries.6

The incidence of isolated levocardia (a left-sided heart) with situs inversus is about 0.6% per 10 000 births and more than 90% have serious heart disease.5


    References
 Top
 Abstract
 Introduction
 Case report
 Discussion
 References
 
1 Kartagener M. Zur pathogenese der bronchiektasien. 1. Bronchiektasien bei situs viscerum inversus. Bietr Kli Tuberk 1933; 83: 489

2 Rubin BK. Immotile cilia syndrome (primary ciliary dyskinesia) and inflammatory lung disease. Clin Chest Med 1988; 9: 657–68[ISI][Medline]

3 Belmonte JCI. How the body tells left from right. Sci Am 1999; June: 38–43

4 Friedman WF. Congenital heart disease in the adult. In: Isselbacher KJ, Braunwald E, eds. Harrison’s Principles of Medicine. 13th Edn. New York: McGraw-Hill Inc., 1994; 1046

5 Nugent EW. The pathology, abnormal physiology, clinical recognition, and medical and surgical treatment of congenital heart disease. In: Hurst, ed. The Heart. 7th Edn. New York: McGraw-Hill Inc., 1990; 178

6 Friedman WF. Congenital heart disease. In: Wintrobe MM, Thorn GW, eds. Harrison’s Principles of Medicine. 6th Edn. New York: McGraw-Hill Inc., 1970; 1182





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