Brazilian Journal of Anesthesiology
https://bjan-sba.org/article/doi/10.1016/j.bjane.2023.10.002
Brazilian Journal of Anesthesiology
Letter to the Editor

Primary ciliary dyskinesia: a case of complete Kartagener's syndrome in a patient undergoing cesarean section

Paula Daniele Lopes da Costa, Thaiza Oliveira Marinho, Norma Sueli Pinheiro Módolo, Paulo do Nascimento Junior

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Abstract

Dear Editor,

We recently anesthetized a patient with complete Kartagener's syndrome undergoing a cesarean section and would like to share our experience conducting this rare case.

Kartagener's syndrome is a subtype of primary ciliary dyskinesia, a rare genetic disorder and its pathophysiology involves the dysfunction or lack of arms of the ciliary protein dynein, responsible for the mechanics of ciliary movement, causing chronic respiratory symptoms. The triad bronchiectasis, rhinosinusitis, and situs inversus, with or without dextrocardia, which defines the syndrome as complete or incomplete, is classic for the diagnosis of the disease. In women, ciliary dyskinesia of the fimbriae impairs the conduction of the oocyte along the uterine tubes, increasing the chance of infertility and ectopic pregnancy. Due to the fertility problems in Kartagener's syndrome, a cesarean section is a very rare situation for anesthesiologists. In a recent published review of 99 cases, only two were in obstetric patients.

References

1. Leigh MW, Pittman JE, Carson JL, et al. Clinical and genetic aspects of primary ciliary dyskinesia/Kartagener syndrome. Genet Med. 2009;11:473−87.

2. Blyth M, Wellesley D. Ectopic pregnancy in primary ciliary dyskinesia. J Obstet Gynaecol. 2008;28:358.

3. Cheng L, Dong Y, Liu S. Anesthetic Management of Patients With Kartagener Syndrome: A Systematic Review of 99 Cases. J Cardiothorac Vasc Anesth. 2023;37:1021−5.

4. Mathew PJ, Sadera GS, Sharafuddin S, et al. Anaesthetic considerations in Kartagener’s syndrome ‒ a case report. Acta Anaesthesiol Scand. 2004;48:518−20.

5. Ortega HA, Vega Nde A, Santos BQ, et al. Primary ciliary dyskinesia: considerations regarding six cases of Kartagener syndrome. J Bras Pneumol. 2007;33:602−8.

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