Avoiding harm: central venous access in Kartagener syndrome and other vascular anomalies

Placement of internal jugular catheters is more likely to be complicated if a left-sided approach is used, assuming normal anatomy. Kartagener syndrome is the sine qua non of sidedness confusion and results in cognitive challenges that increase the risk of adverse patient outcomes. The altered anatomy can cause profound disorientation from our usual processes. In normal circumstances the right-sided approach is used for placement of internal jugular catheters, but in Kartagener syndrome the left-sided approach should be preferred. Surgical volume and use of ultrasound guided techniques are positively correlated with better outcomes. Clinical experience may be a detriment to performance. Knowledge of these issues will help clinicians maintain vigilance and avoid error. Keywords: Kartagener syndrome, central venous access, superior vena cava, landmark technique, internal jugular vein catheterization cognitive bias

Ciliary Dyneins and Dynein Related Ciliopathies

Although ubiquitously present, the relevance of cilia for vertebrate development and health has long been underrated. However, the aberration or dysfunction of ciliary structures or components results in a large heterogeneous group of disorders in mammals, termed ciliopathies. The majority of human ciliopathy cases are caused by malfunction of the ciliary dynein motor activity, powering retrograde intraflagellar transport (enabled by the cytoplasmic dynein-2 complex) or axonemal movement (axonemal dynein complexes). Despite a partially shared evolutionary developmental path and shared ciliary localization, the cytoplasmic dynein-2 and axonemal dynein functions are markedly different: while cytoplasmic dynein-2 complex dysfunction results in an ultra-rare syndromal skeleto-renal phenotype with a high lethality, axonemal dynein dysfunction is associated with a motile cilia dysfunction disorder, primary ciliary dyskinesia (PCD) or Kartagener syndrome, causing recurrent airway infection, degenerative lung disease, laterality defects, and infertility. In this review, we provide an overview of ciliary dynein complex compositions, their functions, clinical disease hallmarks of ciliary dynein disorders, presumed underlying pathomechanisms, and novel developments in the field.

Anatomical variants of pulmonary segments and uni-portal thoracoscopic segmentectomy for lung cancer in a patient with Kartagener syndrome: a case report

Situs inversus totalis (SIT) is an extremely uncommon congenital disease where the major organs of the body are transposed through the sagittal plane. Kartagener syndrome is a complication of SIT with immotility of bronchial cilia, bronchiectasis, and chronic sinusitis. There is no report describing patients with Kartagener syndrome who accept uni-portal segmentectomies for lung cancer in past studies. Here we report a 74-year-old female patient with both Kartagener syndrome and a small early-stage lung cancer lesion located in the apical segment of the left upper lobe (LS1). The pulmonary segment anatomy of the left upper lobe in this case, which had very rare variants, was presented and interpreted in detail. This patient underwent an anatomic segmentectomy to the LS1 and a partial excision to the left middle lobe with bronchiectasis through a single 3 cm length incision. We believe that the case can give surgeons some experience and inspiration.