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Interact CardioVasc Thorac Surg 2007;6:404-405. doi:10.1510/icvts.2006.141994A
© 2007 European Association of Cardio-Thoracic Surgery

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Case report - Cardiac general

ICVTS on-line discussion A Thoracic bifurcation and Klippel-Feil syndrome

Narcis Hudorovic

University Hospital Sestre Milosrdnice, Zagreb 10000, Croatia

Patients with Klippel-Feil syndrome should have imaging of the subclavian artery and its branches prior to coronary artery bypass grafting

eComment: The article by Paul et al. [1] proposes that any patient with Klippel-Feil syndrome for CABG, should have imaging of the subclavian and internal mammary arteries preoperatively as there is likely to be an associated anomaly. My search of our database finds that we performed 2506 CEA procedures (last ten years), and one patient was followed up for a Klippel-Feil anomaly (plain radiography, CT scan, MRI, DSA, ultrasound of the kidneys, intravenous pyelography, hearing loss evaluation). The patient presented with bilateral thoracic carotid bifurcation and the internal carotid artery (ICA) being anterior and medial to the external carotid artery (ECA) on both sides. Until today, the number of documented cases remains insufficient to draw a significant conclusion of association between the Klippel-Feil anomaly and subclavian artery supply disruption sequence (SASDS). It is generally accepted that the precursor of the CCA-ECA trunk arises from the aortic sac and secondarily migrates toward the third aortic arch to constitute carotid bifurcation. Alternatively, it has been proposed that the ventral pharyngeal artery arises directly from the third arch at the location of the future carotid bifurcation. In both cases, persistence of the ductus caroticus associated with partial or complete involution of the third will increase the distance separating the CCA and ECA embryologic origins. Migration of the ECA toward the carotid axis then being incomplete or absent, resulting either in a low position of the carotid bifurcation or in separate origins of the ECA and ICA. The association of low carotid bifurcations with a Klippel-Feil anomaly is interesting in that it might point to a segmental developmental disorder leading both to the fusion of cervical vertebral bodies and to the third aortic arch anomaly facilitating SASDS. Intrathoracic carotid bifurcations may also represent diagnostic pitfalls by lying outside the region of interest during pre-operatively diagnostic evaluation. As anomalies of carotid bifurcation are conjoint with subclavian as well as internal mammary artery it could be wise to SASDS be represented by the above conditions. Since this is a syndrome with a constellation of possible abnormalities, no set of definitive contraindications for cardiovascular surgical subspecialty exists. If a surgeon believes that an operation is indicated, it is incumbent upon her/him to make certain none of the other conditions that could cause morbidity or mortality are present. A thorough workup of the patient (especially preoperative imaging techniques) is imperative prior to cardiovascular surgical intervention. Greater interest, observations, and endeavors in this field should offset the problems of diagnosis.


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  1. Paul I, Badmanaban B, Campalani G. Patients with Klippel-Feil syndrome should have imaging of the subclavian artery and its branches prior to coronary artery bypass grafting. Interact CardioVasc Thorac Surg 2007; 6:403–405.[Abstract/Free Full Text]

Related Article

Patients with Klippel-Feil syndrome should have imaging of the subclavian artery and its branches prior to coronary artery bypass grafting
Ian Paul, Balaji Badmanaban, and Gianfranco Campalani
Interactive CardioVascular and Thoracic Surgery 2007 6: 403-404. [Abstract] [Full Text] [PDF]




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