How often is Klippel-Feil Syndrome associated with congential heart disease presentation of five cases and a review of the literature
Published on: 3rd September, 2019
OCLC Number/Unique Identifier: 8270717914
Introduction: Klippel-Feil syndrome (KFS), is a bone disorder characterized by the abnormal joining (fusion) of two or more spinal bones in the neck (cervical vertebrae), which is present from birth. Three major features result from this abnormality: a short neck, a limited range of motion in the neck, and a low hairline at the back of the head. In some individuals, KFS can be associated with a variety of additional symptoms and physical abnormalities which contribute in the deterioration and complication of the condition of the child.
Aim of presentation: Here, we report five children from Kosovo with KFS associated with different heart abnormalities, clinical presentation, diagnosis, management, and outcomes of selected conditions in resources-limited settings.
Methods: Retrospectively we analysed medical reports of five children, diagnosed at different age with congenital disease and clinical and lab signs of Klippel-Feil syndrome.
Conclusion: Basing on our cases, all diagnosed in a small country as a Kosovo, we can conclude that KFS is not such a rare condition. In addition, such syndrome is not so rarely associated with different congenital heart disease. In four cases cardiac surgery was indicated and successfully was done abroad Kosovo in the lack of such services in Kosovo.
Anomalies of coronary artery origin: About two cases
Published on: 13th September, 2019
OCLC Number/Unique Identifier: 8270707766
Anomalies of coronary artery origin are congenital malformations characterized by the abnormal birth of a coronary artery from the controlateral coronary aortic sinus (birth of the left coronary artery from the right sinus or birth of the right coronary artery from the left sinus). The artery concerned has an abnormal initial path between the aorta and the pulmonary artery; this segment is most often intramural, in the aortic wall. They are rare with a prevalence (0.1% to 0.3% of the population).
They pose a high risk of sudden stress death related to exercise myocardial ischemia. The most common mode of discovery is aborted sudden death, but sometimes fortuitously. The contribution of multi-round CT is described for the positive diagnosis of these anatomical variations, sometimes delicate in coronary angiography, but also for the distinction between “benign” and “malignant” forms, potentially responsible for myocardial ischemia. Treatment is usually surgical in symptomatic forms. We report the cases of two patients with coronary connection abnormalities discovered in adulthood.
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