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December 23, 2005
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Omaida C. Velazquez, M.D., Peter Mattei, M.D., Ronald M. Fairman, M.D..
University of Pennsylvania, Philadelphia, PA, USA.
Objective: Vascular pathologies of the aorta in the pediatric population represent a heterogeneous group of exceedingly rare conditions for which classic vascular approaches may now be enhanced by state-of-the-art advances in the vascular field. In the current era of surgical sub-specialization, the highly infrequent nature of these challenging clinical scenarios makes it difficult to formally train surgical fellows from pediatric or vascular surgical backgrounds. The aim of this work was to review our center’s experience with pediatric vascular anomalies with focus on diagnoses and treatment of pathology in the aorta and its major named branches.
Methods: We retrospectively reviewed all pediatric vascular consultations addressed to our center’s division of vascular surgery from all major pediatric hospitals in the region. We focused on extracting and formulating an updated understanding of etiologies and current strategies for multidisciplinary approach at diagnosis, treatment, and follow-up, such as to optimize long-term favorable outcomes.
Results: In the last three years, 31 pediatric vascular consultations were performed by our vascular surgery division, for patients ages 1 d to 18 yr of age. Pathology of the aorta and/or its major named branches accounted for 68% (21/31) of the requested consultations. The type of aortic pathology was equally divided into stenotic/occlusive (50%) and aneurismal disease (50%). Four patients had both aneurismal and stenotic/occlusive disease. The etiologies for the aortic pathologies (in order of frequency) were idiopathic in 57%, or associated with Takayasu’s arteritis (9%), neurofibromatosis (9%), fibromuscular dysplasia (9%), William’s syndrome (4%), Tuberous sclerosis (4%), traumatic (4%), and mycotic (4%). The most common presentation for stenotic/occlusive cases was hypertension whereas the majority of aneurysm cases were diagnosed incidentally upon imaging performed for unrelated symptoms. Individualized complex vascular surgical interventions were performed in 53% of evaluated patients with zero operative morbidity and mortality. Innovative treatments that utilized endovascular approaches, alternative conduits such as cryopreserved arterial homografts, and multidisciplinary techniques such as renal autotransplantation (in collaboration with transplantation team), cardiopulmonary bypass for circulatory management (in collaboration with cardiothoracic team), and intra-operative neuromonitoring (in collaboration with neurology team), were required in several of these highly complex vascular reconstructions. Pre-, intra-, and post-operative close collaboration with nephrology, cardiology, genetics, and anesthesia teams has been paramount to achieve excellent outcomes.
Conclusions: Extremely rare and complex anomalies of the aorta and its major named branches in children can be successfully reconstructed taking advantage of recent vascular surgical advances and following a thorough multidisciplinary approach to diagnoses, treatment, and follow-up.
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Current Strategies to Treat Anomalies of the Aorta and its Branches in Children
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Omaida C. Velazquez, M.D., Peter Mattei, M.D., Ronald M. Fairman, M.D..
University of Pennsylvania, Philadelphia, PA, USA.
Objective: Vascular pathologies of the aorta in the pediatric population represent a heterogeneous group of exceedingly rare conditions for which classic vascular approaches may now be enhanced by state-of-the-art advances in the vascular field. In the current era of surgical sub-specialization, the highly infrequent nature of these challenging clinical scenarios makes it difficult to formally train surgical fellows from pediatric or vascular surgical backgrounds. The aim of this work was to review our center’s experience with pediatric vascular anomalies with focus on diagnoses and treatment of pathology in the aorta and its major named branches.
Methods: We retrospectively reviewed all pediatric vascular consultations addressed to our center’s division of vascular surgery from all major pediatric hospitals in the region. We focused on extracting and formulating an updated understanding of etiologies and current strategies for multidisciplinary approach at diagnosis, treatment, and follow-up, such as to optimize long-term favorable outcomes.
Results: In the last three years, 31 pediatric vascular consultations were performed by our vascular surgery division, for patients ages 1 d to 18 yr of age. Pathology of the aorta and/or its major named branches accounted for 68% (21/31) of the requested consultations. The type of aortic pathology was equally divided into stenotic/occlusive (50%) and aneurismal disease (50%). Four patients had both aneurismal and stenotic/occlusive disease. The etiologies for the aortic pathologies (in order of frequency) were idiopathic in 57%, or associated with Takayasu’s arteritis (9%), neurofibromatosis (9%), fibromuscular dysplasia (9%), William’s syndrome (4%), Tuberous sclerosis (4%), traumatic (4%), and mycotic (4%). The most common presentation for stenotic/occlusive cases was hypertension whereas the majority of aneurysm cases were diagnosed incidentally upon imaging performed for unrelated symptoms. Individualized complex vascular surgical interventions were performed in 53% of evaluated patients with zero operative morbidity and mortality. Innovative treatments that utilized endovascular approaches, alternative conduits such as cryopreserved arterial homografts, and multidisciplinary techniques such as renal autotransplantation (in collaboration with transplantation team), cardiopulmonary bypass for circulatory management (in collaboration with cardiothoracic team), and intra-operative neuromonitoring (in collaboration with neurology team), were required in several of these highly complex vascular reconstructions. Pre-, intra-, and post-operative close collaboration with nephrology, cardiology, genetics, and anesthesia teams has been paramount to achieve excellent outcomes.
Conclusions: Extremely rare and complex anomalies of the aorta and its major named branches in children can be successfully reconstructed taking advantage of recent vascular surgical advances and following a thorough multidisciplinary approach to diagnoses, treatment, and follow-up.
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