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Although conditions afflicting the extracardiac vascular system during infancy and childhood have been described for well over two centuries, a comprehensive literature dedicated to the management of pediatric vascular surgery patients remains lacking. Despite the presence of a number of textbooks devoted to the focused topic of pediatric cardiac surgery, a unified resource collating common themes and describing surgical techniques and principles does not exist for vascular surgery of children and the young adult. This can partly be explained by the range of vascular surgery conditions encountered in the modern era of pediatric surgery and by the diversity of providers who render treatment in this therapeutic area. Beyond pediatric surgeons with expertise in advanced vascular surgery reconstruction, adult vascular surgeons, pediatric and adult cardiothoracic surgeons, transplant surgeons, urologists, plastic surgeons, and adult general surgeons may all at one time or another operate on the blood vessels of pediatric patients. It is our hope in this introductory chapter to provide a broad overview of concepts and techniques that are particularly relevant to the pediatric population and that may prove useful to those who, either rarely or frequently, may be called upon to treat the vascular problems of this unique age group.
One might trace the origins of pediatric vascular surgery to the 16th century when Guido Guidi first described a congenital vascular malformation after observing pulsatile varices protruding from a young man’s scalp. Giovanni Morgagni, a precocious Italian anatomist and a pioneer in the development of pediatric surgery, was instrumental in further expanding this early experience with vascular anomalies. His original description of coarctation of the thoracic aorta in 1760 was just one of his many original discoveries. Sixty years later, August Meckel reported the associated collateral circulation and rib erosion that is seen in this condition. In 1835 Schlessinger was the first to discover that aortic coarctation is not limited to the aortic isthmus and, later in the same century, Pierre-Carl Pontain reported on arterial hypertension as a pathophysiologic consequence of coarctation. Although much less frequently encountered, constriction of the subisthmic aorta was noted by Schlessinger during his autopsy of a 15-year-old girl whose distal thoracic aorta was nearly obliterated. Quain reported a similar malady of the abdominal aorta in a 50-year-old man in 1847. The first clinical recognition of renovascular hypertension in a child was in 1938 by Wyland Leadbetter and Carl Burkland. They further noted that removal of the child’s kidney, which had become ischemic from fibromuscular dysplasia, corrected the hypertension. These are just a few of the myriad discoveries that have provided a framework for our current understanding of many pediatric vascular disorders.
For many years, operative repair of vessels in infants was deemed too hazardous to undertake. Thomas Starzl overcame this barrier in July 1967 with his pioneering efforts to complete the first successful pediatric liver transplant. This operation, carried out on a 19-month-old child with a large hepatoma necessitating multiple vascular anastomoses, was a major milestone in pediatric vascular surgery. Further technical advances were developed by Dean, Stanley, and Fry in their landmark work on pediatric patients with renovascular hypertension. , Over the past 3 to 4 decades, pediatric, cardiac, and vascular surgeons have continued to accrue significant clinical and technical experience that has translated into more consistent results and allowed more sophisticated vascular reconstructions to be undertaken in infants and children.
William Ladd and Robert Gross are often cited as the founders of pediatric surgery, and much of the current-day training of pediatric surgeons can be traced to these two men and their many pioneering contributions. Publication of their textbook Abdominal Surgery of Infancy and Childhood in 1941 was instrumental in establishing pediatric surgery as a unique and independent surgical subspecialty. This text emphasized the paramount importance of gentle tissue handling, one of William Halsted’s fundamental surgical principles, when operating on children. Although there are many similarities between children and adults regarding surgical management and operative technique, there are also a number of important differences that warrant highlighting. These range from fluid management and anesthetic concerns to issues related to the future growth of organs and their surrounding critical elements. From a vascular surgical standpoint, one must take into consideration that as an infant or child grows, the demands on blood vessels will also increase.
If clinically permissible, vascular reconstruction in small infants should be delayed to allow for further development as reconstructing vessels less than 2 mm in diameter is technically challenging and has been associated with comparatively inferior results. For example, deferring renal revascularization in children with renovascular hypertension until the child is at least 3 years old has been shown to increase the likelihood of a successful outcome. Fortunately, the incidence of neonatal hypertension is quite low, ranging from 0.2% to 2%, and prolonging the use of antihypertensive medications in an effort to delay surgery has not been associated with significant long-term adverse effects. However, occasionally, it can prove challenging to control high blood pressure in an infant, given the variable onset and duration of oral antihypertensive medications as well as unpredictable responses to these agents. Persistent, uncontrolled hypertension in these young patients can lead to congestive heart failure and cardiogenic shock, which may compel surgical reconstruction of the renal arteries regardless of the child’s age. Although the preference is to defer surgical repair until the patient is of appropriate age and size, renal artery reconstruction has been safely performed in the very young; the University of Michigan has described their successful treatment of a 3-month-old, 4700-g baby with bilateral renal artery occlusions.
In contrast to the recommendation to delay the surgical correction of renovascular hypertension, early repair of thoracic coarctation is typically advised, given low reported perioperative mortality rates and a relatively low risk of reintervention or recurrent coarctation following reconstruction. More importantly, early repair minimizes the incidence of vascular dysfunction and persistent hypertension.
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