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Solid Organ Transplantation in Children

A retrospective review of the United Network for Organ Sharing Database (UNOS) from 1987–2012 recently estimated that over 2 million life-years have been saved by solid organ transplant, including both adult and pediatric patients. The ability to successfully perform solid organ transplantation in children has led to a remarkable improvement in survival and quality of life. In this chapter, each of the abdominal solid organ transplant…

Choledochal Cyst and Gallbladder Disease

Choledochal Cyst A choledochal cyst is a congenital dilatation of the biliary tract. The dilatation can be found along any portion of the biliary tract. However, the most common site is the choledochus. The diameter of the bile duct varies according to the child’s age. The normal diameter of the common bile duct (CBD) is seen in Table 44.1 . Any diameter of the bile duct…

Biliary Atresia

Biliary atresia (BA) is a relatively rare obstructive condition of the bile ducts causing neonatal jaundice that first appeared as a distinct entity in the Edinburgh Medical Journal in 1891. The concept of “correctable” and “noncorrectable” forms was introduced in 1916. BA is classified according to anatomic and cholangiographic findings. Type I is atresia of the common bile duct, type IIa is atresia of the common…

Appendicitis

Appendicitis is the most common surgical emergency in children. The incidence of appendicitis in the United States is approximately 1 per 1000. Eighty-six cases of appendicitis per 100,000 children are estimated to occur annually, and this number is increasing. The overall lifetime risk is estimated to be around 8%, with an age peak during the teen years. Appendicitis is increasing in Hispanics, Asians, and Native Americans,…

Inflammatory Bowel Disease

Inflammatory bowel disease (IBD) is the broad term that encompasses Crohn disease (CD), chronic ulcerative colitis (UC), and indeterminate colitis. The incidence of IBD continues to rise and is as high as 11.4 new cases per 100,000 children per year. Regardless of which specific disease entity is present, the physicians caring for these patients are faced with difficult medical and surgical challenges. Clinical, radiographic, and pathologic…

Meckel Diverticulum

Wilhelm Fabricius Hildanus, a German surgeon, first described the presence of a small bowel diverticulum in 1598. However, the diverticulum is named for Johann Meckel, a German anatomist, who further described the anatomy and embryology in 1809. Meckel diverticulum is a remnant of the embryologic vitelline (omphalomesenteric) duct that connects the fetal gut with the yolk sac and normally involutes between the 5th and 7th weeks…

Alimentary Tract Duplications

Alimentary tract duplications have been described for hundreds of years, and multiple terms have been used in the literature. The term duplication of the alimentary tract was coined by William Ladd in 1937. He described three common findings: a well-developed smooth muscle coat, an epithelial lining, and attachment to the alimentary tract. In 1952 Gross et al. reported the first large series describing 67 patients with these…

Intussusception

Intussusception is the most frequent cause of bowel obstruction in infants and toddlers. It is an acquired invagination of the proximal bowel (intussusceptum) into the distal bowel (intussuscipiens). It was first described in 1674 by Paul Barbette of Amsterdam, defined by Treves in 1899, and operated on successfully in 1873 by John Hutchinson. Pathophysiology The intussusceptum telescopes into the distal bowel by peristaltic activity. There may…

Acquired Anorectal Disorders

Perianal and Perirectal Abscess Perianal or perirectal abscesses are often encountered during infancy. The abscess typically presents as a fluctuant, tender mass in the perianal region ( Fig. 37.1 ). A history of stool abnormalities is typically not elicited. Perianal abscesses are much more common in male infants younger than 1 year of age and are infrequent in toddlers and older children. Crohn disease, immunodeficiency, glucose…

Fecal Incontinence and Constipation

Fecal incontinence can have serious implications on the social development of children and young adults, which, in turn, can provoke serious psychological and behavioral sequelae. It is a problem that affects more children than previously appreciated, including those born with anorectal malformations (ARMs), Hirschsprung disease (HD), spinal cord problems or spinal injuries, and severe functional (idiopathic) constipation. True fecal incontinence must be distinguished from pseudoincontinence. Children…

Anorectal Atresia and Cloacal Malformations

“Imperforate anus” has been a described entity for centuries. In fact, an early description suggested that “an infant whose anus is not visible should be rubbed with oil and stood in the sun, and where it shows transparent it should be torn cross-wise with a barley grain.” Fortunately, the surgical approach to these conditions has advanced significantly since then. For many centuries, individuals who practiced medicine…

Hirschsprung Disease

Hirschsprung disease (HD), also known as “congenital megacolon,” is characterized by the absence of ganglion cells in the myenteric and submucosal plexuses of the intestine. The first known description of this condition was by ancient Hindu surgeons in the Shushruta Samheta, and the first descriptions in the modern medical literature were from the 17th century. In 1887, Harald Hirschsprung, a pediatrician from Copenhagen, described two cases…

Necrotizing Enterocolitis

Despite nearly two centuries of recognition and five decades of increasingly intensive study, necrotizing enterocolitis (NEC) remains an unsolved problem. Reports from France as early as the 1820s describing “gangrenous necrosis” of the intestine were echoed in Vienna 30 years later. Not until the mid-20th century was the disease process systematically observed in Switzerland in 1944 and in New York in the 1960s. A condition of…

Meconium Disease

Intestinal obstruction is one of the most common admitting diagnoses to the neonatal intensive care unit (NICU), accounting for as many as one-third of all admissions. Failure to pass meconium within the first 24–48 hours of life, feeding intolerance, abdominal distension, and bilious emesis are hallmarks of intestinal obstruction in the newborn, and evoke a differential diagnosis of obstruction based on anatomic, metabolic, and functional considerations.…

Malrotation

Normal rotation of the intestine requires transformation from a simple, straight alimentary tube into the mature fixed and folded configuration normally present at birth. Through precise embryologic events, the duodenojejunal junction becomes fixed in the left upper abdomen while the cecum is anchored in the right lower quadrant. The midgut, defined as the portion of the intestine supplied by the superior mesenteric artery (SMA), is thus…

Duodenal and Intestinal Atresia and Stenosis

Congenital intestinal obstruction occurs in approximately 1 in 2000 live births and is a common cause of admission to a neonatal surgical unit, accounting for up to one-third of all admissions. Morphologically, congenital defects related to continuity of the intestine can be divided into either atresia or stenosis. Together, they constitute one of the most common etiologies of neonatal intestinal obstruction. See Chapter 29 for information…

Lesions of the Stomach

The stomach forms from the foregut and is recognizable by the fifth week of gestation. It then elongates, descends, and dilates to form its familiar structure by the seventh week of gestation. The vascular supply to the stomach is very robust, and ischemia of the stomach is rare. The stomach is supplied by the right and left gastric arteries along the lesser curvature, the right and…

Gastroesophageal Reflux

Gastroesophageal reflux (GER) is a condition that is commonly encountered in infants but usually resolves by 2 years of age. GER is defined as the involuntary retrograde passage of gastric contents into the esophagus, with or without regurgitation or vomiting. It is thought to be the result of transient relaxations of the lower esophageal sphincter (LES), independent of swallowing, which allow the gastric contents to reflux…

Esophageal Atresia and Tracheoesophageal Fistula Malformations

Esophageal atresia (EA) and tracheoesophageal fistula (TEF) anomalies present the pediatric surgeon with a unique and complex congenital disease, which tests both the diagnostic and technical skill of the surgeon. Most pediatric surgeons consider the surgical correction of these malformations to be the height of neonatal surgical care. In 1959, Dr. Willis Potts wrote, “To anastomose the ends of an infant’s esophagus, the surgeon must be…

The Esophagus

This chapter provides a brief overview of the surgical anatomy, physiology, and pathologic conditions of the esophagus. Conditions of interest to the pediatric surgeon include congenital disorders, achalasia, foreign bodies, caustic injuries, perforation, and strictures. Surgical approaches for esophageal replacement are also reviewed. Esophageal atresia (EA), tracheoesophageal fistula (TEF), and gastroesophageal reflux (GER) disease are covered in detail in other chapters. Neoplasms of the esophagus are…