Physical Address

304 North Cardinal St.
Dorchester Center, MA 02124

Endoscopic Retrograde Cholangiopancreatography

Endoscopic retrograde cholangiopancreatography (ERCP) is a combined endoscopic and radiologic procedure used for diagnostic and, more commonly, therapeutic interventions within the pancreatic and biliary tree. Although interventional radiology and interventional endoscopic ultrasound (EUS) approaches exist, ERCP has been and remains the gold standard endoscopic platform for pancreatic and biliary interventions. The procedure utilizes the simultaneous acquisition of high-resolution endoscopic and fluoroscopic images, and radiographic images are…

Colonoscopy, Polypectomy, and Related Techniques

The development of the colonoscope followed that of upper panendoscopes. Rigid proctoscopes were developed in the late 1800s, and fiberoptic techniques were adapted to visualize the sigmoid and descending colon in the 1960s. In the 1970s, the colonoscope was lengthened with four-way tip deflection, allowing for modern day colonoscopic techniques. The first mass-produced video instruments were introduced in the 1980s and 1990s. Pediatric colonoscopes with a…

Esophagogastroduodenoscopy and Related Techniques

The earliest gastrointestinal endoscopies were performed in the late 1880s using rigid instruments, looking initially at the esophagus and rectum. The semiflexible gastroscope was developed in the early 1930s by Schindler and Wolf utilizing a series of short-focal-length lenses and a semiflexible tube. Fiberoptic endoscopes represent a significant advance in endoscopy and were first developed in the 1950s by Basil Hirschowitz, along with two physicists at…

Stomas of the Small and Large Intestine

Acknowledgments The authors wish to thank those who wrote the previous text versions and the following individuals for their contributions to this chapter: Meagan Costedio, MD; enterostomal therapists Coleen Potts, RN, Susan Booth, RN, and Jeanette Gatan, RN; Lisa Rhome, RN; and the patients who inspire us every day. Introduction The creation of a stoma in the pediatric patient requires careful consideration of the associated risks…

Newborn Abdominal Wall Defects

Historical Background Omphalocele was first described by Ambrose Paré during the 16th century and was associated with poor prognosis; gastroschisis was likely described by Lycosthenes near the same time. The present-day diagnoses were established in 1953 by Moore and Stokes, who described omphalocele and gastroschisis by distinguishing the location of the umbilical cord, the presence or absence of a covering sac, and the appearance of eviscerated…

Small and Large Bowel Stenosis and Atresias

Intestinal atresia and stenosis are common causes of neonatal intestinal obstruction and can affect any portion of the gastrointestinal tract. “Atresia,” derived from the Greek word atretos, means not perforated. This term is used to describe a congenital absence or pathologic closure of a body opening or passage, and these comprise approximately 95% of congenital gastrointestinal obstructions. When used to describe gastrointestinal anatomy, this word refers…

Abnormal Rotation and Fixation of the Intestine

Normal intestinal growth and development follows a stereotypical pattern of mesenteric rotation and subsequent intestinal fixation to the body wall during fetal life. The term malrotation has been widely used to describe various disorders of abnormal intestinal rotation and fixation. Clinically symptomatic abnormalities of intestinal rotation and fixation are encountered infrequently. The major life-threatening problem associated with malrotation is the potential for the intestine to twist…

Imperforate Anus

The term imperforate anus is a misnomer; it is commonly used to refer to a spectrum of anorectal malformations, ranging from a benign defect that requires a minor operation and results in an excellent prognosis to complex malformations with a high incidence of associated defects requiring sophisticated and specialized surgical procedures with less than optimal functional results. Most cases are not actually “imperforate,” but rather have…

Hirschsprung Disease

Hirschsprung disease (HD) is characterized by the absence of ganglion cells in the myenteric and submucosal plexuses of the intestine. The first known description of HD appeared in an ancient Hindu document known as the Sushruta Samheta. There were multiple case reports of congenital megacolon during the 17th century, but the two cases described by Harald Hirschsprung in 1887 resulted in the disease being named for…

Meckel Diverticula and Other Omphalomesenteric Duct Remnants

Introduction Meckel diverticula are located along the antimesenteric border of the distal ileum and may contain rests of ectopic tissue. They are the most common congenital anomaly of the alimentary tract with an estimated prevalence of 1% to 4% in the general population and are more common in children born with major malformations of the umbilicus, alimentary tract, central nervous system, and cardiovascular system. , The…

Inguinal Hernias and Hydroceles

Inguinal hernia repair is the most common surgical procedure performed by pediatric surgeons. A hernia can be thought of as protrusion of contents through a defect or opening. The many different types of hernias found in children are defined by their location. An epigastric hernia is protrusion of preperitoneal fat through a small defect in the linea alba and presents in the midline above the umbilicus.…

Intussusception in Infants and Children

Acknowledgments The authors would like to acknowledge the previous authors Anthony T. Manning and Danny C. Little for their assistance with the 6th edition of this chapter. Introduction Intussusception is the most common cause of intestinal obstruction in children less than 2 years of age. Intussusception is defined as the invagination of a segment of intestine into the lumen of an adjacent segment of intestine that…

Appendicitis

Introduction Acute appendicitis is one of the most common surgical conditions afflicting children and adults. Approximately 250,000 cases occur annually in the United States, with the highest incidence in patients 10 to 19 years of age. The lifetime risk of developing appendicitis has been estimated at 8.6% for males and 6.7% for females, with a lifetime risk of undergoing an appendectomy estimated at 12% for males…

Other Diseases of the Small Intestine and Colon

Small Intestinal Bacterial Overgrowth The microbiota of the small intestine is essential for proper immune regulation and nutrient absorption. Typically, the concentration of bacteria is less in the proximal small intestine compared with the distal small intestine. Bacterial concentration of the proximal to mid–small intestine is approximately 10 4 organisms per milliliter compared with the terminal ileum, in which the bacterial concentration may be as high…

Neoplasms of the Liver

Hepatic tumors make up only 1% to 4% of pediatric solid tumors, and most of these are metastatic lesions from an extrahepatic site. The symptoms leading to their diagnosis are usually nonspecific and may be erroneously attributed to an underlying gastrointestinal condition. Being able to recognize these hepatic neoplasms is important as a prompt and correct diagnosis can be crucial to the survival of the patient.…

Neoplasms of the Gastrointestinal Tract

Acknowledgment We would like to acknowledge the authors of the prior edition, Zev H. Davidovics, Karen F. Murray, and Laura S. Finn, for the structure and a significant portion of the content of this chapter. Many thanks to Thomas Plesec, MD, for pathology images along with Bo Shen, MD, and Matthew Kalady, MD, for endoscopic images. Neoplasms of the gastrointestinal (GI) tract are relatively rare in…

Disorders of the Anorectum: Fissures, Fistulas, Prolapse, Hemorrhoids, Tags

The anal sphincter consists of an inner ring of smooth muscle, the internal anal sphincter, the intersphincteric space, and an outer ring of skeletal muscle, the external anal sphincter. The internal sphincter is an involuntary muscle that maintains anal tone. It is in a continuous state of partial contraction and relaxes in response to rectal distension. The external sphincter is a voluntary muscle extending from the…

Neonatal Necrotizing Enterocolitis

Necrotizing enterocolitis (NEC) is the most common gastrointestinal emergency in the neonatal intensive care unit (NICU). It is primarily a disease of prematurity, with only 10% of affected infants born after 36 weeks of gestation. NEC contributes significantly to short-term and long-term morbidity as well as mortality of preterm (PT) infants and is the most common cause for pediatric intestinal failure. Fourteen percent of pediatric intestinal…

Chronic Intestinal Pseudo-Obstruction

Chronic intestinal pseudo-obstruction (CIPO) is an umbrella term for heterogeneous disorders that result in the inability of the gastrointestinal (GI) tract to move contents through an unobstructed lumen and are thus unified by similarities in their clinical presentation, evaluation, and management. Therefore, it is the most severe form of GI motility disorder and can lead to significant morbidity and mortality. Although much progress has been made…

Ulcerative Colitis in Children and Adolescents

Ulcerative colitis (UC) is an important pediatric gastrointestinal disease, given its potential for significant morbidity and even mortality during childhood, its chronicity, and its premalignant nature. Although significant advances in our understanding of its immunologic basis have led to novel approaches to its therapy, UC remains medically incurable. Nevertheless, current medical and surgical therapeutic options have improved the overall outlook for children with this condition. Over…