The most frequent abnormality of the pancreas is annular pancreas in which a thin flat band of pancreatic tissue surrounds the first part of the small intestine (duodenum) and continues on either side into the head of the pancreas.
Acute appendicitis is one of the most common causes of abdominal pain in childhood. This diagnosis must be considered in all age groups but is more common between the ages of 4 and 15 years. The function of the appendix is unknown. However, in rabbits and other animals the cecum is similar in shape to the appendix and plays a role in digestion of food. The middle, inside portion of the appendix may be irregular and somewhat narrow because of the presence of lymph nodes in the wall of the appendix.
In normal infants bile drains from the liver to the duodenum (the first portion of the small intestine) through a series of ducts. Ducts are like pieces of hollow tubing. Thousands of very small ducts in the liver become two large ducts that join to form a single hepatic duct that drains into the intestine. The gallbladder is a reservoir for bile that empties into the hepatic duct halfway between the liver and the duodenum. After the gallbladder joins the main duct draining the liver, the duct is called the common bile duct. Biliary atresia is a disorder occurring in infants in which the bile ducts disappear or scar closed, preventing the passage of bile out of the liver.
A problem that occurs mostly in older children and adults is biliary dyskinesia. This happens when the gallbladder does not squeeze properly and the bile does not drain out of the gallbladder properly.
Cholecystitis Acalculous - (Gallbladder inflammation without gallstones)
Acalculous cholecystitis may occur as a complication during treatment of various diseases. This condition may occur in newborns but is more common in older children. Boys and girls are affected equally. Patients are often severely ill as a result of prior surgery; a severe burn; extensive trauma; massive blood transfusion; and various infections, including pneumonia, a generalized infection (sepsis), typhoid, salmonella, otitis media with meningitis, giardiasis, and Kawasaki disease. The cause is unknown, but may be in part due to dehydration, ileus (a condition where the bowel does not squeeze properly), gallbladder stasis (bile not being squeezed out of the gallbladder), treatment with total parenteral nutrition (TPN), and breakdown of blood from multiple transfusions.
When bile is made in the liver it is first stored in the gall bladder, which connects to the common bile duct, and the gallbladder empties when fat enters the intestine. Bile contains digestive enzymes as well as the breakdown products of old red blood cells, which can no longer fulfill their function. When there is any disturbance in bile flow, liver damage may result. If there is a blockage, bile builds up in the blood and the patient becomes jaundiced with a yellow color to the eyes and skin.
Gallstones are relatively uncommon in blacks (except in sickle cell disease), exceptionally common in groups of Native Americans and Hispanics, and a frequent occurrence in whites. Gallstones currently are being recognized in children with increased frequency. Whether this increase in diagnosis is related to an increase in frequency of the disease or an increase in recognition because of the widespread use of ultrasound for abdominal complaints is unclear. The incidence currently is reported to be 0.15% to 0.22% in children. In comparison, gallstones are found in approximately 10% of adults.
Crohn's disease (CD), also known as regional enteritis, is a type of inflammatory bowel disease (IBD) described by Crohn in 1932. The most common affected site of bowel inflammation is the distal part of the small intestine known as the ileum, although the colon and other segments of the intestinal tract can be involved as well. The other common type of IBD is termed ulcerative colitis, although this disease is limited to the colon (large intestine).
Gastroesophageal Reflux and Other Disorders of Esophageal Function
Fundoplication is currently either the first or the second most commonly performed intra-abdominal procedure in infants and children in most pediatric surgical centers. Since the 1970s, surgical correction for gastroesophageal reflux (GER) has been performed by wrapping the upper stomach around the esophagus either a partial (Thal, Boix-Ochoa, Dor, Toupet) or complete (Nissen) fundoplication. In general, it seems that the Nissen fundoplication is more effective in preventing GER, but it may lead to difficulty swallowing and troublesome bloating in some patients compared with a partial fundoplication, which seems not as effective in preventing reflux. These symptoms after the Nissen procedure seem to be minimized with use of an appropriate-sized esophageal tube used as a “sizer” and division of the blood vessels that attach the stomach to the spleen for sufficient mobilization of the stomach.
Prior to the 1960s, the outcome of infants born with holes in their abdominal wall (abdominal wall defects) was poor. The development of modern breathing machines for infants, nutrition that could be delivered directly into the blood, and man made synthetics that could act as a covering for the abdomen greatly improved the survival of these patients. Recently, the ability to diagnose these defects during pregnancy, further advances in the neonatal intensive care unit (NICU) management of infants, and the development of the modern operating room techniques and technology have led to most patients surviving.
Descriptions of children with Hirschsprung's Disease date back to the 17th century, when Ruysch (1691), a Dutch anatomist, described a 5-year-old girl who died of intestinal obstruction. The first clinical description of Hirschsprung's Disease was presented at the Berlin Society of Pediatrics in 1886 by Hirschsprung. He thought that the disease was caused by distention of the colon, as evidenced by the title of his presentation: "Constipation in Newborns Due to Dilation and Hypertrophy of the Colon." As a result of Hirschsprung's presentation, however, attention was focused on the consequences of the abnormality rather than the actual cause of the disease. Hirschsprung’s disease often presents in newborns as intestinal blockage or obstruction occurring most commonly in the colon or rectum. The child may or may not have an infection called enterocolitis, resulting from the obstruction. This is caused by to many bacteria (overgrowth) in the affected segment of intestine. Although the incidence of enterocolitis is variable, this complication makes the diagnosis and early treatment of Hirschsprung’s disease urgent. In the mildest case, delayed passage of meconium (the stool first passed by a baby) may be the only abnormality.
Hydrops (Swelling) of the Gallbladder
Acute swelling of the gallbladder in the absence of gallstones is a well-recognized problem. Hydrops may occur as a newborn and in older children. This condition is characterized by the development of a fluid collection around the gallbladder and main bile duct that drains the liver.
Congenital hyperinsulinism, also called nesidioblastosis, is a problem in babies in which insulin control is abnormal, causing low blood sugar. Patients with this syndrome may have signs of low blood sugar without actually having a high insulin level, showing that they have a problem with insulin control.
A series of developmental steps are involved in the formation of the normal anatomy of the lower end of the anus, rectum, and genitourinary (GU) tract. By the fourth week of development, the cloaca and a structure called the cloacal membrane are present. The cloaca is a normal structure in birds and is present for a short period of time during the development of the human before birth. A cloaca is a structure into which the colon, urinary tract and genitalia all drain and exit the body with a single opening. A human goes thorough a stage of development where a cloaca normally exists and then goes on to develop separate openings for the rectum, urinary tract and in girls, the vagina. This normal development is in much part due to the development of a structure called the cloacal membrane. If the membrane does not develop normally the cloaca may persist at birth in girls or boys will develop some variant of imperforate anus.
There are many forms and causes of intussusception, but the most common in children is where the last portion of the intestine (the ileum) goes into the first portion of the colon (cecum). This intussusception is an important cause of belly pain and intestinal blockage in infants and requires immediate medical attention. Intussusception occurs in the United States in 2 to 4 per 1000 live births and more commonly (60%) in boys. Intussusception of infancy is seen most commonly between 6 and 10 months of age, with 65% of the children being less than 1 year of age. There seems to be a seasonal incidence, with two peaks occurring (1) in spring and summer and (2) in the middle of winter, corresponding to times of the year with an increased incidence of viral gastroenteritis (GI virus), colds, and flu. While there is no obvious cause, it generally is believed that GI virus leads to swelling of lymph glands in the intestine which leads to one piece of intestine "telescoping" into another.
The need to have one’s gallbladder removed is generally due to the formation of gallstones. Gallstones form when there is an imbalance in the components in the bile that is normally stored in the gallbladder. For most people, there is no known reason why gallstones develop. Nevertheless, there are diseases that can predispose your child to form gallstones. Children who have problems with blood cells being broken down easily such as can happen in spherocytosis and sickle cell anemia have increased incidence of gallstone formation.
Malrotation is an abnormal arrangement or twisting of the intestine inside the abdomen, which is also known as volvulus, that may result in loss of blood flow to the intestine. This abnormal arrangement or twisting of the intestine can also cause blockages of the intestines by causing a kinking of the intestine without loss of blood flow. A delay in recognizing these conditions can result in damage to the intestine as well as danger to the life of the child.
Meckel's diverticulum is an out pouching from the intestine caused by an abnormality during the development of the fetus and it occurs between 5 and 7 weeks after conception.
Meconium ileus is the intestinal blockage which is due to cystic fibrosis. Approximately 15% to 20% of infants with CF present with intestinal obstruction related to meconium ileus. Although it originally was thought that CF primarily affected the pancreas, it now is known that it also affects the secretions from the intestine.
Meconium Plug/Small Left Colon Syndrome
Meconium plug and neonatal small left colon syndromes share sufficient similarities to be discussed together. Patients have transient colonic obstruction most likely due to poor function. The principles of diagnosis and management are similar.
Necrotizing Enterocolitis (NEC)
Necrotizing enterocolitis (NEC) is the most serious and frequent stomach and intestinal (gastrointestinal, GI) problem of low-birth-weight infants. The result of dramatic improvements in the management of the lung and nutritional needs of premature infants has improved the immediate survival of the infants so now they are living longer and, thus, have a greater chance of developing NEC.
An omphalocele is a covered defect of the umbilicus (belly button) with a “sac” which contains abdominal contents. The skin and the muscle of the abdomen are missing. This problem is thought to start in the third week of development when the intestines elongate and normally reside in the yolk sac outside of the abdomen. Omphaloceles are covered by a sac composed of an outer layer and an inner layer. The umbilical cord s right into the sac. A separate compartment containing a jelly-like material also may be observed. If the omphalocele is above the umbilicus there may be other defects involving the diaphragm, sternum, and heart. If the omphalocele is below the umbilicus there may be other defects affecting the bladder, rectum, and lower spinal cord.
Pancreas divisum results when the back of the pancreas drained by one pancreatic tube and the front of the pancreas drained by another tube fail to join before joining with the main bile tube. The chances of someone having pancreas divisum are believed to be 5% to 10%, but drainage is normal in most of these people without any site of blockage.
Papillary neoplastic cysts, which are rare in childhood, should be assumed to cause cancer. Only by looking at the cyst under the microscope after surgery can doctors tell if the cyst is cancerous. They are easily ruptured, and the fluid inside the cyst is extremely irritating to the inside of the belly.
The pancreas is very important to a child’s normal growth and development. It sits in the back of the abdomen, behind the stomach and does two things: 1) It produces enzymes, which help with digesting food and 2) it produces hormones such as insulin and glucagon, which help control blood sugar. In the fetus and newborn baby, the pancreas produces almost no pancreatic enzymes (amylase and lipase); then, the child’s pancreas starts making these enzymes, which help in the digestion of food. Production of these enzymes is at full adult levels by age 2 years.
Perianal and Perirectal Abscess
Perianal abscesses (pus collection) and fistulas (tunnels that connect the inside of the anus to the skin outside) are seen commonly in male infants younger than 1 year of age with a peak incidence at 4 months of age.
Pyloric stenosis is the most common disorder causing vomiting in infancy which requires surgery.
There are numerous definitions for short-bowel syndrome (SBS). The simplest definition is that there is inadequate intestine to maintain normal nutrition by eating. Because infants and children require increased calories to grow and develop, SBS can have a more devastating effect in these patients.
The spleen was long believed to be an unnecessary organ that could be removed without causing any problems. Important functions of the spleen have now been discovered, however, including the removal of bacteria and waste from the blood, the making of antibodies, and the making of red blood cells.
Ulcerative colitis (UC) was described almost 150 years ago. Despite significant advancements in the knowledge of inflammatory bowel disease (IBD), this disorder’s cause and treatment remain unresolved. Although classically thought of as a disorder of adults, many children are affected. The pediatric surgeon is often instrumental in caring for patients with this disabling disease.
Umbilical (belly button) Hernia
After birth, when the end of the umbilical cord, along with the umbilical vein, umbilical arteries, and urachal remnant, dry up, a small opening is left in the abdominal wall. Failure of the muscles to close around this opening causes an umbilical hernia.
After normal separation of the umbilical cord; pink, healing tissue called granulation tissue may persist in the belly button and enlarge into a mass called an umbilical granuloma . This is often associated with umbilical drainage and swelling with redness of the surrounding skin.
Umbilical Infection (omphalitis)
Umbilical infections in older children are usually due to bacterial growth in a cavity from a left over fetal structure, most commonly a structure that was once attached to the bladder called the urachus.
Beginning in the fourth week of fetal life the front of the abdomen develops by tissue folding in from the sides, top and bottom. Between 6 and 10 weeks’ gestation, some of the fetus’ intestine is located outside of the main body cavity but it returns to the developing abdomen by the 10th week. By 12 weeks’ gestation, the large abdominal wall muscles in the front, called the rectus muscles, join each other in the midline except at the site of the umbilical ring (belly button), where the muscles are separated by tissue.