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List of Pediatric Surgical Conditions
Achalasia
Achalasia is a motor disorder usually affecting the entire length of the esophagus. Although the cause of achalasia is unclear, approximately 10% of cases occur in children. Even though this disease is relatively uncommon in children, it seems to be increasing in frequency.
Ambiguous Genitalia
Ambiguous genitalia refers to a condition of the genitals in which there is a question about whether the sex of the child is male or female. Proper male or female assignment to a newborn with ambiguous genitalia should be expeditious and timely, but it is crucial that it be proper and accurate. Proper gender assignment should entail an approach that includes, but is not limited to, input from pediatric endocrinology, surgery, urology, psychiatry, and radiology consultants and the parents. When an appropriate sex assignment has been made, it is possible to proceed with an operation procedure in a timely fashion, if one is required.
Annular Pancreas
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.
Appendicitis
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.
Biliary Atresia
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.
Biliary Dyskinesia
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.
Branchial Cysts, Sinuses and Remnants
Branchial arches are structures that appear in the fourth week of development on either side of the head and neck. Paired outpouchings from the inner lining of the early part of the mouth and throat along with clefts (inward indentations) appear along with muscular, nervous structures, and connective tissue in the neck area. Continued development in this area, from the outpouchings and from the clefts results in the ear canal, tonsil, throat structures, parathyroid glands and thymus. There are four distinct clefts, each giving rise to abnormalities if incomplete development occurs or there is a failure of these primitive structures to go away.
Breast Problems
Pediatric breast problems can be categorized to those that arise in infancy and those later in childhood. They may manifest as nipple discharge, masses or lumps, with redness or swelling of the breast area. It is not uncommon for some children to have extra breast tissue or extra nipple tissue. This extra tissue rarely causes any physical symptoms but can be removed if it leads to emotional or psychological problems.
Cervical Cysts, Sinuses, and other Neck Lesions
Cysts (fluid filled lumps), solid lumps, and sinuses (small openings) found about the head and neck are frequently residual structures from embryologic development that have failed to resorb completely or mature. Some lumps may be caused by inflammation, infection or even tumors affecting lymph nodes in the neck area.
Chest (Mediastinal) Cysts
Mediastinal cysts and tumors are relatively common in infants and children. Although cysts of the mediastinum are often asymptomatic, they usually require removal. If cysts are symptomatic, they may present with chest pain, cough, breathing problems, bloody cough or bloody vomiting, and difficulty swallowing. The various cysts encountered include bronchogenic cysts, thymic cysts, enterogenous cysts, neurenteric cysts, dermoid cysts, cystic hygroma, and pericardial cysts.
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.
Choledochal Cyst
Choledochal cyst is one of the common abnormalities that affect the biliary system of children who present with complaints of upper abdominal pain and jaundice.
Cholelithiasis (Gallstones)
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.
Congenital Diaphragmatic Hernia
Congenital diaphragmatic hernia (CDH) is a condition where there is a hole in the diaphragm and organs from the abdomen go up into the chest cavity. CDH was described in 1848 and the first repair in a baby was by Robert Gross in 1946.
Conjoined Twins
Conjoined twinning is a rare and challenging congenital malformation, which has stimulated human interest from early times. For example, conjoined twins have been depicted in sculpture and art dating back from before the time of Christ. Early, though unsuccessful efforts were undertaken to separate the conjoined twins, but occasional successful separation was not reported until the 1960s. More routine success with separation has only occurred over the last 15 to 20 years.
Crohn's Disease
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).
Cystic Hygroma
Cystic hygromas are varying sized cystic abnormalities of the lymphatic system occurring in perhaps 1 in 10,000 births. The word is derived from the Greek meaning moist or watery tumor and can occur equally in boys and girls. Approximately 50 or 60 percent are present at birth and about 80-90 percent appear or are detected before the end of the second year of life.
Empyema
Empyema is a chest infection. Unlike pneumonia, which is an infection in the lung, empyema occurs in the space between the lung and the chest wall known as the pleural space (or pleural cavity). In children, empyema is usually a complication of pneumonia. Inflammatory reaction to the pneumonia produces fluid in the pleural space (“effusion”). If the infection from the pneumonia spreads to this fluid, pus may accumulate, resulting in empyema.
Epididymitis and Orchitis
Epididymitis is a collection of tissue just behind the testicle. Epididymitis may have an infectious or inflammation. Most commonly, epididymitis occurs from the reflux of infected urine or from sexually acquired disease caused by gonococci and Chlamydia . This distinction is important because different antibiotics are needed for the different causes. Occasionally, epididymitis develops after excessive straining or lifting and the reflux of urine into the vas deferens, which causes a chemical epididymitis; this usually resolves promptly. Any non–sexually active child, especially a prepubertal child who develops epididymitis, should be evaluated for a urinary tract abnormality.
Foreign Bodies and Bezoars
Ingestion of foreign bodies is a problem because of the tendency for small children to put everything into their mouths. There may or may not be history of ingestion of a foreign body. If a foreign body does not become stuck in the duodenum or at the first part of the small intestine (ligament of Treitz), it ordinarily passes through the intestinal tract. A foreign body that perforates the intestine may produce obstruction.
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.
Gastroschisis
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.
Hirschsprung's Disease
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 too 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. top 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.topHyperinsulinism
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.
Hyperthyroidism
Grave’s disease (hyperthyroidism) is generally diagnosed in adults, but can be seen in children. This can cause enlargement of the thyroid gland (goiter). Although this usually occurs in older children, it can be seen in infants of mother’s with Grave’s disease. Infants generally respond to medicines (iodine and propylthiouracil) and do not require surgery.
Imperforate Anus
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.
Inguinal Hernia and Hydrocele
The occurrence of a congenital inguinal hernia and undescended testis is related to descent of the testis into the scrotum. The testes start developing in the abdomen and eventually drop into the scrotum. As the testes drop into the scrotum a membrane surrounding all the abdominal contents gets pulled into the scrotum with the testes. This is called the processus vaginalis. At approximately 3 months’ gestation, the testis descends into the scrotum because of the influence of hormones. The testes usually reaches its final destination in the scrotum by the third trimester. In approximately 90% of children, the processus vaginalis seals shut and becomes a thin band of tissue without a lumen or opening. If all or any portion of the processus remains open it may cause either a congenital inguinal hernia, hydrocele or a communicating hydrocele. (Fig. 42-1). The frequency that the processus vaginalis remains open is related to the gestational age of the infant and whether the testis drop into the scrotum or not. In girls, hernias are less common than in boys though inguinal hernia and, more rarely, a hydrocele may occur.
Intussusception
Meckel’s diverticulum contains all the normal intestinal layers. This out pouching has its own blood supply. Variations of this abnormality include a blind ending passage from the belly button or umbilicus, called a sinus, or a cyst inside the abdominal cavity. In other instances, there may be a cord-like attachment from the undersurface of the umbilicus to the intestine and the intestine may twist around the band resulting in blockage of the intestine.
Laparoscopic Cholecystectomy
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
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
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
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.
Neck Abnormalities
Cysts (fluid filled lumps), solid lumps, and sinuses (small openings) found about the head and neck are frequently residual structures from embryologic development that have failed to resorb completely or mature. Some lumps may be caused by inflammation, infection or even tumors affecting lymph nodes in the neck area.
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.
Neuroblastoma
Neuroblastoma is a tumor that may arise at any site in the sympathetic nervous system, including the brain, neck (3%), chest next to the spine (20%), abdomen on either side of the aorta (24%), pelvis (3%), and adrenal gland (50%) (Figure 1). It is the second most common solid tumor of infancy and childhood, being exceeded only by brain tumors. More than 25% of cases are diagnosed before 1 year of age; 50% of cases by age 2 years, and 90% by age 8 years.
Omphalocele
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
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.
Pancreas Transplantation
Pancreas transplantation has been used rarely in children, but it is likely to be used more often in the future. This is because more recent improvements in immunosuppressive drugs and surgical technique have provided better results with fewer risks, and pancreatic cell (islet cell) transplantation is now being done. Pancreas transplantation is performed either as a single-organ transplant or at the same time as a kidney transplant in patients with severe kidney disease.
Pancreas Tumors
Malignant tumors of the pancreas are rare in infants and children. In most patients, by the time the diagnosis is made, a mass can be felt, and yellowing of the skin and eyes is often present. Patients with malignant pancreatic tumors range in age from 3 months to 16 years with most patients younger than 10 years old.
Pancreatic Cysts
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.
Pancreatitis
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.
Parathyroid
There are four parathyroid glands along the behind the thyroid glands and near the edges of the gland. They are pea-sized and control calcium levels in the body. The most common disease of the parathyroid glands in children is Hyperparathyroidism.
Pectus Carinatum
Protrusion deformities of the anterior chest wall are 10 times less frequent than depression deformities. Associated disorders, including congenital heart disease, Marfan’s syndrome, spine abnormalities, and musculoskeletal defects are as frequent as in patients with pectus excavatum. The deformity typically is mild or absent in early childhood and becomes increasingly prominent during the rapid growth in early adolescence.
Pectus Excavatum
Pectus excavatum, or funnel chest, is a congenital malformation of the anterior chest characterized by a prominent depression of the body of the sternum, usually involving its lower half to two-thirds. The lower rib cartilages bend posteriorly to form a depression. The first and second ribs, and the upper sternum are essentially normal. Asymmetric deformities are common, with the depression being deeper on the right with the sternum being rotated posteriorly to that side. In most instances however, the depression is involves the lower half of the sternum and is symmetrical with a decrease in the depth of the chest cavity.
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
The true cause of pyloric stenosis is unknown. It is believed to begin as the overworked muscle around the outside of the pyloric opening at the bottom of the stomach grows too thick. This enlarged muscle blocks the passage of food from the stomach through the pylorus into the downstream intestine. After the operation, the pyloric muscle becomes completely normal. Approximately seven percent of infants with pyloric stenosis are born with other medical problems.
Short-Bowel Syndrome
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.
Solid Chest (Mediastinal) Tumors
Mediastinal tumors are fairly common in children and can be either benign (noncancerous) or cancerous (malignant). Approximately 60% are malignant. These tumors include Hodgkin’s disease, non-Hodgkin’s lymphoma, neurogenic tumors, thymomas, teratomas, lipomas, lipoblastoma, germ cell tumors, and other rare lesions.
Spleen Problems
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.
Testicular Torsion
Torsion of Testicular Appendix Torsion of an appendix testis and appendix epididymis are common causes of torsion. Both of these are small tags of normal tissue attached to the testicle.
Thyroglossal Duct Cysts and Sinuses
Embryology: The thyroid gland, although situated in the lower portion of the neck around the trachea, originates in the mouth at the back of the tongue and then moves down the neck during development. As the thyroid gland moves down to its normal position, there is a connection to the base of the tongue that should disappear by the time the thyroid reaches its final position. If it does not, there may be a persistent hollow tube that may allow accumulation of mucoid material and the formation of a cyst at the end.
Thyroid Cancer
The frequency of thyroid cancer has increased since the 1960’s, and is one of the most common pediatric cancers.
Thyroid Nodules
A thyroid nodule is a lump found in the gland. They can be malignant (cancer) or benign (not cancer). Although thyroid nodules are more common in adults than children, they are more likely (20-30%) to be malignant in children.
Thyroid Tissue at the Wrong Site (Ectopic)
Ectopic tissue is normal tissue that is found in a part of the body where it is not usually meant to be. During normal development, the thyroid forms at the base of the tongue, and later drops down into the neck. A remnant is left behind which usually involutes (goes away). If the thyroid does not descend normally, it may remain at the base of the tongue (called a lingual thyroid) or within the muscles of the neck.
Thyroiditis
Thyroiditis is enlargement of the thyroid gland from an autoimmune (antibodies to one’s own body) reaction. In this disease, the thyroid cells are damaged by antibodies. This is most commonly called Hashimoto’s thyroiditis, or autoimmune chronic lymphocytic thyroiditis. It is a common cause of enlargement of the thyroid in children, although it is more common in adults. Hashimoto’s thyroid is 10 times more common in females.
Torsion of Testicular Appendix
Torsion of an appendix testis and appendix epididymis are common causes of torsion. Both of these are small tags of normal tissue attached to the testicle.
Torticollis
Torticollis, or wry neck, results from scarring and a mass in the middle portion of the large muscle in the neck known as the sternocleidomastoid muscle.
Ulcerative Colitis
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.
Umbilical Granuloma/Polyp
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.
Umbilical Problems
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.
Undescended Testis
An empty scrotum is found in 20% to 30% of premature infants and 1.2% to 4% of mature newborns. This means that there is no testicular tissue that can be found in the scrotum. The diagnosis of true undescended testis may be difficult to determine before age 6 months, particularly in premature infants.
Urachal Abnormalities
The urachus is a fetal structure that extends up from the top of the bladder to the lower part of the umbilicus (belly button). While it may remain open throughout life, the urachus usually collapses and becomes occluded after birth. The urachus may produce symptoms if some or all of it remains open after birth.
Wilms' Tumor
Wilms’ tumor is a cancer of childhood that arises in the kidney. It is made up of cells that originate from immature tissue. Approximately 500 new cases are seen in the United States each year, representing slightly more than 10% of all cases of childhood cancer.
“Adopted from the American Pediatric Surgical Association, Parent and Family Resource website” |
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