By Laith Farid Gulli M.D.,M.S., Nicole Mallory M.S.,PA-C, Abraham F. Ettaher M.D., Robert Ramirez B.S.The Gale Group Inc., Gale…. Gale Encyclopedia of Surgery, 2004more »
Definition
Pediatric surgery is a specialized field of surgery for the treatment of conditions that can be surgically corrected in a baby, child, or adolescent.
Purpose
The purpose of pediatric surgery varies with the procedure. In general, the purpose is to surgically correct a congenital condition, disease, traumatic injury, or other disorder in the pediatric patient.
Demographics
Pediatric surgeons provide treatment for young patients—newborns up through late adolescence.
Description
Pediatric surgery is the surgical branch that uses operative techniques to correct certain pediatric conditions (i.e., congenital abnormalities, tumors, chronic diseases, and traumatic injuries). There are different specialties within the field that include:
pediatric neurological (brain and spinal cord) surgery
pediatric plastic (reconstructive and cosmetic) surgery
The American Academy of Pediatrics has established specific guidelines for referral to subspecialists. The pediatric patient has special considerations that differentiate him or her, both physically and psychologically, from an adult. A neonate (newborn) poses great challenge in surgical treatment since the tiny structures and immature organ systems may not cope with disease-induced stress and the physical demands of a major operative procedure. A newborn infant may still be developing key bodily functions, or may have special requirements. Key areas of concern in the newborn include:
special requirements for fluid, electrolyte (necessary elements such as sodium, potassium, and calcium) and nutrition
The pediatric surgeon must take into account the special requirements unique to the young surgical patient. The pediatric surgeon is trained to treat the entire spectrum of surgical illnesses. The following is an overview (with symptoms) of the more common pediatric conditions that require surgery typically performed by the pediatric surgeon.
Alimentary tract obstruction
Obstruction of the alimentary tract (tubes of digestion extending from the mouth to the anus) is characterized by four cardinal symptoms:
abdominal distention (an abdomen that becomes large and appears swollen)
PYLORIC ATRESIA AND RELATED CONDITIONS. Pyloric atresia is a condition that occurs when the pyloric valve, located between the stomach and duodenum, fails to open. Food cannot pass out of the stomach, resulting in vomiting clear gastric juice at attempted feedings. Maternal polyhydramnios is present before birth in more than 60% of cases.
Other areas of the colon (duodenum, jejunum, ileum) can be obstructed during development, with symptoms present at birth. Most of these disorders share the four cardinal symptoms of alimentary obstruction.
INTUSSUSCEPTION. Intussusception accounts for 50% of intestinal obstruction in patients who are three months to one year of age. Eighty percent of cases are observed by the child's second birthday. The cause of intussusception is not known, and it is more common in males who are well nourished and apparently healthy. The symptoms include a sudden onset of abdominal pain characterized by episodic screaming and drawing up of the legs. In 60% of patients, vomiting and blood in the stool are common findings (either bright red or occult [hidden] blood). Typically, the bowel movements look like currant jelly, consisting of mucus and blood mixed together. Currant jelly stool is the most common clinical observation for patients with intussusception. During physical examination, patients will exhibit abdominal distention, and in 65% of cases there is a sausage-shaped mass that can be felt in the upper right portion of the abdomen toward the mid-abdomen. Ultrasound studies are a reliable method of diagnosis.
FAILURE TO PASS MECONIUM. Failure to pass meconium (meconium ileus) is associated with cystic fibrosis (a genetic disorder), colonic obstruction (colonic atresia), meconium plug syndrome, and aganglionic mega-colon (also called Hirschsprung's disease, a congenital absence of the nerves that provide gastrointestinal tract mobility).
Anorectal anomalies
There are many different types of anorectal anomalies common to male and female neonates, as well as deformities that are gender-specific since involvement of genitalia can occur. The surgery for these cases is complicated, and must be performed by an experienced pediatric surgeon. Complications of these procedures could result in permanent problems.
Necrotizing enterocolitis (NEC)
NEC affects 1–2% of patients admitted to a neonatal intensive care unit. It is a life-threatening illness characterized by abdominal distention, bilious vomiting, lethargy, fever, occult (not obvious) or gross (clearly seen) rectal bleeding. Additionally, affected patients may exhibit signs of hypothermia (temperature less than 96.5°F or 35.8°C), bradycardia (slow heart rate), abdominal mass (felt during palpation), oliguria, jaundice, and episodes of breathlessness (apnea). Survival of NEC surgery can be expected for 60–70% of patients.
Abdominal wall defects
Omphalocele is a defect that involves protrusion of abdominal contents into an external sac. This disorder occurs in one per 5,000 births. More than 50% of omphalocele patients have serious genetic deformities involving these body systems: cardiovascular (heart), musculoskeletal (muscle and bones), genitourinary (genital and bladder systems), and central nervous (brain and spinal cord). The overall survival rate for infants with omphalocele varies, and depends on defect size, other associated genetic abnormalities, and age of newborn. (Many infants with omphalocele are premature.) Approximately 33% of patients with omphaloceles do not survive.
GASTROSCHISIS. Gastroschisis is a defect in the abdominal wall to the side (lateral) of the umbilicus. It usually occurs to the right of an intact normal umbilical cord. The cause is unknown. The bowel protrudes to the outside of the abdomen during intrauterine life (while the embryo is developing inside the uterus). The amniotic fluid has an irritating effect on the exposed bowel, and causes infection of the bowels. The problem can be detected by ultrasound studies during pregnancy. Some pediatric surgeons and obstetricians recommend cesarean section (early elective delivery) to spare bowel trauma. The newborn patients typically require surgery, tube feedings for three to four weeks, and hospitalization for several weeks. The current survival rate for infants with gastroschisis is greater than 90%.
Congenital diaphragmatic hernia (CDH)
CDH can be diagnosed by the fourth month of pregnancy via ultrasound studies. Of the infants with congenital diaphragmatic hernia (CDH), 44–66% have other congenital abnormalities as a result of developmental malformations. Anatomically, patients with CDH have a defect in development that allows a communication between the chest and abdomen. Through this defect, the abdominal contents enter the lung cavity and interfere with normal lung development. The incidence is approximately one per 2,200 live births, and males are more commonly affected than females. Usually the infants are full-term, and the defect occurs on the left side in the majority—88%—of patients.
Treatment is extensive, and usually requires three major areas:
Postoperatively, the infant is monitored in the neonatal intensive care setting. The postoperative period is more critical if a lung is severely underdeveloped.
Pyloric stenosis (PS)
Pyloric stenosis is an obstruction in the intestine due to a larger-than-normal size of the muscle fibers of the pylorus (lower stomach opening). Pyloric stenosis is a common hereditary condition that affects males more than females, and occurs in one per 750 births. The typical symptoms include a progressive, often projectile, vomiting after attempted feedings. The gastric vomitus (bloody in 80% of patients) usually begins during the second and third week of life, and increases in force and frequency. Typically, the infant fails to gain weight, and the number of bowel movements and rate of urination decreases.
Physical examination is usually helpful in establishing a diagnosis. Palpation of the enlarged muscle fibers can be felt as an olive-shaped mass located in the midline approximately one-third to one-half of the distance from the umbilicus to the xiphoid (end of the breast bone), when the stomach is empty. Careful abdominal examination and palpation can usually identify the pyloric mass in 85% of cases.
Gastroesophageal reflux
Gastroesophageal reflux (GER) is a common disorder in infancy, and usually disappears by the baby's first birthday. The largest group of patients with clinically significant GER are those who have neurologic impairment. Symptoms often include vomiting, repeated lung infections (from aspirating gastric contents during regurgitation of foodstuffs), and delayed gastric emptying. The success rate with infants who have procedures necessary to correct GER is over 90%.
Meckel's diverticulum
Meckel's diverticulum occurs in approximately 2% of the U.S. population. The diverticulum is an outgrowth of intestine is located in a portion of the intestines called the ileum. Symptoms of obstruction are more often observed in infants, and bleeding is more common in patients after age four.
Intestinal polyps
Juvenile polyps are usually present between the ages of four and 14 years, and tend to be inflammatory. The most common symptom of intestinal polyps is rectal bleeding, which is commonly due to a solitary polyp (80% of cases). Diagnosis can be done by proctosigmoidoscopy, which allows visualization of 85% of polyps.
Acute appendicitis
Acute appendicitis is a relatively common surgical emergency that is misdiagnosed in 28% of patients due to a broad spectrum of symptoms that can confuse the clinician. The classic clinical symptom of acute appendicitis is the onset of pain in the middle region of the abdomen that is followed by anorexia, nausea, and vomiting. The pain is persistent and radiates to the right lower abdomen, becoming more intense and localized. The physical and abdominal examinations must be carefully and accurately performed. Patients with acute appendicitis usually have an increased white blood cell (cells that fight infection) count.
Once the diagnosis is established, the child is prepared for surgery. Preoperative antibiotics are started at least one-half hour before the operation. If the appendix is perforated (ruptured), complications can occur as a result of kidney (renal) failure, seizures due to fever, and gram-negative sepsis (an infection that enters the bloodstream and interferes with life-saving chemical reactions). Patients who are very young, or those who were misdiagnosed and incurred long delays in treatment, are susceptible to death.
The diagnosis of inflammatory bowel disease is usually based on presenting clinical symptoms, laboratory analysis results, endoscopic appearance, and radiologic findings. Approximately 50–60% of patients have bloody diarrhea, severe cramping, abdominal pain, and urgency.
CROHN'S DISEASE. The symptoms of Crohn's disease includes cramping abdominal pain, diarrhea, and strictures (constriction) resulting from bowel obstruction. Removal of diseased portions in children with Crohn's disease may be temporarily beneficial, but recurrence after surgical removal occurs in about 50% of cases within four years. Chronic symptoms may remain into adult life, making long-term follow-up essential.
ULCERATIVE COLITIS. Ulcerative colitis is limited to the colon. A surgical procedure known as colectomy is curative, and indicated for intractable disease (64% of patients). Colectomy is the removal of the entire colon, or the inflamed part of it.
Biliary tract disorders
A variety of biliary tract conditions may be present at birth, some requiring surgical correction.
NEONATAL JAUNDICE. Neonatal jaundice is common, and results from an immature system not capable of some basic biochemical reactions. Food intake can help speed these reactions, which usually resolves the condition within seven to 10 days. Jaundice that persists for over two weeks is abnormal, and could be caused by over 30 possible disorders.
BILIARY ATRESIA. Biliary atresia is a disease that causes inflammation of the ducts within the biliary system, resulting in fibrosis of these ducts. The incidence of biliary atresia is one per 15,000 live births, and is more common in females. Time is critical, and most patients must have surgery by two months of life. Approximately 25–30% of patients who receive early operative intervention have long-term successful outcomes. Some patients may require liver transplantation, and 85–90% of these patients survive.
CHOLELITHIASIS. Gallbladder obstruction in infants and young children is usually caused by pigmented (colored) stones resulting from blood disorders. Removal of the gallbladder (laparoscopic cholecystectomy) is the treatment of choice.
Trauma
Accidents are the leading cause of death in children between the ages of one and 15 years, and accounts for 50% of all deaths in the pediatric age group. More than half of these deaths are due to motor vehicle accidents, followed by falls, bicycle injuries, drowning, burns, child abuse, and birth trauma. Head trauma is the single most common organ associated with traumatic death. Within recent years, the number of fatalities related to the use of firearms and violence has increased.
More than 20 million children each year sustain injuries requiring treatment. These injuries account for 100,000 cases of permanent pediatric disability. Response to trauma in pediatric patients is significantly different from older patients. Pediatric patients require special attention concerning temperature regulation, blood volume, metabolic rate and requirements, and airway maintenance. Other special pediatric considerations include response to stress, communication difficulties, psychological trauma, a different pediatric trauma score system, smaller airway diameter, and increased risk of aspirating gastric contents (which could cause pneumonia). Pediatric trauma patients should have access to appropriate pre-hospital transportation, and must receive medical attention in a pediatric trauma center capable of providing the complex level of care necessary for serious pediatric trauma situations.
Neck masses
Neck masses during infancy and childhood may be caused by tumors or infections, or they may be congenital. Lymphadenitis is an infection of a lymph node that becomes enlarged and tender. Most cases are resolved by treating the primary source of infection (i.e., middle ear infection and tonsillitis). Some inflamed nodes may require an incision and drainage of infection.
Hernias
INGUINAL HERNIA AND HYDROCELE. Inguinal (groin) hernia is the most frequent disorder requiring surgery in the pediatric age group. Clinically, a right-sided inguinal hernia is more common in males (60% of cases), and there is a familial tendency. The incidence is higher in full-term infants (3.5–5%). Full-term infants and older children (without underlying diseases) can receive surgical repair in an outpatient setting. An inguinal hernia may result in herniation of the scrotum, and a communicating hydrocele (hernia with a small connection to the peritoneal cavity).
UMBILICAL HERNIA. Umbilical hernia is a defect of the umbilical ring, and is more common in females and African American children. Spontaneous involution occurs in 80% of cases. Larger defects may be observed for several years without complications, and their spontaneous resolution is possible. If the umbilical hernia persists, patients may develop feeding intolerance, pain, and local skin breakdown.
Undescended testes
Undescended testes are observed in 1–2% of full-term males. Approximately 30% of preterm males may have an undescended testis. Undescended testis in premature infants may descend by the first year of life, and observation is often the treatment during that time.
Tumors
Wilm's tumor (nephroblastoma) is a tumor in the kidneys that forms during embryonic development. The tumor is due to a genetic abnormality; and approximately 80% of children are diagnosed between one and five years of age. In about 75–95% of cases, the patient has an abdominal mass that is detected by a parent during bathing. Blood in the urine (hematuria) occurs in 10–15% of cases, and high blood pressure (hypertension) is present in 20–25% of cases. Hypertension is the result of the tumor compressing the kidney in a specific area, causing it to release a chemical called renin, which elevates blood pressure. During physical examination, the Wilm's tumor is a smooth, round, hard, nontender flank mass. The treatment of Wilm's tumor depends on its stage, and may include surgery, chemotherapy, or radiotherapy.
BOOKS
Townsend, Courtney. Sabiston Textbook of Surgery 16th ed. St. Louis: W. B. Saunders Company, 2001.
PERIODICALS
Coran, A. "American Academy of Pediatrics: Guidelines for Referral to Pediatric Surgical Specialists." Pediatrics 110, no.1 (July 2002).
Okada P. J., B. Hicks. "Pediatric Surgical Emergencies: Neonatal Surgical Energencies." Clinical Pediatric Emergency Medicine 3, no.1 (March 2002:).
ORGANIZATIONS
The American Pediatric Surgical Association. 60 Revere Drive Suite 500 Northbrook, Il 60062. (847) 480-9576. Fax: (847) 480-9282 E-mail: eapsa@eapsa.org.
Laith Farid Gulli, M.D.,M.S.
Nicole Mallory, M.S.,PA-C
Abraham F. Ettaher,M.D.
Robert Ramirez, B.S.
WHO PERFORMS THE PROCEDURE AND WHERE IS IT PERFORMED?
Pediatric surgery is performed by a pediatric surgeon who has had five years of general surgery training, along with further specialized instruction and experience, and is certified in pediatric general surgery or in a specific pediatric specialty.
