Etiology

The exact cause is unknown, although infectious, genetic, and environmental factors are important. There is an increased familial incidence, and the disease is increased in persons with blood group O.

There is a significant relationship between the bacterium Helicobacter pylori (H. pylori) and ulcers. H. pylori is a microaerophilic, gram-negative, slow-growing, spiral-shaped, and flagellated bacterium known to colonize the gastric mucosa in about half of the population of the world (Cover and Blaser, 1996). It has been identified in 90% to 100% of adult patients with PUD. H. pylori synthesizes the enzyme urease, which hydrolyses urea to form ammonia and carbon dioxide. Ammonia then absorbs acid to form ammonium, thus raising the gastric pH. Also, its flagella allow the bacterium to swim across the viscous gastric mucus and reach the more neutral gastric pH below the mucus (Sgouros and Bergele, 2006). H. pylori may cause ulcers by weakening the gastric mucosal barrier and allowing acid to damage the mucosa. It is believed that it is acquired via the fecal-oral route, and this hypothesis is supported by finding viable H. pylori in feces.

In addition to ulcerogenic drugs, both alcohol and smoking contribute to ulcer formation. There is no conclusive evidence to implicate particular foods, such as caffeine-containing beverages or spicy foods, but polyunsaturated fats and fiber may play a role in ulcer formation. Psychologic factors may play a role in the development of PUD, and stressful life events, dependency, passiveness, and hostility have all been implicated as contributing factors.

Pathophysiology

Most likely, the pathology is due to an imbalance between the destructive (cytotoxic) factors and defensive (cytoprotective) factors in the GI tract. The toxic mechanisms include acid, pepsin, medications such as aspirin and nonsteroidal antiinflammatory drugs (NSAIDs), bile acids, and infection with H. pylori. The defensive factors include the mucus layer, local bicarbonate secretion, epithelial cell renewal, and mucosal blood flow. Prostaglandins play a role in mucosal defense because they stimulate both mucus and alkali secretion. The primary mechanism that prevents the development of peptic ulcer is the secretion of mucus by the epithelial and mucus glands throughout the stomach. The thick mucus layer acts to diffuse acid from the lumen to the gastric mucosal surface, thus protecting the gastric epithelium. The stomach and the duodenum produce bicarbonate, decreasing acidity on the epithelial cells and thereby minimizing the effects of the low pH (Chelimsky and Czinn, 2001). When abnormalities in the protective barrier exist, the mucosa is vulnerable to damage by acid and pepsin. Exogenous factors, such as aspirin and NSAIDs, cause gastric ulcers by inhibition of prostaglandin synthesis.

Zollinger-Ellison syndrome may occur in children who have multiple, large, or recurrent ulcers. This syndrome is characterized by hypersecretion of gastric acid, intractable ulcer disease, and intestinal malabsorption caused by a gastrin-secreting tumor of the pancreas.

Diagnostic Evaluation

Diagnosis is based on the history of symptoms, physical examination, and diagnostic testing. The focus is on symptoms such as epigastric abdominal pain, nocturnal pain, oral regurgitation, heartburn, weight loss, hematemesis, and melena (Box 24-9). History should include questions relating to the use of potentially causative substances such as NSAIDs, corticosteroids, alcohol, and tobacco. Laboratory studies may include a CBC to detect anemia, stool analysis for occult blood, liver function tests (LFTs), sedimentation rate, or CRP to evaluate IBD; amylase and lipase to evaluate pancreatitis; and gastric acid measurements to identify hypersecretion. A lactose breath test may be performed to detect lactose intolerance.

Radiographic studies such as an upper GI series may be performed to evaluate obstruction or malrotation. An upper endoscopy is the most reliable procedure to diagnose PUD. A biopsy is taken to determine the presence of H. pylori. It can also be diagnosed by a blood test that identifies the presence of the antigen to this organism. The C-urea breath test measures bacterial colonization in the gastric mucosa. This test is used to screen for H. pylori in adults and children. Polyclonal and monoclonal stool antigen tests are an accurate noninvasive method both for the initial diagnosis of H. pylori and for the confirmation of its eradication after treatment (Gisbert, de la Morena, and Abraira, 2006).

Therapeutic Management

The major goals of therapy for children with PUD are to relieve discomfort, promote healing, prevent complications, and prevent recurrence. Management is primarily medical and consists of administration of medications to treat the infection and to reduce or neutralize gastric acid secretion.

Antacids are beneficial medications to neutralize gastric acid.

Histamine (H₂) receptor antagonists (antisecretory drugs) act to suppress gastric acid production. Cimetidine (Tagamet), ranitidine (Zantac), and famotidine (Pepcid) are examples of these medications. These medications have few side effects.

PPIs, such as omeprazole and lansoprazole, act to inhibit the hydrogen ion pump in the parietal cells, thus blocking the production of acid. Controlled studies of these drugs have been done in adults, and these drugs are now commonly used to treat ulcers in children. They appear to be well tolerated and to have infrequent side effects (e.g., headache, diarrhea, nausea and vomiting).

Mucosal protective agents, such as sucralfate and bismuth-containing preparations, may be prescribed for PUD. Sucralfate is an aluminum-containing agent that forms a protective barrier over ulcerated mucosa to protect against acid and pepsin. Sucralfate is available in both pill and liquid forms. Because sucralfate blocks the absorption of other medications, it should be given separately from other medications.

Bismuth compounds are sometimes prescribed for the relief of ulcers, but they are used less frequently than PPIs. Although these compounds inhibit the growth of microorganisms, the mechanism of their activity is poorly understood. In combination with antibiotics, bismuth is effective against H. pylori. Although concern has been expressed about the use of bismuth salts in children because of potential side effects, none of these side effects has been reported when these compounds have been used in the treatment of H. pylori infection.

Triple drug therapy is the recommended treatment regimen for H. pylori (Ford, Delaney, Forman, and others, 2004). Combination therapy has demonstrated 90% effectiveness in eradication of H. pylori when compared with antibiotic monotherapy. Examples of drug combinations used in triple therapy are (1) bismuth, clarithromycin, and metronidazole; (2) lansoprazole, amoxicillin, and clarithromycin; and (3) metronidazole, clarithromycin, and omeprazole. Common side effects of medications include diarrhea, nausea, and vomiting. The yearly relapse rate of 80% for duodenal ulcer and 60% for gastric ulcer can be reduced to less than 5% after successful H. pylori eradication (Ford, Delaney, Forman, and others, 2004).

In addition to medications, the child with PUD should be given a nutritious diet and advised to avoid caffeine. Adolescents are warned about gastric irritation associated with alcohol use and smoking.

Children with an acute ulcer who have developed complications, such as massive hemorrhage, require emergency care. The administration of IV fluids, blood, or plasma depends on the amount of blood loss. Replacement with whole blood or packed cells may be necessary for significant loss.

Surgical intervention may be required for complications such as hemorrhage, perforation, or gastric outlet obstruction. Ligation of the source of bleeding or closure of a perforation is performed. A vagotomy and pyloroplasty may be indicated in children with recurring ulcers despite aggressive medical treatment.

Nursing Care Management

The primary nursing goal is to promote healing of the ulcer through compliance with the medication regimen. If an analgesic-antipyretic is needed, acetaminophen, not aspirin or NSAIDs, is used. Critically ill neonates, infants, and children in intensive care units should receive H₂ blockers to prevent stress ulcers. Critically ill children receiving IV H₂ blockers should have their gastric pH values checked at frequent intervals.

The role of stress in ulcer formation should be considered for nonhospitalized children with chronic illnesses. In children, many ulcers occur secondary to other conditions, and the nurse should be aware of family and environmental conditions that may aggravate or precipitate ulcers. Children may benefit from psychologic counseling and from learning how to cope constructively with stress.

Etiology

Hepatitis is an acute or chronic inflammation of the liver that can result from several different causes (e.g., virus, chemical or drug reaction, or other diseases). Nonviral causes of hepatitis include autoimmune hepatitis, Wilson disease, α₁-antitrypsin deficiency, and steatohepatitis. The following six viruses cause 90% of cases of viral hepatitis (Table 24-4):

Diagnostic Evaluation

Diagnosis is based on the history (especially regarding possible exposure to a hepatitis virus); physical examination; and serologic markers (antibodies or antigens) indicating the presence of active infection with hepatitis A, B, or C or previous infection. Because the liver has a large functional reserve, abnormal laboratory tests may be the only indication of hepatitis. However, LFTs are not specific for the diagnosis of viral hepatitis. Although serum aspartate and alanine aminotransferase (AST and ALT) levels are markedly elevated in viral hepatitis, other diseases or conditions may cause their elevation. Serum bilirubin levels peak 5 to 10 days after clinical jaundice appears. When hepatitis is severe, albumin levels are depressed and prothrombin times are increased.

Diagnosis of viral hepatitis is based on the presence of specific viral markers. Diagnosis of acute HAV infection is based on the presence of anti-HAV immunoglobulin (immunoglobulin M [IgM]) antibody in the serum. HBV diagnosis depends on the presence of hepatitis B surface antigen (HBsAg) or anti-HBV core (anti-HBc) IgM antibody. Chronic HBV infection is associated with the persistence of HBsAg and HBV DNA markers. The diagnosis of HCV is based on the detection of anti-HCV antibodies and confirmation by polymerase chain reaction for hepatitis C RNA.

An abdominal ultrasound provides measurement of liver size, detection of cystic lesions and stones, and imaging of the gallbladder. Cholescintigraphy radionuclide imaging detects abnormalities in liver uptake, concentration, and excretory function. Finally, a liver biopsy aids in assessing the severity of the disease.

Pathophysiology

Pathologic changes occur primarily in the parenchymal cells of the liver and result in varying degrees of swelling, infiltration of liver cells by mononuclear cells, subsequent degeneration, necrosis, and fibrosis.

Hepatitis can be self-limited, and complete regeneration of liver cells without scarring may occur. However, some forms of hepatitis do not result in complete return of liver function. These include fulminant hepatitis, which is characterized by a severe, acute course and massive destruction of the liver, resulting in liver failure and death in 1 to 2 weeks. Subacute or chronic active hepatitis is characterized by progressive liver destruction, uncertain regeneration, scarring, and potential cirrhosis.

The initial anicteric (absence of jaundice) phase usually lasts 5 to 7 days and is often mistaken for influenza. Symptoms include nausea, vomiting, extreme anorexia, malaise, easy fatigability, arthralgia, skin rashes, slight to moderate fever, and epigastric or upper right quadrant abdominal pain. Dark urine is a symptom of the icteric (jaundice) phase. Pruritus may accompany jaundice and can be bothersome, but many children with acute viral hepatitis do not develop jaundice.

Therapeutic Management

Treatment options for viral hepatitis are limited. The goals of management include early detection, recognition of chronic liver disease, support and monitoring, and prevention of spread of the disease.

HAV infection is an acute disease that resolves with support and management of symptoms. HBV and HCV treatment is directed at managing the viral load to prevent further destruction of the liver. Currently, HBV and HCV are treated with interferons, naturally occurring proteins that exert antiviral, antiproliferative, and immunomodulatory effects. A recent interferon formulation, pegylated interferon, can be administered once a week and has been found to sustain plasma levels and enhance viral suppression (Karnam and Reddy, 2003). Lamivudine and adefovir are two other interferon analogs that suppress the replication of HBV (Yuen and Lai, 2001). A combination of α-interferon and ribavirin has resulted in a sustained response in only 50% of patients with HBV and HCV (Waters and Nelson, 2006).

Another important aspect of the therapeutic management of hepatitis involves hospitalization. Hospitalization is necessary if coagulopathy or fulminant hepatitis is present.

Nursing Care Management

Nursing objectives depend on the severity of the hepatitis, the medical management, and factors influencing the control and transmission of the disease. Children with benign viral hepatitis are frequently cared for at home, and the clinic or office nurse must explain the medical therapy and control measures. If further assistance is needed for parents to comply with therapy, a public health nursing referral may be necessary.

A well-balanced diet and a realistic schedule of rest and activity adjusted to the child’s condition are encouraged. HAV is not infectious within a week after the onset of jaundice, and children may feel well enough to resume school. Parents are cautioned about administering any medication to the child, since normal dosages of many drugs may become dangerous because of the liver’s inability to detoxify and excrete them. Hand washing is the single most critical measure in reducing risk of transmission. The nurse should explain to parents and children the ways in which HAV (oral-fecal route) and HBV (parenteral route) are spread.

Nurses caring for young people with HBV infection and a known or suspected history of illicit drug use should help these teens realize the dangers of drug abuse. Nurses should stress the parenteral mode of transmission of hepatitis and encourage them to seek counseling through a drug program. HBV and HCV are chronic diseases that require frequent monitoring and management. Many communities have multidisciplinary clinics dedicated to the management of these diseases.

Therapeutic Management

Therapy is directed toward (1) frequent assessment of liver status with physical examination and LFTs and (2) management of specific complications. The only successful treatment for end-stage liver disease and liver failure may be liver transplantation, which has improved the prognosis substantially for many children with cirrhosis. Currently, the 1-year survival rate for liver transplantation is 85%. Increasing numbers of recipients are reaching their second decade after transplant. The increasing life span after transplantation is related to advances in surgical techniques and improved preoperative, intraoperative, and postoperative care (Atkison, Ross, Williams, and others, 2002).

Nursing Care Management

Nursing care of the child with cirrhosis is determined by the cause of the cirrhosis, the severity of complications, and the prognosis. The prognosis for life is poor unless successful liver transplantation occurs. Nursing care of this child is similar to that for any child with a life-threatening illness (see Chapter 18). Hospitalization is usually required when complications occur.

Etiology and Pathophysiology

The exact cause of BA is unknown. Because BA has two distinct forms, postnatal and fetal-embryonic, different pathogenic mechanisms are suggested. Postnatal BA represents 65% to 90% of cases and is probably the result of infection or an immune-mediated mechanism. Jaundice, manifesting with yellow discoloration of the skin or sclerae, is the most common early symptom of BA. Jaundice, indicating cholestasis (the accumulation of compounds that cannot be excreted because of occlusion or obstruction of the biliary tree), can be visible at a total serum bilirubin concentration as low as 5.0 mg/dl. An abnormal direct bilirubin has been designated as greater than 1.0 mg/dl if the total bilirubin is less than 5 mg/dl or a value of direct bilirubin that represents more than 20% of the total bilirubin if it is greater than 5 mg/dl (Emerick and Whitington, 2006). Direct hyperbilirubinemia first appears after the resolution of physiologic jaundice. Jaundice is often associated with pale stool and dark urine. Histologic study demonstrates bile duct remnants and a progressive inflammatory process. In the fetal embryonic form of BA, which represents 10% to 35% of cases, there is a congenital absence of biliary ductal patency and an absence of bile duct remnants. Many infants have associated congenital anomalies. Varying degrees of cholestasis occur, resulting in retention of irritants and toxins. Injury to the liver occurs as the result of the inflammation caused by the cholestasis.

Diagnostic Evaluation

Early diagnosis is the key to the survival of the child with BA. Infants who undergo surgery in the first 60 days of life have an 80% chance of establishing bile flow. Between 60 to 90 days of life, the chance of reestablishing flow drops to 50%, and after 90 days to 10% (Chen, Chang, Du, and others, 2006). The typical infant is thriving, appears well, and has only very mild jaundice during the first 6 to 8 weeks (Emerick and Whitington, 2006) but will soon begin failing to thrive. Several clinical signs may indicate the presence of BA (Box 24-10). Blood tests should include a CBC, electrolytes, bilirubin, and liver enzymes. Additional laboratory analyses, including α₁-antitrypsin level, TORCH titers (see Maternal Infections, Chapter 9), hepatitis serology, α-fetoprotein, urine cytomegalovirus, and a sweat test, are indicated to rule out other conditions that cause persistent cholestasis and jaundice. Abdominal ultrasonography allows inspection of the liver and biliary system. Hepatobiliary scintigraphy demonstrates biliary patency but does not provide diagnostic certainty. Endoscopic retrograde cholangiopancreatography (ERCP) is performed in very young infants. This procedure, which is done using general anesthesia, has an 80% reported diagnostic accuracy. Percutaneous liver biopsy is highly reliable when the biopsy contains specimens from a number of portal areas. Definitive diagnosis of BA is obtained during surgical laparotomy and an intraoperative cholangiogram.

Therapeutic Management

The primary treatment of BA is hepatic portoenterostomy (Kasai procedure), in which a segment of intestine is anastomosed to the resected porta hepatis to attempt bile drainage. Bile drainage is achieved in approximately 80% to 90% of infants who undergo surgery when younger than 10 weeks of age (Ohi, 2001). However, progressive cirrhosis still occurs in many children, necessitating liver transplantation. Prophylactic antibiotics are given after the Kasai procedure to minimize the risk of ascending cholangitis.

Medical management is primarily supportive. It includes nutritional support with infant formulas that contain medium-chain triglycerides and essential fatty acids. Supplementation is usually required with fat-soluble vitamins; a multivitamin; and minerals, including iron, zinc, and selenium. Aggressive nutritional support with continuous tube feedings or TPN is indicated for moderate to severe failure to thrive. The enteral solution should be low in sodium. Ursodeoxycholic acid is used to treat pruritus and hypercholesterolemia.

Nursing Care Management

Nursing interventions for the child with BA include support of the family before, during, and after surgical procedures and education regarding the treatment plan. In the postoperative period of a portoenterostomy, nursing care is similar to that after major abdominal surgery. Family members need education relating to the proper administration of medications and nutritional therapy, including special formulas, vitamin and mineral supplements, tube feedings, or parenteral nutrition. Pruritus can often be relieved by drug therapy or comfort measures such as baths.

Children and their families also need psychosocial support. The uncertain prognosis, discomfort, and waiting for transplantation produce stress, and hospitalizations, pharmacologic therapy, and nutritional therapy impose financial burdens on the family. Families can receive help from the Children’s Liver Disease Foundation,^* an organization that provides educational materials, programs, and support systems.

Etiology

Cleft deformities may be an isolated anomaly, or they may occur with a recognized syndrome. CL with or without CP is distinct from isolated CP. Clefts of the secondary palate alone are more likely to be associated with syndromes than is isolated CL or CL with CP.

CL and CP may be caused by exposure to teratogens such as alcohol, anticonvulsants, steroids, and retinoids, but there is little evidence to link isolated clefts to any single teratogenic agent with the exception of phenytoin. Use of phenytoin during pregnancy is associated with a tenfold increase in the incidence of CL. The incidence of CL among mothers who smoke during pregnancy is twice as great as the incidence in mothers who do not (Eppley, van Aalst, Robey, and others, 2005).

Pathophysiology

Cleft deformities represent a genetic defect in cell migration that results in a failure of the maxillary and premaxillary processes to come together between the third and twelfth week of embryonic development. Although often appearing together, CL and CP are distinct malformations embryologically, occurring at different times during the developmental process. Merging of the upper lip at the midline is completed between the seventh and eleventh weeks of gestation. Fusion of the secondary palate (hard and soft palate) takes place later, between the seventh and twelfth weeks of gestation. In the process of migrating to a horizontal position, the palates are separated by the tongue for a short time. If there is delay in this movement, or if the tongue fails to descend soon enough, the remainder of development proceeds but the palate never fuses.

Diagnostic Evaluation

CL with or without CP is apparent at birth. The defect elicits severe emotional reactions in parents. CP is less obvious than CL and may not be detected without a thorough assessment of the mouth. CP is identified when the examiner places a gloved finger directly on the palate. Clefts of the hard palate form a continuous opening between the mouth and the nasal cavity. The severity of the CP has an impact on feeding; the infant is unable to generate negative pressure and create suction in the oral cavity. This impairs feeding, even though in most cases the infant’s ability to swallow is normal.

Prenatal diagnosis with fetal ultrasound is not reliable until the soft tissues of the fetal face can be visualized at 13 to 14 weeks. The sensitivity of fetal ultrasound for facial clefting is almost 100% when CL/P is associated with other structural anomalies. An intact lip is the most difficult to diagnose prenatally (Wilkins-Haug, 2003).

Therapeutic Management

Treatment of the child with CL is surgical and involves no long-term interventions other than possible scar revision. The management of CP involves the cooperative efforts of a multidisciplinary health care team, including pediatrics, plastic surgery, orthodontics, otolaryngology, speech/language pathology, audiology, nursing, and social work. Management is directed toward closure of the cleft(s), prevention of complications, and facilitation of normal growth and development in the child. Until recently, repair of cleft deformities in the neonate was not considered safe. Surgery is now possible in younger neonates because of advances in pediatric anesthesiology and neonatology. However, the infant must be free of any oral, respiratory, or systemic infections. In deformities of both the lip and palate, the palate is repaired first to avoid disrupting the lip after it has been repaired.

Nursing Care Management

The immediate nursing problems for an infant with CL/P deformities are related to feeding and dealing with the parental reaction to the defect. Facial deformities are particularly disturbing to parents. CL is a visible defect that may produce a strong negative response in parents, which could influence maternal- and paternal-infant attachment. However, a study of infants with CL or CP indicated that maternal-infant attachment was not negatively affected when measured at 1 year of age (Speltz, Endriga, Fisher, and others, 1997). During the initial phase following birth, it is important for the nurse to emphasize not only on the infant’s physical needs, but also the parents’ emotional needs. The manner of handling the infant should convey to the parents that the infant is a precious human being. (See Chapter 18 for interventions in assisting parents in accepting a birth defect.) Throughout the course of therapy, parents need explanations of the immediate and long-range problems associated with CP. They may be unaware that more is involved than repairing the defect. Whenever possible, they should be referred to a comprehensive CP team.

Etiology

Esophageal atresia (EA) with or without an associated TEF is the most common esophageal malformation, occurring in approximately 1 in 3500 live births (Shaw-Smith, 2006). There appears to be an equal sex incidence, but the birth weight of most affected infants is significantly lower than average, and incidence of prematurity is unusually high. A history of maternal polyhydramnios is present in approximately 50% of infants with the defects. EA/TEF is often present with the VATER or VACTERL syndromes, acronyms for syndromes involving a combination of Vertebral, Anorectal, Cardiovascular, Tracheoesophageal, Renal, and Limb abnormalities. The cardiac and renal anomalies occur most frequently with EA/TEF.

Pathophysiology

The cause of EA/TEF is unknown. In the most frequently encountered form of EA and TEF (80% to 95% of cases), the proximal esophageal segment terminates in a blind pouch, and the distal segment is connected to the trachea or primary bronchus by a short fistula at or near the bifurcation (see Fig. 24-4, C). The second most common variety (5% to 8%) consists of a blind pouch at each end, widely separated and with no communication to the trachea (see Fig. 24-4, A). Less frequently, an otherwise normal trachea and esophagus are connected by a common fistula (see Fig. 24-4, E). Extremely rare anomalies involve a fistula from the trachea to the upper esophageal segment (see Fig. 24-4, B) or to both the upper and the lower segments (see Fig. 24-4, D).

Diagnostic Evaluation

The disorder is suspected on the basis of clinical manifestations (Box 24-11). EA should also be suspected in cases of maternal polyhydramnios. Although the diagnosis is established on the basis of clinical signs and symptoms, the exact type of anomaly is determined by radiographic studies. A radiopaque catheter is inserted into the hypopharynx and advanced until it encounters an obstruction. Chest films are taken to ascertain esophageal patency or the presence and level of a blind pouch. Sometimes fistulas are not patent, which makes them more difficult to diagnose. The presence of gas in the stomach or small bowel is indicative of a coexisting TEF.

Therapeutic Management

EA is a surgical emergency. The treatment includes maintenance of a patent airway, prevention of pneumonia, gastric or blind pouch decompression, and surgical repair of the anomaly. When EA/TEF is suspected, the infant is immediately taken off oral intake, started on IV fluids, and placed in the position least likely to cause aspiration of either mouth or stomach secretions. Removal of secretions from the mouth and upper pouch requires frequent or continuous suction. Because aspiration pneumonia is almost inevitable and appears early, broad-spectrum antibiotic therapy is often instituted.

Primary surgical correction consists of a thoracotomy with division and ligation of the TEF and an end-to-side anastomosis of the esophagus. This may consist of one operation or be staged with two or more procedures. For infants who are preterm, have multiple anomalies, or are in poor condition, a staged procedure is preferred that involves palliative measures, including gastrostomy, ligation of the TEF, and provision of constant drainage of the esophageal pouch. A delayed esophageal anastomosis is usually attempted after several weeks to months when the upper pouch elongates. Further surgical techniques may be performed later to facilitate esophageal lengthening. If an esophageal anastomosis still cannot be accomplished, a cervical esophagostomy (to allow drainage of saliva) and gastrostomy are performed.

A primary anastomosis may be impossible because of insufficient length of the two segments of the esophagus. In these cases, an esophageal replacement procedure using a part of the colon, or gastric tube interposition may be necessary to bridge the missing esophageal segment. Endotracheal intubation may be required, since many infants (10% to 20%) also have tracheomalacia, a weakness in the tracheal wall that occurs when a dilated proximal pouch compresses the trachea in early fetal life or when the trachea does not develop normally because of a loss of intratracheal pressure.

Complications of a primary repair include an anastomotic leak, strictures resulting from tension or ischemia, esophageal motility disorders causing dysphagia, and GER.

Nursing Care Management

Nursing responsibility for detection of this malformation begins immediately after birth. Ideally, the diagnosis should be made before the initial feeding, but often it is not. If fed, the infant swallows normally but suddenly coughs and struggles, and the fluid is aspirated or returns through the nose and mouth. For this reason, it is customary for the nurse to give the infant the first feeding of plain water or to be present when a parent feeds the child to observe the infant’s response. Early breastfeeding should not be prevented unless there is a strong suspicion of EA.

Cyanosis is usually the result of laryngospasm caused by overflow of saliva into the larynx from the proximal esophageal pouch. It normally clears after removal of the secretions from the oropharynx by suctioning. Any suspicion of TEF is reported immediately. The infant is placed in an incubator or a radiant warmer, and oxygen is administered to help relieve respiratory distress. Intubation and assisted mechanical ventilation may be necessary if the infant is in respiratory distress. When a newborn is suspected of having a TEF, the most desirable position is supine with the head elevated at least 30 degrees. This position minimizes the reflux of gastric secretions up the distal esophagus into the trachea and bronchi.

It is imperative that the source of aspiration be removed at once. Oral fluids are withheld, and the infant’s fluid needs are met parenterally or via gastrostomy. Until surgery the blind pouch is kept empty by intermittent or continuous suction through an indwelling nasal catheter that extends to the end of the pouch. The catheter needs attention because it has a tendency to become clogged with mucus. It is usually replaced daily. In the event that a staged repair is performed, a gastrostomy tube is inserted and left open so that air entering the stomach through the fistula can escape, thus minimizing the danger that gastric contents will be regurgitated into the trachea. The tube empties by gravity drainage. Feedings through the gastrostomy tube and irrigations with fluid are contraindicated before surgery in the infant with a distal TEF. Nursing interventions include respiratory assessment, airway management, thermoregulation, fluid and electrolyte management, and possibly nutritional support.

Pathophysiology

The circular muscle of the pylorus thickens as a result of hypertrophy (increased size) and hyperplasia (increased mass). This produces severe narrowing of the pyloric canal between the stomach and the duodenum, causing partial obstruction of the lumen (Fig. 24-5, A). Over time, inflammation and edema further reduce the size of the opening, resulting in complete obstruction. The hypertrophied pylorus may be palpable as an olivelike mass in the upper abdomen. Pyloric stenosis is not a congenital disorder. There is now substantial evidence to support decreased expression of neuronal nitric oxide synthase in the nerve fibers of the pyloric circular muscle in infants with HPS (Huang, Tiao, Lee, and others, 2006). In most cases HPS is an isolated lesion; however, it may be associated with intestinal malrotation, esophageal and duodenal atresia, and anorectal anomalies.

Diagnostic Evaluation

The diagnosis of HPS is often made after the history and physical examination. The olivelike mass is easily palpated when the stomach is empty, the infant is quiet, and the abdominal muscles are relaxed. Vomiting usually occurs 30 to 60 minutes after feeding and becomes projectile as the obstruction progresses. Emesis is nonbilious, usually consisting of stale milk. Often these infants become dehydrated and lethargic and appear significantly malnourished.

If the diagnosis is inconclusive from the history and physical signs (Box 24-13), ultrasonography will demonstrate an elongated, sausage-shaped mass with an elongated pyloric channel. If ultrasound fails to demonstrate a hypertrophied pylorus, then upper GI radiography should be done to rule out other causes of vomiting. Laboratory findings reflect the metabolic alterations created by severe depletion of both fluid and electrolytes from extensive and prolonged vomiting. There are decreased serum levels of both sodium and potassium, although these may be masked by the hemoconcentration from ECF depletion. Of greater diagnostic value is a decrease in serum chloride levels and increases in pH and bicarbonate (carbon dioxide content) characteristic of metabolic alkalosis. The BUN level will be elevated as evidence of dehydration.

Therapeutic Management

Surgical relief of the pyloric obstruction by pyloromyotomy is the standard treatment for this disorder. The procedure is performed through a right upper quadrant incision (laparotomy) and consists of a longitudinal incision through the circular muscle fibers of the pylorus down to, but not including, the submucosa (see Fig. 24-5, B). The procedure has a high success rate when infants receive careful preoperative preparation to correct fluid and electrolyte imbalances.

Feedings are usually begun 4 to 6 hours postoperatively, beginning with small, frequent feedings of glucose water or electrolyte solutions followed by formula or breast milk as tolerated. Another procedure, laparoscopy, may be performed for infants with HPS. The use of a small incision for the laparoscope results in shorter surgical time, more rapid postoperative feeding, and quicker discharge.

Nursing Care Management

The diagnosis of HPS is considered in the very young infant who appears alert but fails to gain weight and has a history of vomiting after meals. Assessment is based on observation of eating behaviors and evidence of other characteristic clinical manifestations.

Pathophysiology

Intussusception occurs when one portion of bowel invaginates into a more distant portion of the bowel, pulling the mesentery with it. Lymphatic and venous congestion and bowel wall edema can cause obstruction of the intestine, and infarction and perforation of the bowel wall can occur (Huppertz, Soriano-Gaabarro, Grimprel, and others, 2006). Venous engorgement also leads to leaking of blood and mucus into the intestinal lumen, forming the classic currant jelly stools. The most common site is the ileocecal valve (ileocolic), where the ileum invaginates into the cecum and colon (Fig. 24-6). Other forms include ileoileal (one part of the ileum invaginates into another section of the ileum) and colocolic (one part of the colon invaginates into another area of the colon, usually in the area of the hepatic or splenic flexure or at some point along the transverse colon).

Diagnostic Evaluation

Frequently subjective findings lead to the diagnosis (Box 24-14), which can be confirmed by ultrasound. Spontaneous reduction occurs in up to 10% of patients.

Therapeutic Management

Conservative treatment consists of radiologist-guided pneumoenema (air enema) with or without water-soluble contrast or ultrasound-guided hydrostatic (saline) enema, the advantage of the latter being that no ionizing radiation is needed (Huppertz, Soriano-Gaabarro, Grimprel, and others, 2006). Recurrence of intussusception after conservative treatment occurs in about 1 in 10 patients; no predictable risk factors for recurrence have been identified.

IV fluids, NG decompression, and antibiotic therapy may be used before hydrostatic reduction is attempted. If these procedures are not successful, the child may require surgical intervention. Surgery involves manually reducing the invagination and, when indicated, resecting any nonviable intestine.

Nursing Care Management

The nurse can help establish a diagnosis by listening to the parent’s description of the child’s physical and behavioral symptoms. It is not unusual for parents to state that they thought something was seriously wrong before others shared their concerns. The description of the child’s severe colicky abdominal pain combined with vomiting is a significant sign of intussusception.

As soon as a possible diagnosis of intussusception is made, the nurse prepares the parents for the immediate need for hospitalization, the nonsurgical technique of hydrostatic reduction, and the possibility of surgery. It is important to explain the basic defect of intussusception. A model of the defect is easily demonstrated by pushing the end of a finger on a rubber glove back into itself or using the example of a telescoping rod. The principle of reduction by hydrostatic pressure can be simulated by filling the glove with water, which pushes the “finger” into a fully extended position.

Physical care of the child does not differ from that for any child undergoing abdominal surgery. Even though nonsurgical intervention may be successful, the usual preoperative procedures, such as maintenance of NPO status, routine laboratory testing (CBC and urinalysis), signed parental consent, and preanesthetic sedation, are performed. For the child with signs of electrolyte imbalance, hemorrhage, or peritonitis, additional preparation, such as replacement fluids, whole blood or plasma, and NG suctioning, may be needed. Before surgery the nurse monitors all stools.

Postprocedural care includes observations of vital signs, blood pressure, intact sutures and dressing, and the return of bowel sounds. After spontaneous or hydrostatic reduction, the nurse observes for passage of water-soluble contrast material (if used) and the stool patterns, since the intussusception may recur. Children may be admitted to the hospital or monitored on an outpatient basis. A recurrence of intussusception is treated with the conservative reduction techniques described above, but a laparotomy is considered for multiple recurrences.

Because hospitalization may be the child’s first separation from the parents, it is important to preserve the parent-child relationship by encouraging rooming-in or extended visiting. It may be the parents’ first experience with hospitalization, necessitating their preparation for procedures such as IV therapy, frequent vital sign and blood pressure monitoring, dressings, and NPO. Because of the rapidity of the onset, diagnosis, and treatment, parents may feel stunned or numb. They may ask few questions, or they may constantly make inquiries, sometimes the same ones several times. If the nurse realizes the circumstances surrounding this condition, the parents’ reactions are more likely to be understood and accepted.

Diagnostic Evaluation

It is imperative that malrotation and volvulus be diagnosed promptly and surgical treatment instituted quickly. An upper GI series is the definitive procedure to diagnose this condition.

Therapeutic Management

Surgery is indicated to remove the affected area. Because of the extensive nature of some lesions, short-gut syndrome is a postoperative complication.

Nursing Care Management

Preoperatively the nursing care is the same as that provided to an infant or child with intestinal obstruction. Postoperatively the nursing care is similar to that provided to the infant or child who has undergone abdominal surgery.

Diagnostic Evaluation

Checking for patency of the anus and rectum is a routine part of the newborn assessment and should include observations regarding the passage of meconium. Inspection of the perineal area reveals absence of the normal anal opening; however, the appearance of the perineum alone does not accurately predict the level of the lesion. Genitourinary and pelvic anomalies associated with anorectal malformations should be considered.

In the newborn the presence of meconium on the perineum does not always indicate anal patency (particularly in girls), since a fistula may be present and allow evacuation of meconium through the vagina. Fistulas may not be apparent at birth but may become obvious as peristalsis gradually forces the meconium through the fistula. Rectourinary fistulas should be suspected if there is meconium in the urine. Anal stenosis may not be identified until the child is older and comes to the physician with a history of difficult defecation, abdominal distention, and ribbonlike stools.

Abdominal ultrasound is performed to determine the existence of other malformations. An IV pyelogram and voiding cystourethrogram are recommended for an infant with a high malformation to identify anomalies of the urinary tract. Further examination is also indicated when there is evidence of urinary tract infection or other symptoms. If a syndrome is suspected, cardiac evaluation and spinal films should be obtained.

Therapeutic Management

Successful treatment for anal stenosis is generally accomplished by manual dilations. The procedure is initiated by a physician and repeated on a regular basis by the nurses in the hospital. Parents are taught to continue the dilations at home. Perineal fistulas are treated by anoplasty during the newborn period. The opening is moved to the center of the external sphincter, and dilations are begun. More extensive defects are usually managed with a colostomy and corrective surgical repair performed later in the first year.

The type of defect, the sacral anatomy, and the quality of muscles influence the long-term prognosis. In general, if the newborn has a deep midline groove, two well-formed buttocks, and an anal dimple, the prognosis for bowel control is better than if the infant has a flat or “rocker” bottom and no midline groove because of associated neurologic problems. A functioning interior anal sphincter is important to achieve continence. In its absence, the child may need a bowel program to achieve socially acceptable bowel continence. Other potential complications after surgical treatment include strictures, recurrent rectourinary fistula, mucosal prolapse, and constipation.

Nursing Care Management

The first nursing responsibility is identification of undetected anorectal malformations. A poorly developed anal dimple, a genitourinary fistula, or vertebral abnormalities suggest a high lesion. A newborn who does not pass a stool within 24 hours of birth requires further assessment. In addition, meconium that appears at an inappropriate orifice is reported. Preoperative care includes diagnostic evaluation, GI decompression, and IV fluids.

Nursing care after an anorectoplasty is directed toward healing the surgical site without infection or complications. Care involves keeping the anal area as clean as possible with scrupulous perineal care. A temporary dressing and drain may be placed initially to manage the continuous passage of stool. Protective ointments such as zinc oxide and occlusive dressings such as hydrocolloids decrease skin irritation from frequent loose stools. The preferred position is a side-lying prone position with the hips elevated or a supine position with the legs suspended at a 90-degree angle to the trunk to prevent pressure on perineal sutures.

There may be an NG tube for abdominal decompression and IV feedings. The infant is given formula when normal peristalsis is noted. Care of the infant with a colostomy involves frequent dressing changes, meticulous skin care, and correct application of a collection device (see Chapter 22).

Pathophysiology

Genetic predisposition is an essential factor in the development of celiac disease. Membrane receptors involved in preferential antigen presentation to CD4+ T cells play a crucial role in the immune response characteristic of celiac disease. Genes located on the HLA region of chromosome 6, namely HLA-DQ2 or HLA-DQ8, are found in almost 100% of those affected with celiac disease (Murdock and Johnston, 2005). Once the inflammatory reaction is activated by gluten, CD4+ T cells produce cytokines, which are likely to contribute to the intestinal damage. The damage consists of infiltration of the lamina propria, crypt hyperplasia, and villous atrophy and flattening. With sufficient villous atrophy, malabsorption occurs.

Diagnostic Evaluation

Classic symptoms of celiac disease are GI manifestations usually noted several months after the introduction of gluten-containing grains into the diet, typically between the ages of 6 months and 2 years (Box 24-15). Typically, children are seen with impaired growth, chronic diarrhea, abdominal distention, muscle wasting with hypotonia, poor appetite, and lack of energy. The clinical manifestations are usually insidious and chronic. The first evidence may be failure to thrive and diarrhea. Less typical presentation has been observed in children ages 5 to 7 years, who have abdominal pain; nausea; vomiting; bloating; constipation; or extraintestinal manifestations, including short stature, pubertal delay, iron deficiency, dental enamel defects, and abnormal LFTs. Older children have been found to have osteoporosis. Untreated celiac disease can evolve into celiac crisis, characterized by abdominal distention, explosive watery diarrhea, and dehydration with electrolyte imbalance, leading to hypotensive shock and lethargy.

The diagnosis of celiac disease is based on a biopsy of the small intestine demonstrating the characteristic changes of mucosal inflammation, crypt hyperplasia, and villous atrophy (Dieterich, Esslinger, and Schuppan, 2003). Within a day or two of instituting the diet, most children with celiac disease demonstrate a favorable response, including weight gain and improved appetite. Within a few weeks there is resolution of the diarrhea and steatorrhea.

Commercially available serologic tests for celiac disease include antigliadin antibodies of both the immunoglobulin A and G classes (IgA and IgG); antiendomysium IgA; and antitissue transglutaminase IgA and IgG antibodies for screening first-degree relatives of known celiac disease patients and those with known celiac disease–associated disorders such as type 1 diabetes, thyroiditis, arthritis, primary biliary cirrhosis, Down syndrome, Turner syndrome, Williams syndrome, and osteopenia or osteoporosis. False-positive results are likely when only one serologic test is used because patients with these disorders can also test positive for these antibodies. Use of more than one test increases diagnostic accuracy (Gelfond and Fasano, 2006). Ruling out total IgA deficiency is necessary to minimize false-negative results.

Therapeutic Management

Treatment of chronic celiac disease is primarily dietary. Although the diet is called “gluten free,” it is actually low in gluten, since it is impossible to remove every source of this protein. Because gluten is found primarily in the grains of wheat and rye, but also in smaller quantities in barley and oats, these four foods are eliminated. Corn and rice become substitute grain foods.

Children with untreated celiac disease may have associated lactose intolerance related to intestinal mucosal lesions, which usually improves with gluten withdrawal and intestinal healing. Specific nutritional deficiencies are treated with appropriate supplements, including vitamins, iron, and calories.

Nursing Care Management

The main nursing consideration is helping the child adhere to dietary management. Considerable time is involved in explaining to the child and the parents the disease process, the specific role of gluten in aggravating the condition, and those foods that must be restricted. Also, a lactose-free diet, which necessitates eliminating all milk products, may be needed initially. It is especially difficult to maintain a diet indefinitely when the child has no symptoms and temporary transgressions result in no difficulties. However, evidence indicates that most individuals who relax their diet experience a relapse of their disease and possibly exhibit growth retardation, anemia, or osteomalacia. There is also the risk of developing malignant lymphoma of the small intestine or other GI malignancies.

Although the chief source of gluten is cereal and baked goods, grains are frequently added to processed foods as thickeners or fillers. Gluten is also added to many foods as “hydrolyzed vegetable protein.” The nurse must advise parents to read carefully all ingredients on labels to avoid hidden sources of gluten. Many gluten-containing products are easily eliminated from the infant’s or young child’s diet, but monitoring the diet of a school-age child or adolescent is more difficult. Many “favorite” foods, such as hot dogs, pizza, and spaghetti, are chief offenders. Luncheon preparation away from home is particularly difficult, since bread, luncheon meats, and instant soups are not tolerated.

Generally, management includes a diet high in calories and proteins, with simple carbohydrates, such as fruits and vegetables, but low in fats. Initially the bowel may be inflamed as a result of the pathologic process, so high-fiber foods, such as nuts, raisins, raw vegetables, and raw fruits with skin, are avoided until inflammation has subsided. In a survey of 253 patients with celiac disease in New York State, Lee and Newman (2003) found that 86% experienced difficulties eating out and 82% experienced difficulties traveling because of the constraints of a gluten-free diet.

Several organizations and resources are available to help families cope with this condition. The Celiac Sprue Association/United States of America^* provides support, guidance, and educational materials to families concerning a gluten-free diet, food sources, recipes, and travel information. Several published cookbooks contain gluten-free recipes.†

Therapeutic Management

The goals of treatment are (1) to preserve as much length of bowel as possible during surgery; (2) to maintain the child’s nutritional status, growth, and development while intestinal adaptation occurs; (3) to stimulate intestinal adaptation with enteral feeding; and (4) to minimize complications related to the disease process and therapy.

Nutritional support is the long-term focus of care. The initial phase of therapy includes TPN as the primary source of nutrition. The second phase is the introduction of enteral feeding, which usually begins as soon as possible after surgery. Elemental formulas containing glucose, sucrose and glucose polymers, hydrolyzed proteins, and medium-chain triglycerides facilitate absorption. Usually these formulas are given by continuous infusion through an NG or gastrostomy tube. As the enteral feedings are advanced, the TPN solution is decreased in terms of calories, amount of fluid, and total hours of infusion per day. The final phase of nutritional support occurs when growth and development are sustained exclusively by enteral feedings. When TPN is discontinued, there is a risk of nutritional deficiency secondary to malabsorption of fat-soluble vitamins (A, D, E, K) and trace minerals (iron, selenium, zinc). Serum vitamin and mineral levels should be obtained, and enteral supplementation of vitamins and minerals may be required. Pharmacologic agents have been used to reduce secretory losses. H₂ blockers, PPIs, and octreotide inhibit gastric or pancreatic secretion. Cholestyramine is often prescribed to improve diarrhea that is associated with bile salt malabsorption. Growth factors have also been used to hasten adaptation and to enhance mucosal growth, but these uses are still experimental.

Numerous complications are associated with SBS and long-term TPN (see Chapter 22). Infectious, metabolic, and technical complications can occur. Catheter sepsis can occur after improper care of the catheter. The GI tract can also be a source of microbial seeding of the catheter. Bowel atrophy may foster increased intestinal permeability of bacteria. A lack of adequate sites for central lines may become a significant problem for the child in need of long-term TPN. Hepatic dysfunction, hepatomegaly with abnormal LFTs, and cholestasis may also occur.

Bacterial overgrowth is likely to occur when the ileocecal valve is absent or when stasis exists as a result of a partial obstruction or a dilated segment of bowel with poor motility. Alternating cycles of broad-spectrum antibiotics are used to reduce bacterial overgrowth. This treatment may also decrease the risk of bacterial translocation and subsequent central venous catheter infections. Other complications of bacterial overgrowth and malabsorption include metabolic acidosis and gastric hypersecretion.

Many surgical interventions, including intestinal valves, tapering enteroplasty or stricturoplasty, intestinal lengthening, and interposed segments, have been used to slow intestinal transit, reduce bacterial overgrowth, or increase mucosal surface area. Intestinal transplantation has been performed successfully in children. Only children with a permanent dependence on TPN or severe complications of long-term parenteral nutrition are candidates for transplantation.

Nursing Care Management

The most important components of nursing care are administration and monitoring of nutritional therapy. During TPN therapy, care must be taken to minimize the risk of complications related to the central venous access device (i.e., catheter infections, occlusions, dislodgment, or accidental removal). Care of the enteral feeding tubes and monitoring of enteral feeding tolerance are also important nursing responsibilities.

When long-term parenteral nutrition is required, preparing the family for home care is a major nursing responsibility that should be initiated early to prevent a lengthy hospitalization with subsequent problems such as family dysfunction and developmental delays. Many infants and children can be successfully cared for at home with enteral and parenteral nutrition when the family is prepared and provided with adequate support services. Follow-up by a multidisciplinary nutritional support service is essential. The nurse plays an active and important role in the success of a home nutrition program. Home infusion companies provide portable equipment, which enables the child and family to maintain a more normal lifestyle.

When hospitalization is prolonged, the child’s developmental and emotional needs must be met. This often requires special planning to promote normal family adjustment and adaptation of the hospital routines. Care of the hospitalized child is discussed in Chapter 21.