27. FEVER 135 this will only be heard on serial exams, because it may not be present at onset of fever. Muffled or distant heart sounds may be a clue to pericardial effusion as part of viral pancarditis or due to septic pericardial effusion. 9. Abdomen. Examination often requires diversionary tactics. Exam findings such as localized tenderness often need to be repeated from different approaches to validate finding. Rupture of the appendix before operative treatment is the rule in infants and young toddlers. Tenderness at McBurney point, if elicited, is reliable as a sign of appendicitis. Liver size, as measured by distance of the edge below the right costal margin at the mid- clavicular line (MCL), requires knowledge of changing anatomic ratios with growth. A liver edge 3 cm below the right costal margin at the MCL may be normal in a newborn but marks hepatomegaly in a 10-year-old child. Tenderness of the cos- tovertebral angle (CVA) in older toddlers and children points to a renal source of infection. 10. GU system. Perform a GU exam to evaluate for pelvic inflam- matory disease in a sexually active febrile adolescent. Consider UTI in a febrile girl without other evidence of an infec- tious focus. Physical findings (eg, CVA tenderness) are less reliable in younger children. Male adolescents must be assessed for testicular tenderness of epididymitis. 11. Extremities. Trauma from childhood play can be noted on the extremities, and evidence of infecting cellulitis should be sought. The punctum of cat-scratch disease is most often seen on extremities (upper > lower) as this is the site of most human contact with cats. Extremity findings can be seen in Kawasaki disease, dermatomyositis, SLE, and vasculitic syndromes (eg, septic vasculitis). B. Laboratory Data 1. CBC with differential. Often overutilized in well-appearing febrile children. Total WBC is a risk factor for bacteremia in highly febrile child. Low total WBC is not a reliable predictor of meningitis because low WBC counts are seen in viral infection, overwhelming infection (including meningitis), and immune deficiency states. 2. Lumbar puncture. Remains the gold standard for diagnosis of meningitis and must be performed, if not contraindicated, when history and physical exam cannot convincingly rule out bacter- ial meningitis. 3. Blood culture. Has little practical value to assess for occult bacteremia (bacteremia unexpected on clinical grounds). Most of these episodes are benign and resolve without treatment. Children who develop serious deep infections often present for medical care before positive testing of the blood culture. Multiple (three or four) blood cultures are warranted when 136 I: ON CALL PROBLEMS certain diseases (eg, osteomyelitis, endocarditis) are suspected to increase their yield. Blood cultures should be obtained through central lines if present. 4. Urinalysis. A useful test in female children without other evi- dence of infectious foci; it has a significantly lesser yield in male children but should be considered in uncircumcised boys during infancy if fever is not self-limited. Urine nitrites, leuko- cyte esterase, Gram stains, and direct cell visualization add to the immediate diagnostic value of urinalysis. 5. Urine culture. The gold standard for diagnosing UTI. 6. Other cultures. Throat culture and rapid antigen tests can be useful in diagnosing streptococcal pharyngitis; occasional cul- ture from the maximum area of induration of a cellulitis yields an infecting organism. Stool culture in selected patients may lead to a diagnosis of enteric infection (bloody diarrhea, elevat- ed fecal leukocytes, or protracted diarrhea). 7. Miscellaneous tests. Consider cultures of central lines, if present. Hepatic transaminases may suggest viral disease and lead to more specific hepatitis studies. C-reactive protein and ESR, although nonspecific, can occasionally help direct diag- noses or assess progress of treatment in some infectious diseases. C. Radiographic and Other Studies 1. Chest x-ray. Useful in patients who have fever without localiz- ing signs, particularly if physical exam findings raise suspicion of pulmonary involvement (especially tachypnea). 2. Abdominal imaging. “Blind” abdominal imaging for clues to an abdominal source of fever seldom is useful. Abdominal imag- ing, as well as imaging in general, should be guided by clinical suspicion. 3. Ultrasound. By virtue of its rapid availability, often a useful study if clinical signs or symptoms direct an evaluation to a given area. May reveal abscesses or other fluid collections. 4. Bone scan or MRI. Particularly useful if bone infection is sus- pected. 5. Thoracentesis, arthrocentesis, bone aspirate. As a general rule, obtaining material for culture from locations of fluid col- lections has a high yield and is warranted whenever possible. Perform whenever possible prior to antimicrobial treatment, because it can direct treatment. 6. Echocardiogram. Can be useful to assess for myocardial dys- function, as seen in viral myocarditis, acute rheumatic fever, and Kawasaki disease. May also implicate valvular disease of acute rheumatic fever, infective endocarditis, and coronary dilation or aneurysm of Kawasaki disease. V. Plan. Age of the involved child is a critical ingredient in the clinical decision tree. Any ill-appearing child requires thorough evaluation. 27. FEVER 137 A. Young Infants. Approach each febrile infant with the goal of first ruling out meningitis or overwhelming sepsis. Neonates must be considered functionally “immunocompromised” as they not only often fail to localize infection but also have a limited repertoire of clinical responses. 1. Infants, especially those who are younger than 1 month or who appear ill, require thorough evaluation with blood culture, urine evaluation and culture, and cerebrospinal fluid (CSF) evalua- tion with culture and appropriate CSF polymerase chain reac- tion (eg, herpes, enterovirus). 2. Admission and empiric treatment to cover group B Streptococcus, Listeria monocytogenes, and gram-negative enteric organisms is warranted, as well as consideration of empiric treatment of herpesvirus with acyclovir. A third-generation parenteral cephalosporin or an aminoglycoside (usually gentamicin) coupled with ampicillin is the current treatment of choice in most settings. Clinician must still rule out meningitis in this patient. B. Children One Month to 2 Years of Age 1. Treatment approach. Evaluation and management of this age group requires the most consideration. First, a compulsive search for the source of the fever must be performed. If found, treatment can proceed by clinical diagnosis as long as clinician recognizes that the diagnosed clinical syndrome does not nec- essarily eliminate more worrisome diagnoses. 2. Fever Without Localizing Signs (FWLS). If no source of infec- tion is found, child fits into the diagnostic group of FWLS. Although most infants with FWLS have self-limited viral dis- ease, a rare but real number of such patients are early in the course of a serious infection. Choices for therapeutic manage- ment include: a. “Sepsis workup” on all such patients, with subsequent hos- pitalization and empiric antibiotic treatment. This aggressive approach will treat hundreds perhaps even a thousand such patients to avoid missing the single patient at the early stage of an illness, either viral or occult bacteremia, that is des- tined to go on to bacterial meningitis. This option is fraught with problems, including issues of medical complications (eg, phlebitis, medication errors) and the psychosocial dis- ruption of hospitalization. b. Evaluation and empiric treatment of all infants who are “toxic,” while assuring close follow-up in FWLS infants who look well despite fever. Clinician may choose to perform acute phase testing, CBC and differential, C-reactive pro- tein, and urine studies to add diagnostic comfort to the choice to follow patient expectantly. Follow-up in this instance requires that infant’s parents or caregiver realize they are assuming a small risk in not hospitalizing child. Parents must be given appropriate information to enable 138 I: ON CALL PROBLEMS them to recognize progression of the illness (irritability, lethargy, loss of interest in feeding, petechiae or purpura, seizures or neurologic alteration) and respond to those changes (return immediately for care). Follow-up when these elements cannot be put in place may require hospital- ization for observation without empiric treatment. C. Children Older Than 2 Years of Age. Manage as for an older child. In this age group, child’s response to serious illnesses is sufficiently developed to be recognized (eg, nuchal rigidity is a reliable finding of meningeal irritation). D. Infant With Otitis Media. In an infant with otitis media, meningi- tis must be ruled out.The finding of a source of infection (eg, acute otitis media) in a highly febrile infant does not remove the onus on clinician to rule out serious deep infection. Although data suggest that a patient with one focus of infection is unlikely to have a second source of infection, the first diagnosis source does not protect patient from a second, more serious, source. VI. Problem Case Diagnosis. Evaluation in the emergency department showed a febrile infant (103.1°F) who was irritable but consolable by his parents. Physical findings were negative for an infectious focus. Lumbar puncture was deferred in favor of antipyretic treatment with acetaminophen to clarify role of fever in infant’s altered behavior. Upon reevaluation 1 hour later, infant was laughing and actively engaged in play with his father. Information was provided to parents regarding risks and findings that warrant return and reevaluation, and infant was sent home. Phone follow-up found that fever persist- ed for the next 3 days but occasional antipyretic therapy confirmed infant’s well-being. On the third day, infant developed a diffuse, blanching, erythematous macular rash. Fever and other symptoms simultaneously resolved. Diagnosis is roseola (herpesvirus 6). VII. Teaching Pearl: Question. What are the key considerations when assessing an infant with high fever? VIII. Teaching Pearl: Answer. Assessment is dependent on child’s age. Most neonates require a sepsis workup, with admission and empiric antibiotic treatment. Patients beyond the neonatal period, but younger than 2 years, require concise review of the history to assess for altered risk indicators (immune compromise) and infectious con- tacts, and thorough physical exam to search for an infectious focus. Management is then based on the clinical syndrome (pneumonia, cellulitis, meningitis, FWLS) or degree of toxicity. Fever itself, rather then the source of the fever, may cause irritability and lethargy. Antipyretic therapy may have its most substantive role in the ill but nontoxic infant in which defervescence allows a more effective assessment of infant’s status. Patients older than 2 years of age can be managed based on their clinical syndrome and degree of toxicity as one would older children. 28. FEVER OF UNKNOWN ORIGIN 139 REFERENCES Malatack JJ, Consolini DM. Fever without localizing signs and occult bacteremia. In: Klein JD, Zaoutis TE, eds. Pediatric Infectious Disease Secrets. Hanley & Belfus, 2003:211–220. Shapiro ED. Fever without localizing signs. In: Long SS, Pickering LK, Prober CG, eds. Pediatric Infectious Disease. Churchill Livingstone, 1997:110–114. 28. FEVER OF UNKNOWN ORIGIN I. Problem. A 7-year-old boy who has had daily fever for 2 weeks is brought to the clinic for evaluation. II. Immediate Questions A. What is the degree of fever and who has documented it? Normal body temperature is highest in children who are preschool aged. Several studies have documented that peak temperature tends to be in the afternoon and is highest at about 18–24 months of age when many normal children will have a temperature of 101°F. It is important to document fever (usually in an office setting) prior to beginning extensive testing. B. Is this truly fever of unknown origin (FUO)? Definition in adults is 2 weeks of outpatient fever and 1 week in hospital without a diagnosis. In children, variable definitions have been used. Generally, most clinicians would accept fever documented for more than 1 week in which initial cultures and other investigations fail to yield a diagnosis. This is quite different from fever without localizing signs (FWLS), which is a more common and acute dis- order in pediatrics, often involving risks and outcomes of bac- teremia (for further discussion, see Chapter 27, Fever, p. 132). Another key question is whether this is a “periodic” fever inter- spersed with wellness, pointing to additional possible diag- noses. C. What symptoms does patient have now? At onset? Clues to diagnosis of FUO are often obtained from the history, including meticulous review of systems (eg, rashes, skin breaks, and GI complaints). D. What testing has been done? Initial effort should be to ensure complete data collection (ie, cultures, laboratory work, x-rays, antibody titers). E. Are there known exposures? In difficult cases patients and fam- ilies may, with careful questioning, recall exposures (eg, insect or tick bites, animal contact, other children or adults with illness). F. What treatment has been initiated previously? At times, prior treatment may mask the fever history, make cultures negative, suppress bacterial growth (eg, urine or throat), or be the source of fever in the form of a drug reaction. G. Has patient traveled outside the country or to an endemic area? Certain areas are far more likely to be sources of individual 140 I: ON CALL PROBLEMS illnesses (eg, Lyme disease, Salmonella infection), and a history of travel to these areas may provide valuable clues. III. Differential Diagnosis. The list of potential etiologies of FUO is enormous, but with care, a systematic approach using key major screening tests and categories will prove useful. A. Infection. In almost all reviews of FUO in pediatric patients, infec- tion is the largest category, with a figure of at least 50% of all final diagnoses. It is important to recognize uncommon manifestations of common disorders (infectious mononucleosis with hepatitis or pneumonia) rather than unusual or uncommon infections, such as tularemia. About half of the localized infections involve the respi- ratory tract, and a careful history and x-rays may confirm this diagnosis. Other locations that are sources of prolonged fever include urinary tract, bone, and CNS. A random search for abscesses may not be warranted, but if patient has abdominal symptoms with FUO, a CT scan may be useful. Look for clues to more generalized infections (Epstein-Barr virus, enteric infection, cat-scratch disease, tuberculosis, and cytomegalovirus) in which there may be evidence of multiple organ involvement. B. Collagen or Connective Tissue Disease. Juvenile rheumatoid arthritis may present with a long duration of fever before a diag- nosis is established (ie, fever precedes evidence of joint or skin involvement). Additional causes include Kawasaki disease, sys- temic lupus erythematosus, rheumatic fever, and other vasculitic syndromes, such as Wegener granulomatosis. Most of these con- ditions produce additional physical findings, but patients with Kawasaki disease who are younger than 1 year of age may have “incomplete” or atypical presentations with only a few manifesta- tions of the disorder. C. Neoplasia. Most common in this group are lymphoreticular malig- nancies (eg, lymphoma, leukemia). If there are joint symptoms, these may, at times, be confused with juvenile rheumatoid arthri- tis. Neuroblastoma and occasionally other sarcomas may present with fever as the major symptom. D. Inflammatory Bowel Disease. This is an unusual cause of iso- lated FUO because other symptoms (eg, diarrhea, weight loss, poor growth) are usually present. E. Miscellaneous. There are always rare causes not evident on an initial search. Examples are ectodermal dysplasia with poor ther- mal regulation, diabetes insipidus with dehydration and fever in infancy, and central fever in patients with disordered thermoregu- lation. Another rare cause is so-called inflammatory pseudotu- mor, usually found in the abdomen. F. Pseudo FUO. This entity is likely much more common than true FUO because frequent, minor, viral illness may be overinterpreted. A careful recording of illnesses and overall function of child and family is necessary, including school attendance. 28. FEVER OF UNKNOWN ORIGIN 141 G. Periodic Fever. This is a separate entity in which fever is truly episodic, followed by “normal” times. This category includes peri- odic fever with aphthous stomatitis, pharyngitis (PFAPA) and familial Mediterranean fever and variants. Many of these latter dis- orders are being delineated using newer genetic techniques as well as by studying pathways of inflammation. IV. Database A. Physical Exam Key Points 1. Vital signs and growth parameters. Fever should be con- firmed and weight loss or growth failure recorded. Hypertension may be a clue to renal involvement. Respiratory rate may be elevated in patients with chest disorders. 2. Skin. Examination should be meticulous, evaluating for skin breaks, nodules, and rashes, which may be clues to the diag- nosis. Petechiae may be another clue. 3. EENT. Conjunctivae may demonstrate injection or even splin- ter hemorrhages in endocarditis. Fundi and disk margins should be examined. EENT exam should include pneumatic otoscopy (otitis media is overdiagnosed) and clinical exam of the sinuses. 4. Lymph nodes, organomegaly. Generalized disorders often include generalized adenopathy and enlargement of liver spleen, or both. Regional lymph node enlargement may be a clue to disorders such as cat-scratch disease. 5. Chest and lungs. Changes in breath sounds or adventitious sounds may confirm a localized process. 6. Heart. A new murmur may be a result of infection or a disorder such as rheumatic fever. 7. Abdomen and perianal area. Assess for pain, masses, and bowel sounds. Patients with a previously ruptured, walled-off appendix may present with prolonged fever and diarrhea. Regional enteritis commonly involves the anus; skin tags and fissures may be clues. 8. Extremities, bones, back. A search for localized tenderness may be critical to making a correct diagnosis. Be certain to examine the spine for flexibility and paraspinous muscle spasm. B. Laboratory Data 1. Cultures. Be certain that key cultures (usually blood, urine, throat, and occasionally stool or local lesions) have been taken for analysis. 2. Screening tests. Several studies have documented that inflam- matory markers are strong evidence of more serious causes of prolonged fever: increased ESR, elevated C-reactive protein, and low albumin with reversal of the albumin– globulin ratio. These tend to be sensitive but not specific for seri- ous disorders. Patients without markers of chronic inflammation 142 I: ON CALL PROBLEMS may warrant observation rather than intense investigation. Although a specific diagnosis is usually not made, CBC with platelets and comprehensive metabolic profile should be ordered for most patients, especially to screen renal and hepatic function. 3. Titers. Several disorders are best diagnosed with antibody titers (eg, Lyme disease, Epstein-Barr infection, tularemia, cat- scratch disease). If these are likely diagnoses, titers should be sent rapidly because paired titers may be necessary. 4. Bone marrow exam. Should not be routine, except perhaps in immunocompromised patients. If there are reductions in at least two cell lines from a CBC, marrow exam may be useful to look for malignancy. C. Radiographic and Other Studies. Radiology consultation is extremely useful when making decisions about the best imaging test. 1. Chest x-ray. 2. Bone scan. Useful with localized tenderness. 3. CT scan. May be useful, especially when there are localized findings in areas such as chest, abdomen, bones, and CNS. Routine use of CT scan in all patients without localizing find- ings is not useful and may give misleading information. 4. Leukocyte tagged scans. Occasionally useful to localize infection, but there are reports of false-negative results. 5. MRI scan. Rapidly becoming the most useful test to evaluate certain areas (eg, bone, spine, and CNS). V. Plan. Treatment is based on whether or not patient has an identifi- able condition. Inflammatory markers help clinician to decide whether further extensive testing is necessary. Even when present, at times there must be a period of watchful waiting and repeat examination, seeking additional information from history or physical exam. A. Pursue Clues, Only. Most clinicians approach patients with FUO by random testing (x-rays, CT scans, etc) without any clear infor- mation as to a possible diagnosis. B. Seek Additional Information From History and Exam. This is clinician’s strength: New data may lead to the correct diagnosis. Examples may be new exposures, hearing a new murmur, a new skin rash. C. Use Laboratory Wisely. Repeat cultures may be helpful. Blood may be sent for additional titers or for “vasculitis” screens (eg, ANCA). VI. Problem Case Diagnosis. Patient had an increased ESR, low albu- min, and negative throat, blood, urine, and stool cultures as an out- patient. He had mild abdominal pain at onset, which was improving. Additional history revealed exposure to a neighbor’s new kitten. Physical exam uncovered several resolving old papules on the right 29. FOREIGN BODY: GASTROINTESTINAL TRACT 143 arm where he had been scratched, as well as axillary adenopathy. Sonogram and abdominal CT scan, performed because of initial abdominal pain, showed typical granulomatous lesions in the liver. Titers for Bartonella henselae were strongly positive, confirming a diagnosis of systemic cat-scratch disease. VII. Teaching Pearl: Question. A patient presents with generalized adenopathy and fever of 8 days’ duration. The patient had periorbital edema early in the illness and now has splenomegaly and mild hep- atitis. What diagnosis should be considered? VIII. Teaching Pearl: Answer. Periorbital edema is seen occasionally in infectious mononucleosis and is known as Hoagland sign. Mild hep- atitis is seen almost universally in the 2nd week of illness. REFERENCES Gartner JC Jr. Fever of unknown origin. Adv Pediatr Infect Dis 1992;7:1–4. Miller M, Szer I, Yogev, R, Bernstein B. Fever of unknown origin. Pediatr Clin North Am 1999;42:999–1015. 29. FOREIGN BODY: GASTROINTESTINAL TRACT I. Problem. A 2-year-old boy old is brought to the physician’s office 1 hour after a gagging episode. His mother states that he has had some difficulty swallowing, has been acting normally, but is not inter- ested in drinking or eating. The boy is unwilling to open his mouth for his mother. II. Immediate Questions A. Is there a history of gagging, drooling, vomiting, sore throat, or dysphagia? Many of these symptoms indicate the presence of a foreign body in the GI tract. B. Is patient refusing food? All of these symptoms could point to an esophageal foreign body. C. Is there a history of midepigastric or chest pain? Lodging of a foreign body in the esophagus can lead to abdominal and chest pain. D. Does patient have shortness of breath, coughing, or wheez- ing? If the object is compressing the trachea, these respiratory symptoms would be expected. E. Were symptoms sudden in onset? The timing can be helpful in identifying a discrete event. F. Is fever associated with the pain? These symptoms may indi- cate perforation, which is a very rare complication of foreign body ingestion. G. Was the foreign body ingestion witnessed? Some patients with known foreign body ingestion are asymptomatic. In 30–40% of [...]... headache(s)? 3 What was patient doing before and after episode(s)? 4 Are there any precipitants to headache(s)? Any alleviating factors for headache(s)? D Pertinent Historical Information 1 What was the prenatal and neonatal course? May identify risks for long-term neurologic sequelae 33 HEADACHE 161 2 Were developmental milestones achieved at appropriate ages? 3 Any significant medical or surgical... last from minutes to days 162 I: ON CALL PROBLEMS D Chronic Daily Headache Extremely frequent form of tensiontype headache Occurs at least 20 days per month Patients can often recall the exact date of onset of these frequent and bothersome headaches E Cluster Headaches Severe, unilateral headaches classically associated with ipsilateral nasal congestion, conjunctival injection, and lacrimation Pain... Can be managed with symptomatic medication use (ie, “when headache occurs, take an analgesic medication”) These medications include acetaminophen, aspirin, NSAIDs, and a variety of combination medications (containing butalbital, caffeine, and codeine), such as Midrin and Fioricet Triptans (eg, sumatriptan, rizatriptan, zolmitriptan, 33 HEADACHE 165 and naratriptan) can be used for infrequent headaches... intrahepatic or extrahepatic causes of portal hypertension; rarely in association with congenital heart disease or vascular malformations Portal vein thrombosis is a common cause of extrahepatic obstruction Risk factors include omphalitis, history of umbilical vein cannulation, and dehydration Intrahepatic portal hypertension is caused by hepatic parenchymal disorders More common associated diagnoses... reveals unilateral aeration disturbance such as air trapping (“ball-valve” phenomenon), atelectasis (complete obstruction), or consolidation Many foreign bodies are not visualized on plain x-rays because they are nonradiopaque, but the aforementioned findings will suggest aspiration 2 Inspiratory and expiratory films May be required if plain film is not revealing Often these additional films show unilateral... than once per week Triptan medications are effective in treating migraine headache along with accompanying symptoms of nausea and vomiting Dose can usually be repeated in 2 hours 2 Prophylactic treatment Various agents from many medication classes have been used to treat headaches prophylactically Choose medications based on efficacy in various headache types Symptomatic medications are used to treat... pulmonary hypertension Is there a history of prematurity? Premature infants, especially those with low birth weight, are more likely to have persistent patent ductus arteriosus Has there been a prior cardiac evaluation? Many patients with congenital heart disease are identified prenatally by fetal echocardiography and have had subsequent postnatal evaluations Information from any prior cardiac evaluations,... cramping and vomiting C Gingivostomatitis Usually presents with oral pain, drooling, and anorexia D Airway foreign body May compress the esophagus, leading to painful swallowing and dysphagia IV Database A Physical Exam Key Points 1 ABCs Assess airway, breathing, and circulation first 2 General appearance and vital signs Often normal if the foreign body has passed beyond the proximal esophagus 3 Oropharynx... the appearance of blood Food coloring is contained in fruit juices and gelatins Breast-feeding infants may swallow maternal blood from cracked nipples D Is bleeding coming from GI system? Does patient have any history of nasopharyngeal trauma, chronic nasal congestion, or 156 I: ON CALL PROBLEMS epistaxis? Swallowed nasopharyngeal bleeding from trauma or mucosal ulceration can cause hematemesis E Was... coagulation cascade (ie, hemophilia) or secondary to liver disease or disseminated intravascular coagulation as a result of overwhelming infection 32 GASTROINTESTINAL BLEEDING: UPPER TRACT 157 E Varices Variceal bleeding is rare in infancy, although gastroesophageal varices associated with portal hypertension are the most common cause of significant GI bleeding in older children Gastroesophageal varices . the AP projection, a battery appears as a double-density shadow and in the lateral projec- tion, the edges are rounded and reveal a step-off at the junc- tion of the anode and cathode. Several. school-aged, has patient had any extraintestinal manifes- tations? Symptoms such as rash, arthralgias, anorexia, and weight loss are all indicative of inflammatory bowel disease, although this condition. of age. Clinical spectrum ranges from imme- diate-type reactions, including urticaria and angioedema, to inter- mediate and late-onset reactions, such as atopic dermatitis, gastroesophageal reflux,