The crying child: What are they trying to tell you? Part I

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This activity has been planned and implemented in accordance with the Essential Areas and policies of the Accreditation Council for Continuing Medical Education (ACCME) through the joint sponsorship of CME², Inc. ("cme²") and Contemporary Pediatrics. cme² is accredited by the ACCME to provide continuing medical education for physicians.

cme² designates this educational activity for a maximum of 1.0 AMA PRA Category 1 Credit™. One AMA PRA Category 1 Credit™ will be awarded after the successful completion of Part I and II, which is scheduled to appear in the June issue of Contemporary Pediatrics. Physicians should only claim credit commensurate with the extent of their participation in the activity.

Target audience: Pediatricians and primary care physicians

EDUCATIONAL OBJECTIVES

• Cite life- and limb-threatening diagnoses that may present as irritability or persistent crying in a young child
• Discuss the typical clinical course for simple colic
• Explain the utility and limits of the laboratory evaluation of the irritable young child
• Describe an algorithm for the initial evaluation and treatment of the irritable young child

To earn CME credit for this activity
Participants should study Parts I and II of this article and log on to www.contemporarypediatrics.com, where they must pass a post-test and complete an online evaluation of the CME activity. After passing the post-test and completing the online evaluation, a CME certificate will be e-mailed to them. The release date for this activity is May 1, 2007. The expiration date is June 1, 2008.

Disclosures
Editors Toby Hindin, Jeannette Mallozzi, Jeff Ryan, and Karen Woldman disclose that they do not have any financial relationships with any manufacturer in this area of medicine. Manuscript reviewers disclose that they do not have any financial relationships with any manufacturer in this area of medicine.

Author Robert Bolte, MD, discloses that he does not have any financial relationships with any manufacturer in this area of medicine.

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A crying child universally evokes not only pity but stress. This stress is intensified for the physician confronting a young, non-verbal child whose anxious parents are seeking an explanation for their child's distress. Add to this the vast number of etiologies of intractable crying in infancy and early childhood, and stress levels easily mount up. Therefore, an organized approach to this common presenting complaint is vital for any physician providing acute care to children.

Pathologies and patterns

Crying is a non-specific response in an infant, which may be a symptom of an underlying disease process-either obvious or occult. Indeed, crying can be a major feature in various infectious diseases, traumatic and surgical problems, as well as toxicological, nutritional, and metabolic disorders.¹

Crying is also a necessary feature of normal psychomotor development in early infancy, and begins as a response to a physiologic stress such as hunger, discomfort, over- or under-stimulation, or temperature change.² As the infant becomes conditioned to expect a response to his/her cry, crying becomes a more purposeful tool for controlling their environment.

A different pattern of crying also emerges in the first few weeks of life in almost all normal thriving babies. This type of crying, classified as "paroxysmal fussing" or colic, is characterized by cyclic discrete periods of crying, usually on a daily basis. This type of crying is intractable and does not cease through the obvious methods of relief offered by parents (e.g., feeding, burping, diaper change, rocking, etc.). Moreover, afflicted babies tend to be hypersensitive to stimuli, less predictable in their sleep-awake state regulation, and have a diminished ability to regulate crying duration. These traits make the colicky baby certainly more challenging. As a result, parental self-doubt and tension often develop, sometimes leading to abusive behavior.³

In 1962, Brazelton published a study of crying in normal infants.⁴ In the 80 infants studied, the median daily crying time was one hour and 45 minutes at 2 weeks of age, peaking at two hours and 45 minutes at 6 weeks, and thankfully decreasing to less than one hour by 12 weeks of life. The peak time for crying occurred from 3 p.m. to 11 p.m.-when most busy parents are frantically attempting to spend precious quality time with their new baby. Sharing this information with prospective parents may be the most effective argument for family planning that you can ever deliver.

Brazelton's data fits well with Wessel's classic definition of infantile colic, which is defined as crying lasting longer than three hours per day, three days per week, and continuing for more than three weeks in infants younger than 3 months old.⁵

Making a differential diagnosis

Crying may accompany almost any pediatric illness and, as previously discussed, is also a normal feature of psychomotor development in young infants. A concerning subset of patients are young, non-verbal children who present with a relatively abrupt onset of intractable crying. The following discussion will focus on some of the more common and/or serious conditions associated with intractable crying in infancy and early childhood (see Table 1).

1. Infectious

Any infectious process can result in a crying, irritable infant. Etiologic considerations range from benign viral upper respiratory tract or gastrointestinal infections to immediate life-threatening disease.

Meningitis and sepsis occur with their highest frequency during infancy. The presenting signs/symptoms for either of these conditions can be very non-specific throughout this period, but especially during the first 12 weeks of life. Fever, although a cardinal sign of infection, may be absent (particularly in the first month of life). Additionally, hypothermia is not uncommon in the neonate with sepsis or meningitis. The infant may also exhibit paradoxical irritability (i.e., "wanting to be left alone"), manifesting as an increase in crying when being held. A full fontanelle and/or vomiting may be seen, and petechiae may also be present. Nuchal rigidity, however, is an inconsistent finding in the infant with meningitis, and is almost never present in patients less than 3 months of age.

Viral meningoencephalitis has a non-specific presentation in infants similar to that of bacterial meningitis, although the level of toxicity is generally less. Fortunately, the frequency of pediatric bacterial meningitis has decreased dramatically with the introduction of effective Hib (Haemophilus influenza type b) and pneumococcal vaccines. Therefore, in young febrile patients, clinicians should make it a point to ascertain the child's vaccination status.

Urinary tract infection (UTI) can present as an inconsolable infant and is the most frequent significant bacterial infection in this young age group. Although generally considered a disease of females, UTIs occur with about equal frequency in males during the first three months of life, and are relatively frequent in uncircumcised males up to 1 year of age. Most, but not all, young children with UTIs are febrile. A clinically important feature among infants with culture-proven UTIs is that they often have seemingly normal urinalysis results-up to half the time in infants less than 3 months of age. This fact underscores the need for a reliable culture in all infants suspected of having a UTI.

Septic arthritis and osteomyelitis may also present as a crying infant. The frequent lack of fever or system toxicity in these cases must be emphasized. Rather, the most common presenting complaint is lower extremity involvement. Infants with a septic hip generally lie quiet, holding their leg in an abducted and externally rotated position. Patients may also exhibit pseudoparalysis and pain with passive movement. Infants with osteomyelitis may present with an overlying cellulitis, secondary to rupture of the thin, bony cortex. Point tenderness is also a common feature, but localization may be difficult in an apprehensive infant. Lastly, discitis represents an osteomyelitis of the vertebral end plate. It may present as a crying infant, often associated with fever combined with a refusal to sit or bear weight.

Otitis media is one of the most common etiologies for intractable crying in an infant, and must be diligently sought out despite the frequent technical challenges in its diagnosis. Fever is variable in these cases. In Poole's 1991 study of afebrile infants with intractable crying, otitis media was the most common specific diagnosis (18%).⁶ The presence of otitis media does not, however, exclude a more serious concomitant infection, such as sepsis or meningitis. In the infant less than 3 months of age, you should be very hesitant initiating presumptive treatment for this diagnosis without a more extensive evaluation.

Pneumonia can occasionally account for a crying, irritable young child. Tachypnea, retractions, grunting, and hypoxia are typical but not absolute features. The absence of classic auscultatory findings does not exclude significant pulmonary pathology, which may be apparent only on a roentgenogram of the chest.

Kawasaki disease, a systemic vasculitis of unclear etiology that mimics an infectious process, can cause extreme irritability in an infant or young child. These children are generally highly febrile for several days with multisystem involvement, for example, mucous membrane and extremity changes, nonpurulent conjunctivitis, erythematous rash, adenopathy, etc.

2. Traumatic

Infants are at high risk for serious physical abuse.⁷ Although evidence of external trauma is usually absent in shaken babies, clinicians should keep in mind that a crying, listless, "septic-appearing" infant may indeed be the victim of a shake injury. Retinal hemorrhages in such cases are essentially pathognomonic but problematic to diagnose if the child is conscious. In an irritable or lethargic infant, bloody cerebrospinal fluid that does not clear or clot, or the presence of xanthochromic fluid, should raise the suspicion of a subarachnoid hemorrhage associated with a shake injury. However, a lumbar puncture should obviously not be performed if an intracranial bleed is suspected. If suspected, this diagnosis should be confirmed by computerized axial tomography.⁸

Any form of skeletal or soft tissue trauma may present as a crying infant. Point tenderness can usually be elicited during a careful physical exam. Again, a high index of suspicion for physical abuse should be maintained in these circumstances. In the older infant (i.e., one able to pull to a standing position) and the toddler, acute onset of severe crying may be seen with a nondisplaced spiral fracture of the distal tibia ("toddler's fracture"), combined with a refusal to bear weight. Toddler's fracture, however, is not usually considered indicative of non-accidental trauma. Spiral fractures of the femur, on the other hand, are often associated with abuse.

Corneal abrasion is a relatively common-but usually surprising-cause of abrupt, intractable crying in a young infant.⁶ Both suggestive history and eye redness are often absent. A foreign body, such as an eyelash, may be present even in the absence of a corneal abrasion.

3. Gastrointestinal

Between 2 months and 5 years of age (peak 3 months to 2 years), intussusception is the most common cause of intestinal obstruction. Paroxysms of crying are usually an early presenting symptom. During these paroxysms, the child will appear to have severe abdominal pain, but in the interim initially appears relatively well. In early cases, plain film roentgenograms are usually normal. As the paroxysms persist, the infant may become quite lethargic and appear toxic. This stage may precede the classic "currant jelly stool," and therefore these infants may be misdiagnosed as being septic.

It should be noted that some infants with intussusception may present with striking lethargy without paroxysmal crying at the time of initial presentation, thus a high index of suspicion needs to be maintained with this age group.^9,10 A digital rectal exam is mandatory as it may reveal an unexpected bloody stool, and the leading edge of the intussusceptum may rarely be palpable. Once the diagnosis of intussusception is suspected, it should be confirmed by ultrasound or air enema. Fortunately diagnosis is somewhat simplified, since infant colic syndrome has its onset in infants less than 1 month of age, and is cyclic and recurrent in nature.

Volvulus may account for a sudden onset of crying in a young child. Midgut volvulus (usually associated with malrotation) is the most common variety and is an immediately life-threatening condition. Most, but not all, cases present in the first month of life and are associated with a constant but not crampy pain. Although the initial presentation of this condition may be coupled with crying, it should be emphasized that most young infants at diagnosis do not appear irritable or toxic.¹¹ Rather, bilious vomiting is the cardinal symptom. Abdominal tenderness and bloody stools, if present, suggest significant intestinal compromise. Sigmoid and gastric volvulus may also rarely present as intractable crying.

Appendicitis is hardly ever seen in the first two years of life, but it does occur. Perforation at the time of diagnosis in this age group is an extremely common complication.

Reflux esophagitis can potentially be the root of inconsolable crying in an infant. In such cases there is often a history of frequent, non-forceful, non-bilious regurgitation, usually following feedings. A history of "aversive" feeding behavior is also common. The infant will take the first few sips avidly, but then turn away from the breast or bottle and proceeds to cry. Occasionally, there could be associated aspiration pneumonitis. Occult blood loss or hematemesis may also take place. Intermittently, the infant might exhibit an associated torticollis-like positioning of the neck (Sandifer syndrome). Consideration of this diagnosis is particularly important if the onset of symptoms is later than "typical colic," or if the symptoms are increasing as the baby approaches 3-4 months of age, when simple colic should be subsiding.

Constipation is a fairly common cause of crying in infancy. Cases are characterized by stools that are hard and usually infrequent. Passage may be difficult and associated with increased straining. In addition, there may be anal fissures which cause severe pain at defecation. You would typically find a small amount of bright red blood streaking the surface of the stool, combined with fissures that are generally obvious on inspection.

An incarcerated inguinal hernia can be another cause of an abrupt onset of crying that may be overlooked during a hasty physical examination. It is a relatively common cause of bowel obstruction in the young infant.

Henoch-Schönlein purpura (non-thrombocytopenic) has been reported as early as 6 months of age with a peak incidence between 4 and 5 years. Abdominal pain (sometimes severe) may precede the characteristic rash by up to two weeks, obscuring the diagnosis.

Hemolytic-uremic syndrome is a disease that predominantly affects infants and young children. In these instances, the child may present with irritability and abdominal pain. Diarrhea, often bloody, usually precedes other symptoms of this illness. Proteinuria and hematuria are detectable at presentation. Thrombocytopenia, microangiopathic hemolytic anemia, and severe azotemia also develop in most patients.

4. Nutritional

Underfeeding can result in an irritable, crying infant. These children will typically appear cachectic and ravenous. Clinicians must rule out other causes of organic failure to thrive, such as renal failure or Celiac disease, before reaching a diagnosis of malnutrition. Improper feeding techniques (e.g., inadequate burping, supine feeding, etc.) may also occasionally account for significant crying in an infant.

Food allergies could potentially be at the heart of inconsolable crying. Young infants, for example, may exhibit an allergy to proteins in cow's milk, resulting in irritability and crying. Generally, these cases present with small-volume intestinal bleeding and possibly diarrhea. These infants typically have a similar intolerance to soy protein.

5. Respiratory

Hypoxemia or hypercapnia can also cause an infant to cry and be irritable. In such cases, the normal signs of respiratory distress (i.e., tachypnea, retractions, nasal flaring, wheezing) may be difficult to assess. Only when hypoxemia is marked will cyanosis be evident.

6. Metabolic

Occassionally, a crying infant will have an underlying metabolic abnormality. In instances of infant hyponatremia or hypernatremia, for example, the child may be quite irritable. Usually, there is a history of inappropriate dietary intake (e.g., prolonged clear liquids, overdiluted formula, boiled milk, etc.) and/or a history of vomiting and diarrhea with the clinical appearance of dehydration. A young infant with hyponatremia secondary to congenital adrenal hyperplasia (CAH) generally has a history of vomiting and poor feeding. Ambiguous genitalia or scrotal hyperpigmentation may also be seen in the infant with CAH.

Intractable crying would be a very atypical presentation for glucose or calcium abnormalities. Irritability, jitteriness, or lethargy can be features of these disorders.

The infant with metabolic acidosis may be quite irritable, listless, and dehydrated. In general, there is a history of several days of diarrhea or, rarely, salicylate intake. Despite adequate hydration, the infant may have a significant metabolic acidosis.

Iron deficiency anemia could be another source of extreme irritability and crying, although the onset is typically not abrupt. Pallor is often difficult to clinically assess in such cases. Among term babies with normal iron stores, iron deficiency anemia is usually not seen prior to 6 months of age, unless there is occult blood loss. Premature and low-birth-weight infants can develop this problem as early as 2 months of age.

7. Integument

Severe diaper dermatitis, atopic eczema, burns, or an open safety pin (this one is on the endangered species list) may account for an intractably crying infant. An encircling hair may lead to strangulation of a toe or finger. Rarely the penis, clitoris, or even the uvula may be involved. An encircling hair may be overlooked if the examination is not thorough, but is usually not indicative of abuse.

Various bites and stings can also cause an acute onset of crying in an infant. The black widow spider bite in an infant is a serious but relatively infrequent problem. The site of the bite may appear trivial and the presentation, sometimes mimicking an acute abdomen, can be quite confusing.

8. Drugs and toxins

Irritability and crying are the most common manifestations of neonatal narcotic withdrawal. Onset of symptoms usually occurs from 12 hours to 4 days of age; however, symptoms of methadone withdrawal may be occasionally delayed until 2 weeks of age.

Therapies such as barbiturates, ethanol, and dilantin administered to mothers may also create withdrawal symptoms in their newly born infants. Toxicity from decongestants, antihistamines, amphetamines, or cocaine (transferable in breast milk), can also be a factor for extreme irritability in an infant.¹²

Ingestion of high doses of vitamin A can also result in extreme irritability, secondary to increased intracranial pressure and bone pain. Lastly, infants suffering from carbon monoxide toxicity may be quite irritable, although somnolence is a more typical presentation. Historical clues should be sought. Pulse oximetry will be misleading.

9. Outside the box

Ingestion of a foreign body, such as a coin or pin, may be the cause of inconsolable crying in an older infant/young child. A foreign body in the ear canal (a cockroach is the classic example) may be one reason for an abrupt onset of intractable crying in a small child.

Teething can be yet another source of discomfort in an otherwise normal infant. Onset should be no earlier than 4 months of age, and is associated with a swollen, tender gum but does not account for significant fever.¹³

Somewhat surprisingly, supraventricular tachycardia (SVT) was found to be the etiology in 4% of afebrile infants presenting to an emergency department with "unexplained intractable crying" in a 1991 study by Poole.⁶ The pulse rate in SVT patients is generally greater than 230 and shows no beat-to-beat variability. At times, a diagnosis can be problematic as SVT episodes may be intermittent. During the asymptomatic period, the presence of delta waves on the EKG could serve as a potential indicator of this problem.

Nontraumatic causes of increased intracranial pressure (e.g., hydrocephalus or tumor) may occasionally present as a crying, irritable infant. Bulging fontanelle, vomiting, large head circumference, etc., are all signs and symptoms that could accompany an increase in intracranial pressure.

Torsion of the testes is an unusual cause of inconsolable crying in infancy, and is usually found among older children and adolescents.

Caffey's disease (usually presenting in first three months of life, and associated with fever and skeletal swelling, often of the jaw and thorax) and congenital glaucoma (associated with large corneas and photophobia) are rare causes of intractable crying in infancy.

There are a variety of genetic syndromes, such as Smith-Lemli-Opitz, that may be associated with hyperirritability and crying in infancy. In these unusual cases, striking phenotypic findings are typically present.

The dreaded colicky baby

Each of the aforementioned etiologies has demonstrated incidence among intractably crying infants and young children. But perhaps the most common cause of severe crying in afebrile, otherwise thriving children, is infant colic syndrome (see discussion in the Pathophysiology and patterns section).

This syndrome can be characterized by recurrent paroxysms of usually nocturnal crying beginning at 1 to 4 weeks of age, and greatly diminishing in intensity by 3 to 4 months of age. During the paroxysms, the baby's abdomen may be somewhat tense and the legs are often flexed. Additionally, parents may report that the infant is "very gassy" and appears to experience temporary relief after passing flatus. Vomiting, diarrhea, and fever, however, are not features of infant colic syndrome.

The exact underlying cause of infant colic syndrome remains controversial, with a growing body of evidence suggesting that this condition probably represents the extreme of normal infant temperament/behavioral development.²

Editor's Note:Look for "The crying child: Evaluation and management, Part 2" in the June 2007 issue of Contemporary Pediatrics.

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References

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2. Barr RG: Changing our understanding of infant colic. Arch Pediatr Adolesc Med 2002;156:1172

3. Reijneveld SA, van der Wal MF, Brugman E, et al: Infant crying and abuse. Lancet 2004;364:1340

4. Brazelton TB: Crying in infancy. Pediatrics 1962;29:579

5. Wessel MA, Cobb JC, Jackson EB, et al: Paroxysmal fussing in infancy, sometimes called "colic". Pediatrics 1954;14:421

6. Poole SR: The infant with acute, unexplained, excessive crying. Pediatrics 1991;88:450

7. Jenny C, Hymel KP, Ritzen A, et al: Analysis of missed cases of abusive head trauma. JAMA 1999;281:621

8. Alexander R, Crabbe L, Sato Y, et al: Serial abuse in children who are shaken. Am J Dis Child 1990;144:58

9. Heldrich FJ: Lethargy as a presenting symptom in patients with intussusception. Clin Pediatr 1986;25:363

10. Kuppermann N, O'Dea T, et al: Predictors of intussusception in young children. Arch Pediatr Adolesc Med 2000;154:250

11. Bonadio WM, Clarkson T, Naus J: The clinical features of children with malrotation of the intestine. Pediatr Emerg Care 1991;7:348

12. Kolecki P: Inadvertent methamphetamine poisoning in pediatric patients. Pediatr Emerg Care 1998;14:385

13. Macknin ML, Piedmonte M: Symptoms associated with infant teething: A prospective study. Pediatrics 2000;105:747