Patient Presentation
A 4 month old male came to the emergency room with fever to 103° and cough for 48 hours. The coughing had been much worse over the past day but there was no apnea or cyanosis. The patient had not had anything to drink for the past 8 hours. The past medical history showed a full-term infant without neonatal problems. He was current on immunizations. The family history showed no pulmonary disease.

The pertinent physical exam revealed a tired appearing male with a respiratory rate of 62, pulse of 114, with normal blood pressure and temperature. His pulse oximeter was 88% on room air. His capillary refill was 3 seconds. HEENT showed clear rhinitis. Lungs had some mild coarse breath sounds throughout the fields with decreased sounds on the right. The rest of his examination was normal. The work-up included a venous blood gas of pH= 7.34, CO2 = 38 and O2 of 56 with a base of -6. A respiratory viral panel was negative for influenza, respiratory syncytial virus and other viruses. A pertussis nasal swab was also negative. The radiologic evaluation of a chest radiograph showed a right upper lobe consolidation with a moderate apical/anterior pneumothorax and small pneumomediastinum. The diagnosis of bilateral lower lobe pneumonia and spontaneous pneumothorax and pneumomediastum was made. He was treated conservatively with oxygen at 100% by nasal canula, IV fluids and antibiotics for community-acquired pneumonia. He was slowly improving clinically after 5 days.

Figure 105 – 06-20-13 – AP view of the chest demonstrates right upper lobe collapse, patchy bibasilar infiltrates felt to represent bacterial pneumonia, and pneumomediastium outlining the inferior border of the heart – a continuous diaphragm sign.
Figure 106 – 06-20-13 – Left lateral decubitus view of the chest demonstrates a small right pneumothorax.

Discussion
“A pneumothorax is a collection of air in the pleural space, and it can be categorized into spontaneous, traumatic or iatrogenic. Spontaneous pneumothorax can be further classified into primary with no clinical evidence of underlying lung disease or secondary due to pre-existing lung disease.”

Spontaneous pneumothorax is a condition that is relatively rare in pediatrics. There is a bimodal age distribution – neonates and late adolescence. It is caused by tearing of the visceral pleural. Clinical signs include chest pain, dyspnea, tachycardia, tracheal deviation towards contralateral side, hypotension, cyanosis.

There is a wide variation in treatment practices particularly for large pneumothoraces. For small ones, most are treated conservatively with or without oxygen therapy, and treatment for an underlying cause if present. Large pneumothoraces can be treated conservatively, by aspiration, chest tube, pleurodesis and/or surgery. The pneumothorax is seen on AP radiographs, but decubutus radiographs often make the pneumothorax more prominent. Because air will track anteriorly on a supine chest radiograph often used in small children, pneumothorax in these children can easily be missed on the AP but not on the decubitus radiograph.

To review the complications of pneumonia and its common infectious disease causative agents, see What Are the Complications of Pneumonia?.

Learning Point

Causes of secondary spontaneous pneumothorax include:

• Airway disease
  • Asthma, associated with
  • Bronchopulmonary dysplasia
  • Bronchiectesis
  • Cystic fibrosis
• Congenital lung disease
  • Congenital lobar emphysema
  • Cystic adenomatoid malformation
• Interstitial lung disease
  • Saroidosis
  • Langerhans cell histiocytosis
• Infectious disease
  • Measles
  • Pneumonia or abscess
  • Pneumocystis jirovecii
  • Parasitic, especially ecchinococcal
  • Tuberculosis
• Connective tissue disease
  • Marfan
  • Dermatomyositis
  • Ehler-Dahlos
  • Polymyositis
  • Systemic lupus erythematosis
• Other
  • Catamenial pneumothorax or intrathoracic endometriosis
  • Foreign body
  • Malnutrition

Questions for Further Discussion
1. What is the pathophysiology behind treating with oxygen for pneumothorax?
2. How should a recurrent pneumothorax be treated?

Related Cases

Disease: Spontaneous Pneumothorax | Pleural Disorders | Lung Disease

Symptom/Presentation: Fever and Fever of Unknown Origin | Respiratory Distress

Specialty: Allergy / Pulmonary Diseases | Radiology / Nuclear Medicine / Radiation Oncology | Surgery

Age: Infant

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for this topic: Pleural Disorders

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

Michel JL. Spontaneous pneumothorax in children. Arch Pediatr. 2000 Mar;7 Suppl 1:39S-43S.

O’Lone E, Elphick HE, Robinson PJ. Spontaneous pneumothorax in children: when is invasive treatment indicated? Pediatr Pulmonol. 2008 Jan;43(1):41-6.

Robinson PD, Cooper P, Ranganathan SC. Evidence-based management of paediatric primary spontaneous pneumothorax. Paediatr Respir Rev. 2009 Sep;10(3):110-7.

Roberts D, Wacogne I. Question 3. In patients with spontaneous pneumothorax, does treatment with oxygen increase resolution rate? Arch Dis Child. 2010 May;95(5):397-8.

Kurihara M, Kataoka H, Ishikawa A, Endo R. Latest treatments for spontaneous pneumothorax. Gen Thorac Cardiovasc Surg. 2010 Mar;58(3):113-9.

ACGME Competencies Highlighted by Case

Patient Presentation
While working on the general pediatric inpatient service, a pediatrician had 4 patients with similar stories.
All were under 1 year of age and were admitted for respiratory distress, cough and dehydration due to respiratory syncytial virus.
Each had a prolonged illness or needed readmission because each also had an underlying airway malacia that had been diagnosed by bronchoscopy – laryngomalacia, tracheomalacia, tracheobronchomalacia and bronchomalacia.
The patient with laryngomalacia also had inspiratory stridor during this admission. Those with lower airway malacias originally were diagnosed after having a chronic cough, poor weight gain, and/or had additional underlying disease problems (i.e. genetic syndrome).
The residents all had noted that the farther down in the airway the malacia was, the more ill the patient was clinically and/or had other underlying disease problems.
This offered a good chance to review how airway malacias can present.

Discussion
Stridor is a variably pitched sound caused by increased turbulence and airflow through a narrowed part of the large airway. Usually due to narrowing of the larynx or extrathoracic trachea, stridor is usually inspiratory.
Biphasic stridor usually is due to a fixed airway obstruction at the level of the glottis or subglottis but may also extend to the mid-thoracic trachea.

Stridor is different than stertor which is a heavy-snoring, inspiratory sound occurring in coma or deep sleep, sometimes due to obstruction of the larynx or upper airways. Causes of stertor include choanal stenosis, enlarged tonsils and/or adenoids, and redundant upper airway tissues above the larynx.

Causes of upper airway obstruction that can cause stridor include:

• Acute laryngotracheobronchitis (i.e. croup)
• Epiglottitis
• Hereditary angioneurotic edema
• Intubation, post-endotracheal
• Laryngomalacia
• Laryngeal cleft
• Laryngeal cysts
• Micrognathia or retrognathia
• Mucus retention cysts
• Subglottic stenosis
• Thyroglossal duct remnant
• Vocal cord paralysis – forceps delivery, Chiari malformation

Learning Point
Malacia means a softening of the tissues and is named by its location.

Laryngomalacia usually presents with noisy breathing and inspiratory stridor. There can be collapse of the arytenoids, epiglottis, and aryepiglottic folds.
Symptoms usually present around 10 days, worsen for the next several months, and then resolve by 2 years of age.
It is considered a developmental process and is usually benign.
Laryngomalacia can be associated with other airway malacias. In one study, concomitant tracheomalacia (29%) bronchomalacia (10%) and tracheobronchomalacia (7%) were identified.

Recurrent wheeze, chronic cough and recurrent respiratory tract illnesses were common problems seen in patients with both laryngomalacia and another airway malacias.

Tracheomalacia is a localized or generalized “…weakness of the trachea which causes luminal obstruction at times of increased intrathoracic pressure, such as expiration or coughing.” Tracheobronchomalacia involves both the trachea and bronchi, and bronchomalacia involves the bronchus.
Patients with all 3 intrinsic airway malacia types may have minor respiratory infections but they can also cause apnea, cyanosis and life-threatening airway obstruction. Chronic coughing and wheezing are common presentations. Most have increased illness severity during acute illnesses and take longer to resolve.
The airway malacias may be part of other congenital anomalies (including cardiac anomalies) and therefore be more difficult to treat. Treatment can include surgical (e.g. aortopexy, tracheopexy, external or internal airway stenting), and mechanical (positive airway pressure). These airway malacias often do not resolve and therefore may require treatment.

Questions for Further Discussion
1. What are indications for bronchoscopy?
2. What different delivery methods are there for oxygen therapy?

Related Cases

Disease: Airway Malacia | Breathing Problems | Respiratory Diseases | Trachael Disorders

Symptom/Presentation: Respiratory Distress | Stridor

Specialty: Otolaryngology

Age: Infant

To Learn More
To view pediatric review articles on these topics from the past year check
PubMed – Laryngomalacia,
PubMed – Tracheomalacia, and
PubMed – Bronchomalacia.

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for these topics: Breathing Problems and Tracheal Disorders.

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

Eber E. Evaluation of the upper airway. Paediatr Respir Rev. 2004 Mar;5(1):9-16.

Vijayasekaran D, Gowrishankar NC, Kalpana S, Vivekanandan VE, Balakrishnan MS, Suresh S. Lower airway anomalies in infants with laryngomalacia. Indian J Pediatr. 2010 Apr;77(4):403-6.

Masters IB, Zimmerman PV, Pandeya N, Petsky HL, Wilson SB, Chang AB. Quantified tracheobronchomalacia disorders and their clinical profiles in children. Chest. 2008 Feb;133(2):461-7.

Calkoen EE, Gabra HO, Roebuck DJ, Kiely E, Elliott MJ. Aortopexy as treatment for tracheo-bronchomalacia in children: an 18-year single-center experience. Pediatr Crit Care Med. 2011 Sep;12(5):545-51.

Goyal V, Masters IB, Chang AB. Interventions for primary (intrinsic) tracheomalacia in children. Cochrane Database Syst Rev. 2012 Oct 17;10:CD005304.

Midyat L, Cakır E, Kut A. Upper airway abnormalities detected in children using flexible bronchoscopy. Int J Pediatr Otorhinolaryngol. 2012 Apr;76(4):560-3.

ACGME Competencies Highlighted by Case

Patient Presentation
A 2-year-old female came to clinic after 16 hours of severe right ear pain. She had fever to 38.5° and was being given ibuprofen. Previously she had rhinorrhea for 3 days but no cough. The past medical history showed an episode of otitis media 7 months previously. The review of systems was otherwise negative. The pertinent physical exam showed a cranky female with normal vital signs and growth parameters in the 90-95% for age. HEENT revealed moderate rhinorrhea, normal pharynx and eyes. Her left tympanic membrane was erythematous with mild bulging, distorted landmarks and immobility. Her right tympanic membrane was very erythematous with an orange hue, and was dramatically bulging with 3 blisters on the lower 1/2 of the membrane. The rest of her examination was negative. The diagnosis of bullous myringitis was made and amoxicillin-clavalaunic acid was given. The parents were told to use acetaminophen or ibuprofen for pain relief and told that because the blisters were relatively friable there was a chance that one could break and the child would have otorrhea. The patient was to follow-up in about 4 weeks to recheck the ear.

Discussion
Bullous myringitis (BM) is felt to be a variation of acute otitis media (AOM) with more severe symptoms. Bullae (blisters or “balloons”) on the tympanic membrane occur between the outer epithelial layer and middle fibrous layers of the tympanic membrane. The exact reason for this is unknown but felt to be probably due to a strong inflammatory reaction in the middle ear begun by viral or bacterial pathogens. The pain is felt to be due to irritation of the highly innervated outer epithelial layer. The most common pathogens are the same as AOM but Streptococcus pneumoniae is detected more often. The bullae can occur on the tympanic membrane but also extend to the proximal aspect of the external ear canal (in about 10% of BM cases). Bullae that only involve the external canal are due to otitis externa and should be distinguished from BM. Symptoms that are present more often in patients with BM than AOM include severe earache and fever, but also ear rubbing, poor sleep, more crying and decreased appetite.

While most cases are due to infectious diseases, one case in the literature reported BM due to organic solvent (paint thinner) entering the nasal cavity and into the middle ear with what appeared to be direct cellular damage to the structures.

The American Academy of Pediatrics recently updated their clinical practice guidelines for the treatment of acute otitis media in children. See To Learn More below.

Learning Point
Overall, BM is felt to occur in < 10% of patients with AOM. One prospective longitudinal cohort study found of 2028 children followed from 2-24 months, they had 1876 visits for AOM (1876/2028 = 92.5%) in 2683 ears. Eighty-six visits were for BM (86/1876 = 4.6%) in 92 ears. Bullae spread from the tympanic membrane to the external canal in 9 ears (9/92 = 9.8%).

Questions for Further Discussion
1. What is the difference between pneumatic otoscopy and tympanometry? How do they help to determine if AOM is present?
2. What other treatment(s) besides antibiotics can be offered for BM?

Related Cases

Disease: Bullous Myringitis | Ear Disorders | Ear Infections

Symptom/Presentation: Ear Pain | Fever and Fever of Unknown Origin | Rhinitis

Specialty: General Pediatrics | Infectious Diseases | Otolaryngology

Age: Toddler

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for this topic: Ear Infections

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

McCormick DP, Saeed KA, Pittman C, Baldwin CD, Friedman N, Teichgraeber DC, Chonmaitree T. Bullous myringitis: a case-control study. Pediatrics. 2003 Oct;112(4):982-6.

Minoda R, Miwa T, Sanuki T, Yumoto E. An unusual cause of bullous myringitis with acute otitis media. Otolaryngol Head Neck Surg. 2011 Nov;145(5):874-5.

Kotikoski MJ, Palmu AA, Puhakka HJ. The symptoms and clinical course of acute bullous myringitis in children less than two years of age. Int J Pediatr Otorhinolaryngol. 2003 Feb;67(2):165-72.

Liberthal AS, Carroll AE, Chonmaitree T et al. The Diagnosis and Management of Acute Otitis Media. Pediatrics. 2013. Available from the Internet at: http://pediatrics.aappublications.org/content/early/2013/02/20/peds.2012-3488.abstract (rev. 2/25/13, cited 2/25/13).

ACGME Competencies Highlighted by Case

Patient Presentation
An emergency medicine physician called a regional children’s hospital for consultation with a general pediatrician. His question was could a school age child who had elevated transaminase levels (2-3x normal), elevated bilirubin and mild clinical jaundice but no other clinical symptoms be infected by Epstein Barr Virus? The brief story was that the child had a fever for 2 days and the parent noted scleral icterus. There was no abdominal pain. The general pediatrician was fairly sure that EBV could cause these types of problems, and knew that it could cause splenomegaly, and was a major complication of solid organ transplants particularly liver transplants. However, the general pediatrician wasn’t sure of treatments so he offered the emergency room physician to be reconnected with a pediatric gastroenterologist. He assumed the consultation with the gastroenterologist went well, because he had told the emergency room physician to contact him if there were any problems contacting the gastroenterologist, and he had not heard back from the physician.

Discussion

Infectious mononucleosis is caused by an Epstein-Barr Virus (EBV) infection causing the triad of fever, sore throat and adenopathy.

The differential diagnosis of clinical presentations similar to EBV includes:

• Viral
  • Cytomegalovirus
  • Herpes simplex
  • Hepatitis A, B, C
  • HIV
  • Varicella
• Bacterial/Spirochete
  • Brucellosis
  • Leptospirosis
  • Syphilis
  • Q fever
• Miscellaneous
  • Autoimmune hepatitis
  • Drug side effects
  • Ischemia
  • Wilson Disease

Treatment for EBV infections is mainly supportive. Anti-viral medications such as ganciclovir are usually used for severe problems. Liver failure has been treated by transplant.
Refraining from activities which could cause abdominal trauma while splenomegaly is apparent is recommended as is medications that are hepatotoxic until liver function tests normalize.

Learning Point
EBV infections are usually asymptomatic with 90-95% of people by age 18 becoming seropositive.
EBV is known have many different clinical presentations which can be reviewed here.
A monospot test for EBV can be falsely positive and reasons for this can be reviewed here.

Gastrointestinal problems related to EBV include:

• Mildly elevated transaminase levels (2-3x normal) that are asymptomatic clinically
• Laboratory test abnormalities
  • Alkaline phosphatase – 60%
  • Bilirubin – 45%
• Mild abdominal pain – 15%
• Jaundice – < 5 %
• Hepatitis (with moderate 5-10 x transaminase elevation) – 80-90%
• Hepatosplenomegaly 6-14%
• Splenomegaly 50-60%
• Splenic rupture
• Liver failure

Liver function test abnormalities occur usually during the second week of illness and resolve within 2-6 weeks.
Severe liver problems are rare but can occur and may be deadly. Fulminant cases are usually associated with a concomitant immunodeficiency.
Chronic EBV infections may also be implicated with autoimmune hepatitis and hepatocellular carcinoma.

Questions for Further Discussion
1. List other clinical presentations of EBV infections?
2. When do heterophil antibodies become positive after infection?

Related Cases

Disease: Infectious Mononucleosis

Symptom/Presentation: Fever and Fever of Unknown Origin | Abnormal Laboratory Test

Specialty: General Pediatrics
| Gastroenterology | Infectious Diseases

Age: School Ager

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com, the National Guideline Clearinghouse and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for this topic: Infectious Mononucleosis.

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

Feranchak AP, Tyson RW, Narkewicz MR, Karrer FM, Sokol RJ. Fulminant Epstein-Barr viral hepatitis: orthotopic liver transplantation and review of the literature. Liver Transpl Surg. 1998 Nov;4(6):469-76.

Crum NF. Epstein Barr virus hepatitis: case series and review. South Med J. 2006 May;99(5):544-7.

Kelly DA. Current issues in pediatric transplantation. Pediatr Transplant. 2006 Sep;10(6):712-20.

ACGME Competencies Highlighted by Case