Patient Presentation
A 2-week-old male came to clinic with his parents for his health supervision visit. He had surpassed his birth weight by 10%. The parents voiced usual concerns, but the father also asked about anything special he should do because he himself had chronic Hepatitis C. He was an older father and had contracted it through a blood transfusion during a surgery as a teenager. He only knew about it several years later when he was told he could not donate blood. He was being monitored by his physician and his wife was Hepatitis C negative during pregnancy.

The pertinent physical exam showed a healthy newborn with growth parameters in the 50-90% He had some erythema toxicum on his face. The diagnosis of a healthy newborn was made. The pediatrician wasn’t sure if there were specific recommendations for newborns and other children living in a family with a Hepatitic C positive parent. After looking at several articles online including recommendations from two professional groups in different countries, the pediatrician contacted the father. The infant had already had Hepatitis B vaccine and would receive additional doses plus Hepatitis A at the proper time. The father was told to be careful with any blood or body fluids that the child over time could come in contact with. He was also advised to not share toothbrushes or razors with the child as he grew. The pediatrician also recommended continued monitoring by the father and mother’s own physicians.

Discussion
Hepatitis C virus (HCV) is a single-stranded RNA Flavivus that was first identified in 1989 and universal screening in the blood supply was begun in 1992 in the United States. Overall incidence of acute HCV in children under age 19 is 0.1 per 100,000.

In adults, the transmission is mainly from contaminated blood and body fluids, primarily intravenous drug use. It is the most common reason for liver transplantation in adults. Of those that acquire the acute infection, about 70% go on to become chronically infected. Adults have a slow progression of their disease with 20% having cirrhosis within 20 years. Being male, older, increased duration of infection, co-infections (particularly HIV and Hepatitis B), immunosuppression, hepatotoxic drug and alcohol use all increase the risk of cirrhosis. There is a 3-4% chance of hepatocellular carcinoma developing in chronic HCV patients with cirrhosis.

In children the transmission is mainly vertical. Among family members, transmission is uncommon but inapparent or direct percutaneous or mucosal exposure to blood could occur. Of infants born to HCV-positive mothers, about 5-10% will acquire HCV. Of those that are acutely infected, only about 50-60% become chronically infected, and 25-75% of those will spontaneously resolve by 2-3 years of age. Unfortunately school age children and teenagers who acquire the infection have a natural history that is like adults. Most children who were infected at birth have no symptoms and may have normal, or elevated transaminases and viral levels. Long-term studies (10-20 years) show perinatally acquired HCV patients only have a 5-10% chance of significant fibrosis and < 5% develop cirrhosis.

Testing is by specific immunoassays which detect IgG antibodies. Those for IgM are not available. Treatment recommendations are different for children infected through vertical transmission and older children and adults. Because younger children have a high spontaneous resolution rate, treatment may not be necessary particularly since there are side effects to the medications. Recommendations are also changing particularly in the adult populations as new medications are available and are studied more. Factors regarding treatment include: age, presumed transmission method, co-infection, genotype of HCV virus (type 1 is the most common in the US), and actual liver disease. Biomarkers for liver disease are available but are not as thoroughly developed and as useful in the pediatric population.

Learning Point
For families living with a HCV+ family member some general recommendations are advised. All patients should be vaccinated against Hepatitis A and B. HCV patients should also avoid hepatotoxic medications and excessive alcohol. Universal precautions with prompt treatment and clean-up of bloody wounds should be advised. There also should be no sharing of toothbrushes and razors. Breastfeeding is not contraindicated by a HCV+ mother. Patients who are HCV+ can go to a group-based childcare facility. For HCV+ people, changes in sexual practices are not recommended if a steady partner is maintained, but the partner should be informed of the risks and ways to prevent transmission. People with multiple sexual partners are recommended to use condoms and decrease the number of partners.

Questions for Further Discussion
1. When should infants be tested for HCV if their mother is HCV positive?
2. What is the incidence of Hepatitis C in your local area?

Related Cases

Disease: Hepatitis C

Symptom/Presentation:Health Maintenance and Disease Prevention

Specialty: Infectious Diseases

Age: Newborn

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: Hepatitis C

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

To view images related to this topic check Google Images.

American Academy of Pediatrics. Hepatitis C, In Pickering LD, Baker CJ, Kimberlin DW, Long SS, eds. Red Book: 2009 Report of the Committee on Infectious Diseases. 28th edit. Elk Grove Village, IL: American Academy of Pediatrics; 2009; Accessed from the Internet on 3/6/13.

Jhaveri R. Diagnosis and management of hepatitis C virus-infected children. Pediatr Infect Dis J. 2011 Nov;30(11):983-5.

Porto AF, Tormey L, Lim JK. Management of chronic hepatitis C infection in children. Curr Opin Pediatr. 2012 Feb;24(1):113-20.

Lagging M, Duberg AS, Wejstal R, Weiland O, Lindh M, Aleman S, Josephson F; Swedish Consensus Group. Treatment of hepatitis C virus infection in adults and children: updated Swedish consensus recommendations. Scand J Infect Dis. 2012 Jul;44(7):502-21.

ACGME Competencies Highlighted by Case

Patient Presentation
A 5-year-old female was admitted to the inpatient service after a cuneiform to cuboid wedge surgery for recurrent talipes equinovarus on the left foot. The past medical history showed bilateral congenital clubfoot that was treated by the Ponseti method of serial casting and application of nightime foot orthosis for 2 years. Since that time the parents had noted an increase in left foot adduction and pain. The pertinent physical exam showed a healthy appearing female who was sleeping post-op. The left foot was in a plaster cast that had some sanguinous fluid on the cast at the heel. There was good capillary refill and mild swelling of the toes. The diagnosis of post-operative recurrent talipes equinovarus was confirmed. Overnight, the patient’s clinical course revealed that her pain was well-controlled with oral medication. The next day, physical therapy was able to get the patient up on crutches and she was sent home to followup in 6 weeks with orthopaedics.

Figure 104 – AP radiographs of both feet show a persistent metatarsus adductus deformity in the left foot. There is a dysplastic appearing medial cuneiform bone with associated widening at the first tarsal-metatarsal joint.

Discussion
Newborn foot deformities are relatively common with an incidence of ~4.2%. About 75% of these are due to metatarsus adductus. Talipes equinovarus, calcaneovulgus foot and vertical talus are the other most common abnormalities.

Congenital talipes equinovarus or clubfoot deformity occurs in 1-2/1000 births. The mneumonic CAVE identifies the major findings of Cavus, forefoot Adduction, hindfoot Varus and Equinus. Clubfoot can be mild to severe with the more severe forms being associated with other problems.

The Ponseti method is currently considered the main treatment method with better short-term outcomes compared to other techniques. Dr. Ignatio Ponseti at the University of Iowa developed a technique of serial casting of the feet. Most patients require 5-7 casts changed weekly with longer treatment for more severely affected feet. A period of stabilization with ankle-foot orthoses (AFOs) is required to maintain the desired outcome. Sometimes a tendon transfer is also necessary.

Learning Point
Originally, Dr. Ponsetti had a 50% relapse rate with his early patients, but later this was decreased to 7% by overcorrection of the initial deformity with the initial casting and use of the night AFO splinting.

Overall initial correction of deformity is as high as 93-100% of cases, but there can be persistent or recurrent deformity. Persistent deformity is one that has been incompletely corrected initially before being placed into the AFO. Recurrent deformity is one that was initially corrected, properly placed and maintained in the AFO, but recurs months to years later. Overall relapse rates range from 4-41%. Of patients that are compliant with AFO use, this is decreased from 0-21%.

There are 3 main reasons for relapse:

• Non-compliance with the AFO use – caregivers fail to understand the importance of consistent use of the AFO. Incidence of non-compliance with AFO use is 0-51%
• Idiopathic
• Increased risk – underlying disorder such as arthrogryposis, unrecognized or known neuropathy (e.g. Charot-Marie Tooth, myasthenia gravis, myotonic dystrophy, meningomyelocoele), and syndromes (e.g. Larsen syndrome).
  Non-idiopathic clubfeet have a higher rate of relapse; from 24-56% depending on the population.

Treatment of relapse includes repeated casting and/or tenotomy, tibialis anterior tendon transfer and other surgical techniques depending on the specific area of deformity.

Questions for Further Discussion
1. What is the difference between calcaneovalgus foot and posteromedial bowing of the tibia?
2. How is metatarsus adductus treated?

Related Cases

Disease: Talipes Equinovarus | Birth Defects | Foot Injuries and Disorders

Symptom/Presentation: Foot Pain

Specialty: Orthopaedic Surgery and Sports Medicine | Radiology / Nuclear Medicine / Radiation Oncology

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 these topics: Birth Defects and Foot Injuries and Disorders.

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

To view images related to this topic check Google Images.

Sankar WN, Weiss J, Skaggs DL. Orthopaedic conditions in the newborn. J Am Acad Orthop Surg. 2009 Feb;17(2):112-22.

Jowett CR, Morcuende JA, Ramachandran M. Management of congenital talipes equinovarus using the Ponseti method: a systematic review. J Bone Joint Surg Br. 2011 Sep;93(9):1160-4.

Gray K, Pacey V, Gibbons P, Little D, Frost C, Burns J. Interventions for congenital talipes equinovarus (clubfoot). Cochrane Database Syst Rev. 2012 Apr 18;4:CD008602. doi: 10.1002/14651858.CD008602.pub2.

Chu A, Lehman WB. Persistent clubfoot deformity following treatment by the Ponseti method. J Pediatr Orthop B. 2012 Jan;21(1):40-6.

ACGME Competencies Highlighted by Case

Patient Presentation
A 6-month-old female came to clinic with mild fever, copious rhinitis and poor fluid intake for 48 hours. Children at her daycare had respiratory syncytial virus and her symptoms were getting worse over the past 8 hours with no fluid intake. The past medical history showed a healthy infant who had received influenza vaccine.

The pertinent physical exam showed a respiratory rate of 62, pulse of 119, temperature of 37.8° and an oxygen saturation of 87%. HEENT showed copious clear rhinorrhea. Lungs had mild wheezing. She had intercostal retractions and nasal flaring, but no abdominal breathing and no trachael tugging. The rest of her examination was normal. The diagnosis of probable respiratory syncytial virus (RSV) and hypoxia was made. She was placed on oxygen at 45% FiO₂ with nasal prongs and her saturations increased to 95%. She was admitted to the hospital for oxygen therapy. Over the next 48 hours she was slowly weaned off oxygen and was orally rehydrated. The laboratory evaluation confirmed RSV.

Discussion
Oxygen is the most common element on earth. It makes up 21% of air, 89% of seawater and 46% of the earth’s crust. It is a highly reactive element (including being highly flammable) that must be combined to be stable (O₂ molecular form) and non-reactive at ambient temperature and pressure.

A continuous supply of oxygen is necessary for human life and lack of oxygen leads to hypoxic brain damage and other end organ damage such as the liver, kidneys and heart. Thus in hypoxic situations, emergency oxygen use is necessary.

Situations where O₂ is necessary in high concentrations includes major trauma, shock, sepsis, cardiac arrest, and poisoning with cyanide or carbon monoxide. Other situations where oxygen is likely needed to keep within the saturation range of 94-98% include asthma, pneumonia, heart failure and pulmonary embolism. Additional situations where a lower saturation range (88-92%) are wanted but oxygen is still necessary include diseases where an element of hypercapnea is normal and include cystic fibrosis, chronic neuromuscular disease, hypoventilation syndromes and morbid obesity, COPD and some congenital heart disease. Oxygen therapy is also used for altitude sickness, decompression sickness, wound therapy and other indications.

The target saturations are based on S-shaped oxygen-hemoglobin saturation curve. Below ~88% saturation the curve’s slope is steep and small changes in the partial pressure of oxygen correspond to inadequate oxygen binding to hemoglobin and thus ineffectively delivery of adequate oxygen to tissues.

Learning Point
Oxygen is relatively inexpensive itself, but the equipment to deliver it and monitor the therapy can be more expensive. Equipment varies by availability, ease of patient use or preference, amount of flow or rate, oxygen concentration and the ability to apply positive pressure.

Basic options for non-invasive oxygen therapy include:

• Nasal canula or prongs (View Image) – easy to adjust flow and concentration at any time, comfortable for patients to use and relatively inexpensive.
• Simple facemask (View Image) – similar to nasal canula but other patients find this more comfortable.
• Venturi mask (View Image) – specifically controls concentration and flow, good for patients who are oxygen-sensitive.
• Reservoir mask (View Image) – also known as a non-rebreather, for patients who are critically hypoxemic or ill, often used until other oxygen delivery methods can be used.

Basic options for non-invasive ventilation include:

• CPAP (View Image) – continuous positive airway pressure, has a tight-fitting mask or helmet nasalpharyngeal prongs. The patient usually is spontaneously breathing, but PEEP (positive end expiratory pressure) is increased from normal. NPCPAP is commonly used in ill preterm infants. Nightime CPAP is often used for obstructive sleep apnea.
• NIPSV – non-invasive pressure support ventilation is sometimes called BIPAP (bilevel positive airway pressure) or Bilevel PS (bilevel pressure support). This uses a ventilator to assist the patient by applying both PEEP at the end of expiration but also additional pressure during inspiration. The patient is not intubated in the traditional sense but the ventilator assists the patient.

Options for ventilators today mainly include positive pressure ventilation, and high frequency oscillatory ventilation.

Among these various basic delivery methods are variations and refinements.

In developed countries these oxygen delivery methods are widely available, but in resource-limited settings across the world where more than 99% of the mortality of children <5 years occurs simple delivery methods may not be available. Pneumonia accounts for more than 1,400,000 annual deaths worldwide and recent recommendations for expanding basic and advanced life support in these countries have been proposed. Basic equipment including a stethoscope, metered dose inhaler and spacer, +/- bronchodilator therapy are recommended. For higher levels of care then nasal prongs and oxygen concentrator are recommended. As saturation monitors are usually not available clinical indicators of hypoxemia are recommended including evaluation for RR > 70 bpm, head nodding, grunting, chest retractions, nasal flaring, lethargy, central cyanosis and inability to eat or drink.

Questions for Further Discussion
1. What are the oxygen delivery methods available locally?
2. How can a respiratory therapist be helpful in the evaluation and treatment of a patient with respiratory distress?
3. What other medication can be delivered along with oxygen therapy?

Related Cases

Disease: Respiratory Syncytial Virus Infections | Oxygen Therapy

Symptom/Presentation: Respiratory Distress

Specialty: Allergy / Pulmonary Diseases | Critical Care | Pharmacology / Toxicology

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, and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for these topics: Oxygen Therapy and Respiratory Syncytial Virus Infections.

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

Masip J. Non-invasive ventilation. Heart Fail Rev. 2007 Jun;12(2):119-24.

Rojas MX, Granados Rugeles C, Charry-Anzola LP.Oxygen therapy for lower respiratory tract infections in children between 3 months and 15 years of age. Cochrane Database Syst Rev. 2009 Jan 21;(1):CD005975.

O’Driscoll R. Emergency oxygen use. BMJ 2012;345:e6856.

Encycloaedia Britannica Online, s. v. “oxygen (O),” accessed March 05, 2013, http://www.britannica.com/EBchecked/topic/436806/oxygen.

Ralston ME, Day LT, Slusher TM, Musa NL, Doss HS. Global paediatric advanced life support: improving child survival in limited-resource settings. Lancet. 2013 Jan 19;381(9862):256-65.

ACGME Competencies Highlighted by Case

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