Pediatric Pulmonary Hypoplasia Follow-up

Updated: Aug 11, 2017
  • Author: Terry W Chin, MD, PhD; Chief Editor: Girish D Sharma, MD, FCCP, FAAP  more...
  • Print
Follow-up

Further Outpatient Care

Since chronic lung disease is common in survivors of pulmonary hypoplasia, these infants and children have an increased risk of fatality and serious morbidity from upper respiratory tract infections (URTIs) and lower respiratory tract infections (LRTIs). Antiviral and antibiotics should be administered based on clinical symptoms and signs.

Children may be given bronchodilators and/or inhaled corticosteroids for the treatment of wheezing episodes and/or reactive airway disease.

Respiratory syncytial virus (RSV) prophylaxis should be considered during RSV season in infants younger than two years who have been treated with oxygen or medication for chronic lung disease within 6 months of the start of RSV season. Palivizumab is a humanized monoclonal antibody (IgG) directed against the fusion protein of RSV and has been shown to reduce the risk of hospitalization from RSV infection in high-risk pediatric patients by 55%. RSV season in most parts of the United States is from October to March. The dose is 15 mg/kg via intramuscular injection monthly throughout RSV season.

Children with pulmonary hypoplasia should receive the influenza vaccine at the start of every influenza season, which in the United States, while varying from season to season, begins as early as October. The influenza season peaks in January or February and continues as late as May.

Children with chronic lung disease are considered at high risk for invasive pneumococcal disease. If younger than two years, they should be administered the 13-valent pneumococcal conjugate vaccine (PCV13) 4-dose series at ages two, four, and six months, with a booster dose at 12-15 months. If aged 24 months to five years, they should receive 1 or 2 doses of PCV13 if they have not already completed the 4-dose series. Anyone over the age of two, with chronic lung disease, should also receive 1 dose of PCV23.

Next:

Inpatient & Outpatient Medications

Various aerosolized medications such as bronchodilators and corticosteroids should be considered if symptoms suggest reactive airway disease or obstructive airway disease.

Persistent pulmonary arterial hypertension can be treated with various pulmonary vasodilators such as inhaled nitric oxide and sildenafil, and endothelin receptor inhibitors such as bosentan.

Previous
Next:

Complications

Complications in pediatric pulmonary hypoplasia are as follows:

  • Mortality due to acute respiratory failure in the neonatal period

  • Chronic respiratory failure or insufficiency

  • Pneumothorax, either spontaneous or as a result of ventilatory support

  • Persistent pulmonary hypertension caused by a reduced pulmonary vascular bed and worsened by hypoxia or a coexisting left-to-right intracardiac shunt

  • Chronic lung disease of infancy caused by prolonged ventilatory support

  • Airway abnormalities, including tracheobronchial compression and tracheomalacia caused by the displaced aorta and enlarged left pulmonary artery

  • Restrictive lung disease due to reduced total lung capacity

  • Recurrent respiratory infections

  • Recurrent wheezing episodes

  • Reduced exercise tolerance

  • Scoliosis in adolescent years due to abnormal thoracic cage development

  • Nutritional, musculoskeletal, neurological, and gastrointestinal comorbidities

  • Delayed growth and development

Previous
Next:

Prognosis

Mortality has traditionally been very high. In a retrospective study of 76 premature infants less than 35 weeks’ gestation, 20 had prolonged rupture of membrane of more than 5 days and were clinically diagnosed with pulmonary hypoplasia. Of those 20 infants with pulmonary hypoplasia, 18 died. In another retrospective study of 117 infants of less than 37 weeks’ gestation who had prolonged rupture of membrane of more than 99 hours, 11 died and were considered to have pulmonary hypoplasia. The median age of death was 20 hours (range, 12-48 h), mostly commonly from respiratory failure.

While antepartum amnioinfusions for treatment of oligohydramnios have significantly reduced the risk of pulmonary hypoplasia, longitudinal follow-up studies are lacking on the long-term outcomes of these children.

Of children with pulmonary hypoplasia secondary to congenital diaphragmatic hernia (CDH), the postnatal survival rate of CDH at tertiary centers has improved, with reported rates of 70-92%. [71] However, the survival rates do not account for the cases of CDH that are stillborn, died outside a tertiary center, or died as a result of spontaneous or therapeutic abortion.

CDH survivors have a high incidence of respiratory, nutritional, musculoskeletal, neurological, and gastrointestinal morbidities. [71] In a prospective study of 41 CDH survivors, abnormal muscle tone was found in 90% at age 6 months and 51% at age 24 months. While almost half (49%) had normal scores for neurocognitive and language skills, 17% had mildly delayed and 15% had severe delayed scores. Likewise, in psychomotor testing, while 46% had normal scores, 23% and 31% scored as mildly delayed and severely delayed, respectively. Autism was present in 7%. Studies of brain maturation using MRI show delayed structural brain development and other abnormalities that may lead to long-term neurologic complications. [72]

In a retrospective follow-up study of 55 children survivors with scimitar syndrome followed at one center, a high rate of respiratory complications was observed. All (100%) of the children had right lung hypoplasia of varying degrees of severity. The median duration of follow-up was 7.2 years. Pulmonary infections were reported in 38%, and 43% of children reported wheezing episodes during the last 12 months of follow-up. A restrictive pattern of lung function was observed in the majority of patients, likely related to right-sided lung hypoplasia. Lower total lung capacity values were seen in children with the infantile form of scimitar syndrome, possibly reflective of the severity of pulmonary hypoplasia in these children. [73]

Right-sided hypoplasia, typically secondary to right sided CDH, seems to carry a higher mortality. This is likely due to higher risk of recurrent herniation, increased risk of pulmonary complications, requiring pulmonary vasodilator therapy and tracheostomy. However, no differences in neurodevelopmental outcomes was found. [74, 75]

A minimum lung volume of 45% compared with age-matched control subjects has been shown to be a predictor of survival in neonates with diaphragmatic hernia treated with extracorporeal membrane oxygenation (ECMO). Similarly, a functional residual capacity of 12.3 mL/kg, about one half the normal capacity, has been thought to be a predictor of survival in pulmonary hypoplasia with CDH.

Previous