Pediatric Pulmonary Hypoplasia Treatment & Management

  • Author: Terry W Chin, MD, PhD; Chief Editor: Michael R Bye, MD   more...
 
Updated: Mar 5, 2009
 

Medical Care

  • In fetuses with pulmonary hypoplasia, before delivery and depending on the underlying lesion, a few interventions can be performed to increase the fetal lung volume and improve lung development.
    • Preterm rupture of membranes without signs of fetal distress or intrauterine infection is treated conservatively with or without tocolytics, antibiotics, and steroids in various combinations. Attempts have been made to seal the defect in the membranes by transcervically using "fibrin glue." However, this technique requires a preliminary cerclage, increases the risk of infection, and has limited efficacy.
    • Serial amnioinfusions are increasingly used in cases of preterm rupture of membranes at less than 32 weeks' gestation. This procedure, if successful, has been shown to decrease the risk of pulmonary hypoplasia and significantly improve perinatal outcome.
  • After delivery, the infant needs respiratory support, which can range from supplying supplemental oxygen to mechanical ventilation, including high-frequency ventilation and extracorporeal membrane oxygenation (ECMO). Ventilatory strategies have veered toward the use of permissive hypercapnia, especially in cases of congenital diaphragmatic hernia (CDH), with an increased survival rate in several reports. Partial liquid ventilation has also been used, without definite advantages.
    • ECMO is particularly indicated in infants with diaphragmatic hernia associated with pulmonary hypoplasia and when mild hypoplasia is complicated by persistent pulmonary hypertension. A decreased response to nitric oxide is believed to occur with pulmonary hypertension associated with hypoplasia.
    • Surfactant administration of 4 mL/kg in pulmonary hypoplasia secondary to CDH has been shown to be efficacious in improving oxygenation, decreasing the need for ECMO, and improving the survival rate if prophylactically administered at birth. Animal studies have shown that surfactant significantly improved lung mechanics and gas exchange and that lung tissue stores of surfactant protein B and phosphatidylcholine were low in diaphragmatic hernia.
    • Dialysis for support of renal function is provided in some cases, but it should be started only after careful consideration. Patients with severe chronic renal impairment with pulmonary hypoplasia have a poor prognosis; the ultimate outcome is difficult to improve, even with optimal renal and respiratory support.
    • Some studies suggest that strict infection control may improve the outcome of neonates with CDH without the need for ECMO.[20]
  • CDH has been associated with chronic pulmonary damage as well as cardiovascular disease, GI disease, failure to thrive, neurocognitive defects and musculoskeletal abnormalities.[21]
  • Management of any of the congenital cystic lung abnormalities such as cystic adenomatoid malformation (CAM) needs to consider the spontaneous improvement and possible resolution that occurs over months to years in many of these lesions.[22]
    • Their management must be individualized with very large lesions resulting in lung hypoplasia or fetal hydrops with possible fetal surgery required.[23]
    • In most cases of fetal lung lesions, continued observation with postnatal therapy occurs.[24]
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Surgical Care

  • In patients with severe CAM who have an extremely poor prognosis, fetal surgery is possible in certain centers. A multidisciplinary team with expertise in fetal surgery should evaluate both the fetus and the pregnant mother. A major indication for fetal surgery is the presence of hydrops and a gestation of less than 32 weeks. Thoracocentesis can allow for drainage of fluid from the CAM, but the fluid usually rapidly reaccumulates.
  • Intrauterine vesicoamniotic shunts and endoscopic ablation of posterior urethral valves are other techniques that are currently used in fetuses with urinary tract obstruction and pulmonary hypoplasia. With careful case selection, pulmonary hypoplasia is prevented, and postnatal renal and respiratory function is improved.
  • Percutaneous fetal endoluminal tracheal occlusion (FETO) with a balloon, inserted at 26-28 weeks' gestation, can be considered for infants with isolated CDH with poor prognosis.[25] This procedure was found to be minimally invasive, may reverse pulmonary hypoplasia changes, and may improve survival rate in these highly selected cases. In addition, the airways can be restored before birth.
    • In experimental animals, fetal tracheal occlusion (TO) induces lung growth and morphologic maturation. Fetoscopic TO with a clip may lead to accelerated lung growth and prevent pulmonary hypoplasia. However, one study showed that fetal TO used to treat severe CDH resulted in modest improvements in neonatal pulmonary function that are of questionable clinical significance
    • After delivery, surgery to correct diaphragmatic hernia, to correct CAM, and to decompress pleural effusions may be life-saving and curative in some cases.
  • The optimal time of surgery and the duration of ventilatory support used before surgery are controversial. The decision is made based on the lesion and the center's preferences.
  • Management of any of the congenital cystic lung abnormalities such as CAM needs to consider the spontaneous improvement and possible resolution that occurs over months to years in many of these lesions.[22] The risks of subsequent cancer are poorly understood and "probably overstated," and the degree of compensatory lung growth are also not well defined.
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Consultations

  • These patients should be followed by a pediatric pulmonologist after birth. Appropriate diagnostic tests can be performed. If early surgery is not performed during infancy, close follow-up of these patients are needed. As indicated above, some cystic lung abnormalities can spontaneously resolve over months to years. Newborns who have been referred for a cystic lesion observed by fetal ultrasonography may have complete resolution on chest CT scanning. Also, the occurrence of pneumonia or repeated respiratory infections may suggest surgical intervention is needed in a patient who has been conservatively managed.
  • Consult a pediatric surgeon for CDH, CAM, or any other lesion that requires surgery. Also, consult a pediatric surgeon in cases that involve pulmonary hypertension or respiratory failure that requires ECMO.
  • Consult a nephrologist and a urologist if a renal obstructive, cystic, or agenetic lesion is the cause of the pulmonary hypoplasia.
  • Consult a cardiologist and cardiothoracic surgeon if the patient has a causative or coexisting cardiac lesion, such as anomalous pulmonary venous connection.
  • Consult a neurologist in cases of congenital neuromuscular diseases.
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Contributor Information and Disclosures
Author

Terry W Chin, MD, PhD  Associate Director, Pediatric Allergy/Immunology/Pulmonology, Miller Children's Hospital, Long Beach Memorial Medical Center; Associate Professor, Department of Pediatrics, University of California, Irvine, School of Medicine

Terry W Chin, MD, PhD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Association of Immunologists, American College of Allergy, Asthma and Immunology, American College of Chest Physicians, American Thoracic Society, California Thoracic Society, Clinical Immunology Society, and Western Society for Pediatric Research

Disclosure: Nothing to disclose.

Coauthor(s)

Girija Natarajan, MD  Assistant Professor, Division of Neonatology, Children's Hospital of Michigan & Wayne State University

Girija Natarajan, MD is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Nothing to disclose.

Ibrahim Abdulhamid, MD  Assistant Professor of Pediatrics, Wayne State University; Director of Pediatric Pulmonary Medicine, Clinical Director of Pediatric Sleep Laboratory, Children's Hospital of Michigan

Ibrahim Abdulhamid, MD is a member of the following medical societies: American Academy of Pediatrics, American Academy of Sleep Medicine, and American Thoracic Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Susanna A McColley, MD  Associate Professor, Department of Pediatrics, Northwestern University, The Feinberg School of Medicine; Director of Cystic Fibrosis Center, Head, Division of Pulmonary Medicine, Children's Memorial Medical Center of Chicago

Susanna A McColley, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, American Sleep Disorders Association, and American Thoracic Society

Disclosure: Genentech Honoraria Speaking and teaching; Genentech Honoraria Consulting; Boston Scientific Consulting fee Consulting; Gilead Honoraria Speaking and teaching; Caremark Consulting fee Consulting; Vertex Pharmaceuticals Honoraria Speaking and teaching

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Pharmacy Editor, eMedicine

Disclosure: Nothing to disclose.

Heidi Connolly, MD  Associate Professor of Pediatrics and Psychiatry, University of Rochester; Director, Pediatric Sleep Medicine Services, Strong Sleep Disorders Center

Heidi Connolly, MD is a member of the following medical societies: American Academy of Pediatrics, American Thoracic Society, and Society of Critical Care Medicine

Disclosure: Nothing to disclose.

Mary E Cataletto, MD  Associate Director, Division of Pediatric Pulmonology, Winthrop University Hospital; Professor of Clinical Pediatrics, State University of New York at Stony Brook; Director of Children's Sleep Services, Winthrop University Hospital

Mary E Cataletto, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Chest Physicians

Disclosure: Shering Plough Pharmaceuticals Honoraria Consulting

Chief Editor

Michael R Bye, MD  Professor of Clinical Pediatrics, Division of Pulmonary Medicine, Columbia University College of Physicians and Surgeons; Attending Physician, Pediatric Pulmonary Medicine, Morgan Stanley Children's Hospital of New York Presbyterian, Columbia University Medical Center

Michael R Bye, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, and American Thoracic Society

Disclosure: Nothing to disclose.

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Chest radiograph of a newborn with primary pulmonary hypoplasia of the right lung showing shift of the mediastinum to the right hemithorax.
CT scan of the same patient (a newborn with primary pulmonary hypoplasia of the right lung) showing absence of the right lung. Note branching of the left lower lobe bronchus (horizontal arrow) and absence of airways in the right side (vertical arrow).
A posteroanterior radiograph of a 3-month-old infant with primary pulmonary hypoplasia of the right lung.
Lateral view of the same patient (a 3-month-old infant with primary pulmonary hypoplasia of the right lung) showing one dome of the diaphragm.
Bronchogram of the same patient (a 3-month-old infant with primary pulmonary hypoplasia of the right lung) showing absence of the airways in the right side and presence of the left main bronchus and its branches.
A chest radiograph of a 14-year-old child with primary pulmonary hypoplasia of the right side causing secondary scoliosis.
A chest radiograph of a newborn with achondroplasia and small chest causing hypoplasia of both lungs.
A chest radiograph of a newborn with diaphragmatic hernia in the right hemithorax shortly after birth.
CT scan of the same child (a newborn with diaphragmatic hernia in the right hemithorax shortly after birth) showing the presence of abdominal contents in the right hemithorax. Note the presence of the left lower bronchus and its main branches (horizontal arrow) and absence of the right lower lobe bronchus. The liver in the right hemithorax is indicated by the upper arrow.
A chest radiograph of a 10-month-old child after repair of a right diaphragmatic hernia showing loss of lung volume in the right hemithorax.
MRI of the same patient (a 10-month-old child after repair of a right diaphragmatic hernia) showing loss of right lung volume and smaller right pulmonary artery than the left pulmonary artery (arrow).
 
 
 
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