Physician Toolkit: Managing Fetal Lung Lesions

Lung Lesion Experience at CHOP: 1995-2014 More than 1,700Patients referred to the Center for Fetal Diagnosis and Treatment for treatment of lung lesions More than 1,200Patients received postnatal surgical care for lung lesions 24Open fetal surgeries performed on lung lesion patients 31EXIT deliveries95Thoracoamniotic shunts All numbers except deliveries reflect data from 1995-March 2014 at The Children's Hospital of Philadelphia. Lung lesions are extremely rare, serious diagnoses; so rare that most obstetricians may see only one case in their entire career. The Center for Fetal Diagnosis and Treatment has managed more than 1,700 pregnancies prenatally diagnosed with fetal lung lesions, the largest volume of these patients of any center in the world.

We have treated patients presenting with all types of lung lesions, including congenital cystic adenomatoid malformation (CCAM, also known as congenital pulmonary airway malformation, or CPAM), bronchopulmonary sequestration (BPS), hybrid lesions, chronic high airway obstruction syndrome (CHAOS), pleural effusion, hydrothorax and other pulmonary diagnoses.

Through this experience, we have learned that lung lesions are unpredictable and challenging to diagnose, requiring meticulous prenatal diagnosis to properly manage the broad spectrum of presentations, from a very small mass to a very large growth that can cause significant problems for both fetus and mother.

To better partner with you and share the lessons we’ve learned through our experience managing fetal lung lesions, the Center has created a number of professional-focused resources about fetal lung lesions, including prenatal management and diagnostic tools, a collection of recent research, and educational resources that you can share with your patients.

Resources for professionals

Prognostic tool: CCAM volume ratio, or CVR

Prenatal management and the overall prognosis of a lung lesion depends on the size and growth of the mass. Accurate diagnosis is critical.

At the Center for Fetal Diagnosis and Treatment, prenatal management of lung lesions includes sonographic surveillance during which we obtain measurements that can indicate the severity of the condition and confirm the prenatal differential diagnosis of the lesion.

More than 15 years ago, our imaging specialists developed the CCAM Volume Ratio, also called the CVR, as a prognostic tool to measure the size of lung lesions before birth. This ratio helps determine the frequency of sonographic evaluation required over the duration of the pregnancy, and in many cases, the CVR enables us to predict the likelihood of hydrops developing.

Download the CVR prognostic tool »

In Utero Insights newsletter

The fetal lung lesion edition of In Utero Insights, the Center's newsletter to the professional community, focuses on the diagnosis and management of fetal lung lesions, both before and after delivery.

This issue offers a detailed look at the care and resources available to families whose pregnancies are diagnosed with lung lesions, as well as our research into improved therapeutic interventions. Topics include:

  • A look at the rare level of expertise we offer in the diagnosis and care of lung lesions
  • Increasing understanding of the genetic and molecular mechanisms responsible for CCAM through research
  • The role of imaging in the comprehensive evaluation of lung lesions, before and after birth
  • A look into the holistic care provided by the Center, from diagnosis through long-term follow-up and annual patient/family reunions
  • Providing anesthesia for patients with congenital lung lesions
  • A case study of thoracoscopic lobectomy for asymptomatic CCAM

Online CME

The Center for Fetal Diagnosis and Treatment offers a free online continuing medical education module on the Management of Fetal Lung Lesions. This educational activity seeks to:

  • Provide participants a foundation and framework for confidently identifying challenging prenatal lung anomalies and determining appropriate level of perinatal management.
  • Provide evidence-based diagnostic techniques, current options and criteria for fetal intervention, considerations for appropriate delivery planning, and guidelines for neonatal and pediatric surgical management.
  • Enhance clinicians’ skill in diagnosing, counseling and managing pregnancies complicated by these anomalies and caring for neonates with these defects.

The CME is available for 1.5 AMA PRA Category 1 CreditTM for physicians and is offered free of charge. Visit our online CME page for course details, accreditation information, and to register and take the course.

Recent publications and research on lung lesions at CHOP

Our team members have written extensively about the management of fetal lung lesions. Visit our publications page for an extensive list of fetal lung lesion publications »

Resources you can share with patients

Condition content

The Center has detailed diagnosis-specific information about lung lesions, specifically CCAM and BPS, as well as other fetal anomalies, written for families.

Patient stories

Several of our patient families have shared their experiences about what it was like to be diagnosed and treated for fetal lung lesions, with the goal of helping other families who are facing a similar diagnosis.

If your patient is looking for more information about what to expect, here are some stories for them to learn more about other families’ personal experiences with fetal lung lesions.

Bronchopulmonary sequestration (BPS)

Congenital cystic adenomatoid malformation (CCAM)

Chronic high airway obstruction syndrome (CHAOS)

Video about fetal lung lesions

In our DVD, Breathing Easier: Fetal Lung Anomalies, Center team members describe different fetal lung anomalies, including CCAM and BPS. The video explains the treatment options available and the multidisciplinary care provided for both mother and baby at The Children's Hospital of Philadelphia. Created for clinicians and patients, it offers educational information about these complex conditions.

To request a copy of the DVD, call 1-800-IN-UTERO (1-800-468-8376) or complete our online form.

Reviewed on February 28, 2014