Rare and Sometimes Disappearing: The Tough Life of the Sonographic Expert

Everyday obstetricians hear the same questions: “Is my baby OK?” “Is there anything abnormal?” or, and this is may be the worst, “Should I be worried about anything?”

While innocent enough, these questions get for far more difficult when you consider that in the patient’s mind sonographic experts should be able to “see everything.” For Hershkovitz scanpatients, ultrasound has become a routine obstetrical practice that experts should be able to use to perform every type of diagnosis at the earliest gestational age—with 100% accuracy. This perception, however, means that developmental anomalies or disappearing findings are very confusing for the patient and her spouse.
An example of such a confusing sonographic condition is prenatal diagnosis of congenital lung lesions. One of the rarest lung lesions is Congenital Lobar Emphysema (CLE). This is a rare developmental anomaly of the lower respiratory tract characterized by hyperinflation of one or more of the pulmonary lobes and subsequent air trapping. The main fetal sonographic features of CLE include a bright echogenic lung with or without cystic or mixed cystic lesions (see image) without abnormal blood flow. A mediastinal shift, polyhydramnios, and fetal hydrops can also be observed and are predictors of severe respiratory distress or mortality.

CLE can decrease in size during pregnancy and even disappear on prenatal sonography or become apparent at postnatal evaluation, even though it is not observed during pregnancy. This can, obviously, become confusing for the patient. The main differential diagnosis of CLE is with congenital cystic adenomatoid malformation, pulmonary sequestration, and congenital high airway obstruction syndrome (CHAOS).

Once such a diagnosis is suspected, the patient is usually invited to a multidisciplinary meeting with the pediatric pulmonologypulmonologist, geneticist, and surgeon. Evaluation of the fetus is usually performed every 2 weeks and sometimes prenatal MRI is also suggested.

In many cases, depending on the size of the CLE and whether the fetus is hydropic by the time the fetus is delivered, the neonate is not in respiratory distress immediately after birth. However, sometimes CLE can lead to neonatal respiratory distress, and these neonates need to undergo surgical resection of the affected lobe. In the past, this has meant open thoracotomy, although recent advances in minimally invasive thoracoscopic surgery have resulted in decreased morbidity associated with resection of these lesions.

While innocent enough, the question, “Should I be worried about anything?” can get confusing and difficult when dealing with conditions like CLE. How would you handle it?

In a case like this how would you answer the question, “Should I be worried about anything?” How and when do you provide counseling to patients? Comment below or let us know on Twitter: @AIUM_Ultrasound.

Professor Reli Hershkovitz is the Head of the Ultrasound Unit at the Department of Obstetrics and Gynecology of the Soroka University Medical, located in Beer Sheva in the Southern part of Israel. This medical center serves nearly 2 million people, with an average of 16,000 deliveries a year.

Leave a Reply

Please log in using one of these methods to post your comment:

WordPress.com Logo

You are commenting using your WordPress.com account. Log Out / Change )

Twitter picture

You are commenting using your Twitter account. Log Out / Change )

Facebook photo

You are commenting using your Facebook account. Log Out / Change )

Google+ photo

You are commenting using your Google+ account. Log Out / Change )

Connecting to %s