SONOWORLD : Fetal bronchopulmonary sequestration

Username

Password

182,063 Registered Members as of 08/02/2020.

JavaScript must be enabled in order for you to use Sonoworld.com in standard view. However, it seems JavaScript is either disabled or not supported by your browser. To use standard view, enable JavaScript by changing your browser options, then try again.

Obstetrics » Obstetrics 2nd And 3rd Trimester

Fetal bronchopulmonary sequestration with cystic adenomatoid malformation

« Back to Listing

Author(s) :

Wesley Lee, MD

Presentation This is a 30 year old female who presented to the ultrasound unit at a gestational age of 18.5 menstrual weeks with diamniotic, dichorionic twins. Her past history was remarkable for a prior fetus with Turner's syndrome. The initial study indication was for fetal growth evaluation and the anatomic survey for both twins was unremarkable. She had a repeat scan for growth evaluation at 25.3 menstrual weeks. At that time, one of the twins demonstrated bilateral pleural effusions (Figures 1 and 2). The right lung appeared to be replaced by an echogenic complex mass that measured 4.2 x 4.4 x 2.5 cm. Several fluid-filled cysts (about 2-3 mm size) were present throughout the mass. Doppler ultrasonography did not suggest unusual vascularity.

Caption: Fetal chest - sagittal view

Description: Echogenic lung mass (arrow) is surrounded by pleural effusion.

Caption: Fetal Chest - axial View

Description: Echogenic lung mass is surrounded by bilateral pleural effusions.

Caption: Postnatal Radiograph

Description: Lung mass (dark area) compresses the fetal heart towards the left side.

Caption: Autopsy specimen

Description: Cross section demonstrates macrocysts that suggest cystic adenomatoid malformation. Note the aberrant blood supply to the aorta (arrow).

Caption: Pathology specimen - heart and lungs

Description: The light tan tissue is the extralobar bronchopulmonary sequestration with aberrant arterial blood supply(arrow).

Differential Diagnosis The differential diagnosis for this chest mass includes cystic adenomatoid malformation, pulmonary sequestration. bronchogenic or enteric cyst, bronchial atresia, mediastinal cystic hygroma, and neuroblastoma (1).

Final Diagnosis The final diagnosis was a bronchopulmonary sequestration with areas containing cystic adenomatoid malformation.

Discussion The most common etiologies for a fetal chest mass with hydrops is cystic adenomatoid malformation, pulmonary sequestration, and congenital diaphragmatic hernia. King and co-workers (1) presented a nice review of echogenic lung lesions by prenatal sonography. The prenatal diagnosis was correct in 13 of 17 fetuses with echogenic lung masses. Cystic adenomatoid malformation has been traditionally divided into three different types based on the presence of cysts (2). Type I consists mainly of large cysts, Type II is mixed, and Type III involves the presence of microcysts. In our case, the presence of cystic structures within this mass strongly suggested a cystic adenomatoid malformation. This abnormality can also be confused with bronchopulmonary sequestration where a part of the lung has become disconnected with the bronchial tree. These lesions can occur within (intralobar) or outside (extralobar) the main lung parenchyma. Congenital anomalies, such as diaphragmatic hernia, cardiac defects, and gastric communications, are more commonly found (15-60%) with extralobar sequestration (3). In the current case, we were unable to document an aberrant vessel by color Doppler sonography. An example of a systemic vascular supply is demonstrated in another fetus with pulmonary sequestration (Figure 6). Cystic adenomatoid malformation and bronchopulmonary sequestration are traditionally considered to have distinctive embryology, histologic characteristics, and natural history. However, Cass and co-workers (4) have described six fetuses who were diagnosed with fetal chest masses between 19-30 menstrual weeks. All six were thought to have the sonographic appearance of cystic adenomatoid malformation because an aberrant systemic vessel was not identified. After delivery, all 6 lesions were confirmed to have an aberrant systemic vessel directly from the aorta. The surgical findings indicated an bronchopulmonary sequestration of the extralobar variety in 1 case and intralobar type in the others. Nonetheless, all 6 lesions had histologic evidence of cystic adenomatoid malformation. These "hybrid" cases suggest a similar embryological origin for both types of pulmonary lesions and underscores the difficulty with trying to differentiate between the two lesions by prenatal sonography.

Case References 1. King SJ, Pilling DW, Walkinshaw S. Fetal echogenic lung lesions: prenatal ultrasound diagnosis and outcome. Pediatr Radiol 1995;25:208-10.
2. Stocker JT, Madewell JE, Drake RM. Congenital cystic adenomatoid malformation of the lung. Hum Pathol 1977; 8:155-71.
3. Savic B, Birtel FJ, Tholen W, Funke HD, Knoche R. Lung sequestration: report of seven cases and review of 540 published cases. Thorax 1979; 34:96-101.
4. Cass DL, Crombleholme TM, Howell LJ, Stafford PW, Ruchelli ED, Adzick NS. Cystic lung lesions with systemic arterial blood supply: a hybrid of congenital cystic adenomatoid malformation and bronchopulmonary sequestration. J Pediatr Surg 1997;32:986-90.

Follow Up The patient developed preterm labor and delivered twins. The fetus with the lung mass and pleural effusions expired.

Other Cases in This Category

Obstetrics >> Obstetrics 2nd And 3rd Trimester

Placenta previa
Because the patient reported intermittent vaginal bleeding, a sonogram was performed in the second trimester of this pregnancy.

Succenturiate lobe of placenta
This sonogram was performed in the second trimester for the purpose of determining gestational age and screening for anomalies.

Idiopathic infantile arterial calcification
This is a 35 year old G4P2 patient whose previous child was stillborn due to non-immune hydrops. During the current pregnancy, she had an ultrasound at 20 weeks, showing a normal fetal survey. ...

Autosomal recessive polycystic kidney disease
A 29 year old woman, G1, P0, A0 presented for antenatal ultrasound. The gestational age of the fetus at the time of examination was 30w 3d.

Antenatal cystic hygroma
A 25 year old woman referred for gestational age assessment.

Gastroschisis
This is a routine second trimester antenatal ultrasound that was performed at 14 weeks gestational age.

Prenatal detection of cystic adenomatoid malformation of the lung
This is a routine antenatal scan at 22 weeks of gestation.

Thanatophoric dysplasia, third trimester
This 18 year old woman, primigravida, presented for a routine antenatal scan at 31 weeks of gestation. She has no significant past medical history.

Bilateral cleft lip and cleft palate
A woman presented for a routine second trimester antenatal scan.

Anencephaly
A 21 year old woman, primigravida, presented for routine antenatal ultrasound.

Umbilical vein varix
A 26 year old primigravida presented for a routine third trimester ultrasound. Fetal gestational age corresponded to 34 weeks.

Intracranial arteriovenous malformation with cardiomegaly
This is a routine asymptomatic third trimester ultrasound.

Pregnancy in a uterus with synechia
These are serial scans of a 32-year-old pregnant woman with a history of prior C-section.

Cord around the neck of the fetus
A young woman presented for routine obstetric scan.

Cystic hygroma with hydrops
This is the fetal scan in a woman who presented for a routine second trimester antenatal ultrasound examination at 20 weeks of gestation.

Beckwith Wiedemann syndrome
This is the ultrasound in a 32-year-old gravida at 34 weeks of gestation. She had an ultrasound performed outside of our hospital which showed macrosomia.

Fetal hydrops
A woman presented for routine second trimester antenatal ultrasound

Osteogenesis Imperfecta with Agenesis of Corpus Callosum
A 23 years old primagravida presented for routine sonographic scan with no significant complaints. According to the patient, menstrual age was approximately 30 weeks.

Fetal Spongiform Myocardium
A 32 year old gravida 1, para 1 female came for her 1st ultrasound at 28 weeks of gestation. The fetus had a normal anatomic survey except for the appearance of the heart.

Split hand and foot syndrome

39 week gestation with fetal gallstones
A patient came in for a biophysical profile. She had a failed non-stress test. She is 29 years old and is morbidly obese. This is her first pregnancy. Other than her obesity she has no other ...

multicystic Dysplastic Kidney
This was a 2nd trimester scan to corfirm dates. There were no risk factors. The mother was 26 years old with a previous normal pregnancy.

Succenturiate Lobe of Placenta
Primigravida with 20 wks gestation came for a routine scan.

Vein of Galen Aneurysm with Hydrocephalus
Routine third trimester scan.