Radiological Category: Pediatrics
Principal Modality (1): CT
Principal Modality (2): CXR
Case Report
Submitted by:
John Knight, MS4
Faculty reviewer:
Sandra Oldham, MD
Date accepted:
August 27, 2014
Case History
•
The patient is a two-week old female born at 34 weeks 6 days gestation who
presents for follow-up of a cystic lesion noted in the right lung on prenatal
ultrasound.
Birth History:
•
•
•
•
•
Delivered 34+6 weeks via primary C/S secondary to pregnancy-induced
hypertension
APGARs 8 & 9
Twin Gestation
Pregnancy also complicated by: well-controlled gestational Diabetes Mellitus,
twin to twin transfusion syndrome
Status-post laser ablation of placental anastamosis at 21 weeks
Case History
•
•
•
•
•
Birth itself uncomplicated
Patient and twin admitted to NICU due to prematurity
Hemodynamically stable
No labored breathing, oxygen desaturation, or clinical signs of hypoxia
Patient spent 16 days in the NICU prior to discharge to home
Chest X-Ray, Day of Life 1
Chest CT with Contrast, Day of Life 14
Chest CT with Contrast, Day of Life 14
Chest CT with Contrast, Day of Life 14
Chest CT with Contrast, Day of Life 14
Chest CT with Contrast, Day of Life 14
Chest CT with Contrast, Day of Life 14
Test Your Diagnosis
Which one of the following is your choice for the appropriate diagnosis?
• Intrapulmonary bronchogenic cysts
• Pleuropulmonary Blastoma
• Congenital Pulmonary Airway Malformation, Type I
• Congenital Pulmonary Airway Malformation, Type II
• Congenital Lobar Overinflation
Findings and Differentials
Findings:
Multiple hyperlucent foci within the right upper lobe, with the largest measuring 1.3 x
1.1 x 1.2 cm.
No cystic lesions are noted in the right middle and lower lobes, or the left lung.
The heart size, mediastinal structures, and abdomen are within normal limits
Differential:
• CPAM Type I
• CPAM Type II
• Pleuropulmonary Blastoma
Discussion: Differential Diagnosis
•CPAM Type I
– Characterized by large intercommunicating airfilled cysts (>2 cm) surrounded by multiple smaller
cysts
– Lined by bronchial-type epithelium without
cartilage
– Communicate with surrounding lung; may see airfluid levels within cysts
– Most common type of CPAM (60 – 70% of cases)
– Diagnosis often made with prenatal ultrasound
•CPAM Type II
– Characterized by mixture of solid tissue and small
air-filled cysts (<2 cm)
– Consistent with the small, hyperlucent foci noted
on the patient’s CT
– 15 – 20% of all cases
– 60% of cases associated with other congenital
anomalies
Discussion: Differential Diagnosis
• Pleuropulmonary Blastoma
– Relatively rare malignancy of the lung
– Typically diagnosed in first 4 years of life
– Often radiographically indistinguishable from
CPAM Type IV and Type I
– Histology key to differentiating from CPAM
– Pathology in our patient showed pulmonary
parenchyma with cysts lined by ciliated epithelium
– Inconsistent with PPB, which is composed of
malignant mesenchymal cells
Diagnosis
Congenital Pulmonary Airway Malformation Type II
Congenital Pulmonary Airway Malformation
• History
– First described in 1949 by Ch’in and Tang
– Classification system developed in 1977 by Stocker, which he expanded in 2001
– Also known as Congenital Cystic Adenomatoid Malformation prior to these changes
• Epidemiology
– 1 in 8,300 – 35,000 live births
• Pathophysiology
– Failure of normal broncho-alveolar development
– Possibly triggered by in utero airway obstruction, atresia
– Results in hamartomatous proliferation of terminal respiratory units in a gland-like,
“adenomatoid” pattern
– Lack of normal alveolar development in affected region
–Five different patterns seen clinically
Congenital Pulmonary Airway Malformation
• Type 0
– < 3% cases
– No cysts, or very small ones
– Usually involves entire lung
– Incompatible with life
• Type I
• Type II
• Type III
– Solid adenomatoid malformation with minor
cystic components
– 5 – 10% of cases
• Type IV
– Large cysts up to 10 cm
– 10 – 15% of cases
– Difficult to distinguish from Type I CPAM, PPB
– Increasing evidence that all or most Type IV
CPAM cases might be PPB
Imaging Options
• Prenatal
– Ultrasound: microcystic lesions appearing echogenic and solid. Relatively sensitive.
– MRI: equally sensitive and more specific
– Lesions will often stabilize or regress prior to birth
• Asymptomatic neonates
– If prenatal diagnosis made, patient should have CXR in neonatal period
– CXR may be normal in up to 40% of cases
– Follow-up CT chest at 4 – 6 weeks of age to confirm and evaluate lesion
– CT more sensitive than CXR
• Postnatal Diagnosis
– Typically made with CXR
– CT also useful
Congenital Pulmonary Airway Malformation
• Clinical course
– Typically diagnosed on prenatal ultrasound
– Presentation after birth variable; depends on size of the lesion
– Large lesions may cause pulmonary hypoplasia and progressive pulmonary distress in
the neonatal period resulting in death without intervention
– Small lesions, if not caught on imaging, may cause no symptoms and go undiscovered
into adulthood
• Treatment Options
– Resection is the treatment of choice due to risk of infection, malignancy
– Surveillance
– Fetal Surgery
Discussion
Discussion
Discussion
Chest X-Ray, Post-surgical
References
Kao SW, Zuppan CW, Young LW. AIRP best cases in radiologic-pathologic correlation: type 2
congenital cystic adenomatoid malformation (type 2 congenital pulmonary airway malformation).
Radiographics 2011; 31(3): 743-748.
Priest JR, Williams GM, Hill DA, Dehner LP, Jaffé A. Pulmonary cysts in early childhood and the risk
of malignancy. Pediatr Pulmonol. 2009;44(1):14-30.
Oermann, Christopher M. Congenital pulmonary airway (cystic adenomatoid) malformation. In:
UpToDate, Post, TW (Ed), UpToDate, Waltham, MA, 2014.
Weerakkody, Yuranga and Frank Gaillard. Congenital Pulmonary Airway Malformation.
Radiopaedia. Retrieved August 23, 2014. <http://radiopaedia.org/articles/congenital-pulmonaryairway-malformation>.
Lung: Pleuropulmonary Blastoma. Atlas of Genetics and Cytogenetics in Oncology and
Haematology. Retrieved August 25, 2014.
<http://atlasgeneticsoncology.org/Tumors/PleuropulmblastomID6040.html>.
Mak, S., B. Annan, S. P. G. Padley, A. G. Nicholson. Congenital Pulmonary Airways Malformation: an
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