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Case 384

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A 5-month-old male infant is brought to the emergency department for fever of 100,4 °F (38 °C) at home, productive cough, worsening of the respiratory pattern (with signs of strong respiratory effort) and a micropapular rash on the face and thorax for 12 days. Nasal saline lavage and a short-acting β-agonist prescribed, without success. Chest X-ray (image 3) and ultrasound performed in the gestation period, raised the suspicion of lung malformation, confirmed by fetal MRI (image 1). The baby was born at full term and was appropriate for gestational age, with a birth weight of 3.730g. There is a history of respiratory distress, requiring supplemental oxygen and CPAP within the first hours of life. The physician ordered a thorax CT (Image 2).

Based on the images and the clinical scenario, which of the following is the most likely diagnosis?

a) Bronchopulmonary sequestration

25%

b) Congenital pulmonary airway malformation

25%

c) Bronchial atresia

25%

d) Bronchogenic cyst

25%
   

Image analysis

Image 1 – Fetal magnetic resonance (MRI), T2-weighted, showing uterus with an only fetus on longitudinal position, cephalic presentation, back turned right. (A) coronal view; (B) sagittal view. On fetal morphology, there is a cystic mass (red arrow) with lobulated contours, imprecise limits, and a few internal septations of well-defined limits, located on the right perihilar region. Its biggest component measures 20 x 18 mm and is hyperintense, which suggests cystic etiology. Fetal MRI can bring additional information to the gestational US in about 60% of the cases.

Image 2 – High-resolution computed tomography (HRCT) of the thorax, axial view, lung window. Loculated cystic mass (yellow arrow) with lobulated contours, thin walls, located on the posterior segment of the upper right lung, perihilar position, measuring 23 x 19 mm on the axial view.

Image 3 – Chest radiographs, anteroposterior (A) and lateral (B) view. Cystic mass (green arrow) on the right perihilar region with well-defined contours and homogeneous content.

Highlights 

−      Congenital cystic adenomatoid malformation (CCAM)  is the most common congenital lung lesion. It is the result of abnormal proliferation of bronchial structures;

−      The diagnosis is made prenatally upon obstetric ultrasound and confirmed by fetal MRI;

−      The differential diagnosis must include: bronchopulmonary sequestration, bronchial atresia and bronchogenic cyst;

−      CCAM is classified accordingly to the location of the malformation: type 0 (tracheobronchial), type 1 (bronchial or bronchiolar), type 2 (bronchiolar), type 3 (bronchiolar or alveolar), and type 4 (distal acinar);

−      CCAM is treated by surgical resection in the neonatal period if there is significant respiratory distress. If the patient is asymptomatic, surgical intervention can be done until the 10th month of life to avoid complications (e.g. infection and malignant transformation).

References 

−     AK Sfakianaki, JA Copel. Congenital Cystic Lesions of the Lung: Congenital Cystic Adenomatoid Malformation and Bronchopulmonary Sequestration. Rev Obstet Gynecol. 2012; 5(2):85-93. doi: 10.3909/riog0183a

−     DR Biyyam, T Chapman, MR Ferguson, G Deutsch, MK Dighe. Congenital Lung Abnormalities: Embryologic Features, Prenatal Diagnosis, and Postnatal Radiologic-Pathologic Correlation. RadioGraphics 2010; 30:1721-1738. doi: 10.1148/rg.306105508

−     S. Kotecha, A. Barbato, A. Bush, F. Claus, M. Davenport, C. Delacourt, J. Deprest, E. Eber, B. Frenckner, A. Greenough, AG Nicholson, JL Antón-Pacheco, F. Midulla. Antenatal and Postnatal Management of Congenital Cystic Adenomatoid Malformation. Paediatric Respiratory Reviews. 13(2012);162-171. doi: 10.1016/j.prrv.2012.01.002

−     CM Oermann, JA Garcia-Prats, G Redding, AG Hoppin. Congenital Pulmonary Airway (Cystic Adenomatoid) Malformation. UpToDate (accessed on 30/08/2019).

−     CA Rezende, MIR Vieira, VO Ganem, TIM Mastachtakow, SF Assis, IMP Teodoro, GB Lasmar, FMB Filho, JLSS Zanini, LMLBF Lasmar. Abordagem da Malformação Pulmonar Congênita de Vias Aéreas: Atualização Através de um Caso. Rev Med Minas Gerais 2018;28 (Supl.6). doi: https://dx.doi.org/10.5935/2238-3182.20180105 

Author

Felipe Eduardo Fagundes Lopes, 5th year medical student at Federal University of Minas Gerais School of Medicine (UFMG).

Mail: felipelopes.med[at]outlook.com

Supervisor

Dr. Marconi Augusto Aguiar Reis, professor at the Department of Pediatrics of UFMG.

Mail: marconiaugusto[at]hotmail.com 

Dr. Diego Corrêa Andrade, radiologist at Hospital das Clínicas of UFMG.

Reviewers

Lara Hemerly De Mori, Mateus Nardelli, Letícia de Melo Elias, Victoria Costa, Ana Luísa Melgaço Almeida, Professors José Nelson Mendes Vieira and Viviane Santuari Parisotto Marino.

Test question

(Brazilian Society of Pediatrics) The presence of some cysts of varied dimensions on the thorax CT, the bigger one measuring 2cm, in the right lower lung of a newborn with a few hours of life is compatible with the diagnosis of:

a) Bronchial atresia

25%

b) Bronchogenic cyst

25%

c) Right sided congenital diaphragmatic hernia

25%

d) Cystic adenomatoid malformation

25%

e)

25%
   

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