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Case 267

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21-year old female patient, living in Moeda-MG, is referred to HC-UFMG’s Centro de Medicina FETAL (CEMEFE, Center of Fetal Medicine) due to rotine morfology obstetric ultrasound’s finding of lung abnormalities, without further complaints. This is her first pregnancy and it was planned. Smoking, alcohol drinking, drug abuse and exposition to teratogens are absent. The child’s father is healthy as well and there is no consaguinity in the couple. There are no reports of malformations in the family. Prenatal serologies are negative.

After analysing the patient’s history and image findings, what is the most likely diagnosis?

a) Bronchopulmonary sequestration

25%

b) Congenital diaphragmatic hernia

25%

c) Congenital cystic adenomatoid malformation

25%

d) Bronchogenic cyst

25%
   

IMAGE ANALYSIS

Image 1 - Analysis: Obstetric morphology ultrasound (US), transversal plane at thoracic level, in a 22 week and 5 days old fetus showing enlarged lung with multiple cysts (red), which dislodge the heart towards left.

Imagem 2 - Analysis: Obstetric morphology ultrasound (US), transversal plane at thoracic level, performed at HC-UFMG’s Centro de Medicina Fetal (CEMEFE), in a 27 week and 6 days old fetus showing multiple cysts in chest topography (red), which suggest type II congenital cystic adenomatoid malformation and polyhydramnios.

HIGHLIGHTS

- Prenatal diagnosis of congenital cystic adenomatoid malformation (CCAM) is based on ultrasonography, which shows macrocystic lesions and/or solid mass on the fetus’ thorax;

- Advantages of early (prenatal) diagnosis of CCAM include the identification of potential candidates to prenatal intervention and better planning of the birth;

- The natural history of CCAM varies from complete intrauterine regression to life-threatening hydrops fetalis;

- CCAM’s prognosis depends on the presence of hydrops fetalis, which has high mortality rates and is the major indication of prenatal intervention;

- CCAM’s postnatal surgical treatment depends on the fetus’ prenatal status and on the presence of symptoms.

REFERENCES

- Sfakianaki AK, Copel JA. Congenital Cystic Lesions of the Lung: Congenital Cystic Adenomatoid Malformation and Bronchopulmonary Sequestration. Reviews in Obstetrics and Gynecology. 2012;5(2):85-93.

- David M, Lamas-Pinheiro R, Henriques-Coelho T. Prenatal and postnatal management of congenital pulmonary airway malformation. Neonatology 2016;110:101–115.

- DynaMed [Internet]. Ipswich (MA): EBSCO Information Services. Record No. 116291, Pulmonary sequestration; [updated 2016 Nov 18, cited 23/02/2017]. Available at: http://search.ebscohost.com/login.aspx?direct=true&db=dnh&AN=116291&site=dynamed-live&scope=site.

- DynaMed [Internet]. Ipswich (MA): EBSCO Information Services. Record No. 115523, Bronchogenic cyst; [updated 2016 Aug 31, cited 23/02/2017]. Available at: http://search.ebscohost.com/login.aspx?direct=true&db=dnh&AN=115523&site=dynamed-live&scope=site. Registration and login required.

- Stone A. Cystic Adenomatoid Malformation. Bye M, editor. Medscape; 2015. Available at: http://emedicine.medscape.com/article/1001488-overview.

- Egloff A, Bulas D. Prenatal diagnosis and management of congenital pulmonary airway malformation. UpToDate; 2017.

AUTHOR

Raíra Cesar e Cezar, 6th year medical student at Universidade Federal de Minas Gerais

E-mail: rairacezar[at]gmail.com

SUPERVISOR

Dalila Magioni MD, obstetrician, fetal medicine specialist by Hospital das Clínicas da UFMG

E-mail: dalilamagioni[at]hotmail.com

REVIEWERS

Fernando Bottega, Luísa Bernardino, Juliana Albano and professor Viviane Parisotto

TRANSLATED BY

Thiago de Oliveira Heringer, 5th year medical student at Universidade Federal de Minas Gerais

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