Document Detail

Congenital cystic adenomatoid malformation: is there a need for pregnancy termination?
Jump to Full Text
MedLine Citation:
PMID:  22474453     Owner:  NLM     Status:  PubMed-not-MEDLINE    
Abstract/OtherAbstract:
Aim. Congenital cystic adenomatoid lung malformation is a rare unilateral dysplasia of the lung. Three pathologic types are described in the literature: type I with cysts >2 cm, type II with cysts <1 cm, and type III with microcysts. The aim of this paper is to present a case of a fetus with congenital cystic adenomatoid lung malformation and discuss the necessity for pregnancy termination according to its prognosis and future mortality. Case. A 36-year-old pregnant woman (para: 1, gravida: 1) presented in our department for anatomy ultrasound screening at 20 + 1 weeks of gestation. The ultrasound detected a cystic adenomatoid right lung malformation measuring 1.45 × 1.67 cm which caused mediastinal shift of the heart and the lung to the left side. Other findings were cysts of the choroid plexus and echogenic intracardiac foci. The parents after genetic counseling decided pregnancy termination. The pregnant received cabergoline for ablactation. Conclusion. Congenital cystic adenomatoid lung malformation has different prognosis according to the type (69% in type I, 0% in types II and III). Fetal hydrops, cardiac and skeletal anomalies, Potter's syndrome, and gastrointestinal atresia are common cofindings. Genetic counseling is necessary, and pregnancy termination is proposed to the cases with poor prognosis.
Authors:
C Iavazzo; M Eleftheriades; A M Bacanu; D Hassiakos; D Botsis
Related Documents :
1752603 - Effects of clomiphene citrate on serum hormone levels and endometrial thickness in an i...
7580023 - Involvement of ovarian factors magnified by pharmacological induction of multiple folli...
3241293 - Intrauterine death of a single fetus in twin pregnancies.
Publication Detail:
Type:  Journal Article     Date:  2012-03-14
Journal Detail:
Title:  Case reports in medicine     Volume:  2012     ISSN:  1687-9635     ISO Abbreviation:  Case Rep Med     Publication Date:  2012  
Date Detail:
Created Date:  2012-04-04     Completed Date:  2012-08-23     Revised Date:  2013-02-21    
Medline Journal Info:
Nlm Unique ID:  101512910     Medline TA:  Case Rep Med     Country:  United States    
Other Details:
Languages:  eng     Pagination:  397508     Citation Subset:  -    
Affiliation:
2nd Department of Obstetrics and Gynecology, University of Athens, Aretaieion Hospital, Athens, Greece.
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Descriptor/Qualifier:

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

Full Text
Journal Information
Journal ID (nlm-ta): Case Report Med
Journal ID (iso-abbrev): Case Report Med
Journal ID (publisher-id): CRIM
ISSN: 1687-9627
ISSN: 1687-9635
Publisher: Hindawi Publishing Corporation
Article Information
Download PDF
Copyright © 2012 C. Iavazzo et al.
open-access:
Received Day: 4 Month: 4 Year: 2011
Revision Received Day: 3 Month: 1 Year: 2012
Accepted Day: 6 Month: 1 Year: 2012
Print publication date: Year: 2012
Electronic publication date: Day: 14 Month: 3 Year: 2012
Volume: 2012E-location ID: 397508
ID: 3312219
PubMed Id: 22474453
DOI: 10.1155/2012/397508

Congenital Cystic Adenomatoid Malformation: Is There a Need for Pregnancy Termination?
C. IavazzoI1*
M. EleftheriadesI1
A. M. BacanuI1
D. HassiakosI1
D. BotsisI1
2nd Department of Obstetrics and Gynecology, University of Athens, Aretaieion Hospital, Athens, Greece
Correspondence: *C. Iavazzo: christosiavazzo@hotmail.com
[other] Academic Editor: André Mégarbané

1. Introduction

Congenital cystic adenomatoid lung malformation (CCAM) is a rare, usually, unilateral dysplasia of the lung. The entity was first reported in 1787 with the description of the absence of the lungs [1]. It results from an abnormal maturation of the bronchopulmonary tree, especially an overgrowth of the terminal bronchioles [2]. The etiology of the entity remains unknown. Hamartomatous change in the terminal bronchioles or an arrest in their embryological development between 7 and 15 weeks of gestation is suspected [3]. Decreased apoptosis also plays a role [3]. The related genes in the pathogenesis are HOXB5, Fgf7, and PDGFB [4, 5].

The incidence of CCAM ranges from 1 : 35,000 up to 1 : 25,000 [6]. According to Rosado-de-Christenson and Stocker, three pathologic types are described: type I: cysts diameter >2 cm, type II: cysts diameter <1 cm, and type III: microcysts [7]. In parallel, Adzick et al. differentiated CCAM to macrocystic and microcystic types [8]. The aim of this paper is to present a case of a fetus with congenital cystic adenomatoid lung malformation and discuss the need for pregnancy termination in the cases with poor prognosis.


2. Case

A 36-year-old pregnant woman (para: 1, gravida: 1) presented in our department for anatomy ultrasound screening at 20 + 1 weeks of gestation. No problems are mentioned in her medical or obstetric history. Her current pregnancy was uncomplicated. The ultrasound detected a cystic adenomatoid right lung malformation measuring 1.45 × 1.67 cm which caused mediastinal shift of the heart and the lung to the left side (Figure 1). Other findings were cysts of the choroid plexus, as well as echogenic intracardiac foci. No polyhydramnios or hydrops fetalis were found. The parents after genetic counseling (chromosome analysis) decided pregnancy termination, although they were informed regarding the increased possibility of excellent prognosis. The pregnant received cabergoline for ablactation.


3. Discussion

CCAM is a rather rare entity which is usually identified in the anatomy ultrasound scanning of the second trimester. Prenatal diagnosis of CCAM has increased due to the advances in antenatal sonography. The positive predictive value and the sensitivity of 2D sonographic evaluation range between 45–57% and 70–81%, respectively [9]. Moreover, the 3D sonographic evaluation allows all cystic lesions within the studied volume to be seen as opaque or echogenic [10]. MRI is another useful tool for imaging CCAM [11]. In our case, the diagnosis was performed by using 2D ultrasound.

CCAM is usually unilateral [12]. The macrocystic type has large cysts of variable size with thin echogenic areas, whereas the microcystic type has a defined echogenic area with cysts <5 mm in diameter [12]. In our case, a left unilateral anechoic cyst <2 cm was identified. Usually, no association with aneuploidy is found, and for this reason karyotyping is not indicated. Although, in our case, the parents opted for amniocentesis, the karyotype was normal. Some patients especially of type I CCAM demonstrate compression of the heart and the vena cava leading to mediastinal shift, polyhydramnios, and fetal hydrops [13]. Moreover, skeletal anomalies, Potter's syndrome, and gastrointestinal atresia are other common findings [14].

The differential diagnosis includes bronchopulmonary sequestration, bronchogenic cyst, neurenteric cysts, congenital lobar emphysema, and diaphragmatic hernia [11, 15].

The prognosis is usually optimal. Poor prognostic findings are hydrops fetalis, ascites, polyhydramnios, bilateral lung involvement, and a final lung-to-thorax transverse area ratio of less than 0.25 [16, 17]. It should also be mentioned that congenital cystic adenomatoid lung malformation has different prognosis according to the type (69% in type I, 0% in types II and III). The cystic adenomatoid malformation volume ratio—which is defined as the estimated volume of the CCAM divided by head circumference—is used to predict prognosis. A ratio >1.6, leads to a poorer prognosis [18]. In our case, the ratio was less than 1.6 and for this reason termination of pregnancy was not proposed, although the parents opted differently.

Genetic counseling is necessary and pregnancy termination is frequently wrongly proposed due to “poor prognosis.” The good prognosis could be explained by the fact that the cyst decreases in size in utero in the majority of cases [19]. The peak CCAM growth is expected to occur by the 28th gestational week and regression usually occurs in 20% of cases after 29 weeks [19]. Women should be advised to give birth in tertiary centres with a pediatric unit. Antenatally diagnosed CCAM has an excellent prognosis when signs of severe fetal distress are absent. Nakata et al. showed that, in cases with hydrops fetalis, fetal intervention is necessary [6]. Different treatment techniques are proposed in heavier cases such as shunting, laser, and postpartum surgery. Fetal intervention such as cyst-amniotic shunting or tumor resection before 32 weeks of gestation could also be used [20]. Another in utero technique is percutaneous laser ablation [21]. Tran et al. after a retrospective study of 34 cases with CCAM proposed that asymptomatic neonates should have a postnatal CT even if the CCAM appears to have resolved or decreased on antenatal ultrasound [22]. However, in asymptomatic patients elective operation is proposed in 3–6 months of life to avoid respiratory infections, pneumothorax, or even malignancy [23], while a partial lung resection using an axillary skin crease incision is also proposed to obtain a good postoperative quality of life [6]. Such interventions are life saving. Although rare entities, the most common relevant malignancies are bronchoalveolar cancer and rhabdomyosarcoma [24, 25]. Usually, a lobectomy in such cases is performed, and then the mortality rates range from 9 to 49% [26].

In our case, the pregnant woman was advised not to terminate the pregnancy; however, the couple decided to do so because of the increased fear of developing a fetus with hydrops and comorbidity.


References
1. Harmath A,Csaba A,Hauzman E,Hajdú J,Pete B,Papp Z. Congenital lung malformations in the second trimester: prenatal ultrasound diagnosis and pathologic findingsJournal of Clinical UltrasoundYear: 200735525025517373682
2. Argeitis J,Botsis D,Kairi-Vassilatou E,Hasiakos D,Papakonstantinou K,Kondi-Pafiti A. Congenital cystic adenomatoid lung malformation: report of two cases and literature reviewClinical and Experimental Obstetrics and GynecologyYear: 20083517680
3. Luján M,Bosque M,Mirapeix RM,Marco MT,Asensio O,Domingo C. Late-onset congenital cystic adenomatoid malformation of the lung: embryology, clinical symptomatology, diagnostic procedures, therapeutic approach and clinical follow-upRespirationYear: 200269214815411961429
4. Volpe MA,Pham L,Lessin M,et al. Expression of Hoxb-5 during human lung development and in congenital lung malformationsBirth Defects Research AYear: 2003678550556
5. Jancelewicz T,Nobuhara K,Hawgood S. Laser microdissection allows detection of abnormal gene expression in cystic adenomatoid malformation of the lungJournal of Pediatric SurgeryYear: 20084361044105118558180
6. Nagata K,Masumoto K,Tesiba R,et al. Outcome and treatment in an antenatally diagnosed congenital cystic adenomatoid malformation of the lungPediatric Surgery InternationalYear: 200925975375719662430
7. Rosado-de-Christenson ML,Stocker JT. Congenital cystic adenomatoid malformationRadiographicsYear: 19911158658861947321
8. Adzick NS,Harrison MR,Crombleholme TM,Flake AW,Howell LJ. Fetal lung lesions: management and outcomeAmerican Journal of Obstetrics and GynecologyYear: 199817948848899790364
9. Gornall AS,Budd JL,Draper ES,Konje JC,Kurinczuk JJ. Congenital cystic adenomatoid malformation: accuracy of prenatal diagnosis, prevalence and outcome in a general populationPrenatal DiagnosisYear: 20032312997100214663837
10. Hata N,Wada T,Chiba T,Tsutsumi Y,Okada Y,Dohi T. Three-dimensional volume rendering of fetal MR images for the diagnosis of congenital cystic adenomatoid malformationAcademic RadiologyYear: 200310330931212643560
11. Epelman M,Kreiger PA,Servaes S,Victoria T,Hellinger JC. Current imaging of prenatally diagnosed congenital lung lesionsSeminars in Ultrasound, CT and MRIYear: 2010312141157
12. Margi M,Kaddouri N,Abdelhak M,Barahioui M,Benhmamouch MN. Cystic adenomatoid malformations of the lung: a retrospective study of 12 casesRevue de Pneumologie CliniqueYear: 200965314314619524802
13. Chen WS,Yeh GP,Tsai HD,Hsieh CT. Prenatal diagnosis of congenital cystic adenomatoid malformations: evolution and outcomeTaiwanese Journal of Obstetrics and GynecologyYear: 200948327828119797020
14. Orpen N,Goodman R,Bowker C,Lakhoo K. Intralobar pulmonary sequestration with congenital cystic adematous malformation and rhabdomyomatous dysplasiaPediatric Surgery InternationalYear: 200319861061114551713
15. Lecomte B,Hadden H,Coste K,et al. Hyperechoic congenital lung lesions in a non-selected population: from prenatal detection till perinatal managementPrenatal DiagnosisYear: 200929131222123019911412
16. Bunduki V,Ruano R,da Silva MM,et al. Prognostic factors associated with congenital cystic adenomatoid malformation of the lungPrenatal DiagnosisYear: 200020645946410861709
17. Usui N,Kamata S,Sawai T,et al. Outcome predictors for infants with cystic lung diseaseJournal of Pediatric SurgeryYear: 200439460360615065037
18. Matsushita M,Ishii K,Tamura M,et al. Perinatal magnetic resonance fetal lung volumetry and fetal lung-to-liver signal intensity ratio for predicting short outcome in isolated congenital diaphragmatic hernia and cystic adenomatoid malformation of the lungJournal of Obstetrics and Gynaecology ResearchYear: 200834216216718412776
19. Duncombe GJ,Dickinson JE,Kikiros CS. Prenatal diagnosis and management of congenital cystic adenomatoid malformation of the lungAmerican Journal of Obstetrics and GynecologyYear: 2002187495095412388984
20. Adzick NS,Harrison MR,Crombleholme TM,Flake AW,Howell LJ. Fetal lung lesions: management and outcomeAmerican Journal of Obstetrics and GynecologyYear: 199817948848899790364
21. Davenport M,Warne SA,Cacciaguerra S,Patel S,Greenough A,Nicolaides K. Current outcome of antenally diagnosed cystic lung diseaseJournal of Pediatric SurgeryYear: 200439454955615065026
22. Tran H,Fink MA,Crameri J,Cullinane F. Congenital cystic adenomatoid malformation: monitoring the antenatal and short-term neonatal outcomeAustralian and New Zealand Journal of Obstetrics and GynaecologyYear: 200848546246619032660
23. Dell’Agnola C,Tadini B,Mosca F,Colnaghi M,Wesley J. Advantages of prenatal diagnosis and early surgery for congenital cystic disease of the lungJournal of Perinatal MedicineYear: 19962466216319120745
24. Granata C,Gambini C,Balducci T,et al. Bronchioloalveolar carcinoma arising in congenital cystic adenomatoid malformation in a child: a case report and review on malignancies originating in congenital cystic adenomatoid malformationPediatric PulmonologyYear: 199825162669475333
25. Murphy JJ,Blair GK,Fraser GC,et al. Rhabdomyosarcoma arising within congenital pulmonary cysts: report of three casesJournal of Pediatric SurgeryYear: 19922710136413671403524
26. Duan M,Wang L,Cao Y,et al. Results of surgical treatment of congenital cystic lung diseaseThoracic and Cardiovascular SurgeonYear: 2005531616415692922

Article Categories:
  • Case Report


Previous Document:  Concurrent cemento-osseous dysplasia and osteogenic sarcoma: report of two cases.
Next Document:  Sprengel's Deformity Associated with Musculoskeletal Dysfunctions and Renal Anomalies: A Case Report...