Obstet Gynecol Sci.  2014 Jan;57(1):73-76.

An unusual prenatal manifestation of a huge congenital cystic adenomatoid malformation with favorable perinatal outcome

• Suk HJ
• Won HS
• Lee EJ
• Lee MY

Abstract

Congenital cystic adenomatoid malformation (CCAM) is a rare condition which is easily detectable by prenatal ultrasonography. Fetuses with large CCAMs associating with hydrops are predisposed to perinatal mortality, therefore prenatal intervention is required. While macrocystic CCAM is treated prenatally by thoracentesis or thoraco-amniotic shunt, microcystic or mixed CCAM is difficult to manage in the fetus. In these latter lesions, fetal lobectomy, sclerotherapy, or laser ablation was used to treat lesions directly. We present an unusual prenatal case of mixed CCAM associating with hydrops and marked ascites, which was conservatively managed with prenatal abdomino-amniotic shunting and successfully treated by postnatal surgery.

Keyword

Congenital; Cystic adenomatoid malformation of lung; Fetal therapies; Hydrops fetalis; Prenatal diagnosis

MeSH Terms

Ascites
Cystic Adenomatoid Malformation of Lung, Congenital*
Edema
Fetal Therapies
Fetus
Hydrops Fetalis
Laser Therapy
Perinatal Mortality
Prenatal Diagnosis
Sclerotherapy
Ultrasonography, Prenatal

Figure

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  Fig. 1 (A) Ultrasonography showing a hyperechoic mass of 70 mm with an enclosed cystic lesion of 25 mm in the right lung with massive ascites surrounding the liver. (B) The mass is supplied by the pulmonary artery (arrow), and the heart is severely deviated to the left side of the thoracic cavity. Lt, left; Rt, right.

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  Fig. 2 (A) Placement of the abdomino-amniotic shunt (arrow) in situ to eliminate ascites. (B) Ultrasonographic findings at 31+2 weeks of gestation showing the left lung with alleviated mediastinal shifting. (C) Postnatal chest computed tomography showing the right lung with a centrally located congenital cystic adenomatoid malformation (arrow) and the left normal lung. Lt, left; Rt, right.

Reference

1. Adzick NS, Harrison MR, Crombleholme TM, Flake AW, Howell LJ. Fetal lung lesions: management and outcome. Am J Obstet Gynecol. 1998; 179:884–889.

2. Witlox RS, Lopriore E, Oepkes D. Prenatal interventions for fetal lung lesions. Prenat Diagn. 2011; 31:628–636.

3. Adzick NS. Open fetal surgery for life-threatening fetal anomalies. Semin Fetal Neonatal Med. 2010; 15:1–8.

4. Bermudez C, Perez-Wulff J, Arcadipane M, Bufalino G, Gomez L, Flores L, et al. Percutaneous fetal sclerotherapy for congenital cystic adenomatoid malformation of the lung. Fetal Diagn Ther. 2008; 24:237–240.

5. Ruano R, da Silva MM, Salustiano EM, Kilby MD, Tannuri U, Zugaib M. Percutaneous laser ablation under ultrasound guidance for fetal hyperechogenic microcystic lung lesions with hydrops: a single center cohort and a literature review. Prenat Diagn. 2012; 32:1127–1132.

6. Higby K, Melendez BA, Heiman HS. Spontaneous resolution of nonimmune hydrops in a fetus with a cystic adenomatoid malformation. J Perinatol. 1998; 18:308–310.

7. Tsao K, Hawgood S, Vu L, Hirose S, Sydorak R, Albanese CT, et al. Resolution of hydrops fetalis in congenital cystic adenomatoid malformation after prenatal steroid therapy. J Pediatr Surg. 2003; 38:508–510.

8. Leung WC, Ngai C, Lam TP, Chan KL, Lao TT, Tang MH. Unexpected intrauterine death following resolution of hydrops fetalis after betamethasone treatment in a fetus with a large cystic adenomatoid malformation of the lung. Ultrasound Obstet Gynecol. 2005; 25:610–612.

9. Peranteau WH, Wilson RD, Liechty KW, Johnson MP, Bebbington MW, Hedrick HL, et al. Effect of maternal betamethasone administration on prenatal congenital cystic adenomatoid malformation growth and fetal survival. Fetal Diagn Ther. 2007; 22:365–371.

10. Curran PF, Jelin EB, Rand L, Hirose S, Feldstein VA, Goldstein RB, et al. Prenatal steroids for microcystic congenital cystic adenomatoid malformations. J Pediatr Surg. 2010; 45:145–150.

11. Morris LM, Lim FY, Livingston JC, Polzin WJ, Crombleholme TM. High-risk fetal congenital pulmonary airway malformations have a variable response to steroids. J Pediatr Surg. 2009; 44:60–65.

12. Miller JA, Corteville JE, Langer JC. Congenital cystic adenomatoid malformation in the fetus: natural history and predictors of outcome. J Pediatr Surg. 1996; 31:805–808.