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01-12-2003 | Pediatric

Bronchopulmonary foregut malformations: embryology, radiology and quandary

Authors: N. A. Barnes, D. W. Pilling

Published in: European Radiology | Issue 12/2003

Abstract.

Bronchopulmonary foregut malformations (BPFM) are a heterogeneous group of pulmonary developmental anomalies that present at varying ages and with overlapping symptoms, signs and radiology. This article discusses the embryology of these lesions with reference to possible common origins and the link between aetiology and radiological appearance. The radiology of each lesion, both antenatally and postnatally, is described and illustrated. A number of quandaries exist in the prediction of prognosis and subsequent treatment of BPFM. We discuss the radiological features that may help to elucidate an individual prognosis and aid in the planning of treatment. The treatment options available for BPFM are briefly discussed. Finally, the link between BPFM, in particular cystic adenomatoid malformations and malignancy, is discussed. We aim to provide a comprehensive overview of the embryology, radiology, prognosis and treatment highlighting contentious issues of BPFM.

Wang NS, Chen MF, Chen FF (1999) The glandular component in congenital cystic adenomatoid malformation of the lung. Respirology 4:147–153CrossRefPubMed

Cass DL, Quinn TM, Liechty KW, Crombleholme TM, Flake AW, Azick NS (1998) Increased cell proliferation and decreased apoptosis characterize congenital cystic adenomatoid malformation of the lung. J Pediatr Surg 33:1043–1047PubMed

Conran RM, Stocker JT (1999) Extralobar sequestration with frequently associated congenital cystic adenomatoid malformation, type 2: report of 50 cases. Pediatr Dev Path 2:454–463CrossRef

Winters WD, Effmann EL, Nghiem HV, Nyberg DA (1997) Disappearing fetal lung masses: importance of postnatal imaging studies. Pediatr Radiol 27:535–539CrossRefPubMed

Murphy JJ, Blair Gk, Fraser GC, Ashmore PG, LeBlanc JG, Sett SS, Rogers P, Magee JF, Taylor GP, Dimmick J (1992) Rhabdomyosarcoma arising within congenital pulmonary cysts: report of three cases. J Pediatr Surg 27:1364–1367PubMed

D'Agostino S, Bonoldi SD, Meli S, Cappellari F, Musi L (1997) Embryonal rhabdomyosarcoma of the lung arising in cystic adenomatoid malformation: case report and review of the literature. J Pediatr Surg 32:1381–1383PubMed

McAlister WH, Wright JR, Crane JP (1987) Main-stem bronchial atresia: intrauterine sonographic diagnosis. Am J Roentgenol 148:364–366

Takeda S, Miyoshi S, Inoue M, Omori K, Okumura M, Yoon, H, Minami M, Matsuda H (1999) Clinical spectrum of congenital cytic diseae of the lung in children. Eur J Cardiothorac Surg 15:11–17CrossRefPubMed

Clements BS, Warner J (1987) Pulmonary sequestration and related congenital bronchopulmonary-vasculture malformations: nomenclature and classification based on anatomical and embryological considerations. Thorax 42:401–408PubMed

10.

Cass DL, Crombleholme TM, Howell LJ, Stafford PW, Ruchelli ED, Adzick NS (1997) Cystic lung lesions with systemic arterial blood supply: a hybrid of congenital cystic adenomatoid malformation and bronchopulmonary sequestration. J Pediatr Surg 32:986–990PubMed

11.

Laurin S, Hagerstrand I (1999) Intralobar bronchopulmonary sequestration in the newborn: a congenital malformation. Pediatr Radiol 29:174–178CrossRefPubMed

12.

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

13.

Samuel M, Burge DM (1999) Management of antenatally diagnosed pulmonary sequestration associated with congenital cystic adenomatoid malformation. Thorax 54:701–706PubMed

14.

Stocker JT, Madewell JE, Drake RM (1977) Congenital cystic adenomatoid malformation of the lung: classification and morphological spectrum. Hum Pathol 8:15–71

15.

Evrard V, Ceulemans J, Coosemans W, De Baere T, De Lyn P, Deneffe G, Devlieger H, De Boeck C, Van Raemdonck D Lerut T (1999) Congenital parenchymatous malformations of the lung. World J Surg 23:1123–1132CrossRefPubMed

16.

McManus DT, O'Hara MD (1996) Extralobar srequestration on type II congenital cystic adenomatoid malformation in an infant with congenital diaphragmatic hernia. Pediatr Pathol Lab Med 16:637–642CrossRefPubMed

17.

Adzick NS, Harrison MR, Glick PL, Golbus MS, Anderson RL, Mahony BS, Callen PW, Hirsch JH, Luthy DA, Filly RA et al. (1985) Fetal cystic adenomatoid malformation: prenatal diagnosis and natural history. J Pediatr Surg 20:483–488PubMed

18.

Schwartz DS, Reyes-Mugica M, Keller MS (1999) Imaging of surgical diseases of the newborn chest. Radiol Clin North Am 37:1067–1078PubMed

19.

Sohaey R, Zwiebel WJ (1996) The fetal thorax: noncardiac chest anomalies. Semin Ultrasound CT MRI 17:34–50

20.

Diwan RV, Brennan JN, Philipson EH, Jain S, Bellon EM (1983) Ultrasonic prenatal diagnosis of type III congenital cystic adenomatoid malformation of lung. J Clin Ultrasound 11:218–221PubMed

21.

Dähnert W (ed) (1999) Bronchopulmonary sequestration. In: Radiology review manual, 4th edn. Williams and Wilkins, Baltimore, pp 394–396

22.

Morotti RA, Cangiarella J, Gutierrez MC, Jagirdar J, Askin F, Singh J, Profitt SA, Wert SE, Whitsett JA, Greco MA (1999) Congenital cystic adenomatoid malformation of the lung (CCAM): evaluation of the cellular components. Hum Pathol 30:618–625PubMed

23.

Samuel M, Burge DM (1996) Extra-lobar intra-abdominal pulmonary sequestration. Eur J Pediatr Surg 6:107–109PubMed

24.

Pilling DW (1998) Fetal lung abnormalities: What do they mean? Clin Radiol 53:789–795PubMed

25.

Ng KJ, Gray ES, Jeffrey RR, Youngson GG (1994) Intralobar bronchopulmonary sequestration: antenatal diagnosis. Thorax 49:379–380PubMed

26.

Zangwill BC, Stocker JT (1993) Congenital cystic adenomatoid malformation within an extralobar pulmonary sequestration. Pediatr Pathol 13:309–315PubMed

27.

Chandran H, Upadhyay V, Pease PW (2000) Congenital cystic adenomatoid malformation and extralabar sequestration occurring independently in the ipsilateral hemithorax. Pediatr Surg Int 16:102–103CrossRefPubMed

28.

Dähnert W (ed) (1999) Bronchial atresia. In: Radiology review manual, 4th edn. Williams and Wilkins, Baltimore, p 387

29.

Kravitz RM (1994) Congenital malformations of the lung. Pediatr Clin North Am 41:453–472PubMed

30.

Dähnert W (ed) (1999) Bronchogenic cyst. In: Radiology review manual, 4th edn. Williams and Wilkins, Baltimore, p 393

31.

Carrol ED, Campbell ME, Shaw NJ, Pilling DW (1996) Congenital lobar emphysema in congenital cytomegalovirus infection. Pediatr Radiol 26:900–902PubMed

32.

Al-Bassam A, Al-Raeeah A, Al-Nassar S, Al-Mobaireek K, Al-Rawaf A, Banjer H, Al-Mogari I (1999) Congenital cystic disease of the lung in infants and children (experince with 57 cases). Eur J Pediatr Surg 9:364–368PubMed

33.

Bagolan P, Nahom A, Giorlandino C, Trucchi A, Bilancioni E, Inserra A, Gambuzza G, Spina V (1999) Cystic adomatoid malformation of the lung: clinical evolution and management. Eur J Pediatr 158:879–882CrossRefPubMed

34.

Karnak I, Senocak ME, Ciftci AO, Buyukpamukcu N (1999) Congenital lobar emphysema: diagnostic and therapeutic considerations. J Pediatr Surg 34:1347–1351PubMed

35.

King SJ, Pilling DW, Walkinshaw S (1995) Fetal echogenic lung lesions: prenatal ultrasound diagnosis and outcome. Pediatr Radiol 25:208–210PubMed

36.

Van Leeuwen K, Teitelbaum DH, Hirschl RB, Austin E, Adelman SH, Polley TZ, Marshall KW, Coran AG, Ngent C, Arbor A (1999) Prenatal diagnosis of congenital cystic adenomatoid malformation and its postnatal presentation, surgical indications and natural history. J Paediatr Surg 34:794–799

37.

McCullagh M, MacConnachie I, Garvie D, Dykes E (1994) Accuracy of prenatal diagnosis of congenital cystic adenomatoid malformation. Arch Dis Child 71:F111–F113PubMed

38.

Lacy DE, Shaw NJ, Pilling DW, Walkinshaw S (1999) Outcome of congenital lung abnormalities detected antenatally. Acta Paediatr 88:454–458CrossRefPubMed

39.

Bromley B, Parad R Estroff JA, Benacerraf BR (1995) Fetal lung masses: prenatal course and outcome. J Ultrasound Med 14:927–936PubMed

40.

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

41.

Barret J, Chitayat D, Sermer M, Amankwah K, Morrow R, Toi A, Ryan G (1995) The prognostic factors in the prenatal diagnosis of the echogenic fetal lung. Prenat Diagn 15:849–853PubMed

42.

MacGillivray TE, Harrison MR, Goldstein RB, Adzick NS (1993) Disappearing fetal lung lesions. J Pediatr Surg 28:1321–1325PubMed

43.

De Santis M, Masini L, Noia G, Cavaliere AF, Oliva N, Caruso A (2000) Congenital cystic adenomatoid malformation of the lung: antenatal ultrasound findings and fetal-neonatal outcome. Fifteen years experience. Fetal Diagn Ther 15:246–250CrossRefPubMed

44.

Sapin E, Lejeune V, Barbet JP, Carricaburu E, Lewin F, Baron JM, Barbotin-Larrieu F, Helardot PG (1997) Congenital adenomatoid disease of the lung: prenatal diagnosis and perinatal management. Pediatr Surg Int 12:126–129CrossRef

45.

Bunduki V, Ruano R, da Silva MM, Miguelez J, Miyadahira S, Maksoud JG, Zugaib M (2000) Prognostic factors associated with congenital cystic adenomatoid malformation of the lung. Prenat Diagn 20:459–464CrossRefPubMed

46.

Kamata S, Ishikawa S, Usui N, Sawai T, Kitayama Y, Nose K, Okuyama H, Imura K, Okada A (1999) Clinical significance of the lung/thorax transverse-area ratio in fetuses with cystic lung disease. Pediatr Surg Int 15:470–474CrossRefPubMed

47.

Lipshutz GS, Lopoo JB, Jennings RW, Farrrell J, Harrison MR, Albanese CT (1999) Are bilateral fetal lung masses double trouble? Fetal Diagn Ther 14:348–350CrossRefPubMed

48.

Chaoui R, Kalache K, Tennstedt C, Lenz F, Vogel M (1999) Pulmonary arterial Doppler velocimetry in fetuses with lung hypoplasia. Eur J Obstet Gynecol Reprod Biol 84:179–185CrossRefPubMed

49.

Winters WD, Effmann EL, Nghiem HV, Nyberg DA (1997) Congenital masses of the lung: changes in cross sectional area during gestation. J Clin Ultrasound 25:372–377CrossRefPubMed

50.

Hubbard AM, Adzick NS, Crombleholme TM, Coleman BG, Howell LJ, Haselgrove JC, Mahboubi S (1999) Congenital chest lesions: diagnosis and characterisation with prenatal MR imaging. Radiology 212:43–48PubMed

51.

Achiron R, Strauss S, Seidman DS, Lipitz S, Mashiach S, Goldman B (1995) Fetal lung hyperechogenicity: prenatal ultrasonographic diagnosis, natural history and neonatal outcome. Ultrasound Obstet Gynecol 6:40–42CrossRefPubMed

52.

Dähnert W (ed) (1999) Cystic adenomatoid malformation. In: Radiology review manual, 4th edn. Williams and Wilkins, Baltimore, p 399

53.

Dähnert W (1999) Congenital lobar emphysema. In: Radiology review manual, 4th edition. Williams and Wilkins, Baltimore, p 399

54.

Kim WS, Lee KS, Kim IO, Suh YL, Im JG, Yeon KM, Chi JG, Han BK, Han MC (1997) Congenital cystic adenomatoid malformation of the lung: CT-pathologic correlation. Am J Roentgenol 168:47–53

55.

Sugiyama M, Honna T, Kamii Y, Tsuchida Y, Kawano T, Okai T, Isoda T (1999) Management of prenatally diagnosed congenital cystic adenomatoid malformation of the lung. Eur J Pediatr Surg 9:53–57PubMed

56.

Adzick NS, Harrison MR, Flake AW, Howell LJ, Golbud MS, Filly RA (1993) Fetal surgery for cystic adenomatoid malformation of the lung. J Pediatr Surg 28:806–812PubMed

57.

Seo T, Ando H, Watanabe Y, Harada T, Ito F, Kaneko K, Mimura S (1999) Acute respiratory failure associated with intrathoracic masses in neonates. J Paediatr Surg 11:1633–1637

58.

Waszak P, Claris O, Lapillonne A, Picaud JC, Basson E, Chappuis JP, Salle BL (1999) Cystic adenomatoid malformation of the lung: neonatal management of 21 cases. Pediatr Surg Int 15:326–331CrossRefPubMed

59.

Ueda K, Gruppo R, Unger F, Martin L, Bove K (1997) Rhabdomyosarcoma of lung arising in congenital cystic adenomatoid malformation. Cancer 40:383–388

60.

Priest JR, McDermott MB, Bhatia S, Watterson J, Manivel JC, Dehner LP (1997) Pleuropulmonary blastoma: a clinicopathologic study of 50 cases. Cancer 80:147–161CrossRefPubMed

61.

Tagge EP, Mulvihill D, Chandler JC, Richardson M, Uflacker R, Othersen HB (1996) Childhood pleuropulmonary blastoma: caution against nonoperative management of congenital lung cysts. J Pediatr Surg 31:187–190PubMed

62.

Ribet ME, Copin MC, Soots JG, Gosselin BH (1995) Bronchoalveolar carcinoma and congenital cystic adenomatoid malformaton. Ann Thorac Surg 60:1126–1128CrossRefPubMed

63.

Conran AG, Drongowski R (1999) Congenital cystic disease of the tracheobronchial tree. Experience with 44 consecutive cases. Arch Surg 129:521–527

64.

Dell'Agnola C, Tadini B, Mosca F, Colnaghi M, Wesley J (1996) Advantages of prenatal diagnosis and early surgery for congenital cystic disease of the lung. J Perinat Med 26:621–631

65.

Schwartz MZ, Ramachandran P (1997) Congenital malformations of the lung and mediastinum: a quarter century of experience from a single institution. J Pediatr Surg 32:44–47PubMed

Title: Bronchopulmonary foregut malformations: embryology, radiology and quandary
Authors: N. A. Barnes
D. W. Pilling
Publication date: 01-12-2003
Publisher: Springer-Verlag
Published in: European Radiology / Issue 12/2003
Print ISSN: 0938-7994
Electronic ISSN: 1432-1084
DOI: https://doi.org/10.1007/s00330-002-1812-5

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Bronchopulmonary foregut malformations: embryology, radiology and quandary

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