Surgical Endoscopy

, Volume 23, Issue 8, pp 1750–1753 | Cite as

Intralobar sequestration associated with cystic adenomatoid malformation: diagnostic and thoracoscopic pitfalls

  • Smart ZeidanEmail author
  • Geraldine Hery
  • Ferderic Lacroix
  • Guillaume Gorincour
  • Alain Potier
  • Jean Christophe Dubus
  • Jean-Michel Guys
  • Pascal de Lagausie



Intralobar sequestration (ILS) associated with congenital cystic adenomatoid malformation (CCAM) is a well-known entity. This hybrid form has many considerations for an appropriate management. This report aims to analyze pitfalls in the diagnosis, treatment, and thoracoscopic procedure pertaining to this association.


Between May 2004 and September 2007, 23 fetal lung lesions were diagnosed prenatally and managed at the authors’ institution. Seven of these lesions were the hybrid form of ILS-CCAM. The records for these lesions were reviewed retrospectively.


In all seven fetuses, prenatal ultrasound diagnosed congenital lung abnormalities. In three cases, fetal magnetic resonance imaging (MRI) demonstrated a macrocystic lesion supplied by an aberrant vessel. In all cases, the intrauterine evolution remained uncomplicated. All the newborns were asymptomatic at birth. Chest computed tomography (CT) performed at the age of 1 month showed the aberrant vessel in five cases. A regression of the lesion was noted in one patient and that of the aberrant vessel in another patient. Elective thoracoscopic lobectomy of the affected lobe was performed for six patients. The average age at the time of intervention was 5 months. At this writing, one patient is under observation due to resolution of the lesion. The operating time ranged from 80 to 160 min. One conversion to thoracotomy was required. All surgically treated patients had an uneventful hospital course. Histopathologic examination confirmed the association of intralobar sequestration with Stocker type 2 CCAM in five cases and with CCAM type 1 in one case.


Prenatal diagnosis of the ILS-CCAM association was possible, as was resolution of one or both components of this lesion. Preoperative identification of the aberrant vessel is important for prevention of operative morbidity. This should be controlled before the lobectomy is begun.


Congenital lesions Cystic adenomatoid malformation Lobectomy Lung Neonate Sequestration Thoracoscopy 


  1. 1.
    Ch’in KY, Tang MY (1949) Congenital adenomatoid malformation of one lobe of a lung with general anasarca. Arch Pathol 48:221–229Google Scholar
  2. 2.
    Rokitansky C (1855–1861) Lehrbuch der Pathologischen Anatomie, 3rd edn. Braumuller, Vienna, pp 44Google Scholar
  3. 3.
    Pryce DM (1946) Lower accessory pulmonary artery with intralobar sequestration of lung: a report of seven cases. J Pathol Bacteriol 58:457–467CrossRefGoogle Scholar
  4. 4.
    Dibden LJ, Fischer JD, Zuberbuhler PC (1998) Pulmonary sequestration and congenital cystic adenomatoid malformation in an infant. J Pediatr Surg 21:731–733Google Scholar
  5. 5.
    Morin C, Filiatrault D, Russo P (1998) Pulmonary sequestration with histologic changes of cystic adenomatoid malformation. Pediatr Radiol 19:130–132CrossRefGoogle Scholar
  6. 6.
    Conran RM, Stocker JT (1999) Extralobar sequestration with frequently associated congenital cystic adenomatoid malformation, type 2: report of 50 cases. Pediatr Dev Pathol 2:454–463PubMedCrossRefGoogle Scholar
  7. 7.
    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 adenomatoid malformation and bronchopulmonary sequestration. J Pediatr Surg 37:986–990CrossRefGoogle Scholar
  8. 8.
    de Lagausie P, Bonnard A, Berrebi D, Petit P, Dorgeret S, Guys JM (2005) Video-assisted thoracoscopic surgery for pulmonary sequestration in children. Ann Thorac Surg 80:1266–1269PubMedCrossRefGoogle Scholar
  9. 9.
    Dhingsa R, Coakley FV, Albanese CT, Filly RA, Goldstein R (2003) Prenatal sonography and MR imaging of pulmonary sequestration. AJR Am J Roentgenol 180:433–437PubMedGoogle Scholar
  10. 10.
    Wesley JR, Heidelberger KP, DiPietro MA, Cho KJ, Coran AG (1986) Diagnosis and management of congenital cystic disease of the lung in children. J Pediatr Surg 21:202–207PubMedCrossRefGoogle Scholar
  11. 11.
    Adzick NS, Harrison MR, Crombleholme TM, Flake AW, Howell LJ (1998) Fetal lung lesions: management and outcome. Am J Obstet Gynecol 179:884–889PubMedCrossRefGoogle Scholar
  12. 12.
    Davenport M, Warne SA, Cacciaguerra S, Patel S, Greenough A, Nicolaides K (2004) Current outcome of antenally diagnosed cystic lung disease. J Pediatr Surg 39:549–556PubMedCrossRefGoogle Scholar
  13. 13.
    Illanes S, Hunter A, Evans M, Cusick E, Soothill P (2005) Prenatal diagnosis of echogenic lung: evolution and outcome. Ultrasound Obstet Gynecol 26:145–149PubMedCrossRefGoogle Scholar
  14. 14.
    Van Leeuwen K, Teitelbaum DH, Hirschl RB, Austin E, Adelman SH, Polley TZ, Marshall KW, Coran AG, Nugent C (1999) Prenatal diagnosis of congenital cystic adenomatoid malformation and its postnatal presentation, surgical indications, and natural history. J Pediatr Surg 34:794–798PubMedCrossRefGoogle Scholar
  15. 15.
    Lacin T, Batirel HF, Ozer K, Demirutku A, Ahiskali R, Yuksel M (2007) Safety of a thermal vessel sealer on main pulmonary vessels. Eur J Cardiothorac Surg 31:482–485PubMedCrossRefGoogle Scholar
  16. 16.
    Albanese CT, Sydorak RM, Tsao K, Lee H (2003) Thoracoscopic lobectomy for prenatally diagnosed lung lesions. J Pediatr Surg 38:553–555PubMedCrossRefGoogle Scholar
  17. 17.
    Weatherford DA, Stephenson JE, Taylor SM, Blackhurst D (1995) Thoracoscopy versus thoracotomy: indications and advantages. Am Surg 61:83–86PubMedGoogle Scholar
  18. 18.
    Adzick NS, Harrison MR, Glick PL, Golbus MS, Anderson RL, Mahony BS, Callen PW, Hirsch JH, Luthy DA, Filly RA (1985) Fetal cystic adenomatoid malformation: prenatal diagnosis and natural history. J Pediatr Surg 20:483–488PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2008

Authors and Affiliations

  • Smart Zeidan
    • 1
    Email author
  • Geraldine Hery
    • 1
  • Ferderic Lacroix
    • 2
  • Guillaume Gorincour
    • 3
  • Alain Potier
    • 4
  • Jean Christophe Dubus
    • 5
  • Jean-Michel Guys
    • 1
  • Pascal de Lagausie
    • 1
  1. 1.Department of Pediatric SurgeryTimone-Enfants HospitalMarseilleFrance
  2. 2.Department of Anesthesiology-ReanimationTimone-Enfants HospitalMarseilleFrance
  3. 3.Department of RadiologyTimone-Enfants HospitalMarseilleFrance
  4. 4.Department of Prenatal DiagnosisTimone-Enfants HospitalMarseilleFrance
  5. 5.Department of Pediatrics, Pulmonary unitTimone-Enfants HospitalMarseilleFrance

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