Pulmonary sequestration

Synonyms :
- congenital pulmonary sequestration
- congenital bronchopulmonary sequestration

 

Description

Pulmonary sequestration (PS) consists of an island of lung parenchyma that does not communicate with the bronchial tree and is fed by the systemic rather than the pulmonary circulation.

There are two types of PS: intralobar and extralobar. Prenatal diagnosis concerns the latter type only, because intralobar sequestration is not visible on ultrasound, despite accounting for three quarters of the PS cases detected after birth. 63% of extralobar PS affect the lower lobes and the left side is more affected than the right.

Illustration - Séquestration pulmonaire

Incidence

about 1/100 of the population
(2nd most common lung malformation)

3.5 boys for 1 girl in extralobar pulmonary sequestration

 

Codification :
CIM-10 : Q33.2
HPO : 0100632

extralobar sequestration :
HPO : 0006544

 

A little story

Several theories persist but the most commonly adopted suggests that a pulmonary sequestration would be the result of the formation of an accessory lung bud between the 4th and the 8th week of embryonic development.

 

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During a quick appointment, share with us the signs that, you have spotted on the ultrasound. We will replay the exam in Sonio Diagnostics. These appointments are reserved for healthcare professionals.

Ultrasound diagnosis

ultrasound signs detectable in the 1st trimester

   ***

Ultrasound signs detectable in the 2nd & 3rd trimester

  • 4 chamber view :
    well-defined homogeneously hyperechoic, unilateral and roughly triangular mass (often the left lower lobe)

  • diaphragmatic level axial view :
    to visualize the rare subdiaphragmatic variants

  • sagittal views :
    assessment of the caudal extension of the mass
    detection and characterization of subdiaphragmatic extralobar sequestrations
    recognition, on power or color Doppler, of the feeding artery branching off the descending aorta

    The nourishing artery of sequestration most often arises from the descending thoracic aorta, but can also arise from the abdominal aorta, and more rarely from the splenic artery or the celiac trunk.

Differential Diagnosis

  • solid, microcystic variant of CAML (similarity of the echogenicity)
    attention - tip Sonio

    A more triangular shape, a location in the left lower lung area, and, above all, the recognition of the feeding vessel all favor a PS
  • a more triangular shape, a location in the left lower lung area, and, above all, the recognition of the feeding vessel all favor a PS
    information - tip Sonio

    in the very rare instance in which a feeding vessel is identified entering the solid component of a prevalently cystic CAML, the lesion is probably a rare case of mixed CAML + PS lesion

Associated anomalies

There is a close relationship from a histological and pathogenetic point of view between PS and MAKP, the associated anomalies are very close.
An associated abnormality is found in 65% of cases of extralobar PS.

  • hydrops (cardiac compression)
  • polyhydramnios (esophageal compression)
  • tracheoesophageal fistulas
  • pulmonary artery branching abnormalities
  • diaphragmatic hernia (16%)
  • pulmonary malformations (25%) :
  • pulmonary hypoplasia
  • pulmonary emphysema
  • bronchogenic cyst

Risk of chromosomal anomalies

Very low

 

Risk of nonchromosomal syndromes

Very low

Acrocephalopolydactylous Dysplasia

information - tip Sonio

The etiology of PS is unknown, although it has been suggested that it might share the same pathogenesis with CAML, due to the fact that the two lesions are frequently associated

 

 

 

 

Etiologies - Others

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Sonio Diag_ écran signes à vérifier_EN-1

Some key figures

 

300

Recognisable syndromes

700

Recognisable signs

50

Prenatal diagnosis practitioners have already adopted it
echographie-sonio-1

References

  • N Zhang, Q Zeng, C Chen, J Yu, X Zhang. Distribution, diagnosis, and treatment of pulmonary sequestration: Report of 208 cases. J Pediatr Surg. 2019 Jul;54(7):1286-1292.
  • J-H Hung, S-H Shen, W-Y Guo, C-Y Chen, K-C Chao, M-J Yang, C-Y S Hung. Prenatal diagnosis of pulmonary sequestration by ultrasound and magnetic resonance imaging. J Chin Med Assoc. 2008 Jan;71(1):53-7.
  • R Ruano, A Benachi, M-C Aubry, Y Revillon, S Emond, Y Dumez, M Dommergues. Prenatal diagnosis of pulmonary sequestration using three-dimensional power Doppler ultrasound. Ultrasound Obstet Gynecol. 2005 Feb;25(2):128-33.
  • M R Curtis, D P Mooney, T J Vaccaro, J C Williams, M Cendron, N A Shorter, S K Sargent
    Prenatal ultrasound characterization of the suprarenal mass: distinction between neuroblastoma and subdiaphragmatic extralobar pulmonary sequestration. J Ultrasound Med. 1997 Feb;16(2):75-83.
  • Min Kyong Cho, Mi-Young Lee, Jisik Kang, Juhee Kim, Hye-Sung Won, Pil-Ryang Lee, Euiseok Jeong, Byong Sop Lee, Ellen Ai-Rhan Kim, Heemang Yoon, Jin Seoung Lee, Minkyu Han. Prenatal sonographic markers of the outcome in fetuses with bronchopulmonary sequestration. J Clin Ultrasound. 2020 Feb;48(2):89-96.
  • G Xu, J Zhou, S Zeng, M Zhang, Z Ouyang, Y Zhao, H Yuan, L Tong, C Yin, Q Zhou. Prenatal diagnosis of fetal intraabdominal extralobar pulmonary sequestration: a 12-year 3-center experience in China Sci Rep. 2019 Jan 30;9(1):943.
  • Z A D L-Ureña, S Sadowinski-Pine, L Jamaica-Balderas, [Pulmonary sequestration associated with congenital pulmonary airway malformation]. J Penchyna-Grub.. Bol Med Hosp Infant Mex. 2018;75(2):119-126.
  • A Bhide, D Murphy, B Thilaganathan, J S Carvalho. Prenatal findings and differential diagnosis of scimitar syndrome and pulmonary sequestration. Ultrasound Obstet Gynecol. 2010 Apr;35(4):398-404.
  • E. Ben Jemia Ben Zekri, S. Zairi, M. Abdenadher et H. Zribi, « Séquestration pulmonaire diagnostiquée à l’âge adulte : à propos de 25 cas », Revue des Maladies Respiratoires, 23e Congrès de Pneumologie de Langue Française, vol. 36,‎ 1er janvier 2019, A205–A206
  • Harriet J Corbett et Gillian M.E Humphrey, « Pulmonary sequestration », Paediatric Respiratory Reviews, vol. 5, no 1,‎ mars 2004, p. 59–68.
  • Monni G, Paladini D, Ibba RM, et al. Prenatal ultrasound diagnosis of congenital cystic malformation of the lung: a report of 26 cases and review of the literature. Ultrasound Obstet Gynecol 2000; 16: 159–62
  • Adzick NS, Harrison MR, Crombleholme TM, et al. Fetal lung lesions: management and outcome. Am J Obstet Gynecol 1998;179: 884–9
  • Lopoo JB, Goldstein RB, Lipshultz GS, et al. Fetal pulmonary sequestration: a favourable congenital lung lesion. Obstet Gynecol 1999; 94: 567–71
  • Pumbergera W, Hörmann M, Deutingerc J, et al. Longitudinal observation of antenatally detected congenital lung malformations (CLM): natural history, clinical outcome and long-term follow-up. Eur J Cardiothorac Surg 2003; 24: 703–11
  • Avni EF, Vanderelst A, Van Gansbeke D, et al. Antenatal diagnosis of pulmonary tumors: report of two cases. Pediatr Radiol 1986; 16: 190–2