Pulmonary sequestration, also called accessory lung, refers to aberrant formation of segmental lung tissue that has no connection with the bronchial tree or pulmonary arteries. It is a bronchopulmonary foregut malformation (BPFM).
The estimated incidence is 0.1%. Some authors propose a greater male prevalence (this may be the case for the extralobar type) ref. The age of presentation is dependent on the type of sequestration and this, in turn, determines the clinical presentation.
ELS more commonly presents in newborns, whereas ILS presents in late childhood or adolescence with recurrent pulmonary infections.
Pulmonary sequestration can be divided into two distinct groups based on the relationship of the aberrant segmental lung tissue to the pleura:
intralobar sequestration (ILS)
- accounts for the majority (75-85% of all sequestrations 4-5,7)
- present later in childhood with recurrent infections
extralobar sequestration (ELS)
- less common (15-25% of all sequestrations 4-5,7)
- usually present in the neonatal period with respiratory distress, cyanosis and/or infection
- recognized male predilection M:F ratio ~4:1
- can be infra-diaphragmatic in ~10% of cases
The two types of sequestration are similar in their relationship to the bronchial tree and arterial supply/venous drainage but differ in their relationship to the pleura.
By definition, there is no communication with the tracheobronchial tree. In the vast majority of cases, the abnormal lung tissue has a systemic arterial supply which is usually a branch of the aorta:
- intralobar sequestrations
- venous drainage most commonly through the systemic veins into the right atrium (but is variable)
- separated from any surrounding lung by its own pleura
Almost all cases occur sporadically.
Overall, sequestration preferentially affects the lower lobes. 60% of intralobar sequestrations affect the left lower lobe, and 40% the right lower lobe. Extralobar sequestrations almost always affect the left lower lobe, however ~10% of extralobar sequestrations can be subdiaphragmatic 8.
Associated disease is common with the extralobar type (50-60%):
- congenital pulmonary airway malformation (CPAM): which is then sometimes termed a hybrid lesion
- congenital heart disease
- congenital diaphragmatic hernia
- Scimitar syndrome 15
- radiographs will often show an opacity in the affected segment
- may show cystic spaces if infected
- both ILS and ELS can rarely have air bronchograms as they may be connected with the gastrointestinal tract
The sequestrated portion of lung is usually more echogenic than the rest of the lung. On antenatal ultrasound, an extralobar sequestration may be seen as early as 16 weeks gestation and typically appears as a solid well-defined triangular echogenic mass 8. Colour Doppler may identify a feeding vessel (in-utero cases) from the aorta. If the sequestration is sub diaphragmatic, it may appear as an echogenic intra abdominal mass.
- cross sectional imaging frequently demonstrates the arterial supply by the descending aorta
- they may arise below the diaphragm in 20% of patients
- usually doesn't contain air unless infected
- 3D reconstructions can be particularly helpful in detecting 7
- anomalous arterial vessels
- concurrent anomalous veins
- differentiating between intralobar and extralobar sequestrations
Not part of routine investigation but is the gold standard in determining arterial supply.
- T1: the sequestrated segment tends to be of comparatively high signal to normal lung tissue 14
- T2: also tends to be of comparatively high signal 14
Can be helpful in demonstrating anomalous arterial supply.
Treatment and prognosis
Traditionally treatment has been a surgical resection. Extralobar sequestrations with their separate pleural investments can usually be removed sparing normal lung, tissue, although with an intralobar type, segmental resection or even lobectomy will be necessary.
Coil embolisation has also been successfully trialled in selected cases 4. Spontaneous involution has been reported in occasional cases 10.
- frequent respiratory tract infection
- in neonates can be complicated by high output cardiac failure
General imaging differential considerations include:
- 1. Blickman JG, Parker BR, Barnes PD. Pediatric radiology, the requisites. Mosby Inc. (2009) ISBN:0323031250. Read it at Google Books - Find it at Amazon
- 2. Berrocal T, Madrid C, Novo S et-al. Congenital anomalies of the tracheobronchial tree, lung, and mediastinum: embryology, radiology, and pathology. Radiographics. 24 (1): e17. doi:10.1148/rg.e17 - Pubmed citation
- 3. Ferretti GR, Jouvan FB, Coulomb M. MDCT demonstration of intralobar pulmonary sequestration of the right upper lobe in an adult. AJR Am J Roentgenol. 2005;185 (6): 1663-4. doi:10.2214/AJR.05.0155 - Pubmed citation
- 4. Tokel K, Boyvat F, Varan B. Coil embolization of pulmonary sequestration in two infants: a safe alternative to surgery. AJR Am J Roentgenol. 2000;175 (4): 993-5. AJR Am J Roentgenol (full text) - Pubmed citation
- 5. Dhingsa R, Coakley FV, Albanese CT et-al. Prenatal sonography and MR imaging of pulmonary sequestration. AJR Am J Roentgenol. 2003;180 (2): 433-7. AJR Am J Roentgenol (full text) - Pubmed citation
- 6. Franco J, Aliaga R, Domingo ML et-al. Diagnosis of pulmonary sequestration by spiral CT angiography. Thorax. 1998;53 (12): 1089-92. doi:10.1136/thx.53.12.1089 - Free text at pubmed - Pubmed citation
- 7. Lee EY, Boiselle PM, Cleveland RH. Multidetector CT evaluation of congenital lung anomalies. Radiology. 2008;247 (3): 632-48. doi:10.1148/radiol.2473062124 - Pubmed citation
- 8. Dhingsa R, Coakley FV, Albanese CT et-al. Prenatal sonography and MR imaging of pulmonary sequestration. AJR Am J Roentgenol. 2003;180 (2): 433-7. AJR Am J Roentgenol (full text) - Pubmed citation
- 9. Ko SF, Ng SH, Lee TY et-al. Noninvasive imaging of bronchopulmonary sequestration. AJR Am J Roentgenol. 2000;175 (4): 1005-12. AJR Am J Roentgenol (full text) - Pubmed citation
- 10. García-peña P, Lucaya J, Hendry GM et-al. Spontaneous involution of pulmonary sequestration in children: a report of two cases and review of the literature. Pediatr Radiol. 1998;28 (4): 266-70. - Pubmed citation
- 11. West MS, Donaldson JS, Shkolnik A. Pulmonary sequestration. Diagnosis by ultrasound. J Ultrasound Med. 1989;8 (3): 125-9. J Ultrasound Med (abstract) - Pubmed citation
- 12. Pessar ML, Soulen RL, Kan JS et-al. MRI demonstration of pulmonary sequestration. Pediatr Radiol. 1988;18 (3): 229-31. - Pubmed citation
- 13. Oliphant L, Mcfadden RG, Carr TJ et-al. Magnetic resonance imaging to diagnose intralobar pulmonary sequestration. Chest. 1987;91 (4): 500-2. doi:10.1378/chest.91.4.500 - Pubmed citation
- 14. Au VW, Chan JK, Chan FL. Pulmonary sequestration diagnosed by contrast enhanced three-dimensional MR angiography. Br J Radiol. 1999;72 (859): 709-11. Br J Radiol (abstract) - Pubmed citation
- 15. Vida VL, Padalino MA, Boccuzzo G et-al. Scimitar syndrome: a European Congenital Heart Surgeons Association (ECHSA) multicentric study. Circulation. 2010;122 (12): 1159-66. doi:10.1161/CIRCULATIONAHA.109.926204 - Pubmed citation