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Eur J Cardiothorac Surg 2001;19:388-395
© 2001 Elsevier Science NL
a Department of Thoracic Surgery, University Hospital Gasthuisberg, Herestraat 49, B-3000 Leuven, Belgium
b Department of Pediatric Pulmonology, University Hospital Gasthuisberg, Herestraat 49, B-3000 Leuven, Belgium
c Department of Respiratory Medicine, University Hospital Gasthuisberg, Herestraat 49, B-3000 Leuven, Belgium
d Department of Pathology, University Hospital Gasthuisberg, Herestraat 49, B-3000 Leuven, Belgium
Received 8 October 2000; received in revised form 15 January 2001; accepted 24 January 2001.
Corresponding author. Tel.: +32-16-34-6823; fax: +32-16-34-6824; URL:http://www.kuleuven.ac.be/~p7568020/
e-mail: dirk.vanraemdonck{at}uz.kuleuven.ac.be
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Abstract |
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 Top Abstract 1. Introduction2. Material and methods3. Results4. DiscussionAppendix A. Conference...References |
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16 years: n=13; mean age, 3±5 years) versus the adult group (>16 years: n=15; mean age, 33±13 years). Results: No significant differences between both groups were observed in sex, side, type of sequestration, pulmonary venous drainage, associated anomalies, hospital and late outcome, and patient's overall score. Patients (n=21) with the intralobar type of sequestration presented significantly more often with an infection when compared with patients (n=7) with the extralobar type (91 versus 14%; P=0.0033). When compared with the pediatric group, patients in the adult group had significantly more respiratory infections (87 versus 38%; P=0.016), and also required a lobectomy more often (67 versus 31%; P=0.056). Conclusions: The extralobar type of sequestration often remains asymptomatic, and is usually an incidental finding during infancy. The intralobar type mostly presents with recurrent infections in adulthood resulting in more lobectomies. We believe these findings support our current policy to remove any pulmonary malformation whenever diagnosed in order to: (1), prevent infection and other potentially serious late complications which may compromise the surgical outcome; and (2), enhance the chance of a parenchymal-sparing resection.
Key Words: Broncho-pulmonary malformation • Pulmonary sequestration • Congenital • Systemic artery • Surgery
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1. Introduction |
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TopAbstract1. Introduction2. Material and methods3. Results4. DiscussionAppendix A. Conference...References |
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Numerous case reports of PS have been published in the literature. Large single institutional reports, however, are extremely rare [5–7]. We have previously reported our experience with seven adult cases operated at the University Hospital Pellenberg [8]. The results in our pediatric cases were published as part of a review on congenital broncho-pulmonary malformations operated at the University Hospital Gasthuisberg [9]. In the present study, we wanted to compare the characteristics and the outcomes between pediatric and adult patients that have been operated for a PS at the University Hospitals of Leuven between 1978 and 1997. We were specifically interested to see if there was any difference between both groups that could help us in deciding whether or not to remove an asymptomatic PS in the future.
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2. Material and methods |
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TopAbstract1. Introduction2. Material and methods3. Results4. DiscussionAppendix A. Conference...References |
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2.2. Study design
In this retrospective study, the characteristics and outcomes were compared between patients referred by our adult (MD) versus pediatric (KDB and HD) pulmonologists. The adult group (n=15) and the pediatric group (n=13) were defined as including all patients older or younger than 16 years of age, respectively.
The demographics, symptoms, diagnosis, operative findings, type of resection, associated anomalies, and the hospital and long-term outcomes were analyzed in all patients.
2.3. Follow up
A questionnaire was sent to all surviving patients (n=27). A response was obtained from 20/27 patients (74%). The response rate did not differ between both age groups (80% in the adult group versus 73% in the pediatric group; P=0.97). Patients not responding to the questionnaire were censored as surviving at the last date of follow up. In this questionnaire, information was obtained on residual pain, respiratory symptoms or medication, current medical problems or operations for other congenital anomalies. Finally, patients were asked to score the overall result of their operation.
Three patients (11%) were lost to follow up in the first year following surgery. The median follow up in the 27 surviving patients was 107 months. The mean follow up was somewhat longer in the adult patient group (117±23 months) when compared with the pediatric group (83±14 months; P=0.38).
2.4. Statistics
Data are presented as means±SD. The data were analyzed using the statistical package StatView SE+ Graphics version 1.03 (Abacus Concepts, Inc., Berkeley, CA). Comparison between the pediatric versus the adult group and IL-PS versus EL-PS consisted of the Fisher's exact test for discrete variables and the Mann–Whitney non-parametric test for continuous variables. Survival was calculated using the Kaplan–Meier method. A P value of less than 0.05 was accepted as significant.
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3. Results |
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TopAbstract1. Introduction2. Material and methods3. Results4. DiscussionAppendix A. Conference...References |
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3.2. Type, side and localization of sequestration (Table 1)
There were 21 IL-PS and seven EL-PS. The IL-PS/EL-PS ratio was 8/5 in the pediatric group and 13/2 in the adult group (P=0.20).
The malformation was located on the left side in 16 patients and on the right side in 12 patients. The left/right ratio was 5/8 in the pediatric group versus 11/4 in the adult group (P=0.12), and 12/9 in IL-PS versus 4/3 in EL-PS (P=1.00). One male patient (4%) presented with a bilateral sequestration. Only the left sided lesion was resected. Therefore, his right sided lesion is not included in this series.
Of the 21 IL-PS, 19 lesions (90%) were found in the lower lobe and two in the upper lobe. In six out of seven patients with EL-PS, the accessory lobe was located at the base of the thoracic cavity above the diaphragm.
3.3. Arterial supply and venous drainage (Table 1)
The systemic blood supply to the malformation was from a single vessel in 20 cases, from two arteries in four patients and from three arteries in three patients. Finally, in one patient the PS was vascularized by four small arteries. The arteries originated from the thoracic aorta in 17 patients, from the abdominal aorta in ten patients, and from an intercostal artery in two patients. Finally, in one patient, the origin of the systemic vessel was not documented.
The venous drainage was to a systemic vein in seven patients (the azygos system in four and the inferior caval vein in three) and to a pulmonary vein in 20 patients (the inferior pulmonary vein in 18 patients and the superior pulmonary vein in two patients). In one patient, the venous drainage of the lesion was also not documented. The systemic/pulmonary venous return ratio was 5/7 in the pediatric group versus 2/13 in the adult group (P=0.18). As expected, this ratio was significantly different when comparing IL-PS versus EL-PS (2/19 versus 5/1, respectively; P=0.0014).
3.4. Symptoms (Table 1)
Twelve patients had recurrent respiratory infections and six more patients underwent surgical resection after a single episode of infection in a documented pulmonary lesion. Two neonates presented with respiratory distress. One 3-month-old girl presented with high output left ventricular failure resulting from a left to right shunt. Finally, seven patients were asymptomatic (five EL-PS and two IL-PS; five neonates and two adults). The pulmonary malformation in these patients was detected on prenatal ultrasound (n=4) or routine chest X-ray (n=3).
All the patients in the adult group except the two patients with EL-PS (87%) were symptomatic compared with 8/13 (62%) in the pediatric group (P=0.20). Patients with an IL-PS were significantly more often symptomatic when compared with EL-PS (19/21 versus 2/7, respectively; P=0.0038).
When we compare patients who presented with a single or recurrent infection versus those who did not, a significant difference was seen between the pediatric versus the adult group (5/13 versus 13/15; P=0.0016), and also, between IL-PS versus EL-PS (17/21 versus 1/7; P=0.0033).
Symptomatic patients developed their first problem 3±4 years (0–12 years) prior to surgery with no difference between the pediatric and the adult groups.
3.5. Diagnosis
In four patients (14%), an intrathoracic abnormality had already been diagnosed on prenatal ultrasound. In the other patients, abnormal findings on chest X-ray (Fig. 1A)
or CT-scan (Fig. 1B) prompted the physician to refer the patients for surgical exploration.
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No significant differences were seen in the presence/absence of associated anomalies between the adult versus the pediatric group (1/14 versus 5/8, respectively; P=0.069), or between the IL-PS versus the EL-PS (3/18 versus 3/4, respectively; P=0.14).
3.7. Type of resection (Table 2)
Thirteen patients had a lobectomy and one patient a bilobectomy. In 14 patients, only the malformation was removed (sequestrectomy). In the patient with bilateral sequestration (case number 13), a sequestrectomy was performed on the left side, and an aberrant artery going to the right lower lobe was ligated. No pulmonary resection was carried out on the right side.
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3.8. Hospital outcome (Table 2)
Nine patients experienced postoperative problems: residual pneumothorax after removal of chest tube (n=4), bleeding (n=2), respiratory infection (n=2), and pleural effusion (n=1).
One neonate (4%) died of severe pulmonary hypoplasia and pulmonary hypertension 2 days following bilobectomy.
The duration of hospital stay was not documented in two cases. The mean hospital stay was 13±6 days and did not differ between the two age groups (12±6 days in the pediatric group versus 14±7 days in the adult group; P=0.34), nor between the two types of PS (13±6 days in IL-PS versus 12±8 days in EL-PS; P=0.33).
3.9. Late outcome
Of the 20 patients who responded to the questionnaire, 11 patients had no post-thoracotomy pain and nine patients (45%) had occasional discomfort in the thoracotomy incision. None of these patients were taking any pain medication. Five patients (25%) had respiratory symptoms that were attributed to their initial pulmonary problem (sputum production, n=1; dyspnea, n=4; cough, n=1). One of these patients had been hospitalized for recurrent respiratory infections. None of the patients had been reoperated for the same problem. Other medical problems in these patients were hypertension (n=2), esophagitis (n=1), asthma (n=1), hepatitis B (n=1), and rheumatoid arthritis (n=1). Eleven patients had pulmonary function tests done elsewhere in the years after their operation. Unfortunately, the results were not available for further analysis. Patients scored the overall result of their operation as excellent in 60% (n=12), good in 35% (n=7), and moderate in 5% (n=1). No difference in scores was observed between both age groups.
Four patients had another congenital problem that emerged in the years following surgery: pectus excavatum (n=1), Rubinstein–Taybi syndrome (n=1), Stilling–Turk–Duane syndrome (n=1), and epilepsy and mental retardation (n=1; Table 2).
The survival in all patients was 96% at 1 (n=22), 5 (n=16), 10 (n=9) and 20 years (n=2) after the operation (Fig. 3) .
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4. Discussion |
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TopAbstract1. Introduction2. Material and methods3. Results4. DiscussionAppendix A. Conference...References |
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The findings in this series are in accord with the information gathered in collective reviews of published small series and case reports [1,4]. The ratio of IL-PS/EL-PS was 21/7 in our experience, exactly identical to 400/133 in the largest collective series [4]. The aberrant artery was a single vessel in 71% of the patients and originated from the thoracic aorta in 61% and from the abdominal aorta in 36%. The sequestered lung tissue was located on the left side in 57% of the cases. The IL-PS was found in the lower lobe in 90% and the EL-PS was seen in the lower thoracic cavity in 86%. Associated congenital anomalies were seen in 43% of EL-PS and in 17% of IL-PS.
To the best of our knowledge, this study is the first to compare the characteristics and the outcomes between pediatric and adult patients. No significant differences were identified between the two groups in male/female ratio, IL-PS/EL-PS ratio, left/right ratio, systemic/pulmonary venous drainage, or in the presence or absence of associated congenital anomalies, suggesting that the occurrence in both age groups is identical. Patients in the adult group, however, presented with infectious problems more often. The ratio of lobectomy/sequestrectomy was also higher (P=0.056) in the adult group when compared with the pediatric group. This may suggest that early removal of the lesion whenever diagnosed during infancy may result in removal of less pulmonary parenchyma. However, when we excluded patients with EL-PS, because they will rarely present with an infection, the difference in both the infection as an indication for surgery and the type of resection between both age groups disappeared. Interestingly, the hospital and long-term outcomes did not differ between both age groups.
The answer to the question of whether or not to remove an asymptomatic lesion, found incidentally on imaging, is not clear. Numerous reports have described serious complications arising both from IL-PS as well as from EL-PS, such as fungal infection [10], tuberculosis [11], fatal hemoptysis [12], massive hemothorax [13], cardiovascular problems [14,15], benign tumors [16], and even malignant degeneration [17]. It has therefore been our policy to remove a pulmonary malformation not only to establish a correct diagnosis, but also to prevent potentially serious complications. Seven asymptomatic patients in this series (five neonates and two adults) were referred for surgical removal of a pulmonary lesion. It is well known that patients with EL-PS may remain asymptomatic for many years. In this series, only two out of seven patients with EL-PS were symptomatic (one neonate with respiratory distress and one neonate with lung infection resulting from a bronchus communicating with the esophagus [18]). On the other hand, the distinction between EL-PS and IL-PS is not always possible on preoperative imaging. The decision and timing to refer an asymptomatic patient for excision, however, remains controversial [6].
Another matter of debate is the best treatment policy in antenatally diagnosed pulmonary lesions. Four patients in this series had a malformation compatible with a PS detected on prenatal ultrasound. In all four cases, the lesion was removed shortly after birth (three EL-PS and one IL-PS). It is well known that the natural history of these lung masses is variable [19]. On one hand, serial scans may show regression, so that the lesion may become nearly undetectable at birth. On the other hand, these lesions may increase in size and cause postnatal respiratory distress from mediastinal compression, high output cardiac failure, or may present later in life with recurrent chest infections. Some authors therefore recommend surgical resection of a persistent postnatal pulmonary lesion with systemic arterial blood supply as it is unlikely that it will involute. Early surgery may reduce complication related morbidity and parental anxiety [20]. Neonatal pulmonary surgery nowadays is safe, provided no other life-threatening congenital cardiac, renal or neurological problems are involved. It is also well known that alveolar multiplication continues in the first years of life [21]. Compensatory growth of the remaining lung tissue after resection is therefore possible in infants, but not in adults. Removal of an asymptomatic lesion has to be balanced against the possible sequelae of a thoracotomy (scar, risk of positional scoliosis). In our large experience with pediatric thoracic surgery including the primary repair of esophageal atresia, this is usually not a problem. Consent of the parents should be obtained after they have been fully informed on the considerations mentioned above.
Surgical treatment consists of resection of the sequestered lung parenchyma (sequestrectomy). This type of operation is usually not very difficult in EL-PS as the malformation is well separated from the normal lung by its own pleural envelope. In this series, sequestrectomy was possible in all seven patients with EL-PS. In IL-PS, however, inflammatory changes resulting from previous infections may complicate the surgery by destroying the intersegmental plane. Lobectomy, and not resection of the sequestered segment alone, therefore, is often the treatment of choice. In this series, lobectomy was performed in 14/21 (67%) patients with an IL-PS. Theoretically, the removal of these lesions in childhood prior to the development of surrounding chronic inflammation may allow for a parenchymal-sparing, segmental resection.
Distinguishing PS from other pulmonary disorders, such as congenital cystic adenomatoid malformation, pulmonary emphysema, intrapulmonary abscess, bronchiectasis, or malignant tumor, is not always possible on chest X-ray or CT. Selective arteriography may demonstrate the aberrant artery and is the most useful examination for the preoperative diagnosis of PS. The detection and localization of aberrant vessels may be important to the surgeon to avoid any intraoperative bleeding from injury to unexpected aberrant vessels. Nowadays, less invasive imaging techniques, such as duplex-scan [22], CT-scan [23] or MRI [24], have become available to demonstrate the systemic vessel supplying the pulmonary malformation.
Video-assisted thoracoscopic surgery has recently enlarged the armamentarium of the thoracic surgeon, allowing minimally invasive removal of lobes and even complete lungs. There have been some case reports on video-assisted thoracoscopic resection of a PS [25]. The authors emphasized the importance of preoperative detailed analysis of aberrant arteries by selective arteriography for safe manipulation during thoracoscopy. Also, our group recently performed a successful VATS left lower lobectomy in a 54-year-old female patient with IL-PS. This patient was not included in this series.
A shared embryological defect for PS and congenital cystic adematoid malformation has been suggested as they often display the same clinicopathologic features. The association of both types of congenital malformation is well described in both EL-PS and IL-PS [20,26].
In summary, this study has confirmed that the extralobar type will often remain asymptomatic and is usually an incidental finding on prenatal ultrasound in neonates or on chest X-ray in childhood. In contrast, the intralobar type mostly presents with recurrent infections during adulthood. The only significant difference in this study between adult and pediatric patients was the higher rate of infections and the higher number of lobectomies in de adult group. No differences in early and late outcomes were found between both groups.
We believe these findings support our current policy to remove any pulmonary malformation whenever diagnosed in order to: (1), prevent late infections and other potentially serious late complications which may compromise the surgical outcome; and (2), enhance the chance of a parenchymal-sparing resection.
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Acknowledgments |
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Footnotes |
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Appendix A. Conference discussion |
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TopAbstract1. Introduction2. Material and methods3. Results4. DiscussionAppendix A. Conference...References |
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Dr Van Raemdonck: I have not found this term in the literature. I have only seen the term sequestration, and malinosculation (abnormal connection) as well as Clements and Warner (ClementsB.S.WarnerJ.Q.Pulmonary sequestration and related congenital bronchopulmonary-vascular malformations: nomenclature and classification based on anatomical and embryological considerationThorax198742401408).
Ms D. Watson (Norwich, UK): There is interest now amongst neonatologists in observing babies who are identified as having a sequestration in utero or just after birth. You are suggesting that we should remove all sequestrations, and yet I don't think you have demonstrated that your infant population necessarily becomes your adult population. Do you have any experience of following children without an operation?
Dr Van Raemdonck: Your comment is absolutely right. Our figures do not defend that we should really remove an asymptomatic sequestration, however, it is our policy in the hospital that in all patients with asymptomatic lesions, the sequestration will be removed.
It is difficult to really say what is the best for the child, but we have to balance the complication/related morbidity, such as bleeding, hemoptysis, malignant degeneration, versus the operation/related morbidity, like a scar at young age, the risk of positional scoliosis; but it is also known that that there is still alveolar multiplication in the first years of age, and therefore, compensatory lung growth after resection of lung is still possible in infants, but not in adults.
Another point is that we cannot make the differential diagnosis between an intralobar type versus an extralobar type, and we know that the intralobar type most likely will have symptoms of recurrent infection at a later age. So we, in our hospital, would favor early surgical removal, but we have no experience in observing patients that are asymptomatic.
Mr K. Moghissi (Goole, UK): In your abstract, you indicate that five patients were diagnosed in utero. First of all, how were they diagnosed, and secondly, does it imply that, at birth, you would investigate them further and operate on them immediately?
Dr Van Raemdonck: That's correct, the lesions were diagnosed on prenatal ultrasounds; patients had been followed in utero with consecutive ultrasounds. After birth, we observed the patients. Two patients were immediately resected because of respiratory distress and one patient because of high output cardiac failure. The two other patients were asymptomatic and were still operated in the first week after birth.
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References |
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TopAbstract1. Introduction2. Material and methods3. Results4. DiscussionAppendix A. Conference...References |
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This article has been cited by other articles:
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  K. Tetsuka, S. Endo, Y. Kanai, and S. Yamamoto Extralobar pulmonary sequestration presenting as hemothorax Interactive CardioVascular and Thoracic Surgery, September 1, 2009; 9(3): 547 - 548. [Abstract] [Full Text] [PDF]
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 C. Chen and H. Zheng Extralobar Pulmonary Sequestration in the Hilum Asian Cardiovasc Thorac Ann, June 1, 2008; 16(3): 246 - 248. [Abstract] [Full Text] [PDF]
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G. Datta, J. Tambiah, S. Rankin, A. Herbert, and L. Lang-Lazdunski Atypical presentation of extralobar sequestration with absence of pericardium in an adult. J. Thorac. Cardiovasc. Surg., November 1, 2006; 132(5): 1239 - 1240. [Full Text] [PDF]
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