Interact CardioVasc Thorac Surg 2009;9:547-548. doi:10.1510/icvts.2009.209254
© 2009 European Association of Cardio-Thoracic Surgery
Case report - Thoracic non-oncologic |
Extralobar pulmonary sequestration presenting as hemothorax
Kenji Tetsuka,
Shunsuke Endo*,
Yoshihiko Kanai and
Shinichi Yamamoto
Department of General Thoracic Surgery, Jichi Medical University, 1-3311 Yakushiji, Shimotsuke-si, Tochigi, 329-0498, Japan
Received 9 April 2009;
received in revised form 20 May 2009;
accepted 25 May 2009
*Corresponding author. Tel.: +81-285-58-7368; fax: +81-285-44-6271.
E-mail address: tcvtzk{at}jichi.ac.jp (S. Endo).
 |
Abstract
|
|---|
Symptomatic extralobar pulmonary sequestration (EPS) is extremely rare. Herein, we report two male patients (3 and 16 years of age) with EPS presenting as hemothorax. Thoracotomic resections of the sequestrated lungs were uneventful. Pathologic examinations revealed hemothorax caused by circulatory disorders within the EPSs.
Key Words: Extralobar pulmonary sequestration; Infarction; Hemothorax
|
1. Introduction
|
|---|
Symptomatic pulmonary sequestration is usually an intralobar type presenting as infection, hemoptysis and hemothorax caused by communication to the airway [1]. Herein, we report two patients with symptomatic extralobar pulmonary sequestration (EPS) presenting as hemothorax.
1.1. Case 1
A 16-year-old male was referred to a nearby hospital two months previously because of increasing left chest pain, and developed a fever and chills. His past history was unremarkable. Chest radiography revealed a massive left pleural effusion. Blood examination tests showed a white blood cell count of 15,200/mm3, hemoglobin of 16.4 g/dl and a serum C-reactive protein concentration of 18.2 mg/dl. A tube thoracostomy yielded hemothorax to the amount of 1100 ml. Chest computed tomography revealed a mass of 4x5x5 cm adjacent to the inferior ligament, which was markedly thickened (Fig. 1).
View larger version (90K):
[in this window]
[in a new window]
|
Fig. 1. A computed tomography scan of case 1 at the onset of hemothorax, showing a massive left pleural effusion and an irregular tumor of 5x4x5 cm in the paraspinal area medial to the left lower lobe above the diaphragm.
|
|
The patient was admitted to our hospital for further examinations of the tumor. Chest computed tomography revealed a marked reduction in the size of the tumor to 2x2.5x2 cm (Fig. 2), with resolution of the hemothorax and a decrease in the serum C-reactive protein concentration. An exploratory thoracotomy was performed. Adhesion of the inferior ligament was marked. The tumor was separated from the left lower lobe with a dense adhesion to the diaphragm. No anomalous arteries were identified within the inferior ligament. The tumor resection was uneventful.
View larger version (80K):
[in this window]
[in a new window]
|
Fig. 2. A computed tomography scan of case 1 at 2 months after the onset, showing a marked reduction of the tumor size to 2x2.5x2 cm with resolution of the hemothorax at 2 months after the onset.
|
|
Pathologic findings showed an organized pulmonary parenchyma consistent with an infarcted EPS. The small vessels around the bronchus changed the hyaline lesion by occlusion, while no elastic arteries were identified.
1.2. Case 2
A 3-year-old male child presented at the emergency room with progressive chest pain 14 days previously. Physical examination revealed an acute illness and tachypnea (respiratory rate, 38/min). Blood examination tests showed a white blood cell count of 15,000/mm3, hemoglobin of 12.3 g/dl and a serum C-reactive protein concentration of 14.3 mg/dl. Chest radiography revealed a left pleural effusion. A tube thoracostomy yielded hemothorax to the amount of 150 ml. Repeated chest computed tomography scans three days after resolution of the hemothorax revealed a left paraspinal mass of 5x2.5x3 cm in the inferior portion of the left pleural cavity. An exploratory thoracotomy was performed. Thickened pleura was identified over the left lower lobe, diaphragm and chest wall. A soft tumor was located adjacent to the inferior ligament and separated from the left lower lobe with a dense adhesion to the diaphragm. No anomalous systemic arteries supplying the tumor were identified. Tumor resection concomitant with partial resection of the diaphragm was successful. Pathologic examinations revealed that the tumor had an organized pulmonary parenchyma consistent with an infarcted EPS. Small elastic arteries were identified within the tumor.
|
2. Discussion
|
|---|
EPS is an embryonic anomaly. It is considered to be an accessory lobe and was first described in 1861 by Rokitansky and Rektorik. EPS, an entity in which an abnormal lung segment is enclosed within its own pleural membrane, is completely separated from the tracheobronchial tree and accounts for 25% of all pulmonary sequestrations. The location, usually related to the left hemidiaphragm, may vary between the lower lobe and the diaphragm, within the mediastinum, the lung, pleural or pericardial spaces, or the retroperitoneum. The preferential site is reported to be the left inferior ligament with a frequency of 80%. The arterial blood supply to the sequestered lung by aberrant elastic arteries mostly originates from the thoracic or abdominal aorta in most cases with EPS. These arteries may arise above or below the diaphragm, and are usually small and variable [2–5].
EPS is mostly asymptomatic and discovered on routine chest X-ray films or incidental examinations for other diseases. Among the previous reports detected by PubMed using key words for symptomatic EPS such as hemothorax, infarction or torsion, five cases with symptomatic EPS were cited [6–10]. The symptoms of these cases were ipsilateral chest pain and respiratory failure with sudden onset caused by hemothorax and infarction. The age range (2–31 years) for these cases is younger than that for asymptomatic EPS. The location of the EPS was the left costovertebral angle adjacent to the inferior ligament in all patients. Based on these previous reports and our experience, symptomatic EPS may be related to infarction caused by torsion under exposure to drastic pleural cavity movement or by infection associated with bacteremia. The sudden onset of a circulatory disorder may cause hemothorax. The small vessels around the bronchus changed the hyaline lesion by occlusion, while no elastic arteries were identified in case 1. The aberrant vessels may have deteriorated during the two months from the onset of symptoms to surgery when the tumor was drastically reduced. Aberrant arteries were not identified in a previous report in which the patient underwent surgery within a few days after the onset of symptoms. Asymptomatic EPS with a tendency toward infarction may possibly have tiny aberrant vessels.
In conclusion, EPS should be considered when a tumor is located adjacent to the left inferior ligament within hemothorax, and resected soon after diagnosis.
|
References
|
|---|
- Van Raemdonck D, De Boeck K, Devlieger H, Demedts M, Moerman P, Coosemans W, Deneffe G, Lerut T. Pulmonary sequestration: a comparison between pediatric and adult patients. Eur J Cardiothorac Surg 2001;19:388–395.[Abstract/Free Full Text]
- Carter R. Pulmonary sequestration. Ann Thorac Surg 1969;7:68–88.[Medline]
- Sade RM, Clouse M, Ellis FH Jr. The spectrum of pulmonary sequestration. Ann Thorac Surg 1974;18:644–658.[Medline]
- Mattila SP, Ketonen PE, Kyllonen KE. Pulmonary sequestration. Ann Chir Gynaecol Fenn 1975;64:24–29.[Medline]
- Halkic N, Cuenoud PF, Corthesy ME, Ksontini R, Boumghar M. Pulmonary sequestration: a review of 26 cases. Eur J Cardiothorac Surg 1998;14:127–133.[CrossRef][Medline]
- Maull KI, McElvein RB. Infarcted extralobar pulmonary sequestration. Chest 1975;68:98–99.[CrossRef][Medline]
- Hernanz-Schulman M, Stein SM, Neblett WW, Atkinson JB, Kirchner SG, Heller RM, Merrill WH, Fleischer AC. Pulmonary sequestration: diagnosis with color Doppler sonography and a new theory of associated hydrothorax. Radiology 1991;180:817–821.[Abstract/Free Full Text]
- Avishai V, Dolev E, Weissberg D, Zajdel L, Priel IE. Extralobar sequestration presenting as massive hemothorax. Chest 1996;109:843–845.[CrossRef][Medline]
- Huang EY, Monforte HL, Shaul DB. Extralobar pulmonary sequestration presenting with torsion. Pediatr Surg Int 2004;20:218–220.[CrossRef][Medline]
- Chun EJ, Goo JM, Lee HJ, Lee CH, Im JG. Extralobar pulmonary sequestration with hemorrhagic infarction in an adult. J Thorac Imaging 2007;22:166–168.[CrossRef][Medline]
|
|
Top
Abstract
1. Introduction