A Case of Intrapericardial Extralobar Pulmonary Sequestration:
First Case in Korea
- Chul Min Ahn, M.D., Hyung Jung Kim, M.D., Hyun Keun Cho, M.D., Sung Kyu Kim, M.D., Won Young Lee, M.D., Sang Jin Kim, M.D., Hae Kyoon Kim, M.D., Doo Yun Lee, M.D., Ki Bum Lee, M.D.
- Abstract
-
Extralobar pulmonary sequestration, known as accessory lung, is a rare congenital anomaly and intrapericardial lung sequestration is extremely rare.To the best of our knowledge, only four reported cases were intrapericardial. We report a case with intrapericardial extralobar pulmonary sequestration confirmed by operation and morphologic basis.
Key Words
Intrapericardial. Extralobar pulmonary sequestration
- INTRODUCTION
- INTRODUCTION
Extralobar pulmonary sequestration is defined as a mass of pulmonary parenchyma anatomically separate from the normal lung.In extralobar pulmonary sequestration, the tissue has a distinct pleural covering and may be supplied by an aberrant artery from the aorta or its branch, in rare instances, extralobar pulmonary sequestration communicates with the esophagus or stomach1–4).The pulmonary sequestration is found lying mostly at the left lung base but can be found in the right lung and in the mediastinum at any level from the neck to below the diaphragm1,2,5–8).Extralobar pulmonary sequestration is often discovered at the time of routine roentgenogram of the chest or at the time of repair of associated congenital anomalies such as diaphragmatic hernia and fistulous communication with the gastrointestinal tract because extralobar sequestration rarely presents like the intralobar form with recurrent pulmonary infection1,4,6,8).
- CASE REPORT
- CASE REPORT
A 27-year-old man was admitted to Yong Dong Severance Hospital, Yonsei University Medical Center, for evaluation and management of mediastinal mass.Three years prior to admission, he found mediastinal mass on computerized tomographic (CT) scan performed in another institution.At admission, he was noted to be with generalized weakness and right chest pain, but he denied cough, sputum and dyspnea.The temperature was 38.6°C, the pulse rate 72/min and the blood pressure 110/70 mmHg.On examination, he appeared chronically ill, the skin was warm and dry, the head and neck were normal and cervical lymphadenopathy was not found, the lungs were clear and the heart was normal.The abdomen and extremities were normal.The urine was normal and the hemoglobin was 13.5gm/dl; hematocrit 41.9%; white cell count 5,500/mm3 with 64 percent neutrophils, 35 percent lymphocytes and 1 percent eosinophils, the serum electrolytes and liver function tests were normal. T3: 150 ng/dl, T4: 7.1 ug/dl, FT4: 1.6ng/dl and TSH was 1.5 uU/ml.The electrocardiogram showed right axis deviation and tall R wave in right precordial leads, and the sputum exanination did not suggest malignancy or tuberculosis.Chest roentgenogram (Fig. 1) revealed well defined mediastinal mass on the right paratracheal area.Chest CT scan revealed large multi-cystic mass lesion on the right paratracheal area which was attached to the lower pole of right thyroid gland, displaced the superior vena cava anteriorly and extended to the level of the azygous arch vein (Fig. 2).The lung perfusion scan and whole body bone scan were normal and the thyroid scan revealed cold area in the lower pole of right thyroid gland.The fiberoptic bronchoscopy revealed extrinsic mass effect without mucosal lesions on right intermediate bronchus.The pulmonary function studies showed mild restriction with FEV1:2.85 L/sec and FVC: 3.49 L/sec.He underwent operation under the impression of mediastinal mass.8×8 cm sized mass composed of multi-cystic and solid part was located in the pericardium and excised easily from the surrounding structures and the feeding vessel maybe originated from the subclavian artery.
- Discussion
- Discussion
Pulmonary sequestration is an uncommon congenital malformation characterized by the presence of nonfunctioning lung tissue which usually has no communication with the normal bronchial tree and which receives its blood supply from an anomalous systemic artery instead of a pulmonary arterial branch9).In intralobar sequestration, the abnormal pulmonary tissue is incorporated within the normal lung and shares a common covering of visceral pleura, and in extralobar sequestration, the abnormal pulmonary tissue is separate from the normal lung and has its own pleural covering1,3,5,7).“Bronchopulmonary foregut malformation” proposed by Gerle RD and Heithoff as pathogenesis of pulmonary sequestration is generally accepted and their spectrum of bronchopulmonary foregut malformation includes intralobar and extralobar sequestration, esophageal diverticuli, foregut duplication cysts (esophageal cysts) and maybe expanded to include bronchogenic cysts10–21).The incidence rate was 1.1 to 1.8 percent of all lobectomied patients by Carter and 0.15 to 6.4 percent of all congenital pulmonary malformation by Savic.Savic found 133 cases of extralobar sequestration in a review of 547 cases of pulmonary sequestration.The male predominance of patients with extralobar pulmonary sequestration (3:1) was described by Carter and Savic described that extralobar sequstration was located between the diaphragm and lower lobe in 77.4% and on left lung in 78.9% of the cases8).Most of the published cases of extralobar pulmonary sequestration were located outside the pericardium, only four reported cases were intrapericardial15,13,14).Since an extralobar sequestered segment is enveloped in its own pleural sac, the chance of becoming infected are very small, unless there is communication with the gastrointestinal tract8).The extralobar sequestration (42%) is more often associated with other anomalies than intralobar type (14%) such as diaphragmatic hernia and fistulous communication with the gastrointestinal tract1,5,15).Consequently, extralobar sequestration is usually an incidental finding on routine roentgenograms of the chest or during management of some other congenital anomaly and infrequently, extralobar sequestration presents with symptoms similar to those seen with the intralobar form and with a picture of congestive heart failure or pulmonary overcirculation1,5,7,16).In all cases of intrapericardial extralobar pulmonary sequestration, a large quantity of pericardial fluid was found, but no sign of congestive heart failure or cardiac tamponade was noticed13,14).A correct preoperative diagnosis is made in 39 percent of intralobar lung sequestration and only in 9 percent of extralobar lung sequstration by Savic and correct preoperative diagnosis is more difficult in the cases of unusual location.Preoperative angiography is important to confirm the diagnosis and to afford a safe operative approach1,6,7).The sequestratered segment is typically made up of multiple cysts or noncystic mass containing branching bronchi which run in the direction of the aberrant artery. The cysts contain mucus or mucopurulent material or air.Histologically, the sequestration consists of normal lung elements in an abnormal and disorderly arrangement with a variable presence of cartilage, bronchial glands and alveolar parenchyma. The cysts are lined with ciliated respiratory epithelium7,16).
Fig. 2
Chest CT scan revealed large multi-cystic mass lesion on the right paratracheal area which was attached to the lower pole of the right thyroid gland, displaced the superior vena cava anteriorly and extended to the level of the azygous vein.
Fig. 3
The mass is composed of several cystic lesions and yellow solid lesion resembling unexpanded lung tissue.
Fig. 5
The cyst wall shows a bronchial tissue which is composed of ciliated columnar epithelium, submucosal glands and cartilage (×100, H–E).
Fig. 6
The solid portion of the mass shows alveolar spaces filled with inflammatory cells (×200, H–E).
- References
- References
REFERENCES
1. Carter R. Pulmonary sequestration: collective review. Ann Thorac Surg 7:68. 1969.
[Article] [PubMed]2. Deparedes CG, Pierce WS, Johnson DG, Waldhausen JA. Pulmonary sequestration in infants and children: A 20-year experience and review of the literature. J Pediatr Surg 5:136. 1970.
[Article] [PubMed]3. Sade RM, Clouse M, Ellis FH Jr. The spectrum of pulmonary sequestration. Ann Thorac Surg 18:644. 1974.
[Article] [PubMed]4. Flye MW, Conley M, Silver D. Spectrum of pulmonary sequestration. Ann Thorac Surg 22:478. 1976.
[Article] [PubMed]5. Savic B, Birtel FJ, Tholen W, Funke HD, Knoche R. Lung sequestration: report of seven cases and review of 540 published cases. Thorax 34:96. 1976.
[Article] [PubMed] [PMC]6. Buntain WL, Woolley MH, Mahour CH, Issacs H, Payne V. Pulmonary sequestration in children: a 25 year experience. Surg 81:413. 1977.7. O’Mara CS, Baker RR, Jeyasingham K. Pulmonary sequestration. Surg Gynecol Obstet 147:609. 1978.
[PubMed]8. Stocker JT, Kagan-hallet K. Extralobar pulmonary sequestration: analysis of 15 cases. Am J Clin Pathol 72:917. 1978.
[Article]9. Pryce DM. Lower accessory pulmonary artery with intralobar sequestration of the lung: a report of seven cases. J Pathol Bacteriol 58:457. 1946.
[Article] [PubMed]10. Gerle RD, Jaretzki A, Ashley CA, Berne SA. Congenital bronchopulmonary foregut malformation: pulmonary sequestration communicationg with the gastrointestinal tract. N Engl J Med 278:1423. 1968.
[Article] [PubMed]11. Heithoff KB, Sane SM, Williams HJ, Jarvis DJ, Carter J, Kane P, Brennam W. Bronchopulmonary foregut malformation: a unifying etiological concept. Am J Roentgenol 126:46. 1976.
[Article]12. Zumbro GL, Treasure RL, Seitter G, Strevey TE, Brott W, Green DC. Pulmonary sequestration: a broad spectrum of bronchopulmonary foregut malformations. Ann Thorac Surg 20:161. 1975.
[Article] [PubMed]14. Levi A, Findler M, Dolfin T, Segni ED, Vidne BA. Intrapericardial extralobar pulmonary sequestration in a neonate. Chest 98:1014. 1990.
[Article] [PubMed]15. Flye MW, Izant RJ. Extralobar pulmonary sequestration with esophageal communication and complete duplication of the colon. Surgery 71:744. 1972.
[PubMed]