Spectrum of Pulmonary Sequestration M. Wayne Flye, M.D., Martin Conley, M.D., and Donald Silver, M.D.

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1 Spectrum of Pulmonary Sequestration M. Wayne Flye, M.D., Martin Conley, M.D., and Donald Silver, M.D. ABSTRACT Bronchopulmonary sequestration was diagnosed in 17 patients ranging in age from newborn to 64 years. The sequestration was intralobar in 14 patients and extralobar in 3. The spectrum of symptoms could be divided into three patterns: no symptoms (6 patients), respiratory problems (8 patients), and cardiovascular problems (3 patients). Cardiovascular problems usually manifest themselves in the first few weeks or months of life and often have a respiratory component. In older patients the sequestration is first manifested by recurrent pulmonary infections or, if it remains uninfected, an asymptomatic density on chest roentgenogram. The definitive diagnostic study is arteriography. Operative treatment for the intralobar variety consists of segmental resection or, if the inflammatory process is more extensive, lobectomy. An extralobar sequestration may simply be excised. The term pulmonary sequestration is applied to a portion of lung that is separated completely (extralobar) or incompletely (intralobar) from normal functioning lung during early embryological development. It is supplied by an anomalous systemic artery that arises from the aorta or its branches between the base of the neck and the upper abdomen. The available evidence indicates that both types of sequestration develop as congenital foregut anomalies [8, 12, 15, 18, 211. Typically the sequestration is discovered when the patient develops respiratory symptoms secondary to infections or associated cardiac anomalies. Some patients, however, remain asymptomatic, and the sequestraticm is suggested by a chest roentgenogram. This report of a series of pulmonary sequestrations demonstrates the spectrum of clinical manifestations that accompany the anomaly. From the Department of Surgery, Duke University Medical Center, Durham, NC. Accepted for publication Jan 12, Address reprint requests to Dr. Silver, Department of Surgery, University of Missouri School of Medicine, Columbia, MO Clinical Material Between 1960 and 1975, bronchopulmonary sequestration was diagnosed in 17 patients at the Duke University Medical Center (Table); during this period 11,120 patients were evaluated on the Cardio-Thoracic Service. The patients ranged in age from newborn to 64 years, and 10 of the 17 patients were male. The patients demonstrated one of three classic patterns: no symptoms, respiratory symptoms, or cardiovascular problems. In 3 patients the sequestration was extralobar. In the 14 patients with the intralobar variety the sequestration was confined to the lower lobes-8 on the left and 6 on the right. The vascular supply to the sequestration was by way of a single large systemic artery arising from either the thoracic or the abdominal aorta in all but 4 patients. In 3 of these 4 patients multiple smaller arteries from the aorta supplied the sequestration, while in a newborn with extralobar sequestration, postmortem examination demonstrated a single artery arising from the superior mesenteric artery. Venous drainage of the sequestration was through the inferior pulmonary vein to the left atrium in all but l patient, who had a left lower lobe intralobar sequestration that drained through a single intercostal vein above the diaphragm. The vascular anatomy was demonstrated by angiography in 8 patients and by postmortem examination in 2. In 7 patients the diagnosis was made at the time of operation; the correct diagnosis had been suspected preoperatively in 1 of them. In 3 of the 7 an asymptomatic, rounded density had been identified on chest roentgenogram, 2 patients had infected cavities, l had bronchiectasis by bronchogram, and 1 had a rounded density and chest pain. Respiratory symptoms alone were present in 8 patients, 6 of whom gave a history of recurrent pneumonia. In 1 of the 6 patients with pneumonia, bronchography demonstrated bronchiectasis in the area of the sequestration; another patient had hemoptysis. In a seventh patient the 478

2 479 Flye, Conley, and Silver: Spectrum of Pulmonary Sequestration Pulmonary Sequestration in 17 Patients, No. of Patients Age Symptoms Location No. of &Type Arteries Treatment Results Cardiovascular Problems 3 Birth-22 mo Congestive heart failure, 2; RLL, 21; Single, all 3 Ligation, 2; Died, 3 bilateral pneumothorax, 1 R, 1E none, 1 Respiratory Problems yr Recurrent pneumonia, 6; RLL, 31; Single, 6; Lobectomy, 6; Improved, 7; chest pain, 1; LLL, 31; three, 2 pneumonectomy, 1; died, 1 respiratory distress, 1 R, 1E none, 1 L, 1E Asymptomatic yr None, 5; RLL, 11; Single, 4; Lobectomy, 3; Improved, 6 heart murmur, 1 LLL, 51 two, 2 segmentectomy, 2; none, 1 RLL = right lower lobe; R = right pleural cavity; LLL = left lower lobe; L = left pleural cavity; I = intralobar; E = extralobar. abnormality was discovered on chest roentgenogram while anterior chest pain was being evaluated, and in 1 newborn the diagnosis was made at postmortem examination. The associated cardiovascular problems consisted of septal defects in 2 patients (1 ventricular, 1 atrial) and total anomalous pulmonary return with cor biloculare in a third. In the patients with septal defects, the extent to which shunting through the sequestration contributed to the development of congestive heart failure could not be determined. A 24-month-old child with an ostium primum defect did undergo ligation of the anomalous artery alone with symptomatic improvement of congestive heart failure. This patient, however, died at home two months later of uncertain cause. A second patient, with ventricular septal defect and peripheral pulmonic stenosis, died shortly after closure of the VSD and ligation of the anomalous artery. The third patient, who had cor biloculare, died of bilateral pneumothoraces several hours after birth, and the diagnosis was made at postmortem examination. Six of the patients were asymptomatic and ranged in age from 2 to 64 years. In the 2-yearold the sequestration was discovered during cardiac catheterization for evaluation of a heart murmur. The remaining 5 patients were found to have asymptomatic masses by chest roentgenogram. Nine of the 17 patients underwent lobectomy, 2 had segmental resection, 1 had a pneumonectomy, and the 2 patients with septal defects had simple ligation of the systemic artery without removal of the sequestration. In 2 newborns the diagnosis was made at postmortem examination. A 56-year-old woman with an asymptomatic sequestration demonstrated by arteriography refused operation and is currently being followed (Fig 1). All surviving patients have remained asymptomatic. Postoperative complications occurred in 3 patients. A persistent pleural air leak necessitating chest tube suction for fourteen days developed in a 26-year-old woman. Mediastinal tamponade and death six hours following ligation of the anomalous artery and repair of a VSD occurred in a 14-week-old boy. A 32-year-old man developed massive postoperative bleeding following right lower lobectomy for an asymptomatic intralobar sequestration. A preoperative arteriogram had demonstrated that the arterial blood supply was derived from the intercostal and right inferior phrenic arteries (Fig 2). Multiple vessels were encountered in the area where the sequestration adhered to the chest wall. Despite care in ligating them, on reexploration one of these arteries was found to have retracted and was freely bleeding. Ten units of blood were required for transfusion. The patient s postoperative course was sub-

3 480 The Annals of Thoracic Surgery Vol 22 No 5 November 1976 A B Fig 1. (A)Asymptomaticleft lower lobe multilobular sequestration in a 56-year-old woman. (B) Barium swallow reveals no esophageal communication. (C) Selective arteriography demonstrates a single large systemic artery arising from the thoracic aorta and supplying the sequestration. sequently uncomplicated, and he has remained asymptomatic. C Comment Pulmonary sequestration can be viewed as disruption of morphogenesis in the region of the primitive foregut. Although most cases conform to the common features described for the intralobar and extralobar types of sequestration, many anatomical locations, types of vascular supply, and associated gastrointestinal and diaphragmatic anomalies have also been reported which are not well explained by any of the proposed theories [5,10,161. Blesovasky 111 maintains that these are variants of the same process and that an embryonic organizer defect must be the fundamental lesion. The spectrum of clinical symptoms is determined by the combination of anatomical anomalies. Although many lesions may remain asymptomatic, others will develop symptoms.

4 481 Flye, Conley, and Silver: Spectrum of Pulmonary Sequestration A B Fig 2. (A) Asymptomatic right lower lobe sequestration with an air-fluidleuel in a 32-year-old man. (B) Anteroposterior and (C) lateral uiezus ofa selective arteriogram demonstrate that the systemic arterial bloodsupply arisesfrom the right intercostal and inferior phrenic arteries. C Cardiovascular symptoms may result from leftto-right shunting from the systemic artery through the sequestration into the pulmonary venous circuit. In this setting the shunt may be corrected by excision of the sequestration [14, 191 or, if indicated in a critically ill infant with other anomalies, simple ligation of the systemic artery, as was done in 2 of our patients. Hemodynamic symptoms generally become manifest within the first few weeks or months of life. In the older age group, symptoms are almost invariably related to the presence of an airway communication. Recurrent fever, chills, and purulent sputum production are the most common signs. Bronchial communication is virtually unknown in extralobar sequestration [3], although symptoms have been reported secondary to progressive enlargement when a gastrointestinal communication persists [7]. Similarly, bronchial communication is initially absent in intralobar sequestration as well [13]; however, secretion of mucus within an intralobar sequestration causes a cystic swelling with subsequent compression and atelectasis of

5 482 The AM& of Thoracic Surgery Vol 22 No 5 November 1976 the surrounding normal lung tissue. Superimposed infection then spreads easily to the sequestration itself, causing capsular erosion and airway communication and thus producing the clinical picture typically associated with pulmonary sequestration. Any indolent process seen on chest roentgenogram should alert one to the possibility of sequestration, especially if the posterior basilar region is involved [31. If a pneumonic process is present, a multicystic lesion may be confused with pneumatoceles and operation consequently may be delayed. Bronchography may show displacement of adjacent segmental bronchi. The definitive diagnostic study, however, is the demonstration of a systemic arterial blood supply by arteriography [171. Treatment of intralobar sequestration generally is operative resection, preferably after treatment of the acute inflammatory process. Lobectomy has been required in the majority of patients because of changes secondary to infection. Segmentectomy, however, has in 15 to 20% of cases been shown to be technically feasible without additional morbidity [201. If an intralobar sequestration is nonaerated and the arteriogram is pathognomonic, nonoperative management may be considered under unusual circumstances [213. In the case of diagnosed extralobar sequestration, observation rather than operation may be more strongly considered because of the very low incidence of associated problems. The surgeon operating for any cystic or suppurative process within the thorax must be aware of the diagnosis of sequestration and the possibility of unusual vascular connections or unsuspected gastrointestinal communications. The occasional fatal consequence of inadvertent transection of an anomalous systemic artery has been well documented 12, 4, 6, 9, 111. References Blesovasky A: Pulmonary sequestration: a report of an unusual case and a review of the literature. Thorax 22:351, 1967 Butler EF: Discussion of HJ Moersch, OT Clagett: Pulmonary cysts. J Thorac Surg 16:179, 1947 Carter R: Pulmonary sequestration (collective review) Ann Thorac Surg 7:68, Douglas R: Anomalous pulmonary vessels. J Thorac Surg 17:712, Eppinger J, Schauenstein W: Krankheiten der lungen. Ergeb Allg Pathol 1:267, Findlay CW, Maier HC: Anomalies of pulmonary vessels and their surgical significance with review of literature. Surgery 29:604, Flye MW, Izant RJ: Extralobar pulmonary sequestration with esophageal communication and complete duplication of the colon. Surgery 71:744, Gerle RD, Jaretzki A 111, Ashley CA, et al: Congen ital bronchopulmonary-foregut malformation: pulmonary sequestration communicating with the gastrointestinal tract. N Engl J Med 278:1413, 1968 Golding MR, Kwon K, Chiu CJ, et al: Pulmonary sequestration: a report of an unusual case. J Thorac Cardiovasc Surg 54:121, 1967 Halasz NA, Lindskog GE, Liebow AA: Esophagobronchial fistula and bronchopulmonary sequestration. Ann Surg 155:215, 1962 Harris HA, Lewis I: Anomalies of the lungs with special reference to the danger of abnormal vessels in lobectomy. J Thorac Surg 9:666, 1940 Iwai K, Shindo G, Hajikano H, et al: Intralobar pulmonary sequestration with special reference to developmental pathology. Am Rev Resp Dis 107:911, 1973 Kyllonen KEJ: Intralobar pulmonary sequestration and a theory as to its aetiology. Acta Chir Scand 127:307, 1964 Litwin SB, Plauth WH Jr, Nadas AS: Anomalous systemic arterial supply to the lung causing pulmonary artery hypertension. N Engl J Med 283:1098, 1970 Pendse P, Alexander J, Khademi M, et al: Pulmonary sequestration. J Thorac Cardiovasc Surg 64:127, 1972 Pryce DM: Lower accessory pulmonary artery with intralobar sequestration of lung. J Pathol Bacteriol 58:457, 1946 Ranninger K, Valvassori GE: Angiographic diagnosis of intralobar pulmonary sequestration. Am J Roentgen01 Radium Ther Nucl Med 92:540,1964 Sade RM, Clouse M, Ellis FH Jr: The spectrum of pulmonary sequestration (collective review). Ann Thorac Surg 18:644, 1974 Solit RW, Fraimow W, Wallace S, et al: The effect of intralobar pulmonary sequestration on cardiac output. J Thorac Cardiovasc Surg 49:844, 1965 White JJ, Donahoo JS, Ostrow PT, et al: Cardiovascular and respiratory manifestations of pulmonary sequestration in childhood. Ann Thorac Surg 18:286, 1974 Zumbro GL, Treasure RL, Seitler G, et al: Pulmonary sequestration. Ann Thorac Surg 20:161,1975