(Chest. 2004;125:1944-1947.)
© 2004
American College of Chest Physicians
Hemoptysis in a 38-Year-Old Woman Receiving an Oral Contraceptive*
Mehrdad M. Behnia, MD, FCCP;
Phillip W. Catalano, MD and
William S. Brooks, MD
* From St. Joseph Hospital, Augusta, GA.
Correspondence to: Mehrdad M. Behnia, MD, 1500 Johns Rd, Suite 5, Augusta, GA 30904; email: mb{at}behnia.org
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Introduction
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A 38-year-old woman presented with intermittent chest pain and hemoptysis of 1 week in duration, producing less than a cup of blood throughout the week. She denied constitutional symptoms, dyspnea, or orthopnea. Her medical history was unremarkable. She was receiving oral contraceptives and smoked cigarettes. On physical examination, she was afebrile. Bronchial breath sounds and crackles were audible at the left base. The rest of her examination was negative.
Remarkable initial laboratory data demonstrated a hematocrit of 51%, total bilirubin of 1.4 mg/dL, and room air arterial PaO2 of 100 mm Hg and PCO2 of 36 mm Hg. ECG and cardiac enzymes did not suggest myocardial injury. Chest radiography revealed a questionable developing density in the left infrahilar region (Fig 1
). Spiral CT showed an aberrant vessel off the left side of the descending aorta (Fig 2
) with some consolidation in the left lower lobe. No filling defect in the pulmonary circulation suggestive of an embolus was seen.
Bronchoscopy displayed oozing of blood from the medial segment of left lower lobe. Culture of bronchoalveolar washing showed mixed respiratory flora with negative fungal and acid-fast bacteria culture results. Hemoptysis spontaneously abated, and within 48 h, the patient was ready to be discharged home receiving oral antibiotics. A diagnostic test was ordered.
What is your diagnosis? What diagnostic modality and what therapeutic intervention would you recommend next?
Answers: Pulmonary sequestration; aortogram and surgical resection of sequestered segment.
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Discussion
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Sequestration is an infrequent congenital deformity of the bronchopulmonary tree, in which a region of lung parenchyma is isolated, or sequestered, from the remainder of lung and its blood supply is derived from an anomalous branch of aorta, not the pulmonary artery. Of the two types, extralobar and intralobar, the former has its own dedicated pleura and the latter shares a common visceral pleura with the rest of normal lung. Extralobar sequestration is rare and is typically associated with other inborn abnormalities, such as diaphragmatic hernia, cystic adenomatoid malformation, and communication with lower esophagus and stomach.
Of the sequestration theories, one postulates that a branching element of developing bronchopulmonary tree may become disconnected from the rest of lung during embryogenesis. The blood supply of the separated branch fails to develop because it is not in contact with the developing pulmonary circulation. Therefore, the isolated fragment relies on systemic circulation for its blood supply. If this occurs early in embryogenesis, the branch is retained within the pleura, hence becoming an intralobar sequestration.
Our patient had intralobar sequestration, which is the more common type (three fourths of all sequestrations) with an equal distribution among male and female patients. The sequestered lobe is mainly in the medial or posterior segments of left lower lobe. Arterial supply is usually from the aorta, but it can rarely be derived from coronary arteries. Uncommonly, the venous drainage is through veins other than pulmonary veins. Dissimilar to extralobar sequestration, it is infrequently associated with developmental anomalies. The sequestered intralobar parenchyma can become infected because of distorted anatomy, poor aeration, and abnormal drainage, conceivably by migration of bacteria through pores of Kohn.
The patient may be asymptomatic or present with cough, fever, recurrent pneumonias, or hemoptysis with onset often during adolescence, although half of the patients become symptomatic after the age of 20 years. The presentation may be mistaken for bronchiectasis or lung abscess. If the sequestered segment is not resected, infections can become recurrent, leading to necrotizing pneumonia, septicemia, and massive hemoptysis. Sequestration can potentially exacerbate congestive heart failure in subjects with valvular disease and cardiac dysfunction.
The diagnosis of sequestration is contingent on good imaging technique. The chest radiograph has a poor sensitivity and may appear normal to the inexperienced reader. Spiral CT angiography of chest has greater sensitivity because of clear visualization of pulmonary vessels, but it should be followed by an aortogram for definitive localization of the aberrant vessel and surgical planning. Magnetic resonance angiography is an excellent noninvasive tool that can map a three-dimensional configuration of the abnormal artery and the draining vein; its limitations are cost, motion artifact, and difficulty in obtaining high-resolution images of the small vasculature.
The definitive treatment entails surgical resection either through traditional thoracotomy or video assistance. Coil embolization of the feeding artery to ensure safe resection prior to surgery has been reported, but is not routinely required. Regardless of technique, surgery is the only curative option with excellent long-term outcome.
Sequestration, and not just pulmonary embolism, must be a strong consideration in a young patient with hemoptysis. Therefore, a normal or low-probability ventilation-perfusion scan should not dissuade the clinician from ordering spiral CT and/or aortography.
Clinical Course
Prior to discharge, the aortogram showed an aberrant vessel from the aorta that appeared to supply the pulmonary sequestration (Fig 3
). The patient returned within 2 days of discharge with fever, leukocytosis, and hypoxemia. Chest radiography showed left basilar consolidation consistent with pneumonia. CT showed air-fluid interface within the consolidation raising the issue of tissue necrosis. The pneumonia was treated with broad-spectrum IV antibiotics, and the patient was discharged home in stable condition. After resolution of the pneumonia, she underwent dissection of the medial aspect of left lower lobe. A 2 cm in diameter branch of aorta was encountered feeding the sequestered segment. The branch was ligated and dissection performed. Pathology revealed collapse of normal lung structure with obliteration of alveolar spaces and chronic inflammatory exudate. Histology was consistent with bronchiolitis obliterans organizing pneumonia.
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Clinical Pearls
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- Hemoptysis in a young patient should raise suspicion for intralobar pulmonary sequestration.
- Chest radiograph and even ventilation-perfusion scan may appear normal in pulmonary sequestration.
- Spiral CT angiogram followed by aortogram should be obtained in the evaluation of possible sequestration.
- The sequestered segment must be surgically removed to prevent complications, such as hemoptysis, necrotizing pneumonia, and septicemia.
Received for publication April 29, 2003.
Accepted for publication October 22, 2003.
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Suggested Readings
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- Conran, RM, Stocker, JT (1999) Extralobar sequestration with frequently associated congenital cystic adenomatoid malformation, type 2: report of 50 cases. Pediatr Dev Pathol 2,454-463[CrossRef][ISI][Medline]
- Dinkel, HP, Hoppe, H, Striffeler, HU, et al Preoperative arterial embolization of intralobar lung sequestration. Radiologe 2001;41,1001-1004[CrossRef][ISI][Medline]
- Do, KH, Goo, JM, Im, JG, et al Systemic arterial supply to the lungs in adults: spiral CT findings. Radiographics 2001;21,387-402[Abstract/Free Full Text]
- Fabre, OH, Porte, HL, Godart, FR, et al Long-term cardiovascular consequences of undiagnosed intralobar pulmonary sequestration. Ann Thorac Surg 1998;65,1144-1146[Abstract/Free Full Text]
- Franco, J, Aliaga, R, Domingo, ML, et al Diagnosis of pulmonary sequestration by spiral CT angiography. Thorax 1998;53,1089-1092[Abstract/Free Full Text]
- Khalil, KG, Kilman, JW Pulmonary sequestration. J Thorac Cardiovasc Surg 1975;70,928-937[Abstract]
- Rubin, EM, Garcia, H, Horowitz, MD, et al Fatal massive hemoptysis secondary to intralobar sequestration. Chest 1994;106,954-955[Abstract/Free Full Text]
- Samuel, M, Burge, DM Management of antenatally diagnosed pulmonary sequestration associated with congenital cystic adenomatoid malformation. Thorax 1999;54,701-706[Abstract/Free Full Text]
- Savic, B, Birtel, FJ, Tholen, W, et al Lung sequestration: report of seven cases and review of 540 published cases. Thorax 1979;34,96-101[ISI][Medline]
- Wan, IY, Lee, TW, Sihoe, AD, et al Video-assisted thoracic surgery lobectomy for pulmonary sequestration. Ann Thorac Surg 2002;73,639-640[Abstract/Free Full Text]
- Zhang, M, Zhu, J, Wang, Q, et al Contrast enhanced MR angiography in pulmonary sequestration. Chin Med J (Engl) 2001;114,1326-1328
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Introduction
Discussion
