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Extralobar Sequestration Presenting as Massive Hemothorax

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DOI: 10.1378/chest.109.3.843 · Source: PubMed

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DOI 10.1378/chest.109.3.843 1996;109;843-845Chest

 Israel E. PrielVered Avishai, Eran Dolev, Dov Weissberg, Liliana Zajdel and Massive HemothoraxExtralobar Sequestration Presenting as

  http://chestjournal.chestpubs.org/content/109/3/843

and services can be found online on the World Wide Web at: The online version of this article, along with updated information 

ISSN:0012-3692)http://chestjournal.chestpubs.org/site/misc/reprints.xhtml(

without the prior written permission of the copyright holder.No part of this article or PDF may be reproduced or distributed3300 Dundee Road, Northbrook, IL 60062. All rights reserved. Copyright1996by the American College of Chest Physicians,Physicians. It has been published monthly since 1935.

is the official journal of the American College of ChestChest

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Figure 2. Intercostal arteriography performed after the bleedinghad been controlled. Tumor vessels draining into pulmonary veinare present.

DiscussionBalloon tamponade is a useful technique for treating he¬

moptysis. But inserting a balloon catheter through theworking channel of a flexible bronchoscope, as previouslyreported,12 has some demerits. First, insertion of a ballooncatheter into the working channel reduces the suctioncapacity and thus makes it difficult to attain a goodendoscopic view during the procedure, limiting the use ofthe technique to patients with slow bleeding. Second, a

special balloon catheter with reattachable valve2 or compli¬cated maneuver1 is necessary to remove the bronchoscopeand then inflate the balloon. The modified insertion tech¬nique provides a better view during the procedure becauseonly a guide wire needs to be inserted into the workingchannel, rendering minimal influence to suction capacity;this means that the technique could be applied to more

massive bleeding cases. It requires no special catheter or

complicated maneuver and is technically easy. We believemodified balloon tamponade is the preferred procedure in

hemoptysis patients whose bleeding is located at the lobarlevel or beyond.

References

Swersky RB, Chang JB, Wisoff BG, et al. Endobronchial balloontamponade of hemoptysis in patients with cystic fibrosis. AnnThorac Surg 1979; 27:262-64Freitag L. Development of a new balloon catheter for manage¬ment ofhemoptysis with bronchofiberscope. Chest 1993; 103:593

Extralobar SequestrationPresenting as MassiveHemothorax*Vered Avishai, MD; Eran Dolev, MD;Dov Weissberg, MD, FCCP; Liliana Zajdel, MD; andIsrael E. Priel, MD, FCCP

Pulmonary extralobar sequestration is a rare anomaly,usually diagnosed during the first months oflife. A case

of extralobar pulmonary sequestration in an adult,manifesting itselfas massive hemothorax, is presented.

(CHEST 1996; 109:843-45)

CCAM=cystic adenomatoid malformation

Key words: cystic adenomatoid malformation; extralobarsequestration; hemothorax

Bronchial sequestration is a relatively rare anomaly, diag¬nosed usually at infancy. Of the two forms of bron¬

chopulmonary sequestration, intralobar and extralobar, thelatter is less frequently encountered. We describe an adultwith extrapulmonary sequestration in whom massive spon¬taneous hemothorax was the presenting symptom.

Case ReportA 50-year-old healthy man was referred to the hospital because

of a complaint of pain in the lower area of the left side of the chestand the left area of the hypochondrium, 2 h following ergometryperformed as part of a periodic checkup. Physical examination re¬

vealed only a slightly distended abdomen. The patient was admit¬ted for observation. At the time of admission, the ECG was normaland remained so. A chest x-ray film demonstrated a moderatepleural effusion on the left side. Twenty-four hours later, dyspneaand elevation of body temperature to 38°C developed, and a dullpercussion sound with no air entry over the lower part of the leftlung was noted. Another chest x-ray film indicated a significant in¬crease in the amount of the pleural effusion.

Closed-tube thoracostomy yielded non-clotting blood. Since thepatient was experiencing chest pain and hemothorax on the left side,the possibility of dissection of the thoracic aorta was considered.Transesophageal echocardiography did not indicate any abnormalcardiac finding nor evidence of aortic dissection. However, a fairlylarge amount of pleural effusion was present on the left side, witha large partially moving mass within the fluid. A CT scan ofthe chestindicated the presence of a large left-sided pleural effusionsurrounding a mass-like lesion, containing fluid with irregular,high-density foci in its periphery (Fig 1). Angiography of the tho¬racic aorta, celiac trunk, and left lower intercostal arteries showedno evidence of dissecting aneurysm, bleeding, or abnormal vesselssupplying the lung tissue. The chest tube drained 2,800 mL bloodyeffusion within 48 h. Significant clinical improvement followed.However, following another episode ofchest pain, a subsequent CTscan of the chest showed a marked increase in the size of the mass,with a minimal amount of fluid surrounding it.

Subsequently, the patient underwent an explorative thoracot¬omy. A cystic mass was found at the lower aspect of the left lung,*From the Departments of Internal Medicine E. (Drs. Avishai,Dolev, and Pnel), Thoracic Surgery (Dr. Weissberg), and Pathol¬ogy (Dr. Zajdel), The Edith Wolfson Medical Center and SacklerSchool of Medicine, Tel Aviv University, Holon, Israel.

CHEST /109 / 3 / MARCH, 1996 843

 1996 BY THE AMERICAN COLLEGE OF CHEST PHYSICIANS by guest on July 13, 2011chestjournal.chestpubs.orgDownloaded from

Figure 1. Noncontrast CT scan of the lower part of the chestshows a large pleural effusion on the left. Enclosed in the fluid isa 5.2-cm inhomogenous, low-density, well-circumscribed roundmass with high-density foci in its periphery.

adherent to the diaphragm. It contained brown odorless fluid.Vascular connection to the thoracic aorta was found and divided.The lesion was resected. The pathologic finding consisted of a mass10 cm in diameter containing small cyst-like structures. The histo¬logic features established the diagnosis of extralobar lung seques¬tration exhibiting cystic adenomatoid malformation (CCAM) (Fig2). Postoperative recovery was uneventful.

DiscussionExtralobar sequestration, an entity in which an abnormal

segment of the lung enclosed within its own pleural mem¬brane is completely separated from the tracheobronchialtree, accounts for 25% of all pulmonary sequestrations, oc¬

curring predominantly in men with a ratio of 3 to 4:1.lA Itslocation, usually related to the left hemidiaphragm, may varybetween the lower lobe and the diaphragm, in the medi¬astinum, within the lung, in the pleural or pericardial spaces,or in the retroperitoneum.1'5

Arterial blood supply to the sequestered lung may origi¬nate from the thoracic or abdominal aorta (>80%); fromsmaller arteries, such as splenic, gastric, intercostal andsubclavian (15%); or from branches ofthe pulmonary arteryand systemic vessels (5%). The venous drainage usually isconnected to the azygos system; however, occasionally it canbe connected to the intercostal, portal, esophageal, or adre¬nal veins. Pulmonary venous drainage also is possible.4Intriguingly enough, the feeding vessel was not identified onthe angiogram, nor was it detected by an independent ret¬

rospective review of the films. One could only speculatewhether this was the result ofvasospasm, a sharp angle of exitfrom the aorta, or due to the fact that no technique is infal¬lible.

Extralobar sequestration, a developmental lesion, usuallypresents during the first 6 months of life (61%), often withdyspnea, cyanosis, and feeding difficulties.2 Recurrent pul¬monary infections (as in intralobar sequestration) or gas¬trointestinal symptoms in the presence of a communicationwith the gastrointestinal tract (bronchopulmonary foregutmalformation) are uncommon.

Very few cases of CCAM.a developmental anomalycharacterized by multiple cysts of varying sizes as well as a

¦y% h

i {/.*

Figure 2. Top: Cyst-like structures (hematoxylin-eosin, originalx80). Bottom: high-magnification of the ciliated pseudostratifiedepithelium covering a large cyst (hematoxylin-eosin, original x320).network of interconnecting spaces.were reported in theadult.6 Extralobar pulmonary sequestration that histologi¬cally displayed the features ofCCAM type 2 was reported in14 cases, 9 of which were thoracic and 5 of which were ab¬dominal.7 Clinical features of extralobar sequestration aloneor containing CCAM type 2 do not differ significantly.7The occurrence ofhemothorax unrelated to acute trauma

is quite rare. The major diagnostic categories to be consid¬ered include defects of coagulation, malignant tumors, andprimary vascular events.such as aortic dissection and pul¬monary vascular bleeding. Rarely, infections, endometriosis,and costal exostoses can cause hemothorax.8 In this patient,massive hemothorax was the presenting sign of extralobarpulmonary sequestration.The diagnosis of extralobar sequestration in adults is

rather difficult, perhaps because it is rarely thought of.9 It isintriguing that of 66 patients with extralobar sequestrationwho underwent surgery in the series of Savic et al,2 a correct

preoperative diagnosis was made only in 6. Spontaneoushemothorax from bronchopulmonary sequestration is ex¬

ceedingly rare and only has been reported in five cases.10"12To our best knowledge, all previously reported cases withspontaneous hemothorax occurred with intralobar seques¬tration.

In summary, a case of massive hemothorax resulting fromextralobar sequestration diagnosed at thoracotomy was pre-

844 Selected Reports 1996 BY THE AMERICAN COLLEGE OF CHEST PHYSICIANS

by guest on July 13, 2011chestjournal.chestpubs.orgDownloaded from

sented. Hemothorax in intralobar sequestration is exceed¬ingly rare and in extralobar sequestration has not been pre¬viously described.

ACKNOWLEDGMENTS: We thank D. Goncalves, MD, from theDepartment of Radiology, D. Harpaz, MD, from the Heart Insti¬tute at our hospital, and Z. Rubinstein, MD, from the Chaim ShebaMedical Center, Tel-Hashomer, Israel, for their help.

References1 Fraser RG, Pare JAP, Pare PD, et al. Pulmonary abnormalities of

developmental origin. In: Diagnosis of diseases of the chest. 3rded. Vol 2. Philadelphia: WB Saunders, 1989; 701-12

2 Savic B, Birtel FJ, Tholen W, et al. Lung sequestration: report of7 cases and review of 540 published cases. Thorax 1979; 34:96-101

3 Stocker JT. Congenital and developmental diseases. In: Dail DH,Hammer SP, eds. Pulmonary pathology New York: SpringerVerlag, 1988; 53-5

4 Sade RM, Clouse M, Ellis HF. The spectrum of pulmonary se¬

questration. Ann Thorac Surg 1974; 18:644-555 Felker RE, Tonkin ILD. Imaging of pulmonary sequestration.AJR 1990; 154:241-49

6 Avitabile AM, Hulnick DH, Greco MA, et al. Congenital cysticadenomatoid malformation ofthe lung in adults. Am J Surg Pathol1984; 8:193-202

7 Aulicino MR, Reis ED, Dolgin SE, et al. Intra-abdominal pul¬monary sequestration exhibiting congenital cystic adenomatoidmalformation: report of a case and review of the literature. ArchPathol Lab Med 1994; 118:1034-37

8 Martinez FJ, Villanueva AG, Pickering R, et al. Spontaneous he¬mothorax: report of 6 cases and review ofthe literature. Medicine1992; 71:354-66

9 Louie HW, Martin SM, Mulder DG. Pulmonary sequestration:17-year experience at UCLA. Am Surg 1993; 59:801-05

10 Beau A, Collignon P, Guillamot M, et al. Hemothorax spontane,revelateur d'une sequestration pulmonaire. Ann Chir Infant 1966;7:59-62

11 Laurin S, Aronson S, Schuler H, et al. Spontaneous hemothoraxfrom bronchopulmonary sequestration. Pediatr Radiol 1980; 10:54-6

12 Zapatero J, Baamonde C, Bellone JM, et al. Hemothorax as rare

presentation of intralobar sequestration. Scand J Thorac Cardio¬vasc Surg 1983; 17:177-79

Use of Somatostatin Analog forLocalization and Treatment ofACTH Secreting BronchialCarclnoid Tumor*Sophie Christin-Maitre, MD; Nathalie Chabbert-Buffet, MD;Agnes Mure, MD; Rafik Boukhris, MD; andPhilippe Bouchard, MD, PhD

A 29-year-old woman presenting with an ectopicadrenocorticotropic hormone syndrome and a noduleof the upper lobe of the left lung was explored by in-

*From the Units of Endocrinology (Drs. Christin-Maitre, Chab¬bert-Buffet, and Bouchard) and Nuclear Medicine (Dr. Mure),Hopital Saint Antoine, Paris, France; and Cinique Saint Augustin(Dr. Boukhris), Tunis, Tunisia.Manuscript received March 22, 1995; revision accepted August 30.Reprint requests: Dr. Bouchard, Hopital Saint-Antoine, 184 rue deFaubourg Saint-Antoine, 75012 Paris, France

dium 111 (mIn) octreotide scintigraphy. This showeda pathologic uptake by the nodule. Treatment withoctreotide resulted in the rapid control of hypercorti-solism prior to surgery. (CHEST 1996; 109:845-46)

Key words: bronchial carcinoid; Cushing's syndrome: ec¬

topic ACTH secretion; somatostatin receptor scintigraphy

Carcinoid tumors are responsible for 40% of cases ofadrenocorticotropic hormone (ACTH) dependent

Cushing's syndrome.1 Bronchial carcinoid tumors usuallyare small (0.3 to 1.3 cm) and are difficult to localize.2 In ad¬dition, hypercortisolism must be controlled prior to surgery.In this report, we describe, in a patient with ectopic ACTHsyndrome, the rapid normalization of hypercortisolism byoctreotide-only treatment, following the localization of a

bronchial carcinoid tumor by octreotide scintigraphy. Oct¬reotide is a somatostatin analog. This case report suggeststhat when a paraneoplasic ACTH-dependent hypercorticismis suspected, labeled pentetreotide scintigraphy may allowthe exact localization ofthe tumor. Furthermore, the uptakeofradioactive somatostatin (octreotide [Sandostatin; Sandoz;Bale, Switzerland]) suggests the possibility of treating pa¬tients with this drug.

Case ReportA 29-year-old woman was admitted to the hospital for the

exploration of a rapidly evolving hypercortisolism, discovered as thepatient complained of generalized edema, amenorrhea, and hy¬pokalemia (2.8 mmol/L). She weighed 80 kg and was 163-cm tall.She had a typical appearance of Cushing's syndrome. Biologicalevaluation confirmed the ACTH-dependent hypercortisolism. Re¬sults of a high-dose dexamethasone test were negative, and theplasma ACTH level did not increase after cortisol releasing factoradministration, suggesting an ectopic ACTH secretion (Table 1).

Table 1.Biological Evaluation Prior to Treatment, on

Day 24 of SMS Treatment, and 1 Week and 2 MonthsPostsurgery*Initial Treatment 1 Week 2 Months

Values Value Day 24 Postsurg PostsurgUFC, nmol/24 h

Plasma cortisol, nmol/L8h

16 h

Dexamethasone test*Plasma ACTH,pg/mL, basalCRF test*

Plasma LPH,pg/mL

806(<260)

1,493(220-610)

1,103(100-290)

1,166214

(9-52)2461,674

(46-124)

13

116

101

24

123

18

66

33

151

211

32

* Surgery took place on treatment day 60. Normal value ranges are

shown in parentheses. UFC=urinary free cortisol; LPH=lipotropinhormone; pg=picogram.2-day high-dose dexamethasone suppression test (8 mg dexametha-sone/d).

* Corticotropin-releasing factor, 100 pg administered intravenously.

CHEST /109 / 3 / MARCH, 1996 845

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DOI 10.1378/chest.109.3.843 1996;109; 843-845Chest

E. PrielVered Avishai, Eran Dolev, Dov Weissberg, Liliana Zajdel and Israel

Extralobar Sequestration Presenting as Massive Hemothorax

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