Endobronchial Hamartoma Associated with Bronchioloalveolar Cell Carcinoma

mediastinum before terminating in the superior cervical ganglion located at the angle of the jaw medial to the carotid artery and juxtaposed to the carotid sheath. The third-order or postganglionic neurons arise in the superior cervical ganglion and run on the common carotid and internal carotid arteries as a plexus ascending the internal carotid artery to enter the cavernous sinus joining the ophthalmic division for the trigeminal nerve to the orbit. The postganglionic fibers to the face that regulate sweating do not enter the skull but follow the external carotid artery" We postulate that this patients transient Homers syndrome resulted from either irritation to the nerve from instillation of hypertonic (3 percent saline) fluid into the mediastinum or from pressure on the nerve in the mediastinum prior to spontaneous decompression of blood and fluid into the pleural space. An alternative explanation is that the central venous catheter itself caused direct neural irritation. As described previously 1 the presence of a relatively stif( large-bore (8 French) percutaneous introducer used for central venous access most likely predisposed the patient to this complication. Although seen most commonly with catheterization of the external jugular vein, migration into the mediastinum has been reported with insertion of a central venous catheter from any approach. The right subclavian approach, in particular, has an acute angle at the point where the right subclavian vein meets the right brachiocephalic vein, making this a likely point for extravascular migration. Homers syndrome is a recognized complication of carotid arterial catheterization and has been reported infrequently following internal and external jugular cannulation. This usually is ascribed to pressure on the postganglionic fibers in the carotid plexus. 4 Other iatrogenic causes are those associated with manipulation of the pulmonary apex, such as with tube thoracostomy" To our knowledge, there are no reports of Homers syndrome from subclavian catheterization or hydromediastinum, but this may be underdiagnosed, particularly in critically ill patients. The pleural fluid was sampled to exclude other potentially serious causes of pleural effusion, such as empyema. The presence of an RBC of 4O,OOO/ml was likely caused by hemorrhage from the involved vessel. The levels of protein (2.3 g/dl) and LDH (85 IV/dl) are consistent with a transudative effusion, although both were considerably higher than the infusate, which contained no protein. This may have resulted from increased permeability of the mediastinal pleural vessels induced by the hypertonic infusion and the subsequent equilibration with the interstitial spaces of the mediastinum and pleura. The ·diagnosis of migration of a central venous catheter may be subtle and, if not discovered, can progress to respiratory failure or vascular compromise with infusion of fluid into the mediastinum and pleural space. The acute occurrence of an ipsilateral Homers syndrome without obvious cause may be an early clue to this diagnosis and warrants further investigation. Absence of blood return and the observation of fluid similar to the infusate on aspiration of the central venous catheter is diagnostic of extravascular migration of the catheter. A chest roentgenogram should be obtained to assess the location of the central venous catheter 1084

and to search for mediastinal widening or pleural effusion. When the diagnosis is established the infusion should be stopped and the central venous catheter removed. REFERENCES

1 Chute E, Cerra FB. Late development of hydrothorax and hydromediastinum in patients with central venous catheters. Crit Care Med 1982; 10:868-69 2 Moorthy SS, McCammon RL, Deschner WI: Fishel C. Diagnosis and management of mediastinal migration of central venous pressure catheters. Heart Lung 1985; 14:80-3 3 Giles CL, Henderson JW Homers syndrome: an analysis of216 cases. Am J Ophthalmoll958; 46:289-96 4 Teich SA, Halprin SL, Tay S. Homers syndrome secondary to Swan-Ganz catheterization. Am J Med 1985; 78:168-70 5 Kahn SA, Brant LJ. Iatrogenic Homers syndrome: a complication of thoracostomy tube replacement (letter), N Eng} J Med 1986; 312:245

Endobronchial Hamartoma Associated with Bronchioloalveolar cell Carcinoma* Dominique Mompoint, M.D.;t Odile Growsard, M.D.;* Philippe G~niet; M.D.§

Computed tomographic 6ndings in a 60-year-old man with lobar broncbioloalveolar cell carcinoma distal to endobronchial hamartoma are described. (Cheat 1988; 94:1094-96)

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omputed tomography (CT) findings in a case of central, endobronchial hamartoma associated with lobar bronchioloalveolar carcinoma in the subtended lung parenchyma are described. This pathologic association has not, to our knowledge, been previously reported. CASE REpORT

A 60-year-old male smoker, with a history of chronic obstructive pulmonary disease, presented with loss of weight and hemoptysis of three months' duration following an episode ofbronchial infection. Chest roentgenograms showed a persistent ill-defined alveolar consolidation in apical-posterior and inferior lingular segments of the left: upper lobe (Fig 1). Bronchoscopic examination revealed a polypoid, elastic, mobile mass arising from the posterior wall of the left: upper lobe bronchus, extending to its superior division and occluding the lingular bronchus. Bronchoscopic biopsy was negative for cancer. Computed tomographic scans showed a smooth-edged endobronchial intraluminal left: upper lobe mass, 2 cm wide, extended to the lingular lumen; thin-section scans revealed small foci of fat inside the mass and no detectable calcium deposits (Fig 2~ The diagnosis of endobronchial hamartoma was established by CT on the evidence of fat within the lesion. The alveolar consolidation visible in the upper and lower segments of the left upper lobe was considered obstructive pneumonitis. Left: upper lobectomy was performed. The examination of he specimen disclosed a pedunculated endobronchial tumor, 2 cm long, obliterating the apicodorsal bronchus. The tumor base was From the Departments of tRadiology and *Anatomopatholo~ Beaujon Hospital, Clich~ and §Bondy Hospital, Bondy France.

Endobronchial Hamartoma and BronchloloaJveolar Cell Carcinoma(Mompolnt, Grosaard, Grenier)

FIGURE 1. Posteroanterior chest film shows an ill-defined opacity in left upper lobe . implanted on the bifurcation of this bronchus; the head emerged at the surgical section. This white, smooth, firm tumor had a chondroid aspect. There was no wall infiltration; the pulmonary parenchyma of the dorsal and apical segments was infiltrated by an ill-defined, whitish, tumorous tissue (Fig 3). The endobronchial tumor was a hamartochondroma composed of a central, cartilaginous, dystrophic area associated with fibrous and adipose tissue . The surface was lined by a normal bronchial epithelium. The infiltrated dorsal and apical regions corresponded to a bronchioloalveolar cell carcinoma, with mucin-producing cells lining alveolar walls of normal thickness. A lymph node draining the apicodorsal bronchus was involved with carcinoma. DISCUSSION

Thoracic hamartoma, including central, intrabronchial hamartoma, rarely encountered in comparison to the more common intrapulmonary variety, can be confidently diagnosed with CT. Siegelman et al,' using thin-section cr, described the criteria for pulmonary hamartoma (diameter of 2.5 ern or less, smooth edge , focal collections offat or fat

FIGURE 3. Pathologic specimen. Pedunculated endobronchial hamartochondroma implanted on the bifurcation between apical and dorsal bronchi (arTOW ~ To the left, the bronchioalveolar carcinoma accounts for the whitish infiltration of the parenchyma.

alternating with areas of calcification). The same criteria can be confidently extended to endobronchial hamartoma because of its similar histologic composition .2 Chronic lobar consolidation is a rare pattern of bronchioloalveolar cell carcinoma.v' This neoplasm represents 5 percent of primary lung carcinomas.' Bronchioloalveolar earinoma usually appears radiographically as a unique localized nodule or mass, diffuse nodules, or diffuse areas of consolldadon.sRadiographically, chronic lobar consolidation is nonspecific and may be encountered in a variety of situations, including pneumonia, obstructive pneumonia, lymphoma, and tuberculosis. According to 1m et al,' an air bronchogram made of narrow, elongated , rigid bronchi is a characteristic CT finding in infiltrative lobar bronchioloalveolar carcinoma. The prognosis oflobar bronchioloalveolar carcinoma is poor, comparable to that of diffuse disease.' Parenchymal consolidation distal to an endobronchial lesion is usually assumed to be secondary to the bronchial obstruction caused by the lesion. However, in rare circumstances, the possibility of coexistent abnormalities should be kept in mind . Alveolar cell carcinoma has been reported in association with scleroderma, diffuse interstitial fibrosis, and mineral oil aspiration pneurnonia.v' Extensive cicatrization often accompanies bronchioloalveolar cell carcinoma," in up to 50 percent of cases, according to some researchers. Thus, one is inclined to evoke the possibility in this case that postobstructive pneumonia led to alveolar cell carcinoma . However, since there are no other reports of alveolar cell carcinoma distal to an obstructing neoplasm , their coexistence should be viewed as coincidental. REFERENCES

FIGURE 2. cr scan at the level of left upper lobe bronchus shows endoluminal, rounded mass with areas of low density (-30 UH) (arTOW~ A calcification on the anterior bronchial wall is visible .

Siegelman 55, Khouri NF, Scott WW Jr, Leo Fp, Hamper UM, Fishman EK , Zerhouni EA. Pulmonary hamartoma: CT findings. Radiology 1986;160:313-17 2 Bateson EM . Relationship between intrapulmonary and endobronchial cartilage containing tumors (so-called hamartomata). Thorax 1965;20:447-61 3 Hill CA. Bronchioloalveolar carcinoma: a review. Radiology 1984;150:15-20 CHEST / 94 / 5 / NOVEMBER . 1988

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4 Epstein DM, Gefter WB, Miller WI: Lobar bronchioalveolar cell carcinoma. AJR 1982;139:463-68. 5 1m JG, Choi BY, Chang KH, Yeon KM, Han MC, Kim CW cr findings of lobar bronchioloalveolar carinoma. J Comput Assist Tomogr 1986;10:320-22 6 Fraser RG, Pare JAE Diagnosis of disease in the chest. Philadelphia: WB Saunders, 1978:1055 7 Felson B, Ralaisomay G. Carcinoma of the lung complicating lipoid pneumonia. AJR 1983;141:901-907 8 Naidich D~ Zerhouni EA, Hutchins GM, Genieser NB, McCauley DI, Siegelman SSe Computed tomography of the pulmonary parenchyma. Distal air-space disease. J Thorac Imag

1985;1:39-53

not distended or paradoxic. Attempts at plastic repair were unsuccessful, and the tricuspid valve was replaced with a 3M StarrEdwards caged-ball prosthesis. The valve was inserted into the true tricuspid annulus below the coronary sinus orifice. Complete heart block ensued with an escape nodal rhythm at a rate of about 5OIm'. No pacing was necessary postoperatively and the patient became asymptomatic, tolerating heart block well, with regression of cardiomegaly In 1967, however, a permanent epicardial pacemaker was implanted. From 1968 through 1979, the patient underwent subsequent operations for pacemaker complications, without problems thereafter. He is a very active man who leads a normal life, hunting, fishing, and bicycling, besides working full time. His only medication is warfarin, which is carefully monitored. DISCUSSION

Tricuspid Valve Replacement for Ebsteln's Anomaly In Childhood with a Starr-Edwards Caged-Ball Prosthesis· 21-y88r Follow-up Francuco Di LeUo, M.D.; RobertJ FlemmD, M.D.; Donald C. Mullen, M.D.; Leonard H. Kleinman, M.D.; and lbul H. Werner; M.D., F.C.C.P.

A II-year follow-up after tricuspid valve replacement (TVR) with a Starr-Edwards caged-ball prosthesis in a ten-year old boy is described. TVR is performed for EbsteinS anomaly, with strict indications in childhood. Despite the current preference for bioprostheses, good performance can be expected from the Starr-Edwards caged-ball valve. (Chat 1988; 94:1096-97)

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ricuspid valve replacement for Ebsteins anomaly was first performed in 1962,1 and variable results were subsequently reported. CASE REpORT

A four..year-old boy was admitted in 1960 with a heart murmur and history of one cyanotic spell at age six weeks. The heart was not enlarged. The ECG suggested right ventricular hypertrophy Cardiac catheterization disclosed significant valvular pulmonic stenosis, patent foramen ovale, and trivial tricuspid regurgitation. Being very young and asymptomatic, the patient was discharged and followed. Progressive exercise intolerance and cardiomegaly mandated a second catheterization in 1962, confirming the previous diagnosis but showing increased tricuspid regurgitation with marked right atrial enlargement Open pulmonic commissurotomy and closure of the patent foramen ovale were performed. EbsteinS anomal~ noted at surgery was not corrected because of the small size of the patient and the feeling that relief of the right ventricular obstruction would decrease the amount of tricuspid regurgitation. Wonening congestive heart failure from massive tricuspid regurgitation mandated a second operation in 1966. Surgery revealed a septal leaflet that was severely malformed, hypoplastic, and displaced into the right ventricle. The atrialized right ventricle was -From se Lukes and Affiliated Hospitals of the Medical College of Wisconsin, Milwaukee Reprint "'quem: Dr. FlemmD, Cardi0vtJ8cular Surgery Associate&,

2901 West KK lbrkway, Milwaukee 53215 1018

Surgery for EbsteinS anomaly of the bicuspid valve has strict indications in childhood. More than 73 percent of children between 1 and 15 years of age are in New York Heart Association class 1 or 2, and many remain so throughout adolescence; in the same age range surgical mortality is very high, approaching 61%.1 However, progressive heart failure, paradoxic embolism, cyanosis, or other anomalies requiring correction are indications to operate, even in very young children. Since plastic repair of the valve requires a very propitious anatomy to be successful, and this is not frequently the ease," bicuspid valve replacement (1VR) is widely performed. 'The longest documented follow-up is 19 years and concerns the first patient operated on. 4 The valve implanted was used only at the University of Cape Town, South Africa. The prosthesis most commonly used thereafter has been the Starr-Edwards caged-ball valve, with favorable results at follow-ups as long as 13 years, 5-7 sometimes even without anticoagulation." Those patients, however; were mostly adolescents or young adults at the time of the implant, whereas overall results in patients under 15 years of age have not been as favorable. 8.8 Many surgeons now prefer bioprostheses because of their better performance in the right side of the heart, despite the problems related to their use in children and young adults. 5-7.8 Plication of the atrialized segment is controversial. 3 •8 When surgery is indicated, complete correction should be undertaken regardless of age, provided that a good-sized prosthesis can be inserted.' Operating before the atrialized segment becomes hyperdistended and paradoxic provides a better outlook, 10 and our case supports this thesis. Since it is the longest reported follow-up after lVR with a mechanical prosthesis, our report may provide helpful information in reevaluating the possible role of mechanical valves in the controversial therapy of such a polymorphic cardiac malformation. REFERENCES

1 Barnard CN, Schrire \! Surgical correction of EbsteinS malformation with prosthetic tricuspid valve. Surgery 1963;54:302-08 2 Watson H. Natural history of EbsteinS anomaly of tricuspid valve in childhood and adolescence: an international cooperative study of 505 cases. Br Heart J 1974;36:417-27 3 Danielson GK, Maloney JD, Devloo RA. Surgical repair of EbsteinS anomaly Mayo Clio Proc 1979;54:185-92 4 Charles RG, Barnard CN, Beck W lHcuspid valve replacement for EbsteinS anomaly: a 19 year review of the first case. Br Heart J 1981;46:578-80 1HcuapkI VBNe Replacement for Ebsteir1lAnomaly (01 LIllo et _)