manifestations of visceral dissemination. The papules in the esophagus and stomach were probably BA. The recognition of potential BA involvement of deep mucosal surfaces is important, since a biopsy specimen then becomes the key to correct diagnosis. Otherwise, the finding of a purplish, vascular mucosal lesion in an DIV-infected patient would most likely result in a presumptive diagnosis of KS. The consequence might be inappropriate (and ineffective) therapy directed at KS, rather than antibiotics. Awareness of this entity cannot be minimized, lest the diagnosis be overlooked despite a biops~ as occurred in this patient. Findings that distinguish BA from KS are now well described. 10 It is now known that a newly recognized agent of bacteremia in both immunocompromised and immunocompetent hosts, Rochali11Ulea henselae, also causes both BA and bacillary peliosis. 8 ,l1 This pathogen has been found to be most similar to Rochali11Ulea quintana, the agent of trench fever, by 16S ribosomal RNA gene sequencing,12 cellular fatty acid analysis,13 and DNA hybridization. 14 Extracutanoous dissemination of BA is not surprising in light of this organism's ability to cause prolonged bacteremia. Failure to recover it from blood in this case, despite the use of appropriate cultures at a time when rapid progression of lesions would suggest bacteremia, underlines its fastidious nature. In summar~ we have presented the case of a patient with AIDS whose presentation with BA involved not only typical cutaneous lesions, but also polypoid endobronchial lesions and likely visceral involvement. Although not subtle in presentation, such manifestations may be incorrectly diagnosed and treated if the clinician and/or pathologist are unaware of their nature. REFERENCES 1 Stoler MH, Bonfiglio TA, Steigbigel RT, Pereira M. An atypical subcutaneous infection associated with acquired immune syndrome. Am J Clin Patholl983; 80:714-18 2 LeBoit PE, Egbert BM, Stoler MH, Strauchen jA, Wear OJ, Berger TG, et a1. Epithelioid haemangioma-like vascular proliferation in AIOS: manifestation of cat scratc~ disease bacillus infection? Lancet 1988; 1:900-63 3 Angritt ~ Tuur SM, Macher AM, Smith KJ, Park CS, Hobin F~ et a1. Epithelioid angiomatosis in HIV infection: neoplasm or cat scratch disease? Lancet 1988; 1:996 4 Cockerell CJ, Webster GF, Whitlow MA, Friedman-Kien AE. Epithelioid angiomatosis: a distinct vascular disorder in patients with the acquired immunodeficiency syndrome or AIDS-related complex. Lancet 1987; 2:6544-46 5 Koehler JE, LeBoit PE, Egbert BM, Berger TG. Cutaneous vascular lesions and disseminated cat-scratch disease in patients with the acquired immunodeficiency syndrome (AIDS) and AIDS-related complex. Ann Intern Moo 1988; 109:449-55 6 Milam M~ Balenli MJ, Toney JF, Foulis PR, Milam C~ Behnke RH. Epithelioid angiomatosis secondary to disseminated cat scratch disease involving the bone marrow and skin in a patient with acquired immune de6ciency syndrome: a case report. Am J Moo 1990; 88:180-83 7 Kemper CA, Lombard CM, Deresinski SC, Tompkins LS. Visceral bacillary epithelioid angiomatosis: possible manifestations of diseminatOO cat scratch disease in the immunocompromised host: a report of two cases. Am J Moo 1990; 89:216-22 8 Slater LN, Welch OF, Min K-W A HochalifTUJea quintana-like organism causes bacillary angiomatosis and peliosis hepatis.
Arch Intern Moo 1992; 152:602-06 9 Cockerell CJ, Bergstresser PR, Myrie-Wtlliams C, TIerno PM. Bacillary epithelioid angiomatosis occurring in an immunocompetent individual. Arch Dermatoll990; 126:787-90 10 LeBoit PE, Berger TG, Egbert BM, Beckstead JB, Yen YSB, Stoler MH. Bacillary angiomatosis. Am J Surg Pathol 1989; 13:909-20 11 Reiman DA, Falkow S, LeBoit PE, Perkocha LA, Min IC-~ Welch OF, et ale The organism causing bacillary angiomatosis, peliosis hepatis, and fever and bacteremia in immunocompromised patients. N Eng) J Med 1991; 324:1514 12 ReIman DA, Loutit JS, Schmidt TM, Falkow S, Tompkins LS, An approach to the identification of uncultured pathogens: the agent of bacillary angiomatosis. N Engl J Moo 1990; 323:157380 13 Slater LN, Welch OF, Hensel D, Coody DW A newly recognized fastidious Gram-negative pathogen as a cause of fever and bacteremia. N Engl J Med 1990; 323:1587-93 14 Welch OF, Pickett OA, Slater LN, Steigerwalt AG, Brenner DJ. Hochalimaea henselae sp. nov., a cause of septicemia, bacillary angiomatosis, and parenchymal bacillary peliosis. J Clin Microbioi 1992; 30:275-80
Intralobar Pulmonary Sequestration Presenting as Congestive Heart Failure in a Neonate* jay K. Koll&, M.D., F.C.C.~; Michael ~ Kiernan, M.D., F.C.C.~;
Robert J Ascuitto, M.D., Ph.D.; Nancy 7: Ross-Ascuitto, M.D.; and Lawrence S. Fox, M.D., F.C.C.I!
We recently evaluated a premature infant with intralobar pulmonary sequestration which presented with signs of CHF. The infant initially underwent ligation of a PDA but subsequently developed tachycardia, tachypnea, and a continuous murmur which radiated to the back. The diagnosis of a pulmonary sequestration was suggested by Doppler echocardiography and confirmed by aortograph~ The sequestration was successfully treated by surgical (Chat 1992; 102:914-16) removal of the involved lobe. I
u.s =intralobar sequestra-I
ulmonary sequestrations consist of lung tissue that lacks P normal communication with the tracheobronchial tree
and receives aberrant systemic blood suppl~ Extralobar sequestrations typically present in the first year of life because oftheir association with other foregut malformations and cardiac anomalies. Intralobar sequestrations often present later in childhood with infectious complications; however, by virtue of the aberrant systemic blood supply and venous drainage via the pulmonary veins to the left atrium, ·From Louisiana State University Medical Center (Dr. Kolls) and Tulane Medical Center, (Drs. Kolls, Kiernan, Ascuitto, RossAscuitto, and Fox), New Orleans. Presented in part at the 56th Annual Scientific Assembly, American College ofCliest Physicians, Toronto, October 25-29, 1990. Supported by Maternal Child Health grant MCJ-229163 (Dr. Kolls). Reprint requests: Dr. KolL!, LSU Section of PulmonarylCriHctJl Care, Room 3205, 1901 Perdido Strut, New Orleans 70112 Intralobar Pulmonary sequestration (Kolls et 8/)
hemodynamic symptoms can occur.l,a Overt congestive heart failure (CHF) is rare.' We recently evaluated a premature infant with ILS who presented with CHF. This infant with ILS is thought to be the youngest patient presenting with hemodynamic symptoms, and only the third patient reported to have CHF secondary to ILS despite normal intracardiac anatomy. CASE REPORT
An infant weighing 1,800 g who was born at 32 weelcs' gestation developed mild respiratory distress syndrome and required mechanical ventilatory support for two days. A PDA was diagnosed, by Doppler echocardiography, based on the presence of left-toright Bow into the main pulmonary artery during diastole. The patient was transferred to our Institution at 3 weelcs of age for surgical ligation of the PDA after she failed to improve following a course of Indomethacin. After an UDeventful surgical operation, she was transferred back to the referring hospital. However, tachycardia, tachypnea, feeding Intolerance, and a continuous murmur subsequently developed. She was transferred back to our institution at 6 weelcs of age for additional evaluation. The physical eumination revealed a respiratory rate of SO breaths per minute, a heart rate of ISO beats per minute, and a grade 316 continuous murmur heard best at the apex of the heart with radiation to the back. The electrocardiogram showed LV hypertrophy by voltage criteria. 1\vo-dimensional echocardiography demonstrated LV dilatation and hypertrophy and dilatation of the LA with an apparent large vascular channel posterior to the LA. The Intracardiac anatomy otherwise appeared normal. Pu1sed Doppler analysis revealed an increased velocity of Bow from the pulmonary veins to the LA, left-to-right Bow across a widely patent foramen ovale, continuous Bow In the vascular channel posterior to the LA, and retrograde Bow during diastole In the descending thoracic aorta. These findings suggested an abnormal vascular communication between the descending aorta and the pulmonary circulation. Cardiac catheterization revealed a mild step-up In oxygen saturation from the superior vena cava to the right atrium and desaturation to 92 percent in the LA and descending aorta,
FIGURE 2. The venous phase of the aortogram demonstrates venous drainage by a large inferior pulmonary vein to a markedly dilated left atrium. suggesting an Intrapulmonary shunt. The right-sided pressures were normal. The LV end-diastolic pressure was elevated (10 mm Hg) and the pulse pressure In the descending aorta was widened (95127 mm Hg). Aortography showed runolf of contrast material from the descending thoracic aorta to a large aberrant vessel that traversed the left hemidiaphragm and appeared to supply the left lower lobe of the lung (Fig 1). Venous drainage from this lobe was by a large inferior pulmonary vein to a markedly dilated LA (Fig 2). The presumptive diagnosis was pulmonary sequestration with a large aorta-LA shunt. Exploratory thoracotomy confirmed the diagnosis of ILS and a left lower lobectomy with ligation of the aberrant vessel was performed. The patient tolerated the procedure well and the tachycardia. tachypnea and murmur resolved. The patient is presently doing well. DISCUSSION
FIGURE 1. This aortogram demonstrates a large aberrant vessel arising from the infradiaphragmatic aorta which traverses the left hemidiaphragm and supplies the left lower lobe.
Pulmonary sequestrations occur in two types: extralobar and intralobar. Extralobar sequestrations consist ofabnormal lung tissue separated from normal lung by its own visceral pleural investment. These anomalies are typically diagnosed during the first year oflife when they present as an incidental finding on the chest radiograph or with respiratory distress..... Ninety percent ofELS occur in the left lower lobe,7 and are frequently associated with other foregut anomalies such as diaphragmatic hernia, pectus excavatum, pericardia! cyst, and enteric duplications."· Since there is a lack of collateral ventilation, infectious complications are uncommon. The arterial blood supply is usually from the infradiaphragmatic aorta and venous drainage is from the azygous or hemizygous system. Intralobar sequestrations also usually occur in the lower lobes; however, in contrast to ELS, ILS often present with infectious complications later in life. These infections are thought to be secondary to extension of pneumonia in normal lung tissue through the pores of Kohn.•.• The arterial blood supply frequently is by a single, large, aberrant elastic vessel arising from either the descending thoracic aorta or the infradiaphragmatic aorta. Venous drainage typically is CHEST 1102 I 3 I SEPTEMBER. 1992
by the ipsilateral inferior pulmonary vein. 9 This shunt may exacerbate symptoms of heart failure l -3.IO.ll in patients with existing intracardiac anomalies. These patients, as our case illustrates, usually have continuous murmurs located over the chest, back, or axilla on the affected side. 3 •11 •12 Due to the high prevalence of associated cardiac anomalies, it is unclear whether the symptoms of heart failure are due to the volume overload from the sequestration or the underlying heart disease. To our knowledge, there have been only two previous cases of CHF associated with ILS and normal intracardiac anatom~ White and associates ll reported a IO-week-old infant whose symptoms of CHF improved after lobectomy; however, the patient died of acute tracheobronchitis five weeks postoperativel~ Ransom and associateSJ reported a 6month-old infant who initially presented at 4 months 'of age with CHF. The diagnosis of ILS was demonstrated with an aortogram. Our patient who had CHF associated with ILS is the first case to be diagnosed in the neonatal period. Presumably, the patient's prematurity contributed to the early presentation of CHF. The diagnosis of pulmonary sequestration in this case was suggested by two-dimensional echocardiography in conjunction with Doppler How analysis. Recentl~ Doppler color flow mapping has been reported to be useful in the evaluation of such vascular lesions. 13 In conclusion, we describe the case of a neonate who presented with CHF from ILS but who did not have an associated cardiac anomal~ We found two-dimensional echocardiography and pulsed-Doppler analysis helpful in suggesting the diagnosis of pulmonary sequestration. This finding of a pulmonary sequestration was confirmed by aortography, which is currently the best diagnostic tool for this disorder. Surgical lobectomy or segmentectomy has been curative in nearly all cases with hemodynamic symptoms, 1-3 but the decision to resect the lesion requires careful consideration of the volume load on the heart, the presence of underlying cardiac defects and the vascular supply to the sequestrum, since there is some mortality associated with lobectom~ 10.11
REFERENCES 1 Kergin PC. Congenital cystic disease of the lung associated with anomalous arteries. J Thorac Surg 1952; 23:55-65 2 Solit ~ Trainow ~ Wallace S. The effect of intralobar pulmonary sequestration on cardiac output. J Thorac Cardiovasc Surg 1965; 49:844-53 3 Ransom JM, Norton JB, Williams GD. Pulmonary sequestration presenting as congestive heart failure. J Thorac Cardiovasc Surg 1978; 76:378-80 4 Bliek AJ, Mulholland OJ. Extralobar lung sequestration associated with fetal neonatal respiratory distress. Thorax 1971; 26: 125-30 5 Werthammer J~ Hutten M~ Blake WB. Upper thoracic extralobar pulmonary sequestration presenting with respiratory distress in a newborn. Pediatr Radioll980; 9:116-17 6 Piccione ~ Burt ME. Pulmonary sequestration in the neonate. Chest 1990; 97:244-46 7 DeParedes CC, Pierce WS, Johnson DC, Waldhausen JA. Pulmonary sequestration in infants and children: a 2O-year experience and review of the literature. J Pediatr Surg 1970; 5: 136-47 8 Ryckman FC, Rosenkrantz JG. Thoracic surgical problems in infancy and children. Surg Clin North Am 1985; 65:1423-54
9 O'Mara CS, Baker RR, Jeyasingham K. Pulmonary sequestration. Surg Cynecol Obstet 1978; 147:609-16 10 Flye M~ Conley M, Silver D. Spectrum of pulmonary sequestration. Ann Thorac Surg 1976; 22:478-82 11 White JJ, Donahoo JS, Ostrow PT, Murphy J, Haller JA. Cardiovascular and respiratory manifestations of pulmonary sequestration in childhood. Ann Thorac Surg 1974; 18:286-94 12 Sperling DR, Finck RJ. Intralobar bronchopulmonary sequestration: association with a munnur over the back in a child. Am J Dis Child 1968; 115:362-67 13 Newman B. Real time ultrasound and color Doppler imaging in pulmonary sequestration. Pediatrics 1990; 86:620-23
Diagnostic Value of Pleural Fluid Cytology in Occult Boerhaave's Syndrome* Michael Drury, M.D.; William Anderson, M.D.; and
john E. Heffner, M.D., F.C.C.l
When Boerhaave's syndrome presents with atypical clinical features aod eludes prompt diagnosis, delays in surgical therapy increase complications and mortality. We present a patient with occult Boerhaave's syndrome who bad nondiagoostic esophageal contrast studies aod thoracic computed tomography. Pleural 8uid cytologic analysis established the presence of esophageal rupture by detecting undigested food particles. (Cheat 1992; 102:976-78)
espite more than 250 years of clinical experience with the diagnosis, Boerhaaves syndrome (spontaneous esophageal rupture) remains a potentially lethal and frequently elusive medical condition. Prognosis rapidly diminishes when diagnosis and surgical repairs are delayed with mortality rates reported as high as 100 percent in some series after one week of nonsurgical medical care. 1.2 Unfortunately, patients frequently present with a "nonclassical" history without an episode of preceding emesis, and results of the physical examination and laboratory studies may provide atypical, nondiagnostic clues. 3 ... We report the case of a patient with spontaneous esophageal rupture that remained undiagnosed despite a thorough evaluation with contrast esophagography, thoracic computed tomography (C1j, and routine microscopic examination of pleural fluid for food particles. The diagnosis was confirmed by detection of undigested food particles in the pleural fluid cytologic preparation. CASE REpORT
A 64-YeaN>ld man presented 3 h after the sudden onset of left posterior chest pain that started while lifting a heavy object and radiated to his left shoulder and abdomen. The patient had no history of productive cough, fever, chills, or dyspnea before the onset of pain. He denied alcohol abuse, dysphagia, vomiting, or loss of consciousness. Physical examination revealed a thin man with mild respiratory distress and restlessness from thoracic pain. Blood pressure was 14