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5. Romero R, Pilu G, Jeanty P, et al: Prenatal Diagnosis of Congenital Anoma- lies. Norwalk, Conn, Appleton& Lange, 1988, pp 115-118 ABBREVIATIONS USED IN TEXT 6. Benacerraf BR, Greene MF, Holmes LB: The prenatal sonographic features ofNoonan's syndrome. J Ultrasound Med 1989; 8:59-63 CT = computed tomographic 7. Zarabi M, Mieckowski GC, Mazer J: Cystic hygroma associated with Noo- KS = Kaposi's sarcoma nan's syndrome. JCU 1983; 1 1:398-400 8. Macken MB, Grantmyre EB, Vincer MJ: Regression of nuchal cystic hy- groma in utero. J Ultrasound Med 1989; 8:101-103 Report of a Case 9. Brace RA: Effects of outflow pressure on fetal lymph flow. Am J Obstet Gynecol 1989; 160:494-497 The patient, a 35-year-old homosexual man, had viola- 10. Smith DW, Jones KL: Recognizable Patterns of Human Malformation: Ge- ceous skin lesions develop in early 1986, and a skin biopsy in netic, Embryologic and Clinical Aspects, 3rd Ed. Philadelphia, WB Saunders, 1984 11. Garden AS, Benzie RJ, Miskin M, et al: Fetal cystic hygroma colli: Ante- June of that year disclosed Kaposi's sarcoma. Although he natal diagnosis, significance, and management. Am J Obstet Gynecol 1986; had no pulmonary symptoms, a diffuse, interstitial pulmo- 154:221-225 12. Pijpers L, Reuss A, Stewart PA, et al: Fetal cystic hygroma: Prenatal diag- nary infiltrate was noted, and an opportunistic infection was nosis and management. Obstet Gynecol 1988; 72:223-224 suspected. Over the next six months, bronchoalveolar lavage 13. Mostello DJ, Bofinger MK, Siddigi TA: Spontaneous resolution of fetal no was cystic hygroma and hydrops in Turner's syndrome. Obstet Gynecol 1989; was done oh three occasions, but diagnosis estab- 73:862-865 lished. 14. Distell BM, Hertzberg BS, Bowie JD: Spontaneous resolution of a cystic In February 1987, the patient was admittedto the hospital ieck mass in a fetus with normal karyotype. AJR 1989; 153:380-382 15. Abramowicz IS, Warsof SL, Levy DL: Prenatal Diagnosis of Congenital with chills and sweats. On examination he had many cuta- Cystic Hygroma: Poor Prognosis, Irrespective of Karyotype. Presented at the 9th neous KS lesions and enlarged left axillary and right subman- annual meeting of the Society of Perinatal Obstetricians, New Orleans, February 1989 (Abstract 167) dibular lymph nodes. A chest radiograph revealed a diffuse 16. Von Oegen PT,, Kirk J, Comstock CH, et al: Successful Fetal Karyotyping pulmonary interstitial infiltrate and blunting ofthe right cos- from Cystic Hygroma Pluid. Presented at the 9th annual meeting of the Society of Perinatal Obstetricians, New Orleans, February 1989 (Abstract 179) tophrenic angle, suggesting a small pleural effusion. The patient refused a diagnostic evaluation and was discharged against medical advice. Chylothorax and Respiratory The patient was admitted again in April 1987 because of Failure in Kaposi's Sarcoma DANIEL W. PENNINGTON, MD MARTHA L. WARNOCK, MD MICHAEL S. STULBARG, MD San Francisco .jig ... :.' 0. DISSEMINATED KAPOSIfS SARCOMA (KS) is a common problem in patients with the acquired immunodeficiency syndrome, particularly in homosexual men. Intrathoracic involvement occurs clinically in as many as a fifth of patients with KS'-2 and has been reported in 11 of 23 consecutive patients in an autopsy series.3 Pulmonary KS usually, though not always, follows the appearance of characteristic lesions on the skin. Its clinical and radiographic presentation may mimic pneu- monia due to opportunistic infections, although nodular infil- trates and intrathoracic lymph node enlargement are com- .11 .: .... :, -ft-'- ..jTv -, monly seen. Pleural effusions occur in as many as half of -.'. .. I ,i.7j patients with pulmonary KS; they may be bilateral and are ,~. often hemorrhagic. Although they result from tumor im- plants on the visceral pleura, neither cytologic examination ofthe fluid nor pleural biopsies are helpful in establishing the diagnosis.3-5 Because pleural effusions only rarely occur in patients with Pneumocystis carinii pneumonia or other op- portunistic infections, the presence of a pleural effusion in a patient with cutaneous KS suggests the diagnosis of pulmo- nary KS.4 Among patients with pulmonary KS, those with pleural effusions have substantially shortened survival.6 Chylous pleural effusions, on the other hand, have been re- ported previously in only two patients with KS.'8

(Pennington DW, Warnock ML, Stulbarg MS: Chylothorax and respiratory failure in Kaposi's sarcoma. West J Med 1990 Apr; 152:421-422) From the Departments of Medicine (Drs Pennington and Stulbarg) and Pa- thology (Dr Warnock), Cardiovascular Research Institute (Dr Pennington), Univer- sity ofCalifornia, San Francisco, School ofMedicine. Reprint requests to Daniel W. Pennington, MD, Box 0130, Cardiovascular Re- Figure 1.-A, Anteroposterior and, B, right-side-down decubitus search Institute, UCSF, Third and Parnassus, San Francisco, CA 94143. chest films show bilateral large pleural effusions. 422 CASE REPORTS~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~REPORTS

are the commonest cause ofchylothorax; most ofthe rest are due to trauma, including surgical procedures. Among tu- mors, lymphomas and bronchogenic or gastric carcinomas are most often responsible.10 It is thought that invasion of lymph nodes along the course of the thoracic duct results in interruption of the duct and subsequent chylothorax. Here we report the first case of chylothorax due to Kaposi's sar- coma with documented neoplastic involvement of medias- tinal lymph nodes. Two patients with intrathoracic KS have been reported in whom massive chylous pleural effusions developed.',8 There was no evidence of mediastinal adenopathy by CT examina- tion in either case, and the cause ofthe chylous effusions was unknown. The patient reported here had documented involvement Figure 2.-A photomicrograph of a mediastinal lymph node shows of mediastinal lymph nodes with Kaposi's sarcoma. No at- involvement of the capsule (top) and invasion of nodal parenchyma tempt was made at autopsy to find a disruption or obstruction by the vascular interlacing spindled cells of Kaposi's sarcoma (ar- in the thoracic the of involvement rows) (hematoxylin and eosin; original magnification x 80). duct; presence neoplastic in the nodes, however, strongly suggests that such a disrup- abdominal pain; a computed tomographic (CT) scan revealed tion or obstruction occurred. The chylous nature of the the presence of small bilateral pleural effusions that were not pleural effusions is confirmed by the elevated triglyceride detected on chest radiograph. During abdominal exploration concentration.9 The fluid was grossly chylous only intermit- he was found to have diffuse violaceous lesions, with notably tently; this may have been due to his severe malnutrition enlarged spleen and lymph nodes. Biopsies were taken of because the presence ofchyle in the thoracic duct depends on three para-aortic lymph nodes, and all showed Kaposi's sar- oral fat intake. It has been reported previously that chylous in coma. Purulent peritonitis with Staphylococcus aureus bac- pleural effusions (defined by the presence ofchylomicrons teremia and wound dehiscence developed postoperatively. the fluid) were often described grossly as "bloody" or rather than His pleural effusions progressively enlarged; at the same "yellow turbid" chylous.9 time, anasarca developed and he had severe malnutrition Recognizing the diagnosis ofchylothorax in patients with be with a serum albumin level of less than 20 grams per liter. Kaposi's sarcoma is important because morbidity may In addition to the Twenty-one days after laparotomy he suffered a respiratory substantial and treatment may be beneficial. of due to the accumulation arrest that was attributed to large pleural effusions (Figure problem ventilatory compromise thoracentesis of pleural fluid, as seen in this patient, severe malnutrition 1). He required mechanical ventilation, and and immune result from the loss of removed 500 ml of fluid from the left pleural space. His dysfunction large amounts of fat, and thora- ventilation improved, and the endotracheal tube was re- protein, lymphocytes by repeated centesis or chest tube drainage. Bowel rest and total paren- moved. During the next week he underwent thoracentesis teral nutrition may be necessary to reduce the flow of chyle five times, with obvious but transient improvement in his and to nourish the patient adequately. Pleurodesis with a ventilation each time. The fluid was cloudy on four occasions sclerosing agent such as tetracycline may help to limit fluid and serosanguineous once. The protein concentration in the accumulation and protein and fat losses. Although no satis- pleural fluid was 30 to 50 grams per liter, the leukocyte count factory treatment of pulmonary KS exists, radiation therapy was 200 to 500 x 106 per liter, and the triglyceride concen- has been used with limited success.1 In addition, mediastinal tration was 1.93 mmol per liter (171 mg per dl). A cytologic irradiation is helpful in controlling chylothorax due to lym- examination showed no abnormalities. He refused a chest phoma and carcinoma. Thus, radiation therapy should be tube placement. No chest pain was reported. His clinical considered in cases ofchylothorax due to Kaposi's sarcoma. condition deteriorated, and he died on the 42nd hospital day. An autopsy examination of the chest revealed bilateral REFERENCES serosanguineous pleural effusions: 1,300 ml in the right 1. Ognibene FP, Steis RG, Macher AM, et al: Kaposi's sarcoma causing pulmo- nary infiltrates and respiratory failure in the acquired immunodeficiency syndrome. pleural space and 600 ml in the left. A red-brown macule was Ann Intern Med 1985; 102:471-475 noted on the left parietal pleura and was not examined histo- 2. Garay S, Belenko M, Fazzini E, et al: Pulmonary manifestations of Kaposi's sarcoma. Chest 1987; 91:39-43 logically. There were violaceous macules in the airways. 3. Meduri GU, Stover DE, Lee M, et al: Pulmonary Kaposi's sarcoma in the Mediastinal lymph nodes were enlarged, firm, and matted acquiredimmune deficiency syndrome. AmJMed 1986; 81:11-18 together. Sectioning of the nodes revealed hyperemic gray 4. Zibrak JD, Silvestri RC, Costello P, et al: Bronchoscopic and radiologic features of Kaposi's sarcoma involving the respiratory system. Chest 1986; parenchyma, and microscopic examination of pretracheal 90:476-479 and carinal nodes showed sarcoma 5. Ognibene FP, Shelhamer JH: Kaposi's sarcoma. Clin Chest Med 1988; Kaposi's (Figure 2). 9:459-465 an infection. There was no evidence of opportunistic 6. Gill P, Akil B, Colletti P, et al: Pulmonary Kaposi's sarcoma: Clinical find- ingsandresults of therapy. AmJMed 1989; 87:57-61 Discussion 7. Schulman LL, Grimes MM: Metastatic Kaposi's sarcoma and bilateral chylo- thorax. NY State J Med 1986; 86:205-206 or obstruction of the Chylothorax is caused by disruption 8. Pandya K, Lal C, Tlchschmidt J, et al: Bilateral chylothorax with pulmonary thoracic duct. An acute onset of pleural effusion and the Kaposi's sarcoma. Chest 1988;94:1316-1317 of turbid fluid that does not clear when 9. Staats BA, Ellefson R, Budahn L, et al: The lipoprotein profile ofchylous and presence pleural nonchylous pleural effusions. Mayo Clin Proc 1980; 55:700-704 centrifuged or that contains at least 1.24 mmol per liter (110 10. Light RW: Chylothorax and pseudochylothorax, chap 21, In Pleural Dis- mg per dl) triglyceride establish the diagnosis.9 Neoplasms eases. Philadelphia, Lea & Febiger, 1983, pp 209-219