Caroli disease

A 45-year-old woman was admitted to the hospital with a brain abscess due to asymptomatic pulmonary arteriovenous malformation (PAVM). The brain abscess was removed by craniotomy and excision following antibiotic therapy. The stapled wedge excision of the lung with the PAVM was successfull under videoassisted thoracoscopic surgery.

(CHEST 1995; 108:1724-27)

PAVM=pulmonary arteriovenous malformation

Key words: brain abscess; pulmonary arteriovenous malformation; thoracoscopy

Pulmonary arteriovenous malformation (PAVM) may occur as an isolated anomaly or as one of the lesions with hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease). It has a right to left shunt and induces chronic hypoxemia or paradoxical embolism (often infectious). As the patients are asymptomatic, appropriate treatments are required to avoid a series of complications.[1] Recently, thoracoscopic surgery has advanced. 2 To our knowledge, however, there have been no reports of patients with PAVM treated by means of thoracoscopic surgery. We describe a 45-year-old woman with asymptomatic single PAVM and brain abscess as an initial clinical manifestation. She underwent successful video-assisted thoracoscopic surgery without complications.

CASE REPORT

A 45-year-old woman without any significant past or family history presented with a headache and left homonymous hemianopia. After 10 days, she also complained of severe headache and fever, and was admitted to Toyoshina Red Cross Hospital. On hospital admission, body temperature was 36.4[degrees]C, pulse rate was 72 beats/min, and BP was 110/70 mm Hg. She was alert and oriented. She had left homonymous hemianopia without other neurologic defects and without physical signs suggesting hereditary hemorrhagic telangiectasia. A continuous vascular bruit was auscultated on the seventh intercostal space at the right anterior axillary line.

Laboratory data included the following: leukocyte count, 8,400/[mm.sup.3]; hemoglobin level, 13.6 g/dL; erythrocyte count, 4.35x106/[mm.sup.3] ; platelet count, 168,000/[mm.sup.3] C-reactive protein level, 0.2 mg/dL; pH, 7.489; [Pco.sub.2], 35.5 mm Hg; and [Po.sub.2], 100.3 mm Hg.

CT of the brain showed a ring enhancement in the left occipital region with surrounding brain edema and shifting of the ventricle. The chest radiograph showed a nodular shadow in the light lower peripheral lung field. The chest CT scan with contrast enhancement also showed a high-density nodular lesion with connecting vessels in the subpleural region of the right lower lung (Fig. 1). Echocardiogram was normal, but continuous Doppler wave analysis revealed abnormal continuous blood flow inside the chest wall with vascular bruit by auscultation.

The brain abscess was successfully removed by craniotomy and excision followed by systemic administration of antibiotics. Bacteriologic culture of abscess grew Gram-positive cocci and Gram-negative rods. After the operation, pulmonary angiography confirmed a single small arteriovenous malformation just under the pleura of the right middle lobe. Digital subtraction angiogram revealed that there was a single malformation with one feeding artery and one draining vein (Fig 2). Video-assisted thoracoscopic surgery was performed under the aid of double-lumen endotracheal tube anesthesia with the patient in a left lateral decubitus position. Three intercostal small incisions were made to introduce a rigid thoracoscope and manipulators through thoracoports. The pulsating lesion of PAVM and connecting vessels were identified by thoracoscopy. To confirm the precise position of the PAVM and course of the connecting vessels, a pulsed Doppler velocimeter was useful (Fig 3, top). The stapled wedge excision of the anterior lower margin of the middle lobe of the lung with PAVM was carried out successfully with a stapler (ENDO Gia 3.0V Stapler; US Surgical Corp; Norwalk, Conn) three times (Fig 3, bottom). After assuring no air leak and bleeding at the margin of the resection, a 24F chest tube was placed and the incisions were closed. Operating time was 49 min. Pathohistologic findings of the resected tissue showed dilated vessels with feeding vessels.

The patient's postoperative course was without complication and required only minimal administration of analgesics without epidural analgesia. She was discharged from the hospital after 11 days.

DISCUSSION

PAVM, which has been reported since 1897,[3] has been considered an unusual but not a rare abnormality. Cerebral complications included cerebral thrombosis and brain abscess. Most patients with PAVM and cerebral complications also had hereditary hemorrhagic telangiectasia.[4] Recently, cases of brain abscess associated with asymptomatic isolated PAVM were reported.[5,6] In treatments of brain abscess, craniotomy and excision or aspiration are generally carried out; both methods had good outcome with early diagnosis and administration of antibiotics.[6] When examining infection and the etiology of a brain abscess, the presence of PAVM should be considered. In this case, there were no signs of hereditary hemorrhagic telangiectasia, but auscultation and the chest radiograph led to the suggestion of PAVM. Chest CT showed the presence of an abnormal mass lesion with a feeding artery. Pulmonary arteriogram confirmed the single isolated PAVM.

When treatment of PAVM is considered, it should be based on size, number, location, complications of PAVM, and the patient's condition. As the natural history of PAVM is not benign due to progression and complications, the most radical and least invasive treatment should be considered in all cases.1 Before 1978, surgical resection of PAVM was the only method, and variations to conserve normal pulmonary tissue were developed. Since 1978, however, percutaneous transcatheter embolism by coil or balloon for multiple PAVMs has been reported by interventional radiologist[7] recent advances of interventional radiology are remarkable; this therapy is less invasive and has lower complications than surgery and is the first choice in most cases of PAVM in institutions with the necessary expertise and facilities. Effectiveness of the treatment was evaluated in a morphologic study with chest CT[8] or in a physiologic study with exercise test.9 The radiologist may replace the traditional surgeon in the treatment of PAVM[10]

However, the technologic advance in surgical procedures also may be a less invasive and safe method. Recently, video-assisted thoracoscopic surgery was developed in association with an improvement of video camera technology and a development of percutaneous endoscopic staplers. The study groups reported that video-assisted thoracic surgery appeared safe and might be advantageous for some procedures in reducing postoperative analgesia requirements and shortening hospital stay.[2,11]

In the examination of our patient, we had information that the PAVM lesion was single, small (<2 cm in diameter), placed at just the subpleural position, and had one feeding artery and one draining vein. We decided to carry out video-assisted thoracoscopic stapled wedge excision for the PAVM.[12] The operation was successfully performed and the patient had a short clinical course and no complications. PAVMs exist usually just under the visceral pleura, in a lower lobe in 55 to 84% of patients, and as a single lesion in 42 to 65% of patients.[1] Therefore, more than half of the patients with PAVM may have an indication for video-assisted thoracoscopic stapled wedge excision.

This case demonstrated one of the new therapeutic methods for PAVM. Further experiences and development of medical technology will be required to select add estimate the best therapy for the individual patient with PAVM.

ACKNOWLEDGMENTS: The authors thank the medical staff of Toyoshina Red Cross General Hospital, and Dr. Shinichi Okubo, Shinshu University School of Medicine, for his thoughtful comments.

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REFERENCES

[1] Burke CM, Safai C, Nelson DP, et al. Pulmonary arteriovenous malformations: a critical update. Am Rev, Respir Dis 1986; 134:334-39 [2] Hazelrigg SR, Nunchuck SK, LoCicero J. 3-D video assisted thoracic surgery study group data. Ann Thorac Surg 1993; 56:1039-43 [3] Churton T. Multiple aneurysms of pulmonary artery. BMJ 1897; 1:1223 [4] Prager RL, Laws KH, Bender HW. Arteriovenous fistula of the lung. Ann Thorac Surg 1983; 36:231-39 [5] Caroli M, Arienta C, Rampini PM, et al. Recurrence of brain abscess associated with symptomatic arteriovenous malformation of the lung. Neurochirurgia 1992; 35:167-70 [6] Momma F, Ohara S, Ohyama T, et al. Brain abscess associated with congenital pulmonary arteriovenous fistula. Surg Neurol 1990; 34:439-41 [7] Taylor BG, Cockerill EM, Manfredi F, et al. Therapeutic embolization of the pulmonary artery in pulmonary arteriovenous fistula. J Med 1978; 64:360-65 [8] Remy J, Remy-Jardin M, Wattinne L, et al. Pulmonary, aretriovenous malformations: evaluation with CT of the chest before and after treatment. Radiology 1992; 182:809-16 [9] Pennington DW, Gold WM, Gordon RL, et al. Treatment of pulmonary arteriovenous malformations by therapeutic embolization. Am Rev Respir Dis 1992; 145:1047-51 [10] Hughes JM, Allison DJ. Pulmonary arteriovenous malformations: the radiologist replaces the surgeon. Clin Radiol 1990; 41:297-98 [11] Allen MS, Deschamps C, Lee RE, et al. Video-assisted thoracoscopic stapled wedge excision for indeterminate pulmonary nodules. J Thorac Cardiovasc Surg 1993; 106:1048-52 [12] Landreneau RJ Hazelrigg SR, Ferson PF, et al. Thoracoscopic resection of 85 pulmonary, lesions. Ann Thorac Surg 1992; 54: 415-19

(*) From the Departments of Cardiology, Internal Medicine (Drs. Watanabe and Hirayama), Surgery (Drs. Munakata and Ogiwara), and Neurosurgery (Drs. Miyatake and Nakagawara), Toyishima Red Cross General Hospital Toyoshima, Town Nagano Prefecture. Japan. Reprint requests: Dr. Watanabe, TAguchi 2nd Maitsu, 1-13-5 Yanagibara, Adachi-Ku, Tokyo 120, Japan

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