Ann Thorac Surg 2002;73:1956-1957
© 2002 The Society of Thoracic Surgeons
Case report
Chyothorax associated with massive osteolysis (Gorham’s syndrome)
Koichi Fujiu, MDa*,
Ryuzo Kanno, MDa,
Hiroyuki Suzuki, MDa,
Naoya Nakamura, MDb,
Mitsukazu Gotoh, MDa
a First Department of Surgery, Fukushima Medical University, Fukushima, Japan
b First Department of Pathology, Fukushima Medical University, Fukushima, Japan
Accepted for publication December 5, 2001.
* Address reprint requests to Dr Fujiu, First Department of Surgery, Fukushima Medical University, 1 Hikarigaoka, Fukushima 960-1295, Japan
e-mail: kfujiu{at}fmu.ac.jp
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Abstract
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We report a 15-year-old boy with bilateral chylothorax complicating Gorham’s syndrome. Thoracic duct ligation failed to prevent fluid reaccumulation. The patient died of lymphocytopenia. Autopsy revealed vascular proliferation in the parietal and visceral pleura as well as in the ribs, which seemed to cause persistent chylothorax responsible for the poor prognosis of this patient. We reviewed treatments provided to 22 patients reported in the literature with Gorham’s syndrome and chylothorax.
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Introduction
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Gorham’s syndrome, or massive osteolysis, is a rare disorder of unknown etiology characterized by proliferation of thin-walled vascular structures in the bone with progressive osteolysis and often extending into the surrounding soft tissues and adjacent bones [1]. Chylothorax is an uncommon but often fatal complication of Gorham’s syndrome [2, 3]. Based on our patient and the review of 21 other patients with Gorham’s syndrome complicated by chylothorax, we discuss the treatment of this rare disease.
A 15-year-old boy was admitted to our hospital for examination of bilateral pleural effusion. The family history was unremarkable. His past history included fracture of the right clavicle and right scapula 3 years earlier, which was subsequently complicated by osteolysis of the right scapula. A biopsy specimen showed proliferation of vascular structures and marked cortical bone thinning. On admission, the chest roentgenogram and computed tomographic scans showed a large amount of pleural effusion bilaterally. Pleural aspiration yielded chylous effusion. Laboratory studies, including white blood count (4,800/mm3), absolute lymphocyte count (864/mm3), routine blood chemistry, and serum 
-globulin (1.16 g/dL) were normal.
Surgery was performed under video-assisted thoracoscopy. The right thoracic duct was ligated at the level of the diaphragm. After the operation, lymphoscintigraphy revealed no thoracic duct above the level of the ligated site (Fig 1),
but chest tube drainage persisted at a rate of approximately 2 L per day. Subsequently multiple ligatures were placed to disrupt the oozing sites by a right thoracotomy approach, but the rate of accumulation did not change. At that time, the blood count showed leukocytosis (20,000/mm3) and absolute lymphocytopenia (200/mm3). Human normal immunoglobulin was administered at 7.5 g per month, but serum gamma globulin was low (0.22 g/dL). The level of serum interleukin-6 (IL-6) level was high (71.1 pg/mL); normal is < 4.0. Nine months later the patient died of lymphocytopenia.
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Fig 1. (Top) Lymphoscintigraphy before ligation of the thoracic duct. (Bottom) Lymphoscintigraphy after ligation. Note the lack of any duct visible above the level of the diaphragm.
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Autopsy showed disappearance of the right scapula and clavicle, and osteolysis of the right humerus, sternum, and multiple ribs bilaterally. Microscopic examination showed proliferation of vascular structures that were lined with endothelial cells in the right and left ribs, sternum, vertebra, thyroid, aorta in the upper trunk, pericardium, and parietal and visceral pleura. Almost all vascular walls had no muscular tissue, indicating that these vessels-like structures were lymphatic vessels [4]. It is notable that these lymphatic structures were observed not only within the affected ribs, but also within the visceral as well as the parietal pleura (Fig 2).
The number of osteoclasts in the osseous tissues was not found to be increased.
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Fig 2. Microscopic photograph of the autopsy specimen showing proliferation of lymphatic structures (arrow) in the rib and parietal pleura. (Hematoxylin-eosin, original magnification x25.) (P = parietal pleura; R = rib.)
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Comment
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Gorham’s syndrome was first recognized as a unique single disease entity by Gorham and Stout [1] in 1955. It is most commonly seen in children and young adults. The disease may occur in any bone, but most reports describe involvement of the ribs, scapula, clavicle, humerus, pelvis, spine, femur, or mandible. Chylothorax is an uncommon but often fatal complication [2, 3]. Death in this case is usually caused by malnutrition, lymphocytopenia, or infection. Chylothorax probably occurs secondary to direct extension of proliferation of vascular structures into the parietal pleura. The treatment for chylothorax involves the prevention of fluid reaccumulation by disruption of the proliferating vascular structures or surgical joining of the visceral and parietal pleura. Irradiation sometimes prevents further osteolysis. Because endothelial cells of vessels are extremely radiosensitive, the majority of cells are rendered reproductively nonviable. Pleurodesis is sometimes effective for chylothorax [3] because the injected sclerosing agent creates pleuritis that joins the visceral and parietal pleura.
A Medline English literature search for the period of 1960 to 2000, identified 22 reported cases of Gorham’s syndrome complicated by chylothorax [2, 3, 5]. Twelve patients survived, and 10 patients died. Among the 12 surviving patients, operations were performed on 8 [2]. Two patients underwent only thoracic duct ligation (1 of these 2 patients showed only osteolysis of the spine). Six patients underwent thoracic duct ligation as well as ligation of multiple oozing sites, excision of diffuse lymphatic mass, decortication, pleurodesis, or radiotherapy, or a combination thereof. Of the 4 nonsurgical surviving patients, 1 patient was treated with 
-2b interferon, which has an antiangiogenic property [5]. Two patients with osteolysis of the pelvis or spine were successfully treated only by pleurodesis or radiotherapy [3], and 1 patient was treated by pleural drainage alone. On the other hand, treatment in 4 patients with massive osteolysis involving the clavicle, scapula, spine, and ribs was unsuccessful, even though it consisted of an operation combined with pleurodesis and radiotherapy. These results, together with the microscopic findings in our patient, suggest that wide extension of the pathologic process in the thoracic bones may, in the end, involve visceral as well as parietal pleura, which can seldom be controlled even by combined current modalities.
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References
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Gorham L.W., Stout A.P. Massive osteolysis (acute spontaneous absorption of bone, phantom bone, disappearing bone). J Bone Joint Surg Am 1955;37:985-1004.[Abstract/Free Full Text]
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Tie M.L.H., Poland G.A., Rosenow E.C. Chylothorax in Gorham’s syndrome. Chest 1994;105:208-213.[Abstract/Free Full Text]
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Hejgaard N., Olsen P.R. Massive Gorham osteolysis of the right hemipelvis complicated by chylothorax: report of a case in a 9-year-old boy successfully treated by pleurodesis. J Pediatr Orthop 1987;7:96-99.[Medline]
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Nakamura N., Watanabe K., Abe M. An autopsy case of Gorham’s syndrome. Shindanbyori 2000;17:135-136.
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Hagberg H., Lamberg K., Astrom G. -2b interferon and oral clodronate for Gorham’s disease. Lancet 1997;350:1822-1823.[Medline]
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Abstract
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