Ann Thorac Surg 2001;72:937-939
© 2001 The Society of Thoracic Surgeons
Case report
Chylothorax complicating Gorham’s disease
Nicolas Chavanis, MDa,
Philippe Chaffanjon, MDa,
Gil Frey, MDa,
Gabrielle Vottero, MDb,
Pierre-Yves Brichon, MDa
a Department of Thoracic Surgery, Hôpital Albert Michallon, Grenoble, France
b Department of Pneumology, Hôpital Albert Michallon, Grenoble, France
Accepted for publication August 23, 2000.
Address reprint requests to Dr Brichon, Service de Chirurgie Thoracique, Centre Hospitalier Universitaire Albert Michallon, BP 217, F-38043 Grenoble Cedex 09, France
e-mail: pybrichon{at}chu-grenoble.fr
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Abstract
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Gorham’s disease is a rare disorder characterized by a proliferation of thin-walled lymphatic vessels (lymphangiectasia) resulting in an osteolysis. A chylothorax is present in about one-fifth of the patients and carries a poor prognosis. In this circumstance, surgery including thoracic duct ligation, pleurodesis, and excision of involved tissue is probably the treatment of choice. It is facilitated by a precise radiological assessment using a computed tomographic scanning coupled with a lymphography. We report such a case.
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Introduction
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Gorham’s disease is an unusual proliferation of abnormal lymphatic vessels leading to an osteolysis. The occurrence of a chylothorax has a poor prognosis and the treatment of this complication remains controversial. Aggressive surgery supported by a precise radiological assessment seems better than medical therapy.
A 45-year-old woman was referred to us for a left spontaneous chylothorax. Past medical history revealed a left idiopathic facial muscle paralysis and multiple occurrences of lymphoedema of the left upper limb, that appeared 10 years ago during a gestation. No diagnosis was made at that time. X-ray examination of the left hand showed a massive osteolysis, and the diagnosis of Gorham’s disease was suspected at referral. During 1 month, total parenteral hyperalimentation and pleural drainage were unsuccessful, and the chylothorax persisted. A lymphography coupled with a computed tomographic scan was performed and showed a mediastinal lymphomatous process on the left upper mediastinum above a normal thoracic duct (Figs 1 and 2). A left postero-lateral thoracotomy was performed with ligation of the thoracic duct (without division) at the lower part of the chest and excision of the pathologic lymphangiomatous mediastinal tissue that stayed in a subpleural location within the thymic area (a thymectomy was not performed). All connections of this pathologic tissue were carefully ligated. In addition, a parietal pleurectomy and a talc powder application were performed. Convalescence was uneventful. Pathologic study revealed multiple dilated vessels in the subpleural connective tissue which were described predominantly as lymphangiomas with few hemangiomas. One year later, the chylothorax had not recurred but the upper limb lymphoedema reappeared. It previously totally disappeared at the beginning of the chylothorax.
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Fig 1. Chest roentgenogram during lymphography: normal thoracic duct (long arrow) continued by mediastinal lymphangiectasy at the superior part of the chest (thick arrow).
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Fig 2. (A) Computed tomographic scan coupled with lymphography showing the pathologic lymphangiectatic proliferation at the thymic area. Anterior limit (black arrow); lateral limit (white arrowhead). (B) Computed tomographic scan coupled with lymphography showing the pathologic lymphangiectatic proliferation proceeding to the left subclavian vein (black arrow).
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Comment
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Gorham’s disease is first described by Jackson in 1838 [1]. Less than 200 cases are reported. This acquired disease may occur at any age but is most often recognized in children and young adults without sex predilection or inheritance pattern [2]. The etiology is unknown with some coincidental reported histories of trauma. The massive and progressive osteolysis is caused by the proliferation of abnormal thin-walled endothelial-lined capillaries of vascular or lymphatic origin. These hemangiomas or lymphangiomas may extend and destroy adjacent bones, soft tissue, or organs. The mechanism of the bone resorption is unknown but there is neither osteoclastic activity nor osteoporosis [4]. The skeletal distribution is not specific but it occurs mainly in shoulder girdle and pelvic area. If spontaneous arrest has been noted in some cases (causing skeletal sequelae), the ultimate extent may be lethal with cachexia, paraplegia, or pleuropulmonary involvement [3, 4]. Chylothorax is a very rare complication (25 cases reported) and is usually associated with shoulder girdle or thoracic vertebral bony osteolysis [4–7]. The isolated carpal bone osteolysis as described in our case is rare: 4 cases and is usually not associated with a chylothorax [5]. The chylothorax results from invasion of the thoracic duct or penetration of the lymphatic dysplasia into the pleural cavity, as in our case at the level of the thymic area. Medical management of the Gorham’s disease-related chylothorax (drain, parenteral feeding, corticotherapy or radiotherapy) is often ineffective with a 64% mortality rate [4]. However, 1 case of chylothorax was successfully treated with clodronate and alpha 2b interferon [8]. Surgical treatment is reported with a lesser mortality rate of 36% [4]. It usually consists of ligation of the thoracic duct and pleurectomy because of the pleural lymphatic dysplasia [1, 7]. We report a lymphangiectatic mediastinal excision in our case. In this event, the lymphography coupled with a computed tomographic scan is of particular interest, showing the limits of the mediastinal lymphangiectasy to be adequately resected. It seems to us the best radiologic examination. We had no explanation about the reoccurrence of the lymphoedema after the treatment of the chylothorax. We may only speculate that there was, on the left side of the thoracic outlet, a continuity from the left upper limb and shoulder lymphangiectasia with the same pathologic tissue that was resected in the mediastinum. The resection of this tissue and the pleurectomy could have led to the inability of the lymphatic fistula to occur in the pleura and some blockade of the abnormal lymphatic pathways of the left upper limb.
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References
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Abstract
Introduction
