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Ann Thorac Surg 2003;75:1650-1652
© 2003 The Society of Thoracic Surgeons

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Case report

Chylotamponade: an unusual presentation of gorham’s syndrome

^a Department of Surgery, University of Wisconsin, Madison, Wisconsin, USA

Accepted for publication October 27, 2002.

^* Address reprint requests to Dr Weigel, 600 Highland Ave, CSC Room 4-346, University of Wisconsin Medical Center, Madison, WI 53792, USA (Email: weigel{at}surgery.wisc.edu).

	Abstract

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Gorham’s Syndrome, also known as massive osteolysis or "vanishing bone disease" results from lymphangiomatosis with adjacent bone resorption. Chylothorax is a common complication in cases of mediastinal involvement. We report a case of Gorham’s Syndrome presenting as chylotamponade successfully treated with pericardial drainage, early parenteral nutritional support, bilateral pleurodesis for chylous effusions, and adjuvant external beam radiation.

	Introduction

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Gorham’s Syndrome, also known as massive osteolysis or "vanishing bone disease," results from lymphangiomatosis and is often fatal secondary to malnutrition. Nearly 20% of cases are complicated by chylothorax, yet there are only three reported cases of asymptomatic chylopericardium. The following is a report of Gorham’s Syndrome presenting as chylous pericardial tamponade.

A healthy 31-year-old woman presented with new-onset heartburn, wheezing, chest heaviness, and shortness of breath. She was treated with oral ranitidine, steroids, and albuterol, and her heartburn and wheezing resolved. She returned 3 weeks later to the emergency room with an increase in dyspnea and chest pressure.

A chest roentgenogram demonstrated an enlarged cardiac silhouette and a right pleural effusion (Fig 1). Echocardiogram confirmed a large pericardial effusion with tamponade, and a pigtail catheter inserted percutaneously into the pericardial space drained approximately 1.5 L of chylous fluid. The patient was placed on bowel rest and total parenteral nutrition. Chest computed tomographic scan demonstrated osteolytic right posterior ribs 3, 4, and 5 and T7 and T8 vertebral bodies, and a right pleural effusion. A chest tube was placed and drained 1,500 mL of chylous effusion. Lymphangiogram demonstrated a broad plexus of severely ectatic lymphatic ductules running along the posterior chest walls bilaterally with no discernable lymphatic leak or discrete thoracic duct. Lymphatic proliferation was also identified in the pericardium, visceral pleura, right superior posterior chest wall, and left humerus (Figs 2, 3).

View larger version (5K): [in this window] [in a new window]   Fig 1. Admission chest roentgenogram demonstrates enlarged pericardial silhouette, right pleural effusion, and osteolytic right posterior ribs 3, 4, and 5.

View larger version (9K): [in this window] [in a new window]   Fig 2. Postlymphangiogram computed tomographic scan demonstrates diffuse mediastinal and pulmonary lymphatic involvement.

View larger version (6K): [in this window] [in a new window]   Fig 3. Lymphangiogram demonstrates a diffuse, ectatic mediastinal and bilateral chest wall lymphatic system.

	Comment

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Gorham’s Syndrome was originally described by Jackson in 1838, and then by Gorham in 1954, with fewer than 150 cases reported in the literature. The diagnosis is suggested radiographically by osteolytic lesions in association with proliferation of capillary and small lymphatic vessels on lymphangiogram. Histologically, Gorham’s is characterized by proliferation of endothelial-lined, thin-walled capillaries and lymphatic vessels with osteolysis of adjacent boney structures (Fig 4). Patients usually present in their 20s and 30s, with symptoms depending on the area of lymphatic proliferation. Skeletal fractures are common, including vertebral involvement with neurologic symptoms. Pleuritic involvement with pain, dyspnea, or cough is also common. Nearly 20% of patients experience chylothorax; however, there are only three reported cases complicated by asymptomatic chylopericardium [1]. We believe our case is the first report of Gorham’s presenting as chylous tamponade.

View larger version (7K): [in this window] [in a new window]   Fig 4. Photomicrograph of rib biopsy using CD31, an immunohistochemical stain specific for endothelial cells, to highlight marrow space replaced by thin-walled channels containing red blood cells and protein-rich lymphatic channels (hematoxylin & eosin stain, x100).

In cases with significant pleural and mediastinal involvement resulting in chylous effusion(s) or chylopericardium, surgical ligation of the thoracic duct, pleurodesis, and local excision with pleurectomy have all been reported [4–6]. A recent review reported survival rates of 64% with thoracic duct ligation, as opposed to 36% with nonsurgical management [4]. All cases of surgical failure in this report were secondary to inability to define the thoracic duct. Surgical differences in outcome may be due to a more diffuse mediastinal disease process in the medically managed or failed surgical group. Patients such as ours, with extensive thoracic pleural and mediastinal lymphatic collateralization, may be treated with pleurodesis and radiation rather than attempt at surgical ligation, especially if there is no discrete thoracic duct or lymphangiogram. Primary pleurodesis does not preclude a subsequent attempt at thoracic duct ligation if the former is unsuccessful. Deterioration in the patient’s nutritional status should prompt early surgical exploration with an attempt at thoracic duct ligation. When bilateral chylous effusions are associated with diffuse lymphangiomatosis at the level of the diaphragm, bilateral thoracotomies or an abdominal approach may be required.

In summary, we report a case of tamponade and bilateral pleural effusions secondary to Gorham’s Syndrome. Successful treatment included adequate pericardial drainage, aggressive parenteral nutritional support, bilateral pleurodesis, and adjuvant external beam radiation to the areas of mediastinal and chest wall lymphatic proliferation identified on lymphangiogram.

	References

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1. Goldstein MR, Benchimol A, Cornell W, Long DR. Chylopericardium with multiple lymphangioma of bone N Engl J Med 1969;280:1034-1037.[Medline]
2. Dunbar SF, Rosenberg A, Mankin H, Rosenthal D, Suit HD. Gorham’s massive osteolysis. the role of radiation therapy and a review of the literature. Int J Radiat Oncol Biol Phys 1993;26:491-497.[Medline]
3. Handl-Zeller L, Hohenberg G. Radiotherapy of Morbus Gorham stout. the biological value of low irradiation dose. Br J Radiol 1990;63:206-208.[Abstract/Free Full Text]
4. Tie ML, Poland GA, Rosenow III EC. Chylothorax in Gorham’s Syndrome. a common complication of a rare disease. Chest 1994;105:208-213.[Abstract/Free Full Text]
5. Patrick JH. Massive osteolysis complicated by chylothorax successfully treated with pleurodesis J Bone Joint Surg 1976;58:347-349.
6. Pedicelli G, Mattia P, Zorzoli AA, Sorrone A, DeMactino F, Sciotto V. Gorham Syndrome JAMA 1984;252:1449-1451.[Abstract/Free Full Text]

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