Pleuropulmonary and Skeletal Lymphangiomatosis with Chylothorax and Chylopericardium

doi:10.1016/S0003-4975(10)60078-1

The Annals of Thoracic Surgery

Volume 40, Issue 4, October 1985, Pages 398-401

https://doi.org/10.1016/S0003-4975(10)60078-1 Get rights and content

Abstract

Congenital lymphangiomatosis of lung and bone, with or without chylothorax, is a rare but often fatal systemic lymphatic malformation. In those who survive infancy and early childhood, parietal pleurectomy with excision of lymphatic lakes and ligation of the thoracic duct can be successful. Two patients with lymphangiomatosis are described, 1 with chylothorax and chylopericardium with generalized skeletal lesions and the other with pleuropulmonary lesions and chylothorax. Both were successfully treated with parietal pleurectomy, excision of lymphatic lakes, and ligation of lymphatics, including the thoracic duct. To our knowledge, the triad of generalized skeletal lymphangiomatosis, chylopericardium, and chylothorax has not been previously reported.

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Citation Excerpt :
The true prevalence of pulmonary involvement in the total pool of patients with LA is unknown. It has, however, been noted in several studies that patients with LA with pulmonary involvement had significantly higher mortality and morbidity rate than those without (C. Trenor, oral communication, 2nd International Conference on Generalized Lymphatic Anomaly and Gorham-Stout Disease, Atlanta, GA, June 10-11, 2016).4,12,13 Traditionally, imaging modalities of patients with LA have been limited to conventional cross-sectional imaging, such as MR and CT imaging.1,14
Pulmonary involvement in lymphatic anomalies (LA) is associated with significant morbidity and mortality. Dynamic contrast-enhanced magnetic resonance lymphangiography (DCMRL) is capable of imaging the lymphatic system in a variety of pulmonary lymphatic disorders.
The objective of this study is to describe the central lymphatic anatomy in patients with LA and pulmonary involvement on DCMRL.
This prospective observational study enrolled 16 patients with LA (mean age, 17 years; range, 6-63 years; ratio of female to male patients, 9:7) with pulmonary involvement. All patients underwent DCMRL. The lymphatic system was assessed for the presence of mediastinal masses, interstitial lung disease, size and tortuosity of the thoracic duct (TD), and presence of abnormal pulmonary lymphatic flow.
T2-weighted imaging showed the following: mediastinal soft tissue masses in 10 patients, diffuse pulmonary interstitial thickening in 13 patients, and bone involvement in 15 patients. DCMRL revealed abnormal pulmonary lymphatic flow in 14 of 16 patients. Abnormal pulmonary lymphatic flow originated from the TD in three of 14 patients, the retroperitoneum in six of 14 patients, and both the TD and retroperitoneum in four of 14 patients. In nine of 16 patients, the TD was dilated and tortuous. In two patients the TD was not identified, and in five patients it was normal.
Abnormal pulmonary lymphatic flow/perfusion from the TD or retroperitoneum into the lung parenchyma occurred in the majority of patients in this study. These findings can explain the interstitial lung disease and chylothorax resulting in deterioration of respiratory function in these patients. Future studies will determine whether mechanical cessation of this abnormal flow can improve pulmonary function and prolong survival in patients with LA.
Clinicaltrials.gov; No.: NCT02744027; URL: www.clinicaltrials.gov;
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Central conducting lymphatic anomalies (CCLA) may cause chylous leaks and protein-losing enteropathy (PLE) owing to dysfunction of the central lymphatic channels. Most of the treatment strategies for these conditions are palliative and provide transient improvement.
We treated 14 patients with intractable chylous leak and/or PLE using a novel technique of lymphaticovenous bypass of the terminal portion of the thoracic duct. Chylous leaks occurred in multiple different anatomic sites. All patients had CCLA and failure of thoracic duct emptying demonstrated by preoperative intranodal lymphangiography.
Five patients had complete resolution of symptoms, and two patients had partial improvement. There were no major complications. Of 5 patients with PLE, only one improved after lymphaticovenous bypass. Repeat traditional lymphangiography was performed in 4 patients who did not improve, demonstrating patency of the bypass in all cases with persistent sluggish drainage. One patient had repeat MR lymphangiography that did not show the thoracic duct well.
Bypass of the terminal thoracic duct is a novel procedure that offers improvement and a chance of cure for some patients with devastating manifestations of CCLA who lack other effective therapeutic options.
IV.
Cervical and thoracic components of multiorgan lymphangiomatosis managed surgically
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Citation Excerpt :
The origin of this pathologic entity is controversial. Some authors discuss a hamartomatous or neoplastic origin [4]; still others believe that the cysts represent congenital anomalies of lymphojugular sacs in the developing embryo [5]; and in some instances it is associated with malformation of the venous system, (eg, left sided superior vena cava) [6]. The incidence of the syndrome of lymphangiomatosis is not known.
Primary idiopathic chylopericardium in a 2 month old successfully treated without surgery
2000, Journal of Pediatric Surgery
Citation Excerpt :
Skeletal x-ray will show punched out areas showing extrathoracic lymphangiomatosis. Mediastinal neoplasms can cause the effusion by compression of the thoracic duct and have to be ruled out by chest CT and MRI.5,6 Tuberculosis also has to be excluded by repeated cultures and microscopic examination of samples from the pericardial effusion.
The authors report a case of primary idiopathic chylopericardium in a previously healthy 2-month-old infant. He was treated with continuous pericardial drainage and low-fat total parenteral nutrition. He remains asymptomatic after 14 months of follow-up. This is the first report of primary idiopathic chylopericardium successfully treated nonoperatively. J Pediatr Surg 35:646-648. Copyright © 2000 by W.B. Saunders Company.
Successful control of extensive thoracic lymphangiomatosis by irradiation
1997, Clinical Oncology
Lymphangiomas are very rare benign tumours, believed to arise from congenital malformations of the lymphatic system. They grow very slowly, but are capable of massive expansion and infiltration of the surrounding tissues. If left untreated they can cause serious and sometimes fatal complications. The thorax is commonly affected, with involvement of the ribs, pleura, pericardium, lung parenchyma, mediastinum and vertebrae. Treatment depends on the extent of the disease. While local excision is the treatment of choice for localized lesions, radiotherapy is used for more extensive disease. In this report we describe three patients with extensive thoracic lymphangiomatosis who were treated successfully with irradiation alone. The value of early diagnosis and treatment is emphasized.
Massive chylothorax associated with lymphangiomatosis of the bone
1994, Journal of Pediatric Surgery
Chylothorax in the absence of tumor or trauma is uncommon. Lymphangiomatosis of the bone, although extremely rare, has been associated with chylothorax. The authors describe the case of a 12-year-old boy who presented with a symptomatic left chylothorax associated with lymphangiomatosis of the ribs, scapula, and clavicle. Despite tube thoracostomies and the initiation of total parenteral nutrition, massive losses of chyle persisted, resulting in hypoproteinemia and severe lymphopenia. Control of the chylothorax was achieved by a parietal pleurectomy and application of fibrin glue (Tisseel). In the literature there are 16 cases of chylothorax associated with lymphangiomatosis of the bone. Their presentation, treatment, and outcome are reviewed. Conservative treatments such as dietary manipulations or thoracenteses were rarely successful. Thoracotomy with parietal pleurectomy on the side of the effusion is usually effective in controlling the chylothorax. Lymphangiomatosis should be considered a diagnostic possibility for any child who presents with a chylothorax.

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Pleuropulmonary and Skeletal Lymphangiomatosis with Chylothorax and Chylopericardium

Abstract

Ann Thorac Surg

Am J Med

Am J Med

J Thorac Cardiovasc Surg

J Thorac Cardiovasc Surg

Dis Chest

Am J Med

J Pediatr

Chest

J Biol Chem

Skeletal angiomatosis

Clin Orthop