- Email: [email protected]
Spontaneous chylothorax associated with primary lymphedema and a lymphangioma malformation
1480
CASE REPORT DAGENAIS ET AL CHYLOTHORAX AND LYMPHEDEMA
sion of the coronary artery tree by obviating the need for retrograde flow to some vessels with associated decrease in perfusion pressure—the so-called Prizometer principle. It preserves conduit material for further procedures later in life and also allows percutaneous transluminal coronary angioplasty of subsequent stenosis developing distally. The material for performing the repair is readily available and, like the patient’s own, will not in any way be immunogenic. The inflammatory process that affects the aorta and its ostia may also cause stenosis at the origin of either internal mammary artery. For these reasons we believe that patch angioplasty holds undoubted advantage over the alternatives. In our first case the patch was used in conjunction with the left internal mammary artery as an adjunct. Postoperative angiography revealed a widely patent ostia at the site of the patch angioplasty; thus, a patch alone was used in the second case. Pericardial patches are well established in aortic root enlargement. Long-term results of these patches in adults have found the patch to be very stable with no evidence of either aneurysm formation or rupture. Polytetrafluoroethylene and Dacron grafting can cause aneurysmal dilatation at suture lines, which is even more pronounced in the inflammed aorta as in Takayasu’s and Kawasaki’s [4]. Other autologous tissues such as atrial appendage and saphenous vein graft have been used with as yet no long-term follow-up [5]. Other procedures for ostial stenosis are still controversial. Percutaneous transluminal coronary angioplasty has been performed on patients with coronary lesions that result from Kawasaki disease. However, the inherent risks of angioplasty to a left main stem coronary artery are high, with risk of dissection, infarction, embolism, and fatal arrhythmias. Restenosis rate is 50% at 1 year [6]. Transaortic endarterectomy has the additional hazard of dissection in coring out too much of the arterial wall in these often fibrotic and calcified vessels [7]. Coronary artery bypass grafting has been performed successfully in children since 1985. Indications until 1990 were strictly limited because of unknown natural history of these coronary artery lesions. Saphenous vein graft patency rate was considered to be low in younger children (less than 8 years). In addition, saphenous vein grafting to a small coronary artery seemed to require a very high level of surgical skill. Whether growth of the graft after bypass occurs has not been established. Recurrent stenosis at the origin of the vein graft is reported to be 60% at 10 years [8]. It must not be forgotten, however, that the underlying inflammatory process must be treated, and that successful outcome depends not only on the type of procedure, but the timing of that procedure and aggressive medical treatment of the aforementioned underlying process. We believe that although rare, coronary ostial stenosis in children poses a difficult problem for which pericardial patch angioplasty provides an effective and simple solution, making it a far more attractive procedure to perform over the alternatives available. © 1999 by The Society of Thoracic Surgeons Published by Elsevier Science Inc
Ann Thorac Surg 1999;67:1480 –2
References 1. Hitchcock JF. The return of surgical angioplasty of the left main coronary artery. Int J Cardiol 1985;7:311– 4. 2. Epstein FH. International trends in mortality and morbidity from ischemic heart disease. Ann Clin Res 1988;20:21–5. 3. Maeda M, Kobayashi M, Okamoto S, et al. Aortitis syndrome in children: clinical observation of 35 cases in Japan. Acta Paediatr 1997;39:280– 4. 4. Ascer E, Collier P, Gupta SK, Veith FJ. Reoperation for polytetrafluoroethylene bypass failure: the importance of distal outflow site and operative technique in determining outcome. J Vasc Surg 1987;5:298 –310. 5. Kitayama H, D’Orsogna L, Karl T. Isolated nonatherosclerotic coronary stenosis: surgical angioplasty of the left coronary ostium using an anterior approach. Asia Pac Heart J 1996;5:124– 6. 6. Ino T, Akimoto K, Ohkubo M, et al. Application of percutaneous transluminal coronary angioplasty to coronary arterial stenosis in Kawasaki disease. Circulation 1996;93:1709–15. 7. Ohara K, Kasegawa T, Ando T. Surgical treatment for coronary artery disease associated with aortitis syndrome. Kyobu Geka 1986;39:423– 8. 8. Suma K, Takeuchi Y, Shiroma K. Early and late post operative studies in coronary artery lesions resulting from Kawasaki disease in children. J Thorac Cardiovasc Surg 1982;84:224–9.
Spontaneous Chylothorax Associated With Primary Lymphedema and a Lymphangioma Malformation Francois Dagenais, MD, Pasquale Ferraro, MD, and Andre Duranceau, MD Division of Thoracic Surgery, Centre Hospitalier Universitaire de Montre´al, Universite´ de Montre´al, Montre´al, Que´bec, Canada
Spontaneous chylothorax associated with primary lymphedema is an exceedingly rare clinical entity. We report a case of chylothorax in a 38-year-old woman with a history of primary lymphedema of the right leg and an inguinal lymphangioma. Pathophysiology, diagnosis, treatment, as well as literature review are presented. (Ann Thorac Surg 1999;67:1480 –2) © 1999 by The Society of Thoracic Surgeons
G
enerally, chylothoraces result for operative injury to the thoracic duct or from neoplasms of the mediastinum or pleural cavity. Spontaneous chylothorax is an uncommon entity, and its association with other lymphatic malformations has been rarely reported. Establishing the diagnosis and selecting the most appropriate therapy in these cases may be challenging. We describe a unique case of a young patient with a history of primary
Accepted for publication Oct 21, 1998. Address reprint requests to Dr Ferraro, Department of Surgery, HotelDieu Hospital (CHUM), 3840 St Urbain St, Montre´al, PQ, Canada, H2W 1T8; e-mail: [email protected].
0003-4975/99/$20.00 PII S0003-4975(99)00219-2
Ann Thorac Surg 1999;67:1480 –2
lymphedema and a lymphangioma who presented with a spontaneous chylothorax. A 38-year-old woman was admitted to a community hospital for progressive dyspnea. Chest roentgenogram revealed a massive right pleural effusion, and thoracentesis showed the presence of a nonclotting milky fluid. The diagnosis of a chylothorax was confirmed by triglyceride and cholesterol counts within the pleural effusion (triglyceride 3.13 mM/L; cholesterol 0.62 mM/L). Bacterial and cytologic examinations were normal. The chylothorax was initially drained with a pigtail catheter, while the patient ceased all oral intake and total parenteral nutrition was instituted. After a 2-week trial of conservative management, daily drainage persisted fluctuating between 500 and 800 cc. As medical treatment had failed, the patient was transferred to our Thoracic Surgery Unit. Past medical history was remarkable for lymphedema of the right leg diagnosed at 16 years of age. When 18 years old, the patient was operated on for a right inguinal fluctuating mass, which histologically was found to be a lymphangioma. After surgery, lymphedema of the right leg progressed. A lymphangiography was attempted but the patient developed a severe anaphylactic reaction to iodine. The patient was discharged with compressive stockings and preventive measures for lymphangitis. Subsequently, the patient’s health remained stable until the recent appearance of progressive dyspnea extending over a 3-week period. Family history was unremarkable for congenital lymphedema. Other than the presence of the right leg lymphedema, the patient’s physical examination was normal. Her nutritional status was poor, however, as suggested by a plasma albumin count of 10 g/L. A thoracic computed tomographic (CT) scan showed numerous small cysts (, 1 cm), mostly located in the upper lobes, in addition to a residual pleural effusion on the right side. Based on clinical grounds and CT scan imaging, a tentative diagnosis of pulmonary lymphangioleiomyomatosis (LAM) was made, although a radiographic diagnosis of centrolobular emphysema could not be excluded. Pulmonary function test showed a mild obstructive pattern. The patient was explored with a right thoracoscopy. Inspection showed absence of cysts on the lung surface. Lung biopsies were taken in the upper and middle lobes, and a subtotal pleurectomy with a talc poudrage were performed. No smooth muscle cell proliferation was found on biopsies, thus excluding the diagnosis of LAM. Histologic changes were compatible with centrolobular emphysema. After surgery, pleural drainage of chyle persisted (500 cc or more/day). Considering the patient’s severe iodine allergy, a lymphangiogram was not possible, and thus the investigation was completed with a lymphoscintigraphy. The lymphoscintigraphy demonstrated obstruction and dilation of the lymphatics of the right lower leg. Progression of the technetium marker was normal in other limbs. Visualization of the lymphatic anatomy in the thorax was not possible. After 3 weeks of conservative medical management, the pleural effusion
CASE REPORT DAGENAIS ET AL CHYLOTHORAX AND LYMPHEDEMA
1481
did not subside. With the persistence of the chyle leak, a second surgical intervention was undertaken. The patient was approached through a right postero-lateral thoracotomy in the eighth intercostal space. A thoracic duct leak at the level of the inferior pulmonary ligament was identified and oversewn. In addition, mass ligation of the supradiaphragmatic thoracic duct and completion of the parietal pleurectomy were also performed. The postoperative course was uneventful and the patient was discharged 10 days after the second operation. A 6-month follow-up visit showed the patient in excellent health with no detectable signs of a pleural effusion on chest roentgenogram.
Comment This case illustrates a rare etiology of chylothorax. The most common causes of chylothorax are trauma and neoplasms, whereas spontaneous chylothorax is infrequently encountered. Spontaneous chylothorax in a woman of childbearing age should alert the physician to a possible diagnosis of LAM [1]. This rare condition is characterized by the proliferation of smooth muscle in the peribronchial, perivascular, and perilymphatic regions of the lung. The clinical presentation is usually one of exertional dyspnea or pneumothorax. Isolated chylothorax as an initial presentation is unreported [1]. Among diagnostic methods, the chest CT carries a high sensitivity and specificity for the diagnosis of LAM [2]. Thinwalled cysts less than 10 mm in diameter, distributed throughout both lung fields, are distinctive features found on CT. Our patient presented with cysts distributed primarily in the upper lobes. In addition, pathologic examination of the lung tissue did not reveal the characteristic smooth muscle cell proliferation found in LAM. The association of lymphedema, lymphangioma, and chylothorax in our patient suggests a more generalized lymphatic malformation. Primary lymphedema is divided into three subgroups: congenital, praecox, and tarda, according to the age at which the lymphedema develops. A small subset of patients with primary lymphedema will present chylous complications of their disease such as chylous ascites, protein-losing enteropathy, chyluria, chylometrorrhea, or chylous vesicles of the lower extremity. Pulmonary manifestations in primary lymphedema are primarily characterized by pleural exudates. Patients with primary lymphedema and pleural effusions often have yellow dystrophic nails. Such a triad is refered to as the “yellow nail syndrome,” first described by Samman and White in 1964 [3]. Pleural effusion is attributed to a dysfunction of the pleural and pulmonary lymphatics [4]. Chylothorax associated with primary lymphedema is rare. Chylothorax with congenital lymphedema has occasionally been encountered [5, 6]. Interestingly, Pauwels and colleagues [7] have reported a case of congenital lymphedema associated with chylothorax developing at an adult age. Two cases with similar presentations have been reported in the literature [8, 9]. The association of
1482
CASE REPORT DAVIES AND VAUGHAN MESH STENT FOR BOERHAAVE’S
congenital lymphedema and chylothorax is found in a spectrum of generalized congenital lymphatic malformations. Generalized lymphatic disorders have also been described in patients with hereditary lymphedema or Nonne-Milroy-Meige disease. This autosomal dominant transmission disorder is characterized by bilateral lower limb lymphedema. An association with chylothorax rarely has been reported. Furthermore, at least one nondominant familial transmission of unilateral lymphedema associated with chylothorax has been reported [10]. In our case, no family history of lymphedema was obtained. In addition to the lymphedema and chylothorax, our patient presented a lymphangioma in the right inguinal region. To our knowledge, this represents only the second reported case of a chylothorax associated with primary lymphedema- and lymphangioma-like manifestations. Diagnosis in these cases is difficult and relies on a high index of suspicion. More frequent etiologies of chylothorax should be excluded by a detailed history, physical examination, and chest CT. Lymphangiography may be helpful in defining the site of the chyle leak in the chest. Patients may present on lymphangiography a hyperplastic or a hypoplastic thoracic duct with collateral lymphatic channels. These anomalous thoracic ducts may rupture and produce a spontaneous chylothorax. Treatment may be difficult if the site of leakage is unknown. Although medical therapy may cause a decrease in chyle flow, it is frequently unsuccessful in completely abolishing the chyle fistula. Chemical pleurodesis is useful as an initial operative procedure, especially in the presence of a hypoplastic thoracic duct. When facing a thoracic duct fistula, we recommend a formal posterolateral thoracotomy with direct ligation of the thoracic duct leak and mass ligation of the supradiaphragmatic thoracic duct. Adjunct of a parietal pleurectomy may be performed to enhance pleurodesis. Persistent chylothorax after these different operative measures may be approached by implantation of a pleuroperitoneal. In summary, we report a rare case of spontaneous chylothorax associated with primary lymphedema and a lymphangioma. Other etiologies of chylothorax must be ruled out, and a complete investigation with lymphangiography is mandatory. Treatment is difficult and depends to a great extent on the thoracic duct morphology found with the lymphangiogram.
References 1. Taylor JR, Ryu J, Colby T. Lymphangioleiomyomatosis. NEJM 1990;323:1254– 60. 2. Merchant RN, Pearson MG, Rankin RN, Morgan WKC. Computed tomography in the diagnosis of lymphangioleiomyomatosis. Am Rev Respir Dis 1985;131:295–7. 3. Samman PD, White WF. The “yellow-nail” syndrome. Br J Dermatol 1964;76:153–7. 4. Lewis M, Kallenbach J, Zaltzman M, Conlan A, Zwi S, Abramowitz J. Pleurectomy in the management of massive pleural effusion associated with primary lymphoedema: demonstration of abnormal pleural lymphatics. Thorax 1983; 38:637–9. 5. Levine C. Primary disorders of the lymphatic vessels: a unified concept. J Pediatr Surg 1989;24:233– 40. © 1999 by The Society of Thoracic Surgeons Published by Elsevier Science Inc
Ann Thorac Surg 1999;67:1482–3
6. Kitahara M. A case report of congenital lymphedema with chylothorax. Japan J Thorac Dis 1985;23:106–13. 7. Pauwels R, Omen C, Huybrechts W, Van Der Straeten M. Chylothorax in adult age in association with congenital lymphedema. Eur J Respir Dis 1986;69:285–7. 8. Kinmonth JB, Taylor GW, Jantet GH. Chylous complications of primary lymphedema. J Cardiovasc Surg 1964;5:327– 45. 9. Mine H, Tamura K, Tanegashina K, Shinozaki Y. Nontraumatic chylothorax associated with diffuse lymphatic dysplasia. Lymphology 1984;17:111–2. 10. Mithaescu T, Veres L, Zbranca V. Chylothorax in familial lymphedema of the Meige type. Chest 1992;101:290–1.
Expanding Mesh Stent in the Emergency Treatment of Boerhaave’s Syndrome Andrew P. Davies, MRCS, and Roger Vaughan, FRCS Department of Thoracic Surgery, Norfolk and Norwich Hospital, Norwich, United Kingdom
Boerhaave’s syndrome, or spontaneous esophageal perforation, is a life threatening condition demanding early diagnosis and rapid aggressive management to prevent fulminant mediastinitis and death. We describe a patient treated in an emergency situation with an expanding esophageal mesh stent to bridge the esophageal rent together with chest and mediastinal drainage, resulting in complete recovery and return to the community. We also describe the partial recovery of the stent in the convalescent period. (Ann Thorac Surg 1999;67:1482–3) © 1999 by The Society of Thoracic Surgeons
B
oerhaave’s syndrome is potentially lethal and presents not only a diagnostic but also a therapeutic challenge. Most series report a substantial morbidity and mortality rate [1, 2]. The accumulation within the mediastinum and pleural spaces of corrosive gastric juices, enzymes, and food results in fulminant mediastinitis and severe cardiorespiratory embarrassment. Patients are, therefore, extremely unwell on admission and require urgent resuscitation prior to the emergency operation to drain the mediastinal and pleural spaces and to effect a repair of the esophageal perforation. What is required, therefore, is a fast, efficient means of sealing the esophageal rent. A previously independent, but rather frail, 85-year-old lady was presented as an emergency with severe epigastric pain of sudden onset after a bout of vomiting. The diagnosis was confirmed by contrast swallow that showed a long, left-sided tear in the distal third of the Accepted for publication Oct 22, 1998. Address reprint requests to Mr Vaughan, Department of Thoracic Surgery, Norfolk and Norwich Hospital, Brunswick Rd, Norwich, NR1 3SR, UK.
0003-4975/99/$20.00 PII S0003-4975(99)00119-8
CASE REPORT DAGENAIS ET AL CHYLOTHORAX AND LYMPHEDEMA
sion of the coronary artery tree by obviating the need for retrograde flow to some vessels with associated decrease in perfusion pressure—the so-called Prizometer principle. It preserves conduit material for further procedures later in life and also allows percutaneous transluminal coronary angioplasty of subsequent stenosis developing distally. The material for performing the repair is readily available and, like the patient’s own, will not in any way be immunogenic. The inflammatory process that affects the aorta and its ostia may also cause stenosis at the origin of either internal mammary artery. For these reasons we believe that patch angioplasty holds undoubted advantage over the alternatives. In our first case the patch was used in conjunction with the left internal mammary artery as an adjunct. Postoperative angiography revealed a widely patent ostia at the site of the patch angioplasty; thus, a patch alone was used in the second case. Pericardial patches are well established in aortic root enlargement. Long-term results of these patches in adults have found the patch to be very stable with no evidence of either aneurysm formation or rupture. Polytetrafluoroethylene and Dacron grafting can cause aneurysmal dilatation at suture lines, which is even more pronounced in the inflammed aorta as in Takayasu’s and Kawasaki’s [4]. Other autologous tissues such as atrial appendage and saphenous vein graft have been used with as yet no long-term follow-up [5]. Other procedures for ostial stenosis are still controversial. Percutaneous transluminal coronary angioplasty has been performed on patients with coronary lesions that result from Kawasaki disease. However, the inherent risks of angioplasty to a left main stem coronary artery are high, with risk of dissection, infarction, embolism, and fatal arrhythmias. Restenosis rate is 50% at 1 year [6]. Transaortic endarterectomy has the additional hazard of dissection in coring out too much of the arterial wall in these often fibrotic and calcified vessels [7]. Coronary artery bypass grafting has been performed successfully in children since 1985. Indications until 1990 were strictly limited because of unknown natural history of these coronary artery lesions. Saphenous vein graft patency rate was considered to be low in younger children (less than 8 years). In addition, saphenous vein grafting to a small coronary artery seemed to require a very high level of surgical skill. Whether growth of the graft after bypass occurs has not been established. Recurrent stenosis at the origin of the vein graft is reported to be 60% at 10 years [8]. It must not be forgotten, however, that the underlying inflammatory process must be treated, and that successful outcome depends not only on the type of procedure, but the timing of that procedure and aggressive medical treatment of the aforementioned underlying process. We believe that although rare, coronary ostial stenosis in children poses a difficult problem for which pericardial patch angioplasty provides an effective and simple solution, making it a far more attractive procedure to perform over the alternatives available. © 1999 by The Society of Thoracic Surgeons Published by Elsevier Science Inc
Ann Thorac Surg 1999;67:1480 –2
References 1. Hitchcock JF. The return of surgical angioplasty of the left main coronary artery. Int J Cardiol 1985;7:311– 4. 2. Epstein FH. International trends in mortality and morbidity from ischemic heart disease. Ann Clin Res 1988;20:21–5. 3. Maeda M, Kobayashi M, Okamoto S, et al. Aortitis syndrome in children: clinical observation of 35 cases in Japan. Acta Paediatr 1997;39:280– 4. 4. Ascer E, Collier P, Gupta SK, Veith FJ. Reoperation for polytetrafluoroethylene bypass failure: the importance of distal outflow site and operative technique in determining outcome. J Vasc Surg 1987;5:298 –310. 5. Kitayama H, D’Orsogna L, Karl T. Isolated nonatherosclerotic coronary stenosis: surgical angioplasty of the left coronary ostium using an anterior approach. Asia Pac Heart J 1996;5:124– 6. 6. Ino T, Akimoto K, Ohkubo M, et al. Application of percutaneous transluminal coronary angioplasty to coronary arterial stenosis in Kawasaki disease. Circulation 1996;93:1709–15. 7. Ohara K, Kasegawa T, Ando T. Surgical treatment for coronary artery disease associated with aortitis syndrome. Kyobu Geka 1986;39:423– 8. 8. Suma K, Takeuchi Y, Shiroma K. Early and late post operative studies in coronary artery lesions resulting from Kawasaki disease in children. J Thorac Cardiovasc Surg 1982;84:224–9.
Spontaneous Chylothorax Associated With Primary Lymphedema and a Lymphangioma Malformation Francois Dagenais, MD, Pasquale Ferraro, MD, and Andre Duranceau, MD Division of Thoracic Surgery, Centre Hospitalier Universitaire de Montre´al, Universite´ de Montre´al, Montre´al, Que´bec, Canada
Spontaneous chylothorax associated with primary lymphedema is an exceedingly rare clinical entity. We report a case of chylothorax in a 38-year-old woman with a history of primary lymphedema of the right leg and an inguinal lymphangioma. Pathophysiology, diagnosis, treatment, as well as literature review are presented. (Ann Thorac Surg 1999;67:1480 –2) © 1999 by The Society of Thoracic Surgeons
G
enerally, chylothoraces result for operative injury to the thoracic duct or from neoplasms of the mediastinum or pleural cavity. Spontaneous chylothorax is an uncommon entity, and its association with other lymphatic malformations has been rarely reported. Establishing the diagnosis and selecting the most appropriate therapy in these cases may be challenging. We describe a unique case of a young patient with a history of primary
Accepted for publication Oct 21, 1998. Address reprint requests to Dr Ferraro, Department of Surgery, HotelDieu Hospital (CHUM), 3840 St Urbain St, Montre´al, PQ, Canada, H2W 1T8; e-mail: [email protected].
0003-4975/99/$20.00 PII S0003-4975(99)00219-2
Ann Thorac Surg 1999;67:1480 –2
lymphedema and a lymphangioma who presented with a spontaneous chylothorax. A 38-year-old woman was admitted to a community hospital for progressive dyspnea. Chest roentgenogram revealed a massive right pleural effusion, and thoracentesis showed the presence of a nonclotting milky fluid. The diagnosis of a chylothorax was confirmed by triglyceride and cholesterol counts within the pleural effusion (triglyceride 3.13 mM/L; cholesterol 0.62 mM/L). Bacterial and cytologic examinations were normal. The chylothorax was initially drained with a pigtail catheter, while the patient ceased all oral intake and total parenteral nutrition was instituted. After a 2-week trial of conservative management, daily drainage persisted fluctuating between 500 and 800 cc. As medical treatment had failed, the patient was transferred to our Thoracic Surgery Unit. Past medical history was remarkable for lymphedema of the right leg diagnosed at 16 years of age. When 18 years old, the patient was operated on for a right inguinal fluctuating mass, which histologically was found to be a lymphangioma. After surgery, lymphedema of the right leg progressed. A lymphangiography was attempted but the patient developed a severe anaphylactic reaction to iodine. The patient was discharged with compressive stockings and preventive measures for lymphangitis. Subsequently, the patient’s health remained stable until the recent appearance of progressive dyspnea extending over a 3-week period. Family history was unremarkable for congenital lymphedema. Other than the presence of the right leg lymphedema, the patient’s physical examination was normal. Her nutritional status was poor, however, as suggested by a plasma albumin count of 10 g/L. A thoracic computed tomographic (CT) scan showed numerous small cysts (, 1 cm), mostly located in the upper lobes, in addition to a residual pleural effusion on the right side. Based on clinical grounds and CT scan imaging, a tentative diagnosis of pulmonary lymphangioleiomyomatosis (LAM) was made, although a radiographic diagnosis of centrolobular emphysema could not be excluded. Pulmonary function test showed a mild obstructive pattern. The patient was explored with a right thoracoscopy. Inspection showed absence of cysts on the lung surface. Lung biopsies were taken in the upper and middle lobes, and a subtotal pleurectomy with a talc poudrage were performed. No smooth muscle cell proliferation was found on biopsies, thus excluding the diagnosis of LAM. Histologic changes were compatible with centrolobular emphysema. After surgery, pleural drainage of chyle persisted (500 cc or more/day). Considering the patient’s severe iodine allergy, a lymphangiogram was not possible, and thus the investigation was completed with a lymphoscintigraphy. The lymphoscintigraphy demonstrated obstruction and dilation of the lymphatics of the right lower leg. Progression of the technetium marker was normal in other limbs. Visualization of the lymphatic anatomy in the thorax was not possible. After 3 weeks of conservative medical management, the pleural effusion
CASE REPORT DAGENAIS ET AL CHYLOTHORAX AND LYMPHEDEMA
1481
did not subside. With the persistence of the chyle leak, a second surgical intervention was undertaken. The patient was approached through a right postero-lateral thoracotomy in the eighth intercostal space. A thoracic duct leak at the level of the inferior pulmonary ligament was identified and oversewn. In addition, mass ligation of the supradiaphragmatic thoracic duct and completion of the parietal pleurectomy were also performed. The postoperative course was uneventful and the patient was discharged 10 days after the second operation. A 6-month follow-up visit showed the patient in excellent health with no detectable signs of a pleural effusion on chest roentgenogram.
Comment This case illustrates a rare etiology of chylothorax. The most common causes of chylothorax are trauma and neoplasms, whereas spontaneous chylothorax is infrequently encountered. Spontaneous chylothorax in a woman of childbearing age should alert the physician to a possible diagnosis of LAM [1]. This rare condition is characterized by the proliferation of smooth muscle in the peribronchial, perivascular, and perilymphatic regions of the lung. The clinical presentation is usually one of exertional dyspnea or pneumothorax. Isolated chylothorax as an initial presentation is unreported [1]. Among diagnostic methods, the chest CT carries a high sensitivity and specificity for the diagnosis of LAM [2]. Thinwalled cysts less than 10 mm in diameter, distributed throughout both lung fields, are distinctive features found on CT. Our patient presented with cysts distributed primarily in the upper lobes. In addition, pathologic examination of the lung tissue did not reveal the characteristic smooth muscle cell proliferation found in LAM. The association of lymphedema, lymphangioma, and chylothorax in our patient suggests a more generalized lymphatic malformation. Primary lymphedema is divided into three subgroups: congenital, praecox, and tarda, according to the age at which the lymphedema develops. A small subset of patients with primary lymphedema will present chylous complications of their disease such as chylous ascites, protein-losing enteropathy, chyluria, chylometrorrhea, or chylous vesicles of the lower extremity. Pulmonary manifestations in primary lymphedema are primarily characterized by pleural exudates. Patients with primary lymphedema and pleural effusions often have yellow dystrophic nails. Such a triad is refered to as the “yellow nail syndrome,” first described by Samman and White in 1964 [3]. Pleural effusion is attributed to a dysfunction of the pleural and pulmonary lymphatics [4]. Chylothorax associated with primary lymphedema is rare. Chylothorax with congenital lymphedema has occasionally been encountered [5, 6]. Interestingly, Pauwels and colleagues [7] have reported a case of congenital lymphedema associated with chylothorax developing at an adult age. Two cases with similar presentations have been reported in the literature [8, 9]. The association of
1482
CASE REPORT DAVIES AND VAUGHAN MESH STENT FOR BOERHAAVE’S
congenital lymphedema and chylothorax is found in a spectrum of generalized congenital lymphatic malformations. Generalized lymphatic disorders have also been described in patients with hereditary lymphedema or Nonne-Milroy-Meige disease. This autosomal dominant transmission disorder is characterized by bilateral lower limb lymphedema. An association with chylothorax rarely has been reported. Furthermore, at least one nondominant familial transmission of unilateral lymphedema associated with chylothorax has been reported [10]. In our case, no family history of lymphedema was obtained. In addition to the lymphedema and chylothorax, our patient presented a lymphangioma in the right inguinal region. To our knowledge, this represents only the second reported case of a chylothorax associated with primary lymphedema- and lymphangioma-like manifestations. Diagnosis in these cases is difficult and relies on a high index of suspicion. More frequent etiologies of chylothorax should be excluded by a detailed history, physical examination, and chest CT. Lymphangiography may be helpful in defining the site of the chyle leak in the chest. Patients may present on lymphangiography a hyperplastic or a hypoplastic thoracic duct with collateral lymphatic channels. These anomalous thoracic ducts may rupture and produce a spontaneous chylothorax. Treatment may be difficult if the site of leakage is unknown. Although medical therapy may cause a decrease in chyle flow, it is frequently unsuccessful in completely abolishing the chyle fistula. Chemical pleurodesis is useful as an initial operative procedure, especially in the presence of a hypoplastic thoracic duct. When facing a thoracic duct fistula, we recommend a formal posterolateral thoracotomy with direct ligation of the thoracic duct leak and mass ligation of the supradiaphragmatic thoracic duct. Adjunct of a parietal pleurectomy may be performed to enhance pleurodesis. Persistent chylothorax after these different operative measures may be approached by implantation of a pleuroperitoneal. In summary, we report a rare case of spontaneous chylothorax associated with primary lymphedema and a lymphangioma. Other etiologies of chylothorax must be ruled out, and a complete investigation with lymphangiography is mandatory. Treatment is difficult and depends to a great extent on the thoracic duct morphology found with the lymphangiogram.
References 1. Taylor JR, Ryu J, Colby T. Lymphangioleiomyomatosis. NEJM 1990;323:1254– 60. 2. Merchant RN, Pearson MG, Rankin RN, Morgan WKC. Computed tomography in the diagnosis of lymphangioleiomyomatosis. Am Rev Respir Dis 1985;131:295–7. 3. Samman PD, White WF. The “yellow-nail” syndrome. Br J Dermatol 1964;76:153–7. 4. Lewis M, Kallenbach J, Zaltzman M, Conlan A, Zwi S, Abramowitz J. Pleurectomy in the management of massive pleural effusion associated with primary lymphoedema: demonstration of abnormal pleural lymphatics. Thorax 1983; 38:637–9. 5. Levine C. Primary disorders of the lymphatic vessels: a unified concept. J Pediatr Surg 1989;24:233– 40. © 1999 by The Society of Thoracic Surgeons Published by Elsevier Science Inc
Ann Thorac Surg 1999;67:1482–3
6. Kitahara M. A case report of congenital lymphedema with chylothorax. Japan J Thorac Dis 1985;23:106–13. 7. Pauwels R, Omen C, Huybrechts W, Van Der Straeten M. Chylothorax in adult age in association with congenital lymphedema. Eur J Respir Dis 1986;69:285–7. 8. Kinmonth JB, Taylor GW, Jantet GH. Chylous complications of primary lymphedema. J Cardiovasc Surg 1964;5:327– 45. 9. Mine H, Tamura K, Tanegashina K, Shinozaki Y. Nontraumatic chylothorax associated with diffuse lymphatic dysplasia. Lymphology 1984;17:111–2. 10. Mithaescu T, Veres L, Zbranca V. Chylothorax in familial lymphedema of the Meige type. Chest 1992;101:290–1.
Expanding Mesh Stent in the Emergency Treatment of Boerhaave’s Syndrome Andrew P. Davies, MRCS, and Roger Vaughan, FRCS Department of Thoracic Surgery, Norfolk and Norwich Hospital, Norwich, United Kingdom
Boerhaave’s syndrome, or spontaneous esophageal perforation, is a life threatening condition demanding early diagnosis and rapid aggressive management to prevent fulminant mediastinitis and death. We describe a patient treated in an emergency situation with an expanding esophageal mesh stent to bridge the esophageal rent together with chest and mediastinal drainage, resulting in complete recovery and return to the community. We also describe the partial recovery of the stent in the convalescent period. (Ann Thorac Surg 1999;67:1482–3) © 1999 by The Society of Thoracic Surgeons
B
oerhaave’s syndrome is potentially lethal and presents not only a diagnostic but also a therapeutic challenge. Most series report a substantial morbidity and mortality rate [1, 2]. The accumulation within the mediastinum and pleural spaces of corrosive gastric juices, enzymes, and food results in fulminant mediastinitis and severe cardiorespiratory embarrassment. Patients are, therefore, extremely unwell on admission and require urgent resuscitation prior to the emergency operation to drain the mediastinal and pleural spaces and to effect a repair of the esophageal perforation. What is required, therefore, is a fast, efficient means of sealing the esophageal rent. A previously independent, but rather frail, 85-year-old lady was presented as an emergency with severe epigastric pain of sudden onset after a bout of vomiting. The diagnosis was confirmed by contrast swallow that showed a long, left-sided tear in the distal third of the Accepted for publication Oct 22, 1998. Address reprint requests to Mr Vaughan, Department of Thoracic Surgery, Norfolk and Norwich Hospital, Brunswick Rd, Norwich, NR1 3SR, UK.
0003-4975/99/$20.00 PII S0003-4975(99)00119-8