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Chylous vaginal discharge in a patient with lymphangioleiomyomatosis

2008; Elsevier BV; Volume: 199; Issue: 3 Linguagem: Inglês

10.1016/j.ajog.2008.04.007

1097-6868

Ichiro Iwamoto, Toshinori Fujino, Tsutomu Douchi,

Eosinophilic Disorders and Syndromes

We present the case of a patient having chylous vaginal discharge, which may be a symptom of the secondary to an established diagnosis of the systemic disorder, lymphangioleiomyomatosis, a disease predominantly affecting women during their reproductive years. Lymphangioleiomyomatosis causes dyspnea, pneumothorax, chylous pleural effusions, and eventually respiratory failure. We present the case of a patient having chylous vaginal discharge, which may be a symptom of the secondary to an established diagnosis of the systemic disorder, lymphangioleiomyomatosis, a disease predominantly affecting women during their reproductive years. Lymphangioleiomyomatosis causes dyspnea, pneumothorax, chylous pleural effusions, and eventually respiratory failure. Chylous vaginal discharge is a rare disorder first described by Watson in 1953.1Watson J. Chronic lymphoedema of extremities and its management.Br J Surg. 1953; 41: 31-37Crossref PubMed Scopus (9) Google Scholar Chyle is formed in intestinal lacteals, drains into mesenteric lymphatics, and eventually into the thoracic duct via the cisterna chyli. Chyle is not normally found in areas other than those described above. However, chylous reflux has been recognized.The chylous reflux syndrome is caused by the stasis and eventual retrograde flow of chyle from its normal route into retroperitoneal or peritoneal lymphatics. This syndrome may manifest itself as lymphedema, chyluria, chylous metrorrhea, or chylous vaginal discharge.2Shahlaee A.H. Burton E.M. Sabio H. Plouffe Jr., L. Teeslink R. Primary chylous vaginal discharge in a 9-year-old girl: CT-lymphangiogram and MR appearance.Pediatr Radiol. 1997; 27: 755-757Crossref PubMed Scopus (7) Google Scholar The primary form of the syndrome may be due to anomaly of the lymphatic drainage system. The causes of secondary chylous reflux are surgical complications, irradiation side effects, filarial infection, or neoplasm. Here we present the case of a patient having chylous vaginal discharge, which may be a symptom of the systemic disorder lymphangioleiomyomatosis (LAM).LAM is a systemic disorder predominantly affecting women during their reproductive years. It is characterized by the proliferation of abnormal smooth muscle cells (LAM cells) in the pulmonary interstitium and along the axial lymphatics of the thorax and abdomen.3Corrin B. Liebow A.A. Friedman P.J. Pulmonary lymphangiomyomatosis A review.Am J Pathol. 1975; 79: 348-382PubMed Google Scholar LAM cells are thought to be derived from perivascular cells along the walls of lymphatic vessels. The disease causes dyspnea, pneumothorax, chylous pleural effusions, hemoptysis, and eventually respiratory failure.4Johnson S.R. Tattersfield A.E. Clinical experience of lymphangioleiomyomatosis in the UK.Thorax. 2000; 55: 1052-1057Crossref PubMed Scopus (210) Google ScholarCase ReportA 37-year-old woman (gravida 2, para 1) occasionally experienced heavy whitish, watery vaginal discharge for 3 years. She started to suffer from exertional dyspnea 2 years before the present examination, and the symptom gradually worsened. She consulted a physician in our university hospital and was diagnosed as having LAM based on high-resolution computerized tomography and pulmonary biopsy findings (Figure 1).She was referred to the outpatient clinic of our department regarding her occasional heavy milky vaginal discharge. However, when she initially visited our outpatient clinic, the amount and color of her vaginal discharge were normal. Pelvic examination showed no abnormal findings except for a slight echo-free space in the pouch of Douglas that seemed to be within the normal range.Later the amount of her vaginal discharge became transiently moderate. She was administered with a GnRH agonist by her attending physician to treat the LAM-induced respiratory symptoms. Afterward, she again visited our outpatient clinic because of the reappearance and increased volume of vaginal discharge. Upon examination, milky discharge was observed dripping from her vagina (Figure 2). A vaginal discharge specimen was collected and submitted for laboratory analysis, which demonstrated the presence of chyle (Table 1). Her pulmonary symptoms gradually worsened, and at present, she is waiting for lung transplantation.FIGURE 2Vaginal dischargeShow full captionHeavy milky vaginal discharge (arrows) dripping from the vagina.Iwamoto. Chylous vaginal discharge lymphangioleiomyomatosis. Am J Obstet Gynecol 2008.View Large Image Figure ViewerDownload Hi-res image Download (PPT)TABLEAnalysis of the milky vaginal discharge1) supernatant after centrifugal separation : turbid2) solubility in ether : dissolved in ether3) character of biochemistry : total cholesterol 64mg/dl neutral fat 412mg/dl phospholipid 113mg/dl Open table in a new tab CommentIn the present case, chylous vaginal discharge was considered to be caused by an obstruction of the valvular system or a nonfunctional valvular system owing to the proliferation of LAM cells along the lymphatics of the abdomen as well as in the pulmonary interstitium. We did not perform lymphangiography to confirm the lymphatic lesion in the pelvis because we thought that the procedure would not contribute to any improvement of prognosis for our patient, who eventually developed severe pulmonary symptoms.LAM is most often sporadic, although it has also been reported to occur in about 3% of female patients with tuberous sclerosis complex, a genetically transmitted disorder.5Moss J. Barnes P. Litzenberger R. et al.Incidence of lymphangioleiomyomatosis (LAM) in patients with tuberous sclerosis complex.Am J Respir Crit Care Med. 2000; 164: 169-171Google Scholar The present patient was considered a sporadic case having no family history of this disorder.Progesterone has been widely used for LAM treatment.6Chu S.C. Horiba K. Usuki J. et al.Comprehensive evaluation of 35 patients with lymphangioleiomyomatosis.Chest. 1999; 115: 1041-1052Crossref PubMed Scopus (285) Google Scholar However, the effects of progesterone have been variable to date. Other drugs, such as tamoxifen and a GnRH agonist, were reported to be less effective than progesterone.7Johnson S.R. Lymphangioleiomyomatosis: clinical features, management and basic mechanisms.Thorax. 1999; 54: 254-264Crossref PubMed Scopus (179) Google Scholar Although our patient has been administered a GnRH agonist, her LAM-induced pulmonary symptoms and chylous vaginal discharge have been gradually worsening. Lung transplantation is considered only in patients with advanced disease, such as in this case.It should be emphasized that the vaginal discharge was not the presenting symptom of LAM in this patient and that LAM diagnosis was established during the evaluation of exertional dyspnea. However, chylous vaginal discharge should not be taken lightly because it may be a presenting symptom, 1 of the symptoms of LAM in rare cases. Early detection of LAM through detailed history taking by gynecologists and immediate treatment by specialists may improve prognosis in the future. Chylous vaginal discharge is a rare disorder first described by Watson in 1953.1Watson J. Chronic lymphoedema of extremities and its management.Br J Surg. 1953; 41: 31-37Crossref PubMed Scopus (9) Google Scholar Chyle is formed in intestinal lacteals, drains into mesenteric lymphatics, and eventually into the thoracic duct via the cisterna chyli. Chyle is not normally found in areas other than those described above. However, chylous reflux has been recognized. The chylous reflux syndrome is caused by the stasis and eventual retrograde flow of chyle from its normal route into retroperitoneal or peritoneal lymphatics. This syndrome may manifest itself as lymphedema, chyluria, chylous metrorrhea, or chylous vaginal discharge.2Shahlaee A.H. Burton E.M. Sabio H. Plouffe Jr., L. Teeslink R. Primary chylous vaginal discharge in a 9-year-old girl: CT-lymphangiogram and MR appearance.Pediatr Radiol. 1997; 27: 755-757Crossref PubMed Scopus (7) Google Scholar The primary form of the syndrome may be due to anomaly of the lymphatic drainage system. The causes of secondary chylous reflux are surgical complications, irradiation side effects, filarial infection, or neoplasm. Here we present the case of a patient having chylous vaginal discharge, which may be a symptom of the systemic disorder lymphangioleiomyomatosis (LAM). LAM is a systemic disorder predominantly affecting women during their reproductive years. It is characterized by the proliferation of abnormal smooth muscle cells (LAM cells) in the pulmonary interstitium and along the axial lymphatics of the thorax and abdomen.3Corrin B. Liebow A.A. Friedman P.J. Pulmonary lymphangiomyomatosis A review.Am J Pathol. 1975; 79: 348-382PubMed Google Scholar LAM cells are thought to be derived from perivascular cells along the walls of lymphatic vessels. The disease causes dyspnea, pneumothorax, chylous pleural effusions, hemoptysis, and eventually respiratory failure.4Johnson S.R. Tattersfield A.E. Clinical experience of lymphangioleiomyomatosis in the UK.Thorax. 2000; 55: 1052-1057Crossref PubMed Scopus (210) Google Scholar Case ReportA 37-year-old woman (gravida 2, para 1) occasionally experienced heavy whitish, watery vaginal discharge for 3 years. She started to suffer from exertional dyspnea 2 years before the present examination, and the symptom gradually worsened. She consulted a physician in our university hospital and was diagnosed as having LAM based on high-resolution computerized tomography and pulmonary biopsy findings (Figure 1).She was referred to the outpatient clinic of our department regarding her occasional heavy milky vaginal discharge. However, when she initially visited our outpatient clinic, the amount and color of her vaginal discharge were normal. Pelvic examination showed no abnormal findings except for a slight echo-free space in the pouch of Douglas that seemed to be within the normal range.Later the amount of her vaginal discharge became transiently moderate. She was administered with a GnRH agonist by her attending physician to treat the LAM-induced respiratory symptoms. Afterward, she again visited our outpatient clinic because of the reappearance and increased volume of vaginal discharge. Upon examination, milky discharge was observed dripping from her vagina (Figure 2). A vaginal discharge specimen was collected and submitted for laboratory analysis, which demonstrated the presence of chyle (Table 1). Her pulmonary symptoms gradually worsened, and at present, she is waiting for lung transplantation.TABLEAnalysis of the milky vaginal discharge1) supernatant after centrifugal separation : turbid2) solubility in ether : dissolved in ether3) character of biochemistry : total cholesterol 64mg/dl neutral fat 412mg/dl phospholipid 113mg/dl Open table in a new tab A 37-year-old woman (gravida 2, para 1) occasionally experienced heavy whitish, watery vaginal discharge for 3 years. She started to suffer from exertional dyspnea 2 years before the present examination, and the symptom gradually worsened. She consulted a physician in our university hospital and was diagnosed as having LAM based on high-resolution computerized tomography and pulmonary biopsy findings (Figure 1). She was referred to the outpatient clinic of our department regarding her occasional heavy milky vaginal discharge. However, when she initially visited our outpatient clinic, the amount and color of her vaginal discharge were normal. Pelvic examination showed no abnormal findings except for a slight echo-free space in the pouch of Douglas that seemed to be within the normal range. Later the amount of her vaginal discharge became transiently moderate. She was administered with a GnRH agonist by her attending physician to treat the LAM-induced respiratory symptoms. Afterward, she again visited our outpatient clinic because of the reappearance and increased volume of vaginal discharge. Upon examination, milky discharge was observed dripping from her vagina (Figure 2). A vaginal discharge specimen was collected and submitted for laboratory analysis, which demonstrated the presence of chyle (Table 1). Her pulmonary symptoms gradually worsened, and at present, she is waiting for lung transplantation. CommentIn the present case, chylous vaginal discharge was considered to be caused by an obstruction of the valvular system or a nonfunctional valvular system owing to the proliferation of LAM cells along the lymphatics of the abdomen as well as in the pulmonary interstitium. We did not perform lymphangiography to confirm the lymphatic lesion in the pelvis because we thought that the procedure would not contribute to any improvement of prognosis for our patient, who eventually developed severe pulmonary symptoms.LAM is most often sporadic, although it has also been reported to occur in about 3% of female patients with tuberous sclerosis complex, a genetically transmitted disorder.5Moss J. Barnes P. Litzenberger R. et al.Incidence of lymphangioleiomyomatosis (LAM) in patients with tuberous sclerosis complex.Am J Respir Crit Care Med. 2000; 164: 169-171Google Scholar The present patient was considered a sporadic case having no family history of this disorder.Progesterone has been widely used for LAM treatment.6Chu S.C. Horiba K. Usuki J. et al.Comprehensive evaluation of 35 patients with lymphangioleiomyomatosis.Chest. 1999; 115: 1041-1052Crossref PubMed Scopus (285) Google Scholar However, the effects of progesterone have been variable to date. Other drugs, such as tamoxifen and a GnRH agonist, were reported to be less effective than progesterone.7Johnson S.R. Lymphangioleiomyomatosis: clinical features, management and basic mechanisms.Thorax. 1999; 54: 254-264Crossref PubMed Scopus (179) Google Scholar Although our patient has been administered a GnRH agonist, her LAM-induced pulmonary symptoms and chylous vaginal discharge have been gradually worsening. Lung transplantation is considered only in patients with advanced disease, such as in this case.It should be emphasized that the vaginal discharge was not the presenting symptom of LAM in this patient and that LAM diagnosis was established during the evaluation of exertional dyspnea. However, chylous vaginal discharge should not be taken lightly because it may be a presenting symptom, 1 of the symptoms of LAM in rare cases. Early detection of LAM through detailed history taking by gynecologists and immediate treatment by specialists may improve prognosis in the future. In the present case, chylous vaginal discharge was considered to be caused by an obstruction of the valvular system or a nonfunctional valvular system owing to the proliferation of LAM cells along the lymphatics of the abdomen as well as in the pulmonary interstitium. We did not perform lymphangiography to confirm the lymphatic lesion in the pelvis because we thought that the procedure would not contribute to any improvement of prognosis for our patient, who eventually developed severe pulmonary symptoms. LAM is most often sporadic, although it has also been reported to occur in about 3% of female patients with tuberous sclerosis complex, a genetically transmitted disorder.5Moss J. Barnes P. Litzenberger R. et al.Incidence of lymphangioleiomyomatosis (LAM) in patients with tuberous sclerosis complex.Am J Respir Crit Care Med. 2000; 164: 169-171Google Scholar The present patient was considered a sporadic case having no family history of this disorder. Progesterone has been widely used for LAM treatment.6Chu S.C. Horiba K. Usuki J. et al.Comprehensive evaluation of 35 patients with lymphangioleiomyomatosis.Chest. 1999; 115: 1041-1052Crossref PubMed Scopus (285) Google Scholar However, the effects of progesterone have been variable to date. Other drugs, such as tamoxifen and a GnRH agonist, were reported to be less effective than progesterone.7Johnson S.R. Lymphangioleiomyomatosis: clinical features, management and basic mechanisms.Thorax. 1999; 54: 254-264Crossref PubMed Scopus (179) Google Scholar Although our patient has been administered a GnRH agonist, her LAM-induced pulmonary symptoms and chylous vaginal discharge have been gradually worsening. Lung transplantation is considered only in patients with advanced disease, such as in this case. It should be emphasized that the vaginal discharge was not the presenting symptom of LAM in this patient and that LAM diagnosis was established during the evaluation of exertional dyspnea. However, chylous vaginal discharge should not be taken lightly because it may be a presenting symptom, 1 of the symptoms of LAM in rare cases. Early detection of LAM through detailed history taking by gynecologists and immediate treatment by specialists may improve prognosis in the future.