Postgrad Med J: first published as 10.1136/pgmj.70.827.655 on 1 September 1994. Downloaded from Postgrad Med J (1994) 70, 655 - 657 © The Fellowship of Postgraduate Medicine, 1994

Recurrent chylothorax in a woman with primary amenorrhoea Mangalam Sridhar, lain Boyle' and Stephen Banham

Department ofRespiratory Medicine and' University Department ofMedicine (Endocrinology), Glasgow Royal Infirmary, Glasgow, UK

Summary: A 47 year old woman receiving oestrogen replacement therapy for primary amenorrhoea presented with recurrent chylothorax. The clinical and radiological features of her illness were characteristic of pulmonary lymphangio-leiomyomatosis, a rare hormone-dependent disease. The condition was treated with pleurodesis and withdrawal of oestrogen therapy.

Introduction Chylothorax, the collection of chylous fluid in the hormones as a growth stimulant. Menstruation pleural space is a rare cause of pleural effusion.' was induced by oestrogen therapy (piperazine The aetiology includes malignancy involving the oestrone sulphate 1.5 mg daily). A detailed endo- mediastinal structures causing obstruction of the crinological assessment was performed once more thoracic duct (especially lymphomas and metasta- at the age of 28, prior to the patient's proposed ses from gastric carcinoma), surgical and non- emigration to Canada. Cortisol response to insulin- by copyright. surgical trauma to the thoracic duct, and throm- induced hypoglycaemia and tetracosactin (Synac- bosis of the jugular and subclavian veins. Other then) were normal. Plasma and urinary gonado- more unusual causes are tuberculosis, filariasis, trophin levels were low. There was a subnormal yellow nail syndrome and lymphangio- response to a luteinizing hormone releasing test. A leiomyomatosis (LAM).2'3 The latter condition is a diagnosis of hypogonadotrophic hypogonadism rare cause ofchylothorax and lung disease affecting was made on the basis of these investigations. A women of child-bearing age. In recent years there routine chest X-ray performed at this time was has been particular interest in the apparent sex- reported as showing prominent lung markings at hormone dependence of this condition.4 We pres- both lung bases but no action was taken as the

ent here a case of recurrent chylothorax due to patient was asymptomatic. The patient remained in http://pmj.bmj.com/ LAM, with an unusual clinical course which pro- good health until the onset of her present com- vides further support for the importance of hor- plaint, save for intermittent problems associated monal manipulation in control of disease progres- with oestrogen therapy, which required temporary sion. withdrawal of therapy on a number of occasions. There was no history ofthoracic surgery or trauma. On examination the patient was of short stature Case report (height 135 cm) and thin (weight 35 kg). There was no finger clubbing or lymphadenopathy. There on September 30, 2021 by guest. Protected A 47 year old school teacher presented with a 3 were signs of a right pleural effusion. A computed month history of progressive breathlessness. tomographic (CT) scan ofthe thorax confirmed the Her first contact with the hospital was at the age effusion and showed in addition a small pneumo- of 17 when she was investigated for short stature thorax on the right as well as scattered cystic and and primary amenorrhoea. Bone age was found to parenchymal abnormalities in both lower lung be less than chronological age but thyroid, pitui- fields (Figures 1 and 2). The mediastinum was tary function and sex chromosome constitution normal. Aspiration of the pleural effusion revealed were normal. The patient was placed on thyroid a milky white fluid which showed the presence of triglycerides and chylomicrons on biochemical analysis, confirming it to be a chylothorax. Pleural Correspondence: M.K. Sridhar, M.B., M.R.C.P. (U.K.)., biopsies showed no specific abnormality. Lung Department of Respiratory Medicine, Glasgow Royal function tests performed after plerual aspiration Infirmary, 16 Alexandra Parade, Glasgow G31 2ES, UK. showed poor lung function (forced vital capacity: Accepted: 13 January 1994 1.4 litre; forced expiratory volume in the first Postgrad Med J: first published as 10.1136/pgmj.70.827.655 on 1 September 1994. Downloaded from 656 CLINICAL REPORTS

Figure 1 Right-sided chylothorax (computed tomo- Figure 2 Cystic and parenchymal abnormalities of left graphy of thorax). lung.

second: 1.1 litre). A clinical diagnosis of lymph- combination of clinical and radiological features, angioleiomyomatosis was made on the basis of the in particular the presence ofthin-walled cysts in the typical clinical features and CT scan appearances.5 lung parenchyma on computerized tomography, is A lung biopsy was not performed in view of the considered diagnostic.5 In our case the presence of poor lung function. a chylothorax, a pneumothorax and pulmonary Kaolin pleurodesis ofthe right pleural space was parenchymal abnormalities in a woman, in theby copyright. undertaken following failure of conservative man- absence of any other cause for these conditions, agement by tube drainage and altered diet. Three rendered LAM the most likely diagnosis. weeks later the patient presented with a chylo- Since LAM occurs exclusively in women of thorax on the left side which was treated in a similar childbearing age, it is believed to be a hormone- manner. Oestrogen therapy was withdrawn. Lung dependent condition.4 Oestrogen has been postu- function tests performed 6 weeks after the second lated to play a causative role, and treatment has pleurodesis showed a decrease in lung function therefore included attempts at hormonal manipu- measured by spirometry (forced vital capacity: lation by oophorectomy,7 tamoxifen therapy8 and 1 litre). At her last review 21 years from the initial progesterone therapy.9 The disease is described as presentation with a chylothorax, the patient was being relentlessly progressive with death due to http://pmj.bmj.com/ asymptomatic and in good health. A recent chest respiratory failure occurring within 10 years of X-ray showed prominent lung markings at both diagnosis.'" lung bases but no evidence of deterioration of the Our case is of interest for a number of reasons. disease. Spirometric values have also remained Although oestrogen is strongly implicated in the stable. pathogenesis of LAM and LAM has been des- cribed in post-menopausal women on oestrogen supplementation,3 to our knowledge this is the first Discussion case of LAM to be reported in a primarily amenor- on September 30, 2021 by guest. Protected rhoeic woman in whom the only oestrogenic stimu- Lymphangioleiomyomatosis (LAM) is a disease of lus has been entirely exogenous. Also of interest is women of child-bearing age characterized by pro- the relatively long interval of 18 years between the liferation of smooth muscle along a lymphatic date of appearance of a radiological abnormality distribution, in the absence of cancer.6 Although and the clinical presentation. We believe that the the disease affects mainly the lungs causing spon- slow and atypical progression of the disease was taneous pneumothorax, chylothorax or rarely due to the lack ofa sustained oestrogenic stimulus, haemoptysis, peritoneal involvement is also docu- the patient having been on intermittent oestrogen mented.3 In some cases the disease is associated therapy only. Should the patient continue to with features of tuberous sclerosis. The disease is remain in a state ofclinical remission as she has for rare with fewer than a hundred cases having been the past 2+ years following the withdrawal of reported. oestrogen therapy, her case would provide direct The diagnosis of LAM is usually made on the evidence for the hormone-dependent nature of basis ofa lung biopsy but sometimes the distinctive LAM. Postgrad Med J: first published as 10.1136/pgmj.70.827.655 on 1 September 1994. Downloaded from CLINICAL REPORTS 657

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