A young lady with severe dyspnea in postpartum period after long term infertility treatment / Uzun donem infertilite tedavisinden sonra postpartum daneminde ciddi dispne ile basvuran genc kadin hasta.
Lymphangioleiomyomatosis (LAM) is a disease that predominantly affects young females and generally progresses to respiratory failure and death. A 33-year-old postpartum woman was admitted with progressive dyspnea and high CA-125 levels. She had bilateral chylopneumothorax, chylous ascites and was diagnosed as LAM by open lung biopsy. Her management was very complex as her chylous ascites and pleural effusions were very resistant to therapy. Betadine pleurodesis was very successful in the management of chylothorax. There were no LAM cases presenting with high CA-125 levels. We believe that CA-125 levels could be used as a useful parameter in patient follow-up. (Tur Toraks Der 2011; 12: 127-30)
Key words: Lymphangioleiomyomatosis, pleurodesis, betadine, CA-125
Received: 25.11.2008 Accepted: 03.03.2009
Lenfanjioleimyomatozis (LAM) cogunlukla genc kadinlari etkileyen, genellikle solunum yetmezligi ve olume ilerleyebilen bir tablodur. 33 yasinda postpartum periyodda kadin hasta ilerleyici nefes darligi ve yuksek CA-125 seviyeleri ile basvurdu. Hastada bilateral silopnomotoraks, siloz asit mevcut idi ve acik akciger biyopsisi ile LAM tanisi aldi. Hastanin takibi oldukca karmasikti ve siloz asit ve plevral efuzyonlar tedaviye cok direncliydi. Betadin plorodezisi silotorakslann tedavisinde oldukca basarilt oldu. Literaturde yuksek CA-125 seviyeleri ile basvuran LAM vakasi sunulmamistir. Takipte CA-125 seviyelerinin yararii bir parametre olabilecegini dusunmekteyiz. (Tur Toraks Der 2011; 12: 127-30)
Anahtar seactikler: Lenfanjioleyiomyomatozis, plarodezi, betadin, CA-125
Gelis Tanhi: 25.11.2008 Kabul Tarihi: 03.03.2009
A 33-year-old chemistry teacher was referred in December 2006 with a one month history of progressive breathlessness in the last trimester of her first pregnancy. She had a history of intense hormonal therapy for infertility for the previous six years and she gave birth to twins after invitro fertilization by cesarian section one day earlier.
Physical examination showed orthopnea, tachypnea, tachycardia, dullness and diminished breath sounds in both hemithoraxes. There was no fever, cyanosis or clubbing.
Her arterial blood gas analysis in room air was pH: 7.47, [PCO.sub.2]: 30 mmHg, [PO.sub.2]: 50 mmHg and [SatO.sub.2]: 80%. Biochemical tests showed only two abnormal results: high triglycerides (760 mg/dl) and Ca-125 (250U/ml) in her serum which was otherwise normal.
Radiologic examination revealed a left-sided hydropneumothorax and right sided hydrothorax. HRCT scan of the chest showed diffuse bilateral thin walled microcysts in the lung parenchyma (Figure 1). Abdominal CT revealed multiple paraaortic lymhadenopathies and massive peritoneal fluid.
We inserted bilateral chest tubes immediately and air with white opaque fluid drained from both sites at an average of 800 cc per day for each site. High triglyceride, low cholesterol level, cholesterol/triglyceride ratio<1 were detected in both pleural fluid and peritoneal fluid and total parenteral nutrition was started. After 14 days, as the chylous drainage continued in the same degree, we performed thoracic duct ligation and open lung biopsy with right thoracotomy (Figure 2).
Pathologic examination confirmed the diagnosis of lymphangioleiomyomatosis (LAM) (Figure 3).
Bilateral chylous drainage had persisted in large amounts from the chest tubes although we continued total parentheral nutrition (TPN). We then decided to perform right thoracotomy for thoracic duct ligation and to obtain a lung biopsy (Table 1). Thoracic duct ligation eliminated the left chylothorax but right chylothorax persisted. Firstly talc pleurodesis was tried but unfortunately this was unsuccessful. A good pleurodesis was followed by betadine.
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We started an octreotide infusion at 2 mg/kg/hour and planned to give this for 5 days as suggested in the literature when chylous ascites was over 10 liters. Two days later, the dose was increased to 4 mg/kg/hour. The chylous drainage gradually diminished from day 2 to day 5 of octreotide infusion. Her chylous ascites persisted in a minimal amount.
In the second month of her hospitalization she was diagnosed as acute pulmonary embolism with spiral tomography of thorax. Her anticoagulation therapy was ended at 6 months as her congenital thrombophilic markers were found negative.
While CA-125 levels were very high (250 U/ml) on admission, it decreased to normal limits (0-35 U/ml) with therapy.
She is still in follow-up for 2.5 years with medical therapy with GnRH analogue. She has mild exertional dyspnea and we performed high resolution computerised tomography (HRCT) of the lungs, pulmonary function tests (PFT), diffusion capacity of lungs (DLCO) and abdominal USG at her clinical visits. Pneumothoraxand chylothorax did not rcurt, and minimal abdominal chylous ascites persisted, her last DLCO showed a moderate decrement. She is now clinically stable and working as a chemistry teacher.
LAM is a disease that predominantly affects young females and generally progresses to respiratory failure and death. The clinical features result from progressive cystic destruction of the lungs and the accumulation of LAM cells within the lungs and axial lymphatics. The disease tends to present between the menarche and the menopause, with the mean age of onset being 34 yrs. Occasionally, post-menopausal females will present with the disease; however, these patients are often receiving oestrogen replacement therapy. The disease is rare (termed sporadic LAM) with a prevalence of 1 in 1,000,000 population (1), (2).
The pulmonary symptoms tend to dominate the clinical course, with the most common features being pneumothorax progressive dyspnoea and chylous pleural effusions (2-4).
Chylothorax may contribute to symptomatic dyspnoea in LAM and occurs by one of three mechanisms: 1) obstruction of the thoracic duct and its tributaries; 2) leakage from pleural lymphatics; and 3) transdiaphragmatic flow from chylous ascites. Treatment is aimed at obliterating the pleural space to prevent lymphatic accumulation, or ligation of the thoracic duct. The natural history of chylous collections is variable; while some will have small persisting effusions that require no treatment, for moderate or large effusions associated with dyspnoea, treatment is required. In most cases, simple aspiration or chest tube drainage results in re-accumulation in a short time. Patients with persisting effusions after aspiration have been successfully treated by pleurodesis, pleurectomy and, in some cases, thoracic duct ligation. Early experience with thoracic duct ligation was poor; however, this now appears to be a relatively safe procedure in patients with LAM (5).
Table 1. Clinical progress of the patient Date Problem Treatment Result 08.12.2006 Infertility Six year intense Gave birth hormonal therapy+in with cesario vitro fertilization section 09.12.2006 L Chest tube Air and hydropneumothorax chylous leakage 01.12.2006 R Chest tube Chylous hydropneumothorax drainage High amount chy 14 days TPN No response 22.12.2006 Sustained R thoracotomy (Ductus L bilateral chy thorasicus lig+Open chylothorax lung biopsy) succesful, R chylothorax no response 31.12.2006 R Talc pleurodesis Failure for hydropneumothorax chy, successful for px 02.01.2007 R chylothorax Betadine pleurodesis Successful 16.01.2007 Chylous ascites Drainage (10 liter) Failure Sustained chylous Somatostatin infusion Successful ascites (4mg/kg iv for 5 days) 17.01 Pulmonary Anticoagulation Successful .2007 embolism 30.04.2007 L pneumothorax Betadine pleurodesis Successful R: right, L: left, chy: chylous effusion, hg: ligation, px: pneumothorax, lv: intravenous, TPN: total parenteral nutrition
Ooopherectomy is generally performed for LAM, and gonadotrophin-releasing hormone agonists can produce the same reduction in oestrogen levels as ovarian ablation; however, evidence for their use is restricted to case reports (6).
In the literature, a case report about exacerbation of pulmonary LAM by exogenous oestrogen used for infertility treatment was reported earlier (5), (7). The presence of LAM should be considered before initiating infertility treatment.
Although we know that talc pleurodesis is very effective, in this case betadine (povidone-iodine) produced good results in pleurodesis (8), (9). We consider that betadine, a very acidic agent, could have destroyed lipid particles in the chylothorax.
While CA-125 levels were very high (250 U/ml) on admission, it decreased to normal limits (0-35 U/ml) with therapy. Zahner et al. reported that CA-125 could be a suitable screening parameter for LAM, which might additionally be of prognostic value, and he recommended further studies for sensitivity and specificity of CA-125 (10). We did not find any LAM case presenting with high CA-125 levels. In our case, while CA-levels continued high, it decreased to normal limits when the patient was in a stable clinical condition. We believe that CA-125 levels could be used as a useful parameter in follow-up.
As is clearly seen from our patient's progress table above, diagnosis and management of LAM cases could be a very chaotic process and it often requires emergency decisions and an efficient team to cope with this disease.
In this new millennium, great progress has been achieved for infertility problems and women have a tendency to give birth to their first babies in their late thirties or even forties. However, these intense therapies could produce some hormone-related pathologies in women. For this reason, both gynecologists and pulmonary physicians should be aware of such problems in these patients.
(1.) Johnson SR, Tattersfield AE. Decline in lung function in lymphangioleiomyomatosis: relation to menopause and progesterone treatment. Am J Respir Crit Care Med 1999;160:628-33.
(2.) Urban T, Lazor R, Lacronique J, et al. Pulmonary lymphangioleiomyomatosis. A study of 69 patients. Groupe d'Etudes et de Recherche sur les Maladies "Orphelines" Pulmonaires (GERM"0"P). Medicine (Baltimore) 1999;78:321-37.
(3.) Johnson SR, Tattersfield AE. Clinical experience of lymphangioleiomyomatosis in the UK. Thorax 2000;55: 1052-7.
(4.) Kitaichi M, Nishimura K, Itoh H, Izumi T. Pulmonary lymphangioleiomyomatosis: a report of 46 patients including a clinicopathologic study of prognostic factors. Am J Respir Crit Care Med 1995;151:527-33.
(5.) Johnson Sr. Lymphangioleiomyomatosis Series "Rare Interstitial Lung Diseases" Edited By Vogelmeier C And Costabel U. Number 1 Eur Respir J 2006;27:1056-65.
(6.) Desurmont S, Bauters C, Copin MC, Dewailly D, Tonnel AB, Wallaert B. Treatment of pulmonary lymphangioleiomyomatosis using a GnRH agonist. Rev Mal Respir 1996;13:300-4.
(7.) Yano S. Exacerbation of pulmonary lymphangioleiomyomatosis by exogenous oestrogen used for infertility treatment. Thorax 2002;57;1085-6.
(8.) Brissaud O, Desfrere L, Mohsen R, Fayon M, Demarquez JL. Congenital idiopathic chylothorax in neonates: chemical pleurodesis with povidone-iodine (Betadine). Arch Dis Child Fetal Neonatal Ed 2003;88:F531-3.
(9.) Kilic D, Sahin E, Gulcan 0, Bolat B, Turkoz R, Hatipoglu A. Octreotide for treating chylothorax after cardiac surgery. Tex Heart Inst 1 2005;32:437-9.
(10.) Zahner J, Strauer BE. CA-125-a tumor marker of lymphangioleiomyomatosis of diagnostic and prognostic importance? Geburtshilfe Frauenheilkd 1993;53:492-4.
Nese Dursunoglu, (1) Seyda Ors, (2) Samil Gunay, (2) Ferda Bir, (3) Sevin Baser, (1) Sibel Ozkurt, (1) Nevzat Karabulut, (4)
(1.) Pamukkale Universitesi Tip Fakultesi, GegLis Hastaliklan Anabilim Dali, Denizli, Turkey
(2.) Pamukkale Universitesi Tip Fakultesi, GibgCls Cerrahisi Anabilim Dali, Denizli, Turkey
(3.) Pamukkale Universitesi Tip Fakultesi, Patoloji Anabilim Dali, Denizli, Turkey
(4.) Pamukkale Universitesi Tip Fakultesi, Radyodiyagnostik Anabilim Dal', Denizli, Turkey
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|Title Annotation:||CLINICAL PROBLEMS / KLINIK SORUNLAR|
|Author:||Dursunoglu, Nese; Ors, Seyda; Gunay, Samil; Bir, Ferda; Baser, Sevin; Ozkurt, Sibel; Karabulut, Nevz|
|Publication:||Turkish Thoracic Journal|
|Date:||Sep 1, 2011|
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