Successful surgical treatment of thoracic multiorgan lymphangiomatosis

Steinacher, Ina; Lamprecht, Bernd; Lobendanz, Markus; Zoller, Heinz; Dartevelle, Philippe; Fadel, Élie; Studnicka, Michael

doi:10.1007/s00508-009-1249-9

Cited by 6 publications

(2 citation statements)

References 14 publications

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“…6,7 In patients with large symptomatic chylothorax, repeated pleurocentesis, thoracoscopic thoracic duct ligation and pleurodesis have been reported. 3 Other measures include pleurodectomy, 3 pulmonary lymphangiectomy 8 and more recently, bilateral lung transplant. 9 Unfortunately the rarity of the disease can only allow for limited case series and no definitive treatment has been found thus far.…”

Section: Lymphangioleiomyomatosis (Lam) -Diffuse Lymphatic Proliferatmentioning

confidence: 99%

A Pleural Effusion with an Unusual Cause

Tan

Ong

Takano

2011

Proceedings of Singapore Healthcare

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Background and Aims: Peritoneal dialysis (PD) is a rare cause of pleural effusion. It appears as a complication of PD in approximately 2% patients. Hydrothorax in this situation is called "sweet hydrothorax" as hypertonic glucose solution fills the pleura. Methods: 62-year-old woman with end stage renal disease presented with dyspnea on exertion and chest pain for about 4 weeks after initiation of PD six months back. A chest x-ray was done showed massive unilateral right-sided pleural effusion. She underwent small bore chest drainage for massive pleural effusion.Pleural fluid was crystal clear with high glucose concentration of 735 mg/dl.The pleural fluid protein was 0.2g/dl .Cytological and microbiological examination of the pleural fluid showed no abnormalities .Pleural fluid analysis was consistent with transudate and pleural fluid glucose concentration was higher than patient serum glucose concentration suggestive of sweet hydrothorax.She was switched over to haemodialysis for 4 weeks. After 4 weeks she was started back on peritoneal dialysis with reduced fluid volume and reduced dwell time and increased frequency of fluid change without recurrence for six months under followup. Results: Hydrothorax may develop several weeks or months after starting of PD. Diagnostic pleural tapping and pleural-fluid analysis are often diagnostic, revealing a crystal clear pleural fluid with a low protein and a high glucose concentration. In the patient presented here, glucose concentration in the pleural fluid was much higher than that in the serum drawn concomitantly. Conclusions: It is advisable to keep sweet hydrothorax as a differential diagnosis while evaluating hydrothorax in patients on peritoneal dialysis.

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Section: Lymphangioleiomyomatosis (Lam) -Diffuse Lymphatic Proliferatmentioning

confidence: 99%

A Pleural Effusion with an Unusual Cause

Tan

Ong

Takano

2011

Proceedings of Singapore Healthcare

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“…Finally, 26 case reports and 36 participants were included in the pooled analysis. [7,13–15,19–40] As shown in Table 1, all screened patients were male. The median age of the participants was 34 years (IQR: 1–79).…”

Section: Resultsmentioning

confidence: 99%

Clinical features and outcomes of male patients with lymphangioleiomyomatosis: A review

Zhang

Wang

et al. 2022

Medicine

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Background: Lymphangioleiomyomatosis (LAM) is a rare disease involving multiple systems, which is divided into sporadic LAM (S-LAM) and tuberous sclerosis complex-LAM, mostly affecting women who are in childbearing age stage. Data on male patients are limited and scattered. Therefore, it is necessary to conduct a systematic review to investigate the clinical features, diagnosis, treatment, and outcomes of LAM in male. Methods: We performed a literature review by searching for all the published reported cases of LAM in male during the past 35 years (April 1986–October 2021). Results: 36 male patients described in 26 references were included in this article. The median age of onset was 34 years (interquartile range: 1–79). The most common initial manifestations were cough, dyspnea, respite, and hemoptysis, with pulmonary complications such as pneumothorax and chylothorax. Five patients (13.9%) were asymptomatic at admission. Nearly half of the 36 male patients had thin-walled air-filled cysts that were visible throughout both lungs. Considering the abovementioned atypical clinical features, misdiagnosis was committed in 8 patients (22.2%). In addition, patients with tuberous sclerosis complex lymphangioleiomyomatosis often have no pulmonary manifestations at onset but present multiple extrapulmonary manifestations and have higher rates of renal angiomyolipomas than patients with S-LAM ( P < 0.01). Eventually, 4 patients with S-LAM eventually died. Conclusion: Physicians should increase the awareness of LAM in male. Early monitoring of various systems should be recommended to ensure early management and active follow-up. Tuberous sclerosis complex patients should immediately be tracked for the onset of LAM disease to improve prognosis.

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Lymphangiomas and Lymphangiomatosis

Chen

2014

Atlas of Genetic Diagnosis and Counseling

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Successful surgical treatment of thoracic multiorgan lymphangiomatosis

Cited by 6 publications

References 14 publications

A Pleural Effusion with an Unusual Cause

A Pleural Effusion with an Unusual Cause

Clinical features and outcomes of male patients with lymphangioleiomyomatosis: A review

Lymphangiomas and Lymphangiomatosis

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