Serious complications of pulmonary biopsy in a boy with chylopericardium and suspected pulmonary lymphangiomatosis

Abstract

Lymphangiomatosis is a rare disorder. It is commonly localised in the skeletal system and called Gorham's disease, and in thoracic or abdominal organs. Involvement of the pericardium is rare and has been described in less than 20 patients worldwide. The case of a 14-year-old boy presenting with asymptomatic chylopericardium and interstitial lung disease is presented. After lung biopsy, performed to confirm the diagnosis of pulmonary lymphangiomatosis, he developed chylothorax and massive loss of chyle via chest drains. Thoracic duct ligation did not result in clinical improvement. Treatment with interferon α2b was given and because of clinical deterioration radiotherapy was added to the treatment. This resulted in a rapid decrease of chyle production in the patient.

Transthoracic ultrasound: an essential diagnostic tool in a very rare case of thoracic lymphangiomatosis

“…On the other hand, in this particular case, a less invasive diagnostic method was of major importance and led to the final diagnosis, taking into account the altered respiratory status, that posed a greater anesthetic risk if a fourth intervention would have been performed. Another important issue is represented by the potential severe complications of open biopsy in patients with TL as mentioned in the literature [8], which supports the need of a minimal invasive diagnostic approach, such as a US-guided biopsy. Concomitant with the advances of endoscopic ultrasound FNA, which is feasible even for paraesophageal lung tumors [9], it is obvious that the intrathoracic organs benefit from another technique that can procure minimally invasive biopsies.…”

Diffuse pulmonary lymphangiomatosis

“…We suspect that chyloptysis is due to the suddenly decreased pressure in lymphatic vessels after bronchoscopy. Chylothorax has been reported after open lung bioscopy [13] or surgical removal of the mediastinal mass. [14] We suggest that non-invasive methods for diagnosis should be first considered, especially in asymptomatic patients [13] or in patients with severely enlarged mediastinum or with thin-walled translucent vesicle under endoscopy.…”

Primary pulmonary lymphatic flow disorder associated plastic bronchitis

“…However, diagnostic pulmonary biopsy was not performed due to the risk of massive chyloptysis or refractory chylothorax. 3,4 Splenic cystic lesions were observed on abdominal ultrasonography, suggesting extrathoracic involvement. No diseasecausing mutations were found in genes associated with congenital lymphatic dysplasia.…”