Primary Pulmonary Lymphatic Flow Disorder Associated Plastic Bronchitis

Lymphatic plastic bronchitis (PB) most commonly occurs in children with congenital heart disease as a result of secondary pulmonary lymphatic flow disorder (PLFD). However, PB caused by primary PLFD is rare. The clinical symptoms of lymphocytic PB are nonspecific; thus, the diagnosis may be long delayed, especially in the absence of chylothorax. We made a retrospective analysis of two children diagnosed with PB due to primary PLFD, in order to contribute to further understanding of these disorders. Patient 1, an eight-year-old boy, presented with chronic productive cough and expectorated milky-white mucous plugs accompanied by intermitted wheezing for one year. Patient 2, a nine-month-old girl, presented with episodes of acute respiratory distress with expectoration of milky-white bronchial casts for four months. There was no obvious evidence of infection in either child. Bilateral thickening of bronchovascular bundles and interlobular septal, as well as multiple patchy ground-glass opacities were seen on chest computed tomography (CT) in both patients. Lymphangioscintigraphy demonstrated pulmonary lymph reflux in both patients and slowed lymphatic drainage of the lower limbs in patient 1. Primary PLFD was considered for both patients, and a diagnosis of yellow nail syndrome was made in patient 1. Both patients received lymphatic interventional treatment, but all experienced recurrence following the procedure.

Yellow nail syndrome

Yellow nail syndrome is an extremely rare syndrome, mainly in people over 50 years of age, occurring both systemically and in isolation and requiring the most careful collection of anamnesis, since this condition has a close relationship with respiratory diseases, malignant neoplasms of internal organs and rheumatoid arthritis. Moreover, this rare disease is not sufficiently studied to fully understand its pathogenesis and effective treatment. Patients pay attention to the yellow color of the nails, associated with the deposition of melanin, bile pigments and hemosiderin in the submarginal space, slowing down the growth and thickening of the nail. It should be noted that the change in the nail plates can be observed long before the other clinical manifestations of this syndrome are detected, and probably this can in some cases serve as a harbinger of incipient changes in the lung tissue, neoplasms and changes in the lymphatic vessels. In this regard, it is extremely important to clearly differentiate this condition and refer patients to related specialists for verification of the diagnosis and further treatment.

Respiratory manifestation of yellow nail syndrome: a case report and literature review

Yellow nail syndrome (YNS) is a rare disorder, and diagnosis is based on the clinical findings and the exclusion of other possible causes; the pathogenesis is poorly understood. YNS can be an isolated condition or associated with other diseases; however, YNS associated with multiple myeloma (MM) is rare. A 53-year-old male patient presented with coughing and shortness of breath, and he was diagnosed with YNS with MM. He underwent chemotherapy and achieved a good response. Although the etiology of YNS remains unknown, treating the underlying disease may help prevent or relieve the clinical signs.

Unusual presentation of a man with recurrent chylous ascites

We describe an interesting case of a 77-year-old man presenting with refractory chylous ascites of unknown aetiology. After extensive diagnostic workup, unifying diagnosis of an intriguing condition of yellow nail syndrome was reached. This case is unusual as it describes a rare cause of chylous ascites in this age group. Despite refractory ascites and the need for recurrent paracentesis, this patient has a good prognosis with no significant impact on overall mortality.