Yellow nail syndrome resulting from cardiac mitral valve replacement
Yellow nail syndrome is a rare disease with unknown etiology, Attributed to functional anomalies or disturbance in lymphatic drainage. This condition is characterized by triad of nail discoloration, respiratory or intrathoracic manifestations and lymphedema.
Twenty days after mitral valve replacement for severe rheumatic mitral valve stenosis, 39 years old woman presented with face tenderness and hearing problems besides stuffy and clogged nose and underwent routin rhinosinusitis therapy. She came back to ears, nose and throat service with persistent rhinosinusitis as well as relapsing preoperative couphs and dyspnea besides lower extremities edema and toenails discoloration. After some modulations of treatment, she was introduced to pulmonary clinic on post -operative day = 30. Chest x ray showed a lot of left pleural effusion then she was returned to our service (cardiac surgery) on post- operative day = 33. The pigtail catheter was secured and we attained a significant amount of milky fluid which conformed with chylothorax. Finally Yellow nail syndrome was diagnosed with her on post–operative day = 35. Early conservative therapy such as bed rest, legs massage, low fat diet with medium chain triglycerides, diuretics, bronchodilator inhaler was not be able to satisfy us (chylous out put > 330 cc/d). Therefore the catheter replacement with chest tube was carried out followed by pleurodesis using Talc and doxycycline besides transition of oral intake to total parentral nutrition and vitamine E supplement, on post - operative day = 41. After that chylous leakage gradually subsided and patient was discharged to home on post- operative day = 47. At 4 weeks follow ups, chest x ray was clear without effusion and nails discoloration and legs lymphedema resolved.
We reported the third post cardiac surgery Yellow nail syndrome which is an unclear entity with a set of associated signs and symptoms. Two prior reports involved with coronary artery bypass graft whereas we performed mitral valve replacement. In angiogram thoracic duct was not identified so that it seems post cardiac surgery Yellow nail the syndrome has iatrogenic origin due to the thoracic duct or its tributaries injury and requires meticulous assessment and management.
KeywordsYellow nail syndrome Mitral valve replacement Thoracic duct injury Chylothorax Pleurodesis Pleural effusion Total parentral nutrition lymphedema
Two dimensional echocardiography
Anomalous pulmonary venous
Coronary artery bypass grafting connection
Chest X ray
Ears, nose and throat
Human phenotype ontology
Left internal mammary
Mitral valve replacement
Patent ductus arteriosus
Recurrent respiratory infection
Short – acting beta agonists
Superior vena cava
Tetralogy of fallot
Ventricular septal defect
Yellow nail syndrome
Yellow nail syndrome (YNS) is an idiopathic rare condition attributed to functional abnormalities of lymphatic drainage. It is characterized by three cardinal signs related to yellow nails, lymphedema and respiratory manifestations . That yellowing represents a subset of chromonychia, defined as pathological nail discoloration, especially xanthonychia (yellow nail coloration). It is a syndrome that associated with conditions as different as diseases implicating the lymphatic system, autoimmune diseases or cancers [2, 3]. The first case of YNS was probably reported by Heller in 1927 , but Samman and White described the first series of patients with YNS accompanied by lymphedema in 1964 . Whose report consisted of 13 patients whom had slow measured nail growth associated with nail discoloration ranging from pale yellow to dark greenish . According to the information comes from a databases called HPO, the most manifestations that may be in as high as 80–99% of patients are: Bronchiectasis, nail dysplasia, lymphatic vessels hypoplasia, lymphedema and yellow nails followed by couph, dyspnea, pleuritis, RRI and rhinosinusitis in 30 -79% of patients [7, 8]. Emerson added pleural effusion to the diagnostic criteria . Although two criteria from first group are required to diagnosis, it is difficult to call the entity YNS without nail discoloration. In addition to being yellow, nails may lack cuticle [10, 11], grow very slowly and become detached (onycholysis) [12, 13, 14]. Respiratory problems include chronic couph, bronchiectasis and pleural effusion. The complete Triad is present only in 27–69% of patients [15, 16, 17]. YNS often occurs in adults (age > 50 years) with no sex predominance . Estimated prevalence is < 1/10000000 [19, 20]. YNS in pediatrics is very rare [21, 22].
Discussion and conclusions
The authors wish to thank: Professor Zahra Sepehrmanesh, Dr. Soroush Sarmast & Dr. Behina Sarmast; for their assistance in this study. Also Dr. Kevin Brady & Dr. Fariba Brady from the USA state of New Jersey, For their editorial assistance.
As we are from lower income country, the processing charge has been waived.
Availability of data and materials
The datasets during the current study available from the corresponding author on reasonable request and for this purpose, the author received written consent from patient.
Both authors have contributed to the conception & writing the manuscript as well as have approved to the submission of the manuscript.
Ethics approval and consent to participate
In 2018, the ethics committee was held in the cardiac surgery center hospital of Damascus university and Department review board approval to publish a case report study entitled “ Yellow nail syndrome resulting from cardiac mitral valve replacement “ was obtained. As well as written consent was received from patient.
Consent for publication
Written consent for publication was obtained from patient.
The authors declare that they have no competing interests.
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