Yellow nail syndrome resulting from cardiac mitral valve replacement

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 [1]. 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 [4], but Samman and White described the first series of patients with YNS accompanied by lymphedema in 1964 [5]. Whose report consisted of 13 patients whom had slow measured nail growth associated with nail discoloration ranging from pale yellow to dark greenish [6]. 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 [9]. 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) [12‐14]. Respiratory problems include chronic couph, bronchiectasis and pleural effusion. The complete Triad is present only in 27–69% of patients [15‐17]. YNS often occurs in adults (age > 50 years) with no sex predominance [18]. Estimated prevalence is < 1/10000000 [19, 20]. YNS in pediatrics is very rare [21, 22].

YNS has remained vague entity owing to lack of information about this very rare condition.There are little available data determining exact prevalence of YNS, as fewer than 400 cases have been published in the literature. Although two instances of familial cases have already been reported that in one sample, two siblings and in the other example, four offsprings during two generations had YNS [23, 24], the very few reported familial cases mimic a dominant inheritance pattern, which is not supported by any genetic evidence. In the other words, it has not been suggested any genetic link [25, 26]. It often occurs on its own even though is occasionally associated with autoimmune disease [27], lymphatic disease or cancers immunodeficiency disorders, such as common variable immunodeficiency, combined T- and B-cell deficiency [28, 29], Guillain–Barré syndrome [30], nephritic syndrome [31, 32], Hashimoto’s thyroiditis, severe hypothyroidism or hyperthyroidism [33, 34], xanthogranulomatous pyelonephritis [35] and rheumatoid arthritis even without thiol-analog use [36]. Very rare ocular involvement has been reported: chemosis, corneal micropannus (vascularized sheet of fibroustissue overlying the cornea), eyelid lymphedema, thickened conjunctiva [37]. Anecdotal associations have also been described: anhydrosis, pectus excavatum, eosinophilia–myalgia syndrome, bullous stomatitis, sarcoidosis and Raynaud’s phenomenon, cerebralaneurysm and pancytopenia. YNS is very rarely associated with primary intestinal lymphangiectasia (Waldmann’s disease) (OMIM 152800, ORPHA90362) or lymphedema – distichiasis syndrome (OMIM 153400, ORPHA33001), suggesting that these entities have overlapping characteristics, including lymphatic impairment [38, 39]. YNS treatment is not codified. YNS may resolve in few months without treatment [40] or, when it is a paraneoplastic syndrome, after cancer therapy [41]. Symptomatic treatments are prescribed. Patients may receive antibiotics for acute exacerbation of bronchiectasia, whereas, for patients with poor symptom control and/orrecurrent exacerbations, low-dose antibiotic prophylaxis, such as oral azithromycin (usually 250 mg 3 times/week), achieved attenuation of chest symptoms for the majority of them. Physiotherapy training (postural drainage, chest physiotherapy, flutter valve), combined or not with antibiotic prophylaxis, is also prescribed to help patients self-manage their chronic expectoration. Vaccinations against flu and pneumococci are strongly recommended. Surgical intervention of recurrent and/or large pleural effusions is useful: decortication/pleurectomy, pleurodesis (talc, picibanil quinacrine) and pleural–peritoneal shunts were the most effective treatments of symptomatic pleural effusions with, respectively, 89, 82 and 67% partial or complete responses [42, 43]. Octreotide, a somatostatin analog, was also used to treat YNS pleural effusions or chylous ascites and lymphedema, and generated positive responses [44‐47]. We reported the third post cardiac surgery YNS. The first was reported in 2009 and an 80 years old male presented with YNS after CABG. The second report in 2018, belongs to a 62 years old man who endured CABG too and after that suffered from YNS. In both of them, there was coexistence regarding the LIMA harvesting and chylothorax (involving 81%), suspiciously owing to tight proximity between LIMA and thoracic duct. How is about our case? Our operation nowise correlated with LIMA. We don’t have any information about patient lymphatic system prior to the operation whether it was normal or aberrantly abnormal [48‐52]. In our case the lymphangiogram demonstrated diffused abnormal indocyanine pattern in lower extremities especially on the left side but was not be able to identify the cycterna chyli and thoracic duct while showed sparse droplets of lipidol in left pleural cavity without any apparent leakage site (Fig. 5). On the other hand, doppler echocardiography precluded possibility of central venous embolization. All of the aforementioned evident findings are saying that, damage to the thoracic duct or lymphatic vessels is undertaken unless proven otherwise. According to the observations in majority of series, post thoracotomic chylothorax has been reported in the frequency of 0.2% [52‐57]. Blalock and coworkers for the first time noted chylothorax following SVC ligation in 1936 . Then other surgeons observed similar findings (chylothrax) after surgical corrections such as: aortic coarctation, BTS, vascular slings, PDA, TOF, TS, VSD and APVC. Nobody other than two authors has reported full blown post cardiac surgery YNS and our report is third.