A case of pregnancy associated with the rare yellow nail syndrome: An overview and review of literature

Abstract

The yellow nail syndrome (YNS) is a rare disorder characterized by the classic triad of yellow and dystrophic nails, lymphedema along with pleural effusion, resulting from malformations of the lymphatic system. The first case of yellow nail syndrome was probably reported by Heller in 1927, but Samman & White described the first series of patients who had yellow nails associated with lymphedema in 1964.The pathogenesis of YNS still remains poorly defined. The diagnosis is established on the basis of characteristic clinical features including abnormal nails, lymphedema and any respiratory manifestations. The clinical course is usually benign, and current treatment plan aims at controlling the various clinical manifestations of this obscure disease process.Here we report an antenatal case who reported to us in her third trimester for safe confinement and delivery, already a diagnosed case of yellow nail syndrome with occasional bouts of cough on and off with no associated breathlessness. She had associated gross bilateral lower limb oedema and characteristic yellow nail changes. Physical examination revealed uterus corresponding to term gestation, relaxed and fatal heart sound good.She never had any history of associated chest pain, palpitation, haemoptysis or required hospitalization for these episodes. She gives no further association with exacerbations of any respiratory ailments. Antenatal scan along with Doppler study revealed Breech presentation at 37-38 weeks gestation, normal Doppler, a small subserous fibroid 5x4 cms near to fundus noted. The patient was taken up for an elective caesarean section in view of the present condition of the patient and the further anticipated complications. Our case gives a glimpse of the pregnancy associated with this rare syndrome where appropriate medical treatment for their respiratory symptoms and oedema should be carried out and close monitoring of the patient is of utmost requirement in view of the anticipated complications. The yellow nail syndrome (YNS) is a rare disorder of unknown cause characterized by the triad of yellow and thickened nails, lymphedema and respiratory manifestations. The pathogenesis along with clinical presentations and natural history of this disorder remain, for the most part, obscure. Owing to the absence of large-scale studies in relation to YNS, there is no consensus on treatment strategy. Management decisions are typically based on anecdotal evidence, case reports and intuition. Treatment is predominantly supportive, but it should be further noted that successful treatment of one component of the triad often causes symptomatic improvement in the others.The YNS remains a rare and intriguing disorder of unknown cause. Lymphatic dysfunction probably represents the pathogenic mechanism responsible for the various clinical manifestations associated with this disorder. Most clinical manifestations of YNS are generally managed symptomatically with supportive measures, and the long-term prognosis generally appears favourable.