dyskeratosis congenita ( dc ) is an inherited disorder with progressive multi - system involvement leading to bone marrow failure ( bmf ) , squamous cell carcinoma , pulmonary fibrosis ( pf ) , etc . liver transplantation ( lt ) for cirrhosis secondary to dc has not been reported previously and we describe here the perioperative management of the same . a patient with diagnosed dc at 25 years of age presented with esld to our institute in his 34 year . past history included haematopoietic stem cell transplant ( hsct ) for bmf secondary to dc . patient was diagnosed with cirrhosis post - hsct and was kept on medical management for 9 years . at the time of presentation he was not on any immunosuppressant . clinical examination revealed multiple dental caries , reticulate skin pigmentation , nail dystrophy , severe clubbing , epiphora , enlarged liver with irregular margins , splenomegaly and ascites . pre - operative liver function tests showed hypoalbuminemia ( 2.6 g / dl ) with slightly elevated transaminases . on arterial blood gas ( abg ) analysis , partial pressure of oxygen ( pao2 ) was 51.3 mm hg with widened alveolar arterial oxygen gradient ( pao2pao2 = 59.7 mm hg ) [ table 1 ] . abg with 100% o2 supplementation demonstrated an increased pao2 ( 158 mm hg ) and spo2 of 100% [ table 1 ] . arterial blood gas values at various time points high resolution computerized tomography ( ct ) demonstrated mild bilateral interstitial lung disease ( ild ) [ figure 1 ] . saline contrast echocardiography ( sce ) suggested severe right to left intrapulmonary shunting and macroaggregated albumin scan ( maa ) demonstrated a 21% shunt fraction . child - turcotte - pugh score was 10 ( child c ) and model for end stage liver disease score was 14 . patient was diagnosed with cirrhosis secondary to dc and posted for urgent living donor liver transplantation ( ldlt ) . high resolution computerized tomography thorax showing mild bilateral interstitial lung disease apart from standard anaesthesia monitoring , invasive haemodynamic monitoring ( central venous pressure , invasive arterial blood pressures and continuous cardiac output monitoring using flowtrac ev1000 edwards life sciences ) were used . anaesthesia was induced with propofol ( 1.5 mg / kg ) , fentanyl ( 1.5 gm / kg ) , and rocuronium ( 0.9 mg / kg ) with modified rapid sequence induction . maintenance of anaesthesia was with isoflurane in air ( minimum alveolar concentration 11.2 ) , with infusions of atracurium and fentanyl . baseline abg after induction of anaesthesia showed pao2 of 140 mm hg with fio2 of 0.5 . persistent svv > 13% in absence of sustained major vascular occlusion or arrhythmias was treated with 250 ml boluses of 5% albumin . noradrenaline and vasopressin infusion were titrated for a targeted mean blood pressure > 65 mm hg . after reperfusion , fio2 requirements increased from 0.5 to 0.7 to maintain the pao2 above 100 mm hg . estimated blood loss during the surgical procedure was 2100 ml . patient was gradually weaned from mechanical ventilation and vasopressors and extubated on post - operative day 2 in the intensive care unit . post - extubation intermittent non - invasive bilevel positive airway pressure ( bipap ) support was provided . immunosuppression was provided with steroids , tacrolimus and mycophenolate . on post - operative day 7 , patient developed tachypnoea , increased o2 requirement , fever and productive cough . chest auscultation revealed bilateral basal crepitations and basal lobe consolidation on chest x - ray . oxygen requirements were increased and were provided via high concentration o2 mask with reservoir bag to maintain acceptable levels of saturation . on availability of sputum culture reports ( culture demonstrated escherichia coli sensitive to carbapenems ) , patient was shifted from broad spectrum empirical antibiotics to culture specific antibiotics and patient gradually improved with decrease in oxygen requirements . patient was discharged from the hospital on post - operative day 29 with a pao2 of 70 mm of hg on room air , which at 2 months was 78 mm of hg . dyskeratosis congenita is a rare multisystem disorder characterized by the triad of abnormal skin pigmentation , nail dystrophy and mucosal leucoplakia . patients exhibit considerable clinical and genetic heterogeneity with x - linked recessive , autosomal dominant and recessive forms . bone marrow failure and pf are the most common causes of mortality(6070% for bmf/15% for pf ) in these patients . hsct is the only curative option for bmf but increases the risk of pulmonary complications . hepatic involvements including hepatomegaly , hemosiderosis , fibrosis , and cirrhosis have been reported in 10% of cases . presence of severe hypoxemia and orthodeoxia and absence of ild led to a probable diagnosis of hepatopulmonary syndrome ( hps ) . imbalance between vasoconstrictors and vasodilators leading to pulmonary vascular dilatation at the pre - capillary and capillary level in patients with liver disease results in hps . subsequent intrapulmonary shunting and v - q mismatch causes progressive hypoxia , dyspnoea , and cyanosis . the patient reported here was fulfilling the criteria for hps : ( 1 ) chronic liver disease ( cld ) , ( 2 ) alveolar - arterial oxygen gradient > 15 mm of hg and intrapulmonary shunt ( demonstrated by sce and maa ) . severe intrapulmonary shunting ~21% and pao2 of 51.5 mm of hg on room air placed him in almost the very severe category of type i hps . lt being a definitive treatment for hps and severity of hypoxaemia limiting time for medical therapy trial , lt was offered to the patient on an urgent basis . the prevalence of hps in patients with advanced liver disease is approximately 1633% . without lt the 5 years survival rate for cirrhotic patients with hps however , more recently gupta et al . in their study have reported a mortality rate of 9% in patients with severe hps in peri - transplant period . it has also been demonstrated that severity of pre - operative hypoxaemia correlates with the peri - transplant mortality . rapid progression of pre - operative hypoxaemia in these patients mandates minimal transplant waiting time . ldlt reduces transplant waiting time and prevent functional status deterioration and thus provide good results in patients with severe hps . the low acceptable thresholds of pao2 values were kept keeping in mind pre - operative severe hps and hypoxaemia . restrictive fluid therapy using svv along with lung protective ventilation strategies ( low tidal volume ) were used to minimize pulmonary complications . other supportive therapies like o2 supplementation , incentive spirometry , intermittent non - invasive ventilatory support , adequate attention to asepsis and antibiotics were provided as per institute 's protocol . hps can be an important cause of hypoxemia in patients of dc presenting with cld . directed pre - operative evaluation to determine the primary cause of hypoxaemia can help in identifying patients who may benefit from lt .