Spider nevi or angiomas are one of the cutaneous manifestations seen in liver cirrhosis patients, more commonly in alcohol-associated liver disease. It is identified by the presence of central red arteriole and radiating vessels resembling a spider.1 Although small spider nevi are common in cirrhosis, giant-sized spider nevi are rarely reported in literature 2. Further, they are not known to bleed profusely, and it is unclear whether they resolve after transplant. Here we report a rare case of cirrhosis with hepatopulmonary syndrome, intracranial bleed, and giant spider angioma that resolved soon after live donor liver transplantation.
Case report
A 45-year-old male patient presented to our center with alcohol-related decompensated liver cirrhosis with jaundice, ascites, and hydrothorax. He had a giant (3.5 × 3 cm) vascular reddish purple-colored soft tumor-like lesion on forehead for the last 5 months, which was later diagnosed as spider angioma. He had a history of recurrent bleeding from this lesion started 2 months ago, which was controlled with local compression and correction of underlying coagulopathy. Smaller-sized spider nevi were also present on the chest and arms. He also complained of shortness of breath, and his room air SpO2 varied between 85 and 90%, while before transplant on ABG analysis, PaO2 was 70 mm. Hepatopulmonary syndrome (HPS) was confirmed on CT pulmonary angiography and bubble contrast echo. In view of advanced liver failure with MELD-Na of 18 and CTP class C, he was worked up for liver transplant. During admission, he developed an altered sensorium without any focal neurological deficit. He was started on standard treatments, including lactulose and rifaximin, and also underwent an MRI brain with angiogram that showed left temporal and gangliothalemic bleed without any significant mass effect or midline shift. There was no arteriovenous malformation present. His sensorium improved with medical therapy only. His follow-up brain imaging showed significant resolution of hematoma, and he was cleared by the neurosurgery and neurology team for liver transplant surgery.
He underwent live donor-related transplantation on March 8, 2021, the donor being his wife. He received a modified right lobe graft with a graft recipient weight ratio of 1.6. The transplant surgery was uneventful, and in the postoperative period, blood pressure was kept on the lower side (maintained within 25% of the preoperative blood pressure) in view of the previous intracranial bleed. Cyclosporin was used in place of tacrolimus in the post-transplant immunosuppressive regime as cyclosporin is less neurotoxic. The patient made a smooth recovery in the post-transplant period, and his giant spider nevi on the forehead started to decrease in size, and by the end of the second-week post-transplant, it was hardly noticeable (Figure 1, Figure 2). After the liver transplant his SpO2 and PaO2 gradually improved and normalized by the 12th week. However, we did not perform contrast ECHO or chest imaging to confirm the reversal of HPS after liver transplant. At 3 months follow-up, he is asymptomatic with normal liver function tests and his forehead spider nevus has resolved completely.
Figure 1.
Forehead giant spider nevus before transplant.
Figure 2.
Significant resolution of spider nevus 3 weeks post-transplant.
Discussion
Underlying etiopathogenesis of spider angiomas in cirrhosis is not completely understood and increased estrogen to testosterone ratio and hyperdynamic circulatory state are supposed to play a role.3 Other than cirrhosis, these lesions also seen in thyrotoxicosis, pregnancy, and patients on oral contraceptive pills. Lesions can be single or multiple and are usually seen in the superior vena cava distribution area involving the chest, arms, necklace area and face4 Few studies have associated them with the degree of liver dysfunction and hepatopulmonary syndrome.4,5 Spider angiomas and hepatopulmonary syndrome occur due to hyperdynamic circulation, pulmonary and cutaneous vasodilation and opening of A-V (arteriovenous) shunts and likely represent the spectrum of cardiovascular abnormalities seen in cirrhosis. Association of spider nevi and hepatopulmonary syndrome in liver cirrhosis has been termed as hepato-pulmonary-cutaneous syndrome.6 Prevalence of these lesions is about 33% in cirrhosis, but giant spider nevi of more than 2 cm in size are very rare.7 We came across only a few case reports of large spider angioma lesions in cirrhotic patients, including one in which a patient with multiple nevi underwent liver transplantation.8,9 However, resolution of such a giant lesion following liver transplantation has not been described before in literature. Skin and cerebral AV malformations occur in some rare hereditary neurocutaneous syndromes, but the association of cutaneous spider angiomas in cirrhosis patients with intracranial bleed has not been reported before. Erythrocytosis due to chronic hypoxemia in HPS and systemic emboli reaching to cerebral circulation because of intrapulmonary shunting may be the two plausible explanations for the intracranial events in these patients.10,11 Further studies are warranted given the high prevalence of spider angiomas (33%) in cirrhosis and association of these lesions with intracranial bleed in cirrhotic patients may be clinically significant. Our case report signifies that spider angiomas in liver cirrhosis can be a part of the hepato-pulmonary-cutaneous syndrome, and even giant spider angiomas in liver cirrhosis patients do not need a specific therapy and resolve with correction of underlying portal hypertension and liver failure. Association between giant spider angioma and intracranial bleed needs further evidence.
Credit authorship contribution statement
Dr. Shekhar Singh Jadaun, MD DM, draft writing, concept, revision. Dr. Sanjiv Saigal, MD DM, draft writing, critical revision. Dr. Ana Hasnain, MBBS MSc, draft writing, Dr. Mukesh Kumar, MS DNB, draft writing, Dr. Dhiraj Agrawal, MD DM, draft writing. Dr. Shweta A. Singh, MD, draft writing. Dr. Dibyajyoti Das, MBBS, IDCCM, draft writing. Dr. Shaleen Agarwal, MS, MCH, draft writing. Dr. Subhash Gupta, MS, FRCSED, FRCS, critical revision.
Conflicts of interest
The authors have none to declare.
Funding
No grant or financial support was taken for this research.
Disclosure
None of the authors have any financial, professional or personal conflicts that are relevant to the manuscript.
Note
Consent was taken for the publication of this case report.
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