Early experience of thalidomide therapy for high-grade peripheral and facial arteriovenous malformations.

BACKGROUND: Arteriovenous malformations (AVMs) are developmental defects in the vascular system with abnormal connections between arteries and veins. A minority of AVMs are characterized by aggressive growth and continue to proliferate despite maximal surgical and interventional therapy. We report our outcomes with the use of thalidomide as the only UK specialist center adopting this novel approach for the management of AVMs refractory to conventional therapy. METHODS: This was a retrospective case series which included only complex and proliferative AVM lesions (Schobinger grade III and IV). All patients prescribed thalidomide on a compassionate basis between September 2006 and August 2022 after attempts at embolosclerotherapy without satisfactory response were reviewed. RESULTS: Eleven patients were included in our study. The median total duration of thalidomide use was 10 months. Two thirds of patients with pain (six of nine) reported an improvement, three quarters reported a reduction in swelling (six of eight) and all who presented with bleeding reported improvement in overall volume or frequency (four of four). Over the study period, 45% achieved a non-proliferative state with no further target vessel demonstrable on angiography. Mild, tolerable side effects such as fatigue were common (73%). There was only one major adverse reaction (neutropenia) necessitating cessation of therapy. CONCLUSIONS: We can conclude that thalidomide is able to reduce the symptom burden for patients with complex and proliferative AVMs that were refractory to established treatment modalities. Adverse effects are common, but the benefit achieved from taking thalidomide in otherwise treatment resistant cases outweighs the risks, most of which are manageable.

Efficacy and safety of embolo-sclerotherapy of arteriovenous malformations with foam sodium tetradecyl sulphate.

BACKGROUND: To evaluate the efficacy and safety of embolo-sclerotherapy (EST) particularly with foamed sclerotherapy in the treatment of arteriovenous malformations (AVMs). METHODS: All patients with AVM who underwent interventional therapy i.e. EST from January 1st, 2015 - December 31st, 2019 were identified through a prospective database. Types of AVM were classified according to Schobinger's classification. The outcome measures assessed efficacy and complications. The former was divided into four groups: no response, mild response, moderate response, and complete response. Complications were defined as any tissue or functional damage, distal embolization or tissue reaction. Continuous variables were compared using analysis of variance (ANOVA) F test and discrete variables were analysed using χ2 tests. P<0.05 was considered significant. RESULTS: A total of 65 patients were included. There was no statistical difference amongst the volume of foam STS 3% or alcohol used across all types of AVM. Overall, majority of patients (86.2%) reported some degree of improvement following interventional therapy. Six (9.2%) patients experienced complications including necrosis and amputation. The proportions of complication were significantly different across the categories (P=0.009). Patients with type III AVM seemed to report more complications than others. CONCLUSIONS: Foam sclerotherapy was clinically effective and safe for patients with AVM. This study showed that foam sclerotherapy with STS 3% provided a safe and efficacious alternative sclerosant to ethanol despite it was not often reported to be used to treat AVM. However, a combination of embolic agents is likely required to treat type IV AVMs.

Early follow-up quality of life and mental health of patients with congenital vascular malformations cared for in a multi-disciplinary specialist centre.

OBJECTIVE: The study aimed to evaluate the early follow-up quality of life (QoL), pain and mental health of patients with congenital vascular malformation (CVM) from a variety of treatment options. METHODS: All patients with CVM who received care and had follow-up between February 1st 2018 and January 31st 2020 were included. The health-related QoL, pain, and mental health were assessed with RAND Health Care 36-Item Short Form Survey (SF-36), visual analogue score for pain (VAS-P) and Hospital Anxiety and Depression Scale (HADS). Paired t-test was used for all analyses. p < .05 were considered significant. RESULTS: In total, 110 patients with a mean age of 36.9 years were included in this study. In all patients following care, significant improvement was found in the bodily pain domain of SF-36 and VAS-P (both p = .01). This was largely driven by high-flow vascular malformation patients who responded better to embolo-sclerotherapy, which revealed significant improvement in the bodily pain domain of SF-36 (p = .002) and VAS-P (p = .02). Patients who received supportive treatment only reported significant improvement in mental health (p = .004) and social functioning (p = .03) domains of SF-36. Meanwhile, patients treated with embolo-sclerotherapy reported significant improvement only in VAS-P (p = .02). CONCLUSIONS: This study concluded that the effects of care on early follow-up QoL, pain and mental health of patients with CVM were heterogenous. Future research should therefore, include larger sample size and longer term follow-up to understand the various factors that affect the QoL and mental health of these patients, as well as the holistic approaches to manage them.

Efficacy and safety of foam sclerotherapy with sodium tetradecyl sulfate as preferred sclerosant of venous malformations based on experience from a single specialist center.

OBJECTIVE: We have assessed the efficacy and safety of interventional therapy for venous malformations (VMs), with foam sclerotherapy as the treatment of choice according to our experience at a single specialist center. METHODS: All the patients with VMs who had undergone interventional therapy (ie, embolo-sclerotherapy and/or open surgery) from January 1, 2015 to December 31, 2019 were identified through a prospective database. The VM types were classified according to the Puig classification. The outcome measures assessed included the efficacy and complications. The former was divided into four groups: no response, mild response, moderate response, and complete response. The complications were defined as any tissue or functional damage, distal embolization, or tissue reaction. The continuous variables were compared using the analysis of variance F test, and discrete variables were analyzed using the χ2 tests. P values < .05 were considered statistically significant. RESULTS: A total of 207 patients were included. Puig type I lesions were significantly less likely to have received foam sclerotherapy using sodium tetradecyl sulfate (STS) 3% (P ≤ .001) and more likely to have been surgically excised (P ≤ .001). At the patient's first procedure during the study period, the volumes of foam STS 3% were significantly different across all types of VM (P ≤ .001). The patients with type I VMs had received a lower volume of STS 3% compared with those with type II and III VMs. The efficacy outcome categories were significantly different across all types of VMs (P ≤ .001). Overall, only 14 patients (6.8%) had reported no improvement in efficacy, and 38 patients (18%) had not attended follow-up. Therefore, 154 patients (74.8%) had experienced some form of efficacious outcome. Ten patients (4.8%) had developed complications such as hematoma, thrombophlebitis, and ulceration. The incidence of complications differed significantly across the categories (P = .030), with more complications reported for those with type I VMs. CONCLUSIONS: We found that intervention with foam sclerotherapy using STS 3% is clinically effective and safe for patients with VMs and was most successful for those with Puig type I and II VMs.

Quality of life and mental health of patients with vascular malformations in a single specialist center in the United Kingdom.

OBJECTIVE: Patients with vascular malformations suffer from chronic debilitating symptoms that have been shown to contribute negatively to their quality of life (QoL) and mental health. Despite this, the current literature evaluating the QoL and mental health of patients with vascular malformations remains scarce. Our aim was to evaluate the QoL and mental health of patients with vascular malformations. METHODS: We prospectively analyzed the validated health-related QoL (HRQoL) questionnaires: the RAND Health Care 36-Item Short Form Survey (SF-36), Hospital Anxiety and Depression Scale (HADS), and visual analogue score for pain reported by 253 patients with vascular malformations in a specialist center of vascular anomalies in the UK over 2 years. RESULTS: Patients with vascular malformations reported significantly poorer SF-36 scores in all domains compared with the UK general population. Patients with low-flow vascular malformations and arteriovenous malformations reported little variations in SF-36, HADS, and visual analogue score for pain scores. No significant association was found between age and any of the health-related QoL scores, other than the physical functioning in SF-36. Female patients reported significantly lower physical and social functioning of SF-36 and worse HADS-Depression than their male counterparts. Patients with syndromic vascular malformations reported significantly lower SF-36 scores in role-physical, role-emotional and bodily pain than nonsyndromic vascular malformations. CONCLUSIONS: This study concluded that patients with vascular malformations reported worse QoL than the UK general population. Therefore, the assessment and management of QoL and mental health should be incorporated into the overall treatment strategies of patients with vascular malformations.

Incidence of major complications from embolo-sclerotherapy of head and neck vascular malformations in a single specialist centre.

OBJECTIVE: Current data on the nature and rate of major complications for embolo-sclerotherapy (EST) of vascular malformations are scarce. However, even fewer studies focus on vascular malformations specific to the head and neck, which confer an increased specific risk of airway compromise, neurologic and ophthalmologic injury. More understanding is required surrounding the type and incidence of complications to improve treatment planning and informed consent. Therefore, this study aimed to review major complications secondary to EST of head and neck vascular malformations over a 5-year period in a single specialized multidisciplinary centre for vascular anomalies. METHODS: All interventions were decided by the multidisciplinary team. Demographic, procedural and complication data between 1st January 2013 and 31st December 2017 were prospectively documented in a dedicated database and analysed. EST of high-flow vascular malformations (HFVMs) was performed by selective catheter angiography or direct injection, and by direct injection only for low-flow vascular malformations (LFVMs). Major complications were defined as any tissue or functional damage caused by direct injection, distal embolization or tissue reaction and were decided by the multidisciplinary team. RESULTS: Forty-eight patients (median age of 35 years; range of 14-70 years; 18 men and 30 women) had 100 EST procedures for head and neck vascular malformation. Of these, 14 patients had EST for HFVM and 34 patients for LFVM, total 43 and 57 procedures, respectively. Overall, five patients with HFVM developed major complications from EST when compared with two patients with LFVM (p = 0.0167). Two patients required pre-emptive tracheostomy due to risk of post-operative airway compromise. Overall, seven (14.6%) patients experienced major complication from EST. In the HFVM group, major complications from EST occurred in five patients; four cases of tissue ulceration and necrosis (two needed debridement, one healed with resultant fibrosis that impeded speech and one resolved spontaneously) and one post-procedural airway compromise requiring tracheostomy. Meanwhile, in the LFVM group, major complications occurred in two patients; one case of severe necrosis involving the alar cartilage, lip and cheek requiring debridement and reconstruction under plastics and one simple cellulitis. No patients sustained stroke or vision impairment. CONCLUSIONS: EST is relatively safe for head and neck vascular malformations in a high-volume experienced centre. Our major complication rate of 14.6% per patient (35.7% for HFVM; 5.9% for LFVM) or 7% per procedure (11.6% for HFVM; 3.5% LFVM) compares favourably with published data from other centres. These data will improve treatment planning and informed consent for EST for both HFVM and LFVM of the head and neck.

Single Center Experience of Sirolimus Therapy in Head and Neck Low-flow Vascular Malformations.

OBJECTIVE: Recently, studies have shown that sirolimus is clinically efficacious in the treatment of some low-flow vascular malformations (LFVM). This study aimed to assess the efficacy and safety of sirolimus in treating complex head and neck (H&N) LFVM that were challenging and/or refractory to standard treatment. METHODS: Each patient had baseline and 6-months assessments consisting of clinical history and examination, quality of life (QoL) questionnaires, laboratory investigations, MRI and medical photography. Patients were followed up 1-week and then 1-monthly for 6-months. Wilcoxon signed-rank test was used to compare pre-and 6-months treatment in all 8 domains of RAND 36-Item Short Form Health Survey (SF-36), hospital anxiety and depression scale (HADS), and visual analog score for pain (VAS-P). P < 0.05 was considered significant. RESULTS: Seven patients (median age 43 years, range 23-65 years) were recruited. Six patients completed the six-months course of therapy with 1 patient withdrawing due to intolerable side effects. All six patients reported reduction of swelling with and without other symptom improvement related to the vascular malformations while on treatment. However, at 1-month review after discontinuation of sirolimus, 5 patients reported return of initial symptoms. Overall, patients demonstrated an improvement in QoL six-months treatment but there was no statistical significance (P > 0.05) in all 8 domains of SF-36, HADS and VAS-P. Five patients demonstrated a minimum 10% decrease in lesion size six-months treatment (median 21%, range 13-40%). A Wilcoxon signed-rank test showed that sirolimus treatment did elicit a statistically significant change in lesion size in either direction (Z = -1.992, P = 0.046). The most common side effects found were dyslipidaemia (n-4) and mouth ulcers (n = 2). CONCLUSION: In our preliminary experience, sirolimus is effective and safe in treating patients with complex H&N LFVM. This provides an alternative treatment where standard treatment is challenging and/or refractory.

Patient radiation exposure from embolo-sclerotherapy of peripheral vascular malformations.

OBJECTIVE: Embolo-sclerotherapy (EST) is the mainstay therapy for peripheral vascular malformations that involves the exposure of patients to ionizing radiation. We analyzed the radiation exposure to patients from EST of peripheral vascular malformations during a 5-year period in a single specialist center. METHODS: All patients who had undergone EST at a single specialist center for peripheral vascular malformations from January 1, 2013 to January 8, 2018 were identified from a prospectively collected database. Data collection included basic demographics, procedure date, anatomic site, type of vascular malformations, and procedural details. Radiation exposure, measured as the dose-area product (DAP) and fluoroscopy time, of all patients who had undergone EST during the study period were retrospectively reviewed. Statistical analysis was performed using the Mann-Whitney U and Kruskal-Wallis tests for comparison between subgroups. P < .05 was considered statistically significant. RESULTS: A total of 237 patients (median age, 30 years; range, 1-73 years) had undergone 419 EST sessions during the study period. Of the 237 patients, 61 (25.7%) had had arteriovenous malformations (AVMs) and had undergone 140 EST sessions (33.4%) and 176 (74.3%) had had venous and lymphatic malformations and had undergone 279 EST sessions (66.6%). Patients with AVMs had undergone a median of 2 procedures (range, 1-13) compared with a median of 1 (range, 1-6) for venous and lymphatic malformations within the study period. The median DAP for the single and cumulative EST for peripheral vascular malformations was 1.26 Gycm2 (range, 0.00-698.36 Gycm2) and 1.91 Gycm2 (range, 0.00-1300.24 Gycm2), respectively. The median fluoroscopy time for single and cumulative EST was 19 seconds (range, 1-3846 seconds) and 30 seconds (range, 1-5843 seconds), respectively. Significantly greater patient radiation exposure, in DAP and fluoroscopy time, was measured for single and cumulative EST for AVMs compared with venous and lymphatic malformations (P < .01 for both; Mann-Whitney U test). A significant difference in DAP but not fluoroscopy time was found when the anatomic areas of vascular malformations were compared. CONCLUSIONS: Patient radiation exposure for EST for peripheral vascular malformations, measured in DAP and fluoroscopy time, appeared to be generally less than that reported for endovascular arterial and deep venous interventions. However, some patients with peripheral vascular malformations received relatively high radiation doses. Further studies to investigate the risk factors and long-term side effects of radiation exposure in these patients and strategies to reduce these are required.

Incidence of major complication following embolo-sclerotherapy for upper and lower extremity vascular malformations.

OBJECTIVE: The current literature on the major complications of embolo-sclerotherapy of upper and lower extremity vascular malformations is scarce. Evaluating and understanding the rates and types of potential major complications of embolo-sclerotherapy of vascular malformations help treatment planning and informed consent. Therefore, this study reviewed major complications following embolo-sclerotherapy of all upper and lower extremity vascular malformations in a single specialized multidisciplinary vascular malformation center over a 5-year period. METHODS: All patients with vascular malformations underwent multidisciplinary directed intervention. Demographic, procedural, follow-up, and complication data were collected prospectively in a dedicated database, and reviewed retrospectively. Major complications for upper and lower extremity vascular malformations from 1 January 2013 to 31 December 2017 were analyzed. All embolo-sclerotherapies of high-flow vascular malformations (HFVMs) were performed under selective catheter angiography and direct injection, but low-flow vascular malformations (LFVM) with direct injection only. Major complications were defined as any tissue or functional damage caused by direct injection, distal embolization, or tissue reaction. RESULTS: Seventy patients (median age of 25 years; 44 males and 26 females) had 150 embolo-sclerotherapy procedures for upper extremity vascular malformation. Of these, 28 patients had embolo-sclerotherapy for HFVM and 42 patients for LFVM; total 78 and 72 procedures, respectively. A total of 107 patients (median age of 26 years; 42 males and 65 females) had 160 embolo-sclerotherapy interventions for lower extremity vascular malformations. Of these, 18 patients had embolo-sclerotherapy for HFVM and 89 patients for LFVM; total of 30 and 130 procedures, respectively. The overall major complication rates following embolo-sclerotherapy of upper and lower extremity vascular malformations were 14.3% and 4.7%, respectively (P = 0.030). In the upper extremity HFVM group, major complications from embolo-sclerotherapy occurred in five patients; three ischemic fingers requiring amputation and two skin ulcerations. Meanwhile, in the upper extremity LFVM group, major complications occurred in five patients; one median nerve injury requiring nerve grafting and hand therapy, one hand contracture requiring tendon release, and three skin ulcerations. There was only one major complication, which was cellulitis in the lower extremity HFVM group. In the lower extremity LFVM group, major complications occurred in four patients; two skin ulcerations, one cellulitis, and one deep vein thrombosis. CONCLUSIONS: Embolo-sclerotherapy is relatively safe for upper and lower extremity vascular malformations in a high-volume experienced center where our major complication rates were 14.3% and 4.7%, respectively, which compare favorably or similar to those reported in most recent literature. These outcomes will direct treatment strategies to avoid local and systemic toxic complications in the upper and lower extremity, for both HFVM and LFVM, and to improve informed consent.

"Switching off the light bulb" - venoplasty to relieve SVC obstruction.

In superior vena cava obstruction, one of the signs on computed tomography is an arterially enhancing pseudolesion in segment IV adjacent to the falciform ligament due to collateral flow via the veins of Sappey, sometimes termed the "lightbulb sign." We describe a case where venoplasty was performed to restore flow in superior vena cava with disappearance of the pseudolesion on subsequent computed tomography, thus "switching off the lightbulb."

Successful Repair of a Vasculopathic Aneurysmal Brachial Artery in a Patient with Type 1 Neurofibromatosis.

Vasculopathy is a well-recognized abnormality associated with neurofibromatosis type 1(NF1) and may cause stenoses, aneurysms, and arteriovenous malformations. We report a challenging case of a woman with NF1, who presented with spontaneous rupture of a brachial aneurysm around her right elbow, on a background of previous debulking and soft tissue reconstructive surgery in the same arm. She underwent successful delayed reconstruction of the brachial artery using an autologous great saphenous vein graft.

Evaluation of Clinical Prognostic Factors and Further Delineation of the Effect of Mesenteric Fibrosis on Survival in Advanced Midgut Neuroendocrine Tumours.

BACKGROUND: Small intestinal neuroendocrine tumours (SI NETs) represent 30-50% of small bowel neoplasms and often present at an advanced stage. To date, there is relatively limited literature regarding prognostic factors affecting overall survival (OS) in stage IV disease. In addition, the prevalence of mesenteric fibrosis (MF) in SI NETs and its effect on OS have not been sufficiently explored in the literature. AIM: The primary aim of this study was to perform a large-scale survival analysis in an institutional cohort of 387 patients with metastatic (stage IV) SI NETs. The secondary aim was to provide epidemiological information regarding the prevalence of MF and to evaluate its effect on OS. RESULTS: The median OS was 101 months (95% CI 84, 118). Age > 65 years, mesenteric metastases with and without desmoplasia, liver metastases, carcinoid heart disease (CHD) and bone metastases were associated with a significantly shorter OS, while primary tumour resection was predictive of a longer OS. The benefit of surgical resection was limited to symptomatic patients. MF was present in approximately 50% of patients with mesenteric lymphadenopathy. Elevated urinary 5-HIAA levels correlated strongly with the presence of CHD (p < 0.001) and to a lesser extent (p = 0.02) with MF. MF and CHD did not usually co-exist, suggesting that different mechanisms are likely to be involved in the development of these fibrotic complications. CONCLUSIONS: This study has identified specific prognostic factors in a large cohort of 387 patients with advanced SI NETs and has provided useful epidemiological data regarding carcinoid-related fibrotic complications.

Use of transabdominal ultrasound-guided transjugular portal vein puncture on radiation dose in transjugular intrahepatic portosystemic shunt formation.

PURPOSE: Transjugular intrahepatic portosystemic shunt (TIPS) creation is used to treat portal hypertension complications. Often the most challenging and time-consuming step in the procedure is the portal vein (PV) puncture. TIPS procedures are associated with prolonged fluoroscopy time and high patient radiation exposures. We measured the impact of transabdominal ultrasound guidance for PV puncture on duration of fluoroscopy time and dose. METHODS: We retrospectively analyzed the radiation dose for all TIPS performed over a four-year period with transabdominal ultrasound guidance for PV puncture (n=212, with 210 performed successfully and data available for 206); fluoroscopy time, dose area product (DAP) and skin dose were recorded. RESULTS: Mean fluoroscopy time was 12 min 9 s (SD, ±14 min 38 s), mean DAP was 40.3±73.1 Gy·cm2, and mean skin dose was 404.3±464.8 mGy. CONCLUSION: Our results demonstrate that ultrasound-guided PV puncture results in low fluoroscopy times and radiation doses, which are markedly lower than the only published dose reference levels.

The role of interventional radiology in the treatment of hepatocellular carcinoma.

The incidence of hepatocellular carcinoma (HCC) in the UK is increasing. The role of interventional radiology in the treatment of HCC is well established. We discuss the minimally invasive treatment options that are available. It is important that procedures are undertaken in and postprocedure imaging is reviewed by centers with accredited hepatobiliary units. This is when the best outcomes are achieved. Advances in tumor biology and in technology will continue to expand the role of interventional radiology in the treatment of HCC in the future.

Acute gastrointestinal bleeding: CT angiography with multi-planar reformatting.

Acute gastrointestinal bleeding is a common medical emergency, which carries a significant mortality. CT Angiography is an important non-invasive diagnostic tool, which can be used to plan subsequent endovascular or surgical management. The cases presented demonstrate that a meticulous and systematic approach to image interpretation is necessary, in particular, to detect focal sites of contrast extravasation and small pseudoaneurysms.

Transjugular renal biopsy: our experience and technical considerations.

The purpose of this study was to describe the indications for and technique of transjugular renal biopsy (TJRB) and evaluate the efficacy and complications of this method. We performed a retrospective review of 59 patients who underwent TJRB using the Quick-core needle biopsy system (Cook, Letchworth, UK) over a 4-year period. The indications for obtaining renal biopsy included acute renal failure, chronic renal failure, nephrotic syndrome, and proteinuria with or without other associated disease. Indications for the transjugular approach included coagulopathy, biopsy of a solitary kidney or essentially single functioning kidney, simultaneous renal and hepatic biopsy, morbid obesity, and failed percutaneous biopsy. All but four cases were performed via the right internal jugular vein. The right, left, or both renal veins were cannulated in 41, 14, and 4 cases, respectively. Combined liver and renal biopsies were obtained in seven cases. Diagnostic biopsy specimens were obtained in 56 of 59 patients (95%). The number and size of tissue cores ranged from 1 to 9 mm and from 1 to 20 mm, respectively. The mean numbers of glomeruli per procedure on light microscopy and electron microscopy were 10.3 and 2.6, respectively. Specimens for immunohistology were acquired in 49 cases, of which 40 were adequate. Of the 56 successful TJRB procedures, 34 (61%) were associated with isolated capsular perforation (19), contained subcapsular leak (10), isolated collecting system puncture (1), and concurrent collecting system and capsular perforation (4). There was a significant increase in capsular perforation with six or more needle passes, although no significant correlation was seen between number of needle passes and complication. Six patients had minor complications defined as hematuria or loin pain. Seven patients developed major complications, of whom five received blood transfusion alone. Two required intervention: in one an arteriocalyceal fistula was embolized and the patient was temporarily dialyzed; the remaining patient required ureteric stenting. In conclusion, TJRB provides an adequate yield for diagnosis. Complication rates are relatively high, but patients are also at high risk from the conventional percutaneous approach. Patient selection and optimization are critical to avoid major complications.

Natural history of hepatic sarcoidosis and its response to treatment.

BACKGROUND: Liver involvement in sarcoidosis is variable and can occur in the absence of pulmonary disease. Data on the natural history of hepatic sarcoid and response to therapy are lacking. This study investigates hepatic dysfunction complicating lung disease and significant liver involvement presenting independent of pulmonary sarcoid. PATIENTS AND METHODS: One hundred and eighty patients were included in the study. The minimum follow-up was 2 years. RESULTS: Fifty per cent of the study population had derangement of liver function attributable to hepatic sarcoid. Twenty-three patients (13%) had liver involvement without lung disease. Sixty-three patients were administered corticosteroids; approximately one-third had a complete clinical response, one-third a partial response and one-third showed no response. Fourteen patients (8%) were cirrhotic at presentation, and two progressed to cirrhosis despite steroid therapy. Sixteen patients received a second-line immunosuppressive agent; one-half of these showed a response to treatment augmentation (four patients azathioprine, three patients methotrexate, one patient both drugs). Six patients required liver transplantation, with disease recurrence in one recipient. In four patients, sarcoid as the aetiology of end-stage liver disease was diagnosed only on examination of the explanted liver. CONCLUSION: Sarcoidosis can cause end-stage chronic liver disease, which is often unrecognized until examination of the explanted liver. Response to conventional immunosuppression is variable and unpredictable. Transplantation is feasible and safe in this population but recurrence is possible.