Demographics, presentation, causes and management of patients with downhill varices
| Authors | Age | Sex | Variceal bleeding | Comorbidities | Endoscopic findings | Cause | Management | Follow-up |
|---|---|---|---|---|---|---|---|---|
| Gebreselassie A et al. [5] | 55 | F | Yes | ESRD on HD Hypothyroidism | Moderate sized mid-esophageal varices | SVC syndrome secondary to central venous dialysis catheters | Conservative management | Stable on follow-up with pending vascular appointment |
| Yaşar B & Kılıçoğlu G [6] | 31 | M | Yes | Behçet’s disease | Prominent esophageal varices in the upper half of the esophagus with an overlying clot | SVC syndrome secondary to Behçet’s disease | Conservative management DMARDs Steroids | Follow-up data N/A |
| Gholam S et al. [7] | 87 | F | No | Cameron ulcers Aortic stenosis Pulmonary hypertension | Large varices in the upper third of the esophagus and Cameron lesions | Severe pulmonary hypertension secondary to aortic stenosis | Conservative management | Follow-up data N/A |
| Berkowitz JC et al. [8] | 32 | F | Yes | ESRD MCTD | Esophageal varices in the upper and middle esophagus | SVC syndrome secondary to central venous dialysis catheters | Endoscopic band ligation of a proximal varix | One year follow-up with non-bleeding grade I varices |
| Loudin M et al. [9] | 22 | F | Yes | ESRD Henoch-Schönlein purpura | Large varices in the proximal esophagus with positive red wale sign | SVC syndrome secondary to central venous dialysis catheters | Balloon dilation of the stenotic SVC | One year follow-up without recurrent bleeding |
| Yasar B & Abut E [10] | 45 | M | Yes | Seminoma Pelvic radiotherapy | Varices in the upper third of the esophagus | Bilateral brachiocephalic truncus stenosis due to mediastinal fibrosis | Conservative management | Stable hemoglobin at follow-up visits |
| Gessel L & Alcorn J [11] | 39 | M | Yes | ESRD Cerebral palsy | Mid and upper esophageal varices | Stenosis of the SVC secondary to scoliosis and central venous dialysis catheters | Conservative management | Follow-up data N/A |
| Inoue Y et al. [12] | 66 | M | No | Thymoma SVC and BCV resection | Mid and upper esophageal varices | Postoperative SVC graft occlusion | Conservative management | Stable at 3, 6, 18 months follow-ups |
| Pillai U et al. [13] | 73 | M | Yes | ESRD Hypertension CAD PAD Diastolic heart failure | Extensive mid esophageal varices | SVC stenosis from HeRO graft placement | Endoscopic band ligation | No recurrent bleeding at follow-ups |
| Basar N et al. [14] | 54 | M | No | AV block Epicardiac pacemaker | Upper esophageal varices | Bilateral subclavian veins DVTs secondary to pacemaker | Patient refused treatment | Patient demise at 4 months due to hospitalization refusal |
| Lim EJ et al. [15] | 68 | M | Yes | Small and large bowel resection Short gut syndrome Total parenteral nutrition via right subclavian vein Hickman’s catheter | Upper esophageal varices | SVC syndrome secondary to bilateral brachiocephalic vein stenosis | Endoscopic variceal band ligation Hickman’s catheter removal | Follow-up data N/A |
| Mönkemüller K et al. [16] | 82 | M | No | Diabetes mellitus type II Hyperlipidemia Parkinson’s disease Retrosternal goiter | Upper esophageal downhill varices | Obstruction of the thyroid veins secondary to retrosternal goiter | Conservative management (patient preferred) | Follow-up data N/A |
| Vorlop E et al. [17] | 42 | F | Yes | Multiple myeloma Antiphospholipid syndrome | Upper esophageal downhill varices | SVC occlusion secondary to a central venous port | Angioplasty and stenting of the SVC Removal of the indwelling catheter | Normal endoscopy at 4 weeks follow-up |
| Froilán C et al. [18] | 49 | M | Yes | Hypertension Diabetes mellitus ESRD | Mid to upper esophageal varices | Stenosis of the SVC secondary to central venous dialysis catheters | Proximal sclerotherapy Angiographic metal stenting | Follow-up data N/A |
| Calderwood AH & Mishkin DS [19] | 55 | F | Yes | ESRD Ischemic cardiomyopathy Left upper-extremity deep vein thrombosis | Proximal esophagus varices | Stenosis of the SVC secondary to central venous dialysis catheters | Endoscopic variceal ligation with band placement Angiographic balloon dilation and stent placement | Symptom resolution at 3 months follow-up |
| Greenwell MW et al. [20] | 55 | F | Yes | ESRD Hypertension | Mid to upper esophageal varices | SVC stenosis/stricture secondary to previous venous catheterizations | Esophageal band ligation Angioplasty with stenting of the SVC stricture | No recurrent GI bleeding at follow-ups |
| Ibis M et al. [21] | 35 | F | Yes | History of subtotal thyroidectomy and multinodular goiter | Upper esophageal varices | Downhill varices secondary to recurrent multinodular goiter | Esophageal band ligation Inferior thyroid artery embolization Repeat subtotal thyroidectomy | Follow-up data N/A |
| Tavakkoli H et al. [22] | 42 | M | Yes | Behçet’s disease | Upper esophageal varices | SVC obstruction secondary to Behçet’s disease | Esophageal band ligation | Variceal eradication at 1 and 6 months interval on follow up |
| van der Veldt AA et al. [23] | 77 | F | Yes | COPD Multinodular goiter | Grade II–III upper esophageal varices | Right internal jugular vein compression secondary to multinodular goiter | Subtotal thyroidectomy | Stable at 20 months follow-up |
| Areia M et al. [24] | 89 | M | Yes | Diabetes mellitus Pulmonary embolism on warfarin Severe pulmonary hypertension | Grade II upper esophageal varices | Pulmonary hypertension and oral anticoagulant use | Conservative management | Stable at 3 months follow-up |
| Bédard EL et al. [25] | 68 | F | Yes | Retrosternal goiter | Upper esophageal varices | Extrinsic compression of the right innominate vein secondary to retrosternal goiter | Thyroidectomy | Follow-up data N/A |
| Serin E et al. [26] | 60 | F | No | None | Upper esophageal varices | Increase blood drainage from the tumor into the esophageal veins | Tumor removal | Resolution of varices at 15 months follow-up |
| Blam ME et al. [27] | 42 | F | No | Pulmonary sarcoidosis Uveitis ESRD | Grade II varices in the mid to distal esophagus | SVC syndrome secondary to central venous dialysis catheters | Conservative management | Follow-up data N/A |
| Chakinala RC et al. [28] Case 1 | 56 | M | Yes | ESRD Rheumatoid arthritis PAD Esophageal varices | Upper and middle esophageal varices | Chronic SVC and right brachiocephalic vein occlusion secondary to venous catheters | Esophageal band ligation Failed SVC stenting | Follow-up data N/A |
| Chakinala RC et al. [28] Case 2 | 56 | M | Yes | Gastroparesis Diabetes mellitus type II ESRD on HD Atrial flutter on warfarin | Upper and middle esophageal varices | Chronic SVC and right brachiocephalic vein occlusion secondary to venous catheters | Conservative management | Follow-up data N/A |
| Hussein FA et al. [29] | 43 | F | Yes | ESRD on HD Hypertension PAD | Upper esophageal varices | SVC stenosis/occlusion secondary to SVC catheter later replaced by graft | Variceal banding Refused SVC angioplasty | Resolution of varices at follow-ups |
| Chandra A et al. [30] | 55 | M | Yes | Diabetes mellitus type II ESRD on HD | Middle and upper esophageal varices | SVC syndrome secondary to thrombosis from HD catheter | Balloon angioplasty with stenting | Follow-up data N/A |
| Pratap et al. [31] | 26 | M | Yes | ESRD on HD | Upper third esophageal varices | Left brachiocephalic vein and SVC obstruction secondary to HD catheter | Venous angioplasty with balloon dilation | No GI bleeding at 7 months follow-up |
| Ennaifer R et al. [32] | 31 | M | Yes | Behçet’s disease | Upper esophageal varices | SVC syndrome secondary to Behçet’s disease | Conservative management DMARDs Steroids | Follow-up data N/A |
| Muthyala U et al. [33] | 31 | F | Yes | Interstitial nephritis ESRD | Proximal esophageal varices | SVC stenosis/obstruction due to multiple central venous accesses | Angioplasty | No GI bleeding at 9 months follow-up |
| Harwani YP et al. [34] | 55 | F | yes | Liver cirrhosis Chronic rheumatic heart disease, severe mitral and tricuspid regurgitation Pulmonary hypertension | Upper and lower esophageal varices | Dilated SVC due to pulmonary hypertension | Variceal banding | Follow-up data N/A |
| Rhoades DP et al. [35] | 57 | M | Yes | Hepatitis C cirrhosis Human immunodeficiency virus infection Hemophilia A | Upper esophageal varices | Idiopathic | Variceal banding | Stable hemoglobin at 1, 3 months follow-ups |
| Nguyen LP et al. [36] | 39 | F | Yes | Diabetes Hypertension ESRD on HD Recurrent AV fistula thrombosis | Large esophageal varices 25 cm to the distal esophagus just above the gastroesophageal junction | SVC thrombosis secondary to catheter | Angioplasty with stenting | Subsequent EGD showed variceal resolution |
| Nayudu SK et al. [37] | 48 | M | Yes | ESRD on HD Seizure disorder Dyslipidemia Hypertension | Upper esophageal varices | SVC occlusion secondary to dialysis catheter | Angioplasty | Stable hemoglobin at follow-ups |
| Shirakusa T et al. [38] | 26 | M | No | Hepatitis | Upper esophageal varices | Excessive blood flow into the esophageal wall from a giant lymphoma | Thoracotomy | Follow-up data N/A |
| Pop A et al. [39] | 52 | F | Yes | Hypertension, diabetes mellitus type II, PAD ESRD | Proximal to mid-esophageal varices | SVC thrombosis secondary to thrombosis from HD catheter | Failed balloon angioplasty Gore-Tex graft bypass with an end-to-side anastomosis | No recurrent GI bleeding at 5 months follow-up |
| Sorokin JJ et al. [40] | 46 | F | No | Subtotal thyroidectomy | Upper esophagus and gastroesophageal junction varices | SVC obstruction secondary to mediastinum fibrosis | Conservative management | Follow-up data N/A |
| Johnson LS et al. [41] | 85 | F | Yes | None | Upper esophageal varices | SVC obstruction due to retrosternal thyroid | Total thyroidectomy | Resolution of varices at 2 and 24 months follow-ups |
| Orikasa H et al. [42] | 59 | M | No | Behçet’s disease | Four upper esophageal varices | SVC syndrome secondary to Behçet’s disease | Conservative management | Follow-up data N/A |
| Maton PN et al. [43] | 34 | F | Yes | Idiopathic vasculitis | Upper esophageal varices | Vasculitis | Conservative management | Hemoglobin improvement over next 18 months |
| Basaranoglu M et al. [44] | 34 | F | Yes | Asthma | Grade II upper esophageal varices | SVC obstruction secondary to fibrosing mediastinitis | Conservative management | Persistent grade II varices on follow-up imaging |
M: male; F: female; ESRD: end-stage renal disease; HD: hemodialysis; N/A: not available; DMARDs: disease-modifying antirheumatic drugs; DVT: deep venous thrombosis; MCTD: mixed connective tissue disease; BCV: brachiocephalic vein; AV: atrioventricular; GI: gastrointestinal; COPD: chronic obstructive pulmonary disease; PAD: peripheral arterial disease; EGD: esogastroduodenoscopy; CAD: coronary artery disease; HeRO: HD reliable outflow
