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strategies and treatment. Curr Opin Hematol. 2008; 15:456–464. Sitter T, Spannagl M, Banas B, Schiffl H. Prevalence of heparin- induced PF4-heparin antibodies in hemodialysis patients. Nephron.. 1998; 79:245–246. Palomo I, Pereira J, Alarcon M, et al. Prevalence of heparin-induced antibodies in patients with chronic renal failure undergoing hemodialysis. J Clin Lab Anal. 2005; 19:189–195. Zhao D, Sun X, Yao L, et al. The clinical significance and risk factors of anti-platelet factor 4/heparin antibody on maintenance hemodialysis patients: A two-year prospective follow-up. PLoS ONE. 2013; 8:e62239. Warkentin TE. Heparin-induced thrombocytopenia: Pathogenesis and management. Br J Haematol. 2003; 121:535–555.

9 Lo GK, Juhl D, Warkentin TE, Sigouin CS, Eichler P, Greinacher A. Evaluation of pretest clinical score (4 T’s) for the diagnosis of heparin-induced thrombocytopenia in two clinical settings. J Thromb Haemost. 2006; 4:759– 765. 10 Kolde HJ, Dostatni R, Mauracher S. Rapid and simple IgG specific test for the exclusion of heparin induced thrombocytopenia (HIT). Clin Chem Lab Med. 2011; 49:2065– 2068. 11 Tun NM, Bo ZM, Ahluwalia M, Guevara E, Villani GM. A rare case of intracerebral hemorrhage complicating heparin-induced thrombocytopenia with thrombosis: A clinical dilemma ameliorated by novel use of plasmapheresis. Int J Hematol. 2012; 96:513–515.

A miniseries of spontaneous intramural esophageal hematoma in hemodialysis patients: A rare cause of dysphagia Vinod KUMAR, Mallikarjuna HM, Gokulnath Department of Nephrology, St. John’s Medical College and Hospital, Bangalore, India

Abstract Intramural esophageal hematoma (IEH) is an uncommon clinical condition, with a prognosis that is essentially benign. In most cases, a predisposing factor may be seen, with the most common being the history of esophagic instrumentation, food impactions, and thrombocytopenia. We report a miniseries of 3 patients on hemodialysis, who developed IEH. All 3 of them presented with sudden onset and progressively worsening dysphagia and hematemesis. Diagnosis of IEH was established by upper gastrointestinal endoscopy. All patients were managed conservatively, and symptoms of dysphagia and chest pain improved within 6–8 days. Good resolution of hematoma was noted by repeat endoscopy within 2–3 weeks. The reported case of IEH in hemodialysis patients is rare and needs to be identified early when patients present with dysphagia, as anticoagulation during hemodialysis would possibly worsen the clinical condition, the course of which is otherwise benign. Key words: Hematoma, hemodialysis, endoscopy, dysphagia

Correspondence to: Gokulnath, MD, DM, DNB, FRCP, St. John’s Medical College and Hospital, Bangalore 560034, India. E-mail: [email protected] Conflict of interest: None. Disclosure: None.

INTRODUCTION Intramural esophageal hematoma (IEH) is an uncommon clinical condition, with a prognosis that is essentially benign with very few cases reported in hemodialysis

© 2014 International Society for Hemodialysis DOI:10.1111/hdi.12143

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secondary to hepatitis C viral infection with coagulopathy. All of them had received heparin anticoagulation during dialysis. Cardiac evaluation and chest X-ray was normal. Rapid decline in hemoglobin levels were noted in all the patients. Coagulation parameters were deranged (activated partial thromboplastin time—prolonged) in all the patients and patient 3 with CLD had thrombocytopenia and prolonged prothrombin time. These parameters were corrected by giving vitamin K injection, fresh frozen plasma, cryoprecipitate, and blood transfusion as needed. After stabilization, upper gastrointestinal (GI) endoscopy was performed, which showed large IEH in all 3 patients with variable extension (Figure 1a). Patient 1 underwent computed tomography (CT) chest with contrast, which demonstrated an intramural soft tissue density with moderate luminal compression (Figure 2). All patients were managed conservatively with liquid diet, proton pump inhibitors, antiemetics, saline hemodialysis, and correction of coagulation abnormalities. Symptoms of dysphagia and chest pain improved within 6–8 days. However, in patient 1, melena persisted for 10 days. Good resolution of hematoma was noted by repeat endoscopy within 2–3 weeks (Figure 1b) in all the patients and also confirmed by repeat CT chest in patient 1.

patients so far. In most cases, a predisposing factor may be seen, with the most common being the history of esophagic instrumentation, food impactions, and thrombocytopenia. Patients commonly present with a triad of chest pain, dysphagia, and hematemesis. Spontaneous intramural esophageal hematoma (SIEH) generally has a benign course and usually resolves within 3 weeks of conservative management.

CASE REPORT We report a miniseries of 3 patients on hemodialysis, who developed IEH. Clinical and laboratory parameters of the 3 patients are mentioned in Table 1. All 3 of them presented with sudden onset progressively worsening dysphagia and hematemesis. Patient 1 had acute retrosternal chest pain mimicking angina; however, cardiac evaluation was normal and he also had melena. Patients 1 and 2 had significant vomiting probably due to uremia and were newly initiated on dialysis. Patient 1 was in intensive care unit due to severe renal failure and uremic encephalopathy where he was intubated and was on ventilator. Patient 3 had completed 6 months of dialysis but had chronic liver disease (CLD) with portal hypertension Table 1 Clinical and laboratory parameters of all three patients Characteristics

Patient 1

Patient 2

Age (y) Gender Native kidney disease

61 Male Diabetic nephropathy

52 Male Diabetic nephropathy

Other comorbidities

Hypertension, IHD

Hypertension

Clinical presentation

Acute retrosternal chest pain, dysphagia, hematemesis, melena Preceding history of vomiting Present Dialysis vintage Newly initiated Anticoagulation during HD Heparin Prolonged APTT Yes Prolonged PT No Thrombocytopenia No Diagnosis Upper GI endoscopy, CT chest with contrast Management Liquid diet, PPIs, saline HD, FFP, and cryoprecipitate Improvement in symptoms 10 d Resolution of hematoma 3 wk

Patient 3

Dysphagia, hematemesis

48 Male Renal allograft recipient—graft loss, CKD—stage 5 HCV positive status, CLD with portal hypertension Dysphagia, hematemesis

Present Newly initiated Heparin Yes No No Upper GI endoscopy

Present 6 months Heparin Yes Yes Yes Upper GI endoscopy

Liquid diet, PPIs, saline HD, Liquid diet, PPIs, saline HD, FFP, and cryoprecipitate FFP, and cryoprecipitate 7d 8–10 d 2 wk 3 wk

APTT = activated partial thromboplastin time; CKD = chronic kidney disease; CLD = chronic liver disease; CT = computed tomography; FFP = fresh frozen plasma; GI = gastrointestinal; HCV = hepatitis C virus; HD = hemodialysis; IHD = ischemic heart disease; PPI = proton pump inhibitors; PT = prothrombin time.

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Figure 1 Upper gastrointestinal endoscopy showing (a) near total occlusion of esophageal lumen and (b) complete resolution of hematoma.

DISCUSSION Esophageal wall injuries may present as a tear (MalloryWeiss tear), an esophageal rupture (Boerhaave’s syndrome) and, more rarely, as an intramural hematoma. Intramural esophageal hematomas are an uncommon form of an esophageal injury and have been described in the literature as esophageal apoplexy, intramural hemorrhage, and intramural dissection. According to Shima et al.1 and Furukawa et al.,2 esophageal injury may be divided broadly into mechanical and chemical injuries. Mechanical injury is further classified into traumatic and spontaneous types; the former is caused by accidental ingestion of foreign objects or medical procedures such as endoscopic examination and procedures, intubation of nasogastric tube, and dilatation of constrictions; the latter accompanies increased intraluminal pressure in the esophagus due to nausea, emesis, and/or blood coagulation abnormalities.

Figure 2 Contrast computed tomography chest showing near total occlusion of esophageal lumen.

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The most common clinical manifestation among patients with IEH is acute thoracic pain (87%), followed by hematemesis (70%), dysphagia (26%), and pain while swallowing (26%).3 The triad of chest pain, dysphagia, and hematemesis is present in 32% of individuals.4 Other symptoms may include epigastric pain and odynophagia. Since the presenting symptoms are nonspecific and SIEH is rare, other cardiovascular and GI conditions are usually considered before the diagnosis is established.5–7 One of our patients had classical acute retrosternal chest pain and all of them had dysphagia and hematemesis. The classical finding on a gastrografin swallow study is the “double barrel” sign, but more frequently, an intraluminal filling defect is demonstrated. A CT scan may detect an intraluminal or intramural soft tissue density. The upper GI endoscopy often reveals a friable mucosa with a bluish longitudinal hematoma, with or without evidence of mucosal breach. There is a controversy about its routine use as many intramural hematomas are contained perforations that could be worsened by the insufflation of air.4 Endoscopic ultrasound may also be helpful in establishing the diagnosis. Similar findings were seen in upper GI endoscopy in all our patients, and in one patient, it was corroborated by contrast CT chest. It is important to note that in up to 60% of the cases, there is a history of a predisposing factor, most commonly a history of esophageal instrumentation, food impaction, and bleeding dyscrasias such as thrombocytopenia.3,8,9 Well-documented cases of IEH secondary to anticoagulation therapy are sparse in worldwide literature.10,11 Only few cases of IEHs have been reported in hemodialysis patients.12,13 All our patients received heparin anticoagulation during hemodialysis and one of the patient had associated thrombocytopenia and prolonged prothrombin time due to CLD. Spontaneous IEHs (SIEHs) generally have a benign course and resolve within 3 weeks of conservative management,14 as seen in our case series where the hematoma resolved in 2–3 weeks time. However, on rare occasions, the hematoma can progress to cause complete esophageal obstruction and severe dysphagia,15 and in some cases, spontaneous drainage of hematoma with massive bleeding, leading to hemodynamic instability and death.16,17 Rare cases of esophageal perforation has been reported, which carries a mortality of 10% to 20%.15 This case series has been reported to highlight a rare cause of dysphagia in hemodialysis patients—IEH, which usually has a benign course if identified early and appropriately managed. This condition, though rare, should be a differential diagnosis for the treating physician in managing patients with dysphagia on hemodialysis, as clinical course might worsen with potential life-threatening

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complications in a small percentage of patients and it may not be prudent to continue exposure to anticoagulation in these patients with IEH.

Manuscript received August 2013; revised December 2013.

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8 Younes Z, Johnson DA. The spectrum of spontaneous and iatrogenic esophageal injury: Perforations, Mallory-Weiss tears, and hematomas. J Clin Gastroenterol. 1999; 29:306–317. 9 Ashman FC, Hill MC, Saba GP, Diaconis JN. Esophageal hematoma associated with thrombocytopenia. Gastrointest Radiol. 1978; 3:115–118. 10 Thomasset SC, Berry DP. Spontaneous intramural esophageal hematoma. J Gastrointest Surg. 2005; 9:155– 156. 11 Yamashita K, Okuda H, Fukushima H, Arimura Y, Endo T, Imai K. A case of intramural esophageal hematoma: Complication of anticoagulation with heparin. Gastrointest Endosc. 2000; 52:559–561. 12 Lien JWK, Dufresne LR, Daly DS. Intramural esophageal bleeding in a hemodialysis patient. Can Med Assoc J. 1974; 111:1230–1231. 13 Klausner JM, Epstein L, Peer G, Lelcuck S, Skornick Y, Rozin RR. Perforation of the esophagus (Boerhaave’s syndrome) during hemodialysis. Nephron. 1985; 40: 372–373. 14 Jung KW, Lee OJ. Extensive spontaneous sub mucosal dissection of the esophagus: Long-term sequential endoscopic observation and treatment. Gastrointest Endosc. 2002; 55:262–265. 15 Soulellis CA, Hilzenrat N, Levental M. Intramucosal esophageal dissection leading to esophageal perforation: Case report and review of the literature. Gastroenterol Hepatol (N Y). 2008; 4:362–365. 16 Mion F, Bernard G, Valette P, Lambert R. Spontaneous esophageal hematoma: Diagnostic contribution of echoendoscopy. Gastrointest Endosc. 1994; 40:503–505. 17 Amott DH, Wright GM. Dissecting hematoma of the oesophagus masquerading as acute myocardial infarction. Med J Aust. 2006; 184:182–183.

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A miniseries of spontaneous intramural esophageal hematoma in hemodialysis patients: a rare cause of dysphagia.

Intramural esophageal hematoma (IEH) is an uncommon clinical condition, with a prognosis that is essentially benign. In most cases, a predisposing fac...
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