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Pseudomelanosis of the small bowel: A systematic review [Meeting Abstract]

Deliwala, S; Hussain, M; Ponnapalli, A; Wiggins, B T; Beere, T; Kunaprayoon, L; Lakshman, H; Obeid, M; Bala, A; Gurvits, G
INTRODUCTION: Pseudomelanosis duodeni is identified by a tigroid mucosa with brown-black or grey-black speckling. It is not associated with laxatives, unlike its colonic variant. Over the years, isolated reports of pseudomelanosis across the duodenum, jejunum, and ileum have been reported that were not captured in previous reviews. We aim to summarize all affected cases and provide clinical characteristics of this visually striking disease.
METHOD(S): We conducted a systematic review to identify all published reports of pseudomelanosis in the small bowel. A search string ("pseudomelanosis duodeni") OR ("duodenal melanosis") OR ("melanosis duodeni") OR ("melanosis ilei") OR ("pseudomelanosis ilei") OR ("melanosis intestini") OR ("pseudomelanosis intestini") OR ("melanosis jejuni") OR ("pseudomelanosis jejuni") was applied across databases (Figure 1). All cases were analyzed for characteristics with outcomes.
RESULT(S): 134 patients were identified. 82% of cases were identified by an EGD, while 5% used capsule endoscopy. Commonly affected segments were duodenum (70%), ileum (11%), stomach (7%), jejunum (5%), while 7% appeared endoscopically unaffected. Histology revealed a lack of fibrosis or cellular injury, reacting to prussian blue (43%), Fontana-Masson (27%), and PAS (16%) stains. Iron accumulations were frequently noted in samples (34%). The mean age was 61.23 +/- 17 years, with 56% males. The most common coexisting comorbidities were HTN (63%), CKD (55%) with 13% on hemodialysis, iron deficiency anemia (23%), diabetes mellitus (22%), and heart failure (6%). Hiatal hernias were seen in less than 10% of cases. Anti-hypertensives (44%), iron supplements (41%), diuretics (31%), beta-blockers (26%), and multivitamins (12%) were most commonly implicated. Anti-reflux therapy was used in less than 5% of patients, while mortality was seen in 5% of all cases, mainly to renal failure (42%) and sepsis (14%).
CONCLUSION(S): Pseudomelanosis of the upper GI tract maintains a distinct pathophysiology. The majority are diagnosed on endoscopy, and tissue diagnosis can be reassuring to the patient. There seems to be a firm association with chronic illness, that may contribute to impaired iron utilization. It is possible that defects in absorption can affect nutrients as well, contributing to GI symptoms of dyspepsia and abdominal discomfort, although the majority remain asymptomatic. Most cases resolve spontaneously, although the persistence of deposits is an emerging feature of this disease
EMBASE:633658972
ISSN: 1572-0241
CID: 4720442

Pseudomelanosis duodeni in consecutive endoscopies: An indirect marker of long-standing kidney disease? [Meeting Abstract]

Deliwala, S; Hussain, M; Ponnapalli, A; Wiggins, B T; Beere, T; Kunaprayoon, L; Lakshman, H; Obeid, M; Bala, A; Gurvits, G
INTRODUCTION: Much has been written about melanosis coli; in contrast, occurrences of pigmentation in the upper gastrointestinal (GI) tract remains relatively rare. Pseudomelanosis duodeni (PD) appears as a striking black-brown speckle across the mucosa, presenting a diagnostic challenge to endoscopists. The paucity of reported cases and minimal understanding makes management arbitrary. Follow-up data on the persistence of these accumulations and its long-term outcomes remain to be explored. CASE DESCRIPTION/METHODS: An 86-year-old female presented with fatigue, dizziness, and melena. She had a history of end-stage renal disease requiring hemodialysis, valvular heart disease, history of recurrent GI bleeding, anemia, and diabetes mellitus. Home medications included amlodipine, carvedilol, hydralazine, insulin (detemir and aspart), and oral iron supplements. On arrival, her vitals read
EMBASE:633658029
ISSN: 1572-0241
CID: 4720512

Black Esophagus Trends Across American College of Gastroenterology (ACG) Scientific Meetings over a Decade: 2010-2020 [Meeting Abstract]

Deliwala, S; Wiggins, B T; Ponnapalli, A; Hussain, M; Beere, T; Kunaprayoon, L; Lakshman, H; Obeid, M; Bala, A; Gurvits, G
INTRODUCTION: Acute Esophageal Necrosis (AEN) is a rare syndrome characterized by a diffuse necrotic black appearing mucosa preferentially affecting the distal esophagus with various proximal extensions and an abrupt demarcation at the GE junction. Its etiology is multifactorial, a combination of ischemic injury, massive reflux of gastric contents, and impaired esophageal mucosal defense systems. AEN incidence is 0.01-0.28% in selected studies, and population-based information regarding AEN is sparse. Presentation in literature is typically based on published reports, but scientific abstracts are often overlooked. We aim to present a systematic review of a large number of previously presented abstracts from 2010 to 2020 ACG annual meetings.
METHOD(S): We identified all ACG published abstracts over the past ten years using "acute esophageal necrosis" OR "black esophagus" OR "acute necrotizing esophagitis" across databases (Figure 1).
RESULT(S): Amongst 89 abstracts, 97 patients were identified. Mean age was 63.68 +/- 14 years, while 65% were males. Most common presenting features were hematemesis (34%), melena (22%), coffee-grounded emesis (16%), abdominal pain (11%), dysphagia (9%) and odynophagia (6%). Risk factors included diabetes mellitus (39%), CKD (33%), HTN (32%), cirrhosis (25%), alcohol use (23%), CAD (18%) and cancer (18%). Patients received an EGD within 3.41 +/- 10.34 days from symptom onset. Over 95% of cases were confirmed visually on endoscopy and remaining on autopsy. Affected segments were middle-distal (34%), pan-esophageal (28%), distal (20%), middle (5%), while proximal was seen in less than 1%. Histology findings uniformly confirmed necrosis, although biopsies were not performed in 54%. Majority received medical management with PPIs (80%), antibiotics (43%), and sucralfate (12%). Patients treated medically remained NPO for 5 +/- 2.52 days, with 9% receiving TPN. Follow-up endoscopies were seen in 32%, with a mean of 31.84 +/- 64.95 days. Majority demonstrated significant improvement, with complete resolution in 10%, as early as 3 days. Complications included perforation (18%), strictures (5%) and infections (4%), and a mortality rate of 23%.
CONCLUSION(S): AEN syndrome is increasingly being reported as awareness increases, with the majority of all ACG abstracts being published over the past decade. Patients with chronic diseases are more commonly afflicted. Endoscopy is diagnostic. Increased awareness of the disease may lead to a rise in prevalence
EMBASE:633657143
ISSN: 1572-0241
CID: 4720572

Black esophagus (acute esophageal necrosis) and diabetes mellitus: Is it time to reconsider their association? [Meeting Abstract]

Deliwala, S; Beere, T; Kunaprayoon, L; Lakshman, H; Obeid, M; Bala, A; Wiggins, B T; Ponnapalli, A; Hussain, M; Gurvits, G
INTRODUCTION: As awareness for Acute Esophageal Necrosis syndrome increases, so does our ability to understand its associations and causations. It classically presents as a striking necrotic appearance of black mucosa preferentially affecting the distal esophagus and extending proximally to various degrees while stopping at the GEJ. Its etiology is multifactorial and related to tissue hypoperfusion, massive reflux of gastric contents, and compromised local mucosal barriers. Historically, diabetes mellitus was seen in nearly 36% of AEN cases, although its association with hyperglycemia has never been parsed out. We aim to present a case of diabetic ketoacidosis (DKA) and review the literature with a focus on hyperglycemia and acute esophageal necrosis (AEN). CASE DESCRIPTION/METHODS: A 34-year-old female had called emergency services after experiencing coffee-ground emesis, abdominal pain, and pre-syncopal like symptoms for 5 days. She was found hypotensive, tachycardic with a blood sugar over 400 mg/dL. She was an active smoker with a history of diabetes mellitus type 1 with her last hemoglobin A1c at 16.9%. On arrival to the ED, her labs revealed blood glucose greater than 1000 mg/dL, pH of 7.1, bicarbonate ,10 mEq/L, anion gap over 30 mEq/L with acute kidney injury and cystitis. She was initiated on an insulin drip and antibiotics, made NPO, and IV esomeprazole was started. Esophagogastroduodenoscopy (EGD) revealed circumferential black appearing mucosa and a necrotic complexion in the middle and distal segments (Figures 1-3). She was managed conservatively with antiacid therapy. Enteral feeds were restarted shortly, and she was discharged home. DISCUSSION: Despite growing literature revealing DKA as a common precipitant for black esophagus, a bonafide association to hyperglycemia has not been shown. A literature review across all reported cases revealed diabetes mellitus as its most common risk factor (39%). Blood glucose over 150 mg/dL was seen in 15% of all black esophagus cases, while 67% of all hyperglycemic patients had blood glucose levels over 350 mg/dL. Average hemoglobin A1c was 11.46%, and approximately 13% that met DKA criteria developed black esophagus. Cases of DKA reported unusually high glucose levels, with the highest recorded at 1294 mg/dL. The mortality amongst patients with black esophagus and hyperglycemia nears 19%, while patients that develop concomitant black esophagus and DKA, mortality is 15%. (Figure Presented)
EMBASE:633658754
ISSN: 1572-0241
CID: 4720472

Acute Esophageal Necrosis (Gurvits Syndrome) Presenting as Globus and Altered Phonation

Deliwala, Smit S; Bala, Areeg; Haykal, Tarek; Elbedawi, Mamoon M; Bachuwa, Ghassan; Gurvits, Grigoriy E
BACKGROUND Acute esophageal necrosis (AEN), also known as black esophagus or Gurvits syndrome, is an infrequently seen clinical condition distinguishable by a visually striking endoscopic appearance of necrotic esophageal mucosa that involves the distal esophagus with proximal extensions ending at the gastroesophageal junction. Since its early recognition pathologically in the 1960s and endoscopically in the 1990s, AEN, despite its rarity, is being increasingly recognized as a demonstratable cause of upper gastrointestinal bleeding. Cases of pan-esophageal necrosis are sparsely reported, leaving management guidance to isolated case reports. CASE REPORT An 80-year-old female smoker with advanced chronic obstructive pulmonary disease presented with signs and symptoms of acute pharyngitis and globus sensation that had been evolving over the preceding weeks. An esophagogastroduodenoscopy revealed circumferential necrotic mucosa encircling the entire lumen of the esophagus. The patient was made nil-per-os and started on high-dose anti-reflux therapy with adequate hemodynamic resuscitation. CONCLUSIONS AEN is multifactorial but primarily a combination of decreased tissue perfusion and a massive influx of gastric contents in settings of impaired local defense barriers. Despite its dramatic presentation, the majority of cases resolve with conservative medical management, foregoing surgical interventions.
PMID: 32881843
ISSN: 1941-5923
CID: 4583462

Nuances in diagnosis and management of acute esophageal necrosis

Gurvits, Grigoriy E
PMCID:7315703
PMID: 32624671
ISSN: 1108-7471
CID: 4518112

Black Esophagus in the Setting of Alcohol Abuse after External Beam Radiation [Case Report]

Deliwala, Smit S; Lakshman, Harini; Congdon, Douglas D; Elbedawi, Mamoon M; Bachuwa, Ghassan; Gurvits, Grigoriy E
Acute esophageal necrosis (AEN), black esophagus, or Gurvits syndrome is a rare clinical disorder characterized by a striking endoscopic appearance of necrotic esophageal mucosa, universally affecting the distal esophagus and ending abruptly at the gastroesophageal junction. It has been gaining traction as a demonstrable cause of upper gastrointestinal bleeding in the 21st century. Its pathophysiology is multifactorial affecting men and the elderly disproportionally, with a mortality rate nearing 36%. AEN has been associated with numerous conditions in the past, and we aim to present an unusual case of AEN on the setting of chronic alcohol use.
PMCID:7506255
PMID: 32999646
ISSN: 1662-0631
CID: 4615852

Important points on black oesophagus

Gurvits, Grigoriy E
PMID: 32299287
ISSN: 1758-1133
CID: 4383762

When gastroenterology meets radiology: air under right diaphragm

Gurvits, Grigoriy E
PMCID:7043082
PMID: 32133121
ISSN: 2041-4137
CID: 4339802

Metastatic Cutaneous Squamous Cell Carcinoma of the Colon Presenting as Transfusion-Dependent Hematochezia [Case Report]

Dornblaser, David; Hajdu, Cristina; Rosenberg, Jonathan; Gurvits, Grigoriy
Squamous cell carcinoma (SCC) of the colon is an exceedingly rare clinical diagnosis with few cases reported in the literature. We report a case of a 61-year-old man with a medical history of cutaneous SCC of the penis who presented with hematochezia and was found to have metastatic SCC to the distal transverse colon. To our knowledge, this is the first case of colonic SCC presenting as a metastatic disease from a primary penile site.
PMCID:7145165
PMID: 32309497
ISSN: 2326-3253
CID: 4402062