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Amir
Maqbul Khan, Rangit Hundal, Vijaya Ramaswamy, Mark Korsten, Sunil
Dhuper, North Central Bronx and Veterans Affair Hospital, New
York 10710, USA
Correspondence to: Amir Maqbul Khan, 59 beaumont circle #3
Yonkers, NY 10710, USA. dramirkhan@hotmail.com
Telephone: +1-718-5849000 Ext. 6753
Received: 2003-12-12
Accepted: 2004-02-18
Abstract
Acute esophageal necrosis (AEN) or "black esophagus"
is a clinical condition found at endoscopy. It is a rare entity the
exact etiology of which remains unknown. We describe a case of
'black esophagus', first of its kind, in the setting of liver
cirrhosis and hepatic encephalopathy.
Khan AM, Hundal R,
Ramaswamy V, Korsten M, Dhuper S. Acute esophageal necrosis and
liver pathology, a rare combination. World J Gastroenterol 2004; 10(16): 2457-2458
http://www.wjgnet.com/1007-9327/10/2457.asp
INTRODUCTION
Endoscopic discovery of a 'black esophagus' or 'acute esophageal
necrosis' (AEN) that is unrelated to ingestion of caustic or
corrosive agents is quite exceptional. It has been described only a
few times in the past. Lacey et al. in 1991 reported 25 cases[1].
A similar report by Benoit et al.[2] in 1999 cited
27 cases after an extensive review of the literature. Since then
there have been sporadic case reports. It is difficult to estimate
its true incidence as we think this disease is largely
underreported. The exact etiology of 'black esophagus' still remains
unknown. We describe a case of 'black esophagus', first of its kind,
in the setting of liver cirrhosis and hepatic encephalopathy.
CASE REPORT
A 59-year-old hispanic male with a known past medical history of
hypertension, chronic alcohol abuse, liver cirrhosis, chronic
pancreatitis and depression was admitted to the psychiatry service
for aggressive behaviours, depression and suicidal ideation. On d 2,
he was transferred to the medical floor after evaluation for a
change in mental status with confusion, disorientation and impulsive
behaviours.
A detailed history was
elicited on further interrogation. Patient was known to have
hypertension for 10 years for which he required monopril, metoprolol
and clonidine. The history also revealed alcohol abuse for 45 years,
still an active abuser, alcoholic liver cirrhosis diagnosed for 4
years and negative hepatitis B and C serologies. He had multiple
previous admissions for hepatic encephalopathy and earlier in the
same year underwent chemotherapy for a biopsy proven hepatocellular
carcinoma. He denied smoking and illicit drug abuse.
On examination the
patient was drowsy but arousable, responding to verbal commands,
oriented to 'place and person', but not 'time'. He was afebrile and
hemo-dynamically stable. General physical examination was normal.
Cardiac, pulmonary, abdominal and neurological examinations did not
show any abnormalities. Rectal examination was normal with a
negative guaiac test.
Laboratory data on
admission were: WBC 5.1 n/L with 48% granulocytes, hemoglobin (H)/
hematocrit (Hct) of 10.3/ 31, MCV of
80.3 fl, RDW of 15.4%, platelets of 174 /n.
Na
141 mE , K 4.5 mE, Cl 108 mE, CO2 26.1 mE, BUN 32 mg/dL,
Creatinine 2.1 mg, Gluc 134 mg, Total bili 0.6 (0.3 direct), albumin
2.8 gm, proteins 6.7 gm, alkaline phosp 217 U/L, SGOT 50 U/L, SGPT
39U/L, LD 225 U/L, PT 11.4, aPTT 27.6 s and INR 1.05 s, Ammonia
139.8 um.
Ethanol on admission was
56.2, serum Osm 312, normal TSH, syphilis(-), ferritin 593, TIBC
168, total iron 58 and iron saturation 35%, B12/folate 226/22.7. CT
scan of the head was negative for bleed/metastasis or any mass. CT
scan of the abdomen revealed a few scattered diverticuli and
accessory spleen.
A diagnosis of hepatic
encephalopathy, anemia and intra-vascular volume depletion was
established based on the clinical and laboratory data. Hydration
with normal saline at 125 cc/h and treatment with lactulose 445 cc
qid (titrated to bowel movement) were given. The rest of the
management included administration of resperidal, thiamine, folate
and ativan (prn). His anti- hypertensive medications were continued
and patient was placed under close observation for alcohol
withdrawal.
During the following 2 d
(d 4 and 5) the patient's mental status improved to full orientation
without any signs of confusion, agitation and/or suicidal ideation.
Ammonia level dropped to 85 um. A drop in H/Hct (9.7/29.7) was
related to intravascular volume depletion.
On d 6 of admission, the
patient's clinical status deteriorated with a recurrence of
confusion and lethargy. Patient was found to have tachycardia (heart
rate of 120/min), a blood pressure of 105/70, a positive stool
guaiac test, BUN/Creatinine of 68/3, ammonia of 105 um and a drop in
his H/Hct (7.8/22). His management at this point included continuous
intravenous hydration, blood transfusion with 3 units of packed red
blood cells and intravenous proton pump inhibitors. His anti-hypertensive
medications were held. The patient was transferred to a monitored
setting for further observation and an esophageal gastro-duodenoscopy
(EGD) was scheduled.
EGD revealed a continuous
segment (15-35 cm) of necrosis with exudates, ulcerations and
friable mucosa in the middle and distal parts of the esophagus. The
gastro-esophageal (GE) junction showed no evidence of varices,
stomach and duodenum linings were normal. A biopsy taken from the
necrotic area confirmed the findings of fibrinoid necrotic debris
with hemosiderin deposits and acute inflammatory cells on
pathological examination of the specimen. An ultrasound of the
abdomen revealed a medical renal disease without obstruction, liver
texture consistent with cirrhosis with reversal of portal venous
flow and mild ascites.
The patient made an
uneventful recovery over the next 3 d. He was transferred to the
medical floor from where he was discharged with a follow up in the
outpatient clinic. No recurrences of any GI symptoms were reported
in his 3-mo-follow-up.
DISCUSSION
'Black esophagus' has been described primarily in post-mortem
studies[3,4]. Goldenberg et al.[5] gave
us a detailed endoscopic description of 2 cases in the early 1990s.
In a one-year prospective study conducted in Rouen University
Hospital, France, 8 (0.2%) cases of 'acute esophageal necrosis' were
identified among the 3 900 patients who underwent EGD[6].
Since then there have been only a handful of case reports. The
numbers reported in the literature may highly underestimate the real
incidence of this condition as suggested by this prospective trial.
Acute esophageal bleeding is the most common presentation. The
condition is generally seen in the elderly and those having
co-morbid conditions.
A variety of mechanisms
have been proposed to account for the development of this condition,
and low systemic perfusion seems to play the dominant role. Other
mechanisms cited include direct toxic effect[7,8], as
well as indirect mucosal breakdown and acid effect[9],
however, none of these have been proved. The frequent involvement of
distal 1/3 esophagus[10], absence of gastric lesions,
presence of necrosis of mucosal/submucosal necrosis, presence of
thrombus microscopically and rapid regression of disease after
hemodynamic stability were similar to ischemic colitis[11,12]
and strongly support the ischemic basis for the insult.
Other conditions
associated with this disease entity were prolonged hypertension[13],
ischemia[5,14], hyperglycemia[1],
hypersensitivity to antibiotics[15], herpetic infection[16],
gastric volvulus[17], posterior mediastinal haematoma and
aortic dissection[3,18], anti-cardiolipin antibodies[19]
and Steven Johnson syndrome[20]. In short, a
multi-factorial etiology to absence of any disease (idiopathic) is
possible. The pathogenesis of ischemic insult in our case can be
accounted for by the low systemic perfusion with reversal of portal
venous flow and worsening of hepatic encephalopathy.
Diagnosis is established
with endoscopy with or without biopsy. The differential diagnosis
included melanosis[21], pseudo-melanosis[22],
and acanthosis nigrans[23].
The main reported
complications of AEN were esophageal stenosis and stricture
formation[1,2]. Lacy et al.[1] reported
a 15% complication rate, whereas other case series have reported
very low to none. Recurrence was less than 10%[6,24] and
mortality ranged between 0-33%[1,6,25]. The prognosis
depends on the patient's general status rather than the extent of
local esophageal necrotic lesions.
Generally there is no
standardized treatment but the overall consensus favours
conservative treatment with intra-venous proton pump inhibitors and
short-term parenteral nutrition. The main aim is to avoid extension
of insult with time for a spontaneous and aided recovery.
In short, AEN is still
uncommon with exact etiology and pathogenesis largely unclear. The
majority do not have any long-term sequel, only a few develop
strictures and stenosis. Prognosis varies, and a majority recover
with conservative treatment with death being an uncommon outcome.
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Edited
by
Zhu LH
Proofread by Xu FM
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