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Case
Report - A 50-year-old Female with Abdominal Pain
and HIV
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Lindsay
Gennari, MD
A
56 year old HIV positive (CD4 count of 418) black female
with a history of chronic abdominal pain presented to the
emergency department complaining of mental status changes.
She had not been taking her prescribed HAART (Highly Active
Anti-retroviral Therapy) regimen for the past two weeks.
A head CT scan was negative for hemorrhage or lesions, and
lumbar puncture was negative for infection. Based on these
findings she was diagnosed with HIV related dementia versus
encephalopathy. On the third day of her admission she complained
of midepigastric abdominal pain that radiated to her back.
It was chronic in nature and not associated with meals.
She denied any hematemesis, melanic stools, BRBPR, constipation,
diarrhea, reflux symptoms or dyspnea.
The
patient’s past medical history was significant for
a cholecystectomy in 2001 due to recurrent cholecystitis.
She had a history of chronic abdominal pain for many years
which required the use of maintenance narcotics. The patient
was diagnosed with HIV in 1987. She had previously been
on HAART, however the medications had been on hold for two
weeks due to her incarceration. It is also unclear if she
was compliant with her medications prior to that time. The
patient also had untreated hepatitis C. Her past medical
history also included chronic renal failure, congestive
heart failure, hypertension, and peripheral neuropathy.
In addition, she had a history of 20 pack-years of tobacco
use, social alcohol consumption, and intravenous drug use
15 years ago.
Her
family history was significant for renal disease, cancer,
and coronary artery disease. There was no history of gastrointestinal
disease.
Her
medications during the admission included:
Lactulose 30 mg po twice daily
Hydralazine 50 mg po three times a day
Nexium 40mg po daily
Metoprolol 50 mg po twice daily
Fe Sulfate 325 mg po daily
Fentanyl duragisic patch 75mg every 72 hours
Epogen 8000 units every Monday, Wednesday, and Friday
Fosamax 70 mg po every week
Lexapro 10mg po daily
Azithromycin 1200 mg po once weekly
Morpine 1-2 mg IV Q 4 hours as needed
The
patient’s vital signs on the third day of hospitalization
were as follows: temperature 98.6, pulse 94, respiratory
rate 20, blood pressure 176/98. The patient was moderately
responsive. Her sclerae were normal appearing, oral pharynx
was clear, and no lymphadenopathy was present. Lung examination
was significant for end expiratory crackles. Cardiovascular
exam revealed a regular rate and rhythm, no murmurs, rubs,
or gallops were present. Abdominal exam revealed a rigid
abdomen with right upper quadrant to midepigastric tenderness
to palpation. Murphy’s sign was not present. The abdomen
was nondistended with normoactive bowel sounds. There was
a well healed midline abdominal scar. Her skin showed no
sign of jaundice. Extremities were warm and dry without
evidence of edema. She was alert and orientated to person,
place, and time.
The
differential diagnoses included early appendicitis, bowel
obstruction, perforated peptic ulcer, bowel perforation,
pancreatitis, retained gallstone causing biliary colic,
renal or urethral colic, intrabdominal abscess, hepatitis
or hepatic abscess, duodenitis, colitis, pneumonia, angina,
MI, pericarditis, and dissecting aneurysm. Cardiac and pulmonary
disease was low on the list of differentials. Due to her
symptoms and physical examination, gastrointestinal disease
most likely. The leading diagnosis was a retained gallstone
based on her history of cholelithiasis, bowel obstruction,
previous surgeries, and pancreatitis secondary to prior
use of HAART medications and polypharmacy.
Table 1: Laboratory Data at onset of abdominal pain
| WBC
count |
8.2/mm3 |
| Hemoglobin |
10.1
gm/dl (Low) |
| Hematocrit |
29.2
% (Low) |
| MCV |
103.6 |
| Platelets |
48 |
| Prothrombin
time |
13.2
seconds (High) |
| INR |
1.6 |
| Sodium |
136
MEQ/L |
| Potassium |
4.2
MEQ/L |
| Chloride |
111
MEQ/L (High) |
| Bicarbonate |
23
MMOL/L (Low) |
| Blood
Urea Nitrogen |
37
mg/dL (High) |
| Creatinine |
1.9
mg/dL (High) |
| Glucose |
155
mg/dL (High) |
| Total
bilirubin |
1.1
mg/dL |
| Alkaline
phosphatase |
102
IU/L |
| AST |
66
IU/L (High) |
| ALT |
28
IU/L |
| Amylase |
744
IU/L (High) |
| Lipase |
875
IU/L (High) |
A
right upper quadrant ultrasound was performed and the preliminary
report was read as showing a normal common bile duct at
4 mm in diameter with no evidence of stones, no pericholecystic
fluid, and a contracted gallbladder. The exam was limited
due to gallbladder contraction. Abdominal CT done without
contrast was also normal. No gallbladder was noted on the
CT scan report.
ASSESSMENT
The
laboratory values would point to pancreatitis as the most
likely etiology for the patient’s abdominal pain.
In regard to imaging, the right upper quadrant ultrasound
finding of a contracted gallbladder in a patient who reports
a history of a cholecystectomy and with an abdominal scar
evident on exam was concerning.
The patient was made NPO and IVFs were initiated. Her HAART
therapy remained on hold and hydralazine was stopped. However,
the patient continued to have abdominal pain, nausea, vomiting
and her amylase and lipase remained elevated for 16 days
despite being NPO and on TPN.
The
gastroenterology service was consulted at that time due
to the nonresolving nature of her abdominal pain and elevated
pancreatic enzymes. Since the patient was not able to have
radiographic contrast due to her renal insufficiency, an
MRCP was performed. The results were significant for an
absent gallbladder, normal hepatic and pancreatic ducts,
and no common bile duct stone. A 1.1 cm cystic lesion seen
at the head of the pancreas in the region of the distal
common bile duct was suggestive of a choledochal cyst (type
II vs. type III). The right upper quadrant ultrasound was
re-viewed at this time. It was then believed that what was
initially believed to be a contracted gallbladder was actually
a cyst in the common bile duct. It was believed that the
acute pancreatitis and chronic abdominal pain were due to
obstruction of the common bile duct by a choledochal cyst
causing pancreatitis or possible leak of enzymes through
the weak epithelium of the cyst. An attempt at an ERCP was
done, however, it was unsuccessful due to oversedation and
tachypnea. A decision by the patient and her family was
made not to repeat the ERCP or surgery.
The
patient remained symptomatic and had been on TPN for greater
than one month when a gastrojejunostomy tube was placed.
Finally, two months later she was tolerating a full diet
and her abdominal pain clinically resolved. Interestingly,
her amylase and lipase remained elevated in the 400s.
DISCUSSION
Bile
duct cysts, or choledochal cysts, are a cystic dilation
in the bile duct. In 1959 Alonso-Lej et al. proposed a classification
of these cysts. The three original categories are type I,
which is a diffuse dilation of the extrahepatic bile duct;
type II, which is a diverticulum of the common bile duct;
and type III, which is a dilation of the intraduodenal segment
of the biliary system and is also called a choledochocele1,2
. Type IIIA represents a choledochocele that presents in
the intraluminal duodenum and contains the terminal pancreatic
and common bile duct as a common channel, type IIIB contains
separate ductal structures with an intraluminal cyst, and
type IIIC shows a cyst completely contained within the intramural
portion of the duodenum7.
A type IV A, which consists of multiple cysts of intrahepatic
and extrahepatic bile ducts; type IV B, multiple cysts of
the extrahepatic bile duct and a type V, which is a cystic
dilation of the intrahepatic biliary tract (Caroli’s
disease), were later introduced by Todani et al3.
Bile
duct cysts are predominantly diagnosed in children or young
adults. Approximately 60% of choledochal cysts present before
10 years of age, but more than 20% of patients are older
than 20 years at the time of discovery. They are about 3
or 4 times more common in women than men. Choledochal cysts
are rare in Western countries and are more common in Asia
where over 3000 cases have been reported and the incidence
is estimated at between 1/100,000 and 1/150,000 inhabitants4.
Prenatal ultrasounds have facilitated the antenatal diagnosis
of choledochal cysts.
The
pathology of choledochal cysts is unclear. They may be congenital,
acquired or associated with other anomalies. Theories include
congenital weakness of the wall, primary mucosal abnormality,
fetal virus infection, biliary autonomic dysfunction with
a lack of ganglion cells in the common bile duct, and abnormal
anatomy of the pancreaticobiliary junction resulting in
pancreatic reflux which destroys the mucosal lining and
enhances dilation. An abnormal pancreaticobiliary junction
(APBJ) is present in 70% of the cases. An APBJ is characterized
by a long common channel. Moreover, this abnormal pancreaticobiliary
junction may cause reflux of pancreatic enzymes into the
bile duct and this could also lead to their passage through
the denuded epithelium of the cystic wall into the blood
stream causing hyperamylasemia and hyperlipasemia. This
may lead to a false diagnosis of acute pancreatitis, and
as a consequence, a long history of recurrent abdominal
pain and hyperamylasemia5.
This was likely the case in our patient, who had recurrent
abdominal pain with a persistently elevated amylase and
lipase.
The
diagnosis of choledochal cyst is usually made via ultrasound,
which provides essential information about the common bile
duct, intrahepatic and extrahepatic dilation, or the present
of stones, in the least invasive manner. Computerized tomography
offers additional anatomic information, such as hepatic
and pancreatic disease, and defines the relationship of
the cyst to the other portal structures. Contrast enhanced
images are far superior in this regard to noncontrast. Diagnosis
is confirmed at the time of surgery with an intraoperative
cholangiogram to provide the clearest picture of the cysts
in relation to the biliary tree6.
However, these noninvasive techniques do not allow for a
projectional (cross sectional and cholangiographic) display
of the exact anatomy. Endoscopic Retrograde Cholangiopancreatography
(ERCP) and percutaneous transhepatic cholangiography seem
to be the best tests for the diagnosis and allow the benefit
of intervention.
Magnetic
Resonance Cholangiopancreatography has recently been used
for diagnosis. Studies have found there to be no statistically
significant difference between the accuracy in the assessment
of biliary tract disease or in distinguishing benign from
malignant lesions. MRCP has been very useful in finding
the overall causes of pancreatitis, including choledochal
cysts, an abnormal pancreaticobiliary junction, and pancreatic
divisum. MRCP has been found to be equivalent or superior
to conventional cholangiography in identifying choledochal
cysts and an abnormal pancreaticobiliary junction. MRCP
may also be useful in certain patient populations where
anesthesia and the associated risks of the procedure may
outweigh the benefits, including the development of pancreatitis7,8.
It also has a benefit for patients that can not receive
contrast. Endoscopic ultrasound is also used to identify
biliary cysts.
Abnormal
Laboratory findings may be abnormal but are nondiagnostic
for choledochal cysts. Elevations of gamma-glutamyltranspeptidase,
alkaline phosphatase, bilirubin, transaminases, amylase
and lipase are commonly found but not specific.
The classic triad of presentation includes recurrent pain,
jaundice and palpable abdominal mass. Stringer et al., in
a retrospective study reported an incidence of this triad
in only 6% of patients. That study looked at children only,
the majority of the patients presented with jaundice (69%),
either as the sole symptom (28%), or in combination with
abdominal pain (32%), or an abdominal mass (4%)9.
Older children and adults typically present with abdominal
pain.
Hyperamylasemia
and repeated attacks of pancreatitis are common features
in patients. Recurrent pancreatitis associated with a type
III choledochal cyst was first described in 1985 by Greene
et al. The authors found that their patient who had a 25
year history of recurrent pancreatitis with an unknown causative
agent was found to have a type III choledochal cysts diagnosed
by ERCP3.
Since then pancreatitis has been frequently described in
association with choledochal cysts. Other complications
include recurrent cholangitis, stone formation, stricture,
biliary cirrhosis, portal hypertension, liver cirrhosis,
liver fibrosis, liver abscess, cyst rupture and the most
feared complication malignancy.
Biliary
cysts are associated with an increased risk of cancer, especially
cholangiocarcinoma. Review of the literature found that
the incidence of cancer was 0.7 percent in patients less
than 10 years of age, 6.8 percent in patients 11 to 20 years
of age, and 14.3 percent in patients over 20 years of age.
An incidence as high as 50 percent was found in older patients10.
Unfortunately, for unknown reasons those patients that have
undergone cyst resection may continue to have an increased
risk for developing malignancies. Theories for this include
incomplete cyst removal.
Management
of choledochal cysts usually results in surgical removal
because of the high malignant potential as well as reducing
the incidence of the complications of the cysts such as
cholangitis, cirrhosis and pancreatitis. Most surgeons recommend
complete cyst removal and restoration of biliary drainage
by Roux-Y hepaticojejunostomy. ERCP is very useful in relieving
symptoms and reducing complications. Interventions include
sphincterotomy, stent placement and stone extraction11.
However ERCP does not reduce the malignant potential.
In
regards to our patient it is unclear if she had a case of
pancreatitis that would not resolve due to the choledochal
cyst or if this patient with recurrent abdominal pain and
persistently elevated amylase and lipase just had a leak
of enzymes due the cyst causing damage to the epithelium.
REFERENCES
1.
Alonso-Leg F, Rever W, Pessagno DJ. Congenital
choledochal cysts, with a report of 2 and an analysis of
94 cases. Int Abstr Surg 1959; 108:1.
2. Todani T, Watanabe Y, Narusue M, et al.
Congenital bile duct cysts: Classification, operative procedures,
and review of thirty-seven cases including cancer arising
from choledochal cysts. Am J Surg 1977; 134; 263-269.
3. Greene F et al. Choledochocele and Recurrent
Pancreatitis. Diagnosis and Surgical Management. The
American journal of Surgery 1985; 149:306-309.
4. Dussaulx-Garin L, Pagenault M, Berre-Heresbach
NL, et al. Obstructive pancreatitis due to mucus
produced by metaplastic choledochal cyst epithelium. Gastrointestinal
Endoscopy 2000; 52:1-8.
5. Gianpiero M, Cavalleram A, Ragozzino A, et al.
Acute pancreatitis in adult type IV congenital cysts of
bile ducts: Report of two cases. Journal of Clinical
Gastroenterology 1999; 28:70-73.
6. Lee CC, Levine DA, Tunik MG, Crupi RS.
A case report: Type I choledochal cyst induced pancreatitis
in a 15 month old child. Pediatric Emergency Care
2000; 16: 265-267.
7. Shimizu T, Suzuki R, Yamashiro Y, Segawa O et
al. Magnetic Resonance Cholangiopancreatography
in Assessing the Cause of Acute Pancreatitis in Children.
Pancreas 2001:196-199.
8. Kim SH, Lim JH, Yoon HK et al. Choledochal
Cyst: Comparison of MR and Conventional Cholangiography.
Clinical Radiology 2000; 55: 378-383.
9. Stringer M, Dhawan A, Davenport et al.
Choledochal cysts: Lessons from a 20 year experience. Arch
Dis Child 1995; 73: 528-531.
10. http://www.uptodate.com
11. Jordan PH, Goss JA, Rosenberg WR, Woods K.
Some Considerations for the management of choledochal cysts.
American Journal of Surgery 2004; 187: 600-607.
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