A 25-Year-Old Girl With Severe Abdominal Pain After a Hug

Background
A 25-year-old girl presents to the local emergency department (ED) with a sudden onset of
severe abdominal pain following what she describes as a "bear hug from a friend." The pain
began a couple of hours before arrival to the ED. She describes the pain as sharp, constant, most
intense in the right upper quadrant, and radiating to her right shoulder.
The patient also reports having mild, dull abdominal discomfort and a feeling of progressive
abdominal fullness for the past few months but has not sought medical attention for these
symptoms. She also complains of having a diffuse, itchy rash that seems to have appeared
around the same time as the onset of the abdominal discomfort.
The patient has no history of food allergy and has not eaten any new foods before this episode.
She denies having any fevers, nausea, or abnormal bowel movements. She has not had any
changes in her skin coloration. She reports occasional use of acetaminophen in the past 2 weeks
for the abdominal discomfort but is not otherwise taking any regular medications. She has no
chronic medical conditions or past surgical history. She reports no significant family history. She
has two dogs that she cares for in her house, but no other pets are present.

Physical Examination and Workup

Upon physical examination, the patient is in obvious discomfort. Her body temperature is 99.1°F
(37.3°C), she has a blood pressure of 110/70 mm Hg, and her pulse is 110 beats/min. Her skin is
pale and without jaundice, but she does have a diffuse urticarial rash that is most prominent on
the trunk and proximal extremities. She appears well nourished and well developed.
Her chest has symmetrical movements during respiration, and clear breath sounds are noted on
auscultation. Her heart sounds are normal, with a regular rhythm and no detectable murmurs. A
firm mass overlying the liver edge in the right upper quadrant is noted on palpation. The entire
upper abdomen is markedly tender and rigid, particularly in the right subcostal region.
Her laboratory testing is remarkable for leukocytosis, with a white blood cell (WBC) count of
18.6 × 103/µL (18.6 × 109/L) and 40% neutrophils (0.40), 22% lymphocytes (0.22), 8%
monocytes (0.08), and 21% eosinophils (0.21) (reference ranges: WBC, 4.5-11 × 10 3/µL;
neutrophils, 40%-70%; lymphocytes, 22%-44%; monocytes, 4%-11%; eosinophils, 0%-8%). An
elevated total bilirubin level of 1.98 mg/dL (33.8μmol/L) was also noted (reference range, 0.3-
1.0 mg/dL). Her aspartate aminotransferase (AST) level is 101 U/L and her alanine
aminotransferase (ALT) level is 104.7 U/L. Her hematocrit and platelet counts are normal.
An upright x-ray of the abdomen shows a nonspecific bowel gas pattern and no findings of
pneumoperitoneum. An ultrasound is performed for a suspicion of possible gallbladder disease;
it reveals a large hypoechogenic zone in the liver, with irregular margins and a small amount of
free fluid around the liver. A CT scan of the abdomen is subsequently performed (Figures 1 and
2).

Figure 1.
Figure 2.
Discussion
The CT scan of the abdomen reveals a fluid-filled cystic mass with an irregular margin in the
fourth segment of the liver. The mass communicates with the gallbladder and is associated with a
small amount of free fluid around the liver and in the peritoneal cavity. These imaging findings
in the setting of eosinophilia, an associated allergic reaction, and a history of acutely worsening
abdominal pain with sudden pressure applied to the abdomen are consistent with a
ruptured hydatid cyst.
Echinococcosis, otherwise known as hydatid or alveolar cyst disease, is an infection caused by
the larval stage of small taeniid-type tapeworms of the Echinococcus species. Echinococcus is a
zoonotic parasite primarily in the lifecycle between domestic dogs (definitive host) and domestic
ungulates (intermediate host). Echinococcosis is found in worldwide studies and has a
prevalence rate between 1% and 10% in endemic areas, such as China, the Middle East, and
South America.
Three forms of human hydatid disease are recognized: Echinococcus
granulosus and Echinococcus vogeli produce unilocular cystic lesions, whereas Echinococcus
multilocularis produces multilocular alveolar lesions that are locally invasive. E vogeli is
uncommon and is occasionally found in the South American highlands. E multilocularis is more
common than E vogeli, but it is probably not the etiologic organism in this case. It is different
from E granulosus in that it remains in a proliferative phase, is always multilocular, and survives
in wild canines as the definitive hosts and small rodents as the intermediate hosts.
The adult form of E granulosus (3-5 mm long) inhabits the intestines of definitive hosts (which
are most commonly dogs, but it can also be found in coyotes or wolves). It has three
proglottides: immature, mature, and gravid. The gravid proglottid splits into eggs that can be
found in the feces of the definitive host. Intermediate hosts, such as humans, sheep, cattle, and
goats, get infected by consuming plants that are contaminated by the feces of affected animals or
by direct contact with an affected animal. After humans ingest the eggs, they hatch into embryos
in the small intestine. The embryos penetrate the intestinal mucosa, enter the portal circulation,
and are carried to the liver. Some are destroyed in the liver while others form into hydatid cysts.
A small percentage of the eggs may pass through the liver and form cysts in other parts of the
body, including the lungs, central nervous system (CNS), spleen, and pancreas. After the
developing embryos localize in a specific organ, they transform and develop into larval
echinococcal cysts. This process is referred to as primary echinococcosis.
The cyst is composed of two layers: the endocyst, which is filled with clear fluid, and the
pericyst, which is a fibrous capsule that develops as a host response to the growth of the
echinococcal cyst. Nutritive substances that contribute to the cyst's growth pass through the
pericyst. The pericyst encompasses the endocyst, which is of larval origin. It is composed of an
outer laminated layer, or hyaline membrane, and an inner multipotential germinal layer.
Daughter cysts develop from the inner aspect of the germinal layer, as do germinating cystic
structures called brood capsules. New larvae, called protoscoleces, develop in large numbers
within the brood capsule. The cysts typically expand slowly over a period of years, at a rate of
approximately 1-3 cm per year.

In primary echinococcosis, approximately two thirds of patients experience liver hydatid cysts.
In 85% of cases, the cysts are located in the right lobe of the liver.[5] The second most
commonly involved organ is the lungs. Because of the slow rate of growth of the cysts, patients
with simple uncomplicated cysts are usually asymptomatic. The cysts are often discovered
incidentally on routine imaging studies. In patients with liver echinococcosis, the most common
symptoms are mild abdominal pain and an upper abdominal mass. Upon physical examination,
hepatomegaly may be present in addition to a palpable abdominal mass. In about 10% of the
patients, an elevated eosinophil count is noted.

More dramatic findings are present when complications of hydatid cyst disease occur. The most
frequent complication in hepatic echinococcosis is intrabiliary rupture, which occurs in
approximately 10-15% of patients. This results in biliary obstruction manifested by jaundice and
biliary colic. In some cases, cholangitis or, even more rarely, pancreatitis may ensue. Infection of
the cyst may also occur, and it is usually caused by bacteria residing in the biliary system. This
may result in fever, leukocytosis, and possible formation of a liver abscess. Patients may be
septic and should be treated aggressively with broad-spectrum antibiotics if signs of systemic
infection are present.

Rupture of a hydatid cyst into the peritoneal cavity may happen spontaneously or may be caused
by trauma, as in this case. Symptoms following the rupture are often dramatic and may include
severe abdominal pain, syncope, or fever. Some patients exhibit signs of an allergic reaction,
such as pruritus, urticaria, eosinophilia, or even anaphylaxis. Intraperitoneal rupture usually
results in secondary implantation of cysts into the peritoneal cavity. Some rare but possible
complications of liver echinococcosis include ascites, portal hypertension, Budd-Chiari
syndrome, or compression of the vena cava.

Rupture of the cyst into the vena cava is a very rare but universally fatal complication.
Pulmonary hydatid cysts, when symptomatic, can cause chest pain, chronic cough, or
hemoptysis. They may rupture into the bronchial tree and cause expectoration of a cyst fluid.
Rupture of the hydatid cyst into the pleural cavity leads to pleuritic chest pain and dyspnea.
Although rare, localization of hydatid cysts in the CNS can cause neurologic symptoms related
to mass effect, including headache and seizures. Infection of skeletal tissue can cause pathologic
fractures as a result of invasion of the medullar cavity and slow bone erosion. Cardiac
involvement may result in pericarditis or conduction abnormalities.
Various radiographic and related imaging techniques can be used in detecting and evaluating
echinococcal cysts in different parts of the body. Plain x-rays may show pulmonary cysts as
round masses with uniform density. Hydatid cysts are not typically seen on plain abdominal x-
rays unless calcification is present. Ultrasonography is the method of choice in detecting and
evaluating hepatic hydatid cysts. It can reveal well-defined cysts with thick or thin walls in
otherwise solid organs.[4] It can also elucidate the density of the fluid inside the cyst.

The most pathognomonic finding on ultrasonography is the presence of daughter cysts; however,
small cysts under 2 cm in size and peripherally located cysts can be missed by ultrasonography.
Hydatid cysts may be unable to be distinguished from simple benign cysts if no signs of daughter
cysts are present. CT scanning is no more sensitive or specific than ultrasonography, but it is
useful in localization of the cyst and defining its relation to other structures, such as large blood
vessels or biliary structures. This is very valuable when the clinician is considering surgery.

Immunologic diagnosis is highly sophisticated and is used to distinguish simple benign cysts
from hydatid cysts. It is also used for postsurgical monitoring of persistent disease. The most
valuable serologic test in the diagnosis of human hydatid disease is immunoelectrophoresis. It is
highly specific but requires high levels of antibodies for sensitivity. It is also the most valuable
test for postsurgical monitoring because of its relative rapid reversion to negative when the
organism is cleared. Enzyme-linked immunosorbent assay (ELISA) is a valuable test for primary
diagnosis, but it is not useful in postsurgical follow-up because it takes years to revert to
negative. Latex agglutination or indirect hemagglutination tests may be also be used for
diagnosis. The sensitivity of these tests is best for hepatic disease, but it is much less sensitive for
detecting lung or other organ disease.
Surgery is the traditional treatment of choice for hydatid cysts, but numerous cysts are now
treated with percutaneous aspiration, infusion of scolicidal agents, and reaspiration (PAIR).
PAIR may be a reasonable approach for treating patients with inoperable disease, and it is
typically performed while patients are on antihelminthic therapy to decrease the risk for cyst
dissemination. Ultrasonographic staging is used to determine the ideal method of treatment.

The success of the surgical approach depends on the location and size of the cyst and the
potential for injury to surrounding structures. PAIR is contraindicated for superficially located
cysts, cysts communicating with the biliary tree, and cysts with multiple internal septal divisions.
Surgery is still preferred for complicated cysts under these conditions. Care must be made to
remove cysts without contaminating the surrounding tissues, as anaphylaxis and dissemination of
infectious protoscoleces may result. In cases of intraperitoneal rupture, the peritoneal cavity
should be searched for any hydatid elements and very thoroughly lavaged with large quantities of
saline.
Albendazole and mebendazole are used for the medical treatment of echinococcosis in patients
with contraindications for surgery. Albendazole is the preferred agent because of its greater
absorption from the gastrointestinal tract, which results in higher serum levels. Multiple factors
can be used to predict the patient response to treatment. These drugs may also be used for
perioperative prophylaxis. A systemic review and meta-analysis concluded that treatment
outcomes are better when surgery or PAIR is combined with benzimidazole drugs administered
either preoperatively or postoperatively.[6] This study also found that combined treatment with
albendazole plus praziquantel resulted in higher scolicidal and anti-cyst activity and was more
likely to result in cure or improvement, compared with use of albendazole alone.
This patient was admitted to the surgery department and surgically treated the same night. The
intraoperative findings included intraperitoneal and intrabiliary rupture of a hepatic hydatid cyst.
Evacuation of the cyst fluid and its elements, as well as sterilization with hypertonic saline
solution, was performed. Cholecystectomy was performed and a T-tube was inserted. In the
postoperative period, she was treated with albendazole and discharged to home after
approximately 2 weeks. She was instructed to continue on the albendazole for 3 months. At a 1-
month postoperative follow-up visit, she was doing well and was without complications.
Albendazole and mebendazole may be used for the medical treatment of echinococcosis in
patients with contraindications for surgery. Albendazole is the preferred agent because of its
greater absorption from the gastrointestinal tract, which results in higher serum levels. Multiple
factors can be used to predict the patient response to treatment. These drugs may also be used for
perioperative prophylaxis.
PAIR is contraindicated for superficially located cysts, cysts communicating with the biliary tree,
and cysts with multiple internal septal divisions. Surgery is still preferred for complicated cysts
under these conditions.
Hydatid cysts are not typically seen on plain abdominal x-rays unless calcification is present.
Ultrasonography is the method of choice in detecting and evaluating hepatic hydatid cysts.
ELISA is a valuable test for primary diagnosis, but it is not useful in postsurgical follow-up
because it takes years to revert to negative. Immunologic diagnosis is highly sophisticated and is
used to distinguish simple benign cysts from hydatid cysts. It is also used for postsurgical
monitoring of persistent disease.
Three forms of human hydatid disease are recognized: E granulosus and E vogeli produce
unilocular cystic lesions, whereas E multilocularis produces multilocular alveolar lesions that are
locally invasive.
The most frequent complication in hepatic echinococcosis is intrabiliary rupture, which occurs in
approximately 10%-15% of patients. This results in biliary obstruction manifested by jaundice
and biliary colic.