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16 Biliary Tract Emergencies
nancy [221]. The incidence ranges from 1/7000
to 1/20,000 deliveries [222–224]. The onset is
usually between 28 and 40weeks’ gestation, with
an average of 35weeks [223]. It may occur during any gestation and is not always diagnosed
before delivery. The risk factors are twin pregnancies [223], and 7% of triplet pregnancies are
complicated by AFLP [225]. AFLP is more frequent in primiparas (70%) and pregnancies carrying a male (72%) fetus [223].
Pathophysiology
Approximately 39% of AFLP cases are secondary
to a urinary or respiratory infection [226]. The
pathophysiology of the disease is obscure. The
fetus’ deciency of long-chain 3- hydroxyacyl-CoA
dehydrogenase (LCHAD) may be implicated in
pathogenesis [227]. A fetus with this enzyme deciency accumulates long- chain fatty acids that have
not undergone oxidation. These fatty acids enter the
mother’s serum and are hepatotoxic. Furthermore,
the placenta may produce excess fatty acids and further elevate maternal serum-free fatty acids (FFAs).
Mothers heterozygous for LCHAD deciency also
have a greater risk of developing AFLP [227]. The
long-chain LCHAD deciency in the mitochondria
determines the cell’s accumulation of long- and
medium-chain fatty acids. This defective enzyme is
determined by a gene mutation (E47Q) [228] with
an incidence of 1:150–1:200 in the population.
Other hypotheses above normal (for pregnancy)
levels of estrogens potentiating the effects of an otherwise tolerable hormonal insult to the mitochondria in the third trimester.
Clinical Presentation
Most women present with a 1- to 2-week history
of anorexia, nausea, vomiting, epigastric, right
upper quadrant pain, headache, and jaundice
[229]. In the past, jaundice was almost always
present, but patients without jaundice are diagnosed because of earlier diagnosis, prompt delivery, and milder cases. The size of the liver is
usually normal or small. Patients with AFLP
rarely have pruritus. Hypertension and proteinuria, the main signs of preeclampsia, are found in
up to half the patients [230, 231]. Patients may
demonstrate asterixis and encephalopathy in
severe forms, with or without coma. Esophagitis
and Mallory–Weiss syndrome related to severe
vomiting and bleeding secondary to these esophageal lesions have been reported. Genital bleeding is frequent. Associated coagulation disorders
exacerbate these hemorrhages. Ascites may be
present. It is partially related to portal hypertension. Polyuria and polydipsia (without DM) have
been noted in 5% with AFLP [230] and are almost
pathognomonic in this setting. AP is a rare but
potentially severe complication (see Chap. 17).
Sometimes, patients present with fetal distress
rst, and emergent CS is indicated [232].
Diagnosis
The serum aminotransferase levels are raised but
usually not as high as in acute viral hepatitis. The
bilirubin level is almost always increased. Patients
may demonstrate hypoglycemia, which is uncommon in other liver diseases unique to pregnancy. In
severe cases, the prothrombin time increases, and
the brinogen level decreases. These coagulation
disorders are caused by hepatic insufciency, disseminated intravascular coagulation, or both. A
low platelet count is usual in AFLP and is not
always associated with other signs of disseminated
intravascular coagulation. Thrombocytopenia may
be the most striking laboratory feature and normalizes spontaneously after delivery. The diagnosis of AFLP should always be considered when
thrombocytopenia occurs during late pregnancy
and should always prompt liver function tests.
Both acute renal failure, commonly acute tubular
necrosis [226], and hyperuricemia are usual. The
US of the liver may show increased echogenicity.
An abdominal CT helps in the diagnosis. The liver
density that is lower than usual may be demonstrated by Hounseld unit values, which are equal
to or lower than those in the spleen [229]. An
abdominal CT is more sensitive than US, which is
normal in half of the patients with AFLP [233].
These complementary examinations should not
delay delivery, particularly in severe cases.
Diagnosis is highly suspected on clinical grounds
with routine blood tests (serum liver tests, glycemia, creatininemia, electrolytes, uricemia, full
blood count including platelets, and prothrombin
time).
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A liver biopsy conrms AFLP, but it is not
always performed because it is invasive. Also,
noninvasive procedures can demonstrate fat in the
liver and exclude other liver diseases, such as viral
hepatitis. Nevertheless, a liver biopsy is helpful in
atypical cases, particularly if the appropriate treatment (delivery) is delayed. The overall architecture of the liver is not altered. The characteristic
picture is a microvesicular fatty inltration of the
hepatocytes, which are swollen. The droplets are
minute and surround centrally located nuclei so
that the cytoplasm has a foamy appearance. The
droplets stain with oil red O, which is specic for
fat. Electron microscopy conrms the presence of
fat droplets [234]. A stain specic for fat or electron microscopy conrms the diagnosis in patients
with ballooning cytoplasm but no evident vacuolization [229]. Therefore, whenever AFLP is suspected, a piece of the liver biopsy specimen
should be reserved before parafn embedding and
processed appropriately with special stains to
conrm the presence of fat in the hepatocytes. The
pathologic changes reverse rapidly after delivery,
and AFLP is not associated with progression to
cirrhosis [231].
stabilization. Close monitoring of renal function
and uid status is of the utmost importance.
Invasive hemodynamic monitoring may be necessary to assess the intravascular volumes and
maintain renal perfusion.
Immediate termination of pregnancy (<24h),
emergently after maternal stabilization via the
infusion of glucose and reversal of the coagulation disturbances, effectively improves the prognosis of AFLP [224, 236]. Plasma exchange
combined with continuous renal replacement
therapy eliminates toxins and replenishes nutrients, thus shortening the recovery time [237].
Meanwhile, the articial liver has been used for
AFLP patients [238].
Hypoglycemia is common, and glucose levels
should be monitored until liver function normalizes [239]. The improvement of hepatic dysfunction heralds the resolution of the disease. Liver
enzymes, ammonia, cholesterol, and coagulation
begin to normalize, followed by a decrease in
serum creatinine, as long as permanent damage
to the renal parenchyma has not occurred. Once
these measurements show improvement, serum
amylase and lipase normalize [226].
Dierential Diagnosis
Liver failure and derivative complications are
rare with HELLP syndrome, except when spontaneous liver rupture develops (see Sect. 26.1).
Patients complain of malaise (90%), epigastric or
right upper quadrant pain (90%), or nausea or
vomiting (50%), and some will have nonspecic
viral syndrome-like symptoms [15]. Acute kidney injury is more frequent 820–100%) and profound with AFLP than with HELLP syndrome
(7–36%) [235]. Hypoglycemia is almost exclusive to AFLP [235]. However, in some cases, it is
impossible to distinguish AFLP syndrome from
severe HELLP syndrome partially because some
AFLP patients have preeclampsia.
Treatment
The treatment of AFLP is predominantly supportive. Patients with encephalopathy may
require intubation and mechanical ventilation.
Profound coagulopathy may necessitate the
transfusion of blood products for correction and
Prognosis
When AFLP was described, it was considered
fatal [221], with maternal mortality up to 85%
[240]. Nowadays, maternal mortality is 12.5–18%
[226, 240–243], recently lowered to 4% [224].
Shortly before delivery, the recovery duration correlated with total serum bilirubin, PT, plasma
brinogen level, and platelet counts [224].
A neonatal mortality rate was 7–66% [242,
243], recently lowered to 23–25% [226, 240, 241].
Intrauterine fetal death is attributed to placental
hypoperfusion due to profound maternal hypotension. No evidence of growth restriction or phenotypic abnormalities suggests other causes [226].
16.2.5 Diagnosis
16.2.5.1 Laboratory Findings
Liver tests in pregnant women with biliary AP are
frequently normal. The transaminase levels are
less than 5× the normal upper limits in 89% of
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16 Biliary Tract Emergencies
patients and less than 3× the normal upper limits
in 80%. One possibility is that the increased
metabolism of maternal transaminases by the
placenta leads to relatively normal maternal levels of liver enzymes [43].
16.2.5.2 Transabdominal Ultrasound
The US is the initial imaging method for evaluating the biliary system. Diagnostic accuracy for
cholelithiasis is 97% [47, 115]. However, accuracy is lower for the detection of CBD (50%) and
the pancreas (partial visualization in 60% with
unremarkable ndings) [120].
16.2.5.3 MRCP
MRCP is the best imaging option for biliary diseases in pregnancy if the abdominal US is not
diagnostic. MRCP is rarely used due to the rarity
of the disease, emergent presentation, and limited
availability [120, 244, 245]. MRCP can differentiate between CBD stones and Mirizzi syndrome
(Fig. 16.4). Cholecystectomy for Mirizzi syndrome improves jaundice without endoscopic
sphincterotomy or exploration of the
CBD. Preoperative diagnosis of Mirizzi syndrome eliminates the need for ERCP and radiation exposure. 3D MRCP sequences further
improved MRCP, allowing the reconstruction of
overlapping slices <1mm. With a reported accuracy near 100% in determining the presence and
level of biliary obstruction, MRCP has replaced
diagnostic ERCP.
16.2.5.4 ERCP
Indications
Baillie etal., in 1990, reported the rst ERCP to
treat complicated gallstone disease [246].
Guidelines on AP management in the general
population state that urgent therapeutic ERCP
should be performed for biliary AP, cholangitis,
or dilated CBD. ERPC should be performed
within 72 h from the onset of pain. Patients
undergoing early ERCP for severe biliary AP
require endoscopic sphincterotomy [247].
However, consideration should be given to
extending the therapeutic indications for emergency papillotomy to mild and idiopathic types
of gestational biliary AP.
Timing
There is a recommendation to avoid endoscopy
in the rst trimester whenever possible [248].
Women submitted to ERCP in the rst trimester
have the lowest percentage of term pregnancies
(73%), the highest risk of PTD (20%), and low
birth weight newborns (21%) [196]. However,
the development of hepatobiliary disease in the
rst trimester of pregnancy could be associated
with PTD or low birth weight and not the procedure itself. The recommendation is “to intervene
appropriately as early as possible.”
For radiation issues and techniques, see Chap.
1. Another issue is the risk of electrocautery use
on the fetus. Amniotic uid is a possible conductor of current to the fetus. Thus, the uterus should
not be between the grounding pad and the electrical catheter. The pad should be placed higher in
the posterior thoracic wall (rather than the hip).
Bipolar electrocautery should be preferred to
minimize this risk [248, 249].
ERCP may be the best therapeutic option during pregnancy compared to surgery or percutaneous transhepatic cholangiography.
ERCP in pregnancy tends to be safe for
both the mother and the fetus. It should be
restricted to therapeutic indications with
additional intraprocedural safety measures
[250]. ERCP should continue to be the pro-
cedure of choice for bile duct decompres-
sion in pregnancy to prevent potentially
life-threatening complications to both
mother and fetus [251].
Sedation
See Chap. 2.
Complications
ERCP during pregnancy is technically exacting and should be performed by experienced
biliary endoscopists [68, 159, 250, 252, 253].
The complications in pregnancy consist
mainly of post- ERCP AP (6–16%) [131, 196,
253], PTL, and post-sphincterotomy bleeding
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[196, 249, 250, 253, 254]. There is no difference in perforation, infection, and bleeding
rates of ERCPs performed in pregnant and
nonpregnant women [255]. Pregnancy is an
independent risk factor (OR 2.8, CI 2.1–3.8)
for post-ERCP AP [255], although the pregnancy results in fewer pancreatic stents.
Potential mechanisms are (1) a tendency to
minimize radiation leads to more difficult cannulation, (2) the physicians could be less prone
to give large volumes of IV fluid during/after
ERCP or NSAIDs (nonsteroidal antiinflammatory drugs), and (3) physiology that
inherently predisposes pregnant women to
PEP [255]. In uncomplicated cases, amylase
normalization occurs within 36–48h [256].
No randomized, controlled study compared
ERCP with surgery regarding efcacy and safety.
Also, ERCP is not the rst-line treatment for
CBD stones and acute cholangitis in pregnancy
from choledochal cysts (see Sect. 16.3).
16.2.5.5 Endoscopic Ultrasound
Endoscopic US (EUS), a semi-invasive procedure, is rarely used as a denitive diagnostic tool
to identify stones in the distal CBD. EUS is
appropriate before therapeutic ERCP, where noninvasive imaging such as MRCP is unavailable,
contraindicated, or inconclusive. Also, it is a conrmatory tool after sphincterotomy stone extraction. Without radiation exposure, it is safe with
minimal sedation-related risk. If a CBD stone is
detected, an ERCP with sphincterotomy can be
performed following the EUS during the same
sedation.
When a CBD stone is suspected, EUS has a
high positive predictive value near 100%, even
for small stones ≤2mm or sludge [257, 258].
EUS is considered the best imaging study to
evaluate the CBD but requires expensive equipment, IV sedation, and technical expertise. It is
superior to MRCP, an imaging method providing a large multiplanar eld of view images of
the biliopancreatic ductal system [259]. It
reduces unnecessary interventions in patients
with low or moderate probabilities for CBD
stones [258].
16.2.6 Treatment
No consensus on the best management of biliary
AP in pregnancy exists. ERCP is safe in pregnancy, and biliary stenting can be performed
[260]. Previous studies have shown low rates of
PTL that were not signicantly different from
those of conservative therapy in patients undergoing preterm surgery or endoscopic intervention
for simple bile stones.
16.2.6.1 Laparoscopic
Cholecystectomy After ERCP
No consensus has been reached on whether LC
should be performed after successful ERCP with
stone extraction. There are several options.
Wait-and-See Approach
In one study, ve patients underwent ERCP due
to CBD stones, and two patients developed
AC.After LC, the remainder of the pregnancy
was uneventful [68]. All ve patients had healthy
babies at term vaginal delivery. In nonpregnant
patients, the prospective randomized trial demonstrated a conversion rate of 55% in patients allocated to a wait-and-see policy after ERCP and a
23% conversion rate in the elective LC group.
This result implies that LC after ERCP is mandatory (see next section).
Mandatory Cholecystectomy
The number of emergency department visits,
recurrent biliary symptoms, and hospitalizations
is signicantly higher with conservative treatment than with cholecystectomy (see Sect.
16.1.8.4) or ERCP. In practice, some strongly
advocate an LC within 6weeks of the initial biliary event. In the pregnant population, ERCP is
highly effective for major maternal complications [261].
The interval between ERCP and LC during
pregnancy is not dened. An increased risk of
conversion to OC exists 2–6weeks after ERCP
in the general population. A higher conversion
rate could be that ERCP leads to inammation
around the gallbladder, including the hepatoduodenal ligament, making an LC more demanding
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[262]. Adhesions, operation time, and bile duct
damage were not different [262]. Others claim
that time interval is of no importance. Male gender, bilirubin levels during ERCP, severe adhesions during LC, and pre-LC CRP levels were
associated with an adverse outcome for an LC
after ERCP.
LC should be performed 24–48 h after
ERCP to shorten the hospitalization, avoid
another hospitalization, and reduce the
possibility of recurrent biliary events in the
interval between ERCP and LC [154, 262,
263]. ERCP for CBD stones and acute
cholangitis in pregnancy is preferred over
the surgical approach [68, 159, 196, 246,
250, 252–254].
16.2.6.2 Hybrid Laparoendoscopic
Approach
As in the nonpregnant population, simultaneous
(rendezvous) ERCP with LC is feasible [264].
The hybrid approach offers the therapeutic
advantage of selective bile duct cannulation,
decreasing the risk of complications that could be
catastrophic for the mother and the fetus. Also,
treatment-related costs and hospital stay would
be reduced.
16.2.6.3 Common Bile Duct
Exploration
Although postoperative ERCP remains essential
for managing retained CBD stones, routine preoperative ERCP in the general population with
suspected CBD stones has declined due to low
yield and an increased risk of complications and
laparoscopic CBD exploration. Six cases of laparoscopic [134, 244, 262] and 20 cases of open [8,
10, 36, 130, 133] CBD explorations were
described. There was no maternal or fetal morbidity or mortality. Multiple studies have demonstrated the safe and effective management of
CBD stones in pregnancy with ERCP and sphinc-
terotomy with subsequent LC [36, 68, 246, 252,
262]. Therefore, indications for laparoscopic
CBD exploration in pregnant patients following
an episode of biliary AP are yet to be dened.
Some recommend IV 1 mg of glucagon for
sphincter of Oddi relaxation.
Transcystic Approach
In general, laparoscopic transcystic clearance of
duct calculi is successful in 80–90% of and is an
alternative to ERCP [262]. This approach eliminates the teratogenic effects of radiation exposure
when ERCP is used during the rst trimester and
the inability to appropriately shield the fetus from
radiation during the third trimester [254].
However, such recommendations for laparoscopic management of biliopancreatic disease to
include laparoscopic CBD exploration in pregnancy are yet to be dened.
The location of the bile duct stones, size, number, and biliary anatomy should be considered
when choosing between a transcystic approach
and choledochotomy. Indications for a laparoscopic transcystic approach include [262] the
following:
• Small (<0.8cm) stones in the CBD,
• A limited number of CBD stones (≤5),
• The absence of stones in the common
hepatic duct,
• Cystic duct joining the CBD on its lateral or posterior aspect.
Choledochotomy
Indications for choledochotomy are [262] as
follows:
• The transcystic approach fails or is
contraindicated,
• Biliary lithotripsy needed,
• CBD >7mm.
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Intraoperative Cholangiography
Since Pablo Luis Mirizzi rst described IOC in
1934 [262], the technique has developed from
static views to dynamic real-time uoroscopic
cholangiography and, recently, three- dimensional
dynamic cholangiography [262]. In nonpregnant
patients, a dynamic real-time intraoperative uoroscopic cholangiogram is achieved with the
mobile C-arm X-ray equipment, using 10–40mL
(200 mg/mL) as a contrast medium and 1 mL
glucagon IV to release any papillary spasm, or in
cases of DM, 1–2mL (20mg/mL) IV butylscopolamine. IOC with LC was described in eight
reports [8, 18, 20, 36, 130, 140, 265, 266]. IOC
was used frequently, along with cholecystectomy,
until the early 1990s. Currently, IOC is recommended only during the exploration of CBD
[140]. From these reports, maternal morbidity or
mortality was nil [265].
ERCP and MRCP minimized the need for
IOC.Despite the radiation exposure being in a
safe range (see Chap. 1), potential risks should be
discussed with the patient.
extraction balloon is done. The SpyGlass
SpyScope was exchanged over the guidewire,
and cholangioscopy directly visualized the
CBD, common hepatic duct, and left and right
intrahepatic ducts. Saline lavage through the
cholangioscope ushes debris from the CBD
into the duodenum. No uoroscopy is needed.
To date, seven pregnant patients undergoing
SpyGlass cholangioscopy-assisted ERCP have
been reported [267–269]. The use of non-uoroscopy interventions may prolong the overall
duration of the procedure due to the learning
curves and technical experiences of endoscopists and hence increase the risk, especially in
complex cases. Furthermore, in daily practice,
non-uoroscopy modalities are not often used.
Therefore, one should not hesitate to use uoroscopy if required, knowing that limited radiation exposure is safe during pregnancy [250].
Intraoperative Ultrasound
Laparoscopic US can be used instead of IOC to
exclude retained CBD stones [134].
Choledochoscopy
Choledochoscopy could be used during CBD
exploration (open or laparoscopic) or after
sphincterotomy. Wire-guided CBD cannulation
with sphincterotomy and biliary stones or
sludge removal can be performed without uoroscopy. Choledochoscopy can conrm ductal
clearance [267, 268]. If choledochoscopy is not
available, an alternative approach is to use
EUS-guided extraction balloon sweeps. The
technique with the SpyGlass Direct
Visualization System (Boston Scientic,
Natick, MA, USA) is described. A 4.4 Fr
sphincterotome is angled in the biliary orientation, and a hydrophilic 0.35″ guidewire is gently advanced into the major papilla resulting in
bile ow around the guidewire. The sphincterotome is advanced over the wire, and the aspiration of 10mL of clear yellow bile conrmed the
location within the bile duct. A biliary sphincterotomy is performed. Sweep with a 9 mm
16.2.6.4 Puerperium
Due to the high incidence of CBD stones in the
puerperium, some recommended routine IOC
even without traditional risk factors [270].
Missed CBD stones were found in 9% [270]. In
nonemergent cases, MRCP eliminates radiation
exposure.
16.2.7 Prognosis
16.2.7.1 Maternal Outcome
Conservative treatment has an incidence of recurrent biliary AP of 7–70% [199], leading to severe
consequences [131]. Hernandez etal. described 6
pregnant women who underwent cholecystectomies for biliary AP, with a mean hospital stay of
7.8 days and no postoperative complications,
recurrence of AP, or fetal loss; 4 pregnant patients
with biliary AP were clinically managed, with a
50% recurrence rate and 1 fetal loss [38].
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After a single ERCP (with the complete calculi extraction), post-ERCP AP incidence is
6–16% [131, 196, 253]. When biliary drainage
by ERCP is performed in the rst phase, without
the preoperative use of somatostatin to prevent
AP, the incidence of post-ERCP AP was 2.9%
[271].
As for the fetal outcome, the maternal outcome after surgical treatment is also excellent. In
all 6 cases of laparoscopic and 20 cases of open
CBD explorations, maternal morbidity or mortality was nil [187].
16.2.7.2 Fetal Outcome
The underlying pathology inuences the fetal
outcome after ERCP and whether diagnostic or
therapeutic ERCP is performed. The main
cause of fetal mortality and morbidity is the
underlying disease, not the procedure itself.
One woman had a PTD out of 18 who underwent ERCP with biliary sphincterotomy for
CBD stones during all trimesters [253]. At a
median follow-up of 6years, all babies were
healthy. Others claim 1 AP, 2 neonatal deaths,
and 1 abortion (3months following ERCP) in
23 pregnant patients undergoing ERCP [254].
Another 17 patients in the third trimester
treated by two-stage ERCP described a fullterm labor rate of 67%, with 2 patients with
self-limiting post-ERCP bleeding (12%) and
no post-ERCP AP.
Therapeutic ERCP has a low incidence of
maternal complications, prolongs gestational
weeks, and reduces the induced labor rate [271].
ERCP for gallstone-induced acute cholangitis in
the third trimester resulted in 66.7% deliveries at
term. The risk of fetal complications related to
ERCP is <5% [272]. The remaining patients
delivered by elective CS because of the selfdecision, not fetal distress. A trend toward a
higher rate of fetal mortality (8.0% vs. 2.6%) in
the conservative group suggests earlier LC (and
biliary tract clearance when necessary) during
pregnancy [187].
The prognosis after CBD surgery during pregnancy is excellent. Six laparoscopic and 20 open
CBD explorations resulted in zero fetal morbidity or mortality [187].
16.3 Symptomatic Choledochal
Cysts
16.3.1 Historical Perspective
Since the rst pathological description by Vater
in 1723 [273] and the rst clinical description by
Douglas in 1852 [274], more than 400 cases have
been recorded in the general population. Seyffert,
in 1888, published the rst symptomatic case in
pregnancy, when jaundice began after childbirth
[275]. In 1958, Friend published the rst rupture
of the choledochal cyst in puerperium after normal vaginal delivery [276], and the rst rupture
during pregnancy was by Saunders and Jackson
in 1969 [277].
16.3.2 Incidence
A choledochal cyst is a rare congenital abnormality of the biliary tract. The condition is
reported to be common in Japan and Korea and
relatively uncommon in Europe and America.
One-third of the cases reported have been from
Japan [278]. Incidence varies from 1:100,000–
1:150,000 live births [279] to 1:13,000 births in
Japan [280]. Usually diagnosed during childhood, choledochal cysts present for the rst time
during adulthood in 25% of patients. It is four
times more common in females [279]. Until
1944, 14 cases of choledochal cysts during pregnancy and puerperium were published [281]. Up
to 1978, there were approximately 50 cases of
ruptured choledochal cysts published, in the
general population, including pregnancy and
puerperium [282]. Up to 2007, there was 21 case
diagnosed during pregnancy and a signicant
number during labor and early puerperium [275,
276, 281, 283–312]. Most patients were nullipa-
rous [303]. The Todani I type was found in 82%
[303].
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16.3.3 Pathophysiology
Choledochal cysts are an uncommon cause of
obstructive jaundice, but there is a tendency to
present in pregnancy [281, 283]. The cyst expansion with pain and jaundice in pregnancy may be
due to hormonal effects (progesterone-induced
stasis, relaxin), compression of the bile duct
lumen and cyst by the gravid uterus, and increase
in intra-abdominal pressure during pregnancy
and labor [286, 303, 305, 309].
16.3.4 Clinical Presentation
Clinical manifestations are nonspecic and variable. In late pregnancy, the symptoms may exacerbate due to hormonal effects and CBD
compression by the enlarging uterus [304]. The
Charcot’s triad (right upper quadrant abdominal
pain, jaundice, and fever) is seldom in adults and
one-third of pregnant patients [285, 309]. Due to
anatomic dislocations during pregnancy, the cyst
is not easily detected, especially in advanced
pregnancy [306]. If silent, it can be unnoticed
during pregnancy or found during routine abdominal US screening. Most (75%) patients present
with abdominal pain in the upper right quadrant
due to enlarged or palpable mass, jaundice (50%),
and nausea or vomiting (50%) [303].
Complications include cholangitis, AP [284,
306], a cystic rupture with biliary peritonitis
[285], and malignancy within the choledochal
cyst [287]. When the biliary obstruction is present, the symptoms are similar to those of CBD
stones (see Sect. 16.2). Perforation occurs mostly
in the late third trimester or during labor [282],
with the highest intra-abdominal pressure.
Another possibility is the destruction of the cystic
wall by inammation (cholangitis) associated
with biliary obstruction.
nancy, it is usually due to viral hepatitis or
ICP.The most common etiologies are presented
in Table16.1.
16.3.6 Diagnosis
Pregnancy makes diagnosing this disease more
difcult because similar symptoms are often
encountered during normal pregnancy. Routine
hematological and liver function tests are often
of limited value. The radiographic study is limited by fetal exposure. The transabdominal US
is the initial screening examination to evaluate
(painful) abdominal mass, acute abdomen, or
hepatobiliary conditions. However, difculties
may arise due to distortion of the normal
abdominal anatomy and gravid uterus during
pregnancy. The cyst may be misdiagnosed as
an ovarian tumor or mucocele [311]. ERCP or
CT may provide more accurate information,
but ionizing radiation should probably be
avoided in pregnancy [288]. MRI is the preferred method due to the high resolution of the
biliary tree without the problems associated
with exposing the mother and the fetus to ionizing radiation [312]. MRI can even dene the
type of choledochal cyst (Figs. 16.11 and
16.12).
16.3.5 Dierential Diagnosis
Due to various possible presentations, the differential diagnosis is different. When a choledochal
cyst presents as obstructive jaundice in preg-
Fig. 16.11 MR of the abdomen at 22 weeks of pregnancy shows gallbladder calculi and choledochal cyst
(white arrow) lled with calculi. (Reproduced with permission from [302])
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16 Biliary Tract Emergencies
a b
Fig. 16.12 Coronal (a) and axial (b) view of MR cholangiopancreatography shows a large Todani I type choledochal
cyst in 22weeks of pregnancy. (Reproduced with permission from [313] under CC BY 4.0)
16.3.7 Treatment
Although choledochal cysts rarely occur in pregnancy, clinicians must be aware of the condition,
as delayed or inappropriate therapy may be catastrophic for both mother and child. Once the
diagnosis is established, patients should be
referred to specialized centers where treatment
can be carefully planned due to the likelihood of
cyst-related complications in the short and long
term. The timing of the operation depends on the
type of presentation (elective or emergent presentation), and the type of operation depends on the
type of cyst according to the Todani classication. The same surgical principles in the postpartum period as during pregnancy are
recommended—emergent presentation requires
an emergent operation [307]. Otherwise, elective
operation in puerperium or after the termination
of breastfeeding minimizes postoperative complications or medication therapy related to the
newborn.
16.3.7.1 Asymptomatic
CholedochalCyst
An asymptomatic (palpable) mass without other
symptoms should be checked regularly. Several
treatment strategies exist. First, denitive treatment is postponed after labor and puerperium to
minimize the inuences of the operation on the
mother and fetus. Second, after elective term CS,
denitive treatment of the choledochal cyst is
performed in the same act. The rst option sounds
more logical and is recommended because during
pregnancy: (a) complex surgical procedures
result in a long recovery and possible complications which affect both mother and fetus, (b)
hemostasis may be difcult due to a hyperemic
state in pregnancy, and (c) the operative view
might be obscured by the gravid uterus. There are
no recommendations about timing after CS,
denitive surgery 6 weeks after delivery [304,
309], or delayed denitive surgery when the
patient’s general physiological condition
becomes normal after elective CS [304].
Indications for the semi-urgent surgical intervention of an asymptomatic choledochal cyst in
pregnancy include (a) symptomatic cysts, (b) an
increase in size on serial US screening, or (c) suspected/conrmed cholangiocarcinoma.
In elective settings, recommended surgical
technique in pregnant and nonpregnant women is
complete excision of the extrahepatic duct, cholecystectomy, and Roux-en-Y hepaticojejunostomy. Cyst excision eliminates the risk of
malignant degeneration, which is as high as 30%
[314], or cyst inammation. For large cysts,
bilateral Roux-en-Y hepaticojejunostomy could
be performed [311]. Complete excision of the
extrahepatic bile duct from the hepatic hilum to
the pancreaticobiliary duct junction is the most
radical solution. In some cases, it is combined
16.3 Symptomatic Choledochal Cysts
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with pancreaticoduodenectomy or hepatic resection. If the structures in the porta hepatis are not
identiable, especially after repeated attacks of
cholangitis leading to adhesion formation. In
such cases, choledochocystoduodenostomy is
performed. A side-to-side anastomosis is performed between the most dependent part of the
choledochal cyst and the second part of the duodenum. The endoscopic sphincterotomy of type
III cysts is the method of choice. Treatment is
still controversial for type IVa cysts (combined
extra and intrahepatic biliary dilation). Total cyst
excision, including hepatectomy, has been recommended in general [315] and pregnant [288]
populations. Concerning type V cysts, some recommend hepatic resection for unilobar Caroli’s
disease [316].
16.3.7.2 Complicated
CholedochalCyst
Acute Cholangitis
The complication in the form of acute cholangitis
can occur: (a) as the progression of the natural
history of the disease and (b) it was also described
after CS possibly due to kinking of the
CBD.Acute cholangitis (with or without biliary
obstruction) can be treated rst by percutaneous
cystic decompression under US, CT, or MRCP
guidance. Antibiotics should be administered.
After labor and ideally puerperium, denitive
excision of the cyst and a hepaticojejunostomy
with Roux-en-Y reconstruction are recommended [120, 306]. In late pregnancy, elective
CS followed by percutaneous decompression
after 6weeks is recommended [304]. Although
no recommendations exist, some perform denitive surgery 6weeks after delivery [304, 309].
However, complications such as AP [284,
286] or biliary obstruction [120] require a more
urgent approach.
Choledochal Cyst Rupture
Preoperative denitive diagnosis is rare with
acute abdomen due to the rarity of the disease
and additional nonspecic symptoms. During
451
Fig. 16.13 Intraoperative picture of the choledochal cyst
with perforation in the anterior wall of the bile duct.
(Reproduced with permission from [317])
exploration, the bile-stained intra-abdominal
free uid with a dilated CBD should raise the
possibility of a ruptured choledochal cyst [289].
The intraoperative cholecystography [310] or
transcystic cholangiography after a cholecystectomy [285] is conrmatory. Sometimes rupture
is evident intraoperatively (Fig. 16.13). After
detecting the condition, the initial step is thorough lavage and insertion of a T-tube. This is followed by a denitive operation that comprises
the excision of the choledochal cyst with the restoration of biliary-enteric continuity. This is
done electively after MR cholangiography or
postoperative cholangiography [317]: (a) in the
second trimester [290] when fetal complications
are the least common, (b) as part of the CS in the
third trimester [287], or (c) in the postpartum
period [308, 317].
16.3.7.3 Obstetric Management
The route of delivery for a patient with a choledochal cyst is controversial. Cyst rupture due to
increased intra-abdominal pressure during labor
is possible if untreated. After external percutaneous drainage for cyst decompression, vaginal
delivery is not contraindicated [305, 308].
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