Atypical Presentation of Choledochal Cyst Type IV-A in a Hispanic Girl: A case report-Juniper Publishers
Juniper Publishers-Journal of Pediatrics
Abstract
A type IV-A choledochal cyst is the second most
common type of this rare congenital entity that is characterized by
multiple dilatations of the intrahepatic and extrahepatic biliary tract.
The most common clinical findings of this entity is abdominal pain,
palpable right upper quadrant mass and jaundice. However, there are
other clinical presentations that differ from the classical presentation
that can delay the adequate management of the choledochal cyst. The
optimal treatment is total surgical excision along with a
hepaticoduodenostomy or hepaticojejunostomy. We present a case of a 22
month old female with an unusual presentation of a choledochal cyst.
Introduction
Choledochal cysts are rare congenital entities
characterized by single or multiple dilatations of the intra and/or
extrahepatic biliary tree [1]. Its incidence in Asian population is
1:1000, but in western populations is 1:100000 to 1:150000 [2]. There is
an unexplained female:male preponderance of 1.5:1 in the pediatric
population and up to 4.9:1 in the adult population [1,3]. There are
numerous theories of choledochal cyst etiology. The most accepted theory
explaining the pathogenesis of choledochal cyst is the Babbitts theory.
This theory is based in the anomalous pancreaticobiliary ductal
junction system. It attributes choledochal cyst formation to the
presence of an abnormal pancreaticobiliary junction outside the ampulla
of Vater, resulting in a long common channel that allows the reflux of
pancreatic juice in the bile duct. This reflux in turn activates
pancreatic enzymes, causing inflammation and weakness of the bile duct
wall and leading to cyst formation [4].
The anatomy of choledochal cyst is categorized in five types named by Todani. Choledochal cyst
- Type I is a saccular or fusiform dilatation of a portion or entire common bile duct with normal intrahepatic duct;
- Type II consists of an isolated diverticulum protruding from the common bile duct;
- Type III is a dilatation of intraduodenal segment of the common bile duct (choledocele);
- Type IV consists of multiple dilatations of the
intrahepatic and extrahepatic biliary tract (IV-A) or multiple
dilatations involving the extrahepatic biliary tree
(IV-B) . - Type V consists of cystic dilatation of the intrahepatic biliary ducts (Caroli’s disease).
Choledochal cyst Type I is the most common type presenting in 50-80% of cases. Choledochal cyst Type IV-A
is the second most common one with an incidence of 5 to 83%in different
series [1,5,6] (Figure 1). The clinical presentation of choledochal
cyst varies among patients. The most common frequent presentation is
abdominal pain (93.8%) followed by jaundice (58.3%). These cysts are
typically a surgical problem of infancy or childhood, however, the
diagnosis is delayed until adulthood in nearly 20% of patients [7].
Choledochal cyst carry a long – term burden of morbidity and mortality,
therefore the optimal treatment is total surgical excision and possibly
surgical diversion [8]. We present a case report of a 22 month old
Hispanic girl with an atypical presentation and a rare case of
choledochal cyst type IV-A.
Case Report
Case of a 22 month old female born premature at 32 weeks
complicated with low birth weight.
She was doing well until 2 months ago that she started presenting
generalized pruritus. She was treated with conservative
management for skin lesions without significant improvement.
Patient was brought to the emergency room due to worsening
generalized pruritus and multiple skin lesions.
Upon physical examination she had multiple skin lesions and
a distended, nontender abdomen with a palpated mass in the
right upper quadrant. Mother referred that abdominal distention
became evident close to the time she started presenting pruritus.
Mother denied abdominal pain, nausea, vomiting, diarrhea, or
jaundice. Laboratory evaluation upon admission was significant
for elevated white blood cell count of 14.8x 103 cells/μL, elevated
erythrocyte sedimentation rate of 71mm/h, and elevated bilirubin
levels at 2.26 mg/dL.
An abdominal ultrasound was performed and revealed a large
cystic mass measuring 9.1 X 6.7 X 6 cm, suggestive of a choledochal
cyst. A magnetic resonance cholangiopancreatography was
ordered to confirm diagnosis and study revealed a marked dilatation
of the intrahepatic and common hepatic ducts with severe
fusiform dilatation of the common bile duct. These findings were
consistent with a Type IV-A choledochal cyst.
Patient was started on ursodiol treatment as well as intravenous
antibiotics (ceftriaxone and clindamycin) for concomitant
skin infections. Also, she was started on topical treatment with
hydrocortisone 1% cream for suspected dermatitis. Patient was
taken to the operating room for excision of choledochal cyst and
anastomosis. A hepaticoduodenostomy and division of an intrahepatic
membrane were performed successfully. Histological sections
of the cyst surgical margins did not reveal malignancy (Figure
2). The post operative course was uneventful and the patient
was discharged in the postoperative day # 11, tolerating diet and
stooling adequately for age, as well as significant improvement in
her symptoms. During follow up examination she was doing well.
Bilirubin levels had decreased to normal values and pruritus had
improved.
Discussion
A choledochal cyst is a rare congenital dilatation of the biliary
tract characterized by single or multiple dilatations of intra and/
or extra hepatic biliary tree. The incidence of choledochal cyst was
reported to be less than 1 in 13,000 to 2 million population [9]. As
previously mentioned, it has a female preponderance of 1.5:1. The
choledochal cyst Type IV is the second most common choledochal
cyst type. Clinical choledochal cyst presentations varies and depends
largely on the age of the child at presentation [1]. Literature
often reports that both pediatric and adult populations presents
most commonly with abdominal pain, nausea and vomits, and
jaundice, in that order of occurrence. However, the classic triad
of abdominal pain, jaundice and palpable mass was found only in
10.5% of the children [2]. Hau [10] also reported that the classic
triad of abdominal pain, jaundice and abdominal mass were not
common in newborns or infants with choledochal cyst [10]. Pediatric
cases frequently exhibited cystic lesions with jaundice and
abdominal pain which were more apparent than in newborn and
infants [10].
Todani reported that patients with Type IV-A are commonly
older children and adolescents in comparison with those without
intrahepatic involvement. Most likely due to development of
symptoms appears to be delayed until the intrahepatic cysts are
filled with infected bile. Other presenting features of choledochal
cysts are cholangitis, pancreatitis, and biliary peritonitis from
cyst rupture [11,10]. Our patient presented with pruritus and
without abdominal pain, which is an extremely rare presentation.
Ultrasound is the initial imaging modality of choice for children,
whereas ERCP (endoscopic retrograde cholangiopancreatography)
is most commonly used in adults [9,12]. Surgical treatment
of choledochal cyst should be recommended to reduce the risk of
serious complications such as cholangitis, pancreatitis, rupture,
portal hypertension, cirrhosis and cholangiocarcinoma [9].
The primary complete cyst excision and bilioenteric anastomosis
with wide stoma is the treatment of choice for choledochal
cysts and should be done as early as possible to reduce the risk
of malignancy. Hepaticoduodenostomy or Roux-en-Y- hepaticojejunostomy
are the preferably approaches. Recently, laparoscopic
approaches are being used more often with good results. Our
patient had a primary complete cyst excision and a hepaticoduodenostomy
with concomitant division of biliary ducts performed
with success.
In conclusion choledochal cyst are rare congenital entities.
They can present at different ages with variable symptoms. A high
index of suspicion is necessary since if left untreated choledochal
cysts have an increased rate of malignant transformation and serious
complications.
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