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Case 320

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Female patient, 8 years old, admitted due to severe diffuse abdominal pain started 7 days ago. The mother informs that the pain is recurrent since 2 years old, associated with vomiting, constipation and occasions in which the child gets yellowed skin and darkened urine. On physical examination, the child is icteric and has a palpable mass in the right hypochondrium. Ultrasonography (US) of the liver and biliary tract was performed on admission.

Based on the pictures and clinical history described, which diagnosis is most likely?

a) Primary sclerosing cholangitis

25%

b) Drug hepatitis

25%

c) Choledochal cyst

25%

d) Biliary atresia

25%
   

Image analysis

 

Image 1: Ultrasonography of liver and biliary tract, gallbladder and proximal choledochus region, transverse and longitudinal sections. There is a cystic lesion in the proximal portion of the common bile duct (black arrow), without continuity with the gallbladder (red arrow).

 

Image 2: Ultrasonography of liver and bile ducts, proximal choledochus region, oblique transverse section. Notepresence of large cystic formation in the region of the proximal choledochal duct (red arrow).

 

 

Image 3: Ultrasonography of liver and biliary tract, distal choledochus region’s.

Distal portion of choledochus dilated (red arrow), containing echogenic material,

without acoustic shadow (blue arrow) - biliary sludge.

Highlights

- Choledochal cyst is one of the causes of cholestasis in childhood. Some differential diagnoses are biliary atresia, primary sclerosing cholangitis and congenital infections;

- Abdominal pain associated with jaundice or palpable mass in the abdomen should raise suspicion for the presence of a common bile duct;

- The ultrasound is the examination of choice for the initial evaluation, capable of demonstrating cystic dilatation of the common bile duct. It has good specificity (71-96%), sensitivity (71-97%) and accessibility;

- Secondary biliary cirrhosis and neoplasms such as cholangiocarcinoma are possible complications. The incidence of malignancy reported in the literature ranges from 2.5 to 17.5% and tends to be late;

- The treatment depends mainly on the Todani classification, being surgical in most cases.

 References

 -Bhavsar MS, Vora HB, Giriyappa VH. Choledochal cysts: A review of literature. Saudi J Gastroenterol 2012; 18: 230-6

-Cecil R, Goldman L, Ausiello D, Schafer A. Cecil treated internal medicine. 24th ed. London: Elsevier Health Sciences Spain; 2013.

-Soares K, Arnaoutakis D, Kamel I, Trawling N, Anders R, Maithel S et al. Choledochal Cysts: Presentation, Clinical Differentiation, and Management. Journal of the American College of Surgeons. 2014; 219 (6): 1167-1180.

 Author

Lucas de Oliveira Cantaruti Guida, academic of the 11th period of Medicine at UFMG.

E-mail: lucas_cantaruti [at] hotmail.com

 Supervisor

 Paula Vieira Teixeira Vidigal

Associate Professor of the Medical School at UFMG

pvidigal [at] medicina.ufmg.br

 Reviewers

Bruno Campos, Gabriel Santos, Vinícius Avelar, Mateus Nardelli, Guilherme Rocha, Prof. José Nelson M. Vieira, Profa. Viviane Santuari Parisotto Marino

 Translated by

Luana Fonseca de Almeida, 4 th year medical student at Universidade Federal de Minas Gerais.

E-mail: luana.fonseca.almeida[at]gmail.com

Test question

 (UFU - 2016) Raul, 40 days, is brought to the clinic for being yellow. He was born of normal birth, in term, birth weight equal to 3200g, in good condition. Discharged from hospital with his mom. Mother has reported jaundice since the first week of life, having made use of Picão tea. She sought the doctor when he had fifteen days and it was prescribed guided sunbath and replaced human milk by starting formula. Baby is icteric +++ / 4, hydrated, with globular abdomen, liver 4 cm from the right costal margin and spleen 3 cm from the left costal margin, Current weight = 4 kg. No further changes. During the visit he presented darkened urine and whitish feces.

All of the following may be possible diagnoses, except:

a) Biliary atresia

25%

b) Hemolytic anemia

25%

c) Alagille's syndrome

25%

d) Choledochal cyst

25%

e)

25%
   

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