Congenital Absence of Hepatic Segments

 Right lobe: Segments 5 and 8 (anterior segments)


image Left lobe: Segment 4





TOP DIFFERENTIAL DIAGNOSES




• Acquired atrophy with cirrhosis
image Atrophy of anterior and medial segments commonly follows development of focal confluent fibrosis in cirrhosis

image Look for signs of portal hypertension (varices, ascites, etc.)

• Postsurgical resection
image For hepatic resection, incisions can be made along longitudinal or transverse scissurae or both combined

• Post chemoembolization or ablation
image Hyperattenuation of atrophic liver parenchyma

image Check prior imaging studies and medical records for evidence of prior tumor and treatment


CLINICAL ISSUES




• Usually asymptomatic


DIAGNOSTIC CHECKLIST




• Conditions including cirrhosis, atrophy secondary to biliary obstruction, hepatic surgery, and trauma can mimic agenesis and should be ruled out 1st

image
(Left) Axial CECT shows a very small medial segment lying between the falciform ligament cleft image and the gallbladder image. The anterior segments are also small.


image
(Right) Axial CECT in the same case shows the colon image and omental fat filling the gap between the right and left hepatic lobes in the same patient with congenital hypoplasia of the anterior and medial segments.

image
(Left) Axial CECT shows hypoplasia of the anterior and medial segments. Note a clip image from a prior cholecystectomy; the gallbladder normally lies in the interlobar plane. Also note the lateral and cephalic herniation of the stomach image and colon image.


image
(Right) Axial NECT of a more caudal section in the same patient shows the large gap created by congenital hypoplasia of the anterior and medial segments. The gap is filled with omental fat and the hepatic flexure of the colon image.


TERMINOLOGY


Synonyms




• Agenesis or hypoplasia of hepatic lobes or segments


Definitions




• Lobar agenesis or hypoplasia is an uncommon developmental anomaly with absence of liver tissue usually to right or left of gallbladder fossa without prior surgery or disease


IMAGING


General Features




• Best diagnostic clue
image Absence of right or left hepatic vein, portal vein and its branches, and intrahepatic ducts
– If none of these structures are visible, agenesis is substantiated

Nov 16, 2016 | Posted by in GASTROINTESTINAL IMAGING | Comments Off on Congenital Absence of Hepatic Segments

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