Home About us Editorial board Search Ahead of print Current issue Archives Submit article Instructions Subscribe Contacts Login 
Print this page Email this page Users Online: 447

  Table of Contents  
CASE REPORT
Year : 2020  |  Volume : 13  |  Issue : 4  |  Page : 410-412  

Hypoplasia of the right hepatic lobe associated with portal hypertension and retroperitoneal fibrosis


Department of Radiodiagnosis, Shri M. P. Shah Government Medical College and Shri Guru Gobind Singh Government Hospital, Jamnagar, Gujarat, India

Date of Submission29-Mar-2019
Date of Acceptance09-Sep-2019
Date of Web Publication20-Jul-2020

Correspondence Address:
Jay Kantilal Satapara
Department of Radiodiagnosis, Shri M. P. Shah Government Medical College and Shri Guru Gobind Singh Government Hospital, P. N. Marg, Jamnagar - 361 008, Gujarat
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/mjdrdypu.mjdrdypu_98_19

Rights and Permissions
  Abstract 


Hypoplasia of the right hepatic lobe is a rare and usually asymptomatic anomaly, which sometimes may be associated with portal hypertension but rarely with retroperitoneal fibrosis. We report one such rare case of hypoplastic right hepatic lobe associated with portal hypertension and retroperitoneal fibrosis. A 45-year-old nonalcoholic male presented with a chief complaint of hematemesis. Serum investigations were normal and inconclusive, so computed tomography was performed which showed right hepatic lobe hypoplasia, esophageal varices, and retroperitoneal fibrosis. Since all other causes of acquired liver atrophy had been ruled out, we considered this case to be of congenital origin. Although it is a rare anomaly, it should be kept in mind to avoid fatal surgical complication.

Keywords: Computed tomography, congenital anomaly, hypoplasia of the right hepatic lobe, portal hypertension, retroperitoneal fibrosis


How to cite this article:
Verma S, Satapara JK, Kumari L, Chudasama S. Hypoplasia of the right hepatic lobe associated with portal hypertension and retroperitoneal fibrosis. Med J DY Patil Vidyapeeth 2020;13:410-2

How to cite this URL:
Verma S, Satapara JK, Kumari L, Chudasama S. Hypoplasia of the right hepatic lobe associated with portal hypertension and retroperitoneal fibrosis. Med J DY Patil Vidyapeeth [serial online] 2020 [cited 2020 Aug 8];13:410-2. Available from: http://www.mjdrdypv.org/text.asp?2020/13/4/410/290182




  Introduction Top


Liver development starts from hepatic diverticulum at the distal part of the foregut in the 2nd week of embryonic life which grows within the transverse septum. Congenital anomalies of the liver are rare.[1] Hypoplasia or agenesis of the right hepatic lobe is one such anomaly, which may be associated with other anomalies such as abnormally positioned gallbladder, compensatory left hepatic lobe hypertrophy, or portal hypertension.[2] Retroperitoneal fibrosis is usually idiopathic and may also cause portal hypertension.[3] Most of the patients are asymptomatic and found incidentally at the time of autopsy or intraoperatively.[4] We report a case of hypoplasia of the right hepatic lobe with portal hypertension and retroperitoneal fibrosis on computed tomography (CT).


  Case Report Top


A 45-year-old male patient presented with complaints of abdominal pain and bloody vomiting for 7 days. The patient had no complaints of fever, weight loss, yellowish discoloration of sclera or urine, or any other complaints. The patient was nonalcoholic and had no significant history, drug history, or family history of liver disease. On clinical examination, spleen was palpable just below the costal margin; rest of general systemic examination was unremarkable.

Routine serum investigations were performed to find the cause of hematemesis, which included liver function tests, complete blood count, hepatitis B surface antigen, and hepatitis C test, all of which came out to be normal.

CT was performed which revealed hypoplastic right hepatic lobe with compensatory hypertrophy of the left lobe. Right kidney and hepatic flexure were displaced upward, and gallbladder was displaced to the right. Spleen was mildly enlarged. Mild ascites was noted [Figure 1] and [Figure 2]. Portal vein was dilated with prominent left gastric vein and multiple varices in the lower part of esophagus and proximal part of lesser curvature, which suggested portal hypertension and the cause of hematemesis [Figure 3]. Extensive fat stranding was noted around inferior mesenteric artery and bilateral anterior pararenal spaces and in the pelvic cavity, which suggests retroperitoneal fibrosis [Figure 4]. Consequently, after ruling out all other causes of acquired liver atrophy, a diagnosis of hypoplasia of the right hepatic lobe associated with portal hypertension and retroperitoneal fibrosis was established.
Figure 1: Plain abdominal computed tomography demonstrates (a) hypoplasia of right hepatic lobe (red arrow) with compensatory hypertrophy of left lobe of the liver (white arrow). (b) There is upward migration of the right kidney. Right kidney is higher than the left kidney. Spleen appears mildly enlarged. RK: Right kidney, LK: Left kidney, S: Spleen

Click here to view
Figure 2: Computed tomography axial view showing ascites (red arrow). The gallbladder is displaced to the right (white arrow)

Click here to view
Figure 3: Contrast-enhanced computed tomography scan: (a) Portal vein is dilated (red arrow) with prominent left gastric vein (blue arrow). (b) Varices are seen in the lower part of esophagus

Click here to view
Figure 4: Computed tomography scan demonstrates extensive fat stranding around inferior mesenteric artery and aorta suggestive of retroperitoneal fibrosis

Click here to view



  Discussion Top


Liver anomalies are rare and usually asymptomatic but do not always remain latent. Agenesis or hypoplasia of the liver is defined as congenital absence or incomplete development of the liver parenchyma involving either right or left lobe in the absence of prior surgery or hepatic disease.[1] Hypoplasia of the left lobe of the liver may cause gastric volvulus, whereas hypoplasia of the right lobe may either remain clinically latent or lead to portal hypertension.[1]

Majority of these anomalies are usually diagnosed as an incidental finding at ultrasonography, CT, or sometimes intraoperatively. However, they may be associated with other abnormalities of the liver or the biliary tract.[5]

Imaging findings include hypoplasia or agenesis of the right hepatic lobe, compensatory enlargement of either medial or lateral segments of the left lobe or caudate lobe of liver, or sometimes both.[6] Gallbladder is displaced in the retrohepatic location.[6] There is upward migration of the hepatic flexure of colon and right kidney; it may lie above the level of splenic flexure and left kidney, respectively.[2],[6]

The differential diagnosis of this congenital anatomic anomaly includes other acquired causes of liver atrophy such as hepatic cirrhosis, cholangiocarcinoma of the hilum, or liver trauma. The diagnosis of congenital hypoplasia of the right hepatic lobe can only be made by ruling out these acquired causes, as in our case.[2]

Hypoplasia or agenesis of the right hepatic lobe may be associated with portal hypertension of the intrahepatic presinusoidal type.[7] Esophageal varices can be an additional complication. Decrease in the number of intrahepatic portal vein branches is not completely compensated by the hypertrophied left lobar portal vein branches, resulting in increased resistance in portal vein and thus portal hypertension.[8] Another hypothesis for portal hypertension is a potential shunt between the hepatic artery and the portal vein.[8]

Retroperitoneal fibrosis is defined as idiopathic fibrous tissue overgrowth in the retroperitoneum, especially in the periaortic region.[3] On CT, it is seen as periaortic soft-tissue mass with variable degree of enhancement depending on the disease activity.[7] Idiopathic retroperitoneal fibrosis may also be associated with portal hypertension.[7]

Although hypoplasia of the right hepatic lobe is a very rare anomaly and may itself be of no clinical significance, its knowledge should be kept in mind as it may be associated with other abnormalities or may present itself with findings of portal hypertension as in our case.[4],[8] Moreover, awareness and familiarity of these anomalies is necessary for surgeons to avoid fatal surgical complications.[8]

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Champetier J, Yver R, Létoublon C, Vigneau B. A general review of anomalies of hepatic morphology and their clinical implications. Anat Clin 1985;7:285-99.  Back to cited text no. 1
    
2.
Kabaroudis A, Papaziogas B, Atmatzidis K, Argiriadou E, Paraskevas A, Galanis I, et al. Hypoplasia of the right hepatic lobe combined with a floating gallbladder. Acta Chir Belg 2003;103:425-7.  Back to cited text no. 2
    
3.
Kottra JJ, Dunnick NR. Retroperitoneal fibrosis. Radiol Clin North Am 1996;34:1259-75.  Back to cited text no. 3
    
4.
Sethi SK, Solanki RS. A case report hypoplastic right hepatic lobe a rare anomaly. Indian J Radiol Imaging 2004;14:53-54.  Back to cited text no. 4
  [Full text]  
5.
Sato N, Kawakami K, Matsumoto S, Toyonaga T, Ishimitsu T, Nagafuchi K, et al. Agenesis of the right lobe of the liver: Report of a case. Surg Today 1998;28:643-6.  Back to cited text no. 5
    
6.
Chou CK, Mak CW, Lin MB, Tzeng WS, Chang JM. CT of agenesis and atrophy of the right hepatic lobe. Abdom Imaging 1998;23:603-7.  Back to cited text no. 6
    
7.
Hisatomi K, Ohya T, Asayama M, Shibata M, Ito M, Sakurai Y, et al. Hypoplasia of the right hepatic lobe associated with portal hypertension and idiopathic retroperitoneal fibrosis. J Gastroenterol 2004;39:685-91.  Back to cited text no. 7
    
8.
Alicioglu B. Right liver lobe hypoplasia and related abnormalities. Pol J Radiol 2015;80:503-5.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

Top
   
 
  Search
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

 
  In this article
   Abstract
  Introduction
  Case Report
  Discussion
   References
   Article Figures

 Article Access Statistics
    Viewed62    
    Printed4    
    Emailed0    
    PDF Downloaded11    
    Comments [Add]    

Recommend this journal