|Year : 2020 | Volume
| Issue : 4 | Page : 410-412
Hypoplasia of the right hepatic lobe associated with portal hypertension and retroperitoneal fibrosis
Sumit Verma, Jay Kantilal Satapara, Lata Kumari, Shilpa Chudasama
Department of Radiodiagnosis, Shri M. P. Shah Government Medical College and Shri Guru Gobind Singh Government Hospital, Jamnagar, Gujarat, India
|Date of Submission||29-Mar-2019|
|Date of Acceptance||09-Sep-2019|
|Date of Web Publication||20-Jul-2020|
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
Source of Support: None, Conflict of Interest: None
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 Dec 3];13:410-2. Available from: https://www.mjdrdypv.org/text.asp?2020/13/4/410/290182
| Introduction|| |
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. 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. Retroperitoneal fibrosis is usually idiopathic and may also cause portal hypertension. Most of the patients are asymptomatic and found incidentally at the time of autopsy or intraoperatively. We report a case of hypoplasia of the right hepatic lobe with portal hypertension and retroperitoneal fibrosis on computed tomography (CT).
| Case Report|| |
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|
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|Figure 2: Computed tomography axial view showing ascites (red arrow). The gallbladder is displaced to the right (white arrow)|
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|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|
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|Figure 4: Computed tomography scan demonstrates extensive fat stranding around inferior mesenteric artery and aorta suggestive of retroperitoneal fibrosis|
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| Discussion|| |
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. 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.
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.
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. Gallbladder is displaced in the retrohepatic location. 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.,
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.
Hypoplasia or agenesis of the right hepatic lobe may be associated with portal hypertension of the intrahepatic presinusoidal type. 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. Another hypothesis for portal hypertension is a potential shunt between the hepatic artery and the portal vein.
Retroperitoneal fibrosis is defined as idiopathic fibrous tissue overgrowth in the retroperitoneum, especially in the periaortic region. On CT, it is seen as periaortic soft-tissue mass with variable degree of enhancement depending on the disease activity. Idiopathic retroperitoneal fibrosis may also be associated with portal hypertension.
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., Moreover, awareness and familiarity of these anomalies is necessary for surgeons to avoid fatal surgical complications.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]