| Cavernous Transformation and Portal Vein Obstruction | ||
Barbara Haber
| Database Differential Diagnosis Data Gathering Physical Examination Laboratory Aids Therapy Follow-Up Common Questions and Answers Bibliography |
| DATABASE | ||
DEFINITION
Cavernous transformation is defined as the collection of collaterals that develop around an obstructed vessel. In pediatrics, the obstruction is most typically of the portal vein. In portal vein obstruction the main portal vein or splenic vein is obstructed somewhere along its course, between the hilum of the spleen and the porta hepatis.
PATHOPHYSIOLOGY
Patients present with asymptomatic splenomegaly or upper gastrointestinal hemorrhage, resulting from portal hypertension. Less commonly, the patient presents with ascites or failure to thrive. And 50% of portal vein obstructions are idiopathic. Identified etiologies include:
GENETICS
A genetic basis of this problem has not been identified. Congenital abnormalities of the heart, major blood vessels, biliary tree, and renal system are found in 40% of cases.
EPIDEMIOLOGY
Even if the lesion developed in infancy, patients present at any age. Patients in the 70th decade have been reported, but most patients present between birth and 15 years of age. Bleeding is more typical of patients presenting before 7 years of age; splenomegaly in the absence of symptoms is more typical for patients between ages 5 and 15.
COMPLICATIONS
Most of the complications are secondary to portal hypertension, such as variceal hemorrhage and splenomegaly with hypersplenism. In some cases, steatorrhea and protein-losing enteropathy occur secondary to venous congestion of the intestinal mucosa. Ascites may also develop around the time of the onset of portal hypertension. Often, it develops in the period immediately following an upper gastrointestinal (GI) hemorrhage due to acute hepatic decompensation and decreasing albumin. Both the albumin and the ascites resolve in several weeks.
| DIFFERENTIAL DIAGNOSIS | ||
The differential diagnosis must exclude other causes of splenomegaly and portal hypertension. Once portal vein obstruction has been identified, a search for the underlying etiology must be pursued.
| DATA GATHERING | ||
Clinical examination reveals no jaundice or other evidence of chronic liver disease. Splenomegaly is the only intra-abdominal abnormality that can be identified. Ascites is rarely present.
HISTORY
Question: Other causes of splenomegaly?
Significance:
Exposure to infectious mononucleosis, metabolic storage disease, and
malignancy.
| PHYSICAL EXAMINATION | ||
Finding: Splenomegaly and possible hemorrhoids
Significance:
The spleen is measured from the left anterior axillary line at the costal margin
diagonally toward the umbilicus.
| LABORATORY AIDS | ||
Test: CBC
Significance: Leukopenia and thrombocytopenia will
be present if there is hypersplenism
Test: AST/ALT/GGT
Significance: Should be normal
Test: PT/PTT
Significance: May be abnormal if malabsorption
is present
Test: Protein C, protein S, anti-thrombin III
Significance:
Associated with hypercoagulable states
Test: Ultrasound with Doppler
Significance: To examine
portal vein flow and to identify collateral veins if there is cavernous
transformation of the portal vein. The liver may be slightly small, but may be
normal in texture.
Test: Liver biopsy
Significance: Exclude other
etiologies.
Test: Upper endoscopy
Significance: To define extent of
varices
Test: Bone marrow examination
Significance: To determine if
there is an underlying myeloproliferative disease
| THERAPY | ||
Therapy is designed to manage variceal hemorrhage and to identify an underlying etiology to determine if the patient is at risk for additional venous thrombosis or malignancy. The therapy for GI hemorrhage is:
| FOLLOW-UP | ||
Follow-up by the pediatrician should focus on growth parameters and early detection of malabsorption. The gastroenterologist will manage GI hemorrhage and supplemental nutrition, if needed.
PITFALLS
The patient should be advised about activity restrictions due to splenomegaly and should be told to avoid medicines that interfere with platelet function.
| COMMON QUESTIONS AND ANSWERS | ||
Q: What is the long-term prognosis?
A: Good. Upper GI
hemorhage becomes less problematic as the child becomes older. Shunting is
rarely recommended. Most patients undergo prophylactic sclerotherapy. As liver
function is normal, it is rare for encephalopathy to develop.
Q: Should I restrict my child’s activities?
A: Contact
sports should be limited or a spleen guard used. NSAIDs, including aspirin,
should be avoided because of the risk of hemorrhage.
ICD-9-CM 747.49
| BIBLIOGRAPHY | ||
Gitnick G, LaBrecque DR, Moody FG. Diseases of the liver and biliary tract. Philadelphia: Mosby-Year Book, 1992.
Mowat AP. Liver disorders in childhood. Boston: Butterworths, 1987.
Suchy F. Liver disease in children. Philadelphia: CV Mosby, 1994.
Copyright
© 2000 Lippincott Williams & Wilkins
M. William
Schwartz, Louis M. Bell, Jr., Peter M. Bingham, Esther K. Chung, David F.
Friedman and Andrew E. Mulberg, The 5 Minute Pediatric Consult