Non-transplant Management of Portal Hypertension in Children

Fig. 26.1

Hepatic vein measurements and gradients

Diseases causing PH in children can be classified according to the aforementioned pressure-gradient table, which places patients into two principle groups – those with associated significant liver disease and those associated with no significant liver disease. The performance of these pressure-gradient measurements is feasible and safe in children; however, it may not be necessary in the routine clinical care of these patients if the determination of in one of these groups is evident using other modalities.

Children with liver disease are usually cirrhotic, biliary atresia being the most common disease (60% of childhood cirrhosis, of which 70% may have varices); nonetheless, only in a minority are the complications of PH a dominant feature of their disease. Also, in those patients with early decompensation any evidence of PH will prompt liver transplantation. Late decompensation (with preserved liver function in successfully treated children with the Kasai operation) may manifest complications of PH in adolescence and be amenable to therapy other than transplant. Some patients present persistence of a small caliber portal vein with this disease, which may be a consequence to decreases in flow as cirrhosis develops and can make the reconstruction of the portal vein at transplant difficult. Alpha-1-antitrypsin deficiency is the other notable cirrhotic disease in children, occurring only in the PIZZ phenotype (18% of children affected) with the risk of developing jaundice, hepatomegaly, cirrhosis, and possibly hepatocellular carcinoma at various time points of the disease. The manifestations of PH are usually in concert with other evidence of cirrhosis, because the evolution can span from the neonate to the elderly careful follow up for the development and management of PH is necessary. Postnecrotic cirrhosis (viral or toxic) is rare in children; familial cholestatic syndromes, biliary cirrhosis, and cystic fibrosis (only 2% of whom develop clinically significant PH) account for the other causes of PH. Congenital hepatic fibrosis is an intrahepatic disorder which behaves like an extra-hepatic/pre-sinusoidal disease (as in with no associated liver disease) and is a consequence to the deposition of fibrous bands of dense connective tissue in portal and peri-portal areas; complications of PH can occur in 30–70% patients, which prompts aggressive management. Also of consideration is the associated ductal disease of the kidneys which may lead to renal failure and need for Kidney transplantation, at which time consideration for PH management may be required (including transplantation) (Table 26.1).

Table 26.1

Intrahepatic causes of portal hypertension

Intrahepatic
Postsinusoidal	Veno-occlusive disease
Sinusoidal	Cirrhosis (BA, A1A)
	Nodular regenerative hyperplasia
	Hypervitaminosis A
	Postnecrotic
Presinusoisal	Schistosomiasis
	Congenital hepatic fibrosis
	Sarcoidosis
	Portosclerosis
	Hepatic artery-portal vein fistula

Rarely will posthepatic disease cause PH in children, the most notable being constrictive pericarditis particularly tuberculous in origin, and generally in areas of significant endemic disease. Other notable causes for reference are included in Table 26.2.

Table 26.2

Posthepatic causes of portal hypertension

Posthepatic
Heart failure
Cardiomyopathy
Congenital heart disease
Constrictive pericarditis
Inferior vena cava thrombosis
Congenital web in inferior vena cava
Budd-Chiari syndrome
Tumor

Given the success of Liver transplantation for patients with Liver disease, many patients presenting with bleeding complications of PH at this time are in association with no significant Liver disease, which has important management and outcome implications. Common diseases for this form of presinusoidal cause of PH are noted in Table 26.3.

Table 26.3

Prehepatic causes of portal hypertension

Prehepatic
Portal vein thrombosis
Portal vein stenosis
Cavernous transformation of portal vein
Congenital anomalies of portal vein
Tumor

These noncirrhotic diseases comprise what is termed extrahepatic portal vein obstruction (EHPVO), bleeding mostly occurring early in life, can be idiopathic (neonatal sepsis), or secondary to malignancy, trauma, hypercoagulable states, intraperitoneal inflammatory processes (portal vein phlebitis), and umbilical vein catheterization. It can also present in the setting of Liver transplantation, Budd Chiari, and cirrhosis. Over 50% of children with EHPVO develop severe bleeding episodes, however, the mortality rate is low (0–2%, vs. the mortality rate in the setting of cirrhosis which ranges from 2.5% to 20%). This low mortality rate from bleeding is likely due to the absence of other complications seen in patients with cirrhosis, however, these patients do suffer from massive splenomegaly and hypersplenism, growth retardation, neurocognitive impairment, and encephalopathy. The disease is indolent, the age at presentation is quite variable (from infancy to adolescence), and the frequency and intensity of bleeding is similarly varied and unpredictable.

Diagnosis and Management

Irrespective of the cause of PH the clinical consequences are the same: bleeding from gastro-esophageal varices, splenomegaly with hypersplenism, hepatopulmonary syndrome, portopulmonary hypertension, neurocognitive impairment, growth retardation, and encephalopathy; depending on the etiology and Liver reserve, ascites may be a component. The principle emphasis which has guided management has been the control of bleeding.

Portal Hypertension should be suspected in children who present with unexplained gastrointestinal bleeding, evidence of porto-systemic collateral circulation, cyanosis, splenomegaly, hypersplenism, or abdominal distention with ascites. Some patients may be referred by a pediatric hematologist/oncologist after an extensive work up for the non-bleeding complications of PH. Generally, the initial history and physical examination will determine the likelihood of the presence or absence of chronic Liver disease; such a determination will then focus the evaluation and severity of the Liver disease, specifically cirrhosis. In the absence of evidence to support cirrhosis, consideration of EHVO as a cause for the PH is quickly assessed by Doppler ultrasonography of the Liver visualizing the Liver (for parenchymal texture, bile ductular dilations, cysts, vascular anomalies, and surface irregularities), portal vein patency and flow changes (reversal of flow), hepatic vein patency (venous outflow), the presence of splenomegaly, renal abnormalities, and visceral rotations. Upper gastrointestinal endoscopy is the second investigative modality for the assessment and potential management of varices and is specifically performed after a bleeding presentation; there are no evidenced based recommendations regarding screening endoscopy, since therapeutic recommendations used in adults have not been studied in children regarding safety or efficacy in the prevention of the first variceal bleed. Computerized tomography or magnetic resonance imaging, and selective angiography with their various arterial and venous phase imaging are used when no lesion can be detected in the portal vein or there is no evidence of Liver disease. Hepatic vein pressure and gradients can be measured as well and determine other pre and post Liver diseases as the cause of PH; this transjugular vein route may also allow for retrograde visualization of the intrahepatic portal venous anatomy, the presence of angiodysplasias, and the performance of a liver biopsy (if indicated). The use of splenoportograms (through the transcutaneous puncture of the Spleen) has been in disuse for many years due to the risk of splenic bleeding or rupture. All of these diagnostic modalities may assist in the planning of a therapeutic strategy [4].

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