Pediatric Portal Hypertension Case Presentation
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- 23 month old girl presented with hematemesis and melena - History of prematurity and prolonged NICU stay - Investigations revealed esophageal varices, splenomegaly, thrombocytopenia, and portal vein thrombosis - MRI and MRCP showed choledochal cyst and thin caliber portal vein, consistent with extrahepatic portal vein thrombosis - She was diagnosed with portal hypertension secondary to extrahepatic portal vein thrombosis and choledochal cyst
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Pediatric Portal Hypertension Case Presentation
- 2. Case: ⢠23 month old girl, corrected age 19 months,24/52, has been brought to ED: ⢠Hematemesis ⢠Melena ⢠Fever
- 3. What to ask? Liver disease Mallory Weiss tear Acid peptic disease Medications Ingested blood FB/Battery ingestion Obstruction Food Color
- 4. General Examination: ⢠Pale ⢠Lethargic but arousable ⢠No apparent dysmorphism ⢠Not jaundiced or icterus ⢠No clubbing ⢠No palmar erythema ⢠No stigmata of liver disease ⢠Capillary refill 3-5 sec ⢠Vitals: ⢠HR: 130 BP: 77/41 Temp: 36.6C ⢠RR: 44 Spo2: 100% Features of Severe GI bleed Prolonged capillary refill Hypotension Melena Decrease in HGB >2g/dl HR>20 BPM
- 5. Systemic Examination: ⢠Chest: Bilaterally clear, no adventitious sounds ⢠CVS: S1+S2 no murmur heard, pulses well felt and equal, mild tachycardia ⢠GIT: Soft abdomen, non protuberant, normal shaped umbilicus, spleen palpable 3cm BCM, liver not palpable, no abdominal veins, ⢠CNS: GCS: 15/15, lethargic, normal deficit, normal reflex and tone. ⢠Skin: No Rash, spider angiomata ⢠Eyes: Non-icteric ⢠ENT: Normal mucosa
- 6. Treatment in ER ⢠Received IV normal saline bolus twice ⢠IV Ranitidine ⢠IV ondansetron ⢠Investigations were collected
- 7. What Investigations to do? CBC, U/E, creatinine, LFT, Coagulation profile, Blood type and cross, Guaiac testing Radio-imaging: Abdominal x-ray , USS abdomen
- 8. FBC Counts Range WBC 16.3x 10^3/uL 6-18 HGB 8.2 g/dl 11-14 RBC 3.74 x 10^3/uL 3.9-5.1 MCV 70 fl 72-84 RDW 16.9 11.5-14 PLT 164 x 10^3/uL 20-550
- 9. LFT Value Range T.Bil 0.5 6-18 ALT 305 11-14 AST 193 3.9-5.1 GGT 77 72-84 Albumin 3.0 11.5-14 PT 16.7 11-14 APTT 40.7 28-41 RFT Value Range Urea 55 12-40 Na 136 131-145 K 3.7 3.2-5.4 Cl 101 96-111 CO2 19.9 22-28 Creatinine 0.2 0.2-0.4
- 10. Lab Value Range CRP 9.0 <10 PCT 0.45 <0.05 Blood culture No Growth Blood film: Hypochromic Microcytic cells with poikilocytosis, Leukocytosis, neutrophilia with slight shift to the left. Normal platelet count
- 11. Abdominal X-ray: Conclusion : ⢠No free air shadow seen ⢠Normal gaseous bowel distention ⢠No radiopaque FB shadow seen
- 12. USS Abdomen: Findings: Liver: The liver showing homogenous echotexture. No focal lesion could be seen. Spleen: Spleen is enlarged measures about 10 cm in diameter showing homogenous echotexture CYST: An irregular cystic structures seen at the porta hepatis showing a proximal diameter measures about 2.6 cm No comment on ascites, collateral or portal vein size Conclusion: an irregular cystic lesion seen at the porta hepatis : ? choledochal cyst ?
- 13. Provisional Diagnosis ⢠Hematemesis and Melena for investigation â˘Admitted to the ward : ⢠For Diagnostic workup and supportive care
- 14. History: ⢠Birth History: ⢠Antenatal: IVF pregnancy, triplets, TCTA, received betamethsone ⢠Natal: mother had PROM 24 hours prior delivery, 24/52, LSCS, had weak cry electively intubated at birth ⢠Postnatal: She was admitted in NICU for around three months and had a stormy course. ⢠She was managed for following problems: ⢠Respiratory distress syndrome ⢠Right sided pneumothorax ⢠Chronic lung disease (received steroids) ⢠ESBL Klebsiella Sepsis ⢠PDA (closed medically), ⢠Retinopathy of prematurity stage 3 plus disease ⢠neonatal seizures ⢠neonatal jaundice of prematurity ⢠neonatal cholestasis ( TPN associated) ⢠GERD ⢠Neonatal anemia.
- 15. History: ⢠Past medical history: ⢠Readmitted in hospital at 17 months of age due to Enteroviral pneumoniae and Thrombocytopenia. Received platelets. ⢠Had an episode of spontaneously resolving epistaxis 1 month ago. ⢠Persistently low platelets ⢠Vaccination: ⢠Uptodate as per DHA schedule ⢠Feeding History: ⢠Pediasure and normal family diet , following clinical nutritionist ⢠Development: ⢠Mild motor delay for corrected age Family History: ⢠Born to non consanguineous parent. Father has celiac disease. Three other siblings are healthy. On of triplet died postnatally and one triplet had similar course in NICU is alive.
- 16. Summary: ⢠23 months old girl ⢠Ex preterm 24/52 ⢠Prolonged and turbulent postnatal NICU stay. ⢠Acute episode of Hematemesis and Melena ⢠Pallor and Splenomegaly ⢠Persistently low platelets Labs Result FBC HGB 8.2 g/dl PLT 164 x 10^3/uL LFT and coagulation ALT 305 AST 193 U/E: Urea 55 USS abdomen an irregular cystic lesion seen at the porta hepatis
- 17. Differential Diagnosis for UGI bleed ⢠Esophageal Varices ⢠Hemorrhagic Gastritis ⢠Peptic Ulcer disease ⢠Mallory Weiss Tear ⢠Epistaxis (swallowed blood) ⢠Bowel obstruction
- 18. Progress in Ward: Day 1 ⢠Was given PRBC transfusion once ⢠Started on Octreotide infusion ⢠IV Vitamin K ⢠IV Esomeprazole ⢠IV Cefuroxime ⢠Only one episode of melena after admission
- 19. What further investigation would be indicated? ďź USS doppler ďź Endoscopy
- 20. USS doppler Reduced mean flow velocity Dilatation in MPV Reversal of flow Recanalization Collateral vessels/ Cavernoma
- 21. USS Doppler Findings: Day 3rd ⢠Well defined cystic lesion measuring about 1.6x1.4 at the porta hepatis ⢠No color flow seen inside and not separable from the regional PV and hepatic artery. ⢠Enlarged liver measuring about 9.1 cm in MCL. No hepatic focal lesions. ⢠Enlarged spleen , measuring about 10.4 cm. No focal lesions. ⢠Mild free fluid at the upper abdomen. ⢠Conclusion: ⢠Possibility include: 1. Cavernous transformation ? secondary to chronic PV thrombosis. 2. Choledochcal cyst.
- 22. UGI endoscopy â˘Endoscopy is both diagnostic and therapeutic in patients with UGI bleed. â˘Varices: â˘Site, grade â˘Predictors of bleed â˘Portal hypertensive gastropathy Grade 1 Grade 2 Grade 3
- 23. UGI Endoscopic findings ⢠Esophagus: In Lower esophagus there were 4 columns of varices at 3,4,7 and 11 o clock. Grade 2 and 3. ⢠Stomach: Fundal varices ⢠Intervention: ⢠Banding device was applied to varix ⢠Prophylactic Propranolol was started
- 25. Summary: ⢠23 months , ex-preterm 24/52 ⢠Prolonged and turbulent postnatal NICU stay. ⢠Presented with acute episode of Hematemesis and Melena ⢠Pallor and Splenomegaly ⢠History of Persistently low platelets ⢠Low platelets, Mildly deranged liver transaminases ⢠USS doppler: Well defined cystic lesion measuring at the porta hepatis ⢠Endoscopy: esophageal and fundal varices ⢠Thrombophilia screen and autoimmune hepatitis profile was sent
- 26. Day 5 ⢠No active complaints ⢠No Bleeding episodes ⢠Vitally and clinically well ⢠IV octreotide infusion weaned in 72 hours ⢠IV cefuroxime completed for 5 days and stopped ⢠on prophylactic propranolol and esomeprazole ⢠Labs Repeated LFT New Old T.Bil 0.4 0.5 ALT 106 305 AST 63 193 Albumin 3.1 3.0 FBC New Old WBC 6.3x 10^3/uL 16.3x 10^3/uL HGB 9.3 g/dl 8.2 g/dl PLT 122x 10^3/uL 164 x 10^3/uL Viral study Result HIV Negative HBV Negative
- 27. What further investigation may help? MRI abdomen with contrast
- 28. MRI abdomen + MRCP ⢠LIVER: The liver is enlarged however showing normal signal intensity with no detectable focal lesion. ⢠Biliary System: The left intrahepatic biliary tree is dilated. Two cysts are noted arising from the common bile duct posteriorly measuring about 1.6 cm and anteriorly measuring about 1.5 cm and containing a stone measuring about 7 mm. The gallbladder appears normal with dilated cystic duct. No gallstones or acute cholecystitis. ⢠Portal Vein: The portal vein is of thin caliber. ⢠Spleen: The spleen is enlarged measuring about 13 cm. ⢠Ascites: Moderate to large pelvi-abdominal ascites. ⢠Pancreas, both kidneys and bladder appear normal in size, outline and signal intensity. ⢠No retroperitoneal lymphadenopathy, lung bases are showing bilateral collapse-consolidations. ⢠The visualized bony skeleton appears unremarkable ⢠Conclusion: ⢠The MRI findings are likely in favour of type 2 choledochal cyst. ⢠Thin calibre portal vein
- 29. MRCP:
- 30. Repeated USS Doppler ⢠Portal vein appears narrow 3 mm with extrahepatic varicose net of vesselsď Cavernoma ⢠Suggested Diagnosis : 1. Portal vein thrombosis 2. Congenital stenosis of portal vein ?
- 32. Thrombophilia screen Protein C 89% ( Range: 70 to 140) Protein S 73% ( Range: 60 to 160) Anti thrombin 3 87 % ( Range: 80 to 130) Activated protein C resistance Low <0.8 ( Range: 0.86 to 1.10) ALKM Negative ASMA Negative ANA 1:100 weakly positive Autoimmune hepatitis screen
- 33. Hematology consult was taken Following tests were done: ⢠Factor V Leiden Assay (most common cause) ⢠Prothrombin gene mutation (2nd most common cause) ⢠Factor 8 Assay ⢠Homocysteine
- 35. Summary: Acute hematemesis Esophageal and fundal varices Portal hypertension secondary to Portal vein thrombosis Hypersplenism Choledochal cyst (Todani type 2) Failure to thrive Low activated protein C resistance ratio- results awaited
- 36. Portal Hypertension: ⢠It is defined as Hepatic venous Pressure gradient between the IVC and portal vein greater than 5 mm of Hg. ⢠PPG>10mmHg(varices) ⢠PPG>12 mmHg(ascites)
- 37. Pathophysiology
- 38. Classification ⢠Extrahepatic ⢠Intrahepatic Presinusoidal ⢠CirrhosisSinusoidal ⢠Extrahepatic ⢠Intrahepatic Post sinusoidalPre- Sinusoidal Sinusoidal Post- Sinusoidal Causes
- 39. Clinical Features: Features/Type Pre- Sinusoidal sinusoidal Post-sinusoidal Our patient Mean age Children All Ages All Ages 2 years GI Bleed +++ + +/- Present Ascites/pedal edema +/- ++ +++ Present Spleen +++ + + Present Liver +/- ++ +++ USS enlargement Anterior Abdominal veins +/- ++ +++ back veins - Encephalopathy - ++ +/- - Stigmata of LD - +++ ++ - USS PV thrombosis, Cavernoma, Collaterals Coarse liver, Collaterals, dilated PV, Hepatic vein or IVC thrombosis Enlarged liver Thin caliber PV cavernoma
- 41. Management (EHPVO): ⢠of life threatening hemorrhage Emergency treatment ⢠directed at prevention of subsequent bleedingProphylaxis ⢠Rex bypass shunt ⢠Splenorenal shuntSurgery
- 42. Complications of PHT Growth retardation Pubertal delay Varices Hypersplenism Portal hypertensive biliopathy . Hepato- pulmonary syndrome Hepato-renal disease
- 43. Prognosis: ⢠Depending on underlying cause: extrahepatic vs intrahepatic PHT ⢠Intrahepatic: Liver transplantation ⢠Extrahepatic: ⢠frequency of bleeds decreases as they get older ⢠Neurocognitive defects naturally occurring portosystemic shunts ⢠Progressive liver disease can be treated or prevented by the Rex shunt.
Editor's Notes
- Hematemesis was today large in amount around 2 cups, fresh blood 2-3 times ( no bile) Melena was today morning one time with loose stools, moderate to large amount Fever is tactile since past 2 days associated with URTI symptoms With good oral intake and activity until symptoms. A quick history aimed at broadly identifying the Cause : Upper GI bleed: (proximal to ligament of Treitz) Bright red vomitus indicates brisk bleeding with melena (Indicating Gastric acid effect on blood) (( had this been LGI it would have been dark maroon or bright red))
- Cause: Liver disease: jaundice, easy bruising, edema Mallory weiss: episodes of frequent forceful of vomiting followed by hematemesis in a relatively well child Acid peptic disease or GERD with esophagitis : Feeding difficulty/dysphagia, regurgitation, fussiness with eating, in older child retrosternal pain/epigastric pain, poor weight gain Medications: NSAIDS, corticosteroids( with ibuprofen even short term usage can cause gastritis) Ingested blood: recent epistaxis Battery ingestion: No Foreign body ingestion history Obstruction: Intussception, Volvulus : bilious emesis , episodic abdominal pain/irritability, drawing up of legs during episodes with quiet periods in between. Food Color: well appearing coloring agents, tomato skin, ketchup, spinach, licorice, iron, bismuth salicylate
- Previous HGB 10.4 ď 8.2 UGI Bleedď SEVERE GI BLEED Class I. Loss of 15% or less of the total blood volume. clinical manifestations of hypovolemia are minimal or absent. Class II. Loss of 15 to 30% of the blood volume. The clinical findings at this stage may include resting tachycardia and orthostatic changes in heart rate and blood pressure Class III. Loss of 30 to 40% of the blood volume usually marks the onset of hypovolemic shock, with a decrease in blood pressure and urine output Class IV. Loss of more than 40% of blood volume is a harbinger of circulatory collapse. Therefore, when hypovolemia is accompanied by marked hypotension, oliguria, or other evidence of organ failure, prompt volume resuscitation is mandatory.
- CNS: lethargicď¨ due to hypotension GIT: splenomegalyď evidence of PHT Nasopharynx: For evidence of disrupted mucosa or inflamed tonsils suggesting swallowed blood. UGI Bleedď SEVERE GI BLEEDď Portal Hypertension (indeed PHT is Manifested by two principal signs Hematemesis (dilatation of collateral venous channels)+ Splenomegaly , which may cause depression of one or two blood elements )ď presinusoidal, sinusoidal or post sinusoidal
- Principal management:
- A brisk response may also be seen in hemorrhage. Drop in HCT predicting loss of blood in ml: Each unit of blood loss drops the hematocrit by 3 percent points (hemoglobin by 1 mg/dL). Conversely stated if the hematocrit drops by 6% the patient has lost 2 units of blood.
- Bowel obstruction: We are looking for causes of GI bleed that can be seen on xray: that is true especially + clinical signs of obstruction : that is volvulus or intussusception . Signs of Perforation from ulcer. Conclusion : Bowel obstruction: LGI bleed/hematochezia such as volvulus or intussusception ď Normal gaseous bowel distention . Perforation: ďNo free air shadow seen . FB: ď No radiopaque FB shadow seen
- What we expect? PHT: . LIVER: Presinusoidal liver is normal and post sinusoidal it may be normal or coarse depending on duration and sinusoidal it is usually coarse. Splenomegaly Presence of ascites Portal vein : A dilated portal vein (diameter of greater than 13 or 15 mm) is a sign of portal hypertension, dilated in sinusoidal and post sinusoidal whereas in presinusoidal its not visualized with cavernomas. Collaterals: gastro, spleno renal Findings: Liver: The liver showing homogenous echotexture.  No focal lesion  could be seen. Spleen: Spleen is enlarged measures about 10 cm in diameter showing homogenous echotexture CYST: An irregular cystic structures seen at the porta hepatis showing a proximal diameter measures about 2.6 cm No comment on ascites, collateral or portal vein size Conclusion: an irregular cystic lesion seen at the porta hepatis : ? choledochal cyst ?  Repeated USS abdomen next day : INCONCLUSIVE Liver size 6.6cm Moderate ascites
- Interpretation: UGI Bleedď SEVERE GI BLEEDď Portal Hypertensionď presinusoidal, sinusoidal or post sinusoidal ď Presinusoidal (as PV not dilated and spleen enlarged) ( for sinusoidal liver should be coarse/echogenic for post sinusoidal liver should be enlarged signs of thrombus in hepatic or IVC)
- Interpretation: UGI Bleedď SEVERE GI BLEEDď Portal Hypertensionď presinusoidal, sinusoidal or post sinusoidal ď ? Presinusoidal ď history of neonatal sepsis and umbilical catheterization ď presinusioidal probably portal vein thrombosis ?
- Esophageal varices: Hematemesis and melena with spleen ( INTRAHEPATIC: with hepatomegaly and/or stigamata of liver disease such as jaundice, palmar erythema, spider nevi, ascites), EXTRAHEPATIC: no stigmata of liver disease) Gastritis and Peptic ulcer disease: history of fussiness, regurgitation or vomiting with eating Mallory Weiss tear: relatively well looking with History of frequent forceful vomiting Epistaxis : nasal mucosal clots or bleed Bowel obstruction: Intussception and volvulus: Toxic looking with severe abdominal pain (episodic in intussception) and Abdominal distention and ( Bilious vomiting in volvulus)
- Octreotide: : Use: may help in reducing or temporizing GI bleeding in selected cases of variceal bleeding MOA: It reduces portal venous inflow and intravariceal pressure, cause vasoconstriction in the blood vessels Administration : it is usually given as bolus followed by maintenance infusion and if bleeding stops it is tapered over 24 hours. The optimal duration of therapy is unclear Adverse-effects: it emanates from fact it inhibits various GI hormones, GI motilityď abdominal cramps, anorexia , vasoconstriction ď hypertension pancreatic hormones and secretionď malabsorption, inhibit neurotransmittersď abnormal gait, confusion Anti-biotics: According to one study: Bacterial infections are common in cirrhotic patients with acute variceal bleeding, occurring in 20% within 48 h. Outcomes including early rebleeding and failure to control bleeding are strongly associated with bacterial infection According to another study: Intravenous antibiotic therapy should be considered for all patients with variceal bleeding in light of the high risk of potentially fatal infectious complications It remains unsure whether infection or bleeding is the initiating event but prophylactic antibiotics have been proven useful. Short term fluoroquinolones and cephalosporins are the most studied antibiotics. Blood in the intestinal lumen can promote bacterial translocation, leading to peritonitis Bleeding/ Coagulopathy: Platelets: should be administered for levels less than 50 Ă 109/L, coagulopathy : corrected with vitamin K and fresh frozen plasma. Acid suppression: According to studies : Use of agents such as PPI significantly reduces the risk of rebleeding patients with UGI bleed PRBC transfusion: Patients with hemorrhagic shock should receive blood and require definitive treatment for the cause of hemorrhage especially after no improvement on 60ml/kg fluid boluses. Packed red blood cells should be infused in 10 mL/kg boluses Need for blood transfusion (given if hemoglobin <8 g/dL) Decrease in hemoglobin of more than 2 g/dL
- What we looking for ? a portal flow mean velocity of less than 12Â cm/s, dilatation in the MPV are diagnostic of portal hypertension.[14] upperlimit 13-16 Reversal of direction of flow in portal vein (normal direction towards liver) Recanalization of paraumbilical vein: pathognomonic Collaterals vessels or cavernoma
- Cystic lesion ? cavernoma ď PHT Hepatosplenomegaly : 1-<2y Liver M Mean: 8.6 cm (0.85) 3rd 7.1 97th 10.2 3rd Spleen: Mean: 6.4 (1.01) 3rd 4.7 97th 9.8 Liver F Mean: 8.5 cm (1.51) 3rd 6.3 97th 11.1 Spleen: Mean: 6.1 (0.74) 3rd 4.5 97th 7.6 Interpretation: UGI Bleedď SEVERE GI BLEEDď Portal Hypertensionď presinusoidal, sinusoidal or post sinusoidal ď ? Presinusoidal ď history of neonatal sepsis and umbilical catheterization ď presinusioidal probably portal vein thrombosis ? What is the cyst? Incidental findings? Technical difficulty they couldnât identify there was limitation in commenting on portal vein and its flow and cystic lesion
- Guidelines recommend to perform it for cases of UGI bleed especially if it is severe ,acute in 24 to 48 hours . Both for diagnostic and therapeutic purposes. Earlier may be needed if bleeding is uncontrolled. Hemodynamically unstable patient should be stabilized prior to it. Grades of varices: When esophageal varices are discovered, they are graded according to their size, as follows: Grade 1 â Small, straight esophageal varices Grade 2 â Enlarged, tortuous esophageal varices occupying less than one third of the lumen Grade 3 â Large, coil-shaped esophageal varices occupying more than one third of the lumen Predictors of bleed: Larger and more superior varices had a higher bleeding Another endoscopic finding of value in variceal bleeding is the appearance of the vessel wall. finding of "red signs" is related to the variceal bleeding predicting. The red color signs are the result of microteleangioectasia of the varix. chery red spots(dilated subepithelial veins), hemocystic spots (crimson projections)((represent blood exiting from the deeper esophageal veins)) PHT Gastropathy: Fundal varices can also be found on stomach
- Site, grade Predictors of bleed Portal hypertensive gastropathy
- Interpretation: UGI Bleedď SEVERE GI BLEEDď Portal Hypertensionď presinusoidal, sinusoidal or post sinusoidal ď ? Presinusoidal ď history of neonatal sepsis and umbilical catheterization ď presinusioidal probably portal vein thrombosis ((Confirmed varices, treated it)) ď screen fr underlying coagulopathy ď ? What is the cyst? Incidental findings?
- Findings: dilated portal vein +/- mesenteric veins contrast enhancement of paraumbilical vein: pathognomonic collateral vessels/varices: these are many and can include 4
- Magnetic resonance cholangiopancreatography â MRCP does not expose patients to ionizing radiation and does not have the risks of cholangitis and pancreatitis associated with ERCP. In many cases, it is the test of choice for diagnosing and evaluating biliary cysts. Its sensitivity for biliary cysts is between 73 and 100 percent
- To study portal vein
- APC resistance Positive ANA ? Significant
- Factor 5 leidin mutation: MC: replacement of arginine with glutamine at amino acid 506 of factor 5 gene causes Removes the site of action for APC in Factor 5ď Increased level of activated factor 5aď Hypercoagulable state Prothrombin gene mutation: Sometimes referred to as the factor II mutation or simply the prothrombin mutation. Mechanism:increased prothrombin biosynthesis without affecting the rate of transcription; increased glycosylation that promotes protein stability increased prothrombin levels rendering it unable to be controlled by degradation of factor 5 which is usually destroyed by APC associated with G20210A prothrombin gene mutation could affect the results of activated protein C (APC) resistance phenotype and increase the risk of venous thrombosis Factor 8 assays: aPC resistance has been described in patients with elevated levels of coagulation factor VIII (figure 1). Circulating levels of factor VIII can be increased in inflammatory disorders Homocytine: in vitro homocysteine treatment of factor V can protect Îą-thrombin-derived factor Va from inactivation by APC. The most probable mechanism by which homocysteine inhibits APC inactivation of factor Va is by forming heterologous disulfide bond
- The normal portal venous pressure is approximately 7âŻmmâŻHg Hepatic Venous pressure gradient is measured by inserting a catheter equipped with a balloon into the hepatic venous system via IVC and indirectly measuring portal venous pressures. The clinical features of the various forms of portal hypertension may be similar, but the associated complications, management, and prognosis can vary significantly
- Portal hypertension can result from obstruction to portal blood flow anywhere along the course of the portal venous system. Cirrhosis which increases intrahepatic resistance to blood flow or portal vein thrombosis. Once PHT develops it creates collateral vessel formation (esophagus, stomach, SI and rectum)( promoted VEGF42-44 and placental growth factor (PlGF)) and arterial vasodilation including splanchnic and systemic vasodilation ( NO is the most important vasodilator molecule that contributes to excessive vasodilation ) This helps to increase the blood flow into the portal vein, which exacerbates portal hypertension A hyperdynamic circulation is achieved by tachycardia, an increase in cardiac output, and decreased systemic vascular resistance. Consequently varices develps particularly in the esophagus or anorectal region along with ascites
- Pre-sinusoidal: Extrahepatic: Splenic or portal vein thrombosis (hypercoagulable states: protein c and s deficiency, antithrombin c, tumor invasion such as RCC , neonatal sepsis, umbilical vein catheterization, NEC,peritonitis ), Congenital stenosis of portal vein, Intrahepatic: NCPF Sinusoidal: Cirrhosis: replacement of liver parenchyma by fibrotic tissue due to plethora of causes : infecive: Hepatits B, C, genetic: Cystic fibrosis, Wilson, Metabolic: GSD type 4 , autoimmune Post-Sinusoidal: extrahepatic: Budd-chiari Syndrome , IVC obstruction (Hypercoagulabel states, Malignancy(RCC)) Intrahepatic: VOD
- Presinusoidal: Its classical presentation is with UGI bleed with splenomegaly with out a preceding history of liver disease and in the absence of stigmata of liver diseases such jaundice, SN, Abdominal veins, pedal edema or ascites (if present it is transient) or encephalopathy. USS will support findings of Portal vein obstruction such as narrowing, cavernoma or presence of clot. Hemorrhage, particularly in children with portal vein obstruction, can be precipitated by minor febrile, intercurrent illness Sinusoidal: UGI bleed is often not the presentation and in case of PHT leading to UGI bleed these patients with underlying hepatic disease the physical examination might show jaundice and stigmata of cirrhosis such as palmar erythema and vascular telangiectasia. 3. Post sinusoidal: Acute: patient develops symptoms rapidly severe right upper quadrant pain and hepatomegaly [4]. Jaundice and ascites may not be apparent initially but often develop rapidly. Subacute and chronic Budd-Chiari syndrome : Patients who are asymptomatic often have large hepatic vein collaterals. However, ascites and lower extremity edema may occur because chronic occlusion of the hepatic veins. Splenomegaly: Single most important diagnostic sign of PHT. Corelates well with the type of portal hypertension rather than severity. Hence, Hypersplenism is predominant in Presinusoidal Dilated abdominal veins: Presence supports the diagnosis of PHT (Sinusoidal and postsinusoidal) Primarily indicates intrahepatic portal hypertension and absence doesnât exclude PHT. Back vein especially indicates post sinusoidal. Ascites: Presence supports the diagnosis of PHT. Its not frequent presentation in presinusoidal causes. Though Its presence gives clue to sinusoidal and post sinusoidal causes especially sudden accumulation of ascites points to HVOO Liver: Consistency is more significant than size. Normal soft liver in Presinusoidal PHT. Firm nodular and even enlarged in sinusoidal causes. Liver can be very enlarged and tender in Post sinusoidal causes. Its non cirrhotic in post sinusoidal in acute and subacute stages whereas in sinusoidal disease the liver is frequently have feature of cirrhosis which corelated with its clinical stigmata. . GI bleed: Usually the first presentation in Presinusoidal causes and is well tolerated as liver function is intact. Unlike in sinusoidal causes where there is significant mortality associated with it. Encephlopathy: it is predominantly seen in sinusoidal causes but can be seen in other two especially after GI bleed when it may be transient. USS: Variation of splenic and SMV diameter: normally increases but not in PHT Collaterals: gastro, spleno renal Thickness of omentum: ratio of omental thickness to diameter of aorta>1.7 =PHT Presinusoidal liver is normal and post sinusoidal it may be normal or coarse depending on duration and sinusoidal it is usually coarse. Portal vein : dilated in sinusoidal and post sinusoidal whereas in presinusoidal its not visualized with cavernomas.
- Clinical: UGI bleed+splenomegaly Endoscopy: is the most reliable method for detecting esophageal varices and for identifying the source of gastrointestinal bleeding USS /Dopplerâ: experienced ultrasonographer should be able to demonstrate the patency of the portal vein, and Doppler flow ultrasonography can demonstrate the direction of flow within the portal system. Reversal of flow.
- EMERGENCY: Correction of coagulopathy by administration of vitamin K and/or infusion of platelets or fresh-frozen plasma may be required An H 2 -receptor blocker or proton pump inhibitor should be given intravenously Care should be taken in fluid resuscitation of children after bleeding to avoid producing an excessively high venous pressure Pharmacologic therapy to decrease portal pressure: The somatostatin analog octreotide is more commonly used, and it decreases splanchnic blood flow with fewer side effects. Endoscopy: Without bleeding: Endoscopic can still be done to look for predictors of bleed With Bleeding: After an episode of variceal hemorrhage or in patients in whom bleeding cannot be controlled, endoscopic sclerosis or elastic band ligation of esophageal varices are important options Prophylaxis: Although bleeding can be controlled acutely in most cases, further sessions of sclerotherapy/banding are required to achieve temporary obliteration of the varices Beta blockers Surgery: With no bleeding: no surgical option is required in most patient with EHPVO (as they improve with time) If recurrent bleeding , thrombocytopenia: ( not always advocated if natural history of disease is self improving, vein too thin) Meso-rex bypass shunt :is ideal surgery: which connects the junction of the superior mesenteric and splenic veins to the left portal vein using an internal jugular jump graft.1,5 This procedure bypasses the obstruction and restores nutritive blood flow to the liver Splenorenal shunt: it decreases the portal flow by diverting the splenic vein flow to the vena cava
- Portal hypertensive biliopathy ; Patients with portal vein obstruction as a result of external compression of bile ducts by cavernous transformation of the portal vein can develop Cholestasis and Progressive liver disease hepatopulmonary syndrome, which develops in âĽ10% of patients with portal hypertension. It is defined as an arterial oxygenation defect induced by intrapulmonary microvascular dilation, resulting from release of a number of endogenous vasoactive molecules, including endothelin-1 and nitric oxide into the venous circulation. Liver transplantation is the only effective therapy Hepatorenal disease: Renal insufficiency in patient with liver failure in the absence of any other cause.
- In patients with intrahepatic disease, the combination of portal hypertension and poor liver synthetic ability (coagulopathy) can make bleeding much more difficult to control = poorer prognosis, ultimately require liver transplantation In patients with portal vein obstruction and normal hepatic function, the bleeding episodes becomes less frequent but may have intermittent bouts of life-threatening hemorrhage.