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Home , Ascites, Hematemesis, Melena, Splenomegaly

| Case Presentation | No. 07-2020 | A 12-year-old girl presenting with , and gradual

Mukesh Khadga, Md. Benzamin, Rubaiyat Alam, Nasreen Sultana and Md. Rukunuzzaman

Article Info Presentation of Case had , moderate underweight and stun- ting, huge , ultrasonography of Department of Pediatric Gastroenterol- Dr. Md. Benzamin (MD Resident): A 12-year-old the whole showed coarse hepatic ogy and Nutrition, Faculty of Pediatrics, nd Bangabandhu Sheikh Mujib Medical girl, 2 issue of non-consanguineous parents, echotexture and huge splenomegaly. So, we University, Shahbag, Dhaka, Bangladesh immunized as per EPI schedule, presented with differentially thought of chronic (MK, MB, RA, MR); National Institute of the history of hematemesis and melena 3 (compensated) with . Nuclear Medicine and Allied sciences, months back and abdominal distension for 1 Bangabandhu Sheikh Mujib Medical year. After the episodes of hematemesis and Chronic (compensated) with University, Shahbag, Dhaka, Bangladesh portal hypertension (NS) melena, she received 1 unit of trans- fusion. She had no history of fever, , Dr. Mukesh: The term abdominal trauma, umbilical catheterization, For Correspondence: implies a longstanding irreversible change in umbilical , severe , offending Mukesh Khadga the hepatic structure that may end in compli- [email protected] drug intake and abdominal trauma or any cations like , portal hypertension . leading to premature death.1 The main On general examination, the child was ill- etiologies of chronic liver disease are chronic Received: 25 January 2020 looking and severely anemic. The stigmata of viral (Hepatitis-B), Wilson’s Disease Accepted: 29 May 2020 and autoimmune liver disease. Patients of Available Online: 16 June 2020 chronic liver disease was absent. The vital signs were normal. She was moderately underweight chronic liver disease with PHTN commonly and moderately stunted. On abdominal exami- present with , , jaundice, pallor, ISSN: 2224-7750 (Online) nation, the abdomen was soft and distended variceal , splenomegaly, stigmata of 2074-2908 (Print) 2 over the left hypochondrium. There was a huge chronic liver disease. Compensated cirrhosis DOI: 10.3329/bsmmuj.v13i2.47604 splenomegaly (14 cm) (Figure 1A). The liver refers to the condition where hepatic synthetic was not palpable. Ascites was absent. Other functions are preserved despite the cirrhosis, in systemic examination revealed normal findings. contrast to decompensated cirrhosis, where patients suffer from progressive complications Investigations revealed severe anemia (hemo- Cite this article: of liver disease (fatigue, ascites, variceal bleed- Khadga M, Benzamin M, Alam R, Sultana globin 5.1 g/dL), ( ing, hepatic ) with associated N, Rukunuzzaman M. A 12-year-old girl count 35,000/mm3), leucopenia (total white hepatic dysfunction.3, 4 presenting with hematemesis, melena blood cells 1,500/mm3) (Table I). Peripheral and gradual abdominal distension. blood film showed microcytic hypochromic Dr. Benzamin: Serum alanine aminotransferase Bangabandhu Sheikh Mujib Med Univ J. (20 U/L), prothrombin time and albumin levels 2020; 13: 58-62. anemia with no features of . Prothrombin time (12 sec, INR- 1.02), activated (56 g/L) were normal. The HBsAg was partial thromboplastin time and serum albumin negative. The serum ceruloplasmin level was Copyright: level were normal (56 g/L). Ultrasonography of normal. The eye evaluation for the KF ring and The copyright of this article is retained sunflower cataract were negative. The total IgG by the author(s) [Atribution CC-By 4.0] the whole abdomen showed coarse hepatic echotexture and huge splenomegaly. level was normal and ANA was negative. So, chronic liver disease was excluded. Available at: www.banglajol.info Dr. Mukesh: Portal hypertension is a disease Provisional Diagnosis which occurs due to the formation of portal- A Journal of Bangabandhu Sheikh Mujib systemic collaterals, that shunts a portion of the Medical University, Dhaka, Bangladesh Portal hypertension (extra-hepatic cause) portal blood flow to the systemic circulation, bypassing the liver.5 Diagnosis is done by upper gastrointestinal .6 Differential Diagnosis Dr. Rubaiyat Alam (Assistant Professor): To reach Dr. Mukesh Khadga (Resident): As the patient our diagnosis we did the upper gastrointestinal presented with a history of gastrointestinal endoscopy which showed large varices in the bleeding (hematemesis, melena) with gradual lower part of . There are several abdominal distension and on examination she classifications for grading of esophageal BSMMU J 2020; 13: 58-62 59

Figure 1: Splenomegaly (A), Grade IV (B), and doppler ultrasonography (C) showing cavernous transformation of portal vein the development of extrahepatic portal hyper- Table I tension. Among them, the most common cause is extrahepatic portal vein obstruction.8 So, we did Laboratory investigations color doppler ultrasonography and it showed Investigations Results Reference cavernous transformation of portal vein with Hemoglobin (g/dL) 5.1 13-17 evidence of portal hypertension (Figure 1C). White blood cells (/mm3) 1,500 4,500-5,500 Nasreen Sultana (Professor): Extrahepatic portal vein Platelet (/mm3) 35,000 1,50,000- obstruction is a primary vascular condition 4,50,000 characterized by chronic long-standing blockage Peripheral blood film (PBF) Microcytic hypo- Microcytic hypo- and the cavernous transformation of the portal vein chromic anemia with chromic anemia with or without additional involvement of intra- no features of hemol- with no features of hepatic branches, splenic or superior mesenteric ysis. hemolysis. vein.9 It is the predominant cause of pediatric portal Serum albumin (g/dL) 5.6 3.5-5.5 hypertension in developing countries. It is a prehepatic type of portal hypertension in which Serum bilirubin (mg/dL) 1 0.2-1.2 liver functions and morphology are preserved to Prothrombin time (sec) 12 12-16 date. International normalized ratio 1.02 <1.4 Doppler ultrasound is an accurate non-invasive Serum alanine aminotransferase 20 35-50 method for evaluation of portal hemodynamics that (U/L) provides valuable information on morphological, Total IgG (g/L) Normal 0-16 qualitative and quantitative changes.10 ANA Negative Negative In the normal portal vein, spectral doppler Serum ceruloplasmin (mg/dL) 35 >20 shows antegrade or hepatopetal flow Eye evaluation for K-F ring, Absent Absent and a waveform above the baseline. Abnormally sunflower cataract slow portal venous flow is diagnostic when peak HBsAg Negative Negative velocity is less than 16 cm/sec. In some cases, the flow is still hepatopetal but spectral analysis may demonstrate loss of respiratory phasicity and more varices, like- Dagradi classification, Conn’s classifi- pronounced cardiac periodicity which can progress cation, Paquet’s classification, Westaby classifi- to an absence of end-diastolic flow, arterialized flow cation, Soehendra classification, Cale’s or bidirectional to-and-fro flow. Slow or reverse classification. Paquet’s classification for grading of (hepatofugal) flow are two of the most specific esophageal varices is as follows, Grade I: findings. Absent flow in the portal vein may be due microcapillaries located in the distal esophagus or to stagnant flow in portal hypertension. Recanaliza- esophago-gastric junction, Grade II: one or two tion occurs in some cases of but more small varices located in the distal esophagus, Grade frequently if persists, portal III: medium-sized varices of any number and Grade flow is reestablished via cavernous transformation IV: large-sized varices in any part of the esophagus.7 (portal vein undergoes fibrosis and a tangle of So, according to Paquet’s classification, our patient tortuous collateral veins are seen along the usual had Grade IV varices (Figure 1B). course of the portal vein). The dilated superior Dr. Benzamin: Several etiologies are responsible for mesenteric vein and splenic vein may be seen. In 60 BSMMU J 2020; 13: 58-62

some cases, reversed flow may be detected. The endoscopy.15-17 most specific finding is the development of porto- Dr. Nazmul Hassan (Resident): What is the systemic collaterals. On doppler, collateral vessels differentiating point between EHPHTN and chronic demonstrate continuous flow similar to that of the liver disease with portal hypertension? portal vein. The most common sites are paraumbili- cal vein, gastro-esophageal or short gastric, spleno- Dr. Khadga: EHPHTN is usually present on early renal or gastro-renal veins, inferior mesenteric age with a repeated history of hematemesis and veins.11 melena, without deteriorating general health condition and absence of stigmata of chronic liver The use of the congestive index has been disease.6 recommended in helping to diagnose. It is the ratio of the portal vein cross-sectional area divided by the Dr. A. Z. M. Raihanur Rahman: What investigation is mean portal flow velocity. In the normal subject, done to diagnose portal hypertension, where endos- this ratio is less than 0.7.12 copy is not available? Dr. Benzamin: Barium swallow is done. It will show a worm-like filling defect in esophagus.18 Dr. Benzamin’s Diagnosis Dr. Nazmul Ahamed (Resident): What is the impor- Portal hypertension due to extrahepatic portal vein tance of consanguinity in EHPTH? obstruction Dr. Khadga: Protein C, protein S deficiency is auto- The child was treated with endoscopic variceal somal recessive disease and also one of etiology of ligation, followed by Tab. extrahepatic portal vein obstruction.19 1 mg/kg/day in three divided doses. Dr. Maimuna Sayeed (Resident): What are the predic- tors of variceal bleeding? Discussion Md. Rukunuzzaman (Professor): Predictors of variceal bleeding include 1. Portal vein-hepatic vein gra- Dr. Alam (Assistant Professor): Portal hypertension is dient >12 mmHg, 2. Large, tense varices, red wale defined as portal vein pressure >5 mmHg or a marks, 3. Red spots on varices, 4. Severity of portal vein to hepatic vein gradient of >10 mmHg.13 underlying liver disease, 5. Presence of gastric 14 is helpful in diagnosis. varices and 6. Extrahepatic portal vein obstruction. Splenomegaly is common and sometimes massively Dr. Aysha Siddiqua (Resident): What are the prophy- enlarged. In extrahepatic portal hypertension no lactic therapy given to prevent variceal bleeding? . In cirrhosis patient, the liver is usually small and shrunken, but children with Dr. Khadga: Beta-blocker (propranolol), endoscopic , the liver may be moderately therapy.6 enlarged. In Budd–Chiari syndrome or congenital Dr. Sharmin Akter (Resident): What is primary hepatic fibrosis, the liver may be massively prophylaxis and what is secondary prophylaxis? enlarged. Ascites is generally only present when portal hypertension is at the sinusoidal level.14 Dr. Benzamin: Prophylactic therapy given before the 1st episode of hematemesis and melena is known as The four main portals to systemic vein collateral primary prophylaxis and after the episode of systems that become prominent in portal hematemesis and melena is known as secondary hypertension. Varices developed due to increased prophylaxis.6 pressure in the portal to esophageal and gastric collateral system. Caput medusa result from Dr. Ferdous Ara Begum (Resident): How propranolol Increased pressure in the paraumbilical venous acts? network. With overfilling of the perirectal collateral Dr. Rukunuzzaman: Propranolol is a non-selective β venous system, appear.14 blocker. It primarily blocks β2-adrenoceptors of the Investigations modalities include endoscopy and splanchnic bed, leaving unopposed α-adrenoceptor duplex sonography. The along stimulation and thus decreased splanchnic and with some common etiology (chronic , portal perfusion. It also decreases heart rate by β1- Wilson disease, , etc.) are adrenoreceptor blockade, thus lowering cardiac essential to exclude the chronic liver disease. output and portal perfusion. It also decreases colla- Esophageal and are best examined teral circulation (e.g. azygous vein blood flow).6 by endoscopy. Magnetic resonance angiography is Dr. Sharmistha Ghosal (Resident): What are the endos- newer, less invasive radiologic techniques. Intra- copic therapyies available for portal hypertension? venous computed tomography using a helical CT scanner can be identified as changes in the Dr. Benzamin: Sclerotherapy and ligation therapy.6 esophageal and gastric vasculature earlier than BSMMU J 2020; 13: 58-62 61

Dr. Aysha Sabiha (Resident): What is the emergency up with the child? management for variceal bleeding? Dr. Khadga: The follow-up endoscopy is done Dr. Khadga: Firstly, management includes mainte- frequently until small varices are seen. When nance of patients’ airway, breathing and circulation, varices become small, then endoscopy should be including fluid resuscitation with crystalloids or red done every 1-2 yearly.23 blood cell transfusion cautiously. Simultaneously, Dr. Nayma Rahman (Resident): What is the prognosis starting of inj. terlipressin, vasopressin, or soma- of extrahepatic portal vein obstruction? tostatin (octreotide) is equally important. A naso- gastric tube is essential and blood/ blood product Dr. Benzamin: Regular endoscopic and medical transfusion if indicated. After stabilization endos- treatment ensure a good long term prognosis.24 copic therapy and propranolol to prevent further bleeding. If needed, surgical management is the choice.6 Final Diagnosis Dr. Bodhrun Nahar (Resident): Why nasogastric tube is given on acute variceal bleeding? Portal hypertension due to extrahepatic portal vein obstruction Dr. Benzamin: A nasogastric tube is given to see any active bleeding present or not and removal of blood (a protein source that may precipitate encephalo- pathy). Moreover, blood in the increases Conflict of Interest splanchnic blood flow and potentially could worsen Authors declare no conflict of interest portal hypertension and ongoing hemorrhage.6 Dr. Kaniz Fathema (Resident): What are the indica- tions of blood/blood product transfusion in acute References variceal bleeding management? 1. Dhole SD, Kher AS, Ghildiyal RG, Tambse MP. Dr. Khadga: Packed red blood cells is transfused if Chronic liver in children: Clinical profile the hemogobin level is less than 8 mg/dL, platelet and histology. J Clin Diagn Res. 2015; 9: 4-7. transfusion if <50,000/mm3. Fresh frozen plasma 2. Hanif M, Raza J, Qureshi H, Issani Z. Etiology of 6 transfusion if prothrombin time is prolonged. chronic liver disease in children. J Pakistan Med Dr. Khondokar Mobbasher Ahmed (Resident): How is Assoc. 2004; 54: 119-22. inj. octreotide given and when to stop? 3. Hsu EK, Murray KF. Cirrhosis and chronic liver Dr. Benzamin: Inj. octreotide is given 1-2 µg/kg stat failure. In: Liver disease in children. Suchy FJ, Sokol RJ, Balistreri WF (eds). 4th ed. Cambridge, at a bolus dose followed by 2-5 µg/kg/hour Cambridge University Press. 2014, pp 51-67. maintenance dose to continue the infusion for at least 5 days in patients at risk of rebleeding.20, 21 4. Benzamin M, Islam R, Subha NE, Begum F. A 12- year old boy presented with jaundice, abdominal Dr. Saidul Sumon (Resident): What are surgical distension and leg edema. Bangabandhu Sheikh management options for EHPHTN? Mujib Med Univ J. 2020; 13: 22-26. Dr. Khadga: The portosystemic procedures 5. Bosch J, Abraldes JG, Groszmann R. Current include mesocaval shunt, portocaval shunt, spleno- management of portal hypertension. J Hepatol. renal shunt, meso Rex bypass and splenectomy6 2003; 38: S54–68. Dr. Mahmudul Hasan (Resident): Is there any chance 6. Shneider BL. Portal hypertension. In: Liver disease of liver function impairment in EHPHTN? in children. Suchy FJ, Sokol RJ, Balistreri WF (eds). 4th ed. Cambridge, Cambridge University Press, Dr. Benzamin: Liver function may be impaired if 2014, pp 68-87. there is the development of portal biliopathy.8 7. Philips CA, Sahney A. Oesophageal and gastric Dr. Rafiqul Islam (Resident): What advice was given varices: Historical aspects, classification and gra- to the patient during discharge? ding: Everything in one place. Gastroenterol Rep. 2016; 4: 186-95. Dr. Benzamin: Fever, dehydration, and cough should be treated on a priority basis. 8. Khadga M, Benzamin M, Karim AB. Extrahepatic Propranolol should continue and if there is portal venous obstruction in children: Approach gastrointestinal bleeding occur. Propranolol should and management. Mediscope 2019; 6: 79-85. be withheld and hospitalized for emergency 9. Sarin SK, Khanna R. Non-cirrhotic portal hyper- management.22 tension. Clin Liver Dis. 2014; 18: 451-476. Dr. Naznin Sarkar (Resident): How will you follow- 10. Pargewar SS, Desai SN, Rajesh S, Singh VP, Arora 62 BSMMU J 2020; 13: 58-62

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