| Case Presentation | No. 07-2020 | A 12-year-old girl presenting with hematemesis, melena and gradual abdominal distension Mukesh Khadga, Md. Benzamin, Rubaiyat Alam, Nasreen Sultana and Md. Rukunuzzaman Article Info Presentation of Case had anemia, moderate underweight and stun- ting, huge splenomegaly, ultrasonography of Department of Pediatric Gastroenterol- Dr. Md. Benzamin (MD Resident): A 12-year-old the whole abdomen 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 liver disease (MK, MB, RA, MR); National Institute of the history of hematemesis and melena 3 (compensated) with portal hypertension. Nuclear Medicine and Allied sciences, months back and abdominal distension for 1 Bangabandhu Sheikh Mujib Medical year. After the episodes of hematemesis and Chronic liver disease (compensated) with University, Shahbag, Dhaka, Bangladesh portal hypertension (NS) melena, she received 1 unit of blood trans- fusion. She had no history of fever, jaundice, Dr. Mukesh: The term chronic liver disease abdominal trauma, umbilical catheterization, For Correspondence: implies a longstanding irreversible change in umbilical sepsis, severe dehydration, offending Mukesh Khadga the hepatic structure that may end in compli- [email protected] drug intake and abdominal trauma or any cations like cirrhosis, portal hypertension surgery. 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 (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 edema, ascites, jaundice, pallor, ISSN: 2224-7750 (Online) nation, the abdomen was soft and distended variceal bleeding, 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), thrombocytopenia (platelet ing, hepatic encephalopathy) 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 hemolysis. 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 endoscopy.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 esophagus. 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 esophageal varices (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 ultrasound 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 thrombosis but more small varices located in the distal esophagus, Grade frequently if portal vein thrombosis 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