Introduction:

Esophageal varices can be a source of life-threatening massive upper gastrointestinal hemorrhage. After gastric and duodenal ulcers, they are the third most frequent cause of upper GI bleeding. The mortality rate of acute episodes ranges from 5-19% in children which further increases in the case of rebleeding. In almost 60% of cases, recurrent variceal hemorrhage is reported. Esophageal varices are dilated submucosal distal esophageal veins which develop as a consequence of portal hypertension. The development of varices is caused by increased resistance to portal blood flow because of prehepatic, intrahepatic, and suprahepatic obstructions.

Because of higher incidence of prehepatic portal hypertension in children, a better overall survival rate and quality of life is seen in comparison to adults. But serious morbidities associated with varicealhaemorrhage impact mortality and acquire attention for consideration of prophylactic or therapeutic interventions for children at risk. Herein we report an emergent case of esophageal varices rupture in a 6 year old boy admitted in our pediatric department.

Case Presentation:

A 6-years-old boy was admitted with a complaint of coffee-ground vomiting and abdominal pain with black tarry stool. During the first hours of hospitalization, the patient had a second episode of coffee-ground vomiting and each time passed stool black color. There was no history of umbilical venous catheterization or umbilical diseases during the neonatal period. There was no indication of an immune deficiency disease, and he was allergic to amoxicillin and clavulanic acid.

The patient was alert and eupneic. He was hemodynamically stable (heart rate 120 beats per minute, blood pressure 110/73 mmHg with respiratory rate of 25 breaths per minute. The patient weighed 20 kg at the 50th percentile and measured 115 cm tall at the 50th percentile for stature. Apart from mild abdominal distension, other clinical findings were not significant.

Laboratory investigations indicated anemia withhemoglobin concentration of 7g/dL. Hematocrit was 18%, WBC count was 12.3 103 uL/L, RBCS count was 2.54 mil uL/L, and platelet count was 171 uL/L. Stool test was positive for blood. EBV PCR was negative, but both EBV IgG nuclear and capsid were positive. The serum levels of Alfa 1 antitrypsin were high( 2.2 g/l )(reference range: 1-2 g/l). Blood smear showed normocytic normochromic anemia with no abnormal cells. Other investigations including CRP, liver function tests, renal function tests serum electrolytes, calcium magnesium phosphorus D dimmer, blood glucose level, coagulation profile, serum ammonia serum amylase and serum organic acids, G6pd, comb test, hemoglobin electrophoresis, calprotectin, TTG, SMA screen, LKM, Upper respiratory panel, MRSA, CMV, ANA, thrombotic screen were within normal range.

Case Presentation:

Chest x ray showed left pleural effusion. Ultrasound of chest revealed small size left basilar echo lucent and non septated pleural effusion in association with collapse of the adjacent left lower lobe. Ultrasound of abdomen showed cavernous transformation of portal vein with splenomegaly. CT scan of abdomen with contrast was done which showed tortuous appearance of extrahepatic portion of portal vein and multiple small tortuous veins consistent with cavernous transformation in intrahepatic portion. Splenomegaly ( size 13.7 cm ) with multiple small tortuous veins was seen in the splenic hilum. Multiple tiny tortuous veins along the distal esophagus was present suggesting esophageal varices. Upper gastrointestinal endoscopy revealed esophageal varices grade II in the lower third of the esophagus, as well as dilated congested veins proximal to the varices and hyperemic stomach mucosa with visible erosions and bleeding stigmata in the fundus area.

Based on above findings, final diagnosis of esophageal varices was given. During the hospitalization, blood transfusions were administered twice. Glucose and electrolytes was administered in order to compensate for fluid loss. The patient was treated with spironolactone, propranolol, amoxiclave, esomeprazole, and furosemide. After treatment, there were no further gastrointestinal symptoms. Hemoglobin values returned to normal, indicating resolution of gastrointestinal bleeding and the report of the endoscopy performed at the end of the treatment period confirmed the non-bleeding esophageal varices.

Discussion:

The formation of collateral vessels is well-described in patients with portal hypertension. Various studies reported that 70% of children with biliary atresia or portal vein thrombosis have esophageal varices, and bleeding occurs in 85% of children with portal hypertension over the age of 5 to 10 years. Esophagealvarices mostly bleed suddenly and unexpectedly, presenting as severe hematemesis. We presented a case of an 6 year old boy with hematemesis caused by ruptured esophagealvarices due to portal hypertension. The provisional diagnosis was based on medical history, clinical examination, laboratory and radiology findings and was finally confirmed by endoscopy.

There was no history of umbilical venous catheterization or umbilical diseases during the neonatal period in our patient. However, Rahman et al. (2000), Rogvi et al. (2016), and Ugwu et al. (2020) reported cases of upper GI bleeding after umbilical catheterization in children aged 2.6, 7, and 9, respectively. Risk factors for the development of portal venous thrombosis with the placement of an umbilical venous catheter include lack of use of an anticoagulant, malposition of
the catheter tip, and prolongation of the catheterization period.

Poddar U et al. (2007) determined extrahepatic portal venous obstruction and cirrhosis to be major causes of portal hypertension in children, with extrahepatic portal venous obstruction being the predominant cause of variceal bleeding. The liver function test in our patient was normal. However, serum levels of Alfa 1 antitrypsin were elevated and EBV IgG nuclear and capsid were positive. The ultrasound and CT scan with contrast of abdomen showed splenomegaly and tortuous
appearance of extrahepatic portion of portal vein and multiple small tortuous veins consistent with cavernous transformation in intrahepatic portion suggesting portal hypertension.

Upper gastrointestinal endoscopy is the gold standard for detection of esophageal varices which can be can be undertaken either before (primary) or after (secondary) an initial variceal bleed. Grade I esophageal varices are small and flatten with insufflation; grade II varices arenodular, moderately enlarged; grade III varices are markedly enlarged and are not flattened by insufflation. Our patient had grade II esophageal varices in the lower third of the esophagus.

The initial treatment of variceal hemorrhage is to stabilize the patient by effective fluid resuscitation including blood transfusion and controlling of bleeding. According to Kravetz D et al, patients with variceal bleeding should be transfused conservatively to a hematocrit of only 27% to avoid exacerbating bleeding by increasing portal pressure. We transfused the patient with two units of fresh whole blood because the haemoglobin value was less than 7 g/dl. According to the Baveno V consensus, there are two main strategies for the primary management of medium or large varices, which include either the administration of non-selective beta-blockers or repeated sessions of endoscopic variceal ligation until variceal eradication. The definitive treatments include endoscopic procedure (sclerotherapy or band ligation) and surgical therapies (portosystemic shunts). Our patient was successfully treated with spironolactone, propranolol, amoxiclave, esomeprazole, and furosemide. Hemoglobin returned to normal level after the treatment, indicating resolution of gastrointestinal bleeding.

Conclusion:

We highlighted the importance of early detection and management of esophageal varices, a common complication of portal hypertension.In children with portal hypertension, splenomegaly, thrombocytopenia, or liver disease, it is important to take into account the possibility of esophageal varices as it is associated with significant morbidity and mortality.