Congenital Internal Hernia Presented with Life Threatening Extensive Small Bowel Strangulation
A 6-year-old boy visited pediatric emergency department due to sudden onset abdominal pain which developed 1 hour ago. He had complained diffuse abdominal pain and vomited clear fluid twice in the hospital. Vital sign was stable and the physical examination revealed dehydrated tongue and diffuse abdominal tenderness. Laboratory test showed unremarkable except elevated white blood cell count of 24,310/mm3 with normal differentiation. Plain abdominal radiograph showed nonspecific small bowel gas which suggested mild ileus () and abdominal ultrasound revealed diffuse small bowel wall thickening with ascites (). A contrast-enhanced abdominal computed tomography (CT) was recommended, but his father refused to undergo the further evaluation and discharged against our medical advice.\nFifteen hours later, he was rushed by ambulance to the pediatric emergency department again with altered mentality. He did not respond to the painful stimuli, his both pupils were dilated to 5mm and showed severe abdominal distension. His blood pressure was uncheckable with weakly palpable femoral pulse, percutaneous oxygen saturation was 77% and blood sugar level was 22 mg/dL. Pretibial intraosseous cannulation was performed urgently due to failure of the intravenous catheterization and fluid and medications were infused through the intraosseous cannulation. He was in shock state and arterial blood gas analysis showed pH of 7.15 and bicarbonate of 7.7 mM. Hemoglobin and hematocrit levels were 10.1 g/dL and 27.7%, but rapidly decreased to 4.7 g/dL and 13% respectively, just one hour later. Also he was in disseminated intravascular coagulopaty state with antithrombin III of 38.9%, D-dimer of 9.18 mg/L, prothrombin time of 1.61 international normalized ratio, activated partial thromboplastin time of 43.9 seconds and lactic acid of 10.9 mmol/L. After the initial hydration, central venous catheter could be placed via the left subclavian vein and massive fluid, transfusions, and inotropics could be delivered. The contrast-enhanced abdominal CT scan performed immediately after maintaining systolic blood pressure of 90 mmHg. CT scan showed large amount of ascites, bowel wall thickening with poor or absent enhancement of the strangulated bowel segment. The serrated beak sign and whirl sign of the obstructive bowel loop were identified at the transition zone which might indicate closed loop obstruction (). Surgical exploration was performed immediately. On the operation field, three quarters of the small bowel was herniated through the two fingerbreadth defect of mesentery located on near the ileocecal valve (). All herniated small bowel showed diffusely hemorrhagic, edematous and inflammatory change. Under the diagnosis of congenital IH with strangulated small bowel, resection of more than 2 meters of gangrenous small bowel was followed by the anastomosis of proximal jejunum and terminal ileum.\nAfter four-day treatment at pediatric intensive care unit, he was improved enough to move to general ward and started soft diet on the 5th day after operation. He did not fully recover yet due to hypertension after renal ischemic injury and short bowel syndrome, fortunately, his cognitive function is normal and he is gradually improving conditions.