Surgical Treatment of Kawasaki Disease with Intestinal Pseudo-obstruction

Abstract

A 5-year-old boy suffering from abdominal pain accompanied by a fever of up to 39.5°C for 2 days was admitted to the hospital. Although Flomoxef was administered following admission, the boy's fever persisted and abdominal distension gradually worsened. On the 4th day, dry lips, red eyes and a strawberry tongue were noted. An echocardiogram revealed pericoronary enhancement with mild mitral valve regurgitation and a small degree of pericardial effusion, characteristics compatible with Kawasaki disease. Although intravenous immunoglobulin was administered, the fever and abdominal distension persisted. On the 8th day, a pediatric surgeon was consulted and an exploratory laparotomy was arranged. During the operation, intestinal pseudo-obstruction and fibrin coatings around the intestine near the splenic flexure were found. A colostomy was performed for decompression of the dilated bowel and a biopsy of the lymph node surrounding the splenic flexure was taken. The fever subsided dramatically after decompression of the bowel and the recovery course was uneventful. The pathologic report revealed necrotic lymphadenitis. We report this rare case and review the literature.

Intestinal Involvement in Kawasaki Disease

“…Plain radiograph, ultrasonography, and computed tomography (CT) showed pseudoobstruction as the most frequent sign of gastrointestinal involvement (multiple dilated intestinal loops with multiple fluid and gas levels and/or concentric thickening of the intestinal wall) in 38 cases (77%). [2][3][4][5][6]8,9,[12][13][14][15][16][17][19][20][21][22][23][27][28][29][30][31][32][33] However, pseudoobstruction was accompanied by ischemic colitis in 1 case (2%), 4 hydrops of the gallbladder in 1 case (2%), 6 mesenteric and splenic ischemia 28 in 1 case (2%), hemorrhagic duodenitis 15 in 2 cases (4%), and appendicular involvement in 10 cases (20%). 15,21,25,27,28 Only 1 case showed mechanical obstruction with intussusception, 24 and 1 patient whose radiograph showed presence of free subdiaphragmatic intraperitoneal air developed a perforated ulcer in the descending duodenum.…”

“…15,21,25,27,28 Only 1 case showed mechanical obstruction with intussusception, 24 and 1 patient whose radiograph showed presence of free subdiaphragmatic intraperitoneal air developed a perforated ulcer in the descending duodenum. 29 Regarding surgical procedures, 25 (51%) patients underwent surgery, 5 (10%) underwent exploratory laparotomy, 2,8,15,29,30 8 (16%) required resection of the restricted loop and temporary colostomy, 3,6,9,12,14,23,28,32 and 1 (2%) required enterolysis. 4 Endoscopy for hematemesis was performed in 2 cases (4%).…”

“…15,29 Cardiac involvement was documented in 21 (43%) of the cases considered. [3][4][5][6]9,[12][13][14][15][16][20][21][22][23]27,28,31,32 All patients except 3 2,3 received medical treatment: 12 patients (25%) were administered immunoglobulin intravenous (IGIV) alone, and 18 patients (37%) were administered IGIV with aspirin. 2,5,13,16,17,[20][21][22]24,33 The outcome was good in 28 patients (57%, including the 3 patients who did not receive medical treatment).…”

“…2,5,13,16,17,[20][21][22]24,33 The outcome was good in 28 patients (57%, including the 3 patients who did not receive medical treatment). 4,6,12,17,19,[21][22][23][24]27,[29][30][31][32][33][34] Seven patients (14%) showed persistence of CAAs, 5,15,16,20,21 and 1 (2%) patient developed cyanosis, and later, left hand and forefoot gangrene. 3 Three patients (6%) died: in 1 case autopsy revealed massive liver necrosis and progressive duodenal infarction with systemic arteritis and periarteritis involving vessels of all sizes 3 ; the second patient died 2 days after the exploratory laparotomy 2 ; the third patient died because of Pseudomonas septic shock.…”