Journal
of Pediatric
Surgery
VOL 25, NO 11
NOVEMBER
Surgical Complications of the Hemolytic-Uremic By Mary
L. Brandt,
Sean
O’Regan,
Elisabeth
Montreal, 9 Hemolytic-uremic syndrome (HUS) of childhood is a triad of acute hemolytic anemia, thrombocytopenia, and acute renal failure associated with a gastrointestinal prodrome. From 1977 to 1988, 134 patients with HUS were admitted to this institution. All patients presented with abdominal pain and diarrhea, which was virtually always bloody. Seventy-eight patients (80%) required dialysis. Five patients died (4%). One patient died as a result of colon perforation, the other four patients died of other nonsurgical complications of HUS. Three patients underwent exploratory laparotomy. One patient had a hemoperitoneum from mesenteric and transmural bleeding of the entire intraabdominal colon. Another patient had undergone surgery elsewhere for presumed intussusception with pancolitis found at exploration. .Fourteen days postoperatively, he had a spontaneous perforation of the transverse colon. The third patient presented with pancolitis and perforation of the transverse colon. Despite surgical intervention he died on the sixth postoperative day. One other patient was treated conservatively for pancreatitis, which developed 3 weeks after her presentation with HUS. Complications requiring surgical intervention in HUS are rare, potentially lethal, and usually involve the colon. 0 1990 by W.B. Saunders Company. INDEX ration:
WORDS: pancreatitis.
Hemolytic-uremic
syndrome;
bowel
perfo-
H
EMOLYTIC-UREMIC syndrome (HUS) consists of the triad of thrombocytopenia, hemolytic anemia, and acute nephropathy almost invariably preceded by a prodrome of gastrointestinal or upper respiratory infection. HUS is the most frequent cause of renal failure in children. Several excellent reviews of the hemolytic uremic syndrome have been published recently.le6 Although noninfectious etiologies are known to cause HUS in the adult,7 most cases in children are caused by infection. 8*9Although many infectious agents associated with HUS have been identified, the most common are the verotoxic strains of Escherichia coli, especially E coli 0157:H7.7~10-‘3 Patients with HUS may be seen initially by a surgical service, because they often present with severe abdominal pain and tenderness mimicking a surgical abdomen.‘4*‘5
Journal
of Pediatric
Surgery,
Vol 25,
No
11 (November).
1990:
pp
1109-l
Rousseau,
and
Salam
1990
Syndrome Yazbeck
Quebec
Complications of HUS are usually medical, and include encephalopathy, pancreatitis, cardiomyopathy, hypertension, and seizures.7p’6 Surgical complications of HUS are rare, and have been generally reported as individual cases of colonic necrosis with or without perforation’53’7-19 or colonic stenosis.*“*’ MATERIALS
AND
METHODS
The recordsof 134 patients with during the periodfrom 1977to 1988
HUS admitted to this hospital were reviewed.There were 75 males (56%) and 59 females (44%). The average age was 3 years 4 months (range, 6 months to 19 years). Seventy-eight patients required dialysis (60%). Five patients died (4% mortality). No surgical complications resulting from dialysis or other iatrogenic injuries were considered for this report. Three patients developed complications requiring surgical intervention, and a fourth patient developed pancreatitis as the result of HUS.
Case I-Hemoperitoneum Pancolitis
Secondary to Transmural
A 2-year 4-month-old boy was transferred from another hospital with a 3-day history of bloody diarrhea and fever to 38.6OC. His physical examination was remarkable for diffuse abdominal tenderness with guarding and grossly bloody diarrhea. Because of anuria a catheter was placed for peritoneal dialysis. Initial laboratory values included a hematocrit of 34.9%. white blood cell count (WBC) count of 31,500, a platelet count of 70,000, and a blood urea nitrogen (BUN) of 9 mg/dL (3 mmol/L). On the second hospital day his abdominal examination worsened, with increased tenderness and guarding. His WBC increased to 47,500, his BUN increased to 30 mg/dL (I 1.5 mmol/L), and his platelet count decreased to 30,000. However, the effluent from the Tenckhoff was clear. On the third hospital day, his abdominal examination continued to worsen and the drainage from the Tenckhoff catheter became bloody. At
From the Departments of Surgery and Pediatrics, Hopital Ste-Justine. Montreal, Quebec. Presented at the 21st Annual Meeting of the Canadian Association of Paediatric Surgeons, Edmonton, Alberta, September 20-23, 1989. Address reprint requests to Salam Yarbeck. MD, Department de Chirurgie, Hopital Ste Justine, 3175 Cote Ste Catherine, Montreal, Quebec H3S I C3, Canada. 0 1990 by W.B. Saunders Company. 0022-3468/90/251 l-0001 $03.00/O
112
1109
BRANDT
1110
surgical exploration he was found to have 200 mL of free blood in the peritoneal cavity with diffuse oozing from the entire surface of the colon, most markedly from the splenic flexure. There was also a hematoma noted in the mesentery of the colon. There were no perforations or other sites of bleeding found. Cultures of stool and peritoneal fluid were positive for E co/i 06:Hl and E coli 075:NM. His postoperative course was complicated by a right pleural effusion, which resolved spontaneously, and persistent renal failure, requiring peritoneal dialysis for I week postoperatively. He required a total transfusion of 520 mL of packed red blood cells (PRBCs) (42 mL/kg). He recovered and was discharged on postoperative day 12.
Case 2-Colonic
Perforation
A 4-year 6-month-old boy was transferred after evaluation and treatment at another hospital for bloody diarrhea and abdominal pain. The diagnosis of intussusception was made without a barium enema. At laparotomy his colon was found to be diffusely inflamed with no intussusception or other pathology found. Postoperatively he was noted to be anuric and was treated with multiple fluid boluses. At the time of transfer to this institution, on the third postoperative day, he weighed 27 kg, a gain of 6.3 kg over his usual weight, and had anasarca and pulmonary edema. His hematocrit was 23%. WBC count was 34,100, platelet count was 57,000, and BUN was 50 mg/dL (20 mmol/L). A peritoneal catheter was placed for emergency dialysis. The effluent was clear. Despite an overall improvement, he was never able to tolerate a diet because of nausea, vomiting, and mild abdominal pain, and required intravenous hyperalimentation for nutritional support. Plain abdominal radiographs were unremarkable. Serum amylase and WBC count were within normal limits. Esophagogastroduodenoscopy was performed on the 1 I th postoperative day, and showed a mild esophagitis and gastritis. On the 14th postoperative day he had a sudden onset of abdominal pain with tachycardia. The Tenckhoff effluent became cloudy with multiple organisms on Gram stain, an amylase of 1,200 U/L, and 7,000 WBC/mL. At laparotomy he was found to have a spontaneous perforation of the right transverse colon. The remainder of the colon and the pancreas appeared normal. A segmental resection, colostomy and mucous fistula were performed. Upon opening the specimen there was an area of totally denuded mucosa measuring 6 x 4 cm surrounding the perforation. Histological examination showed acute and chronic inflammation. Cultures of the peritoneal fluid were positive for E coli 0157:H7. His postoperative course was uncomplicated with adequate recovery of his renal function and closure of the colostomy 1 year later.
Case 3-Colonic
ET AL
botic microangiopathy of the small vesselsof the terminal ileum with areas of necrosis present, and acinar dilitation of the pancreas.
Case I-Pancreatitis A 2-year-old girl was transferred after admission elsewhere for dehydration and bloody diarrhea. On arrival her hematocrit was 28%, WBC count was 15,700, platelet count was 44,000, and BUN was 104 mg/dL (37 mmol/L). She underwent placement of a Tenckhoff catheter for emergency dialysis. Physical examination showed no abdominal abnormalities. Her hospital course was complicated by encephalopathy, presumed secondary to microangiopathy and the development, on the 20th hospital day, of vomiting and epigastic pain. Serum amylase was 780 U/L (normal ~200 U/L). Two days later her amylase was 579 U/L and and serum lipase was 1,048 U/L (normal
of Pediatric
Surgery
VOL 25, NO 11
NOVEMBER
Surgical Complications of the Hemolytic-Uremic By Mary
L. Brandt,
Sean
O’Regan,
Elisabeth
Montreal, 9 Hemolytic-uremic syndrome (HUS) of childhood is a triad of acute hemolytic anemia, thrombocytopenia, and acute renal failure associated with a gastrointestinal prodrome. From 1977 to 1988, 134 patients with HUS were admitted to this institution. All patients presented with abdominal pain and diarrhea, which was virtually always bloody. Seventy-eight patients (80%) required dialysis. Five patients died (4%). One patient died as a result of colon perforation, the other four patients died of other nonsurgical complications of HUS. Three patients underwent exploratory laparotomy. One patient had a hemoperitoneum from mesenteric and transmural bleeding of the entire intraabdominal colon. Another patient had undergone surgery elsewhere for presumed intussusception with pancolitis found at exploration. .Fourteen days postoperatively, he had a spontaneous perforation of the transverse colon. The third patient presented with pancolitis and perforation of the transverse colon. Despite surgical intervention he died on the sixth postoperative day. One other patient was treated conservatively for pancreatitis, which developed 3 weeks after her presentation with HUS. Complications requiring surgical intervention in HUS are rare, potentially lethal, and usually involve the colon. 0 1990 by W.B. Saunders Company. INDEX ration:
WORDS: pancreatitis.
Hemolytic-uremic
syndrome;
bowel
perfo-
H
EMOLYTIC-UREMIC syndrome (HUS) consists of the triad of thrombocytopenia, hemolytic anemia, and acute nephropathy almost invariably preceded by a prodrome of gastrointestinal or upper respiratory infection. HUS is the most frequent cause of renal failure in children. Several excellent reviews of the hemolytic uremic syndrome have been published recently.le6 Although noninfectious etiologies are known to cause HUS in the adult,7 most cases in children are caused by infection. 8*9Although many infectious agents associated with HUS have been identified, the most common are the verotoxic strains of Escherichia coli, especially E coli 0157:H7.7~10-‘3 Patients with HUS may be seen initially by a surgical service, because they often present with severe abdominal pain and tenderness mimicking a surgical abdomen.‘4*‘5
Journal
of Pediatric
Surgery,
Vol 25,
No
11 (November).
1990:
pp
1109-l
Rousseau,
and
Salam
1990
Syndrome Yazbeck
Quebec
Complications of HUS are usually medical, and include encephalopathy, pancreatitis, cardiomyopathy, hypertension, and seizures.7p’6 Surgical complications of HUS are rare, and have been generally reported as individual cases of colonic necrosis with or without perforation’53’7-19 or colonic stenosis.*“*’ MATERIALS
AND
METHODS
The recordsof 134 patients with during the periodfrom 1977to 1988
HUS admitted to this hospital were reviewed.There were 75 males (56%) and 59 females (44%). The average age was 3 years 4 months (range, 6 months to 19 years). Seventy-eight patients required dialysis (60%). Five patients died (4% mortality). No surgical complications resulting from dialysis or other iatrogenic injuries were considered for this report. Three patients developed complications requiring surgical intervention, and a fourth patient developed pancreatitis as the result of HUS.
Case I-Hemoperitoneum Pancolitis
Secondary to Transmural
A 2-year 4-month-old boy was transferred from another hospital with a 3-day history of bloody diarrhea and fever to 38.6OC. His physical examination was remarkable for diffuse abdominal tenderness with guarding and grossly bloody diarrhea. Because of anuria a catheter was placed for peritoneal dialysis. Initial laboratory values included a hematocrit of 34.9%. white blood cell count (WBC) count of 31,500, a platelet count of 70,000, and a blood urea nitrogen (BUN) of 9 mg/dL (3 mmol/L). On the second hospital day his abdominal examination worsened, with increased tenderness and guarding. His WBC increased to 47,500, his BUN increased to 30 mg/dL (I 1.5 mmol/L), and his platelet count decreased to 30,000. However, the effluent from the Tenckhoff was clear. On the third hospital day, his abdominal examination continued to worsen and the drainage from the Tenckhoff catheter became bloody. At
From the Departments of Surgery and Pediatrics, Hopital Ste-Justine. Montreal, Quebec. Presented at the 21st Annual Meeting of the Canadian Association of Paediatric Surgeons, Edmonton, Alberta, September 20-23, 1989. Address reprint requests to Salam Yarbeck. MD, Department de Chirurgie, Hopital Ste Justine, 3175 Cote Ste Catherine, Montreal, Quebec H3S I C3, Canada. 0 1990 by W.B. Saunders Company. 0022-3468/90/251 l-0001 $03.00/O
112
1109
BRANDT
1110
surgical exploration he was found to have 200 mL of free blood in the peritoneal cavity with diffuse oozing from the entire surface of the colon, most markedly from the splenic flexure. There was also a hematoma noted in the mesentery of the colon. There were no perforations or other sites of bleeding found. Cultures of stool and peritoneal fluid were positive for E co/i 06:Hl and E coli 075:NM. His postoperative course was complicated by a right pleural effusion, which resolved spontaneously, and persistent renal failure, requiring peritoneal dialysis for I week postoperatively. He required a total transfusion of 520 mL of packed red blood cells (PRBCs) (42 mL/kg). He recovered and was discharged on postoperative day 12.
Case 2-Colonic
Perforation
A 4-year 6-month-old boy was transferred after evaluation and treatment at another hospital for bloody diarrhea and abdominal pain. The diagnosis of intussusception was made without a barium enema. At laparotomy his colon was found to be diffusely inflamed with no intussusception or other pathology found. Postoperatively he was noted to be anuric and was treated with multiple fluid boluses. At the time of transfer to this institution, on the third postoperative day, he weighed 27 kg, a gain of 6.3 kg over his usual weight, and had anasarca and pulmonary edema. His hematocrit was 23%. WBC count was 34,100, platelet count was 57,000, and BUN was 50 mg/dL (20 mmol/L). A peritoneal catheter was placed for emergency dialysis. The effluent was clear. Despite an overall improvement, he was never able to tolerate a diet because of nausea, vomiting, and mild abdominal pain, and required intravenous hyperalimentation for nutritional support. Plain abdominal radiographs were unremarkable. Serum amylase and WBC count were within normal limits. Esophagogastroduodenoscopy was performed on the 1 I th postoperative day, and showed a mild esophagitis and gastritis. On the 14th postoperative day he had a sudden onset of abdominal pain with tachycardia. The Tenckhoff effluent became cloudy with multiple organisms on Gram stain, an amylase of 1,200 U/L, and 7,000 WBC/mL. At laparotomy he was found to have a spontaneous perforation of the right transverse colon. The remainder of the colon and the pancreas appeared normal. A segmental resection, colostomy and mucous fistula were performed. Upon opening the specimen there was an area of totally denuded mucosa measuring 6 x 4 cm surrounding the perforation. Histological examination showed acute and chronic inflammation. Cultures of the peritoneal fluid were positive for E coli 0157:H7. His postoperative course was uncomplicated with adequate recovery of his renal function and closure of the colostomy 1 year later.
Case 3-Colonic
ET AL
botic microangiopathy of the small vesselsof the terminal ileum with areas of necrosis present, and acinar dilitation of the pancreas.
Case I-Pancreatitis A 2-year-old girl was transferred after admission elsewhere for dehydration and bloody diarrhea. On arrival her hematocrit was 28%, WBC count was 15,700, platelet count was 44,000, and BUN was 104 mg/dL (37 mmol/L). She underwent placement of a Tenckhoff catheter for emergency dialysis. Physical examination showed no abdominal abnormalities. Her hospital course was complicated by encephalopathy, presumed secondary to microangiopathy and the development, on the 20th hospital day, of vomiting and epigastic pain. Serum amylase was 780 U/L (normal ~200 U/L). Two days later her amylase was 579 U/L and and serum lipase was 1,048 U/L (normal
