- Email: [email protected]
Kawasaki disease mimicking an acute appendicitis
REFERENCES
Kawasaki disease mimicking an acute appendicitis To the Editor: We read with interest the recent paper by Zulian et al1 regarding acute surgical abdomen as the presenting form of Kawasaki disease (KD). We would like to report a child that we recently treated at our children’s hospital. A 4-year-old boy presented with a 1-day history of fever, vomiting, and abdominal pain. Fever (408 C), tachycardia, a systolic murmur, and severe diffuse abdominal pain were found. Blood count showed 24,200 leukocytes/mm3, neutrophilia, and normal platelets; he had sterile pyuria and C-reactive protein was 149 mg/L. Abdominal radiographs revealed intestinal loop distension and edema, and ultrasound showed no evidence of gallbladder disease. Abdominal pain increased within a few hours, and McBurney sign and rebound tenderness were found by 3 different experienced pediatric surgeons. He underwent surgery with a presumptive diagnosis of acute appendicitis, but had a normal appendix. Mesenteric adenopathy was found at surgery. The appendix was removed by intussusception and no biopsy or cultures were obtained. Fever persisted for 4 days. Two days after surgery he developed an erythematous rash in thorax, abdomen, and scrotum, as well as conjunctival injection, cervical adenopathy, oral mucositis, strawberry tongue, and edematous red palms. On day 5, an echocardiogram showed a pericardial effusion, fusiform dilation of the left coronary artery (LCA), but no aneurysms. antistreptolysin O titer and serologies for Epstein Barr virus, cytomegalovirus, and parvovirus B19 were negative, as were bacterial cultures. A diagnosis of KD led to treatment with intravenous immunoglobulin (2 g/kg) and acetylsalicylic acid (100 mg/kg/day), with dramatic improvement the next day. He was discharged home with acetylsalicylic acid treatment. A repeat echocardiogram one week after showed resolution of pericardial effusion, but this time he had dilation of both coronary arteries and an LCA aneurysm. Kawasaki disease uncommonly manifests as an acute surgical abdomen.1-5 Abdominal pain in some of these children occurs secondary to large-vessel vasculitis including the aorta and mesenteric arteries, with subsequent ischemia and pain.5 However, mesenteric adenitis may also occur. Because the other organs were visibly normal and no appendicular vasculitis was proven,1 we consider mesenteric adenitis to be a likely explanation for his abdominal symptoms. Presented in part as an abstract at the ‘‘40th Annual Meeting of the Infectious Diseases Society of America (IDSA),’’ Chicago, Illinois, October 24-27, 2002, Abstract No. 636.
Letters
1. Zulian F, Falcini F, Zancan L, Martini G, Secchieri S, Luzzatto C, et al. Acute surgical abdomen as presenting manifestation of Kawasaki disease. J Pediatr 2003;142:731-5. 2. Chiba T. Two cases of appendicitis in Kawasaki disease. Arch Jpn Chir 1998;67:69-71. 3. Chung CJ, Rayder S, Meyers W, Long J. Kawasaki disease presenting as focal colitis. Pediatr Radiol 1996;26:455-7. 4. Wheeler RA, Najmaldin AS, Soubra M, Griffiths DM, Burge DM, Atwell JD. Surgical presentation of Kawasaki disease (mucocutaneous lymph node syndrome). Br J Surg 1990;77:1273-4. 5. Mercer S, Carpenter B. Surgical complications of Kawasaki disease. J Pediatr Surg 1981;16:444-8.
Reply
Rolando Ulloa-Gutierrez, MD, Servicio de Infectologı´a Rafael Gutierrez-Alvarez, MD, Servicio de Cardiologı´a Maria L. Avila-Aguero, MD, Servicio de Infectologı´a Departamento de Pediatrı´a Hospital Nacional de Nin˜os de Costa Rica San Jose´, Costa Rica YMPD769
To the Editor: We read with interest the letter of Ulloa-Gutierrez et al reporting a 4-year-old boy with acute appendicitis as presenting sign of Kawasaki disease (KD). Since the publication of our study,1 three more patients with acute abdominal symptoms as the presenting manifestation of KD have been referred to us for a second opinion. In Ulloa-Gutierrez’s patient, as in the majority of the patients in our series, the sudden onset of fever, vomiting, and abdominal pain were the confounding features that induced the surgeons to perform laparotomy and partially delayed the diagnosis. However, in three of our patients whose diagnosis was made early, intravenous immunoglobulin treatment could not arrest the development of the abdominal complications or prevent the coronary involvement. This finding seems to confirm that the onset KD with abdominal symptoms represents the severe side of KD and the expression of a more aggressive disease. In the reported case, it would have been of some interest to obtain the histologic examination of appendix to show the presence of appendicular arteritis. Indeed it would have been useful to know if there was elevation of hepatic transaminase. In our opinion, a mild elevation of transaminases, unusual in classic appendicitis,2 can represent a helpful variable to be considered in the differential diagnosis. Mesenteric adenitis could be a reasonable explanation for the abdominal symptoms in the reported case, but histologic examination in this case would not have helped in the differential diagnosis because the inflammatory picture is usually unspecific (personal observation). Mesenteric lymph node hyperplasia could represent the early response to an intestinal pathogen acting as trigger for the disease in these particular patients. In fact, as suggested by some authors, it is possible that in patients with acute abdominal symptoms, bacteria colonizing the small intestine mucosa may produce exotoxins acting as superantigens with subsequent Vb2 T-cells clonal expansion.3,4 Francesco Zulian, MD Rheumatology Unit Department of Pediatrics University of Padua 35128 Padua, Italy YMPD770
10.1016/j.jpeds.2004.01.060
10.1016/j.jpeds.2004.01.061
691
Kawasaki disease mimicking an acute appendicitis To the Editor: We read with interest the recent paper by Zulian et al1 regarding acute surgical abdomen as the presenting form of Kawasaki disease (KD). We would like to report a child that we recently treated at our children’s hospital. A 4-year-old boy presented with a 1-day history of fever, vomiting, and abdominal pain. Fever (408 C), tachycardia, a systolic murmur, and severe diffuse abdominal pain were found. Blood count showed 24,200 leukocytes/mm3, neutrophilia, and normal platelets; he had sterile pyuria and C-reactive protein was 149 mg/L. Abdominal radiographs revealed intestinal loop distension and edema, and ultrasound showed no evidence of gallbladder disease. Abdominal pain increased within a few hours, and McBurney sign and rebound tenderness were found by 3 different experienced pediatric surgeons. He underwent surgery with a presumptive diagnosis of acute appendicitis, but had a normal appendix. Mesenteric adenopathy was found at surgery. The appendix was removed by intussusception and no biopsy or cultures were obtained. Fever persisted for 4 days. Two days after surgery he developed an erythematous rash in thorax, abdomen, and scrotum, as well as conjunctival injection, cervical adenopathy, oral mucositis, strawberry tongue, and edematous red palms. On day 5, an echocardiogram showed a pericardial effusion, fusiform dilation of the left coronary artery (LCA), but no aneurysms. antistreptolysin O titer and serologies for Epstein Barr virus, cytomegalovirus, and parvovirus B19 were negative, as were bacterial cultures. A diagnosis of KD led to treatment with intravenous immunoglobulin (2 g/kg) and acetylsalicylic acid (100 mg/kg/day), with dramatic improvement the next day. He was discharged home with acetylsalicylic acid treatment. A repeat echocardiogram one week after showed resolution of pericardial effusion, but this time he had dilation of both coronary arteries and an LCA aneurysm. Kawasaki disease uncommonly manifests as an acute surgical abdomen.1-5 Abdominal pain in some of these children occurs secondary to large-vessel vasculitis including the aorta and mesenteric arteries, with subsequent ischemia and pain.5 However, mesenteric adenitis may also occur. Because the other organs were visibly normal and no appendicular vasculitis was proven,1 we consider mesenteric adenitis to be a likely explanation for his abdominal symptoms. Presented in part as an abstract at the ‘‘40th Annual Meeting of the Infectious Diseases Society of America (IDSA),’’ Chicago, Illinois, October 24-27, 2002, Abstract No. 636.
Letters
1. Zulian F, Falcini F, Zancan L, Martini G, Secchieri S, Luzzatto C, et al. Acute surgical abdomen as presenting manifestation of Kawasaki disease. J Pediatr 2003;142:731-5. 2. Chiba T. Two cases of appendicitis in Kawasaki disease. Arch Jpn Chir 1998;67:69-71. 3. Chung CJ, Rayder S, Meyers W, Long J. Kawasaki disease presenting as focal colitis. Pediatr Radiol 1996;26:455-7. 4. Wheeler RA, Najmaldin AS, Soubra M, Griffiths DM, Burge DM, Atwell JD. Surgical presentation of Kawasaki disease (mucocutaneous lymph node syndrome). Br J Surg 1990;77:1273-4. 5. Mercer S, Carpenter B. Surgical complications of Kawasaki disease. J Pediatr Surg 1981;16:444-8.
Reply
Rolando Ulloa-Gutierrez, MD, Servicio de Infectologı´a Rafael Gutierrez-Alvarez, MD, Servicio de Cardiologı´a Maria L. Avila-Aguero, MD, Servicio de Infectologı´a Departamento de Pediatrı´a Hospital Nacional de Nin˜os de Costa Rica San Jose´, Costa Rica YMPD769
To the Editor: We read with interest the letter of Ulloa-Gutierrez et al reporting a 4-year-old boy with acute appendicitis as presenting sign of Kawasaki disease (KD). Since the publication of our study,1 three more patients with acute abdominal symptoms as the presenting manifestation of KD have been referred to us for a second opinion. In Ulloa-Gutierrez’s patient, as in the majority of the patients in our series, the sudden onset of fever, vomiting, and abdominal pain were the confounding features that induced the surgeons to perform laparotomy and partially delayed the diagnosis. However, in three of our patients whose diagnosis was made early, intravenous immunoglobulin treatment could not arrest the development of the abdominal complications or prevent the coronary involvement. This finding seems to confirm that the onset KD with abdominal symptoms represents the severe side of KD and the expression of a more aggressive disease. In the reported case, it would have been of some interest to obtain the histologic examination of appendix to show the presence of appendicular arteritis. Indeed it would have been useful to know if there was elevation of hepatic transaminase. In our opinion, a mild elevation of transaminases, unusual in classic appendicitis,2 can represent a helpful variable to be considered in the differential diagnosis. Mesenteric adenitis could be a reasonable explanation for the abdominal symptoms in the reported case, but histologic examination in this case would not have helped in the differential diagnosis because the inflammatory picture is usually unspecific (personal observation). Mesenteric lymph node hyperplasia could represent the early response to an intestinal pathogen acting as trigger for the disease in these particular patients. In fact, as suggested by some authors, it is possible that in patients with acute abdominal symptoms, bacteria colonizing the small intestine mucosa may produce exotoxins acting as superantigens with subsequent Vb2 T-cells clonal expansion.3,4 Francesco Zulian, MD Rheumatology Unit Department of Pediatrics University of Padua 35128 Padua, Italy YMPD770
10.1016/j.jpeds.2004.01.060
10.1016/j.jpeds.2004.01.061
691