We describe a 2 year-old boy with severe vasculitis who presented with a typical Kawasaki disease complicated with an intestinal pseudo-obstruction, gallbladder hydrops, myocarditis and transient coronary abnormalities despite early administration of intravenous immunoglobulin treatment.
Describimos el caso de un niño de 2 años con vasculitis grave que presentó un cuadro típico de enfermedad de Kawasaki complicada con una pseudo-obtrucción intestinal, hidrops vesicular, miocarditis y anormalidades coronarias transitorias, a pesar de la administración temprana de tratamiento con inmunoglulina intravenosa
Keywords: Kawasaki disease, cardiovascular abnormalities, intestinal pseudo-obstruction, abdominal pain, acute surgical abdomen
Palabras Clave: Enfermedad de Kawasaki, Alteracions cardiovasculares, pseudo-obstrucción intestinal, dolor abdominal, abdomen agudo quirúrgico
Kawasaki disease (KD) is the acute vasculitis responsible for most cases of acquired heart disease in children around the world. Surgical presentation of KD is not uncommon, including children who develop atypical forms of the disease. Recently, there have been reports of KD patients1-3 who presented as intestinal pseudo-obstruction or acute surgical abdomen pictures, which resulted in delayed diagnosis and treatment. Many of these children consequently developed coronary aneurysms, the most common and fearful complication of KD.
We report a child with typical KD who developed intestinal pseudo-obstruction, gallbladder hydrops, myocarditis, and transient coronary abnormalities despite early intravenous immunoglobulin (IVIG) treatment.
A 2-year-old boy presented to our children's hospital with a 5-day history of fever and 2 days prior to admission he progressively developed a maculopapular rash in thorax and genitals, had erythema and edema in palms and soles, bilateral conjunctival injection, swollen red lips, and a strawberry tongue. On the day of admission he developed a distended and painful abdomen, diarrhea, and vomiting.
On examination he was febrile (39.8ºC), heart rate was 180 beats/minute, met 5 major clinical diagnostic criteria for KD, and also presented a diffusely distended tender but soft abdomen, with no rebound sign. He received 1 dose of IVIG [2 g/Kg] and was started on acetylsalicylic acid (ASA) [100 mg/Kg/day]. Because of persistent abdominal distention and vomiting, a plain abdominal radiograph was performed which showed air fluid levels with diffuse small bowel loops distention, all of these consistent with an intestinal pseudoobstruction. An abdominal ultrasound revealed gallbladder hydrops, fluid-filled loops, and intestinal wall edema. He was seen by the pediatric surgeons who recommended conservative management with intravenous fluids and an open nasogastric tube, but no need for surgical intervention. Hemoglobin was 10.4g/dL, leukocytes 9930/mm3, (68% neutrophils), and platelets were 210,000/mm3; C-reactive protein was 322 mg/L, he had sterile pyuria, sodium was 129 mmol/L, potassium 3.5 mmol/L, and ALT and AST values were 68 IU/L and 163 IU/L, respectively. Abdominal distention persisted and the nasogastric tube drained 900 mL during a 24 hour period.
On day 2 of admission, an echocardiogram showed a mild dilatation of the left coronary artery, a moderate left ventricular dysfunction with a fractional shortening (FS) of 20%, for which oral furosemide (10 mg bid) and enalapril treatment were started. He improved dramatically on the same day and started to tolerate oral intake. A repeat echocardiogram on day 5 of admission showed a normal ventricular function with a FS of 30% and normal coronary arteries. He was discharged home with oral ASA, enalapril and furosemide. Two weeks later a new echocardiogram was normal and ASA was prescribed for 1 month more.
Intestinal pseudo-obstruction occurs in 2-3% of children with KD4. Some of these children present early with dilated coronary arteries, that could be related sometimes with a delay of more than 10 days in IVIG administration1,5. However, other children as our patient develop coronary abnormalities despite early IVIG administration2,4. Some of the severe abdominal complications including intestinal pseudo-obstruction, may reflect severe vasculitis to some extent, and therefore clinicians should pay especial attention to coronary lesions in children with similar abdominal manifestations as ours2,4.
This is of special concern in children in whom surgical manifestations develop prior to the onset of typical KD features, as recently described by Akikusa1 and Zulian2,3. In a series by Miyake and colleagues4, 5 of 7 children who developed intestinal pseudo-obstruction had coronary artery disease. Zulian et al.2 reported 50% of their patients with surgical-onset KD having coronary artery abnormalities.
Our patient had evidence of multiple organ system inflammation and diffuse vasculitis, as suggested by the presence of myocarditis, coronary abnormalities, gallbladder hydrops, and intestinal pseudo-obstruction. The majority of these patients do not require surgery and improve dramatically after IVIG treatment6, although steroids have been used in some of these children4. Fortunately, our patient developed only transient coronary abnormalities as confirmed with the repeat echocardiograms.
1.Akikusa JD, Laxer RM, Friedman JN. Intestinal pseudoobstruction in Kawasaki disease. Pediatrics. 2004;113:e504-e506.
Available at www.pediatrics.org/cgi/content/full/113/5/e504
2. Zulian F, Falcini F, Zancan L, et al. Acute surgical abdomen as presenting manifestation of Kawasaki disease. J Pediatr. 2003;142:731-735
3. Zulian F. Reply to: Kawasaki disease mimicking an acute appendicitis. J Pediatr. 2004;144:691-692.
4. Miyake T, Kawamori J, Yoshida T, Nakano H, Kohno S, Ohba S. Small bowel pseudoobstruction in Kawasaki disease. Pediatr Radiol. 1987;17:383-386
5. Fang SB, Lee HC, Huang FY, Chen MR. Intestinal pseudo-obstruction followed by major clinical features of Kawasaki disease: report of one case. Acta Paediatr Taiwan 2001;42:111- 114
6. 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;77:1273-1274
Comment of the reviewer Patricia Muñoz Casas del Valle MD, PhD Profesora de Pediatría y de Parasitología. Vicedecana de la Facultad de Medicina. Universidad de los Andes
Santiago de Chile. Chile
El caso clínico, su tratamiento y la evolución de la enfermedad están muy bien descritos y constituyen una buena contribución para los clínicos, pues alertan al Pediatra y al Cirujano infantil sobre una complicación infrecuente en esta enfermedad que es de manejo conservador como está señalado en el artículo.
La publicación de casos como el descrito permiten ir acumulando experiencias que a la fecha son escasas y tener presente esta complicación en niños con enfermedad de Kawasaki que presentan síntomas de abdomen agudo.
Comment of the reviewer Antonio Félix Conde Martín. Pathologist. Hospital Universitario Perpetuo Socorro. Badajoz, Spain.
The present paper is a report of a rare complication in Kawasaki disease. Intestinal pseudo-obstruction may occur up to 2-3% as reported in literature1,2.
It is important to recognize this atypical clinical presentation in order to not to delay the diagnosis and treatment of the disease. Such delay may be responsible of increased rate in coronary complications when this onset is present.
1.- Miyake T, Kawamori J, Yoshida T, Nakano H, Kohno S, Ohba S. Small bowel pseudo-obstruction in Kawasaki disease. Pediatr Radiol. 1987;17 :383 –386[Medline]
2.- 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 –1274[Medline]
Fabiola Acon-Rojas, MD. Servicio de Infectología, Hospital Nacional de Niños de Costa Rica, "Dr. Carlos Sáenz Herrera".
PO Box 3742-1000. San José, Costa Rica. Tele/Fax: (506) 258- 2173. E-mail: fabiacon @ yahoo.com.mx
Received , August 5, 2005
Published August 25, de 2005