Kawasaki Disease Presenting as Acute Intestinal Obstruction.

Kawasaki disease (KD) is an acute febrile illness of childhood associated with vasculitis of medium-sized arteries especially the coronary arteries. Typical clinical features involving the skin, mucous surfaces, etc., occur sequentially over a few days. We report a rare presentation of KD as a surgical abdomen in a 2-year-old boy. Awareness of this presentation is important as it can otherwise lead to a delay in starting potentially life-saving intervention like intravenous immunoglobulins for cardiac complications kept cryptic by the manifest acute abdomen.

INTRODUCTION

Kawasaki disease (KD), first described in Japan in 1962, is an acute febrile illness of early childhood characterized by multi-organ inflammation and diffuse arteritis.[1] Abdominal complications are unusual and most commonly involve the gallbladder, but necrosis of the liver, duodenum, and jejunum has been described.[2] Intestinal obstruction is rare, unlike ileus which can accompany the acute phase of the disease. We describe a child who presented with intestinal obstruction and was subsequently diagnosed with KD.

CASE REPORT

A 2-year-old male child presented to the emergency with chief complaints of abdominal pain and distension for 5 days along with reduced appetite. There were low-grade fever and constipation for the past 3 days with 2–3 episodes of bleeding per rectum. The child was dehydrated, lethargic and had cheilitis and mild conjunctival erythema. The abdomen was distended and diffusely tender. The perrectal examination was unremarkable. The erect abdominal radiograph was suggestive of intestinal obstruction [Figure 1]. Ultrasound abdomen showed edematous bowel in the right iliac fossa with target sign, suspicious of intussusception. The child was therefore taken up for emergency laparotomy. The entire large bowel was found to be edematous and markedly inflamed, especially in the ileocecal region. Mesenteric lymph nodes were prominent. The small bowel appeared normal except for mild distal ileal dilatation and a broad-based uninflamed Meckel's diverticulum. These findings were not suggestive of a resolved intussusception. The diverticulum was resected, and the ends brought out as a divided ileostomy. Full thickness biopsies were also taken from the ascending colon, transverse colon and rectosigmoid area just above the peritoneal reflection with a suspicion of inflammatory bowel disease/ulcerative colitis.

Figure 1

Air fluid levels on the erect abdominal radiograph suggestive of intestinal obstruction

The child was allowed orally on the 2nd postoperative day as the stoma began to function satisfactorily. However, he continued to be irritable and unwilling to sit or hold anything in his hands. There was generalized edema with intermittent bleed from the stoma. A week after surgery, the cheilitis and conjunctivitis became florid, and he began to develop periungual desquamation. A pediatric gastroenterology consultation was taken with a suspicion of KD. Histopathological examination of all the colonic biopsies showed diffuse nonspecific inflammatory changes with small and medium vessel vasculitis [Figure 2a and b]. Echocardiography on postoperative day 21 revealed transient coronary artery ectasia. The platelet count rose from 328,000/μL on admission to 630,000/μL by day 10 and 1,080,000/μL by day 20 postoperative. He was started on an anti-inflammatory dose of aspirin at 75 mg/kg/day and intravenous immunoglobulins at 2 g/kg over 24 h till he became afebrile. His general condition slowly improved and he was discharged 4 weeks after surgery in a satisfactory condition. Anti-thrombotic dose of aspirin (5 mg/kg/day) was continued till a repeat echocardiography 8 weeks later showed resolution of cardiac changes. A distal cologram performed 3 months later showed free passage without any strictures and the stoma was closed. Histopathological examination of the cut stomal edges showed vasculitis with moderate lymphocytic infiltrate of lamina propria. At 1 year follow-up, he is doing well; passing stools satisfactorily without any bleeding episodes and the abdominal wound has healed properly. He will be kept on long-term follow-up.

Figure 2

(a) Photomicrograph from the full thickness biopsy of the colon along the affected site showing medium sized sub-mucosal vessel exhibiting features of a healed vasculitis which is occluded by fibrin along with fibrosis of the vessel wall. (Hematoxylene eosin, ×240). (b) Medium power photomicrograph of the mesenteric lymph node showing a small sized capsular blood vessel exhibiting endothelial swelling and acute inflammatory cell infiltration. (Hematoxylene eosin, ×240)

DISCUSSION

The diagnosis of KD can be made by fulfilling five characteristic criteria in the absence of any specific diagnostic tests, namely, prolonged fever for at least 5 days together with four of a collection of five clinical features, including rash, non-purulent conjunctivitis, oropharyngeal changes, lymphadenopathy, and skin changes/desquamation of the peripheries.[34] As approximately 20%–25% of untreated patients can have life-threatening cardiac involvement like coronary thrombosis and myocardial infarction, early diagnosis is essential. Other non-aneurysmal cardiac complications include dilatation of coronary artery, pericardial effusion, pericarditis, myocarditis, and mitral incompetence.[3]

This disease causes severe vasculitis resulting in exudative infiltration of lymphocytes and large mononuclear cells into the media and adventitia of all blood vessels, particularly medium-sized vessels with endothelial necrosis and multi-systemic involvement.[56] Unusual symptoms can, therefore, be expected.

Gastrointestinal (GI) involvement is not common with a prevalence of only 2.3%.[78] The diagnosis of KD with predominant GI involvement may be delayed by 4–6 weeks as there may not be enough characteristic clinical criteria in the acute febrile phase. There was a delay in the index case, although by a lesser margin of 3 weeks, following the onset of abdominal pain and intestinal obstruction features. Diarrhea and vomiting are the most common GI complaints. Elevated liver enzymes and gallbladder hydrops are well-known while splenic infarct, pancreatitis, and ascites occur less frequently. Jejunal stricture and obstruction, intussusception, intestinal pseudo-obstruction, ischemic colitis, bowel edema, and hemorrhagic enteritis have been reported.[789] Zulian et al. reported 12 patients with KD with surgical abdominal complications including gallbladder hydrops with cholestasis (n = 3), small intestinal occlusion (n = 4), functional obstruction caused by paralytic ileus (n = 3), ischemic colitis (n = 1) and abdominal vasculitis with massive liver necrosis (n = 1).[9]

There is no clarity on the most common location of colonic wall edema in the literature. While edema may be due to inflammatory changes and vasculitis, more severe cases might produce nodular areas or intestinal obstruction when surgery cannot be avoided. The entire colon, especially the cecum, was involved in our case. There is an interesting hypothesis that the GI tract could be one of the primary sites of entry of bacterial toxins which then act as superantigens. Nagata et al. studied the jejunal mucosa in 19 patients in the acute phase of KD and associated Gram-negative microbes such as Neisseria mucosa along with Gram-positive cocci in its pathogenesis. These microbes with superantigen properties by eliciting heat shock proteins can induce immune activation triggering high expression of heat shock proteins in endothelial cells. Although all the isolated bacteria in their study were antibiotic resistant, they postulate the future role of specific antibiotics in treatment.[10]

Since no diagnostic tests are available for KD, the diagnosis is absolutely based on clinical criteria. In our patient, some complications preceded some of the typical acute clinical features leading to difficulty in early diagnosis. KD tends to be under-recognized or misdiagnosed with other febrile illnesses and systemic diseases but should be kept in mind in a child with evolving clinical features as seen in our patient. Children with KD can have lifelong morbidity and our patient who still had evidence of vasculitis at the time of stoma closure will be kept under long-term close observation.

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Conflicts of interest

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