An 11-Year-old Male Presenting With Fever and Vomiting

CASE An 11-year-old male presented in April 2020 to the pediatric emergency department at a tertiary academic hospital in Turkey with fever, malaise and vomiting. The day before, he had had sudden abdominal pain, followed by vomiting and fever (38 °C) after a heavy meal at the end of a 16-hour religious fast. For the past few months, he had intermittently experienced widespread rash over his face and extremities. His medical history revealed no known comorbidities. At presentation, he appeared mildly ill and dehydrated, but he was hemodynamically stable with a heart rate of 100 beats/min and blood pressure of 100/60 mm Hg. His body temperature was 37.2 °C, and oxygen saturation was 97%. Respiratory, cardiac and abdominal examinations were normal. He had no skin rash, but bilateral mild conjunctival hyperemia was present. According to our local COVID-19 pandemic triage protocol for children with fever, a nasopharyngeal swab for SARS-CoV-2 polymerase chain reaction was obtained, and he was admitted for observation. Chest radiograph was unremarkable. Complete blood count revealed white blood cell count of 13,000/mm3 (neutrophil 93%, monocyte 5%, lymphocyte 1% and eosinophil 1%), hemoglobin of 13.6 g/dL, and platelet count of 249,000/mm3. His laboratory parameters showed elevated inflammatory markers with C-reactive protein of 14.6 mg/dL (normal range [NR]: 0–0.8) and procalcitonin of 8.046 ng/mL (NR: 0–0.1), hyponatremia with serum sodium 127 mEq/L (NR: 136–146) and mild hypoalbuminemia with albumin of 3.07 g/dL (NR: 3.5–5.2). Venous blood gas analysis was normal. Blood urea nitrogen was 20 mg/dL (NR: 5–18), and urine specific gravity was 1.019, compatible with dehydration. Six hours after admission, the patient suddenly became hemodynamically unstable with blood pressure of 75/35 mm Hg, extensive urticarial-like rash over his face and extremities, and fever (38.4 °C) refractory to antipyretics. Bilateral conjunctival hyperemia became more evident. A sepsis evaluation was initiated, including blood cultures and coagulation tests. Other markers of inflammation, including ferritin, triglycerides and fibrinogen were also obtained. Treatment for hypotension and suspected septic shock was initiated with crystalloid boluses (up to 40 mL/kg) and broad-spectrum antibiotics with meropenem and clindamycin. Within a few hours, the patient’s vital signs stabilized, and the fever subsided. However, the urticarial-like rash persisted. The international normalized ratio was 1.9 (0.8–1.2). Ferritin, triglyceride, and fibrinogen were normal, and SARS-CoV-2 polymerase chain reaction was negative. The next morning, bilateral desquamation of fingertips and slight abdominal tenderness became evident. With fever, rash, conjunctivitis, hemodynamic instability, and acral desquamation, Kawasaki shock syndrome or possible Kawasaki-like illness caused by pediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 (PIMS-TS) was considered as other possible diagnoses, prompting cardiac evaluation. Electrocardiogram, echocardiogram and troponin I were all normal. Because of the vomiting, abdominal pain, and fever without a focus, abdominal ultrasonography (US) was performed, which revealed the true diagnosis. For Denouement see P. 277. DENOUEMENT Continued from P. 276. Sonography showed a heterogeneous cystic lesion with echogenic solid components in the left hepatic lobe adjacent to the falciform ligament and another cystic lesion in the right hepatic lobe. Abdominal computed tomography (CT) scan with intravenous contrast revealed a partially collapsed large cyst with detached membranes near the falciform ligament, consistent with a ruptured hepatic hydatid cyst (Fig. 1) and an intact cyst in the right hepatic lobe. Significant free fluid was noted throughout the perihepatic and pericolic regions. Thoracic and cranial CT scans were normal.FIGURE 1.: Axial CT demonstrating partially collapsed hepatic cyst with detached membranes (arrow).The patient underwent surgery via a transabdominal approach. One ruptured cyst in the left and one nonruptured cyst in the right hepatic lobe, both with a diameter of approximately 6 cm, were visualized. Cystostomy was performed for both cysts. Contents of the cysts, including laminated membranes, were removed, and the cavities were flushed with 3% hypertonic saline. The cyst walls were carefully inspected, confirming that there were no open biliary radicals. The intraabdominal purulent fluid was aspirated, and the peritoneum was washed with sponges soaked in 3% hypertonic saline. Pathology results confirmed the diagnosis of hydatid cyst, and albendazole was initiated (10 mg/kg/d PO). On the seventh postoperative day, meropenem and clindamycin were discontinued because fever, hypotension, rash, conjunctival hyperemia had resolved and laboratory findings had returned to normal, and the patient was discharged on treatment with albendazole. At his latest outpatient follow-up visit 5 months after the operation, he was asymptomatic and had a normal physical examination and abdominal US. He remains on albendazole with an intention to complete a course of 9–12 months. This patient presented with fever and malaise during the COVID-19 pandemic, and deteriorated shortly after his admission, suggesting the differential diagnoses of septic shock, Kawasaki disease (KD) and PIMS-TS. However, he was eventually and unexpectedly diagnosed with a rare complication of an endemic illness: anaphylactic shock secondary to ruptured hepatic hydatid cyst. Hydatid cyst (echinococcosis) is a parasitic infection caused by Echinococcus granulosus, which most frequently affects the liver. The disease affects 2–3 million people worldwide and is endemic in Turkey, other Mediterranean countries, South Africa, the Middle East, South America, and New Zealand. In Turkey, the prevalence of echinococcal disease is 50–400 per 100,000 and the incidence is 3.4 per 100,000.1 In humans, hydatid disease involves the liver in approximately 75% of cases, the lung in 15%, and other anatomic locations in 10%. Patients often present with complications related to the involved organ (due to cyst rupture or infection) or at distant anatomic sites.2 In the liver, cysts may remain asymptomatic, may regress spontaneously or may produce nonspecific symptoms. Symptomatic cysts can cause increased abdominal girth, hepatomegaly, palpable mass, vomiting or abdominal pain. Ultrasonography and CT are helpful in the diagnosis, which can be confirmed by intraoperative examination and histopathology, as in the present patient. Treatment requires surgical removal of the cysts and antihelminthic treatment with albendazole or mebendazole.1 The treatment modality should be based on the characteristics of the cyst (ultrasonographic stage and localization). Medical treatment with albendazole alone (10–15 mg/kg/d orally divided twice daily for 1–6 months; maximum 800 mg/d) may result in a high rate of cure for cysts smaller than 5 cm in diameter. Mebendazole (40–50 mg/kg/d) is an alternative oral drug. In uncomplicated larger cysts, techniques that are less invasive (such as endoscopic, laparoscopic or puncture-aspiration-injection-reaspiration) and medical treatment can be combined. Surgical treatment is preferred for ruptured cysts (as in our patient), infected cysts, bone or central nervous system cysts, large hepatic cysts with multiple daughter cysts, large pulmonary cysts, cysts communicating with the biliary tract and cysts compressing vital organs. Albendazole treatment should be initiated days to weeks before surgery to decrease the risk of spilling live scolices into the abdomen and continued for 1–3 months after surgery.3.4 Because our patient experienced a life-threatening complication and required emergency surgery, he did not receive preoperative albendazole. Therefore, our clinical judgment favored a much more extended course of postoperative albendazole. Rupture of the hydatid cyst, which can occur spontaneously, with minor trauma or during surgery, is a serious complication that can be associated with IgE-mediated immediate hypersensitivity reactions, such as anaphylaxis, due to the antigenic nature of the cyst fluid. Risk factors for cyst rupture include large cysts, superficial cysts and young age.5 Sudden abdominal pain and subsequent vomiting in our patient during the preceding evening had been attributed to a heavy meal after a fast, when in fact, it might have been caused by the rupture of the hydatid cyst. Acute abdomen caused by ruptured hydatid cyst requires emergency surgical removal of the cyst, together with postoperative antihelminthic treatment. In some cases, cyst rupture may be clinically silent. While ultrasonography and CT can both demonstrate ruptured cysts, CT is superior for detecting cyst infection. Infection may occur only after rupture of both the pericyst and the endocyst, which allows bacteria to pass easily into the cyst.2 Scolices may spread to the abdomen during surgery, particularly during emergency surgery for ruptured cysts. Operative management typically starts with the isolation of the liver from the rest of the abdomen, usually with sponges soaked in hypertonic saline to prevent spillage of the cyst material. All components of the cyst should be carefully removed. Hypertonic saline, which deactivates cyst contents, can be injected into nonruptured cysts after aspiration of the cyst fluid and can also be used to flush the cavity of ruptured cysts and after cyst excision. The peritoneal cavity should then be washed with a scolicidal agent (cetrimide-chlorhexidine, povidone-iodine [10%], silver nitrate [0.5%], hypertonic saline solution [3%–30%], chlorhexidine [0.4%] or praziquantel).6 Several studies have shown that particularly high concentrations of hypertonic saline solution may cause sclerosing cholangitis and acute pancreatitis.7–9 Therefore, we prefer a low concentration (3%) of hypertonic saline solution in our practice. Anaphylaxis is the most frequent cause of death by hydatid disease.10 Sudden deaths caused by hydatid disease after heavy exercise or trauma have been reported in Turkey and in other countries. Most of these sudden deaths were attributed to anaphylaxis caused by ruptured or nonruptured cysts.10–12 Anaphylaxis is caused by leakage of the antigenic cyst fluid into blood vessels or anatomic cavities. Even unruptured cysts may erode a small vessel in the liver while enlarging, and a small quantity of cyst fluid can spread into the bloodstream, triggering an anaphylactic response.10,13 Our patient’s history included occasional urticarial-like rash, which may be explained by this mechanism. The reported frequency of minor allergic reactions after traumatic or spontaneous rupture of hydatid cysts ranges from 16.7% to 25%, and the incidence of more severe reactions is 1% to 12.5%.14 As most of the published literature consists of case reports, there are no data regarding the incidence of hydatid cyst rupture and anaphylaxis in Turkish children. Given the patient’s clinical features in the context of the COVID-19 pandemic, it was important to consider the newly described PIMS-TS in the differential diagnosis. PIMS-TS is defined as fever, inflammation and evidence of single- or multiorgan dysfunction, after the exclusion of other microbial causes, with positive or negative testing for SARS-CoV-2. This syndrome shares common features with other pediatric inflammatory conditions including KD, KD shock syndrome, staphylococcal and streptococcal toxic shock syndromes, bacterial sepsis and macrophage activation syndrome.15 Our patient was 11 years old, which is an unusual age for classical KD but appropriate for PIMS-TS.16 He presented with fever and unusual abdominal symptoms with greatly elevated inflammatory markers, with accompanying hypoalbuminemia and hyponatremia, which are also considered as markers of hyperinflammation.17 A tendency to prioritize the consideration of COVID-19 and associated diseases in the triage, assessment and management of every patient is an inherent consequence of this pandemic. This patient, who had presenting signs and symptoms compatible with the suspected case definition of COVID-19, but deteriorated rapidly, highlights the importance of keeping atypical manifestations of endemic diseases in mind when considering the differential diagnosis. Permission was obtained from the family before proceeding with this case report.

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