Rare case of infective aortitis with aortic rupture and cardiac tamponade in a young child
- Manas Datta ,
- Sangeetha Pradeep and
- Mainak Aditya
- Department of Paediatrics, Broomfield Hospital, Mid and South Essex NHS Foundation trust, Chelmsford, UK
- Correspondence to Dr Mainak Aditya; m.aditya@nhs.net
Abstract
Aortitis and aortic dissection are very rare in children. The clinical presentation of aortitis varies across a spectrum, ranging from incidental findings to fatal aortic dissection and rupture. A high index of suspicion is needed to establish an accurate and timely diagnosis. Here, we present an unfortunate case of fatal infective aortitis with aortic rupture and cardiac tamponade in a healthy toddler. Postmortem report implicated Kingella kingae as the causative organism of aortic pseudoaneurysm and rupture, leading to the instantaneous death of the child.
Background
Aortitis is a rare life-threatening condition, involving inflammation of the aorta, which carries blood at high pressure from the heart to the vital organs of the body. Aortitis is usually seen in systemic inflammatory conditions and rarely in some infections.
Causes of aortitis can be broadly divided into three groups: Non-infectious causes may be associated with autoimmune conditions where our own body’s natural immune system attacks the healthy tissues. Infectious causes include tuberculosis, syphilis, Salmonella, Staphylococcus spp, Streptococcus pneumoniae and Rocky Mountain spotted fever. Isolated aortitis includes idiopathic aortitis and chronic periaortitis (eg, Ormond’s disease) where other vessels are not affected.1
Kingella kingae is a gram-negative facultative anaerobic Beta haemolytic coccobacilli (family: Neisseriaceae). It belongs to the HACEK group (Haemophilus species, Aggregatibacter actinomycetemcomitans, Cardiobacterium hominis, Eikenella corrodens and Kingella species). This is commonly found as a commensal of the throat in young children. In one longitudinal study, it was found that an average of 27.5% of children attending daycare centres were colonised with K. kingae at any given time.2 The colonised epithelium is the source of bloodstream invasion and dissemination to distant sites. K. kingae outbreaks occur sporadically in childcare centres but remain poorly understood and sometimes difficult to identify.
Case presentation
This patient was a healthy young boy with no comorbidities and his height and weight were >90 th centile. He developed intermittent high-grade fever with drooling of saliva for 8–9 days. His parents arranged a COVID-19 PCR test for themselves and the child which was found to be negative. The mother consulted the general practitioner (GP) who prescribed oral phenoxymethylpenicillin on his seventh day of illness for suspected upper respiratory infection. The GP found an erythematous throat on clinical examination; the rest of the examination was unremarkable. He took oral antibiotics for 2 days and was much improved on that morning; he was playful and even went to nursery. At approximately 1600 hours, he was sitting on his high chair and just had his oral antibiotic dose, he suddenly developed a cough and vomited and made a shrill cry, and then became stiff and unresponsive. His mother laid him down on the floor and began giving him chest compressions and called for help. Paramedics arrived within 10 min and found him unresponsive and deemed to be in cardiac arrest. They began full resuscitation along with medications. He was then airlifted to the nearest hospital at around 1710 hours. Resuscitation continued at the emergency department, but with no successful outcome, and treatment was withdrawn at 1735 hours on that day. The coroner’s inquiry started and a postmortem was conducted at the department of histopathology in the referral hospital.
Investigations
Postmortem findings:
The postmortem appearances were consistent with those of aortitis with pseudoaneurysm that had ruptured to cause haemopericardium and cardiac tamponade.
Postmortem CT scan showed the presence of hyperdense fluid (depth of approximately 1.6 cm) around the heart and represented antemortem pericardial haemorrhagic effusion, which possibly led to pericardial tamponade. The visceral examination also showed the heart was encased in a thick thrombus.
A pseudoaneurysm (approximately 13 mm × 10 mm × 8 mm) with a perforation at its apex, was found between the left atrial appendage, pulmonary trunk and aorta. There was a defect on the anterolateral wall of the ascending aorta also (size 10 mm × 8 mm) situated approximately 10 mm above the sinotubular junction. The defect showed a stellate appearance and from its angles, small linear tears extended centrifugally into the wall of the aorta. There was fibrous induration of the soft tissues between the aorta and pulmonary trunk and it is perforated anterolaterally to form a direct communication between the lumen of the aorta and the pericardial sac. There was dense inflammatory cell infiltration in the connective tissue between the aorta and pulmonary trunk and formed microabscesses. There were also focal endothelialitis in the wall of the aorta along with coagulative necrosis and disruption of the aortic wall. The right atrium and left and right ventricles and interventricular septum were unremarkable. Mitral and tricuspid valve leaflets also appeared normal.
Postmortem virology tests detected enterovirus and parechovirus from the larynx. Also, adenovirus DNA was detected from respiratory secretions. 16s rDNA PCR test from the heart and necrotic aortic tissues showed the presence of K. kingae.
Treatment
K. kingae infections are usually sensitive to common oral and parenteral β-lactam antibiotics prescribed to young children, as most invasive diseases run a benign clinical course, except infective endocarditis, which can be complicated by embolic phenomenon like cerebral infarction.3 Kingella infective endocarditis children are mostly treated with intravenous second or third-generation cephalosporins alone or in combination with aminoglycosides for at least 4 weeks. Emergency cardiac surgery is often necessary for them. Chemoprophylaxis with oral rifampin alone or in combination with amoxicillin, is also recommended for children in daycare centres, where an outbreak of invasive K. kingae disease has occurred.4 Screening and chemoprophylaxis of siblings of age group 6–36 months of the diagnosed case, has also been proposed.4
Outcome and follow-up
K. kingae is usually implicated as an organism for osteomyelitis, septic arthritis, spondylodiscitis and bacteraemia in otherwise healthy children aged 6–36 months.2 Along with other members of the HACEK group, it is also implicated to cause infective endocarditis and mitral valvulitis in children and adults with pre-existing cardiac conditions. With recent advancements in culturing methods, more and more cases of invasive diseases of these commensal organisms are identified. With this case report, we want to increase awareness among clinicians, regarding the emergence of atypical and complicated K. kingae infections.
Discussion
Skeletal infections remain the most common clinical presentation of K. kingae. Recent studies have shown that K. kingae has displaced S. aureus as the most common cause of acute septic arthritis in children of all ages.5 K. kingae is part of the normal oropharyngeal flora of young children. In a longitudinal study of young children in Israel, a cohort of 716 children between the ages of 2 and 30 months were screened. K. kingae carriage rate was nil below 6 months of age, 1.5% at 6 months, 9.6% at 12 months, remained stable between 10.4% and 12% during the second year of life and decreased significantly to 5.3% at 30 months.6 (figure 1)
The percentage of oropharyngeal carriage of Kingella kingae between 12 and 30 months of age in a longitudinal study conducted in Israel in the year 2005–2006 period. Figure 1 is used by permission from Oxford university press from the Journal: Amit et al. 6
Clinical data suggest that the organism may gain access to the bloodstream in the course of an upper respiratory viral infection or stomatitis. In our case report also, coexistent enterovirus, adenovirus and parechovirus were detected in the respiratory secretions and excessive drooling of saliva (suspected stomatitis) was present during the initial presentation. Few cases of confirmed K. kingae meningitis are also reported in children and young adults.7 There is also a case report of chorioamnionitis and intrauterine infection leading to premature rupture of membrane and miscarriage due to K. kingae infection.8
More and more cases of unusual and complicated presentations of K. kingae infections are reported in previously healthy children. Except for patients with endocardial involvement, it is believed that children with K. kingae infection often show a mild, prolonged course of illness, needing more awareness among clinicians. One recent study in Israel compared the clinical features of Kingella and non-Kingella endocarditis in children.9 Throughout the study period, an increased incidence of Kingella endocarditis was noted. Also, they observed that Kingella endocarditis patients had some special features as compared with children with Staphylococcal and Streptococcal endocarditis. Kingella predominantly affected boys and displayed higher temperature at admission and recent history of aphthous ulcers.9 In 2010, there was a rare case report of Kingella endocarditis resulting in a perforated aortic root abscess and fistulous connection between the sinus of Valsalva and the left atrium in a child.10 There was another case report of a subtle presentation of K. kingae mitral valve infective endocarditis in a normal-cardiac infant.11 It has been found that K. kingae produces type IV pili and autotransporter adhesion protein called knh (Kingella NhhA homolog), which facilitates mucosal colonisation of oropharyngeal epithelial cells.12 13 Kingella also secretes a potent RtxA toxin (belonging to the RTX family of toxins) with broad-spectrum cytotoxicity, which helps in the penetration of the respiratory epithelial barrier.14 15 The presence of the polysaccharide capsule and the exopolysaccharide also enhances the resistance of the organism in the bloodstream and deep body tissues.13 16 Isolation of K. kingae in the routine solid medium is suboptimal, and detection of this organism is significantly increased by inoculation of exudates in the aerobic blood culture bottles and the use of PCR-based assays (ie, 16s rDNA gene assay, reverse transcription polymerase chain reaction tests). It is believed that many cases of previous culture-negative septic arthritis and endocarditis might have been caused by K.kingae, as it remained undetected in normal culture methods. A recent study report from Israel has proposed that persistent high fever in young healthy children (<36 months of age) without any obvious focus (especially boys), with or without congenital heart defects, and with a recent history of oral aphthous ulcer or stomatitis, should raise suspicion for K. kingae endocarditis.9
Learning points
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With advancements in microbiological and immunological techniques, subtle and complicated presentations of Kingella kingae infections are reported in previously healthy young children.
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Many cases of previous culture-negative septic arthritis and endocarditis might have been caused by Kingella, as it remained undetected in normal culture methods.
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Kingella infections can be life-threatening and fatal, even in healthy children, if not recognised and treated early.
Ethics statements
Patient consent for publication
Acknowledgments
We would like to thank the parents of the child who kindly agreed to give their consent to publish this rare case report in the journal. We also wish to thank the doctors and staff of the Department of Histopathology, Great Ormond Street Hospital for Children NHS foundation trust, London, who performed the detailed post-mortem examination and gave their expert opinion about the cause of death of this child. We also want to thank Oxford University Press for giving kind permission to use Figure 1 in our case report.
Footnotes
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Contributors MD, SP and MA were jointly involved in the management of this case as well as discussion with parents. MA collected the necessary documents, consent from parents and written this case report for publication in the BMJ. Manuscript has been reviewed and approved by all three authors.
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Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
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Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.
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Competing interests None declared.
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Provenance and peer review Not commissioned; externally peer reviewed.
- © BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.
References
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