Invasive pneumococcal disease presenting as purpura fulminans in a child with Mondini's Dysplasia/Purpura fulminans ile basvuran Mondini Displazi tanili cocukta invaziv pnomokokkal hastalik.Introduction
Purpura fulminans (PF) is a condition characterized by disseminated intravascular coagulation
(DIC) presenting with ecchymotic skin lesions, fever, bacteremia and hypotension. In the majority of cases, PF is usually associated with Neisseria meningitidis sepsis. However, even if it is a rare agent, Streptococcus pneumoniae can be responsible for purpura fulminans (1,2). Other encapsulated bacteria such as Haemophilus influenzae and group A and B streptococci have been implicated in infants and adults. Staphylococcus aureus as a cause of purpura fulminans is considered rare, probably because these cases are reported as toxic shock syndrome, but may be as common as other nonmeningococcal organisms (3).
Cases secondary to pneumococcal infections usually occur in those with underlying predisposing conditions such as congenital (primary) or acquired immunodeficiencies (e.g. HIV infection) or splenic dysfunction. In the pediatric age group, splenic dysfunction is the most frequent cause of invasive pneumococcal infections (4,5). However, other underlying factors like temporal bone defects, or congenital anomalies like Mondini's dysplasia are also possible risk factors for invasive disease. Here we present a patient who had Mondini's dysplasia with invasive pneumococcal infection related purpura fulminans.
A five year old girl was admitted to the hospital because of fever. She had an implant in her
left ear because of Mondini's dysplasia (Figure 1) and findings of inflammation were seen on her eardrum. Amoxicillin-clavunate therapy was started according to these findings but loss of consciousness in follow up led the physicians to consider the diagnosis of an intracranial infection. Parenteral antibiotherapy with ceftriaxone and vancomycin was started after a computerized tomography of the cranium was performed. Thereafter she was transferred to our hospital because of the presence of subarachnoid bleeding and sepsis. On admission, she was unconscious, dehydrated and was in septic shock. There were disseminated palpable purpuric lesions on her skin as a sign of purpura fulminans and disseminated intravascular coagulation. She was entubated and intraarterial blood pressure monitoring was started. Clinical and laboratory measurements showed signs of septic shock. She was treated with fluid replacement and antibiotics. Because of the septic state and subarachnoid bleeding; lumber puncture for diagnostic purposes could not be performed. Her blood pressure was high and antihypertensive drugs were needed to regulate blood pressure. Further tests to show possible agents of purpura fulminans and sepsis were first evaluated in urinary antigen test (BinaxNOW[R]) with Streptococcus pneumoniae antigen positivity. Also blood culture results revealed a penicillin resistance species of Streptococcus pneumoniae in the antibiogram. Other antibiotics such cephalosporins were effective for this agent. After adequate supportive treatment and antibiotherapy; the treatment was terminated within 2 weeks and she completely recovered in 3 months with continued physical therapy to prevent spasticity on both lower extremities, which was represented as a sequel after subarachnoid bleeding.
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Our patient was diagnosed as invasive pneumococcal disease and purpura fulminans. Even if they are unusual agents in purpura fulminans, pneumococci are responsible for this disease if there is an underlying condition, and it is rarely described in healthy infants (6). In the presented patient the main risk factor was the Mondini's dysplasia, which is a developmental anomaly of the middle ear characterized by cochlear malformation with dilation of the vestibular aquaduct, vestibule and ampullar ends of the semicircular canals (7). She was operated on and an implant was placed in her left ear three years previously. the blood brain barrier was intact and there was no proof of cerebrospinal fluid (CSF) leakage even on examination after recovery. Also there was no proof of immunodeficiency; her immunoglobulin levels and antibody responses were consistent with her age group.
Like other temporal bone defects or other congenital anatomic causes of recurrent meningitis; in Mondini dysplasia the CSF leaks into the middle ear through a deficient foramen ovale, which is a risk factor for recurrent pneumococcal infections (8). Therefore prophylaxis plays an important role for preventing sequelae through central nervous system infections. However, the efficacy of prophylactic use of antibiotics in preventing meningitis in children could not be proved and might possibly do harm by increasing the risk of infection due to antibiotic-resistant organisms. Such use of antibiotics has been more often been recommended for patients with complement or immunoglobulin subclass deficiency.
Another preventive method is to vaccinate the patients. Meningococcal vaccine is recommended for patients who have terminal complement component deficiency and for those who have asplenia, but the vaccine's clinical efficacy has not been documented in such patients. Although the heptavalent pneumococcal conjugate vaccine is not sufficient for older children and adults in high risk groups, immunization with 23-valent polysaccaride vaccine is recommended for older children with recurrent infections (7). In our case, prophylactic penicillin was administered monthly before she was admitted to our hospital and she was vaccinated once with 23-valent polysaccaride vaccine. The question of whether it is possible to protect the patients with penicillin prophylaxis is controversial, because the responsible species of Streptococcus pneumoniae in our case is also resistant to penicillin. Even there is no certain evidence, it can be hypothesized that previously used penicillin prophylaxis could be the reason for infection with a resistant strain and this strain is already the cause of invasive disease.
In conclusion we suggest that patients presenting with purpura fulminans should be examined for pneumococcal disease, especially if there is an underlying condition like Mondini's dysplasia.
Gelis Tarihi: 10.11.2010
Kabul Tarihi: 10.01.2011
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Basak Yildiz, Doga Ceren Tekguc, Dilek Yilmaz Ciftdogan, Fadil Vardar
Department of Pediatric Infectious Diseases, Faculty of Medicine, Ege Unive rsity, Izmir, Turkey
Dr. Basak Yildiz Department of Pediatric Infectious Diseases, Faculty of Medicine, Ege University, 35100, Izmir, Turkey
Phone.: +90 232 390 10 01
Fax: +90 232 390 13 57