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Chromobacterium violaceum necrotizing fasciitis: a case report and review of the literature.

Abstract

Necrotizing fasciitis is a severe, rapidly progressive infection of the subcutaneous tissue that causes significant destruction. It is rarely encountered in the pediatric population. We describe the case of a 14-year-old boy who was diagnosed with Chromobacterium violaceum necrotizing fasciitis and subsequently found to have autosornal recessive chronic granulornatous disease.

Introduction

Necrotizing fasciitis (NF) is a serious, rapidly progressive infection of the subcutaneous tissues that is associated with significant morbidity and mortality. It is rare among children. NF is usually caused by either non-group A beta-hemolytic streptococci, Staphylococcus aureus, or coagulase-negative staphylococci. Other causative organisms include Bacteroides, Peptostreptococcus, Klebsiella, Enterobacter, Proteus, Clostridiurn, Vibrio, Serratia, and Salmonella species, Escherichia coli, Pseudornonas aeruginosa, and fungi. (1)

To the best of our knowledge, no case of NF caused by Chrornobacterium violaceum infection has been previously reported in the literature. We describe what we believe is the first such documented case, which occurred in a young teenager who was subsequently diagnosed with autosomal recessive chronic granulomatous disease.

Case report

A 14-year-old boy with a history of attention-deficit/ hyperactivity disorder and allergic rhinitis presented to the emergency department with poorly localized central facial pain and a fever (maximum temperature: 103.6[degrees]F) of 1 day's duration. The previous day, he had gone swimming in a lake, and he recalled coming into contact with clay and rocks at the lake's bottom. His surgical history was remarkable for a lymph node resection to treat presumed cat scratch disease when he was 2 years old and urethral surgery to correct an abnormal urinary stream at 5 years of age. His immunizations were up to date. He was allergic to amoxicillin/clavulanate. The remainder of his medical history, review of systems, family history, and social history were unremarkable.

The patient was initially diagnosed with acute bacterial sinusitis and discharged on oral clindamycin. However, his facial pain worsened, and he developed persistent sneezing with copious clear rhinorrhea. He presented to his primary care physician, who noted erythema and edema over the left nasal ala. Maxillofacial computed tomography (CT) demonstrated findings consistent with maxillary sinusitis.

The patient was prescribed a course of cefuroxime and oxymetazoline nasal spray for presumed persistent bacterial sinusitis. However, his facial pain, erythema, and edema persisted, and he developed tenderness over his maxillary incisors and canines (figure, A). He was admitted to an outside hospital and started on intravenous ceftriaxone, clindamycin, and fluconazole, still with a presumed diagnosis of complicated bacterial sinusitis.

[FIGURE OMITTED]

Despite 2 days of IV antibiotics, the patient's symptoms worsened. He developed mobility of the left maxillary central and lateral incisors with surrounding purulence. The left facial swelling and erythema had spread to include the left orbit, maxilla, and palatal mucosa (figure, B).

The patient was taken to the operating room by the oral and maxillofacial surgery service. It is important to note that immediately prior to surgery, there was a noticeable increase in the dark discoloration of the left nasal ala (figure C), and the discoloration and necrosis progressively worsened during the operation itself. Intraoperatively, purulence around the maxillary incisors and a thick, boggy sinus mucosa were encountered. The left maxillary incisors were extracted, and soft-tissue biopsies were obtained.

Given the rapid progression of the patient's infection despite the broad-spectrum antibiotics, he was transferred to a tertiary care facility for further evaluation and treatment. In light of concerns about possible NF, the pediatric infectious disease service started him on vancomycin, ceftazidime, gentamicin, clindamycin, and amphotericin B, and the ENT service was consulted.

Laboratory findings upon admission were significant for leukocytosis (white cell count: 20.2 x [10.sup.9]/L), mild anemia (hemoglobin: 10.2 g/dl; hematocrit 28.7%), hyponatremia (sodium: 129 mEq/L), hypocalcemia (calcium: 8.3 mg/dl), and coagulopathy (prothrombin time: 22.9 sec; international normalized ratio: 2.0; activated partial thromboplastin time: 31.7 sec).

Shortly after arrival, the patient became somnolent, and a marked progression of his facial erythema and edema was observed. He was emergently taken to the operating room by the ENT team. Intraoperatively, a partially necrotic left nasal ala and an area of denuded septal mucosa were treated with aggressive debridement. The surgery resulted in removal of the left nasal ala and a portion of the soft tissue crossing the nasolabial fold (figure, D).

Over a period of 10 minutes, healthy bleeding tissue within the freshly debrided wound quickly progressed to dusky, nonviable, necrotic tissue. Subsequent debridements were performed until healthy tissue was encountered. Intraorally, teeth 11 and 12 were loose and extracted. The overlying gingiva was easily stripped from the associated maxillary alveolar ridge because there was no underlying periosteum, and the maxilla was partially resected because it was also nonviable (figure, E).

Multiple wound and tissue cultures and biopsies were obtained intraoperatively. Pathology of the specimens demonstrated severe multifocal acute inflammation and necrosis consistent with NE Postoperatively, the patient was admitted to the pediatric intensive care unit for management. Shortly thereafter, biopsy and blood cultures from the outside hospital revealed the presence of C violaceum sensitive to ciprofioxacin, ertapenem, gentamicin, and metronidazole. Thus, ciprofloxacin was added to the patient's antibiotic regimen. The cultures obtained at the tertiary care facility also grew C violaceum sensitive to piperacillin/tazobactam, imipenem, cefepime, gentamicin, tobramycin, and trimethoprim/ sulfamethoxazole (TMP/SMZ).

To further evaluate the extent of the patient's infection, CT scans of the head, chest, and abdomen were obtained. These studies were significant for ground-glass opacities in both lungs and prominent retroperitoneal lymphadenopathy. Alumbar puncture and echocardiography ruled out meningitis and endocarditis, respectively.

The patient was extubated on postoperative day 3, and his antibiotic regimen was switched to imipenem and gentamicin in response to confirmation of the C violaceum and its sensitivities. Although the patient did not have a history of immunodeficiency or recurrent infections, the C violaceum infection prompted a neutrophil function study, the results of which were consistent with autosomal recessive chronic granulomatous disease.

Because the patient's surgical defects (figure, F) prevented him from maintaining adequate oral nutrition, he underwent laparoscopic placement of a gastrostomy tube. After a 2-week hospitalization, he was discharged home to complete a 6-week course of imipenem and gentamicin. Thereafter, he has been maintained on itraconazole, TMP/SMZ, and interferon gamma-1b, and he has had no further significant infections. During the 3 years since his initial surgery, he has undergone multiple reconstructive procedures and a bone graft, and he is awaiting placement of dental implants.

Discussion

C violaceum is a ubiquitous, saprophytic, anaerobic, gram-negative bacillus that lives in soil and stagnant bodies of water in tropical and subtropical climates. (2-5) Its colonies are smooth, convex, and violet as a result of the production of the pigment violacein. (2) Despite the fact that it produces a lipopolysaccharide endotoxin, (4) C violaceum rarely causes disease in humans. Infrequently, however, the bacterium gains entry through abrasions or lesions in the skin or conjunctiva (6) of susceptible persons and disseminates locally and hematogenously, causing severe and often fatal infections.

C violaceum has previously been reported to cause abscesses (liver, lung, kidney, brain, spleen, abdomen, and skin), lymphadenitis, cellulitis, sepsis, and meningitis. Since it was first described in humans in 1927, approximately 150 cases of human infection have been reported worldwide. (4,5,7) Reports have originated from all continents except Antarctica.

To the best of our knowledge, ours is the first case of C violaceum NF to be reported in the literature. Classically, NF is associated with gram-positive (staphylococcal and streptococcal) organisms, but it is important to consider other microorganisms, including C violaceum.

C violaceum infections are associated with significant morbidity and mortality. Case series have reported mortality rates ranging from 53 to 80%, with higher mortality noted in patients with disseminated disease. (2,3,6) Our patient, who was a previously healthy teenager, spent approximately 2 weeks critically ill in the hospital. He lost significant facial soft and bony tissues and dentition, and he required placement of a gastrostomy tube. Although he has continued to do well 3 years after this initial infection, reconstructive procedures are still ongoing.

While prompt diagnosis and appropriate surgical intervention are imperative, this case also illustrates the importance of selecting the appropriate antimicrobial agents. Previous reports of C violaceum isolates demonstrate uniform susceptibility to chloramphenicol, gentamicin, fluoroquinolones, tetracyclines, imipenem, TMP/SMZ, and semisynthetic penicillins. (2) Isolates are often resistant to cephalosporins, penicillin, ampicillin, and antistaphylococcal penicillins, and they are universally resistant to vancomycin and rifampin. (2)

Despite the initial broad-spectrum antibiotic coverage administered to our patient, it was not until the cultures from the outside hospital reported C violaceum that an effective antibiotic regimen was started. In a review by Sirinavin et al, fatality rates were 24% for patients with C violaceum infections who received effective antimicrobial agents and 100% for those who received ineffective therapy. (6)

Our case involved a 14-year-old boy with no known history of immunodeficiency who was ultimately diagnosed with autosomal recessive chronic granulomatous disease after C violaceum infection. Chronic granulomatous disease is a rare hereditary immunodeficiency. It is characterized by recurrent life-threatening bacterial and fungal infections and granuloma formation secondary to a mutation in the nicotinamide adenine dinucleotide phosphate oxidase complex. (8) Such mutations prevent phagocyte respiratory burst and destruction of intracellular organisms.

Recurrent infections such as pneumonia, abscesses, osteomyelitis, sepsis, lymphadenitis, and cellulitis are characteristic in patients with chronic granulomatous disease. (8) Catalase-positive bacteria, gram-negative Enterobacteriaceae, and fungi are common pathogens in patients with chronic granulomatous disease. (8) It is interesting that C violaceum has a predilection for patients with disorders of neutrophil function. (2,3)

In response to our patient's diagnosis of chronic granulomatous disease, he was started on antimicrobial therapy to prevent infection. This therapy has been shown to reduce the incidence of serious bacterial infections, the number of surgical interventions, and the number of hospitalizations in patients with chronic granulomatous disease. (9) Ultimately, this diagnosis and appropriate prophylactic treatment will decrease our patient's morbidity, improve his quality of life, and increase his chances for survival.

References

(1.) Leung AK, Eneli I, Davies HD. Necrotizing fasciitis in children. Pediatr Ann 2008;37(10):704-10.

(2.) Fisher RG. Miscellaneous non-Enterobacteriaceae fermentative bacilli. In: Feigen RD, Cherry JD, Demmler-Harrison GJ, Kaplan SL, eds. Textbook of Pediatric Infectious Diseases. 6th ed. Philadelphia: Saunders; 2009:1632-4.

(3.) Yang CH, Li YH. Chromobacterium violaceum infection: A clinical review of an important but neglected infection. J Chin Med Assoc 2011;74(10):435-41.

(4.) Martinez P, Mattar S. Fatal septicemia caused by Chromobacterium violaceum in a child from Columbia. Rev Inst Med Trop Sao Paulo 2007;49(6):391-3.

(5.) Shao PL, Hsueh PR, Chang YC, et al. Chromobacterium violaceum infection in children: A case of fatal septicemia with nasopharyngeal abscess and literature review. Pediatr Infect Dis J 2002;21 (7):707-9.

(6.) Sirinavin S, Techasaensiri C, Benjaponpitak S, et al. Invasive Chromobacterium violaceum infection in children: Case report and review. Pediatr Infect Dis J 2005;24(6):559-61.

(7.) Macher AM, Casale TB, Fauci AS. Chronic granulomatous disease of childhood and Chromobacterium violaceum infections in the southeastern United States. Ann Intern Med 1982;97(1):51-5.

(8.) Goldblatt D, Thrasher AJ. Chronic granulomatous disease. Clin Exp Immunol 2000;122(1):1-9.

(9.) Weening RS, Kabel R Pijman R Roos D. Continuous therapy with sulfamethoxazole-trimethoprim in patients with chronic granulomatous disease. J Pediatr 1983;103(1):127-30.

Jonathan K. Seigel, MD; Michael E. Stadler, MD; Jennifer L. Lombrano, DDS; Jeffrey S. Almony, MD, DDS; Marion E. Couch, MD, PhD; Thomas H. Belhorn, MD, PhD

From the Department of Pediatrics, Duke University School of Medicine, Durham, N.C. (Dr. Seigel); the Department of Otolaryngology & Communication Sciences, Medical College of Wisconsin, Milwaukee (Dr. Stadler); private dental practice, Anchorage, Alaska (Dr. Lombrano); the Dental Clinic, Womack Army Medical Center, Fort Bragg, N.C. (Dr. Almony); the Division of Otolaryngology/Head and Neck Surgery, Department of Surgery, University of Vermont, Burlington (Dr. Couch); and the Department of Pediatrics, University of North Carolina School of Medicine, Chapel Hill (Dr. Belhorn). The case described in this article occurred at the Womack Army Medical Center and the University of North Carolina.

Corresponding author: Thomas H. Belhorn, MD, PhD, UNC Pediatrics-Infectious Diseases, 2242 Genome Sciences, CB #7231, 250 Bell Tower Dr., Chapel Hill, NC 27599-7231. Email: tom_belhorn@ med.unc.edu
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Title Annotation:ORIGINAL ARTICLE
Author:Seigel, Jonathan K.; Stadler, Michael E.; Lombrano, Jennifer L.; Almony, Jeffrey S.; Couch, Marion E
Publication:Ear, Nose and Throat Journal
Article Type:Case study
Date:Nov 1, 2012
Words:2040
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