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Case report: Candida and Herpes Simplex Virus supraglottitis in a patient with cyclical neutropenia
International Journal of Pediatric Otorhinolaryngology Extra 7 (2012) 85–88
Contents lists available at SciVerse ScienceDirect
International Journal of Pediatric Otorhinolaryngology Extra journal homepage: www.elsevier.com/locate/ijporl
Case report
Case report: Candida and Herpes Simplex Virus supraglottitis in a patient with cyclical neutropenia Yvonne L. Richardson a, Amy E. Lawrason b, Tulio A. Valdez b,c,* a
Tuba City Regional Health Care Corporation, 167 North Main Street, PO Box 600, Tuba City, AZ 86045, United States University of Connecticut, Department of Surgery, Division of Otolaryngology, Farmington, CT, United States c Connecticut Children’s Medical Center, Hartford, CT, United States b
A R T I C L E I N F O
A B S T R A C T
Article history: Received 29 November 2011 Accepted 24 January 2012
In the post-Haemophilus influenza type b vaccination era, the etiology of pediatric supraglottitis has been evolving. In addition to traditional bacteriology, viral and fungal infectious agents must also be considered, particularly in immunocompromised patients. We report the case of a 15 year old female with cyclic neutropenia, a rare hematologic disorder, who presented with airway compromise secondary to Candida and Herpes Simplex Virus 1 (HSV1) supraglottitis. A review of viral causes of supraglottitis is also presented. ß 2012 Published by Elsevier Ireland Ltd.
Keywords: Neutropenia Supraglottitis Candida HSV Fungal Epiglottitis
1. Introduction
2. Methods
Cyclic neutropenia is a rare hematologic disorder characterized by regularly recurring episodes of neutropenia lasting typically 3– 6 days, and occurring approximately every 3 weeks [1]. Supraglottitis is inflammation of the laryngeal structures above the level of the vocal folds, including epiglottal cartilage and arytenoids, and can rapidly lead to life-threatening airway obstruction. Traditionally Haemophilus influenza type b (Hib), was the most common cause of epiglottitis, however the introduction of the Hib vaccine has resulted in a dramatic decrease in the incidence of epiglottitis. Despite this decrease, the most common cause continues to be bacterial, with only rare cases of fungal and viral supraglottitis reported in immunocompromised patients. We present a case of a 15 year old female with cyclic neutropenia who developed airway compromise secondary to acute Candida and HSV1 supraglottitis during a neutropenic episode. To the best of our knowledge this is the first case of supraglottitis reported in a patient with this hematological condition.
Approval was obtained from the Connecticut Children’s Institutional Review Board. A 15 year old female with a known history of cyclic neutropenia presented with a 2 day history of sore throat, fevers, and a 4–5 day history of oral and genital ulcerations. At presentation, she had an absolute neutrophil count of 17 cells/ml. She was transferred to our institution from an outside hospital secondary to hypoxia and increased work of breathing requiring supplemental oxygen. The patient had a family history of cyclic neutropenia with her mother and sister being affected by this condition. Upon arrival the patient was started on broad spectrum antibiotics, an antiviral, as well as granulocyte colony-stimulating factor (G-CSF). Her lateral neck X-rays showed a thickened epiglottis (thumb sign) and aryepiglottic folds (see Fig. 1). Otolaryngology was consulted, and on examination she had a slightly increased work of breathing, hot potato voice, inspiratory stridor and inability to tolerate supine position. Flexible nasopharyngoscopy revealed an erythematous epiglottis with exudate and significant arytenoid edema. The true vocal folds could not be visualized secondary to supraglottic edema. Steroids were initiated. Due to airway compromise, the patient was taken immediately to the operating room for intubation and cultures as well as biopsy of the ulcerative lesions in the tongue.
* Corresponding author. Tel.: +1 928 283 2699; fax: +1 928 283 2795. E-mail address: [email protected] (T.A. Valdez). 1871-4048/$ – see front matter ß 2012 Published by Elsevier Ireland Ltd. doi:10.1016/j.pedex.2012.01.004
Y.L. Richardson et al. / International Journal of Pediatric Otorhinolaryngology Extra 7 (2012) 85–88
86
Fig. 1. Lateral neck X-ray with evidence of thumb sign of the epiglottis.
Fig. 2. Swelling of the epiglottis with evidence of Candida infection involving the tongue and supraglottic structures after securing airway with endotracheal tube.
Intraoperatively, spontaneous ventilation was maintained while general mask anesthesia was induced. Direct laryngoscopy showed multiple oral and palatal lesions, an extremely friable base of tongue, and a very erythematous and edematous epiglottis with clumps of white material. The patient was intubated under direct visualization with a 5.5 endotracheal tube over an endoscope (see Fig. 2). Cultures from the epiglottis were positive for Herpes Simplex Virus 1 (HSV1) and Candida albicans. Fluconazole was added to her treatment. Repeat examination 4 days later showed improvement in supraglottic edema, and she was extubated without incident. Her absolute neutrophil count recovered, and she was discharged home 2 days after extubation.
Staphylococcus aureus, Klebsiella pneumoniae, Haemophilus parainfluenzae, and beta-hemolytic streptococci [8]. Vigilance must remain high to diagnose impending airway obstruction. We report a case of concomitant acute HSV1 and Candida supraglottitis in a patient with cyclic neutropenia. Candida supraglottitis is rare and usually opportunistic. It is largely reported in the immunocompromised setting, typically in patients with HIV [9,10], hematologic malignancies [11], and/or chemotherapy induced granulocytopenia [12]. Additionally, candidiasis may be an acute complication of an underlying laryngeal malignancy [13]. Findings of oropharyngeal thrush may not be present, and supraglottitis should be suspected in the immunocompromised patient with refractory sore throat. Only one case of acute Candida supraglottitis has been reported in a non-debilitated, healthy patient [14]. Diagnosis requires a positive culture and histopathologic examination. Special staining with periodic acidSchiff and Grocot–Gomori may reveal hyphae or mycelia in the mucosa [6]. Systemic and local anti-fungal treatment should be initiated if cultures are positive. Viral supraglottitis is an extremely uncommon cause of supraglottis, with only a few cases of documented HSV supraglottitis reported in the literature [15–17]. In non-emergent situations, culture is the most sensitive and specific method of diagnosis, with ELISA being a more rapid diagnostic test in emergency conditions. In cases where viral numbers are low, ELISA may be more sensitive than culture [16]. In our patient, HSV1 was diagnosed by positive culture. Use of steroids during HSV1 infection is controversial due to their immune suppressive properties. In the setting of suspected viral infection, it may be wise to limit steroid use once the airway is secured. Other viral etiologies reported include parainfluenza virus, varicella-zoster virus, human immunodeficiency virus, and Epstein-Barr virus [18]. Immunocompromised [19], neonatal [20], and burn patients [3] are at risk for disseminated disease (Table 1). This case illustrates that a short period of neutropenia as seen in cyclic neutropenia can precipitate an opportunistic laryngeal fungal infection and viral infection. In our patient, oral and genital ulcerations preceded severe odynophagia and airway compromise. To the best of our knowledge, this is the first report of concomitant HSV and Candida supraglottitis in the setting of cyclic neutropenia.
3. Discussion Cyclic neutropenia is a rare hematological disorder with an estimated incidence of 1–2 per million [2]. This condition affects neutrophil production and presents with regular oscillations of numbers of circulating neutrophils from near normal to severely low levels. The periodicity has been described to be 19–21 days, with the duration of neutropenia lasting 3–6 days [3]. Symptom severity varies and symptoms tend to become milder in adulthood. Patients typically present in their first year of life with regularly occurring episodes of anorexia, malaise, oral ulcerations, pharyngitis, headaches, myalgias, fever, and cervical lymphadenopathy [4]. Incidence can be sporadic or inherited, with the familial cases typically showing an autosomal dominant form of inheritance [5]. Diagnosis is made using complete blood count with differential at least three times weekly for two months. Treatment with G-CSF may lessen the severity of the disease [1]. Heterozygous mutations in the gene encoding neutrophil elastase (ELA2) have been associated with both cyclic neutropenia as well as another disease called Severe Congenital Neutropenia. [6] In addition, several identified mutations within the ELA2 gene have been correlated with more severe phenotypes, and raises the possibility of using molecular screening for diagnostic purposes [6]. Historically the most common cause of supraglottitis was H. influenza type b (Hib). The introduction of the Hib vaccine in 1985, resulted in a decline in the incidence of supraglottitis in all geographic areas [7]. In the postvaccination era the most common infectious agents causing epiglottitis include group A Streptococcus,
Reference
Age
Sex
Comorbidities
Symptoms
Laryngoscopy
Organism
Treatment
Outcome
[21]
31
M
None
Methylprednisolone, acyclovir
Full resolution
57
M
Hypertension, hyperlipidemia
2+ right tonsil, edema and erythema right arytenoids with patchy mucosal ulceration Erythema of epiglottis, edema of arytenoids, VC, and postcricoid area
HSV1 and HSV2
[18]
Parainfluenza virus type 2
Elective intubation, supportive measures
Full resolution
[16]
18
F
None
Ceftriaxone, steroids
Full resolution
31
F
Hypothyroid
Edema and fibrinous exudates over left AE fold, decreased mobility left true VC Edematous soft palate, throat, uvula, pharynx, and supraglottis with a smooth polypoid mass
HSV
[17]
Right sore throat, odynophagia, globus, mild dysphonia Severe sore throat, dysphagia, dysphonia, dry cough, rhinorrhea, fatigue, conjunctivitis, fevers/chills, stridor Fever, rhinorrhea, cough, progressive dysphagia & dysphonia Bizarre behavior, muffled voice, drooling
HSV 1 and methicillin resistant Staphylococcus aureus
Intubation, ceftriaxone, clindamycin. Then, tracheostomy on hospital day #9 and acyclovir, vancomycin
[15]
44
F
Asthma, schizophrenia, cocaine abuse 3 young infants (1) 4 months old (2) 6 months old (3) 14 week
Agitation, tachypnea, shortness of breath
HSV 1
Fever, rhinorrhea, dysphonia, brassy cough, refusal to drink, reluctance to speak, intermittent stridor Fever, brassy cough, lethargy, stridor
Multiple intubation prior; edema and ulcerations false cords, AE folds, polypoid lesion epiglottis (1) Marked erythema of epiglottis, arytenoids, AE folds, and false cords (2) No laryngoscopy (3) Epiglottis, AE folds, surrounding tissue red and edematous Normal epiglottis, marked inflammation of AE folds and false VC
Parainfluenza type 3
Intubation 2 (accidental extubation 1), tracheotomy, IV acyclovir 2 weeks (1) Nasotracheal intubation, IV ampicillin and chloramphenicol (2) IV ampicillin (3) intubation, IV ampicillin, IV chloramphenicol, IV decadron Intubation, IV chloramphenicol
Full resolution after starting acyclovir and vancomycin, successfully decannulated. Full resolution, decannulation day 21 (1) Full resolution (2) Full resolution (3) Full resolution
Influenza type b virus
Intubation, IV chloramphenicol
[22]
(1) N/A (2) M (3) M
[23]
1.75
F
None
[23]
3.5
M
Mild asthma
Viral prodrome, lack of fever, stomatitis
AE, aryepiglottic; VC, vocal cord; IV, intravenous; HSV, Herpes Simplex Virus.
Inflammation of epiglottis, false vocal cords, AE folds
(1) Parainfluenza (2) H. influenzae (3) Blood cultures negative
Extubated at 100 h. Full resolution at 1 month Extubated after 7 days
Y.L. Richardson et al. / International Journal of Pediatric Otorhinolaryngology Extra 7 (2012) 85–88
Table 1 Viral etiologies of supraglottitis reported in the literature.
87
88
Y.L. Richardson et al. / International Journal of Pediatric Otorhinolaryngology Extra 7 (2012) 85–88
4. Conclusion In the post Hib vaccination age, acute supraglottitis has become less common in the pediatric population. Vigilance must remain high for this serious condition, particularly in the immunocompromised population. If supraglottitis is diagnosed, viral and fungal causes must be considered in the differential diagnosis, in addition to traditional bacterial etiology. Conflict of interest statement The authors have no financial or other conflicts of interest to disclose. References [1] D.C. Dale, A.A. Bolyard, A. Aprikyan, Cyclic neutropenia, Semin. Hematol. 39 (2) (2002) 89–94. [2] D.C. Dale, ELANE-related neutropenia, in: R.A. Pagon, T.D. Bird, C.R. Dolan, K. Stephens (Eds.), GeneReviews [Internet], University of Washington, Seattle (WA), 2002, Available at: http://www.ncbi.nlm.nih.gov.online.uchc.edu/books/NBK1533/ (accessed 23.08.11). [3] A. Peppercorn, L. Veit, C. Sigel, et al., Overwhelming disseminated herpes simplex virus type 2 infection in a patient with severe burn injury: case report and literature review, J. Burn Care Res. 31 (3) (2010) 492–498. [4] D.C. Dale, W.P. Hammond, Cyclic neutropenia: a clinical review, Blood Rev. 2 (3) (1988) 178–185. [5] S.M. Stein, D.C. Dale, Molecular basis and therapy of disorders associated with chronic neutropenia, Curr. Allergy Asthma Rep. 3 (5) (2003) 385–388. [6] C. Bellanne´-Chantelot, S. Clauin, T. Leblanc, et al., Mutations in the ELA2 gene correlate with more severe expression of neutropenia: a study of 81 patients from the French Neutropenia Register, Blood 103 (11) (2004) 4119–4125.
[7] B.A. Senior, D. Radkowski, C. MacArthur, R.C. Sprecher, D. Jones, Changing patterns in pediatric supraglottitis: a multi-institutional review, 1980 to 1992, Laryngoscope 104 (11 Pt 1) (1994) 1314–1322. [8] S.E. Sobol, S. Zapata, Epiglottitis and croup, Otolaryngol. Clin. North Am. 41 (3) (2008) 551–566, ix. [9] N. Sharma, D.M. Berman, G.B. Scott, G. Josephson, Candida epiglottitis in an adolescent with acquired immunodeficiency syndrome, Pediatr. Infect. Dis. J. 24 (1) (2005) 91–92. [10] L. Israel, M. Sadigh, Candida epiglottitis in an adult with acquired immunodeficiency syndrome treated with oral fluconazole, J. Laryngol. Otol. 109 (4) (1995) 337–339. [11] A.C.W. Lee, S.Y. Lam, Life threatening acute epiglottitis in acute leukemia, Leuk. Lymphoma 43 (3) (2002) 665–667. [12] T.J. Walsh, W.C. Gray, Candida epiglottitis in immunocompromised patients, Chest 91 (4) (1987) 482–485. [13] N. Hirshoren, R. Eliashar, J.M. Weinberger, Candida epiglottitis: a rare emergent condition – appearance, treatment and pitfalls, Eur. J. Intern. Med. 19 (8) (2008) e84–e85. [14] R.S. Haberman, M.E. Becker, C.N. Ford, Candida epiglottitis, Arch. Otolaryngol. 109 (11) (1983) 770–771. [15] A.J. D’Angelo, S. Zwillenberg, J.P. Olekszyk, F.I. Marlowe, J. Mobini, Adult supraglottitis due to herpes simplex virus, J. Otolaryngol. 19 (3) (1990) 179–181. [16] U.M. Musharrafieh, G.F. Araj, N.S. Fuleihan, Viral supraglottitis in an adult: a case presentation and literature update, J. Infect. 39 (2) (1999) 157–160. [17] V. Bengualid, S. Keesari, V. Kandah, D. Gitler, J. Berger, Supraglottitis due to herpes simplex virus type 1 in an adult, Clin. Infect. Dis. 22 (2) (1996) 382–383. [18] K.J. Vigil, V.E. Mulanovich, R.F. Chemaly, et al., Severe parainfluenza virus type 2 supraglottitis in an immunocompetent adult host: an unusual cause of a paramyxoviridae viral infection, J. Intern. Med. 265 (3) (2009) 397–400. [19] G.W. Herget, U.N. Riede, A. Schmitt-Gra¨ff, et al., Generalized herpes simplex virus infection in an immunocompromised patient – report of a case and review of the literature, Pathol. Res. Pract. 201 (2) (2005) 123–129. [20] C.A. Jones, K.S. Walker, N. Badawi, Antiviral agents for treatment of herpes simplex virus infection in neonates, Cochrane Database Syst. Rev. (3) (2009) CD004206. [21] A. Lotfizadeh, D.K. Chhetri, Viral supraglottitis in an adult, Ear Nose Throat J. 88 (2) (2009) 768–770. [22] J.L. Goldhagen, Supraglottitis in three young infants, Pediatr. Emerg. Care 5 (3) (1989) 175–177. [23] T. Grattan-Smith, M. Forer, H. Kilham, J. Gillis, Viral supraglottitis, J. Pediatr. 110 (3) (1987) 434–435.
Contents lists available at SciVerse ScienceDirect
International Journal of Pediatric Otorhinolaryngology Extra journal homepage: www.elsevier.com/locate/ijporl
Case report
Case report: Candida and Herpes Simplex Virus supraglottitis in a patient with cyclical neutropenia Yvonne L. Richardson a, Amy E. Lawrason b, Tulio A. Valdez b,c,* a
Tuba City Regional Health Care Corporation, 167 North Main Street, PO Box 600, Tuba City, AZ 86045, United States University of Connecticut, Department of Surgery, Division of Otolaryngology, Farmington, CT, United States c Connecticut Children’s Medical Center, Hartford, CT, United States b
A R T I C L E I N F O
A B S T R A C T
Article history: Received 29 November 2011 Accepted 24 January 2012
In the post-Haemophilus influenza type b vaccination era, the etiology of pediatric supraglottitis has been evolving. In addition to traditional bacteriology, viral and fungal infectious agents must also be considered, particularly in immunocompromised patients. We report the case of a 15 year old female with cyclic neutropenia, a rare hematologic disorder, who presented with airway compromise secondary to Candida and Herpes Simplex Virus 1 (HSV1) supraglottitis. A review of viral causes of supraglottitis is also presented. ß 2012 Published by Elsevier Ireland Ltd.
Keywords: Neutropenia Supraglottitis Candida HSV Fungal Epiglottitis
1. Introduction
2. Methods
Cyclic neutropenia is a rare hematologic disorder characterized by regularly recurring episodes of neutropenia lasting typically 3– 6 days, and occurring approximately every 3 weeks [1]. Supraglottitis is inflammation of the laryngeal structures above the level of the vocal folds, including epiglottal cartilage and arytenoids, and can rapidly lead to life-threatening airway obstruction. Traditionally Haemophilus influenza type b (Hib), was the most common cause of epiglottitis, however the introduction of the Hib vaccine has resulted in a dramatic decrease in the incidence of epiglottitis. Despite this decrease, the most common cause continues to be bacterial, with only rare cases of fungal and viral supraglottitis reported in immunocompromised patients. We present a case of a 15 year old female with cyclic neutropenia who developed airway compromise secondary to acute Candida and HSV1 supraglottitis during a neutropenic episode. To the best of our knowledge this is the first case of supraglottitis reported in a patient with this hematological condition.
Approval was obtained from the Connecticut Children’s Institutional Review Board. A 15 year old female with a known history of cyclic neutropenia presented with a 2 day history of sore throat, fevers, and a 4–5 day history of oral and genital ulcerations. At presentation, she had an absolute neutrophil count of 17 cells/ml. She was transferred to our institution from an outside hospital secondary to hypoxia and increased work of breathing requiring supplemental oxygen. The patient had a family history of cyclic neutropenia with her mother and sister being affected by this condition. Upon arrival the patient was started on broad spectrum antibiotics, an antiviral, as well as granulocyte colony-stimulating factor (G-CSF). Her lateral neck X-rays showed a thickened epiglottis (thumb sign) and aryepiglottic folds (see Fig. 1). Otolaryngology was consulted, and on examination she had a slightly increased work of breathing, hot potato voice, inspiratory stridor and inability to tolerate supine position. Flexible nasopharyngoscopy revealed an erythematous epiglottis with exudate and significant arytenoid edema. The true vocal folds could not be visualized secondary to supraglottic edema. Steroids were initiated. Due to airway compromise, the patient was taken immediately to the operating room for intubation and cultures as well as biopsy of the ulcerative lesions in the tongue.
* Corresponding author. Tel.: +1 928 283 2699; fax: +1 928 283 2795. E-mail address: [email protected] (T.A. Valdez). 1871-4048/$ – see front matter ß 2012 Published by Elsevier Ireland Ltd. doi:10.1016/j.pedex.2012.01.004
Y.L. Richardson et al. / International Journal of Pediatric Otorhinolaryngology Extra 7 (2012) 85–88
86
Fig. 1. Lateral neck X-ray with evidence of thumb sign of the epiglottis.
Fig. 2. Swelling of the epiglottis with evidence of Candida infection involving the tongue and supraglottic structures after securing airway with endotracheal tube.
Intraoperatively, spontaneous ventilation was maintained while general mask anesthesia was induced. Direct laryngoscopy showed multiple oral and palatal lesions, an extremely friable base of tongue, and a very erythematous and edematous epiglottis with clumps of white material. The patient was intubated under direct visualization with a 5.5 endotracheal tube over an endoscope (see Fig. 2). Cultures from the epiglottis were positive for Herpes Simplex Virus 1 (HSV1) and Candida albicans. Fluconazole was added to her treatment. Repeat examination 4 days later showed improvement in supraglottic edema, and she was extubated without incident. Her absolute neutrophil count recovered, and she was discharged home 2 days after extubation.
Staphylococcus aureus, Klebsiella pneumoniae, Haemophilus parainfluenzae, and beta-hemolytic streptococci [8]. Vigilance must remain high to diagnose impending airway obstruction. We report a case of concomitant acute HSV1 and Candida supraglottitis in a patient with cyclic neutropenia. Candida supraglottitis is rare and usually opportunistic. It is largely reported in the immunocompromised setting, typically in patients with HIV [9,10], hematologic malignancies [11], and/or chemotherapy induced granulocytopenia [12]. Additionally, candidiasis may be an acute complication of an underlying laryngeal malignancy [13]. Findings of oropharyngeal thrush may not be present, and supraglottitis should be suspected in the immunocompromised patient with refractory sore throat. Only one case of acute Candida supraglottitis has been reported in a non-debilitated, healthy patient [14]. Diagnosis requires a positive culture and histopathologic examination. Special staining with periodic acidSchiff and Grocot–Gomori may reveal hyphae or mycelia in the mucosa [6]. Systemic and local anti-fungal treatment should be initiated if cultures are positive. Viral supraglottitis is an extremely uncommon cause of supraglottis, with only a few cases of documented HSV supraglottitis reported in the literature [15–17]. In non-emergent situations, culture is the most sensitive and specific method of diagnosis, with ELISA being a more rapid diagnostic test in emergency conditions. In cases where viral numbers are low, ELISA may be more sensitive than culture [16]. In our patient, HSV1 was diagnosed by positive culture. Use of steroids during HSV1 infection is controversial due to their immune suppressive properties. In the setting of suspected viral infection, it may be wise to limit steroid use once the airway is secured. Other viral etiologies reported include parainfluenza virus, varicella-zoster virus, human immunodeficiency virus, and Epstein-Barr virus [18]. Immunocompromised [19], neonatal [20], and burn patients [3] are at risk for disseminated disease (Table 1). This case illustrates that a short period of neutropenia as seen in cyclic neutropenia can precipitate an opportunistic laryngeal fungal infection and viral infection. In our patient, oral and genital ulcerations preceded severe odynophagia and airway compromise. To the best of our knowledge, this is the first report of concomitant HSV and Candida supraglottitis in the setting of cyclic neutropenia.
3. Discussion Cyclic neutropenia is a rare hematological disorder with an estimated incidence of 1–2 per million [2]. This condition affects neutrophil production and presents with regular oscillations of numbers of circulating neutrophils from near normal to severely low levels. The periodicity has been described to be 19–21 days, with the duration of neutropenia lasting 3–6 days [3]. Symptom severity varies and symptoms tend to become milder in adulthood. Patients typically present in their first year of life with regularly occurring episodes of anorexia, malaise, oral ulcerations, pharyngitis, headaches, myalgias, fever, and cervical lymphadenopathy [4]. Incidence can be sporadic or inherited, with the familial cases typically showing an autosomal dominant form of inheritance [5]. Diagnosis is made using complete blood count with differential at least three times weekly for two months. Treatment with G-CSF may lessen the severity of the disease [1]. Heterozygous mutations in the gene encoding neutrophil elastase (ELA2) have been associated with both cyclic neutropenia as well as another disease called Severe Congenital Neutropenia. [6] In addition, several identified mutations within the ELA2 gene have been correlated with more severe phenotypes, and raises the possibility of using molecular screening for diagnostic purposes [6]. Historically the most common cause of supraglottitis was H. influenza type b (Hib). The introduction of the Hib vaccine in 1985, resulted in a decline in the incidence of supraglottitis in all geographic areas [7]. In the postvaccination era the most common infectious agents causing epiglottitis include group A Streptococcus,
Reference
Age
Sex
Comorbidities
Symptoms
Laryngoscopy
Organism
Treatment
Outcome
[21]
31
M
None
Methylprednisolone, acyclovir
Full resolution
57
M
Hypertension, hyperlipidemia
2+ right tonsil, edema and erythema right arytenoids with patchy mucosal ulceration Erythema of epiglottis, edema of arytenoids, VC, and postcricoid area
HSV1 and HSV2
[18]
Parainfluenza virus type 2
Elective intubation, supportive measures
Full resolution
[16]
18
F
None
Ceftriaxone, steroids
Full resolution
31
F
Hypothyroid
Edema and fibrinous exudates over left AE fold, decreased mobility left true VC Edematous soft palate, throat, uvula, pharynx, and supraglottis with a smooth polypoid mass
HSV
[17]
Right sore throat, odynophagia, globus, mild dysphonia Severe sore throat, dysphagia, dysphonia, dry cough, rhinorrhea, fatigue, conjunctivitis, fevers/chills, stridor Fever, rhinorrhea, cough, progressive dysphagia & dysphonia Bizarre behavior, muffled voice, drooling
HSV 1 and methicillin resistant Staphylococcus aureus
Intubation, ceftriaxone, clindamycin. Then, tracheostomy on hospital day #9 and acyclovir, vancomycin
[15]
44
F
Asthma, schizophrenia, cocaine abuse 3 young infants (1) 4 months old (2) 6 months old (3) 14 week
Agitation, tachypnea, shortness of breath
HSV 1
Fever, rhinorrhea, dysphonia, brassy cough, refusal to drink, reluctance to speak, intermittent stridor Fever, brassy cough, lethargy, stridor
Multiple intubation prior; edema and ulcerations false cords, AE folds, polypoid lesion epiglottis (1) Marked erythema of epiglottis, arytenoids, AE folds, and false cords (2) No laryngoscopy (3) Epiglottis, AE folds, surrounding tissue red and edematous Normal epiglottis, marked inflammation of AE folds and false VC
Parainfluenza type 3
Intubation 2 (accidental extubation 1), tracheotomy, IV acyclovir 2 weeks (1) Nasotracheal intubation, IV ampicillin and chloramphenicol (2) IV ampicillin (3) intubation, IV ampicillin, IV chloramphenicol, IV decadron Intubation, IV chloramphenicol
Full resolution after starting acyclovir and vancomycin, successfully decannulated. Full resolution, decannulation day 21 (1) Full resolution (2) Full resolution (3) Full resolution
Influenza type b virus
Intubation, IV chloramphenicol
[22]
(1) N/A (2) M (3) M
[23]
1.75
F
None
[23]
3.5
M
Mild asthma
Viral prodrome, lack of fever, stomatitis
AE, aryepiglottic; VC, vocal cord; IV, intravenous; HSV, Herpes Simplex Virus.
Inflammation of epiglottis, false vocal cords, AE folds
(1) Parainfluenza (2) H. influenzae (3) Blood cultures negative
Extubated at 100 h. Full resolution at 1 month Extubated after 7 days
Y.L. Richardson et al. / International Journal of Pediatric Otorhinolaryngology Extra 7 (2012) 85–88
Table 1 Viral etiologies of supraglottitis reported in the literature.
87
88
Y.L. Richardson et al. / International Journal of Pediatric Otorhinolaryngology Extra 7 (2012) 85–88
4. Conclusion In the post Hib vaccination age, acute supraglottitis has become less common in the pediatric population. Vigilance must remain high for this serious condition, particularly in the immunocompromised population. If supraglottitis is diagnosed, viral and fungal causes must be considered in the differential diagnosis, in addition to traditional bacterial etiology. Conflict of interest statement The authors have no financial or other conflicts of interest to disclose. References [1] D.C. Dale, A.A. Bolyard, A. Aprikyan, Cyclic neutropenia, Semin. Hematol. 39 (2) (2002) 89–94. [2] D.C. Dale, ELANE-related neutropenia, in: R.A. Pagon, T.D. Bird, C.R. Dolan, K. Stephens (Eds.), GeneReviews [Internet], University of Washington, Seattle (WA), 2002, Available at: http://www.ncbi.nlm.nih.gov.online.uchc.edu/books/NBK1533/ (accessed 23.08.11). [3] A. Peppercorn, L. Veit, C. Sigel, et al., Overwhelming disseminated herpes simplex virus type 2 infection in a patient with severe burn injury: case report and literature review, J. Burn Care Res. 31 (3) (2010) 492–498. [4] D.C. Dale, W.P. Hammond, Cyclic neutropenia: a clinical review, Blood Rev. 2 (3) (1988) 178–185. [5] S.M. Stein, D.C. Dale, Molecular basis and therapy of disorders associated with chronic neutropenia, Curr. Allergy Asthma Rep. 3 (5) (2003) 385–388. [6] C. Bellanne´-Chantelot, S. Clauin, T. Leblanc, et al., Mutations in the ELA2 gene correlate with more severe expression of neutropenia: a study of 81 patients from the French Neutropenia Register, Blood 103 (11) (2004) 4119–4125.
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