Accepted Manuscript Title: Otologic Complications of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Author: Jeffrey M. Hotaling Andrew J. Hotaling PII: DOI: Reference:
S0165-5876(14)00186-4 http://dx.doi.org/doi:10.1016/j.ijporl.2014.03.037 PEDOT 7067
To appear in:
International Journal of Pediatric Otorhinolaryngology
Received date: Revised date: Accepted date:
8-2-2014 27-3-2014 29-3-2014
Please cite this article as: J.M. Hotaling, A.J. Hotaling, Otologic Complications of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis, International Journal of Pediatric Otorhinolaryngology (2014), http://dx.doi.org/10.1016/j.ijporl.2014.03.037 This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
*Title Page
Article Title: Otologic Complications of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis
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Authors: Jeffrey M. Hotaling, M.D. Affiliation: Loyola University Medical Center, Department of Otolaryngology – Head and Neck Surgery Address: 2160 S. First Avenue Building 105 Room 1870 LUMC Maywood, Illinois 60153 Email: [email protected]
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Andrew J. Hotaling, M.D. Affiliation: Loyola University Medical Center, Department of Otolaryngology – Head and Neck Surgery Address: 2160 S. First Avenue Building 105 Room 1870 LUMC Maywood, Illinois 60153 Email: [email protected]
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Send Proofs/Correspondence to: Andrew J. Hotaling, M.D. Email: [email protected] Phone: (708)216-4785 Address: 2160 S. First Avenue Building 105 Room 1870 LUMC Maywood, Illinois 60153
Page 1 of 8
Title: Otologic Complications of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Authors: Jeffrey M. Hotaling, M.D.; Andrew J. Hotaling, M.D.
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Abstract: This case report demonstrates the otologic complications of StevensJohnson Syndrome and Toxic Epidermal Necrolysis (SJS/TEN). A 5 year-old male was admitted to the burn unit with the diagnosis of SJS/TEN believed secondary to acetaminophen use. After resolution of the acute phase, the patient was left with a severe auricular deformity with scarring and complete occlusion of the right external auditory canal (EAC). The patient underwent meatoplasty, canaloplasty, and tympanoplasty with subsequent placement of a molded stent in order to maintain patency of his EAC. Four years later, the patient has a patent right EAC with adequate hearing.
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Keywords: Stevens-Johnson Syndrome; Toxic Epidermal Necrolysis; Ear; Ear Auricle; Complication
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Background/Introduction:
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Stevens Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are two disease entities that exist on a spectrum of severe adverse cutaneous reactions. Both are characterized by the formation of erythema and hemorrhagic erosions with lesions commonly affecting external skin and the mucous membranes of the mouth and eyes. Blister formation leads to eventual sloughing and detachment of the skin – the extent of body surface area (BSA) involvement determining whether a reaction is categorized as SJS (30% BSA). SJS and TEN are most commonly believed to be adverse immunologic reactions to drugs, including allopurinol, carbamazepine, sulfonamides, acetaminophen, sulfa drugs, non-steroidal anti-inflammatory drugs (NSAID's) and others although an association with mycoplasma pneumoniae and other infections has also been suggested1. Treatment includes removal of the offending agent, supportive care including referral to a burn unit if necessary, and the use of immunomodulating agents when indicated. Despite these measures, patients afflicted with SJS and TEN can suffer considerable morbidity and even mortality2. We present an otologic complication of SJS/TEN in a pediatric patient and its management. A comprehensive PUBMED search yielded no reported cases of otologic manifestations of SJS and TEN.
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Case Report:
Page 2 of 8
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A three-year-old male was admitted to the burn unit at Loyola University Medical Center in August of 2006 with a severe SJS-TEN reaction, presumed secondary to acetaminophen and ibuprofen. This disease process resulted in extensive skin loss involving >50% of his body surface area ultimately requiring tracheotomy and a long hospital course (Figure 1). Particular to this case, the patient had experienced composite tissue loss of his right auricle for which he had previously undergone excision and debridement with skin grafting by the burn service. After the patient’s acute issues had been resolved, the otolaryngology service was consulted for evaluation and management of the patient’s resulting auricular injury and subjectively decreased hearing on the right. On initial physical examination 14 months post-injury, significant right auricular deformity with anterior displacement of the pinna was noted. The right external auditory canal (EAC) also appeared occluded by exquisitely tender scar tissue, which limited examination at that time. The left auricle and EAC were intact but there was a middle ear effusion noted on the left. An audiogram at that time demonstrated normal thresholds in the left ear but was unable to be completed in the right ear due to patient discomfort. A more detailed examination under anesthesia confirmed complete occlusion of the right EAC, as well as left middle ear effusion for which a left pneumatic equalization tube was also placed.
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Given his complete EAC stenosis, the patient subsequently a right meatoplasty, canaloplasty, tympanoplasty and otoplasty with the goal of restoring a patent EAC. During the procedure, it was noted that the conchal cartilage had fused to the tragal cartilage, the tympanic membrane was markedly thickened, and squamous debris consistent with a canal cholesteatoma partially filled the right EAC. With the exception of a partially lateralized and scarred tympanic membrane, the middle ear appeared grossly normal with an intact ossicular chain. Due to concern for further lateralization of the tympanic membrane from the malleus causing a further conductive hearing loss, the tympanic membrane was subtotally resected with a fascial graft placed in standard underlay fashion. The conchal cartilage was also released from the tragal cartilage and fastened to the skin overlying the mastoid to prevent recurrent anterior folding of the auricle onto itself. The patient initially did well post-operatively but 3 months later developed re-stenosis of the EAC, requiring two revision meatoplasty and canaloplasty procedures. To prevent further re-stenosis, a custom, open-vented ear mold was fabricated and used to stent open the right EAC (Figure 2). This mold was worn by the patient at night and is well-tolerated. Through multiple follow-up examinations, the right EAC has remained patent with rare granulation tissue requiring debridement. The patient has a small, stable, central right tympanic membrane perforation. However, 7 years after the initial injury, the patient developed stenosis in the contralateral (left) EAC,
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with profuse granulation and narrowing at the bony-cartilaginous junction. This has been managed with debridement to date but may require further procedures if the granulation progresses in a fashion similar to the right side. Discussion:
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SJS/TEN represent two ends of a spectrum of severe adverse cutaneous reactions that can involve any epidermal or mucosal surface in the body, with mucous membranes of the eyes, mouth, nose and genitalia involved in up to 90% of cases1. In severe cases, other organ systems including the gastrointestinal, renal, and bronchopulmonary systems can also be involved. While the ophthalmologic complications of SJS/TEN including corneal scarring and loss vision are welldescribed in the literature, many otolaryngologic complications, including otologic complications, remain undescribed and, perhaps, underreported. In fact, a PubMed search using the terms “Stevens-Johnson Syndrome,” “Toxic Epidermal Necrolysis,” “Ear,” and “Otic” failed to find any articles dedicated to the otologic complications of SJS/TEN. However, in the context of the novelty of this report, an important distinction in this case is that the patient’s auricular deformity and scarring were not due to a disease process specific to the auricle and external canal, but rather to the more global immunologic process affecting the patient’s auricle.
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In fact, strictures and stenoses secondary to SJS/TEN are nothing new, with upper aerodigestive tract strictures and stenoses well-documented among the roughly 20% of patients with TEN with involvement of the tracheal and bronchial epithelium3. As it is well known that opposing raw or denuded surfaces will adhere and cicatrize, it follows that the patient’s stricture was the result of a similar process whereby opposing, denuded epithelial surfaces in the patient’s EAC scarred and granulated, thereby occluding it. An additional consideration in this case was the composite tissue loss of the patient’s auricle, leaving few landmarks to aid in the patient’s reconstruction. While these sequelae were initially managed surgically by means of meatoplasty, canaloplasty, tympanoplasty and otoplasty, the patient’s EAC rapidly re-stenosed secondary to granulation tissue, requiring two revision procedures. In order to maintain patency of the EAC and potentially abate the need for additional surgeries, a stent was molded to the patient’s EAC in order to maintain its patency. Similar to a keel in upper airway reconstruction, this stent served to prevent contact between the opposing healing epidermal surfaces, in this case preventing restenosis of the external canal. This case thereby serves as a reminder that this devastating disease can affect ANY epidermal or mucosal surface in the body, including the auricle and the external canal. The continued granulation in the patient’s right ear and the new involvement in the contralateral (left) EAC indicate the long-term sequelae of this disease which continue even after the acute
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Page 4 of 8
phase of the reaction has abated. It is therefore crucial that these patients are followed long-term and that physicians expand their armamentarium beyond traditional surgical approaches in treating SJS/TEN patients. Conclusion:
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The long term care of patients with SJS/TEN requires an interdisciplinary approach and it is important that otolaryngologists be aware of these potential and challenging otologic complications. Given the often exuberant granulation and scarring experienced by these patient’s, molded stent placement may serve as an important adjunct to surgery in the treatment of stenoses of the external auditory canal.
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References:
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1. Gerull R, Nelle M, Schaible T. Toxic epidermal necrolysis and Stevens-Johnson syndrome: A review. Crit Care Med. 2011;39(6):1521-32. 2. Letko E, Papaliodis DN, Papaliodis GN, Daoud YJ, Ahmed AR, Foster CS. Stevens-Johnson syndrome and toxic epidermal necrolysis: a review of the literature. Ann Allergy Asthma Immunol. 2005;94(4):419-36. 3. Mockenhaupt M. The current understanding of Stevens-Johnson syndrome and toxic epidermal necrolysis. Expert Rev Clin Immunol. 2011;7(6):803-13.
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Figure 1
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Figure 2
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Figure 1: 3 year-old patient upon admission to burn ICU after sustaining a severe SJSTEN reaction involving >50% of his body surface area.
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Figure 2: Patient wearing his molded, open-vented external auditory canal stent.
Page 8 of 8
S0165-5876(14)00186-4 http://dx.doi.org/doi:10.1016/j.ijporl.2014.03.037 PEDOT 7067
To appear in:
International Journal of Pediatric Otorhinolaryngology
Received date: Revised date: Accepted date:
8-2-2014 27-3-2014 29-3-2014
Please cite this article as: J.M. Hotaling, A.J. Hotaling, Otologic Complications of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis, International Journal of Pediatric Otorhinolaryngology (2014), http://dx.doi.org/10.1016/j.ijporl.2014.03.037 This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
*Title Page
Article Title: Otologic Complications of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis
cr
ip t
Authors: Jeffrey M. Hotaling, M.D. Affiliation: Loyola University Medical Center, Department of Otolaryngology – Head and Neck Surgery Address: 2160 S. First Avenue Building 105 Room 1870 LUMC Maywood, Illinois 60153 Email: [email protected]
an
us
Andrew J. Hotaling, M.D. Affiliation: Loyola University Medical Center, Department of Otolaryngology – Head and Neck Surgery Address: 2160 S. First Avenue Building 105 Room 1870 LUMC Maywood, Illinois 60153 Email: [email protected]
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Send Proofs/Correspondence to: Andrew J. Hotaling, M.D. Email: [email protected] Phone: (708)216-4785 Address: 2160 S. First Avenue Building 105 Room 1870 LUMC Maywood, Illinois 60153
Page 1 of 8
Title: Otologic Complications of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Authors: Jeffrey M. Hotaling, M.D.; Andrew J. Hotaling, M.D.
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Abstract: This case report demonstrates the otologic complications of StevensJohnson Syndrome and Toxic Epidermal Necrolysis (SJS/TEN). A 5 year-old male was admitted to the burn unit with the diagnosis of SJS/TEN believed secondary to acetaminophen use. After resolution of the acute phase, the patient was left with a severe auricular deformity with scarring and complete occlusion of the right external auditory canal (EAC). The patient underwent meatoplasty, canaloplasty, and tympanoplasty with subsequent placement of a molded stent in order to maintain patency of his EAC. Four years later, the patient has a patent right EAC with adequate hearing.
an
Keywords: Stevens-Johnson Syndrome; Toxic Epidermal Necrolysis; Ear; Ear Auricle; Complication
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Background/Introduction:
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Stevens Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are two disease entities that exist on a spectrum of severe adverse cutaneous reactions. Both are characterized by the formation of erythema and hemorrhagic erosions with lesions commonly affecting external skin and the mucous membranes of the mouth and eyes. Blister formation leads to eventual sloughing and detachment of the skin – the extent of body surface area (BSA) involvement determining whether a reaction is categorized as SJS (30% BSA). SJS and TEN are most commonly believed to be adverse immunologic reactions to drugs, including allopurinol, carbamazepine, sulfonamides, acetaminophen, sulfa drugs, non-steroidal anti-inflammatory drugs (NSAID's) and others although an association with mycoplasma pneumoniae and other infections has also been suggested1. Treatment includes removal of the offending agent, supportive care including referral to a burn unit if necessary, and the use of immunomodulating agents when indicated. Despite these measures, patients afflicted with SJS and TEN can suffer considerable morbidity and even mortality2. We present an otologic complication of SJS/TEN in a pediatric patient and its management. A comprehensive PUBMED search yielded no reported cases of otologic manifestations of SJS and TEN.
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1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 42 43 44 45 46 47 48 49 50 51 52 53 54 55 56 57 58 59 60 61 62 63 64 65
Case Report:
Page 2 of 8
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A three-year-old male was admitted to the burn unit at Loyola University Medical Center in August of 2006 with a severe SJS-TEN reaction, presumed secondary to acetaminophen and ibuprofen. This disease process resulted in extensive skin loss involving >50% of his body surface area ultimately requiring tracheotomy and a long hospital course (Figure 1). Particular to this case, the patient had experienced composite tissue loss of his right auricle for which he had previously undergone excision and debridement with skin grafting by the burn service. After the patient’s acute issues had been resolved, the otolaryngology service was consulted for evaluation and management of the patient’s resulting auricular injury and subjectively decreased hearing on the right. On initial physical examination 14 months post-injury, significant right auricular deformity with anterior displacement of the pinna was noted. The right external auditory canal (EAC) also appeared occluded by exquisitely tender scar tissue, which limited examination at that time. The left auricle and EAC were intact but there was a middle ear effusion noted on the left. An audiogram at that time demonstrated normal thresholds in the left ear but was unable to be completed in the right ear due to patient discomfort. A more detailed examination under anesthesia confirmed complete occlusion of the right EAC, as well as left middle ear effusion for which a left pneumatic equalization tube was also placed.
te
d
Given his complete EAC stenosis, the patient subsequently a right meatoplasty, canaloplasty, tympanoplasty and otoplasty with the goal of restoring a patent EAC. During the procedure, it was noted that the conchal cartilage had fused to the tragal cartilage, the tympanic membrane was markedly thickened, and squamous debris consistent with a canal cholesteatoma partially filled the right EAC. With the exception of a partially lateralized and scarred tympanic membrane, the middle ear appeared grossly normal with an intact ossicular chain. Due to concern for further lateralization of the tympanic membrane from the malleus causing a further conductive hearing loss, the tympanic membrane was subtotally resected with a fascial graft placed in standard underlay fashion. The conchal cartilage was also released from the tragal cartilage and fastened to the skin overlying the mastoid to prevent recurrent anterior folding of the auricle onto itself. The patient initially did well post-operatively but 3 months later developed re-stenosis of the EAC, requiring two revision meatoplasty and canaloplasty procedures. To prevent further re-stenosis, a custom, open-vented ear mold was fabricated and used to stent open the right EAC (Figure 2). This mold was worn by the patient at night and is well-tolerated. Through multiple follow-up examinations, the right EAC has remained patent with rare granulation tissue requiring debridement. The patient has a small, stable, central right tympanic membrane perforation. However, 7 years after the initial injury, the patient developed stenosis in the contralateral (left) EAC,
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1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 42 43 44 45 46 47 48 49 50 51 52 53 54 55 56 57 58 59 60 61 62 63 64 65
Page 3 of 8
with profuse granulation and narrowing at the bony-cartilaginous junction. This has been managed with debridement to date but may require further procedures if the granulation progresses in a fashion similar to the right side. Discussion:
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SJS/TEN represent two ends of a spectrum of severe adverse cutaneous reactions that can involve any epidermal or mucosal surface in the body, with mucous membranes of the eyes, mouth, nose and genitalia involved in up to 90% of cases1. In severe cases, other organ systems including the gastrointestinal, renal, and bronchopulmonary systems can also be involved. While the ophthalmologic complications of SJS/TEN including corneal scarring and loss vision are welldescribed in the literature, many otolaryngologic complications, including otologic complications, remain undescribed and, perhaps, underreported. In fact, a PubMed search using the terms “Stevens-Johnson Syndrome,” “Toxic Epidermal Necrolysis,” “Ear,” and “Otic” failed to find any articles dedicated to the otologic complications of SJS/TEN. However, in the context of the novelty of this report, an important distinction in this case is that the patient’s auricular deformity and scarring were not due to a disease process specific to the auricle and external canal, but rather to the more global immunologic process affecting the patient’s auricle.
te
d
In fact, strictures and stenoses secondary to SJS/TEN are nothing new, with upper aerodigestive tract strictures and stenoses well-documented among the roughly 20% of patients with TEN with involvement of the tracheal and bronchial epithelium3. As it is well known that opposing raw or denuded surfaces will adhere and cicatrize, it follows that the patient’s stricture was the result of a similar process whereby opposing, denuded epithelial surfaces in the patient’s EAC scarred and granulated, thereby occluding it. An additional consideration in this case was the composite tissue loss of the patient’s auricle, leaving few landmarks to aid in the patient’s reconstruction. While these sequelae were initially managed surgically by means of meatoplasty, canaloplasty, tympanoplasty and otoplasty, the patient’s EAC rapidly re-stenosed secondary to granulation tissue, requiring two revision procedures. In order to maintain patency of the EAC and potentially abate the need for additional surgeries, a stent was molded to the patient’s EAC in order to maintain its patency. Similar to a keel in upper airway reconstruction, this stent served to prevent contact between the opposing healing epidermal surfaces, in this case preventing restenosis of the external canal. This case thereby serves as a reminder that this devastating disease can affect ANY epidermal or mucosal surface in the body, including the auricle and the external canal. The continued granulation in the patient’s right ear and the new involvement in the contralateral (left) EAC indicate the long-term sequelae of this disease which continue even after the acute
Ac ce p
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 42 43 44 45 46 47 48 49 50 51 52 53 54 55 56 57 58 59 60 61 62 63 64 65
Page 4 of 8
phase of the reaction has abated. It is therefore crucial that these patients are followed long-term and that physicians expand their armamentarium beyond traditional surgical approaches in treating SJS/TEN patients. Conclusion:
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cr
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The long term care of patients with SJS/TEN requires an interdisciplinary approach and it is important that otolaryngologists be aware of these potential and challenging otologic complications. Given the often exuberant granulation and scarring experienced by these patient’s, molded stent placement may serve as an important adjunct to surgery in the treatment of stenoses of the external auditory canal.
an
References:
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1. Gerull R, Nelle M, Schaible T. Toxic epidermal necrolysis and Stevens-Johnson syndrome: A review. Crit Care Med. 2011;39(6):1521-32. 2. Letko E, Papaliodis DN, Papaliodis GN, Daoud YJ, Ahmed AR, Foster CS. Stevens-Johnson syndrome and toxic epidermal necrolysis: a review of the literature. Ann Allergy Asthma Immunol. 2005;94(4):419-36. 3. Mockenhaupt M. The current understanding of Stevens-Johnson syndrome and toxic epidermal necrolysis. Expert Rev Clin Immunol. 2011;7(6):803-13.
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Figure 1
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Figure 2
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Figure 1: 3 year-old patient upon admission to burn ICU after sustaining a severe SJSTEN reaction involving >50% of his body surface area.
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Figure 2: Patient wearing his molded, open-vented external auditory canal stent.
Page 8 of 8
