ILLUSTRATIVE CASE
Stridor Is Not Always Croup Leslie W. King-Schultz, MD,* Laura J. Orvidas, MD,† and Mark S. Mannenbach, MD*‡§ Abstract: Respiratory distress and stridor are common presenting symptoms for children in the emergency department. Most of these children will have common illnesses such as bronchiolitis or croup. Clinicians, however, must maintain a broad differential diagnosis and a healthy skepticism in the approach to each child's case so as not to miss uncommon or atypical presentations. We describe the case of a child with stridor in whom an airway hemangioma was ultimately diagnosed. Key Words: croup, hemangioma, stridor (Pediatr Emer Care 2015;31: 140–143)
CASE A 39-day-old girl born at term after an uncomplicated pregnancy and delivery was seen at the emergency department (ED) with symptoms of respiratory distress and stridor in January. For the previous 5 days, she had had symptoms of intermittent cough without rhinorrhea or fever. She had been evaluated 3 days earlier by her primary pediatrician who diagnosed an upper respiratory illness, probably viral, and recommended supportive cares. She had a 2-year-old brother who also had had cough and rhinorrhea for the previous week. There was no family history of asthma. When she began to experience increased work of breathing, noisy breathing, and decreased interest in and duration of feeding, her parents brought her to the ED for evaluation. Upon arrival to the ED, she had a rectal temperature of 37.5°C. She was tachycardic with a heart rate (HR) of 180 beats per minute. Her respiratory rate (RR) was 32 breaths per minute with notable subcostal and suprasternal retractions. She had an audible stridor and an expiratory “wheezing.” Blow-by oxygen was administered, and saturations remained higher than 96%. Chest radiography demonstrated bilateral perihilar interstitial prominence consistent with bronchial inflammation but no focal consolidation. Her weight was 5.2 kg. She was given a 4-mg dose of dexamethasone through intramuscular injection and a nebulizer treatment with 0.5 mL of 2.25% racemic epinephrine, which resulted in improvement in her work of breathing and stridor. She was observed for 4 hours in the ED, but the retractions and stridor returned 2 hours after the nebulizer treatment was given. A second 0.5-mL dose of 2.25% racemic epinephrine was given, which again improved her symptoms. She was admitted to the general pediatrics unit for observation with a presumed diagnosis of croup. At admission, she was afebrile with an HR of 153 beats per minute and an RR of 22 breaths per minute. She was breathing comfortably with no further wheezing, stridor, or retractions. A mild cough continued overnight, but she fed well from a bottle. Because her symptoms had improved considerably in the
morning, she was discharged home with planned follow-up with her primary care provider. The day after discharge, she was brought back to the ED for recurrence of respiratory symptoms, along with new symptoms of rhinorrhea and sneezing. Her rectal temperature was 36.8°C, her HR was 177 beats per minute, her RR was 28 breaths per minute, and her oxygen saturation was 95% on room air. On examination, she had subcostal and supraclavicular retractions as well as mild intercostal retractions. She had stridor with both inspiration and expiration that was worse when lying supine. Examination of her skin revealed a small hemangioma in her left antecubital fossa (Fig. 1). An otolaryngologist was consulted, and bedside flexible laryngoscopy was performed. The glottic structures and epiglottis appeared normal. The subglottis demonstrated a left-sided fullness. Full neck radiographs showed subglottic narrowing (Fig. 2). The patient was subsequently given a 0.5-mL dose of 2.25% racemic epinephrine, which improved her stridor and work of breathing. She was admitted to the pediatric intensive care unit for close monitoring. Because she was stable, further evaluation of her airway was deferred until the morning when a pediatric otorhinolaryngologist would be available. She required additional doses of racemic epinephrine and received a 4-mg dose of dexamethasone overnight for continued stridor. At admission, she was tachycardic up to 270 beats per minute. Electrocardiography revealed supraventricular tachycardia (SVT). Throughout the night, she had short runs of tachycardia, but her blood pressure, perfusion, and mental status remained normal. No intervention was required to abort the SVT because it resolved each time on its own. She underwent microlaryngoscopy and bronchoscopy the following day in the operating room. A left-sided glottic mass was visualized in the airway (Fig. 3), although a 2.7-mm endoscope could easily pass beside it. The mass appeared to be compressible and blanching with some hypervascularity. It was difficult, however, to determine through visualization alone whether it was a cystic structure or a hemangioma. Because no fluid could be aspirated from the mass, it was diagnosed as a hemangioma.
From the Departments of *Pediatric and Adolescent Medicine, and †Otorhinolaryngology—Head and Neck Surgery, and ‡Division of Pediatric and Adolescent Emergency Medicine, §Division of Pediatric Emergency Medicine, Department of Emergency Medicine, Mayo Clinic, Rochester, MN. Disclosure: The authors declare no conflict of interest. Reprints: Mark S. Mannenbach, MD, Division of Pediatric and Adolescent Emergency Medicine, Mayo Clinic, 200 First St SW, Rochester, MN 55905 (e‐mail: [email protected]). Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved. ISSN: 0749-5161
FIGURE 1. Photograph of the forearm showing a small hemangioma.
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FIGURE 2. Soft tissue lateral neck radiograph showing subglottic narrowing (arrow).
The patient remained intubated after the procedure in the event of postoperative swelling from the procedure. Magnetic resonance imaging of the area was attempted, but because of compression from the endotracheal tube, no mass could be visualized. No further hemangiomas or cysts were noted on magnetic resonance imaging of the chest and abdomen. A pediatric cardiologist was consulted to evaluate her tachycardia. She underwent Holter monitoring, which showed 6 instances of SVT during approximately 20 hours of monitoring. As with earlier episodes, the rhythm spontaneously converted on each occasion, and she was otherwise asymptomatic. Her highest HRs were noted with dosing of epinephrine, but she continued to have SVT even after racemic epinephrine was discontinued. Echocardiography demonstrated a structurally normal heart. She was initiated on propranolol therapy at a dosage of 2 mg/kg per day divided every 8 hours for treatment of the hemangioma. The pediatric cardiologist recommended increasing the dosage of propranolol to 1 mg/kg per dose or 3 mg/kg per day divided every 8 hours to treat the SVT in addition to the hemangioma. She was returned to the operating room for extubation and
Stridor Is Not Always Croup
FIGURE 4. Follow-up intraoperative bronchoscopic photograph of airway. The mass was considerably smaller (arrow) after 2 days of propranolol therapy.
repeated airway endoscopy 2 days later, at which time the airway mass appeared markedly smaller (Fig. 4). Her tachycardia improved and she had no further documented episodes of SVT. She remained on continuous monitoring throughout her 6-day hospital course. She experienced occasional decreases in HR to 80 beats per minute, but she remained normotensive. Her blood glucose level was monitored after each dose of propranolol and remained within normal limits. Two weeks later, a repeated endoscopy demonstrated a somewhat larger hemangioma compared with that at the time of extubation. This was believed to be caused by compression from the endotracheal tube. The mass was still smaller, however, than what was seen on initial evaluation. Subsequent endoscopy at 1 month and 3 months showed a steady decrease in hemangioma
TABLE 1. Differential Diagnosis for Stridor in Young Children Supraglottic
Glottic
Subglottic
Laryngomalacia Glottic web Stenosis Laryngocele or cyst Glottic atresia Subglottic cyst Epiglottitis Laryngeal Croup cleft Retropharyngeal or Glottic Tracheomalacia peritonsillar stenosis abscess Angioneurotic Laryngitis Vascular edema Neoplasm Vocal cord Ring/sling Craniofacial Paralysis Bacterial abnormality Macroglossia Hematoma Tracheitis Cystic Cystic hygroma hygroma Neoplasm Laryngeal cleft FIGURE 3. Initial bronchoscopic photograph of airway. A mass can be seen in the 7-o'clock position (arrow).
Other Hemangioma Lymphangioma Papilloma Foreign body
Neck or facial Trauma Caustic Ingestion
Data from the following references: Sulman et al,6 Holinger,7 Cherry,12 Ryckman and Rodgers,13 Zalzal,14 and Ledoux et al.15
© 2015 Wolters Kluwer Health, Inc. All rights reserved.
Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.
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size with an accompanying increase in airway diameter. On followup in the division of cardiology both 6 weeks and 6 months after discharge, she had no further SVT on electrocardiography or Holter monitoring. The cardiologist believed that she is no longer required propranolol therapy for the SVT. She remained on propranolol for the hemangioma, however, until repeated endoscopy at the age of approximately 1 year. Because the hemangioma remained stable and the patient was asymptomatic, propranolol therapy was discontinued. On subsequent Holter monitoring, she was noted to have a recurrent SVT and resumed on β-blocker therapy.
surgical excision proves to be too challenging, tracheostomy may be required until full resolution occurs.5,8,9 In the present case, the patient also experienced SVT. This was believed to be idiopathic because it persisted, even despite discontinuation of racemic epinephrine. Propranolol, which is the treatment of choice for SVT in infants, was also the treatment chosen for the hemangioma. Dosing was increased to more adequately treat the SVT. This case highlights the importance of considering a broad differential diagnosis for stridor (Table 1), especially when the patient's case does not meet the typical parameters for common conditions. Although this infant's condition manifested in the midst of the peak respiratory illness season and there most likely was a precipitating viral illness for her symptoms, croup was atypical because of the patient's young age; croup typically presents between the ages of 6 months and 6 years (most are 6 months to2 y).11 In addition, although her symptoms improved with dexamethasone and racemic epinephrine, she had a recurrence of stridor very quickly (
Stridor Is Not Always Croup Leslie W. King-Schultz, MD,* Laura J. Orvidas, MD,† and Mark S. Mannenbach, MD*‡§ Abstract: Respiratory distress and stridor are common presenting symptoms for children in the emergency department. Most of these children will have common illnesses such as bronchiolitis or croup. Clinicians, however, must maintain a broad differential diagnosis and a healthy skepticism in the approach to each child's case so as not to miss uncommon or atypical presentations. We describe the case of a child with stridor in whom an airway hemangioma was ultimately diagnosed. Key Words: croup, hemangioma, stridor (Pediatr Emer Care 2015;31: 140–143)
CASE A 39-day-old girl born at term after an uncomplicated pregnancy and delivery was seen at the emergency department (ED) with symptoms of respiratory distress and stridor in January. For the previous 5 days, she had had symptoms of intermittent cough without rhinorrhea or fever. She had been evaluated 3 days earlier by her primary pediatrician who diagnosed an upper respiratory illness, probably viral, and recommended supportive cares. She had a 2-year-old brother who also had had cough and rhinorrhea for the previous week. There was no family history of asthma. When she began to experience increased work of breathing, noisy breathing, and decreased interest in and duration of feeding, her parents brought her to the ED for evaluation. Upon arrival to the ED, she had a rectal temperature of 37.5°C. She was tachycardic with a heart rate (HR) of 180 beats per minute. Her respiratory rate (RR) was 32 breaths per minute with notable subcostal and suprasternal retractions. She had an audible stridor and an expiratory “wheezing.” Blow-by oxygen was administered, and saturations remained higher than 96%. Chest radiography demonstrated bilateral perihilar interstitial prominence consistent with bronchial inflammation but no focal consolidation. Her weight was 5.2 kg. She was given a 4-mg dose of dexamethasone through intramuscular injection and a nebulizer treatment with 0.5 mL of 2.25% racemic epinephrine, which resulted in improvement in her work of breathing and stridor. She was observed for 4 hours in the ED, but the retractions and stridor returned 2 hours after the nebulizer treatment was given. A second 0.5-mL dose of 2.25% racemic epinephrine was given, which again improved her symptoms. She was admitted to the general pediatrics unit for observation with a presumed diagnosis of croup. At admission, she was afebrile with an HR of 153 beats per minute and an RR of 22 breaths per minute. She was breathing comfortably with no further wheezing, stridor, or retractions. A mild cough continued overnight, but she fed well from a bottle. Because her symptoms had improved considerably in the
morning, she was discharged home with planned follow-up with her primary care provider. The day after discharge, she was brought back to the ED for recurrence of respiratory symptoms, along with new symptoms of rhinorrhea and sneezing. Her rectal temperature was 36.8°C, her HR was 177 beats per minute, her RR was 28 breaths per minute, and her oxygen saturation was 95% on room air. On examination, she had subcostal and supraclavicular retractions as well as mild intercostal retractions. She had stridor with both inspiration and expiration that was worse when lying supine. Examination of her skin revealed a small hemangioma in her left antecubital fossa (Fig. 1). An otolaryngologist was consulted, and bedside flexible laryngoscopy was performed. The glottic structures and epiglottis appeared normal. The subglottis demonstrated a left-sided fullness. Full neck radiographs showed subglottic narrowing (Fig. 2). The patient was subsequently given a 0.5-mL dose of 2.25% racemic epinephrine, which improved her stridor and work of breathing. She was admitted to the pediatric intensive care unit for close monitoring. Because she was stable, further evaluation of her airway was deferred until the morning when a pediatric otorhinolaryngologist would be available. She required additional doses of racemic epinephrine and received a 4-mg dose of dexamethasone overnight for continued stridor. At admission, she was tachycardic up to 270 beats per minute. Electrocardiography revealed supraventricular tachycardia (SVT). Throughout the night, she had short runs of tachycardia, but her blood pressure, perfusion, and mental status remained normal. No intervention was required to abort the SVT because it resolved each time on its own. She underwent microlaryngoscopy and bronchoscopy the following day in the operating room. A left-sided glottic mass was visualized in the airway (Fig. 3), although a 2.7-mm endoscope could easily pass beside it. The mass appeared to be compressible and blanching with some hypervascularity. It was difficult, however, to determine through visualization alone whether it was a cystic structure or a hemangioma. Because no fluid could be aspirated from the mass, it was diagnosed as a hemangioma.
From the Departments of *Pediatric and Adolescent Medicine, and †Otorhinolaryngology—Head and Neck Surgery, and ‡Division of Pediatric and Adolescent Emergency Medicine, §Division of Pediatric Emergency Medicine, Department of Emergency Medicine, Mayo Clinic, Rochester, MN. Disclosure: The authors declare no conflict of interest. Reprints: Mark S. Mannenbach, MD, Division of Pediatric and Adolescent Emergency Medicine, Mayo Clinic, 200 First St SW, Rochester, MN 55905 (e‐mail: [email protected]). Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved. ISSN: 0749-5161
FIGURE 1. Photograph of the forearm showing a small hemangioma.
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Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.
Pediatric Emergency Care • Volume 31, Number 2, February 2015
FIGURE 2. Soft tissue lateral neck radiograph showing subglottic narrowing (arrow).
The patient remained intubated after the procedure in the event of postoperative swelling from the procedure. Magnetic resonance imaging of the area was attempted, but because of compression from the endotracheal tube, no mass could be visualized. No further hemangiomas or cysts were noted on magnetic resonance imaging of the chest and abdomen. A pediatric cardiologist was consulted to evaluate her tachycardia. She underwent Holter monitoring, which showed 6 instances of SVT during approximately 20 hours of monitoring. As with earlier episodes, the rhythm spontaneously converted on each occasion, and she was otherwise asymptomatic. Her highest HRs were noted with dosing of epinephrine, but she continued to have SVT even after racemic epinephrine was discontinued. Echocardiography demonstrated a structurally normal heart. She was initiated on propranolol therapy at a dosage of 2 mg/kg per day divided every 8 hours for treatment of the hemangioma. The pediatric cardiologist recommended increasing the dosage of propranolol to 1 mg/kg per dose or 3 mg/kg per day divided every 8 hours to treat the SVT in addition to the hemangioma. She was returned to the operating room for extubation and
Stridor Is Not Always Croup
FIGURE 4. Follow-up intraoperative bronchoscopic photograph of airway. The mass was considerably smaller (arrow) after 2 days of propranolol therapy.
repeated airway endoscopy 2 days later, at which time the airway mass appeared markedly smaller (Fig. 4). Her tachycardia improved and she had no further documented episodes of SVT. She remained on continuous monitoring throughout her 6-day hospital course. She experienced occasional decreases in HR to 80 beats per minute, but she remained normotensive. Her blood glucose level was monitored after each dose of propranolol and remained within normal limits. Two weeks later, a repeated endoscopy demonstrated a somewhat larger hemangioma compared with that at the time of extubation. This was believed to be caused by compression from the endotracheal tube. The mass was still smaller, however, than what was seen on initial evaluation. Subsequent endoscopy at 1 month and 3 months showed a steady decrease in hemangioma
TABLE 1. Differential Diagnosis for Stridor in Young Children Supraglottic
Glottic
Subglottic
Laryngomalacia Glottic web Stenosis Laryngocele or cyst Glottic atresia Subglottic cyst Epiglottitis Laryngeal Croup cleft Retropharyngeal or Glottic Tracheomalacia peritonsillar stenosis abscess Angioneurotic Laryngitis Vascular edema Neoplasm Vocal cord Ring/sling Craniofacial Paralysis Bacterial abnormality Macroglossia Hematoma Tracheitis Cystic Cystic hygroma hygroma Neoplasm Laryngeal cleft FIGURE 3. Initial bronchoscopic photograph of airway. A mass can be seen in the 7-o'clock position (arrow).
Other Hemangioma Lymphangioma Papilloma Foreign body
Neck or facial Trauma Caustic Ingestion
Data from the following references: Sulman et al,6 Holinger,7 Cherry,12 Ryckman and Rodgers,13 Zalzal,14 and Ledoux et al.15
© 2015 Wolters Kluwer Health, Inc. All rights reserved.
Copyright © 2015 Wolters Kluwer Health, Inc. All rights reserved.
www.pec-online.com
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size with an accompanying increase in airway diameter. On followup in the division of cardiology both 6 weeks and 6 months after discharge, she had no further SVT on electrocardiography or Holter monitoring. The cardiologist believed that she is no longer required propranolol therapy for the SVT. She remained on propranolol for the hemangioma, however, until repeated endoscopy at the age of approximately 1 year. Because the hemangioma remained stable and the patient was asymptomatic, propranolol therapy was discontinued. On subsequent Holter monitoring, she was noted to have a recurrent SVT and resumed on β-blocker therapy.
surgical excision proves to be too challenging, tracheostomy may be required until full resolution occurs.5,8,9 In the present case, the patient also experienced SVT. This was believed to be idiopathic because it persisted, even despite discontinuation of racemic epinephrine. Propranolol, which is the treatment of choice for SVT in infants, was also the treatment chosen for the hemangioma. Dosing was increased to more adequately treat the SVT. This case highlights the importance of considering a broad differential diagnosis for stridor (Table 1), especially when the patient's case does not meet the typical parameters for common conditions. Although this infant's condition manifested in the midst of the peak respiratory illness season and there most likely was a precipitating viral illness for her symptoms, croup was atypical because of the patient's young age; croup typically presents between the ages of 6 months and 6 years (most are 6 months to2 y).11 In addition, although her symptoms improved with dexamethasone and racemic epinephrine, she had a recurrence of stridor very quickly (
