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Spontaneous air in the retropharyngeal space presenting with hypoxemia and neck pain in a child with neuroblastoma: A case report
International Journal of Pediatric Otorhinolaryngology Extra (2008) 3, 71—74
www.elsevier.com/locate/ijporl
CASE REPORT
Spontaneous air in the retropharyngeal space presenting with hypoxemia and neck pain in a child with neuroblastoma: A case report Mona Shete *, Jerome Thompson Otolaryngology-Head & Neck surgery, University of Tennessee HSC, Court Avenue, Ste B226, Memphis, TN 38163, United States Received 3 September 2007; accepted 30 October 2007 Available online 26 December 2007
KEYWORDS Retropharyngeal air; Cryptogenic organizing pneumonia; Airway obstruction
Summary A 9-year-old white male with a stage IV neuroblastoma of thorax since 4 years of age presented to the routine clinic visit with several days history of nonproductive cough, neck pain and progressive dyspnea. The oxygen saturation was 87%. His head and neck exam was insignificant and there was no crepitus or tenderness to neck palpation. Chest X-ray was normal but neck X-ray showed a strip of air in the retropharyngeal space. Last transplant was allogeneic BMT 9 months back followed by grade-III chronic graft versus host disease of skin and gut. The CT scan of neck and chest confirmed pneumomediastinum with retropharyngeal air and bilateral faint pulmonary infiltrates. Systemic steroids were added to the treatment to stabilize his respiratory condition. After 10 days of ICU stay his condition improved and he was discharged home. Repeat neck X-ray after 14 days showed near complete resolution of retropharyngeal air. From the clinical, radiological picture as well as therapeutic response to steroids the most likely diagnosis was cryptogenic organizing pneumonia formerly known as ‘‘bronchiolitis obliterans with organizing pneumonitis’’ (BOOP). Pneumomediastinum is reported in the literature as a rare cause of spontaneous air in the retropharyngeal space. Usually pneumomediastinum is a terminal event but in our patient it was a presenting symptom of BOOP. We also report a subtle presentation of BOOP and complete resolution with steroids. # 2007 Elsevier Ireland Ltd. All rights reserved.
1. Introduction Pneumoretropharynx or air in the retropharyngeal space has been reported secondary to blunt or * Corresponding author. Tel.: +1 901 730 0260; fax: +1 901 448 5120. E-mail address: [email protected] (M. Shete).
penetrating trauma to neck, soft tissue infections, cancer, dental procedures, esophageal perforations (Boerhaave’s syndrome) and tracheobronchial ruptures [1]. Other occasional causes of spontaneous air in the retropharyngeal space include pneumomediastinum or pneumothorax. The likely events responsible for occurrence of spontaneous air in the retropharynx are barotrauma from violent
1871-4048/$ — see front matter # 2007 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.pedex.2007.10.006
72
Fig. 1
M. Shete, J. Thompson
Strip of air on soft tissue X-ray neck on admission.
coughing, physical exertion, forceful swallowing or various situations producing valsalva maneuver [2]. In this report we present a case that was predisposed to this occurrence because of the patient’s underlying illness and prior therapies.
2. Case report A 9-year-old white male with a stage IV neuroblastoma, primarily of thoracic origin since 4 years of
Fig. 2
age presented for routine clinic visit with several days history of nonproductive cough, neck pain and progressive dyspnea. His history was otherwise negative for trauma, fever, upper respiratory tract infection, dysphagia or severe vomiting. On examination, his oxygen saturation was 87%. Auscultation of lungs revealed bilateral rales. His head and neck exam was insignificant and there was no crepitus or tenderness to neck palpation. Chest X-ray was normal but neck X-ray showed a strip of air in the retropharyngeal space (Fig. 1). His white cell count was 6800. Past medical history was significant for bone marrow transplant (BMT) twice. Last transplant was allogeneic BMT 9 months back followed by grade-III chronic graft versus host disease (c-GVHD) of skin and gut. His immunosuppressive medications were stopped because of neutropenia but he was taking antiviral agents for several episodes of positive PCR for cytomegalovirus. He was admitted to the hospital and supportive treatment was started with humidified oxygen with a facemask and intravenous antibiotics. His condition became worse with increasing respiratory distress so he was transferred to the ICU. The CTscan of neck and chest was done which showed pneumomediastinum and bilateral faint pulmonary infiltrates along with retropharyngeal air (Fig. 2). Systemic steroids were added to the treatment to stabilize his respiratory condition. After 10 days of ICU stay his condition improved and he was transferred to the floor and was slowly weaned of oxygen. Repeat CT scan after 10 days showed decreasing air in retropharynx with persistent pneumomediastinum and bilateral interstitial opacities (Fig. 3). Based on clinical improvement patient was discharged home. He did not develop fever or leucocytosis during entire hospital course. Repeat neck X-ray after 14
CT soft tissue neck confirming pneumomediastinum as a source of retropharyngeal air.
Retropharyngeal air in Neuroblastoma
Fig. 3
73
Continued air in the mediastinum despite resolution of symptoms and retropharyngeal air.
days showed near complete resolution of retropharyngeal air (Fig. 4). From the clinical and radiological picture as well as therapeutic response to steroids the most likely diagnosis was cryptogenic organizing pneumonia (COP) formerly known as bronchiolitis obliterans with organizing pneumonitis (BOOP). Fungal, viral or bacterial origin of pneumonia was less likely in this patient.
3. Discussion This patient presented with neck pain and retropharyngeal air as the first manifestation of BOOP
Fig. 4 Near complete resolution of retropharyngeal air in 6 weeks.
complicating c-GVHD. In contrast to other patients with pneumomediastinum described in the literature, this presentation was very subtle [3]. The prompt recognition of the underlying cause is essential as BOOP can be a life threatening complication of c-GVHD in post-BMT patients [2,4]. History of nonproductive cough and absence of trauma, severe vomiting, fever or other signs of infection excludes other causes of retropharyngeal air like blunt or penetrating trauma to neck, soft tissue infections, cancer, esophageal perforations (Boerhaave’s syndrome) and tracheobronchial ruptures. The pathophysiology of pneumomediastinum involves extravasation of air through perivascular interstitial tissues secondary to any condition that creates a gradient between intra-alveolar and perivascular interstitial pressures [5]. Transient increase in the intrathoracic pressure (e.g., Valsalva maneuver or coughing) precipitates alveolar rupture into the bronchovascular sheath, with subsequent dissection through the pulmonary interstitium towards and into the mediastinum. A continuum of fascial planes connects retropharyngeal or prevertebral space with the mediastinum and retroperitoneum [6]. Air in the soft tissues may produce obstruction to the upper airway structures and neck pain as seen in our case report. In our patient, we attribute the spontaneous mediastinal emphysema to paroxysms of cough with proliferating bronchiolitis as a pathological feature of COP [7]. Although the occurrence of ‘‘pneumoretropharynx’’ can be very bothersome to the affected individual, it is by itself a benign condition that generally resolves spontaneously as seen in our patient [6,8]. Prompt recognition of the underlying cause is essential as certain trauma-related causes may require surgical intervention, but the routine
74
M. Shete, J. Thompson
use of chest tubes, tracheostomy, or mediastinal drains is not recommended [6,10,11]. Our patient had a progressive c-GVHD complicating allogeneic BMT for neuroblastoma with bone marrow involvement. His prophylactic immunosuppressive medications were on hold for neutropenia. He was 9 months post-BMT and his immune system was normal (WBC, 6800). Pulmonary complications occur in 40—60% of patients who undergo BMT and are more commonly seen after allogeneic BMT. This may be due to the effects of c-GVHD, as well as the intense immunosuppressive therapy that is used to prevent it [3,7]. Late-phase complications of BMT occur 100 days or more after transplant when patient’s immune system is near normal as in our patient. They could be infective or noninfective in nature. CMV and Aspergillus fungal infections were reported and may be responsible for early termination of prophylactic immunosuppressive therapy as in our patient [9]. The normal chest X-ray and absence of nodules that are surrounded by a halo of ground glass attenuation (‘‘halo sign’’) or pleural-based, wedge-shaped areas of consolidation on CT scan rules out the possibility of Aspergillus origin in our patient [9]. The noninfectious pulmonary complications include bronchiolitis obliterans (BO), and cryptogenic organizing pneumonia (COP) which were reported in the literature with spontaneous pneumomediastinum and one report of the same condition with air in the nasopharynx [3]. Our patient’s clinical and radiological picture as well as his therapeutic response to steroid support the diagnosis of COP and exclude other causes of pneumonia. Patients who have bilateral faint pulmonary infiltrates and restrictive physiology may have COP as the predominant pathology. BO presents with an obstructive pattern on pulmonary function tests and clear lung fields. BO is often a progressive condition and is unresponsive to therapy. In contrast, steroids achieve resolution in approximately two-third of patients who have idiopathic COP [7]. The association of COP with invasive pulmonary aspergillosis is indicative of more severe pulmonary disease [2,4]. In summary, we report a case with an unusual and subtle presentation of cryptogenic organizing pneumonia as spontaneous air in the retropharyngeal
space in a child with c-GVHD after bone marrow transplantation. We also noticed good response to steroids with resolution of air in the retropharynx and clinical improvement to the degree of discharging patient home although pneumomediastinum was still persistent.
References [1] C.-C. Lee, T.-J. Chen, Y.-H. Wu, K.-C. Tsai, A. Yuan, Spontaneous retropharyngeal emphysema and pneumomediastinum presented with signs of acute upper airway obstruction, Am. J. Emerg. Med. 23 (3 May) (2005) 402—404. [2] M. Vogel, H. Brodoefel, W. Bethge, C. Faul, J. Hartmann, H. Schimmel, et al., Spontaneous thoracic air-leakage syndrome in patients following allogeneic hematopoietic stem cell transplantation: causes, CT-follow up and patient outcome, Eur. J. Radiol. 60 (3 Dec) (2006) 392—397 (Epub 2006 Aug 17). [3] M. Bitan, I.B. Resnick, R. Or, A. Rokach, U. Laxer, A. Ackerstein, et al., Rhinolalia as a presenting sign of pneumomediastinum complicating post peripheral blood stem cell transplantation bronchiolitis obliterans, Am. J. Hematol. 74 (3 Nov) (2003) 182—186. [4] S. Kumar, A. Tefferi, Spontaneous pneumomediastinum and subcutaneous emphysema complicating bronchiolitis obliterans after allogeneic bone marrow transplantation–—case report and review of literature, Ann. Hematol. 80 (7 Jul) (2001) 430—435. [5] V. Caraballo, R.A. Barish, D.J. Floccare, Pneumomediastinum presenting as acute airway obstruction, J. Emerg. Med. 4 (2 Mar—Apr) (1996) 159—163. [6] R.J. Maunder, D.J. Pierson, L.D. Hudson, Subcutaneous and mediastinal emphysema, Pathophysiol. Diagn. Manage. 44 (7 Jul) (1984) 1447—1453. [7] K.T. Yen, A.S. Lee, M.J. Krowka, C.D. Burger, Pulmonary complications in bone marrow transplantation: a practical approach to diagnosis and treatment, Clin. Chest. Med. 25 (1 Mar) (2004) 189—201. [8] R. Crausman, D. Mellman, E. Bloomfield, C. Jennings, Pulmonary barotrauma as the cause of pneumoretropharynx in pulmonary lymphangioleiomyomatosis, Am. J. Emerg. Med. 14 (3 May) (1996). [9] S. Waite, J. Jeudy, C.S. White, Acute lung infections in normal and immunocompromised hosts, Radiol. Clinics North. Am. 44 (2 Mar) (2006) 295—315. [10] E. Skogvoll, A.T. Grammeltvedt, P. Aadahl, U. Mostad, S. Slordahl, Life-threatening upper airway obstruction in a child caused by retropharyngeal emphysema, Acta Anaesthesiol. Scand. 45 (3 Mar) (2001) 393—395. [11] D.A. Breznick, J.L. Saporito, Iatrogenic retropharyngeal emphysema with impending airway obstruction, Arch. Otolaryngol. Head Neck Surg. 115 (11 Nov) (1989) 1367—1372.
Available online at www.sciencedirect.com
www.elsevier.com/locate/ijporl
CASE REPORT
Spontaneous air in the retropharyngeal space presenting with hypoxemia and neck pain in a child with neuroblastoma: A case report Mona Shete *, Jerome Thompson Otolaryngology-Head & Neck surgery, University of Tennessee HSC, Court Avenue, Ste B226, Memphis, TN 38163, United States Received 3 September 2007; accepted 30 October 2007 Available online 26 December 2007
KEYWORDS Retropharyngeal air; Cryptogenic organizing pneumonia; Airway obstruction
Summary A 9-year-old white male with a stage IV neuroblastoma of thorax since 4 years of age presented to the routine clinic visit with several days history of nonproductive cough, neck pain and progressive dyspnea. The oxygen saturation was 87%. His head and neck exam was insignificant and there was no crepitus or tenderness to neck palpation. Chest X-ray was normal but neck X-ray showed a strip of air in the retropharyngeal space. Last transplant was allogeneic BMT 9 months back followed by grade-III chronic graft versus host disease of skin and gut. The CT scan of neck and chest confirmed pneumomediastinum with retropharyngeal air and bilateral faint pulmonary infiltrates. Systemic steroids were added to the treatment to stabilize his respiratory condition. After 10 days of ICU stay his condition improved and he was discharged home. Repeat neck X-ray after 14 days showed near complete resolution of retropharyngeal air. From the clinical, radiological picture as well as therapeutic response to steroids the most likely diagnosis was cryptogenic organizing pneumonia formerly known as ‘‘bronchiolitis obliterans with organizing pneumonitis’’ (BOOP). Pneumomediastinum is reported in the literature as a rare cause of spontaneous air in the retropharyngeal space. Usually pneumomediastinum is a terminal event but in our patient it was a presenting symptom of BOOP. We also report a subtle presentation of BOOP and complete resolution with steroids. # 2007 Elsevier Ireland Ltd. All rights reserved.
1. Introduction Pneumoretropharynx or air in the retropharyngeal space has been reported secondary to blunt or * Corresponding author. Tel.: +1 901 730 0260; fax: +1 901 448 5120. E-mail address: [email protected] (M. Shete).
penetrating trauma to neck, soft tissue infections, cancer, dental procedures, esophageal perforations (Boerhaave’s syndrome) and tracheobronchial ruptures [1]. Other occasional causes of spontaneous air in the retropharyngeal space include pneumomediastinum or pneumothorax. The likely events responsible for occurrence of spontaneous air in the retropharynx are barotrauma from violent
1871-4048/$ — see front matter # 2007 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.pedex.2007.10.006
72
Fig. 1
M. Shete, J. Thompson
Strip of air on soft tissue X-ray neck on admission.
coughing, physical exertion, forceful swallowing or various situations producing valsalva maneuver [2]. In this report we present a case that was predisposed to this occurrence because of the patient’s underlying illness and prior therapies.
2. Case report A 9-year-old white male with a stage IV neuroblastoma, primarily of thoracic origin since 4 years of
Fig. 2
age presented for routine clinic visit with several days history of nonproductive cough, neck pain and progressive dyspnea. His history was otherwise negative for trauma, fever, upper respiratory tract infection, dysphagia or severe vomiting. On examination, his oxygen saturation was 87%. Auscultation of lungs revealed bilateral rales. His head and neck exam was insignificant and there was no crepitus or tenderness to neck palpation. Chest X-ray was normal but neck X-ray showed a strip of air in the retropharyngeal space (Fig. 1). His white cell count was 6800. Past medical history was significant for bone marrow transplant (BMT) twice. Last transplant was allogeneic BMT 9 months back followed by grade-III chronic graft versus host disease (c-GVHD) of skin and gut. His immunosuppressive medications were stopped because of neutropenia but he was taking antiviral agents for several episodes of positive PCR for cytomegalovirus. He was admitted to the hospital and supportive treatment was started with humidified oxygen with a facemask and intravenous antibiotics. His condition became worse with increasing respiratory distress so he was transferred to the ICU. The CTscan of neck and chest was done which showed pneumomediastinum and bilateral faint pulmonary infiltrates along with retropharyngeal air (Fig. 2). Systemic steroids were added to the treatment to stabilize his respiratory condition. After 10 days of ICU stay his condition improved and he was transferred to the floor and was slowly weaned of oxygen. Repeat CT scan after 10 days showed decreasing air in retropharynx with persistent pneumomediastinum and bilateral interstitial opacities (Fig. 3). Based on clinical improvement patient was discharged home. He did not develop fever or leucocytosis during entire hospital course. Repeat neck X-ray after 14
CT soft tissue neck confirming pneumomediastinum as a source of retropharyngeal air.
Retropharyngeal air in Neuroblastoma
Fig. 3
73
Continued air in the mediastinum despite resolution of symptoms and retropharyngeal air.
days showed near complete resolution of retropharyngeal air (Fig. 4). From the clinical and radiological picture as well as therapeutic response to steroids the most likely diagnosis was cryptogenic organizing pneumonia (COP) formerly known as bronchiolitis obliterans with organizing pneumonitis (BOOP). Fungal, viral or bacterial origin of pneumonia was less likely in this patient.
3. Discussion This patient presented with neck pain and retropharyngeal air as the first manifestation of BOOP
Fig. 4 Near complete resolution of retropharyngeal air in 6 weeks.
complicating c-GVHD. In contrast to other patients with pneumomediastinum described in the literature, this presentation was very subtle [3]. The prompt recognition of the underlying cause is essential as BOOP can be a life threatening complication of c-GVHD in post-BMT patients [2,4]. History of nonproductive cough and absence of trauma, severe vomiting, fever or other signs of infection excludes other causes of retropharyngeal air like blunt or penetrating trauma to neck, soft tissue infections, cancer, esophageal perforations (Boerhaave’s syndrome) and tracheobronchial ruptures. The pathophysiology of pneumomediastinum involves extravasation of air through perivascular interstitial tissues secondary to any condition that creates a gradient between intra-alveolar and perivascular interstitial pressures [5]. Transient increase in the intrathoracic pressure (e.g., Valsalva maneuver or coughing) precipitates alveolar rupture into the bronchovascular sheath, with subsequent dissection through the pulmonary interstitium towards and into the mediastinum. A continuum of fascial planes connects retropharyngeal or prevertebral space with the mediastinum and retroperitoneum [6]. Air in the soft tissues may produce obstruction to the upper airway structures and neck pain as seen in our case report. In our patient, we attribute the spontaneous mediastinal emphysema to paroxysms of cough with proliferating bronchiolitis as a pathological feature of COP [7]. Although the occurrence of ‘‘pneumoretropharynx’’ can be very bothersome to the affected individual, it is by itself a benign condition that generally resolves spontaneously as seen in our patient [6,8]. Prompt recognition of the underlying cause is essential as certain trauma-related causes may require surgical intervention, but the routine
74
M. Shete, J. Thompson
use of chest tubes, tracheostomy, or mediastinal drains is not recommended [6,10,11]. Our patient had a progressive c-GVHD complicating allogeneic BMT for neuroblastoma with bone marrow involvement. His prophylactic immunosuppressive medications were on hold for neutropenia. He was 9 months post-BMT and his immune system was normal (WBC, 6800). Pulmonary complications occur in 40—60% of patients who undergo BMT and are more commonly seen after allogeneic BMT. This may be due to the effects of c-GVHD, as well as the intense immunosuppressive therapy that is used to prevent it [3,7]. Late-phase complications of BMT occur 100 days or more after transplant when patient’s immune system is near normal as in our patient. They could be infective or noninfective in nature. CMV and Aspergillus fungal infections were reported and may be responsible for early termination of prophylactic immunosuppressive therapy as in our patient [9]. The normal chest X-ray and absence of nodules that are surrounded by a halo of ground glass attenuation (‘‘halo sign’’) or pleural-based, wedge-shaped areas of consolidation on CT scan rules out the possibility of Aspergillus origin in our patient [9]. The noninfectious pulmonary complications include bronchiolitis obliterans (BO), and cryptogenic organizing pneumonia (COP) which were reported in the literature with spontaneous pneumomediastinum and one report of the same condition with air in the nasopharynx [3]. Our patient’s clinical and radiological picture as well as his therapeutic response to steroid support the diagnosis of COP and exclude other causes of pneumonia. Patients who have bilateral faint pulmonary infiltrates and restrictive physiology may have COP as the predominant pathology. BO presents with an obstructive pattern on pulmonary function tests and clear lung fields. BO is often a progressive condition and is unresponsive to therapy. In contrast, steroids achieve resolution in approximately two-third of patients who have idiopathic COP [7]. The association of COP with invasive pulmonary aspergillosis is indicative of more severe pulmonary disease [2,4]. In summary, we report a case with an unusual and subtle presentation of cryptogenic organizing pneumonia as spontaneous air in the retropharyngeal
space in a child with c-GVHD after bone marrow transplantation. We also noticed good response to steroids with resolution of air in the retropharynx and clinical improvement to the degree of discharging patient home although pneumomediastinum was still persistent.
References [1] C.-C. Lee, T.-J. Chen, Y.-H. Wu, K.-C. Tsai, A. Yuan, Spontaneous retropharyngeal emphysema and pneumomediastinum presented with signs of acute upper airway obstruction, Am. J. Emerg. Med. 23 (3 May) (2005) 402—404. [2] M. Vogel, H. Brodoefel, W. Bethge, C. Faul, J. Hartmann, H. Schimmel, et al., Spontaneous thoracic air-leakage syndrome in patients following allogeneic hematopoietic stem cell transplantation: causes, CT-follow up and patient outcome, Eur. J. Radiol. 60 (3 Dec) (2006) 392—397 (Epub 2006 Aug 17). [3] M. Bitan, I.B. Resnick, R. Or, A. Rokach, U. Laxer, A. Ackerstein, et al., Rhinolalia as a presenting sign of pneumomediastinum complicating post peripheral blood stem cell transplantation bronchiolitis obliterans, Am. J. Hematol. 74 (3 Nov) (2003) 182—186. [4] S. Kumar, A. Tefferi, Spontaneous pneumomediastinum and subcutaneous emphysema complicating bronchiolitis obliterans after allogeneic bone marrow transplantation–—case report and review of literature, Ann. Hematol. 80 (7 Jul) (2001) 430—435. [5] V. Caraballo, R.A. Barish, D.J. Floccare, Pneumomediastinum presenting as acute airway obstruction, J. Emerg. Med. 4 (2 Mar—Apr) (1996) 159—163. [6] R.J. Maunder, D.J. Pierson, L.D. Hudson, Subcutaneous and mediastinal emphysema, Pathophysiol. Diagn. Manage. 44 (7 Jul) (1984) 1447—1453. [7] K.T. Yen, A.S. Lee, M.J. Krowka, C.D. Burger, Pulmonary complications in bone marrow transplantation: a practical approach to diagnosis and treatment, Clin. Chest. Med. 25 (1 Mar) (2004) 189—201. [8] R. Crausman, D. Mellman, E. Bloomfield, C. Jennings, Pulmonary barotrauma as the cause of pneumoretropharynx in pulmonary lymphangioleiomyomatosis, Am. J. Emerg. Med. 14 (3 May) (1996). [9] S. Waite, J. Jeudy, C.S. White, Acute lung infections in normal and immunocompromised hosts, Radiol. Clinics North. Am. 44 (2 Mar) (2006) 295—315. [10] E. Skogvoll, A.T. Grammeltvedt, P. Aadahl, U. Mostad, S. Slordahl, Life-threatening upper airway obstruction in a child caused by retropharyngeal emphysema, Acta Anaesthesiol. Scand. 45 (3 Mar) (2001) 393—395. [11] D.A. Breznick, J.L. Saporito, Iatrogenic retropharyngeal emphysema with impending airway obstruction, Arch. Otolaryngol. Head Neck Surg. 115 (11 Nov) (1989) 1367—1372.
Available online at www.sciencedirect.com