ILLUSTRATIVE CASE
Chest Pain and Anxiety in an Adolescent An Unusual Etiology Katherine R. Peeler, MD,* Jennifer Hasvold, MD,† and Margaret Wolff, MD‡ Abstract: Chest pain in children is commonly caused by benign etiologies but may be caused by conditions that carry significant morbidity if not treated. Emergency medicine physicians must identify the patients that require further evaluation and treatment. We describe a case of a 13-yearold boy with 10 months of progressive chest pain that had been attributed to anxiety and was ultimately diagnosed as an esophageal duplication cyst requiring surgical repair. Key Words: chest pain, anxiety, adolescent, esophageal duplication cyst (Pediatr Emer Care 2014;30: 812–813)
C
hest pain is a common presenting complaint in the pediatric emergency department (ED).1 Although the etiology is usually benign, the differential also includes conditions that can carry significant morbidity and mortality, including cardiac causes such as myocardial infarction or pericarditis, pulmonary causes such as pneumothorax or pulmonary embolism, or rarely gastrointestinal etiologies such as esophageal rupture or duplication. The more benign, and by far more common, causes of chest pain such as gastroesophageal reflux, costochondritis, musculoskeletal pain, and anxiety generally require only supportive care. The challenge for the ED physician is to identify the patients that require further evaluation or treatment. The aim of this illustrative case was 2-fold: (1) to describe the presentation and treatment of esophageal duplication cyst as an etiology of adolescent chest pain and (2) to highlight the importance of searching for nonpsychological sources of chest pain in a patient with longstanding severe anxiety.
CASE A 13-year-old boy with a history of anxiety presented to the ED for increased anxiety and chest pain. For the past 10 months, he had experienced intermittent chest pain accompanied by tachycardia. Previous evaluation included a normal electrocardiogram and chest radiograph. One week prior to presentation, he developed severe constant chest pain, frequent palpitations, and numbness and tingling of his hands. He also endorsed nausea and intermittent non-bloody, non-bilious emesis resulting in decreased oral intake and a 5-lb weight loss. He denied fever, difficulty swallowing, shortness of breath, abdominal pain, diarrhea, or constipation. Past medical history was significant for generalized anxiety disorder diagnosed 2 years prior to his presentation. Family history revealed generalized anxiety disorder, depression, and ulcerative colitis. Social history was remarkable for bullying. From the Departments of *Division of Critical Care Medicine, Department of Anesthesiology, Perioperative and Pain Medicine, Boston Children's Hospital, Boston, MA; †Internal Medicine and Pediatrics, Rapid City Regional Hospital and Founders Park Clinic, Rapid City, SD; and ‡Emergency Medicine, University of Michigan, Ann Arbor, MI. Disclosure: The authors declare no conflict of interest. Reprints: Katherine R. Peeler, MD, Division of Critical Care Medicine, Department of Anesthesiology, Perioperative and Pain Medicine Boston Children's Hospital, 300 Longwood Ave, Bader 634, Boston, MA 02115 (e‐mail: [email protected]). Copyright © 2014 by Lippincott Williams & Wilkins ISSN: 0749-5161
812
www.pec-online.com
On arrival to the ED, he was noted to be anxious appearing with the following vital signs: temperature of 37.1°C, pulse rate of 104 beats per minute, blood pressure of 107/67 mm Hg, respiratory rate of 20 breaths per minute, and oxygen saturation of 98% while breathing room air. Physical examination revealed slightly dry mucus membranes with a capillary refill of 2 seconds. Cardiovascular examination demonstrated tachycardia with a regular rhythm and without murmurs. Chest pain was not reproducible with palpation. His lungs were clear with good aeration. His abdomen was soft and nontender with normoactive bowel sounds. No rashes or skin lesions were noted. The rest of his physical examination was otherwise unremarkable. He was placed on monitors, intravenous access was obtained, and he was given a 20 mL/kg bolus of normal saline, ondansetron for nausea, and lorazepam for anxiety. A 12-lead electrocardiogram showed tachycardia with a sinus rhythm. Chest radiograph was unremarkable. Significant laboratory results included a white blood cell count of 17,500 WBC/mm3 with 85% neutrophils, bicarbonate of 17 mMol/L, erythrocyte sedimentation rate of 70 mm/h, and C-reactive protein of 6.2 mg/dL. Troponins, thyroid studies, and antistreptolysin O were all unremarkable. Given the inability to tolerate adequate oral intake and markedly elevated inflammatory markers, he was admitted for further evaluation. While an inpatient, he continued to have persistent chest pain. Transthoracic echocardiogram was obtained, which demonstrated normal biventricular function and no evidence of effusion. Because of persistent retching and poor oral intake, upper gastrointestinal series was performed, which demonstrated barium contrast in a longitudinal tubular structure adjacent to the esophagus. Subsequently, a chest computed tomography scan showed a second lumen approximately 12.5 cm in length, anterior to and communicating with the esophagus, consistent with an esophageal duplication cyst (Fig. 1). There was also wall thickening of the second lumen suggestive of an inflammatory process (Fig. 2).
FIGURE 1. Computed tomography showing esophageal duplication cyst with communication with true esophagus superiorly and fluid-contrast and air-fluid levels inferiorly. Pediatric Emergency Care • Volume 30, Number 11, November 2014
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Pediatric Emergency Care • Volume 30, Number 11, November 2014
FIGURE 2. Computed tomography showing esophageal duplication cyst anteriorly with wall thickening of the cyst.
Operative repair using a thoracoscopic approach was attempted, but the procedure was later converted to an open thoracotomy. The duplication cyst was resected, and the communication was repaired. After surgical repair, he was started on sertraline for generalized anxiety, and his diet was slowly advanced to solid foods. He was seen in follow-up 1 month after discharge at which point he endorsed complete resolution of his chest pain and significant improvement in both his mood and appetite.
DISCUSSION An esophageal duplication cyst is a rare etiology of chest pain in children. The estimated incidence is 1 in 8200 with a 2:1 male predominance.2 Esophageal duplication cysts occur as the result of an embryologic duplication of a portion of the muscle and submucosa of the esophagus without epithelial duplication. During the third through sixth weeks of gestation, secretory vacuoles fail to coalesce creating a cyst. If the defect occurs early in gestation, the cyst will occur more proximally in the foregut, such as the esophagus, whereas later errors will manifest more distally.3,4 Presenting symptoms vary based on the location of the cyst. Upper esophageal cysts generally present before the age of 2 years with tachypnea, stridor, cough, or difficulty feeding.2,5 A duplication cyst in the mid or lower esophagus can be asymptomatic until the cyst becomes infected or inflamed due to retention of esophageal contents. In rare cases, esophageal cysts can rupture or even hemorrhage. Presenting symptoms include dysphagia, epigastric discomfort, and retrosternal pain similar to our patient.2,6 Less common symptoms include anorexia, regurgitation, weight loss, or even cardiac dysrhythmias secondary to myocardial irritation.5,7 Diagnosis is made through various modes of imaging. Prenatally, esophageal duplication cysts may be detected on fetal ultrasonography.8 After birth, a chest radiograph may show a mass in
© 2014 Lippincott Williams & Wilkins
Chest Pain and Anxiety in an Adolescent
the middle or posterior mediastinum, and ultrasound may show a characteristic “bowel signature” sign of an outer hypoechoic rim and an inner echogenic rim.9 Barium swallow esophagram typically reveals extrinsic compression of a portion of the wall of the esophagus but may also show complete duplication.4 Both chest computed tomography and magnetic resonance imaging can help to further characterize the anatomy in pre-surgical planning.4,9 The management of esophageal duplication cysts is primarily surgical, but the urgency of surgery depends on the size of the cyst and presence or absence of associated symptoms. If a cyst is less than 4 cm and is detected incidentally in an asymptomatic patient, expectant management with scheduled followup and routine ultrasound or magnetic resonance imaging is appropriate.9 However, because alimentary tract duplication cysts can be complicated by obstruction, infection, bleeding, and rarely the development of malignancy, surgical repair will ultimately be necessary.9 Complicating this presentation was the patient's known history of significant anxiety that had increased over the past year in the setting of school bullying. Given his history and that historically up to 1 in 6 cases of pediatric chest pain are attributable to psychosocial stressors or underlying psychiatric illness, it is not surprising that his chest pain was attributed to his anxiety.1 His progression of symptoms and involvement of other systems, however, warranted further observation and workup.
CONCLUSIONS Esophageal duplication cysts are a rare cause of pediatric chest pain. The constellation of retrosternal chest pain, inability to tolerate oral intake, and vomiting suggests this diagnosis and requires further imaging. ACKNOWLEDGMENTS The authors would like to thank Dr William Masch for his technical support with formatting the images in this case. REFERENCES 1. Kocis KC. Chest pain in pediatrics. Pediatr Clin North Am. 1999;46: 189–203. 2. Arbona JL, Fazzi JG, Mayoral J. Congenital esophageal cysts: case report and review of literature. Am J Gastroenterol. 1984;79:177–182. 3. Bremer JL. Diverticula and duplications of the intestinal tract. Arch Pathol. 1944;38:132–140. 4. Berrocal T, Torres I, Gutierrez J, et al. Congenital anomalies of the upper gastrointestinal tract. Radiographics. 1999;19:855–872. 5. Sodhi KS, Saxena AK, Narasimha Rao KL, et al. Esophageal duplication cyst: an unusual cause of respiratory distress in infants. Pediatr Emerg Care. 2005;21:854–856. 6. Argarwal A, Singla S, Bansal M, et al. Infected esophageal duplication cyst masquerading as pericarditis. Intern Med. 2012;51:689–690. 7. Salo JA, Ala-Kulju KV. Congenital esophageal cysts in adults. Ann Thorac Surg. 1987;44:135–138. 8. Bianchi DW, Crombleholme TM, D'Alton ME, et al. Other cystic lesions of the chest. In: Fried A, Davis K, eds. Fetology: Diagnosis and Management of the Fetal Patient. 2nd ed. New York, NY: McGraw Hill Medical; 2010: 273–278. 9. Langer JC, Somme S. Duplications of the alimentary tract. In: Spitz L, Coran A, eds. Operative Pediatric Surgery. 6th ed. New York, NY: Oxford University Press; 2006:433–444.
www.pec-online.com
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
813
Chest Pain and Anxiety in an Adolescent An Unusual Etiology Katherine R. Peeler, MD,* Jennifer Hasvold, MD,† and Margaret Wolff, MD‡ Abstract: Chest pain in children is commonly caused by benign etiologies but may be caused by conditions that carry significant morbidity if not treated. Emergency medicine physicians must identify the patients that require further evaluation and treatment. We describe a case of a 13-yearold boy with 10 months of progressive chest pain that had been attributed to anxiety and was ultimately diagnosed as an esophageal duplication cyst requiring surgical repair. Key Words: chest pain, anxiety, adolescent, esophageal duplication cyst (Pediatr Emer Care 2014;30: 812–813)
C
hest pain is a common presenting complaint in the pediatric emergency department (ED).1 Although the etiology is usually benign, the differential also includes conditions that can carry significant morbidity and mortality, including cardiac causes such as myocardial infarction or pericarditis, pulmonary causes such as pneumothorax or pulmonary embolism, or rarely gastrointestinal etiologies such as esophageal rupture or duplication. The more benign, and by far more common, causes of chest pain such as gastroesophageal reflux, costochondritis, musculoskeletal pain, and anxiety generally require only supportive care. The challenge for the ED physician is to identify the patients that require further evaluation or treatment. The aim of this illustrative case was 2-fold: (1) to describe the presentation and treatment of esophageal duplication cyst as an etiology of adolescent chest pain and (2) to highlight the importance of searching for nonpsychological sources of chest pain in a patient with longstanding severe anxiety.
CASE A 13-year-old boy with a history of anxiety presented to the ED for increased anxiety and chest pain. For the past 10 months, he had experienced intermittent chest pain accompanied by tachycardia. Previous evaluation included a normal electrocardiogram and chest radiograph. One week prior to presentation, he developed severe constant chest pain, frequent palpitations, and numbness and tingling of his hands. He also endorsed nausea and intermittent non-bloody, non-bilious emesis resulting in decreased oral intake and a 5-lb weight loss. He denied fever, difficulty swallowing, shortness of breath, abdominal pain, diarrhea, or constipation. Past medical history was significant for generalized anxiety disorder diagnosed 2 years prior to his presentation. Family history revealed generalized anxiety disorder, depression, and ulcerative colitis. Social history was remarkable for bullying. From the Departments of *Division of Critical Care Medicine, Department of Anesthesiology, Perioperative and Pain Medicine, Boston Children's Hospital, Boston, MA; †Internal Medicine and Pediatrics, Rapid City Regional Hospital and Founders Park Clinic, Rapid City, SD; and ‡Emergency Medicine, University of Michigan, Ann Arbor, MI. Disclosure: The authors declare no conflict of interest. Reprints: Katherine R. Peeler, MD, Division of Critical Care Medicine, Department of Anesthesiology, Perioperative and Pain Medicine Boston Children's Hospital, 300 Longwood Ave, Bader 634, Boston, MA 02115 (e‐mail: [email protected]). Copyright © 2014 by Lippincott Williams & Wilkins ISSN: 0749-5161
812
www.pec-online.com
On arrival to the ED, he was noted to be anxious appearing with the following vital signs: temperature of 37.1°C, pulse rate of 104 beats per minute, blood pressure of 107/67 mm Hg, respiratory rate of 20 breaths per minute, and oxygen saturation of 98% while breathing room air. Physical examination revealed slightly dry mucus membranes with a capillary refill of 2 seconds. Cardiovascular examination demonstrated tachycardia with a regular rhythm and without murmurs. Chest pain was not reproducible with palpation. His lungs were clear with good aeration. His abdomen was soft and nontender with normoactive bowel sounds. No rashes or skin lesions were noted. The rest of his physical examination was otherwise unremarkable. He was placed on monitors, intravenous access was obtained, and he was given a 20 mL/kg bolus of normal saline, ondansetron for nausea, and lorazepam for anxiety. A 12-lead electrocardiogram showed tachycardia with a sinus rhythm. Chest radiograph was unremarkable. Significant laboratory results included a white blood cell count of 17,500 WBC/mm3 with 85% neutrophils, bicarbonate of 17 mMol/L, erythrocyte sedimentation rate of 70 mm/h, and C-reactive protein of 6.2 mg/dL. Troponins, thyroid studies, and antistreptolysin O were all unremarkable. Given the inability to tolerate adequate oral intake and markedly elevated inflammatory markers, he was admitted for further evaluation. While an inpatient, he continued to have persistent chest pain. Transthoracic echocardiogram was obtained, which demonstrated normal biventricular function and no evidence of effusion. Because of persistent retching and poor oral intake, upper gastrointestinal series was performed, which demonstrated barium contrast in a longitudinal tubular structure adjacent to the esophagus. Subsequently, a chest computed tomography scan showed a second lumen approximately 12.5 cm in length, anterior to and communicating with the esophagus, consistent with an esophageal duplication cyst (Fig. 1). There was also wall thickening of the second lumen suggestive of an inflammatory process (Fig. 2).
FIGURE 1. Computed tomography showing esophageal duplication cyst with communication with true esophagus superiorly and fluid-contrast and air-fluid levels inferiorly. Pediatric Emergency Care • Volume 30, Number 11, November 2014
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Pediatric Emergency Care • Volume 30, Number 11, November 2014
FIGURE 2. Computed tomography showing esophageal duplication cyst anteriorly with wall thickening of the cyst.
Operative repair using a thoracoscopic approach was attempted, but the procedure was later converted to an open thoracotomy. The duplication cyst was resected, and the communication was repaired. After surgical repair, he was started on sertraline for generalized anxiety, and his diet was slowly advanced to solid foods. He was seen in follow-up 1 month after discharge at which point he endorsed complete resolution of his chest pain and significant improvement in both his mood and appetite.
DISCUSSION An esophageal duplication cyst is a rare etiology of chest pain in children. The estimated incidence is 1 in 8200 with a 2:1 male predominance.2 Esophageal duplication cysts occur as the result of an embryologic duplication of a portion of the muscle and submucosa of the esophagus without epithelial duplication. During the third through sixth weeks of gestation, secretory vacuoles fail to coalesce creating a cyst. If the defect occurs early in gestation, the cyst will occur more proximally in the foregut, such as the esophagus, whereas later errors will manifest more distally.3,4 Presenting symptoms vary based on the location of the cyst. Upper esophageal cysts generally present before the age of 2 years with tachypnea, stridor, cough, or difficulty feeding.2,5 A duplication cyst in the mid or lower esophagus can be asymptomatic until the cyst becomes infected or inflamed due to retention of esophageal contents. In rare cases, esophageal cysts can rupture or even hemorrhage. Presenting symptoms include dysphagia, epigastric discomfort, and retrosternal pain similar to our patient.2,6 Less common symptoms include anorexia, regurgitation, weight loss, or even cardiac dysrhythmias secondary to myocardial irritation.5,7 Diagnosis is made through various modes of imaging. Prenatally, esophageal duplication cysts may be detected on fetal ultrasonography.8 After birth, a chest radiograph may show a mass in
© 2014 Lippincott Williams & Wilkins
Chest Pain and Anxiety in an Adolescent
the middle or posterior mediastinum, and ultrasound may show a characteristic “bowel signature” sign of an outer hypoechoic rim and an inner echogenic rim.9 Barium swallow esophagram typically reveals extrinsic compression of a portion of the wall of the esophagus but may also show complete duplication.4 Both chest computed tomography and magnetic resonance imaging can help to further characterize the anatomy in pre-surgical planning.4,9 The management of esophageal duplication cysts is primarily surgical, but the urgency of surgery depends on the size of the cyst and presence or absence of associated symptoms. If a cyst is less than 4 cm and is detected incidentally in an asymptomatic patient, expectant management with scheduled followup and routine ultrasound or magnetic resonance imaging is appropriate.9 However, because alimentary tract duplication cysts can be complicated by obstruction, infection, bleeding, and rarely the development of malignancy, surgical repair will ultimately be necessary.9 Complicating this presentation was the patient's known history of significant anxiety that had increased over the past year in the setting of school bullying. Given his history and that historically up to 1 in 6 cases of pediatric chest pain are attributable to psychosocial stressors or underlying psychiatric illness, it is not surprising that his chest pain was attributed to his anxiety.1 His progression of symptoms and involvement of other systems, however, warranted further observation and workup.
CONCLUSIONS Esophageal duplication cysts are a rare cause of pediatric chest pain. The constellation of retrosternal chest pain, inability to tolerate oral intake, and vomiting suggests this diagnosis and requires further imaging. ACKNOWLEDGMENTS The authors would like to thank Dr William Masch for his technical support with formatting the images in this case. REFERENCES 1. Kocis KC. Chest pain in pediatrics. Pediatr Clin North Am. 1999;46: 189–203. 2. Arbona JL, Fazzi JG, Mayoral J. Congenital esophageal cysts: case report and review of literature. Am J Gastroenterol. 1984;79:177–182. 3. Bremer JL. Diverticula and duplications of the intestinal tract. Arch Pathol. 1944;38:132–140. 4. Berrocal T, Torres I, Gutierrez J, et al. Congenital anomalies of the upper gastrointestinal tract. Radiographics. 1999;19:855–872. 5. Sodhi KS, Saxena AK, Narasimha Rao KL, et al. Esophageal duplication cyst: an unusual cause of respiratory distress in infants. Pediatr Emerg Care. 2005;21:854–856. 6. Argarwal A, Singla S, Bansal M, et al. Infected esophageal duplication cyst masquerading as pericarditis. Intern Med. 2012;51:689–690. 7. Salo JA, Ala-Kulju KV. Congenital esophageal cysts in adults. Ann Thorac Surg. 1987;44:135–138. 8. Bianchi DW, Crombleholme TM, D'Alton ME, et al. Other cystic lesions of the chest. In: Fried A, Davis K, eds. Fetology: Diagnosis and Management of the Fetal Patient. 2nd ed. New York, NY: McGraw Hill Medical; 2010: 273–278. 9. Langer JC, Somme S. Duplications of the alimentary tract. In: Spitz L, Coran A, eds. Operative Pediatric Surgery. 6th ed. New York, NY: Oxford University Press; 2006:433–444.
www.pec-online.com
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
813
