Stridor secondary to a bronchogenic cyst in a neonate

November 22, 2011 by Rowley S. Busino, MD, Huma A. Quraishi, MD, and Ian T. Cohen, MD

Abstract
We present the case of a female neonate with expiratory stridor that was caused by a bronchogenic cyst that led to tracheobronchomalacia. Rigid endoscopy revealed that a severe anterior compression of the tracheobronchial tree had caused tracheomalacia and right bronchomalacia. Computed tomography of the chest with intravenous contrast demonstrated the presence of a mediastinal mass anterior to the trachea and bronchus. Following a complete excision via a median sternotomy, histopathologic examination identified the mass as a bronchogenic cyst with respiratory epithelial lining and cartilage elements. The patient experienced a complete resolution of her respiratory symptoms postoperatively.

Introduction
Tracheobronchomalacia is an abnormal collapse of the tracheal and bronchial walls on expiration. It can be classified as primary or secondary.1 In primary tracheobronchomalacia, the defect is intrinsic to the tracheobronchial tree. The diagnosis can be made with bronchoscopy when the cartilage-to-membrane ratio changes from 4.5:1 to 2:1.1 Although the primary type is usually seen in premature infants, it has also been found in full-term infants. Previous presentation: The information in this article has been updated from its original presentation as a poster at the annual meeting of the American Society of Pediatric Otolaryngology; May 2-4, 2008; Orlando, Fla. Secondary tracheobronchomalacia occurs as the result of a variety of extrinsic pathologies that can cause compression or damage to the airway; among these pathologies are an esophageal atresia/tracheoesophageal fistula, a vascular anomaly, a congenital cardiac defect, and a cystic lesion, such as a bronchogenic cyst. External compression affects the integrity of the tracheal and bronchial walls and increases the compliance of the overlying affected tissue. Treatment is rarely necessary for primary presentations, but it is typically required for cases of secondary tracheobronchomalacia.2 Determination of the etiology is imperative for proper treatment. In this article, we describe an unusual presentation of secondary tracheobronchomalacia in a full-term neonate.

Case report
A 1-month-old girl was brought to our institution with intermittent expiratory stridor and feeding difficulties that had been present since birth. She had no other significant medical history. The stridor was not progressive, and the patient had no history of intubation. Physical examination was significant for intermittent expiratory stridor, tachypnea, and ankyloglossia. The patient was taken to the operating room for a frenulectomy, direct laryngoscopy, and bronchoscopy. She was found to have severe tracheomalacia and right bronchomalacia, which appeared to be the result of anterior

compression (figure 1). No other airway abnormalities were found. The endoscopic findings prompted a further evaluation to determine the source of the external compression. A vascular ring was suspected.

Figure 1. Preoperative rigid bronchoscopy shows the anterior extrinsic compression at the mid-trachea (A) and at the level of the carina (B), particularly at the right mainstem bronchus.

Computed tomography (CT) of the chest with intravenous contrast revealed the presence of a 2.1 x 2.3 x 2.3-cm cystic mediastinal mass located anterior to the trachea and bronchus (figure 2). The mass splayed the aortic arch and superior vena cava, which resulted in severe anterior tracheal and bronchial compression. Based on the anterior and central location of the cyst, complete excision was achieved via a median sternotomy rather than via the usual approach with a right lateral thoracotomy. The cyst was found in the right middle mediastinum deep to the superior vena cava, lateral to the aorta, and anterior to the tracheobronchial tree.

Figure 2. Axial chest CTs at the distal third of the trachea superior to the carina (A) and at the level of the carina (B) show the mediastinal cyst (arrows) compressing the anterior tracheal and bronchial walls.

Findings on histopathologic examination were consistent with a bronchogenic cyst with respiratory epithelial lining and cartilage elements (figure 3).

Figure 3. Histopathology shows that the cyst lining is made up of pseudostratified ciliated columnar epithelium with underlying mucous glands and cartilage.

Postoperatively, the patient did well, and she experienced no further respiratory issues.

Discussion
Bronchogenic cysts are an uncommon source of stridor in the pediatric population. They are congenital lesions that arise from an abnormality of the normal budding of the ventral foregut, and they develop around the fourth to eighth gestational week.3Bronchogenic cysts are lined with respiratory epithelium and filled with clear fluid or mucus, and they contain other elements normally found in the respiratory tract, such as cartilage and smoothmuscle, glandular, and connective tissue. They represent 7% of all mediastinal masses that occur in this age group.4 The differential diagnosis of benign mediastinal masses in the pediatric population includes teratoma, thymic cyst, ganglioneuroma, enteric duplication cyst, and hemangiolymphangioma. Our review of the literature identified many case reports of bronchogenic cysts of the middle mediastinum in the pediatric population, but all of them had caused compression from a location either posterior to or lateral to the tracheobronchial tree.5-10 To the best of our knowledge, ours is the first published report in which tracheobronchoscopy revealed a severe anterior compression. In view of the anatomic area affected, the mass was initially thought to be a vascular abnormality, specifically a vascular ring.11 Some common congenital vascular anomalies that cause symptomatic compression of the trachea and bronchus include aberrant innominate artery, double aortic arch, right aortic arch with a left ligamentum arteriosum and an aberrant left subclavian artery, and pulmonary artery sling. Bronchogenic cysts occur in patients of all ages, and their clinical presentation is variable. Signs and symptoms of airway compression are more common in infants and children than in adults. Their location in relation to the tracheobronchial tree determines the presence of compressive symptoms. In 1995, Ribet et al reported that 75% of bronchogenic cysts in a cohort of children were in a critical area above or at the level of the tracheal carina, and that 70.8% of the entire cohort was symptomatic.12 In contrast, only 60% of adults in that series were symptomatic, and only 53% of cysts were above or at the level of the carina. The treatment of bronchogenic cysts entails complete surgical excision. The prognosis following treatment is excellent.12 In conclusion, the possibility of an extrinsic source of tracheobronchomalacia should always be considered in the differential diagnosis of stridor. Bronchogenic cysts are an unusual but treatable cause of secondary tracheobronchomalacia in the pediatric population. Cyst location determines both the presence of compressive symptoms and the type of surgical management.

From the Department of Otolaryngology, Rady Children's Hospital, San Diego (Dr. Busino); the Department of Otolaryngology-Head and Neck Surgery, University of Medicine and Dentistry of New Jersey-New Jersey Medical School, Newark (Dr. Quraishi); and the Division of Pediatric Surgery, Department of Surgery, Monmouth Medical Center, Long Branch, N.J. (Dr. Cohen). The case described in this article occurred at the University of Medicine and Dentistry of New Jersey-New Jersey Medical School. Corresponding author: Rowley S. Busino, MD, Department of Otolaryngology, Rady Children's Hospital, 3030 Children's Way, San Diego, CA 92123.

References
1. 2. 3. 4. 5. 6. 7. 8. Benjamin B. Tracheomalacia in infants and children. Ann Otol Rhinol Laryngol 1984; 93 (5 Pt 1):438-42. Carden KA, Boiselle PM, Waltz DA, Ernst A. Tracheomalacia and tracheobronchomalacia in children and adults: An in-depth review. Chest 2005; 127 (3): 984-1005. Maier HC. Bronchiogenic cysts of the mediastinum. Ann Surg 1948; 127 (3): 476-502. Bower RJ, Kiesewetter WB. Mediastinal masses in infants and children. Arch Surg 1977; 112 (8): 1003-9. Grafe WR, Goldsmith EI, Redo SF. Bronchogenic cysts of the mediastinum in children. J Pediatr Surg 1966; 1 (4): 384-93. Eraklis AJ, Griscom NT, McGovern JB. Bronchogenic cysts of the mediastinum in infancy. N Engl J Med 1969; 281 (21): 1150-5. Yerman HM, Holinger LD. Bronchogenic cyst with tracheal involvement. Ann Otol Rhinol Laryngol 1990; 99 (2 Pt 1):89-93. Lazar RH, Younis RT, Bassila MN. Bronchogenic cysts: A cause of stridor in the neonate. Am J Otolaryngol 1991; 12 (2): 117-21.

9. Artz GJ. Stridor in an infant. Clin Pediatr (Phila) 2006; 45 (6): 578. 10. Reilly J, Mattei P. Stridor in an infant: Commentary. Diagnosis: Bronchogenic cyst. Clin Pediatr (Phila) 2006; 45 (6): 578-81. 11. Shah RK, Mora BN, Bacha E, et al. The presentation and management of vascular rings: An otolaryngology perspective. Int J Pediatr Otorhinolaryngol 2007; 71 (1): 57-62. 12. Ribet ME, Copin MC, Gosselin B. Bronchogenic cysts of the mediastinum. J Thorac Cardiovasc Surg 1995; 109 (5): 1003-10.