This Article  • Extract  • PDF  • Send to a friend  • Save in My Folder  • Save to citation manager  • Permissions  Citing Articles  • Contact me when this article is cited  Related Content  • Similar articles in this journal  Topic Collections  • Diagnosis  • Alert me on articles by topic

Dirk Eichmann, MD; Sylvia Engler, MD; Hans-Deiter Oldigs, MD; Heinz Schroeder, MD; Carl-Joachim Partsch, MD
From the Departments of Pediatrics (Drs Eichmann, Schroeder, and Partsch) and General and Thoracic Surgery (Dr Engler), Christian-Albrechts-University, Kiel, and the Department of Pediatrics (Dr Oldigs), Diakonissen Krankenhaus, Flensburg, Germany.

Arch Pediatr Adolesc Med. 2001;155:1067-1068.

AT AGE 3 WEEKS, a full-term newborn developed recurrent cough and stridor without other signs of respiratory distress. She had no feeding problems and was thriving. At age 6 months, inspiratory and expiratory stridor became more pronounced, and she developed dyspnea. A chest radiograph was obtained to evaluate the airway, upper mediastinum, and esophagus (Figure 1). The vertebrae were normal. Magnetic resonance imaging followed (Figure 2). Surgery was performed, and the stridor and respiratory distress ceased immediately after surgery.

Figure 1.

Figure 2.

Denouement and Discussion: Congenital Esophageal Duplication Cyst as a Rare Cause of Neonatal Progressive Stridor

Figure 1. Lateral chest radiograph shows narrowing and anterior displacement of the trachea (arrow).

Figure 2. Sagittal T1-weighted (A) and axial magnetic resonance imaging T2-weighted (B) scans show a prevertebral cystic mass measuring 4 x 4 x 1.8 cm and filled with fluid. A pleural effusion is present.

Athoracotomy performed because of progressive stridor and dyspnea showed a fluid- and blood-filled mass adherent to the esophagus. It was completely resected. Histopathological examination showed esophageal mural structure with gastric mucosa (Figure 3). The diagnosis was an esophageal duplication cyst without connection to the lumen of the esophagus.

Esophageal duplications are congenital malformations resulting from aberration of ventral budding of the lung primordium from the embryonic foregut at 3 to 4 weeks' gestation.^{1, 2} They are tubular or cystic mediastinal masses with or without associated congenital anomalies (such as duplication of the small intestine, esophageal atresia, tracheoesophageal fistula, and spinal and/or vertebral anomalies). The overall incidence is estimated as 1 in 8200, with a predominance in males.³ Cystic duplications (or enteric cysts) are more rare and communicate with the esophageal lumen in 10% of patients.⁴

Although most cases are asymptomatic, swallowing difficulty, failure to thrive, or respiratory distress can be caused by displacement or compression of mediastinal structures. These and other clinical signs, including fever or cough, may be produced by enlargement of the cyst as a result of infection or bleeding, most likely to occur in duplication cysts, which contain gastric mucosa. There are few cases of enteric cysts as the cause of stridor in neonates or young infants.^{5, 6}

The differential diagnosis of masses in the upper posterior mediastinum includes bronchogenic cysts and neurogenic neoplasms. Bronchogenic cysts arise from the same embryonic esophageal bud and are more frequent than enteric cysts.⁷ Magnetic resonance imaging and computed tomographic scans clearly demonstrate esophageal duplication cysts.⁸ Barium esophagram may detect duplications of the esophagus, but in cases of a cystic mass of the upper mediastinum, it is not helpful because of the infrequent connection of enteric cysts to the esophageal lumen. Surgical resection of the cyst is the treatment of choice. Thoracoscopic surgery may be used as a method of less invasive treatment for this benign disorder.⁹

Our patient presented with congenital stridor and progressive dyspnea caused by an esophageal duplication cyst. Stridor is a relatively frequent symptom in newborns and young infants and is caused by a variety of diseases or abnormalities. A mass in the upper mediastinum causing inspiratory and expiratory stridor is a rare finding. Respiratory distress necessitated surgery, and the patient was cured by resection of the cyst.

AUTHOR INFORMATION
Accepted for publication February 23, 2000.

Reprints: Carl-Joachim Partsch, MD, Department of Pediatrics, Christian-Albrechts-University Kiel, Schwanenweg 20, D-24105 Kiel, Germany.

REFERENCES
1. Phillapart Al, Farmer DL. Benign mediastinal cysts and tumors. In: O'Neill JA, Rowe MI, Fonkalsrud EW, Coran AG, eds. Pediatric Surgery. St Louis, Mo: Mosby–Yearbook Inc; 1998:839-851. 2. Harmon CM, Coran AG. Congenital anomalies of esophagus. In: O'Neill JA, Rowe MI, Fonkalsrud EW, Coran AG, eds. Pediatric Surgery. St Louis, Mo: Mosby–Yearbook Inc; 1998:941-967. 3. Arbona JL, Fazzi JGF, Mayoral J. Congenital esophageal cysts: case report and review of the literature. Am J Gastroenterol. 1984;79:177-182. ISI | PUBMED 4. Market DJ, Grumbach K, Haney PJ. Thoracoabdominal duplication cyst: prenatal and postnatal imaging. J Ultrasound Med. 1996;15:333-336. PUBMED 5. Stewart RJ, Bruce J, Beasley SW. Oesophageal duplication cyst: another cause of neonatal respiratory distress. J Pediatr Child Health. 1993;29:391-392. 6. Essodegui F, Benjelloun A, Zamiati W, Gharbi A, Ksiyer M. Upper esophageal duplication: apropos of a case disclosed by respiratory distress [in French]. Ann Radiol (Paris). 1996;39:193-196. 7. Nobuhara KK, Gorski YC, LaQuaglia MP, Shamberger RC. Bronchogenic cysts and esophageal duplications: common origins and treatment. J Pediatr Surg. 1997;32:1408-1413. FULL TEXT | ISI | PUBMED 8. Rafal RB, Markisz JA. Magnetic resonance imaging of an esophageal duplication cyst. Am J Gastroenterol. 1991;86:1809-1811. PUBMED 9. Merry C, Spurbeck W, Lobe TE. Resection of foregut-derived duplications by minimal-access surgery. Pediatr Surg Int. 1999;15:224-226. PUBMED

SECTION EDITOR: BEVERLY P. WOOD, MD