Esophageal duplication cyst presenting with stridor in a child with congenital pulmonary airway malformation: A case report and literature review

Abstract

Rationale: Esophageal duplication cyst (EDC) is a rare developmental aberration originated from the embryonic foregut. It may remain asymptomatic but produce local mass effect on surrounding organs if rapid enlarges. EDC may sometimes accompany with other congenital malformations. Congenital pulmonary airway malformation (CPAM) is a congenital lung malformation with an unknown chance of developing symptoms. Here we report a rare case of esophageal duplication cyst with type 2 congenital pulmonary airway malformation (CPAM).

Patient concerns: A 16-month old boy with a prenatal diagnosis of type 2 CPAM presented progressive stridor and respiratory distress and was admitted to our hospital under the diagnosis of pneumonia. The patient responded poorly to antibiotics. A chest Xray (CXR) showed consolidation over the left upper lobe with trachea deviated to right side. A chest computed tomography (CT) revealed a cystic lesion sized 3.3 × 3.3 cm in the superior mediastinum.

Diagnoses: Post-operative pathological report confirmed the diagnosis of esophageal duplication cyst.

Interventions: We pre-medicated the patient with steroids and inhaled bronchodilators for airway maintenance. Then the patient received tumor resection via median sternotomy.

Outcomes: The patient recovered without complication and discharged smoothly 4 days after the surgery.

Lessons: EDC is a rare but potentially life-threatening disease owning to compression of large airways. Chest CT scan could detect the lesion non-invasively and should be considered in patients with persistent stridor, as well as CXR findings of the trachea deviated by a mass lesion in mediastinum, especially for those with CPAM.

Figure 1

(A) The chest CT after birth showing a cystic lesion measuring 2.1 × 1.3 cm at left upper lung field with some solid component, consistent with CPAM. (B) The CXR showed consolidation in the left upper lobe and the trachea was deviated to the right side. (C) The CT showed a well-encapsulated cystic lesion up to 3.3 × 3.3 cm in the left superior mediastinum, which contained homogeneous content and alveolar consolidation lesion in the left upper lung consistent with lung infection. (D) The cyst contained some yellowish turbid fluid and there was no fistula, adhesion, nor focal inflammatory change.

Figure 2

(A) The cystic epithelial lining presents predominantly stratified squamous epithelium. (B) A focal ciliated columnar epithelium is noted without associated gland or cartilage.