Intrathoracic Foregut Duplication Cysts in Neonates

Abstract

Alimentary tract duplications are rare congenital anomalies that can present diagnostic and therapeutic difficulties. Although they can occur anywhere from the mouth to the anus, they are commonly seen in relation to the ileum.1,2 Intrathoracic foregut duplications, on the other hand, are even less common and present with a variety of symptoms, most commonly airway or esophageal obstruction. The high incidence of associated thoracic or cervical vertebral anomalies with foregut duplications should provide an early clue to the diagnosis, as management delay in such cases may be fatal. 3 The purpose of this report is to present two newborns with this anomaly and to discuss aspects of diagnosis and management. Case Reports Case 1A 10-day-old male infant was referred to our hospital because of severe respiratory distress. He was a product of a full-term normal vaginal delivery to a G4 P3 mother, and had an Apgar score of 8 and 9 at 1 and 5 minutes. At the age of 3 hours, he developed respiratory distress and was diagnosed with meconium aspiration. His examination was normal, apart from tachypnea with intercostal and subcostal retraction as well as diminished air entry on the left upper chest with a few crepitations on the right side. His chest x-ray showed hemivertebrae in the midthoracic region and haziness on the left upper lobe. The trachea was deviated to the left side and there was a mass on the right side. His CT scan of the chest revealed congenital anomalies of the upper thoracic vertebrae, including hemivertebrae. There was a large posterior mediastinal mass on the right side, which extended from the upper tracheal region to the diaphragm, and caused displacement of the heart, trachea, and esophagus. His MRI of the chest (Figure 1) showed a large prevertebral cystic mass of about 4 x 3.5 x 5 cm, mainly on the right side. There was also a cleft in the central area of upper thoracic vertebral bodies at the D4-D5 level. The cleft in the midline was connected to the cystic mass, most likely via dorsal stalk as well as tethering of the cord at the site of the cleft anteriorly. The patient underwent a right thoracotomy, which revealed a large cystic mass in the posterior mediastinum. There was a good line of cleavage between the mass and the esophagus. The mass was excised totally and the neurosurgeon dissected the stalk until about half the depth of vertebra, where it ended and was ligated and divided. There was no sign of any CSF leakage and the cyst was probably attached to the dura only. Histology revealed a 5 x 3 cm cyst lined partly with respiratory epithelium and stratified squamous epithelium, but mainly with intestinal epithelium, with some neurogenous elements as well.

Congenital cystic adenomatoid malformation associated with esophageal duplication cyst

“…EDCs are usually found incidentally in asymptomatic patients, but they can present in early infancy with respiratory or esophageal obstructive symptoms if they attain a large size or if they develop complications such as infection or hemorrhage. 2 In our patient, EDC cyst was an incidental finding both radiologically and intraoperatively.…”

“…EDCs are known to be associated with other congenital anomalies such as spinal abnormalities, midgut duplications and tracheoesophageal fistula. 2 , 3 When foregut duplication cysts are associated with vertebral malformations, they are called neuroenteric cysts to differentiate them from simple foregut duplication cysts. 2 The coexistence of both congenital EDC and congenital bronchopulmonary malformations is rare, but has been reported.…”

“…Intrathoracic foregut duplication cysts are also rare, and depending on their size, may be asymptomatic and discovered incidentally, or may present with a variety of symptoms, most commonly airway or esophageal obstruction. 2 The coexistence of both CCAM and esophageal duplication cyst (EDC) is extremely rare. 3 , 4 This is a case report of CCAM in association with an EDC in a 12-year-old child.…”

Yenidoğan döneminde nadir bir solunum sıkıntısı nedeni: posterior mediyastinal gastroenterik kist

“…Enteric cyst is a developmental disorder of the foregut containing esophageal duplication and neuroenteric cysts (6). The term of "neuroenteric cyst" is used to define the loss of association of notachord connection secondary to vertebral or neural anomalies to the foregut.…”

Case report: Antenatal MRI diagnosis of esophageal duplication cyst

“…[1] Antenatal presentation is very rare. It is one of the causes of acute respiratory distress in infancy and childhood.…”