www.elsevier.com/locate/jpedsurg

Journal of Pediatric Surgery (2008) 43, 1401–1404

Prenatal diagnosis of an intrathoracic gastric duplication:a case report☆

Paul Daher, Lamisse Karam, Edward Riachy⁎

Department of Pediatric Surgery, Hotel Dieu de France University Hospital-Beirut, Saint Joseph University,Achrafieh, PO Box 16-6830, Beirut, Lebanon

Received 9 January 2008; revised 14 March 2008; accepted 17 March 2008

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Key words:Gastric duplication;Intrathoracic cyst;Prenatal diagnosis

Abstract We report an intrathoracic gastric duplication cyst detected antenatally on obstetricalultrasonography at 33 weeks of gestation. After birth, the newborn was directly intubated. At day 19,the intrathoracic cyst was removed, and the patient discharged on his 27th day of life. Histologic findingsconfirmed the diagnosis of a gastric duplication cyst. This appears to be the first reported case of anintrathoracic gastric duplication detected antenatally. Intrathoracic gastric duplication should be amongthe differential diagnosis of any mediastinal cyst discovered antenatally; its detection allows appropriatemanagement at birth.© 2008 Elsevier Inc. All rights reserved.

Alimentary tract duplications are considered to be arelatively rare congenital abnormality. They can be foundanywhere from the tongue to the anus, and only 20% ofthem are located in the thorax. Gastric duplications accountfor less than 4% of all enteric duplications [1]. They areusually cystic, not communicating with the stomach andlocated most commonly in the greater curvature, antrum, orpylorus [2]. The presence of gastric mucosa suggests thatthey should be rapidly resected once diagnosed to avoidpotential morbidity and mortality [3-5]. Prenatal detection of

☆ All authors cited in this article have made substantial contributions toll of the following: (1) the conception, analysis, and interpretation of therticle, (2) drafting the article and revising it critically for importanttellectual content, and (3) final approval of the version to be submitted.⁎ Corresponding author. Tel.: +961 3 998923; fax: +961 1 615300x7325.E-mail addresses: jmpaul@dm.net.lb (P. Daher),

ddy_riachy@msn.com (E. Riachy).

022-3468/$ – see front matter © 2008 Elsevier Inc. All rights reserved.oi:10.1016/j.jpedsurg.2008.03.046

gastric duplication allows appropriate management andapproach at birth. As far as we know, all previously detectedgastric duplications were located in the abdomen [6-9]. Wereport what we believe is the first case of an isolatedintrathoracic gastric duplication detected on a routineprenatal ultrasound examination.

1. Case report

A routine obstetrical ultrasonography was performed at33 weeks of gestation of an otherwise uneventfulpregnancy. It showed oligohydramnios, ascites, and a 52-mm-wide right thoracic cyst (Fig. 1). At 37 weeks ofgestation, she delivered a male by cesarean delivery with aweight of 2400 g, a height of 34.5 cm, and headcircumference of 37 cm.

He was rapidly intubated at birth to prevent a respiratorydistress. Chest x-ray showed a bilateral pleural effusion,

Fig. 1 A routine obstetrical ultrasonography showing a 52-mm-wide right thoracic cyst of the fetus.

ig. 3 Chest x-ray showing a deviation of the nasotracheal tubethe left by an opaque mass in the right hemithorax. Vertebralalformations can also be seen.

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predominantly on the right. Thoracocentesis was performedusing a thoracic drain and left for 5 days. Cardiac andabdominal ultrasounds showed no abnormalities. Renalfunction was within the normal limits. The newborn wasextubated on day 14 but still needed to be placed under ahood with FiO2 of 60%. A cervicothoracic magneticresonance imaging was performed and showed manyvertebral abnormalities including C6 through C7 fusionand hemivertebras, syringomyelia, and a cyst of the righthemithorax measuring about 52 mm without any spinalcommunication (Fig. 2).

He was transferred to our neonatal intensive care unit onthe 18th day of life where he was intubated and ventilated ashe still presented a respiratory distress. Potential diagnosisincluded bronchogenic cyst and a neurenteric cyst oranother form of foregut duplication. The x-ray showed adeviation of the nasotracheal tube to the left by an opaquemass in the right hemithorax (Fig. 3).

Fig. 2 A, A magnetic resonance imaging showing syringomyelia (nehemithorax without any spinal communication.

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Surgery was performed at day 19 by a right lateralthoracotomy. A thoracoscopic resection was discussed, butthe large size of the mass, the low weight of the newborn, andthe risk of a systemic vascularization made us prefer an openaccess. A space-occupying cyst of the right hemithorax wasfound, explaining the neonatal respiratory distress syndrome.The cyst was easily excised by electrocautery. Its serouscontent was sent to culture. No communications were seenwith other thoracic structures including esophagus andanterior vertebral bodies. The esophagus was easilyidentified during surgery, thereby did not necessitate anyadditional procedure to verify its integrity. Postoperatively,the newborn recovered appropriately permitting us a rapidweaning from ventilation and discharging him from thehospital on his 27th day of life. On pathologic examination,the cyst was entirely lined by simple mucinous columnargastric epithelium (ie, no squamous epithelium was found)

xt to the “x” symbol). B, A space occupying cyst (⁎) of the right

Fig. 4 Pathologic examination showing on the left a simple mucinous columnar gastric epithelium; and on the right, a well-definedmuscle wall.

1403Intrathoracic gastric duplication

and had a well-defined muscle wall making the diagnosis ofgastric duplication (Fig. 4).

2. Discussion

Thoracic cysts pose a diagnostic challenge, with multiplepotential pathologic findings. Possibilities include broncho-genic cysts, neurenteric cysts, and other forms of alimentarytract duplications including gastric duplication. Thoracicforegut duplications may present at any age, although themajority present before the age of 2 years [10-11]. Antenataldetection of the cyst allows appropriate management at birth,early investigations, and quick removal [12]. We believe thatthis is the first reported case of an intrathoracic prenatallydetected gastric duplication. Prenatal detection is very crucialas it leads to prompt management and rapid removal.

Conservative treatment is still controversial as asympto-matic patients may later present with symptoms related tocyst growth [13]. On the other hand, early neonatal resectionof the duplication cyst has been advocated because ofpotential complications [14]. Infection of a mediastinal cystmay lead to serious mediastinitis. Malignant transformationhas also been described [2,15]. The presence of an ectopicgastric tissue in the lining of the cyst may result in life-threatening hematemesis or hemoptysis if the cyst fistulatesinto the bronchus. The risk of bleeding was confirmed in theseries of Foley et al [16] and Nakazawa et al [6]—thehistopathologic examination of the cyst revealed gastrictissue with mucosal erosions. In our case, the surgicalapproach was advocated because of the space occupyingeffect of the cyst. In fact, the 52-mm-wide cyst occupiedalmost the whole right hemithorax, which made it imperativeto directly intubate the newborn at birth to avoid a neonatalrespiratory distress syndrome.

The etiology of enteric duplication remains speculative.The split notochord theory proposes that abnormal separa-tion of the notochord from the endoderm causes entericduplications [17]. It remains that gastric duplication is a rareentity especially in its thoracic location [18-22]. However, itis still a benign lesion, and its treatment should be simple.

The outcome of the infants who undergo surgical treatmentduring the neonatal period is usually uneventful [14].

In conclusion, it seems that our case is the first isolatedantenatally diagnosed intrathoracic gastric duplicationreported in the literature. Although intrathoracic gastricduplication is very rare, it should be among the differentialdiagnosis of any mediastinal cyst.

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