Journal of Pediatric Surgery (2008) 43, 1401–1404
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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
Key words: Gastric duplication; Intrathoracic cyst; Prenatal diagnosis
Abstract We report an intrathoracic gastric duplication cyst detected antenatally on obstetrical ultrasonography 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 findings confirmed the diagnosis of a gastric duplication cyst. This appears to be the first reported case of an intrathoracic gastric duplication detected antenatally. Intrathoracic gastric duplication should be among the differential diagnosis of any mediastinal cyst discovered antenatally; its detection allows appropriate management at birth. © 2008 Elsevier Inc. All rights reserved.
Alimentary tract duplications are considered to be a relatively rare congenital abnormality. They can be found anywhere from the tongue to the anus, and only 20% of them are located in the thorax. Gastric duplications account for less than 4% of all enteric duplications [1]. They are usually cystic, not communicating with the stomach and located most commonly in the greater curvature, antrum, or pylorus [2]. The presence of gastric mucosa suggests that they should be rapidly resected once diagnosed to avoid potential morbidity and mortality [3-5]. Prenatal detection of
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All authors cited in this article have made substantial contributions to all of the following: (1) the conception, analysis, and interpretation of the article, (2) drafting the article and revising it critically for important intellectual content, and (3) final approval of the version to be submitted. ⁎ Corresponding author. Tel.: +961 3 998923; fax: +961 1 615300x7325. E-mail addresses:
[email protected] (P. Daher),
[email protected] (E. Riachy). 0022-3468/$ – see front matter © 2008 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2008.03.046
gastric duplication allows appropriate management and approach at birth. As far as we know, all previously detected gastric duplications were located in the abdomen [6-9]. We report what we believe is the first case of an isolated intrathoracic gastric duplication detected on a routine prenatal ultrasound examination.
1. Case report A routine obstetrical ultrasonography was performed at 33 weeks of gestation of an otherwise uneventful pregnancy. It showed oligohydramnios, ascites, and a 52mm-wide right thoracic cyst (Fig. 1). At 37 weeks of gestation, she delivered a male by cesarean delivery with a weight of 2400 g, a height of 34.5 cm, and head circumference of 37 cm. He was rapidly intubated at birth to prevent a respiratory distress. Chest x-ray showed a bilateral pleural effusion,
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Fig. 1 A routine obstetrical ultrasonography showing a 52-mmwide right thoracic cyst of the fetus.
predominantly on the right. Thoracocentesis was performed using a thoracic drain and left for 5 days. Cardiac and abdominal ultrasounds showed no abnormalities. Renal function was within the normal limits. The newborn was extubated on day 14 but still needed to be placed under a hood with FiO2 of 60%. A cervicothoracic magnetic resonance imaging was performed and showed many vertebral abnormalities including C6 through C7 fusion and hemivertebras, syringomyelia, and a cyst of the right hemithorax measuring about 52 mm without any spinal communication (Fig. 2). He was transferred to our neonatal intensive care unit on the 18th day of life where he was intubated and ventilated as he still presented a respiratory distress. Potential diagnosis included bronchogenic cyst and a neurenteric cyst or another form of foregut duplication. The x-ray showed a deviation of the nasotracheal tube to the left by an opaque mass in the right hemithorax (Fig. 3).
P. Daher et al.
Fig. 3 Chest x-ray showing a deviation of the nasotracheal tube to the left by an opaque mass in the right hemithorax. Vertebral malformations can also be seen.
Surgery was performed at day 19 by a right lateral thoracotomy. A thoracoscopic resection was discussed, but the large size of the mass, the low weight of the newborn, and the risk of a systemic vascularization made us prefer an open access. A space-occupying cyst of the right hemithorax was found, explaining the neonatal respiratory distress syndrome. The cyst was easily excised by electrocautery. Its serous content was sent to culture. No communications were seen with other thoracic structures including esophagus and anterior vertebral bodies. The esophagus was easily identified during surgery, thereby did not necessitate any additional procedure to verify its integrity. Postoperatively, the newborn recovered appropriately permitting us a rapid weaning from ventilation and discharging him from the hospital on his 27th day of life. On pathologic examination, the cyst was entirely lined by simple mucinous columnar gastric epithelium (ie, no squamous epithelium was found)
Fig. 2 A, A magnetic resonance imaging showing syringomyelia (next to the “x” symbol). B, A space occupying cyst (⁎) of the right hemithorax without any spinal communication.
Intrathoracic gastric duplication
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Fig. 4 Pathologic examination showing on the left a simple mucinous columnar gastric epithelium; and on the right, a well-defined muscle wall.
and had a well-defined muscle wall making the diagnosis of gastric duplication (Fig. 4).
2. Discussion Thoracic cysts pose a diagnostic challenge, with multiple potential pathologic findings. Possibilities include bronchogenic cysts, neurenteric cysts, and other forms of alimentary tract duplications including gastric duplication. Thoracic foregut duplications may present at any age, although the majority present before the age of 2 years [10-11]. Antenatal detection of the cyst allows appropriate management at birth, early investigations, and quick removal [12]. We believe that this is the first reported case of an intrathoracic prenatally detected gastric duplication. Prenatal detection is very crucial as it leads to prompt management and rapid removal. Conservative treatment is still controversial as asymptomatic patients may later present with symptoms related to cyst growth [13]. On the other hand, early neonatal resection of the duplication cyst has been advocated because of potential complications [14]. Infection of a mediastinal cyst may lead to serious mediastinitis. Malignant transformation has also been described [2,15]. The presence of an ectopic gastric tissue in the lining of the cyst may result in lifethreatening hematemesis or hemoptysis if the cyst fistulates into the bronchus. The risk of bleeding was confirmed in the series of Foley et al [16] and Nakazawa et al [6]—the histopathologic examination of the cyst revealed gastric tissue with mucosal erosions. In our case, the surgical approach was advocated because of the space occupying effect of the cyst. In fact, the 52-mm-wide cyst occupied almost the whole right hemithorax, which made it imperative to directly intubate the newborn at birth to avoid a neonatal respiratory distress syndrome. The etiology of enteric duplication remains speculative. The split notochord theory proposes that abnormal separation of the notochord from the endoderm causes enteric duplications [17]. It remains that gastric duplication is a rare entity especially in its thoracic location [18-22]. However, it is still a benign lesion, and its treatment should be simple.
The outcome of the infants who undergo surgical treatment during the neonatal period is usually uneventful [14]. In conclusion, it seems that our case is the first isolated antenatally diagnosed intrathoracic gastric duplication reported in the literature. Although intrathoracic gastric duplication is very rare, it should be among the differential diagnosis of any mediastinal cyst.
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