Eur J Cardiothorac Surg 2002;21:133-135
© 2002 Elsevier Science NL
Giant dumbbell-shaped gastroenteric cyst presenting with total situs inversus
Murat Akala*,
Erkan Dikmena,
Ay
e Sertçelikb,
Serpil Dizbaysakb
a Department of Thoracic Surgery, Ankara University School of Medicine, 
bn-i Sina Hospital, 06100 Sihhiye, Ankara, Turkey
b Department of Pathology, Ankara University Faculty of Medicine, 06100 Sihhiye, Ankara, Turkey
Received 24 July 2001;
received in revised form 17 October 2001;
accepted 17 October 2001.
* Corresponding author. Tezel Sokak 9/12, 06550 Asa
iayranci, Ankara, Turkey. Tel.: +90-312-4284695
e-mail: akal{at}medicine.ankara.edu.tr
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Abstract
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We present a case of an 8-month-old boy with total situs inversus, who had a giant dumbbell-shaped gastroenteric cyst. The concurrence of these entities has never been reported previously. The cyst occupied a large space in the mediastinum and involved both hemithoraces.
Key Words: Gastroenteric cyst • Total situs inversus
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1. Introduction
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Gastroenteric cysts are unusual developmental cysts encountered in the posterior mediastinum of patients who usually have vertebral abnormalities [1–3]. Other abnormalities are rare. They are more frequent in males and in infants [4]. We are not aware of any report of a gastroenteric cyst occupying the entire mediastinum and presenting with total situs inversus.
We herein report a case of an 8-month-old boy with total situs inversus, who had a giant dumbbell-shaped gastroenteric cyst, located in the mediastinum and involving both hemithoraces.
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2. Case report
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An 8-month-old boy was presented to the pediatric emergency unit with cough, fever and dyspnea. He was referred to our clinic with mediastinal cyst and total situs inversus. He had a history of recurrent pneumonia. Physical examination revealed peripheral cyanosis, 3/6 systolic murmur and absence of breath sounds in the left hemithorax. Heart sounds were audible from the right hemithorax. Arterial blood gas analyses showed hypoxemia and decreased levels of oxygen saturation. Other laboratory data were within the normal limits. Posteroanterior chest roentgenogram showed homogenous opacity localized in the entire left hemithorax, enlarged mediastinum and cardiac opacity in the right hemithorax. Computed tomographic (CT) scan showed a cystic mass; the density of the fluid was about +15 Hounsfield units. One widened portion of the cyst extended into the left hemithorax and other widened portion projected into the right hemithorax. In the left hemithorax, the upper end of the lesion was identified at the thoracic inlet and the lower end, at the crus of the diaphragm. (Fig. 1a). CT scan also showed total situs inversus (Fig. 1b). No vertebral abnormalities were seen. We did not perform bronchoscopy and esophagoscopy because of the poor condition of the infant, and barium contrast study to avoid any aspiration of the radiopaque material into the tracheobronchial tree. We decided an immediate thoracotomy. On left thoracotomy, we observed a cystic lesion measuring 15x10x5 cm3 in the posterior mediastinum, which occupied the whole left hemithorax, extending into the right hemithorax and causing collapse of the left lung. The cyst, which was localized between descending aorta and heart, deplaced left main bronchus, left lower lobe bronchus, also heart and proximal part of inferior vena cava, to anterior. In addition, the cyst caused compression of esophagus but there was no communication with alimantary tract or tracheobronchial tree. The cyst was totally excised. On histological examination, gastric type mucosa, with focal areas of small intestinal differentiation, was observed. Mucosa was ulcerated in some areas. All histologic layers of gastrointestinal wall were present. The cyst was diagnosed as a gastroenteric cyst (Fig. 2). Postoperative course was uneventful. The patient remained well and disease-free 3 years after surgery.
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Fig. 1. (a) CT scan showing a giant dumbbell-shaped cystic mass (arrows outline the borders of the cyst). (b) CT scan showing the contrast localization of liver (L) and spleen (S) consistent with total situs inversus.
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3. Discussion
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Congenital foregut cysts of the mediastinum are extremely rare lesions [5,6]. They constitute approximately 6% of mediastinal lesions in adults, and up to 18% in children. Gastroenteric cysts account for approximately 15% of the foregut cysts [7–9].
Gastroenteric cysts are spherical, unilocular masses with smooth, thin walls [1]. They are commonly located posterior to the carina [1,8,9]. Although two-thirds of these cysts are seen in the right hemithorax, in our case it was mainly located in the left hemithorax and extended into the right hemithorax.
Gastroenteric cysts are often attached to the spine and a great proportion is associated with vertebral abnormalities such as scoliosis, anterior spina bifida, hemivertebrae, butterfly vertebrae or vertebral fusion or scoliosis, whereas other abnormalities are rare [1,2,3]. According to our knowledge, the presented case is the first ever reported gastroenteric cyst presenting with total situs inversus in the literature. These cysts may also be closely associated with the esophagus. They may rarely communicate with the tracheobronchial tree [1,3,7]. In our case, there was no communication either with the tracheobronchial tree or the esophagus.
Gastroenteric cysts are usually present in children, however, 25–30% are found in adults. Adults are usually asymptomatic and the cysts are found incidentally. Bleeding, infection or epithelial secretions may cause mediastinal enlargement and symptomatic compression of the aerodigestive tract [1,4,6]. Infants and children commonly suffer from symptoms of severe airway obstruction or recurrent pneumonia as in the present case. Because of this, routine CT scan is suggested for the children with recurrent pneumonia.
Chest X-rays and CT scan are efficient for diagnosis. Radiologically, foregut cysts manifest as well-marginated, homogenous, spherical mediastinal masses ranging in size from 2 to 10 cm [1]. Bronchoscopy and esophagoscopy also may be helpful in the diagnosis. The presented cyst attained a large size in the mediastinum and involved both hemithoraces, causing a deteriorated condition of the infant. Thus, we preferred an urgent thoracotomy following radiological investigation including chest X-ray and CT.
Despite the extreme rarity of malignancy in mediastinal foregut cysts, definitive diagnosis is obtained by histopathologic examination, and complete excision should always be considered when diagnosed, even in patients without symptoms [3,7]. Partial excision with ‘de-epithelization’ of the residua can be performed if complete excision is not possible. When surgery is not possible, bronchoscopic or thoracoscopic needle drainage may be an alternative. However, these procedures may cause severe complications and in addition, the risk of malignant transformation will still remain [1,3,6,10]. Complete excision was successfully performed in our case despite the large size of the lesion.
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Abstract
1. Introduction
