Journal of Postgraduate Medicine
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Year : 1994  |  Volume : 40  |  Issue : 4  |  Page : 228-30  

Intrathoracic enteric foregut duplication cyst.

BJ Birmole, BK Kulkarni, AS Vaidya, SS Borwankar 
 Dept of Paediatric Surgery, KEM Hospital, Parel, Bombay, Maharashtra.

Correspondence Address:
B J Birmole
Dept of Paediatric Surgery, KEM Hospital, Parel, Bombay, Maharashtra.


A one month old male child presented with respiratory distress since day 10 of life. There was intercostal retraction and decreased air entry on the right side. Investigations revealed a well defined cystic mass in the posterior mediastinum with vertebral anomalies, the cyst was excised by posterolateral thoracotomy. Histopathology revealed it to be an enteric foregut duplication cyst.

How to cite this article:
Birmole B J, Kulkarni B K, Vaidya A S, Borwankar S S. Intrathoracic enteric foregut duplication cyst. J Postgrad Med 1994;40:228-30

How to cite this URL:
Birmole B J, Kulkarni B K, Vaidya A S, Borwankar S S. Intrathoracic enteric foregut duplication cyst. J Postgrad Med [serial online] 1994 [cited 2023 Sep 24 ];40:228-30
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  ::   IntroductionTop

Duplications of alimentary tract are spherical or tubular structures occurring anywhere from tongue to the anus. The characteristic features described are (1) attachment to at least one point of alimentary tract (2) well developed coat of smooth muscle and (3) epithelium resembling some part of alimentary tract. Among alimentary duplication, 20% occur in thorax[1]. The majority of duplications are found in children, the frequency being almost triple to that found in adults[1]. Foregut duplication cysts constitute 10% of all mediastinal tumours[2],[3]. Three types of foregut cysts are described: bronchogenic, intramural esophageal and enteric. The diagnosis is basically histological, however vertebral anomalies associated with posterior mediastinal cyst is a feature of enteric cyst. The commonest mode of presentation in neonatal period is respiratory distress. Enteric cyst is uncommon and out of 15 cases of foregut cyst in children reported by Cohen, et al[4] none was of enteric variety. This article reports a case with all the characteristic features of enteric variety of foregut duplication cyst.

  ::   Case reportTop

A one-month-old male child weighing 2.75 kg presented with respiratory distress since day 10 of life. There was no history of cough, fever, cyanosis or vomiting after feeds. On examination, the child was thriving well and had a good cry and activity. There was intercostal retraction and air entry was decreased on the right side. Plain Xray chest [Figure:1] revealed a rounded radioopaque mass on right side of midline, emphysematous right lung with pleural herniation and vertebral anomalies extending from T1 to T4. Esophagogram showed deviation of esophagus to left. Computerized tomographic scan [Figure:2] revealed encysted, well defined mass about 3 cm in diameter just anterior to vertebra on right side of midline. There was no intraspinal extension. Right posterolateral thoracotomy was performed.

The cyst could be easily dissected all around [Figure:3]. It was attached by a fibrous cord to the higher vertebra. The cyst was excised.

Postoperative course was uneventful. On histopathology, the cyst wall was found to be lined by columnar epithelium, flattened at places. Goblet cells were seen and had two well developed muscle layers, No gastric mucosa was seen.

  ::   DiscussionTop

The earliest description of foregut duplication was that of Blassius and Bremer[5] in 1711. In 1881, Roth described the association of oesophageal duplications and vertebral anomalies. The first surgical excision was performed by Sauerbuch in 1931. Gray and Skanalakis[6] describe three categories of intrathoracic foregut cysts: bronchogenic, intramural oesophageal and enteric. No single theory can account for all the observed types of thoracic foregut cysts. Bronchogenic cysts result from abnormal budding of the bronchial tree. Intramural oesophageal cysts result from defects in vacuolation process and at e never associated with vertebral anomalies. The most accepted theory for enteric cyst is that of the split notochord syndrome with persistence of a neurenteric canal. Enteric cysts are usually separate from the oesophagus and lie in the posterior mediastinum. They are commonly associated with vertebral anomalies and are part of split notochord syndrome. In 1952, Veenklaas theorised that it separation of the notochord from the entoderm was not complete, a diverticulum of entoderm could be withdrawn from the primitive foregut which would form a cyst and the attachment of the cyst to the notochord could prevent fusion of the vertebral bodies, resulting in a spinal anomaly.

Intrathoracic duplications can present with wide spectrum of symptoms and can be life threatening. Precise location and type decide the symptoms. Dyspnoea, stridor or persistent cough is the commonest presentation[7],[8]. Respiratory distress occurs most commonly in small infants because of pressure on bronchi or lung. Dysphagia due to pressure on oesophagus and cardiac arrhythmias due to retrocardiac duplication is known. Various other diagnoses such as bronchial asthma, vascular ring, foreign body and pneumonia may be entertained[4]. Accidental incision and drainage of supra-clavicular extension of duplication cyst has been reported[8]. Chest pain, epigastric pain, recurrent respiratory tract infection is the mode of presentation in older children. The foregut cysts sometimes remains asymptomatic for months or years[4],[9]. Vertebral anomalies can produce limitation of movement of the neck, scoliosis, root compression and pain. Peptic ulceration within a duplication can incite bleeding into the lung (haemoptysis), pleural cavity or oesophagus. Penetration of aorta has occurred with fatal terminus. Intraspinal anomalies can coexist in 25% of patients and later on can cause symptoms either by compression or infection. Only 2 out of 6 had presented before 1 month of age[10]. In Cohen's, et a1[4] series of 15 cases one of the patient was under the age of 1 month. In a collected series of 25 patients with thoracoabdominal duplications there were 12 neonates presenting mainly with respiratory symptoms[11].

Plain Xray chest showing mass in posterior mediastinum mainly on right side, vertebral anomalies usually of higher vertebra is diagnostic of enteric cyst. All patients except one had vertebral anomalies in Ahmed, et al's series. The cysts may be considerably caudal to the vertebral anomaly[12]. Mediastinal mass may contain air if a communication exists with some part of the alimentary tract[9]. Only 20% of patients had obstructive emphysema in Cohen et al's series and such patients presented early. Communication with gastrointestinal tract and deviation of oesophagus can be demonstrated by esophagogram[4]. Intraspinal anomalies coexisted with mediastinal masses in almost 25% of patients in Superina, et al's series[7] and were often initially asymptomatic. He recommended that myelography be carried out in all patients with cystic duplication of esophagus who have vertebral anomalies. CT scan done in our patient ruled out the pathology.

The tendency of enteric cyst to enlarge and produce airway obstruction is sufficient reason for early surgical removal[4]. If there are two components of involvement, the symptomatic part should be excised first. It both mediastinal and intraspinal masses are asymptomatic, the spinal lesion should probably be removed first to avoid neurological deterioration caused by the spinal cyst at the time of removal of mediastinal cyst.

About two thirds of foregut duplications are right sided[13]. Patients who have one duplication are found to have another[9]. Enteric variety has variable types of epithelium and well-developed muscular wall, which resembles that of the intestine. Ectopic mucosa representing any part of intestine can be found, most common being gastric mucosa making it liable to perforate and bleed[4]. Cartilage is never found in the wall. All the characteristic histological features were present in our patient. Although adults have had malignancy developing in duplication, it has not been reported in children[14].


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