|Year : 2012 | Volume
| Issue : 6 | Page : 287-289
Foregut duplication cyst: An unusual presentation during childhood
Ahmad Hammoud, Mohammad Hourani, Mouniat Akoum, Mariam Rajab
Department of Pediatrics, Makassed General Hospital, Beirut, Lebanon
|Date of Web Publication||14-Jun-2012|
Department of Pediatrics, Makassed General Hospital, Riad El-Solh Street, P.O. 6301, Beirut 11072210
Source of Support: None, Conflict of Interest: None
Congenital duplications can occur anywhere in the GIT, one third of all duplications are foregut duplications (esophagus, stomach, first and second part of duodenum). Respiratory symptoms are the most common symptoms in foregut duplications, most cases present with respiratory distress which may be present from birth, or symptoms may be insidious with cough, wheeze, or recurrent respiratory infections. We are presenting a 2-year-old boy presenting with cough and fever. Radiological investigation showed left mediastinal mass that was removed by excisional biopsy and revealed an esophageal cyst. Cough with or without fever could be rare presentations for esophageal cyst.
Keywords: Foregut duplication cyst, Congenital malformation, Esophageal duplication
|How to cite this article:|
Hammoud A, Hourani M, Akoum M, Rajab M. Foregut duplication cyst: An unusual presentation during childhood. North Am J Med Sci 2012;4:287-9
|How to cite this URL:|
Hammoud A, Hourani M, Akoum M, Rajab M. Foregut duplication cyst: An unusual presentation during childhood. North Am J Med Sci [serial online] 2012 [cited 2021 Jan 16];4:287-9. Available from: https://www.najms.org/text.asp?2012/4/6/287/97213
| Introduction|| |
Congenital duplications can occur anywhere in the GIT, one third of all duplications are foregut duplications (esophagus, stomach, first and second part of duodenum).  These duplications may be proximal or distal, usually proximal or mediastinal occur early in embryonic life. 
Foregut duplications show predominance in girls especially if there is broncho-pulmonary involvement.  Diagnosis is usually made early in life, with a mean age of diagnosis of 18 months, but diagnosis has been made as early as at birth, and as late as the end of the first decade, asymptomatic cases may escape detection until adulthood. 
Respiratory symptoms are the most common in foregut duplications, due to its position. Most cases present with respiratory distress, which may be present from birth, sometimes the symptoms may be of sudden onset with cough, wheeze, or recurrent respiratory infections. In rare cases the cyst may perforate into the bronchial tree, and the patient may present with respiratory distress and hemoptysis. 
Extension of the cyst into the infradiaphragmatic area may cause gastrointestinal symptoms; and extension into the neural canal can cause signs of spinal cord compression. 
Gastric mucosa is the predominant mucosa of foregut duplication cysts.  More than one epithelial cell type may line the duplication cyst. Adenocarcinoma has been reported as malignant degeneration in an intrathoracic duplication cyst of foregut origin. 
We are presenting a case of esophageal duplication cyst in a 2-year old, presenting with cough and fever.
| Case Report|| |
The patient is a 2-year-old boy, product of cesarean section, to a G2P1A1 mother. The boy was well vaccinated except for PCV and ROTA vaccine, otherwise up to date. He was previously healthy, until he presented with cough of 10 days and fever of 3 days duration.
The cough was dry and mainly at bed time and was not relieved by cough syrup, with no change in character since 10 days and no cyanotic attacks. The fever was moderate with no chills and responding well to antipyretics. There was also slightly decreased in appetite and activity, loose stool, no vomiting, and no respiratory changes with activity.
His past medical history showed acute tonsillitis 3 month prior to presentation with no history of previous hospitalization and no history of surgical intervention. Family history showed a history of lung cancer with the boy's grandmother.
Upon presentation, the boy was well looking, not in distress, and the complete physical examination was normal for a child in his age. Chest radiography was done and showed left posterior mediastinal mass.
Upper gastrointestinal series (Barium Swallow) showed normal esophagus, stomach and duodenum, with a rounded left basal mediastinal mass but not affecting the esophagus or compressing it [Figure 1].
|Figure 1: (a and b) Chest X-ray showing mediastinal mass (a) PA view, retro-cardiac in position (b) Lateral view, Posterior mediastinal in position. (c and d) Upper Gastrointestinal series (Barium swallow) showing normal esophagus, stomach and duodenum, with a rounded left basal Mediastinal mass but not affecting the esophagus. Follow the tip of the white arrows in all images.|
Click here to view
CT scan of chest and mediastinum was then done and showed a 3.5 × 3 × 2 cm3 well-defined, encapsulated, cyst-like lesion of the posterior mediastinum in a left para-aortic/para-vertebral location. The lesion is in close relation to the aorta and bronchus to the left lower lobe. There is an enhancing capsule with enhancing content with evidence of peri-lesional thickening of pleura.
Next step was an MRI of the dorsal spine, which showed a left-sided posterior mediastinal mass extending along T6 through T8 vertebrae. It is relatively well defined, encasing the aorta, abutting the vertebrae, costovertebral and costotransverse joints, and is associated with lung infiltrates over lied by a thin layer of pleural fluid [Figure 2].
|Figure 2: MRI of dorsal spine showing a left-sided posterior mediastinal mass, extending from T6 to T8, relatively well defined associated with lung infiltrates over lied by a thin layer of pleural fluid. Follow the tip of the white arrows in both images (a and b)|
Click here to view
In order to establish a diagnosis an excisional biopsy, so surgery was done and pathology result showed regular non-keratinizing stratified squamous epithelium, focally blending with the respiratory type peuodostratified columnar epithelium. The cyst contained around 50 ml of white turbid fluid. Smears, cytospin and cell block showed superficial mature keratinocytes with no evidence of atypia.
| Discussion|| |
The first case of esophageal duplication cyst was reported in 1711 by Balasius. 
An embryonic defect in vacuolization of the esophagus results in duplication, and it normally occurs in the sixth week of gestation. , When the foregut epithelium develops and elongates, the lumen forms then undergoes dextro-rotations; therefore, the majority of esophageal duplications occur distally and on the right.  Our case is a proximal duplication cyst located on the left.
Robert Carachi and Amir Azmy compared 21 patients with foregut duplication at the Royal Hospital for Sick Children from 1957 to 1999. Out of 21, 13 had respiratory symptoms, with 3 having cough, one with hemoptysis, one with tachypnea and one had chronic cough. The mean age at diagnosis was 18 months.  A retrospective study done by Takeda et al., addressed the symptoms of mediastinal congenital cysts, out of 105 patients 4 had esophageal duplications, and out of these 4, 3 were asymptomatic and 1 had dyspnoea and dysphagia.  New onset cough and fever as the presentation for esophageal duplication cyst was rarely reported. Nakao et al.  reported a case, were a 12-year old girl presented with fever and cough of 7 days duration and was found to have an infected duplication cyst, in this case the girl had a rapidly growing cyst which was infected, back to our case the child had cough and fever, the fever had no apparent focus, physical examination, labs, and cultures could not explain the cause of fever. Surgery and pathology showed no signs of infection with in the cyst, so there was no apparent infection to link the fever to.
Chronic cough has been described as a symptom of esophageal duplicated cyst, but the cases were mostly adult cases, that remained asymptomatic till the time they presented with this chronic cough. , Therefore, cough has been reported before in adult cases, but nearly all presented as chronic cough, acute onset of cough without any other respiratory symptom (dyspnoea, tachypnea, distress, and hemoptysis) has only been reported in few cases such as Nakao et al.'s  and ours.
| Conclusion|| |
Esophageal duplicated cyst, although rare, should be considered as one of the differential diagnoses of a mediastinal mass. It may have various presentations, it usually presents before the age of 2 year and is usually found on the right side. Symptoms are usually respiratory or gastrointestinal; of the respiratory symptoms, cough is a rare presentation, especially a cough of new onset and not associated with any other respiratory symptoms. The presence of fever without apparent infection also makes this a new presentation. So this makes our case: A 2-year-old boy having a left mediastinal esophageal cyst, presenting with new onset of cough and fever, a case worth reporting.
| References|| |
|1.||Hocking M, Young DG. Duplications of the alimentary tract. Br J Surg 1981;68:92-6. |
|2.||Gray SM, Skandalakis VE. The embryological basis for the treatment of congenital defects. In: Gray SM, Skandalakis VE, editors. Embryology for surgeons. 1 st ed. Philadelphia: WB Saunders; 1972. p. 63-100. |
|3.||Ildstad ST, Tollerud DJ, Weiss RG, Ryan DP, McGowan MA, Martin MW. Duplications of the alimentary tract. Clinical characteristics, preferred treatment and associated malformations. Ann Surg 1988;208:184-9. |
|4.||Dresler CM, Patterson GA, Taylor BR, Moore DJ. Complete foregut duplication. Ann Thorac Surg 1990;50:306-8. |
|5.||Burgner DP, Carachi R, Beattie TJ. A thoracic foregut duplication cyst presenting with neonatal respiratory distress and haemoptysis. Thorax 1994;49:287-8. |
|6.||Velasco-Siles JM, Paredes E, Escanero A, Alcala H. Spinal cord compression due to cystic duplication of the primitive digestive tract. Childs Nerv Syst 1986;2:159. |
|7.||Bissler JJ, Klein RL. Alimentary tract duplications in childhood case and literature review. Clin Pediatr (Phila) 1988;27:152-7. |
|8.||Chnang MT, Barba FA, Kaneko M, Tierstein AS. Adenocarcinoma arising in an intrathoracic duplication of foregut origin: A case report and review of the literature. Cancer 1981;47:1887-90. |
|9.||Arbona JL, Fazzi JG, Mayoral J. Congenital esophageal cysts: Case report and review of literature. Am J Gastroenterol 1984;79:177-82. |
|10.||Shew SB, Holcomb GW III. Alimentary tract duplications. In: Ashcraft KW, Murphy JP, Holcomb GW III, editors. Pediatric Surgery. 4 th ed. Amsterdam: Elsevier; 2005.p. 543-52. |
|11.||Lund DP. Alimentary tract duplication. In: Grosfeld J, O'neill J, Fonkalsrud E, Coran A, editors. Pediatric surgery. Toronto: Judith Fletcher; 2006. p. 1389-98. |
|12.||Nazem M, Amouee AB, Eidy M, Khan IA, Javed HA. Duplication of cervical oesophagus: A case report and review of literatures. Afr J Paediatr Surg 2010;7:203-5. |
|13.||Carachi R, Azmy A. Foregut duplications. Pediatr Surg Int 2002;18:371-4. |
|14.||Takeda SI, Miyoshi S, Minami M, Ohta M, Masaoka A, Matsuda H. Clinical spectrum of mediastinal cysts. Chest 2003;124:125-32. |
|15.||Nakao A, Urushihara N, Yagi T, Choda Y, Hamada M, Kataoka K, et al. Case report: Rapidly enlarging esophageal duplication cyst. J Gastroenterol 1999;34:246-9. |
|16.||Bravo LO, Walls JG, Ly JQ, Lisanti CJ, Roberts SP. Esophageal duplication cyst presenting as chronic cough. Chest 2003; 124:263-4. |
|17.||Bowton DL, Katz PO. Esophageal cyst as a cause of chronic cough. Chest 1984;86:150-2. |
[Figure 1], [Figure 2]