Eur Arch Otorhinolaryngol (2007) 264:1357–1360 DOI 10.1007/s00405-007-0364-3
MI SCE LLANEOUS
Mediastinal foregut duplication cyst presenting as a rare cause of breathing diYculties in an adult A. Espeso · S. Verma · P. Jani · H. SudhoV
Received: 25 March 2007 / Accepted: 23 May 2007 / Published online: 27 June 2007 © Springer-Verlag 2007
Abstract Foregut duplication cysts are rare congenital anomalies of enteric origin. The diagnosis is usually made in infancy. We report the unusual case of a 71-year-old female presenting to the ENT department with shortness of breath and stridor due to an oesophageal reduplication cyst. The presentation, diagnosis and management of this potential pitfall for the unwary are outlined. Keywords
Foregut · Duplication cysts · Stridor
Introduction Reginald Fitz [15] Wrst used the term intestinal duplication in 1844 when he published a paper suggesting that alimentary tract duplications developed from persistent omphalomesenteric remnants. Current literature consists mainly of small case series and case reports rather than any large single or multi-institutional series. It has been postulated that enteric duplications may represent abnormal migration of cells from the primitive foregut [21]. These cysts are found along the course of the oesophagus and are named gastroenteric cysts or oesophageal reduplication cysts. Intrathoracic foregut duplication cysts are rare congenital foregut malformations that usually produce respiratory symptoms in early infancy [20]. We report an unusual case of mediastinal foregut duplication cyst in an adult patient presenting with shortness of breath and stridor.
A. Espeso (&) · S. Verma · P. Jani · H. SudhoV Department of Otolaryngology and Skull Base Surgery, Addenbrooke’s Hospital, Cambridge, UK e-mail:
[email protected]
Case report A 71-year-old female patient was referred by her general practioner to the ENT on-call team as an emergency due to increasingly noisy breathing. In fact, the patient had 5 days of increasing shortness of breath on exertion which progressed to dull retrosternal chest pain and stridor at rest. Three weeks earlier she had suVered from a viral upper respiratory tract infection that had settled. There was no dysphagia or odynophagia reported. She had a past medical history of type 2 diabetes mellitus and had been a lifelong non-smoker. On examination, the patient managed to walk into the examination room, but had obvious stridor together with a dry, irritating cough. Chest examination indicated bronchial breathing. Flexible nasoendoscopy showed mild Reinke’s oedema of the vocal cords but full movement and no other laryngeal pathology. The subglottis was unremarkable and a normal looking proximal trachea was easily visualised. An initial chest X-ray showed no pathology. Lung function tests were also normal. A computerised tomography (CT) scan of the patient’s chest demonstrated a 4.8 £ 3.4 £ 4.0 cm well circumscribed, rounded lesion in the mediastinum, arising just above the level of the aortic arch behind the origin of the great vessels and in close relation to the trachea and the oesophagus. This represented a mediastinal foregut duplication cyst which was compressing the posterior wall of the trachea anteriorly and to the right, reducing the tracheal lumen to 10% of its original size (Fig. 1). Further imaging with a barium swallow (Fig. 2) showed a well deWned extrinsic impression on the oesophagus at the junction of the upper and middle thirds, with mild deviation of the oesophagus to the right corresponding to the position of the foregut duplication cyst demonstrated on the CT scan. The oesophagus was otherwise normal in appearance
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successfully within 3 days and was discharged home with regular out-patient and radiological follow-up.
Discussion
Fig. 1 Chest CT scan showing circumscribed rounded lesion in the mediastinum (arrow)
Fig. 2 Barium swallow showing a well deWned extrinsic impression on the oesophagus at the junction of the upper and middle thirds, with a deviation of the oesophagus to the right (arrow)
and motility. Interestingly, barium contrast was seen to pass freely along the oesophagus. Surgical excision via left posterior thoracotomy was performed 7 days after admission. The patient recovered
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Oesophageal reduplication cysts are developmental cysts arising from the primitive foregut [23]. The general incidence of duplication cysts is very rare. Potter [32] reported only two cases after performing 9,000 foetal and neonatal autopsies. Approximately 75% of duplications have been reported to be located within the abdominal cavity, while the remaining are pure intrathoracic (20%), or thoracoabdominal (5%). Duplication cysts can also originate from within the tongue [10, 12], Xoor of mouth [22], hypopharynx [11] and stomach [21, 24]. In this paper we report the unusual case of an oesophageal reduplication cyst presenting in late adulthood with symptoms of respiratory compromise, and recapitulate the presentation, diagnosis and management of this rare clinical problem. Enteric reduplication cysts are usually detected in childhood (80%). Approximately two thirds of all intestinal duplications are discovered within the Wrst 2 years of life, with one third identiWed in the newborn period [29]. In contrast, the incidence of intrathoracic oesophageal cysts in adults is extremely rare [39]. Our extensive literature review showed only an utmost of seven case reports of intrathoracic oesophageal cysts in adults. Oesophageal reduplication cysts cause symptoms by compression of surrounding structures. It has been reported in the literature that congenital oesophageal cysts presenting in children aVect the lower third of the oesophagus in 60% of cases [34] causing swallowing diYculties due to compression. 20% occur in the upper third of the oesophagus leading to respiratory diYculty, recurrent chest infections, feeding diYculties [2, 14, 28, 35] and haemoptysis [3, 27, 33, 37] usually due to gastric mucosa present within the cystic lumen. The remaining 20% occur in the middle third of the oesophagus presenting clinically as retrosternal chest pain and swallowing diYculty. There are also reports of oesophageal cysts presenting as tracheoesophageal Wstula and oesophageal atresia [18, 20, 25]. In contrast, CioY et al. [7] reported a series of 11 adult patients with a mean age of 46 years presenting with oesophageal duplication cysts present throughout the gastrointestinal tract. Eight patients were symptomatic complaining of dysphagia (four cases), chest pain (two cases), and epigastric pain (two cases). The remaining three patients were asymptomatic. On searching the world literature for cases of oesophageal duplication cysts presenting in adulthood in the intrathoracic region we were able to locate only seven reports. These previous reports cite adult patients presenting with dysphagia [5, 8, 9]; epigastric pain
Eur Arch Otorhinolaryngol (2007) 264:1357–1360
and nausea [24, 36]; post ingestion aphagia, presternal pain and weight loss [31] or retrosternal pain [9, 26, 30]. Till date there has been no reported case in the literature of an oesophageal duplication cyst presenting with stridor in adulthood. The diagnosis of oesophageal reduplication cysts in adulthood is diYcult and is mostly an incidental radiological Wnding in an otherwise asymptomatic patient [1, 16]. Plain radiographs of the chest and abdomen should be routinely obtained; however, because of the nonspeciWcity of their results, making a preoperative diagnosis on the basis of radiographic Wndings is unlikely. Endoscopic ultrasound-Wne needle aspiration (EUS-FNA) of mediastinal cysts with smaller-gauge needles, and antibiotic prophylaxis appears safe and can provide a deWnitive diagnosis in atypical mediastinal cystic lesions [6, 38]. EUS was superior compared with CT scanning in characterizing foregut duplication cysts [13]. EUS-FNA is safe and accurate in the diagnosis of foregut duplication cysts [13]. CT scanning must be a complementary diagnostic technique which is not only used preoperatively but also as follow-up imaging as there is a signiWcant incidence of synchronous lesions. MRI has less of a role in imaging than CT, but may be necessary if neurological symptoms of spinal cord compression exist and with evidence of bony spinal abnormalities on plain Wlm or CT scan. These symptoms may be indicative of intraspinal cysts. Medical therapy has no role in the management of oesophageal cysts [4]. Intestinal duplications are considered to be benign lesions but they may result in signiWcant morbidity and mortality if left untreated [17, 39]. A deWnitive diagnosis of an oesophageal cyst will grant surgical intervention as cystic duplications can be completely excised. Previously, a posterolateral thoracotomy was required to remove the cyst or the duplication; however, minimally invasive thoracoscopy is currently the procedure of choice to enucleate cysts and resect duplications [7]. Surgical resection of this anomaly is usually followed by permanent cure. Recurrence of oesophageal duplication cysts is rare, especially if the entire cyst was excised [19, 20]). In conclusion, we have highlighted an unusual case of oesophageal reduplication cyst presenting with stridor in adulthood. Such a presentation has previously not been reported in the literature and presents a pitfall for the unwary if not recognised and investigated further.
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