Article Type : Case Report
Authors : Sivalokanathan S, Magee C, Pereira SP and Goodchild G
Keywords : Eosinophilic oesophagitis; Boerhaave’s Syndrome; EoE
Eosinophilic Oesophagitis (EoE) is a chronic disease of the
oesophagus characterised by mucosal eosinophilia and oesophageal dysfunction.
It is a common but under-recognised cause of oesophagitis, most commonly
affecting young males with a history of atopy.1 The estimated prevalence ranges
from 13 and 49 cases per 100,000, although this is likely to be underestimated
due to reduced awareness of this condition.2 Oesophageal rupture as a
complication of EoE has been widely reported in association with food impaction
and as a complication of endoscopy. Spontaneous oesophageal perforation
(Boerhaave’s syndrome) is less common.3 we present an unusual case of
Boerhaaves’s syndrome as a presenting feature of EoE in a previously
asymptomatic patient. To our knowledge this has not been previously reported.
Eosinophilic
Oesophagitis (EoE) is an increasingly recognised chronic, allergic,
inflammatory condition of the oesophagus, with the incidence varying from 1 to
20 new cases per 100,000 people per year. While EoE was initially described in
the 1970s, it has only been a recognised clinic pathological disorder since the
1990s. Adults typically complain of dysphagia, food bolus impaction or chest
pain. There is heterogeneity in the presentation of EoE and dysphagia may
present later which may contribute to delay in diagnosis. The oesophagus, in
the normal state, is devoid of eosinophils, and inflammation driven by
eosinophils is characteristic of EoE. Nonetheless, there is significant
variability in eosinophil concentrations throughout the length of the oesophagus,
with a more distal location of eosinophilia steering towards a diagnosis of
gastro-oesophageal reflux disease (GORD). Thus, confirmation of EoE involves
obtaining 2-4 biopsies from the proximal, mid and distal oesophagus - a
protocol which has been shown to improve diagnostic accuracy [1]. Treatment
options include dietary restrictions (usually a 6-food elimination diet) as
well as pharmacological treatment with acid suppression and topical
corticosteroids. A typical regimen initially involves acid suppression with an
eight-week course of a proton pump inhibitor followed by repeat gastroscopy and
biopsy after 8-12 weeks to assess endoscopic and histological response. If
either symptoms or endoscopic evidence of EoE persist, topical corticosteroid
therapy is commenced with dose titration based on clinical and/or histological
response [1,2].
An adolescent male patient, with a background of mild asthma treated with salbutamol as-required, was referred to our centre with persistent pyrexia, night sweats and weight loss. He denied any gastrointestinal symptoms including dyspepsia, dysphagia or chest pain. Routine bloods revealed a C-reactive protein of 52, normal white cell count and normal eosinophil count. A contrast-enhanced computed tomography (CT) scan of the thorax revealed a 4.4cm mediastinal mass with associated lymphadenopathy. Linear endoscopic ultrasound (EUS) demonstrated multiple inflammatory appearing posterior mediastinal lymph nodes but the mass could not be demonstrated. The EUS endoscope was subsequently exchanged for a diagnostic gastroscope, which revealed longitudinal furrowing and trachealisation of the oesophagus in keeping with eosinophilic oesophagitis. This diagnosis was later confirmed histologically with biopsies showing 66 eosinophils per high-powered field (hpf). The patient was commenced on omeprazole 20mg twice daily and swallowed (rather than inhaled) fluticasone 440mcg twice daily. A post 8-week therapy thoracic magnetic resonance imaging (MRI) scan showed a reduction in the size of the inflammatory mass from 4.4cm to 1.5cm in diameter and resolution of lymphadenopathy. Eight months after starting treatment, the patient has remained well and continues on topical fluticasone and omeprazole (Figures 1-3).
Figure
1a: Contrast enhanced computed tomography (CT) scan
demonstrating a 45mm peri-oesophageal anterior mediastinal mass with
displacement of the left diaphragmatic crus (white arrows).
Figure 1b: Follow-up magnetic resonance (MR) thorax demonstrating a thickened gastro-oesophageal junction measuring 17mm by 6mm (white arrow) reduced in size in comparison to index CT scan and with resolution of lymphadenopathy.
Figure 2: Gastroscopy showing longitudinal furrowing & trachealisation of the oesophagus.
Figures
3a: Eosinophilic oesophageal inflammation in
eosinophilic oesophagitis.
Figure
3b: High-power view showing large numbers of
eosinophils accumulating preferentially towards the luminal surface.
EoE is an
increasingly recognised condition with a rapidly increasing incidence [1-4]. In
adults, dysphagia and GORD type symptoms predominate. Endoscopic examination
may often be normal [1-3]. Although typical endoscopic findings include
longitudinal furrows, concentric rings (trachealisation), white exudates and
loss of vascular markings [5]. Biopsies demonstrating more than 15
eosinophils/hpf are required for diagnosis [4]. The practical management of EOE
can be challenging due to minimal data on optimal length of treatment and
long-term outcomes. There is emerging evidence that maintenance treatment with
topical corticosteroids, particularly in cases of oesophageal perforation, may
slow progression and reduce further complications. Long-standing uncontrolled
eosinophilic inflammation of the oesophagus can lead to cellular hyperplasia,
mucosal fragility, and wall re-modelling and stricture formation [6]. The
resultant narrowing of the oesophagus pre-disposes to food bolus impaction
which can lead to secondary perforation. Endoscopic dilation of strictures can
lead to iatrogenic perforation. Our case illustrates both the variability in
the presentation of EOE and the significance of the vulnerability of the
oesophageal wall. The case demonstrates an important consequence of
uncontrolled chronic oesophageal inflammation. Oesophageal perforation, or
Boerhaave’s syndrome, can lead to significant consequences such as
mediastinitis, abscesses, infection of the spinal cord and empyema which may
lead to organ failure and death. Clinicians should remain aware of EoE as a
potential cause of oesophageal perforation even when, as in our case, there are
no preceding oesophageal symptoms.
All authors declare no conflict of interest related to this study.