Zenker’s diverticulum: a rare clinical condition with unusual oral manifestation

  1. Anuj Dadhich 1,
  2. Harish Saluja 2,
  3. Seemit Shah 1 and
  4. Kumar Nilesh 3
  1. 1 Department of Oral and Maxillofacial Surgery, Pravara Institute of Medical Sciences, Loni, India
  2. 2 OMFS, Pravara Rural Dental College, Ahmednagar, India
  3. 3 Oral and Maxillofacial Surgery, Krishna Institute of Medical Sciences Deemed University, Karad, India
  1. Correspondence to Dr Kumar Nilesh; drkumarnilesh@yahoo.com

Publication history

Accepted:17 Oct 2020
First published:31 Oct 2020
Online issue publication:31 Oct 2020

Case reports

Case reports are not necessarily evidence-based in the same way that the other content on BMJ Best Practice is. They should not be relied on to guide clinical practice. Please check the date of publication.

Abstract

Oral mucosa exhibit clinical manifestations of a variety of systemic conditions and can serve as an indicator of overall health of an individual. Various systemic conditions like autoimmune, endocrine and haematological disorders can present with oral mucosal lesions, which can serve as an important diagnostic feature. These oral lesions can vary from oral ulceration, bleeding gums, xerostomia, chronic glossitis, to erosion and sensitivity of teeth. It is important that a clinician must be aware of the oral presentations of systemic conditions, so that timely diagnosis can be made and the necessary treatment is executed. This paper presents an unusual case of severe oral mucosal ulcerations and dysphagia in an elderly patient diagnosed with Zenker’s diverticulum. Patient was relieved of all oral symptoms once the underlying gastrointestinal tract pathology was diagnosed and surgically corrected.

Background

Most of the oral lesions encountered in routine clinical practice arise due to conditions primarily associated within the oral cavity. However, various systemic conditions can adversely affect the oral apparatus. It can clinically manifest as oral ulcers, as seen in dermatological (eg, lichen planus, erythema multiforme, pemphigus vulgaris), haematological (eg, neutropenia, non-Hodgkin’s lymphoma) and gastrointestinal (GI) tract disorders (eg, Crohn’s disease, ulcerative colitis). White patches on oral mucosa are commonly due to candidiasis and can also be associated with the use of drugs like cocaine, pappilomas, white sponge nevus and hairy leukoplakia seen in HIV infection. Mucosal swellings can present as oral manifestation of Crohn’s disease, sarcoiodosis, Sturge-Weber syndrome and tuberous sclerosis. While spontaneous oral bleeding is seen in leukaemias, thrombocytopenia, haemophillia and Christmas disease, erosion and increased sensitivity of teeth can be associated with gastro-oesophageal reflux disease, while hallitosis can be a clinical feature of end-stage renal disease, liver failure and diabetic ketoacidosis. Xerostomia can occur secondary to radiotherapy, uncontrolled diabetes mellitus, Sjogren’s syndrome, sarcoiodosis and HIV infection.1 2

Oral lesions at times can be the first and the only clinical representation of a systemic disease. Therefore, it is imperative that not only the dental surgeon but also the medical practitioner must have adequate understanding of these oral manifestations for efficient diagnosis of these conditions.2 Zenker’s diverticulum (ZD) is an upper aerodigestive tract diverticulum characterised by herniation of the mucosal and submucosal layers through the posterior wall of the pharyngoesophageal junction, known as Killian’s triangle.3 ZD typically occurs in elderly patients, and the most prominent clinical features include dysphagia and regurgitation. Other features of ZD include cough, noisy swallowing, belching and hoarseness of voice. Transoral endoscopic repair with or without diverculectomy is the common treatment for ZD.3 This report highlights an unusual case of severe oral mucosal ulcerations and dysphagia in an elderly male patient. Initial misdiagnosis resulted in failure of treatment. Once the diagnosis of ZD was made and treated, patient was relieved of all oral symptoms. Various differential diagnoses for multiple oral ulcers that must be kept in mind in such situation includes aphthous ulcer, erythema multiforme, pemphigus vulgaris, neutropenia, non-Hodgkin’s lymphoma, Crohn’s disease and ulcerative colitis.

Case presentation

A 66-year-old male patient was referred to oral and maxillofacial surgery clinic with report of burning sensation in the oral cavity, associated with difficulty in eating and swallowing food for last 4 months. General physical examination revealed an otherwise healthy patient with average built. The medical history was non-contributory. Intraoral examination revealed the presence of ulcerative lesions on the lower lip, dorsal surface of tongue and buccal mucosa bilaterally (figures 1 and 2). Lesions were reddish in colour and were covered with whitish slough. Patient reported extreme burning sensation with the lesions, which aggravated during eating. The lesions were tender on palpation and bleed spontaneously. Detailed history revealed that patient had lost almost 10 kg weight in the past 4 months due to burning sensation experienced during eating. Patient had no adverse oral habits and did not give history of any pre-existing systemic condition. Patient had visited many clinicians over the period of last 4 months and received treatment including oral antibiotics, anti-inflammatory agents and oral antifungal therapy. He was also prescribed topical corticosteroid therapy, which did not relieve the symptoms.

Figure 1

Lesions present on lip and dorsal surface of tongue.

Figure 2

Lesions seen on buccal mucosa.

On initial examination and considering the possibility of presence of an underlying undiagnosed systemic condition, palliative therapy with topical application of local anaesthetic gel and multivitamin supplements was initiated and the patient was advised to undergo routine blood examination and blood sugar evaluation. After 3 days of palliative therapy, patient reported back with no improvement in the clinical situation. The blood reports were within normal range including the fasting and postprandial blood glucose levels. During the second visit, under topical anaesthesia, the whitish slough was removed from the lesions, and considering the possibility of presence of dermatological disorder, patient was prescribed topical Kenacort (triamcenolone acetonide), along with topical benzocaine gel application for a period of 1 week. On the third visit, patient had slight reduction in the burning sensation. However, the lesions showed no improvement and despite reduction in the burning sensation, the patient reported an increase in dysphagia from the first visit. Considering the possibility of GI tract pathology like oesophageal strictures, presenting with oral manifestation, the patient was referred to a gastroenterologist, for further evaluation.

Investigations

Barium swallow test was advised, which revealed dilation and pouching of the oesophagus, where the barium uptake was more. Narrowing of cricopharyngeus muscle was also evident (figure 3). Based on the radiographic features, the conditioned was diagnosed as ZD.

Figure 3

Barium swallow showing posteriorly based pouch.

Treatment

Patient underwent endoscopic diverticulectomy under general anaesthesia. He was advised soft diet and was kept on nutrition supplements and oral antacids (pantoprazole 40 mg 24 hourly) for 3 weeks

Outcome and follow-up

At 1-month postsurgery recall visit, it was noticed that all the ulcerative lesions on the lip, buccal mucsa and tongue had healed. There was no burning sensation while eating and there was no dysphagia (figure 4).

Figure 4

Lesions of tongue and lip resolved after endoscopic diverticuletomy.

Discussion

ZD is a rare condition involving the upper part of the GI tract. The condition was first described by German pathologists Friedrich Albert von Zenker and Hugo Wilhelm von Ziemssen in 1877.4 The is a rare disease with an incidence of 0.01%–0.11%.3 4 It usually affects patients between sixth and ninth decade of life.3 ZD is commonly located on the posterior pharyngeal wall within the upper oesophageal sphincter (UES) between the lower pharynx and oesophagus.5 ZD is the consequence of oesophageal mucus membrane weakening with coexisting increase in UES tension. The increase in pressure during swallowing pushes oesophageal tissue layers outside the oesophagus towards the mediastinum and the retropharyngeal space, forming a pouch with gates limited by muscles.4 Increase in pressure of the cricopharyngeal muscle results in decreased UES relaxation and subsequent further increase in the pressure within the sphincter, leading to creation of an alternative route for ingesta. The condition most commonly presents as dysphagia, cough, regurgitation, loud swallowing of liquids and hoarseness of voice.6 7 However, to best of our knowledge, there is no report of oral mucosal lesions developing due to ZD. In the present case, the patient has multiple areas of oral ulcerations, burning sensation and dysphagia.

The diagnosis of ZD is made by performing barium swallow, which characteristically demonstrates a posteriorly based pouch.8 Esophagopharyngeal reflux or regurgitation of swallowed food from the pouch back into the pharynx may be seen on flexible endoscopic evaluation of swallowing. Within a few seconds, the swallowed bolus disappears below the hypopharynx and is generally a pathognomonic sign for ZD.9 Treatment of ZD is diverculectomy, which can be performed as an open surgery or an endoscopic procedure. Release of cricopharyngeal stricture is necessary for successful management of ZD.10

As the lifespan increases and the medical care becomes ever more complex and effective, it is likely that the numbers of individuals with oral manifestations of systemic disease will continue to rise. These manifestations can be variable both in frequency and clinical presentation. A plethora of systemic conditions like autoimmune disorders, haematological, endocrine conditions, neoplastic diseases and chronic diseases produce pathognomonic changes in the oral mucosa. The primary healthcare providers often are the ones who first attend the patients and play an important role in diagnosis of such systemic conditions, which can affect the overall outcome of the treatment. Thus, it is important to report these findings so that primary healthcare as well as oral healthcare providers are aware of the clinical manifestations of this rare condition for timely diagnosis and correct treatment.

Learning points

  • Oral mucosa exhibit clinical manifestations of a variety of systemic conditions. Various systemic conditions like autoimmune, endocrine and haematological disorders present with oral mucosal lesions, which serve as an important diagnostic feature.

  • Zenker’s diverticulum is an upper aerodigestive tract disorder characterised by acquired herniation of the mucosal and submucosal layers through the posterior wall of the pharyngoesophageal junction.

  • It most commonly presents with dysphagia, cough, regurgitation and hoarseness of voice.

  • This paper presents an unusual case of severe oral mucosal ulcerations and dysphagia in an elderly patient diagnosed with Zenker’s diverticulum.

  • Both oral as well as general healthcare providers must be aware of the rare clinical manifestations of this condition, for timely diagnosis and correct treatment.

Footnotes

  • Contributors KN, AD and HS were involved in manuscript preparation, literature review and final approval of the paper. AD and SS were involved in patient care.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent for publication Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

References

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