Unusual association of diseases/symptoms BMJ Case Rep: first published as 10.1136/bcr-2018-227610 on 26 August 2019. Downloaded from Case report Unusual cause of dysphagia Dion Koh, Udit Thakur, Wei Mou Lim Upper Gastrointestinal Surgery, SUMMARY was accompanied by substantial weight loss of 5 kg Monash Health, Clayton, In this case, we describe a unique case of large renal over the preceding 2 weeks. He denied any asso- Victoria, Australia hydronephrosis in a 79-year-old Indian male patient ciated fever, cough or other coryzal symptoms. who had initially presented with 3 months of progressive He also denied symptoms of bowel obstruction Correspondence to dysphagia and loss of weight. His dysphagia was such as new constipation or significant abdominal Dr Dion Koh, dion. koh@ icloud. com initially thought to be related to the atypical diagnosis distension. of achalasia and was being considered for an elective The patient had a recent presentation to another Accepted 10 August 2019 laparoscopic Heller myotomy. On performing CT of the large metropolitan hospital due to aspiration pneu- abdomen, a large renal mass was discovered. However, monia in October 2017. During that admission, he predicament remained regarding the exact aetiology was investigated for dysphagia. A barium swallow of this renal mass. This case highlights a tremendously test revealed the following features: ‘bird beak’ intriguing case of dysphagia with an underlying aetiology appearance, marked distal oesophageal dilation that has not been reported elsewhere previously. with abnormal peristalsis and tertiary peristaltic waves; however, subsequent oesophageal manom- etry (lower oesophageal sphincter basal pressure: 8.9 mm Hg, residual pressure: 9.3 mm Hg, upper BACKGROUND oesophageal sphincter basal pressure: 48.9 mm Hg, We chose to present this patient, as this is an unusual residual pressure: 7.5 mm Hg) was not typical of cause of dysphagia that mimicked achalasia. The achalasia. He consequently underwent a gastros- patient presented to our hospital with symptoms copy, which showed oesophageal dilation and of dysphagia, vomiting, and loss of weight. Prior gastritis. The patient was given the diagnosis of to his admission, he had a recent admission at achalasia and managed conservatively at that stage another metropolitan hospital for aspiration pneu- and was referred to the upper gastrointestinal monia. During that admission, he was investigated surgical unit at Monash Medical Centre for surgical and worked up for dysphagia. The investigations management of achalasia. performed included barium swallow test, which His medical history includes a previous Heller http://casereports.bmj.com/ showed ‘birds beak’ appearance, inconclusive myotomy at the age of 17 and neurofibromatosis. oesophageal manometry and gastroscopy. Initial The indication for the Heller myotomy remains differential diagnoses were achalasia, gastro-oe- unclear as it was completed overseas with no records sophageal tumours, gastro-oesophageal reflux available in regard to this. He has no known drug disease (GORD) and hiatal hernia. allergies and does not take any regular medications. This patient presented as a diagnostic dilemma Clinical examination was unremarkable. His with an atypical clinical history that only became abdomen was soft and slightly distended and no clear after further imaging. As there are no other organomegaly was appreciated. Abdominal pain case reports with similar presentations, this would was not elicited on deep palpation. serve as a good eye opener for other clinicians—in terms of when to consider CT abdomen and pelvis on September 27, 2021 by guest. Protected copyright. in patients presenting with vague gastrointestinal INVESTIGATIONS symptoms. As mentioned above, the patient had barium swallow study and oesophageal manometry done during his recent admission in another hospital. CASE PRESENTATION The barium swallow study had shown ‘bird beak’ A 79-year-old Indian male patient presented to a appearance, which is a key radiological feature of tertiary centre in Melbourne with 3 months of achalasia. He then underwent oesophageal manom- progressive dysphagia, which significantly wors- etry and the following test pressures were recorded: ened over the 3 weeks prior to admission. He was ► Lower oesophageal sphincter basal pressure: © BMJ Publishing Group admitted under the upper gastrointestinal surgical 8.9 mm Hg, residual pressure: 9.3 mm Hg. Limited 2019. Re-use unit for further evaluation and consideration of an ► Upper oesophageal sphincter basal pressure: permitted under CC BY-NC. No commercial re-use. See rights elective Heller myotomy. 48.9 mm Hg, residual pressure: 7.5 mm Hg. and permissions. Published The patient presented with the primary complaint Such pressures are not typical of achalasia. The by BMJ. of dysphagia; unable to tolerate any solids or patient was further investigated with gastroscopy, liquids without regurgitating it back up. The which showed oesophageal dilation and gastritis. To cite: Koh D, Thakur U, Lim WM. BMJ Case Rep vomit contained undigested food with no apparent During his admission at our hospital, initial blood 2019;12:e227610. features of haematemesis such as coffee ground investigations on presentation were significant for doi:10.1136/bcr-2018- appearance. The patient did however complain of elevated white cell count (16.2×109/L) and neutro- 227610 mild epigastric pain while vomiting. This dysphagia philia (13.5×109/L). Other blood investigations Koh D, et al. BMJ Case Rep 2019;12:e227610. doi:10.1136/bcr-2018-227610 1 Unusual association of diseases/symptoms BMJ Case Rep: first published as 10.1136/bcr-2018-227610 on 26 August 2019. Downloaded from Figure 2 CT abdomen and pelvis showing a dilated oesophagus secondary to compression of the stomach by the large left http://casereports.bmj.com/ hydronephrosis. Figure 1 CT abdomen and pelvis showing a very large left of acid-fast bacilli detected on the stain and the culture after hydronephrosis. 6 weeks. A nephrostomy was left in situ to allow any further drainage. To evaluate the nature of the mass, a left nephrostogram was were unremarkable on admission. Additionally, an erect chest requested which showed contrast pooling in the previous mass, X-ray performed initially was also normal with no evidence of with no flow entering the left ureter or renal pelvis. Further- hiatal hernia. more, a renal nuclear scan with 99m-Tc MAG-3 concluded that A CT abdomen and pelvis (CTAP) revealed a large left intra- there was likely long standing left pelviureteric junction obstruc- on September 27, 2021 by guest. Protected copyright. renal multiloculated lesion of simple fluid density measuring tion given the absence of left kidney perfusion and excretion 110×201×212 mm which appeared to be arising from the of radioisotope into the left kidney collecting system. The right renal pelvis, was compressing and displacing the stomach and kidney contributed to 100% of the total renal function. Due to other abdominal organs (figure 1). The left distal ureter was not the inconclusive investigations, the patient also underwent a CT dilated and had no evidence of calcification or mass. There was intravenous pyelogram, which showed a collapsed left-sided marked dilation of the oesophagus with air fluid level (figure 2). non-functional multicystic dysplastic kidney. A repeated oesoph- It was suggested that the large cystic renal mass was most likely ageal manometry was not considered postdrainage of the cystic secondary to a chronic pelviureteric junction obstruction. Small mass as the patient’s symptoms had improved soon after. On a right-sided renal cysts were also observed. There were no masses later admission under the general medical team, a gastroscopy external to the ureter seen to suggest external obstruction. and endoscopic ultrasound was performed to biopsy a retro- An ultrasound-guided interventional cystic drainage was peritoneal lymph node which had suspicious features on a new subsequently organised to drain the left renal cystic mass as CTAP that was organised to investigate for fevers of unknown recommended by the consulting urology team. A total of 4 L origin and weight loss. The gastroscopy had showed dilated of fluid was drained. The initial 200 mL was sent for micros- distal oesophagus without features of achalasia. The endoscope copy, culture and sensitivity, acid-fast bacilli culture, as well as was able to progress into the stomach without difficulties. fluid cytology. Microscopy of the fluid showed debris, inflam- matory cells, macrophages and small clumps of epithelial cells with no features of malignancy. There was no microorganism growth after a period of incubation. There was also no evidence 2 Koh D, et al. BMJ Case Rep 2019;12:e227610. doi:10.1136/bcr-2018-227610 Unusual association of diseases/symptoms BMJ Case Rep: first published as 10.1136/bcr-2018-227610 on 26 August 2019. Downloaded from DIFFERENTIAL DIAGNOSIS biopsy and showed dilated distal oesophagus without features When the patient first presented, the differential diagnoses of achalasia. He was commenced on long-term antibiotic treat- considered were achalasia, gastro-oesophageal tumours, GORD ment for tuberculosis (rifampicin and isoniazid) and was looked and hiatus hernia. Achalasia was considered due to radiological after by the infectious disease team. The discovery of abdom- evidence of ‘bird beak’ appearance. Furthermore, with a signif- inal tuberculosis was unrelated to when he first presented with icant