TERN Christmas Cases 2022

A cause other than Carol singing for a hoarse voice this Christmas..

What can be more joyous at Christmas than the shared singing of Christmas carols. Although overindulging in this festive pastime can certainly cause a hoarse voice, this was not the cause of this man’s hoarse voice. This case report describes the presentation of a 39 year old to the Emergency Department with a 2 month history of a new hoarse voice and exertional dyspnoea following a preceding flu-like illness. His COVID-19 test was negative but COVID-19 antibody testing was positive. His chest radiograph showed cardiomegaly and his pro b-type natriuretic peptide was elevated at 2,444 pg/mL. Nasopharyngoscopy showed a left vocal cord paresis. This gentleman was admitted to the hospital’s cardiology service and was found to have severe global systolic function on cardiac catheterisation and was medically managed with a diagnosis of presumed viral-mediated non ischaemic cardiomyopathy.

Learning points 

1. The eponymous syndrome is named after the 19th century Austrian physician Norbert Ortner who first described it.
2. When cardiovascular structures enlarge a consequence can be compression of the left recurrent laryngeal nerve which presents as a hoarse voice due to a unilateral vocal cord palsy
3. The recurrent laryngeal nerve is a terminal branch of the vagus nerve and sits between the aortic arch and the pulmonary artery in a space known as the aortopulmonary window which can be as small as 4 mm. 
4. The most common aetiology structurally is a dilated left atrium secondary to mitral stenosis. Other causes include an enlarged pulmonary artery, pulmonary hypertension and a thoracic aortic aneurysm. 
5. The recurrent laryngeal nerve supplies motor function to all the laryngeal muscles but also to the cricothyroid muscle.

Bottom Line

The combination of a new hoarse voice and cardiac symptoms should prompt you to think about Ortner’s or cardiovocal syndrome.

Reference

 Kravitz MS, Dagan AS. Man With Hoarse Voice and Dyspnea on Exertion. Ann Emerg Med. 2022 Jan;79(1):18-30. doi: 10.1016/j.annemergmed.2021.07.109. PMID: 34949405; PMCID: PMC8685409.

The cheese course

Sener et al report a case of resistant lactic acidaemia which will be of interest to anyone who is tempted to take up cheese making as a hobby in the new year. The emergency physicians from Istanbul describe the presentation of a 59-year-old normally well male who attended with vomiting and general weakness after accidentally drinking a glass of cheese starter culture that contained lactic acid-producing bacteria. The patient arrived conscious, with normal observations, and a venous lactate level of 4.2mmol/L, and a pH of 7.32. After four hours the lactate had risen to 7.2mmol/L despite treatment with crystalloid. The patient was commenced on supportive treatment and admitted to hospital. No other explanation for the persistent lactic acidaemia was identified and within 24 hours the serum lactate had normalised. The patient was discharged from hospital two days later.

Learning points

Lactate is typically produced as the end product of anaerobic glycolysis which may be the result of a wide range of disease processes and is frequently employed as a means of predicting patients at risk of having a poor outcome, and whilst it is generally accepted that transiently elevated lactate levels do not necessarily confer a poor prognosis, persistently elevated levels are associated with an increase in mortality.

Lactic acid bacteria (LAB) are found naturally in the gut microflora. After food intake, these bacteria produce lactic acid as the major metabolic end-product of carbohydrate fermentation. It is shown that in case of overproduction, lactic acid enters the bloodstream and causes high anion gap metabolic acidosis 

Cheese starter cultures are traditionally used in the production of cheeses and contain bacteria which cause lactic acid from lactose; in this case it is likely that the ingested cheese starter culture produced large quantities of lactic acid in the intestines, sufficient to cause a resistant lactic acidaemia.

Bottom Line

Lactic acidosis may occur due to exogenous as well as endogenous causes

Reference

Sener K, Arslan B, Ozselcuk S, Guven R. Resistant Lactic Acidemia Due to Accidental Cheese Starter Culture Ingestion. Am J Emerg Med. 2022;60:228.e1-228.e2. doi:10.1016/j.ajem.2022.07.046

Another cause for Santa’s Rosy cheeks?

75-year-old man presented to the dermatology clinic with erythema of the face. He had recently started a new medication, mirtazapine, and was concerned about a possible drug reaction. However, he also noted a 1-month history of progressive swelling of the face and arms, along with dizziness with position changes. His medical history was notable for sick sinus syndrome, for which he had undergone placement of a pacemaker 8 years earlier. Two years before the current presentation, the pacemaker leads had been replaced after a lead had fractured.

The physical examination was notable for erythema and oedema of the face and neck, with mild cyanosis of the lips, diffuse varicosities across the anterior chest, oedema of both arms, and distended jugular veins. Radiography of the chest showed a dual-chamber pacemaker with appropriately positioned transvenous leads. Subsequent computed tomographic venography of the chest revealed occlusion of the upper superior vena cava caused by thrombus encasement of the transvenous leads and extensive venous collateral vessels within the lower neck and superior mediastinum. The patient was admitted to the hospital, and intravenous heparin was initiated. A month later, he underwent pacemaker extraction with placement of a leadless system. At follow-up 3 days after the procedure, his symptoms had resolved. 

Learning Points

Superior Vena Cava (SVC) syndrome is a very rare but debilitating complication after pacemaker lead implantation. 

The frequency of pacemaker-induced SVC syndrome is difficult to ascertain; it can either be acute or chronic, with many patients being asymptomatic. The asymptomatic prevalence may be as high as 30% of all patients, with partial or complete venous obstruction. Symptoms depend on how quickly the obstruction establishes; however, the insurgence of thrombosis caused by pacemaker leads seems to be unrelated to the time elapsed from the procedure. In a large series from Rice et al a pacemaker was considered to be the cause of SVC syndrome in only 1 out of 78 cases (1.28%).

The pathophysiology behind this phenomenon is likely endothelial disruption from mechanical stress or infection of the pacemaker leads, leading to inflammation and fibrosis and subsequently, thrombosis. Several predictors may increase the propensity of thrombus formation and venous occlusion: upgrade of pacemaker devices; more than one pacemaker lead; device and/or lead infection; severed pacemaker leads.

Management of pacemaker lead thrombosis can be either medical or surgical. Medical forms of management include anti-coagulation or thrombolysis, while surgical interventions rely on percutaneous endovascular intervention or lead extraction. The anticoagulation treatment of upper extremity deep vein thrombosis (UE-DVT) has not been standardised yet; however, the current practice is to start warfarin after 5 to 7 days of low-molecular-weight heparin. Currently, there are no completed randomised trials of direct oral anticoagulants (DOACs) in UE-DVT; nevertheless, DOACs are increasingly used in real-world experiences with an adequate profile of efficacy and safety. Thus, treatment of iatrogenic superior vena cava thrombosis with a DOAC represents an interesting new approach, to be validated in prospective trials against the current standard (heparin followed by warfarin).

The most common cause of SVC syndrome is malignancy (85%): lung cancer, lymphomas; metastasis to the mediastinum from breast cancer or gastrointestinal tumours, primary mediastinal tumours. The mechanisms most involved are extrinsic compression and neoplastic infiltration of SVC.

Less commonly, non-oncologic causes may occur. These include infections, spontaneous thrombosis, and iatrogenic causes. Among the latter, radiotherapy on the mediastinum and thrombosis or infections of intravascular devices (central vein catheters, cardiac defibrillators, and pacemaker wires) are becoming increasingly common.

Bottom Line

Pacemaker lead thrombosis is a possible cause for SVC syndrome.

References

1. Miller A, Schneider J. Pacemaker-Associated Superior Vena Cava Syndrome. N Engl J Med. 2021;384(17):e63. doi:10.1056/NEJMicm2031975
2. Rice TW, Rodriguez RM, Light RW. The superior vena cava syndrome: clinical characteristics and evolving etiology. Medicine (Baltimore). 2006;85(1):37-42. doi:10.1097/01.md.0000198474.99876.f0
3. Kokotsakis J, Chaudhry UA, Tassopoulos D, et al. Surgical management of superior vena cava syndrome following pacemaker lead infection: a case report and review of the literature. J Cardiothorac Surg. 2014;9:107. Published 2014 Jun 19. doi:10.1186/1749-8090-9-107
4. Mumoli N, Mazzone A, Evangelista I, Cei M, Colombo A. Superior vena cava syndrome after pacemaker implantation treated with direct oral anticoagulation. Thromb J. 2021;19(1):84. Published 2021 Nov 8. doi:10.1186/s12959-021-00321-7

The dangers of veganuary!

 A previously fit and well 40-year-old women presented with a one-hour history of sudden onset left sided abdominal pain. The pain was severe and radiated to the epigastric area. No associated features. No history of alcohol or drug use. On examination she had mild epigastric pain. Point of care ultrasound showed no gallstones. Blood tests showed a lipase >6000 (reference range 11-82) and mildly elevated lactate (1.8mmol). On further questioning the patient had revealed that she had started a strict oatmeal and soymilk diet, which included drinking 64 oz (nearly 1.9 L) of soymilk a day. CT confirmed acute pancreatitis.

Soybean contains a component which is a trypsin inhibitor. It is thought that the large quantity of soybean trypsin inhibitor reduced the proteolytic activity of the intestine removing the negative feedback on the pancreatic acinar cells which results in an inappropriate increase in intrapancreatic trypsin that overwhelmed the normal levels of pancreatic secretory trypsin inhibitor leading to pancreatitis.

Learning Points

The most common causes of pancreatitis in the UK are alcohol and gallstones. However, this article does suggest another potential cause, however I would file it next to scorpion bites in the differential diagnosis/ GET SMASHED mnemonic. 

G – Gallstones 

E – Ethanol 

T – Trauma

S – Steroids

M – Mumps/ malignancy 

A – autoimmune

S – Scorpion sting/ SOY MILK

H – Hypertriglycerides or hypercalcaemia

E – ERCP

D – Drugs

Bottom Line

In the age of increasing use of fad diets, it is important to ask patients about their diet as part of an abdominal pain history.

Reference

deSouza IS, Lipsitt A. The soymilk diet: A previously unknown etiology of acute pancreatitis. American journal of Emergency Medicine. 2021 46(798): 798.e5-798.e6 

Walking in the air..

This case will likely seem familiar. A 33-year-old male is involved in a road traffic collision, appears intoxicated, and is difficult evaluate. This patient had observations in normal range and no pathology found on point-of-care ultrasound examinations including a FAST scan. Given the mechanism and difficulty clinically evaluating the patient due to the alcohol, the patient was sent for a trauma CT scan. Shortly after the injection of the IV contrast with a power injector, the patient was noted to cough vigorously, become tachypnoeic, appear cyanotic and desaturate to 80% on room air. A CT of the chest showed air in the venous system from the left brachiocephalic vein extending into the right ventricle and pulmonary artery. The patient was treated with supplemental oxygen and placement in the left lateral decubitus position in an attempt to keep the buoyant air within the right heart. A repeat CT at 5-hours showed radiological resolution alongside clinical resolution

Learning Points

1. Venous air embolism is the presence of air within the venous circulation

2. Venous air embolisms are rarely caused by penetrating trauma. The cause is often iatrogenic during the placement and removal of venous cannulas, particularly central lines. 

3. They can also occur during contrast-enhanced CT with one study showing the prevalence to be as high as 11.7% when a power injector is used. (2)

4. Fatal air embolism can occur with as little as 100mL. (3)

5. Whilst air should be absorbed within the lung and not enter the cerebral venous circulation, it can in the presence of a right to left shunt e.g., patent foramen ovale.

Bottom line

Be particularly vigilant of the potential for air embolism during contrast-enhanced CT using a power injector. Flush your lines and minimize any delays in leaving a cannula open to air whilst changing connection.

References

1. Buckridge N, Frisch S, Sinert R. Iatrogenic Pulmonary Air Embolism with Rapid Resolution: A Case Report. J Emerg Med. 2021 Aug;61(2):172-173. doi: 10.1016/j.jemermed.2021.02.039. Epub 2021 May 15. PMID: 34006417.
2. Groell R, Schaffler GJ, Rienmueller R, et al . Vascular air embolism: location, frequency, and cause on electron-beam CT studies of the chest. Radiology 1997;202:459–62 . 
3. Yeakel AE . Lethal air embolism from plastic blood-storage container. JAMA 1968;204:267–9

A festive headache can lead to more than one problem

This case describes a 59-year-old woman who presented to the emergency department with the unusual combination of chest pain, left arm numbness and acute memory loss. Her symptoms had started 1-2 hours prior to presentation and the history had to be obtained from her son. The patient had a long-standing 41-year history of migraines and had taken her newly prescribed Sumatriptan after the onset of migrainous aura symptoms. She had a period of global amnesia that lasted 30-60 minutes with her returning to normal en-route to hospital with her last memory being her migrainous aura. The patient’s ECG did not show any acute ischaemia, but her cardiac troponins showed a rise and fall above the upper reference limit and a diagnosis of non-ST elevation myocardial infarction was made. Her CT head was normal and her clinical examination normal. Following neurology review a diagnosis of transient global amnesia was given explaining the memory loss.

Learning points

1. Following the use of simple analgesia, Sumatriptan remains the NICE first line therapy for acute migraine relief treatment. Triptans are highly effective and reduce symptoms from 30 – 90 minutes in 70-80% of patients. Triptans are taken as soon as possible after the onset of the pain or aura 
2. Triptans are a 5-HT agonist and work on 1B and 1D receptor subtypes at blood vessels and nerve endings in the brain
3. Transient global amnesia is a temporary episode of memory loss which cannot be attributed to other more common neurological conditions such as a stroke or a seizure
4. Sumatriptan can cause vasoconstriction and both transient global amnesia and acute coronary syndrome have been described as secondary to triptan induced vasoconstriction. This case highlights the potential for both.

Bottom Line

Sumatriptan mechanism of action is through vasoconstriction and may be contraindicated in patients with multiple comorbidities

Reference

Lee E, Ghafoor N, Jefri M, Black AD, Calello DP, Santos CD. Acute coronary syndrome and transient global amnesia with sumatriptan. Am J Emerg Med. 2022 Mar;53:283.e1-283.e3. doi: 10.1016/j.ajem.2021.09.019. Epub 2021 Sep 11. PMID: 34625330

A BOGOF deal you don’t want this Boxing Day!

A 72-year-old lady presented with progressive dyspnoea and reduced exercise tolerance with a recent pickup of a right lower lobe lung mass, left pleura and right breast lesions on PET scan. Clinically in the emergency department she had features of obstructive shock (HR132, BP 102/72, RR24, Sats 95% room air). Her ECG showed a sinus tachycardia with low voltage QRS and flattened T waves in the inferior and lateral leads. Chest radiograph showed cardiomegaly and bilateral pleural effusions. Bedside echocardiogram showed a large pericardial effusion with right ventricular diastolic collapse, right atrial systolic collapse, kissing left ventricular walls, swinging heart and dilated inferior vena-cava. A diagnosis of cardiac tamponade was made. The patient underwent emergency pericardiocentesis using a subcostal approach with >800ml of haemorrhagic fluid drained and significant clinical improvement. 

However, several hours later she acutely deteriorated with worsening dyspnoea, tachycardia, tachypnoea and increasing oxygen demand. A CT pulmonary angiogram found a submissive PE with evidence of right heart strain. The patient was treated with unfractionated heparin and the decision made not to thrombolyse given the pericardiocentesis. She improved from this acute episode and was discharged on Apixaban. The patient was diagnosed with a primary breast adenocarcinoma with lung metastasis.

Learning Points

1. Occam’s razor is an abductive (logical inference) heuristic which guides clinicians’ diagnostic reasoning towards the simpler explanation where there are competing differentials e.g., the one with the fewest parameters or with the least assumptions.
2. Hickam’s dictum is a counterargument to Occam’s razor which essentially states “A man can have as many diseases as he damn well pleases”.
3. Both PE and pericardial effusions are well known to be caused by malignancy. The concurrent presentation of both with life threatening clinical features is less common.
4. Although Occam’s razor usually serves us well, beware of heuristics and cognitive bias e.g., premature closure that can miss the second pathology

Bottom Line

Beware that more than one acute life-threatening pathology can be concurrently present; Occum’s razor is not always correct

Reference

Pathak V, Manivel V. Defying Occam’s Razor: A Case of Pulmonary Embolism Masked by Cardiac Tamponade. J Emerg Med. 2022 Feb;62(2):e23-e27. doi: 10.1016/j.jemermed.2021.09.020. Epub 2022 Jan 5. PMID: 34998628.

An unexpected complication of not chewing your turkey this Christmas!

A 56-year-old female patient presented to the emergency department with an acutely painful right foot which was cold to touch with absent pulses on palpation and doppler. The pain started almost immediately after an unfortunate event while she was out for dinner. She had choked on a piece of meat and a quick-thinking bystander preformed abdominal thrusts that relieved her airway obstruction. A CT angiogram was performed which showed thromboembolic occlusion of the distal lower limb vessels, just above the ankle. She was treated with a heparin infusion and subsequent operative revascularisation.

Her vascular occlusion was due to cholesterol embolisation as a result of the abdominal thrusts.

Learning points

• Abdominal thrusts can cause acute vascular injury.

• This can be in the form of acute aortic thrombus or dissection, aortic valve injury, and aortic stent displacement.

• This case report describes cholesterol embolisation – the contents of a proximal atherosclerotic plaque are embolised and lodge in distal, narrower vessels. This can happen spontaneously and is also described with vascular procedures such as coronary angioplasty and AAA resection.

Bottom Line

Abdominal thrusts can cause acute vascular injury.

References

1. Pawlukiewicz et al. Cholesterol embolization and arterial occlusion from the Heimlich Manouevre. AJEM. 2021; 290.e2
2. Chillag S, Krieg J, Bhargava R. The Heimlich maneuver: breaking down the complications. South Med J. 2010;103(2):147–50.
3. Kronzon I, Saric M. Cholesterol embolization syndrome. Circulation. 2010;122(6):631–41.

A heart-breaking Christmas story

Paramedics were called to a 36-year male patient who collapsed at home after reportedly encountering the smell of gasoline, feeling immediately nauseated and dizzy. He was initially conscious but confused when paramedics arrived, this improved with fluids and oxygen. On arrival to the ED he reported a significant headache, but no other symptoms. An ABG showed a carboxyhaemoglobin level of 20%, which after 4 hrs of high flow oxygen therapy, reduced to 0.6%. ECG showed sinus tachycardia with non-specific S-T and T wave changes. Given the collapse and ECG changes serial troponins were measured, with results consistent with an acute coronary syndrome (ACS). Medical ACS treatment was started, and an angiogram was performed. This showed patent coronary arteries but apical ballooning.

Acute carbon monoxide poisoning had revealed Takutsubo cardiomyopathy.

Learning points

• Carbon monoxide has a 200-240 times greater affinity for binding with haemoglobin than oxygen, and displaces bound oxygen to form carboxyhaemoglobin, significantly reducing the oxygen carrying capacity of blood resulting in tissue hypoxia.
• Carboxyhaemoglobin also has a greater affinity than oxygen for myoglobin and poisoning can cause myocardial injury.
• Myocardial injury generally takes 2 forms:
• Younger patients develop global left ventricle hyperkinesis and features of cardiogenic shock.
• In patients with pre-existing coronary vessel disease, typically older patients, the demand supply mismatch of oxygenated blood and available myoglobiin binding sites can lead to ischaemic damage.
• In this case the CO poisoning was the only identifiable trigger for inducing Takutsubo cardiomyopathy.

Bottom Line

Carbon monoxide poisoning can cause Takutsubo Cardiomyopathy

References

1. Lakhani M et al. The Poisoned Heart: A Case of Takutsubo Cardiomyopathy Induced by Carbon Monoxide Poisoning. J Emerg Med 2021;60(5);651-654
2. Satran D et al. Cardiovascular manifestation of moderate to severe carbon monoxide poisoning. J Am Coll Cardiol 2005;45:1513–6.

Gingerbread lattes shouldn’t be the only thing you drink this Christmas…

A 43-year-old female patient was brought to the ED who had previously had a Roux-en-Y gastric bypass and had experienced subsequent complications resulting in intestinal malabsorption and chronic pain. Her ED presentation was due to acute agitation, confusion, and lethargy. She had also developed ataxia, lower limb oedema, breathlessness, and a violaceous rash on her hands and lower legs. On initial assessment she was found to be febrile, tachycardic, hypotensive, tachypnoeic and with oxygen saturations of 86% in room air. She had reduced GCS.

Her family relayed to the treating team that for the previous year her diet had consisted almost entirely of a popular coffee franchise’s blended latte drink.

Bloods showed a lactate >11, a haemoglobin of 85 g/L, and an elevated troponin. She was intubated and ventilated and required a high FiO2 and PEEP to maintain adequate oxygenation. Fluid resuscitation and vasopressors resulted in improved haemodynamics but raised lactate persisted. Echo showed evidence of pulmonary hypertension and high output cardiac failure. Severe nutritional deficiency was suspected. She was treated with thiamine and folate supplementation, and also IV antibiotics due to blood cultures positive for group G streptococcus. With these treatments respiratory failure and encephalopathy resolved.

This patient was suffering from beriberi – severe thiamine deficiency

Learning Points

• Nutritional deficiencies can be a potentially reversible cause of critical illness.
• Thiamine has a crucial role in oxidative metabolism and so deficiency primarily effects the nervous and cardiovascular systems due to their high energy demand.
• Beri beri is classified into two forms: 
• Wet beriberi – high output cardiac failure.
• Dry beriberi – peripheral polyneuropathy, ataxia, encephalopathy.
• Wernicke’s encephalopathy is a severe form of dry beriberi and is the most common form seen in developed countries, usually among alcohol dependent patients.
• Treatment of severe thiamine deficiency is with high dose IV thiamine such as Pabrinex.

Bottom Line

Intestinal malabsorption and restricted diets can result in significant nutritional deficits.

References

1. Self M e tal. A Beriberi Unhealthy Latte: Encephalopathy and Shock from Severe Nutritional Deficiency. J Emerg Med 2021;61930;314-319
2. Bal BS , Finelli FC , Shope TR , Koch TR . Nutritional deficiencies after bariatric surgery. Nat Rev Endocrinol 2012;8:544–56 .
3. Chisolm-Straker M , Cherkas D . Altered and unstable: wet beriberi, a clinical review. J Emerg Med 2013;45:341–4 .

Loperamide may not be the answer to your leftover fuelled diarrhoea!

Loperamide AKA Imodium is a readily available and commonly used anti diarrhoeal medication. However, is an emerging drug of abuse due to mu opioid agonist properties. 

In this case a 22-year-old man presented to emergency department after a number of witnessed syncopal episodes. In the department the patient had a collapse episode in which he was pulse less and on telemetry was noted to have polymorphic ventricular tachycardia (pVT). He was defibrillated and given 4g of magnesium which led to ROSC. Post ROSC ECG showed a prolonged QTc with an interval of 667, ST elevation in v1 -v3 with a “coved appearance” followed by a negative T wave consistent with type 1 Brugada. 

The patient reported that he had been self-administering 600mg of loperamide 2 to 3 times a week for 2 months to treat anxiety. The patient was admitted for monitoring and subsequently treated with an isoprenaline infusion due to episodes of bradycardia. The infusion was slowly weaned. The repeat ECG at this point showed a normal QTC of 495 with no Brugada pattern. Genetic testing showed a variant of the GPD1L gene which may be associated with Brugada syndrome. However, this variant has not been previously identified as a cause for Brugada syndrome. 

Learning points

• Beware the collapsing patient and always get an ECG. 
• Consider the potential cardiac effect of commonly used drugs. Other drugs that are commonly used and can lead to long QTc include:
  • Macrolide antibiotics (e.g., erythromycin and clarithromycin) 
  • Antihistamines, (e.g., loratadine, Diphenhydramine)
  • Antiemetics (e.g., ondansetron, cyclizine, metoclopramide), 
  • Antidepressants (e.g., amitriptyline, citalopram, fluoxetine, sertraline, ECT)
  • Antipsychotics (e.g., haloperidol, olanzapine, amisipride, chlorpromazine)
  • Antiarrhythmics (e.g., flecanide, amiodarone, sotalol)
• Other cause of prolonged QTC include hypokalaemia, hypomagnesia and hypocalcaemia. 
• In genetically susceptible patient, supratherapeutic and even therapeutic doses can prolong QTC and lead to potential complication. There are several cases studies when patients have had cardiac arrhythmia unmasked due to medication and electrolyte abnormalities ^2,3.   Therefore, consider performing ECG before using drugs especially if using in high doses or multiple drugs that may prolong QTc e.g., multiple antiemetic in a vomiting patient.
• For more information on Brugada syndrome see the two following excellent summaries, RCEM Learning ST elevation with infarction and LITFL “what is Brugada syndrome” ^4,5.

Bottom Line

Remember to ask about over the counter drugs when taking a drug history. Patient don’t always volunteer this information. 

References

1. Simon M, Rague J. A case report of torsade de pointes and Brugada Pattern associated with Loperamide Misuse and supratherpeutic Loperamide concentrations. The Journal of emergency medicine. 2021;61(4):e54-e59. 
2. Sethi P, Treece J, Pai V, Onweni C. Long QT syndrome unveiled by a fatal complication of medication and electrolyte abnormalities. Cureus 2017;9(8):e1581
3. Tiver FD, Dharmaprani D, Quah JX, lahiri A, Waddell-smith KE, Ganesan AN. Vomiting, electrolyte disturbance, and medications; the perfect storm for acquired long QT syndrome and cardiac arrest: a case report. Journals of medical reports 2022; 16: 9. 
4. RCEM Learning. ST Elevation without Infarction. Obtained on 15/12/22
5. Life in the Fast Lane. Brugada Syndrome. Obtained on 15/11/22

The ghost of Christmas past. Beware the recurrent abdominal pain!

A patient presenting with recurrent abdominal pain can often feel like a heartsink moment. This case demonstrates the need to keep an open mind at each presentation and consider unusual diagnoses. 

In this case, 32-year-old women presented with a 10-year history of episodes of severe, diffuse abdominal pain, which she rated at 10/10. These episodes occurred every month or two and usually lasted for several days. They were unrelated to her menstrual cycles and occurred without an apparent precipitant. Between episodes, she reported having mild, cramping mid-abdominal pain almost every day, with the pain fluctuating in severity throughout the day. The pain was not associated with food intake but was aggravated by running. She had been hospitalised three times for episodes of severe pain. Episodes of severe pain preceded episodes of constipation. Blood tests and CT examination were normal except the patient was noted to have hyponatraemia during two visits. Subsequent colonoscopy was also normal. The patient was also noted to have episodes of psychiatric symptoms during the flare up. During the patient’s most recent admission to hospital it was decided to test for porphyria due to the patient’s recurrent severe abdominal pain, hyponatraemia and transient neuropsychiatric manifestation. A urine porphyrin panel was obtained and revealed elevated levels of porphobilogen, and subsequent genetic testing revealed the diagnosis if acute intermittent porphyria. In retrospect the nursing staff report the patient’s urine being purple.  

Learning points

Acute porphyrias are a group of rare genetic disorders in which there is a build-up porphyins or similar chemicals used to make porphins. This is an essential part of haem synthesis. They are notoriously difficult to diagnose. About 1 In 75,000 people suffer from them.  2

Consider the diagnosis in patients presenting with:

• Recurrent episodes of severe abdominal, back, arms or leg pain
• Associated with nausea, vomiting and constipation
• Hyponatraemia 
• Confusion 
• Convulsion of muscular twitching 

See article for detailed summary or visit porphyria.org.uk 1,2

However, after excluding a potential medical cause it is also important to consider the complex psychophysiology of recurrent abdominal pain. See this excellent article published in the EMJ by Daniels et al³. which makes some clinical recommendation of how to manage this overrepresented and poorly understood group. The recommendation suggests using the ERROR mnemonic to approach these patients.

E – Exclude medical emergency
R – Review attendance and care plan – review previous attendances or care plans
R – Record relevant factors – assessment of relevant psychosocial factors
O – Offer credible explanation of pain – 

• State that no evidence of medical emergency 
• Compassionately acknowledge the patient pain, validate the patient distress and recognise that pain in distressing regardless of the origin. 
• Offer credible biopsychosocial explanation of pain (More details in article)
• Advise of factors that may inhibit recovery. 

R – Refer on. 

Bottom Line

Recurrent abdominal pain is a common presentation. This is a poorly understood group. A careful history and examination are essential even if they have seen many clinicians in the past. You may catch something that somebody else has missed. If there are no medical cause treat patients with compassion and a credible explanation for their pain may help the patient manage their symptoms and prevent re-attendance.

References

1. Fredrick TW, Braga Neto MB, Johnsrud DO, Camilleri M and Chedid VG. Turning purple with pain. New England journal of medicine. 2021;385(6):549-554
2. British Porphyria Association. What is Porphyria? Obtained on 15/12/22. 
3. Daniels J, Griffiths M, Fisher E. Assessment and management if recurrent abdominal pain in the emergency department. 2019;37(8):515-521