By Monzur Ahmed
Pharyngeal pouches (diverticula) are a common cause of dysphagia; they are associated with various morbidities and a decreased quality of life. There are several types of diverticula which are classified according to the anatomical location of their origin relative to the cricopharyngeal muscle. These include Zenker's, Killian-Jamieson's, and Laimer's diverticula. By far the most common is Zenker's diverticula, characterised by a posterior outpouching originating from Killian's dehiscence of the inferior constrictor muscle, between the cricopharyngeal and thyropharyngeal muscles. The Killian-Jamieson diverticulum is much rarer and is a herniation through the anterolateral located superolateral to the longitudinal muscle of oesophagus and inferior to the cricopharyngeal muscle. The rarest variant of pharyngeal diverticulum is often referred to as Laimer's or Laimer-Haeckerman's diverticulum. Similar to the Killian-Jamieson diverticulum, it originates between cricopharyngeus and the longitudinal muscle of the oesophagus; however, it is located posteriorly and midline, from the area termed Laimer-Haeckerman's triangle, and is covered only by the circular muscles of the oesophagus. Pharyngeal pouches can make endoscopic intubation difficult and even hazardous, with a risk of perforation. Hence, if a pouch is suspected or proven, then it is important to appreciate the anatomy of the pharyngeo-oesophageal region in order to safely intubate the oesophagus. The video shows a recent case of an elderly co-morbid patient where previous attempts at intubation were unsuccessful (see video)…
THE PACKAGE DEAL
by Monzur Ahmed
“He who controls the Spice, controls the Universe.” Baron Harkonnen, Dune
In the science fiction world of Frank Herbert’s “Dune,” the drug, Spice, was the most essential and valuable commodity in the universe. Found only on the desert planet Arrakis, the drug gave the user a longer life span, greater vitality, and heightened awareness; it could also unlock prescience in some humans, depending upon the dosage and the consumer's physiology. In real life, Spice or the “Zombie drug” seems to have similar importance in UK prisons. Here is a case from our hospital of a Spice smuggling prisoner who was admitted handcuffed and chained to a prison officer...
A 28 year old male prisoner had a history of epilepsy, personality disorder and drug abuse. Eleven days ago he swallowed drug package containing “Spice” (synthetic cannabinoid) with a street value of £1000. The package did not pass through his system as he intended. So, 5 days ago, he swallowed an USB stick and one AA battery to push the package along! Unfortunately this strategy did not work and he presented to hospital one day ago because the package still had not come out the other end. The initial AXR and CXR showed a AA sized battery and a USB stick in the stomach. In addition there was an oval shaped outline of what could have been the drug package in the fundus of the stomach. An urgent out of hours OGD was performed by a colleague. A battery and a large oval package were visible in the stomach but it was not possible to extract the package with a snare because the package would not pass through the upper oesophageal sphincter. An overtube was then used, but again the procedure was unsuccessful. The next day, the surgeons were preparing to take him to theatre but asked me to have a last ditch attempt at endoscopic extraction (see video)… The procedure was performed with the prisoner still handcuffed and chained to one of the two accompanying prison officers!
Spice is the name given to a synthetic cannibinoid (SCB) which emerged around 2013. SCB are highly potent CB1 cannabinoid receptor agonists falsely marketed and sold as safe and legal drugs. Recreational use of SCB is an increasing public health problem specifically in Western societies, with teenagers, young adults, and the prison population being the most affected. Some of these SCB are analogs of tetrahydrocannabinol, aminoalkylindoles, and other phytocannabinoid analogs have been detected in herbal preparations generically called "Spice." Spice, "K2" or "fake cannabis" is a general term used for variable herbal mixtures of unknown ingredients or chemical composition. Few drugs have achieved such notoriety in so little time, wreaking chaos within the prison service and placing huge pressures on the emergency services. In every town centre, spice users can be found begging for the small amount of loose change they need to buy their next fix. Spice started life as a “legal high” – a laboratory-engineered chemical that claimed to mimic the effects of cannabis but has since been banned, but post-ban, the drug continues to devastate the lives of two of society’s most vulnerable – and ignored – groups who don’t show up in the official figures: the street homeless and prisoners, with membership of one group often conferring membership of the other.
1.Davidson C, Opacka-Juffry J, Arevalo-Martin A et al,Adv Pharmacol. 2017;80:135-168. Spicing Up Pharmacology: A Review of Synthetic Cannabinoids From Structure to Adverse Events
2.Spaderna M, Addy PH, D’Souza DC,Psychopharmacology (Berl),2013 Aug;228(4):525-40. Spicing things up: synthetic cannabinoids
PRADER WILLI SYNDROME AND PHYTOBEZOAR
by Monzur Ahmed
One of my favourite TV shows is the forensic crime drama “CSI: Crime Scene Investigation" (the original Las Vegas version, starring William Peterson as Gil Grissom, not the subsequent spin-offs). The show ran for 15 seasons. In one memorable episode entitled “Dog Eat Dog,” the body of a man is found in a dumpster; cause of death unknown . What appears to be blood on his lips turns out to be cranberry sauce. Initially foul play is suspected but it transpires that the man died from asphyxia caused by gastric distention. The postmortem revealed vast amounts (and variety) of food in the stomach. After some detective work, Grissom diagnoses Prader-Willi syndrome, a condition associated with an insatiable appetite. The dead man literally ate himself to death having won a food-eating contest along the way! When I first watched this episode of CSI, I marvelled at the ingenuity of the script writers. A few days ago, I was reminded of “Dog Eat Dog" by a patient...
A 22 year old man was admitted to my hospital with a one day history of severe abdominal pain, distention and nausea but no vomiting. He was still opening his bowels and passing wind. He had a history Prader-Willi syndrome and had spinal surgery for scoliosis at age 10 years. There was also a history of anxiety. For 3 years he had been living in a Residential Home where his meals were regulated. He was a vegetarian. On examination, he was not obese (BMI 20 kg/m^2) and had a distended, tender abdomen. Initial AXR showed a very distended stomach containing food material. A CT scan confirmed these findings and worryingly the report mentioned “imminent danger of perforation.” The surgeons were called in, presumably with a view to surgery, but instead they asked me to do an OGD to see if there was anything that could be done endoscopically. A large phytobezoar was found in the stomach which proved challenging to treat (see video)...
Prader-Willi syndrome (PWS) was first described by Prader et al. in 1956 and is now recognised as a multisystemic complex genetic disorder caused by lack of expression of genes on the paternally inherited chromosome 15q11.2-q13 region [2,3]. There are three main genetic subtypes in PWS: paternal 15q11-q13 deletion (65–75 % of cases), maternal uniparental disomy 15 (20–30 % of cases), and imprinting defect (1–3 %). Clinical manifestations change with age with hypotonia and a poor suck resulting in failure to thrive during infancy. As the individual ages, other features such as short stature, food seeking/ hyperphagia with excessive weight gain, developmental delay, cognitive disability and behavioural problems become evident. The phenotype is likely due to hypothalamic dysfunction, which is responsible for hyperphagia, temperature instability, high pain threshold, hypersomnia and multiple endocrine abnormalities including growth hormone and thyroid-stimulating hormone deficiencies, hypogonadism and central adrenal insufficiency. Obesity and its complications are the major causes of morbidity and mortality in PWS. Hyperphagia (constant eating due to an insatiable appetite) is a common feature in individuals with PWS. In one study, four reports of unexpected mortality due to gastric rupture and necrosis were found in 152 reported deaths in individuals with PWS, accounting for 3% of mortality . Four additional individuals were suspected to have gastric rupture. Vomiting and abdominal pain, although rare in PWS, were frequent findings in this cohort. A bezoar is an indigestible conglomeration trapped in the gastrointestinal tract. This indigestible mass can be formed by a variety of materials that were intentionally or accidentally ingested . Bezoars are mainly classified into four types depending on the material constituting the indigestible mass of the bezoar: phytobezoars, trichobezoars, pharmacobezoars, and lactobezoars. Gastric bezoars can cause outlet obstruction, ulcerative lesions and subsequent bleeding, whereas small intestinal bezoars present with small bowel obstruction and ileus. The currently available treatment options for a gastric phytobezoar include dissolution of the bezoar by Coca-Cola®, removal by endoscopic devices, laparotomy, and laparoscopic surgery. To my knowledge, phytobezoar as a result of hyperphagia has not been previously reported as a complication of PWS.
1. CSI: Crime Scene Investigation, season 6, episode 9, "Dog Eat Dog"
2. Prader A, Labhart A, Willi H. Ein Syndrom von Adipositas, Kleinwuchs, Kryptorchismus und Oligophrenie nach myatonieartigem Zustand im neugeborenenalter. Schweiz Med Wochenschr. 1956;86:1260–1261.
3. Angulo MA, Butler MG, and Cataletto ME, J Endocrinol Invest. 2015; 38: 1249–1263. Prader-Willi syndrome: a review of clinical, genetic, and endocrine findings
4. Stevenson DA et al J Pediatr Gastroenterol Nutr. 2007 Aug; 45(2): 272–274., Gastric Rupture and Necrosis in Prader-Willi Syndrome
5. Iwamuro M et al, World J Gastrointest Endosc. 2015 Apr 16; 7(4): 336–345.Review of the diagnosis and management of gastrointestinal bezoars
by Monzur Ahmed
A 25 year old female gave a 6 months history of high dysphagia and choking. The symptoms were worse with solids. She had lost one stone in weight. 3 weeks ago, an urgent (2 week wait) OGD was performed by one of our Nurse Endoscopists. This showed an impassable high oesophageal stricture (not biopsied). A subsequent barium swallow showed a short narrowing at the junction of the hypopharynx and oesophagus. Routine blood tests revealed iron deficiency anaemia (Hb 8.0 g/d, MCV 66). On direct questioning, the patient admitted to longstanding menorrhagia. There was no PR bleeding or haematuria and she was not a vegetarian. She was referred for a further OGD with a view to dilatation (see video…)
In 1906 D. R. Paterson described, in The British Medical Journal, the condition of inflammation, accompanied by spasm and stenosis, of the lower pharynx . Paterson pointed out that this may be a cause for dysphagia, and in 1919, discussing dysphagia in women, he noted that cheilosis, glossitis, pharyngitis, and postcricoid carcinoma could be a sequel . Anaemia was not mentioned in either of Paterson's papers, but, also in 1919, A. Brown-Kelly added this feature to the account given by Paterson , and thus the British eponym Paterson Brown-Kelly (PB-K) syndrome was introduced. In the United States, Mayo clinic physician, H. S. Plummer described (though not published) a series of patients with long-standing iron deficiency anaemia, whom he said tended to develop hysterical dysphagia! It was not until 1922 that P. P. Vinson, his pupil also at the Mayo clinic, published a full account which emphasised that the spasm was secondary to anaemia . Neither Plummer nor Vinson mentioned postcricoid carcinoma as a complication. Should the condition be called Patterson Brown-Kelly (PB-K) syndrome or Plummer Vinson (PV) syndrome? In an assessment of the merits of the various contenders for priority, F. C. Ormerod considers that Brown-Kelly and Paterson have the edge on their rivals . J. Waldenstrom emphasized that a low level of iron in the plasma (sideropenia) rather than anaemia was a basic feature of the syndrome . He described a woman with postcricoid dysphagia, but who was not anaemic, whose dysphagia was cured by iron. Other manifestations of iron deficiency, such as soreness of the tongue and fissures at the corners of the mouth, which were present in this patient, were also cured by the iron therapy. Waldenstrom suggested that the formation of a postcricoid "web" (which is shown by a barium swallow) and the dysphagia were due to iron deficiency, and subsequently this association has been recognized frequently. Nowadays, PB-K syndrome is said to be characterized by the classic triad of dysphagia, iron-deficiency anemia and esophageal web. However, even after a century since the first case reports, the aetiology of PB-K syndrome remains largely unknown. Although genetic predispositions and several other mechanism have been postulated, the evidence remains weak although iron deficiency appears to consistently play an important role. This is partly due to studies which have reported an improvement in dysphagia with iron supplementation whereas iron deficiency is suspected to cause mucositis leading to web formation. As patients with PB-K syndrome may also suffer from malnutrition, deficiency of vitamin B has also been suggested as a cause although the evidence is weak and inconclusive. Other disorders reported to be associated with PB-K syndrome include celiac disease, Crohn's disease, rheumatoid arthritis and thyroid disease raising the possibility immune dysregulation may be involved in its pathogenesis although this remains to be proven [7-9]. Patients with PB-K syndrome have an excellent outcome with most symptomatic patients requiring only one OGD with dilatation for complete relief of symptoms in conjunction with iron replacement therapy. Patients are at an increased risk of developing squamous cell carcinoma of hypopharynx or upper oesophagus which may be related to chronic iron deficiency. This is believed to to cause irreversible mucosal changes leading to malignant degeneration .
1. Paterson, D. R., Brit. Med. J., 1906, 2: 353.
2. Paterson D.R., J. Laryng., 1919, 34: 289.
3. Kelly, A. Brown, J. Laryng., 1919, 34: 285.
4.Vinson, P. P., Minn. Med., 1922, 5: 107.
5. Ormerod, F. C., J. Laryng., 1966, 80: 894.
6. Waldenstrom, J., Acta med. scand., Supplement, 1938, 90: 380.
7. Hefaiedh R, Boutreaa Y, Ouakaa-Kchaou A et al. Plummer Vinson syndrome association with coeliac disease. Arab J Gastroenterol. 2013;14(4):183-5.
8. Medrano M. Dysphagia in a patient with rheumatoid arthritis and iron deficiency anemia. MedGenMed. 2002; 28;4(3):10.
9.Park JM, Kim KO, Park CS, Jang BI. A case of plummer-vinson syndrome associated with Crohn's disease. Korean J Gastroenterol. 2014; 63(4):244-7.
10. Watts JM. The importance of the Plummer-Vinson syndrome in the aetiology of carcinoma of the upper gastrointestinal tract. Postgrad Med J. 196;37:523-33.
Monzur Ahmed, Consultant Gastroenterologist