by Monzur Ahmed
Here is an unusual case which most gastroenterologists won’t see too often. A 36 year old lady presented at the age of 16 with abdominal pain. She was noted to have oral pigmentation and a clinical diagnosis of Peutz-Jeghers syndrome was made. There was no family history of this condition. A small bowel enema showed 3 large ileal polyps causing intussusception and she underwent small bowel (SB) resection and polyp excision. At the age of 21, she had a right hemicolectomy, SB resection and SB polypectomy, again for intussusception.
Over the years, she had numerous surveillance OGDs, colonoscopies and gynaecological / breast screening. Two years ago, a video capsule endoscopy showed duodenal/ jejunal polyps. Hence a oral route double balloon enteroscopy (DBE) was performed and 3 upper SB polyps were resected. A year ago, a further video capsule endoscopy showed ileal polyps, including one measuring ~2cm. Therefore, she now attends for rectal route DBE to remove the ileal polyp (see video)...
Peutz-Jeghers syndrome (PJS) is part of a heterogeneous group of disorders, known as hamartomatous polyposis syndromes that involve the growth of multiple polyps in the gastrointestinal tract of affected individuals. PJS first described in a pair of twin sisters with dark pigment spots on their lips and oral mucosa by J.T. Connor in 1895. These symptoms were attributed to a familial syndrome in 1921, when Jan Peutz described four affected siblings. The syndrome was then defined as a distinct entity by Harold Jeghers in 1949 when he described 10 cases and was subsequently named Peutz Jeghers syndrome in 1954. The gene causing PJS (STK-11/LKB1) was identified in 1998 and allows early detection of the disease and screening of family members.
PJS affects males and females in equal numbers and can occur in any racial or ethnic group. The birth prevalence of PJS is estimated to be between 1/50,000 and 1/200,000. PJS is characterised by multiple hamartomas on the mucous lining of the gastrointestinal system and spots of dark blue to dark brown skin freckling (melanocytic macules) around the mouth, eyes, nostrils, fingers, oral mucosa and anus. These melanocytic macules can appear as early as the first year of life and are present in most affected children under five years of age. They tend to fade away with age and might completely disappear in puberty or adulthood, although they tend to persist in the oral mucosa. Polyps also begin to grow within the first years of life, but associated symptoms typically arise between 10 to 30 years of age. Around half of patients with PJS have to undergo surgery by age 18 because of polyps-related complication. Polyps most often tend to develop in the small intestine (60-90%), especially in the jejunum, but can also arise in the stomach and large intestine. Rarely, polyps can grow outside the gastrointestinal tract and affect the ureters, bladder, lungs, bronchi, and gallbladder. Gastrointestinal polyps can cause abdominal pain, vomiting, diarrhoea, intestinal obstruction and rectal bleeding, which can lead to anaemia. They can also provoke intussusception, which can lead to severe abdominal pain and emergency surgery.
Individuals with PJS are at a highly increased risk of developing gastrointestinal and other cancers including breast, cervical, uterine, pancreas, and lung. The lifetime risk of developing cancer in affected individuals can be as high as 93%. The cumulative risk of GI cancers (excluding pancreatic cancer) has been reported to be around 33% at the age of 60, increasing to 57 % at the age of 70 years. Individuals that develop cancer are usually affected around their fifth decade of life (age 40-49 years). Affected females have an increased risk for a benign ovarian tumour called SCTAT (sex cord tumours with annular tumours) for which symptoms may include irregular or heavy periods or early puberty. Usually before age 20, affected males can develop Sertoli cells carcinoma of the testes that secretes oestrogen and can lead to gynaecomastia.
PJS is an autosomal dominant condition caused by mutations in the STK11/LKB1 gene which is located at 19p13.3. The abnormal gene can be inherited from either parent, or can be the result of a new mutation (de novo) in the affected individual. Approximately 60-78% of individuals with PJS have an affected relative. Around 80-94% of PJS patients have an identified mutation in the STK11 gene, which means that other genes are possibly involved in the disease. The penetrance of the STK11 gene mutation is thought to be 100% (which means that someone with a pathogenic mutation has a 100% chance of developing the disease), so genetic testing can give a definite diagnosis before symptoms appear. The STK11 gene produces a protein that is involved in the regulation of cell division and apoptosis (programmed cell death). It also interacts with p53, a major tumour suppression protein. Pathogenic mutations in STK11 lead to either cessation or dysfunction of protein production by the gene and uncontrolled cell growth, which can in turn lead to the development of hamartomas and cancer.
The dark pigmented spots (melanocytic macules) are thought to be caused by inflammation and blockage of melanin migration from cells where it is produced (melanocytes) to cells forming the outermost layer of the skin (keratinocytes).
A clinical diagnosis of PJS can be made when any one of the following criteria is present:
1. Presence of at least 2 histologically confirmed PJS polyps
2. Any number of PJS polyps with a positive family history of PJS
3. Presence of characteristic mucocutaneous pigmentations with a positive family history of PJS
4. Any number of PJS polyps in an individual with characteristic mucocutaneous pigmentation
Surveillance of the GI tract in PJS patients has two purposes:
1. To detect GI polyps that may cause complications (bleeding, anaemia, intussusception) and should be removed (in particular small-bowel polyp-related complications are the predominant clinical problem);
2. To detect cancer (mainly occurring in adults) at an early stage.
After initial diagnosis, it is recommended that individuals older than 8 years or having symptoms undergo endoscopic and small bowel examination. The latter can be done with magnetic resonance imaging of the intestines (magnetic resonance enterography, MRE) or video capsule endoscopy, (VCE). Gynaecologic and breast examination are also recommended for women older than 18 years. Testicular examination is recommended for men.
The ESGE recommendations for PJS are:
1. Perform a baseline OGD and colonoscopy at the age of 8 years in asymptomatic individuals with PJS.
2. Start routine OGD and colonoscopy surveillance at the age of 18 if the baseline endoscopy is negative.
3. An interval of 1 – 3 years based on phenotype for OGD and colonoscopy.
4. Small-bowel surveillance from the age of 8 years in asymptomatic individuals with PJS.
5. An interval of 1 – 3 years based on phenotype for small-bowel surveillance.
6. Either MRI studies or video capsule enteroscopy for small-bowel surveillance.
7. Elective polypectomy should be performed for small-bowel polyps >15–20mm to prevent intussusception. In a symptomatic patient, smaller polyps causing obstructive symptoms should be removed.
8. Device-assisted enteroscopy for the removal of polyps. Based on phenotype, intraoperative enteroscopy could be considered.
These guidelines are similar to those of the BSG / ACPGBI/ UKCGG.
1. Beggs AD, Latchford AR, Vasen HFA et al; Gut 2010 Jul;59(7):975-86.
Peutz-Jeghers syndrome: a systematic review and recommendations for management
2.Peutz Jeghers Syndrome, NORD
3.van Leerdam ME, Roos VH, van Hooft JE et al, Endoscopy 2019; 51
Endoscopic management of polyposis syndromes: European Society of Gastrointestinal Endoscopy (ESGE) guideline
4.Monahan KJ, Bradshaw N, Dolwani S, Hereditary CRC guidelines eDelphi consensus group, et al. Gut 2020;69:411-444.
Guidelines for the management of hereditary colorectal cancer from the British Society of Gastroenterology (BSG)/Association of Coloproctology of Great Britain and Ireland (ACPGBI)/United Kingdom Cancer Genetics Group (UKCGG)
by Monzur Ahmed
A 76 year old man was admitted with a 3 week history of painless jaundice, dark urine and one stone weight loss. Initial investigations were as follows:
- Bilirubin 181 umol/L, ALT 105, Alk Phosp 270 (NR<130)
- WBC 8.4 increasing to 16, Hb 14.2 g/dL, Platelets 370
- CT scan:
- large calcified stone in low CBD
- dilated CHD/ IHD
It was thought that he had cholangitis secondary to a CBD stone. An ERCP, performed 2 days ago by colleagues, showed a bulky papilla and despite a needle knife precut, it was not possible to enter the bile duct. He was therefore referred for a second ERCP 2 days later (see video)...
Cannulation again proved difficult and the needle knife was again used to perform a further precut and fistulotomy. The CBD was eventually accessed but the stone proved stubborn. A sphincterotomy and sphincteroplasty were necessary before we were able to extract the stone with a balloon (dilatation assisted stone extraction, DASE). Finally, a pigtail stent was inserted because there was an additional short stricture (??) in the mid bile duct. Despite the messy procedure, the patient did not have any complications and went home a couple of days later with a view to having another ERCP 6 weeks later to remove the stent and reassess the CBD.
By Monzur Ahmed
An 81 year old man presented with chronic diarrhoea but no rectal bleeding or weight loss. An index colonoscopy, performed by a colleague 4 weeks ago showed 4 polyps. Three of the smaller polyps (1-2cm) were removed from the transverse and descending colon and histology showed tubular adenoma with low grade dysplasia. The fourth polyp was about 6cm in diameter and located in the upper rectum behind a valve of Houston. This polyp was not biopsied and he was referred for a further procedure (see video). The video highlights:
- use of gastroscope to improve access in an awkwardly situated polyp
- using large volume of lift solution - working in retroflexion to clear the oral aspects of the polyp
- using Coagrasper to coagulate vessels on base
- using APC to treat the edges of the defect
By Monzur Ahmed
Recently I had to perform two back to back urgent endoscopies in patients who had swallowed foreign bodies. Both procedures were done under GA with the patient intubated. One patient swallowed button batteries and magnets (a potentially deadly combination) whilst the other ingested a metal nail file and one limb of a pair of tweezers. The video shows the use of Roth net, snare and Gaurdus overtube.
19 year old female
History of personality disorder
Multiple previous admissions for swallowed foreign bodies
- admitted after staggered paracetamol overdose over 3 days and ingestion of several button batteries + neodymium magnets
- AXR: multiple button batteries / magnets in stomach
- Urgent out of hours OGD under GA by a colleague: stomach full of food, unable to retrieve foreign bodies
Next day: repeat OGD under GA (see video)
31 year old female
History of schizophrenia, learning difficulties, personality disorder, asthma
Multiple ingestion of foreign bodies in past
Admitted after swallowing metal nail file + tweezers 24 hrs ago
AXR: nail file in stomach, tweezers in small bowel
OGD performed 48 hours after ingestion, under GA (see video).
Most ingested foreign bodies (80%–90 %) pass spontaneously. However, approximately 10 %–20 % of cases of foreign body ingestion require endoscopic removal, while less than 1 % will need surgery for foreign body extraction or to treat complications. The ESGE 2016 guidelines  on foreign body removal may be summarised as follows:
-diagnostic evaluation based on the patient’s history and symptoms. Physical examination focused on the patient’s general condition and to assess signs of any complications.
-CT scan in all patients with suspected perforation or other complication that may require surgery.
-plain radiography to assess the presence, location, size, configuration, and number of ingested foreign bodies if ingestion of radiopaque objects is suspected or type of object is unknown.
-clinical observation without the need for endoscopic removal for management of asymptomatic patients with ingestion of blunt and small objects (except batteries and magnets). If feasible, outpatient management is appropriate.
-close observation in asymptomatic individuals who have concealed packets of drugs by swallowing (“body packing”). We recommend against endoscopic retrieval. We recommend surgical referral in cases of suspected packet rupture, failure of packets to progress, or intestinal obstruction.
-radiological evaluation for patients with non-bony food bolus impaction without complications.
-barium swallow, because of the risk of aspiration and worsening of the endoscopic visualisation.
B. ENDOSCOPIC MEASURES
-emergent (preferably within 2 hours, but at the latest within 6 hours) therapeutic OGD for foreign bodies inducing complete oesophageal obstruction, and for sharp-pointed objects or batteries in the oesophagus.
-urgent (within 24 hours) therapeutic OGD for other oesophageal foreign bodies without complete obstruction.
-treatment of food bolus impaction in the oesophagus by gently pushing the bolus into the stomach. If this procedure is not successful, retrieval should be considered. The effectiveness of medical treatment of oesophageal food bolus impaction is debated. It is therefore recommended, that medical treatment should not delay endoscopy.
-In cases of food bolus impaction, ESGE recommends a diagnostic work-up for potential underlying disease, including histological evaluation, in addition to therapeutic endoscopy.
-urgent (within 24 hours) therapeutic OGD for foreign bodies in the stomach such as sharp-pointed objects, magnets, batteries and large/long objects. We suggest nonurgent (within 72 hours) therapeutic OGD for medium-sized blunt foreign bodies in the stomach.
-use of a protective device in order to avoid oesophagogastric / pharyngeal.damage and aspiration during endoscopic extraction of sharp pointed foreign bodies. Endotracheal intubation should be considered in the case of high risk of aspiration.
-ESGE suggests the use of suitable extraction devices according to the type and location of the ingested foreign body.
-After successful and uncomplicated endoscopic removal of ingested foreign bodies, ESGE suggests that the patient may be discharged. If foreign bodies are not or cannot be removed, a case-by-case approach depending on the size and type of the foreign body is suggested.
1.Birk M, Bauerfeind P, Deprez PH et al 2016, Removal of foreign bodies in the upper gastrointestinal tract in adults: European Society of Gastrointestinal Endoscopy (ESGE) Clinical Guideline, Endoscopy 2016; 48: 1–8
By Monzur Ahmed
A 68 year old man was admitted with a 2 week history of painless jaundice, dark urine, 2-3 stone weight loss and poor appetite. Initial investigations were as follows:
- Bilirubin 330 umol/L, ALT 230, Alk Phosp 420 (NR < 130)
- Hb15.3 g/dL, WBC 14, platelets 210
A CT scan showed a 5cm tumour in head of the pancreas with a dilated pancreatic duct (14mm) and dilated common hepatic duct and intrahepatic ducts. There was no evidence of metastasis or vascular invasion. It was thought that the appearances were compatible with pancreatic adenocarcinoma. He was discussed with the local HPB unit. In view of the COVID-19 pandemic, surgery was deferred and the recommendation was an ERCP with biliary stenting with subsequent “downstaging” chemotherapy as a prelude to surgery once the COVID situation improves! Hence the patient attended for ERCP. There was a surprise finding (see video)...
Intraductal papillary mucinous neoplasms (IPMNs) result from the intraductal proliferation of mucin producing neoplastic epithelium, leading to hypercellular outgrowths that secrete frank, mucinous fluid. IPMNs were first described in 1982 by Ohhashi et al, who described four patients with successfully resected main duct IPMN (MD-IPMN) . The 1996 World Health Organization (WHO) classification designated these tumours as intraductal papillary mucinous tumors  and this was revised to intraductal papillary mucinous neoplasm in the 2010 publication .
IPMNs are classified as main duct (MD-IPMN), branch duct (BD-IPMN) or mixed type according to the anatomic involvement of the pancreatic ductal system. MD-IPMN have a higher risk of malignant transformation; the risk of malignancy has been found to range from 19% to 30% in BD-IPMNs and as high as 40% to 60% in MD-IPMN [4-7].
Histologically, IPMNs can be classified into four subtypes based on morphological and immunohistochemical characteristics: gastric, intestinal, pancreatobiliary and oncocytic . While not always the case, gastric type IPMNs typically displays low-grade dysplasia, intestinal type often displays moderate to high-grade dysplasia, while the less common pancreatobiliary type and oncocytic type often display high-grade dysplasia. The two main histopathological types of invasive IPMN are colloid carcinoma, which typically arises from intestinal type IPMN, and tubular carcinoma, which arises from pancreatobiliary type IPMN. Histologic subtypes are typically only determined following surgical resection and currently may not be used to dictate clinical management.
Endoscopic ultrasound (EUS) should be considered as a ‘second-level’ diagnostic modality after CT and MRI. EUS is an invasive, operator-dependent imaging technique with the possibility of performing fine-needle aspiration (FNA) for cyst fluid analysis. EUS is useful in differentiating IPMNs from other pancreatic cysts, including macrocystic serous cystadenomas (SCAs), mucinous cystic neoplasms (MCNs) or pseudocysts. By analysing CEA level, amylase/lipase, glucose as well as cytology in the cyst fluid, it may be possible to increase the diagnostic yield in their differential diagnosis. A cut-off ≥192 ng/ml of cyst fluid carcinoembryonic antigen (CEA) has been suggested for distinguishing mucinous from non-mucinous cysts, with a sensitivity of 38–78% and specificity of 63–99%, but it is not helpful in distinguishing between benign and malignant mucinous cysts. Similar results are reported for cytological examination. At ERCP, IPMNs are characterised by the "fish mouth papilla” which is said to be diagnostic.
The management of IPMNs is dependent on the accurate assessment of the risk of malignant transformation. Surgical resection remains the best treatment to avoid this unfavourable outcome. However, two major problems remain: first, many BD IPMN (75–82%) will never progress to malignancy; and second, pancreatic surgery inherent morbidity is not negligible. Therefore, it is crucial to identify the patients who will actually need surgical resection. Several guidelines have been established to define the surgery criteria, depending on the presence or not of “worrisome signs” and “high risk stigmata of malignancy” (i.e., high grade dysplasia and invasive carcinoma [4,5,6,7,9,10]. In these algorithms, the patient’s performance status is taken into account to determine the surgery benefit/risk ratio.
The Sendai and Fukuoka consensus conferences of 2006 and 2012 / 2017 respectively [9,4,5], established some baseline clinical and radiological criteria, especially in terms of tumour size and associated lesions (lymphadenopathy), to guide the decision concerning the patient management (surveillance or surgical treatment). This consensus separated criteria in “worrisome” and “high-risk stigmata” that allow or not the surgical option. The Sendai guidelines , recommended resection for all MD-IPMNs and mixed type IPMNs if patients are good surgical candidates. Resection was also recommended for symptomatic patients with BD-IPMNs, or BD-IPMNs with main duct dilatation > 6mm or the presence of mural nodules. It was also recommended that lesions > 3 cm be resected, with the caveat that more data was required to determine whether all BD-IPMNs > 3 cm should be resected immediately.
The revised Fukuoka guidelines of 2012  decreased the threshold of main duct dilatation for characterisation of MD-IPMN to > 5 mm without other causes of obstruction. Resection was recommended for all surgically fit patients with MD-IPMN. Both “high risk stigmata” and “worrisome features” were defined, with surgical resection recommended for high-risk stigmata, namely obstructive jaundice in a patient with a cystic lesion in the head of the pancreas, enhancing mural nodules and main pancreatic duct diameter ≥ 10 mm.
The above Japanese guidelines are complemented by the European and American guidelines as important decision-making aids [6,7,10]. In the latest update of the European guidelines of 2018, recommendations include a conservative approach for asymptomatic IPMN measuring <40 mm without an enhancing nodule . Relative indications for surgery in IPMN include a main pancreatic duct (MPD) diameter between 5 and 9.9 mm or a cyst diameter ≥40 mm. Absolute indications for surgery in IPMN, due to the high-risk of malignant transformation, include jaundice, an enhancing mural nodule >5 mm, and MPD diameter >10 mm. Lifelong follow-up of IPMN is recommended in patients who are fit for surgery.
1. Ohhashi K. Four cases of mucous secreting pancreatic cancer. Prog Digest Endosc. 1982;20:348–351.
2. Klöppel GSE, Longnecker DS, et al., editors. Histologic Typing of Tumours of the Exocrine Pancreas. 2. New York, NY: Springer-Verlag; 1996.
3. Bosman FTCF, Hruban RH, Theise ND, editors. WHO Classification of Tumours of the Digestive System. Lyon, France: IARC Press; 2010. Tumours of the Pancreas.
4.Tanaka M, Fernández-del Castillo C, Adsay V, Chari S, Falconi M, Jang J-Y, et al. International consensus guidelines 2012 for the management of IPMN and MCN of the pancreas. Pancreatol Off J Int Assoc Pancreatol IAP Al. 2012;12:183–97.
5. Tanaka M, Fernández-Del Castillo C, Kamisawa T, Jang JY, Levy P, Ohtsuka T, et al. Revisions of international consensus Fukuoka guidelines for the management of IPMN of the pancreas. Pancreatol Off J Int Assoc Pancreatol IAP Al. 2017;17:738–53
6. Vege SS, Ziring B, Jain R, Moayyedi P. Clinical Guidelines Committee, American Gastroenterology Association. American gastroenterological association institute guideline on the diagnosis and management of asymptomatic neoplastic pancreatic cysts. Gastroenterology. 2015;148:819–22
7. Elta GH, Enestvedt BK, Sauer BG, Lennon AM. ACG clinical guideline: diagnosis and Management of Pancreatic Cysts. Am J Gastroenterol. 2018;113:464–79.
8. Furukawa T, Klöppel G, Volkan Adsay N, et al. Classification of types of intraductal papillary-mucinous neoplasm of the pancreas: a consensus study. Virchows Archiv. 2005;447:794–799.
9. Tanaka M, Chari S, Adsay V, et al. International consensus guidelines for management of intraductal papillary mucinous neoplasms and mucinous cystic neoplasms of the pancreas. Pancreatology. 2006;6:17–32.
10. European Study Group on Cystic Tumours of the Pancreas. European evidence-based guidelines on pancreatic cystic neoplasms. Gut. 2018;67:789–804.
By Monzur Ahmed
A 40 year old man was enjoying an evening meal of steak with his wife. Whilst eating his well done steak, he developed “choking” and then dysphagia. His wife slapped his back but to no avail. Initially, the dysphagia was at the level of the high sternum and then the mid-sternum. The dysphagia persisted such that he was unable to keep down water. He presented to hospital several hours later. A lateral cervical X-Ray and chest X-Ray were unremarkable. He did not respond to iv Glucagon. Hence an urgent endoscopy was performed, some 16 hours after the onset of symptoms (see video)…
First described in 1963 by Norton et al, "the steakhouse syndrome" is a condition in which food impaction of the oesophagus occurs after eating a piece of food, especially a meat bolus, without adequate chewing . Most food bolus impactions resolve without intervention, either by moving forward to the stomach or by the patient regurgitating the ingested contents. When symptoms of obstruction persist and/or are accompanied by substantial chest discomfort, patients will seek medical attention.
In the classic presentation of the steakhouse syndrome, not surprisingly, impactions occur more often when patients are eating meat and generally when they do not chew their food sufficiently. Contributing conditions could be poor dentation, ill-fitting dentures, the use of alcohol, or a predisposition to eat too quickly . The most commonly impacted foods are beef, chicken, pork, and al dente-cooked vegetables.
Patients with a food bolus impaction that persists to the extent that they present in the emergency department should receive a chest radiograph to rule out evidence of perforation or a radiopaque object in the oesophagus. Once a foreign object is ruled out, endoscopy should be considered. Several factors should be considered regarding the timing of the endoscopy. The status of the patient's airway and ventilation needs to be evaluated to ensure adequate control and assess the risk of aspiration. Patients experiencing excessive sialorrhea who are unable to handle their secretions have an indication for urgent or emergent endoscopy. Further, it is known that food bolus impactions that persist more than 12–24 hours confer more risk for serious complications, including oesophageal perforations.
Small doses of glucagon administered intravenously can be given to patients who are under evaluation for management of a food bolus impaction. This may help to relax the oesophagus and allow spontaneous passage. However, it should not delay definitive investigation and management by endoscopy. Placement of an oesophageal overtube or endotracheal intubation should be considered when copious oesophageal contents are encountered to minimise risk of aspiration. Regarding food bolus impaction, the ESGE guidelines  recommend:
-Oesophageal foreign objects and food bolus impacted in the oesophagus should be removed within 24 hours because delay decreases the likelihood of successful removal and increases the risk of complications. The risk for major complications (i. e., perforation with or without mediastinitis, retropharyngeal abscess and aortoesophageal fistula) increases 14.1 times with foreign bodies impacted for more than 24 hours in the oesophagus.
-ESGE suggests treatment of food bolus impaction in the oesophagus by gently pushing the bolus into the stomach. If this procedure is not successful, retrieval should be considered (weak recommendation, low quality evidence).
-The effectiveness of medical treatment of oesophageal food bolus impaction is debated. It is therefore recommended, that medical treatment should not delay endoscopy (strong recommendation, low quality evidence).
- In cases of food bolus impaction, ESGE recommends a diagnostic work-up for potential underlying disease, including histological evaluation, in addition to therapeutic endoscopy (strong recommendation, low quality evidence).
An underlying oesophageal pathology is found in more than 75 % of patients presenting with food bolus impaction. The most frequently associated abnormalities are oesophageal (mainly peptic) strictures (more than 50 %) and eosinophilic oesophagitis (about 40 %). Less frequently, oesophageal cancer or oesophageal motility disorders, such as achalasia, diffuse oesophageal spasm, and nutcracker oesophagus, are causes of food bolus impaction. Lack of appropriate follow-up for patients has been shown to be a predictor for recurrent food impactions. Therefore, in all patients a diagnostic work-up after extraction of foreign bodies is recommended to detect any underlying disease.
1.Norton RA, King GD. “Steakhouse Syndrome“: The Symptomatic Lower Esophageal Ring. Lahey Clin Found Bull. 1963;13:55–59. https://pubmed.ncbi.nlm.nih.gov/14078124/
2.Enomoto S, Nakazawa K, Ueda K et al, World J Gastrointest Endosc. 2011 May 16; 3(5): 101–104. Steakhouse syndrome causing large esophageal ulcer and stenosis
3.Birk M, Bauerfeind P, Deprez P et al , ESGE Practice Guideline, 2016 May;48(5):489-96.Removal of foreign bodies in the upper gastrointestinal tract in adults: European Society of Gastrointestinal Endoscopy (ESGE) Clinical Guideline
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)…
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
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.