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mechanical obstruction that might require Postgrad Med J: first published as 10.1136/pgmj.2002.004127a on 5 January 2004. Downloaded from SELF ASSESSMENT ANSWERS urgent enterolithotomy.4 The recurrence rate of gall stone is less than 2%.2 Abdominal pain and vomiting Clinical features Usually abdominal pain is a prominent Final diagnosis in an elderly diabetic woman symptom, and associated illnesses such as diabetes and cardiovascular disease are com- Gall stone ileus. Q1: What abnormalities are seen on mon. It causes signs of small bowel obstruc- the plain film (fig 1; see p 709) and References tion like nausea, vomiting, abdominal 1 Foss HL, Summers JD. Intestinal obstruction from computed tomogram (fig 2; see p 709) distension, and absence of bowel sounds in gall stones. Ann Surg 1942;115:721–35. of the abdomen? cases of complete obstruction. Signs of 2 Reisner RM, Cohen JR. ileus: a review Plain film shows air in the biliary tract and intestinal obstruction are seen mostly if the of 1001 reported cases. Am Surg gall bladder, dilated loops of small intestine, gall stone impacts at the ileocaecal junction. 1994;60:441–6. and multiple air-fluid levels in small bowel However, the characteristic features of intest- 3 Clavien PA, Richon J, Burgan S, et al. Gallstone loops. The computed tomogram shows large inal obstruction are found in only 50% to 70% ileus. Br J Surg 1990;77:737–42. of patients.23This may be because as the gall 4 Williams IM, Hughes ODM, Hicks E, et al. Gall gall stone impacted in jejunum and multiple stone ileus following multiple endoscopic air-fluid levels in small intestine. stone ‘‘tumbles’’ through the gastrointestinal retrograde cholangiopancreatographies. J R Coll tract, it impacts and disimpacts, producing Surg Edinb 1997;42:423–4. Q2: What is the diagnosis? intermittent mechanical obstruction. 5 Deitz DM, Standage BA, Pinson PW, et al. Gall stone ileus. Improving the outcome in gallstone ileus. Am J Surg 1986;151:572–6. Q3: What other variants of this disease Investigations 6 Rodriguez-Sanjuan JC, Casado F, Fernandez MJ, The diagnosis of gall stone ileus is often are known to occur? et al. and closure versus difficult to make. enterolithotomy alone in gallstone ileus. Br J Surg The complications of cholelithiasis are shown The classic radiographic signs of gall stone 1997;84:634–7. in table 1. Gall stones can get impacted at ileus on abdominal plain film are pneumobi- various sites, the commonest being the lia, mechanical small , and ileocaecal junction. The other described sites the presence of a new stone or changed of gall stone impaction with in the bowel are position of a previously identified stone, Proptosis in an asthmatic the jejunum, stomach, colon, duodenum, and known as Rigler’s triad.1 patient pylorus (Bouveret’s syndrome). Ultrasound reveals diseased gall bladder, GALL STONE ILEUS whether there is gas in it or in the bile ducts Q1: What is the diagnosis of this or both, and fluid filled bowels that can be respiratory condition? Gall stone ileus is a mechanical bowel followed to the stone in the intestine. The obstruction caused by passage of gall stones presence of stones in the gall bladder will The diagnosis in this case is allergic broncho- from the biliary system through a biliary- modify the planned operative procedure in pulmonary aspergillosis (ABPA). ABPA occurs enteric fistula with impaction within lumen the treatment of gall stone ileus. in 6%–20% of individuals with asthma and of the bowel. approximately 10% of individuals with cystic The characteristic features of Rigler’s triad 1 It is an uncommon complication of biliary are easily identified on computed tomogra- fibrosis. It is characterised by repeated stone disease, accounting for only 2% of all phy even if abdominal plain film is subtle. episodes of exacerbations interspersed with cases of intestinal obstruction. Gall stone Even if abdominal radiography reveals the periods of remissions. Diagnostic criteria ileus is, however, more common in the characteristic signs of small bowel obstruc- include (1) asthma, (2) fleeting pulmonary elderly and accounts for approximately 25% tion, computed tomography is useful for opacities on chest radiographs, (3) positive of all cases of intestinal obstruction in skin tests for Aspergillus fumigatus, (4) periph- 1 excluding complications (for example, stran- patients over 65 years of age. gulation). When unexplained bowel obstruc- eral blood eosinophilia, (5) precipitating The gall stone that causes ileus is usually tion is present, particularly in the elderly, the antibodies to A fumigatus, (6) raised serum more than 2.5 cm in diameter. Multiple IgE levels, and (7) central bronchiectasis.2 12 early use of computed tomography is strongly stones are present in 3%–15% of cases. recommended. Other minor criteria include expectoration Gall stones usually enter the intestinal Endoscopy has been the main diagnostic of golden brown sputum plugs, positive http://pmj.bmj.com/ lumen through a cholecystoenteric fistula, procedure for Bouveret’s syndrome. The sputum culture for aspergillus species and and 68% of these are between the gall bladder diagnosis was made endoscopically in more late (Arthus-type) skin reactivity to A and the duodenum. A history of prior biliary than 90% of the cases.4 fumigatus. tract disease is present in almost 50%–60% of If the disease is not diagnosed early and patients with gall stone ileus.2 treated adequately, lung damage continues The most frequent site of stone impaction Treatment to progress silently leading to fibrosis in is the ileum (.60% of cases), as it is the Gall stone ileus is a serious geriatric surgical the upper lobes.3 The upper lobe fibrosis of narrowest part of the bowel. Other sites of emergency. It has a high morbidity (15%– ABPA can mimic pulmonary tuberculosis obstruction are the jejunum (16%), stomach 18%) and mortality (17%). and patients are often unnecessarily on October 1, 2021 by guest. Protected copyright. (14%), colon (4%), and duodenum (3%). The management of gall stone ileus is treated with antituberculosis therapy as in Gastric outlet obstruction, or Bouveret’s controversial. The choice is between perform- our case. syndrome, occurs when the gall stone lodges ing simple enterolithotomy or a single stage in the duodenal bulb (1%).1 procedure involving enterolithotomy, chole- Q2: Which respiratory diseases have cystectomy, and fistula closure. Current coexistent sinus and orbital reports favour enterolithotomy only, with involvement and what was the cause in definitive biliary surgery performed later if Table 1 Complications of this patient? cholelithiasis symptoms persist. Advocates of the combined procedure contend that it prevents recurrent Allergic and granulomatous diseases com- gall stone ileus, cholangitis, and gall bladder monly involve the lung, sinuses, and the orbit Complication Percent 4 carcinoma complications that occur in nearly (box 1). The diagnosis in this condition was 70–80 one third of patients who undergo entero- allergic fungal sinusitis with orbital aspergil- Acute 10 lithotomy only. Simple enterolithotomy car- losis in a patient with allergic bronchopul- Emphysematous cholecystitis ,1 ries a mortality of 11.7% compared with monary aspergillosis. Mirizzi’s syndrome ,1 16.9% for one stage procedure. The most Hydrops of the gall bladder ,1 common source of operative morbidity is Q3: What is the significance of C-ANCA Small bowel obstruction 1 wound infection, occurring in 30%–40% of in chronic lower respiratory tract (gall stone ileus) cases.56 infections? , Perforation of gall bladder 1 In duodenal stone impaction extracorpor- Positive C-ANCA is characteristic of Acute biliary pancreatitis 12 eal shock wave lithotripsy is successful in Wegener’s granulomatosis. However, in a Acute suppurative/obstructive – cholangitis fragmenting the stone. Endoscopic stone study done by Ohno et al raised myeloper- removal is especially indicated in poor risk oxidase ANCA and bactericidal/permeability patients. A dislodged impacted stone can increasing protein ANCA levels were found migrate distally and cause small bowel in patients with chronic lower respiratory

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controlling local inflammation. It is also degenerative changes are seen at the C5–C6 Postgrad Med J: first published as 10.1136/pgmj.2002.004127a on 5 January 2004. Downloaded from Box 1: Allergic and granulomatous recommended that systemic antifungals be intervertebral disc space. diseases used to prevent progression to invasive forms of fungal sinusitis. Intravenous amphoteri- Q2: What abnormalities are seen on cin-B has been found to be more beneficial the MRI scan (p 711)? than the less toxic agents of the azoles group N Allergic group: Sagittal T2 weighted MRI scan of the cervical in view of the poor in vitro activity of these spine shows narrowing of the bony spinal – ABPA. agents.6 canal at the arch of C1 vertebra. This in turn N In conclusion, patients with ABPA should Granulomatous group: gives rise to generalised narrowing and be evaluated for the presence of coexistent distortion of the spinal cord at the cranio- – Classical Wegener’s granulomato- allergic fungal sinusitis and orbital aspergil- medullary junction. There is some signal sis. losis to avoid further complications. change within the cord itself at this level. – Necrotising sarcoid granulomas. Final diagnosis – Behc¸ets disease. Q3: What is the likely diagnosis? Allergic fungal sinusitis with orbital aspergil- – Midline granulomas. losis in a patient with allergic bronchopul- Anterior atlantoaxial subluxation involving supra-axial spine. – Polyarteritis nodosa. monary aspergillosis. – Churg-Strauss syndrome. Q4: How can this condition be treated? – Relapsing polychondritis. References Atlantoaxial subluxation, when symptomatic 1 Slavin R, Stanevyak D, Lonigro A, et al. Allergic – Benign lymphocytic angitis. or is severe, can be treated by posterior bronchopulmonary aspergillosis: a North cervical fusion with or without stabilisation – Nasopharyngeal lymphomas with American rarity. Am J Med 1969;47:306–8. 2 Rosenberg M, Patterson R, Mintzer R, et al. at this level. Arthrodesis with autologous pulmonary Hodgkin’s disease. Clinical and immunological criteria for the corticocancellous bone graft is augmented by diagnosis of allergic bronchopulmonary various stabilisation techniques. aspergillosis. Ann Intern Med 1977;86:405–14. 3 Shah A, Panjabi C. Allergic bronchopulmonary Discussion infection.5 Proteinase-3 C-ANCA was also aspergillosis: a review of a disease with positive in a single case each of the 21 diffuse worldwide distribution. J Asthma Rheumatoid arthritis is a chronic, inflamma- panbronchiolitis and 16 bronchiectasis 2002;39:273–89. tory disorder characterised by symmetric patients included in the study. The raised C- 4 Seaton A, Seaton D, Leitch AG. Pulmonary polyarthritis involving multiple joints. The ANCA levels found in our patient can thus be angiitis and granulomatosis. Crofton and cervical spine can be involved in 17%–86% of Douglas’s respiratory diseases. 4th Ed. New patients with rheumatoid arthritis. It can explained by the fact that he had underlying Delhi: Oxford University Press, 1989:748–60. chronic respiratory tract infection. involve the supra-axial or subaxial spine with 5 Ohno S, Saku N, Sugiyma Y. ANCA the craniovertebral junction being most often (antineutrophil cytoplasmic autoantibody) in affected.1 Cervical instability is the most chronic lower respiratory tract infection. Chest serious and potentially lethal manifestation Discussion 2001;120(suppl):4. of rheumatoid arthritis. Also, a patient with Allergic fungal sinusitis is a saprophytic 6 McClay John E, Marple Bradley. Allergic fungal sinusitis. Accessed 28 December 2001 rheumatoid arthritis and an unstable cervical fungal growth involving one or more para- (www.emedicine.com/ent /topic 510.htm); spine will present a major anaesthetic pro- nasal sinuses. Allergic fungal sinusitis volume 2, number 12. blem if not stabilised. Atlantoaxial subluxa- appears to be more prevalent in the tempe- 7 Shah A, Panchal N, Agarwal AK. Concomitant tion can be anterior, posterior, or lateral with rate regions and around areas of high relative allergic bronchopulmonary aspergillosis and the anterior type being most common.2 It is humidity with equal preponderance in both allergic aspergillus sinusitis a review of an usually the result of the destruction of joints, sexes.6 It can manifest itself either with signs uncommon association. Clin Exp Allergy 2001;31:1896. ligaments, and bone caused by erosive and symptoms of nasal obstruction and synovitis involving atlantoaxial, atlanto- allergic rhinitis or purulent rhinorrhoea, odontoid, and atlanto-occipital joints.3 The headache, and epistaxis. Orbital aspergillosis inflammatory destruction via synovitis of the occurs as an extension of allergic fungal Rheumatoid arthritis and neck transverse ligament leads to anterior sub- http://pmj.bmj.com/ sinusitis into the adjacent spaces, which may luxation of the atlas on the axis. Erosion of have a dramatic clinical presentation such as pain the odontoid process frequently coincides diplopia or visual loss due to compression of with this process. Protrusion of the odontoid the ophthalmic nerve. The gross facial dis- Q1: What are the features seen in the process posteriorly into the spinal canal can figurement and orbital abnormalities consist- lateral radiographs of the cervical result in clinical symptoms or signs such as ing of proptosis and telecanthus are more spine (p 711)? suboccipital pain or neuralgia and myelo- often seen in children than in adults. The Lateral radiographs of the cervical spine show pathy. Further progression of the disease diagnosis of allergic fungal sinusitis is princi- increased atlas-dens interval anteriorly and involves loss of alar and capsular ligament pally based on pathological findings in the reduced posterior atlas-dens interval in integrity and leads to further erosion of the on October 1, 2021 by guest. Protected copyright. specimens obtained from the paranasal flexion views but normal intervals in the dens.1 If the inflammation persists in the sinuses. These findings are similar to those 6 extension views. The spinal canal shows atlantoaxial joints, their cartilage and bone seen in the mucoid impaction of ABPA. corresponding reverse changes. In addition, structures will be eroded, the joint spaces will Concomitant occurrence of ABPA and narrow down, and the atlas falls down allergic fungal sinusitis is well recognised 7 around the axis (atlantoaxial impaction). with substantial data available on the same. When the atlantoaxial joint surfaces are It has been postulated that the hallmark of Box 1: Groups of C1–C2 this saprophytic colonisation is an identical involvement in rheumatoid pathophysiology occurring in ABPA, allergic arthritis based on the radiological fungal sinusitis, and orbital aspergillosis. features Box 2: Ranawat scale used for There are several case reports of coexisting neurological assessment in ABPA with allergic fungal sinusitis7 and rheumatoid arthritis allergic fungal sinusitis with orbital aspergil- N Group 1 (46%): severe joint space losis.6 The presence of ABPA and allergic narrowing and subchondral sclerosis fungal sinusitis in the same patient is often with lateral mass collapse. N Grade 1: pain, no neurological deficit. overlooked when either is being treated by N Group 2 (44%): joint space narrowing N Grade 2: subjective weakness, hyper- two different specialties. reflexia, dysaesthsia. The management of allergic fungal sinusi- and subchondral sclerosis occurs without tis with orbital involvement warrants an lateral mass collapse. N Grade 3a: objective weaknesss, long immediate surgical removal of the fungal N Group 3 (10%): lateral subluxation with- tract signs, ambulatory. growth including resection of any recurring out joint space narrowing or subchon- N Grade 3b: objective weaknesss, long disease. Medical management includes the dral sclerosis. tract signs, non-ambulatory. use of corticosteroids as in ABPA, while topical corticosteroids may be effective in

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eroded they become rough and the instability The preoperative PADI value is a more recommended in cases with extensive sub- Postgrad Med J: first published as 10.1136/pgmj.2002.004127a on 5 January 2004. Downloaded from decreases (box 1).24 reliable indicator of the development and luxation or gross instability even without Anterior atlantoaxial subluxation implies a severity of paralysis. Moreover, PADI is a neurological deficit to avoid development of widening of the joint space between the better predictor than AADI of whether myelopathy. anterior arch of the atlas and the odontoid postoperative neurological recovery is The commonest surgical procedure is pos- process. Radiographic diagnostic criteria have expected. This is because PADI correlates terior C1–C2 fusion and wiring5 with addi- been developed as descriptors of existence closely with the space available for the spinal tional halovest stabilisation or C1–C2 and advancement of atlantoaxial subluxa- cord.15 transarticular screw fixation. Atlantoaxial tion. Two of the most commonly used criteria Patient controlled flexion and extension subluxation that is not reducible may require are anterior and posterior atlantoaxial inter- views are evaluated to determine the AADI removal of the posterior arch of atlas for cord vals (AADI and PADI respectively). The and PADI. Instability is present with a 3 mm decompression followed by occiput to axis determination of these intervals involves of AADI difference in flexion and extension fusion. An AADI of .7–10 mm or a posterior constructing a line that connects the cen- views, although radiographic instability in space (PADI) of less than 13 mm is a relative troids of the anterior and posterior rings of rheumatoid arthritis is common and is not an contraindication to surgery in other areas and the atlas on a lateral plain radiograph at indication for surgery. Cervical spine surgery the spine should be stabilised first. maximal flexion. The AADI is the distance is seldom indicated solely by radiographic along this line that measures the difference findings. These patients should be examined Final diagnosis between the posterior surface of the anterior by with MRI to get more information Anterior atlantoaxial subluxation of the arch of the atlas and the anterior surface of about possible spinal cord compression and cervical spine in rheumatoid arthritis. the dens. The PADI, which is complementary also to visualise other soft tissues, such as to AADI, is the distance between posterior pannus, before a final decision on surgical surface of dens and anterior surface of treatment.2 References posterior arch of atlas. The normal AADI is The assessment of cervical disease in 1 Puttlitz CM, Goel VK, Clark CR, et al. 1 to 2 mm in adults (sometimes referred to as patients with rheumatoid arthritis can be Biomechanical rationale for the pathology of atlantodens interval, ADI).3 In children, the difficult due to coexisting systemic illnesses, rheumatoid arthritis in the craniovertebral ADI may be as much as 4.5 mm and can neurological abnormalities, and rheumatoid junction. Spine 2000;25:1607–16. show an increase of 0.5 mm in flexion. polyarthropathy.5 The Ranawat scale is often 2 Neva MH, Kaarela K, Kauppi M. Prevalence of radiological changes in the cervical spine—a Atlantoaxial subluxation is defined as the used to grade rheumatoid myelopathy cross sectional study after 20 years from ( 1 AADI .3 mm or PADI 14 mm. because its coarse grading structure accepts presentation of rheumatoid arthritis. J Rheumatol Atlantoaxial subluxation of 9 mm reduces the major musculoskeletal disability in rheu- 2000;27:90–3. the area of the spinal canal (space available matoid arthritis (see box 2). 3 Tucker SK, Taylor BA. Spinal canal capacity in for the cord) to 60%. Theoretically, full Rheumatoid cervical spine changes are simulated displacements of the atlanto-axial rotation of 47˚ would further reduce the usually treated conservatively. The indica- segment—a skeletal study. J Bone Joint Surg Br spinal canal to 21% which must cause tions for surgery are well established in the 1998;80:1073–8. compression of the cord (as cord normally symptomatic patient. Severe pain may be 4 Halla JT, Hardin Jr JG. The spectrum of atlantoaxial facet joint involvement in rheumatoid occupies 27 to 30% of the spinal canal). The alleviated and neurological deterioration arthritis. Arthritis Rheum 1990;33:325–9. rheumatoid pannus also contributes to relieved. Controversy still exists, however, 5 Eyers KS, Gray DH, Robertson P. Posterior medullary compression in cases of atlanto- over the role of prophylactic procedures in surgical treatment for the rheumatoid cervical axial subluxation.3 asymptomatic patients.5 Surgery has been spine. Br J Rheumatol 1998;37:756–9.

ECHO ...... QRS duration: a simple marker for predicting cardiac mortality in ICD patients with heart failure http://pmj.bmj.com/ L Bode-Schnurbus, D Bo¨cker, M Block, R Gradaus, A Heinecke, G Breithardt, M Borggrefe Background: Patients resuscitated from ventricular tachyarrhythmias benefit from implantable cardioverter-defibrillators (ICDs) as opposed to medical treatment. Patients with increased QRS duration receiving an ICD in the presence of heart failure are at greatest risk of cardiac death and benefit most from ICD therapy.

Objective: To determine whether an increased QRS duration predicts cardiac mortalityin on October 1, 2021 by guest. Protected copyright. Please visit the Postgraduate ICD recipients. Medical Design: Consecutive patients with heart failure in New York Heart Association functional Journal class III were grouped according to QRS duration (, 150 ms, n = 139, group 1; v >150 ms, website [www. n = 26, group 2) and followed up for (mean (SD)) 23 (20) months. postgradmedj. Patients: 165 patients were studied (80% men, 20% women); 73% had coronary artery com] for a link to the full text disease and 18% had dilated cardiomyopathy. Their mean age was 62 (10) years and mean of this article. ejection fraction (EF) was 33 (14)%. They presented either with ventricular tachycardia (VT) or ventricular fibrillation (VF). Main outcome measures: Overall and cardiac mortality; recurrence rates of VT, fast VT, or VF. Results: Mean left ventricular EF did not differ between group 1 (33 (13)%) and group 2 (31 (15)%). Forty patients died (34 cardiac deaths). There was no difference in survival between patients with EF . 35% and #35%. Cardiac mortality was significantly higher in group 2 than in group 1 (31.3% at 12 months and 46.6% at 24 months, v 9.5% at 12 months and 18.2% at 24 months, respectively; p = 0.04). The recurrence rate of VT was similar in both groups. Conclusions: Within subgroups at highest risk of cardiac death, QRS duration—a simple non-invasive index—predicts outcome in ICD recipients in the presence of heart failure. m Heart 2003;89:1157–1162.

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