Ligate the cephalic vein proximal to the origin of the antecubital vein to allow basilic vein drainage. Complications Graft stenosis can be treated by angioplasty to prevent thrombosis and eventual graft occlusion. It can be avoided by rotating the sites of venepuncture during dialysis Infection due to frequent puncture can lead to vascular access loss. Back table preparation prepare iced container and remove kidney from packaging in aseptic conditions. Fill the bladder with warm saline ± methylene blue before cystostomy and ureteric anastomosis for easy identification and to avoid opening the peritoneum. Follow the standard reimplantation Lich­Gregoir technique (extravesical neocystostomy) using a ureteric stent. Postoperative care Start immunosuppression (this may be started in the preoperative period for recipients of live donor grafts). In cases of ureteric necrosis, a Boari flap or ureteroureterostomy may be required. Tips and tricks Cold ischaemia time should be minimized for donors after cardiac death (cadaveric donors exposed to warm ischaemia)-best results are achieved with times less than 12h. Anatomy the left kidney has a longer renal vein and is potentially easier to remove if there is one artery. Preoperative preparation Confirm cross-match suitability with recipient and laterality of procedure. Position and theatre setup Dependent on kidney side, up to five working ports may be needed with an 8cm pfannensteil incision premarked on the side to be extracted. Check blood group compatibility (no requirement to wait for crossmatch with donor, cf. Position and theatre set-up Back table preparation Check the paperwork enclosed with the organs for donor details, organ perfusion times, reported damages, pathology, and reported vascular anatomy. Procedure hepatectomy proceeds by identifying and dividing the bile duct, ha, and pV in sequence close to the liver. Closure ensure haemostasis, place large-bore drains around the liver and next to the biliary anastomosis. Day 1 aLt/aSt (alanine/aspartate transaminase) is a marker of ischaemia reperfusion injury. Complications hepatic artery thrombosis (2­5%) presents with failure of pt and lactate to normalize post-implantation, graft dysfunction in the first week, and bile leak in the first 2­3 weeks. Full inspection of organs to ensure suitability for transplantation and absence of pathology. Postoperative care organs flushed to ensure microcirculation clear, anatomy and vessel integrity confirmed. Complications Common injuries include liver capsular tears on mobilization, cuts to the arterial, venous patches, division of accessory vessels (polar renal arteries, accessory left and right hepatic arteries), and parenchymal injury (pancreas). It is a double-layered fold of smooth muscle tissue, blood vessels, neurones, skin, and mucous membrane that covers the glans penis and protects the urinary meatus. The external meatus is a highly innervated mucocutaneous zone of the penis near the tip of the foreskin. The smegma is a whitish pollution of the glans and prepuce, and arises due to bacterial colonization of the desquamated epithelium. The nerve supply to the penis is important to understand as circumcision can be performed with a local anaesthetic penile ring block. Preoperative preparation Patient consented for circumcision; discuss the following specific side-effects (marked with an asterisk throughout this chapter) on the basis of the British association of Urological Surgeons procedure specific consent form*: Common side-effects (>1 in 10): swelling of the penis lasting several days. In certain situations when there is a severe degree of phimosis, the foreskin cannot be retracted to make the inner incision. In this situation a dorsal slit incision is made through the outer and inner skin layers at the same time in the midline. Tips and tricks Ensure no adrenaline in local anaesthetic agent for penile ring block. The venous drainage occurs through the Pampiniform plexus, which eventually becomes reduced to a single vein known as the testicular vein. There is a small risk of injury to the testicular artery during surgery on the epididymis. Preoperative preparation Consent patient*; discuss the following complications: Common (>1 in 10): swelling of the testis lasting several days and seepage of yellowish discharge from the surgical wound. Procedure make either a midline raphe incision or a transverse incision in the hemi-scrotum. The bladder is located in the anterior pelvis and is surrounded by extraperitoneal fat and connective tissue. The surrounding structures of the urinary bladder include the peritoneum anteriorly at the dome of the bladder and structures formed by the reflection of the pelvic fascia and true ligaments of the pelvis. The advantage of a flexible cystoscopy is that the procedure can be performed using local anaesthesia. The commonest local anaesthesia used for a flexible cystoscopy is Instillagel, which contains lidocaine hydrochloride (local anaesthetic) 2. The flexible cystoscope can be manipulated at the tip, which makes visualization of the bladder at different angles possible. Rigid cystoscopy has the advantage of better views during the procedure due to superior optics and irrigation volume. Instruments such as biopsy forceps and injection devices for intravesicle injections are more easily passed via a rigid cystoscope. Procedure Clean and drape patient When a patient is undergoing a flexible cystoscopy, the procedure can be performed using local anaesthesia. In patients undergoing a rigid cystoscopy, a general/spinal anaesthetic is required. During the procedure an irrigant is used to fill the bladder and this allows superior views in comparison to the flexible cystoscope due to the option of continuous irrigation. The advantage of a rigid cystoscope is the versatility of additional treatments that can be performed, which includes biopsy, diathermy, and insertion of stents. When a biopsy of the bladder needs to be performed, this can be done with either a flexible cystoscope or rigid cystoscope. The advantage of flexible cystoscopy biopsies is that the procedure can be performed without a general anaesthetic. The disadvantage is the small biopsies performed due to the biopsy forceps and the risk of an inability to control haemostasis in the event of bleeding, as the irrigation is not on a continuous flow. The advantage of rigid cystoscopy and biopsy is that the biopsy volume is larger and haemostasis is better controlled due to continuous irrigation systems. The commonest cause of acute ureteric obstruction is renal calculi and chronic obstruction is benign/malignant pelvic or retroperitoneal pathology. Indications for ureteric stent insertion Urinary sepsis secondary to acute ureteric obstruction. There are different types of hydroceles: primary hydrocele, secondary hydrocele, infantile hydrocele, congenital hydrocele, and encysted hydrocele of the cord. Normally the processus vaginalis becomes obliterated along the entire length apart from where the process vaginalis surrounds the testis itself. When the central part of the processus vaginalis remains patent, fluid secreted by the peritoneum accumulates and forms a hydrocele around the testis. Preoperative preparation Patient is consented for hydrocele repair after discussing the following specific side-effects on the basis of the British association of Urological Surgeons procedure specific consent form*: Common (>1 in 10): swelling of the scrotum lasting several days. Possible infection of the incision or the testis requiring further treatment with antibiotics or surgical drainage. The best method to separate the hydrocele sac from the surrounding Dartos is via blunt-finger dissection, ensuring haemostasis as there can be prominent veins in this layer. During opening of the hydrocele sac care must be taken not to damage the spermatic cord posteriorly. It is important to ensure that the eversion suture is not made too tight in order to prevent strangulation of the spermatic cord. Commence the eversion from the free edge of the tuniva vaginalis with 3-0 absorbable sutures at 1cm intervals. Complications Occasional (between 1 in 10 and 1 in 50) Blood collection around testis, which resolves slowly or requires surgical removal. Tips and tricks Place a drain postoperatively in large hydrocele to prevent the risk of a postoperative haematoma. In patients with previous lower abdominal surgery/scars there is a risk of the bowel being adhered to the abdominal wall and bladder, risking injury to both these structures during catheter insertion. Patient is consented for suprapubic catheter insertion after discussing the following complications*: Common (>1 in 10): temporary mild burning or bleeding during urination. The anatomical location of the puncture site is two to three finger-breadths above the pubic symphysis. Testicular torsion results from a twist of the spermatic cord, resulting in strangulation of the blood supply of the testis, including the epididymis. It is hence important to perform an orchidopexy of the opposite side in order to prevent a further torsion. The attachment between the tunica vaginalis and scrotum is mal-developed whereupon there is an incomplete fixation of the gubernaculum to the scrotal wall, resulting in the entire testis and tunica vaginalis twisting in a vertical axis of the entire spermatic cord. Complications of a missed torsion include antisperm antibodies, which can lead to delayed infertility. Preoperative preparation Consent for torsion of testis after discussing the following complications*: Common (>1 in 10): it may be necessary to remove the affected testis if it is too damaged to recover. Procedure the patient normally has an emergency general anaesthetic following rapid induction. Do the fixation with 3-0 non-absorbable sutures at medial, lateral, and infero-anterior positions. In the event of there being a question as to whether the testis is viable or not, open the tunica albugenia to assess if the seminiferous tubules are viable. It is the most commonly used modality for assessing the prostate and is the commonest method used to biopsy the prostate. The prostate is an extraperitoneal structure, lying anterior to the rectum and at the bladder neck. The prostate encircles the urethra and it empties its secretions into the urethra. It comprises of glands that are surrounded by smooth muscle and connective tissue. The prostate has three glandular regions: the central, the peripheral, and the transition zones. Thus, for clinical purposes the important regions are the peripheral and transition zones. The options are to perform the procedure with local anaesthetic, sedation, or in some cases general anaesthetic. Regular interval biopsies on patients on active surveillance for low-risk prostate cancer (biopsies). Preoperative preparation Consent after discussing the following complications*: Common (>1 in 10): patients may experience blood in urine, blood in the semen for up to 6 weeks post biopsy, blood in stools, urinary infection (10% risk), discomfort from the prostate due to bruising, haemorrhage (bleeding) causing inability to pass urine (2% risk). Procedure Place patient in lateral position and perform a digital rectal examination. Some surgeons also place either a paracetamol or diclofenac suppository at the end of the procedure. Tips and tricks Ensure patient is aware that negative transrectal biopsy does not exclude prostate cancer. Vasectomy involves surgical division of both vas deferens to allow permanent contraception. Preoperative preparation Take a thorough history from the patient and ensure that both the patient and partner are aware of the operative methodology and potential side-effects of the procedure, as there are significant medico-legal issues. Once made, dissect each vas deferens and isolate it from the spermatic cord and bring it to the level of the skin. Diathermise the ends of the vas deferens with bipolar diathermy and suture ligate with a 3-0 absorbable suture. Carry out haemostasis meticulously Close the dartos and skin in layers with a 3-0 absorbable suture. Postoperative care Patients are instructed to continue with contraceptives methods till two consecutive semen analyses are negative. The commonest aetiology of scrotal abscess is an infected hair follicle or infections on the scrotum related to either scrotal lacerations or postoperative wound infections. Deeper abscesses that form within the scrotum are normally secondary to progressive acute epididymitis. The infection produces ischaemia and necrosis of the tunica albugnia and surrounding tissue. Very rarely a perforated appendix can lead to pus within the scrotum due to a patent processes vaginalis. Preoperative preparation Consent highlighting the risks of bleeding, infection, wound dehiscence, need for second look, orchidectomy, and drain placement. Procedure the options of incision are either transverse or vertical in the hemiscrotum. Preoperative preparation the decision to proceed with a radical inguinal orchiectomy in suspected testicular cancer is made after careful consideration of all available data, including clinical findings, imaging studies, and serum tumour markers. Permission is needed to biopsy the other testis if it is small, abnormal, or has not descended properly. Procedure make an incision on the side of the tumour-bearing testis similar to an inguinal herniorrhaphy. Complications of prosthesis insertion (extrusion from scrotum, scrotal contraction and migration, chronic pain, haematoma, and infection). The transition zone is usually the smallest of the three and occupies only 5% of the prostate volume in men younger than 30 years.

Additional information:

• Rivastigimine
• Prazosin
• Thioridazine

Hard metal interstitial lung disease: High-resolution computed tomography appearance. Beryllium sensitization and disease among long-term and short-term workers in a beryllium ceramics plant. Pulmonary brosis in aluminum oxide workers: Investigation of nine workers with pathologic examination and microanalysis in three of them. Value of beryllium lymphocyte transformation tests in chronic beryllium disease and in potentially exposed workers. Sarcoidosis diagnosed after 11 September 2001, among adults exposed to the World Trade Center disaster. Beryllium particulate exposure and disease relations in a beryllium machining plant. Machining risk of beryllium disease and sensitization with median exposures below 2 mg/m3. Risks of beryllium disease related to work processes at a metal, alloy, and oxide production plant. The anatomy of the exposures that occurred around the World Trade Center site: 9/11 and beyond. Indirect and direct gas exchange at maximum exercise in beryllium sensitization and disease. Recent chronic beryllium disease in residents Beryllium and Related Granulomatous Responses 313 surrounding a beryllium facility. Short- and long-term response to corticosteroid therapy in chronic beryllium disease. Developing effective health and safety training materials for workers in beryllium-using industries. Giant cell interstitial pneumonia in a 60-year-old female without hard metal exposure. Twodimensional analysis of elements and mononuclear cells in hard metal lung disease. Beryllium lymphocyte proliferation test surveillance identi es clinically signi cant beryllium disease. Ground-glass computed tomography pattern in chronic beryllium disease: Pathologic substratum and evolution. Pathologic spectrum and lung dust burden in giant cell interstitial pneumonia (hard metal disease/cobalt pneumonitis): Review of 100 cases. A case control etiologic study of sarcoidosis: Environmental and occupational risk factors. Ef cacy of serial medical surveillance for chronic beryllium disease in a beryllium machining plant. Interstitial lung granulomas as a possible consequence of exposure to zirconium dust. Beryllium medical surveillance at a former nuclear weapons facility during cleanup operations. Major histocompatibility locus genetic markers of beryllium sensitization and disease. Maintenance of alveolitis in patients with chronic beryllium disease by beryllium-speci c helper T cells. Secondary ion mass spectroscopy demonstrates retention of beryllium in chronic beryllium disease granulomas. Process-related risk of beryllium sensitization and disease in a copper­beryllium alloy facility. Variable response to long-term corticosteroid therapy in chronic beryllium disease. Ef cacy of a program to prevent beryllium sensitization among new employees at a copper­beryllium alloy processing facility. Beryllium and Related Granulomatous Responses 315 United States Department of Labor. Exposure and genetics increase risk of beryllium sensitisation and chronic beryllium disease in the nuclear weapons industry. Screening for beryllium disease among construction trade workers at Department of Energy nuclear sites. Establishing causation is often complex, all the more so given the multifactorial nature of many chronic conditions. Well-studied and relatively frequent entities such as chronic obstructive pulmonary disease, ischaemic heart disease and diabetes lend themselves to epidemiologic investigation, delineating the major risk factors for disease and their relative contributions to risk at the population level. Uncommon diseases present added challenges to attribution, especially those for which the underlying pathological processes are poorly elucidated and the condition is typically considered idiopathic. Due to their infrequency, very uncommon processes are dif cult to study using standard epidemiologic techniques, even using case-referent approaches. Only infrequently are potential associations for such diseases amenable to assessments that conform to Bradford­Hill criteria or other rigorous standards of causation. In particular, the links between occupational factors and uncommon respiratory conditions are often rst described in isolated case reports or limited case series. These accounts, even with their acknowledged limitations, * serve to document the potential association between a disease and an exposure scenario and disseminate that information to a wider clinical and academic community. As additional case reports appear with a similar occupational association, the initial suspicion of cause and effect can be strengthened, although this may be counterbalanced by a publication bias against the appearance of further, similar case reports due to a perceived lack of novelty in similar reports. Systematic literature reviews of multiple cases or case series and, where feasible, case-referent investigations, can serve to further support the validity of perceived causality. Animal data can also be critical, presuming that an experimental model exists for the disease process in question. The goal of this chapter is to consider relatively uncommon, generally idiopathic interstitial respiratory tract conditions in relation to potentially causative occupational exposures. In particular, we will address idiopathic pneumonias as well as certain other interstitial disease processes not typically considered as falling within the accepted canon of standard occupational lung diseases. These conditions are all related to chronic or subacute exposures that often occur over a number of years, although sometimes the duration of exposure has been Imaging boxes by Sue Copley. We also do not address chronic interstitial lung processes covered elsewhere in this text; for example, the classic pneumoconioses. In April 1992, two young women who worked at a textile factory were treated for interstitial lung disease and severe pulmonary insuf ciency at the hospital of Alcoi in the Autonomous Community of Valencia, Spain (Moya et al. The illnesses were noti ed to the local authorities, who linked them to another case involving a young woman who had worked at the same factory and who had succumbed to respiratory failure a few months before. This prompted an investigation of all printing textile factories that used similar spraying techniques in the area of Alcoi. Clinical and radiological data, together with biopsy samples from 71 employees, delineated the extent of the outbreak of organising pneumonia. The concentration ranged from 5 to 16 mg/ m3 (mean: 10 mg/m3) in Factory A and from 1 to 3 mg/ m3 (mean: 2 mg/m3) in Factory B. The Ardystil story is exceptional because the magnitude of the initial outbreak did allow for classic epidemiologic study and, unfortunately, a further outbreak under similar conditions con rmed the initial observations. Finally, experimental data later gave additional support for establishing causality. Each of these associations has been reported in an isolated case, and in several of these, the purported exposure was not well characterised. He manually transferred spice mix from sacks into the hopper and generated signi cant dust in the process. Unfortunately, the precise ingredients of the spice mix were unavailable to the authors, and they also were not permitted access to the workplace, such that the nature of the exposure could not be further characterised. In another case report, a cleaner was reported to have developed severe dyspnoea, cough and fever, requiring hospitalisation, 2 weeks after a cleaning agent spill at work that resulted in benzalkonium compound vapour inhalation. The patient worked with ortho-phenylenediamine for 6 months before developing episodes of fever, productive cough, dyspnoea and radiographic pulmonary in ltrates. Mineralogical analysis indicated the presence of titanium dioxide nanoparticles in both the paint and in a lung biopsy, and this was posited to be the causal agent (Cheng et al. Finally, organising pneumonia temporally associated with gold dust inhalation was reported in a 47 year old restorer of religious art. He presented with a 3-week history of asthenia, myalgia, dry cough and fever, which responded to systemic corticosteroids (Ribeiro et al. Because the medicinal use of gold is associated with pneumonitis (Tomioka and King, 1997), the case of organising pneumonia linked with gold dust inhalation (likely to be a rare exposure scenario) may have the greatest biological plausibility among this group of individual reports. The diagnosis requires that known causes of interstitial lung disease (such as drug toxicity, connective tissue disease and domestic and occupational or environmental exposures) be excluded (Travis et al. They identi ed a signi cant asbestos bre burden in two of the cases (8%), leading to an alternative diagnosis of asbestosis. Extrinsic alveolitis is covered extensively elsewhere in this text and will not be considered further here. To date, there have been 13 case-referent studies yielding a substantial body of literature on this topic (Scott et al. Collectively, these studies are limited by the use of self-reported workplace exposure information, which is vulnerable to recall bias and confounding, as well as a failure to collect cumulative exposure data, a data gap that precludes investigation of dose­response relationships. Rare earth pneumoconiosis is a term applied to a homogenous category of exposure (rare earth elements), but subsumes a heterogeneous group of parenchymal pulmonary pathologies, including diffuse brosis. Exposure of photoengravers to carbon arc lamp fume features prominently in the early rare earth pneumoconiosis case report literature. The earliest report described a case in which in ltrates documented by chest radiograph developed in a worker in the photographic department of a German printing plant. The worker was known to have been exposed to carbon arc lamp fume for several years, which prompted analysis of workroom ue dust. Examination of 67 men working under similar conditions demonstrated that radiographic changes were correlated with years of exposure to carbon dusts (Heuck and Hoschek, 1968). Interstitial lung brosis and restrictive lung function were reported in ve reproduction photographers with more than a decade each of exposure to carbon arc lamp fume. X-ray microanalysis and electron diffraction revealed the presence of rare earth minerals (mainly cerium compounds), supporting a diagnosis of cerium pneumoconiosis (Vogt et al. A 58 year old man who worked for 46 years in a photoengraving laboratory and was exposed to smoke emitted from carbon arc lamps presented with a 2 year history of progressive dyspnoea and minimally productive cough, a single case that was described in two separate publications (Sabbioni et al. Lung biopsy showed peri-bronchiolar in ltrates and foci of sclerotic thickening of the connective septal tissue. The weight in ng/g of rare earth elements and thorium were determined using neutron activation analysis for the patient and for 11 controls who lived in the same district of North Italy but did not have occupational exposure: the patient had elevated levels of rare earth elements in the tissue compared to regional controls. Although thorium (a rare earth co-contaminant) was also elevated, its concentration was two orders of magnitude lower than the maximal permissible concentration for occupational exposure to natural thorium-232, suggesting that the rare earths but not thorium were related to the pulmonary brosis observed. A case of pulmonary brosis has also been described in another photoengraver with 13 years exposure who presented 17 years after his exposure ended (Sulotto et al. A 60 year old man was described as having diffuse interstitial lung brosis secondary to a distant 12 year exposure to rare earth dusts that occurred during work as a movie projectionist with arc lamp fume exposure. Rare earths were present in signi cantly higher concentrations in the lung biopsy of this patient when compared Organising Pneumonia and Other Uncommon Interstitial Disorders 321 to controls; there was an absence of any other identi able cause for lung brosis (Porru et al. Waring and Watling (1990) described abnormally high tissue concentrations of rare earth elements in another movie projectionist with 25 years of occupational exposure to carbon arc lamp fume without the occurrence of respiratory disease, but also enumerated 21 cases of rare earth pneumoconiosis. Dendriform pulmonary ossi cation has been described in association with rare earth pneumoconiosis (Yoon et al. A 38 year old man presented with a non-productive cough of several months of duration. He had a history of working for 3 years as a polisher at a crystal factory 20 years previously. He recalled that his workplace had been poorly ventilated and heavily contaminated with greenish polishing powder. There were also emphysematous changes and the bone windows showed a branching, twig-like ossi ed mass in the right lower lobe and a few dot-like ossi cations in both lower lobes. An open lung biopsy showed organising pneumonia, interstitial brosis, peripheral emphysema, dendriform pulmonary ossi cation and the presence of particulate matter. Analytic transmission electron microscopy with energy-dispersive X-ray analysis demonstrated the presence of cerium oxide and lanthanum, with cerium and lanthanum both being rare earth elements. Particles other than rare earth metals, such as quartz, feldspar, mica, kaolinite, halloysite, talc and TiO2, were also present, but were detected only infrequently. Other cases of rare earth pneumoconiosis have been reported among cerium rare earth processors (Nappée et al. Further details for one of these cases, who had also worked as a part-time projectionist, were provided in a separate case report (Pairon et al. As noted earlier, thorium does not appear to be a confounder explaining rare earth pneumoconiosis, but inhaled radionuclides have otherwise been implicated in occupational pulmonary brosis. The study population comprised 326 plutonium-exposed workers and 194 unexposed referents. The severity of chest radiograph interstitial abnormalities between the two groups was compared using the profusion scoring system. There was a signi cantly higher proportion of abnormal chest radiographic profusion scores (by International Labour Organization scoring) among plutonium-exposed workers (17. He presented with 10 months of increasing dry cough, night sweats, progressive breathlessness, anorexia and weight loss. The co-locating presence of indium and tin was demonstrated with X-ray energy spectrometry. In addition, the patient was found to have a high serum indium level (290 µg/L), approximately 3000-fold above a referent value (0.

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As the bladder fills, it rises out of the true pelvis and separates the peritoneum from the anterior abdominal wall. Hence the distended bladder may be punctured or approached surgically for insertion of suprapubic catheter/vesicostomy, without breaching the peritoneum. Indications these are simple forms of incontinent urinary diversion, employed when bladder empties inadequately in conditions like: Posterior urethral valves when endoscopic primary valve ablation is not feasible. Preoperative preparation Appropriate case selection and choice of procedure after having an indepth discussion with the family. Procedure Formation of vesicostomy (Blocksom) Make a 2cm transverse skin incision halfway between the umbilicus and the pubis. Tips and tricks the risk of prolapse is minimized by securely suturing the dome of the bladder to the rectus fascia. The renal vascular pedicle, comprising of a single renal artery and vein, enters the kidney via the renal hilum medially. Indications Non-functioning/poorly functioning kidney (secondary to congenital malformations, such as obstruction at pelvi-ureteric junction, vesicoureteric junction, vesico-ureteric reflux, and dysplasia). Position and theatre set-up Open surgery Supine position with a folded towel under the ipsilateral flank. Laparoscopic-transperitoneal Supine position with flank of the affected kidney elevated using a pillow and patient secured with straps. Retroperitoneoscopic approach Lateral decubitus or prone with appropriate support/padding and patient secured with straps. Procedure Open simple nephrectomy Anterior muscle splitting, extra-peritoneal approach, which is explained here. Dissect the perinephric fat off the kidney using blunt dissection and bipolar diathermy. Renal vein anteriorly and artery posteriorly, should be individually isolated, ligated in continuity, and divided. The distal ureteric stump can be left open if vesico-ureteric reflux was not demonstrated on preoperative investigations. Hemi-nephroureterectomy Following dissection of the peri-nephric fat off the lower pole of the duplex kidney, identify both upper and lower moiety ureters. Tips and tricks In hemi-nephroureterectomy, if the state of the vesico-ureteric reflux is not known, an in-dwelling urinary catheter is recommended to be left longer, for up to 5 days, to reduce the incidence of urine leak. Regardless of the technique used, the principles of repair are: Correction of ventral curvature of the penis (chordee). Surgical anatomy the penis comprises three cylindrical bodies of erectile cavernous tissue: the paired corpora cavernosa and the single corpus spongiosum ventrally. The corpus spongiosum tapers and runs on the underside of the corpora cavernosa and then expands to cap them as the glans penis. Indications Moderate and severe hypospadias that are expected to interfere with voiding and sexual function. If residual chordee is present, one or two of the following steps may have to be carried out to correct the curvature complete: Dorsal tunica albuginea plication or ventral dermal patch graft. If the plate is narrow, a dorsal relieving incision of the urethral plate (Snodgrass repair) will enable an adequate-calibre urethroplasty. Meatoplasty and glanuloplasty are then carried out by raising lateral flaps of glans tissue and approximating over the distal neo-urethra. Excise the hooded prepuce and re-fashion the penile shaft skin in such a way as to provide adequate ventral skin coverage and circumcised appearance of the penis. In severe hypospadias, following correction of chordee, staged repair is carried out by using genital (preputial) skin or extra-genital skin/tissue (such as post-auricular skin or buccal mucosa) graft in the first stage, followed by tubularization of the grafted tissue to form the neo-urethra with reconstruction of the penis, at least 6 months later, as a 2nd stage. Secure the urethral stent and apply a non-adherent, compressive dressing Postoperative care Allow the patient to go home on the same day in distal hypospadias repair and after 24h in proximal hypospadias repair. Tips and tricks Double diapers for ease of postoperative care-urethral stent drains into the outer nappy. It often includes the formation of a continent catheterizable abdominal stoma fashioned using appendix-appendicovesicostomy, commonly referred as Mitrofanoff channel. Surgical anatomy De-tubularized bowel segments provide capacity at lower pressure. In children, 20­30cm of distal ileum, 20cm from the ileo-caecal valve, will reach for the proposed anastomosis to the bladder. Indications Small capacity, poorly compliant bladder-often secondary to neuropathic bladder, posterior urethral valves, or bladder exstrophy. Ensure a 16Fr suprapubic catheter is inserted and secured well before completing the anastomosis. Postoperative care Suprapubic catheter on free drainage for 3 weeks and then clamp/ release 3-hourly. Tips and tricks Anastomose the bowel segment to the posterior bladder first; this can be performed from within the bladder. Procedure Muscle-splitting sub-costal incision, care taken to dissect the adventitial tissue between each muscle layer to maximize exposure through the smallest wound possible. Blunt dissection in this plane will cause the kidney to drop posteriorly and inferiorly, bringing the renal pelvis into view. If passing urine and no flank pain, then patient can go home for stent removal 5­7 days postoperative. Position place the patient supine in a head-up position with the neck extended and rotated to the opposite side. Be gentle, particularly around the bifurcation as the carotid body lies here and manipulation can lead to blood pressure instability or bradycardia. Complications haematoma can occur, particularly if the patient coughs excessively when rousing from anaesthetic. Tips and tricks Mark the landmarks with a skin marker prior to making the skin incision. Most aneurysms are infra-renal, although they may be juxtarenal, suprarenal, or thoraco-abdominal. Preoperative preparation patients should be seen in a vascular anaesthetic clinic and optimization of risk factors carried out. Theatre set-up As a prosthetic graft is used, ideally the operation should be carried out in a theatre with laminar flow, and the number of people and traffic in the theatre should be minimized. Complications Limb ischaemia can occur either due to graft occlusion or emboli dislodging from the aneurysm. Tips and tricks Leave the top of the clamp open but still in position after proximal anastomosis in case proximal control is required for any reason. Indications Classical clinical features of a palpable mass, hypotension and sudden onset of back pain may be present. Preoperative preparation Insert a large-bore cannula in the arms and give crystalloids cautiously. Differences to elective repair Once the diagnosis has been made, contact the relevant staff and transfer the patient to theatre urgently. Tips and tricks Close the abdomen using a temporary mesh in order to prevent compartment syndrome, if primary abdominal closure compromises respiratory or renal functions. Despite these relative contraindications, more challenging aneurysmal aortas can be stented in experienced hands. Procedure this description is for the Cook Medical Zenith device, although other devices like the Gore or AneuRx devices are also available. Preoperative preparation Meticulous preoperative planning of the stent using a Ct scan on a workstation is essential. Surgery transverse incisions are made in both groins and the common femoral arteries are exposed and vascular slings applied. Iliac angiography is carried out to ensure that the left internal iliac is patent. Endoleaks there are four types of endoleaks: type 1 is when there is contrast seen to leak at the attachment sites. Indications Severe diffuse iliac occlusive disease involving long segments of the iliacs or distal aortic occlusion not amenable to endovascular intervention. Procedure Exposure Expose the femoral vessels prior to opening the abdomen to minimize intra-abdominal fluid loss and ensure the femoral vessels are suitable for the distal anastomosis. Distal anastomosis Clamp the femoral vessels and perform an arteriotomy in the common femoral artery. Postoperative care the patient will require at least high-dependency care, most likely intensive care. If this should occur, urgent re-exploration improves the chances of successful revascularisation. Tips and tricks you may only be able to fully retract the small intestine to the right once the retroperitoneum has been opened. Ensure that there is enough space under the inguinal ligament so that the graft is not compressed. Preoperative preparation Review imaging and plan the point of proximal and distal anastomoses. Position place patient supine with the knee rested on a sterile saline bag to allow slight flexion of the hip and knee joint. Procedure Exposure of the femoral artery Make a vertical incision over the femoral vessels. Exposure of the popliteal artery above the knee joint Make an incision longitudinally above the medial femoral condyle over the medial aspect of the leg. Complications Immediate complications like bleeding and graft thrombosis and delayed complications like graft occlusion and infection. Early detection and treatment of these lesions or narrowing in the outflow or inflow vessels by angioplasty or surgery prevents graft occlusion. Femoral to anterior tibial artery bypass Lateral approach Make an incision 10cm long over the lateral part of the leg between the tibia and fibula running parallel to the tibia. Tips and tricks Distal bypasses below the knee are best performed with conduits using long saphenous vein. Anatomy Emboli most commonly preferentially travel down the superficial femoral artery. Indications Acute onset of signs and symptoms of an acutely ischaemic leg in the absence of a history suggestive of pre-existing peripheral vascular disease. Preoperative assessment If the history is highly indicative of an embolic cause for limb ischaemia without cause for concern that one may encounter long-standing arterial disease, it would be appropriate to transfer the patient to theatre for embolectomy without further imaging. Theatre set-up Ensure a variety of sizes of Fogarty catheter are available, including multiples of the most commonly used sizes as balloon rupture is common. Postoperative care Continue heparin infusion for 24­48h until adequate anticoagulation is established. Tips and tricks place the Fogarty catheter alongside the leg to give you an idea of how far you are inserting the catheter. Indications Acute onset of signs and symptoms of an acutely ischaemic arm in the absence of a history suggestive of pre-existing peripheral vascular disease. It is therefore occasionally appropriate to manage such patients conservatively if the operative risks are prohibitively high. Position and theatre set-up place the patient supine with the affected arm placed on an arm-board. A longitudinal arteriotomy may be made and closed with a vein patch later if necessary. If inflow is poor, pass a Fogarty catheter appropriate to the calibre of the artery (usually size 4 or 5) proximally, inflate the balloon, and trawl the catheter down the artery. Close the subcutaneous layer with Vicryl followed by interrupted or subcuticular skin closure. Postoperative care Commence the patient on intravenous heparin provided there are no contraindications. Tips and tricks you may need to bend the tip of the catheter slightly to help guide it down the ulnar artery. Indications to prevent compartment syndrome or to treat this if it has already occurred after peripheral revascularization in an ischaemic limb or after trauma to the leg. Postoperative care Leave the wounds open and cover with kaltostat or Mepitel dressings. Complications Avoid injury to the saphenous vein and nerve while performing the medial compartment fasciotomy. An amputation that yields a healthy stump with good muscle cover allows the patient to recover quicker and, if appropriate, regain mobility with the aid of prostheses. Below knee amputation Indications Chronic or acute ischaemia of limb that fails to respond to surgical revascularization. Preoperative assessment the most distal site where healing will reliably occur is chosen. Operative steps (Burgess long posterior flap) Measure the circumference of the leg a hands breadth below the tibial tuberosity. Skew flap technique Draw a circumferential line, a hands breadth below the tibial tuberosity. Postoperative care Start physiotherapy as soon as possible after the surgery to allow for knee extension. Anaesthesia this is most commonly performed under general anaesthesia, but can be performed under spinal anaesthesia and sedation if patient fitness precludes a general anaesthetic. A sciatic nerve block with or without an infusion catheter can be performed at this stage prior to transecting the nerve as proximally as possible. Simple opiates or gabapentin and amitriptyline may help ameliorate some of the symptoms. Preoperative preparations All patients with varicose veins should have a Duplex scan first. Position and theatre set-up For high tie and stripping, place the patient supine in a trendelenberg position. Procedure Make a transverse incision in the groin just medial to the femoral pulse. Complications Bruising and haematoma formation are well-recognized complications and can be managed conservatively in most cases.

Usage: q.i.d.

In murine models, as many as twice as many islets are required to reverse diabetes when hyperglycemia is not controlled with insulin treatment [11]. This nonspecific inflammatory response is proposed to contribute to substantial islet loss in some recipients [6, 7, 38, 39]. Anti-inflammatory approaches are currently under study to target this potential source of islet loss [8]. Thus, as ambient blood glucose rises, glucose travels freely into the beta cell through an insulin-independent glucose transporter and stimulates the secretion and synthesis of insulin. The pulsatile pattern of insulin secretion, which is present in the native pancreas, is restored in intrahepatic islet transplant recipients [43]. Islet function and insulin dependence are highly dependent upon the islet mass transplanted [21, 44]. When islets are transplanted intrahepatically, the alpha cells produce normally basal and arginine (protein)-stimulated glucagon. This appears to be a transplant site­specific defect; when a portion of the islets are placed in the peritoneal cavity, a normal counter-regulatory rise of glucagon during hypoglycemia is restored [51]. Once transplanted into the intrahepatic environment, islets require several weeks to months to engraft and reestablish a vascular supply. However, remarkably once transplanted and engrafted, islets can function nearly normally and sustain function for years. Islet autotransplant outcomes after total pancreatectomy: a contrast to islet allograft outcomes. Similar islet function in islet allotransplant and autotransplant recipients, despite lower islet mass in autotransplants. Evidence for instant blood-mediated inflammatory reaction in clinical autologous islet transplantation. Evidence of endoplasmic reticulum stress mediating cell death in transplanted human islets. Beta-cell death and mass in syngeneically transplanted islets exposed to short- and long-term hyperglycemia. A new enzyme mixture to increase the yield and transplant rate of autologous and allogeneic human islet products. Effect of core pancreas temperature during cadaveric procurement on human islet isolation and functional viability. American Journal of Transplantation: Official Journal of the American Society of Transplantation and the American Society of Transplant Surgeons 2009;9:2383­2391. A proposed threshold for dispersed-pancreatic tissue volume infused during intraportal islet autotransplantation after total pancreatectomy to treat chronic pancreatitis. Quality of life improves for pediatric patients after total pancreatectomy and islet autotransplant for chronic pancreatitis. Correlation between beta cell mass and glycemic control in type 1 diabetic recipients of islet cell graft. Proceedings of the National Academy of Sciences of the United States of America 2006;103:17444­17449. Islet oxygen consumption rate dose predicts insulin independence for first clinical islet allotransplants. Islet beta cell mass in diabetes and how it relates to function, birth, and death. Severely fibrotic pancreases from young patients with chronic pancreatitis: evidence for a ductal origin of islet neogenesis. Reinnervation of islets and regulation of insulin secretion by the hepatic sympathetic nerves. Three-dimensional islet graft histology: panoramic imaging of neural plasticity in sympathetic reinnervation of transplanted islets under the kidney capsule. Revascularization of transplanted pancreatic islets and role of the transplantation site. Increased numbers of low-oxygenated pancreatic islets after intraportal islet transplantation. Pancreatic islet production of vascular endothelial growth factor-a is essential for islet vascularization, revascularization, and function. Central necrosis in isolated hypoxic human pancreatic islets: evidence for postisolation ischemia. Small islets transplantation superiority to large ones: implications from islet microcirculation and revascularization. Islet size index as a predictor of outcomes in clinical islet autotransplantation. Cytotoxic effects of cytokines on human pancreatic islet cells in monolayer culture. Isolated human islets trigger an instant blood mediated inflammatory reaction: implications for intraportal islet transplantation as a treatment for patients with type 1 diabetes. Elevation of high-mobility group box 1 after clinical autologous islet transplantation and its inverse correlation with outcomes. Patient satisfaction and cost-effectiveness following total pancreatectomy with islet cell transplantation for chronic pancreatitis. Intrahepatic transplanted islets in humans secrete insulin in a coordinate pulsatile manner directly into the liver. Factors associated with insulin and narcotic independence after islet autotransplantation in patients with severe chronic pancreatitis. Long-term outcomes after total pancreatectomy and islet cell autotransplantation: is it a durable operation Total pancreatectomy with islet autotransplantation for chronic pancreatitis: do patients with prior pancreatic surgery have different outcomes Defective glucagon secretion during hypoglycemia after intrahepatic but not nonhepatic islet autotransplantation. This results in a type of impaired glucose metabolism known as pancreatogenic diabetes [1]. The American Diabetic Association classifies this type of diabetes mellitus as "other specific type of diabetes mellitus" [2] as opposed to the previous classification in 2003 as type lll. Pancreaticogenic diabetes following surgical resection differs from type 1 and type 2 diabetes in a number of respects. In particular, as type 1 diabetes mellitus is caused by cell-mediated autoimmune destruction of beta cells, it carries a significant risk of hyperglycemia and ketoacidosis both of which are uncommon with pancreaticogenic diabetes. Pancreaticogenic diabetes is also unlike type 2 diabetes mellitus, which is characterized by insulin resistance and relative insulin deficiency, because patients with pancreatic diabetes are sensitive to insulin [1]. In addition, the increased peripheral sensitivity to insulin and reduced glucagon levels with pancreaticogenic diabetes means that exogenous insulin administration frequently causes hypoglycemic attacks, and this response is the reason for the use of the term "brittle diabetes. As a consequence, glycemic control can be extremely challenging to manage with HbA1c levels, which are generally high and not infrequently associated with chronic diabetic complications (nephropathy, neuropathy, and retinopathy) in the longer term [6, 9]. Fear of this "brittle" diabetes is one of the main reasons that referral for surgery is often much delayed, which further contributes to the potential for complications in the long term [10, 11]. Hence, long-term assessment of graft function must include QoL assessment, in addition to physiological parameters. The European Organisation for Research and Treatment of Cancer Pancreatitis: Medical and Surgical Management, First Edition. The same study also demonstrated that constant pain regardless of severity was significantly associated with higher levels of hospitalization [19]. Those with constant pain were more likely to have been hospitalized on more than 10 occasions in the last year and have a higher need for incapacity benefit compared with sufferers of intermittent pain of all severities (42. Based on these assessments, the authors made the suggestion that treatments that eradicate pain (radical surgery) rather than therapeutic options that reduce pain scores (celiac plexus block, Puestow (drainage) procedure, and pancreatic enzyme supplementation) are the most effective strategies. As the management of these patients improved, consideration was given to its use for other (nonadenocarcinoma) tumors and benign conditions such as chronic pancreatitis. Unfortunately, prior to the advent of islet autotransplantation, the inevitable consequence of the surgery was immediate and complete exocrine and endocrine deficiency, which produced malabsorption and "brittle" diabetes [26]. The principle problem that affects the QoL in these patients is poor glycemic control despite the use of frequently very complex insulin regimens [27]. Several groups have recently reexamined the QoL in these patients in the light of further advances in surgical technique and progress in the management of the diabetes. Ninety one percent of patients complained of hypoglycemia (at least once a week in 72%), and steatorrhea and abdominal pain were found in 66%. These problems resulted in major impairments of leisure and work activities in 56% and 31%, respectively. Narcotic use fell dramatically following surgery with a 63% rate of narcotic independence (at last outpatient visit), which was also associated with a significantly improved QoL (analysis of the 36-item short-form health survey and the McGill pain questionnaire) [29]. In addition, more than 60% of patients were insulin independent or required minimal insulin [32]. The study found a very low 30-day mortality of between 1% and 2% (median of 0%) and insulin independence rates of 4. Dong and colleagues did not examine the results for QoL related to insulin independence, reduced requirements, or glycemic control but this was examined in a study from the Cleveland Clinic this year [33]. The stated aim of the study was "to improve QoL by alleviating pain and discontinuing narcotics while preventing or minimizing surgical diabetes". A visual analog pain scale was used to assess global pain and diabetes was examined by the use of HbA1c. Depression and anxiety were classed as mild, moderate, severe, and extremely severe and the effect on family/home responsibilities, recreation, social activity, occupation, sexual behavior, self-care, and life-support activities studied. Pre-operative state Family/home responsibilities Recreation Social activities Occupation Sexual behavior Self care Life support activity Depression Anxiety Pain scale 12 (61%) 16 (80%) 13 (66%) 14 (70%) 11 (55%) 6 (39%) 9 (45%) 4 (19%) 1 (4%) 11 (55%) Post-operative state 2 (10%) 4 (20%) 3 (15%) 3 (20%) 2 (10%) 0 (0%) 1 (10%) 0 (0%) 1 (4%) 2 (10%) the surgery and islet autotransplant on those activities severely affected are shown in Table 17C. These results are extremely encouraging and demonstrate that there has been a steady improvement in the QoL of the patients over the last three decades, brought about by a standardization of surgical technique, the involvement of multidisciplinary teams in the postoperative period and refinements in islet isolation [34­38]. Assessment of graft function Long-term follow-up protocols have been employed in Leicester since 1994. Patients are routinely assessed for the development of diabetes and attention paid to changes in blood glucose levels with adjustments in insulin doses as appropriate by a consultant diabetologist. In addition, there is protocol evaluation of diabetic status at 1, 3, 6 and 12 months and at yearly intervals thereafter. This includes the response to an oral glucose tolerance test as well as measurement of levels of C-peptide and HbA1c. Formal evaluation of QoL is performed periodically using regular assessments by a medical psychologist. In this way, a full picture of the effects of surgery can be obtained, which includes the following: 1 Insulin requirements. Chapter 17C: Total pancreatectomy and islet cell autotransplantation: long-term assessment of graft function 311 4 Formal assessment of QoL, including changes in pain and narcotic use performed by a consultant medical psychologist. Annual ultrasound examination is part of the standard follow up of patients with bilioenteric anastomoses to prevent complications following an insidious bilioenteric stenosis. In addition, infusion of islets into the portal venous system may induce long-term structural changes in the liver. Several authors have described periportal hepatic steatosis in patients who have undergone islet transplantation [39, 40]. The hepatic steatosis seen in islet transplant patients differs from that seen in the general population in that it produces a heterogeneous nodular or granular pattern of liver involvement. The images demonstrate nodular echogenicity of the liver found in 25% of the patients. The changes occur from 6 to 12 months following the islet infusion and are not found in patients following a total pancreatectomy alone. It is a benign condition thought to result from the paracrine action of high local concentrations of insulin and is believed to be a positive finding acting as a surrogate marker for persistent islet graft function. Background C-peptide secretion will lessen the severity of diabetes even if some exogenous insulin is required, allowing easier control of blood glucose. The improved diabetic control will reduce or abrogate the onset of secondary chronic diabetic complications. Together with the improved control of blood glucose, cessation of narcotics will produce a significantly improved QoL. Clinical outcomes following total pancreatectomy and islet autotransplantation the Minneapolis program Islet autotransplant programs throughout the world attempt to mimic the success of the Minneapolis transplant team (headed by Prof. David Sutherland), which was started in 1977 and now boasts the largest and most experienced team of islet autotransplant physicians and scientists in the world. The procedure has also been used to successfully treat 24 children to date with 56% showing insulin independence at 1 year posttransplant [42]. Ninety percent of patients had statistically significant improvement in their pain and health-related QoL [43, 44]. However, in the aggregate, physical health declined more rapidly for those who were insulin dependent compared with those less dependent on insulin. Previous work by the Minneapolis group has shown that patients who had not had previous pancreatic surgery and those who had previously had procedures to the head of the pancreas were more likely to have a greater islet yield and achieve insulin independence. Intraoperative problems related to a small number of patients where the calcification/fibrosis of the gland prevented the recovery of a significant or sufficient number of islets to justify autotransplantation. Unlike islet allotransplantation, optimal graft function was not achieved until 1 year following transplantation as evidenced by the highest serum C-peptide levels and lowest requirements of exogenous insulin. Both C-peptide secretion and HbA1c demonstrated some deterioration over time, but overall remained satisfactory throughout the follow-up period. This trend continued beyond 10 years, and at the maximum follow-up in the early patients in the series opiate use approaches zero. Further developments in improving long-term graft function As with all branches of transplant surgery, research and development allows progressive improvements in graft function and survival. A Pubmed search of islet-related research (search term: pancreatic islets) results in 41,298 peer-reviewed articles spanning 1907 to July 2011 and specifically 9039 related to islet transplantation.