शल्यतन्त्र Paper II, Part B PDF

शल्यतन्त्र Paper II PART B  CHAPTER I: diseases of liver  Benign Liver Tumors Benign liver tumors are relatively common. Most are asymptomatic, but some cause hepatomegaly, right upper quadrant discomfort, or intraperitoneal hemorrhage. Most are detected incidentally on ultrasoun...

शल्यतन्त्र Paper II PART B  CHAPTER I: diseases of liver  Benign Liver Tumors Benign liver tumors are relatively common. Most are asymptomatic, but some cause hepatomegaly, right upper quadrant discomfort, or intraperitoneal hemorrhage. Most are detected incidentally on ultrasound or other scans. Liver tests are usually normal or only slightly abnormal. Diagnosis is usually possible with imaging tests but may require biopsy. Treatment is needed only in a few specific circumstances.  Hepatocellular Adenoma: Hepatocellular adenoma is the most important benign tumor to recognize. It occurs primarily in women of childbearing age, particularly those taking oral contraceptives, possibly via estrogen’s effects. Most adenomas are asymptomatic, but large ones may cause right upper quadrant discomfort. Rarely, adenomas manifest as peritonitis and shock due to rupture and intraperitoneal hemorrhage. Rarely, they become malignant. Adenomas due to contraceptive use may regress if the contraceptive is stopped. If the adenoma does not regress or if it is subcapsular or > 5 cm, surgical resection is often recommended.  Focal Nodular Hyperplasia: This localized hamartoma may resemble macronodular cirrhosis histologically. Diagnosis is usually based on MRI or CT with contrast; the classic appearance is a lesion with a central scar. But biopsy may be necessary for confirmation. Treatment is rarely needed.  Hemangiomas: Hemangiomas are usually small and asymptomatic; they occur in 1-5% of adults. Symptoms are more likely if they are > 4 cm; symptoms include discomfort, fullness, and, less often, anorexia, nausea, early satiety, and pain secondary to bleeding or thrombosis. Hemangiomas are found incidentally during ultrasonography, CT, or MRI. Treatment is usually not indicated. Resection can be considered if symptoms are troublesome or if a hemangioma is rapidly enlarging.  Hepatocellular Carcinoma / Hepatoma Hepatocellular carcinoma usually occurs in patients with cirrhosis and is common in areas where infection with hepatitis B and C viruses is prevalent. Hepatoma is the most common type of primary liver cancer. Liver cancer is about 3 times more common in men than in women.  Etiology: Hepatocellular carcinoma is usually a complication of cirrhosis. The presence of HBV increases risk of hepatocellular carcinoma by > 100-fold among HBV carriers. Other disorders that cause hepatocellular carcinoma include cirrhosis due to chronic hepatitis C virus (HCV) infection, hemochromatosis, and alcoholic cirrhosis. Environmental carcinogens may play a role; e.g.: ingestion of food contaminated with fungal aflatoxins is believed to contribute to the high incidence of hepatocellular carcinoma in subtropical regions.  Signs & Symptoms: - Most commonly, previously stable patients with cirrhosis present with abdominal pain, weight loss, right upper quadrant mass, and unexplained deterioration. - Fever may occur. - In a few patients, the first manifestation of hepatocellular carcinoma is bloody ascites, shock, or peritonitis, caused by hemorrhage of the tumor.  Diagnosis: - Alpha-fetoprotein (AFP) measurement - Imaging (CT, ultrasonography, or MRI) In adults, AFP signifies dedifferentiation of hepatocytes, which most often indicates hepatocellular carcinoma; 40-65% of patients with the cancer have high AFP levels (> 400 mcg/L). High levels are otherwise rare, except in teratocarcinoma of the testis, a much less common tumor. If imaging shows characteristic findings and AFP is elevated, the diagnosis is clear. However, rarely, liver biopsy, often guided by ultrasonography or CT, is indicated for definitive diagnosis.  Management: - Transplantation if tumors are within the Milan criteria (one tumor < 5 cm or three tumors < 3 cm without vascular invasion and alfa-fetoprotein < 500 mcg/L). - Treatment of hepatocellular carcinoma depends on its stage and the underlying severity of liver disease. - In selected patients with singular tumors < 5 cm and no portal hypertension, surgical resection is potentially curative, with 5-year survival rates of 60-80%. - For small tumors < 2 cm, radiofrequency ablation (RFA) is potentially curative. - If the tumor is large (> 5 cm), is multifocal, has invaded the portal vein, or is metastatic, prognosis is poor (5-year survival rates of about 5% or less). -> Other Primary Liver Cancers Other primary liver cancers are uncommon. Diagnosis usually requires biopsy. Prognosis is typically poor. Some cancers, if localized, can be resected. Resection or liver transplantation may prolong survival.  Fibrolamellar Carcinoma: This distinct variant of hepatocellular carcinoma has a characteristic morphology of malignant hepatocytes enmeshed in lamellar fibrous tissue. It usually occurs in young adults and has no association with preexisting cirrhosis, hepatitis B virus (HBV), hepatitis C virus (HCV), or other known risk factors. Alpha-fetoprotein (AFP) levels are rarely elevated. Prognosis is better than that for hepatocellular carcinoma, and many patients survive several years after tumor resection.  Cholangiocarcinoma: This tumor originates in the biliary epithelium. It is common in China, where underlying infestation with liver flukes is believed to contribute. Elsewhere, it is less common than hepatocellular carcinoma; histologically, the two may overlap. Prognosis is poor in the vast majority of patients.  Hepatoblastoma: Although rare, hepatoblastoma is one of the most common primary liver cancers in infants, particularly those with a family history of familial adenomatous polyposis. It can also develop in children. An elevated AFP level and abnormal imaging test results may help in the diagnosis.  Angiosarcoma: This rare cancer is associated with specific chemical carcinogens, including industrial vinyl chloride.  Cystadenocarcinoma: This rare disorder is probably secondary to malignant transformation of a cystadenoma and is often multilobular. Treatment is liver resection. -> Metastatic Liver Cancer Metastatic liver cancer is more common than primary liver cancer. Liver metastases are common in many types of cancer, especially those of the gastrointestinal tract, breast, lung, and pancreas. The first symptoms of metastases are usually non-specific (e.g.: weight loss, right upper quadrant discomfort); they are sometimes the first symptoms of the primary cancer. Liver metastases are suspected in patients with weight loss and hepatomegaly or with primary tumors likely to spread to the liver. Diagnosis is usually supported by an imaging test, most often ultrasonography, spiral CT with contrast, or MRI with contrast. Treatment usually involves palliative chemotherapy.  Portal Hypertension Portal hypertension is elevated pressure in the portal vein. The portal vein, formed by the superior mesenteric and splenic veins, drains blood from the abdominal GI tract, spleen, and pancreas into the liver. Within reticuloendothelium-lined blood channels (sinusoids), blood from the terminal portal venules merges with hepatic arterial blood. Blood flows out of the sinusoids via the hepatic veins into the inferior vena cava. Normal portal pressure is 5-10 mmHg, which exceeds inferior vena caval pressure by 4-5 mmHg (portal venous gradient). Higher values are defined as portal hypertension.  Etiology: Portal hypertension results mainly from increased resistance to blood flow in the portal vein. A common cause of this resistance is disease within the liver. Portal hypertension is caused most often by cirrhosis (in developed countries), schistosomiasis (in endemic areas), or hepatic vascular abnormalities.  Pathophysiology: In cirrhosis, tissue fibrosis and regeneration increase resistance in the sinusoids and terminal portal venules. However, other potentially reversible factors contribute; they include contractility of sinusoidal lining cells, production of vasoactive substances, various systemic mediators of arteriolar resistance, and possibly swelling of hepatocytes. Over time, portal hypertension creates portosystemic venous collaterals. They may slightly decrease portal vein pressure but can cause complications. Engorged serpentine submucosal vessels (varices) in the distal esophagus and sometimes in the gastric fundus can rupture, causing sudden, catastrophic gastrointestinal bleeding. Bleeding rarely occurs unless the portal pressure gradient is > 12 mmHg. Gastric mucosal vascular congestion (portal hypertensive gastropathy) can cause acute or chronic bleeding independent of varices. Visible abdominal wall collaterals are common; veins radiating from the umbilicus (caput medusae) are much rarer and indicate extensive flow in the umbilical and periumbilical veins. Collaterals around the rectum can cause rectal varices that can bleed.  Signs & Symptoms: Portal hypertension is asymptomatic; symptoms and signs result from its complications. The most dangerous is acute variceal bleeding. Patients typically present with sudden painless upper gastrointestinal bleeding, often massive. Bleeding from portal hypertensive gastropathy is often subacute or chronic. Ascites, splenomegaly, or portosystemic encephalopathy may be present.   Diagnosis: Usually clinical evaluation Portal hypertension is assumed to be present when a patient with chronic liver disease has collateral circulation, splenomegaly, ascites, or portosystemic encephalopathy. Imaging may help when cirrhosis is suspected. Ultrasonography or CT often reveals dilated intra-abdominal collaterals, and Doppler ultrasonography can determine portal vein patency and flow. Esophagogastric varices and portal hypertensive gastropathy are best diagnosed by endoscopy, which may also identify predictors of esophagogastric variceal bleeding (e.g.: red markings on a varix).  Management: - Ongoing endoscopic therapy and surveillance - Non-selective beta-blockers with or without isosorbide mononitrate - Sometimes portal vein shunting - When possible, the underlying disorder is treated.  Yakṛt Vidradhi / Hepatic Abscess Hepatic abscess is defined as a pus-filled mass in the liver that can develop from injury to the liver or an intra-abdominal infection disseminated from the portal circulation.  Pathogens: - Gram-positive cocci: Streptococcus species (especially S. intermedius group), enterococci, and Staphylococcus aureus. - Anaerobes: Bacteroides species, Fusobacterium sp, Clostridium sp, etc. - Enterobacteriaceae (E. coli, Klebsiella spp., etc) and other Gram-negative bacilli. - Candida species  Risk Factors: - Diabetes - Liver cirrhosis - Immunocompromised state - Male sex - Advanced age - Proton-pump inhibitor use - Hepatic trauma - Peritonitis  Signs & Symptoms: - Fever, Pain, Tenderness with Hepatomegaly - Some may only have non-specific symptoms such as fever (60%) associated with chills and malaise. - Presentation may be subacute or chronic including weight loss, anorexia. - Occasionally, patients may be acutely ill with mental status changes. - Rarely, patients may present with sepsis and peritoneal signs from intraperitoneal rupture of the abscess. - Approximately 50% of patients have a solitary hepatic abscess. - Majority of abscesses involve in the right hepatic lobe (75%), less commonly left (20%) or caudate (5%) lobes. - Diaphragmatic irritation from abscess might refer pain to the right shoulder or result in cough or pleural rub.  Diagnosis: - For pyogenic liver abscess(es), positive blood cultures seen in up to 50%; alkaline phosphatase and WBC counts frequently elevated. Hyperbilirubinemia with or without jaundice occurs in < 50% of patients. - Imaging: CT, USG and MRI - CT or USG-guided percutaneous drainage or surgical drainage should be considered in all cases of hepatic abscess for diagnostic confirmation and culture. - Serology: Positive amebic or echinococcal serology helps differentiate parasitic liver abscess from pyogenic, especially in non-endemic areas.  Management: - Abscess drainage is the optimal therapy for pyogenic liver abscesses. CT- or USG-guided percutaneous needle aspiration +/- catheter drainage. Success in up to 90% of cases. If drainage is inadequate, surgical drainage may be required. Repeated aspiration is required in approximately 50%. Catheter placement should be considered in larger abscesses (> 5 cm diameter). Complications of percutaneous drainage include: perforation of adjacent abdominal organs, pneumothorax, hemorrhage and leakage of abscess contents in peritoneum. - Medical management with antibiotics can be considered in patients at high risk for drainage procedures or with small/multiple abscesses (< 3-5 cm in diameter) not amenable to drainage.  Yakṛtdalyodara -> Refer to Kāyachikitsā, Paper I, Part B, Raktavaha Srotas Vikāra  Hepatomegaly Hepatomegaly is a general medical term for an abnormally enlarged liver. Liver enlargement has many different causes, and by itself does not suggest a specific diagnosis or disease. Instead, it could indicate a variety of different conditions.  Etiology: 1) Infective -> Hepatitis, Hepatic abscess, Malaria, Hydatid cyst, Actinomycosis 2) Metabolic -> Fatty infiltration, Amyloidosis, Gaucher’s disease 3) Neoplastic -> Hepatoma, Hemangioma, Myeloma, Lymphoma 4) Cirrhotic -> Portal, Biliary, Cardio 5) Congenital -> Hemolytic anemia, Polycystic disease 6) Drugs & Toxins -> Alcoholism, Poisoning 7) Miscellaneous -> Budd-Chiari Syndrome (BCS), Hunter Syndrome, Sarcoidosis  Signs & Symptoms: - Enlargement of liver, tenderness - Lethargy, Malaise - Loss of appetite - Loss of weight - Jaundice  Investigations: - Physical evaluation - Abdominal ultrasound - CT scan, MRI - Blood tests, Liver function tests - Sometimes liver biopsy  Management depends on the underlying disease since hepatomegaly is only a symptom or complication, and not a separate diagnosis.  CHAPTER Ii: diseases of gallbladder  Cholelithiasis Cholelithiasis is the presence of one or more calculi (gallstones) in the gallbladder. In developed countries, about 10% of adults and 20% of people > 65 years have gallstones. Risk factors for gallstones include female sex, obesity, increased age, American Indian ethnicity, a Western diet, rapid weight loss, and a family history. Most disorders of the biliary tract result from gallstones.  Pathophysiology: Biliary sludge is often a precursor of gallstones. It consists of calcium bilirubinate (a polymer of bilirubin), cholesterol microcrystals, and mucin. Sludge develops during gallbladder stasis, as occurs during pregnancy or use of total parenteral nutrition. Most sludge is asymptomatic and disappears when the primary condition resolves. Alternatively, sludge can evolve into gallstones or migrate into the biliary tract, obstructing the ducts and leading to biliary colic, cholangitis, or pancreatitis. Cholesterol stones account for > 85% of gallstones in the Western world. For cholesterol gallstones to form, the following is required: - Bile must be supersaturated with cholesterol. Normally, water-insoluble cholesterol is made water soluble by combining with bile salts and lecithin to form mixed micelles. Supersaturation of bile with cholesterol most commonly results from excessive cholesterol secretion (as occurs in obesity or diabetes) but may result from a decrease in bile salt secretion (e.g.: in cystic fibrosis because of bile salt malabsorption). - The excess cholesterol must precipitate from solution as solid microcrystals. Such precipitation in the gallbladder is accelerated by mucin, a glycoprotein, or other proteins in bile. - The microcrystals must aggregate and grow. This process is facilitated by the binding effect of mucin forming a scaffold and by retention of microcrystals in the gallbladder with impaired contractility due to excess cholesterol in bile. Black pigment stones are small, hard gallstones composed of calcium (Ca) bilirubinate and inorganic Ca salts (e.g.: Ca carbonate, Ca phosphate). Factors that accelerate stone development include alcohol-related liver disease, chronic hemolysis, and older age. Brown pigment stones are soft and greasy, consisting of bilirubinate and fatty acids (Ca palmitate or stearate). They form during infection, inflammation, and parasitic infestation. Gallstones grow at about 1 to 2 mm/yr, taking 5 to 20 years before becoming large enough to cause problems. Most gallstones form within the gallbladder, but brown pigment stones form in the ducts.   Signs & Symptoms: About 80% of people with gallstones are asymptomatic. The remainder have symptoms ranging from a characteristic type of pain (biliary colic) to cholecystitis to life-threatening cholangitis. Biliary colic is the most common symptom. Stones occasionally traverse the cystic duct without causing symptoms. However, most gallstone migration leads to cystic duct obstruction, which, even if transient, causes biliary colic. Biliary colic characteristically begins in the right upper quadrant but may occur elsewhere in the abdomen. It is often poorly localized, particularly in diabetics and older patients. The pain may radiate into the back or down the arm. Episodes begin suddenly, become intense within 15 minutes to 1 hour, remain at a steady intensity (not colicky) for up to 12 hours (usually < 6 hours), and then gradually disappear over 30-90 minutes, leaving a dull ache. The pain is usually severe enough to send patients to the emergency department for relief. Nausea and some vomiting are common, but fever and chills do not occur unless cholecystitis has developed Mild right upper quadrant or epigastric tenderness may be present; peritoneal findings are absent. Between episodes, patients feel well.  Diagnosis: - Abdominal ultrasonography - Endoscopic ultrasonography accurately detects small gallstones (< 3 mm) - CT scan, MRI - About 10 to 15% of gallstones are calcified and visible on plain x-rays  Management: - For symptomatic stones: Surgery or stone dissolution - For asymptomatic stones: Expectant management Surgery can be done with an open or a laparoscopic technique. Open cholecystectomy, which involves a large abdominal incision and direct exploration, is safe and effective. Its overall mortality rate is about 0.1% when done electively during a period free of complications. Laparoscopic cholecystectomy is the treatment of choice. Using video endoscopy and instrumentation through small abdominal incisions, the procedure is less invasive than open cholecystectomy. The result is a much shorter convalescence, decreased postoperative discomfort, improved cosmetic results, yet no increase in morbidity or mortality. Laparoscopic cholecystectomy is converted to an open procedure in 2-5% of patients, usually because biliary anatomy cannot be identified or a complication cannot be managed. Older age typically increases the risks of any type of surgery. Stone dissolution For patients who decline surgery or who are at high surgical risk (e.g.: because of concomitant medical disorders or advanced age), gallbladder stones can sometimes be dissolved by ingesting bile acids orally for many months. The best candidates for this treatment are those with small, radiolucent stones (more likely to be composed of cholesterol) in a functioning non-obstructed gallbladder (indicated by normal filling detected during cholescintigraphy or oral cholecystography or by absence of stones in the neck). Ursodeoxycholic acid 4-5 mg/kg orally twice a day or 3 mg/kg orally three times a day (8-10 mg/kg/day) dissolves 80% of tiny stones < 0.5 cm in diameter within 6 months. For larger stones (the majority), the success rate is much lower, even with higher doses of ursodeoxycholic acid. Further, after successful dissolution, stones recur in 50% of patients within 5 years.  Acute Cholecystitis Acute cholecystitis is inflammation of the gallbladder that develops over hours, usually because a gallstone obstructs the cystic duct. Acute cholecystitis is the most common complication of cholelithiasis. In fact, ≥ 95% of patients with acute cholecystitis have cholelithiasis. When a stone becomes impacted in the cystic duct and persistently obstructs it, acute inflammation results. Bile stasis triggers release of inflammatory enzymes (e.g.: phospholipase A, which converts lecithin to lysolecithin, which then may mediate inflammation). The damaged mucosa secretes more fluid into the gallbladder lumen than it absorbs. The resulting distention further releases inflammatory mediators (e.g.: prostaglandins), worsening mucosal damage and causing ischemia, all of which perpetuate inflammation. Bacterial infection can supervene. The vicious circle of fluid secretion and inflammation, when unchecked, leads to necrosis and perforation. If acute inflammation resolves and continues to recur, the gallbladder becomes fibrotic and contracted and does not concentrate bile or empty normally -> Chronic Cholecystitis. Acute Acalculous Cholecystitis: Acalculous cholecystitis is cholecystitis without stones. It accounts for 5-10% of cholecystectomies done for acute cholecystitis. Risk factors include the following: - Critical illness (e.g.: major surgery, burns, sepsis, or trauma) - Prolonged fasting or total parenteral nutrition - Shock - Immune deficiency - Vasculitis (e.g.: systemic lupus erythematosus [SLE], polyarteritis nodosa)  Signs & Symptoms: - Most patients have had prior attacks of biliary colic or acute cholecystitis. - The pain of cholecystitis is similar in quality and location to biliary colic but lasts longer (i.e. > 6 hours) and is more severe. - Vomiting is common, as is right subcostal tenderness. - Within a few hours, the Murphy sign (deep inspiration exacerbates the pain during palpation of the right upper quadrant and halts inspiration) develops along with involuntary guarding of upper abdominal muscles on the right side. - Fever, usually low grade, is common. In older patients, the first or only symptoms may be systemic and non-specific (e.g.: anorexia, vomiting, malaise, weakness, fever). Acute cholecystitis begins to subside in 2-3 days and resolves within 1 week in 85% of patients even without treatment. The symptoms of acalculous cholecystitis are similar to those of acute cholecystitis with gallstones but may be difficult to identify because patients tend to be severely ill (e.g.: in an intensive care unit) and may be unable to communicate clearly. Abdominal distention or unexplained fever may be the only clue.  Complications: Without treatment, 10% of patients develop localized perforation, and 1% develop free perforation and peritonitis. Increasing abdominal pain, high fever, and rigors with rebound tenderness or ileus suggest empyema (pus) in the gallbladder, gangrene, or perforation. When acute cholecystitis is accompanied by jaundice or cholestasis, partial common duct obstruction is likely, usually due to stones or inflammation.  Diagnosis: - Transabdominal ultrasonography - Cholescintigraphy if ultrasonography results are equivocal or if acalculous cholecystitis is suspected - Abdominal CT identifies complications such as gallbladder perforation or pancreatitis  Management: - Supportive care (hydration, analgesics, antibiotics, NSAIDs) - Cholecystectomy  Chronic Cholecystitis Chronic cholecystitis is long-standing gallbladder inflammation. It almost always results from gallstones and prior episodes of acute cholecystitis (even if mild). Damage ranges from a modest infiltrate of chronic inflammatory cells to a fibrotic, shrunken gallbladder. Extensive calcification due to fibrosis is called porcelain gallbladder.  Signs & Symptoms: Gallstones intermittently obstruct the cystic duct and so cause recurrent biliary colic. Such episodes of pain are not necessarily accompanied by overt gallbladder inflammation; the extent of inflammation does not correlate with the intensity or frequency of biliary colic. Upper abdominal tenderness may be present, but usually fever is not. Fever suggests acute cholecystitis. Once episodes begin, they are likely to recur.  Diagnosis: Ultrasonography Chronic cholecystitis is suspected in patients with recurrent biliary colic plus gallstones. Ultrasonography or another imaging test usually shows gallstones and sometimes a shrunken, fibrotic gallbladder.  Management: Laparoscopic cholecystectomy  Obstructive Jaundice Jaundice is a yellowish discoloration of the skin and mucous membranes caused by hyperbilirubinemia. Jaundice becomes visible when the bilirubin level is about 2-3 mg/dL (34-51 micromol/L). Obstructive jaundice is a specific type of jaundice, where symptoms develop due to a narrowed or blocked bile duct or pancreatic duct, preventing the normal drainage of bile from the bloodstream into the intestines.  Etiology: - Cholelithiasis - Cholangitis - Lymph node enlargement near the bile duct - Pancreatic cancer - Pancreatic cysts - Other pancreatic duct obstructions such as scarring  Signs & Symptoms: - Abdominal pain (typically occurs in the right upper quadrant of the body) - Yellow discoloration of the eyes and skin - Dark-colored urine - Pale stools - Diarrhoea - Easy bleeding or bruising - Fever and chills - Itching - Anorexia - Malaise or lethargy - Weight loss Serious symptoms that might indicate a life-threatening condition: - Abdominal swelling, distension and bloating - High fever (38.3° C) - Nausea with or without vomiting - Severe abdominal pain  Investigations: - CT scan, MRI - Blood tests to examine bilirubin levels - Endoscopic retrograde cholangiopancreatography (ERCP) - Endoscopic ultrasound (EUS) - Choledochoscopy - Probe-based confocal endomicroscopy - Narrow band imaging of the bile duct  Management: Treatment options for obstructive jaundice depend on the exact cause and severity of the disease. Examples include: - Antibiotic therapy (if indicated for infection) - Endoscopic retrograde cholangiopancreatography (ERCP), an imaging procedure that allows treatment of some bile duct problems, including removal of gallstones that are causing obstruction - Intravenous fluids and pain medications - Nutritional support - Surgery or other procedures to repair anatomical defects or create alternative pathways for the flow of bile - Transplantation of the liver (if all other methods are unsuccessful and all of the liver is damaged) - Treatment for cancer, if present, which may include surgery, chemotherapy, or radiation therapy  Tumors of Gallbladder & Bile Ducts Gallbladder and bile duct tumors can cause extrahepatic biliary obstruction. Cholangiocarcinomas and other bile duct tumors are rare (1-2/100,000 people) but are usually malignant. Cholangiocarcinomas occur predominantly in the extrahepatic bile ducts: 60-70% in the perihilar region (Klatskin tumors), about 25% in the distal ducts, and the rest in the liver. Risk factors include primary sclerosing cholangitis, older age, infestation with liver flukes, and a choledochal cyst. Gallbladder carcinoma is uncommon (2.5/100,000). It is more common among American Indians, patients with large gallstones (> 3 cm), and those with extensive gallbladder calcification due to chronic cholecystitis (porcelain gallbladder). Nearly all (70-90%) patients also have gallstones. Median survival is 3 months. Cure is possible when cancer is found early (e.g.: incidentally at cholecystectomy). Gallbladder polyps are usually asymptomatic benign mucosal projections that develop in the lumen of the gallbladder. Most are < 10 mm in diameter and composed of cholesterol ester and triglycerides; the presence of such polyps is called cholesterolosis. They are found in about 5% of people during ultrasonography. Other, much less common benign polyps include adenomas (causing adenomyomatosis) and inflammatory polyps. Small gallbladder polyps are incidental findings that do not require treatment.  Signs & Symptoms: Most patients with cholangiocarcinomas present with pruritus and painless obstructive jaundice, typically at age 50-70 years. Early perihilar tumors may cause only vague abdominal pain, anorexia, and weight loss. Other features include fatigue, acholic stool, a palpable mass, hepatomegaly, or a distended gallbladder (Courvoisier sign, with distal cholangiocarcinoma). Pain may resemble that of biliary colic (reflecting biliary obstruction) or may be constant and progressive. Manifestations of gallbladder carcinoma may range from incidental findings at cholecystectomy done to relieve biliary pain to cholelithiasis to advanced disease with constant pain, weight loss, and an abdominal mass or obstructive jaundice. Most gallbladder polyps cause no symptoms.  Diagnosis: Ultrasonography (sometimes endoscopic), followed by CT cholangiography or magnetic resonance cholangiopancreatography (MRCP)  Management: - For cholangiocarcinomas, stenting (or another bypass procedure) or occasionally resection - For gallbladder carcinoma, usually symptomatic treatment  CHAPTER Iii: diseases of pancreas  Pancreatitis Pancreatitis is classified as either acute or chronic. Acute pancreatitis is inflammation that resolves both clinically and histologically. Chronic pancreatitis is characterized by histologic changes that are irreversible and progressive and that result in considerable loss of exocrine and endocrine pancreatic function. Patients with chronic pancreatitis may have a flare-up of acute disease. Pancreatitis can affect both the exocrine and endocrine functions of the pancreas. Pancreatic cells secrete bicarbonate and digestive enzymes into ducts that connect the pancreas to the duodenum at the ampulla of Vater (exocrine function). Pancreatic beta cells secrete insulin directly into the bloodstream (endocrine function). -> Acute Pancreatitis Acute pancreatitis is acute inflammation of the pancreas (and, sometimes, adjacent tissues). The most common triggers are gallstones and alcohol intake. Acute pancreatitis is a common disorder and a major healthcare concern.  Etiology: Gallstones and alcohol consumption account for ≥ 70% of acute pancreatitis cases. Other causes include a number of genetic mutations predisposing to pancreatitis. An autosomal dominant mutation of the cationic trypsinogen gene causes pancreatitis in 80% of carriers; an obvious familial pattern is present. Other mutations have lesser penetrance and are not readily apparent clinically except through genetic testing. The gene that causes cystic fibrosis increases the risk of recurrent acute pancreatitis as well as chronic pancreatitis. Acute pancreatitis is a complication that develops after endoscopic retrograde cholangiopancreatography (ERCP) in about 5% of patients.  Types: 1) Interstitial pancreatitis is defined by the presence of an enlarged pancreas on imaging. Peripancreatic stranding may be seen and is a sign of inflammation. Most patients develop this type of pancreatitis. The majority of cases are self- limiting. 2) Necrotizing pancreatitis is defined by the presence of pancreatic and/or peripancreatic necrosis. It is best seen on contrast-enhanced cross-sectional imaging. Necrotizing pancreatitis occurs in 5-10% of patients with acute pancreatitis and is associated with a prolonged and more severe disease course.  Classification: 1) Mild pancreatitis: Inflammation is confined to the pancreas and its close vicinity. Patients do not have organ failure or systemic or local complications. 2) Moderately severe pancreatitis: Patients have local or systemic complications but no organ failure, or only transient organ failure (resolves within 48 hours). 3) Severe pancreatitis: There is persistent single or multiorgan failure (> 48 hours). Most patients have one or more local complications.  Complications: 1) Local: Pancreatic and peripancreatic fluid collections, splenic vein thrombosis, pseudoaneurysm formation, and gastric outlet dysfunction 2) Systemic: Shock, organ failure  Signs & Symptoms: - An acute pancreatitis attack causes steady upper abdominal pain, typically severe enough to require parenteral opioids. - The pain radiates through to the back in about 50% of patients. - Pain usually develops suddenly in gallstone pancreatitis; in alcoholic pancreatitis, pain develops over a few days. - The pain usually persists for several days. Sitting up and leaning forward may reduce pain, but coughing, vigorous movement, and deep breathing may accentuate it. - Nausea and vomiting are common. - The patient appears acutely ill and sweaty. - Pulse rate is usually 100-140 beats/minute. - Respiration is shallow and rapid. - Blood pressure may be transiently high or low. - Temperature may be normal or even subnormal at first but may increase to 37.7-38.3° C within a few hours. - Scleral icterus is occasionally present because of obstruction of the bile duct by a gallstone or inflammation and swelling of the pancreatic head. - Decreased bowel sounds and abdominal distention. - Marked abdominal tenderness occurs, most often in the upper abdomen. - Infection in the pancreas or in an adjacent fluid collection should be suspected if the patient has a generally toxic appearance with fever and an elevated white blood cell count or if deterioration follows an initial period of stabilization. Patients with severe disease can develop multiorgan failure (cardiovascular, renal, and respiratory).   Diagnosis: - Serum markers (amylase, lipase) - Pancreatitis is suspected whenever severe abdominal pain occurs, especially in a patient with significant alcohol use or known gallstones. - To exclude other causes of abdominal pain and to diagnose metabolic complications of acute pancreatitis, a broad range of tests is usually done at initial evaluation. These include laboratory and imaging tests.  Management: The basic treatment of acute pancreatitis includes: - Early goal-directed fluid resuscitation - Analgesia - Nutritional support Treatment of severe acute pancreatitis and complications includes: - ICU (intensive care unit) care - Enteral nutrition preferred over parenteral nutrition - Antibiotics for extrapancreatic infections and infected necrosis - Necrosectomy (removal of necrotic tissue) for infected necrosis - Endoscopic retrograde cholangiopancreatography (ERCP) for acute pancreatitis and concurrent acute cholangitis - Drainage of pseudocysts -> Chronic Pancreatitis Chronic pancreatitis is persistent inflammation of the pancreas that results in permanent structural damage with fibrosis and ductal strictures, followed by a decline in exocrine and endocrine function (pancreatic insufficiency). Drinking alcohol and smoking cigarettes are two of the major risk factors.  Signs & Symptoms: - Abdominal pain and pancreatic insufficiency are the primary manifestations of chronic pancreatitis. - About 10-15% of patients have no pain but present with malabsorption. - Clinical manifestations of pancreatic insufficiency include flatulence, abdominal distention, steatorrhea, undernutrition, weight loss, and fatigue. - Glucose intolerance may appear at any time, but overt diabetes mellitus usually occurs late in the course of chronic pancreatitis. - Patients also are at risk of hypoglycemia because pancreatic alpha cells, which produce glucagon (a counter-regulatory hormone), are lost.  Diagnosis: X-ray, CT scan, Pancreatig function test, MRI coupled with magnetic resonance cholangiopancreatography (MRCP)  Management: Pain control, Pancreatic enzyme supplements, Management of diabetes and other complications  Pancreatic Cancer The most common pancreatic cancer is ductal adenocarcinoma. Adenocarcinomas of the exocrine pancreas arise from duct cells 9 times more often than from acinar cells; 80% occur in the head of the gland. Adenocarcinomas appear at the mean age of 55 years and occur 1.5-2 times more often in men. Prominent risk factors for pancreatic cancer include smoking, a history of chronic pancreatitis, obesity, being male, and being black. Heredity plays some role. Alcohol and caffeine consumption do not seem to be risk factors.  Signs & Symptoms: - Symptoms of pancreatic cancer such as pain and weight loss are non-specific, leading to a later diagnosis. - By the time of diagnosis, 90% of patients have locally advanced tumors that have involved retroperitoneal structures, spread to regional lymph nodes, or metastasized to the liver or lung. - Most patients have severe upper abdominal pain, which usually radiates to the back. Weight loss is common. - Adenocarcinomas of the head of the pancreas cause obstructive jaundice in 80-90% of patients. - Cancer in the body and tail may cause splenic vein obstruction, resulting in splenomegaly, gastric and esophageal varices, and gastrointestinal hemorrhage. - The cancer causes diabetes in 25-50% of patients, leading to symptoms of glucose intolerance (e.g.: polyuria and polydipsia). - Pancreatic cancer can also interfere with production of digestive enzymes by the pancreas (pancreatic exocrine insufficiency) in some patients and with the ability to break down food and absorb nutrients (malabsorption). This malabsorption causes bloating and gas and a watery, greasy, and/or foul-smelling diarrhea, leading to weight loss and vitamin deficiencies.  Investigations: CT scan, MRI or MRCP, followed by endoscopic ultrasonography  Prognosis: Prognosis for pancreatic cancer varies with stage but overall is poor; 5-year survival rate is < 2%, because patients are usually diagnosed late.  Management: - Whipple procedure (pancreaticoduodenectomy) - Adjuvant chemotherapy and radiation therapy - Symptomatic treatment, analgesics (opioids), pancreatic enzyme supplementation - Ultimately, most patients experience pain and die. Thus, symptomatic treatment is as important as controlling disease. Appropriate end-of-life care should be discussed.  CHAPTER IV: diseases of spleen  Plīhodara -> Refer to Kāyachikitsā, Paper I, Part B, Raktavaha Srotas Vikāra  Splenomegaly Splenomegaly is abnormal enlargement of the spleen. It is almost always secondary to other disorders.  Etiology: In temperate climates, the most common causes are: - Connective tissue disorders - Lymphoproliferative disorders - Myeloproliferative neoplasms - Storage diseases (e.g.: Gaucher disease) In the tropics, the most common causes are infectious diseases (e.g.: malaria, visceral leishmaniasis). If splenomegaly is massive (spleen palpable 8 cm below the costal margin), the cause is usually: - Chronic lymphocytic leukemia - Chronic myeloid leukemia - Gaucher disease - Hairy cell leukemia - Non-Hodgkin lymphoma - Polycythemia vera - Primary myelofibrosis Splenomegaly can lead to cytopenias, a disorder called hypersplenism.  Evaluation of Splenomegaly: 1) History - Most of the presenting symptoms result from the underlying disorder. - Splenomegaly itself may cause early satiety by encroachment of the enlarged spleen on the stomach. - Fullness and left upper quadrant abdominal pain are also possible. - Sudden, severe pain suggests splenic infarction. - Recurrent infections, symptoms of anemia, or bleeding manifestations suggest cytopenia and possible hypersplenism. - In patients who have had blunt abdominal trauma within the last several weeks, splenic enlargement may have resulted from splenic subcapsular hematoma and sudden severe pain and/or shock may indicate splenic rupture.  2) Physical Examination - The sensitivity for detection of ultrasound-documented splenic enlargement is 60-70% for palpation and 60-80% for percussion. - Up to 3% of normal, thin, people have a palpable spleen. Also, a palpable left upper quadrant mass may indicate a problem other than an enlarged spleen such as a hypernephroma. 3) Testing If confirmation of splenomegaly is necessary because the examination is equivocal, ultrasonography is the test of choice because of its accuracy and low cost. CT and MRI may provide more detail of the organ’s consistency. Specific causes suggested clinically should be confirmed by appropriate testing. If no cause is suggested, the highest priority is exclusion of occult infection, because early treatment affects the outcome of infection more than it does most other causes of splenomegaly. Complete blood count (CBC), blood cultures, and bone marrow examination and culture should be considered. If the patient is not ill, has no symptoms besides those due to splenomegaly, and has no risk factors for infection, the extent of testing is controversial but probably includes CBC, peripheral blood smear, liver tests, and abdominal CT. If testing reveals no abnormalities other than splenomegaly, the patient should be reevaluated at intervals of 6 to 12 months or when new symptoms develop.  Management is directed at the underlying disorders. An enlarged spleen itself in an asymptomatic patient needs no treatment unless severe hypersplenism is present. Patients with palpable or very large spleens should avoid contact sports and weight-lifting to decrease the risk of splenic rupture.  Splenic Injury & Rupture Splenic injury usually results from blunt abdominal trauma. A ruptured spleen is a medical emergency that occurs as a result of a break in the spleen's surface.  Etiology: - Significant impact (e.g.: motor vehicle crash) can damage the spleen, as can penetrating trauma (e.g.: knife wound, gunshot wound). - Splenic enlargement as a result of fulminant Epstein-Barr viral disease predisposes to rupture with minimal trauma or even spontaneously. Splenic injuries range from subcapsular hematomas and small capsular lacerations to deep parenchymal lacerations, crush injury, and avulsion from the pedicle.  Signs & Symptoms: - The main immediate consequence is hemorrhage into the peritoneal cavity. - The amount of hemorrhage ranges from small to massive, depending on the nature and degree of injury. Many small lacerations, particularly in children, cease bleeding spontaneously. Larger injuries hemorrhage extensively, often causing hemorrhagic shock. - A splenic hematoma sometimes ruptures, usually in the first few days, although rupture can occur from hours to even months after injury. - The manifestations of major hemorrhage, including hemorrhagic shock, abdominal pain, and distention, are usually clinically obvious. - Lesser hemorrhage causes left upper quadrant abdominal pain, which sometimes radiates to the left shoulder. Patients with unexplained left upper quadrant pain, particularly if there is evidence of hypovolemia or shock, should be asked about recent trauma. Maintain a high index of suspicion for splenic injury in patients who have left rib fractures.  Investigations: CT scan, USG, Exploratory laparotomy  Management: - Observation - Angioembolization - Sometimes surgical repair or splenectomy CHAPTER V: diseases of kidney & ureter  Renal Anomalies The urinary tract is a common location for congenital anomalies of varying significance. Many anomalies are asymptomatic and diagnosed via prenatal ultrasonography or part of a routine evaluation for other congenital anomalies. Other anomalies are diagnosed during evaluation of urinary tract obstruction, urinary tract infection, or trauma.  Duplication Anomalies: Supernumerary collecting systems may be unilateral or bilateral and may involve the renal pelvis and ureters (accessory renal pelvis, double or triple pelvis and ureter), calyx, or ureteral orifice. Duplex kidneys have a single renal unit with more than one collecting system. This anomaly differs from fused kidneys, which involves fusion of two renal parenchymal units maintaining their respective individual collecting systems.  Fusion Anomalies: With fusion anomalies, the kidneys are joined, but the ureters enter the bladder on each side. Horseshoe kidney, the most common fusion anomaly, occurs when renal parenchyma on each side of the vertebral column is joined at the corresponding (usually lower) poles; an isthmus of renal parenchyma or fibrous tissue joins at the midline. The ureters course medially and anteriorly over this isthmus and generally drain well. Pyeloplasty relieves the obstruction and can be done without resecting the isthmus. Crossed fused renal ectopia is the 2nd most common fusion anomaly. The renal parenchyma (representing both kidneys) is on one side of the vertebral column. One of the ureters crosses the midline and enters the bladder on the side opposite the fused kidneys. When ureteropelvic junction obstruction is present, pyeloplasty is the treatment of choice. Fused pelvic kidney (pancake kidney) is much less common. A single pelvic kidney is served by two collecting systems and ureters. If obstruction is present, reconstruction is needed.  Malrotation: Malrotation is usually of little clinical significance. Ultrasonography often shows hydronephrosis. Further evaluation with a magnetic resonance urogram or renal scan may be done when clinicians are concerned about possible obstruction.  Multicystic Dysplastic Kidney (MCDK): In this condition, there is a non-functioning renal unit consisting of non- communicating cysts with intervening solid tissue. Usually, ureteral atresia is also present. The contralateral kidney is usually normal, but up to 10% of patients may have vesicoureteral reflux or ureteropelvic junction obstruction. Frequently, the kidney progressively involutes and eventually is no longer visible on ultrasonography. Nephrectomy may be considered for the presence of solid tissue, progressive enlargement, hypertension or a ruptured cyst that causes pain.  Renal Agenesis: Bilateral renal agenesis as part of a syndrome of oligohydramnios, pulmonary hypoplasia, and extremity and facial anomalies (classic Potter syndrome) is fatal within minutes to hours. Fetal demise is common. Unilateral renal agenesis accounts for about 5% of renal anomalies. Many cases result from complete involution in utero of a multicystic dysplastic kidney. It usually is accompanied by ureteral agenesis with absence of the ipsilateral trigone and ureteral orifice. However, the ipsilateral adrenal gland is unaffected. No treatment is necessary.  Renal Dysplasia: In renal dysplasia, the renal vasculature, tubules, collecting ducts, or drainage apparatus develops abnormally. Diagnosis of renal dysplasia is by biopsy. If dysplasia is segmental, treatment of renal dysplasia is often unnecessary. If dysplasia is extensive, renal dysfunction may necessitate nephrologic care, including renal replacement therapy.  Renal Ectopia: Renal ectopia (abnormal renal location) usually results when a kidney fails to ascend from its origin in the true pelvis; a rare exception occurs with a superiorly ascended (thoracic) kidney. Obstruction and severe reflux may be corrected surgically when indicated (if causing hypertension, recurrent infections, or renal growth retardation).  Renal Hypoplasia: Hypoplasia usually occurs because inadequate ureteral bud branching causes an underdeveloped, small kidney with histologically normal nephrons. If hypoplasia is segmental, hypertension can occur, and ablative surgery may be needed.  Ureteral Anomalies Ureteral anomalies frequently occur with renal anomalies but may occur independently. Complications include: - Obstruction, vesicoureteral reflux, infection, and calculus formation due to urinary stasis - Urinary incontinence due to abnormal termination of the ureter in the urethra, perineum, or vagina Diagnosis of ureteral anomalies may be suggested by abnormalities on routine prenatal ultrasonography and occasionally by physical examination. Ureteral anomalies should be suspected in children with an episode of pyelonephritis or recurrent urinary tract infections and in girls with persistent urinary incontinence. Testing typically involves ultrasonography of the kidneys, ureters, and bladder before and after voiding, and then fluoroscopic voiding cystourethrography. Ureteral anomaly treatments are surgical.  Ectopic Ureteral Orifices: Openings of single or duplicated ureters may be malpositioned on the lateral bladder wall, distally along the trigone, in the bladder neck, in the female urethra distal to the sphincter, in the genital system (prostate and seminal vesicle in male, uterus or vagina in female), or externally. Surgery is needed for obstruction and incontinence and sometimes for vesicoureteral reflux.  Retrocaval Ureter: Anomalous development of the vena cava (pre-ureteric vena cava) allows the infrarenal vena cava to form anterior to the ureter (usually the right); a retrocaval ureter on the left occurs only with persistence of the left cardinal vein system or with complete situs inversus. Retrocaval ureter can cause ureteral obstruction. For significant ureteral obstruction, the ureter is surgically divided with uretero-ureteral anastomosis anterior to the vena cava or iliac vessel.  Ureter Duplication Anomalies: Partial or complete duplication of one or both ureters may occur with duplication of the ipsilateral renal pelvis. Ectopia or stenosis of one or both orifices, vesicoureteral reflux into the lower ureter or both ureters, and ureterocele may occur in complete duplication. Surgery may be necessary if there is obstruction, vesicoureteral reflux, or urinary incontinence. Incomplete duplication is rarely of clinical significance.   Ureteral Stenosis: Narrowing may occur at any location in the ureter, most frequently at the ureteropelvic junction and less commonly at the ureterovesical junction (primary megaureter). Consequences include infection, hematuria, and obstruction. Stenoses often diminish as the child grows. In primary megaureter, ureteral tapering and reimplantation may be needed when dilation increases or infection or obstruction occurs. In ureteropelvic junction obstruction, pyeloplasty (excision of the obstructed segment and reanastomosis) may be done by open, laparoscopic, or robotic techniques.  Ureterocele: Prolapse of the lower end of the ureter into the bladder with pinpoint obstruction may cause progressive ureterectasis, hydronephrosis, infection, occasional calculus formation, and impaired renal function. Ureterocele treatment options include endoscopic transurethral incision and open repair.  Polycystic Kidney Disease (PKD) Polycystic kidney disease (PKD) is a hereditary disorder of renal cyst formation causing gradual enlargement of both kidneys, sometimes with progression to renal failure.  Etiology: Inheritance of PKD is: - Autosomal dominant - Recessive - Sporadic (rare) Autosomal dominant polycystic kidney disease (ADPKD) has an incidence of 1/1000 and accounts for about 5% of patients with end-stage renal disease (ESRD) requiring renal replacement therapy. In contrast, autosomal recessive polycystic kidney disease is rare; incidence is 1/10,000. It frequently causes renal failure during childhood. -> Autosomal Dominant Polycystic Kidney Disease (ADPKD) Early in the disorder, tubules dilate and slowly fill with glomerular filtrate. Eventually, the tubules separate from the functioning nephron and fill with secreted rather than filtered fluid, forming cysts. Hemorrhage into cysts may occur, causing hematuria. Patients are also at higher risk of acute pyelonephritis, cyst infections, and urinary calculi. Vascular sclerosis and interstitial fibrosis eventually develop via unknown mechanisms and typically affect < 10% of tubules; nonetheless, renal failure develops in about 35-45% of patients by age 60. Extrarenal manifestations are common: - Hepatic cysts are present in most patients; these typically do not affect liver function. - Patients also have a higher incidence of pancreatic and intestinal cysts, colonic diverticula, and inguinal and abdominal wall hernias. - Valvular heart disorders (most often mitral valve prolapse and aortic regurgitation) can be detected by cardiac ultrasonography in 25-30% of patients; other valvular disorders may be due to collagen abnormalities. - Aortic regurgitation results from aortic root dilation due to arterial wall changes (including aortic aneurysm). - Coronary artery aneurysms occur. - Cerebral aneurysms are present in about 4% of young adults and up to 10% of elderly patients. Aneurysms rupture in 65-75% of patients, usually before age 50.  Signs & Symptoms: Autosomal dominant polycystic kidney disease usually causes no symptoms initially; one half of patients remain asymptomatic, never develop renal insufficiency or failure, and are never diagnosed. Most patients who develop symptoms do so by the end of their 20s. - Symptoms include low-grade flank, abdominal, and lower back pain due to cystic enlargement and symptoms of infection. - Acute pain, when it occurs, is usually due to hemorrhage into cysts or passage of a calculus. - Fever is common with acute pyelonephritis, and rupture of cysts into the retroperitoneal space may cause a fever that can last for weeks. - Hepatic cysts may cause right upper quadrant pain if they enlarge or become infected. - Signs and symptoms of unruptured cerebral aneurysm can be absent or may include headache, nausea and vomiting, and cranial nerve deficits; these manifestations warrant immediate intervention.   Investigations: USG, Sometimes CT scan, MRI or genetic testing The diagnosis of polycystic kidney disease is suspected in patients with the following: - A positive family history - Typical symptoms or signs - Cysts detected incidentally on imaging studies  Management: - Control of complications (e.g.: hypertension, infection, renal failure) - Supportive measures Strict control of hypertension is essential. Typically, an ACE inhibitor or angiotensin receptor blocker is used. In addition to controlling BP, these drugs help block angiotensin and aldosterone, growth factors that contribute to renal scarring and loss of renal function. Urinary tract infections (UTIs) should be treated promptly. Percutaneous aspiration of cysts may help relieve severe pain due to hemorrhage or compression but has no effect on long-term outcome. Nephrectomy is an option to relieve severe symptoms due to massive kidney enlargement or recurrent UTIs. Hemodialysis, peritoneal dialysis, or kidney transplantation is required in patients who develop chronic renal failure. Supportive measures include increased fluid intake (particularly water) to suppress vasopressin release, even if only partially, in patients who are able to safely excrete the load. -> Autosomal Recessive Polycystic Kidney Disease The kidneys are usually greatly enlarged and contain small cysts; renal failure is common in childhood. The liver is enlarged and has periportal fibrosis, bile duct proliferation, and scattered cysts. Diagnosis of autosomal recessive polycystic kidney disease may be difficult, especially without a family history. Ultrasonography may show renal or hepatic cysts; definitive diagnosis may require biopsy. Ultrasonography in late pregnancy usually allows presumptive in utero diagnosis. If postnatal ultrasonography is not definitive, MRI or CT may be diagnostic. Many neonates die in the first few days or weeks of life from pulmonary insufficiency. Most who survive develop progressive renal failure often requiring renal replacement therapy. When transplantation is done, hypersplenism must be controlled to obviate difficulty with hypersplenism-induced leukopenia, which increases the risk of systemic infection. Portal hypertension may be treated by portacaval or splenorenal shunts, which reduce morbidity but not mortality.  Renal Trauma The kidney is injured in up to 10% of patients who sustain significant abdominal trauma. Overall about 65% of genitourinary (GU) injuries involve the kidney. It is the most commonly injured GU organ from civilian external trauma. Most renal injuries (85-90% of cases) result from blunt trauma, typically due to motor vehicle crashes, falls, or assaults. Most injuries are low grade. The most common accompanying injuries are to the head, central nervous system, chest, spleen, and liver. Penetrating injuries usually result from gunshot wounds and are usually associated with multiple intra-abdominal injuries, most commonly to the chest, liver, intestine, and spleen. Renal injuries are classified according to severity into 5 grades: Grade 1: Subcapsular hematoma and/or renal contusion Grade 2: Laceration ≤ 1 cm in depth without urinary extravasation Grade 3: Laceration > 1 cm without urinary extravasation Grade 4: Laceration involving the collecting system with urinary extravasation; any segmental renal vascular injury; renal infarction; renal pelvis laceration and/or ureteropelvic disruption Grade 5: Shattered or devascularized kidney with active bleeding; main renal vascular laceration or avulsion  Investigations: - Clinical evaluation, including repeated vital sign determination - Urinalysis and hematocrit (Hct) - If a high-grade renal injury is suspected, contrast-enhanced CT with delayed images (done about 10-15 minutes after the initial study)  Management: Most blunt renal injuries, including all grade 1 and 2 and most grade 3 and 4 injuries, can be safely managed non-operatively. Patients should be maintained on strict bed rest until the gross hematuria has resolved. Prompt intervention is required for patients with the following: - Persistent bleeding (i.e. enough to necessitate repeated transfusions) - Expanding perinephric hematoma - Renal pedicle avulsion or other significant renovascular injuries - Ureteropelvic junction disruption Intervention can include surgery, stent placement, or selective angiographic embolization. Penetrating trauma usually requires surgical exploration, although observation may be appropriate for patients in whom the renal injury has been accurately staged by CT scan, blood pressure is stable, and no associated intra-abdominal injuries require surgery.  Ureteral Trauma Most ureteral injuries occur during surgery. Procedures that most often injure the ureter include ureteroscopy, hysterectomy, low anterior colon resection, and open abdominal aneurysm repair. Mechanisms include ligation, transection, avulsion, crush, devascularization, kinking, and electrocoagulation. Non-iatrogenic ureteral injuries account for only about 1-3% of all genitourinary trauma. They usually result from gunshot wounds and rarely from stab wounds. In children, avulsion injuries are more common and occur at the ureteropelvic junction. Complications include peritoneal or retroperitoneal urinary leakage; perinephric abscess; fistula formation; and ureteral stricture, obstruction, or both.  Investigations: Imaging, exploratory surgery, or both  Management: - For minor injuries, percutaneous nephrostomy tube or ureteral stent - For major injuries, surgical repair  Perinephric Abscess A perinephric abscess is a collection of pus that occurs due to a bacterial infection in the perinephric fat and fascia surrounding the kidney. Each kidney is encapsulated in a fibrous capsule, known as the renal capsule, and contained in an area called the perinephric space. Within this space, the kidneys are surrounded by a layer of perinephric fat that protects them from damage. Encompassing the fat layer is a tough, fibrous connective tissue called the renal fascia. The renal fascia towards the anterior (front of the body), is also referred to as Gerota’s fascia. A perinephric abscess often affects the renal capsule, and can extend to Gerota’s fascia, the abdominal cavity, and the pelvis.  Etiology: Most perinephric abscesses are caused by complications due to either a urinary tract infection or due to infections that occur in the kidneys, bladder, ureters, or urethra. Urologic infections, such as urinary tract infections and sexually transmitted infections, can be carried through the blood to the kidneys causing pyelonephritis, which refers to inflammation of the kidney due to bacterial infection. If pyelonephritis is chronic or recurring, this can lead to a collection of infected material and consequent cell death (necrosis) in the perinephric space, forming a perinephric abscess. Other causes may include trauma and infection spreading from other body organs including the liver, cervix, pancreas, gallbladder, and appendix.  Certain medical conditions can predispose individuals to perinephric abscesses. These include diabetes mellitus, pregnancy, urinary tract infection, and structural abnormalities in the urinary tract. Additionally, many individuals with a perinephric abscess have associated kidney stones, which can block the flow of urine and allow bacteria to accumulate in the perinephric space. Risk factors that might worsen outcomes include advanced age, diabetes mellitus, and renal insufficiency.  Signs & Symptoms: The severity of symptoms depends on the extent of the infection and the comorbidities of the individual. - Fever, chills, flank pain, abdominal pain, and fatigue. - The bacterial infection may travel to the groin or leg area, as well as to the right upper abdomen, where the liver is located, causing pain. - If the lungs are affected, this may also lead to pain in the chest. - Tenderness of the costovertebral area, located in the back at the bottom of the ribcage, is also common. - Inflammatory skin changes are sometimes present, and less commonly, dysuria or pain and discomfort with urination may occur.  Investigations: - Physical examination may present with a palpable mass - Blood tests, Urinalysis - CT scan, Sometimes USG  Management: - Antibiotics for 2-3 weeks - Some cases may require percutaneous catheter drainage Percutaneous catheter drainage utilizes imaging guidance (CT and ultrasound) to place a thin needle through the skin and into the perinephric abscess to first obtain a sample of the infected fluid. Then, a drainage catheter is left in place to drain the abscess fluid, which can take up to several days. Perinephric abscesses may be drained depending on the size of the infected area. A perinephric abscess larger than 3 cm is more likely to be drained, while an abscess smaller than 3 cm may initially be treated with an antibiotic. Drainage may also be performed if symptoms and radiological abnormalities persist after several days of appropriate antibiotic treatment. - If antibiotic medication and drainage do not successfully treat the abscess, urologic surgery may be required.  Renal Calculi / Kidney Stones / Nephrolithiasis Nephrolithiasis refers to the presence of stones within the kidneys. Cystolithis refers to stones in the urinary bladder. Ureterolithiasis, on the other hand, refers to stones within the ureter, and urolithiasis refers to stones in any part of the urinary tract (kidneys, ureter, bladder and urethra). Nephrolithiasis is one of the most common kidney diseases in adults. Stone formation occurs when there is an excess of crystal-forming substances that cannot be dissolved in the urine. High urinary excretion of certain substances; for instance, calcium, oxalate, uric acid, and cysteine; can promote stone formation, whereas the excretion of others, such as citrate, has a protective effect. Disturbances in urinary pH, a low urine volume, and a lack of protective substances that prevent the crystals from sticking together may contribute to stone formation. Additionally, environmental factors such as decreased fluid intake, hot climates, and dietary factors play an important role in the development of nephrolithiasis.  Types of Nephrolithiasis: 1) Hypocitraturic ̣Stones The vast majority of kidney stones are made out of calcium salts. Calcium oxalate stones are the most common type, followed by calcium phosphate stones. The main risk factor for developing calcium stones is an increased excretion of calcium (hypercalciuria) and oxalate (hyperoxaluria). These abnormalities can be idiopathic or can arise due to other systemic diseases, such as hyperparathyroidism, renal tubular acidosis, and malabsorption. 2) Struvite Stones Struvite stones are sometimes referred to as infectious stones, since they can be associated with infections of the urinary tract, especially those caused by urea-splitting organisms (Proteus mirabilis, Klebsiella, Staphylococcus, etc.) These bacteria split urea molecules into ammonium and CO2, thereby raising the urine’s pH to neutral or alkaline values, and ultimately leading to the precipitation of solutes, to which the bacteria can adhere. 3) Uric Acid Stones Uric acid stones generally develop due to increased excretion of uric acid (hyperuricosuria) and low urine pH. Risk factors include high-protein diets, gout, inflammatory bowel disease, genetic diseases, and diabetes. Uric acid stones are one of the few kidney stones that can be managed successfully through medical treatment that consists of adequate hydration and supplements to raise the urine’s pH.  4) Cystine Stones Cystine stones are generally caused by cystinuria, a hereditary disease that causes increased excretion of cystine in the urine, as well as low urinary pH. Cystine stones are very hard and can be seen as thin hexagonal crystals in a urine analysis. 5) Drug-induced Stones Drug-induced stones can develop by two mechanisms. In some cases, excessive use of laxatives or diuretics can contribute to metabolic abnormalities that ultimately lead to stone formation. On the other hand, certain medications, such as indinavir or ciprofloxacin, can crystallize in the urine and create stones.  Signs & Symptoms: depend on their size, shape, and location in the urinary tract. - Initially, the stones are lodged in the renal pelvis, where they can be asymptomatic and do not cause any pain or obstruction of the urine flow. - Kidney stones might move from the kidney into the ureter. The passage of the ureteral stone causes increased pressure inside the ureter, followed by spasms of the smooth muscle lining and distension of the walls. This typically presents as a renal colic, with severe flank pain of sudden onset that can irradiate to the inner thigh or the groin. Due to the intense pain, people may find themselves feeling restless or unable to find a comfortable position. Some individuals may even show signs of sweating, or have nausea and vomiting as a physiological response to the intense pain. In addition, stones can damage the ureter, causing hematuria. - When stones reach the bladder, they can irritate and obstruct the bladder, leading to frequent or painful urination. - Ultimately, small stones may spontaneously be eliminated through urine, relieving the pain.  - If the obstruction is not resolved, it can lead to severe complications, including chronic bladder discomfort, hydronephrosis, and a decrease in renal function due to urine build up. - Additionally, the obstructed kidney can become infected, causing obstructive pyelonephritis.  Diagnosis: - Asymptomatic nephrolithiasis may be detected as an incidental finding on imaging techniques or on urinalysis for hematuria. - On the other hand, symptomatic individuals are first assessed through physical examination. A common sign in people with kidney infection or kidney stones is Giordano’s sign, which refers to tenderness upon percussion to the costovertebral angle, formed by the angle between the 12th rib and the lumbar spine. - Next, kidney stones can be identified through imaging techniques, such as X-rays and non-contrast CT scans. The majority of calcium stones are visible on an abdominal X-ray, while some stones such as uric acid and cystine stones are radiolucent, meaning that they cannot be seen on an X-ray due to their composition. A non-contrast CT scan, however, is able to detect most stones, regardless of their size and composition, and can also help determine the type of stone by looking at its density - IVP: An intravenous pyelogram (IVP) is an X-ray examination of the kidneys, ureters and urinary bladder that uses iodinated contrast material injected into veins. It travels through the blood stream and collects in the kidneys and urinary tract, turning these areas bright white on the X-ray images. It allows to view and assess the anatomy and function of the above mentioned structures. IVP is used to detect kidney stones, enlarged prostate, tumors, scarring from urinary tract infection, or congenital anomalies. - An ultrasound is an alternative imaging technique which can be used to assess nephrolithiasis in vulnerable individuals like children or pregnant people. The presence of stones can be seen directly as a dense line with distal shadowing on the kidney or ureter, as well as indirectly by evaluating the dilation of the kidneys (hydronephrosis). For pregnant women with a high clinical suspicion and a negative ultrasound, an MRI can be used to identify kidney stones. - Once an acute episode has subsided, a metabolic evaluation can be recommended, especially for individuals with recurrent episodes of nephrolithiasis. A metabolic evaluation generally includes assessing the characteristics of a 24-hour urine sample and, when possible, analyzing the stone crystals found in the urine. Additionally, blood tests may be performed to detect metabolic abnormalities, such as high calcium and uric acid levels in the blood.  Management: - Pain control; NSAIDs to reduce the inflammation and ureteral spasms, intravenous fluids, and medications to help with nausea and vomiting. Individuals with persistent pain despite treatment can be given opioid medications. - Subsequent treatment depends on the size, composition, and location of the stone, as well as the presence of complications. For small stones (< 1 cm) without complications and effective pain treatment, expectant management (i.e. waiting for the stone to pass spontaneously) is appropriate. Most stones will pass over the course of four weeks. Some cases may be treated with medical expulsive therapy (MET), in which medication such as tamsulosin is given to relax the ureteral smooth muscle and help the stone pass more easily. Larger stones or those that are not able to pass despite MET should be considered for lithotripsy or surgical intervention. Lithotripsy, also known as extracorporeal shock wave lithotripsy (ESWL), is a non- invasive procedure that uses shock waves to fragment the stone, breaking it into smaller pieces that are excreted in the urine. Shock waves can only fragment low density stones, so hard stones like cysteine or calcium oxalate cannot be treated with lithotripsy. When lithotripsy is not possible, surgical removal of the stones is the preferred treatment. Stones can be removed either by ureteral endoscopy or through minimally-invasive surgery, as in percutaneous nephrolithotomy (PCNL). PCNL may be necessary for large stones (> 2 cm) or those that cannot be removed endoscopically because of their shape, such as staghorn stones. The surgery is performed by making a small 1 cm incision in the patient’s flank area. A tube is placed through the incision into the kidney under X-ray guidance. A small telescope is then passed through the tube in order to visualize the stone, break it up and remove it from the body. If necessary, a laser or a lithotripter may be used to break up the stone before it can be removed. In rare cases in which stones are unable to be removed by endoscopy or PCNL, open surgery such as ureterolithotomy or nephrolithotomy may be necessary. - Finally, many people that develop nephrolithiasis are at risk for future recurrence. Because of that, general dietary modifications such as increased fluid intake and decreased sodium and protein consumption may be recommended to prevent future stone formation. Additionally, individuals who develop hypocitraturic or uric acid stones may be recommended supplements to raise the urine’s pH, such as potassium citrate or bicarbonate. In individuals with struvite or infectious stones, removal of the stone should be followed by antibiotic treatment to eliminate the presence of urea- splitting organisms.  Renal Pelvic & Ureteral Cancers Cancers of the renal pelvis and ureters are usually transitional cell carcinomas (TCCs) and occasionally squamous cell carcinomas. TCC of the renal pelvis accounts for about 7-15% of all kidney tumors. TCC of the ureters accounts for about 4% of upper tract tumors. Risk factors are the same as those for bladder cancer (smoking, excess phenacetin use, long-term cyclophosphamide use, chronic irritation, exposure to certain chemicals). Also, inhabitants of the Balkans with endemic familial nephropathy are inexplicably predisposed to develop upper tract TCC.  Signs & Symptoms: Most patients present with hematuria; dysuria and frequency may occur if the bladder also is involved. Colicky pain may accompany obstruction. Uncommonly, hydronephrosis results from a renal pelvic tumor.  Investigations: - USG or CT with contrast - Cytology or Histology  Management: - Radical nephroureterectomy, including excision of a cuff of bladder and regional lymphadenectomy - Neoadjuvant chemotherapy prior to nephroureterectomy is recommended for high-grade and high-stage lesions because other treatments can decrease renal function, often preventing subsequent use of adjuvant chemotherapy. - Post-treatment surveillance with cystoscopy  Renal Cell Carcinoma / Adenocarcinoma of the Kidneys Renal cell carcinoma (RCC) is the most common renal cancer. RCC, an adenocarcinoma, accounts for 90-95% of primary malignant renal tumors. RCC metastasizes most often to the lymph nodes, lungs, adrenal glands, liver, brain, and bone. Less common primary renal tumors include transitional cell carcinoma, Wilm’s tumor (most often in children), and sarcoma. Risk factors include: - Smoking - Obesity - Excess use of phenacetin - Acquired cystic kidney disease in dialysis patients - Exposure to certain radiopaque contrast agents, asbestos, cadmium, and leather tanning and petroleum products - Some familial syndromes, particularly von Hippel–Lindau disease   Signs & Symptoms: - Symptoms usually do not appear until late, when the tumor may already be large and metastatic. - Gross or microscopic hematuria is the most common manifestation, followed by flank pain, fever of unknown origin (FUO), and a palpable mass. - Sometimes hypertension results from segmental ischemia or pedicle compression. - Paraneoplastic syndromes occur in 20% of patients. - Hypercalcemia is common and may require treatment.  Investigations: CT with contrast or MRI  Management: - Radical nephrectomy (removal of kidney, adrenal gland, perirenal fat, and Gerota fascia) is standard treatment for localized RCC and provides a reasonable chance for cure. Results with open or laparoscopic procedures are comparable; recovery is easier with laparoscopic procedures. - Nephron-sparing surgery (partial nephrectomy) is possible and appropriate for many patients, even in patients with a normal contralateral kidney if the tumor is < 4 to 7 cm. Partial nephrectomy is gaining popularity because it results in a lower incidence of chronic kidney disease than radical nephrectomy. - Palliation can include nephrectomy, tumor embolization, and possibly external beam radiation therapy. For some patients, drug therapy reduces tumor size and prolongs life  Wilm’s Tumor / Nephroblastoma Wilm’s tumor is an embryonal cancer of the kidney composed of blastemal, stromal, and epithelial elements. Genetic abnormalities have been implicated in the pathogenesis, but familial inheritance accounts for only 1-2% of cases. Wilm’s tumor usually manifests in children < 5 year of age but occasionally in older children and rarely in adults. Wilm’s tumor accounts for about 6% of cancers in children < 15 years of age.  Signs & Symptoms: - The most frequent finding is a painless, palpable abdominal mass. - Less frequent findings include abdominal pain, hematuria, fever, anorexia, nausea, and vomiting. - Hematuria can be microscopic or gross. - Hypertension may occur and is of variable severity.  Investigations: Abdominal ultrasonography, CT, or MRI; During surgery, locoregional lymph nodes are sampled for pathologic and surgical staging.  Management: Surgery and chemotherapy, Radiation therapy for patients with higher stage/risk disease  CHAPTER Vi: Diseases of urinary bladder  Congenital Anomalies of Urinary Bladder Congenital urinary bladder anomalies often occur without other genitourinary abnormalities. They may cause infection, retention, incontinence, and reflux. Symptomatic anomalies may require surgery.  Bladder Diverticulum: A bladder diverticulum is a herniation of the bladder mucosa through a defect in bladder muscle. It predisposes to urinary tract infections (UTIs) and may coexist with vesicoureteral reflux. It is usually discovered during evaluation of recurrent UTIs in young children. Diagnosis of bladder diverticulum is by voiding cystourethrography. Surgical removal of the diverticulum and reconstruction of the bladder wall may be necessary.  Bladder Exstrophy: In exstrophy, there is a failure of midline closure from the umbilicus to the perineum, resulting in bladder mucosa continuity with the abdominal skin, separation of the pubic symphysis, and epispadias or bifid genitalia. The bladder is open suprapubically, and urine drips from the open bladder rather than through the urethra. Despite the seriousness of the deformity, normal renal function usually is maintained. The bladder can usually be reconstructed and returned to the pelvis, although vesicoureteral reflux invariably occurs and is managed as needed. Additional surgical intervention may be necessary to treat a bladder reservoir that fails to expand sufficiently or has sphincter insufficiency. Reconstruction of the genitals is required.  Megacystis Syndrome: In this syndrome, a large, thin-walled, smooth bladder without evident outlet obstruction develops, usually in girls. Megacystis syndrome is poorly understood. The syndrome may be a manifestation of a primary myoneural defect, especially when intestinal obstruction (e.g.: megacystis-microcolon, intestinal hypoperistalsis syndrome) is also present. Symptoms are related to UTIs, and vesicoureteral reflux is common. Ultrasonography with the bladder empty may disclose normal-appearing upper tracts, but voiding cystourethrography may show reflux with massive upper tract dilation. Ureteral reimplantation may be effective, although some patients benefit from antibacterial prophylaxis, timed voiding with behavioral modification, intermittent catheterization, or a combination.   Neurogenic Bladder: Neurogenic bladder is bladder dysfunction caused by neurologic disorders, including spinal cord or central nervous system abnormalities, trauma, or the sequelae of pelvic surgery (e.g.: for sacrococcygeal teratoma or imperforate anus). The bladder may be flaccid, spastic, or a combination. A flaccid bladder has high- volume, low-pressure, and minimal contractions. A spastic bladder has normal or low-volume, high-pressure, and involuntary contractions. When present, chronically elevated bladder pressure often causes progressive kidney damage, even without infection or reflux. Manifestations include recurrent UTIs, urinary retention and/or incontinence, and potentially renal insufficiency. Management goals include lowering risk of infection, maintaining adequate bladder storage pressure and volume, effective bladder emptying, and achieving social continence. Treatment of neurogenic bladder includes drugs (e.g.: anticholinergics, prophylactic antibiotics), intermittent catheterization, and/or surgical intervention (e.g.: augmentation cystoplasty, appendicovesicostomy, botulinum toxin injections, neurostimulation). Children with neurogenic bladder often also have a neurogenic bowel with constipation and stool incontinence that also require proper management.  Urinary Bladder Trauma External bladder injuries are caused by either blunt or penetrating trauma to the lower abdomen, pelvis, or perineum. Blunt trauma is the more common mechanism, usually by a sudden deceleration, such as in a high-speed motor vehicle crash or fall, or from an external blow to the lower abdomen. The most frequently accompanying injury is a pelvic fracture, occurring in > 95% of bladder ruptures caused by blunt trauma. Other concomitant injuries include long bone fractures and central nervous system and chest injuries. Penetrating injuries, most often gunshot wounds, account for < 10% of bladder injuries. The bladder is the most frequently injured organ during pelvic surgery. Such injuries can occur during transurethral surgery, gynecologic procedures (most commonly abdominal hysterectomy, cesarean section, pelvic mass excision), or colon resection. Predisposing factors include scarring from prior surgery or radiation therapy, inflammation, and extensive tumor burden. Bladder injuries are classified as contusions or ruptures based on the extent of injury seen radiographically. Ruptures can be extraperitoneal, intraperitoneal, or both; most are extraperitoneal. Complications of bladder injuries include urinary ascites (free urine in the peritoneal cavity) due to intraperitoneal rupture, infection (including sepsis), persistent hematuria, incontinence, bladder instability, and fistula. Mortality with bladder rupture from external trauma can approach 20%; this is due to concomitant organ injuries rather than the bladder injury.  Signs & Symptoms: - Suprapubic pain, tenderness, distension - Inability to void urine - Hematuria - Hypovolemic shock (due to hemorrhage) - Blunt bladder ruptures almost always present with a pelvic fracture and gross hematuria. - Bladder injuries occurring during surgery are usually identified intraoperatively. Findings can include urinary extravasation, a sudden increase in bleeding, appearance of the bladder catheter in the wound, and, during laparoscopy, distention of the urinary drainage bag with gas.  Investigations: - Retrograde cystography with plain film x-rays or CT - A rectal examination should be done in all patients with a blunt or penetrating mechanism of injury to assess for blood, which is highly suggestive of a concomitant bowel injury. - Additionally, female patients should undergo a thorough pelvic examination to assess for vaginal involvement.  Management: - Catheter drainage - Sometimes surgical repair Bladder contusions require only catheter drainage until gross hematuria resolves. Most extraperitoneal ruptures require only catheter drainage if urine is draining freely and the bladder neck is spared. With bladder neck involvement, surgical exploration and repair are required to limit the likelihood of incontinence. Similarly, surgical management is indicated in cases of persistent gross hematuria, clot retention, or concomitant rectal or vaginal injury. All penetrating trauma and intraperitoneal ruptures due to blunt trauma require surgical exploration. Most bladder injuries occurring during surgery are identified and repaired intraoperatively.  Ashmarī -> Refer to Kāyachikitsā, Paper II, Part B, Chapter IV: Mūtravaha Srotas Vikāra  Vesical Calculi / Bladder Stones / Cystolithis Vescial calculi are caused by a buildup of minerals. They can occur if the bladder is not completely emptied after urination. Eventually, the leftover urine becomes concentrated and minerals within the liquid turn into crystals. Sometimes, these stones will be passed with urune while they are still small. Other times, bladder stones can get stuck to the wall of the bladder. If this happens, they gradually gather more mineral crystals, becoming larger over time. The smallest bladder stones are barely visible to the naked eye, but some can grow to an impressive size. The largest bladder stone, according to Guinness World Records, weighed almost 1.9 kg and measured 17.9 x 12.7 x 9.5 centimeters.  Etiology & Risk Factors: - Bladder stones start to grow when urine is left in the bladder after urinating. This is often due to an underlying medical condition that prevents complete evacuation, such as neurogenic bladder, prostate enlargement, cystitis, nephrolithiasis, bladder diverticula, cystocele. - Males are at higher risk of developing bladder stones, especially with age.  Signs & Symptoms: Bladder stones may not produce symptoms straight away. But, if the stones grow large enough, they may irritate the bladder or urethra. Symptoms can include the following: - Discomfort or pain in the penis for males - Urination with discomfort and pain - Lower abdominal pain - Frequent urination - Difficult urination or interrupted flow - Starting a stream during urination takes longer - Hematuria - Cloudy or abnormally dark urine  Diagnosis: Physical examination, Urinalysis, CT scan, USG, X-ray, IVP  Management: - Intake of plenty water is sufficient to expel small bladder stones. - Large calculi may require cystolitholapaxy or surgical removal. During a cystolitholapaxy, an instrument called a cystoscope is inserted into the bladder to locate the bladder stone or stones. A laser is used to break up the stones into smaller fragments which are then removed.  Cystitis Cystitis is infectious inflammation of the urinary bladder. It is categorized under lower urinary tract infections. About 95% of cystitis occur when bacteria ascend the urethra to the bladder. It is common in women, in whom cases of uncomplicated cystitis are usually preceded by sexual intercourse (honeymoon cystitis). In men, bacterial infection of the bladder is usually complicated and usually results from ascending infection from the urethra or prostate or is secondary to urethral instrumentation. The most common cause of recurrent cystitis in men is chronic bacterial prostatitis.  Etiology: The bacteria that most often cause cystitis (and pyelonephritis) are the following: - Enteric, usually gram-negative aerobic bacteria (most often) - Gram-positive bacteria (less often) In normal genitourinary tracts, strains of Escherichia coli with specific attachment factors for transitional epithelium of the bladder and ureters account for 75-95% of cases. The remaining gram-negative urinary pathogens are usually other enterobacteria, typically Klebsiella or Proteus mirabilis, and occasionally Pseudomonas aeruginosa. Among gram-positive bacteria, Staphylococcus saprophyticus is isolated in 5-10% of bacterial UTIs. Less common gram-positive bacterial isolates are Enterococcus faecalis (group D streptococci) and Streptococcus agalactiae (group B streptococci), which may be contaminants, particularly if they were isolated from patients with uncomplicated cystitis. In hospitalized patients, E. coli accounts for about 50% of cases. The gram-negative species Klebsiella, Proteus, Enterobacter, Pseudomonas, and Serratia account for about 40%, and the gram-positive bacterial cocci E. faecalis, S. saprophyticus, and Staphylococcus aureus account for the remainder.  Risk Factors: Risk factors for development of UTI in women include the following: - Sexual intercourse - Diaphragm and spermicide use - Antibiotic use - New sex partner within the past year - History of UTIs in 1st-degree female relatives - History of recurrent UTIs - First UTI at early age - Even use of spermicide-coated condoms increases risk of UTI in women. The increased risk of UTI in women using antibiotics or spermicides probably occurs because of alterations in vaginal flora that allow overgrowth of Escherichia coli. In older women, soiling of the perineum due to fecal incontinence increases risk.  - Anatomic, structural, and functional abnormalities are risk factors for UTI. A common consequence of anatomic abnormality is vesicoureteral reflux (VUR), which is present in 30-45% of young children with symptomatic UTI. VUR is usually caused by a congenital defect that results in incompetence of the ureterovesical valve. VUR can also be acquired in patients with a flaccid bladder due to spinal cord injury or after urinary tract surgery. Other anatomic abnormalities predisposing to UTI include urethral valves (a congenital obstructive abnormality), delayed bladder neck maturation, bladder diverticulum, and urethral duplications. - Structural and functional urinary tract abnormalities that predispose to UTI usually involve obstruction of urine flow and poor bladder emptying. Urine flow can be compromised by calculi and tumors. Bladder emptying can be impaired by neurogenic dysfunction, pregnancy, uterine prolapse, cystocele, and prostatic enlargement. - Other risk factors for UTI include instrumentation (e.g.: bladder catheterization, stent placement, cystoscopy) and recent surgery.  Signs & Symptoms: - Cystitis onset is usually sudden - Frequency, urgency, and burning or painful voiding of small volumes of urine. - Nocturia, with suprapubic pain and often low back pain, is common. - Urine is often turbid, and microscopic (or rarely gross) - Hematuria can occur - Pneumaturia (passage of air in the urine) can occur when infection results from a vesicoenteric or vesicovaginal fistula or from emphysematous cystitis. - Low-grade fever may develop  Investigations: Urinalysis, Urine culture, Cystoscopy  Management: - Antibiotics - Occasionally surgery (e.g.: to drain abscesses, correct underlying structural abnormalities, or relieve obstruction such as calculi) First-line treatment of uncomplicated cystitis is nitrofurantoin 100 mg orally twice a day for 5 days (it is contraindicated if creatinine clearance is < 60 mL/min), trimethoprim/sulfamethoxazole (TMP/SMX) 160/800 mg orally twice a day for 3 days, or fosfomycin 3 gm orally once. If cystitis recurs within a week or two, a broader spectrum antibiotic (e.g.: a fluoroquinolone) can be used and the urine should be cultured. Complicated cystitis should be treated with empiric broad-spectrum antibiotics chosen based on local pathogens and resistance patterns and adjusted based on culture results. Urinary tract abnormalities must also be managed.  Interstitial Cystitis / Bladder Pain Syndrome Interstitial cystitis is non-infectious bladder inflammation. Cause is unknown, but pathophysiology may involve loss of protective urothelial mucin, with penetration of urinary potassium and other substances into the bladder wall, activation of sensory nerves, and smooth muscle damage. Mast cells may mediate the process, but their role is unclear. Symptoms include: pain (suprapubic, pelvic, and abdominal), urinary frequency, and urgency with incontinence. Diagnosis is by history and exclusion of other disorders clinically and by cystoscopy and biopsy. Treatment varies but includes dietary changes, bladder training, pentosan, analgesics, and intravesical therapies. With treatment, most patients improve, but cure is rare.  Urinary Bladder Cancer Bladder cancer is usually transitional cell (urothelial) carcinoma. Patients usually present with hematuria (most commonly) or irritative voiding symptoms such as frequency and/or urgency; later, urinary obstruction can cause pain. Risk factors include: - Smoking (the most common risk factor, causing ≥ 50% of new cases) - Excess phenacetin use (analgesic abuse) - Long-term cyclophosphamide use - Chronic irritation (e.g.: in schistosomiasis, by chronic catheterization, or bladder calculi) - Exposure to hydrocarbons, tryptophan metabolites, or industrial chemicals, notably aromatic amines (aniline dyes, such as naphthylamine used in the dye industry) and chemicals used in the rubber, electric, cable, paint, and textile industries.  Types: 1) Transitional cell carcinomas (urothelial carcinoma), which account for > 90% of bladder cancers. Most are papillary carcinomas, which tend to be superficial and well differentiated and to grow outward; sessile tumors are more insidious, tending to invade early and m