SURGERYFinals - 3.1. Pancreas and Spleen

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AUFMED

Dr. Nelson Ayson | October 3, 2015 | SURGERY

THE PANCREAS and SPLEEN

QUIZ ON APPENDICITIS 1. Who coined the word ‘appendicitis’? Reginald Fitz 2. Who first described the McBurney’s incision? McArthur 3. What is the longest recorded length of the appendix? >30 cm 4. Based on the Alvarado’s Scoring, what sign has the highest score? Leukocytosis ≥ 10x109 cells/L 5. Appendectomy done before age 20 was reported to have a protective effect on what disease? Ulcerative colitis 6. Based on the Appendicitis Inflammatory Response Score, what finding has the highest point (3 points)? Strong rebound tenderness 7. What is a common congenital anomaly that can only be ruled out intraoperatively? Meckel’s Diverticulum 8. When should you consider acute appendicitis to a patient? Patient presents with RLQ abdominal pain 9. At what week of embryological development does the appendix appear? 8th week 10. What is the normal luminal content of an appendix in ml? 0.1 mL 

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PANCREAS most unforgiving organ unforgiving due to its tendency to be inflamed and subsequently injury secondary to even the slightest trauma during surgery, e.g., during cholecystectomy, palpation of the head of the pancreas is necessary to rule out common bile duct stones, especially at the distal segments of the pancreas; too much palpation may result to pancreatitis later. weighs 75-100 gm length 15-20 cm long Dorsal Bud (Santorini) Ventral Bud (Wirsung) has 4 parts: head, neck, body, and tail

NEUROANATOMY  Acinar Cells – exocrine function  Islet Cells – endocrine function  Islet Vasculature – both innervated by para and sympathetic system  Parasympathetic – stimulatory  Sympathetic – inhibitory

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EXOCRINE PANCREAS  85% of function  50-800 ml/day  colorless, odorless, alkaline (because of its high Bicarbonate content), isosmotic pancreatic juice  Acinar (enzymes) and duct cells (electrolytes & H2O)

Islets of Langerhans ACINAR CELLS  Secrete all types of enzymes for the digestion of carbohydrates, proteins, and fats  Pyramid shape  Amylase, protease and lipase A. PANCREATIC AMYLASE Active form Hydrolyzes starch and glucagon to glucose, maltose, maltotriose and dextrin Stimulated by CCK releasing peptide, CCK, secretin B. PROTEOLYTIC ENZYMES Inactive form Activation of Trypsinogen by Enterokinases = Trypsin Trypsin activates other proteolytic enzymes

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C. PANCREATIC LIPASE Active form (lipase is actually secreted in its final form, yet it only becomes actively functional in the presence of colipase in the duodenum) Colipase - binds lipase to enhance lipase Phospholilpase A2 – bile salts to be active CENTROACINAR & INTERCALATED DUCT CELLS  Secretes water and electrolytes  Carbonic anhydrase needed in bicarbonate secretion  Secretin – major stimulant for bicarbonate secretions (carbonic anhydrase is necessary for bicarbonate production)

Second phase: longer, sustained, release due to ongoing production of new insulin 

Type I DM Insulin insensitivity, Overexpression or upregualtion of insulin receptors = increase sensitivity

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Type II DM Insulin resistance PANCREATIC ISLET PEPTIDE PRODUCTS HORMONE CELL FUNCTION Glucagon

Alpha

Insulin

Amylin (IAPP)

ENDOCRINE PANCREAS  1 million islet of Langerhans  Alpha cells – glucagon  Beta cells – insulin  Delta cells – stomatostatin  Epsilon cells – ghrelin  PP cells – pancreatic polypeptide  Comprise only 2% of the overall pancreatic function

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Stimulated by: Glucagon GIP, GLP-1 CCK Cholinergic and beta sympathetic fibers Inhibited by (PASA) Pancreastatin Amylin Somatostatin Alpha sympathetic fibers Phases of insulin secretion First phase: stored insulin is released

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Decreased gluconeogenesis, glycogenolysis, fatty acid breakdown, and ketogenesis Increased glycogenesis, protein synthesis Counter regulates insulin secretion and function Decreases insulin and somatostatin release Increases glucagon release Decreases pancreatic exocrine secretion

Pancreatitin

Islets of Langerhans

INSULIN  Best studied pancreatic hormone  Influenced by: arginine, lysine, leucine, and fatty acid

Beta

Opposite effects of insulin; increased hepatic glycogenolysis and gluconeogenesis

Inhibits GI secretion; Inhibits cell growth

Somatostatin

Delta

Ghrelin

Epsilon

Decreases insulin release and insulin

PP

Inhibits pancreatic exocrine and secretion of insulin; Facilitates hepatic effect of insulin

Pancreatic Polypeptide

ACUTE PANCREATITIS   

Surgery will always be the last resort for the management of pancreatitis Inflammatory disease of the pancreas that is associated with little or no fibrosis of the gland Complications Septic Shock Respiratory failure Renal failure PATHOGENESIS OF RENAL FAILURE Massive fluid derangement (hypovolemia)  hypotension acute tubular necrosis  renal failure

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Most common cause (80-90%) Biliary tract stone disease  Gallstone Pancreatitis

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Related to the Common Theory Channel; if a stone from the gallbladder falls and obstructs the distal common channel prior to its exit to the duodenum. the location of the stone, cauing obstruction at the “Y” junction, may cause reflux of pancreatic juices. Another theory, the Laxity of the Sphincter of Oddi, says that upon dislodgement of a stone, the sphincter becomes lax, allowing the reflux of even bile and gastric acids to the pancreas; Another theory, the blockade of the pancreatic duct itself. Alcoholism 

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Other causes (10-20%) trauma surgery

Transverse Colectomy secondary to the traction of a segment of the colon which is anatomically related to the pancreas via all connecting vasculatures. There will be bleeding eventually, which will later require pressure. Splenectomy may also put the pancreas in danger because of the relationship of the tail to the spleen. Nephrectomy on the right may also injure the pancreas -

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drugs hereditary infection toxins drugs: thiazides diuretics, furosemide, estrogens, azathioprine, L-aspariginase, 6-mercaptopurine, methylodopa, sulfonamide; tetracycline, pentamidine, procainamide, ntirofurantoin, dideoxyinosine, valproic acid, acetylcholinesterase inhibitors

o Alcoholism Ethanol, which is the main ingredient among alcoholic beverages, is known to be toxic to pancreatic cells. It destroys the acinar cells. There is also a theory wherein there is a ‘burst ‘of stimulatory effect (parasympathetic) then eventually, there be an inhibitory effect. The burst secretory effect + the effect of alcohol into the sphincter of Oddi (which leads to spasm) will eventually close the sphincter of Oddi. It also increases the permeability of enzymes, thus, they seep out outside the pancreatic tree. So as once they are in the parenchyma, these pro-enzymes become activated. They also found out that ethanol diminishes the secretion of bicarbonate thereby reducing the alkalinity of the pancreatic juice. It also increases the protein content of the pancreatic juice, thus, allowing it to form plugs within the ducts of the pancreas. That is why some patients would have multiple duct obstruction. You will consider gallstone pancreatitis if you have a very good patient and they were able to retrieve within a week stones from the feces. This is what the book says. In reality, we do not really look at our feces. The size of these stones are only very small (8 mm) as they have passed out through the sphincter of Oddi. If they are larger in size, suspect perforation as it is possible that the distal common bile duct (intra-pancreatic) had a fistula with your duodenum. -

Symptoms Low grade fever Severe epigastric pain radiating to the back (retroperitoneal organ) Abdominal tenderness, nausea, emesis and leukocytosis Alterations in Amylase levels  Less specific  Perforated duodenal ulcer  SB obstruction  Renal failure  Salivary disease Change in Lipase levels  Longer ½ half  100% sensitivity and specificity

Other causes (10-20%) o Trauma o Surgery o Drugs o Hereditary o Infection o Toxins o Drugs o Thiazides diuretics, furosemide, estrogens, azathioprine, L-aspariginase, 6-mercaptopurine, methylodopa, sulfonamides o Tetracycline, pentamidine, procainamide, nitrofurantoin, dideoxyinosine, valproic acid, acetylcholinesterase inhibitors Always establish whether the patient may be allergic or not to medications prior to their administration. Always ask the history before prescribing.

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Diagnostics Ultrasound Endoscopic Ultrasound MRCP (Magnetic cholangiopancreatography) CT Scan

Symptoms o Low grade fever o Severe epigastric pain radiating to the back (retroperitoneal organ) o Abdominal tenderness, nausea, emesis and leucocytosis

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Amylase

resonance

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Less specific Perforated duodenal ulcer SB obstruction Renal failure Salivary disease

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Lipase o Longer ½ half o 100% sensitivity and specificity It is more specific than amylase. Lipase may also be elevated in several conditions such as salivary disease and small bowel obstruction.

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Diagnostics o Ultrasound Less invasive, relatively available, and cheap o Endoscopic Ultrasound Used nowadays; more accurate than ordinary ultrasound o MRCP (Magnetic Resonance Cholangiopancreatography) o CT Scan Use MRCP and CT Scan if you are contemplating for complications.

We see the right and left hepatic duct, common hepatic duct, cystic duct (CHD + CD = CBD). The sphincter of Oddi opens at the second portion, medial aspect of the duodenum. It is a smooth muscle sphincter which controls the flow of bile and pancreatic juice into the ampulla of Vater (or hepato-pancreatic ampulla as shown in the picture). Definitely, larger stones will not fall to the CBD (5 mm) because the normal size of the cystic duct measures (2-3 mm).

“COMMON CHANNEL THEORY”: The common channel theory suggests that bile reflux, through a common biliopancreatic channel, triggers acute pancreatitis (Lerch, et al, 1993). The common terminal conduit between the pancreatic duct and the bile duct is, if present at all, much too short in most humans to allow for communication between the two ducts (Opie's common channel) when a gallstone is impacted at the papilla. Even if such a common channel were created not by the stone itself but instead by the sphincter stenosis arising from the passage of the stone, the pancreatic secretory pressure would still exceed the pressure of the bile duct. As a consequence, reflux of pancreatic juice into the biliary tract would result, rather than reflux of bile into the pancreas (NEJM article of Acute Pancreatitis, 1994).

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Multifaceted Gallstone

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*Shows pictures of stones retrieved from actual patients.* This is what you call multifaceted stones (yung parang dice). What is its clinical significance? They are attached to each other prior to breaking.

In common practice, an ERCP is usually directed to the common bile duct.

*Shows picture of the longest gallbladder which he removed.* QUESTION: What is the significance of counting the stones intra-operatively? ANSWER: To verify if you were able to remove all the stones completely as shown among the ancillary procedures done prior to surgery. But, we should also be discrete in reading the results of the ultrasonography as it may have several limitations (intestines filled with gas may obstruct the view of the CBD) Common bile duct obstruction may present with jaundice. To further verify presence of residual stones, an intraoperative cholangiography (IOC) is requested. A contrast material is injected into the cystic duct thru a cannula then an x-ray film of the abdomen is taken. A 1:1 ratio or dilution (of contrast media and normal saline) is used so as to prevent wiping out of small stones. (ex. 15 cc of contrast solution + 15 cc of NSS)

CT-scan of a patient with at least 3 stones in the gallbladder -

Ranson’s Criteria: o Percentage of mortality On admission Within 48 hours Age>55 years Drop in Hct.10% WBC count Fluid deficit >6L >16,000/mm3 Serum glucose: Serum ca 200 mg/dl Serum LDH >350 Hypoxemia (pO2 mg/dl 250 BUN >50 mg/dl IU/L Albumin 50%

In old practice, acute pancreatitis is managed first by ‘resting the pancreas’. However, this is already obsolete as patients who can tolerate eating may be fed. This is an endoscopic instrument doing a sphincteroplasty. It will cut the sphincter of Oddi to dilate the opening.

GENERAL MANAGEMENT If there is absence of rebound tenderness, or muscle guarding, a normal creatining and hematocrit, the patient will not require ICU admission. 

Endoscopic Retrograde Cholangiopancreatography

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Vasoactive Phase o This is the hemodynamics of the patient. You should be very aggressive in resuscitating these patients with fluid plus antibiotics IF NEEDED. Antibiotics are given in a case to case basis. o 1st 2 weeks o Consequences of SIRS (can lead to Multi organ Dysfunction, leading to multi organ failure, eventually leading to death)

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SIRS is the initial response of the body to any inflammation. If you did not correct that problem, the patient will go into MODS (multi organ dysfunction) and if it still not corrected, patient will go into MOFS (multi organ failure) then death.

Late Septic Phase o Infection of pancreatic necrosis and sepsis

A. Resuscitation and Supportive Measure  Surgery: not main treatment  IVF >250 ml/hr  Close monitoring o Respiratory, CV and renal function o IFC and O2 Sat monitoring (PAC/CVP) (PAC- pulmonary artery catheterization. CVP- central venous pressure. This is done because we want to watch out for overhydrationor pulmonary congestion)  Pain Control o Narcotics o Epidural catheter We never give morphine because it causes spasm of the sphincter of oddi because pancreatitis will become worse. 

NGT o o

- Management of Necrosis and Infection  Initial CT scan: 1st week o Focal/diffuse well-marginated zones of nonenhanced pancreatic parenchyma >3 cm or occupying >30 % of the gland  Surgical Indications SURGERY IS THE LAST OPTION! o Documented pancreatic infection (CT guided FNAB) o Perforated viscus Pancreatic Debridement o Removal of all devitalized tissue Debridement is done through suction because it might bleed. Direct pressure cannot be applied because the remaining pancreatic tissue is easily injured and pancreatitis will be aggravated.

Prevent emesis and aspiration Pneumonia

B. Nutrition  NPO – you can feed the patient if your patient can tolerate. If the patient cannot, through fluoroscopic guidance, you thread the NGT up to the jejunum then feed the patient. Too aggressive early feeding done without adequately resuscitating the patient first, you will push the patient into death.  

Saponification TPN Enteral: naso jejunal tube feeding

C. ERCP  Pancreatitis – one of the major complication This is done in very few selected patients wherein the persistence of obstruction is noted, you do not have any choice but to perform ERCP. D. Prophylactic Antibiotics Now, antibiotics is withheld if there is no evidence of infection. If you are going to give them, it is a minimum of 7 days. Broad spectrum antibiotics are used.  7-10 days  Imipenem

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Assurance of post op removal of any products of ongoing inflammation that persist after debridement o Hemostatisis o Blunt dissection o Post op high volume lavage (2L/hr) Goals of pancreatic debridement o First: extensive and gentle necrosectomy is undertaken o Second: etiology of pancreatitis is addressed, and cholecystectomy is performed if indicated and safe If the patient is stable enough, and there are gallstones, cholecystectomy is performed. If the patient is not stable during your operation, do not

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do the surgery tied over the patient in the same hospital event, before discharging the patient you may do the cholecystectomy. o Third: nutritional needs of the patient are addressed with enteral tube positioning o Fourth: closed-suction drainage of the exudative process and persistent pancreatic duct leak is established

possible as the patient has recovered; ideally during the same hospital admission o

In severe gallstone associated with acute pancreatitis, cholecystectomy should be delayed until there is sufficient resolution of the inflammatory response and clinical recovery

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Endoscopic sphinctereotomy – alternative to cholecystectomy in those who are not fit to undergo surgery Its main goal is to relieve the pressure from below.

International Association of Pancreatology: Guidelines for the Surgical Management of Acute Pancreatitis o

Mild acute pancreatitis is not an indication of pancreatic surgery

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The use of prophylactic broad-spectrum antibiotics reduces infection rates in CT proved necrotizing pancreatitis

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FNA for bacteriology should be performed to differentiate between sterile and infected pancreatic necrosis in patients with sepsis syndrome

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Infected pancreatic necrosis in patients with clinical signs and symptoms of sepsis is an indication for intervention including surgery and radiologic drainage

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Patients with sterile pancreatic necrosis should be managed conservatively and should undergo intervention only in selected cases (FNA is used to evaluate if sterile or not.)

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Early surgery within 14 days after onset of the disease is not recommended in patients with necrotizing pancreatitis unless there are specific indications. The patient may die of complications such as bleeding and further pancreatitis. If you are going to perform surgical debridement, your main goal is to preserve as much but remove as much if needed.

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Surgical and other forms of interventional management should favor an organ-preserving approach, which involves debridement or necrosecotmy combined with post op management concept that maximizes postop evacuation of retroperitoneal debris and exudates Cholecystectomy should be performed to avoid recurrence of gallstone associated with acute pancreatitis In mild gallstone associated with acute pacnreatitis, cholecystectomy should be performed as soon as

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CHRONIC PANCREATITIS Same signs and symptoms as acute but look out for complications. The patient feels diabetic because many islet cells may have been destroyed. Malabsorption may occur. Much of the enzymes are destroyed as well. - Ongoing inflammatory process characterized by irreversible destruction of the pancreas parenchyma and ductal architectures - Abdominal pain radiating to the back, nausea, vomiting and weight loss - Diabetes, absorption with steatorrhea and biliary obstruction - CT Scan, ERCP, MRCP, EUS -

Indications for therapeutic endoscopy  Chronic pain  Obstruction of the main pancreatic duct  Pseudocyst  Distal common bile duct stricture

- Indications for surgery in chronic pancreatitis  Chronic abdominal pain  Suspicion of pancreatic cancer  Persistent CBD obstruction unresponsive to endoscopic therapy  Duodenal obstruction  Splenic vein thrombosis with bleeding pancreatic pseudocyst  Persistent pancreatic ascites or fistula All patients complaining of chronic abdominal pain and you are contemplating of pancreatitis, you always consider the possibility of pancreatic cancer. Tumors may be the ones obstructing. Diagnostic procedures must be done such as a CT scan or MRCP. - Surgical procedure for treating pain in chronic pancreatitis 

Duct Drainage Procedure o Lateral pancreaticojejunostomy Rochelle Modification Gillesby Procedure)

Roux-en-Y (Partingtonof Puestow-

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Combined Resection-Drainage Procedure o Pancreaticoduodenecotmy (WHIPPLE) o Local resection for the head of pancreas with longitudinal pancreaticojejunosotmy (FREY) o Duodenum preserving pancreatic head resection (BEGER) Majority of the problem is located in the pancreatic head including tumors. This is where the common bile duct enters.

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Total Pancreatectomy with or without Islet Cell Auto Transplantation

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Distal Pancreatectomy This is done in cases where you have to do splenectomy in trauma patients. In the process, you might clip the tail of the pancreas. If this is happens, it is safer to perform a distal pancreatectomy because he might develop pancreatitis. In reality, they are touching one another. The shape of the spleen is a C-shape and the tail is up to the hilum. In operating, you have to pull it down for it to be easier to manipulate as they are hugging each other. Always be careful when doing splenectomy.

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Subtotal Pancreatectomy Neuroablative Procedure

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Endoscopic celiac ganglion – inject with alcohol o Patients with end-stage or terminally ill pancreatic CA – for pain control Thoracoscopic Splanchnicetectomy o

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Management Scheme Etiology/symptomatology Diagnostic Internal drainage: best way to drain the cyst o Using the posterior wall of the stomach, create an opening, and anastomose the cyst o Fluid would drain to the stomach External drainage o Puncture the cyst and put a tube, and exits the abdominal cavity o More tedious, difficult to maintain, more prone to complications such as infection Non-operative vs operative management o Operative/invasive management  Endocospic drainage  Cystenteric drainage  Pancreatojejunostomy – open up the whole length of duct and anastomose the jejunum to the duct  Pancreatic resection

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PANCREATIC PSEUDOCYSTS One of the reasons why surgeons are being called: to manage pseudocysts Is a collection of pancreatic juice enclosed by a wall of fibrous or granulation tissue which arises as a consequence of acute pancreatitis, trauma or chronic pancreatitis Cystic lesion – fluid-filled mass or tumor, with a wall or capsule Before surgery, make sure that cyst should be mature (meaning, it is already well-organized) o Matures in 4-6 weeks o No surgery is done less than 4-6 weeks o If surgery is done without any capsule, AY PATAY KANG BATA KA, because bleeding would be a main problem o Question ni Ms. Long Hair (haha): How would you know that the cyst has already matured? Is it 4-6 weeks after being diagnosed?  Once the patient has the symptoms and came in to you, he/she would be subjected for diagnostic work-ups. Wait for another 4-6 weeks and then do a CT scan to check for the formation of capsule or cyst. Early satiety, abdominal pain, nausea, vomiting, bloating, biliary obstruction, GI bleeding

PERIAMPULLARY CANCER Always consider possibility of malignancy in patients with pancreatitis, especially those who are complaining of chronic abdominal pain Periampullary o duodenum near the ampulla of vater

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o head of pancreas o distal common bile duct Refers to cancers that arise from the pancreas, ampulla of vater, bile duct or duodenum Pancreatic adenocarcinoma (common)

In periampullary cancer, we are not sure whether it is the pancreas invading the duodenum or the duodenum invading the pancreas. TNM CLASSIFICATION AJCC 6th Edition Pancreatic Ca Primary Tumor (T) Tumor T1: tumor limited to pancreas ≤ 2 cm T2: tumor limited to pancreas > 2 cm T3: tumor extends beyond the pancreas but without involvement of celiac axis or SMA T4: tumor involves celiac axis or SMA Regional Lymph Nodes Metastasis M0: no distant metastasis M1: distant metastasis Stage Grouping 0 Tis N0 M0 IA T1 N0 M0 IB T2 N0 M0 IIA T3 N0 M0 IIB T1 N0 M0 T2 N1 M0 T3 N1 M0 III T4 Any N M0 IV Any T Any N M0 AJCC 6th Edition: Ampulla of Vater Carcinoma Primary Tumor (T) Tumor T1: tumor limited to ampulla of vater or sphincter of Oddi T2: tumor invades duodenal wall T3: tumor invades pancreas T4: tumor invades peripancreatic soft tissues or other adjacent organs or structures

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PANCREATICODUODENECTOMY (Whipple’s Procedure) PLEASE SEE APPENDIX (separate file) FOR PICTURES PERTAINING TO THIS PROCEDURE. There are four (4) main steps in this procedure. Exposure o Incision: bilateral subcostal o NOT midline Mobilization o Preoperatively, you should know that the pancreas is already safe to remove. Meaning, other structures are not yet invaded such as the SMA. o Once SMA is already invaded by the malignant tumor, patient cannot be operated anymore because you cannot take away the SMA, or else the rest of the bowel will die. Resection (of the pancreatic head) Reconstruction (BDP) o Pancreatic anastomosis o Biliary anastomosis  End-to-end anastomosis (of the remaining pancreas)  End-to-side anastomosis (common bile duct to jejunum) o Gastric/Duodenal anastomosis  End-to-side anastomosis Other procedures: Distal gastrectomy You have to have a very good technique in doing anastomosis. If not, it may cause stricture, eventually leading to obstruction.

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SPLEEN Seat of laughter (in older time); ill temper o Thought to be associated with mood Black bile 7-11 cm 150 gm (70-250 gm) 4 functions o Filtration o Host defense o Storage o Cytopoiesis Sequester senescent RBCs Splenic surgery – one of the difficult to manage post-operatively due to complications like bleeding, abscess formation, and overwhelming post-splenectomy infection (OPSI) that can kill the patient

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Ultrasonography

IMAGING Ultrasound CT scan Plain radiograph: very first you should request MRI Angiography Nuclear imaging Splenic index: 120-480 ml

Abdominal Radiograph of Splenomegaly

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Nuclear Imaging INDICATIONS FOR SPLENECTOMY OPSI o overwhelming postsplenectomy infection o side effect of splenectomy especially to children trauma (thoracoabdominal injury): most common surgical indication for splenectomy RBC disorders WBC disorders Hemoglobinopathies Platelet disorders Bone marrow disorders Cyst/tumors Infections Storage disease Infiltrative disorders Active splenomegaly: most common medical indication for splenectomy

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GENERAL INDICATIONS FOR SPLENECTOMY FOR HEMATOLOGIC DISORDERS AUTOIMMUNE DISORDERS ITP TTP Autoimmune Hemolytic Anemias Felty’s Syndrome Sarcoidosis RED CELL DISORDERS Hereditary Spherocytosis Hereditary Pyropoikilocytosis Hereditary Xerocytosis GENETIC Thalassemias Sickle Cell Diseases Gaucher’s Pyruvate Kinase Deficiency G6PD Deficiency MALIGNANCIES Hogkin’s Lymphoma Non-Hodgkin’s Lymphoma Chronic Myelogenous Leukemia Chronic Lymphoblastic Leukemia Hairy Cell Leukemia RBC DISORDERS

Repair of the spleen may be challenging because the parenchyma is easily destroyed when sutures are applied. What surgeons use is a cloth like material known as surgicel (structured non-woven materal, absorbable hemostat). When can splenic trauma not be subjected to repair? When the hilar vessels are involved. This condition may be known as COMPLETE FRACTURE OF THE SPLEEN.

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Congenital 1. Hereditary Spherocytosis a. Erythrocyte membrane proteins: Spectrin, Ankyrin and band 3 protein/Band protein 4.2 b. +/- anemia c. Splenomegaly d. Increase reticulocyte count secondary to increased red blood cell destruction (hemolysis) e. Increase LDH f. Increase unconjugated bilirubin g. Spherocytes ** splenectomy is done at 4-6 y/o (more complication may develop) 2. RBC Enzyme Deficiencies  Pyruvate Kinase Deficiency o Most common – congenital chronic hemolytic anemia o Transfusion dependent o Screening test o Diagnosed at 4 y/o  G6PD Deficiency o Overall most common

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o o o o o

Chronic hemolytic anemia usually is drug induced One of the diseases included in newborn screening Aspirin, antimalarial drugs may precipitate hemolytic anemias among these patients Avoidance of drugs causing hemolysis Surgery NOT warranted

Acquired Warm-Antibody Autoimmune Hemolytic Anemia o Mid life o More common in women o Mild jaundice o Signs and symptoms of anemia o Splenomegaly o Positive Direct coombs test o Corticosteroids Direct Coomb’s Test Direct Antiglobulin Test (+): Immunoglobulin Dependent Warm Antibodies – (+) to IgG Cold Antibodies – (+) to IgM *also related to body temperature or the temperature at which these reactions occur Thalassemia (Hemoglinopathies) Inherited disorder of hemoglobin synthesis Thalassemia major (homozygous) Either alpha (found in fetus or in utero and adult hemoglobin) or beta (usually 4 to 6 months become symptomatic) o Hypochromic microcytic anemia o Target cells (red cells with nucleus present) o Increase reticulocyte and WBC count o Beta thalassemia (4-6 months)  Before 2 y/o o Pallor o Growth retardation o Jaundice o Abdominal enlargement (liver and spleen) Treatment o RBC transfusion o Parenteral chelation therapy with DEFEROXAMINE o Splenecotmy – management of last recourse  Pulmonary hypertension  OPSI (4 y/o) overwhelming post splenectomy infection  Try to do splenectomy beyond the age of 4  How do you prevent OPSI?  Immunize the patient from encapsulated microorganisms  In emergency surgery, the vaccination may be performed 1 – 2 days prior to splenectomy

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In elective cases, vaccination may be done 1 – 2 weeks prior to the procedure

WBC DISORDERS 1. Chronic Lymphocytic Leukemia (CLL) o Weakness o Fatigue o Fever without illness o Night sweats (rule out tuberculosis) o Frequent bacterial/ viral infection o Lymphadenopathy o Splenomegaly 2. Hairy Cell Leukemia o Uncommon o Splenomegaly o Pancytopenia o Abnormal lymphocyte in bone marrow 3. Hodgkin’s Lymphoma o Reed-Sternberg cells: diagnostic for Hodgkin’s lymphoma o 4 histologic type  Lymphocyte predominant type  Nodular sclerosis type  Mixed cellularity type  Lymphocyte depletion type o Lymphadenopathy above the diaphragm  SOB (shortness of breath)  Cough  Obstructive pneumonia 4. Non-Hodgkin’s Lymphoma o All malignancies form the lymphoid system o 3 preodminant lymph cell types  natural killer cell  T cells  B cells o Chemotherapy is very effective if the patient has been diagnosed early  Even a single dose of chemotherapy can diminish the size of a tumor significantly o Splenomegaly

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PLATELET DISORDERS A. Idiopathic Thrombocytopenic Purpura (ITP) o Immune thrombocytopenic purpura o Low platelet count o Mucocutanoeus nad petechial bleeding o Intracranial bleeding o Treatment: predinisone 1-1.5 ml/kg/day B. Thrombotic Thrombocytopenic Purpura (TTP) o Thrombocytopenia o Microangiopathic hemolytic anemia o Neurologic complication o Petechial hemorrhages in lower extremities o Renal failure and arrhythmia o Peripheral smear (schistocytes, basophilic stippling) o Plasma exchange (FFP) *For steroids, do not abruptly stop providing steroids. Taper the doses to prevent adrenal insufficiency (may result to shock, ultimately death).

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BONE MARROW DISORDERS Abnormal cell lines in the bone marrow o Chronic myeloid leukemia o Acute myeloid leukemia o Chronic myelomonocytic leukemia o Essential thrombocytopenia o Polycythemia vera o Myelofibrosis Splenomegaly o Treated with medications  Busulfam  Hydroxyurea  Interferon Alpha o Radiation

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SPLENECTOMY pre op considerations o Elective Splenectomy  vaccination against encapsulated bacteria 1-2 weeks before surgery o Emergency Splenectomy  1-2 days post op  Annual influenza vaccination o DVT Prophylaxis

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Outcomes o Howell-jolly bodies o Siderocytes o Within 1 day post op  Increase WBC count o Within 2 days post op  Increase platelet count

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Complications o Pulmonary o Hemorrhagic o Infectious o Pancreatic o Thromboembolic

RISK MANAGEMENT STRATEGIES OF ASPLENIC PATIENTS Wearing a medical bracelet Carrying a laminated medical alert card Possessing a medical letter with specific empiric therapy Keeping a 5-day supply of stand-by antibiotics

Spleen in a Patient with Chronic Lymphocytic Leukemia (CLL)

PI – Arellano | Gagui | Galvan | Pamintuan | Timbang PROPERTY OF AUFSOM BATCH 2017 v3.1 s2015-2016

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