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Condition With Decreased Platelet Count AND Absent/Deficient Clotting Factors

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Disseminated Intravascular Coagulation (DIC)

What happens in DIC:

• Clotting is triggered systemically (by sepsis, malignancy, obstetric emergencies, massive trauma, etc.)
• As clotting runs out of control, coagulation factors are consumed (factors I, II, V, VIII, and fibrinogen are depleted)
• Platelets are consumed in the microthrombi, causing thrombocytopenia
• Fibrinolysis activates simultaneously, generating elevated D-dimers and FDPs
• Result: the patient paradoxically bleeds despite widespread clot formation

Lab findings in DIC:

"These changes result in clinical features of widespread bleeding...and laboratory findings of prolonged PT, APTT, decreased fibrinogen, elevated D-dimers, and thrombocytopenia." - Henry's Clinical Diagnosis and Management by Laboratory Methods

Common triggers of DIC:

• Sepsis (especially gram-negative, most common cause)
• Obstetric emergencies (abruptio placentae, amniotic fluid embolism, HELLP syndrome)
• Malignancy
• Massive tissue injury / surgery
• Circulatory arrest / hypothermia

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Other Conditions With BOTH Thrombocytopenia AND Clotting Factor Deficiency

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Key Distinction

• In hemophilia A or B, only a single clotting factor is absent (VIII or IX), but platelet count is normal - so this does NOT fit.
• In ITP, platelets are low but clotting factors are normal - does NOT fit.
• DIC is the classic condition where BOTH are simultaneously abnormal due to consumption.

Von Willebrand Factor (vWF)

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What Is It?

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Where Is It Made?

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Two Major Functions

1. Primary Hemostasis - Platelet Adhesion

• At a site of vascular injury, vWF binds exposed subendothelial collagen
• vWF then binds glycoprotein Ib (GPIb) on platelet surface
• This "bridges" platelets to the damaged vessel wall = platelet adhesion
• Also promotes platelet aggregation by binding activated GPIIb/IIIa
• This role is especially important under high shear stress (small vessels)

2. Secondary Hemostasis - Carrier for Factor VIII

• vWF acts as a protective carrier protein for Factor VIII
• Without vWF, Factor VIII is rapidly cleared from circulation (half-life drops from ~12 hours to ~2.4 hours)
• vWF also concentrates Factor VIII at the site of injury, releasing it when activated by thrombin

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vWF Structure

• Individual monomers are linked by disulfide bonds into large multimers (up to 20 million Daltons)
• Higher molecular weight multimers = more hemostatically active
• ADAMTS13 (a metalloprotease) cleaves ultra-large vWF multimers to regulate size

"VWF mediates platelet adhesion through collagen on the injured surface and GPIb on the platelet surface... Circulating VWF binds and protects factor VIII from premature clearance." - Tietz Textbook of Laboratory Medicine

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What Happens When vWF Is Deficient? - Von Willebrand Disease (VWD)

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Connection to TTP

• ADAMTS13 deficiency (congenital or autoimmune) causes accumulation of ultra-large vWF multimers
• These spontaneously aggregate platelets, forming microthrombi
• This is the mechanism of Thrombotic Thrombocytopenic Purpura (TTP)

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Summary in One Line

• Henry's Clinical Diagnosis and Management by Laboratory Methods | Tietz Textbook of Laboratory Medicine

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Von Willebrand Disease (VWD) - Diagnosis and Management

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DIAGNOSIS

Step 1 - Clinical Assessment & Bleeding History

• Bleeding score ≥4 in males or ≥6 in females warrants lab investigation
• Key symptoms: mucocutaneous bleeding (epistaxis, gum bleeding, menorrhagia, easy bruising, prolonged wound bleeding)
• Family history and current medications must be reviewed
• Clinical exam: look for petechiae, ecchymosis, hematomas, signs of connective tissue disorders

Step 2 - Initial Lab Screening

Step 3 - Specific VWD Tests

Important: vWF levels vary with stress, exercise, pregnancy, ABO blood group (Type O = lower levels), and inflammation. Tests should be repeated on two separate occasions several weeks apart to confirm diagnosis.

VWD Types - Key Lab Differences

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MANAGEMENT

1. DDAVP (Desmopressin) - First-line for Type 1

• IV: 0.3 µg/kg in 100 mL normal saline over 20 minutes (max 25-30 µg)
• Intranasal: 300 µg total (150 µg per nostril for patients >50 kg)

• Type 1 VWD - most responsive, first choice
• Types 2A and 2M - may be useful in some patients
• Avoid in Type 2B - can trigger worsening thrombocytopenia (releases abnormal hyperfunctional multimers that aggregate platelets)
• Not useful in Type 3 - no vWF stores to release

• Perform a DDAVP trial first in each patient (measure vWF and FVIII at 30 min and 4 hours post-dose)
• Repeat dosing every 12-24 hours but watch for tachyphylaxis (diminishing response)
• Risk of fluid retention and hyponatremia - restrict fluids to 1500 mL/24 hrs; monitor sodium before repeat doses
• Avoid in elderly patients with cardiovascular risk factors

2. vWF Concentrates - For Types 2 & 3 (or DDAVP failures)

• DDAVP fails or is contraindicated
• Type 2B, Type 3, or severe Type 2A disease
• Major surgery or life-threatening bleeding

3. Tranexamic Acid (Antifibrinolytic)

• Useful adjunct for acute bleeding episodes and dental procedures (7-10 days)
• Can be given orally (15-25 mg/kg three times daily) or IV (15 mg/kg three times daily)
• Associated with minimal thrombotic risk even in high-risk situations
• Avoid if significant hematuria (risk of clot retention/urinary obstruction)

4. Hormonal Therapy

• Combined oral contraceptives or progestins for menorrhagia in women with VWD
• Pregnancy naturally raises vWF levels (especially in Type 1); most women with Type 1 do not need prophylactic treatment in the third trimester

Summary Table - Treatment by Type

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"Treatments include desmopressin, which stimulates release of the patient's own von Willebrand factor, and infusion of von Willebrand factor concentrates. Tranexamic acid is useful for acute bleeding episodes." - Goldman-Cecil Medicine

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VMMC Case Charts - Answers with Explanations

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SLIDE 2 - OGTT Curve Interpretation

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SLIDE 3 - OGTT Graph

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SLIDE 4 - Sugar in Urine at Normal Blood Glucose

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SLIDE 5 - Classic DM Presentation

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SLIDE 6 - Unconscious Type 1 Diabetic

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SLIDE 7 - Crush Injury, Unconscious

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SLIDE 8 - Vomiting + Diarrhea

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SLIDE 9 - Excess Vomiting + Antacids

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SLIDE 10 - Chronic Cough + Dyspnea

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SLIDE 11 - Hysterical Hyperventilation

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SLIDE 12 - Altered Sensorium, Low Glucose

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SLIDE 13 - Liver Function Tests

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SLIDE 14 - 2-Day-Old Baby with Jaundice

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SLIDE 15 - Office Lady with Hepatomegaly

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SLIDE 16 - Yellow Urine + Itching + Colicky Pain

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Jaundice Comparison Summary

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SLIDE 17 - Chest Pain, ECG Changes (MI)

• Troponin I and Troponin T (most specific and sensitive for MI)
• CK-MB (Creatine Kinase-MB isoenzyme)
• LDH (Lactate Dehydrogenase)

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SLIDE 18 - Acute Abdominal Pain + Elevated Amylase

1. Diagnosis: Acute Pancreatitis
2. Normal serum amylase: 50-120 U/L (elevated in this case)
3. Action of amylase: Cleaves α-1,4 glycosidic bonds of starch → produces maltose units (digests carbohydrates)

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SLIDE 19 - Girl with Generalized Swelling

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SLIDE 20 - Child with Night Vision Problems

1. Diagnosis: Night Blindness (Nyctalopia)
2. Deficiency: Vitamin A (Retinol) / β-Carotene
3. Dietary sources: Mangoes, carrots, papaya, green leafy vegetables, egg yolk, liver, fish oil

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SLIDE 21 - 5-Year-Old with Bowed Legs

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SLIDE 22 - Hoarse Voice + Weight Gain + Cold Intolerance

1. Diagnosis: Hypothyroidism
2. Investigations and normal levels:

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SLIDE 23 - Urine Turns Black in Sunlight

• Diagnosis: Alkaptonuria (Ochronosis)
• Deficient enzyme: Homogentisate oxidase (homogentisic acid oxidase)

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SLIDE 24 - Painter with Kayser-Fleischer Rings

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SLIDE 25 - Muscle Cramps + Numbness + Spasms

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SLIDE 26 - Child with Liver Enlargement + KF Rings

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SLIDE 27 - Joint Pain in Non-Vegetarian

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SLIDE 28 - Acute Abdominal Pain + Elevated Amylase AND Lipase

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SLIDE 29 - Child with Self-Mutilation + High Uric Acid

• Elevated uric acid: 11 mg/dl (normal 3.5-7 mg/dl)
• Deficient enzyme: HGPRTase (Hypoxanthine-Guanine Phosphoribosyl Transferase)

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SLIDE 30 - Alcoholic with Recurrent Abdominal Pain (31B)

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SLIDE 31 - Chest Pain Radiating to Left Arm, Early Presentation (17A)

1. Enzymes that elevate: Creatine Phosphokinase (CPK/CK)
2. Normal levels: Total CK: 15-100 IU/L (males), 10-80 IU/L (females)
3. Isoenzyme elevated: CK-MB (CK-2) - cardiac specific

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SLIDE 32 - Obese Patient, Dizziness + Chest Pain (17B)

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Quick Summary Table - All Diagnoses

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CT Abdomen & Pelvis Report - Full Explanation

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INDICATION - What the Scan Was Looking For

1. Possible endometrial leiomyomatous polyp (a fibroid-type polyp of the uterus lining)
2. A known incisional hernia at the infraumbilical (below-umbilicus) surgical scar
3. General post-surgical surveillance

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FINDING 1 - Post Hysterectomy Status

"Post hysterectomy status. Both ovaries are not visualized."

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FINDING 2 - Large Incisional Hernia (MOST IMPORTANT SURGICAL FINDING)

"A large incisional hernia noted in right iliac fossa region containing small bowel loops and defect measuring 6 x 5 cm. No evidence of incarceration. The herniated bowel loops are seen adherent to anterior abdominal wall within the hernial sac. Entero-parietal adhesion noted in infraumbilical region."

• Cause chronic pain
• Lead to bowel obstruction (kinking/pulling of bowel)
• Complicate future hernia repair surgery (harder to separate bowel from sac)

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FINDING 3 - Liver (Normal)

"Liver is normal in size measuring 13.5 cm and shows uniform density. No focal lesion is seen. On IV contrast, no abnormal enhancement. Intrahepatic biliary radicles are normal. Portal and hepatic veins are normal."

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FINDING 4 - Gallbladder & CBD (Normal)

"GB is well distended. Wall thickness is normal. No radioopaque calculus/mass lesion seen. CBD is not dilated."

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FINDING 5 - Pancreas & Spleen (Normal)

"Pancreas is normal in size and uniform density. No calcification, mass or peripancreatic fluid collection seen. The pancreatic duct is not dilated. Spleen is normal in size and density."

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FINDING 6 - Kidneys & Ureters (Normal)

"Both kidneys normal in size and position. Right kidney: 10 x 4.4 cm. Left kidney: 9.5 x 4.5 cm. On IV contrast, both kidneys show prompt cortical enhancement and excretion. No calculus or caliectasis on either side. Ureters are not dilated."

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FINDING 7 - REPLACED RIGHT HEPATIC ARTERY (Anatomical Variant)

"Replaced right hepatic artery noted arising from superior mesenteric artery."

• It is an anatomical variant, NOT a disease
• It is critically important to know before surgery - if a surgeon cuts this vessel thinking it's just a lymphatic or small vessel, the right lobe of the liver loses its blood supply
• Must be communicated to the surgical team before any planned hernia repair or future abdominal surgery

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FINDING 8 - DILATED COLLATERAL VEIN (Important Vascular Finding)

"A dilated collateral vein measuring 1.3 cm in maximum diameter noted extending from proximal aspect of splenic vein, encircling OG junction and seen draining into left renal vein. No evidence of thrombosis or stenosis of main portal vein or splenic vein."

• The portal vein carries blood from the intestines and spleen to the liver
• The splenic vein drains the spleen into the portal system

• Starting from: the proximal splenic vein
• Wrapping around: the Oesophago-Gastric (OG) junction (where oesophagus meets stomach)
• Draining into: the left renal vein

• Collaterals around the OG junction can form varices (dilated, fragile veins) in the oesophagus - risk of variceal bleeding (life-threatening)
• No thrombosis of the main portal or splenic vein is reassuring - the vessels themselves are open
• This warrants endoscopy to assess for oesophageal varices
• Liver function tests and evaluation for portal hypertension should be done

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FINDING 9 - Urinary Bladder (Cystitis)

"Urinary bladder is well distended and shows diffuse mild wall thickening measuring 7 mm. No evidence of calculus or diverticulum. VUJ appears normal."

• Normal bladder wall thickness = 3 mm when distended, up to 5 mm when contracted
• Here it measures 7 mm - mildly thickened
• Diffuse (all-around) thickening of the bladder wall is the pattern seen in cystitis (bladder infection/inflammation)
• No stones, no outpouching (diverticulum), normal VUJ (Vesico-Ureteric Junction)
• Impression confirms: Cystitis - likely needs urine culture and antibiotic treatment

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FINDING 10 - Spine (Mild Degenerative Changes)

"Visualized dorsolumbar vertebrae show mild degenerative changes. No lytic or sclerotic lesion."

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IMPRESSION SUMMARY - What the Radiologist Concluded

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In Simple Terms for the Patient

1. A large hernia at the old surgery scar that has bowel loops inside it - it needs to be surgically repaired
2. A blood vessel abnormality - an extra vein has formed near the food pipe junction - this needs further investigation with a stomach scope (endoscopy) to check for fragile veins that could bleed
3. A bladder infection (cystitis) that needs antibiotics
4. No cancer recurrence - no tumors, no spread to lymph nodes or liver - this is very good news
5. Normal liver, kidneys, and other organs

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Management of Large Incisional Hernia

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What Makes a Hernia "Large"?

• Defects >4 cm in diameter are considered large
• This patient's defect is 6 x 5 cm - classified as large and requires mesh-based repair
• Primary suture repair alone is only acceptable for defects <2 cm (or up to 4 cm with strong surrounding tissue)
• With primary suture repair alone, recurrence rates reach 43% at 3 years; mesh reduces this to ~24% or less

"For larger hernias or hernias associated with multiple small defects, mesh repair is indicated." - Maingot's Abdominal Operations

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STEP 1 - Pre-operative Optimization

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STEP 2 - Choosing the Right Repair

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OPTION A - OPEN REPAIR

A1. Primary Suture Repair (NOT suitable for this patient)

• Only for defects <4 cm
• Fascia is closed with interrupted non-absorbable sutures
• Small bite technique (STITCH trial): 5 mm bites, 5 mm apart, suture:wound ratio ≥ 4:1
• High recurrence (43%) if used alone for large defects
• NOT appropriate for this patient's 6x5 cm defect

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A2. Open Mesh Repair - The Standard for Large Hernias

1. Patient under general anaesthesia, supine, Foley catheter inserted
2. Old scar is incised and dissection carried down to anterior rectus sheath
3. Adhesiolysis - scar tissue between bowel and hernia sac is carefully divided (critical step in this patient who has entero-parietal adhesions)
4. Hernia sac freed, contents reduced back into abdomen
5. Fascia cleared of soft tissue for a 3-4 cm margin all around
6. Mesh placed with ≥5 cm overlap beyond defect edges on all sides
7. Mesh fixed with non-absorbable sutures in interrupted fashion around entire circumference

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A3. Mesh Placement Positions (ICAP Classification 2019)

SKIN
SUBCUTANEOUS FAT
EXTERNAL OBLIQUE ←— ONLAY mesh (on top of fascia)
INTERNAL OBLIQUE
TRANSVERSUS ABDOMINIS
POSTERIOR RECTUS SHEATH ←— RETRORECTUS / SUBLAY mesh (between muscle and posterior sheath)
PREPERITONEAL SPACE ←— PREPERITONEAL mesh
PERITONEUM ←— INTRAPERITONEAL (IPOM) mesh
BOWEL

"Inlay mesh should be avoided due to its extremely high recurrence rates." - Fischer's Mastery of Surgery

• Mesh sits in the retrorectus space (between rectus muscle and posterior sheath)
• Intra-abdominal pressure holds mesh in place (Pascal's principle - pressure pushes mesh against wall rather than pushing it out)
• Allows very wide mesh overlap
• Low recurrence (~10-15%)

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A4. Component Separation Techniques (for giant/complex hernias)

Anterior Component Separation (ACS) - Ramirez Technique (1990)

1. Skin and fat flaps raised off anterior rectus sheath
2. External oblique aponeurosis incised lateral to linea semilunaris, from costal margin to iliac crest
3. External oblique separated from internal oblique in the avascular plane between them
4. This releases tension and allows rectus muscle to advance medially by 5-10 cm per side
5. Midline fascia then closed

Posterior Component Separation with Transversus Abdominis Release (TAR)

• More modern technique
• Transversus abdominis muscle divided from inside
• Allows even wider retromuscular space for mesh
• Preserves blood supply to skin flaps (lower wound complication rate than ACS)
• Mesh can be placed in retromuscular space with wide overlap

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OPTION B - LAPAROSCOPIC REPAIR

Laparoscopic IPOM (Intraperitoneal Onlay Mesh)

1. 3-4 small ports placed in the abdomen
2. Pneumoperitoneum created (CO2 gas inflates abdomen)
3. Adhesiolysis performed (laparoscopically) - for this patient with bowel adhesions, this is technically challenging
4. Barrier-coated mesh placed intraperitoneally (inside abdomen, directly against peritoneum)
5. Mesh fixed with tacking devices + transfascial sutures
6. Must have ≥5 cm overlap beyond all edges of defect

• Smaller incisions → lower wound infection rate
• Less postoperative pain
• Shorter hospital stay
• Lower wound complication rate compared to open

• Bowel adhesions (as in this patient) make laparoscopic adhesiolysis technically difficult and risky
• Intraperitoneal mesh requires special anti-adhesion-coated mesh (more expensive)
• Bowel injury risk during adhesiolysis
• Does not restore the abdominal wall to its correct anatomical layers (bridging repair)
• Cannot perform component separation laparoscopically (in most cases)

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OPTION C - ROBOTIC REPAIR

Robotic Retromuscular Repair (e.g., eTEP - Enhanced-View Totally Extraperitoneal)

• Retrorectus space developed robotically
• Mesh placed in retromuscular position (best position) through small ports
• Component separation (TAR) can also be performed robotically
• Avoids entering the peritoneal cavity entirely → no bowel contact with mesh

• Retromuscular mesh position (lower recurrence than IPOM)
• No need for barrier-coated mesh
• Minimally invasive
• Better ergonomics for surgeon

• Expensive, limited availability
• Long learning curve

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MESH TYPES

"Biologic meshes have been shown to be more resistant to infection than their synthetic predecessors and are more appropriate for use in infected or contaminated fields." - Maingot's Abdominal Operations

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COMPLICATIONS OF REPAIR

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WHAT IS BEST FOR THIS PATIENT?

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Summary Flowchart

LARGE INCISIONAL HERNIA (>4 cm)
         |
   Pre-op optimization
         |
   ┌─────┴──────────────────────────┐
   |                                |
Clean wound                  Contaminated field
   |                                |
Synthetic mesh               Biologic mesh
   |
   ├── No adhesions → Laparoscopic IPOM or Robotic eTEP
   |
   └── With adhesions (like this patient)
            |
            ├── Open Rives-Stoppa (retrorectus mesh) ← PREFERRED
            |
            └── If giant/extreme tension → Component Separation (ACS or TAR) + mesh

• Fischer's Mastery of Surgery, 8th Edition
• Maingot's Abdominal Operations