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HELLP Syndrome

HELLP Syndrome is a severe complication of preeclampsia requiring prompt diagnosis and management, focusing on maternal stabilization, delivery of the baby, and supportive care. Acute Fatty Liver of Pregnancy (AFLP) is a rare obstetric emergency characterized by liver dysfunction, necessitating immediate hospitalization and delivery. Intrahepatic Cholestasis of Pregnancy (ICP) is a liver disorder marked by pruritus and elevated bile acids, requiring maternal symptom relief and careful fetal monitoring.

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0% found this document useful (0 votes)
11 views12 pages

HELLP Syndrome

HELLP Syndrome is a severe complication of preeclampsia requiring prompt diagnosis and management, focusing on maternal stabilization, delivery of the baby, and supportive care. Acute Fatty Liver of Pregnancy (AFLP) is a rare obstetric emergency characterized by liver dysfunction, necessitating immediate hospitalization and delivery. Intrahepatic Cholestasis of Pregnancy (ICP) is a liver disorder marked by pruritus and elevated bile acids, requiring maternal symptom relief and careful fetal monitoring.

Uploaded by

Awais Irshad
Copyright
© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
Available Formats
Download as DOCX, PDF, TXT or read online on Scribd
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HELLP Syndrome is a severe complication of preeclampsia characterized by Hemolysis

(H), Elevated Liver enzymes (EL), and Low Platelet count (LP). It requires prompt diagnosis
and management to prevent serious maternal and fetal complications. Below is an outline of its
management:

Key Principles of Management

1. Stabilize the Mother:


o The first priority is maternal stabilization.
o Manage blood pressure, prevent seizures, and address coagulation abnormalities.
2. Delivery of the Baby:
o The definitive treatment for HELLP syndrome is delivery.
o Timing depends on gestational age, maternal condition, and fetal well-being.
3. Supportive Care:
o Address complications like organ dysfunction, coagulation abnormalities, or
seizures.

Immediate Management

1. Hospitalization:
o HELLP syndrome requires care in a tertiary care facility with access to intensive
monitoring, obstetric, and neonatal services.
2. Monitoring:
o Continuous maternal and fetal monitoring.
o Assess maternal vitals, urine output, and symptoms of worsening disease.
o Frequent laboratory testing:
 Complete blood count (CBC) for hemoglobin, platelets.
 Liver function tests (LFTs): AST, ALT.
 Coagulation profile.
 Serum lactate dehydrogenase (LDH).
3. Blood Pressure Control:
o Target systolic BP <160 mmHg and diastolic BP <110 mmHg.
o Medications:
 Labetalol (IV or oral).
 Hydralazine (IV).
 Nifedipine (oral).
4. Seizure Prophylaxis:
o Administer magnesium sulfate to prevent or treat seizures (eclampsia).
5. Steroids for Fetal Lung Maturity:
o If gestation is <34 weeks and delivery can be delayed for 48 hours:
 Administer betamethasone or dexamethasone to enhance fetal lung
maturity.

Definitive Treatment
1. Timing of Delivery:
o If ≥34 weeks gestation: Immediate delivery.
o If <34 weeks gestation:
 Stabilize the mother and fetus (if possible) before delivery.
 Delivery is indicated if there is maternal deterioration or fetal
compromise.
2. Mode of Delivery:
o Vaginal delivery is preferred if the mother and fetus are stable.
o Cesarean section may be required for obstetric indications or if rapid delivery is
necessary.

Supportive Measures

1. Blood and Blood Products:


o Correct anemia with packed red blood cells (PRBCs).
o Platelet transfusion if:
 Platelet count <20,000/µL, even without bleeding.
 Platelet count <50,000/µL before cesarean section.
o Fresh frozen plasma (FFP) or cryoprecipitate for coagulopathy.
2. Fluid Management:
o Monitor closely to avoid pulmonary edema.
o Maintain adequate urine output (≥30 mL/hour).
3. Pain Management:
o Epigastric or right upper quadrant pain from hepatic involvement may need
analgesia.

Postpartum Care

1. Monitor for Resolution:


o HELLP syndrome often resolves within days after delivery.
o Continue close monitoring of blood pressure, liver function, and platelets
postpartum.
2. Antihypertensive Therapy:
o Continue as needed if hypertension persists.
3. Preventive Measures:
o Discuss risk of recurrence in future pregnancies.
o Aspirin may be recommended in future pregnancies to reduce the risk of
preeclampsia.

Complications to Watch For

 Disseminated intravascular coagulation (DIC).


 Acute renal failure.
 Pulmonary edema.
 Hepatic rupture or subcapsular hematoma.
 Placental abruption.
 Fetal growth restriction or death.

Acute Fatty Liver of Pregnancy (AFLP) is a rare but serious obstetric


emergency characterized by excessive fat accumulation in the liver, leading to liver dysfunction.
It typically occurs in the third trimester or the early postpartum period and can cause life-
threatening complications for both the mother and fetus if not managed promptly.

Etiology and Risk Factors

 Exact cause: Unknown, but it may result from mitochondrial dysfunction in the oxidation
of fatty acids.
 Associated with a mutation in the long-chain 3-hydroxyacyl-CoA dehydrogenase
(LCHAD) enzyme in some cases.
 Risk factors:
o First pregnancy.
o Multiple pregnancies (e.g., twins).
o Male fetus.
o Maternal preeclampsia or HELLP syndrome.

Clinical Features

 Non-specific Symptoms:
o Nausea and vomiting.
o Abdominal pain (often epigastric or right upper quadrant).
o Malaise and fatigue.
o Jaundice.
 Severe Cases:
o Hypoglycemia (common and often severe).
o Encephalopathy (confusion, altered consciousness).
o Coagulopathy (bleeding tendencies due to liver dysfunction).
o Acute kidney injury.
o Ascites or peripheral edema.

Diagnosis

There is no single diagnostic test; diagnosis is clinical and supported by laboratory findings and
imaging.
Key Investigations:

1. Laboratory Tests:
o Elevated liver enzymes (AST, ALT).
o Elevated bilirubin.
o Hypoglycemia.
o Elevated creatinine (due to renal dysfunction).
o Coagulopathy (prolonged PT and aPTT, low fibrinogen).
o Low platelet count (may overlap with HELLP syndrome).
o Elevated ammonia levels (in severe cases).
2. Imaging:
o Ultrasound: May show hepatomegaly or fatty infiltration, though not always
diagnostic.
o CT/MRI: Can sometimes confirm fat deposition in the liver.
3. Swansea Criteria: Diagnosis can be made if 6 or more of the following are present:
o Vomiting.
o Abdominal pain.
o Polydipsia/polyuria.
o Encephalopathy.
o Elevated bilirubin (>14 µmol/L).
o Hypoglycemia (<4 mmol/L).
o Elevated uric acid (>340 µmol/L).
o Elevated AST/ALT.
o Leukocytosis.
o Ascites or bright liver on imaging.
o Renal impairment.
o Coagulopathy.

Differential Diagnosis

 HELLP syndrome.
 Severe preeclampsia.
 Viral hepatitis.
 Drug-induced liver injury.
 Intrahepatic cholestasis of pregnancy.

Management

Immediate hospitalization is essential for maternal and fetal monitoring and management.

1. Stabilization:
o Treat hypoglycemia with IV dextrose.
o Correct coagulopathy with fresh frozen plasma (FFP) or cryoprecipitate.
o Manage renal failure with fluids and, if necessary, dialysis.
2. Delivery:
oThe definitive treatment is prompt delivery, irrespective of gestational age, once
the mother is stabilized.
o Vaginal delivery is preferred if feasible, but cesarean delivery may be necessary
depending on maternal or fetal condition.
3. Supportive Care:
o Intensive care unit (ICU) management for severe cases.
o Monitor liver and renal function closely.
o Manage complications like sepsis, DIC, or multi-organ failure.

Complications

 Maternal:
o Hepatic failure.
o Acute renal failure.
o Coagulopathy and hemorrhage.
o Multi-organ failure.
o Death (if untreated).
 Fetal:
o Preterm birth.
o Fetal growth restriction.
o Fetal distress or stillbirth.

Intrahepatic Cholestasis of Pregnancy (ICP) is a pregnancy-specific liver


disorder characterized by pruritus (itching) and elevated serum bile acids. It typically occurs in
the third trimester and resolves after delivery. ICP can have significant implications for maternal
and fetal health, necessitating prompt recognition and management.

Etiology and Risk Factors

1. Causes:
o Hormonal changes (e.g., increased estrogen and progesterone levels).
o Genetic predisposition (e.g., mutations in bile salt export pump genes).
2. Risk Factors:
o Previous history of ICP.
o Family history of ICP.
o Multiple pregnancies (e.g., twins, triplets).
o Gallbladder disease.
o Advanced maternal age.

Clinical Features

1. Pruritus (Key Symptom):


o Intense itching, often worse at night.
o Commonly affects the palms and soles but may become generalized.
o No rash (unless secondary to scratching).
2. Jaundice (in some cases):
o May occur due to severe cholestasis.
3. Other Symptoms:
o Fatigue.
o Dark urine and pale stools (less common).

Diagnosis

ICP is primarily diagnosed based on clinical features and laboratory findings.

1. Laboratory Tests:
o Serum bile acids: Elevated (>10 µmol/L); levels >40 µmol/L indicate severe
disease.
o Liver function tests (LFTs):
 Elevated transaminases (ALT, AST).
 Elevated alkaline phosphatase (common in pregnancy but may rise further
in ICP).
o Bilirubin: Usually normal or mildly elevated.
2. Exclusion of Other Causes:
o Viral hepatitis (e.g., hepatitis A, B, C, E).
o Autoimmune liver diseases.
o Drug-induced liver injury.

Differential Diagnosis

 Preeclampsia with liver involvement.


 Acute fatty liver of pregnancy (AFLP).
 HELLP syndrome.
 Primary biliary cholangitis.
 Biliary obstruction (e.g., gallstones).

Management

1. Maternal Symptom Relief:


o Ursodeoxycholic acid (UDCA):
 First-line treatment to reduce serum bile acids and improve symptoms.
o Antihistamines (e.g., chlorpheniramine):
 For itching relief (though efficacy is limited).
o Topical emollients or oatmeal baths:
 Soothe itching.
2. Fetal Monitoring:
o Non-stress tests and biophysical profiles.
o Regular fetal growth scans.
3. Timing of Delivery:
o Planned delivery at 36–37 weeks is recommended to reduce the risk of stillbirth,
especially in severe cases (bile acids >100 µmol/L).
4. Vitamin K Supplementation:
o Given to prevent maternal and neonatal bleeding due to reduced fat-soluble
vitamin absorption.

Complications

1. Maternal:
o Increased risk of postpartum hemorrhage due to vitamin K deficiency.
2. Fetal:
o Preterm birth.
o Fetal distress.
o Meconium-stained amniotic fluid.
o Stillbirth (risk increases with higher bile acid levels).

Simplified Steps for Laparoscopic Cholecystectomy:

1. Preparation

 Position the patient supine.


 Apply antiseptic from the nipples to the pubic area.
 Drape to include the costal margin and sternum in the sterile field.

2. Team Positioning

 Surgeon: On the patient’s left side.


 Assistant: To the left of the surgeon.
 Scrub Nurse: On the patient’s right.
 Monitor: Positioned at the patient’s right shoulder.

3. Creating Pneumoperitoneum

 Use an open cut-down or Veress needle technique.


 Make a 1 cm supraumbilical incision.
 Place a stay suture on the midline; incise the sheath and peritoneum.
 Insert a blunt trocar with a 10/11-mm cannula as the optical port.
 Set the operating pressure to 12 mmHg.
4. Initial Inspection

 Insert the laparoscope.


 Inspect the abdominal cavity for any additional pathology.
 Tilt the table head-up and left side down for better visualization.

5. Port Placement

 Add three additional ports under direct vision:


o 10-mm port: Epigastrium.
o 5-mm port: Right upper quadrant.
o 5-mm port: Right lower quadrant.

6. Exposing the Gallbladder

 Divide adhesions around the gallbladder.


 Grasp the gallbladder fundus via the RLQ port and retract it cranially.
 Expose Hartmann’s pouch and Calot’s triangle.

7. Dissection

 Use a diathermy hook or scissors.


 Incise the peritoneum around Hartmann’s pouch.
 Continue dissection along the medial and lateral gallbladder borders.
 Carefully dissect Calot’s triangle to isolate the cystic duct and artery.

8. Critical View of Safety

 Dissect the proximal gallbladder from the liver bed.


 Confirm the identity of the cystic duct and artery.
 Ensure the "critical view of safety" is achieved.

9. Securing and Dividing Structures


 Apply mechanical clips to the cystic artery and duct.
 Carefully divide the clipped structures.

10. Gallbladder Removal

 Dissect the gallbladder from the liver bed.


 Place the gallbladder in a retrieval pouch.
 Remove it through the 10-mm port.

11. Closure

 Suture the wound.


 Apply a sterile dressing.

Simplified Steps for Open Appendectomy:

1. Incision

 Use a gridiron incision (5–8 cm long) across McBurney’s point, following the external
oblique muscle fibers.
 The incision is one-third above and two-thirds below the spino-umbilical line.

2. Opening the Abdomen

1. Skin and Fat:


o Incise the skin and subcutaneous fat to expose Scarpa’s fascia.
o Stop any bleeding.
2. External Oblique:
o Incise the external oblique aponeurosis in the direction of its fibers.
o Use scissors and retract the edges to expose the internal oblique muscle.
3. Internal Oblique and Transversus Abdominis:
o Split these muscles along their fibers using scissors.
o Widen the opening gently with your fingers.
4. Peritoneum:
o Identify the transversalis fascia and peritoneum.
o Pick up the peritoneum with forceps and carefully make a small incision to enter
the cavity.
3. Locating and Mobilizing the Appendix

1. Locate the Appendix:


o Look for the appendix and mobilize it by gently freeing any adherent structures.
2. Handle with Care:
o Use Babcock’s forceps to hold the appendix gently without crushing it.
3. Expose the Mesoappendix:
o View the mesentery against light to identify the blood vessels.

4. Securing the Mesoappendix

1. Clamp and Ligate:


o Pass a clamp through the mesoappendix to isolate vessels.
o If thickened, clamp and ligate in two steps using 2/0 Vicryl.
o Cut the mesoappendix after ligation.

5. Removing the Appendix

1. Prepare the Base:


o Crush the base of the appendix with a clamp.
o Place a ligature (2/0 Vicryl) just above the crushed area.
2. Cut and Remove:
o Cut the appendix distal to the ligature and ensure no bleeding.

6. Closure

 Peritoneum and Sheath: Close with 2/0 Vicryl.


 Skin and Layers: Close in layers and apply a sterile dressing.

Simplified Steps for Open Inguinal Hernia Repair:

1. Incision

 Make a skin incision just above the pubic tubercle, extending two-thirds of the way to the
anterior superior iliac spine.
 Cut through the fascia to expose the external oblique aponeurosis, ligating and dividing
any crossing veins.

2. Exposing the Inguinal Canal

 Identify the external inguinal ring (confirms the inguinal canal).


 Split the external oblique aponeurosis along its fibers with a scalpel, then extend the
opening using scissors.
 Preserve the ilioinguinal nerve to reduce postoperative pain and numbness.

3. Mobilizing the Cord

 Use your finger to create a plane around the cord, freeing it gently.
 Incise the coverings above and below the cord to expose any hernia sac.

4. Examining the Hernia

 Inspect the hernia type:


o Direct Hernia: Bulge in the posterior wall, often weak or diffuse.
o Indirect Hernia: Protrusion along the cord, extending toward the internal ring.

5. Handling an Indirect Sac

1. Access the Sac:


o Split the cremasteric and internal spermatic fascia to expose the sac.
o Separate the sac from the cord carefully to avoid injury.
2. Open the Sac:
o Pick up the sac with forceps and make a small incision.
o Return any contents (e.g., bowel, omentum) to the peritoneal cavity.
3. Ligate the Sac:
o Tie the neck of the sac securely with a Vicryl suture.
o Excise the sac 1 cm distal to the ligature and confirm the stump is secure and
retracted into the internal ring.

6. Mesh Placement
 Place a 6×11 cm mesh over the posterior wall to reinforce it.

7. Closure

 Ensure hemostasis.
 Close the layers:
o Sheath: Vicryl sutures.
o Skin: Close in layers.
 Apply a sterile dressing.

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