ANAESTHETIC

IMPLICATIONS IN
CONCURRENT
DISEASES
Presenter: Dr. Pavani .M. S
Moderator: Dr. Deepak. B.S

Classification
Diseases involving endocrine system
Disorders of nutrition
Diseases involving the cardiovascular system
Diseases of central nervous system, neuromuscular
diseases , mental disorders
Diseases involving kidneys, disorders of electrolytes
Diseases involving GI tract and liver
Diseases involving haematopoiesis and various forms of
cancer
Geriatric diseases

Haematological disorders
Diseases related to erythrocytes:- Anaemia
Polycythemia
Anaemia:- clinically reduced no of circulating RBCs
Defined as Hb < 11.5g/dl /hct< 36%(women) ,
Hb<12.5g % /Hct < 40% (men)
Decrease in HCT >1% every 24hrs acute blood loss or
intravascular haemolysis
Adverse effect of anaemia is impaired tissue oxygen
delivery due to reduced CaO2

Compensatory mechanisms
Right ward shift of oxyhaemoglobin dissociation curve
release of oxygen from Hb to tissues
Tissue redistribution of blood to myocardium, brain,
muscles from skin and kidneys
Increased cardiac output as an indicator of decreased
blood viscosity.

Management of anaesthesia.
Avoid iatrogenic hyperventilation
Avoid hypothermia
Maintaining normovolemic hemodilution and blood
salvage in case of surgical blood loss
Volatile anesthetics less soluable due to decrease in
concentration of lipid rich RBCs uptake increased. But
no clinical differences in rate of induction due to
increased cardiac output

 Transfusion trigger:- 10/30 rule..

But Hb < 6g/dl is definitive indication.
In MI patients Hb > 7g/dl should be maintained
If expected bld loss < 15% no transfusion required
If 15-30% replace with crystalloids
If >40% require blood transfusion
In massive transfusion PRBC: FFP: Platelet to be
transfused in ratio of 1:1:1

Hemolytic anaemias
Can be due to:
Extravascular haemolysis
or
Intravascular haemolysis
Characterized by reticulocytosis, increased MCV,
unconjugated bilirubinemia, increased LDH levels,
decreased serum haptoglobin

Hereditary spherocytosis
AD disease with defect in ankyrin and spectrin
Abnormal osmotic fragility and shortened half life
Haemolytic crisis often precipitated by viral or bacterial
infection
Pigmented gall stones, splenomegaly
Anaesthetic risk depends on severity of anaemia and
whether haemolysis is stable
Episodic anaemia triggered by infection and
cholelithiasis to be asked in preop evaluation
Use of long term CPB excessive haemolysis

Hereditary elliptocytosis
AD disorder due to abnormality in spectrin or glycophorin
Prevalent in malaria endemic areas
Most patients are heterozygous and rarely have hemolysis

Acanthocytosis
Seen with abetalipoproteinemia, cirrhosis and pancreatitis
Cholesterol or sphingomyelin accumulation on outer
membrane of erythrocyte speculated appearance signals
splenic macrophages hemolysis

Paroxysmal nocturnal
hemoglobinuria
Result in complement mediated RBC hemolysis
Due to reduction in glycosophosphatidyl glycan
Are also at risk for venous thrombosis due to compliment activation
In absence of protectin dysplastic or aplastic anemia
Nocturnal hemolysis is due to CO2 retention and subsequent acidosis
Management:- Avoid predisposing factors like hypoxemia,
hypoperfusion, hypercarbia
Inhalational agents and propofol are more advantageous than
thiopental
Maintain adequate hydration

Disorders of red cell metabolism

Embden-meyerhoff pathway:- Defects are associated with red cell
rigidity and reduced life span hemolysis
Phosphogluconate pathway:- lack of glucose -6- phosphate
dehydrogenase or glutathione reductase presence of
denatured Hb precipitates on inner surface hemolysis
Methamoglobin reductase deficiency:- Inability to counteract
oxidation of Hb to methemoglobin, thus Hb is in ferric form that
will not transport oxygen.
Type 1 NAD-diaphorase deficiency have small amounts of
methamoglobin accumulation in the cells
Type II disease have severe cyanosis and Mental retardation

 Glucose-6-phosphate deficiency: X linked disorder.

Half life is 60days
Classification :- Class1(<10% G6PD activity) ,
Class2(intermittent hemolysis),
Class3(10-60% activity)-hemolysis only with
stressors,
Class4&5(no hemolysis)
Management :Avoid precipitating factors of hemolysis( hypothermia, acidosis,
hyperglycemia and infections)
Avoid isoflurane, sevoflurane, diazepam . Also lidocaine, prilocaine,
silver nitrate which cause methamoglobinemia
codeine, midazolam, prpofol, fentanyl, ketamine are considered safe

Pyruvate kinase deficiency

Autosomal recessive disorder
Causes congenital chronic haemolytic anaemia
Accumulation of 2,3DPG which causes right shift of ODC
increased oxygen delivery to tissues
Splenectomy reduces rate of RBC destruction thus
reducing no of transfusions
Management depends upon severity of anaemia

Sickle cell anaemia

Due to substitution of valine for glutamic acid on beta globin chain
Present with haemolytic anemia, end organ damage(bone marrow,
spleen, kidneys and CNS
Vaso occlusive crisis may be associated with concurrent infection,
stress or dehydration
Acute chest syndrome, a pneumonia-like complication, is characterized
by the presence of a new pulmonary infiltrate involvin at least one
complete lung segment plus at least one of the following:chest pain, temperature higher than 38.5 C, tachypnea, wheezing, or
cough.

 Management:- Risk factors include advanced age,

frequent and recurrent episodes of sickling, evidence of
end organ damage.
Preoperative transfusion may be required for moderate to
severe risk procedures to maintain Hct of 30%
Prevent dehydration, acidosis, hypothermia to prevent
perioperative sickling
Torniquets are not contraindicated
Pain in postop period should be aggressively managed
Inhaled N2O reducing PAH and improves oxygenation
Acute chest syndrome develops 2-3 days postop usually
demanding treatment for hypoxemia, pain management
and blood transfusion to correct anemia

Thalassemia
Thalassemia minor:- presents with modest anemia and
rare morbidity due to hemolysis and ineffective
erythropoiesis
Thalessemia intermedia:- presents with more severe
anaemia with prominent microcytosis and hypochromia
May have visceromegaly and skeletal changes
secondary to bone marrow expansion making intubation
and regional anaesthesia probabaly diificult

 Thalessemia major:- severe anemia requiring frequent

blood transfusions
Have 3 defects depressing oxygen carrying capacity:
1. ineffective erythropoiesis
2. Hemolytic anaemia
3. hypochromia with microcytosis
Transfusion therapy may cause iron overload, cirrhosis,
right sided heart failure, endocrinopathy requiring
chelation therapy

Macrocytic/ megaloblastic
anemia

Due to folate or Vit B12 deficiency

Folate deficiency may be due to alcoholism or
malabsorption
Sustained exposure to N20 causes impaired Vit B12
activity
Also Vit B12 deficiency causes peripheral neuropathy
due to degeneration of lateral and posterior columns of
spinal cord
In the presence of neurological symptoms regional
anaesthesia to be avoided
Discouraging use of N20 during general anaesthesia

Microcytic anaemia
Correct the cause
Ferrous salts administered orally to correct anemia.
Increase of 2g/dl in 3weeks is favourable.
Hence postpone elective surgery for 4 weeks and
correct anemia preoperatively

Hemoglobins with altered oxygen

affinity
Increased oxygen affinity shifts ODC to left reducing
tissue oxygen delivery increasing erythrocytosis
polycythemia
Hence in such cases mild reduce in Hct reduces
tissue O2 delivery . If Hct> 55% preoperative
exchange transfusion and preventing hemo
concentration during surgery
Reduced oxygen affinity:- shifts ODC to right. Eg:
methamoglobinemia
Here ferric state of iron moiety is maintained thus
reducing oxygen transport tissue hypoxia

 Management :- invasive arterial monitoring to measure

BP, check saturation, methemoglobin levels by serial
ABG analysis
Acidosis to be corrected
ECG should be closely monitored for signs of ischemia
Avoid local anaesthetics, nitrates and N2O
Treatment:- 1-2mg/kg of 1% methylene blue in saline
over 3-5min. To repeated after 30min.
In patients with G6PD deficiency exchange transfusion
is preferred

Anaemia due to drug and radiation

associated marrow damage
Chemotherapy and many drugs cause pancytopenia
High energy radiation produces anaemia resulting from
marrow damage . Depending upon intensity of radiation
aplastic anaemia may result

Anaemia due to infection associated

marrow damage
Due to direct invasion of marrow by infectious agent as
in military TB
Due to immunosuppression of stem cell growth resulting
in aplastic anaemia as in viral hepatitis, EBV infection,
HIV infection, rubella
Parvo virus B19 causes acute reversible pure red cell
aplasia in patients with congenital haemolytic anemia

Tissue oxygen delivery and

haematocrit

Polycythemia
Can be relative polycythemia or primary
polycythemia( polycythemia vera)
Hct > 55-60% exponential increase in blood viscocity
reduction in blood flow
More prone for thrombotic events
Polycythemia vera due to mutation in JAK-2 gene.
Diagnosis:-elevated Hb level (>18.5 g/dL in men and >16.5 g/dL
in women) and either the presence of the JAK2 mutation or two of
the following:
hypercellularity of the bone marrow, a subnormal serum
erythropoietin level, and endogenous erythroid colony formation.

 Look for budd chairi syndrome, generalised pruiritis,

coronary or cerebral thrombosis, pulmonary HTN.
H/o previous phlebotomies, thrombotic complications,
myelofibrosis
Perioperatively prone for thrombosis and haemorrhage
Hemorrhage due to acquired VWF disease due to low
ultralarge VWF required for platelet adhesion
Phlebotomy and avoiding dehydration reduce risk of
thrombosis and haemorrhage perioperatively
Maintaining Hct <45% preoperatively
Withholding aspirin 7 days prior to surgery
Desmopressin and cryoprecipitate improve levels of VWF

Secondary polycythemia
Hypoxia causes increase in RBC mass with out increase in other
haematopoietic cell lines
High altitudes, congenital heart diseases, pulmonary disease,
extreme obesity, increased affinity heamoglobins, drugs causing
methamoglobinemia can cause secondary polycythemia
Due to increased erythropoietin levels:- as in renal diseases like
polycystic kidney disease, renal cysts, hydronephrosis, renal
tumours, uterine myomas, hepatomas, cerebellar haemangiomas.
Management :- specific to cause ..
Phlebotomy in cases of raised Hct >55% to prevent perioperative
complications.

Porphyrias
Inherited or acquired group of enzymatic defects
involved in haeme biosynthesis
Rate limiting step ALA synthase enzyme. Heme
exhibits negative feedback on ALA synthase.
Thus decrease in heameincrease in ALA synthase
activityincreased porphyrin accumulaton in tissues.
Acute porphyrias cause severe neuropathy neuronal
damage and axonal degeneration due to direcet toxicity
of ALA synthetase enzyme or reduced intraneuronal
haeme level

 Erythropoeitic porphyrias cause skin toxicity but lack

neurotoxicity. PCT is the only hepatic porphyria without
neurotoxicity and acute crisis
Most frequently porphyria crisis is drug precipitated
frequent drug being barbiturates
Fasting, dehydration, infection, psychological stress,
excessive alcohol intake, drugs precipitating factors
for acute porphyria crisis.
Acute attacks occur in only 4 types of porphyrias AIP,
HCP, VP, PLP

 Signs and symptoms include:- severe abdominal pain,

vomiting, anxiety, confusion, autonomic dysfunction
manifested by hypertension, tachycardia, dehydration,
electrolyte abnormalities like hyponatremia,
hypokalemia, hypocalcemia.
Tachycardia is indicator of disease state progression
Early detection of high risk patients by cDNA
sequencing is of great benefit.

 Preoperatively:- check for signs of acute porphyria crisis

If established porphyria look for precipitating factors
and last attack
Neurological evaluation focusing on mental condition,
peripheral neuropathy. And in acute crisis consider
cranial dysfunction and bulbar symptomatology may
lead to respiratory falilure
Premedications to reduce stress. Benzodiazepines are
considered safe. Opioids are safe except for pentazocine

 Intraoperatively :- regional can be given if no neurological involvement

noted
While in crisis to be avoided as patients are uncooperative, labile
hemodynamic stability and hypovolemia are more pronounced with
sympathectomy
Diazepam , midazolam can be safely given in crisis.
Thiopentone, etomidate, ketamine at high concetrations cause crisis .
Propofol can be used as inducing agent
Volatile anaesthetics, vecuronium, succinylcholine,atracurium safe
in porphyria
Pancuronium is contraindicated

 Propranololo given to treat tachycardia and

hypertension in crisis.
Alpha methyl dopa and hydral;azine contraindicated
Anticonvulsant prophylaxis
Monitoring for crisis to be continued postop for 5days.

Thank u