[Downloaded free from http://www.mjdrdypu.org on Friday, December 27, 2019, IP: 45.251.33.

52]

Case Report

Anesthetic management of a case with

hereditary spherocytosis for splenectomy and
open cholecystectomy
Sonal S. Khatavkar, Widya S. Thatte, Syed M. Kazi, Arnab Paul
Department of Anesthesiology, Padmashree Dr. D. Y. Patil Medical College, Hospital and Research Centre, Pune, Maharashtra, India

Access this article online

ABSTRACT
Hereditary spherocytosis (HS) is a familial hemolytic disorder
with marked heterogeneity of clinical features ranging from
asymptomatic condition to a fulminant hemolytic anemia. HS is
characterized by the strong family history of anemia, jaundice,
splenomegaly and cholelithiasis. Anesthetic Management of
HS with liver dysfunction is very challenging since most of the
anesthetic drugs are metabolized by the liver. Hereby, we report
anesthetic management in a case of HS with splenomegaly and
gall stones for elective splenectomy and cholecystectomy.
Keywords: Cholecystectomy, hereditary spherocytosis,
splenomegaly
Introduction
Hereditary spherocytosis (HS) is an extremely rare autosomal
dominant disorder. HS is a familial hemolytic disorder with
marked heterogeneity of clinical features ranging from
asymptomatic condition to a fulminant hemolytic anemia.[1]
It occurs due to an intrinsic defect in the red cell membrane
as a result of which cells have a spherocytic shape.[2] The
estimated prevalence in the Caucasian population ranges
from 1:2000 to 1:5000.[3] It is characterized by a deficiency
of ankyrin or spectrin (transmembrane proteins) that
link the bilayer of red cells to the membrane skeleton.
The spherocytes are susceptible to osmotic lysis. In 80%
instances, the inheritance of HS is autosomal dominant
and in others autosomal recessive.[4] HS is diagnosed by
strong family history of anemia, jaundice, splenomegaly and
cholelithiasis. In two-thirds of the cases, it is inherited as an
autosomal dominant trait, and in the remaining as sporadic
mutations or recessive genes.[5] Altered liver function and
metabolism of anesthetic agents in the liver in these patients
can be very challenging.
Quick Response Code:
Website:
www.mjdrdypu.org
DOI:
10.4103/0975-2870.177686
Case Report
A 32-year-old male presented with fever which was high-
grade continuous nature, pain in the abdomen localized
to the left hypochondrium and yellowish discoloration
of eyes and urine since 15 days. No history of neonatal
jaundice, alcohol intake or blood transfusion. No history of
consanguineous marriage. In his family history, his mother
had similar complaints and died of jaundice. In past, the
patient was hospitalized 3-4 times in a hospital in view of
increased bilirubin levels where he was given palliative
treatment.
On general examination height 160 cm, weight 60 kg,
pulse rate 96/min, blood pressure (BP) 100/60 mmHg,
This is an open access article distributed under the terms of the
Creative Commons Attribution-NonCommercial-ShareAlike 3.0
License, which allows others to remix, tweak, and build upon the
work non-commercially, as long as the author is credited and the
new creations are licensed under the identical terms.
For reprints contact: reprints@medknow.com
How to cite this article: Khatavkar SS, Thatte WS, Kazi SM, Paul A.
Anesthetic management of a case with hereditary spherocytosis
for splenectomy and open cholecystectomy. Med J DY Patil Univ
2016;9:267-70.

Address for correspondence:

Dr. Sonal S. Khatavkar, Flat S2/B1, Tejovalaya, Raviraj CHSL, Warje, Pune - 411 058, Maharashtra, India.
E-mail: drsonalkhatavkar@yahoo.co.in

© 2016 Medical Journal of Dr. D.Y. Patil University | Published by Wolters Kluwer - Medknow 267
[Downloaded free from http://www.mjdrdypu.org on Friday, December 27, 2019, IP: 45.251.33.52]
Khatavkar, et al.: Anesthetic management of hereditary spherocytosis
respiratory rate 16/min. Afebrile, pale, icterus present, and
no lymphadenopathy. Abdominal examination patient had
hepatomegaly-1½ inch below costal margin with grade
II splenomegaly. Rest systemic examination was normal
[Figure 1].
Laboratory investigations revealed hemoglobin 7.7 g%
platelet count 346,000/cc total bilirubin: 15.2 mg/dL
direct bilirubin: 7.2 mg/dL indirect bilirubin: 8.0 mg/dL,
enzymes — alanine transaminase: 122 IU/L, aspartate
transaminase: 102 IU/L, alkaline phosphatase: 128 IU/L.
International Normalization Ratio: 1.3 peripheral blood
smear showed spherocytosis and abnormally shaped
poikilocytes. Osmotic fragility of incubated blood cells
was markedly increased. Direct and indirect Coombs test
were negative hemoglobin electrophoresis was normal.
Ultrasonography suggestive of multiple gall stones and
hepatosplenomegaly with mesenteric lymphadenopathy.
Electrocardiogram (ECG) and chest X-ray findings were
within normal limits.
The patient received 3 units of packed red blood cells
(PRBCs), 2 units of fresh frozen plasma and injection
Vitamin K 10 mg intramuscularly. Vaccination against
pneumococci and hepatitis B was given 14 days prior to
surgery.
The patient was nil orally from 12 midnight. Patient
took in the operating room, 18 gauges cannula secured.
Standard monitors such as 5 lead ECG, noninvasive BP,
pulse oximeter attached. End tidal CO2 and temperature
monitoring were done throughout the surgery. The
baseline heart rate was 70/min, BP: 100/60 mmHg and
SpO 2: 100%. Under all aseptic precaution, epidural
catheter was threaded through the epidural needle no. 18
at T12-L1 level and the test dose was given with 3 mL of
2% lignocaine with adrenaline. An initial dose of 5 mL
Figure 1: Sclera showing icterus
268
0.5% bupivacaine was given 15 min later. Ryle’s tube was
inserted. Premedicated with injection glycopyrolate (0.2
mg), injection ondansetron (4 mg), injection midazolam
(1 mg), and injection fentanyl 60 g intravenously (IV).
General anesthesia given with injection propofol (120
mg), injection succinylcholine (100 mg) IV. Endotracheal
Intubation was done gently with cuffed portex tube no. 8.5
mm. Bilateral air entry checked, cuff inflated, tube fixed and
anesthesia maintained on O2:N2O 50%:50% and isoflurane
0.8 mac intermittent positive pressure ventilation with a
closed circuit.
Injection atracurium 25 mg was given for relaxation and
subsequent top ups 0f 5 mg IV at regular intervals of 30
min. Epidural top ups of 5 mL of 0.25% bupivacaine at an
interval of 40 min. The patient received 2 units fresh frozen
plasma and 1 unit of PRBCs after clamping the splenic
vessels. Total blood loss was 500 mL and urine output
400 mL. The intra-operative temperature was 35°C-35.5°C.
Arterial blood gas (ABG) done was normal. The patient
was extubated uneventfully at the end of the surgery and
then shifted to intensive care for observation. One unit
of packed cell volume was administered postoperatively.
The epidural catheter was removed 24 h after the surgery
under all aseptic precautions after giving inj.bupivacaine
0.125% 5cc and 50 mg of tramadol for postoperative pain
relief [Figure 2].
Discussion
HS is an autosomal dominant disorder. It occurs due to a
defect in the genes coding for proteins ankyrin or β spectrin.
Qualitative or quantitative abnormalities in these proteins
cause the red cells to become spheroidal and increased
susceptibility to lysis. The molecular defect involves the
genes encoding for spectrin, ankyrin, band 3, protein 4.2.
Figure 2: Specimen of spleen
Medical Journal of Dr. D.Y. Patil University | March-April 2016 | Vol 9 | Issue 2
[Downloaded free from http://www.mjdrdypu.org on Friday, December 27, 2019, IP: 45.251.33.52]
Khatavkar, et al.: Anesthetic management of hereditary spherocytosis
In north India, both autosomal and recessive patterns have
been reported but both have a similar presentation, but
underlying protein defect has not been characterized.[6]
Patients with HS present with anemia, jaundice,
splenomegaly and cholelithiasis. Many patients have
compensated hemolysis and a normal hemoglobin level with
reticulocytosis.[7] In children, there is growth retardation due
to hemolysis and bone changes due to marrow hypertrophy.
Splenomegaly is usually mild to moderate. The size of spleen
per se is not an indication to splenectomy.
Pigmented gallstones are seen in more than 50% cases.
The incidence increases with the severity of hemolysis and
with age. The hemolytic crisis may occur, often triggered
by viral infections.
Laboratory diagnosis involves a peripheral blood smear, in
which spherocytes are seen. Acanthocytes or speculated
red cells may also be seen. The mean corpuscular volume
is usually normal, but mean corpuscular hemoglobin
concentration is often increased. The reticulocyte count
is often increased. Osmotic fragility test is often positive.
Immunization with pneumococcal and hemophilus
influenza vaccines is indicated, if splenectomy is considered
and is to be given 14 days prior surgery. If gallstones are
present, cholecystectomy is done either at the same surgery
or subsequently. If splenectomy is done in a patient with
symptomatic gallstone, cholecystectomy should be done
concomitantly.[8]
Commonly recommended peri-operative management
includes erythrocyte transfusion, aggressive hydration,
avoidance of hypoxia, hypothermia, and acidosis.
Perioperative goals in such patients to minimize stress by
providing adequate analgesia, to avoid hepatotoxic drugs,
to maintain hepatic blood flow. Intra-operative blood
loss should be replaced when necessary and normal body
temperature to be maintained to minimize vasoconstriction
and associated circulatory stasis. Administer warm fluids
to maintain proper operating room temperature. ABG
measurements to monitor acid-base status.[9]
Epidural anesthesia was chosen in our patient primarily
because of the potential deterioration in liver function and
liver blood flow that may occur with general anesthesia
alone. Studies have shown that controlled ventilation,
inhalational anesthetics and surgical stress can decrease
liver blood flow.[10] Regional anesthesia probably preserves
liver blood flow as long as normotension is maintained.
Epidural anesthesia is desired as the stress associated with
Medical Journal of Dr. D.Y. Patil University | March-April 2016 | Vol 9 | Issue 2
general anesthesia can lead to the release of catecholamines,
which can decrease liver blood flow. Amide local
anesthetics, are metabolized primarily by microsomal P-450
enzymes in the liver (N-dealkylation and hydroxylation).
The rate of metabolism in liver among amides vary as
follows: Prilocaine > lidocaine > mepivacaine > ropivacaine
> bupivacaine. Hence, bupivacaine is the preferred agent
for epidural anesthesia. Epidural anesthesia also reduces the
requirement of skeletal muscle relaxants and inhalational
anesthetics.
All volatile agents decrease total liver blood flow, the
decrease is maximum with halothane and minimum
with isoflurane. Isoflurane is the most preferred volatile
anesthetic agent in such conditions.
The management of such rare disorders is largely dependent
on the severity of anemia and degree of hemolysis.[11] Anemia
should be corrected preoperatively before a major surgery.
Surgery should be avoided in the presence of hemolytic
crisis.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
References
1. Perrotta S, Gallagher PG, Mohandas N. Hereditary spherocytosis.
Lancet 2008;372:1411-26.
2. Firkin F, Chesterman C, Penington D, Rush B. The haemolytic
anaemias. In: Firkin F, Chesterman C, Penington D, Rush B,
editors. De Gruchy’s Clinical Haematology in Medical Practice.
5th ed. London: Blackwell Science; 1991. p. 182-4.
3. Mariani M, Barcellini W, Vercellati C, Marcello AP, Fermo E,
Pedotti P, et al. Clinical and hematologic features of 300
patients affected by hereditary spherocytosis grouped according
to the type of the membrane protein defect. Haematologica
2008;93:1310-7.
4. Das MR, Ananthakrishnan S. Hereditary spherocytosis in a
family from Tamil Nadu. Indian Pediatr 2005;42:610-1.
5. Rinder CS. Hematologic disorders. In: Hines RL, Marschall KE,
editors. Anesthesia and Co-Existing Disease. 5th ed. Pennsylvania:
Elsevier; 2008. p. 409.
6. Panigrahi I, Phadke SR, Agarwal A, Gambhir S, Agarwal SS.
Clinical profile of hereditary spherocytosis in North India. J
Assoc Physicians India 2002;50:1360-7.
7. Bolton-Maggs PH, Stevens RF, Dodd NJ, Lamont G,
Tittensor P, King MJ, et al. Guidelines for the diagnosis and
management of hereditary spherocytosis. Br J Haematol
2004;126:455-74.
8. Bolton-Maggs PH, Langer JC, Iolascon A, Tittensor P, King MJ;
General Haematology Task Force of the British Committee for
269
[Downloaded free from http://www.mjdrdypu.org on Friday, December 27, 2019, IP: 45.251.33.52]

Khatavkar, et al.: Anesthetic management of hereditary spherocytosis

Standards in Haematology. Guidelines for the diagnosis and
management of hereditary spherocytosis — 2011 update. Br J
Haematol 2012;156:37-49.
9. Gelman S. General anesthesia and hepatic circulation. Can J
Physiol Pharmacol 1987;65:1762-79.
10. Runciman WB, Mather LE, Ilsley AH, Carapetis RJ, Upton RN.
A sheep preparation for studying interactions between blood
flow and drug disposition. III: Effects of general and spinal
anaesthesia on regional blood flow and oxygen tensions. Br J
Anaesth 1984;56:1247-58.
11. Mason R, editor. Medical disorders and anaesthetic problems:
Hereditary spherocytosis. In: Anaesthesia Databook — A
Perioperative and Peripartum Manual. 3 rd ed. London:
Greenwich Medical Media Ltd.; 2001. p. 245-6.

270 Medical Journal of Dr. D.Y. Patil University | March-April 2016 | Vol 9 | Issue 2