|Year : 2016 | Volume
| Issue : 2 | Page : 267-270
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
|Date of Web Publication||1-Mar-2016|
Sonal S Khatavkar
Flat S2/B1, Tejovalaya, Raviraj CHSL, Warje, Pune - 411 058, Maharashtra
Source of Support: None, Conflict of Interest: None
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
|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
|How to cite this URL:|
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 [serial online] 2016 [cited 2020 Jan 22];9:267-70. Available from: http://www.mjdrdypu.org/text.asp?2016/9/2/267/177686
| 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.  It occurs due to an intrinsic defect in the red cell membrane as a result of which cells have a spherocytic shape.  The estimated prevalence in the Caucasian population ranges from 1:2000 to 1:5000.  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.  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.  Altered liver function and metabolism of anesthetic agents in the liver in these patients can be very challenging.
| 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, 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 CO 2 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 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 μg 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 O 2 :N 2 O 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. 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. 
Patients with HS present with anemia, jaundice, splenomegaly and cholelithiasis. Many patients have compensated hemolysis and a normal hemoglobin level with reticulocytosis.  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. 
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. 
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.  Regional anesthesia probably preserves liver blood flow as long as normotension is maintained. Epidural anesthesia is desired as the stress associated with 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.  Anemia should be corrected preoperatively before a major surgery. Surgery should be avoided in the presence of hemolytic crisis.
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Conflicts of interest
There are no conflicts of interest.
| References|| |
Perrotta S, Gallagher PG, Mohandas N. Hereditary spherocytosis. Lancet 2008;372:1411-26.
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. 5 th
ed. London: Blackwell Science; 1991. p. 182-4.
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.
Das MR, Ananthakrishnan S. Hereditary spherocytosis in a family from Tamil Nadu. Indian Pediatr 2005;42:610-1.
Rinder CS. Hematologic disorders. In: Hines RL, Marschall KE, editors. Anesthesia and Co-Existing Disease. 5 th
ed. Pennsylvania: Elsevier; 2008. p. 409.
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.
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.
Bolton-Maggs PH, Langer JC, Iolascon A, Tittensor P, King MJ; General Haematology Task Force of the British Committee for Standards in Haematology. Guidelines for the diagnosis and management of hereditary spherocytosis - 2011 update. Br J Haematol 2012;156:37-49.
Gelman S. General anesthesia and hepatic circulation. Can J Physiol Pharmacol 1987;65:1762-79.
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.
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.
[Figure 1], [Figure 2]