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Year : 2020  |  Volume : 23  |  Issue : 12  |  Page : 1772-1775

Combined Spinal and TAP Blocks for Laparoscopic Cholecystectomy for a Patient with Crigler-Najjar Type 2: A Case Report

Department of Anesthesiology, University of Health Sciences, Gazi Yasargil Diyarbakir Training and Research Hospital, Diyarbakir, Turkey

Date of Submission16-Jan-2020
Date of Acceptance20-Jun-2020
Date of Web Publication23-Dec-2020

Correspondence Address:
Dr. H Akelma
Health Sciences University Gazi Yasargil Training and Research Hospital, Diyarbakir
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/njcp.njcp_19_20

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Crigler-Najjar syndrome is a rare disease which is associated with congenital deficiency of uridine-diphosphate-gulukronyltransferase (UDP-glucuronosyltransferase, UGT) enzyme. In the surgery of these patients, it is necessary to use an anesthetic method that causes less damage to the liver. Spinal anesthesia is a good alternative to general anesthesia in these patients. Transversus abdominis plane block is a new method for ultrasound guided abdominal wall block. It is less invasive and relatively safer than conventional regional anesthetic techniques. Our case is a 30-year-old male patient with the diagnosis of Crigler-Najjar type 2 (Arias syndrome). There was a history of gallbladder edema, multiple stones and thickened gallbladder wall. We applied Transversus abdominis plane block in addition to spinal anesthesia as primary anesthesia for our patient who underwent laparoscopic surgery. We didn't experience any postoperative complications in our patient. In conclusion, laparoscopic surgery performed under combined spinal anesthesia and transvesus abdominis plane block in a Criggler Najjar type 2 (Arias syndrome) patient may be a simple and effective technique.

Keywords: Bilateral transversus abdominis block; crigler-najjar syndrome; spinal anesthesia

How to cite this article:
Bicak M, Akelma H, Salik F, Kaya S. Combined Spinal and TAP Blocks for Laparoscopic Cholecystectomy for a Patient with Crigler-Najjar Type 2: A Case Report. Niger J Clin Pract 2020;23:1772-5

How to cite this URL:
Bicak M, Akelma H, Salik F, Kaya S. Combined Spinal and TAP Blocks for Laparoscopic Cholecystectomy for a Patient with Crigler-Najjar Type 2: A Case Report. Niger J Clin Pract [serial online] 2020 [cited 2023 Jan 26];23:1772-5. Available from:

   Introduction Top

Crigler-Najjar syndrome is a rare disease, which is associated with a congenital deficiency of the uridine-diphosphate-gulukronyl-transferase enzyme, involved in the synthesis of hepatic microsomal bilirubin, and 0.6% per million, shows autosomal recessive transmission. The syndrome is found in two ways: type 1 has a complete enzyme deficiency, type 2 has a partial deficiency of the enzyme and is also called Arias syndrome.[1],[2]

Indirect hyperbilirubinemia is seen in patients due to the defect in the conjugation of bilirubin. The risk of gallbladder stone formation increases in patients with hyperbilirubinemia.[3] The possible mechanism is an increase in calcium bilirubinate compounds secondary to increased levels of unconjugated bilirubin in bile.[4] The picture of hyperbilirubinemia seen in this patient group may worsen due to drugs and metabolic changes in the perioperative period.

We aimed to present a patient with Crigler-Najjar type 2 (Arias syndrome) who underwent successful laparoscopic cholecystectomy under combined spinal anesthesia and bilateral TAP block for the first time.

   Case Report Top

After informed consent received from a 30-year-old, 70 kg, BMI 22.6 male patient who was diagnosed with Crigler-Najjar type 2 (Arias syndrome) was admitted to our hospital with complaints of nausea and vomiting and abdominal pain in the left shoulder. An abdominal ultrasonographic examination of the patient revealed multiple gallstones and thick edema of the gallbladder. It was determined that he had an episode of acute cholecystitis and surgical preparation for elective laparoscopic cholecystectomy was decided after oral intake restriction, antibiotic and analgesia treatment. Informed consent was obtained from the patient for the anesthesia technique to be applied to the patient. In pre-operative laboratory evaluation, total bilirubin level was 26.3 mg/dl, (0.3-1.2 mg/dl), direct bilirubin 0.62 mg/dl (0-0.3 mg/dl), and indirect bilirubin was measured as 25.7 mg/dl. (0-1 mg/dl). Other than these parameters, no pathological data were found in the biochemical evaluation. None of the drugs (phenobarbital, phenytoin, dexamethasone, hydrocortisone, para-aminosalicylic acid, omeprazole, clotrimazole and rifampicin) known to induce preoperative Uridyldiphosphoglucyl Transferase (UDPGT) enzyme were used in our patient.

The patient was given information for anesthesia; 6 hours of solid foods and 4 hours of fluid intake were restricted to the patient. The patient was taken to the operating room without premedication and was monitored for electrocardiography, peripheral oxygen saturation, and non-invasive blood pressure measurements (Dräger Infinity Delta XL Draeger Medical Trade and Service, Turkey). The patient's basal heart rate was 68/min, arterial blood pressure was 120/80 mmHg and peripheral oxygen saturation was 99%. The patient was given saline at 15 ml/kg via an intravenous route through an antecubital vein with 18 gauge cannula. In order to prevent post-operative nausea and vomiting, the patient was preemptively given ondansetron 4 mg (ZOFRAN™ 4 mg/2 ml Ampoule, GlaxoSmithKline) intravenously and position for spinal anesthesia. Spinal anesthesia was given by 17.5 mg heavy Marcaine (Buvasin 0.5% spinal heavy) following free CSF flow by using 27 gauge pencil-point spinal needle (Spinal Needle 27 g × 120 mm Quincke EgemenR Tmt Medical Industry And Trade Inc. İzmir, Turkey) from the Lumbar 3-4 range after the sterilization of the procedure area. The patient was extended to the back and pin-prick test was expected to block up to the level of thoracic 4. After an adequate block level, bilateral transversus abdominis plane block was applied to the patient to provide postoperative analgesia. During the block procedure, bilateral skin, subcutaneous, obligus externu and obligus internus muscles were passed through a block needle (Braun peripheral block needle stimplex 22 gauge) with the help of ultrasonographic image and spread to the fascia of the transversus abdominis muscle at 20 cc in each region. Marcaine (0.5% vial of Buvasin) was injected at a dose of 0.25 mg/ml and the surgical procedure was initiated [Figure 1] and [Figure 2]. The carbon dioxide flow rate was adjusted to 4-6 ml/min intraabdominal pressure <12 mm/H20 during the surgical period. In order to prevent the shoulder pain and discomfort in the patient, remifentanil was given with a continuous infusion of 0.1 mcg/kg/min intraoperatively. The surgical procedure time was 30 min. The patient was hemodynamically stable. Intraoperative blood pressure was 110/80 mmHg, heart rate was 85 atm/min, SpO2 was 99%. The patient did not have any complaints such as pain, restlessness, agitation and respiratory distress during an intraoperative surgical procedure. The patient's heart rate was 60/min, arterial blood pressure was 110/70 mm/Hg and peripheral oxygen saturation was 100 in the postoperative recovery room. Clinically, the patient was taken to the service and postoperative 48th-hour biochemical evaluation showed total bilirubin 25.4 mg/dl, direct bilirubin 0.5 mg/dl and indirect bilirubin value 24.8 mg/dl. No biochemical abnormal data were detected except bilirubin values and the patient was discharged, having been healing.
Figure 1: Probe position in medial transverse abdominis plane (TAP) block

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Figure 2: Ultrasound image of the injection of transverse abdominis plane (TAP) block. Local anesthetics were injected between the internal oblique and transversus abdominis muscle. EO: External oblique muscle. İO: Internal oblique muscle. TA: Transversus abdominis muscle

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   Discussion Top

Crigler-Najjar syndrome is a syndrome of congenital deficiency of the rare udp-glucronyl transferase enzyme, first described in 1952.[5] Due to the total deficiency of the enzyme in type 1, patients often present with kern icterus in the early period, while the partial form of type 2 deficiency defined by Arias in 1962 clinical progression proceeds slower and is often diagnosed in early childhood. Drugs and metabolites used in patients with Crigler-Najjar syndrome and bilirubin levels may increase due to changes in metabolism induced by surgery. Increasing blood bilirubin levels in anesthesia management should be avoided. Since acute infections, trauma and stress may increase the level of bilirubin, elective surgery should be planned in patients who are indicated for surgery and if there is an active infection in the gallbladder, it should be postponed after surgical treatment.[6] In a laboratory study, which was on the effects of drug and drug metabolites on albumin and bilirubin binding (albumin-bilirubin complex), conducted by Brodersen et al. sulfonamide, salicylate, ceftriaxone and ampicillin decreased albumin and bilirubin binding and increased blood-free bilirubin level. They also stated that conditions such as hypercarbia, acidosis, and long-term hunger increase the level of bilirubin.

Triglyceride metabolism increases with prolonged fasting and vomiting, and the resulting free fatty acids cause hyperbilirubinemia by separating albumin-bound bilirubin from its binding sites.

Therefore, it is important in this patient group not to starve for a long time before surgery, to maintain basal glucose infusion and to prevent postoperative vomiting. Therefore, intravenous 5% dextrose fluid should be started to prevent hypoglycaemia. If severe vomiting is present, ondansetron HCl should be given in intravenous fluid. It should also be avoided to use drugs such as furosemide, ceftriaxone, ampicillin, salicylates and sulfonamide, which have been shown to reduce bilirubin binding to albümin.[7]

Many drugs used in anesthesia practice have the potential to compete with bilirubin for UGT1A1. When we look at the agents used in induction and maintenance of anesthesia; although the safe use of thiopental and propofol has been described, there are theoretical concerns regarding both. The thiopental has a high-protein affinity and can likewise cause bilirubin displacement from the albumin binding sites. There is no specific data on protein binding of propofol, but it is known that the replacement of bilirubin-albumin induced by the fatty acid components of propofol results in increased free bilirubin.

Therefore, the anesthetic agents and anesthesia methods to be selected in this patient group should be carefully selected. It is important to avoid thiopental and propofol to prevent bilirubin elevation, especially during general anesthesia. Therefore, if the patient is suitable for regional anesthesia, firstly regional anesthesia should be preferred. Most local anesthetics are absorbed from the regional area. The ester group local anesthetics are significantly metabolized by pseudocholinesterase. Metabolites dissolved in water by ester hydrolysis are excreted in the urine. Amid group local anesthetics are metabolized in the liver by microsomal enzymes. Diseases that slow down the liver's blood flow slow down their metabolism and increase toxic side effects. Very few local anesthetics are excreted in the urine without changing. The bupivacaine amide group we use is a local anesthetic. Despite being excreted from the liver, special attention should be paid to patients with advanced liver disease.

However, there is no warning about its use in liver diseases (except for advanced liver disease). Therefore, we preferred regional anesthesia more predominantly in our patients.

When we evaluate neuromuscular blockers, it is known that rocuronium and vecuronium metabolisms are commonly found in the liver. Another 15% of Cis-atracurium, being a neuromuscular blocker, is protein-dependent. A major part of its metabolism is a benzylisoquinolinium agent known to occur through Hofmann elimination, and a small proportion of it occurs by ester hydrolysis, thus not affecting bilirubin metabolism.[8]

Although inhaled anesthetics such as sevoflurane and isoflurane used in the maintenance of anesthesia did not have a direct effect on the metabolism of bilirubin, it was observed that bilirubin increased in the postoperative period. However, there is no evidence that they cause hepatotoxicity.[9]

In an interesting study, there was no abnormality in liver function tests when sevoflurane or desflurane was given to non-surgical volunteers, so it is thought that the increases observed after surgery may be due to the perioperative changes. Therefore, inhaled anesthetic agents are a safe choice for general anesthesia in patients with Crigler-Najjar syndrome.[9],[10]

In order to avoid long-term hunger in our patient, we limited the hunger duration to 6 hours and ensured that it was taken into operation (corrected). On the other hand, we performed the surgery with spinal anesthesia to reduce the exposure to anesthetic agents and avoid hyperbilirubinemia. (correction was made in the patient presentation section) Spinal anesthesia is an advantage in laparoscopic surgery because it protects spontaneous breathing, decreases the incidence of postoperative nausea and vomiting, contributes to postoperative pain management and has early recovery time.[11] We maintained intraabdominal pressure <12 mm/H2O in order to prevent shoulder pain, which is common in 24%-43% of patients during spinal anesthesia and also we used remifentanil infusion for the duration of the case to prevent intraoperative discomfort.[12] The reason we prefer remifentanil is that it is a synthetic phenylpiperidine derivative with a short duration of action and a context-sensitive half-life, and its metabolism is independent of the liver and non-specific plasma and tissue esterases.[13]

Transversus abdominis plane block application is a very effective method in reducing postoperative pain and related stress response and also in reducing the incidence of postoperative nausea and vomiting.[14]

The bupivacaine used in block administration has a high degree of protein binding, but has a greater affinity for alpha 1 acid glycoprotein compared to albumin, and therefore, remains a safe option.[15] It also undergoes hepatic metabolism but proceeds through alkylation as opposed to glucuronidation.

In our case with Crigler-Najjar type 2 syndrome, we tried to minimize the pharmacological factors that may increase and affect bilirubin metabolism in various ways in the light of previous case information and experiences. We limited the duration of hunger to 6 hours and provided it to be taken as the first case under elective conditions.(corrected) In the intraoperative period, we preferred the regional anesthesia method, which we think is the least effective method for drug-bilirubin interaction metabolism. In order to reduce the discomfort of pneumoperitoneum during laparoscopic surgery, we adjusted intraabdominal pressure <12 cm/H2O and administered remifentanil infusion to reduce possible shoulder pain. We applied the bilateral transversus abdominis plane block to reduce the incidence of postoperative drug use and postoperative nausea and vomiting.

In conclusion, laparoscopic surgery performed under combined spinal anesthesia and transversus abdominis plane block in a patient with Crigler-Najjar type 2 (Arias syndrome) may be a simple and effective technique.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Roy Chowdhury J, and PLM Jansen. “Metabolism of bilirubin.” Hepatology: a Textbook of Liver Disease. 3rd ed. WB Saunders, Philadelphia 1996;323-47.  Back to cited text no. 1
Prager MC, Johnson KL, Ascher NL, Roberts JP. Anesthetic care of patients with Crigler-Najjar syndrome. Anesth Analg 2000;74:162-4.  Back to cited text no. 2
Kagita A, Adachi Y, Kambe A, Kamisako T, Yamamoto T. Type II Crigler-Najjar syndrome with intrahepatic cholestasis. J Gastroenterol 1994;29:214-7.  Back to cited text no. 3
Wittenburg H. Hereditary liver disease: Gallstones. Best Pract Res Clin Gastroenterol 2010;24:747-56.  Back to cited text no. 4
Crıgler JF Jr, Najjar V. Congenital familial nonhemolytic jaundice with kernicterus; A new clinical entity. AMA Am J Dis Child 1952;83:259-60.  Back to cited text no. 5
Robards C, Brull SJ. The anesthetic implications of Crigler-Najjar syndrome. Anesth Analg 2007;104:435-6.  Back to cited text no. 6
Brodersen R, Friis Hansen B, Stern L. Drug-induced displacement of bilirubin from albumin in the newborn. Dev Pharmacol Ther 1983;6:217-29.  Back to cited text no. 7
De Wolf AM, Freeman JA, Scott VL, Tullock W, Smith DA, Kisor DF, et al. Pharmacokinetics and pharmacodynamics of cisatracurium in patients with end-stage liver disease undergoing liver transplantation 1. Br J Anaesth 1996;76:624-8.  Back to cited text no. 8
Bito H, Ikeda K. Renal and hepatic function in surgical patients after low-flow sevoflurane or isoflurane anesthesia. Anesth Analg 1996;82:173-6.  Back to cited text no. 9
Cullen SC, Eger EI 2nd, Cullen BF, Gregory P. Observations on the anesthetic effect of the combination of xenon and halothane. Anesthesiology 1969;31:305-9.  Back to cited text no. 10
Tiwari S, Chauhan A, Chaterjee P, Alam MT. Laparoscopic cholecystectomy under spinal anaesthesia: A prospective, randomised study. J Minim Access Surg 2013;9:65-71.  Back to cited text no. 11
Sinha R, Gurwara AK, Gupta SC. Laparoscopic cholecystectomy under spinal anesthesia: A study of 3492 patients. J Laparoendosc Adv Surg Tech 2009;19:323-7.  Back to cited text no. 12
Tegeder I, Lötsch J, Geisslinger G. Pharmacokinetics of opiods in liver disease. Clin Pharmacokinet 1999;37:17-40.  Back to cited text no. 13
Peng K, Ji FH, Liu HY, Wu SR. Ultrasound guided transversus abdominis plane block for analgesia in laparoscopic cholecystectomy: A systematic review and meta analysis. Med Princ Pract 2016;25:237-2-46.  Back to cited text no. 14
Odor PM, Cavalier AG, Reynolds ND, Ang KS, Parrington SJ, Xu H, et al. Safety and pharmacokinetics of levobupivacaine following fascia iliaca compartment block in elderly patients. Drugs Aging 2019;36:541-8.  Back to cited text no. 15


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