Surg Endosc (2009) 23:1599–1602 DOI 10.1007/s00464-009-0501-8

Laparoscopic cholecystectomy in 427 adults with sickle cell disease: a single-center experience Abdulrahman S. Al-Mulhim Æ Abdulmohsen A. Al-Mulhim

Received: 2 December 2008 / Accepted: 29 March 2009 / Published online: 15 May 2009 Ó Springer Science+Business Media, LLC 2009

Abstract Background Sickle cell disease (SCD) is common in the Eastern Province of Saudi Arabia. Patients with this disease have an increased risk for gallstones. Cholecystectomy is the most common general surgical procedure performed in patients with SCD. Laparoscopic cholecystectomy (LC) is the standard treatment for symptomatic gallstones. To date, only small series of LC in adults with SCD have been reported. We report a large series of LC in these patients. Methods A retrospective study included all adults with SCD who underwent LC for symptomatic gallstones at King Fahad Hospital, Hofuf, Saudi Arabia, during a 13year period. Data analyzed were patient demographics, preoperative blood transfusion, duration of operation, rates of conversion and morbidity, and length of hospital stay. Results There were 427 patients; 56% were women with a mean age of 21 years. Blood transfusion was used in 393 patients (92%). Nineteen patients (4.5%) had endoscopic extraction of common bile duct (CBD) stones before LC. Overall, conversion to open surgery was necessary in 21 patients (5%). However, the conversion rate significantly decreased from 9% in the first half of the study to 2% during the second half (p \ 0.001). The mean operation time was 76 min. There were 31 complications (7%), mostly related to SCD. There was no mortality, and the mean hospital stay was 2.6 days.

A. S. Al-Mulhim Department of Surgery, King Fahad Hospital, Hofuf, Saudi Arabia A. A. Al-Mulhim (&) Department of Surgery, King Fahd Hospital of the University, P.O. Box 1917, Al-Khobar 31952, Saudi Arabia e-mail: [email protected]

Conclusion Provided that standard perioperative care is ensured and the procedure is performed by experienced surgeons, LC in adults with SCD results in low rates of conversion and morbidity, no mortality, and a short hospital stay. Keywords Sickle cell disease
Cholelithiasis
Laparoscopic cholecystectomy
Blood transfusion
Conversion
Morbidity

Sickle cell disease (SCD) is the most common inherited hemolytic anemia worldwide. In Saudi Arabia, the disease is most common in the Eastern Province, where 1.7–3.8% of the population is affected [1, 2]. Patients with SCD suffer from chronic hemolysis, which results in an increased risk of pigment gallstones. This risk increases with age and 50–85% of adults with SCD have gallstones [3–5]. At present, patients with SCD have a longer life expectancy due to improved management. Furthermore, most of them develop gallstone symptoms and require surgical intervention [4, 6]. Hence, cholecystectomy is the most common general surgical operation performed in adults with SCD [6]. For patients without SCD, laparoscopic cholecystectomy (LC) is the optimal treatment of symptomatic gallstones. Compared with open cholecystectomy, LC reduces postoperative pain, morbidity, mortality, scarring, hospital stay, recovery period, and costs [7]. These benefits are very important for patients with SCD, who are immunocompromised and at a higher risk to develop postoperative infection. To date, only small series of LC in adults with SCD have been reported [8–15]. We report our experience with a large series of LCs in adults with SCD.

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Patients and methods This retrospective study was conducted at King Fahad Hospital, Hofuf, Eastern Saudi Arabia, between January 1994 and December 2006. This hospital is the main center for the management of patients with SCD in the region. Included were all patients with SCD (HB-SS) who underwent LC for symptomatic gallstones, e.g., biliary colic, acute cholecystitis, or extrahepatic biliary obstruction. Excluded were (1) patients with other inherited hemolytic disorders, e.g., sickle cell trait, HB-SC, and S-B thalassemia, and (2) those who had incidental LC. All patients had hemoglobin electrophoresis to confirm SCD and ultrasound examination to detect gallstones and evaluate the biliary system. Preoperative selective endoscopic retrograde cholangiopancreatography (ERCP) was adopted based on standard criteria [11]. Initially, we used an aggressive transfusion regimen (simple or exchange transfusion) to achieve a preoperative hemoglobin (Hb) C10 g/dl and a hemoglobin S percent B50. Since 2002, only simple blood transfusion with packed red cells was given to achieve a preoperative Hb C10 g/dl regardless of hemoglobin S percent. Blood transfusion was usually given 1–2 days before LC. Perioperative management A multidisciplinary approach was adopted involving hematologist, anesthetist, and surgeon. All patients were nursed in the general surgical ward. Those with biliary colic were usually admitted 1–2 days before surgery and received a single dose of prophylactic cefuroxime at induction of anesthesia. Patients with acute cholecystitis were operated upon within the first 5 days of presentation. They received ampicillin, metronidazole, and gentamicin on admission, which were stopped 2 days after surgery. Upon admission, patients were encouraged to drink clear fluids freely. On the night before LC, patients received intravenous hydration with crystalloids at 1.5 times the maintenance rate. This was continued postoperatively until resumption of full oral intake. They were kept warm, well oxygenated, and pain-free throughout the perioperative period. Incentive spirometry was used in all patients.

Intraoperative care LC was performed under general anesthesia using the standard four-port technique. A closed method was used to create the CO2 pneumoperitoneum (12 mmHg) and to introduce the subumbilical port. Occasionally, trocars in the right axillary line and midclavicular line were inserted more inferiorly due

Surg Endosc (2009) 23:1599–1602

to hepatomegaly. Foley catheter was used routinely to ensure adequate monitoring of urine output during surgery. Data analyzed were patients’ age and sex, preoperative Hb level and blood transfusion, duration of operation, rates of complications and conversion from laparoscopic to open cholecystectomy, and hospital stay. Fisher’s exact test was used to compare the conversion rates during the first and second halves of the study. A p value \0.05 was considered significant. Results During the study period, 5,245 patients underwent LC. Of these, 427 (8.1%) adult patients had SCD (HB-SS). The mean age was 21.2 (range, 14–34) years, and 56.2% were women (Table 1). The mean preoperative level of Hb was 8.7 (range, 6.5–11.4) g/dl. The mean hemoglobin S (HbS) was 71% (range, 56–91%) and that of hemoglobin F (HbF) was 23.4% (range, 8.4–27%). A total of 257 patients (60.2%) received simple blood transfusion. Partial exchange transfusion was given to 136 patients (31.8%); 34 patients (8%) received no transfusion. Fifty-three patients (12.4%) had preoperative ERCP for suspected common bile duct (CBD) stones. Only 19 of these (35.8%) had CBD stones; they had endoscopic sphincterotomy and stone extraction before surgery. Twenty-one patients (4.9%) were converted to open cholecystectomy: 13 for dense adhesions at Calot’s triangle, and 8 for uncontrolled bleeding from cystic artery. During the first half of the study, the rate of conversion was 9% (16/173). Thereafter, it declined to 2% (5/254). This difference was statistically significant (p \ 0.001). The mean operation time was 76 (range, 35–240) min. Table 1 Outcome of laparoscopic cholecystectomy in 427 adults with sickle cell disease Age (years)

21.2 (14–34)

Male/female

187/240

Preoperative Hb (g/dl)

8.7 (6.5–11.4)

Preoperative transfusion: no (%)

393 (92)

CBD stones: no (%)

19 (4.5)

Conversion: no (%)

21 (4.9)

Operation time (min)

76 (35–240)

Morbidity: no (%)

31 (7.3)

Vaso-occlusive lower limb crisis: no

13

Acute chest syndrome: no

8

Vaso-occlusive abdominal crisis: no

6

Superficial wound infection: no

4

Mortality: no (%)

0

Hospital stay (days)

2.6 (1–9)

CBD common bile duct Values are reported as mean (range) or number (%)

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There were 31 complications (7.3%), including vasoocclusive lower limb crisis (n = 13), acute chest syndrome (ACS, n = 8), vaso-occlusive abdominal crisis (n = 6), and superficial wound infection (n = 4). There was neither CBD injury nor death. There were no long-term complications during a mean follow-up of 18 (range, 7–43) months. The mean hospital stay was 2.6 (range, 1–9) days.

Discussion The true incidence of SCD in adults subjected to cholecystectomy is not known. In our setting, 8% of LCs was performed in patients with SCD. This figure is markedly higher than 0.31% reported in Greece by Bonatsos et al. [10]. This difference may reflect variation in the epidemiology of SCD and gallstones, and the patients studied. Like others [5, 16], we tend to avoid cholecystectomy in patients with SCD with asymptomatic gallstones, because surgical intervention in these patients has an increased risk of complications, particularly those related to SCD [4, 6, 13–16]. Walker et al. showed that only a small proportion of patients with SCD with asymptomatic gallstones develop biliary colic or other gallstone complications [5]. Hence, we believe that prophylactic cholecystectomy for sicklers with asymptomatic gallstones is unwarranted. However, we agree with those who advocate early elective cholecystectomy for sicklers with symptomatic gallstones to prevent serious complications [4, 10]. In addition, cholecystectomy simplifies the management of acute abdomen in these patients. This is because the abdominal pain of hepatic crisis is often indistinguishable from biliary colic. Several preoperative transfusion regimens have been used to reduce the risk of perioperative complications in patients with SCD. These regimens include single or repeated simple transfusion and exchange transfusion

1–15 days before LC. However, at present there is no consensus regarding the value of preoperative transfusion in these patients [4, 6, 13, 14, 17, 18] (Table 2). In addition, the benefits of blood transfusion should always be weighed against the risks of infective agents, hemolysis, and alloimmunization [17]. Early in our experience, we employed an aggressive approach. Later on, we used a more conservative approach. This shift was not associated with increased morbidity. Hence, we advocate simple blood transfusion with packed red cells to achieve a preoperative Hb C10 g/dl regardless of hemoglobin S percent. Based on ERCP, CBD were present in 19 of our 427 patients (4.5%). This figure is lower than that reported by Plummer et al. (31%) [13] and Duncan et al. (37%) [16], who employed routine ERCP or intraoperative cholangiogram, respectively. Despite our practice of selective ERCP, no CBD stones were missed during a mean follow-up of 18 months. Noninvasive tests, such as magnetic resonance cholangiopancreatography [19] and intraoperative ultrasound [20], are being increasingly used to evaluate the biliary ducts in sicklers subjected to LC. A conversion rate of 5% among our patients is within the range reported by others [8, 9, 14], except that of 25% reported by Plummer et al. [13] (Table 2). With increasing experience in the overall management, we succeeded to significantly decrease the conversion rate from 9% in the first half of the study to 2% in the second half. Similar to the experience in patients without SCD, most of the conversions were due to obscure anatomy. In this series, 87% of the postoperative complications were related to SCD. ACS, encountered in eight patients, was the most serious complication. However, all patients with this syndrome had full recovery after aggressive medical management, including supplementary oxygenation, chest physiotherapy, and parenteral antibiotics. None of them, however, required mechanical ventilation or

Table 2 Literature review of laparoscopic cholecystectomy in adults with sickle cell disease (SCD) Meshikhes [8]

Leandros [9]

Bonatsos [10]

Plummer [13]

Leff [14]

Diarra [15]

Present study

No. of patients

71

41

13

16

14

47

427

Mean age (years)

25

24

34.7

28.5

34.5a

25.5a

21.2

Mean preoperative Hb (g/dl)

9

11.8

8.4

8.8

9

10.7

8.7

Preoperative TF (%)

77.5

92.7

69.2

0

0

78.7

92

Conversion (%)

5.6

5

NA

25

7.1

NA

4.9

Mean OT (min)

80

64

NA

108

NA

71.4

76

Total morbidity (%)

14

5

7.7

37.5

35.7

34

7.3

SCD-related morbidity

4

0

0

31

7

15

6

Mortality (%)

1.4

0

0

6.3

0

0

0

Mean total stay (days)

2.5a

2.7

3.3

5.5b

3a

7.7

2.6

Hb hemoglobin; TF transfusion; NA not available; OT operation time a

Median

b

Postoperative stay

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1602

intensive care management. Laparoscopy-related complications were limited to four cases of superficial wound infection. These encouraging findings may be attributable to our perioperative management, which pays attention to details to prevent dehydration, hypoxia, hypercarbia, hypothermia, acidosis, and infection. This practice involves hematologists, anesthetists, and surgeons who are experienced in the medical and surgical management of patients with SCD. It also involves dedicated nursing and physiotherapy staff. In addition, patients in our region have increased levels of HbF, which accounts for a relatively mild form of SCD [21]. The mean total hospital stay of 2.6 days documented herein compares favorably with the figures reported after LC in the general population [7] as well as those with SCD (Table 2). Compared with open cholecystectomy, LC in patients with SCD reduces operation time, postoperative pain, complications, and hospital stay [9]. Hence, most surgeons advocate LC in these patients [6, 8–12, 14]. Yet, two reports have documented an increased incidence of ACS after LC in patients with SCD [13, 15]. These reports, however, emphasize the importance of SCD rather than LC in determining the outcome in these patients. Although we accept the limitations of a retrospective study, large series, such as ours, are necessary to confirm the safety of LC in adults with SCD. However, further studies are needed to assess the natural course of asymptomatic gallstones, and the value of preoperative blood transfusion before LC in adults with SCD. We conclude that in an experienced center LC in SCD can be completed in most patients with low rate of morbidity, short hospital stay, and no mortality.

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