Saudi Journal of Gastroenterology
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Year : 2006  |  Volume : 12  |  Issue : 3  |  Page : 130-134
Laparoscopic cholecystectomy in children with sickle cell disease


Department of Surgery, King Fahad Hospital- Hofuf, Hofuf, Al-Hassa, Saudi Arabia

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   Abstract 

Background : The aim of this study was to report the outcome of laparoscopic cholecystectomy in children with sickle cell disease and to compare the patients who had surgery as emergency with those who were operated as routine. Materials and Methods: A total of 72 patients (males 48, females 24) less than 14 years of age (ranging between 6 and 14 years) underwent cholecystectomy at the King Fahad Hospital, Hofuf, in the last 5 years. The records of these patients were reviewed retrospectively, comparing the outcome of surgery in emergency and routine cases. Results: The mean operating time and the mean hospital stay were longer in the emergency group (91.3 min, 8.5 days) compared to routine group (75.8 min, 4.5 days respectively), with high incidence of postoperative complication in the emergency group. Conclusion: This study showed that elective laparoscopic cholecystectomy by an experienced surgeon and taking necessary precautions are the safety measures to be recommended at the time of diagnosis of cholelithiasis in sickle cell disease children.

Keywords: Children, cholecystectomy, sickle-cell

How to cite this article:
Al-Mulhim AS, Abdulatif MM, Ali AM. Laparoscopic cholecystectomy in children with sickle cell disease. Saudi J Gastroenterol 2006;12:130-4

How to cite this URL:
Al-Mulhim AS, Abdulatif MM, Ali AM. Laparoscopic cholecystectomy in children with sickle cell disease. Saudi J Gastroenterol [serial online] 2006 [cited 2019 Aug 21];12:130-4. Available from: http://www.saudijgastro.com/text.asp?2006/12/3/130/29753


Cholelithiasis is thought to be uncommon in infants and children, but recently the incidence has increased. This may indicate actual increase in the cases or improvement in the detection of gallstones.[1]

The most common condition associated with cholelithiasis in children is hemolytic anemia, as in sickle cell disease. Sickle cell disease (SCD) is an inherited disorder of the red blood cells, which is characterized by hemolysis and recurrent vasoocclusive episodes. In AL-HASA area of the Eastern province of Saudi Arabia, the incidence of sickle cell trait and sickle cell disease is 20.02 and 1.08% respectively.[2]

The incidence of gallstones in sickle cell disease patients varies from 10 to 37% and increases with age.[3],[4] The frequency of cholelithiasis in children with SCD makes cholecystectomy, open or laparoscopic, a common procedure.[5] Children with SCD are at increased risk of complications due to cholelithiasis and postoperative complications.[6]

The aim of this study was to make a comparison between emergency cholecystectomy and elective cholecystectomy in children with sickle cell disease with respect to clinical presentation, surgical management and the postoperative course.


   Materials and Methods Top


From January 1998 to December 2003, 72 patients less than 14 years of age (ranging between 6 and 14 years), underwent cholecystectomy at King Fahad Hospital, Hofuf, AL-HASA, Eastern Province of Saudi Arabia.

The records of these patients were reviewed retrospectively for age, gender, clinical presentation, indication of surgery, duration of surgery, hospital stay, postoperative analgesia requirements and complications.

The risk factors for the development of cholelithiasis include sickle cell disease (54 patients), obesity (7 patients) patients, spherocytosis (6 patients) patients and no etiology (5 patients).

The 54 children with sickle cell disease included 30 girls and 24 boys aged between 6 and 14 years (mean 9.2 years) and with body weight varying from 17 to 52 kg (mean 20.1 kg). These patients were divided into two groups: patients operated as emergency (group I - 33 children) and patients operated as elective (group II - 21 children).

The diagnosis of SCD was based on a positive sickling test and hemoglobin electrophoresis using Helena Laboratories' super Z electrophoresis.

Elective patients were those who underwent evaluation and investigation initially with clinic visit and were admitted one or two days prior to surgery if they needed simple blood transfusion or partial blood exchange to increase the hemoglobin level to 10 g/dl, to achieve a hemoglobin S level below 50% and a hematocrit level between 30 and 40% before elective cholecystectomy.

All patients received intravenous fluid hydration with 5% dextrose in 0.54% normal saline as maintenance requirements depending on the body weight. The hydration started preoperatively and continued postoperatively until resumption of full oral intake.

The emergency patients included those patients who required hospitalization due to complications of their cholelithiasis.

The reasons for admission due to complications included acute biliary colic (10 patients), acute cholecystitis (16 patients), obstructive jaundice (6 patients) and gall stone pancreatitis (1 patient).

Children with right hypochondrial pain and tenderness were considered as biliary colic, whereas children with pain, fever, leukocytosis and positive ultrasonic murphy sign and gall bladder wall thickening (>4 mm) were considered to have acute cholecystitis. Children who presented with jaundice; pain; elevated bilirubin, mainly direct type; dilated common bile duct (CBD >6 mm); or stone in CBD were considered as obstructive jaundice, whereas patients who presented with these findings with increased amylase levels were considered to have gallstone pancreatitis.

Intraoperatively and postoperatively, all patients were monitored with pulse oximetry, and care was taken to avoid hypoxemia, hypothermia, acidosis and hypercarbia. All patients were nursed in the general surgical ward with special attention to adequate hydration, and pain was relieved with appropriate analgesia.

Postoperatively, all patients received analgesic in the form of pethidine (1 mg/kg) every 6 h for the first 24 h, and thereafter, oral and/or suppository paracetamol (10 mg/kg). Prophylactic antibiotics were given at induction, 2 h and 12 h after the operation (ampicillin 20 mg/kg, gentamicin 2 mg/kg, flagyl 7.5 mg/kg) to all elective patients. The emergency cases were given the same antibiotics for 3 days postoperatively.

Patients were discharged home, once they had fully recovered. They were followed up in the outpatient clinic 1 week after discharge, and thereafter, monthly for the next 6 months (clinically).

The laparoscopic technique used was similar to that described by others.


   Results Top


A total of 72 patients underwent cholecystectomy due to different causes {54 (75%) patients due to SCD}.

Thirty-three patients had emergency cholecystectomy due to gallstone complication (group I), while 21 patients had elective cholecystectomy (group II) [Table - 1]. There were 19 girls (57.6%) and 14 boys in the first group (M:F - 1:1.3) and 11 girls (52.4%) and 10 boys in the second group (M:F - 1:1.1). The mean age was 9.1 years (range 6-14) in the first group and 8.9 years (range 7-14) in the second group. The mean hemoglobin was 6.8 (range 6.3-9.8 g/dl) and 7.2 (range 6.7-10.1 g/dl) for the first and second group respectively. The mean hemoglobin S level for the first group was 77.0% (60.3-89.2%); and for the second group, it was 67.2% (55.9-80.1%). The mean hemoglobin F level was 18.4% (3-31.2%) and 23.4% (0-37.1%) for the first and second group respectively.

In the first group, 21 (63.6%) needed simple transfusion and 12 (36.4%) needed partial exchange; while in the second group, 13 patients (62%) needed simple transfusion and 8 (38%) needed partial exchange.

In group I, seven patients needed ERCP - six patients due to obstructive jaundice and one patient due to gallstone pancreatitis. Two patients (11, 12 years) required sphincterotomy and common bile duct stone extraction, but common bile duct stones were not found in the other five cases. All patients underwent ERCP 1 day before surgery. No patient from the elective group needed preoperative ERCP.

All patients in both groups started with laparoscopic cholecystectomy. Four patients in group I (12%) and two patients in group II (9.5%) converted to open.

Laparoscopic cholecystectomy was first utilized in our hospital in 1994 for adults and in 1998 for pediatrics.

The mean operating time was 91.3 ( 14.6) and 75.8 ( 18.3) min for group I and group II respectively. The mean hospital stay for laparoscopic cholecystectomy was 8.5 and 4.5 days for group I and group II respectively.

In group II, after OPD evaluation, 13 patients (62%) were admitted for simple blood transfusion 1 day before surgery; while 8 patients (38%) were admitted 2 days before surgery, to receive partial blood exchange to achieve a hemoglobin S level below 50%, hematocrit >30% and hemoglobin level >10 g/dl.

Intraoperative cholangiography was used only when there was a concern about the anatomy of the cystic or common bile duct, and it was surgeon dependent. It was done in nine patients (27.3 %) in group I and three patients (14.3%) in group II.

There were two patients with wound infection, three with chest infection and one with lower limb vasoocclusive crisis in group I (emergency cases), but no major complication required reoperation. There were no complications in the elective group. There was no mortality in both groups.

The histopathology of the gallbladder in group I was - acute cholecystitis (n = 29), acute in addition to chronic cholecystitis (n = 4); while in group II, it was acute cholecystitis ('n = 4), chronic cholecystitis (n = 17), and all patients were with cholelithiasis.


   Discussion Top


Homozygous hemoglobin S disease is a serious chronic hemolytic anemia. It appears in early childhood and is often fatal before the age of 30 years. HbS makes the red cells rigid, and this is the cause of hemolysis and most of the complications.

In the past, gallstone diseases were thought to be an unusual cause of abdominal pain in children, but recently there has been an increase in the incidence of the disease and it is now considered as one of the differential diagnosis of abdominal pain in children.[7]

Gallstones in children usually occur in a known risk group, including prematurity, necrotizing enterocolitis, ileal resection, cystic fibrosis, total parenteral nutrition, immunosuppression and hemolytic anemia.[8] In some series, hemolytic disease was a less common risk factor than it once had been, and the incidence in children with no apparent predisposing factors is increasing (about 43%);[9] while in this study, hemolytic disease due mainly to sickle cell disease is still the commonest factor 54/72 (75%). In the Eastern province of Saudi Arabia, SCD is a common hemoglobinopathy[2] and so are complications.

The incidence of gallstones in children with sickle cell anemia ranges from 4-55%, and it increases with age.[10] In a study by AL Salem et al. of 305 Saudi children (from the same province) with SCD, the frequency of cholelithiasis was found to be 19.7% overall, and it rose from 8.7% in those less than 10 years of age to 36% in those between 15 and 18 years of age.[11] As gallstones become symptomatic in 75% of patients with SCD in the young age group compared to normal population,[12] cholecystectomy is performed rather frequently for children with SCD.[13] In the past, as a result of high incidence of postoperative complications, cholecystectomy was indicated mainly in symptomatic gallstones in SCD patients,[14] but recently with current knowledge of the pathophysiology of SCD, preoperative and postoperative care including blood transfusion, improved anesthesia technique and surgery are offered to asymptomatic patients.[15]

There was no significant difference regarding age and sex in both the groups. A majority (67%) of patients in this study were admitted as emergency due to complications of cholelithiasis, this emphasizing the importance of elective cholecystectomy for children with SCD to prevent the risk of cholelithiasis-related complications and also to prevent emergency cholecystectomy, which is usually associated with high morbidity and mortality.[16]

The incidence of complications due to gallstones is high in many series,[17] and the findings of this study are similar 33/54 (61%). Due to the high prevalence of choledocholithiasis (14-30%) in children with cholelithiasis,[18],[19] ERCP is considered important in suspected cases of choledocholithiasis. Our main indication for ERCP, which was based on ultrasonic abnormalities, is in accord with other studies;[20],[21] as liver function tests and bilirubin levels are usually abnormal in these patients.

The operative time was significantly longer ( P < 0.005) in the emergency group (complicated) compared to elective cholecystectomy group (noncomplicated). This agrees with the observation of others.[22],[23],[24],[25]

The hospital stay was also significantly longer ( P < 0.005) for the emergency group (8.5 days) compared to 4.5 days for the elective laparoscopic cholecystectomy group.[26] This shows the importance of elective surgery on one hand and laparoscopic surgery on the other.[22],[23],[24],[25]

Postoperative complications related to surgery were minor and occurred in the emergency group only, and careful perioperative management permitted safe elective cholecystectomy in children with SCD.[27],[28] In children, laparoscopic cholecystectomy is a less frequent operation than in adults. However, it is an effective and safe method with all the advantages of a mini-invasive operation.[29],[30],[31]


   Conclusion Top


Because of the incidence of eventual significant complication of cholelithiasis in SCD patients, we believe that elective laparoscopic cholecystectomy is a safe procedure for children with SCD.[32],[33]

It should be recommended at the time of diagnosis of cholelithiasis, if experience and the necessary precautions are availed of.

 
   References Top

1.Holcomb GW 3rd. Laparoscopic cholecystectomy. Semin Pediatr Surg 1993;2:159-67.  Back to cited text no. 1  [PUBMED]  
2.Nasserullah Z, Al Jama AH, Abusrair HA. Neonatal screening for sickle cell disease, glucose- 6- phosphate dehydrogenase deficiency and thalassemia in Qatif and Al Hasa. Ann Saudi Med 1998;18:289-92.   Back to cited text no. 2    
3.Lackman BS, Lazerson J, Starshak RJ, Vaughters FM, Werlin SL. The prevalence of cholelithiasis in sickle cell disease as diagnosed by ultrasound and cholecystectomy. Pediatrics 1979;64:601-3.  Back to cited text no. 3    
4.Rennels MB, Dunne MG, Grossman NJ, Schwartz AD. Cholelithiasis in patients with major sickle hemoglobinopathies. Am J Dis Child 1984;138:66-67.   Back to cited text no. 4  [PUBMED]  
5.Ware R, Filston HG, Schultz WH, Kinney TR. Elective cholecystectomy in childern with sickle hemoglobinopathies. Successful outcome using a preoperative transfusion regimen. Ann Surg 1988;208: 17-22.   Back to cited text no. 5    
6.Gibson TJ, O dell RF, Cathcart RS 3rd, Rambo WM. Treatment of cholelithasis in patients with sickle cell anemia. South med J 1979;72:391-2.   Back to cited text no. 6    
7.Jawad AJ, Kurban K, EL-Bakry A, Al Rabeeah A, Seraj K. Laparoscopic cholecystectomy: The treatment of choice for cholelithiasis in infancy and childhood. Ann Saudi Med 1997;17:410-2.   Back to cited text no. 7    
8.Grosfield JL, Rescorla FJ, Skinner MA, West KW, Scherer LR 3rd. The spectrum of biliary tract disorders in Infants and children: Experience with 300 cases. Arch Surg 1994;12:513-20.   Back to cited text no. 8    
9.Cameron JL, Maddrey WC, Zuidema GD. Biliary tract disease in sickle cell anemia: Surgical consideration. Ann Surg 1971;174:702-10.   Back to cited text no. 9  [PUBMED]  [FULLTEXT]
10.Sarnaik S, Slovis TI, Corbett DP, Emami A, Whitten CF. incidence of cholelithiasis in sickle cell anemia using the ultrasonic gray scale technique. J Pediatr 1980;96:1005-8.  Back to cited text no. 10    
11.Rescoral FJ, Grosfield JL. Cholecystitis and cholelithiasis in children. Semin Pediatr Surg 1992;1:98-106.  Back to cited text no. 11    
12.Alsalem AH, Qaisaruddin S, Al-Dabbous I. Cholelithiasis in children with sickle cell disease. Pediatr Surg Int 1996;11:471-3.  Back to cited text no. 12    
13.Al Abkari HA, Abdulnabi HI, Al Jamah AH, Meshikhes AN. Laparoscopic cholecystectomy in patients with sickle cell disease. Saudi Med J 2001;22:681-5.  Back to cited text no. 13    
14.Al-Salem AH, Nangalia R, Kolar K, Qaisaruddine S, Al-Dabbous I, Al-Jama A. Cholecystectomy in children with sickle cell disease. Perioperative management. Pediatr Surg Int 1995;10:472-4.  Back to cited text no. 14    
15.Haberkern CM, Neumayr LD, Orringer EP, Earles AN, Robertson SM, Black D, et al . Cholecystectomy in sickle cell anemia patients: Perioperative outcome of 364 cases from the national preoperative transfusion study. Preoperative transfusion in sickle cell disease study group. Blood 1997;89:1533-42.  Back to cited text no. 15    
16.Duncan ND, McDonald AH, Mitchell DI. Cholecystectomy in Jamaican children with homozygous sickle-cell disease. Trop Doct 2000;30: 214-6.  Back to cited text no. 16  [PUBMED]  
17.Bakhotmah MA. Symptomatic cholelithiasis in children: A Hospital-Based Review. Ann Saudi Med 1999;19:251-2.  Back to cited text no. 17    
18.Holcomb GW 3rd, Olsen DO, Sharp KW. laparoscopic cholecystectomy in the pediatric patients. J Pediatr Surg 1991;26:1186-90.  Back to cited text no. 18  [PUBMED]  [FULLTEXT]
19.Ware RE, Schultz WH, Filston HC, Kinney TR. Diagnosis and management of common bile duct stones in patients with sickle hemoglobinopathies. J Pediatr Surg 1992;27:572-5.   Back to cited text no. 19  [PUBMED]  [FULLTEXT]
20.Gholson CF, Grier JF, Ibach MB, Favort D, Nall L, Sittig K, et al . Sequential endoscopic/laparoscopic management of sickle hemoglobinopathy-associated cholelithiasis and suspected choledocholithiasis. South Med J 1995;88:1131-5.  Back to cited text no. 20    
21.Al-Salem AH, Nourallah H. sequential endoscopic/laparoscopic management of cholelithiasis and choledocholithiasis in children who have sickle cell disease. J Pediatr Surg 1997;32:1432-5.  Back to cited text no. 21  [PUBMED]  [FULLTEXT]
22.Leandros E, Kymionis GD, Konstadoulakis MM, Albanopoulos K, Dimitrakakis K, Gomatos I, et al . Laparoscopic or open cholecystectomy in patients with sickle cell disease: Which approach is superior? Eur J Surg 2000;166:859-61.  Back to cited text no. 22    
23.Esposito C, Gonzalez Sabin MA, Corcione F, Sacco R, Esposito G, Settimi A. Result and complication of laparoscopic cholecystectomy in children. Surg Endosc 2001;15:890-2.  Back to cited text no. 23  [PUBMED]  
24.Al-Salem AH, Qaisaruddin S, Al-Abkari H, Nourallah H, Yassin YM, Varma KK. Laparoscopic versus open cholecystectomy in children. Pediatr Surg Int 1997;12:587-90.  Back to cited text no. 24  [PUBMED]  
25.Simi M, Schietroma M, Carlei F, Lannucci D, Cianca G, Leardi S. Is laparoscopic cholecystectomy a safe alternative to open cholecystectomy for pediatric patients with cholelithiasis? Endoscopy 1996;28:312-5.  Back to cited text no. 25    
26.Malone BS, Werlin SL. Cholecystectomy and cholelithasis in sickle cell anemia. Am J Dis Child 1988;142:799-800.  Back to cited text no. 26  [PUBMED]  
27.Solanki DL, McCurdy PR. Cholelithiasis in sickle cell anemia: A case for elective cholecystectomy. Am J Med Sci 1979;277:319-24.  Back to cited text no. 27  [PUBMED]  
28.Ware R, Filston HC, Schultz WH, Kinney TR. Elective cholecystectomy in children with sickle hemoglobinopathies. Successful outcome using a preoperative transfusion regimen. Ann Surg 1988;208:17-22.   Back to cited text no. 28    
29.Teyschl O, Tua J, Tecl F. Laparoscopic cholecystectomy in children. Rozhl Chir 2000;79:17-20.  Back to cited text no. 29    
30.Simi M, Schietroma M, Carlei F, Iannucci D, Cianca G, Leardi S. Is laparoscopic cholecystectomy a safe alternative to open cholecystectomy for paediatric patients with cholelithiasis? Endoscopy 1996;28:312-5.  Back to cited text no. 30  [PUBMED]  
31.Wales PW, Carver E, Crawford MW, Kim PC. Acute chest syndrom after abdominal surgery in children with sickle cell disease: Is laparoscopic approach better? J Pediatr Surg 2001;36:718-21.   Back to cited text no. 31  [PUBMED]  [FULLTEXT]
32.de Lagauise P, Eymeri JC, Tavakoli D. Value of elective cholecystectomy in children with homozygote sickle cell anemia. Apropos of 3 recent cases. Ann Chir 1989;43:361-2.  Back to cited text no. 32    
33.Rambo WM, Reines HD. Elective cholecystectomy for the patient with sickle cell disease and asymptomatic cholelithiasis. Am Surg 1986;52:205-7.  Back to cited text no. 33  [PUBMED]  

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Correspondence Address:
AbdulRahman S Al-Mulhim
Department of Surgery, King Fahad Hospital- Hofuf, P.O. Box 1164, Hofuf, Al-Hassa 31982
Saudi Arabia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1319-3767.29753

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