Find best premium and Free Joomla templates at GetJoomlaTemplatesFree.com

Laparopscopic Splenectomy: The Procedure of Choice for Children with Sickle Cell Disease

Ashraf A. Alnosair, Ahmed H. Al-Salem

Department of Pediatric Surgery, Maternity and Children Hospital, Dammam, Saudi Arabia

 

Correspondence:

Ahmed H. Al-Salem

P.O. Box 61015

Qatif 31911 Saudi Arabia

Fax: 966 3 8630009

E-mail: This email address is being protected from spambots. You need JavaScript enabled to view it.

 

Abstract

Background: Sickle cell disease (SCD) is one of the most common hemoglobinopathies worldwide. The spleen is commonly affected early, leading ultimately to auto-splenectomy as a result of the repeated attacks of vasoocclusive crises. However, this is not always the case and in some patients sple- nomegaly may persist into adulthood leading to complications which necessitate splenectomy.

Patients and methods: The records of all children who had splenectomy for hematological diseases at our hospital were reviewed for diagnosis, age at splenectomy, indication for splenectomy, sex, method of splenec- tomy, operative time, hospital stay, postoperative morbidity and mortality.

Results: A total of 315 children had splenectomy for various hematological diseases. There were 174 males and 141 females. The mean age was 7.6 years (1.8 – 13 years). Forty-seven (15%) had com- bined splenectomy and cholecystectomy. Eighty-six of them had laparoscopic splenectomy and 13 (15%) had laparoscopic splenectomy and cholecystectomy. SCD (239 sickle cell anemia and 22 sickle –beta-thalassemia) was the commonest indication for splenectomy (83%). Sixty- nine of those with SCD had laparoscopic splenectomy, 13 (18.8%) had combined laparoscopic sple- nectomy and cholecystectomy. When these were compared with a similar group of 88 children with SCD who had open splenectomy, laparoscopic splenectomy was superior in terms of faster recovery, shorter hospital stay, less postoperative complications and better cosmetic appearance but laparoscopic splenectomy took more operative time.

Conclusion: With good perioperative management, laparoscopic splenectomy is feasible and safe in children with SCD and can be done concomitantly with laparoscopic cholecystectomy. Currently, laparo- scopic splenectomy takes more operative time as these children tend to have large spleens with severe adhesions. Laparoscopic splenectomy however is superior to open splenectomy in terms of postoperative recovery, hospital stay, and cosmetic appearance.

Keywords: sickle cell anemia, splenectomy, cholecystectomy, complications, laparoscopic sple- nectomy, laparoscopic splenectomy and cholecystectomy

 

Introduction

The spleen is one of the most commonly affected organs in patients with sickle cell disease (SCD). It enlarges early and then undergoes progressive atrophy as a result of repeated attacks of vasoocclusive crises leading to autosplenectomy [1, 2]. However, splenomegaly persists in some of these patients to an older age and sometimes in adults. The reason is not known but an elevated level of Hb F is known to contribute to persistence of splenomegaly in these patients [3, 4, 5]. Persistence of splenomegaly is known to be associated with complications necessitating splenectomy. Complications include acute splenic sequestration crisis, splenic abscess, and massive splenic infarction [6, 7]. Another common complication in SCD is cholelithiasis. The frequency of this is variable but it is known to increase with age and overall 70% of patients with SCD develop gallstones [8 – 11]. The treatment of symptomatic gallstones is well established and currently, laparoscopic cholecystectomy is the treatment of choice [12 – 17]. The treatment of asymptomatic gallstones is still controversial [16, 18 – 20].

In the past, open splenectomy was the only option to treat splenic complications of children with SCD. In 1991, Delaitre performed the first laparoscopic splenectomy and since then it has become the procedure of choice to treat patients with hematological diseases requiring splenectomy [21 – 23]. Laparoscopic splenectomy was reported to be feasible and safe both in adults and children. This report describes our experience with laparoscopic splenectomy in children with SCD.

Patients and methods

This is a retrospective study from January 1991 to December 2013. The medical records of all children who had splenectomy for various hematological disorders were reviewed and the following information was collected: diagnosis, age at splenectomy, sex, indication for splenectomy, Hb electrophoresis, method of splenectomy, operative procedures and postoperative complications. All children received pneumococcal, meningococcal and H influenza vaccine at least two weeks prior to splenectomy and received prophylactic oral or intramuscular penicillin for a minimum of 2-3 years depending on the patient age at the time of splenectomy. Prior to splenectomy, all children with SCD had an abdominal ultrasound to roll out associated gallstones. Once gallstones are diagnosed, we advise the parents to have concomitant laparoscopic cholecystectomy. All children were put on a perioperative management protocol which consists of:

(1) Hydration with I.V. fluid at 1 1/2 their maintenance starting the night before surgery using D5 ½ normal saline or D5 ¼ normal saline depending on the patient’s age. This is continued postoperatively till the patient takes enough oral fluids.

(2) Blood transfusion when necessary to increase their Hb level to about 10 g/dl. We do not aim to lower the HbS level preoperatively but we transfuse them to increase their Hb level. We do not perform exchange blood transfusion. Those who have an Hb level above 9 g/dl are not transfused. We do keep typed and cross matched blood and we transfuse them intra-operatively when there is blood loss.

(3) Preoperative prophylaxis antibiotics using a cephalosporin.

(4) Close intraoperative monitoring including adequate hydration and oxygenation, avoiding hypothermia, hypercarbia and acidosis.

(5) Adequate postoperative analgesia, chest physiotherapy including incentive spirometer and early mobilization. These patients are not routinely admitted to the intensive care unit postoperatively.

The technique of laparoscopic splenectomy is similar to that describe previously. We put the patient in the lateral position and we use 3-4 ports. The spleen is put in a bag and removed in pieces. In those who had laparoscopic splenectomy and cholecystectomy, we perform laparoscopic cholecystectomy first.

Those children with SCD who had laparoscopic splenectomy were compared to a similar group of children with SCD who had open splenectomy in term of operative time, hospital stay and postoperative complications. The results were compared using Student t-test.

Results

A total of 315 children had splenectomy for various hematological disorders (Table 1). There were 174 males and 141 females. The mean age was 7.6 years (1.8 – 13 years). Eightysix of them had laparoscopic splenectomy and 13 (15%) had laparoscopic splenectomy and cholecystectomy. There were 50 males and 36 females. The mean age was 7.5 years (1.8 – 12 years).

Table I: Indications for splenectomy in children with hematological disorders - Total No. of patients: 315

INDICATION

NO

%

Sickle cell anemia

239

76

Sickle cell beta thalassemia

22

7

Beta thalassemia

28

9

Thalassemia intermediate

6

2

Hb H disease

3

1

ITP

8

2.5

ALL with thrombocytopenia

2

0.6

Gaucher’s disease with hypersplenism

2

0.6

Autoimmune hemolytic anemia

2

0.6

CML with hypersplenism

1

0.3

Chronic renal failure, amyloidosis and hypersplenism

1

0.3

Hereditary elliptocytosis

1

0.3

ITP: Idiopathic thrombocytopenic purpura, CML: Chronic myeloid leukemia, ALL: Acute lymphoblastic leukemia

The indications for laparoscopic splenectomy are shown in Table 2. The youngest patient who had laparoscopic splenectomy was 1.8 years old, a male child with SCD who had major splenic sequestration crisis. Thirteen (15%) children had combined laparoscopic splenectomy and laparoscopic cholecystectomy. Four (4.7%) required conversion to open splenectomy; one had a very large spleen with severe adhesions, 2 had uncontrolled intraoperative bleeding and one had a very large spleen which was difficult to extract through the bag. The spleen was removed through an incision near the inguinal region.

Table II: Indications for laparoscopic splenectomy - Total No. of patients: 86

INDICATION

NO.

%

Sickle cell disease

69

80

Beta thalassemia major

7

8

Thalassemia intermediate

4

4.7

ITP

4

4.7

Hereditary elleptocytosis

1

1.2

Autoimmune hemolytic anemia

1

1.2

ITP: Idiopathic thrombocytopenic purpura

SCD was the commonest indication for splenectomy. A total of 261 (83%) children with SCD had splenectomy. The indications for splenectomy in those with SCD are shown in Table 3. In 2005, we performed the first laparoscopic splenectomy in a child. Out of 261 children with SCD, 69 had laparoscopic splenectomy. The indications for laparoscopic splenectomy in those patients included recurrent minor splenic sequestration crisis in 63, major splenic sequestration crisis in 5 and one had a large spleen with multiple infarcts.

Table III: Indications for splenectomy in children with sickle cell disease -  Total No. of patients: 261

INDICATION

NO.

%

Acute splenic sequestration crisis

221

84.7

Hypersplenism

18

7

Massive splenic infarction

14

5.4

Splenic abscess

7

2.7

Splenomegaly and nonfunctioning spleen

1

0.4

Those who had laparoscopic splenectomy were compared to a similar group of children with SCD who had open splenectomy and the results are shown in Table 4. Post-operatively, 3 (4.3%) in the laparoscopy group developed complications which included mild acute chest syndrome in 2 and fever of unknown origin in 1. Nine (10.2%) of the open splenectomy group developed complications which included: acute chest syndrome in 4, fever of unknown etiology in 3, bleeding in the splenic bed in 1 and adhesive intestinal obstruction in 1. In those who had laparoscopic splenectomy, the operative time was calculated and the results were divided into two periods, 4.5 years each, and the results were compared so as to evaluate the effect of the learning curve. The mean operative time for the first period was 167 minutes (range 120 - 330 minutes) and the mean operative time for the second period was 121 minutes (mean 50- 180 minutes) (P value = 0.000628).

Table IV: Comparison between open and laparoscopic splenectomy

VARIABLE

LAPAROSCOPIC SPLENECTOMY GROUP

OPEN SPLENECTOMY GROUP

Total No. of patients

69

88

Mean age in years (Range)

7.6 (1.8-12)

7.5 (3.5-13)

Sex (M:F)

36:33

52:36

Mean HbS (Range)

77.3% (71-87.4)

78.7 (68 – 92.6)

Mean Hb F (Range)

19.2% (8.8-27)

 

20.3 (9.2 – 39.6)

Splenectomy and cholecystectomy

13 (18.8%)

16 (18.2%)

Mean operative time in minutes (Range)

154 (55-300)

70 (30-105)

P < 0001

Mean hospital stay in days (Range)

4.5 (3-9)

6.5 (5-12)

P < 0001

Postoperative complications

3 (4.3%)

9 (10.2%)


Discussion

Sickle cell disease is one of the common hemoglobinopathies in the world. It can affect any part of the body and one of the most commonly affected organ is the spleen. This is commonly enlarged during the first decade of life but then undergoes progressive atrophy as a result of vasoocclusive crisis leading ultimately to auto splenectomy. In the Eastern Province of Saudi Arabia, SCD is common and known to be associated with persistence of splenomegaly into an older age group or even adults. This makes them liable to develop complications necessitating splenectomy [1, 2, 5 – 7].

Acute splenic sequestration crisis was the commonest complication necessitating splenectomy in our series. This was the indication in 84.7% followed by hypersplenism. Fourteen of our patients had splenectomy for massive splenic infarction and 7 had splenic abscess. In 2005, we performed the first laparoscopic splenectomy for a child and since then all splenectomies for children with hematological disorders are done laparoscopically. Children with SCD are known to develop more postoperative complications when compared with normal children.

We tried to compare our results of laparoscopic splenectomy to a similar group of children who had open splenectomy. Laparoscopic splenectomy was superior in term of faster recovery, shorter hospital stay, and better cosmetic appearance but it takes more operative time. We also analyzed our operative time over the years, and there was a definite decrease in the operative time with more experience. There is an initial learning curve where the mean operative time was 166 minutes but with experience this decreased to a mean operative time of 120.8 minutes. Add to this, the fact that children with sickle cell disease tend to have large spleens with multiple adhesions. Thirteen (18.8%) of our patients had combined laparoscopic splenectomy and cholecystectomy for concomitant gallstones. All our patients with SCD who require splenectomy undergo preoperative ultrasound to look for gallstones and even in those with asymptomatic gallstones, we recommend combined laparoscopic splenectomy and cholecystectomy. The frequency of cholelithiasis in patients with SCD is however variable depending on the age of the patients and the method of diagnostic used. A frequency ranging from 5% - 55% has been reported in the literature but an overall 70% of patients with SCD will develop gallstones at one stage in their life [8 - 11, 24].

Life expectancy in patients with SCD is known to be shorter than the general population and they usually die of sickle cell related complications. With better understanding of SCD, improved medical and surgical management, and the development of new drugs such as hydroxyurea, the survival of these patients is better and their life expectancy increases [25 – 30]. In the same time, it is expected an increased frequency of cholelithiasis in these patients as the frequency of gallstones increases with age. In a previous study on 305 children with SCD, we found an overall 19.7% frequency of gallstones. This increased from 8.7% in those less than 10 years of age to 36% in those 15- 18 years of age [11]. This makes them liable to develop gallstones related complications including acute cholecystitis, choledocholithiasis, obstructive jaundice, ascending cholangitis and pancreatitis. The treatment of symptomatic cholelithiasis in patients with SCD is cholecystectomy.

The treatment of asymptomatic gallstones is still controversial. In the past and because of a reported perioperative mortality rate as high as 10% and a postoperative complication rate up to 50%, surgery in patients with SCD was not advocated except in symptomatic patients [16, 18, 19]. However, this is not the case nowadays as with better understanding of SCD and improved perioperative care, surgery is safer. We, like others, advocate cholecystectomy for asymptomatic gallstones in patients with SCD. In the era of minimal invasive surgery, laparoscopic cholecystectomy is now the procedure of choice. This is especially true for patients with SCD since laparoscopic cholecystectomy was shown to be feasible, beneficial and safe [14 – 17]. Operating on these patients on an emergency basis and not fully prepared is definitely associated with a higher morbidity and mortality. It is much better to operate on these patients electively when they are well prepared. Add to this the fact that cholecystectomy will obviate the confusion in differentiating abdominal crisis from gallbladder related complications. All our patients who had combined laparoscopic splenectomy and cholecystectomy did well with no added mortality or morbidity.The fact that there is no large abdominal wound, laparoscopic cholecystectomy should be advantageous in patients with SCD. Not only the hospital stay is shorter and cosmetically is better, but the associated postoperative pain is much less. This makes their early mobilization much easier and will not interfere with breathing which may prove beneficial in reducing the incidence of postoperative acute chest syndrome.

In conclusion, laparoscopic splenectomy should be the procedure of choice to treat children with SCD. With good perioperative management, laparoscopic splenectomy is feasible and safe and can be done concomitantly with cholecystectomy. There is a definite learning curve where laparoscopic cholecystectomy takes more operative time. This is specially so for children with SCD who are known to have large spleen with severe adhesions. Laparoscopic splenectomy is however superior to open splenectomy with regard to the hospital stay, postoperative recovery, complications and cosmetic appearance.




References

1. Perrine RP, Pembrey ME, John P, Perrine S, Shoup F. Natural history of sickle cell anemia in Saudi Arabia. Ann Intern Med 1978; 88: 1-6.

2. Al-Awamy BH. Sickle cell anemia, its clinical manifestations and their management. Saudi Med J 1987; 9:553-562.

3. Rees DC, Williams TN, Gladwin MT. Sickle-cell disease. Lancet. 2010; 376:2018-2031.

4. Serjeant GW. Sickle cell disease: the spleen, 1st Ed, New York: Oxford University press, 1985:109 -123.

5. Al-Awamy BH, Naeem M. Splenic and hepatic function in sickle cell anemia with special emphasis on patients from the Eastern Province of Saudi Arabia. Saudi Med J 1986; 7:434-442.

6. Al-Salem AH. Indications and complications of splenectomy for children with sickle cell disease. J Pediatr Surg 2006; 41:1909–1915.

7. Al-Salem AH. Splenic Complications of Sickle Cell Anemia and the Role of Splenectomy. ISRN Hematol 2011: 864257.

8. Rennels MB, Dunne MG, Grossman NJ et al. Cholelithiasis in patients with major sickle hemoglobinopathies. Am J Dis child 1984; 138:66-67.

9. Sarnaik S, Slovis TL, Corbett DP et al: Incidence of cholelithiasis in sickle cell anemia using the ultrasonic gray scale technique. J Pediatr 1980; 96:1005-1008.

10. Adekile AD: Experience with cholelithiasis in patients with sickle cell disease in Nigeria. Am J Pediatr Hematol Oncol 1985; 7: 216-264.

11. Al-Salem AH, Qaisarruddin S, Al-Dabbous I, Bhamidipati P, Abusrair H, Amman H, Al Jam’a A. Cholelithiasis in children with sickle cell disease. Pediatr Surg Int 1996; 11: 471-473.

12. Newman KD, Marmon LM, Attari R et al. Laparoscopic cholecystectomy in pediatric patients. J Pediatr Surg 1991; 26:1184-1185.

13. Rubio PA. Laparoscopic cholecystectomy: experience in 500 consecutive cases. Int Surg 1993; 78:277-279.

14. Holcomb GW III, Olsen DO, Sharp KW. Laparoscopic cholecystectomy in the pediatric patient. J Pediatr Surg 1991; 26:1186-1190.

15. Tagge EP, Othersen HB Jr, Jackson SM et al. Impact of laparoscopic cholecystectomy on the management of cholelithiasis in children with sickle cell disease. J Pediatr Surg 1991; 26:1181-1183.

16. Al-Salem AH. Laparoscopic cholecystectomy in children with sickle cell disease. Annals of Saudi Medicine 2000; 26: 480-482.

17. Hartley RM, Crist D, Howell CG, et al. Laparoscopic cholecystectomy in children with sickle cell disease. Am Surgeon 1995; 61:169-171.

18. Curro G, Meo A, Ippolito D, Pusiol A, Cucinotta E. Asymptomatic cholelithiasis in children with sickle cell disease: early or delayed cholecystectomy? Ann Surg 2007; 245:126-129.

19. Ghoson CF, Sittig K, McDonald JC. Recent advances in the management of gallstones. Am J Med Sci 1994; 307:293- 304.

20. Duncan ND, McDonald AH, Mitchell DI. Cholecystectomy in Jamaican children with homozygous sickle-cell disease. Trop Doct 2000; 30:214-6

21. Tulman S, Holcomb GW III, Karamanoukian HL, et al. Pediatric laparoscopic splenectomy. J Pediatr Surg 1993; 28: 689-692.

22. Pugliese R, Sansonna F, Scandroglio I, et al. Laparoscopic splenectomy: A retrospective review of 75 cases. Int Surg 2006; 91:82-86.

23. Moores DC, McKee MA, Wang H, Fischer JD, Smith JW, Andrews HG. Pediatric laparoscopic splenectomy. J Pediatr Surg 1995; 30:1201-1205.

24. Bond LR, Hatty SR, Horn ME, et al. Gall stones in sickle cell disease in the United Kingdom. Br Med J. 1987; 295: 234-236.

25. Oduro KA, Searle JF. Anesthesia in sickle cell states: A plea for simplicity. BMJ 1972; 4:596-598.

26. Searle JF. Anesthesia in sickle-cell states: A review. Anesthesia 1973; 28:48-58.

27. Platt O, Brambilla DJ, Roses WF, et al. Mortality in sickle cell disease: Life expectancy and risk factors for early death. N Engl J Med 1994; 330:1639-1644.

28. Steinberg MH. Hydroxyurea treatment for sickle cell disease. Scientific World Journal 2002; 2:1706-28.

29. Steinberg MH, Barton F, Castro O, Pegelow CH, Ballas SK, Kutlar A, et al. Effect of hyroxyurea on mortality and morbidity in adult sickle cell anemia: risks and benefits up to 9 years of treatment. JAMA 2003; 289:1645-1651.

30.Vichinsky EP. Comprehensive care in sickle cell disease: its impact on morbidity and mortality. Semin Hematol 1991; 28:220–226.