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Laparoscopic Partial Splenectomies in Children for Hereditary Spherocytosis

P. de Lagausie*, D. Kalfa*, I. Thuret**, S. le Bel***, J.M. Guys*.
* Visceral Pediatric Surgery Department, Hôpital de la Timone Enfants, Marseille, France.
** Hematologic Pediatric Department, Hôpital de la Timone Enfants, Marseille, France.
*** Anesthesiology and Pediatric Intensive Care Department, Hôpital de la Timone Enfants, Marseille, France.

 

Correspondence 

Pascal de lagausie, md, Phd
hopital de la timone enfants
27 bld Jean moulin
13005 marseille, france.
email: This email address is being protected from spambots. You need JavaScript enabled to view it.  


Abstract

Background: The two main surgical options proposed in the hereditary spherocytosis in children used to be laparoscopic total splenectomy or open partial splenectomy. The aim of this study was to describe the feasibility and the results of laparoscopic partial splenectomy in children with hereditary spherocytosis.

Material and methods: This retrospective study included 3 cases (2 boys, 1 girl). The mean age was 5,3 years (range from 23 months to 8 years). The mean weight was 19 kg (range from 11 to 26 kg). Surgery was decided on anaemia (mean preoperative haemoglobin count: 90 g/l) in the three cases. Two patients had 10 or more transfusions before partial splenectomy. The mean spleen size was 12,7 cm X 7,5 cm.

Results: During the surgical procedure, 80-90% of the splenic tissue was removed by anterior approach, and the splenic remnant was conserved in the lower part in the 3 cases. Ultracision Harmonic Scalpel (® Ethicon, Cincinnati, USA) was used to complete transection of the spleen. The mean time for laparoscopic resection was 140 minutes (range from 75 to 180 min). No per-operative complications occurred: no bleeding, no diaphragmatic injury. Neither per-operative transfusion nor conversion were necessary. One postoperative complication (left pleural effusion) occurred but required no further treatment. The mean hospital stay was 4,6 days (range from 3 to 7 days). None of them needed postoperative blood transfusion. No post-splenectomy infectious complication occurred. Mean follow-up time was 41,7 months (range from 37 to 49 months).

Conclusion: Laparoscopic partial splenectomy is feasible and safe in children with hereditary spherocytosis, essentially due to the use of Ultracision Harmonic Scalpel. This surgical procedure offers advantages of both laparoscopic technique and preservation of splenic tissue. A longer follow-up would be useful to evaluate the potential risk of regrowth of the splenic remnant after partial laparoscopic splenectomy.

Key words: laparoscopic partial splenectomy, hereditary spherocytosis, children

 

Introduction

Splenectomy in children can be performed for focal splenic tumours and hematologic diseases. Among these, hereditary spherocytosis (HS) is a relatively common inherited haemolytic anemia, with an estimated incidence in Northern Europe of 1 to 2 in 5000 individuals. Splenectomy is the treatment of choice for moderate-to-severe forms of HS [13]. But its use was restricted because of the risk for overwhelming infections, especially in children. Thus, partial splenectomy, characterized by removal of 80% to 90% of the enlarged spleen, is a logical alternative. Laparoscopic splenectomy, which had been performed on adults for several years [18], has been adapted to children [17] with good results. Thus, the ideal surgical procedure seems to be laparoscopic partial splenectomy, which was difficult until now because of technical limits and bleeding risk. The aim of this study was to describe the feasibility and the results of laparoscopic partial splenectomy in children with hereditary spherocytosis.

Materials and methods

This retrospective study included three children (2 boys, 1 girl) aged 23 months to 8 years old (mean: 5,3 years) who had surgery between January 2005 and January 2006 at our institution. The data collected included gender, age, weight, familial history of HS, clinical presentation, indication for operation, need for transfusions, hematologic values and ultrasound evaluation of spleen size. These data are summarized in table 1. The mean weight was 19 kg (range from 11 to 26 kg). Siblings of the three patients experienced the same illness. Surgery was decided on hypersplenism: anemia (mean preoperative hemoglobin count: 90 g/l) and its consequences in the three cases. Two patients had 10 or more transfusions before partial splenectomy. According to ultrasonography, the mean spleen size was 12,7 cm X 7,5 cm.

Table 1. Patients‘ data

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Surgical technique

The surgical procedure was performed with an anterior approach as described before [5]. Dissection begins by opening the gastrocolic ligament to access the lesser sac. Some of the short gastrocolic vessels may be divided after electrocoagulation. The stomach is grabbed sufficiently high, on the posterior aspect of the greater curvature, for a better exposure of the splenic pedicle. The splenic artery is approached first, pancreatic branches are divided. Branches of the splenic artery to the lower pole are conserved while other branches are divided between two ligatures on each side using 2.0 or 3.0 absorbable suture (intracorporeal knotting), creating a clear line of demarcation. Next, the splenic vein is identified: branches to the lower pole are conserved and other branches are divided as well between two ligatures of 2.0 or 3.0 absorbable suture (intracorporeal knotting). We never use clips or devices, because they are not safe and are expensive for us. At this time, vascularisation of the residual spleen must be checked. Transection of the spleen is performed using Ultracision Harmonic Scalpel (® Ethicon, Cincinnati, USA), which provides an excellent hemostatic control. The conserved splenic remnant is the lower pole in the 3 cases. The dissection is pursued by incising the colosplenic and phrenosplenic ligaments in order to mobilize the upper 90% of spleen, which are now totally free and can be introduced into the Endo-catch (® Tyco, Elancourt, France). The splenic tissue is exteriorized through the left inferior incision after digital fragmentation, and sent to pathology. After peritoneal washing, splenopexy is performed: the lower splenic remnant is sutured to the abdominal wall next to diaphragm (® Mersuture 2.0) ( fig. 1) to avoid torsion and sub-acute necrosis of residual splenic tissue. Then, fresh spleen wall is re-covered by great omentum. No drains are used. If there is an associated cholecystectomy for gallstones (one patient), the left side of the patient is brought to a flat position, the gallbladder is caught by the xiphoid trocar, and we work with the two 5-mm ports. The gallbladder is exteriorized through the 15-mm port.

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Figure 1: Splenopexy is performed: the lower splenic remnant is sutured to the abdominal wall next to diaphragm 

Results

Short-term results are summarized in table 1. During the surgical procedure, 80-90% of the splenic tissue was removed by anterior approach, and the splenic remnant was conserved in the lower part in the 3 cases. The mean time for laparoscopic resection was 140 minutes (range from 75 to 180 min). No per-operative complications occurred: no bleeding, no diaphragmatic injury, no postoperative pancreatic fistula or parenchyma lesion. Neither per-operative transfusion nor conversion were necessary. One postoperative complication (left pleural effusion) occurred but no further treatment was required. The mean hospital stay was 4,6 days (range from 3 to 7 days). Clinical and biological parameters improved in the 3 patients. One month after partial splenectomy; ultrasound showed a vascularised splenic remnant. None of them needed postoperative blood transfusion. No post-splenectomy infectious complication occurred. No patients experienced late complications that led to a secondary total splenectomy. One year after the procedure, US Doppler always find a correct vascularisation of the spleen remnant. Mean follow-up time was 41,7 months (range from 37 to 49 months).

Discussion

Laparoscopic partial splenectomy is feasible, reproducible and safe in children with hereditary spherocytosis. Two surgical procedures to treat hypersplenism in children with hereditary spherocytosis are frequently reported in literature: laparoscopic total splenectomy [3,5,9,11] and open partial splenectomy [1]. Laparoscopic partial splenectomy was first described in 2 cases in 2003 [19]. Now, the experience is larger [20]. Until now, paediatric surgeons had to choose between advantages of the laparoscopic approach and those of the partial splenectomy. The surgical procedure we propose in our report can free surgeons from such a choice, since it associates benefits of both laparoscopic and partial approaches.

One important technical point is the choice of remnant spleen. In our technique, we preferred conserve the lower part, as soon as possible. By a progressive control of the spleen pedicle (always by endo-ligature of resorbable stitch), we performed an upper splenectomy. In few cases, an own lower autonomous pedicle is present, and the technique is easier. By using this technique with conservation of the lower spleen, we think that is easier to perform a true 90% splenectomy. This is not the case in all surgical paediatric team [20]. It is important to precise the necessity to performe a splenopexy at the end of our procedure in order to prevent any torsion of the remnant spleen.

Indeed, partial splenectomy is the best way to prevent post splenectomy infections [7] because it seems to preserve the immune role of the spleen [4, 8] . In our report, no post-splenectomy infectious complication occurred, whereas these are the main life-threatening complications after total splenectomy [6] and can occur irrespective of age and of the time interval after the surgical procedure [1]. Nevertheless, vaccination and antibiotic prophylaxis were prescribed in our 3 cases as recommended by [2]. Previous studies in literature also reported other advantages of partial splenectomy compared to total splenectomy: less secondary atherosclerotic events [14], less pulmonary hypertension, a lower risk of thrombotic events [1]. These benefits are probably not altered by the laparoscopic approach of partial splenectomy we propose here.

Although the hematologic improvement after subtotal splenectomy is less spectacular than that observed after total splenectomy, literature shows subtotal splenectomy to be effective in decreasing the hemolytic rate [16]. This seems to be verified also in laparoscopic partial splenectomy since hemoglobin values in our patients returned to normal values and none of them needed postoperative blood transfusion.

Our study does not report regrowth of the splenic remnant in children neither need for a second operation for a total splenectomy, presented as an adverse effect of partial splenectomy by some authors [4, 21]. This can be explained by the medium follow-up of our study. Anyway, regrowth of the remnant spleen does not have a major impact on the beneficial outcome for other authors [1].

Laparoscopic partial splenectomy performed in our 3 patients also offers advantages of a minimally invasive approach. Laparoscopy is considered as the gold standard for simple splenectomy in children [12]. As a matter of fact, laparoscopy includes less complications such as wound dehiscence, infections, intussusception or pleural effusions. Less postoperative adhesions facilitate a second laparoscopy if completion of the splenectomy is required. According to Minkes and al., laparoscopic splenectomy in children can be performed safely with a low conversion rate (2.9%) [9]. This seems to be confirmed by our experience in laparoscopic partial splenectomy. In children, laparoscopy is also known as reducing pain, cost and hospital stay length. In our report, the mean hospital stay was only 4,6 days. Moreover, the laparoscopic approach in splenectomy has become easier and safer due to technological advances: anterior approach, larger bag used for specimen removal, specialized flexible hilar retractor [3]. But until a few years, peroperative bleeding remained the principal risk of the laparoscopic approach, which made laparoscopic partial splenectomy a tactful surgical option.

In our technique, bleeding risk is limited by initial control of the pedicle (by endoligature) and use of Ultracision Harmonic Scalpel (® Ethicon, Cincinnati, USA). Ultracision Harmonic Scalpel allows to complete a clean and non-hemorrhagic transection of the spleen and makes the laparoscopy safer and with minimal blood loss, as described for decapsulation of splenic cysts in children [10]. Neither per-operative transfusion nor conversion were necessary in our 3 patients. If ongoing blood loss does occur, it can correspond either to the spleen emptying (there is no danger for the patient) or to a non-ligated terminal vessel, feeding the splenic segment to be resected. But Ultracision Harmonic Scalpel increases the operative cost [15] and the surgeon must handle this instrument with greatest caution to avoid electric burns of the single persistent vascular pedicle. Nevertheless, Ultracision Harmonic Scalpel has more advantages than drawbacks. Other teams use a ligature device or the argon beam laser to control bleeding of the remnant spleen. We have no experience with these instruments. Splenopexy is also an important technical point of our technique since it avoids torsion and necrosis of the splenic remnant and thus guarantees the preservation of splenic tissue and function.

Two elements must be more precisely defined to improve partial laparoscopic splenectomy. First, the critical minimal splenic mass: although it has been estimated that between 5% and 40% of the spleen is required to maintain splenic function, most authors believe it is yet to be defined. In our surgical procedure, 80-90% of the splenic tissue was removed and no post-splenectomy infectious complication occurred. Evaluation of splenic volumes wasn’t difficult with the laparoscopic approach. Secondly, do we have to associate systematically cholecystectomy to our technique? As a matter of fact, partial laparoscopic splenectomy does not abolish the risk of formation of gallstones [1]. In our report, cholecystectomy was performed only if ultrasound showed gallstones. 

Conclusion

Laparoscopic partial splenectomy is feasible and safe in children with hereditary spherocytosis, essentially due to the use of Ultracision Harmonic Scalpel. This surgical procedure offers advantages of both a minimally invasive technique and preservation of splenic tissue, reducing the risk of post-splenectomy sepsis. Thus, partial laparoscopic splenectomy could become a very interesting approach to treat hereditary spherocytosis. A longer follow-up would be useful to evaluate the potential risk of regrowth of the splenic remnant after partial laparoscopic splenectomy. 

 

 

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