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ORIGINAL ARTICLE
Year : 2023  |  Volume : 28  |  Issue : 2  |  Page : 149-153
 

Role of partial splenectomy in gaucher's disease in resource challenged nations


1 Department of Pediatric Surgery, Indira Gandhi Institute of Child Health, South Hospital Complex, Dharmaram College Post, Bengaluru, Karnataka, India
2 Affiliated to Indira Gandhi Institute of Child Health, Bengaluru, Karnataka, India

Date of Submission21-Sep-2022
Date of Decision03-Dec-2022
Date of Acceptance18-Dec-2022
Date of Web Publication03-Mar-2023

Correspondence Address:
Vinupriya Dasaratha
No. 535, 3rd Cross, B Block, AECS Layout, Kundalahalli, Bengaluru - 560 037, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiaps.jiaps_132_22

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   Abstract 


Context: Gaucher's disease (GD) is a rare inherited metabolic disorder caused by the defective activity of glucocerebrosidase. Enzyme replacement therapy (ERT) and substrate reduction therapy are the treatment of choice. Total splenectomy has a role when the child develops complications of massive splenomegaly. There are only a few case series of partial splenectomy in GD in the pediatric age group.
Aims: To study the role, technical feasibility, and challenges of partial splenectomy in children with GD with hypersplenism.
Materials and Methods: Retrospective review of children of GD who had partial splenectomy between February 2016 and April 2018. Demographics, clinical findings, laboratory, operative details, transfusion requirements, and perioperative, immediate, and late complications were retrieved. Clinical courses after discharge were obtained from follow-up data.
Results: Eight children with GD underwent partial splenectomy between 2016 and 2018. The median age at surgery was 3 years and 6 months (range −2 years to 8 years). Five children underwent partial splenectomy successfully, of which one child required postoperative ventilatory support for 48 h owing to lung atelectasis. Three children underwent completion splenectomy due to bleed from the cut surface of the splenic remnant. One of the children who underwent completion splenectomy expired on the postoperative day 5 due to refractory shock with multi-organ dysfunction.
Conclusion: Partial splenectomy has a definite role in selected children who present with massive splenomegaly with mechanical effects and/or hypersplenism while awaiting ERT.


Keywords: Enzyme replacement therapy, Gaucher's disease, Hypersplenism, Partial Splenectomy


How to cite this article:
Santanakrishnan R, Dasaratha V, Munianjanappa NB, Javaregowda D, Saroja MG. Role of partial splenectomy in gaucher's disease in resource challenged nations. J Indian Assoc Pediatr Surg 2023;28:149-53

How to cite this URL:
Santanakrishnan R, Dasaratha V, Munianjanappa NB, Javaregowda D, Saroja MG. Role of partial splenectomy in gaucher's disease in resource challenged nations. J Indian Assoc Pediatr Surg [serial online] 2023 [cited 2023 Mar 29];28:149-53. Available from: https://www.jiaps.com/text.asp?2023/28/2/149/371164





   Introduction Top


Gaucher's disease (GD) is a rare inherited metabolic disorder caused by the defective activity of glucocerebrosidase, a lysosomal enzyme that leads to the accumulation of glucocerebroside in the reticuloendothelial system and bone marrow.[1] Enzyme replacement therapy (ERT) and substrate reduction therapy are the treatment of choice, with an aim to treat before the onset of complications.[2] In resource-challenged nations, this treatment is expensive, and children develop complications as they await definitive treatment.

Total splenectomy has a role when a child develops complications of massive splenomegaly like life-threatening effects of thrombocytopenia, splenic infarction, splenic rupture, and mechanical effects.[2] There are only a few case series of partial splenectomy in GD in the pediatric age group; however, the experience of the procedure in a developing country with challenges of late referrals with complications and providing definitive management with advanced disease are lacking.

Aim and objectives

To study the role, technical feasibility, and challenges of partial splenectomy in children with GD with hypersplenism.


   Materials and Methods Top


Retrospective study of children with GD who underwent partial splenectomy between February 2016 and April 2018. Demographics, clinical findings, laboratory, operative details, transfusion requirements and perioperative, immediate, and late complications were retrieved. All children received pneumococcal, meningococcal, and Hemophilus influenza vaccination 2 weeks before planned surgery. Clinical courses after discharge were obtained from follow-up data and additional information by telephonic means about those children who could not visit the center for current follow-up.

Operative technique

The markedly enlarged spleen was delivered either by supra-umbilical transverse or midline incision [Figure 1]. Vascular anatomy was delineated at the hilum, and assessment was done for the feasibility of partial splenectomy. When feasible, splenic vessels were looped and controlled. Based on the configuration of the vascular supply, either the upper pole or lower pole is preserved to preserve the critical remnant spleen, taken roughly as the size of normal spleen [Figure 2]. Following the division of hilar vessels, line of demarcation appeared, devascularized zone is then resected using the bipolar sealing device and individual bleeders were transfixed and ligated/clipped [Figure 3]. Control of intrasplenic vessels forms the mainstay for achieving hemostasis, as the weight of a grossly enlarged spleen can cause vessels to avulse, retract and bleed. Hence, supporting the spleen with careful ligation/clipping of intrasplenic vessels was required. The cut surface was reinforced by hemostat agents [Figure 4]. When the lower pole of the spleen was retained, fixation of the spleen in the left hypochondrium was done to prevent torsion. When the upper pole was preserved, gastrosplenic ligament with short gastric vessels was preserved. An abdominal drain was placed in the left subphrenic space in all cases.
Figure 1: Dissection and delivering the organ

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Figure 2: Louping of polar vessel, eventually ligated

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Figure 3: Division of parenchyma along zone of demarcation with ligation and division of intrasplenic vessels

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Figure 4: Reinforcement of cut surface with hemostat agents

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   Results Top


Twelve children of GD underwent operative management between 2016 and 2018, of which eight children underwent partial splenectomy, and four underwent total splenectomy due to unfavorable vascular anatomy at the splenic hilum. The median age at surgery was 3 years and 6 months (range −2 years to 8 years). All the children had massive splenomegaly, bone marrow infiltration, and pancytopenia during the presentation, along with mechanical effects due to massive splenomegaly.

Five children underwent partial splenectomy successfully, of which one child required postoperative ventilatory support for 48 h owing to lung atelectasis. Three children underwent reexploration in the perioperative period and completion splenectomy due to bleeding from the cut surface of the splenic remnant. One of the children who underwent completion splenectomy expired on the postoperative day 5 due to refractory shock with multi-organ dysfunction [Table 1].
Table 1: Operative and perioperative details

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Five children underwent successful partial splenectomy and had good hematological response postsurgery done 12 days postsurgery [Table 2].
Table 2: Hematological improvement after partial splenectomy

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The median follow-up of these children is 4 years (range 6 years – 3 years). All five children who underwent successful partial splenectomy are on ERT. None of these children had any transfusion requirement or pneumonia or painful bone crisis, or pathological fractures postsurgery till their commencement of ERT. Serial ultrasound measurements of the remnant spleen with measurement of the spleen along the long axis showed gradual regrowth of the spleen [Table 3].
Table 3: Regrowth of spleen

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   Discussion Top


GD has three clinical forms: Type 1 is the most common and typically causes no neurological damage, whereas types 2 and 3 are characterized by neurological impairment.[2] The surgical implications are related to complications associated with massive splenomegaly.[2],[3]

The spleen is one of the largest lymphoid organs and is involved in the regulation of circulating blood volume, hematopoiesis, and immunity and also has a protective role in malignancies.[4] Splenic anatomy as described by Liu et al. showed that 86% of spleens exhibited two lobar vascular units, 12.2% of the specimen showed a third lobar vascular unit and this knowledge of splenic vascular anatomy enables successful splenic tissue saving procedures such as lobectomy, segmentectomy, heterotopic auto-transplantation of the hemi-spleen and allotransplantation.[5]

Total splenectomy has been demonstrated to result in impairment of the immunological system, as reflected in the decreased capacity to produce antibodies to bacterial antigens, the decreased levels of IgM and complement, and the complete lack of tuftsin, a substance that increases the phagocytic activity of the white blood cells, and potential for acceleration of hepatic and bony involvement.[6],[7]

In GD, partial resections are preferred to prevent complications related to total splenectomy and also prevents accelerated disease progression in the liver and bone marrow. The crucial steps for partial resections are, first, ligation and division of lobar vessels at the hilum and second, transection of splenic parenchyma with careful ligation of intrasplenic vessels. The complications anticipated with remnant spleen are bleeding, ischemia, and stasis.[1],[4]

The critical splenic remnant required to carry out splenic functions is controversial. Animal studies suggest that one-third of the normal spleen is sufficient to preserve normal immunological function. In GD, this tissue is pathological. Various studies state one fourth to one-half of normal spleen or remnant spleen equivalent to the size of normal spleen in a pathological spleen like in GD is sufficient to provide functional tissue for defense against overwhelming postsplenectomy sepsis.[4],[8],[9]

The first case report of partial splenectomy for GD was by Govrin-Yehudain and Bar-Maor in 1980, on an 11-year-old male with hypersplenism and remained asymptomatic for 6 months. Subsequently, they performed partial splenectomy on four children with GD with hypersplenism, of which one required completion splenectomy due to postoperative bleeding.[10]

One of the earliest series of partial splenectomies in children with GD was in 1986 by Rubin et al., 7 out of 11 children had a successful partial splenectomy. Conversion to total splenectomy in four children was due to hemorrhage,[1] necrosis,[1] and infarction.[2] All the children who underwent partial splenectomy had marked improvement in hematologic parameters as well as improvement in growth parameters and development.[3]

Other series of partial splenectomy in the pediatric age group by Kimber et al., 12 children underwent partial splenectomy for various indications, of which 3 had GD. All children had ERT, and hence, none of these children had documented splenic overgrowth.[11]

A systematic review by Esposito et al. in 2018, included 218 adults who underwent partial splenectomy for various indications, and showed a morbidity of 8% (range 5%–25%), which included fluid collection, atelectasis, pleural effusion, pulmonary embolism, pancreatic fistula, and portal vein thrombosis. 7/218 required completion splenectomy.[12]

In our series, 5/8 have undergone partial splenectomy successfully and are doing well on follow-up with all of these children on ERT. However, one among these had postoperative morbidity in the form of lung atelectasis. Three children had completion splenectomy due to hemorrhage, however, one among these succumbed on post operative day 5 due to refractory shock and multiorgan failure. As partial splenectomy for GD was not a routine surgery performed, the threshold for conversion to total splenectomy was kept low and hence, the higher conversion rates in the series.

Partial splenic artery embolization is a safer alternative in these sick children. There are few case reports where it has been successful, and many agents such as gel foam, microspheres, and coils have been used.[13],[14],[15],[16]


   Conclusion Top


Partial splenectomy has a definite role in selected children who present with massive splenomegaly with mechanical effects and/or hypersplenism while awaiting ERT. Partial splenectomy on a child with poor cardiovascular and hematological reserves is technically demanding and has a low threshold for completion splenectomy who require higher perioperative transfusion. Partial splenectomy preserves normal splenic function, buys time for definitive management, and prevents accelerated disease progression in the liver and bone marrow.

Acknowledgement

We thank Dr. Sanjeeva G N and Dr. Meenakshi Bhat for their support.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Fleshner PR, Aufses AH Jr., Grabowski GA, Elias R. A 27-year experience with splenectomy for Gaucher's disease. Am J Surg 1991;161:69-75.  Back to cited text no. 1
    
2.
Stirnemann J, Belmatoug N, Camou F, Serratrice C, Froissart R, Caillaud C, et al. A review of gaucher disease pathophysiology, clinical presentation and treatments. Int J Mol Sci 2017;441.  Back to cited text no. 2
    
3.
Rubin M, Yampolski I, Lambrozo R, Zaizov R, Dintsman M. Partial splenectomy in Gaucher's disease. J Pediatr Surg 1986;21:125-8.  Back to cited text no. 3
    
4.
Zer M, Freud E. Subtotal splenectomy in Gaucher's disease: Towards a definition of critical splenic mass. Br J Surg 1992;79:742-4.  Back to cited text no. 4
    
5.
Liu DL, Xia S, Xu W, Ye Q, Gao Y, Qian J. Anatomy of vasculature of 850 spleen specimens and its application in partial splenectomy. Surgery 1996;119:27-33.  Back to cited text no. 5
    
6.
King H, Shumacker HB Jr. Splenic studies. I. Susceptibility to infection after splenectomy performed in infancy. Ann Surg 1952;136:239-42.  Back to cited text no. 6
    
7.
Tahir F, Ahmed J, Malik F. Post-splenectomy sepsis: A review of the literature. Cureus 2020;12:e6898.  Back to cited text no. 7
    
8.
Rice HE, Oldham KT, Hillery CA, Skinner MA, O'Hara SM, Ware RE. Clinical and hematologic benefits of partial splenectomy for congenital hemolytic anemias in children. Ann Surg 2003;237:281-8.  Back to cited text no. 8
    
9.
Catalano M, Rizzo D, Coccia P, Maurizi P, Nanni L, Ruggiero A, et al. Can partial splenectomy preserve humoral immunity in pediatric patients? Risks and benefits of partial splenectomy. Signa Vitae 2018;14:17-9.  Back to cited text no. 9
    
10.
Bar-Maor JA. Partial splenectomy in Gaucher's disease: Follow-up report. J Pediatr Surg 1993;28:686-8.  Back to cited text no. 10
    
11.
Kimber C, Spitz L, Drake D, Kiely E, Westaby S, Cozzi F, et al. Elective partial splenectomy in childhood. J Pediatr Surg 1998;33:826-9.  Back to cited text no. 11
    
12.
Esposito F, Noviello A, Moles N, Cantore N, Baiamonte M, Coppola Bottazzi E, et al. Partial splenectomy: A case series and systematic review of the literature. Ann Hepatobiliary Pancreat Surg 2018;22:116-27.  Back to cited text no. 12
    
13.
Ahuja C, Farsad K, Chadha M. An overview of splenic embolization. AJR Am J Roentgenol 2015;205:720-5.  Back to cited text no. 13
    
14.
Pena AH, Kaplan P, Ganesh J, Clevac E, Marie Cahill A. Partial splenic embolization in a child with Gaucher disease, massive splenomegaly and severe thrombocytopenia. Pediatr Radiol 2009;39:1006-9.  Back to cited text no. 14
    
15.
Serratrice C, Cox TM, Leguy-Seguin V, Morris E, Yousfi K, Monnet O, et al. Splenic artery aneurysms, a rare complication of type 1 Gaucher disease: Report of five Cases. J Clin Med 2019;8:219.  Back to cited text no. 15
    
16.
Zhang L, Zhang ZG, Long X, Liu FL, Zhang WG. Severe complications after splenic artery embolization for portal hypertension due to hepatic cirrhosis. Risk Manag Healthc Policy 2020;13:135-40.  Back to cited text no. 16
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]
 
 
    Tables

  [Table 1], [Table 2], [Table 3]



 

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