Subtotal splenectomy in Gaucher's disease: Towards a definition of critical splenic mass

M, Zer; Freud, Enrique

doi:10.1002/bjs.1800790806

Cited by 6 publications

(4 citation statements)

References 22 publications

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“…The disadvantages of total splenectomy are the risk of overwhelming sepsis and the more rapid accumulation of storage material in the bone marrow, liver, and other tissues rich in reticuloendothelial cells. With partial splenectomy, the protective action against sepsis is preserved and the accumulation of the glucocerbroside in other tissues is slowed [93,94].…”

Section: Surgical Conservation Of Splenic Tissuementioning

confidence: 99%

Asplenic-hyposplenic Overwhelming Sepsis: Postsplenectomy Sepsis Revisited

2001

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Absence of the spleen or splenic function predisposes individuals to risk of overwhelming infection. These infections are most often due to encapsulated organisms, especially pneumococcus, Haemophilus influenzae type b, and meningococcus, but any bacterial agent may cause the rapid onset of septicemia, meningitis, pneumonia, and shock characteristic of the asplenic-hyposplenic condition. The risk is greatest in infants and young children, but asplenic-hyposplenic adults also have an increased risk of infection. Prophylactic antibiotics and immunization with polyvalent pneumococcal, H. influenzae type b, and meningococcal vaccines have reduced the incidence of infections in asplenic-hyposplenic individuals, but even these measures have not eliminated the risk. Surgeons have adopted techniques to save as much splenic tissue as possible and some splenic functions, such as pitting red cells, have been preserved, but conservative surgery has not provided total protection against overwhelming infection. Therapies designed to interrupt the cascade of overwhelming sepsis have not yet been successful. In those cases in which the spleen is surgically removed, the underlying disease or condition leading to splenectomy influences the risk of sepsis. Splenectomy incidental to other operations, such as gastrectomy, results in the lowest risk for overwhelming infection, but this is still some 35-fold greater than the risk for overwhelming infections in the general population. In increasing order of risk, the other main indications for surgical removal of the spleen are idiopathic thrombocytopenia purpura, trauma, transplantation procedures, hereditary spherocytosis, staging Hodgkin's disease, portal hypertension with hypersplenism, and thalassemia. Pathologists should comment on the risk of overwhelming sepsis when spleens are processed as surgical specimens, and should carefully weigh all splenic tissue, including accessory spleens and splenic implants (splenosis), in autopsy cases with and without overwhelming sepsis.

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Section: Surgical Conservation Of Splenic Tissuementioning

confidence: 99%

Asplenic-hyposplenic Overwhelming Sepsis: Postsplenectomy Sepsis Revisited

2001

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“…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.…”

Section: Discussionmentioning

confidence: 99%

“…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 ]…”

Section: Discussionmentioning

confidence: 99%

Role of Partial Splenectomy in Gaucher's Disease in Resource Challenged Nations

Santanakrishnan

Dasaratha

Munianjanappa

et al. 2023

Journal of Indian Association of Pediatric Surgeons

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A BSTRACT 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.

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“…Whenever possible, splenectomy should be postponed until the second decade of life, in an attempt to minimize infectious and osteolytic complications (Ashkenazi et aI., 1986). Partial splenectomy has been employed at several centers (Rodgers et aI., 1987;Guzzetta et aI., 1987;Fleshner et aI., 1991;Zer and Freud, 1992;Petroianu, 1996a). In one series (Rubin et aI., 1986), partial splenectomy was attempted in II children with Gaucher's disease, and was successfully performed in seven.…”

Section: Hypersplen Ism Hypersplenism Is a Syndrome Inmentioning

confidence: 99%

The Complete Spleen

Bowdler¹

2002

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Subtotal splenectomy in Gaucher's disease: Towards a definition of critical splenic mass

Cited by 6 publications

References 22 publications

Asplenic-hyposplenic Overwhelming Sepsis: Postsplenectomy Sepsis Revisited

Asplenic-hyposplenic Overwhelming Sepsis: Postsplenectomy Sepsis Revisited

Role of Partial Splenectomy in Gaucher's Disease in Resource Challenged Nations

The Complete Spleen

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