Elective partial splenectomy in childhood

Elective By C. Kimber,

Partial L. Spitz,

London,

D. Drake,

England;

Splenectomy E. Kiely,

Oxford,

Background

& furpose; The indications and results of elective partial splenectomy in children remain uncertain. The aim of this study was to determine (I) the indications for elective partial splenectomy (EPS), (2) the extent of splenic resection, and (3) the complications of EPS.

/IJetho& Patients who underwent EPS from three centers over a IO-year period (1986 through 1996) were studied. Variables analyzed included clinical presentation and indications for EPS, extent of resection performed, perioperative transfusions, and complications. Postoperatively, splenic viability was determined by regular Doppler ultrasound scan and splenic function on peripheral blood film analysis. /?esu/ts; EPS was attempted in 12 children for various conditions: giant epithelial cyst (n = 6), hypersplenism and metabolic disease (n = 4), pseudotumor (n = I), and abscess (n = I). One procedure was converted to total splenectomy because of thin splenic tissue around an infected giant cyst. No child required transfusion for splenic bleeding. There

URGEONS AND PHYSICIANS have failed to take note of O’Donnell’s1 1929 report describing the fatal septicemia in a child with splenectomy who has hemolytic anemia. The child’s father died similarly. The hazard of removing the spleen was not appreciated until Ring and Shumacker’s report2 in 1952 describing five episodes of severe infection in children after undergoing splenectomy. It is now well established that the spleen is involved in several functions including regulation of circulating blood volume, hematopoiesis, immunity and protection against infections, and malignancy.3,~ The reported risk of overwhelming postsplenectomy sepsis (OPSS) is 4% with a mortality rate of l.5%.4 This risk doubles in children under 4 years of age with hematologic disorders. For these reasons retention of splenic function has been adopted by pediatric surgeons whenever possible.

S

From the Institute of Child Health cowl Great Ormond Street Hospital for Children, London, England: John Radcliffe Hospital, Oxford, England: and Institute of Paediatrics, Universrty of Rome “La Sapiemu, ” Roma, Italy. Presented at the 29th Armual Meeting of the Canadzan Association of Paediatric Surgeons, Ban& Alberta, Canada, October 3-6, 1997. Address reprint requests to Ago&no Pierre, MD, FRCS, Reader and Consultant m Paediatric Surgery, Institute of Child Health and Great Ormond Street Hospital for Children, 30 G&ford St, London WCIN IEH, England. Copyright 0 1998 by WB. Saunders Company 0022-3468/98/3306-0004$03.00/O a26

in Childhood

S. Westaby,

England;

F. Cozzi,

and

A. Pierro

and Rome, Italy

were no postoperative complications or deaths. Only 7 of the 12 children received prophylactic preoperative immunisation. In the 11 successful cases splenic remnant viability remains confirmed by ultrasound scan and normal blood film. There has been no significant splenic regrowth or recurrent hypersplenism in the four children with metabolic disorders. CO~C/US~OIJS; EPS is suitable for benign splenic conditions and can be performed without major blood loss. Preoperative vaccination is advisable. Up to 95% of the spleen can be safely removed, basing the blood supply of the residual spleen tissue on peripheral polar vessels, with adequate postoperative function. J Pediatr Surg 33:826-829. Copyright o 1998 by W.B, Saunders Company. INDEX WORDS: disease, spleen.

Partial

splenectomy,

splenic

cysts,

Gaucher’s

Although several series of partial splenectomies for trauma have been reported, there are only few collective series of elective partial splenectomy (EPS) performed in children primarily for Gaucher’s disease.5-sInitial reports of EPS for this condition described reduction of the spleen to its normal size, and rapid regrowth and hypersplenism occurred.9-10In patients with severe hypersplenism secondary to other metabolic disorders, the role and extent of EPS remains unclear. Other conditions that can benefit from partial splenectomy include splenic cysts, splenic abscesses, and tumors. However, marsupilization or aspiration/sclerotherapy for giant splenic cysts has a significant failure rate. no* Large splenic abscessesassociated with endocarditis are usually treated by total splenectomy to avoid the high mortality rate associated with splenic rupture and recurrent endocarditis.13 This study reports the recent favorable experience of three pediatric surgical centers with the use of EPS. MATERIALS

AND

METHODS

The records of 12 patients in whom EPS was attempted were reviewed The procedures were performed in three pediatric institutions (London, Oxford, and Rome) during the past 10 years (January 1986 through December 1996). Partial splenectomies for trauma were excluded. Varrables analyzed included clinical presentation and indications for EPS. extent of resection performed, p&operative transfusions, and complicatrons. Postoperatively, splenic integrity was determined by regular Doppler ultrasound scan and splenic function on peripheral blood film analysts. Red blood cell pitting and Howell-Jolly bodies, as Journa/

of Pecjiatric

Surgery,

Vol 33, No 6 (June),

1996: pp 926-629

ELECTIVE

PARTIAL

SPLENECTOMY

indtcators of sigmficant peripheral blood films children with Gaucher’s

827

splenic hypofunction. were searched for in all Adjuvant enzyme treatment was given to all disease after surgery.

Anatomy Recent anatomic dissection and latex mjecnon studies have shown that the spleen is supphed by two polar arteries that xise as terminal branches of the splenic artery in 86% of 850 specimens3 The two polar arteries subsequently divtde into five to six segmental lobar arteries. These arteries are end vessels and may communicate at the hilum but not beyond. The venous drainage follows the artenal supply; however, there are multiple connections between segmental lobar veins.

Techkque of Partial Splenectomy A transverse left upper abdominal incision was made. The lesser sac was entered and vessel slings were placed around the splenic artery and vem. The spleen was then mobilized by division of the lienorenal ligament. Hemtsplenectomy was based on ligation of a polar artery and control of intersegmental venous bleeding following splenic division. A 95% splenectomy. as required for Gaucher’s disease or Niemann-Pick’s disease. aimed to preserve a single segmental lobar artery and vein to the upper or lower pole remnant. The polar vessels were identified and the appropriate artery and vein ligated. Occasionally. a smgle polar artery was present and intracapsular dissection with ligation of the segmental lobar vessels was required. A line of ischemic demarcation appeared on the spleen. This line was superficially marked with electrocoagulation followed by intracapsular dissection perfotmed usmg an ultrasonic dtssector (20,000 Hz) or electrocoagulation. Venous connections between segments were meticulously ligated during excision. Hemostasis of the raw surface was achieved with surgicell and mterrupted suturing of the splenic capsule. In three cases omentum was placed around the splenic remnant, If the reman-ring spleen is mobile and at risk of torsion, it may be placed in aperitoneal pouch fashioned in the left hypogastrium (one case).

Giunt Epithelial Cysts Six children presented to Great Ormond Street Hospital for Children, London, with intraabdominal masses that ultrasound scanning identified as unilocular splenic cysts greater than 5 cm in diameter. Upper (n = 5) or lower (n = 1) pole EPS was attempted and was successful in five patients. One procedure was converted to total splenectomy because of thin friable splenic tissue around an infected giant cyst. This 14-year-old girl was a Jehovah’s witness, and she was the only patient in this group to receive preoperative vaccination. Histopathologic findings confirmed congenital epithelial cysts in all cases. There has been no evidence of cyst recurrence (follow-up range, 2 to 10 years). These six cases have been reported previously.14 Metabolic Disorders Three children with Gaucher’s disease type III and one child with Niemann-Pick’s disease were treated at Great Ormond Street Hospital for Children, London. All four children had massive splenomegaly and severe hypersplenism. A 95% EPS was performed. basing the blood supply to the remnant on the peripheral polar vessel3 The three patients with Gaucher’s disease also presented with persistent vomiting secondary to the large intraabdominal mass. Postoperatively they commenced ceradase, an enzyme that prevents splenic regrowth and further cerebral deterioration by blocking B glucocerebrosidase deposition. The child with Niemann-Pick’s disease did not have the echogenic splenic tumors associated with this metabolic disorder.

RESULTS

The age of the 12 children who underwent EPS ranged from 1 to 16 years (Table 1). Older children presented with giant epithelial cysts, whereas hypersplenism was found in the younger age group. No child required transfusion for splenic bleeding. There were no postoperative complications, septic events, or deaths. Only 7 of the 12 children received preoperative prophilactic immunization. In the 11 successful cases, splenic remnant viability was confirmed by Doppler ultrasound scan and normal blood film. The clinical course is described for the individual diseases.

EFtdocarditis With Splenic Abscess A previously well child presented with endocarditis at John Radcliffe Hospital, Oxford. An aortic coarctation was detected and a 5-cm solid lesion in the upper pole of the spleen liquefied and became debris ridden over a 3-week period. She did not respond to antibiotics and underwent coarctation repair (homograft patch) and upper pole EPS in one procedure. A large splenic abscess cavity was found, and no vegetations were identified on the coarct. Three years later she has normal splenic function and no further septic episodes. Inflammatory Pseudotumor

Table

1. Indications,

of Surgery,

Extent Who

Underwent Age

Diagnws

Giant

cyst

rdo

hl

and Outcome

for 12 Patients

EPS

Preoperative Vaccine

Resectmn t%)

6

S-16

1

50

Gaucher’s disease Niemann-PvSs disease Pseudotumour

3 1 1

l-2 3 3

3 1 1

95 95 50

Abscess

1

8

1

50

Conversvm to Total

1

A 3%-year-old child with splenomegaly presented at the Institute of Paediatrics, Rome. A solid upper pole mass was found on ultrasound scanning. All other routine investigation findings were normal. The preoperative diagnosis was unclear, and CT scanning demonstrated some contrast enhancement of the tumor. At laparotomy the tumor was removed with the upper pole of the spleen. The tumor consisted of spindle-shaped fibroblasts mixed

828

KIMBER

with a diffuse inflammatory infiltrate. Two years later the child is well with no evidence of recurrence. DISCUSSION

This series demonstrates the wide applicability and safety of EPS in children. It is the operation of choice for all benign splenic conditions, where total splenectomy is not required for hematologic control. Recent anatomic studies have demonstrated that the spleen is made of two primary lobes, one accessory lobe, and three to five segments.3 Each segmental artery supplies the correspondent segment in a wedge shape with relative avascular planes between segments. Lobar arterial branching occurs usually close to the surface of the splenic hilum necessitating a careful dissection before devascularization Extensive splenic resection is possible even in the presence of massive splenomegaly. The vasculature on the cut surface should be coagulated by monopolar diathermy or clamped and ligated. It is advisable to cover the cut surface with the greater omenturn. In our experience the procedure is safe, relatively simple, and can be performed without the need for perioperative blood transfusion. When the upper pole of the spleen needs to be preserved, the blood supply can be supported by the short gastric vessels as described by Fonkalsrud et al7 For giant splenic cysts, EPS is preferable to the partial decapsulation technique described by Touloukian.15 Removal of the entire cyst eliminates the risk of recurrence or malignancy developing in the retained cyst epithelial 1ining.n’ This important principle can be achieved laparoscopically by marsupilizing the cyst and destroying all remaining epithelium using an argon beam coagulator (penetration depth, 1 mm). Temporary control of tbe main splenic vessels can be achieved endoscopically using a lo-mm diameter detachable intestinal clamp. This technique has been performed successfully in three adolescents by Cuschieri (personal communication). Although we have no experience in this technique and no long-term follow-up is available, it is likely to become the surgery of choice for larger cysts located at either the upper or lower pole. Dissection and vascular control of hilar cysts is likely to be more difficult laparoscopically. EPS has been reported widely as being unsuccessful for control of hypersplenism in Gaucher’s disease, mainly because of the rapid splenic regrowth.5,9,10,17However, in

ET AL

nine children with Gaucher’s disease who underwent total splenectomy, OPSS developed in four patients, and one died.ls Enzyme therapy alone may control the hypersplenism, but it can take years, while the child continues to require multiple transfusions. In type III Gaucher’s with severe hypersplenism, currently we remove 95% of the spleen and give ceradase postoperatively. This should prevent regrowth and should allow preservation of immunologic function. The role of EPS in Niemann-Pick’s disease remains unclear with no collective data available. Small splenic abscess will often resolve with antibiotic therapy alone, however, large collections, and those complicated by endocarditis, require resection.ig EPS, where possible, offers preservation of immunological function, particularly important for patients with associated cardiac disease. Several splenic tumors, including lymphomas, angiosarcomas, teratomas, and inflammatory pseudotumors, can present as an isolated solid mass.20,21Needle biopsy is often inconclusive, particularly with necrotic tumors, and may cause significant bleeding. EPS is safe for the diagnosis and therapy of these conditions. Total splenectomy should be avoided unless malignancy has been diagnosed histologically. Patients undergoing EPS are at risk of requiring a total splenectomy (l/l2 in this series) and warrant preoperative immunization for meningococcus, hemophilus, and pneumococcus. In this series, five children did not receive vaccination, which was in retrospect, a clinical error. There is some evidence that absence of HowellJolly bodies is a useful guide to normal immune function, but little data exist in children for the rare conditions we described.22 Antibody responses to vaccines may be reduced in asplenic individuals but titers cannot be used directly to predict splenic function, and many vaccines still may be effective if given to asplenic individuals. Red cells pitting is thought to be the most sensitive marker of splenic function and was performed in the study together with evaluation of splenic viability by Doppler ultrasound scan. EPS is suitable for benign splenic conditions and can be performed without major blood loss. Up to 95% of the spleen can be safely removed with adequate postoperative filtration function.

REFERENCES 1. O’Donnell FJ: The value of splenectomy m Banti’s disease. Br Med J 1:854,1929 2. King LK, Shumacker HBJ: Susceptibility to infection after splenectomy performed in infancy. Ann Surg 136:239-242.1952 3. Liu DL, Xia S, Xu W, et al: Anatomy of vasculature of 850 spleen specimens and its applicanon in parttal splenectomy. Surgety 119:2733, 1996

4. Holdsworth RJ. Cuschieri A: The eprdemiology, pathogenesis and prevention of postsplenectomy sepsis, m Forbes CA, (ed): Disorders of the Spleen. Oxford, England Blackwell Scientific, 1994, pp 229-239 5. Zimran A, Elstein D, Schiffmann R. et al: Outcome of partial spIenectomy for type I Gaucher disease. J Pedran 126:596-597. 1995 6. Rubin M, Yampolski I, Lambrozo R, et al: Partial splenectomy in Gaucher’s disease. J Pediatr Surg 21:125-128, 1986

ELECTIVE

PARTIAL

SPLENECTOMY

7. Fonkalsrud EW, Philippat M, Feig S: Ninety-five tomy for massive splenomegaly: A new approach, 25:267-269.

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percent splenec.I Pediatr Surg

1990

8. 3ar Maor J, Govrin YJ: Partial splenectomy in children with Gaucher’s disease, Pediatrtcs 76:398-401. 1985 9. Bar Maor J: Partial splenectomy m Gaucher’s disease: Follow-up report. .I Pediatr Surg 28.686-688, 1993 10. Cohen 1.I. Katz K, Freud E. et al: Long-term follow-up of partial splenectomy in Gaucher’s disease. Am J Surg 164:345-347. 1992 11. Moir C, Guttman F. Jequier S, et al: Splemc Cysts: Aspiration, Sclerosis, or Resection. J Pediatr Surg 24:646-648. 1989 12. Musy PA, Roche B. Belli D. et al: Splenic cysts in pediatric patients-A report on 8 cases and review of the literature. Eur J of Pediatr Surg 2:137-140, 1992 13. Senecky JG, Passwell J. Iuchtman M, et al: Rupture of spleen complicating infective endocarditis. Am J Dis Child 135:1144-1145, 1981 14. Kimber C, Pierro A, Drake D, et al: Hemisplenectomy for giant splenic cysts in children. Pediatr Surg Int (in press) 15. Touloukian RJ, Maharaj A, Ghoussoub R, et al: Partial decapsu-

lation of splenic eptthehal cysts: Studies on etiology and outcome. J Pediatr Surg 32:272-274, 1997 16. Eht L, Aylward B: Splenic cyst carcmoma presentmg in pregnancy. Am J Hematol 32.57-60. 1989 17. Kyllerman M, Conrad1 N, Mansson JE, et al: Rapidly progressive type III Gaucher disease: Deterioration following partial splenectomy. Acta Paediatr Stand 79:448-453. 1990 18. Walker W Splenectomy in childhood: A review of cases in England&Wales 1960.64. Br .I Surg 63:36-43. 1976 19. Ting W, Silverman NA, Arzouman DA, et al: Splemc septic emboli in endocarditts. Circulation 5:105-109, 1990 20. Sordillo EM, Sordillo PP. Hajdu SI: Primary hemangiosarcoma of the spleen: Report of four cases. Med Pediatr Oncol9:319-324. 1981 21. Thomas RM, Jaffe ES, Zarate OA, et al: Inflammatory pseudotumar of the spleen. Aclinicopathologic and immunophenotypic study of eight cases. Arch Path01 Lab Med 117:92J-926, 1993 22. Corraza GR. Ginaldi L, 2011 G, et al. Howell-Jolly bodies as a measure of splenic function. A reassessment. Clin Lab Haematol 12:269-275, 1990