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CASE REPORT
Year : 2018  |  Volume : 11  |  Issue : 5  |  Page : 433-435  

Massive splenomegaly associated with multiple splenunculi in B-Thalassemia major


1 Department of Paediatric Surgery, Kota Medical College, Kota, Rajasthan, India
2 Department of Anaesthesiology, Kota Medical College, Kota, Rajasthan, India
3 Department of Pathology, Kota Medical College, Kota, Rajasthan, India

Date of Web Publication5-Sep-2018

Correspondence Address:
Rahul Gupta
Department of Paediatric Surgery, Kota Medical College, Kota, Rajasthan
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/mjdrdypu.MJDRDYPU_244_17

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  Abstract 


A 4-year-old male child with β-thalassemia major presented with massive splenomegaly and hepatomegaly along with increasing transfusion requirements (700 ml/kg/last year). Splenectomy was performed; the meticulous search revealed two small splenunculi embedded in the greater omentum near the splenic hilum, which was removed. Histopathological examination confirmed splenunculi. It is important to remove splenunculi during splenectomy for hematological disorders because failure to remove them may result in the nonresolution of the condition as they take over the function of the original spleen.

Keywords: Accessory spleens, massive splenomegaly, multiple splenunculi, splenectomy, β-thalassemia major


How to cite this article:
Gupta R, Mittal J, Soni N. Massive splenomegaly associated with multiple splenunculi in B-Thalassemia major. Med J DY Patil Vidyapeeth 2018;11:433-5

How to cite this URL:
Gupta R, Mittal J, Soni N. Massive splenomegaly associated with multiple splenunculi in B-Thalassemia major. Med J DY Patil Vidyapeeth [serial online] 2018 [cited 2019 Sep 18];11:433-5. Available from: http://www.mjdrdypv.org/text.asp?2018/11/5/433/240382




  Introduction Top


The treatment of thalassemia major has conventionally been red blood cell (RBC) transfusion therapy, iron chelation therapy, and splenectomy.[1] Splenectomy in thalassemia major is indicated when the disease is not suppressed efficiently by transfusion treatment.[2] Splenunculi may be present in 10%–30% of the population,[3],[4] and it may range between 20% and 44.4% in patients with splenic involvement due to hematological disorders.[4] We are presenting a 4-year-old child with β-thalassemia major who had massive splenomegaly and was found to have multiple splenunculi during splenectomy.


  Case Report Top


A 4-year-old male child, a known case of thalassemia, presented to the Department of Paediatric Surgery for elective splenectomy owing to massive splenomegaly along with increasing transfusion requirements. The patient had suffered repeated episodes of respiratory tract infection, diarrhea, anemia, jaundice, and failure to gain weight at the age of 1 year (approximately 3 years back). Investigations revealed pancytopenia and serum ferritin >2000 ng/ml by chemiluminescence immunoassay. Hemoglobin (Hb) variant analysis by high-performance liquid chromatography showed markedly elevated fetal Hb (HbF – 93.6%, HbA2 – 4.9%, and HbA – 1.2%) which was diagnostic of β-thalassemia major. The patient had received 80 blood transfusions, that is, packed cell volumes (PCVs) over the last 3 years. On examination, the patient was pale with mild icterus, enlarged head size, prominent frontoparietal bossing, prominent zygoma, and other features suggestive of extramedullary hematopoiesis which were confirmed by radiographic evaluation [Figure 1]. The skin showed dirty brown pigmentation due to iron deposition [Figure 2]. Abdominal examination revealed enlarged liver (palpable 6 cm below the costal margin) and palpable spleen (7 cm below the costal margin [Figure 2]). Investigations showed anemia (Hb – 5.8 g%) with hematocrit – 17.5%, jaundice (total bilirubin – 2.1 mg%, indirect – 1.2 mg%), and raised liver enzyme levels (serum glutamate oxaloacetate transaminase – 451 IU/L and serum glutamic-pyruvic transaminase – 304 IU/L). Ultrasound confirmed hepatomegaly and massive splenomegaly. Preoperative vaccination against Streptococcus pneumoniae was administered 2 weeks before surgery. Preoperative optimization was performed. Left subcostal incision was given and splenectomy was performed [Figure 2]. Meticulous search revealed two small splenunculi embedded in the greater omentum near the splenic hilum [Figure 2], which were removed [Figure 2]. Postoperative period was uneventful. Histopathological examination revealed splenunculi having a similar architecture as that of the spleen [Figure 3] and [Figure 4]. On early follow-up, the patient is doing well with fewer transfusion requirements. He has been counseled for iron chelation therapy.
Figure 1: Skull radiographs showing thickened membranous bones of the skull, especially the frontal bone with prominent bossing. The skull is having a granular appearance; radiographs of hands showing mild expansion of metacarpals and phalanges

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Figure 2: Clinical photographs (left) showing skin with dirty brown pigmentation suggestive of iron deposition along with splenohepatomegaly; intraoperative photographs showing (center and right) enlarged spleen being removed; two small splenunculi embedded in the greater omentum near the splenic hilum seen after splenectomy; inset image at the bottom showing specimen of spleen and both the splenunculi

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Figure 3: Microscopic examination of the spleen showing splenic capsule with red pulp and white pulp (white arrows) with supporting network (top × 10); Red pulp of the spleen with venous sinuses and splenic cords (bottom × 100)

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Figure 4: Microscopic examination revealing splenunculi having trabecular network similar to the architecture of the spleen but with paucity of lymph follicles

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  Discussion Top


Thalassemia is a type of hemoglobinopathy, that is, a group of disorders of the RBC protein and Hb. β-thalassemia major (1 in 100,000 live births) is caused by mutations in the gene responsible for producing β-globin chain on the chromosome 11.[2],[5] Transfusion therapy aims to correct anemia and to suppress endogenous marked but ineffective erythropoiesis, thus preventing conspicuous skeletal changes or splenohepatomegaly. Splenectomy is reserved in thalassemia, with marked symptoms related to the extent of splenomegaly, increased transfusion requirements more than 250 ml/kg/year of PCV or more than 400 ml/kg/year of whole blood, and clinically significant complications such as pancytopenia.[1],[2] In our patient, the transfusion exceeded 2.5 times the indicated level (700 ml/kg/last year) and massive splenomegaly was evident. Splenectomy improves anemia and reduces the blood transfusion requirement.[1],[2]

Ectopic splenic tissue can be categorized into splenosis and splenunculi. Splenosis is caused by autotransplantation of viable splenic tissue in the abdominal cavity; typically appearing as numerous small nodules. It usually occurs after splenectomy and trauma.[6] Splenunculi, also known as an accessory spleen, supernumerary spleen, or splenule, are congenital foci of healthy splenic tissues that are found apart from the main body of the spleen due to abnormal deposition of some of the cells from the developing spleen along the path of its formation during embryonic life.[7] Splenunculi have normal splenic histology, while in splenosis, histology usually reveals distorted architecture with absence of hilum and a poorly formed capsule with variable shape and size.[6]

On computed tomography evaluation, splenunculi can be encountered in 11.0%–18.8% of cases,[7],[8] and multiple splenunculi may be seen in up to 14.28% of these cases.[4] The maximum diameter may range from 3 to 79 mm.[7] Splenunculi are usually located near the spleen's hilum, though they may be present anywhere in the peritoneal cavity, that is, gastrocolic ligament, tail of pancreas, greater omentum, greater curvature of stomach, splenocolic ligament, small and large bowel mesentery and pancreaticosplenic ligament, and also retroperitoneal space and very rarely in the scrotum.[1],[4],[9] Ultrasonography (US) may reveal splenunculi as hypoechoic round mass. Preoperative diagnosis of splenunculi may be missed on US, which was also seen in our case.[7],[8]

Splenunculi are usually asymptomatic and discovered incidentally.[10] Their clinical implication falls into three clinical situations: (a) Splenunculi may present as acute abdomen with torsion, spontaneous rupture, hemorrhage, intraperitoneal inflammatory mass, or cyst formation; (b) it may be wrongly implicated as enlarged lymph nodes or abdominal neoplastic mass from the tail of the pancreas, gastrointestinal tract, kidney, adrenal gland, or gonads during radiological imaging; and (c) finally, splenunculi may enlarge after splenectomy and result in the recurrence of hematological disorders for which the splenectomy was performed. A meticulous search for splenunculi is mandatory during splenectomy for hematological disorders (including thalassemia), when the intention is to remove all functional splenic tissue.[1],[10]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Acknowledgment

I am sincerely thankful to faculty and residents of Department of Anaesthesia, Kota Medical College, Kota, for helping in this endeavor.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Porecha M, Udani D, Mehta V, Gami A. Splenectomy in management of thalassemia major – A boon for the little angel. Int J Surg 2009;24:1-11.  Back to cited text no. 1
    
2.
Ammar SA, Elsayh KI, Zahranc AM, Embaby M. Splenectomy for patients with β-thalassemia major: Long-term outcomes. Egypt J Surg 2014;33:232-6.  Back to cited text no. 2
  [Full text]  
3.
Mortelé KJ, Mortelé B, Silverman SG. CT features of the accessory spleen. AJR Am J Roentgenol 2004;183:1653-7.  Back to cited text no. 3
    
4.
Curtis GM, Movitz D. The surgical significance of the accessory spleen. Ann Surg 1946;123:276-98.  Back to cited text no. 4
    
5.
Galanello R, Origa R. Beta-thalassemia. Orphanet J Rare Dis 2010;5:11.  Back to cited text no. 5
    
6.
Fremont RD, Rice TW. Splenosis: A review. South Med J 2007;100:589-93.  Back to cited text no. 6
    
7.
Rashid SA. Accessory spleen: Prevalence and multidetector CT appearance. Malays J Med Sci 2014;21:18-23.  Back to cited text no. 7
    
8.
Romer T, Wiesner W. The accessory spleen: Prevalence and imaging findings in 1,735 consecutive patients examined by multidetector computed tomography. JBR-BTR 2012;95:61-5.  Back to cited text no. 8
    
9.
Budzynski A, Bobrzyński A, Sacha T, Skotnicki A. Laparoscopic removal of retroperitoneal accessory spleen in patient with relapsing idiopathic thrombocytopenic purpura 30 years after classical splenectomy. Surg Endosc 2002;16:1636.  Back to cited text no. 9
    
10.
Wang G, Chen P, Zong L. Accessory spleen arising from the gastric fundus mimicking gastrointestinal stromal tumor following splenectomy: A case report. Exp Ther Med 2014;7:349-51.  Back to cited text no. 10
    


    Figures

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



 

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