Abstract
A thorough understanding of the anatomy, physiology, and development of the spleen is essential for determining the pathophysiological mechanisms underpinning splenic diseases and congenital variations. The aim of this review is to briefly summarize current knowledge regarding the normal development of the spleen, and to provide an overview of clinically relevant congenital splenic variations. These include such variations as asplenia, polysplenia, hyposplenia, lobulation of spleen, accessory spleens, accessory splenic nodules, wandering spleen, splenogonadal and splenopancreatic fusion, splenic cysts, and cavernous haemangioma of the spleen. All of these congenital variations are also mentioned in internationally accepted embryological nomenclature, known as the Terminologia Embryologica. Interestingly, most patients who have these diseases are asymptomatic, and are often diagnosed only after an injury or during unrelated medical procedures. Using examples from published case reports, we highlight how an understanding of the embryology of the spleen and the etiology of its disease states would improve clinical practice.
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References
Abbott RM, Levy AD, Aguilera NS, Gorospe L, Thompson WM (2004) From the archives of the AFIP: primary vascular neoplasms of the spleen: radiologic-pathologic correlation. Radiographics 24(4):1137–1163
Accinni A, Bertocchini A, Madafferi S, Natali G, Inserra A (2016) Ultrasound-guided percutaneous sclerosis of congenital splenic cysts using ethyl alcohol 96% and minocycline hydrochloride 10%: a pediatric series. J Pediatr Surg 51(9):1480–1484
Balli O, Karcaaltincaba M, Karaosmanoglu D, Akata D (2009) Multidetector computed tomography diagnosis of fusion of pancreas and spleen confirmed by magnetic resonance imaging. J Comput Assist Tomogr 33(2):291–292
Benelli G, Bonardi R, Parziale M, Campari PF (1992) Role of echography in the differential diagnosis of accessory lobulations and small capsular traumatic fissures of the spleen. Radiol Med 84(6):770–773
Cesta MF (2006) Normal structure, function, and histology of the spleen. Toxicol Pathol 34(5):455–465
Chen CH, Wu HC, Chang CH (2005) An accessory spleen mimics a left adrenal carcinoma. MedGenMed 7(2):9
Chiu SN, Shao PL, Wang JK, Chen HC, Lin MT, Chang LY, Lu CY, Lee PI, Huang LM, Wu MH (2014) Severe bacterial infection in patients with heterotaxy syndrome. J Pediatr 164(1):99–104
Cirillo RL Jr, Coley BD, Binkovitz LA, Jayanthi RV (1999) Sonographic findings in splenogonadal fusion. Pediatr Radiol 29:73–75
Cortes D, Thorup JM, Visfeldt J (1996) The pathogenesis of cryptorchidism and splenogonadal fusion: a new hypothesis. Br J Urol 77:285–290
De Rosa F, Lapiccirella G (1994) Splenic lobulation simulating a gastric neoformation. The diagnostic value of echography. Radiol Med 87(4):521–522
Drut RM, Drut R, Gilbert-Barness E, Reynolds JF Jr (1993) Abnormal spleen lobulation and short pancreas. Birth Defects Orig Artic Ser 29(1):345–352
Endo A, Ueno S, Yamada S, Uwabe C, Takakuwa T (2015) Morphogenesis of the spleen during the human embryonic period. Anat Rec (Hoboken) 298(5):820–826
Federative International Programme on Anatomical Terminology (2013) Terminologia embryologica: international anatomical terminology. Thieme Medical Publishers, New York
Fowler RH (1913) Cysts of the spleen, a pathological and surgical study. Ann Surg 57:658–690
Ghuliani D, Agarwal S, Thomas S, Pathania OP (2008) Giant cavernous haemangioma of the spleen presenting as massive splenomegaly and treated by partial splenectomy. Singap Med J 49(12):e356–e358
Gomi K, Sato Y, Tanaka M, Ijiri R, Kato K, Aoki I, Tanaka Y (2009) Specificity of splenopancreatic field abnormality in trisomy 13 syndrome: macroscopic and histological analysis in 21 autopsy cases. Pathol Int 59(3):147–151
Hommel B, Galloula A, Simon A, Buffet P (2013) Hyposplenism revealed by Plasmodium malariae infection. Malar J 12:271
Impellizzeri P, Montalto AS, Borruto FA, Antonuccio P, Scalfari G, Arena F, Romeo C (2009) Accessory spleen torsion: rare cause of acute abdomen in children and review of literature. J Pediatr Surg 44(9):e15–e18
Ingle SB, Hinge Ingle CR, Patrike S (2014) Epithelial cysts of the spleen: a minireview. World J Gastroenterol 20(38):13899–13903
Kadota K, Kushida Y, Miyai Y, Katsuki N, Hayashi T, Bando K, Shibuya S, Haba R (2010) Epidermoid cyst in an intrapancreatic accessory spleen: three case reports and review of the literatures. Pathol Oncol Res 16(3):435–442
Kapellerová A, Siman J, Rasková J, Cingel V, Haviar D (1999) Enuresis as a rare symptom of the dystopic spleen in a child. Eur J Pediatr 158(10):870–871
Karakaş HM, Tunçbilek N, Ökten ÖÖ (2005) Splenic abnormalities: an overview on sectional images. Diagn Interv Radiol 11:152–158
Khairat AB, Ismail AM (2005) Splenogonadal fusion: case presentation and literature review. J Pediatr Surg 40(8):1357–1360
Kirkineska L, Perifanis V, Vasiliadis T (2014) Functional hyposplenism. Hippokratia 18(1):7–11
Kothari SS (2014) Non-cardiac issues in patients with heterotaxy syndrome. Ann Pediatr Cardiol 7(3):187–192
Kubo M, Yamane M, Miyatani K, Udaka T, Mizuta M, Shirakawa K (2006) Familial epidermoid cysts of the spleen: report of two cases. Surg Today 36(9):853–856
Lakshmanan PM, Reddy AK, Nutakki A (2014) A surprising content of congenital hernia: complete splenogonadal fusion band. BMJ Case Rep. doi:10.1136/bcr-2014-203640
Lehman AM, McFadden D, Pugash D, Sangha K, Gibson WT, Patel MS (2008) Schinzel-Giedion syndrome: report of splenopancreatic fusion and proposed diagnostic criteria. Am J Med Genet A 146A(10):1299–1306
Lehtinen SJ, Schammel CM, Devane M, Trocha SD (2013) Intrapancreatic accessory spleen presenting as a pancreatic mass. J Gastrointest Oncol 4(4):E23–E26
Magowska A (2013) Wandering spleen: a medical enigma, its natural history and rationalization. World J Surg 37:545–550
Malik RD, Liu DB (2013) Splenogonadal fusion: an unusual case of an acute scrotum. Rev Urol 15(4):197–201
Mandosse P, Bourg S, Paulhac P, Dumas JP, Colombeau P (2000) Splenic lobulation and pseudo-cancer of the left kidney. Prog Urol 10(2):291–294
Marchant LK, Levine MS, Furth EE (1995) Splenic implant in the jejunum: radiographic and pathologic findings. Abdom Imaging 20(6):518–520
Mathew H, Dittus C, Malek A, Negroiu A (2015) Howell-Jolly bodies on peripheral smear leading to the diagnosis of congenital hyposplenism in a patient with septic shock. Clin Case Rep 3(8):714–717
Mirilas P, Mentessidou A, Skandalakis JE (2007) Splenic cysts: are there so many types? J Am Coll Surg 204(3):459–465
Misra AP, Misra R, Kumar A (2013) Giant cavernous haemangioma of the wandering spleen. Indian J Surg 75(1):54–55
Morgenstern L (2002) Nonparasitic splenic cysts: pathogenesis, classification, and treatment. J Am Coll Surg 194(3):306–314
Orlando R, Lumachi F, Lirussi F (2005) Congenital anomalies of the spleen mimicking hematological disorders and solid tumors: a single-center experience of 2650 consecutive diagnostic laparoscopies. Anticancer Res 25(6C):4385–4388
Paraskevas GK, Koutsouflianiotis KN, Nitsa Z, Demesticha T, Skandalakis P (2016) Knowledge of the anatomy and physiology of the spleen throughout Antiquity and the Early Middle Ages. Anat Sci Int 91(1):43–55
Peddu P, Shah M, Sidhu PS (2004) Splenic abnormalities: a comparative review of ultrasound, microbubble-enhanced ultrasound and computed tomography. Clin Radiol 59:777–792
Peres LC, Barbosa GH, Careta RS, Nassif CM, de Pina-Neto JM, Giuliani LR, Martinhago CD, Gomy I (2004) Splenopancreatic field abnormality is not unique to trisomy 13. Pediatr Dev Pathol 7(1):91–94
Richman M, Hiyama DT, Wasson E (2014) Wandering spleen. Surgery 155(4):728
Roberts AS, Shetty AS, Mellnick VM, Pickhardt PJ, Bhalla S, Menias CO (2016) Extramedullary haematopoiesis: radiological imaging features. Clin Radiol 71(9):807–814
Rosenberg HK, Markowitz RI, Kolberg H, Park C, Hubbard A, Bellah RD (1991) Normal splenic size in infants and children: sonographic measurements. Am J Roentgenol 157(1):119–121
Scheuerman O, Bar-Sever Z, Hoffer V, Gilad O, Marcus N, Garty BZ (2014) Functional hyposplenism is an important and underdiagnosed immunodeficiency condition in children. Acta Paediatr 103(9):e399–e403
Sinha CK, Agrawal M (2011) Nonparasitic splenic cysts in children: current status. Surgeon 9(1):49–53
Soleimani M, Mehrabi A, Kashfi A, Fonouni H, Büchler MW, Kraus TW (2007) Surgical treatment of patients with wandering spleen: report of six cases with a review of the literature. Surg Today 37(3):261–269
Sountoulides P, Neri F, Bellocci R, Schips L, Cindolo L (2014) Splenogonadal fusion mimicking a testis tumor. J Postgrad Med 60(2):202–204
Srisupundit K, Piyamongkol W, Tongprasert F, Luewan S, Tongsong T (2011) Reference range of fetal splenic circumference from 14 to 40 weeks of gestation. Arch Gynecol Obstet 283(3):449–453
Steiniger B, Ulfig N, Risse M, Barth PJ (2007) Fetal and early post-natal development of the human spleen: from primordial arterial B cell lobules to a non-segmented organ. Histochem Cell Biol 128(3):205–215
Taskin MI, Baser BG, Adali E (2015) Accessory spleen in the pelvis: a case report. Int J Surg Case Rep 12:23–25
Tawfik AM, Batouty NM, Zaky MM, Eladalany MA, Elmokadem AH (2013) Polysplenia syndrome: a review of the relationship with viscero-atrial situs and the spectrum of extra-cardiac anomalies. Surg Radiol Anat 35(8):647–653
Tillson M (2003) Chapter 70. Spleen. In: Slatter D (ed) Textbook of small animal surgery, 3rd edn. Elsevier Science, Philadelphia, pp 1046–1062
Toutziaris Ch, Kampantais S, Christopoulos P, Papaziogas B, Vakalopoulos I (2013) Compensatory enlargement of an accessory spleen mimicking a retroperitoneal tumor: a case report. Hippokratia 17(2):185–186
Vajda P, Kereskai L, Czauderna P, Schaarschmidt K, Kalman A, Koltai J, Engelis A, Kalman E, Lewicki K, Verebely T, Jainsch M, Petersons A, Pinter AB (2012) Re-evaluation of histological findings of nonparasitic splenic cysts. Eur J Gastroenterol Hepatol 24(3):316–319
Varga I, Gálfiová P, Adamkov M, Danisovic L, Polak S, Kubikova E, Galbavy S (2009) Congenital anomalies of the spleen from an embryological point of view. Med Sci Monit 15(12):RA269–RA276
Vermylen C, Lebecque P, Claus D, Otte JB, Cornu G (1983) The wandering spleen. Eur J Pediatr 140:112–115
Waldman JD, Rosenthal A, Smith AL, Shurin S, Nadas AS (1977) Sepsis and congenital asplenia. J Pediatr 90(4):555–559
Wang W, Li W, Sun Y, Zhao Y, Zhu R, Li J, Zhang H (2016) Intra-gastric ectopic splenic tissue. J Gastrointest Surg 20(1):218–220
William BM, Corazza GR (2007) Hyposplenism: a comprehensive review. Part I: basic concepts and causes. Hematology 12(1):1–13
Yamamoto K, Miwa Y, Abe-Suzuki S, Abe S, Kirimura S, Onishi I, Kitagawa M, Kurata M (2016) Extramedullary hematopoiesis: elucidating the function of the hematopoietic stem cell niche. Mol Med Rep 13(1):587–591
Yildiz AE, Ariyurek MO, Karcaaltincaba M (2013) Splenic anomalies of shape, size, and location: pictorial essay. Sci World J 2013:321810
You JH, Lv GR, Liu XL, He SZ (2014) Reference ranges of fetal spleen biometric parameters and volume assessed by three-dimensional ultrasound and their applicability in spleen malformations. Prenat Diagn 34(12):1189–1197
Acknowledgements
Dedicated to a brilliant Slovak pediatric surgeon and friend, Vladimír Cingel, MD, Ph.D., former head physician of Department of Pediatric Surgery, Comenius University in Bratislava, who passed away suddenly before we could finish this manuscript together.
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Varga, I., Babala, J. & Kachlik, D. Anatomic variations of the spleen: current state of terminology, classification, and embryological background. Surg Radiol Anat 40, 21–29 (2018). https://doi.org/10.1007/s00276-017-1893-0
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DOI: https://doi.org/10.1007/s00276-017-1893-0