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Abdominal pain and wandering spleen in young children: the importance of an early diagnosis
Journal of Pediatric Surgery (2009) 44, 1446–1449
www.elsevier.com/locate/jpedsurg
Abdominal pain and wandering spleen in young children: the importance of an early diagnosis Ida Di Crosta a,⁎, Alessandro Inserra b , Carlos Pueyo Gil a , Mara Pisani c , Antonio Ponticelli b Department of Pediatric Surgery, University Hospital “Joan XXIII”, 43007, Tarragona, Spain Pediatric General and Thoracic Surgery Unit, Ospedale Bambino Gesù, IRCCS, P.za, S. Onofrio 4, 00165 Roma, Italy c Emergency Department (DEA), Area Rossa, Ospedale Bambino Gesù, IRCCS, P.za, S. Onofrio 4, 00165 Roma, Italy a
b
Received 23 October 2008; revised 27 January 2009; accepted 28 February 2009
Key words: Wandering spleen; Abdominal pain; Spleen torsion; Power Doppler ultrasonography; Splenectomy; Splenopexy
Abstract Purpose: The aim of the study is to increase clinical awareness of torsion of wandering spleen (WS) in childhood and the need of a rapid diagnosis. Methods: Four cases operated for torsion of WS are retrospectively reviewed. Ages at presentation were, respectively, 30 months, 5 years, 4 years, and 3 years, without sex preference. All subjects led a history of abdominal pain and a mass on physical examination. Results: Torsion of WS should be suspected in any child presenting with acute abdomen. Moreover, in case of acute abdomen and intermittent abdominal pain, we suggest studying spleen position with ultrasound. Conclusions: Ultrasonography with color Doppler is the best choice for diagnosis of torsion of WS. Computed tomography is a good complementary examination, but it needs to submit young patients to a general anesthesia and delays an emergency situation. © 2009 Elsevier Inc. All rights reserved.
Acute splenic torsion of wandering spleen (WS) is a potentially fatal surgical emergency case, and its correct and early identification continues to represent a challenge [1], especially in children. Causes for spleen hypermobility may be hormonal changes during pregnancy or failure of fusion of the dorsal peritoneum [2]. Congenital absence of splenocolic, splenorenal, or splenophrenic ligaments, which points to the failure of the dorsal mesogastrium to fuse with the posterior abdominal ⁎ Corresponding author. Tel.: +34 636406188. E-mail address: [email protected] (I. Di Crosta). 0022-3468/$ – see front matter © 2009 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2009.02.062
wall during the second month of embryogenesis, is the best explanation for the pathophysiology of WS. This condition may occur in people of all ages, with a male-to-female ratio of 2.5:1 in the first year of life, which converts to 1:1 in the first 10 years of life [3,4]. IgA deficiency has been cited as a risk factor for the development of splenic laxity [5].
1. Material and methods Between 1998 and 2006, in our hospital, 4 cases of WS were diagnosed (Table 1).
Abnormal pain and wondering spleen in young children
1447
Table 1
Results of the operations in our 4 patients
Patient
Age/sex
Sign/symptom
Duration of symptoms
Eco color Doppler
CT scan
Surgical treatment
1 2 3 4
30 mo/female 5 y/female 4 y/male 3 y/male
Abdominal Abdominal Abdominal Abdominal
3d 3 wk 2d 2 wk
Diagnostic Diagnostic Diagnostic No diagnostic
Diagnostic Diagnostic Diagnostic No diagnostic
Splenectomy Splenectomy Splenopexy Splenectomy
pain pain + vomiting + mass pain + vomiting pain + fever + mass
1.1. Case 1 A 30-month-old female infant presented to the emergency department with a 3-day history of abdominal pain. Physical examination showed a tense abdomen with generalized pain. White blood cell count was 18.82 × 103/μL. Plain abdominal x-ray showed dilated large bowel loops, without signs of intestinal obstruction. Ultrasound demonstrated enlarged spleen below its normal position, altered core structure, perisplenic fluid, and free fluid in the peritoneal cavity. Doppler ultrasound showed absent vascularity in the splenic artery (Fig. 1). Computed tomographic (CT) scan confirmed the diagnosis of WS. At laparotomy, a 360° splenic torsion was found (Fig. 2). After detorsion, it did not regain its normal vascularity, and splenectomy was carried out. Histopathologic examination demonstrated congestion and fibrosis of the spleen.
Laboratory results were as follows: hemoglobin level, 10.4 g/dL, and platelets, 1094 × 103/μL. The patient underwent a laparotomy, and a 360° splenic torsion was found with adhesion bands to the abdominal wall and the ileum. Adhesiolysis and splenectomy were performed. Histopathologic examination demonstrated hemorrhagic necrosis of the spleen.
1.3. Case 3 A 4-year-old boy presented after 2 days of abdominal pain and vomiting suggesting intestinal obstruction. Medical history was unremarkable. Physical examination revealed a tense abdomen. Laboratory results were all within the normal range except for an increase in the white blood cell count (21.49 × 103/μL).
1.2. Case 2 A 5-year-old girl was admitted to the pediatric department for abdominal pain and a 3-week history of vomiting; the medical history was positive for respiratory distress and patent ductus arteriosus. Physical examination of the abdomen revealed a midabdominal mass. Ultrasound demonstrated enlarged spleen in abnormal position and free fluid in the peritoneal cavity. The CT scan confirmed a WS, without contrast enhancement, indicating partial or total infarction.
Fig. 1 Total absence of flow within the splenic parenchyma and hilum of the first case.
Fig. 2 An abnormal long torsed splenic vascular pedicle of the first case.
1448 Abdominal x-ray showed multiple air-fluid levels. Ultrasound revealed an anomalous position of the spleen, which was also increased in size. The CT scan showed an enlarged spleen compressing surrounding bowel loops. The patient underwent a laparotomy, and a 360° splenic torsion was found. After detorsion, the spleen regained its normal appearance, and hence, splenopexy was performed with a retroperitoneal pouch. On the ninth postoperative day, an incidental ileal intussusception led the child to be submitted to a relaparotomy, during which the spleen was checked in its new pouch and found well vascularized.
1.4. Case 4 A 3-year-old boy was transferred from an outlying hospital with a diagnosis of appendicitis. He presented a 2-week history of abdominal pain; the pain had become worse within the past 24 hours and was accompanied by fever (38.9°C). The physical examination revealed an abdominal mass. White blood cell count was 27.7 × 103/ μL, hemoglobin level was 10.2 g/dL, and platelet count was 744 × 103/μL. Ultrasound showed a solid mass of about 9 × 7 × 4.5 cm, independent of the other organs. The left hypochondrium was not explored because of meteorism. The CT scan confirmed a mesogastric mass and reported no alterations of spleen volume and position. This radiologic mistake brought the child entering the operating room for a mass biopsy. The patient underwent a laparotomy that showed a hard mass torsed at 360° around its pedicle, with important adhesion bands to the ileum and stomach. The surgical exploration did not find the spleen. The histopathologic examination confirmed the postoperative diagnosis of splenic hemorrhagic necrosis.
2. Discussion The absence or abnormal laxity of splenic attachments because of either acquired or congenital causes [1,6,7], combined with an abnormally long and mobile vascular pedicle, predisposes the WS to a number of complications with torsion [7]. Clinical presentation of a WS can be variable. Affected patients can be asymptomatic, and the condition can be discovered incidentally as an abdominal mass on clinical examination [1] or on imaging done for other unrelated reasons [8]. Acute torsion presents as acute abdomen and causes vascular congestion, infarction, and even gangrene [7]. The most common presentation in children is acute abdominal pain [3], but chronic intermittent torsion causes venous congestion and splenomegaly and presents with intermittent abdominal pain [7].
I. Di Crosta et al. Other clinical symptoms include nausea, vomiting, fever, leukocytosis, signs of peritoneal irritation, and a palpable abdominal or pelvic mass [8]. In our review of patients younger than 6 years, the most common presenting complaint was acute abdominal pain, as previously reported. Complications of acute splenic torsion include gangrene, abscess formation, local peritonitis, intestinal obstruction, variceal hemorrhage, and necrosis of the pancreatic tail [4,5,8]. The literature suggests that Doppler ultrasound should be obtained to check the spleen's position and parenchymal flow. Some authors describe CT scan as the best choice to identify this rare condition, although Buckley et al [9] suggests that magnetic resonance imaging may confer certain advantages because of its lack of ionizing radiation, as well as its superior tissue characterization. Angiography can provide evidence of splenic torsion but is invasive and no longer indicated [4]. Computed tomography and magnetic resonance imaging, in young patients, have the disadvantage of having to submit a child, who is in critical conditions, to general anesthesia, and CT is associated with greater radiation exposure. In our experience, CT scan did not improve our knowledge about diagnosis. Detorsion and splenopexy may be considered a reasonable surgical option even in these patients, when there is no evidence of infarction and thrombosis [3,10,11]. Takayasu [6] reported a case of autotransplantation, in which parts of the splenic tissue are transplanted into pouches created in the omentum.
3. Conclusion In our series of 4 cases, CT scan confirmed the diagnosis obtained through color Doppler sonography in 3 cases; in the other case, CT scan did not add any diagnostic information but emphasized our mistake and delayed the time of surgery. Color Doppler sonography in acute and intermittent abdominal pain should always include spleen visualization. If the spleen cannot be found in its anatomical site, torsion of a WS must be considered in the differential diagnosis. Actually, we believe color Doppler sonography is enough for a fast diagnosis of WS. An early recognition of the vascular flow condition is important for a favorable surgical outcome. Splenopexy must be performed each time the organ regains good vascularity after its detorsion.
References [1] Ben Ely A, Zissin R, Copel L, et al. The wandering spleen: CT findings and possible pitfalls in diagnosis. Clin Radiol 2006;61(11):954-8. [2] Taori K, Ghonge N, Prakash A. Wandering spleen with torsion of vascular pedicle: early diagnosis with multiplaner reformation technique of multislice spiral CT. Abdom Imaging 2004;29(4):479-81.
Abnormal pain and wondering spleen in young children [3] Soleimani M, Mehrabi A, Kashfi A, et al. Surgical treatment of patients with wandering spleen: report of six cases with a review of the literature. Surg Today 2007;37(3):261-9. [4] Danaci M, Belet U, Yalin T, Polat V, et al. Power Doppler sonographic diagnosis of torsion in a wandering spleen. J Clin Ultrasound 2000; 28(5):246-8. [5] Lopez-Tomassetti Fernandez EM, Arteaga Gonzalez I, Martin Malagon A, et al. An unusual case of hemoperitoneum owing to acute splenic torsion in a child with immunoglobulin deficiency. J Postgrad Med 2006;52(1):41-2. [6] Takayasu H, Ishimaru Y, Tahara K, et al. Splenic autotransplantation for a congested and enlarged wandering spleen with torsion: report of a case. Surg Today 2006;36(12):1094-7.
1449 [7] Taori K, Sanyal R, Deshmukh A, et al. Pseudocyst formation: a rare complication of wandering spleen. Br J Radiol 2005;78(935): 1050-2. [8] Sodhi KS, Saggar K, Sood BP, et al. Torsion of a wandering spleen: acute abdominal presentation. J Emerg Med 2003;25(2):133-7. [9] Buckley O, Ward EV, Doody O, et al. MRI of the wandering spleen. Clin Radiol 2007;62(5):504. [10] Fukuzawa H, Urushihara N, Ogura K, et al. Laparoscopic splenopexy for wandering spleen: extraperitoneal pocket splenopexy. Pediatr Surg Int 2006;22(11):931-4. [11] Martinez-Ferro M, Elmo G, Laje P. Laparoscopic pocket splenopexy for wandering spleen: a case report. J Pediatr Surg 2005;40(5): 882-4.
www.elsevier.com/locate/jpedsurg
Abdominal pain and wandering spleen in young children: the importance of an early diagnosis Ida Di Crosta a,⁎, Alessandro Inserra b , Carlos Pueyo Gil a , Mara Pisani c , Antonio Ponticelli b Department of Pediatric Surgery, University Hospital “Joan XXIII”, 43007, Tarragona, Spain Pediatric General and Thoracic Surgery Unit, Ospedale Bambino Gesù, IRCCS, P.za, S. Onofrio 4, 00165 Roma, Italy c Emergency Department (DEA), Area Rossa, Ospedale Bambino Gesù, IRCCS, P.za, S. Onofrio 4, 00165 Roma, Italy a
b
Received 23 October 2008; revised 27 January 2009; accepted 28 February 2009
Key words: Wandering spleen; Abdominal pain; Spleen torsion; Power Doppler ultrasonography; Splenectomy; Splenopexy
Abstract Purpose: The aim of the study is to increase clinical awareness of torsion of wandering spleen (WS) in childhood and the need of a rapid diagnosis. Methods: Four cases operated for torsion of WS are retrospectively reviewed. Ages at presentation were, respectively, 30 months, 5 years, 4 years, and 3 years, without sex preference. All subjects led a history of abdominal pain and a mass on physical examination. Results: Torsion of WS should be suspected in any child presenting with acute abdomen. Moreover, in case of acute abdomen and intermittent abdominal pain, we suggest studying spleen position with ultrasound. Conclusions: Ultrasonography with color Doppler is the best choice for diagnosis of torsion of WS. Computed tomography is a good complementary examination, but it needs to submit young patients to a general anesthesia and delays an emergency situation. © 2009 Elsevier Inc. All rights reserved.
Acute splenic torsion of wandering spleen (WS) is a potentially fatal surgical emergency case, and its correct and early identification continues to represent a challenge [1], especially in children. Causes for spleen hypermobility may be hormonal changes during pregnancy or failure of fusion of the dorsal peritoneum [2]. Congenital absence of splenocolic, splenorenal, or splenophrenic ligaments, which points to the failure of the dorsal mesogastrium to fuse with the posterior abdominal ⁎ Corresponding author. Tel.: +34 636406188. E-mail address: [email protected] (I. Di Crosta). 0022-3468/$ – see front matter © 2009 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2009.02.062
wall during the second month of embryogenesis, is the best explanation for the pathophysiology of WS. This condition may occur in people of all ages, with a male-to-female ratio of 2.5:1 in the first year of life, which converts to 1:1 in the first 10 years of life [3,4]. IgA deficiency has been cited as a risk factor for the development of splenic laxity [5].
1. Material and methods Between 1998 and 2006, in our hospital, 4 cases of WS were diagnosed (Table 1).
Abnormal pain and wondering spleen in young children
1447
Table 1
Results of the operations in our 4 patients
Patient
Age/sex
Sign/symptom
Duration of symptoms
Eco color Doppler
CT scan
Surgical treatment
1 2 3 4
30 mo/female 5 y/female 4 y/male 3 y/male
Abdominal Abdominal Abdominal Abdominal
3d 3 wk 2d 2 wk
Diagnostic Diagnostic Diagnostic No diagnostic
Diagnostic Diagnostic Diagnostic No diagnostic
Splenectomy Splenectomy Splenopexy Splenectomy
pain pain + vomiting + mass pain + vomiting pain + fever + mass
1.1. Case 1 A 30-month-old female infant presented to the emergency department with a 3-day history of abdominal pain. Physical examination showed a tense abdomen with generalized pain. White blood cell count was 18.82 × 103/μL. Plain abdominal x-ray showed dilated large bowel loops, without signs of intestinal obstruction. Ultrasound demonstrated enlarged spleen below its normal position, altered core structure, perisplenic fluid, and free fluid in the peritoneal cavity. Doppler ultrasound showed absent vascularity in the splenic artery (Fig. 1). Computed tomographic (CT) scan confirmed the diagnosis of WS. At laparotomy, a 360° splenic torsion was found (Fig. 2). After detorsion, it did not regain its normal vascularity, and splenectomy was carried out. Histopathologic examination demonstrated congestion and fibrosis of the spleen.
Laboratory results were as follows: hemoglobin level, 10.4 g/dL, and platelets, 1094 × 103/μL. The patient underwent a laparotomy, and a 360° splenic torsion was found with adhesion bands to the abdominal wall and the ileum. Adhesiolysis and splenectomy were performed. Histopathologic examination demonstrated hemorrhagic necrosis of the spleen.
1.3. Case 3 A 4-year-old boy presented after 2 days of abdominal pain and vomiting suggesting intestinal obstruction. Medical history was unremarkable. Physical examination revealed a tense abdomen. Laboratory results were all within the normal range except for an increase in the white blood cell count (21.49 × 103/μL).
1.2. Case 2 A 5-year-old girl was admitted to the pediatric department for abdominal pain and a 3-week history of vomiting; the medical history was positive for respiratory distress and patent ductus arteriosus. Physical examination of the abdomen revealed a midabdominal mass. Ultrasound demonstrated enlarged spleen in abnormal position and free fluid in the peritoneal cavity. The CT scan confirmed a WS, without contrast enhancement, indicating partial or total infarction.
Fig. 1 Total absence of flow within the splenic parenchyma and hilum of the first case.
Fig. 2 An abnormal long torsed splenic vascular pedicle of the first case.
1448 Abdominal x-ray showed multiple air-fluid levels. Ultrasound revealed an anomalous position of the spleen, which was also increased in size. The CT scan showed an enlarged spleen compressing surrounding bowel loops. The patient underwent a laparotomy, and a 360° splenic torsion was found. After detorsion, the spleen regained its normal appearance, and hence, splenopexy was performed with a retroperitoneal pouch. On the ninth postoperative day, an incidental ileal intussusception led the child to be submitted to a relaparotomy, during which the spleen was checked in its new pouch and found well vascularized.
1.4. Case 4 A 3-year-old boy was transferred from an outlying hospital with a diagnosis of appendicitis. He presented a 2-week history of abdominal pain; the pain had become worse within the past 24 hours and was accompanied by fever (38.9°C). The physical examination revealed an abdominal mass. White blood cell count was 27.7 × 103/ μL, hemoglobin level was 10.2 g/dL, and platelet count was 744 × 103/μL. Ultrasound showed a solid mass of about 9 × 7 × 4.5 cm, independent of the other organs. The left hypochondrium was not explored because of meteorism. The CT scan confirmed a mesogastric mass and reported no alterations of spleen volume and position. This radiologic mistake brought the child entering the operating room for a mass biopsy. The patient underwent a laparotomy that showed a hard mass torsed at 360° around its pedicle, with important adhesion bands to the ileum and stomach. The surgical exploration did not find the spleen. The histopathologic examination confirmed the postoperative diagnosis of splenic hemorrhagic necrosis.
2. Discussion The absence or abnormal laxity of splenic attachments because of either acquired or congenital causes [1,6,7], combined with an abnormally long and mobile vascular pedicle, predisposes the WS to a number of complications with torsion [7]. Clinical presentation of a WS can be variable. Affected patients can be asymptomatic, and the condition can be discovered incidentally as an abdominal mass on clinical examination [1] or on imaging done for other unrelated reasons [8]. Acute torsion presents as acute abdomen and causes vascular congestion, infarction, and even gangrene [7]. The most common presentation in children is acute abdominal pain [3], but chronic intermittent torsion causes venous congestion and splenomegaly and presents with intermittent abdominal pain [7].
I. Di Crosta et al. Other clinical symptoms include nausea, vomiting, fever, leukocytosis, signs of peritoneal irritation, and a palpable abdominal or pelvic mass [8]. In our review of patients younger than 6 years, the most common presenting complaint was acute abdominal pain, as previously reported. Complications of acute splenic torsion include gangrene, abscess formation, local peritonitis, intestinal obstruction, variceal hemorrhage, and necrosis of the pancreatic tail [4,5,8]. The literature suggests that Doppler ultrasound should be obtained to check the spleen's position and parenchymal flow. Some authors describe CT scan as the best choice to identify this rare condition, although Buckley et al [9] suggests that magnetic resonance imaging may confer certain advantages because of its lack of ionizing radiation, as well as its superior tissue characterization. Angiography can provide evidence of splenic torsion but is invasive and no longer indicated [4]. Computed tomography and magnetic resonance imaging, in young patients, have the disadvantage of having to submit a child, who is in critical conditions, to general anesthesia, and CT is associated with greater radiation exposure. In our experience, CT scan did not improve our knowledge about diagnosis. Detorsion and splenopexy may be considered a reasonable surgical option even in these patients, when there is no evidence of infarction and thrombosis [3,10,11]. Takayasu [6] reported a case of autotransplantation, in which parts of the splenic tissue are transplanted into pouches created in the omentum.
3. Conclusion In our series of 4 cases, CT scan confirmed the diagnosis obtained through color Doppler sonography in 3 cases; in the other case, CT scan did not add any diagnostic information but emphasized our mistake and delayed the time of surgery. Color Doppler sonography in acute and intermittent abdominal pain should always include spleen visualization. If the spleen cannot be found in its anatomical site, torsion of a WS must be considered in the differential diagnosis. Actually, we believe color Doppler sonography is enough for a fast diagnosis of WS. An early recognition of the vascular flow condition is important for a favorable surgical outcome. Splenopexy must be performed each time the organ regains good vascularity after its detorsion.
References [1] Ben Ely A, Zissin R, Copel L, et al. The wandering spleen: CT findings and possible pitfalls in diagnosis. Clin Radiol 2006;61(11):954-8. [2] Taori K, Ghonge N, Prakash A. Wandering spleen with torsion of vascular pedicle: early diagnosis with multiplaner reformation technique of multislice spiral CT. Abdom Imaging 2004;29(4):479-81.
Abnormal pain and wondering spleen in young children [3] Soleimani M, Mehrabi A, Kashfi A, et al. Surgical treatment of patients with wandering spleen: report of six cases with a review of the literature. Surg Today 2007;37(3):261-9. [4] Danaci M, Belet U, Yalin T, Polat V, et al. Power Doppler sonographic diagnosis of torsion in a wandering spleen. J Clin Ultrasound 2000; 28(5):246-8. [5] Lopez-Tomassetti Fernandez EM, Arteaga Gonzalez I, Martin Malagon A, et al. An unusual case of hemoperitoneum owing to acute splenic torsion in a child with immunoglobulin deficiency. J Postgrad Med 2006;52(1):41-2. [6] Takayasu H, Ishimaru Y, Tahara K, et al. Splenic autotransplantation for a congested and enlarged wandering spleen with torsion: report of a case. Surg Today 2006;36(12):1094-7.
1449 [7] Taori K, Sanyal R, Deshmukh A, et al. Pseudocyst formation: a rare complication of wandering spleen. Br J Radiol 2005;78(935): 1050-2. [8] Sodhi KS, Saggar K, Sood BP, et al. Torsion of a wandering spleen: acute abdominal presentation. J Emerg Med 2003;25(2):133-7. [9] Buckley O, Ward EV, Doody O, et al. MRI of the wandering spleen. Clin Radiol 2007;62(5):504. [10] Fukuzawa H, Urushihara N, Ogura K, et al. Laparoscopic splenopexy for wandering spleen: extraperitoneal pocket splenopexy. Pediatr Surg Int 2006;22(11):931-4. [11] Martinez-Ferro M, Elmo G, Laje P. Laparoscopic pocket splenopexy for wandering spleen: a case report. J Pediatr Surg 2005;40(5): 882-4.