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DOI:10.2214/AJR.05.0672
AJR 2007; 188:W328-W330
© American Roentgen Ray Society


Case Report

Wandering Spleen: An Unusual Association with Gastric Volvulus

Huai-Tzu Michael Liu1,2 and Kenneth K. Lau1

1 Department of Radiology, Monash Medical Center, Rm. 116, 246 Clayton Rd., Clayton South, Victoria 3168, Australia.
2 Present address: Department of Radiology, The Canberra Hospital, Yamba Dr., Garran, ACT 2065, Australia.

Received April 19, 2005; accepted after revision June 17, 2005.

 
Address correspondence to H.-T. M. Liu (htliu2003{at}yahoo.com.au).

WEB This is a Web exclusive article.

Keywords: abdomen • abdominal imaging • anatomy • pediatric radiology • spleen


Introduction
Top
Introduction
Case Report
Discussion
References
 
Wandering spleen is a rare condition characterized by the absence or underdevelopment of one or all of the ligaments that hold the spleen in its normal position in the left upper quadrant of the abdomen. Wandering spleen and gastric volvulus share a common cause, the absence of an intraperitoneal visceral ligament. Herein we describe an unusual case of pediatric wandering spleen in association with gastric volvulus and diaphragmatic hernia.


Case Report
Top
Introduction
Case Report
Discussion
References
 
A 44-month-old girl presented to the accident and emergency department with vomiting and dehydration. She had no history of fever, diarrhea, abdominal pain, toxic ingestion, or trauma. On physical examination, she showed signs of 10% dehydration, with dry mucocutaneous membrane and reduced skin turgor. The physical examination also revealed a firm epigastric mass that was non-tender with abdominal palpation.

Abdominal radiography was unremarkable, showing no dilatation of bowel loops. A barium meal examination was performed because of the clinical suspicion of malrotation. It showed a left-sided congenital diaphragmatic hernia and an intrathoracic gastric volvulus (Fig. 1A). Sonography revealed a medial displacement of the spleen immediately inferior to the right lobe of liver (Fig. 1B). Subsequent CT showed the intrathoracic position of the left kidney and stomach and a gastric volvulus in the mesoaxial direction (along the line joining the greater and lesser curvature) (Fig. 1C). It also confirmed the displacement of the spleen to the right side of the abdomen (Fig. 1D).


Figure 1
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Fig. 1A —44-month-old girl with wandering spleen in association with gastric volvulus and diaphragmatic hernia. Barium meal examination shows leftsided congenital diaphragmatic hernia containing intrathoracic gastric volvulus.

 

Figure 2
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Fig. 1B —44-month-old girl with wandering spleen in association with gastric volvulus and diaphragmatic hernia. Sonogram of upper abdomen reveals abnormal position of spleen, which lies inferior to right lobe of liver.

 

Figure 3
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Fig. 1C —44-month-old girl with wandering spleen in association with gastric volvulus and diaphragmatic hernia. Axial CT image of lower chest shows congenital diaphragmatic hernia containing stomach and left kidney.

 

Figure 4
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Fig. 1D —44-month-old girl with wandering spleen in association with gastric volvulus and diaphragmatic hernia. Axial contrast-enhanced CT image confirms displacement of spleen to inferomedial aspect of right lobe of liver.

 
The child underwent an open laparotomy. At surgery, the findings of a large left-sided diaphragmatic hernia with intrathoracic stomach and left kidney were confirmed. A mesoaxial volvulus was seen. The spleen was noted to be completely free-floating because of the lack of ligamentous attachment. Splenopexy and gastropexy were performed. The patient's recovery was uneventful and she was discharged on the fifth postoperative day.


Discussion
Top
Introduction
Case Report
Discussion
References
 
Gastric volvulus is defined as an abnormal degree of rotation of one part of the stomach around another. Gastric volvulus is rare in pediatric patients and is frequently described in association with congenital diaphragmatic hernia and eventration of the diaphragm [1, 2]. Most cases are mesoaxial and occur with a rotation along the long axis of the gastrohepatic omentum. Diagnostic delay can lead to gastric ischemia, perforation, and death [3, 4].

Wandering spleen is by definition a mobile spleen that is attached only by an elongated vascular pedicle, allowing it to migrate to any part of the abdomen or pelvis. It is a rare congenital malformation resulting from abnormal development of the splenic peritoneal attachments [5, 6]. Most cases in children occur when the child is younger than 1 year; the male-to-female ratio is 6:1 [7, 8]. During fetal development, the dorsal mesogastrium fails to fuse with the posterior peritoneum, leading to a laxity or absence of formation of splenic supporting ligaments [5, 6]. These ligaments include the pancreaticocolic, splenocolic, gastrosplenic, pancreaticosplenic, phrenicocolic, splenorenal, and phrenicosplenic ligaments. For this reason, the splenic pedicle has an increased risk of axial torsion that can lead to splenic congestion and ultimately to splenic infarction [7, 8].

The condition also can occur in adults at 20-40 years because of laxity of the ligamentous support as a result of splenomegaly or pregnancy [9]. Fifteen percent of children with wandering spleen are asymptomatic, whereas 55% present with abdominal pain and 90% present with a palpable mass outside the left upper quadrant [10]. Torsion complicates 64% of pediatric wandering spleens [11]. Splenic torsion is usually clockwise and can cause vascular congestion, infarction, and even gangrene of the spleen [12, 13].

Several imaging studies may be helpful in establishing the diagnosis of wandering spleen, and debate still exists concerning which test is most appropriate. Abdominal radiography may reveal the absence of a splenic silhouette [4]. Abdominal sonography is useful in establishing the absence or presence of a spleen in the left upper quadrant, but it may be obscured by bowel gas [13]. Also, a duplex study can be performed to assess splenic blood flow and is useful in establishing whether splenic torsion is present [13]. CT shows an abnormal location of the spleen or abnormality of surrounding viscera, such as gastric malposition or volvulus [1, 2, 5]. Other findings include necrosis and involvement of the pancreatic tail and surrounding fat due to torsed splenic vessels [2-4].

Splenopexy is the procedure of choice to prevent future torsion while preserving functional splenic tissue when a viable wandering spleen is found at surgery [10-12].

There is a rare association between gastric volvulus and wandering spleen [2, 5]. The two entities share a common cause, the absence or laxity of intraperitoneal visceral ligaments. Prophylactic gastropexy in patients with wandering spleen may therefore be implicated [5].

Our patient also had a coincidental left diaphragmatic hernia.

In conclusion, it is important to recognize a wandering spleen because of the configuration of its vascular pedicle, which makes it prone to splenic torsion. Wandering spleen has a rare association with gastric volvulus. Both splenic torsion and gastric volvulus are potentially life-threatening if not immediately managed surgically.


References
Top
Introduction
Case Report
Discussion
References
 

  1. Munoz JJ, Mansul AJ, Malpas TJ, et al. Intrathoracic gastric volvulus mimicking pyloric stenosis. J Pediatr Child Health 2003; 39:149 -150[CrossRef]
  2. Pelizzo G, Lembo MA, Franchella A, et al. Gastric volvulus associated with congenital diaphragmatic hernia, wandering spleen, and intrathoracic left kidney: CT findings. Abdom Imaging2001; 10:306 -308
  3. Miller DL, Pasquale MD, Seneca RP, et al. Gastric volvulus in the pediatric population. Arch Surg 1991;126 : 1146-1149[Abstract/Free Full Text]
  4. Mayo A, Erez I, Lazar L, et al. Volvulus of the stomach in childhood: the spectrum of the disease. Pediatr Emerg Care 2001; 17:344 -348[CrossRef][Medline]
  5. Spector JM, Chappell J. Gastric volvulus associated with wandering spleen in a child. J Pediatr Surg 2000;35 : 641-642[CrossRef][Medline]
  6. Romero J, Barksdale EM. Wandering spleen: a rare cause of abdominal pain. Pediatr Care 2003;19 : 412-414
  7. Zimmermann ME, Cohen RC. Wandering spleen presenting as an asymptomatic mass. Aust N Z J Surg 2000;70 : 904-906[CrossRef][Medline]
  8. Horwitz JR, Black CT. Traumatic rupture of a wandering spleen in a child: case report and literature review. J Trauma1996; 41:348 -350[Medline]
  9. Taori K, Ghonge N, Prakash A. Wandering spleen with torsion of vascular pedicle: early diagnosis with multiplanar reformation technique of multislice spiral CT. Emerg Radiol 2004;29 : 479-481; Epub 2004 Mar 18
  10. Rodkey ML, Macknin ML. Pediatric wandering spleen: case report and review of literature. Clin Pediatr 1992;31 : 289-294[Abstract/Free Full Text]
  11. Thompson JS, Ross RJ, Pizzaro ST. The wandering spleen in infancy and childhood. Clin Pediatr 1980;19 : 221-224[Abstract/Free Full Text]
  12. Keith BA, Gibbs A. Pediatric wandering spleen: the case for splenopexy—review of 35 reported cases in the literature. J Pediatr Surg 1989; 24:432 -435[CrossRef][Medline]
  13. Nemcek AA, Miller FH, Fitzgerald SW. Acute torsion of a wandering spleen: diagnosis by CT and duplex Doppler and color-flow sonography. AJR 1991; 157:307 -309[Free Full Text]

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R. K. Cribbs, K. W. Gow, and M. L. Wulkan
Gastric Volvulus in Infants and Children
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[Abstract] [Full Text] [PDF]


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