Wandering spleen with splenic torsion in a toddler

Abstract

Rationale: Wandering spleen (WS) is a rare clinical entity characterized by splenic hypermobility caused by absent or abnormal laxity of the suspensory ligaments, which fix the spleen in its normal position. Due to abnormal attachment, the spleen is predisposed to torsion and a series of complications. Pediatric WS is mostly reported in children aged <10 years, especially among infants aged <1 year; it is uncommon among toddlers between 1 and 3 years. To the authors’ knowledge, only seven cases of WS have been described previously. Herein, we present the case of a 3-year-old toddler with WS and splenic torsion. Patient concerns: A 3-year-old boy was presented to the pediatric emergency room with a 2-day history of abdominal pain and vomiting. The ultrasonographic examination revealed a mass in the left upper abdomen cavity and absence of spleen in its normal position. Computed tomography showed an enlarged displaced spleen occupying the left abdomen cavity with an elongated splenic vascular pedicle (whirl sign), suggesting splenic torsion. Diagnoses: The patient was diagnosed that had WS and splenomegaly, with or without complications due to splenic torsion. Interventions: The patient underwent emergency laparotomy and splenectomy due to nonviability after detorsion. Outcomes: The postoperative course was uneventful, and the patient was discharged on the 7th day postoperatively without complications. The patient had favorable outcome over a 1-year follow-up. Lessons: Herein, we reported the case of a toddler with WS with splenic torsion. Moreover, after reviewing relevant studies in literature, we presented our findings on the diagnosis and treatment of toddlers with WS. Toddlers with WS are characterized by acute abdominal pain, unclear history description, examination restrictions, and high rates of life-threatening complications. High level of suspicion, careful physical examination, detailed history collection, and objective investigation are crucial in the management of toddlers with WS.

Wandering splenomegaly reduction after splenopexy

“…Nevertheless, more than 700 papers have been published describing this splenic disorder. This abnormality is found more commonly in women of the reproductive age group, however, in pediatrics, this condition is more detected in boys [1] , [10] , [12] , [16] . The diagnosis is difficult due to nonspecific clinical or laboratory findings, except for occasional pancytopenia, and is defined only by imaging exams [1] , [2] , [7] , [11] , [12] , [15] .…”

“…In this hypermobility the spleen is attached to the hilum only by a long vascular pedicle and the organ “wanders” in the lower abdomen or in the pelvis [1] , [2] , [3] . This condition is predisposed to complications, including the torsion of the splenic pedicle and partial or complete infarction of the spleen associated with splenic vein thrombosis [4] , [5] , [6] , [7] , [8] , [9] , [10] . The WS may be mistaken for an unidentified abdominal mass [1] , [2] , [6] , [10] , [11] , [12] .…”

“…This condition is predisposed to complications, including the torsion of the splenic pedicle and partial or complete infarction of the spleen associated with splenic vein thrombosis [4] , [5] , [6] , [7] , [8] , [9] , [10] . The WS may be mistaken for an unidentified abdominal mass [1] , [2] , [6] , [10] , [11] , [12] . Its long pedicle includes the splenic artery and vein as well as lymphatic ducts and autonomous nerves surrounded by connective tissue.…”

“…As a result, computed tomography (CT) is the best imaging tool to make this diagnosis, although ultrasound may be used as well. The acute presentation with sudden onset of sharp abdominal pain is usually due to torsion of the splenic pedicle [2] , [4] , [5] , [6] , [7] , [8] , [9] , [10] , [11] , [12] , [13] .…”

“…In such circumstances tissue implants from the removed spleen on the greater omentum, to preserve at least part of the splenic functions, should be considered [14] . In the case of WS with no complications, the first-line treatment is elective laparoscopic splenopexy, performed in different techniques, including or not the use of a mesh [1] , [6] , [9] , [10] , [15] , [16] . Elective splenectomy for complicated WS is recommended when the spleen parenchyma is too damaged to recover from several ischemic events, or for the spleen dimension [7] , [11] , [12] .…”