Splenic torsion and the prune belly syndrome

Torsion of the spleen is rare, difficult to diagnose, and potentially life-threatening. The simultaneous occurrence of splenic torsion and the prune belly syndrome has been reported only twice in the literature. We report the clinical findings, treatment, and follow-up in a 9-year-old boy with typical features of the prune belly syndrome in whom surgical exploration of the abdomen revealed torsion and infarction of a wandering spleen. The methods used to diagnose and treat splenic torsion are discussed.     

小儿延迟性脾破裂的诊断与治疗

目的 探讨小儿延迟性脾破裂的发生机理、诊断与治疗。方法 总结１６年来治的６例小儿延迟性脾破裂的临床资料,６例均为腹部闭合性损伤,伤后出现脾破裂症状的时间均在４８小时以上,经腹穿、ＣＴ或了超确诊,手术证实。结果 ６例中５例行脾切除术,１例行脾修补术,术后未发生暴发性感染,均痊愈。结论 小儿延迟性脾破裂容易误诊,应掌握其发生机理,提高对本病的认识和警惕性。有价值的影像学检查是提高诊断率、降低病死率的关     

Wandering spleen: a rare cause of abdominal pain

Torsion of a wandering spleen is a rare cause of abdominal pain in children. The most common presentation is acute abdominal pain, although signs and symptoms vary widely. Due to the risk of splenic infarction, rapid and accurate diagnosis is essential. Wandering spleen and splenic torsion can be diagnosed by Doppler ultrasound and CT scan. Treatment options include splenopexy or splenectomy. This case describes a 9-year-old girl with torsion of a wandering spleen, complicated by splenic infarction and managed by splenectomy.     

Disseminated punctate splenic calcifications in a child

In a 7-year-old girl presenting with lower abdominal pain following a fall from a swing, ultrasonography of the spleen showed disseminated echogenic spots in the splenic parenchyma. A toxoplasmosis indirect fluorescent antibody test showed a titer of 1:64, but the child was free of symptoms of toxoplasmosis infection. A repeat ultrasonographic evaluation of the spleen 6 months later showed the calcifications unchanged. Correspondebce to: J. Mayr     

Multimodal approach to diagnosis of hamartoma of the spleen

Splenic hamartoma is a benign, primary neoplasm which usually causes a mass in the left upper quadrant of the abdomen. If radionuclide studies show a space-occupying lesion in the spleen that appears solid on the ultrasonogram, and selective abdominal arteriography reveals a richly vascular splenic tumor, hamartoma of the spleen should be the preoperative diagnosis.The opinions or assertions contained herein are the private views of the authors and are not to be construed as official or as reflecting the views of the Department of the United States Air Force or the Department of Defense     

Splenic surgery in childhood

In recent years efforts have been made to preserve the spleen after childhood injuries because of the increased risk of lethal sepsis following childhood splenectomy. The recent literature reveals that overwhelming postsplenectomy infection (OPSI) occurs with an incidence of 3.2% among splenectomized children, with a mortality of 45%. The incidence of OPSI is highest between birth and 2 years; children whose splenectomy was due to trauma had an incidence of 1.5%. OPSI after splenectomy for other diseases results in a higher incidence (5.6%). The case mortality rates are 17.6% and 55.6%, respectively. Splenic rupture constitutes an indication for total splenectomy, particularly when the rapture involves the splenic hilum (stage IV rupture, Barret et al. [1]). We performed anatomic studies of the splenic arterial supply, which showed that an identifiable superior splenic artery served the upper splenic pole in 60% of cases, offering the possibility that the upper pole might be salvaged even in cases of serious rupture. We report a series of 23 children treated for splenic injury in the last 5 years. Using fibrin-glueing techniques in stage I and II ruptures and segmental or subtotal resection in Stage III and IV ruptures, we successfully preserved functioning splenic tissue in every patient, even in cases of complete rupture of the main splenic artery. Functional preservation was documented by splenic scintigraphy and screening for hematologic signs of hyposplenism. Spleen ultrasonography was also performed as an additional follow-up examination. We observed no cases of OPSI in the above-mentioned series.Since the German system of socialized health care mandates that determination of fitness for work be made after any school-related trauma, an assessment that in the case of splenic injury may have bearing on the child's future ability to serve in the military or work in tropical countries, objective data from scintigraphy was sought in this study as a means of grading these patients for postoperative disabilityIn honour of Professor Dr. Roland Daum, on the occasion of his 60th birthday     

Current management of liver and splenic blunt trauma in children

Thirty-nine children with blunt liver and/or splenic injury were treated in our department from 1979 to 1987; 23 had a splenic injury, 10 a hepatic injury, and the remaining 6 had both. The diagnosis was suggested by the history and physical examination and was confirmed by CT and radioisotope scanning. Every hemodynamically stable patient was initially managed non-operatively. The children who failed to respond to conservative treatment and had unstable vital signs indicating intractable hemorrhage were eventually operated upon. Every effort was made to preserve the liver and spleen during the operation. Liver repair, splenorraphy alone or in combination with splenic artery ligation, and autotransplantation of splenic tissue were widely used procedures. Sixteen patients were successfully managed nonoperatively, but the remaining 23 required surgery. Only 5 children became completely asplenic and no liver lobe resection was performed. It is suggested that conservative management of children with liver and/or splenic injury is both safe and effective. When surgical intervention is inevitable great effort should be made to preserve liver and spleen.Presented at the XII International Congress of Greek Association of Paediatric Surgeons in Rhodes, 1987 Offprint requests to: G. Tryfonas     

Fungal splenic abscesses: management in childhood leukemia

Fungal splenic abscesses are a potentially fatal complication of childhood leukemia, especially during hematologic relapse. Optimal treatment requires splenectomy combined with amphotericin B therapy. Previous reports have suggested that splenectomy should not be performed prior to complete control of the relapse. Three children with relapsed acute lymphocytic leukemia developed fever and splenomegaly, two during the re-induction phase of chemotherapy and the third within 1 month after a second remission. Imaging studies revealed non-homogeneity of the spleen. Uneventful splenectomy was performed for both diagnostic and therapeutic purposes before the return of normal hematopoiesis. Our results indicate that splenectomy for treatment of fungal abscesses can be successfully undertaken before complete hematologic remission is accomplished. Offprint requests to: C. D. Vinocur     

Intrasplenic masses of “preserved” functioning splenic tissue in sickle cell disease: correlation of imaging findings (CT,ultrasound, MRI,and nuclear scintigraphy)

Purpose. We studied six patients with sickle cell disease (SSD), five homozygous for sickle cell anemia and one with sickle betathalassemia, in whom rounded intrasplenic masses proved to be preserved functioning splenic tissue.Materials and methods. Available images including computed tomography, ultrasonography, bone scans (Tc-99m MDP), liver spleen scans (Tc-99m sulfur colloid), and MRI were evaluated.Results. The masses were low density on CT (in an otherwise calcified spleen), hypoechoic relative to the echogenic spleen on US, and had the imaging characteristics of normal spleen on MRI. They failed to accumulate Tc-99m MDP but did demonstrate uptake of Tc-99m sulfur colloid.Conclusion. In a patient with SSD and intrasplenic masses, proper correlation of multiple imaging modalities will establish the diagnosis of functioning splenic tissue and avoid mistaken diagnosis of splenic abscess or infarction.     

Laparoscopic extirpation of splenic hamartoma

We presented a case of splenic hamartoma. A 12-year-old girl has had an abdominal pain since 9 years of age. Contrast-enhanced computed tomography scan at a local hospital revealed heterogeneous enhancement of a mass which is 5 cm in diameter, located near the surface of the spleen, diagnosed as hemangioma. She had an investigation with Levovist-enhanced ultrasonography and superparamagnetic iron oxide-enhanced magnetic resonance imaging on T2-weighted gradient-echo imaging, which showed a hypervascular echoic mass and a decrease of signal intensity. The imaging diagnosis was splenic hamartoma. She underwent laparoscopic splenectomy to prevent the spontaneous rupture. The splenic artery at the pancreas body was exposed, and doubly ligated by vessel clip and furthermore ligated by 3-0 silk. At the splenic hilum, the splenic artery and vein were dissected with the Endo GIA vascular linear cutting stapler. The spleen was captured into the Endocatch II retrieval bag and removed from a transverse lower abdominal (suprapubic) incision extended up 7 cm in length through a port site. In pathological findings, the tumor was compatible with hamartoma. Splenic hamartomas in pediatric patients have been described extremely rare. In clinical symptoms, spontaneously ruptured splenic hamartoma has been rarely reported.     

Splenic torsion in the presence of renal agenesis

A 14-year-old male with left renal agenesis presented with a history of intermittent abdominal pain for over 1 year. At laparotomy, the cause was found to be splenic torsion. The case is presented with a literature review. A theory of the cause is also presented based on the regional anatomy and embryology. Accepted: 2 May 1997     

Torsion of the spleen in children]

The wandering spleen is caused by congenital absence of fixating ligaments or abnormally long ligaments. It is an uncommon clinical entity, which rarely affects children. The clinical presentation of wandering spleen is variable, but the most dangerous complication is splenic torsion. A 7 year-old boy presented with abdominal pain and vomiting. The abdominal ultrasound scan discovered spleen ischemia. Volvulus of the spleen was evoked. Laparotomy was carried out and the patient underwent splenectomy. In this case the anatomical means of spleen fixity were absent. Because wandering spleen is uncommon in the paediatric population, a heightened awareness of the condition is required for accurate diagnosis and appropriate management. The treatment of choice is splenopexy, while if splenic necrosis is present, splenectomy is required.     

Torsion of wandering spleen: The whorled appearance of the splenic pedicle on CT

Congenital deficiency or acquired laxity of the suspensory ligaments of the spleen may result in extreme splenic mobility. This rare condition, which is termed wandering or ectopic spleen predisposes the elongated splenic pedicle to torsion. We present a case of splenic torsion with a new CT finding consisting of a whorled appearance of the splenic pedicle. To our knowledge this finding has not been described before.     

Infantile splenorenopancreatic mucormycosis complicating neuroblastoma

Herein is described the first case of an infant with neuroblastoma who developed isolated splenorenopancreatic mucormycosis. An 18‐month‐old boy with neuroblastoma who was on intensive chemotherapy was admitted with febrile neutropenia. On abdominal computed tomography, multiple hypodense lesions in the spleen with invasion to the upper pole of the left kidney were demonstrated. Enlargement of splenic lesions with a complete hypoechoic pattern replacing the whole spleen, consistent with splenic abscess were observed on serial ultrasound. On splenectomy the resected spleen appeared to be severely fragmented and necrotic. On pathology, massive infiltration of broad, non‐septate hyphal fragments identified as Mucor with invasion to all blood vessels was seen. Histologically, a piece of the tail of the pancreas also showed involvement by the filamentous Mucor. The present case highlights the necessity of high index of suspicion in susceptible patients, early diagnosis and appropriate management in order to minimize the mortality rate.     

<Superscript>99 m</Superscript>Tc-sulphur-colloid and heat-denatured <Superscript>99 m</Superscript>Tc-labelled red cell scans demonstrating a giant intrapelvic spleen in a girl after splenectomy

A 17 × 12 × 5-cm giant intrapelvic mass in a 14-year-old girl is reported. This mass developed 6 years after a splenectomy for splenic torsion. The heat-denatured ^{99 m}Tc-labelled red cell scan and ^{99 m}Tc- sulphur-colloid scan confirmed the specific red cell sequestration function and reticuloendothelial activity in the giant intrapelvic spleen. The size and development of the giant intrapelvic spleen are unusual. The usefulness of functional images to diagnosis the nature of the intrapelvic mass is well demonstrated. Received: 15 November 1999 Revised 14 August 2000 Accepted: 15 October 2000     

Ultrasound assessment of mild splenomegaly: spleen/kidney ratio

Background. Ultrasound may detect mild splenomegaly before it is clinically palpable. Knowledge of the normal range of spleen size in the population being examined is a prerequisite. Racial differences in splenic length could result in incorrect interpretation of splenic measurements. Objective. To measure the normal values of splenic length in Hong Kong Chinese children and compare the results with Western data; to determine whether there is a constant ratio of the length of the spleen to the left kidney, which could be used to diagnose splenomegaly without reference to a nomogram. Materials and methods. Ultrasound was used to measure maximum splenic and left kidney length in 256 Chinese children to obtain normal values for spleen length and to determine the spleen/kidney ratio. Results. Splenic length in Chinese children is similar to Western children up to the age of about 15 years. The spleen/left kidney ratio is strikingly constant with a mean value of 1. Using 2 SD above the mean as a guide, the upper limit of normal for the spleen/kidney ratio is 1.25. Conclusion. Splenomegaly should be suspected in children if the spleen is more than 1.25 times longer than the adjacent kidney. Received: 15 January 1997 Accepted: 23 June 1997     

Isolated splenic hemangioma presenting with bleeding into serous body cavities

We present two children with massive bleeding into the serous body cavities accompanied by intractable consumption coagulopathy. One had a large spleen palpable at admission, the other developed progressive splenomegaly while in hospital. Neither child had any external evidence of angiomatous lesions. A splenic hemangioma was suspected clinically and on abdominal ultrasound; the diagnosis was confirmed at laparotomy. Splenectomy resulted in a prompt cure in both cases.     

Wandering spleen in children: a report of 3 cases and a brief literature review underlining the importance of diagnostic imaging

Background

Wandering spleen is a rare condition in children that is often caused by loss or weakening of the splenic ligaments. Its clinical presentation is variable; 64% of children with wandering spleen have splenic torsion as a complication.

Objective

To provide up-to-date information on the diagnosis, clinical management and diagnostic imaging approaches for wandering spleen in infants and children and to underline the importance of color Doppler US and CT in providing important information for patient management.

Materials and methods

We report a series of three children with wandering spleen treated at our children’s hospital over the last 6 years. All three underwent clinical evaluation, color Doppler US and CT and were surgically treated. We also reviewed 40 articles that included 55 patients younger than 18 years reported in the Medline database from 2002 to 2012.

Results

We correlated pathological data with imaging findings. Color Doppler US, the first imaging modality in investigating abdominal symptoms in children with suspected wandering spleen, yielded a diagnostic sensitivity of 54.9%, whereas CT achieved about 71.7%.

Conclusion

Radiologic evaluation has a major role in confirming the diagnosis of a suspected wandering spleen and avoiding potentially life-threatening complications requiring immediate surgery.     

Regeneration of autotransplanted splenic tissue is not proportional to total weight and size of transplanted fragments

Young minipigs were used as a model for splenic autotransplantation in children. After splenectomy about 7% of the spleen was implanted in the greater omentum either as a single slice or as three small slices. Six months later there was no difference in the amount of regenerated splenic tissue or lymphocyte content between these two groups. The regenerated tissue weighed 3.98±1.9 g, which is not less than after whole spleen transplantation and is only about 6% of the splenic weight of control minipigs. The regenerated splenic tissue contained only about 3% of normal splenic lymphocyte numbers. Based on data from the literature on splenic regeneration in man and on the present experiments in animals, about 10% of the traumatized spleen seems to be sufficient for splenic regeneration after intraomental implantation. Offprint requests to: R. Pabst     

Ectopic spleen. A sonographic diagnosis

The diagnosis of an ectopic spleen can be made sonographically by recognizing the characteristic parenchymal texture and shape of the spleen. Evaluation of the left upper quadrant is necessary to confirm the diagnosis or to diagnose a mass displacing a normal spleen. A rare case of an ectopic spleen in a child with chronic torsion is presented.