Abstract
Introduction
Congenital splenic cysts are a rare cause of abdominal cystic masses in the fetus. We describe nine cases of this condition, the largest reported experience to date. A review of the literature for similar cases was also performed.
Methods
Cases of congenital splenic cyst were collected from three prenatal diagnostic centers and from a dedicated website of prenatal diagnosis. Information regarding clinical and sonographic findings was obtained by reviewing ultrasound reports and medical records. An electronic search of the Pubmed/MEDLINE database for similar cases, with subsequent manual cross-referencing, was performed and the relevant information from the articles was retrieved.
Results
A total of nine cases were added to the currently existing literature of 32 cases. In all but one of our cases, the prenatal detection of the splenic cyst was made in the third trimester, with a median gestational age at diagnosis of 30 weeks (range 22–37). The splenic cyst was confirmed after delivery in all but one case, which was lost to follow up. Postnatal sonographic examinations performed in the remaining eight cases showed that the cyst increased in size in two (25%), was stable in size in one (12%), decreased in size in one (12%), and completely regressed in the other four cases (50%). No complications associated with the cysts were reported in the infants.
Conclusions
Congenital splenic cyst appears to be a benign condition with no known risk of perinatal complications. Nevertheless, differential diagnosis with other fetal cystic masses of the left upper abdominal quadrant and postnatal sonographic follow up to monitor the size of the cyst are important issues to be considered in the perinatal management of these cases.
Introduction
Prenatal sonographic detection of an abdominal cystic mass poses clinically significant diagnostic and management challenges. 1 – 3 Fetal abdominal cysts are mainly related to anomalies of the gastrointestinal or urogenital tracts and their differential diagnosis and prognosis depend on their sonographic characteristics, location, and subsequent antenatal course. 1 – 4 Congenital splenic cysts are among the less frequent causes of abdominal cystic masses in the fetus. In this report, we describe a series of nine cases diagnosed prenatally by ultrasound and discuss the presenting sonographic features, antenatal and neonatal management, perinatal implications, and outcome. A review of the literature for similar cases is also presented.
Material and methods
This was a retrospective review of cases of congenital splenic cyst detected prenatally by ultrasound. Cases were collected by reviewing the ultrasound databases from three Fetal Medicine centers in Chile and Argentina over a 15-year study period from 2002 to 2016. In addition, cases summarized on an electronic website for prenatal diagnosis (www.thefetus.net) were also identified and the authors contacted and invited to collaborate with this series.
Prenatal diagnosis of a congenital splenic cyst was based on the sonographic detection of a round or oval anechoic structure with smooth walls located in the left upper abdominal quadrant posterior to the stomach (Figure 1). In addition, color Doppler ultrasound was used to rule out the presence of blood flow signals within the lesion (Figure 2). Although in some cases it was difficult to demonstrate that the cyst was located within the spleen at the initial examination, thorough examination upon referral revealed that the other abdominal organs including the stomach, liver, left adrenal gland, and left kidney appeared normal and were clearly separated from the cystic mass, making the diagnosis of splenic cyst straightforward. Information on maternal demographics, sonographic features, subsequent antenatal management, and perinatal outcome was obtained by reviewing the ultrasound reports and images, medical records, and, when required, by contacting the referring obstetrician or the patient herself as part of our standard clinical care. Due to the retrospective nature of this research, permission of the Institutional Review Board was waived.
Transverse sonographic view of the fetal left upper abdomen at 35 weeks shows a single cystic mass (c) measuring 22 mm within the spleen (Sp). Color-Doppler ultrasound at 28 weeks in a fetus with a 7-mm splenic cyst (between calipers) shows no blood flow signals within the mass.

An extensive review of the literature was also performed through a computerized search of the Pubmed/MEDLINE database combining the terms “splenic cyst” and “spleen cyst” with “fetal”, “fetus”, “prenatal diagnosis”, and “antenatal diagnosis” without limits of time and language. Search results were also cross-referenced to identify additional articles.
Results
Prenatal detection of fetal splenic cyst: Clinical cases
MA: maternal age; GA: gestational age; NA: information not available.
In eight of the nine cases, only a single cyst was present, which measured between 7 and 22 mm in maximum diameter at the time of presentation. Only one of these cases had a prenatal follow-up scan, at which time the cyst was seen to minimally increase in size from 8 mm to 10 mm. In the remaining case, two distinct cystic masses measuring 12 mm and 10 mm in diameter were visualized close to each other within the fetal spleen. Both cysts were noted to increase in size on subsequent scans and at the neonatal abdominal scan (Figure 3). In one fetus with a single cyst, a septation within the cyst was identified (Figure 4); in all the other cases the content was homogeneously anechoic. Associated anomalies were noted in only one case in which an ipsilateral megaureter was identified; the renal anomaly was considered as an incidental finding and not related to the splenic cyst. All fetuses were delivered at term; however, information on the type of delivery was available only in eight cases and all but one woman delivered vaginally. In the case delivered by Cesarean section, the indication was unrelated to the presence of the cyst. The physical examinations and clinical courses of the neonates were normal in all cases. Neonatal abdominal ultrasound and subsequent pediatric sonographic follow up was available in all but one case. The splenic cyst was confirmed in all these eight cases and subsequent sonographic examinations showed that the cyst increased in size in two (25%), was similar size in one (12%), decreased in size in one (12%), and completely regressed in the remaining four cases (50%). No complications associated to the cysts were reported in the infants.
Left parasagittal view of the fetal abdomen and thorax shows two fetal splenic cysts (c) measuring 16 mm and 14 mm at 36 weeks. The cysts were first detected at 30 weeks, at which time they measured 12 mm and 10 mm, respectively. Fetal single splenic cyst (between calipers) measuring 17 × 15 mm at 30 weeks, containing a single septation (arrow).

Prenatal detection of congenital splenic cyst: Review of the literature
MA: maternal age; GA: gestational age; NS: not stated; MRI: magnetic resonance imaging; CAT: computerized axial tomography; SGA: small for gestational age.
Diagnosis of associated anomaly missed prenatally.
Information obtained from the corresponding author.
Discussion
The prenatal diagnosis of a congenital splenic cyst was first reported by Lichman and Miller 5 in 1988. In their report, a 13 mm cyst was detected in the left upper abdominal quadrant at 33 weeks of gestation. Although the splenic cyst was confirmed postnatally by ultrasound and no changes in size or shape were noted, information on long-term follow up was not available. A case with a large splenic cyst measuring 45 mm in diameter detected at 32 weeks was reported the following year. 6 As the mass was seen to be distinct from the left kidney, the diagnoses of mesenteric cyst and extraparenchymal renal cyst were considered. A laparotomy with partial splenectomy was performed postnatally due to the increasing size of the cyst to 70 mm and displacement of the neighboring organs. Histopathological examination of the surgical specimen revealed an epidermoid splenic cyst. 6
Subsequent reports, however, have demonstrated that the vast majority of fetal splenic cysts are relatively small and have a benign course in the fetus, remaining stable, decreasing in size, or even completely regressing at subsequent postnatal sonographic examinations. Indeed, Garel and Hassan 10 reported that all three of their prenatally detected cases resulted in resolution of the cyst, an observation that has been subsequently repeated by several other authors (Table 2). Of note, all but one 6 of the infants described in the antenatal literature have remained clinically asymptomatic without any complications attributable to the cyst.
From the histopathologic point of view, splenic cysts are classified into two main categories, true cysts and pseudocysts, depending on the presence or absence of an epithelial lining, respectively. Pseudocysts are secondary to trauma or infection and occur mainly in adults. 26 True cysts, in contrast, are seen more frequently in children and are subclassified into parasitic or nonparasitic forms. 27 Congenital splenic cysts are categorized as a nonparasitic subtype, which includes epidermoid, dermoid, polycystic, or mesothelial (simple) cysts. 27 The pathophysiology of congenital splenic cysts is believed to be due to the involution of pluripotent cells in the splenic parenchyma with subsequent squamous metaplasia. 9 However, confirmation of this theory is difficult to prove since all but one 6 of the reported prenatal cases lack pathological examination of the splenic lesion. The reason for spontaneous resolution noted in some of the prenatally diagnosed cases remains unknown.
Congenital splenic cysts are rare although no accurate figures on their true incidence are available in the literature. This is supported by our review of the literature in which only 32 cases detected prenatally have been reported. 5 – 25 However, as this has been mainly a third-trimester finding, and third-trimester ultrasound examination is not typically performed in the low-risk pregnant population, many cases of fetal splenic cyst likely remain undetected. Until now, the largest prenatal series was reported by Cuillier et al. 21 who described four cases, followed by Garel and Hassan 10 and by Dankovcik et al., 22 describing three cases each. Our series reports an even larger experience, although it was based on the recollection of cases from three referral centers for prenatal diagnosis in addition to those obtained from a dedicated website for prenatal diagnosis. Fetal splenic cysts generally manifest on ultrasound when the axial view of the fetal abdomen is obtained for abdominal circumference measurements. Our earliest diagnosis of splenic cyst was indeed diagnosed in that manner at 22 weeks. In cases in which there was doubt about the exact location, the diagnosis of a splenic cyst was confirmed at referral. Technical factors such as quality of the ultrasound machine, fetal position, maternal habitus, and experience of the operator at the initial scan as well as the rarity of this condition may account for the inability to make the exact diagnosis at initial presentation.
Fetal splenic cyst is typically an isolated finding; however, in one of our cases there was a left megaureter and in other reported cases a cleft lip and palate 9 and talipes 12 were diagnosed postnatally, for an overall 7% (3 of 41) rate of associated anomalies. Although these anomalies seems to be unrelated to the splenic cyst, it is nevertheless recommended that a detailed fetal anatomic survey should be performed whenever this anomaly is found to examine other organs to rule out other anomalies including systemic microcystic disease. After the prenatal identification of a suspected congenital splenic cyst, it is important that a differential diagnosis is formed because the prenatal diagnosis of other masses of the left upper abdominal quadrant may have different managements and prognoses. 4 Differential diagnoses include cystic anomalies involving the upper abdomen such as duodenal obstruction, suprarenal cystic mass, intraabdominal cystic pulmonary sequestration, and polycystic kidney. 4 Therefore, every effort to identify the precise location of the cyst should be performed. Although the use of fetal magnetic resonance imaging has been reported in two cases,20,21 it seems that the current resolution of modern ultrasound equipment is sufficiently accurate to determine the exact location, size, and appearance of the cystic mass.
Based on the available literature and our own experience, there is consensus that congenital splenic cyst is not anticipated to be associated with perinatal complications, although sporadic cases of rupture and hemorrhage have been reported in children and adults.10,27 Once detected prenatally, the parents should be counseled about the typically benign nature of this congenital anomaly, although prenatal follow up should be performed to confirm its exact location and monitor the size of the cyst. Postpartum ultrasound and pediatric follow up should be considered in order to rule out significant growth of the cyst in the postnatal period.
Footnotes
Acknowledgements
This work was supported by an unrestricted research grant to W.S. from the Sociedad Profesional de Medicina Fetal “Fetalmed”, Chile. The authors are indebted to Dr Fabrice Cuillier, Dr Alexandra Benachi, Dr Gabriele Tonni and Dr Juan De Leon-Luis for helping in the collection and translation of important part of the non-English literature. We are also grateful to Dr Daniel Flores and Dr Sergio de la Fuente for their help with the collection of data. Special thanks to Dr Philippe Jeanty for his enormous contribution to prenatal diagnosis through his website
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Declaration of Conflicting Interests
The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Funding
The author(s) disclosed receipt of the following financial support for the research, authorship, and/or publication of this article: Unrestricted research grant to WS from Sociedad Profesinal de Medicina Fetal “Fetalmed” Ltda, Chile.
Ethical approval
Waived by IRB.
Guarantor
WS
Contributors
WS wrote the first draft of the manuscript and reviewed the literature. WS and AEW analyzed patient data and wrote the final version. WS, JHO, DC, FB, EA, and EYA contributed with case reports. All authors reviewed and approved the final version of the manuscript.
