CASE REPORT |
https://doi.org/10.5005/jp-journals-10033-1631 |
Wandering Accessory Spleen: Laparoscopic Approach for an Extremely Rare Condition: A Case Report
1–8Department of General Surgery, S. Elia Hospital, Caltanissetta, Italy
Corresponding Author: Paolo Locurto, Department of General Surgery, S. Elia Hospital, Caltanissetta, Italy, Phone: +3408462527, e-mail: paolo.locurto1986@gmail.com
How to cite this article: Locurto P, Fazzotta S, Farulla MA, et al. Wandering Accessory Spleen: Laparoscopic Approach for an Extremely Rare Condition: A Case Report. World J Lap Surg 2024;17(3):185–188.
Source of support: Nil
Conflict of interest: None
Patient consent statement: The author(s) have obtained written informed consent from the patient for publication of the case report details and related images.
Received on: 17 March 2024; Accepted on: 10 April 2024; Published on: 16 August 2024
ABSTRACT
Background: Wandering accessory spleen (WAS) is a very rare but dangerous condition. Patients are often asymptomatic and the diagnosis can be accidental. An early diagnosis and a correct treatment are fundamental.
Case presentation: A young woman with renal disorders underwent laparoscopic surgery after sudden abdominal pain. Radiological exams show a wandering abdominal mass located in different abdominal areas.
Discussion: Wandering accessory spleen is often asymptomatic. A torsion on its vascular axis leads to emergency surgery. Laparoscopy is the gold standard treatment in the correct management of this rare condition. Definitive diagnosis is based on histological exams.
Keywords: Case report, Laparoscopic surgery, Wandering accessory spleen.
INTRODUCTION
Wandering accessory spleen (WAS) is an extremely rare anatomical anomaly characterized by presence of an accessory spleen with a long vascular pedicle and a normal spleen located in abdomen.1 Although accessory spleen is often asymptomatic in general population and incidentally diagnosed during radiological exams, WAS might be symptomatic and abdominal pain, related to intermittent or acute torsion, can be the onset symptom.2,3
Frequently patients suffer of hematological or renal diseases. Wandering accessory spleen may also mimic abdominal tumors or mass like lymphadenopathy, abscess, organized hematoma or cysts and an accurate diagnosis is necessary.
The US, CT, and MRI usually show a mass with a different localization and a surgical exploration is often necessary. Laparoscopy represents the gold standard in the surgical management of this rare clinical condition and definitive diagnosis is based on histopathological analysis.4
CASE PRESENTATION
A 19-year-old young woman was admitted at the emergency room of our hospital following sudden upper abdominal pain and hypotension.
The patient referred in the previous 2 months a first admission to the Emergency Department due to a sudden onset of nephrotic syndrome with lower limbs edema and contraction of diuresis. At that time, laboratory exams showed proteinuria and hypoalbuminemia; renal biopsy was performed with a subsequently diagnosis of membranous glomerulonephritis and she started therapy with rituximab.
Physical examination on readmission revealed abdominal tenderness mainly in the right flank and upper abdomen with mild peritoneal signs of rebound and guarding. A palpable mass was appreciated in the periumbilical area. Abdominal bloating was remarkable and peristalsis was torpid on auscultation. The patient was pale and very suffering.
Laboratory exams showed a reduction of hemoglobin (10.2 mg/dL), neutrophilic leukocytosis, a slight reduction in albumin values, and an increase in CRP (45.38 mg/L). There was no procalcitonin movement and creatinine was normal.
Contrast-enhanced CT scan showed presence of an oval mass of approximately 6 × 4.5 cm in correspondence of the mesentery, close to the left rectus abdominis muscle (Fig. 1A). The expansive mass showed significant enhancement in the arterial and portal phases and slow wash-out in the late one. The angiographic study showed a vascular supply from mesenteric vessels and the presence of tributary venous circles. Contrast-enhanced CT scan excluded areas of vascular disorders or intestinal ischemia, signs of bowel occlusion or perforation and other diseases of abdominal organs.
Figs 1A and B: (A) Contrast-enhanced CT scan shows WAS in left abdomen; (B) MRI shows WAS in right abdomen
Since the patient’s condition was stable, it was necessary performed an MRI of the upper abdomen to clarify any doubts about the nature of the mass.
Abdominal MRI showed the presence of a solid oval mass of approximately 6.9 × 4.8 × 5.3 cm with regular margins which, compared with the CT scan performed a few hours earlier, was located in the right upper abdomen, in front of the right kidney and close the right rectus abdominis muscle. The MRI showed also the presence of coarse vascular pedicle with abnormal venous tributary circles and a modest free fluid in the Douglas (Fig. 1B).
An emergency explorative laparoscopy was necessary in the hypothesis of an extremely mobile (Fig. 1) and bleeding abdominal mass.
Therefore, an open technique pneumoperitoneum was performed. The exploration of the peritoneal cavity showed a voluminous oval brownish mass of about 5 cm in diameter close the transverse mesocolon. There were no signs of hemoperitoneum but minimal free effusion of dark fluid was present and it was taken for cytological examination. The mass was released from adhesions up to its vascular peduncle which appeared congested by transient and reversible torsion phenomena on its own axis. The pedicle was sectioned with a vascular EndoGIA and then the mass was extracted with an endobag. Control of hemostasis and placement of an abdominal drain concluded the phases of the surgical procedure (Fig. 2).
Figs 2A to D: Laparoscopic procedure: Release of the adhesion and vascular pedicle isolation
The postoperative hospitalization was uneventful. The drain was removed on the second postoperative day and the patient was discharged 4 days after surgery.
Gross examination of specimen revealed a pedunculated nodular mass, brownish-grey colored, large about 5 cm in diameter with own capsule.
Microscopic examination on paraffin embedded sections revealed splenic parenchyma type architecture with thickened fibrous capsule and congested dilated vessels with a sinusoid appearance, follicular formations with diffuse central reactive hyperplasia phenomena and minute arterioles with thickened walls and sometimes fibrotic (penicillary-like arteries).
Hemorrhagic-congestive phenomena were observed with dilatation of sinusoidal vessels and the presence of fibro-sclerosis and sclero-hyalinosis was the expression of chronic ischemic phenomena due the probable torsion of the peduncle. Congo Red stains were negative for amyloid detection. The diagnosis of WAS was made (Fig. 3).
Fig. 3: Histological section shows splenic parenchyma architecture with the presence of red and white pulp and reticular connective tissue
The presence of proteinaceous and fibrinoid material on the arteriolar walls with some concentric sheaths of fibrous tissue like “onion bulb” and sclero-hyalinosis phenomena are also described as a consequence of vascular damage of an autoimmune genesis in patients with chronic nephrotic syndromes or membranous-type glomerulonephritis.
DISCUSSION
The WAS is a rare condition with an estimated incidence of less than 0.5% in general population.5 Two different peaks of prevalence have been described: One in women aged 20–40 years, more frequent, and a second one in children aged less than 10 years.6,7
An important feature of WAS is its hypermobility, caused by the presence of a long vascular pedicle or, more rarely, by a laxity defect of its suspensory ligaments. It can be caused by congenital defects in embryonic development of the dorsal mesogastrium or by acquired defects, such as abdominal wall laxity or hormonal status during pregnancy.8
The extreme mobility gives this rare anatomical condition, is the characteristic of being able to migrate in the abdomen with a possible different localization during radiological exams. Due this characteristic, literature has coined the term of wandering spleen.9,10
Most often WAS remains asymptomatic and is discovered incidentally. Ultrasonography, preferably used with children, but mainly CT and MRI suggest diagnosis. Presence of a spleen in its normal anatomical position in the left hypochondrium and the presence of a mass located somewhere in the abdominal cavity are suspicious signs of WAS.11,12
Spontaneous torsion of WAS on its pedicle can take place. If the torsion occurs suddenly, it can determine an acute abdomen of non-univocal interpretation, as in the case described and, therefore, imposes a correct diagnostic classification through a multidisciplinary evaluation and an exact differential diagnosis (bowel obstruction, perforation, bleeding, or ischemia).1,13,14
Minimally invasive surgery represents the gold standard treatment and laparoscopy allows to resolve the diagnostic doubt by safe and reproducible exploration of the peritoneal cavity and in the same time offers a therapeutic intervention. A recent literature analysis shows as dimensions of the WAS can lead to a laparotomic surgical approach.15
Definitive diagnosis is possible only on histological analysis with a necessary integration of anamnestic, laboratory and radiological information.
CONCLUSION
The WAS is an ectopic accessory spleen characterized by marked anatomical mobility resulting from a defect in the normal embryological development. The WAS is a rare condition that can arise as an acute abdomen and diagnosis may be challenging and often difficult. Laparoscopy is the gold standard treatment.
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